Laparoscopic management of celiac arterycompression syndromeGlen S. Roseborough, MD, FRCS(C), Baltimore, Md
Background: Celiac artery compression syndrome (CACS) remains a controversial diagnosis, despite several reportedseries documenting therapeutic efficacy of CA decompression. Traditional therapy consists of open surgical decompres-sion, but since 2000, five isolated case reports have been published in which CACS has been successfully treated withlaparoscopic techniques. This approach was adopted as the sole initial therapy for CACS at the Johns Hopkins Hospitalin 2002. This article reports the results of a unique surgical series that triples the reported worldwide experience with thistherapy.Methods: Fifteen patients (median age, 40.6 years) diagnosed with CACS underwent laparoscopic decompression by asingle vascular surgeon. CACS was diagnosed by digital subtraction angiography in 14 patients and computedtomography (CT) angiography in one patient, with images acquired in both expiratory and inspiratory phases ofrespiration. CA decompression was offered after the results of a thorough workup for other pathology were negative,including upper and lower endoscopy, CT scanning, gastric and gallbladder emptying studies, upper gastrointestinalseries, and small-bowel follow-through studies. Indications in all patients were abdominal pain and weight loss (average,9 lbs). The procedure consisted of laparoscopic division of the median arcuate ligament and complete lysis of the CA fromits origin on the aorta to its trifurcation.Results: Between November 2002 and September 2007, 15 consecutive patients underwent laparoscopic CA decompres-sion. Median length of follow-up was 44.2 months. There were no operative deaths. Four patients were convertedintraoperatively to an open decompression, all for intraoperative bleeding; only one required a blood transfusion. Averageoperating time was 189 minutes, and the average length of stay was 3.5 days. CA intervention was required in six patients,including three intraoperative procedures (1 patch angioplasty, 1 celiac bypass, 1 percutaneous angioplasty) and six lateprocedures (2 percutaneous angioplasties, 3 percutaneous stents, 1 celiac bypass). One complication occurred, a severecase of pancreatitis that developed 1 week after discharge. On follow-up, 14 of 15 patients subjectively reportedsignificant improvement, and one patient remains symptomatic with no diagnosis.Conclusion: Laparoscopic decompression of the CA may be a useful therapy for CACS, but there is potential for vascularinjury, and adjunctive CA intervention is often required. Surgeons should consider laparoscopic CA decompression as atherapeutic alternative for CACS and should participate in the care of patients with this diagnosis. ( J Vasc Surg 2009;
50:124-33.)
The phenomenon of celiac artery compression syn-drome (CACS) was first described in a case report byHarjola et al1 in 1963. Two years later, Dunbar et al2
published a series of patients with abdominal pain who weretreated successfully with surgical decompression of the CA.These reports were soon followed by a number of publica-tions from various institutions around the world that doc-umented variable but for the most part successful outcomesafter treating this problem with decompression of the CAand correction of any intrinsic stenosis in the vessel whenindicated.3-12
The principle source of skepticism about CACS can betraced primarily to a publication in 1972 by a preeminentvascular surgeon of the time, D. Emerick Szilagyi,13 as wellas one by Brandt and Boley14 published in 1978. Theauthors of these articles conceded that the bulk of thepublished literature reported largely successful outcomes
From the Division of Vascular Surgery, Johns Hopkins University.Competition of interest: none.Correspondence: Glen S. Roseborough, MD, The Johns Hopkins Hospital,
with treating CA compression, and they introduced nomeaningful data of their own to refute the diagnosis, yetthey forcefully argued against the concept of CACS. Botharticles were published before the advent of advanced im-aging modalities such as computed tomography (CT),magnetic resonance imaging (MRI), duplex ultrasound(DUS) imaging, and even catheter-based angiography, so itis understandable that therapeutic failures could be due toerrors of diagnosis and poor patient selection. Other ex-perts have cited difficulty explaining the pathophysiologicmechanism by which symptoms develop; therefore, thediagnosis of CACS has remained controversial to this day.
The traditional therapy for CACS has been opensurgical decompression with surgical correction of in-trinsic disease if it is encountered. Reticence to treatCACS likely stems partly from the concern of performinga nontherapeutic laparotomy, with its attendant morbid-ity rate of up to 40% to 50% that includes ventral hernia,ileus, and bowel obstruction.15-17 Another concern isthe technical difficulty of performing a surgical recon-struction of intrinsic disease in the CA if it is encounteredat the time of laparotomy. However, not a single opera-tive death has been reported in the surgical treatment of
CACS, and if the syndrome is considered as a diagnosis
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of exclusion, CA decompression can be effectively ap-plied to carefully selected patients.
