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eMedicine Specialties gt General Surgery gt Abdomen
Inferior Vena Caval ThrombosisAuthor Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS AssistantClinical Professor of Surgery and Family Practice University of Texas Health Science CenterAdjunct Clinical Professor of Medicine and Nursing University of Texas Arlington ChairmanDivision of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care
Unit Trinity Mother Francis Health System Brigadier General Texas Medical RangersTXSGMBCoauthor(s) Douglas M Geehan MD Associate Professor Department of Surgery Universityof Missouri at Kansas CityContributor Information and Disclosures
Updated Dec 29 2008
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IntroductionThrombosis of the inferior vena cava (IVC) is an underrecognized entity with a variety of clinical presentations The general concepts of deep venous thrombosis (DVT) andthrombophlebitis are discussed in detail in Deep Venous Thrombosis and Thrombophlebitis
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However the implications and complexity of inferior vena caval thrombosis (IVCT) meritspecific attention
From a global standpoint IVCT represents a subset of DVT Virchow recognized and describedthe factors predisposing a patient to venous thrombosis The triad of stasis vessel injury andhypercoagulability formulated by Virchow remain the foundation for our understanding of the
pathophysiology of DVT in general and for IVCT in particular (see image below)
Virchows triadvenous thromboembolism (VTE) risk factors
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Virchows triadvenous thromboembolism (VTE) risk factors
As appreciation of the impact of these factors on the patient has improved therapy has becomemore directed
Problem
Understanding the anatomy of the IVC and its tributaries is essential to understanding thevariability in the clinical presentations of patients with IVCT The IVC is formed by theconfluence of the left and right common iliac veins Numerous paired segmental lumbar veins
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drain into the IVC throughout its length The right gonadal vein empties directly into the cavawhile the left gonadal vein generally empties into the left renal vein The azygous system hasconnections with the IVC or the renal veins at the level of the renal veins The next major veinsencountered are the renal veins followed by the hepatic veins No valves are within the IVCThe cava enters the thoracic cavity through the tendonous portion of the diaphragm andterminates at its junction with the right atrium
Several congenital anomalies of venous anatomy can involve the IVC and their presence canincrease the likelihood of IVCT The symptomatology related to IVCT follows directly from theanatomic location of the thrombus and the degree of the lumen occupied by the thrombus
Frequency
The exact number of patients who have IVCT remains elusive because of the clinical variabilityin presentation By compiling information from several epidemiologic studies that investigatedDVT prevalence the following estimates can be generated
bull The DVT rate in the United States is 60-180 cases per 100000 population per year
bull The frequency of IVCT in patients with DVT is 4-15
bull In the United States 165000-493000 cases of DVT occur each year
bull In the United States 6600-74000 cases of IVCT occur each year
These numbers are estimates generated from various population-based studies Various groupswithin the general population have a greater propensity for IVCT as discussed in Etiology
Etiology
To a large degree the etiology of IVCT mirrors that of DVT in general However specificsituations relate to the IVC only but the wide variety of these situations all relate in one or moreways to Virchows classic description
Tumors
Numerous malignancies have been associated with IVCT Perhaps the most familiar is renal cellcarcinoma The intravascular tumor extends from the renal vein and can propagate as far as theheart The tumor can partially or completely occlude the IVC Not all intravascular irregularitiesof the kidney represent tumor thrombus One case has been reported of a patient who underwentradical nephrectomy for presumed renal cell carcinoma and was subsequently found to have onlyrenal vein thrombosis Other genitourinary tumors that reportedly cause IVCT includeseminomas and teratomas
Numerous other less common tumors reportedly involve the IVC Intuitively any structure thatis anatomically related to the IVC can generate either direct compression or vascular invasionRetroperitoneal leiomyosarcoma adrenal cortical carcinoma and renal angiomyolipoma have all been reported as presenting in association with IVCT Even hepatic hemangioma has caused
IVCT from extrinsic compression Additionally malignancy itself is a risk factor for DVT andthus represents a risk factor for the extension of DVT into the IVC
Compression
Extrinsic compression may also result from nontumoral sources and increase the likelihood of IVCT The distortion of the normal caval anatomy generates both venous stasis and turbulentflow This situation facilitates the formation of thrombus An activity as innocuous as bicycle
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riding has reportedly caused IVCT The spectrum of medical diagnoses that can causecompression of the IVC is determined by those structures anatomically adjacent to the IVC
Aneurysms of the abdominal aorta can compress the vena cava and cause thrombosis Althoughthis clinical situation is somewhat uncommon the implications for surgical repair of theaneurysm are significant The surgeon must be prepared for enlarged venous collaterals and the
possibility of unusual configurations of the tissue planes One reported case describedincorporation of the IVC into the aneurysm In this particular case the wall of the IVC wasactually part of the wall of the aneurysm Knowing that abdominal aortic aneurysm is a risk factor for IVCT should heighten clinical suspicion in appropriate cases
Hepatic abscesses either from amebae or echinococci can also generate thrombosis of the IVCfrom compression Because of the propensity of these processes to evolve over time patientsmay present without symptoms suggestive of IVC occlusion They may only demonstrateevidence of the primary process or of collateral venous hypertrophy The initial presentingsymptom may even be pulmonary embolization
Other retroperitoneal organ systems that have been shown to cause IVCT include the pancreasand the kidneys Polycystic disease of the right kidney has reportedly been clinically associated
with thrombosis of the IVC Pancreatic pseudocysts have been observed to cause thrombosis of the IVC Acute pancreatitis has also been found to generate thrombosis of the IVC The pathophysiology of the evolution of the thrombosis may reflect either the local impact of inflammation of the pancreatic head or the impact of a hypercoagulable state on the IVCAlthough IVCT in the setting of pancreatitis is uncommon this clinical entity may account for anunexplained deterioration in the status of a patient with acute pancreatitis
Hematomatrauma
Other aspects of compression can be attributed to the presence of a hematoma adjacent to thecava or the iliac systems Psoas hematomas and other hematomas of the retroperitoneum have been identified as causing IVCT In one case the hematoma was the result of a common iliacartery injury Because the venous system was not involved the presumed mechanism of compression of the cava by clot seems credible
Unique among causes trauma combines the limbs of the Virchow triad Stasis vessel injury andhypercoagulability may all exist in the same clinical situation Direct trauma to the IVC may bethe result of either penetrating or blunt trauma In the absence of venous laceration bluntendothelial damage has been postulated to cause IVCT Other mechanisms observed secondaryto trauma include extension of hepatic venous thrombosis and thrombus formation after perihepatic packing
Dysfunctional coagulation system
By necessity and function the balance between the coagulation system and the fibrinolyticsystem is delicate and dynamic Disorders that disrupt this balance can cause a situation in which
IVC thrombus formation may occur The nephrotic syndrome is a classic example Patients withthis syndrome have urinary protein losses Both renal vein thrombosis and IVCT have beendescribed The exact mechanism of the hypercoagulability of patients with the nephroticsyndrome has not been fully delineated However these patients have massive urinary proteinloss and diminished levels of antithrombin III have been observed
Iatrogenic
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Patients with a recent history of medical care may present with iatrogenic IVCT The expansionof endovascular technology has led to increased recognition of iatrogenic IVCT Interventionsthat reportedly have identifiable rates of IVCT include (1) hepatic transplantation (2) dialysisaccess (3) femoral venous catheters (4) pacemaker wires and (5) vena caval filters
Awareness of the association of these procedures with IVCT allows clinicians to make educated
decisions Recognizing the association allows an accurate risk-benefit assessment for a given procedure Additionally recognizing these factors may aid in determining a prompt diagnosis in patients who have postprocedural clinical changes
Other
Numerous other clinical situations have been associated with IVCT They may meet someclassification criteria to be listed in one or more of the categories listed above however they arelisted here for clarity and can include (1) developmental anomalies of the IVC (2)retroperitoneal fibrosis (3) pregnancy and (4) oral contraceptives
Although not all-inclusive the foregoing information provides a review of many of the knownclinical situations in which IVCT may be evident Knowledge of the potential for thrombosis of the IVC increases physicians level of clinical awareness in patients who present with theidentified primary processes
Congenital absence of inferior vena cava
Iqbal and Nagaraju reported their experience with a case of congenitally absent inferior venacava (IVC)1 This is an extremely rare anomaly that is associated with idiopathic deep veinthrombosis (DVT) particularly in the young
Symptoms associated with severe venous hypertension (eg bilateral lower extremity edemavaricose veins nonhealing venous ulcers caput medusae other manifestations of collateralvenous system hypertensiondilatation) may be varied in their manifestation and in some cases
may not be apparent until later in life
Retrospectively as Iqbal and Nagaraju have discussed in this case there can be clues indicatingthe presence of such an anomaly from a young age The issue of whether early recognition of thiscondition would affect the prognosis and the treatment in many of these patients still remains indoubt
Case presentation
A 54-year-old man was admitted with 3 weeks of abdominal pain and localized swelling over theright flank Examination revealed palpable ldquosnake-likerdquo tortuous tender lumps on the abdominalwall with overlying bruising He was noted to have bilateral lower limb varicose veins
See image below
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
He was a nonsmoker and there was no significant family history of disease He had no upper or lower gastrointestinal symptoms There was no change in weight or appetite There was nohistory of cardiorespiratory disease and his exercise tolerance was not limited
He was not able to volunteer any further information in regard to his past medical history other than that he was under annual review by nephrologists for mild chronic renal impairment due toan atrophic left kidney This was diagnosed by ultrasound of the renal tract There was noevidence of any other imaging modalities or radiologic investigations undertaken to investigate
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the cause of his atrophic kidney
His past history revealed chronic nonhealing venous leg ulcers as well as varicose veinsnecessitating varicose vein ligation at a very young age The ulcers eventually needed skingrafting
During this current admission he was investigated and diagnosed with DVT
A CT scan performed to search for intra-abdominal cancer revealed the absence of the IVCwith extensive thrombosed collaterals of the superficial abdominal and azygous veins and acongenitally atrophic left kidney
See images below
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
Next Page raquo
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report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
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4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
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8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
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9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
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cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
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24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
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28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
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31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
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40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
[ CLOSE WINDOW ]
Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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eMedicine Specialties gt General Surgery gt Abdomen
Inferior Vena Caval ThrombosisAuthor Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS AssistantClinical Professor of Surgery and Family Practice University of Texas Health Science CenterAdjunct Clinical Professor of Medicine and Nursing University of Texas Arlington ChairmanDivision of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care
Unit Trinity Mother Francis Health System Brigadier General Texas Medical RangersTXSGMBCoauthor(s) Douglas M Geehan MD Associate Professor Department of Surgery Universityof Missouri at Kansas CityContributor Information and Disclosures
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IntroductionThrombosis of the inferior vena cava (IVC) is an underrecognized entity with a variety of clinical presentations The general concepts of deep venous thrombosis (DVT) andthrombophlebitis are discussed in detail in Deep Venous Thrombosis and Thrombophlebitis
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However the implications and complexity of inferior vena caval thrombosis (IVCT) meritspecific attention
From a global standpoint IVCT represents a subset of DVT Virchow recognized and describedthe factors predisposing a patient to venous thrombosis The triad of stasis vessel injury andhypercoagulability formulated by Virchow remain the foundation for our understanding of the
pathophysiology of DVT in general and for IVCT in particular (see image below)
Virchows triadvenous thromboembolism (VTE) risk factors
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Virchows triadvenous thromboembolism (VTE) risk factors
As appreciation of the impact of these factors on the patient has improved therapy has becomemore directed
Problem
Understanding the anatomy of the IVC and its tributaries is essential to understanding thevariability in the clinical presentations of patients with IVCT The IVC is formed by theconfluence of the left and right common iliac veins Numerous paired segmental lumbar veins
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drain into the IVC throughout its length The right gonadal vein empties directly into the cavawhile the left gonadal vein generally empties into the left renal vein The azygous system hasconnections with the IVC or the renal veins at the level of the renal veins The next major veinsencountered are the renal veins followed by the hepatic veins No valves are within the IVCThe cava enters the thoracic cavity through the tendonous portion of the diaphragm andterminates at its junction with the right atrium