Roayaie et al18 reported the first case of CACS treatedwith laparoscopic techniques in 2000. Four other singlecase reports have been published since then,19-22 so thatcurrently the complete published world experience withlaparoscopic CA decompression is five cases. In this article,the results of the first clinical series of patients with CACSwho were treated with laparoscopic decompression arereported. The series includes 15 patients, thus tripling thereported experience to date.
METHODS AND SURGICAL TECHNIQUE
Permission for the study was granted from the JohnsHopkins Institutional Review Board. All cases were per-formed by a single surgeon (G. R.), a vascular surgeon withno advanced fellowship training in laparoscopic surgery.Beginning in 2002, all patients referred for CA decompressionunderwent an attempt at laparoscopic decompression. Allpatients had a thorough workup by an experienced gastroen-terologist before the CA decompression procedure. Workupincluded chemistry tests of liver and pancreatic function, CTscans, MRI, esophagogastroduodenoscopy, colonoscopy, orbarium enema, upper gastrointestinal swallow with small-bowel follow-through in all patients, as well as gallbladderultrasound imaging and gallbladder-emptying studies in pa-tients who had a gallbladder. Angiography was performedwith lateral technique in all patients but one, a patient who hada convincing CT angiogram (CTA; Fig 1); CTA has beenconfirmed to be effective at diagnosing CACS.23,24 Gastric-emptying studies were performed in 14 patients, and threepatients were known to have refractory gastroparesis beforesurgery. Esophageal manometry was not routinely per-formed preoperatively because the result of esophago-gastroduodenoscopy was negative in all, and no patientreported dysphagia or heartburn. All patients but onereported weight loss, with an average weight loss for theentire group of 19.5 lbs (range, 0-50 lbs).
On the operating table, patients were placed supine ona beanbag to allow positioning in steep reversed Trendelen-burg position without slipping. Five trocars were typicallyplaced. Three 12-mm ports were placed: one for the camerain the supraumbilical location, approximately midway be-tween the umbilicus and the xiphoid; another in the rightmidclavicular line at the level of the umbilicus for a fanretractor to elevate the left lobe of the liver; and a third wasin the left midclavicular line for placement of a J retractor toretract the incisura of the stomach caudally. A 5-mm portwas placed in the midline as high as possible under thexiphoid, and another was placed below the left costalmargin in the midclavicular line. In one instance a sixthtrocar was placed in the left upper quadrant to facilitatedissection. The last two cases were performed with a 5-mmcamera and a flexible articulating 5-mm snake retractor,such that the surgery was accomplished with four 5-mmports and only one 12-mm port for the fan retractor, whichretracts the liver (Fig 2). This method leaves only one port
site to close at the end of the operation.
The first case was performed according to the tech-nique described in the one published case report at thetime,18 with mobilization of the gastroesophageal junctionand complete exposure of the supraceliac aorta from theaortic hiatus to the CA, followed by a crural repair at theconclusion of the procedure. In all subsequent patients,dissection of the aortic hiatus and crural repair was elimi-nated because this was found to be unnecessary. Omittingthis step decreases operative time and makes the surgerytechnically much easier, but more importantly, it also elim-inates the possible confounding complication of gastro-esophageal reflux, which a crural dissection and repair canpotentially introduce into the clinical picture. The lastpatient in our series presented after an unsuccessful laparo-scopic Nissen fundoplication, and celiac decompressionwas accomplished in that patient without difficulty.
Dissection was begun directly through the gastrohe-
Fig 1. A, Sagittal reconstruction of a computed tomographyangiogram of a patient with celiac artery compression shows awidely patent celiac artery on inspiration. B, Sagittal reconstruc-tion shows severe stenosis of the celiac artery on expiration.
patic ligament. The peritoneum over the right crus of the
JOURNAL OF VASCULAR SURGERYJuly 2009126 Roseborough
diaphragm was incised, and the right crus was mobilized offthe right lateral wall of the supraceliac aorta. Dissectionproceeded in a caudal direction on the anterior wall of theaorta. Exposure is optimized with caudal retraction on theincisura of the stomach and an angled camera; this istypically a 30° camera, but a 45° camera was necessary in a
Fig 2. The abdominal port sites are shown for last patient treatedin the series. Only one 12-mm port, which was for the fan retractorto retract the liver, and four 5-mm ports were used, thanks toimproved resolution with 5-mm cameras.
Fig 3. A celiac artery has been completely skeletonized and de-compressed. AO, Aorta; HA, hepatic artery; LG, left gastric artery;MAL, median arcuate ligament; SA, splenic artery.
few instances to visualize the celiac axis behind the incisura.
Division of the fibers of the median arcuate ligament(MAL) was best accomplished with a hook cautery, whichallows one to lift the fibers off the aorta as they are divided.Dissection with alternate instruments such as the LigaSure(Valleylab/Tyco Healthcare Group LP, Boulder, Colo)and Harmonic Scalpel (Ethicon Endo-Surgery, Cincinnati,Ohio) was attempted but presented difficulty with dissec-tion because the tips of these instruments tended to pushinto the anterior wall of the aorta. In several cases, finalexposure of the origin of the CA required retrograde dis-section from the more distal CA after first identifying theceliac axis or the hepatic artery distally. In several instancesthe scope was relocated to the subxiphoid or subcostallocation to see the CA origin behind the stomach; however,this positioning tends to be disorienting because the oper-ating surgeon is working directly opposite the perspectiveof the camera.
The fibers of the MAL and the surrounding neuralplexus are completely divided until the CA is completelyexposed from the aorta to the primary branches of the CA(Fig 3). Complete decompression was confirmed by visualinspection. Intraoperative endoscopic US imaging was notused to confirm complete release of the CA, although thishas been described.18 CA decompression was confirmed inall patients with postoperative imaging with US or CTscanning. The facial defects from the 12-mm trocars wereclosed with a Carter-Thompson device, but the fascialdefects from the 5-mm ports were not closed.
All patients underwent postoperative imaging of theCA with mesenteric DUS scans at each follow-up visit.CTA, magnetic resonance angiography (MRA), or digitalsubtraction angiography were used to further evaluate ab-normal DUS findings.
RESULTS
Between December 2002 and May 2008, laparoscopicrepair was attempted in 15 consecutive patients. Previousabdominal procedures in these patients included cholecys-tectomy in 5, appendectomy in 3, uterine surgery in 4,esophageal dilatation in 2, a botulinum toxin injection in 1,and Nissen fundoplication in 1. No patient had a docu-mented psychiatric history. Duration of symptoms beforeintervention ranged from 6 months to several years. Me-dian length of stay was 3.0 days (range 1-10 days).
Four conversions (27%) to open repair were required,all for bleeding. In one patient, a small puncture in the aortawas created during the final division of fibers of the MAL.This could have been repaired with laparoscopic endocor-poreal suturing, but appropriate instruments were notavailable in the room. In the second patient, a phrenicartery was lacerated. In the third and fourth patients, thesuperior wall of the CA was injured. These were all directinjuries during the dissection, and meticulous techniquewith excellent exposure is critical during the dissection. Useof the 45° camera in later procedures has improved theexposure behind the incisura, resulting in conversion ofonly one of the last seven patients. The first of these celiac
injuries was repaired with patch angioplasty with a Dacron
eratio
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patch. The second was repaired with an aortoceliac bypasswith polytetrafluoroethylene graft when an intrinsic steno-sis in the CA was found at the time of conversion. Only oneof these patients, the last patient described, required ablood transfusion.
Mean operating time was 189 minutes (range, 96-395minutes), and length of stay was 3.5 days (range 1-10 days) forthe entire group. Patients who required conversion had sig-nificantly longer operative time than those who did not re-quire conversion (263 � 96 vs 162 � 54 minutes; P � .02, ttest), but length of stay for conversions was not significantlylonger (4.75 � 0.96 days vs 3.0 � 2.72 days; P � .24,t test).Of the 11 patients who underwent successful laparoscopic CAdecompression, one stayed 10 days as a result of difficulty witheating, which was later attributed to gastroparesis. One stayed6 days due to difficulty with pain control in the setting of anextensive history of narcotics use to manage chronic pain. Therest were all discharged between 1 and 3 days postoperatively,and those who stayed beyond day 1 were for lesser degrees ofdifficulty with pain management.