Several congenital anomalies of venous anatomy can involve the IVC and their presence canincrease the likelihood of IVCT The symptomatology related to IVCT follows directly from theanatomic location of the thrombus and the degree of the lumen occupied by the thrombus
Frequency
The exact number of patients who have IVCT remains elusive because of the clinical variabilityin presentation By compiling information from several epidemiologic studies that investigatedDVT prevalence the following estimates can be generated
bull The DVT rate in the United States is 60-180 cases per 100000 population per year
bull The frequency of IVCT in patients with DVT is 4-15
bull In the United States 165000-493000 cases of DVT occur each year
bull In the United States 6600-74000 cases of IVCT occur each year
These numbers are estimates generated from various population-based studies Various groupswithin the general population have a greater propensity for IVCT as discussed in Etiology
Etiology
To a large degree the etiology of IVCT mirrors that of DVT in general However specificsituations relate to the IVC only but the wide variety of these situations all relate in one or moreways to Virchows classic description
Tumors
Numerous malignancies have been associated with IVCT Perhaps the most familiar is renal cellcarcinoma The intravascular tumor extends from the renal vein and can propagate as far as theheart The tumor can partially or completely occlude the IVC Not all intravascular irregularitiesof the kidney represent tumor thrombus One case has been reported of a patient who underwentradical nephrectomy for presumed renal cell carcinoma and was subsequently found to have onlyrenal vein thrombosis Other genitourinary tumors that reportedly cause IVCT includeseminomas and teratomas
Numerous other less common tumors reportedly involve the IVC Intuitively any structure thatis anatomically related to the IVC can generate either direct compression or vascular invasionRetroperitoneal leiomyosarcoma adrenal cortical carcinoma and renal angiomyolipoma have all been reported as presenting in association with IVCT Even hepatic hemangioma has caused
IVCT from extrinsic compression Additionally malignancy itself is a risk factor for DVT andthus represents a risk factor for the extension of DVT into the IVC
Compression
Extrinsic compression may also result from nontumoral sources and increase the likelihood of IVCT The distortion of the normal caval anatomy generates both venous stasis and turbulentflow This situation facilitates the formation of thrombus An activity as innocuous as bicycle
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riding has reportedly caused IVCT The spectrum of medical diagnoses that can causecompression of the IVC is determined by those structures anatomically adjacent to the IVC
Aneurysms of the abdominal aorta can compress the vena cava and cause thrombosis Althoughthis clinical situation is somewhat uncommon the implications for surgical repair of theaneurysm are significant The surgeon must be prepared for enlarged venous collaterals and the
possibility of unusual configurations of the tissue planes One reported case describedincorporation of the IVC into the aneurysm In this particular case the wall of the IVC wasactually part of the wall of the aneurysm Knowing that abdominal aortic aneurysm is a risk factor for IVCT should heighten clinical suspicion in appropriate cases
Hepatic abscesses either from amebae or echinococci can also generate thrombosis of the IVCfrom compression Because of the propensity of these processes to evolve over time patientsmay present without symptoms suggestive of IVC occlusion They may only demonstrateevidence of the primary process or of collateral venous hypertrophy The initial presentingsymptom may even be pulmonary embolization
Other retroperitoneal organ systems that have been shown to cause IVCT include the pancreasand the kidneys Polycystic disease of the right kidney has reportedly been clinically associated
with thrombosis of the IVC Pancreatic pseudocysts have been observed to cause thrombosis of the IVC Acute pancreatitis has also been found to generate thrombosis of the IVC The pathophysiology of the evolution of the thrombosis may reflect either the local impact of inflammation of the pancreatic head or the impact of a hypercoagulable state on the IVCAlthough IVCT in the setting of pancreatitis is uncommon this clinical entity may account for anunexplained deterioration in the status of a patient with acute pancreatitis
Hematomatrauma
Other aspects of compression can be attributed to the presence of a hematoma adjacent to thecava or the iliac systems Psoas hematomas and other hematomas of the retroperitoneum have been identified as causing IVCT In one case the hematoma was the result of a common iliacartery injury Because the venous system was not involved the presumed mechanism of compression of the cava by clot seems credible
Unique among causes trauma combines the limbs of the Virchow triad Stasis vessel injury andhypercoagulability may all exist in the same clinical situation Direct trauma to the IVC may bethe result of either penetrating or blunt trauma In the absence of venous laceration bluntendothelial damage has been postulated to cause IVCT Other mechanisms observed secondaryto trauma include extension of hepatic venous thrombosis and thrombus formation after perihepatic packing
Dysfunctional coagulation system
By necessity and function the balance between the coagulation system and the fibrinolyticsystem is delicate and dynamic Disorders that disrupt this balance can cause a situation in which
IVC thrombus formation may occur The nephrotic syndrome is a classic example Patients withthis syndrome have urinary protein losses Both renal vein thrombosis and IVCT have beendescribed The exact mechanism of the hypercoagulability of patients with the nephroticsyndrome has not been fully delineated However these patients have massive urinary proteinloss and diminished levels of antithrombin III have been observed
Iatrogenic
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Patients with a recent history of medical care may present with iatrogenic IVCT The expansionof endovascular technology has led to increased recognition of iatrogenic IVCT Interventionsthat reportedly have identifiable rates of IVCT include (1) hepatic transplantation (2) dialysisaccess (3) femoral venous catheters (4) pacemaker wires and (5) vena caval filters
Awareness of the association of these procedures with IVCT allows clinicians to make educated
decisions Recognizing the association allows an accurate risk-benefit assessment for a given procedure Additionally recognizing these factors may aid in determining a prompt diagnosis in patients who have postprocedural clinical changes
Other
Numerous other clinical situations have been associated with IVCT They may meet someclassification criteria to be listed in one or more of the categories listed above however they arelisted here for clarity and can include (1) developmental anomalies of the IVC (2)retroperitoneal fibrosis (3) pregnancy and (4) oral contraceptives
Although not all-inclusive the foregoing information provides a review of many of the knownclinical situations in which IVCT may be evident Knowledge of the potential for thrombosis of the IVC increases physicians level of clinical awareness in patients who present with theidentified primary processes
Congenital absence of inferior vena cava
Iqbal and Nagaraju reported their experience with a case of congenitally absent inferior venacava (IVC)1 This is an extremely rare anomaly that is associated with idiopathic deep veinthrombosis (DVT) particularly in the young
Symptoms associated with severe venous hypertension (eg bilateral lower extremity edemavaricose veins nonhealing venous ulcers caput medusae other manifestations of collateralvenous system hypertensiondilatation) may be varied in their manifestation and in some cases
may not be apparent until later in life
Retrospectively as Iqbal and Nagaraju have discussed in this case there can be clues indicatingthe presence of such an anomaly from a young age The issue of whether early recognition of thiscondition would affect the prognosis and the treatment in many of these patients still remains indoubt
Case presentation
A 54-year-old man was admitted with 3 weeks of abdominal pain and localized swelling over theright flank Examination revealed palpable ldquosnake-likerdquo tortuous tender lumps on the abdominalwall with overlying bruising He was noted to have bilateral lower limb varicose veins
See image below
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
He was a nonsmoker and there was no significant family history of disease He had no upper or lower gastrointestinal symptoms There was no change in weight or appetite There was nohistory of cardiorespiratory disease and his exercise tolerance was not limited
He was not able to volunteer any further information in regard to his past medical history other than that he was under annual review by nephrologists for mild chronic renal impairment due toan atrophic left kidney This was diagnosed by ultrasound of the renal tract There was noevidence of any other imaging modalities or radiologic investigations undertaken to investigate
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the cause of his atrophic kidney
His past history revealed chronic nonhealing venous leg ulcers as well as varicose veinsnecessitating varicose vein ligation at a very young age The ulcers eventually needed skingrafting
During this current admission he was investigated and diagnosed with DVT
A CT scan performed to search for intra-abdominal cancer revealed the absence of the IVCwith extensive thrombosed collaterals of the superficial abdominal and azygous veins and acongenitally atrophic left kidney
See images below
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
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9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
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24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
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Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
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Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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eMedicine Specialties gt General Surgery gt Abdomen
Inferior Vena Caval ThrombosisAuthor Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS AssistantClinical Professor of Surgery and Family Practice University of Texas Health Science CenterAdjunct Clinical Professor of Medicine and Nursing University of Texas Arlington ChairmanDivision of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care
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IntroductionThrombosis of the inferior vena cava (IVC) is an underrecognized entity with a variety of clinical presentations The general concepts of deep venous thrombosis (DVT) andthrombophlebitis are discussed in detail in Deep Venous Thrombosis and Thrombophlebitis
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However the implications and complexity of inferior vena caval thrombosis (IVCT) meritspecific attention
From a global standpoint IVCT represents a subset of DVT Virchow recognized and describedthe factors predisposing a patient to venous thrombosis The triad of stasis vessel injury andhypercoagulability formulated by Virchow remain the foundation for our understanding of the
pathophysiology of DVT in general and for IVCT in particular (see image below)
Virchows triadvenous thromboembolism (VTE) risk factors
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Virchows triadvenous thromboembolism (VTE) risk factors
As appreciation of the impact of these factors on the patient has improved therapy has becomemore directed
Problem
Understanding the anatomy of the IVC and its tributaries is essential to understanding thevariability in the clinical presentations of patients with IVCT The IVC is formed by theconfluence of the left and right common iliac veins Numerous paired segmental lumbar veins
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drain into the IVC throughout its length The right gonadal vein empties directly into the cavawhile the left gonadal vein generally empties into the left renal vein The azygous system hasconnections with the IVC or the renal veins at the level of the renal veins The next major veinsencountered are the renal veins followed by the hepatic veins No valves are within the IVCThe cava enters the thoracic cavity through the tendonous portion of the diaphragm andterminates at its junction with the right atrium
Several congenital anomalies of venous anatomy can involve the IVC and their presence canincrease the likelihood of IVCT The symptomatology related to IVCT follows directly from theanatomic location of the thrombus and the degree of the lumen occupied by the thrombus
Frequency
The exact number of patients who have IVCT remains elusive because of the clinical variabilityin presentation By compiling information from several epidemiologic studies that investigatedDVT prevalence the following estimates can be generated
bull The DVT rate in the United States is 60-180 cases per 100000 population per year
bull The frequency of IVCT in patients with DVT is 4-15
bull In the United States 165000-493000 cases of DVT occur each year
bull In the United States 6600-74000 cases of IVCT occur each year
These numbers are estimates generated from various population-based studies Various groupswithin the general population have a greater propensity for IVCT as discussed in Etiology
Etiology
To a large degree the etiology of IVCT mirrors that of DVT in general However specificsituations relate to the IVC only but the wide variety of these situations all relate in one or moreways to Virchows classic description
Tumors
Numerous malignancies have been associated with IVCT Perhaps the most familiar is renal cellcarcinoma The intravascular tumor extends from the renal vein and can propagate as far as theheart The tumor can partially or completely occlude the IVC Not all intravascular irregularitiesof the kidney represent tumor thrombus One case has been reported of a patient who underwentradical nephrectomy for presumed renal cell carcinoma and was subsequently found to have onlyrenal vein thrombosis Other genitourinary tumors that reportedly cause IVCT includeseminomas and teratomas
Numerous other less common tumors reportedly involve the IVC Intuitively any structure thatis anatomically related to the IVC can generate either direct compression or vascular invasionRetroperitoneal leiomyosarcoma adrenal cortical carcinoma and renal angiomyolipoma have all been reported as presenting in association with IVCT Even hepatic hemangioma has caused
IVCT from extrinsic compression Additionally malignancy itself is a risk factor for DVT andthus represents a risk factor for the extension of DVT into the IVC
Compression
Extrinsic compression may also result from nontumoral sources and increase the likelihood of IVCT The distortion of the normal caval anatomy generates both venous stasis and turbulentflow This situation facilitates the formation of thrombus An activity as innocuous as bicycle
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riding has reportedly caused IVCT The spectrum of medical diagnoses that can causecompression of the IVC is determined by those structures anatomically adjacent to the IVC
Aneurysms of the abdominal aorta can compress the vena cava and cause thrombosis Althoughthis clinical situation is somewhat uncommon the implications for surgical repair of theaneurysm are significant The surgeon must be prepared for enlarged venous collaterals and the
possibility of unusual configurations of the tissue planes One reported case describedincorporation of the IVC into the aneurysm In this particular case the wall of the IVC wasactually part of the wall of the aneurysm Knowing that abdominal aortic aneurysm is a risk factor for IVCT should heighten clinical suspicion in appropriate cases
Hepatic abscesses either from amebae or echinococci can also generate thrombosis of the IVCfrom compression Because of the propensity of these processes to evolve over time patientsmay present without symptoms suggestive of IVC occlusion They may only demonstrateevidence of the primary process or of collateral venous hypertrophy The initial presentingsymptom may even be pulmonary embolization