The results are summarized in the Table. One patientwas an outright clinical failure, and he remains symptomaticwithout a diagnosis. He would have been treated anyway,was at least spared a laparotomy, and was discharged with-out complications on postoperative day 2. A second pa-
Table. Results of laparoscopic celiac artery decompression
Patient DOSAge,
y Sex RaceWt loss,
lbs LOSOR,min
1 11/25/02 51.0 F B 4 10 292
2 12/2/02 61.5 F W 30 2 142
3 12/15/03 44.0 F B 19 5 165 C
4 7/15/04 20.5 F W 10 2 1665 8/16/04 40.6 M I 30 2 198
6 8/25/04 45.0 M W 28 4 240 C
7 11/5/04 21.1 F W 8 1 1308 3/24/05 30.7 F W 20 6 251 C
9 6/23/05 20.6 F W 0 2 15910 7/12/05 55.8 F W 40 1 96
11 12/22/05 48.5 F W 25 3 20112 7/18/06 24.2 F W 13 4 395 C
13 12/14/06 38.7 F W 6 6 135
14 9/7/07 22.7 F W 9 1 108
15 5/28/08 50 F W 50 3 151
Avg 19.5 3.5 189
B, Black; DOS, date of surgery; I, Asian Indian; LOS, length of stay; OR, op
tient, the first patient ever treated, was a clinical failure
initially. Postoperatively, she was found to have significantgastroparesis and ultimately required a feeding jejunostomy2 years later. On late follow-up 5 years later, however, she isnow pain-free and eating normally, and the jejunostomyhas been removed.
Only one serious complication occurred, pancreatitis ina patient who was converted for bleeding from an injury tothe anterior wall of the CA. She required patch angioplastyto repair the CA injury. She was discharged after a fairlyunremarkable postoperative course, but several days afterdischarge she was readmitted to another hospital withpancreatitis. She remained hospitalized for 6 months andreceived total parenteral nutrition for 8 months. She nowhas chronic pain related to pancreatitis, which is differentfrom her original pain. Remarkably, she is nevertheless gladthat she had CA decompression because the procedurerestored her ability to eat, and her weight has increasedfrom 118 to 170 lbs.
Three patients report partial improvement with re-duced pain and reversal of weight loss, but some persistentsymptoms. The last patient in the series was a complex case,with severe pain and profound weight loss (50 lbs). Hersymptoms had been attributed to reflux but failed to re-spond to a Nissen fundoplication done a few months beforepresentation. Gallbladder dyskinesis was also discovered
5 consecutive patients
plication Adjunct procedure Result
Gastroparesis, J tube,asymptomatic at 5 y
Celiac stent, 1 mon Much improved, mildpersistent pain
rsion, aorticry
Good
GoodUnchanged, persistent pain,
no diagnosisrsion,nic art
ry
Excellent
Goodrsion, celiacry,creatitis
Patch angioplastyintra-op
Pain from chronicpancreatitis but toleratingfood
ExcellentWeight stabilized, some
painExcellent
rsion, celiacry
Celiac bypassintra-op
Excellent
Celiac PTA intra-op Much improved, eats betterbut some pain
Celiac stent; bypass,1 y
Excellent after bypass
Celiac stent, 2 mon Improved after secondarystenting
during her workup. Because of her failure to thrive, she
JOURNAL OF VASCULAR SURGERYJuly 2009128 Roseborough
underwent simultaneous laparoscopic celiac decompres-sion and cholecystectomy rather than risk further deterio-ration between operations. She was discharged in improvedcondition on postoperative day 3, but returned 1 week later
Fig 4. A, In this lateral expiratory aortogram 2 monthsvisualize despite being documented on mesenteric duplphy. B, Deployment of a stent in the same patient demCompletion angiogram after stenting. D, Follow-up anmonths later, with recurrence of symptoms.
with recurrent pain. A follow-up CT scan showed an intrin-
sic stenosis of the CA. She was treated conservatively for afew more weeks but continued to lose weight. Celiacstenting was performed 2 months after the celiac decom-pression. Since then she has regained 4 lbs and is much
celiac decompression in patient 15, stenosis is difficult torasound imaging and computed tomography angiogra-tes high-grade intrinsic stenosis of the celiac artery. C,
am shows stenosis at the origin of hepatic artery several
afterex ultonstragiogr
improved, but not pain free, and follow-up is limited.