Other retroperitoneal organ systems that have been shown to cause IVCT include the pancreasand the kidneys Polycystic disease of the right kidney has reportedly been clinically associated
with thrombosis of the IVC Pancreatic pseudocysts have been observed to cause thrombosis of the IVC Acute pancreatitis has also been found to generate thrombosis of the IVC The pathophysiology of the evolution of the thrombosis may reflect either the local impact of inflammation of the pancreatic head or the impact of a hypercoagulable state on the IVCAlthough IVCT in the setting of pancreatitis is uncommon this clinical entity may account for anunexplained deterioration in the status of a patient with acute pancreatitis
Hematomatrauma
Other aspects of compression can be attributed to the presence of a hematoma adjacent to thecava or the iliac systems Psoas hematomas and other hematomas of the retroperitoneum have been identified as causing IVCT In one case the hematoma was the result of a common iliacartery injury Because the venous system was not involved the presumed mechanism of compression of the cava by clot seems credible
Unique among causes trauma combines the limbs of the Virchow triad Stasis vessel injury andhypercoagulability may all exist in the same clinical situation Direct trauma to the IVC may bethe result of either penetrating or blunt trauma In the absence of venous laceration bluntendothelial damage has been postulated to cause IVCT Other mechanisms observed secondaryto trauma include extension of hepatic venous thrombosis and thrombus formation after perihepatic packing
Dysfunctional coagulation system
By necessity and function the balance between the coagulation system and the fibrinolyticsystem is delicate and dynamic Disorders that disrupt this balance can cause a situation in which
IVC thrombus formation may occur The nephrotic syndrome is a classic example Patients withthis syndrome have urinary protein losses Both renal vein thrombosis and IVCT have beendescribed The exact mechanism of the hypercoagulability of patients with the nephroticsyndrome has not been fully delineated However these patients have massive urinary proteinloss and diminished levels of antithrombin III have been observed
Iatrogenic
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Patients with a recent history of medical care may present with iatrogenic IVCT The expansionof endovascular technology has led to increased recognition of iatrogenic IVCT Interventionsthat reportedly have identifiable rates of IVCT include (1) hepatic transplantation (2) dialysisaccess (3) femoral venous catheters (4) pacemaker wires and (5) vena caval filters
Awareness of the association of these procedures with IVCT allows clinicians to make educated
decisions Recognizing the association allows an accurate risk-benefit assessment for a given procedure Additionally recognizing these factors may aid in determining a prompt diagnosis in patients who have postprocedural clinical changes
Other
Numerous other clinical situations have been associated with IVCT They may meet someclassification criteria to be listed in one or more of the categories listed above however they arelisted here for clarity and can include (1) developmental anomalies of the IVC (2)retroperitoneal fibrosis (3) pregnancy and (4) oral contraceptives
Although not all-inclusive the foregoing information provides a review of many of the knownclinical situations in which IVCT may be evident Knowledge of the potential for thrombosis of the IVC increases physicians level of clinical awareness in patients who present with theidentified primary processes
Congenital absence of inferior vena cava
Iqbal and Nagaraju reported their experience with a case of congenitally absent inferior venacava (IVC)1 This is an extremely rare anomaly that is associated with idiopathic deep veinthrombosis (DVT) particularly in the young
Symptoms associated with severe venous hypertension (eg bilateral lower extremity edemavaricose veins nonhealing venous ulcers caput medusae other manifestations of collateralvenous system hypertensiondilatation) may be varied in their manifestation and in some cases
may not be apparent until later in life
Retrospectively as Iqbal and Nagaraju have discussed in this case there can be clues indicatingthe presence of such an anomaly from a young age The issue of whether early recognition of thiscondition would affect the prognosis and the treatment in many of these patients still remains indoubt
Case presentation
A 54-year-old man was admitted with 3 weeks of abdominal pain and localized swelling over theright flank Examination revealed palpable ldquosnake-likerdquo tortuous tender lumps on the abdominalwall with overlying bruising He was noted to have bilateral lower limb varicose veins
See image below
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
He was a nonsmoker and there was no significant family history of disease He had no upper or lower gastrointestinal symptoms There was no change in weight or appetite There was nohistory of cardiorespiratory disease and his exercise tolerance was not limited
He was not able to volunteer any further information in regard to his past medical history other than that he was under annual review by nephrologists for mild chronic renal impairment due toan atrophic left kidney This was diagnosed by ultrasound of the renal tract There was noevidence of any other imaging modalities or radiologic investigations undertaken to investigate
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the cause of his atrophic kidney
His past history revealed chronic nonhealing venous leg ulcers as well as varicose veinsnecessitating varicose vein ligation at a very young age The ulcers eventually needed skingrafting
During this current admission he was investigated and diagnosed with DVT
A CT scan performed to search for intra-abdominal cancer revealed the absence of the IVCwith extensive thrombosed collaterals of the superficial abdominal and azygous veins and acongenitally atrophic left kidney
See images below
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
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3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
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9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
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24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
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Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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However the implications and complexity of inferior vena caval thrombosis (IVCT) meritspecific attention
From a global standpoint IVCT represents a subset of DVT Virchow recognized and describedthe factors predisposing a patient to venous thrombosis The triad of stasis vessel injury andhypercoagulability formulated by Virchow remain the foundation for our understanding of the
pathophysiology of DVT in general and for IVCT in particular (see image below)
Virchows triadvenous thromboembolism (VTE) risk factors
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Virchows triadvenous thromboembolism (VTE) risk factors
As appreciation of the impact of these factors on the patient has improved therapy has becomemore directed
Problem
Understanding the anatomy of the IVC and its tributaries is essential to understanding thevariability in the clinical presentations of patients with IVCT The IVC is formed by theconfluence of the left and right common iliac veins Numerous paired segmental lumbar veins
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drain into the IVC throughout its length The right gonadal vein empties directly into the cavawhile the left gonadal vein generally empties into the left renal vein The azygous system hasconnections with the IVC or the renal veins at the level of the renal veins The next major veinsencountered are the renal veins followed by the hepatic veins No valves are within the IVCThe cava enters the thoracic cavity through the tendonous portion of the diaphragm andterminates at its junction with the right atrium
Several congenital anomalies of venous anatomy can involve the IVC and their presence canincrease the likelihood of IVCT The symptomatology related to IVCT follows directly from theanatomic location of the thrombus and the degree of the lumen occupied by the thrombus
Frequency
The exact number of patients who have IVCT remains elusive because of the clinical variabilityin presentation By compiling information from several epidemiologic studies that investigatedDVT prevalence the following estimates can be generated
bull The DVT rate in the United States is 60-180 cases per 100000 population per year
bull The frequency of IVCT in patients with DVT is 4-15
bull In the United States 165000-493000 cases of DVT occur each year
bull In the United States 6600-74000 cases of IVCT occur each year
These numbers are estimates generated from various population-based studies Various groupswithin the general population have a greater propensity for IVCT as discussed in Etiology
Etiology
To a large degree the etiology of IVCT mirrors that of DVT in general However specificsituations relate to the IVC only but the wide variety of these situations all relate in one or moreways to Virchows classic description
Tumors
Numerous malignancies have been associated with IVCT Perhaps the most familiar is renal cellcarcinoma The intravascular tumor extends from the renal vein and can propagate as far as theheart The tumor can partially or completely occlude the IVC Not all intravascular irregularitiesof the kidney represent tumor thrombus One case has been reported of a patient who underwentradical nephrectomy for presumed renal cell carcinoma and was subsequently found to have onlyrenal vein thrombosis Other genitourinary tumors that reportedly cause IVCT includeseminomas and teratomas
Numerous other less common tumors reportedly involve the IVC Intuitively any structure thatis anatomically related to the IVC can generate either direct compression or vascular invasionRetroperitoneal leiomyosarcoma adrenal cortical carcinoma and renal angiomyolipoma have all been reported as presenting in association with IVCT Even hepatic hemangioma has caused
IVCT from extrinsic compression Additionally malignancy itself is a risk factor for DVT andthus represents a risk factor for the extension of DVT into the IVC
Compression
Extrinsic compression may also result from nontumoral sources and increase the likelihood of IVCT The distortion of the normal caval anatomy generates both venous stasis and turbulentflow This situation facilitates the formation of thrombus An activity as innocuous as bicycle
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riding has reportedly caused IVCT The spectrum of medical diagnoses that can causecompression of the IVC is determined by those structures anatomically adjacent to the IVC
Aneurysms of the abdominal aorta can compress the vena cava and cause thrombosis Althoughthis clinical situation is somewhat uncommon the implications for surgical repair of theaneurysm are significant The surgeon must be prepared for enlarged venous collaterals and the
possibility of unusual configurations of the tissue planes One reported case describedincorporation of the IVC into the aneurysm In this particular case the wall of the IVC wasactually part of the wall of the aneurysm Knowing that abdominal aortic aneurysm is a risk factor for IVCT should heighten clinical suspicion in appropriate cases
Hepatic abscesses either from amebae or echinococci can also generate thrombosis of the IVCfrom compression Because of the propensity of these processes to evolve over time patientsmay present without symptoms suggestive of IVC occlusion They may only demonstrateevidence of the primary process or of collateral venous hypertrophy The initial presentingsymptom may even be pulmonary embolization
Other retroperitoneal organ systems that have been shown to cause IVCT include the pancreasand the kidneys Polycystic disease of the right kidney has reportedly been clinically associated
with thrombosis of the IVC Pancreatic pseudocysts have been observed to cause thrombosis of the IVC Acute pancreatitis has also been found to generate thrombosis of the IVC The pathophysiology of the evolution of the thrombosis may reflect either the local impact of inflammation of the pancreatic head or the impact of a hypercoagulable state on the IVCAlthough IVCT in the setting of pancreatitis is uncommon this clinical entity may account for anunexplained deterioration in the status of a patient with acute pancreatitis
Hematomatrauma
Other aspects of compression can be attributed to the presence of a hematoma adjacent to thecava or the iliac systems Psoas hematomas and other hematomas of the retroperitoneum have been identified as causing IVCT In one case the hematoma was the result of a common iliacartery injury Because the venous system was not involved the presumed mechanism of compression of the cava by clot seems credible
Unique among causes trauma combines the limbs of the Virchow triad Stasis vessel injury andhypercoagulability may all exist in the same clinical situation Direct trauma to the IVC may bethe result of either penetrating or blunt trauma In the absence of venous laceration bluntendothelial damage has been postulated to cause IVCT Other mechanisms observed secondaryto trauma include extension of hepatic venous thrombosis and thrombus formation after perihepatic packing
Dysfunctional coagulation system
By necessity and function the balance between the coagulation system and the fibrinolyticsystem is delicate and dynamic Disorders that disrupt this balance can cause a situation in which
IVC thrombus formation may occur The nephrotic syndrome is a classic example Patients withthis syndrome have urinary protein losses Both renal vein thrombosis and IVCT have beendescribed The exact mechanism of the hypercoagulability of patients with the nephroticsyndrome has not been fully delineated However these patients have massive urinary proteinloss and diminished levels of antithrombin III have been observed
Iatrogenic
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Patients with a recent history of medical care may present with iatrogenic IVCT The expansionof endovascular technology has led to increased recognition of iatrogenic IVCT Interventionsthat reportedly have identifiable rates of IVCT include (1) hepatic transplantation (2) dialysisaccess (3) femoral venous catheters (4) pacemaker wires and (5) vena caval filters
Awareness of the association of these procedures with IVCT allows clinicians to make educated
decisions Recognizing the association allows an accurate risk-benefit assessment for a given procedure Additionally recognizing these factors may aid in determining a prompt diagnosis in patients who have postprocedural clinical changes
Other
Numerous other clinical situations have been associated with IVCT They may meet someclassification criteria to be listed in one or more of the categories listed above however they arelisted here for clarity and can include (1) developmental anomalies of the IVC (2)retroperitoneal fibrosis (3) pregnancy and (4) oral contraceptives
Although not all-inclusive the foregoing information provides a review of many of the knownclinical situations in which IVCT may be evident Knowledge of the potential for thrombosis of the IVC increases physicians level of clinical awareness in patients who present with theidentified primary processes
Congenital absence of inferior vena cava
Iqbal and Nagaraju reported their experience with a case of congenitally absent inferior venacava (IVC)1 This is an extremely rare anomaly that is associated with idiopathic deep veinthrombosis (DVT) particularly in the young
Symptoms associated with severe venous hypertension (eg bilateral lower extremity edemavaricose veins nonhealing venous ulcers caput medusae other manifestations of collateralvenous system hypertensiondilatation) may be varied in their manifestation and in some cases
may not be apparent until later in life
Retrospectively as Iqbal and Nagaraju have discussed in this case there can be clues indicatingthe presence of such an anomaly from a young age The issue of whether early recognition of thiscondition would affect the prognosis and the treatment in many of these patients still remains indoubt
Case presentation
A 54-year-old man was admitted with 3 weeks of abdominal pain and localized swelling over theright flank Examination revealed palpable ldquosnake-likerdquo tortuous tender lumps on the abdominalwall with overlying bruising He was noted to have bilateral lower limb varicose veins
See image below
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
He was a nonsmoker and there was no significant family history of disease He had no upper or lower gastrointestinal symptoms There was no change in weight or appetite There was nohistory of cardiorespiratory disease and his exercise tolerance was not limited
He was not able to volunteer any further information in regard to his past medical history other than that he was under annual review by nephrologists for mild chronic renal impairment due toan atrophic left kidney This was diagnosed by ultrasound of the renal tract There was noevidence of any other imaging modalities or radiologic investigations undertaken to investigate
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the cause of his atrophic kidney
His past history revealed chronic nonhealing venous leg ulcers as well as varicose veinsnecessitating varicose vein ligation at a very young age The ulcers eventually needed skingrafting
During this current admission he was investigated and diagnosed with DVT
A CT scan performed to search for intra-abdominal cancer revealed the absence of the IVCwith extensive thrombosed collaterals of the superficial abdominal and azygous veins and acongenitally atrophic left kidney
See images below
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
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6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
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9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
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17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
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24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
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the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
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of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
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61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
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Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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Virchows triadvenous thromboembolism (VTE) risk factors
As appreciation of the impact of these factors on the patient has improved therapy has becomemore directed
Problem
Understanding the anatomy of the IVC and its tributaries is essential to understanding thevariability in the clinical presentations of patients with IVCT The IVC is formed by theconfluence of the left and right common iliac veins Numerous paired segmental lumbar veins
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drain into the IVC throughout its length The right gonadal vein empties directly into the cavawhile the left gonadal vein generally empties into the left renal vein The azygous system hasconnections with the IVC or the renal veins at the level of the renal veins The next major veinsencountered are the renal veins followed by the hepatic veins No valves are within the IVCThe cava enters the thoracic cavity through the tendonous portion of the diaphragm andterminates at its junction with the right atrium
Several congenital anomalies of venous anatomy can involve the IVC and their presence canincrease the likelihood of IVCT The symptomatology related to IVCT follows directly from theanatomic location of the thrombus and the degree of the lumen occupied by the thrombus
Frequency
The exact number of patients who have IVCT remains elusive because of the clinical variabilityin presentation By compiling information from several epidemiologic studies that investigatedDVT prevalence the following estimates can be generated
bull The DVT rate in the United States is 60-180 cases per 100000 population per year
bull The frequency of IVCT in patients with DVT is 4-15
bull In the United States 165000-493000 cases of DVT occur each year
bull In the United States 6600-74000 cases of IVCT occur each year
These numbers are estimates generated from various population-based studies Various groupswithin the general population have a greater propensity for IVCT as discussed in Etiology
Etiology
To a large degree the etiology of IVCT mirrors that of DVT in general However specificsituations relate to the IVC only but the wide variety of these situations all relate in one or moreways to Virchows classic description
Tumors
Numerous malignancies have been associated with IVCT Perhaps the most familiar is renal cellcarcinoma The intravascular tumor extends from the renal vein and can propagate as far as theheart The tumor can partially or completely occlude the IVC Not all intravascular irregularitiesof the kidney represent tumor thrombus One case has been reported of a patient who underwentradical nephrectomy for presumed renal cell carcinoma and was subsequently found to have onlyrenal vein thrombosis Other genitourinary tumors that reportedly cause IVCT includeseminomas and teratomas
Numerous other less common tumors reportedly involve the IVC Intuitively any structure thatis anatomically related to the IVC can generate either direct compression or vascular invasionRetroperitoneal leiomyosarcoma adrenal cortical carcinoma and renal angiomyolipoma have all been reported as presenting in association with IVCT Even hepatic hemangioma has caused
IVCT from extrinsic compression Additionally malignancy itself is a risk factor for DVT andthus represents a risk factor for the extension of DVT into the IVC
Compression
Extrinsic compression may also result from nontumoral sources and increase the likelihood of IVCT The distortion of the normal caval anatomy generates both venous stasis and turbulentflow This situation facilitates the formation of thrombus An activity as innocuous as bicycle
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riding has reportedly caused IVCT The spectrum of medical diagnoses that can causecompression of the IVC is determined by those structures anatomically adjacent to the IVC
Aneurysms of the abdominal aorta can compress the vena cava and cause thrombosis Althoughthis clinical situation is somewhat uncommon the implications for surgical repair of theaneurysm are significant The surgeon must be prepared for enlarged venous collaterals and the
possibility of unusual configurations of the tissue planes One reported case describedincorporation of the IVC into the aneurysm In this particular case the wall of the IVC wasactually part of the wall of the aneurysm Knowing that abdominal aortic aneurysm is a risk factor for IVCT should heighten clinical suspicion in appropriate cases
Hepatic abscesses either from amebae or echinococci can also generate thrombosis of the IVCfrom compression Because of the propensity of these processes to evolve over time patientsmay present without symptoms suggestive of IVC occlusion They may only demonstrateevidence of the primary process or of collateral venous hypertrophy The initial presentingsymptom may even be pulmonary embolization
Other retroperitoneal organ systems that have been shown to cause IVCT include the pancreasand the kidneys Polycystic disease of the right kidney has reportedly been clinically associated
with thrombosis of the IVC Pancreatic pseudocysts have been observed to cause thrombosis of the IVC Acute pancreatitis has also been found to generate thrombosis of the IVC The pathophysiology of the evolution of the thrombosis may reflect either the local impact of inflammation of the pancreatic head or the impact of a hypercoagulable state on the IVCAlthough IVCT in the setting of pancreatitis is uncommon this clinical entity may account for anunexplained deterioration in the status of a patient with acute pancreatitis
Hematomatrauma
Other aspects of compression can be attributed to the presence of a hematoma adjacent to thecava or the iliac systems Psoas hematomas and other hematomas of the retroperitoneum have been identified as causing IVCT In one case the hematoma was the result of a common iliacartery injury Because the venous system was not involved the presumed mechanism of compression of the cava by clot seems credible
Unique among causes trauma combines the limbs of the Virchow triad Stasis vessel injury andhypercoagulability may all exist in the same clinical situation Direct trauma to the IVC may bethe result of either penetrating or blunt trauma In the absence of venous laceration bluntendothelial damage has been postulated to cause IVCT Other mechanisms observed secondaryto trauma include extension of hepatic venous thrombosis and thrombus formation after perihepatic packing
Dysfunctional coagulation system
By necessity and function the balance between the coagulation system and the fibrinolyticsystem is delicate and dynamic Disorders that disrupt this balance can cause a situation in which
IVC thrombus formation may occur The nephrotic syndrome is a classic example Patients withthis syndrome have urinary protein losses Both renal vein thrombosis and IVCT have beendescribed The exact mechanism of the hypercoagulability of patients with the nephroticsyndrome has not been fully delineated However these patients have massive urinary proteinloss and diminished levels of antithrombin III have been observed
Iatrogenic
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Patients with a recent history of medical care may present with iatrogenic IVCT The expansionof endovascular technology has led to increased recognition of iatrogenic IVCT Interventionsthat reportedly have identifiable rates of IVCT include (1) hepatic transplantation (2) dialysisaccess (3) femoral venous catheters (4) pacemaker wires and (5) vena caval filters
Awareness of the association of these procedures with IVCT allows clinicians to make educated
decisions Recognizing the association allows an accurate risk-benefit assessment for a given procedure Additionally recognizing these factors may aid in determining a prompt diagnosis in patients who have postprocedural clinical changes
Other
Numerous other clinical situations have been associated with IVCT They may meet someclassification criteria to be listed in one or more of the categories listed above however they arelisted here for clarity and can include (1) developmental anomalies of the IVC (2)retroperitoneal fibrosis (3) pregnancy and (4) oral contraceptives
Although not all-inclusive the foregoing information provides a review of many of the knownclinical situations in which IVCT may be evident Knowledge of the potential for thrombosis of the IVC increases physicians level of clinical awareness in patients who present with theidentified primary processes
Congenital absence of inferior vena cava
Iqbal and Nagaraju reported their experience with a case of congenitally absent inferior venacava (IVC)1 This is an extremely rare anomaly that is associated with idiopathic deep veinthrombosis (DVT) particularly in the young
Symptoms associated with severe venous hypertension (eg bilateral lower extremity edemavaricose veins nonhealing venous ulcers caput medusae other manifestations of collateralvenous system hypertensiondilatation) may be varied in their manifestation and in some cases
may not be apparent until later in life
Retrospectively as Iqbal and Nagaraju have discussed in this case there can be clues indicatingthe presence of such an anomaly from a young age The issue of whether early recognition of thiscondition would affect the prognosis and the treatment in many of these patients still remains indoubt
Case presentation
A 54-year-old man was admitted with 3 weeks of abdominal pain and localized swelling over theright flank Examination revealed palpable ldquosnake-likerdquo tortuous tender lumps on the abdominalwall with overlying bruising He was noted to have bilateral lower limb varicose veins
See image below
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
He was a nonsmoker and there was no significant family history of disease He had no upper or lower gastrointestinal symptoms There was no change in weight or appetite There was nohistory of cardiorespiratory disease and his exercise tolerance was not limited
He was not able to volunteer any further information in regard to his past medical history other than that he was under annual review by nephrologists for mild chronic renal impairment due toan atrophic left kidney This was diagnosed by ultrasound of the renal tract There was noevidence of any other imaging modalities or radiologic investigations undertaken to investigate
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the cause of his atrophic kidney
His past history revealed chronic nonhealing venous leg ulcers as well as varicose veinsnecessitating varicose vein ligation at a very young age The ulcers eventually needed skingrafting
During this current admission he was investigated and diagnosed with DVT
A CT scan performed to search for intra-abdominal cancer revealed the absence of the IVCwith extensive thrombosed collaterals of the superficial abdominal and azygous veins and acongenitally atrophic left kidney
See images below
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
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59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
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Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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drain into the IVC throughout its length The right gonadal vein empties directly into the cavawhile the left gonadal vein generally empties into the left renal vein The azygous system hasconnections with the IVC or the renal veins at the level of the renal veins The next major veinsencountered are the renal veins followed by the hepatic veins No valves are within the IVCThe cava enters the thoracic cavity through the tendonous portion of the diaphragm andterminates at its junction with the right atrium
Several congenital anomalies of venous anatomy can involve the IVC and their presence canincrease the likelihood of IVCT The symptomatology related to IVCT follows directly from theanatomic location of the thrombus and the degree of the lumen occupied by the thrombus
Frequency
The exact number of patients who have IVCT remains elusive because of the clinical variabilityin presentation By compiling information from several epidemiologic studies that investigatedDVT prevalence the following estimates can be generated
bull The DVT rate in the United States is 60-180 cases per 100000 population per year
bull The frequency of IVCT in patients with DVT is 4-15
bull In the United States 165000-493000 cases of DVT occur each year
bull In the United States 6600-74000 cases of IVCT occur each year
These numbers are estimates generated from various population-based studies Various groupswithin the general population have a greater propensity for IVCT as discussed in Etiology
Etiology
To a large degree the etiology of IVCT mirrors that of DVT in general However specificsituations relate to the IVC only but the wide variety of these situations all relate in one or moreways to Virchows classic description
Tumors
Numerous malignancies have been associated with IVCT Perhaps the most familiar is renal cellcarcinoma The intravascular tumor extends from the renal vein and can propagate as far as theheart The tumor can partially or completely occlude the IVC Not all intravascular irregularitiesof the kidney represent tumor thrombus One case has been reported of a patient who underwentradical nephrectomy for presumed renal cell carcinoma and was subsequently found to have onlyrenal vein thrombosis Other genitourinary tumors that reportedly cause IVCT includeseminomas and teratomas