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The 14th patient in the series represents a comprehen-sive case study of CACS. She was completely asymptomaticfor 1 month after surgery; then recurrent symptoms devel-oped. A follow-up DUS scan showed cessation of hepaticartery flow with expiration, but a follow-up angiogram 3months after decompression looked unremarkable. Hersymptoms persisted, and an MRA 2 months later showed ahigh-grade stenosis at the origin of the CA, so angiographywas repeated. This study did confirm a stenosis at the originof the CA involving the inferior edge of the vessel. A stentwas placed that completely relieved her symptoms for 4months. Recurrent pain then developed, and DUS imagingsuggested an in-stent stenosis. Angiography showed a web-like stenosis at the origin of the hepatic artery, with delayedfilling of the hepatic artery compared with the splenicartery. Angioplasty was used to treat this stenosis, but theappearance of the completion angiogram was unchanged,and her symptoms persisted. Follow-up angiography withintravascular US showed a persistent stenosis at the originof the hepatic artery as well as an in-stent stenosis due tointimal hyperplasia (Fig 4). She recently underwent aorto-celiac bypass with a saphenous vein graft, 11 months afterher original decompression. The explanted stent from thispatient is shown in Fig 5. On short-term follow-up afterthat operation, she is now completely pain-free, eatingnormally, and reports an excellent result, having regained 6lbs.
The other seven patients report good to excellent re-sults and are completely pain-free, with no further issues onintermediate follow-up. In all, 14 of the 15 patients reportcomplete or significant relief of their original symptoms,with one patient reporting no improvement and one pa-tient troubled by persistent symptoms of chronic pancreati-tis. Eight patients (53%) reported weight gain averaging 14lbs, and the weight stabilized in the rest.
Overall, six patients required surgical or percutaneousmanipulation of the CA in addition to laparoscopic decom-pression. The first procedure was a celiac stent that was aplanned second procedure after laparoscopic decompres-sion in a patient who was known preoperatively to have anintrinsic stenosis in addition to extrinsic compression. Shecould have been treated in one stage with surgical decom-pression and bypass or patch angioplasty, but she was theoldest patient in the series and we concluded that a stagedapproach with percutaneous stenting after decompressionwould be safer. She required repeat angioplasty a year afterher stent was placed and remains significantly improved 4years later, tolerating food with weight gain but with somepersistent abdominal pain.
The second procedure was a primary repair of an iatro-genic injury to the CA in the patient who developed pan-creatitis.
The third procedure was also performed to treat aniatrogenic injury to the CA; but on exploration, an intrinsicstenosis was discovered that was not recognized preopera-tively, so she was treated with a celiac bypass.
The fourth patient had undergone inappropriate stent
placement at another institution before the CA decompres-
sion. A follow-up angiogram 1 month later showed thestent had fractured (Fig 5). She underwent intraoperativeangioplasty of the CA immediately after laparoscopic de-compression to correct a stenosis within the fractured stent.
The fifth and sixth patients who required celiac inter-vention, which are the last two patients treated in the seriesdescribed in detail above, both required delayed stenting ofpersistent, intrinsic celiac stenoses that were not evident onpreoperative imaging. The stenoses in both patients weredifficult to demonstrate angiographically; but they werereal, and the patients’ symptoms improved with stenting. Itis possible that intraoperative pressure measurementswould have identified these patients to allow immediatesurgical revascularization.
In summary, four patients had intrinsic disease of theCA that would have required management under any cir-cumstances, one intervention was due to a preventableinjury, and one intervention resulted from a preventableerror in judgment by a radiologist.
DISCUSSION
This article presents the first clinical series of patients
Fig 5. Complications of celiac stents. A, A stent in a celiac arteryplaced before celiac decompression fractured only 1 month afterdeployment. B, Explanted stent from patient 14 with significantintimal hyperplasia.
treated laparoscopically for CACS. Several lessons regard-
JOURNAL OF VASCULAR SURGERYJuly 2009130 Roseborough
ing the technical performance of this procedure werelearned:
First, dissection of the esophageal hiatus and the sub-sequent crural repair that this necessitates, as performed byRoayaie et al, is not required to adequately expose anddecompress the CA and should probably be avoided tosimplify the operation and avoid introducing the con-founding diagnosis of gastroesophageal reflux.
Second, the operation can be performed with just one12-mm port for a fan retractor and four 5-mm ports.
Third, visualization of the CA origin behind the inci-sura of the stomach can be difficult and is facilitated byplacing the camera port as high as possible in the midline, atleast midway between the umbilicus and midline, and byhaving a 45° scope available if necessary.
Fourth, dissection around the aorta is technically diffi-cult and carries with it the risk of bleeding from injury tothe aorta or branch vessels, as evidenced by the need forconversion in four of 15 patients.
One may speculate that conversion may have beenavoided with more advanced laparoscopic training andexperience. The reason for conversion in all cases wasbleeding from injury to the CA, supraceliac aorta, orphrenic artery; CA reconstruction was required in two ofthese patients. Because these injuries can be technically verydifficult to expose and repair, a vascular surgeon should beinvolved in these cases to repair these injuries rapidly if theydo occur.