Numerous other less common tumors reportedly involve the IVC Intuitively any structure thatis anatomically related to the IVC can generate either direct compression or vascular invasionRetroperitoneal leiomyosarcoma adrenal cortical carcinoma and renal angiomyolipoma have all been reported as presenting in association with IVCT Even hepatic hemangioma has caused
IVCT from extrinsic compression Additionally malignancy itself is a risk factor for DVT andthus represents a risk factor for the extension of DVT into the IVC
Compression
Extrinsic compression may also result from nontumoral sources and increase the likelihood of IVCT The distortion of the normal caval anatomy generates both venous stasis and turbulentflow This situation facilitates the formation of thrombus An activity as innocuous as bicycle
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riding has reportedly caused IVCT The spectrum of medical diagnoses that can causecompression of the IVC is determined by those structures anatomically adjacent to the IVC
Aneurysms of the abdominal aorta can compress the vena cava and cause thrombosis Althoughthis clinical situation is somewhat uncommon the implications for surgical repair of theaneurysm are significant The surgeon must be prepared for enlarged venous collaterals and the
possibility of unusual configurations of the tissue planes One reported case describedincorporation of the IVC into the aneurysm In this particular case the wall of the IVC wasactually part of the wall of the aneurysm Knowing that abdominal aortic aneurysm is a risk factor for IVCT should heighten clinical suspicion in appropriate cases
Hepatic abscesses either from amebae or echinococci can also generate thrombosis of the IVCfrom compression Because of the propensity of these processes to evolve over time patientsmay present without symptoms suggestive of IVC occlusion They may only demonstrateevidence of the primary process or of collateral venous hypertrophy The initial presentingsymptom may even be pulmonary embolization
Other retroperitoneal organ systems that have been shown to cause IVCT include the pancreasand the kidneys Polycystic disease of the right kidney has reportedly been clinically associated
with thrombosis of the IVC Pancreatic pseudocysts have been observed to cause thrombosis of the IVC Acute pancreatitis has also been found to generate thrombosis of the IVC The pathophysiology of the evolution of the thrombosis may reflect either the local impact of inflammation of the pancreatic head or the impact of a hypercoagulable state on the IVCAlthough IVCT in the setting of pancreatitis is uncommon this clinical entity may account for anunexplained deterioration in the status of a patient with acute pancreatitis
Hematomatrauma
Other aspects of compression can be attributed to the presence of a hematoma adjacent to thecava or the iliac systems Psoas hematomas and other hematomas of the retroperitoneum have been identified as causing IVCT In one case the hematoma was the result of a common iliacartery injury Because the venous system was not involved the presumed mechanism of compression of the cava by clot seems credible
Unique among causes trauma combines the limbs of the Virchow triad Stasis vessel injury andhypercoagulability may all exist in the same clinical situation Direct trauma to the IVC may bethe result of either penetrating or blunt trauma In the absence of venous laceration bluntendothelial damage has been postulated to cause IVCT Other mechanisms observed secondaryto trauma include extension of hepatic venous thrombosis and thrombus formation after perihepatic packing
Dysfunctional coagulation system
By necessity and function the balance between the coagulation system and the fibrinolyticsystem is delicate and dynamic Disorders that disrupt this balance can cause a situation in which
IVC thrombus formation may occur The nephrotic syndrome is a classic example Patients withthis syndrome have urinary protein losses Both renal vein thrombosis and IVCT have beendescribed The exact mechanism of the hypercoagulability of patients with the nephroticsyndrome has not been fully delineated However these patients have massive urinary proteinloss and diminished levels of antithrombin III have been observed
Iatrogenic
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Patients with a recent history of medical care may present with iatrogenic IVCT The expansionof endovascular technology has led to increased recognition of iatrogenic IVCT Interventionsthat reportedly have identifiable rates of IVCT include (1) hepatic transplantation (2) dialysisaccess (3) femoral venous catheters (4) pacemaker wires and (5) vena caval filters
Awareness of the association of these procedures with IVCT allows clinicians to make educated
decisions Recognizing the association allows an accurate risk-benefit assessment for a given procedure Additionally recognizing these factors may aid in determining a prompt diagnosis in patients who have postprocedural clinical changes
Other
Numerous other clinical situations have been associated with IVCT They may meet someclassification criteria to be listed in one or more of the categories listed above however they arelisted here for clarity and can include (1) developmental anomalies of the IVC (2)retroperitoneal fibrosis (3) pregnancy and (4) oral contraceptives
Although not all-inclusive the foregoing information provides a review of many of the knownclinical situations in which IVCT may be evident Knowledge of the potential for thrombosis of the IVC increases physicians level of clinical awareness in patients who present with theidentified primary processes
Congenital absence of inferior vena cava
Iqbal and Nagaraju reported their experience with a case of congenitally absent inferior venacava (IVC)1 This is an extremely rare anomaly that is associated with idiopathic deep veinthrombosis (DVT) particularly in the young
Symptoms associated with severe venous hypertension (eg bilateral lower extremity edemavaricose veins nonhealing venous ulcers caput medusae other manifestations of collateralvenous system hypertensiondilatation) may be varied in their manifestation and in some cases
may not be apparent until later in life
Retrospectively as Iqbal and Nagaraju have discussed in this case there can be clues indicatingthe presence of such an anomaly from a young age The issue of whether early recognition of thiscondition would affect the prognosis and the treatment in many of these patients still remains indoubt
Case presentation
A 54-year-old man was admitted with 3 weeks of abdominal pain and localized swelling over theright flank Examination revealed palpable ldquosnake-likerdquo tortuous tender lumps on the abdominalwall with overlying bruising He was noted to have bilateral lower limb varicose veins
See image below
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
He was a nonsmoker and there was no significant family history of disease He had no upper or lower gastrointestinal symptoms There was no change in weight or appetite There was nohistory of cardiorespiratory disease and his exercise tolerance was not limited
He was not able to volunteer any further information in regard to his past medical history other than that he was under annual review by nephrologists for mild chronic renal impairment due toan atrophic left kidney This was diagnosed by ultrasound of the renal tract There was noevidence of any other imaging modalities or radiologic investigations undertaken to investigate
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the cause of his atrophic kidney
His past history revealed chronic nonhealing venous leg ulcers as well as varicose veinsnecessitating varicose vein ligation at a very young age The ulcers eventually needed skingrafting
During this current admission he was investigated and diagnosed with DVT
A CT scan performed to search for intra-abdominal cancer revealed the absence of the IVCwith extensive thrombosed collaterals of the superficial abdominal and azygous veins and acongenitally atrophic left kidney
See images below
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
Next Page raquo
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1824
9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
882019 Ivc Thrombosis
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24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
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Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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riding has reportedly caused IVCT The spectrum of medical diagnoses that can causecompression of the IVC is determined by those structures anatomically adjacent to the IVC
Aneurysms of the abdominal aorta can compress the vena cava and cause thrombosis Althoughthis clinical situation is somewhat uncommon the implications for surgical repair of theaneurysm are significant The surgeon must be prepared for enlarged venous collaterals and the
possibility of unusual configurations of the tissue planes One reported case describedincorporation of the IVC into the aneurysm In this particular case the wall of the IVC wasactually part of the wall of the aneurysm Knowing that abdominal aortic aneurysm is a risk factor for IVCT should heighten clinical suspicion in appropriate cases
Hepatic abscesses either from amebae or echinococci can also generate thrombosis of the IVCfrom compression Because of the propensity of these processes to evolve over time patientsmay present without symptoms suggestive of IVC occlusion They may only demonstrateevidence of the primary process or of collateral venous hypertrophy The initial presentingsymptom may even be pulmonary embolization
Other retroperitoneal organ systems that have been shown to cause IVCT include the pancreasand the kidneys Polycystic disease of the right kidney has reportedly been clinically associated
with thrombosis of the IVC Pancreatic pseudocysts have been observed to cause thrombosis of the IVC Acute pancreatitis has also been found to generate thrombosis of the IVC The pathophysiology of the evolution of the thrombosis may reflect either the local impact of inflammation of the pancreatic head or the impact of a hypercoagulable state on the IVCAlthough IVCT in the setting of pancreatitis is uncommon this clinical entity may account for anunexplained deterioration in the status of a patient with acute pancreatitis
Hematomatrauma
Other aspects of compression can be attributed to the presence of a hematoma adjacent to thecava or the iliac systems Psoas hematomas and other hematomas of the retroperitoneum have been identified as causing IVCT In one case the hematoma was the result of a common iliacartery injury Because the venous system was not involved the presumed mechanism of compression of the cava by clot seems credible
Unique among causes trauma combines the limbs of the Virchow triad Stasis vessel injury andhypercoagulability may all exist in the same clinical situation Direct trauma to the IVC may bethe result of either penetrating or blunt trauma In the absence of venous laceration bluntendothelial damage has been postulated to cause IVCT Other mechanisms observed secondaryto trauma include extension of hepatic venous thrombosis and thrombus formation after perihepatic packing
Dysfunctional coagulation system
By necessity and function the balance between the coagulation system and the fibrinolyticsystem is delicate and dynamic Disorders that disrupt this balance can cause a situation in which
IVC thrombus formation may occur The nephrotic syndrome is a classic example Patients withthis syndrome have urinary protein losses Both renal vein thrombosis and IVCT have beendescribed The exact mechanism of the hypercoagulability of patients with the nephroticsyndrome has not been fully delineated However these patients have massive urinary proteinloss and diminished levels of antithrombin III have been observed
Iatrogenic
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Patients with a recent history of medical care may present with iatrogenic IVCT The expansionof endovascular technology has led to increased recognition of iatrogenic IVCT Interventionsthat reportedly have identifiable rates of IVCT include (1) hepatic transplantation (2) dialysisaccess (3) femoral venous catheters (4) pacemaker wires and (5) vena caval filters
Awareness of the association of these procedures with IVCT allows clinicians to make educated
decisions Recognizing the association allows an accurate risk-benefit assessment for a given procedure Additionally recognizing these factors may aid in determining a prompt diagnosis in patients who have postprocedural clinical changes
Other
Numerous other clinical situations have been associated with IVCT They may meet someclassification criteria to be listed in one or more of the categories listed above however they arelisted here for clarity and can include (1) developmental anomalies of the IVC (2)retroperitoneal fibrosis (3) pregnancy and (4) oral contraceptives
Although not all-inclusive the foregoing information provides a review of many of the knownclinical situations in which IVCT may be evident Knowledge of the potential for thrombosis of the IVC increases physicians level of clinical awareness in patients who present with theidentified primary processes
Congenital absence of inferior vena cava
Iqbal and Nagaraju reported their experience with a case of congenitally absent inferior venacava (IVC)1 This is an extremely rare anomaly that is associated with idiopathic deep veinthrombosis (DVT) particularly in the young
Symptoms associated with severe venous hypertension (eg bilateral lower extremity edemavaricose veins nonhealing venous ulcers caput medusae other manifestations of collateralvenous system hypertensiondilatation) may be varied in their manifestation and in some cases
may not be apparent until later in life
Retrospectively as Iqbal and Nagaraju have discussed in this case there can be clues indicatingthe presence of such an anomaly from a young age The issue of whether early recognition of thiscondition would affect the prognosis and the treatment in many of these patients still remains indoubt
Case presentation
A 54-year-old man was admitted with 3 weeks of abdominal pain and localized swelling over theright flank Examination revealed palpable ldquosnake-likerdquo tortuous tender lumps on the abdominalwall with overlying bruising He was noted to have bilateral lower limb varicose veins
See image below
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
He was a nonsmoker and there was no significant family history of disease He had no upper or lower gastrointestinal symptoms There was no change in weight or appetite There was nohistory of cardiorespiratory disease and his exercise tolerance was not limited
He was not able to volunteer any further information in regard to his past medical history other than that he was under annual review by nephrologists for mild chronic renal impairment due toan atrophic left kidney This was diagnosed by ultrasound of the renal tract There was noevidence of any other imaging modalities or radiologic investigations undertaken to investigate
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the cause of his atrophic kidney
His past history revealed chronic nonhealing venous leg ulcers as well as varicose veinsnecessitating varicose vein ligation at a very young age The ulcers eventually needed skingrafting
During this current admission he was investigated and diagnosed with DVT
A CT scan performed to search for intra-abdominal cancer revealed the absence of the IVCwith extensive thrombosed collaterals of the superficial abdominal and azygous veins and acongenitally atrophic left kidney
See images below
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
Next Page raquo
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
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9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
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24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
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Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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Patients with a recent history of medical care may present with iatrogenic IVCT The expansionof endovascular technology has led to increased recognition of iatrogenic IVCT Interventionsthat reportedly have identifiable rates of IVCT include (1) hepatic transplantation (2) dialysisaccess (3) femoral venous catheters (4) pacemaker wires and (5) vena caval filters
Awareness of the association of these procedures with IVCT allows clinicians to make educated
decisions Recognizing the association allows an accurate risk-benefit assessment for a given procedure Additionally recognizing these factors may aid in determining a prompt diagnosis in patients who have postprocedural clinical changes
Other
Numerous other clinical situations have been associated with IVCT They may meet someclassification criteria to be listed in one or more of the categories listed above however they arelisted here for clarity and can include (1) developmental anomalies of the IVC (2)retroperitoneal fibrosis (3) pregnancy and (4) oral contraceptives
Although not all-inclusive the foregoing information provides a review of many of the knownclinical situations in which IVCT may be evident Knowledge of the potential for thrombosis of the IVC increases physicians level of clinical awareness in patients who present with theidentified primary processes
Congenital absence of inferior vena cava
Iqbal and Nagaraju reported their experience with a case of congenitally absent inferior venacava (IVC)1 This is an extremely rare anomaly that is associated with idiopathic deep veinthrombosis (DVT) particularly in the young
Symptoms associated with severe venous hypertension (eg bilateral lower extremity edemavaricose veins nonhealing venous ulcers caput medusae other manifestations of collateralvenous system hypertensiondilatation) may be varied in their manifestation and in some cases
may not be apparent until later in life
Retrospectively as Iqbal and Nagaraju have discussed in this case there can be clues indicatingthe presence of such an anomaly from a young age The issue of whether early recognition of thiscondition would affect the prognosis and the treatment in many of these patients still remains indoubt
Case presentation
A 54-year-old man was admitted with 3 weeks of abdominal pain and localized swelling over theright flank Examination revealed palpable ldquosnake-likerdquo tortuous tender lumps on the abdominalwall with overlying bruising He was noted to have bilateral lower limb varicose veins
See image below
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
[ CLOSE WINDOW ]
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
He was a nonsmoker and there was no significant family history of disease He had no upper or lower gastrointestinal symptoms There was no change in weight or appetite There was nohistory of cardiorespiratory disease and his exercise tolerance was not limited
He was not able to volunteer any further information in regard to his past medical history other than that he was under annual review by nephrologists for mild chronic renal impairment due toan atrophic left kidney This was diagnosed by ultrasound of the renal tract There was noevidence of any other imaging modalities or radiologic investigations undertaken to investigate
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the cause of his atrophic kidney
His past history revealed chronic nonhealing venous leg ulcers as well as varicose veinsnecessitating varicose vein ligation at a very young age The ulcers eventually needed skingrafting
During this current admission he was investigated and diagnosed with DVT
A CT scan performed to search for intra-abdominal cancer revealed the absence of the IVCwith extensive thrombosed collaterals of the superficial abdominal and azygous veins and acongenitally atrophic left kidney
See images below
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
[ CLOSE WINDOW ]
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
[ CLOSE WINDOW ]
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
Next Page raquo
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
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9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
882019 Ivc Thrombosis
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24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
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Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
[ CLOSE WINDOW ]
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
He was a nonsmoker and there was no significant family history of disease He had no upper or lower gastrointestinal symptoms There was no change in weight or appetite There was nohistory of cardiorespiratory disease and his exercise tolerance was not limited
He was not able to volunteer any further information in regard to his past medical history other than that he was under annual review by nephrologists for mild chronic renal impairment due toan atrophic left kidney This was diagnosed by ultrasound of the renal tract There was noevidence of any other imaging modalities or radiologic investigations undertaken to investigate
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the cause of his atrophic kidney
His past history revealed chronic nonhealing venous leg ulcers as well as varicose veinsnecessitating varicose vein ligation at a very young age The ulcers eventually needed skingrafting
During this current admission he was investigated and diagnosed with DVT
A CT scan performed to search for intra-abdominal cancer revealed the absence of the IVCwith extensive thrombosed collaterals of the superficial abdominal and azygous veins and acongenitally atrophic left kidney
See images below
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
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9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
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24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
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Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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the cause of his atrophic kidney
His past history revealed chronic nonhealing venous leg ulcers as well as varicose veinsnecessitating varicose vein ligation at a very young age The ulcers eventually needed skingrafting
During this current admission he was investigated and diagnosed with DVT
A CT scan performed to search for intra-abdominal cancer revealed the absence of the IVCwith extensive thrombosed collaterals of the superficial abdominal and azygous veins and acongenitally atrophic left kidney
See images below
Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
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9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
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24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
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Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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Photo showing dilated superficial abdominal veins (upper quadrant) with bruising andthrombosed large abdominal veins (lower quadrant)
Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
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9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
882019 Ivc Thrombosis
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24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
882019 Ivc Thrombosis
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
[ CLOSE WINDOW ]
Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2224
of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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Abdominal CT scan shows absent inferior vena cava with thrombosis of the veryprominent collateral veins in the abdominal wall corresponding to the right side of theabdomen as seen in the image above
On further review of his medical history it was revealed that he had been diagnosed as a childwith bilateral Perthes disease in addition to nonhealing venous ulcers on the medial aspect of hisright ankle
At the age of 21 years he underwent skin grafting of a nonhealing ulcer One year later he wasreadmitted with recurrence of ulcers in the same region and was then noted to have dilatedvaricose veins and thrombophlebitis that was treated with crepe bandaging for 2 yearsTreponemal serology then was negative In 1977 he had ligation of the varicose vein that wasfeeding the ulcerated part of the leg In 1979 he was discharged from follow-up with completehealing of the leg ulcers
He was commenced on low molecular weight heparin and warfarin Low molecular weightheparin was stopped when the international normalized ratio (INR) was greater than 2
Discussion
Absent IVC is uncommon Anomalies of the IVC have been described more frequently (06-2)
in those with other cardiovascular defects2
and less so in otherwise healthy individuals Variousabnormalities of the IVC have been described including complete absence partial absence or presence of bilateral IVC3
Controversy exists as to whether an absent IVC has a true embryonic etiology or whether it is theresult of perinatal IVC thrombosis causing regression and disappearance of the once presentIVC4
There has been one previous report in the literature of an absent IVC and left renal hypoplasiaand a right hypertrophic kidney5 A more common association recognized is right renal aplasiaas suggested in a review by Gayer et al where all 9 patients with complete absence of the IVChad an absent or very small right kidney6
The association of an absent or hypoplastic kidney is related (or may contribute to an absentIVC) due to perinatal renal vein thrombosis7 Veen and colleagues have proposed to name thiscondition KILT (Kidney and IVC abnormalities with Leg Thromboses) syndrome (whenassociated with DVT)5
It is estimated that DVT occurs 1 case per 1000 patient-years8 In up to 80 of patients who areaffected a risk factor can be identified Ruggeri et al presented 4 cases of absent IVC over a 5-year period presenting with idiopathic DVT in patients younger than 30 years9 This wasestimated to represent 5 of cases of idiopathic DVT in young people
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
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9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
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24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
[ CLOSE WINDOW ]
Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2224
of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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Chee et al similarly noted that up to 5 of 20- to 40-year-old patients presenting with DVT hadan IVC anomaly (4 in total of which 3 had a complete absence of IVC)10 This was much higher than the expected 05
The ideal imaging modality to help diagnose an IVC anomaly must have high diagnosticaccuracy and also be safe and reproducible It is difficult to establish a diagnosis of any IVC
anomaly by ultrasound Various clues are recognized on radiologic imaging that could helpdiagnose an absent IVC or anomaly One of the more common and helpful clues is welldeveloped and possibly dilated intrathoracic hemiazygous andor azygous continuations Thesecollateral circulations as well as other retroperitoneal venous pathways are usually welldeveloped before symptoms present11
The most reliable noninvasive methods to establish a diagnosis of IVC anomalies are CT withintravenous contrast or magnetic resonance scan CT scan unlike ultrasound is a good imagingmodality of the retroperitoneal space12 Another accurate but more invasive imaging modality isvenography which is particularly useful if any surgery is planned
It is hypothesized that blood return with an absent IVC is inadequate despite adequatecollaterals resulting in chronic venous hypertension in the lower extremities and causing venous
stasis that precipitates thrombosis
Gayer et al recommended that all patients with an IVC anomaly be screened for a thrombophilicdisorder13 In their series 7 of 9 patients with an IVC anomaly and DVT had a positivethrombophilic screen6
There have been 3 case reports in the English language medical literature of thromboembolismdue to an IVC anomaly (absence of the infrarenal portion of the IVC infrarenal IVC hypoplasia)In all of these cases the thrombophilic screen was negative141516 It was hypothesized thatmultiple emboli from DVT in the common and superficial femoral veins migrate through thewell-developed hemiazygous andor azygous system to the pulmonary circulation
There is very little evidence available on the surgical correction or the treatment of this
uncommon anomaly A case report of a complete absence of the IVC but patent iliac veins andnonhealing pretibial ulceration described successful treatment with a prosthetic graft from theiliac vein to the intrathoracic azygous vein7 Success was defined as complete healing of the ulcer up to 30 months after surgery
Conclusion
In conclusion this patient had an extensive past medical history of idiopathic varicose ulcerationwith evidence of chronic venous hypertension from a young age He was managed withdifficulty but achieved eventual healing of his ulcers as a young adult In later life he developedextensive DVT with worsening of his lower limb and abdominal varicosities
The very limited data from the literature suggest that in cases of an absent IVC in young people
(some data in patients younger than 30 years other data in patients aged 20ndash40 years) anabdominal CT scan should be performed
In this case with a relevant and extensive past history a review of the limited literature wouldsupport further radiologic investigations to exclude an intra-abdominal deep venous anomaly
The authors concluded that it is unlikely that surgical correction of the IVC was warranted in themanagement of this particular patient1 They also concluded that based on their review of theavailable literature surgical options in this patient population are limited1
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1524
Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
[ CLOSE WINDOW ]
882019 Ivc Thrombosis
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882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1724
Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1824
9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1924
24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
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57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
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Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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Their review revealed no consensus regarding the duration of anticoagulation however it would be reasonable for this particular patient to remain on lifelong anticoagulation given the ongoingrisk of further DVT and pulmonary embolism even if the thrombophilic screen is negative1
Knowledge of the association of other anomalies in patients with an absent IVC such as renal
atrophy or agenesis can highlight underlying vascular anomalies which are in and of themselvesof significant clinical importance
The clinician must have a profound awareness of the associated elements that make up theclinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls
Presentation
Patients with IVCT may present with a spectrum of signs and symptoms This variability is asignificant part of the challenge of diagnosis Using a classification system may help theclinician make the correct diagnosis Patients may present with symptoms that are predominantlythrombotic in origin or predominantly embolic in nature Additionally the thrombotic findings
are dependent on the degree of occlusion of the cava and on the location between the iliacconfluence and the right atrium
The classic presentation of IVCT includes bilateral lower extremity edema with dilated visiblesuperficial abdominal veins Intuitively this constellation makes sense although it is notuniversally found In one study almost 60 of patients did not have bilateral leg edema Inaddition if the thrombus is confined to the cava and does not involve the iliac or femoral systemthe collateral pathways form along the posterior abdominal wall This scenario may havesignificant impact on surgical procedures involving this anatomic region
Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinomaHowever more commonly thrombosis at this level suggests a nephrotic syndrome Occlusivethrombus of the IVC at the juxtarenal level can affect renal function by altering renal perfusion
Budd-Chiari syndrome merits specific attention A discussion of the entire syndrome is beyondthe scope of this article but the essentials as they relate to IVCT are important Patients typicallyhave significant ascites portal hypertension hepatomegaly collateral vein enlargement andhepatic fibrosis The pathophysiology of this syndrome centers on either IVC or hepatic venousthrombosis If at the hepatic venous level 2-3 of the major hepatic veins must be occluded beforethe syndrome can develop Both hypercoagulable states and membranous venous webs have been postulated as the etiologic agents of Budd-Chiari syndrome
Finally patients who have IVCT may present only after having a pulmonary embolism The lack of uniform symptoms and the significant number of asymptomatic patients contribute to thisfeature of IVCT In one retrospective review of all patients who had cavography to document
IVC thrombus 20 had angiographically proven pulmonary embolism with no symptoms of DVT Thus pulmonary embolism may be the first sign of IVCT
Relevant AnatomySee image below
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1524
Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
[ CLOSE WINDOW ]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1624
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1724
Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
Next Page raquo
bull Print This
bull Email This
[ CLOSE WINDOW ]
References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1824
9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1924
24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2024
41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2124
57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
[ CLOSE WINDOW ]
Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2224
of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2324
Academy of Sciences and Society for Surgery of the Alimentary Tract Disclosure AMGEN Royalty Other
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1824
9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1924
24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2024
41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2124
57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
[ CLOSE WINDOW ]
Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2224
of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2324
Academy of Sciences and Society for Surgery of the Alimentary Tract Disclosure AMGEN Royalty Other
Top of Form
medscapecme
Bottom of Form
bull Search for CMECE on This Topic raquo
RELATED EMEDICINE ARTICLESPatient Education
bull Leg Blood Clot Treatment
bull Circulatory Problems Center
bull Leg Blood Clot Overview
bull Leg Blood Clot Causes
bull Leg Blood Clot Symptoms
RELATED MEDSCAPE ARTICLESArticles
bull Venothrombotic Events Evidence-based Risk Assessment Prophylaxis Diagnosis andTreatment
Top of Form
Medscape Medscape CME eMedicine Drug Reference MEDLINE
All
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
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References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1824
9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1924
24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2024
41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2124
57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
[ CLOSE WINDOW ]
Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2224
of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
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We subscribe to theHONcode principles of theHealth On the Net Foundation
All material on this website is protected by copyright Copyrightcopy 1994-2010 by MedscapeThis website also contains material copyrighted by 3rd parties
DISCLAIMER The content of this Website is not influenced by sponsors The site is designed primarily for use by qualified physicians and other medical professionals The information
contained herein should NOT be used as a substitute for the advice of an appropriately qualifiedand licensed physician or other health care provider The information provided here is for educational and informational purposes only In no way should it be considered as offeringmedical advice Please check with a physician if you suspect you are ill
Close
882019 Ivc Thrombosis
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Veins of the abdomen and the thorax superior vena cava inferior vena cavabrachiocephalic veins and azygous veins This lithograph plate is from Grays AnatomyUnless stated otherwise it is from the online edition of the 20th US edition of GraysAnatomy of the Human Body originally published in 1918
More on Inferior Vena Caval Thrombosis
Overview Inferior Vena Caval ThrombosisWorkup Inferior Vena Caval Thrombosis
Treatment Inferior Vena Caval ThrombosisFollow-up Inferior Vena Caval Thrombosis
Multimedia Inferior Vena Caval ThrombosisReferences
Next Page raquo
bull Print This
bull Email This
[ CLOSE WINDOW ]
References1 Iqbal J Nagaraju E Congenital absence of inferior vena cava and thrombosis a case
report J Med Case Reports Feb 12 2008246 [Medline]
2 Anderson RC Adams P Jr Burke B Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava) Report of 15 newcases J Pediatr Sep 196159370-83 [Medline]
3 Bass JE Redwine MD Kramer LA et al Spectrum of congenital anomalies of theinferior vena cava cross-sectional imaging findings Radiographics May-Jun 200020(3)639-52 [Medline]
4 Ramanathan T Hughes TM Richardson AJ Perinatal inferior vena cava thrombosis andabsence of the infrarenal inferior vena cava J Vasc Surg May 200133(5)1097-9 [Medline]
5 Van Veen J Hampton KK Makris M Kilt syndrome Br J Haematol Sep 2002118(4)1199-200 [Medline]
6Gayer G Zissin R Strauss S et al IVC anomalies and right renal aplasia detected on CTa possible link Abdom Imaging May-Jun 200328(3)395-9 [Medline]
7 Dougherty MJ Calligaro KD DeLaurentis DA Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration a surgically treated case J Vasc Surg Jan 199623(1)141-6 [Medline]
8 White RH The epidemiology of venous thromboembolism Circulation Jun17 2003107(23 Suppl 1)I4-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1824
9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1924
24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2024
41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2124
57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
[ CLOSE WINDOW ]
Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2224
of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2324
Academy of Sciences and Society for Surgery of the Alimentary Tract Disclosure AMGEN Royalty Other
Top of Form
medscapecme
Bottom of Form
bull Search for CMECE on This Topic raquo
RELATED EMEDICINE ARTICLESPatient Education
bull Leg Blood Clot Treatment
bull Circulatory Problems Center
bull Leg Blood Clot Overview
bull Leg Blood Clot Causes
bull Leg Blood Clot Symptoms
RELATED MEDSCAPE ARTICLESArticles
bull Venothrombotic Events Evidence-based Risk Assessment Prophylaxis Diagnosis andTreatment
Top of Form
Medscape Medscape CME eMedicine Drug Reference MEDLINE
All
882019 Ivc Thrombosis
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Bottom of Form
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bull About Emedicine
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All material on this website is protected by copyright Copyrightcopy 1994-2010 by MedscapeThis website also contains material copyrighted by 3rd parties
DISCLAIMER The content of this Website is not influenced by sponsors The site is designed primarily for use by qualified physicians and other medical professionals The information
contained herein should NOT be used as a substitute for the advice of an appropriately qualifiedand licensed physician or other health care provider The information provided here is for educational and informational purposes only In no way should it be considered as offeringmedical advice Please check with a physician if you suspect you are ill
Close
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1824
9 Ruggeri M Tosetto A Castaman G et al Congenital absence of the inferior vena cava arare risk factor for idiopathic deep-vein thrombosis Lancet Feb10 2001357(9254)441 [Medline]
10Chee YL Culligan DJ Watson HG Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young Br J Haematol Sep 2001114(4)878-
80 [Medline]11Koc Z Oguzkurt L Interruption or congenital stenosis of the inferior vena cava
prevalence imaging and clinical findings Eur J Radiol May 200762(2)257-66 [Medline]
12Ueda J Hara K Kobayashi Y et al Anomaly of the inferior vena cava observed byCT Comput Radiol May-Jun 19837(3)145-54 [Medline]
13Gayer G Luboshitz J Hertz M et al Congenital anomalies of the inferior vena cavarevealed on CT in patients with deep vein thrombosis AJR Am J Roentgenol Mar 2003180(3)729-32 [Medline]
14Cho BC Choi HJ Kang SM et al Congenital absence of inferior vena cava as a rare
cause of pulmonary thromboembolism Yonsei Med J Oct 31 200445(5)947-51 [Medline]
15DAloia A Faggiano P Fiorina C et al Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism Int J Cardiol Apr 200388(2-3)327-9 [Medline]
16Takehara N Hasebe N Enomoto S et al Multiple and recurrent systemic thromboticevents associated with congenital anomaly of inferior vena cava J ThrombThrombolysis Apr 200519(2)101-3 [Medline]
17Anderson FA Jr Wheeler HB Goldberg RJ et al A population-based perspective of thehospital incidence and case-fatality rates of deep vein thrombosis and pulmonaryembolism The Worcester DVT Study Arch Intern Med May 1991151(5)933-8 [Medline]
18Angle JF Matsumoto AH Al Shammari M et al Transcatheter regional urokinasetherapy in the management of inferior vena cava thrombosis J Vasc Interv Radiol Nov-Dec 19989(6)917-25 [Medline]
19Aswad MA Sandager GP Pais SO et al Early duplex scan evaluation of four vena cavalinterruption devices J Vasc Surg Nov 199624(5)809-18 [Medline]
20Banjo AO Comparative study of the distribution of venous valves in the lower extremities of black Africans and Caucasians pathogenetic correlates of prevalence of primary varicose veins in the two races Anat Rec Apr 1987217(4)407-12 [Medline]
21Campbell DN Liechty RD Rutherford RB Traumatic thrombosis of the inferior venacava J Trauma May 198121(5)413-5 [Medline]
22Carter CJ The natural history and epidemiology of venous thrombosis Prog Cardiovasc Dis May-Jun 199436(6)423-38 [Medline]
23Chang TC Zaleski GX Lin BH et al Treatment of inferior vena cava obstruction inhemodialysis patients using Wallstents early and intermediate results AJR Am J Roentgenol Jul 1998171(1)125-8 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1924
24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2024
41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2124
57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
[ CLOSE WINDOW ]
Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2224
of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2324
Academy of Sciences and Society for Surgery of the Alimentary Tract Disclosure AMGEN Royalty Other
Top of Form
medscapecme
Bottom of Form
bull Search for CMECE on This Topic raquo
RELATED EMEDICINE ARTICLESPatient Education
bull Leg Blood Clot Treatment
bull Circulatory Problems Center
bull Leg Blood Clot Overview
bull Leg Blood Clot Causes
bull Leg Blood Clot Symptoms
RELATED MEDSCAPE ARTICLESArticles
bull Venothrombotic Events Evidence-based Risk Assessment Prophylaxis Diagnosis andTreatment
Top of Form
Medscape Medscape CME eMedicine Drug Reference MEDLINE
All
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2424
Bottom of Form
ltscript language=JavaScript12 type=textjavascript charset=ISO-8859-1src=httpasmedscapecomjsngParamsrichmedia=yesampamptransactionID=25776703ampampsite=1ampampaffiliate=2ampampssp=14ampampartid=10032718ampampenv=0ampamptile=48204791ampampcg=ckbampamppub=230ampamppubs=230ampampct=0ampamppf=0ampampusp=0ampampst=0ampam pocc=0ampamptid=ampamppos=141gtltscriptgt
bull About Emedicine
bull Privacy Policy
bull Terms of Use
bull Help
bull Contact Us
bull Institutional Subscribers
bull Contributor Login
We subscribe to theHONcode principles of theHealth On the Net Foundation
All material on this website is protected by copyright Copyrightcopy 1994-2010 by MedscapeThis website also contains material copyrighted by 3rd parties
DISCLAIMER The content of this Website is not influenced by sponsors The site is designed primarily for use by qualified physicians and other medical professionals The information
contained herein should NOT be used as a substitute for the advice of an appropriately qualifiedand licensed physician or other health care provider The information provided here is for educational and informational purposes only In no way should it be considered as offeringmedical advice Please check with a physician if you suspect you are ill
Close
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 1924
24Farber SP ODonnell TF Jr Deterling RA et al The clinical implications of acutethrombosis of the inferior vena cava Surg Gynecol Obstet Feb 1984158(2)141-4 [Medline]
25Figueroa AJ Stein JP Lieskovsky G et al Adrenal cortical carcinoma associated withvenous tumour thrombus extension Br J Urol Sep 199780(3)397-400 [Medline]
26Fleiner-Hoffmann AF Pfammatter T Leu AJ et al Alveolar echinococcosis of the liversequelae of chronic inferior vena cava obstructions in the hepatic segment Arch InternMed Dec 7-21 1998158(22)2503-8 [Medline]
27Flynn P Zammit-Maempel I Case report computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm Clin Radiol Apr 199853(4)306-7 [Medline]
28Fox RH Turner MJ Gardner AM Pseudothrombosis of the infra-renal IVC duringhelical CT--what causes this pitfall Clin Radiol Oct 199651(10)741 [Medline]
29Girard P Hauuy MP Musset D et al Acute inferior vena cava thrombosis Early resultsof heparin therapy Chest Feb 198995(2)284-91 [Medline]
30Gotoh A Gohji K Fujisawa M et al Renal angiomyolipoma associated with inferior vena caval tumour thrombus Br J Urol May 199881(5)773-4 [Medline]
31Gouge SF Paulson WD Moore J Jr Inferior vena cava thrombosis due to an indwellinghemodialysis catheter Am J Kidney Dis Jun 198811(6)515-8 [Medline]
32Hartman DS Hayes WS Choyke PL et al From the archives of the AFIPLeiomyosarcoma of the retroperitoneum and inferior vena cava radiologic-pathologiccorrelation Radiographics Nov 199212(6)1203-20 [Medline]
33Hausler M Duque D Merz U et al The clinical outcome after inferior vena cavathrombosis in early infancy Eur J Pediatr May 1999158(5)416-20 [Medline]
34Jackson BT Thomas ML Post-thrombotic inferior vena caval obstruction A review of
24 patients Br Med J Jan 3 19701(687)18-22 [Medline]
35Jones AL Ojar D Redhead D et al Case report Use of an IVC filter in the managementof IVC thrombosis occurring as a complication of acute pancreatitis Clin Radiol Jun 199853(6)462-4 [Medline]
36Kouroukis C Leclerc JR Pulmonary embolism with duplicated inferior venacava Chest Apr 1996109(4)1111-3 [Medline]
37Krafte-Jacobs B Sivit CJ Mejia R et al Catheter-related thrombosis in critically illchildren comparison of catheters with and without heparin bonding J Pediatr Jan 1995126(1)50-4 [Medline]
38Kwok CK Horowitz MD Livingstone AS et al Mature testicular teratoma with vena
caval invasion presenting as pulmonary embolism J Urol Jan 1993149(1)129-31 [Medline]
39Lam KK Lui CC Successful treatment of acute inferior vena cava and unilateral renalvein thrombosis by local infusion of recombinant tissue plasminogen activator Am J Kidney Dis Dec 199832(6)1075-9 [Medline]
40Lerut J Gordon RD Iwatsuki S et al Surgical complications in human orthotopic liver transplantation Acta Chir Belg May-Jun 198787(3)193-204 [Medline]
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2024
41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2124
57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
[ CLOSE WINDOW ]
Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2224
of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2324
Academy of Sciences and Society for Surgery of the Alimentary Tract Disclosure AMGEN Royalty Other
Top of Form
medscapecme
Bottom of Form
bull Search for CMECE on This Topic raquo
RELATED EMEDICINE ARTICLESPatient Education
bull Leg Blood Clot Treatment
bull Circulatory Problems Center
bull Leg Blood Clot Overview
bull Leg Blood Clot Causes
bull Leg Blood Clot Symptoms
RELATED MEDSCAPE ARTICLESArticles
bull Venothrombotic Events Evidence-based Risk Assessment Prophylaxis Diagnosis andTreatment
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DISCLAIMER The content of this Website is not influenced by sponsors The site is designed primarily for use by qualified physicians and other medical professionals The information
contained herein should NOT be used as a substitute for the advice of an appropriately qualifiedand licensed physician or other health care provider The information provided here is for educational and informational purposes only In no way should it be considered as offeringmedical advice Please check with a physician if you suspect you are ill
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882019 Ivc Thrombosis
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41Mathew CV Shanabo A Zyka I et al Retroperitoneal fibrosis with large-vesselobstruction An uncommon vascular disorder Acta Chir Scand 1985151(5)475-80 [Medline]
42McWilliams RG Chalmers AG Pseudothrombosis of the infra-renal inferior vena cavaduring helical CT Clin Radiol Nov 199550(11)751-5 [Medline]
43Mulvihill SJ Fonkalsrud EW Complications of superior versus inferior vena cavaocclusion in infants receiving central total parenteral nutrition J Pediatr Surg Dec 198419(6)752-7 [Medline]
44 Nagy KK Duarte B Post-traumatic inferior vena caval thrombosis case report J Trauma Feb 199030(2)218-21 [Medline]
45 Nesbitt JC Soltero ER Dinney CP et al Surgical management of renal cell carcinomawith inferior vena cava tumor thrombus Ann Thorac Surg Jun 199763(6)1592-600 [Medline]
46OBrien WM Lynch JH Thrombosis of the inferior vena cava by seminoma J Urol Feb 1987137(2)303-5 [Medline]
47OSullivan DA Torres VE Heit JA Liggett S King BF Compression of the inferior vena cava by right renal cysts an unusual cause of IVC andor iliofemoral thrombosiswith pulmonary embolism in autosomal dominant polycystic kidney disease Clin Nephrol May 199849(5)332-4 [Medline]
48Palmer MA Inferior vena cava occlusion secondary to aortic aneurysm J CardiovascSurg (Torino) May-Jun 199031(3)372-4 [Medline]
49Paolillo V Sicuro M Nejrotti A et al Pulmonary embolism due to compression of theinferior vena cava by a hepatic hemangioma Tex Heart Inst J 199320(1)66-8 [Medline]
50Park JH Chung JW Han JK et al Interventional management of benign obstruction of
the hepatic inferior vena cava J Vasc Interv Radiol May-Jun 19945(3)403-9 [Medline]
51Park JH Lee JB Han MC et al Sonographic evaluation of inferior vena cavalobstruction correlative study with vena cavography AJR Am J Roentgenol Oct 1985145(4)757-62 [Medline]
52Peck KE Bonoan JT Cunha BA Postlaparoscopic traumatic inferior vena cavalthrombosis Heart Lung Jul-Aug 199827(4)279-82 [Medline]
53Peillon C Manouvrier JL Testart J Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst Am J Gastroenterol Dec 199186(12)1854-6 [Medline]
54Perhoniemi V Salmenkivi K Vorne M Venous haemodynamics in the legs after ligation
of the inferior vena cava Acta Chir Scand Jan 198615223-7 [Medline]55Petersen BD Uchida BT Long-term results of treatment of benign central venous
obstructions unrelated to dialysis with expandable Z stents J Vasc Interv Radiol Jun 199910(6)757-66 [Medline]
56Schreiber D Deep Venous Thrombosis and Thrombophlebitis eMedicine Journal [serialonline] Available at httpemedicinemedscapecomarticle758140-overview Accessed2008
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2124
57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
[ CLOSE WINDOW ]
Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2224
of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2324
Academy of Sciences and Society for Surgery of the Alimentary Tract Disclosure AMGEN Royalty Other
Top of Form
medscapecme
Bottom of Form
bull Search for CMECE on This Topic raquo
RELATED EMEDICINE ARTICLESPatient Education
bull Leg Blood Clot Treatment
bull Circulatory Problems Center
bull Leg Blood Clot Overview
bull Leg Blood Clot Causes
bull Leg Blood Clot Symptoms
RELATED MEDSCAPE ARTICLESArticles
bull Venothrombotic Events Evidence-based Risk Assessment Prophylaxis Diagnosis andTreatment
Top of Form
Medscape Medscape CME eMedicine Drug Reference MEDLINE
All
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2424
Bottom of Form
ltscript language=JavaScript12 type=textjavascript charset=ISO-8859-1src=httpasmedscapecomjsngParamsrichmedia=yesampamptransactionID=25776703ampampsite=1ampampaffiliate=2ampampssp=14ampampartid=10032718ampampenv=0ampamptile=48204791ampampcg=ckbampamppub=230ampamppubs=230ampampct=0ampamppf=0ampampusp=0ampampst=0ampam pocc=0ampamptid=ampamppos=141gtltscriptgt
bull About Emedicine
bull Privacy Policy
bull Terms of Use
bull Help
bull Contact Us
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bull Contributor Login
We subscribe to theHONcode principles of theHealth On the Net Foundation
All material on this website is protected by copyright Copyrightcopy 1994-2010 by MedscapeThis website also contains material copyrighted by 3rd parties
DISCLAIMER The content of this Website is not influenced by sponsors The site is designed primarily for use by qualified physicians and other medical professionals The information
contained herein should NOT be used as a substitute for the advice of an appropriately qualifiedand licensed physician or other health care provider The information provided here is for educational and informational purposes only In no way should it be considered as offeringmedical advice Please check with a physician if you suspect you are ill
Close
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2124
57Shefler A Gillis J Lam A et al Inferior vena cava thrombosis as a complication of femoral vein catheterisation Arch Dis Child Apr 199572(4)343-5 [Medline]
58Simpson AH Kilby JO Inferior vena cava thrombosis following a cycle ride J R SocMed Dec 198881(12)738-9 [Medline]
59Smith L Hamill J Metcalf R et al Caval thrombectomy for severe staphylococcal
osteomyelitis J Pediatr Surg Jan 199732(1)112-4 [Medline]
60Soler R Rodriguez E Lopez MF et al MR imaging in inferior vena cavathrombosis Eur J Radiol Jan 199519(2)101-7 [Medline]
61Storti S Pagano L Marra R et al Urokinase and AT-III concentrate treatment in inferior vena cava thrombosis associated with nephrotic syndrome Blood Coagul Fibrinolysis Dec 19901(6)743-5 [Medline]
62Stringer MD Michell M McIrvine AJ Inferior vena caval thrombosis complicating acute pancreatitis Case report Acta Chir Scand Feb 1988154(2)161-3 [Medline]
63Takayama H Kinouchi T Meguro N et al Renal vein thrombosis misdiagnosed as arenal cell carcinoma with a tumor thrombus in the inferior vena cava Int J
Urol Jan 19985(1)94-5 [Medline]
64Takeuchi M Maruyama K Nakamura M et al Posttraumatic inferior vena cavalthrombosis case report and review of the literature J Trauma Sep 199539(3)605-8 [Medline]
65Tien YC Yang CW Ng KK et al Thrombosis of the inferior vena cava in a pregnantwoman with nephrotic syndrome--diagnostic and therapeutic dilemma Nephrol Dial Transplant Jan 199914(1)210-3 [Medline]
66Toumbouras M Spanos P Konstantaras C et al Inferior vena cava thrombosis due tomigration of retained functionless pacemaker electrode Chest Dec 198282(6)785-6 [Medline]
67Valla D Benhamou JP Obstruction of the hepatic veins or suprahepatic inferior venacava Dig Dis Mar-Apr 199614(2)99-118 [Medline]
[ CLOSE WINDOW ]
Further Reading[ CLOSE WINDOW ]
Keywordsinferior vena caval thrombosis IVC thrombosis IVCT deep venous thrombosis DVTthrombophlebitis renal cell carcinoma renal vein thrombosis RVT hepatic venous thrombosis
HVT Virchow triad Virchows triad Budd-Chiari syndrome[ CLOSE WINDOW ]
Contributor Information and DisclosuresAuthor
Luis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS Assistant ClinicalProfessor of Surgery and Family Practice University of Texas Health Science Center AdjunctClinical Professor of Medicine and Nursing University of Texas Arlington Chairman Division
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2224
of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2324
Academy of Sciences and Society for Surgery of the Alimentary Tract Disclosure AMGEN Royalty Other
Top of Form
medscapecme
Bottom of Form
bull Search for CMECE on This Topic raquo
RELATED EMEDICINE ARTICLESPatient Education
bull Leg Blood Clot Treatment
bull Circulatory Problems Center
bull Leg Blood Clot Overview
bull Leg Blood Clot Causes
bull Leg Blood Clot Symptoms
RELATED MEDSCAPE ARTICLESArticles
bull Venothrombotic Events Evidence-based Risk Assessment Prophylaxis Diagnosis andTreatment
Top of Form
Medscape Medscape CME eMedicine Drug Reference MEDLINE
All
882019 Ivc Thrombosis
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bull About Emedicine
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We subscribe to theHONcode principles of theHealth On the Net Foundation
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DISCLAIMER The content of this Website is not influenced by sponsors The site is designed primarily for use by qualified physicians and other medical professionals The information
contained herein should NOT be used as a substitute for the advice of an appropriately qualifiedand licensed physician or other health care provider The information provided here is for educational and informational purposes only In no way should it be considered as offeringmedical advice Please check with a physician if you suspect you are ill
Close
882019 Ivc Thrombosis
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of Trauma Surgery and Surgical Critical Care Chief of Trauma Surgical Critical Care UnitTrinity Mother Francis Health System Brigadier General Texas Medical Rangers TXSGMBLuis G Fernandez MD KHS FACS FASAS FCCP FCCM FICS is a member of thefollowing medical societies American Association for the Surgery of Trauma American College of Chest Physicians American College of Legal Medicine American College of Surgeons American Society of Abdominal Surgeons American Society of General Surgeons AmericanSociety of General Surgeons American Society of Law Medicine amp Ethics American TraumaSociety Association for Surgical Education Association of Military Surgeons of the USChicago Medical Society Illinois State Medical Society International College of Surgeons NewYork Academy of Sciences Pan American Trauma Society Society of Critical Care MedicineSociety of Laparoendoscopic Surgeons Southeastern Surgical Congress Texas MedicalAssociation and Undersea and Hyperbaric Medical Society Disclosure Nothing to disclose
Coauthor(s)
Douglas M Geehan MD Associate Professor Department of Surgery University of Missouriat Kansas City
Douglas M Geehan MD is a member of the following medical societies American College of Surgeons American Institute of Ultrasound in Medicine American Medical AssociationAssociation for Academic Surgery Phi Beta Kappa Society of American Gastrointestinal andEndoscopic Surgeons and Society of Critical Care Medicine Disclosure Nothing to disclose
Pharmacy Editor
Francisco Talavera PharmD PhD Senior Pharmacy Editor eMedicineDisclosure eMedicine Salary Employment
Managing Editor
Michael A Grosso MD Consulting Staff Department of Cardiothoracic Surgery St Francis
HospitalMichael A Grosso MD is a member of the following medical societies American College of Surgeons Society of Thoracic Surgeons and Society of University Surgeons Disclosure Nothing to disclose
CME Editor
Paolo Zamboni MD Professor of Surgery Chief of Day Surgery Unit Chair of Vascular Diseases Center University of Ferrara ItalyPaolo Zamboni MD is a member of the following medical societies American Venous Forum and New York Academy of Sciences Disclosure Nothing to disclose
Chief Editor
John Geibel MD DSc MA Vice Chairman Professor Department of Surgery Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology YaleUniversity School of Medicine Director of Surgical Research Department of Surgery Yale- New Haven HospitalJohn Geibel MD DSc MA is a member of the following medical societies AmericanGastroenterological Association American Physiological Society American Society of Nephrology Association for Academic Surgery International Society of Nephrology New York
882019 Ivc Thrombosis
httpslidepdfcomreaderfullivc-thrombosis 2324
Academy of Sciences and Society for Surgery of the Alimentary Tract Disclosure AMGEN Royalty Other
Top of Form
medscapecme
Bottom of Form
bull Search for CMECE on This Topic raquo
RELATED EMEDICINE ARTICLESPatient Education
bull Leg Blood Clot Treatment
bull Circulatory Problems Center
bull Leg Blood Clot Overview
bull Leg Blood Clot Causes
bull Leg Blood Clot Symptoms
RELATED MEDSCAPE ARTICLESArticles
bull Venothrombotic Events Evidence-based Risk Assessment Prophylaxis Diagnosis andTreatment
Top of Form
Medscape Medscape CME eMedicine Drug Reference MEDLINE
All
882019 Ivc Thrombosis
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bull About Emedicine
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We subscribe to theHONcode principles of theHealth On the Net Foundation
All material on this website is protected by copyright Copyrightcopy 1994-2010 by MedscapeThis website also contains material copyrighted by 3rd parties
DISCLAIMER The content of this Website is not influenced by sponsors The site is designed primarily for use by qualified physicians and other medical professionals The information
contained herein should NOT be used as a substitute for the advice of an appropriately qualifiedand licensed physician or other health care provider The information provided here is for educational and informational purposes only In no way should it be considered as offeringmedical advice Please check with a physician if you suspect you are ill
Close
882019 Ivc Thrombosis
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bull Search for CMECE on This Topic raquo
RELATED EMEDICINE ARTICLESPatient Education
bull Leg Blood Clot Treatment
bull Circulatory Problems Center
bull Leg Blood Clot Overview
bull Leg Blood Clot Causes
bull Leg Blood Clot Symptoms
RELATED MEDSCAPE ARTICLESArticles
bull Venothrombotic Events Evidence-based Risk Assessment Prophylaxis Diagnosis andTreatment
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bull About Emedicine
bull Privacy Policy
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bull Help
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We subscribe to theHONcode principles of theHealth On the Net Foundation
All material on this website is protected by copyright Copyrightcopy 1994-2010 by MedscapeThis website also contains material copyrighted by 3rd parties
DISCLAIMER The content of this Website is not influenced by sponsors The site is designed primarily for use by qualified physicians and other medical professionals The information
contained herein should NOT be used as a substitute for the advice of an appropriately qualifiedand licensed physician or other health care provider The information provided here is for educational and informational purposes only In no way should it be considered as offeringmedical advice Please check with a physician if you suspect you are ill
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bull Terms of Use
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DISCLAIMER The content of this Website is not influenced by sponsors The site is designed primarily for use by qualified physicians and other medical professionals The information
contained herein should NOT be used as a substitute for the advice of an appropriately qualifiedand licensed physician or other health care provider The information provided here is for educational and informational purposes only In no way should it be considered as offeringmedical advice Please check with a physician if you suspect you are ill
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