The CA was effectively decompressed in 11 of 15patients, and using a combination of surgical and percuta-neous adjunct, we ultimately documented good to excel-lent results in 13 of 15 patients (87%). The patient who wasan outright clinical failure would have been treated anyway,and was at least spared a laparotomy, was discharged with-out complications on postoperative day 2. The patient whohad a serious complication from the procedure is neverthe-less clinically improved, is now able to eat pain-free, and hasgained 52 lbs. The first patient ever treated in this series wasconsidered a clinical failure for several years but ultimatelybecame asymptomatic. Her persistent symptoms wereeventually attributed to gastroparesis. In fact, two groupsof investigators have documented that gastroparesis can becaused by CACS and reversed with its treatment.25,26
Nine vascular interventions on the CA were required insix of the 15 patients, so physicians treating this conditionshould be prepared and able to intervene on the CA whennecessary. Ideally, occult intrinsic disease of the CA wouldbe identified preoperatively or at least intraoperatively, butpercutaneous interventions currently available do allow safeadjunctive therapy that can be used selectively at a later dateto treat persistent intrinsic disease on clinical grounds.
Reilly et al8 demonstrated that good late results withCA decompression correlated highly with persistent pa-tency of the CA, as determined by angiography. Similarly,Takach et al11 reported excellent results with liberal use ofCA revascularization in five of seven patients who weretreated surgically for CACS.11 Lord et al6 found intimal
lesions in six of 12 patients that they treated for celiac
compression and reported excellent results in 11; eightunderwent CA revascularization.
However, the temptation to treat celiac lesions withpercutaneous techniques before release of the extrinsiccompression of the CA should be resisted, as demonstratedby our patient who received a stent before decompressionand whose stent fractured within a month of its placement.Similar failures of percutaneous therapy without surgicaldecompression have been reported by Choghari et al27 andmore recently by Delis et al.28 The same experience hasbeen shown with another vascular compression syndrome,thoracic outlet syndrome, where stenting of the subclavianvein for Paget-Schroetter syndrome before decompressionyields dismal results.29
Percutaneous revascularization with intraoperative an-gioplasty or postoperative stenting was a successful adjunctto laparoscopic decompression in three of four patients sotreated; whereas in another, it gave only short-term reliefbefore a bypass was required. The utility of combininglaparoscopic CA decompression with percutaneous revas-cularization should be measured against an open decom-pression with immediate surgical revascularization. Thiswould be best done in a clinical trial. Meanwhile, laparo-scopic decompression coupled with expectant, selectivemanagement of intrinsic disease when clinically indicatedwith percutaneous revascularization is a reasonable alterna-tive that has the potential to minimize the morbidity ofsurgical intervention on the CA.
CACS remains controversial for several reasons. First, itis vascular surgery dogma that at least two of the threemesenteric vessels must be involved to produce symptomsof mesenteric ischemia. This philosophy is usually attrib-uted to observations of the small retrospective surgicalseries of Hansen30 (12 patients), Rob31 (18 patients), andmore recently, Johnson et al32 (21 patients). It is refuted byMensink et al,33 who found disease was isolated to a singlevessel in 60% of patients in a series of �300 patients, as wellas by the experience of Calderon et al,34 who found singleartery involvement in 25% of patients. Furthermore, mostpatients with chronic mesenteric ischemia are misdiagnosedor diagnosed very late in their course, and it is possible thatearly symptoms could be associated with lesser arterialinvolvement.
There is no human experimental evidence whatsoeverto support the claim that at least two of three mesentericvessels must be diseased to cause gut ischemia. Meanwhile,human experimental evidence does suggest that CA com-pression causes gut ischemia. Stanley and Frye35 demon-strated in 1971 that d-xylose absorption was impaired inpatients with CACS and that this was reversed with CAdecompression. Using a physiologic study known as gastrictonometry, other investigators have recently demonstratedthat CACS can cause gastric ischemia directly, and that suc-cessful reversal of this ischemia with CA decompression cor-relates with positive clinical outcomes.36-38 Unfortunately,this test is not available in our institution, but would ideally be
applied to select future patients for treatment.
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Fig 6. Lateral aortogram shows a patent celiac artery on (A) inspiration and (B) expiration. Selective angiogram of thesuperior mesenteric artery shows (C) good filling of distal branches of superior mesenteric artery on inspiration and (D)poor filling of distal branches on expiration, with shunting of blood flow into hepatic artery on expiration, a typical
example of intramesenteric steal.
JOURNAL OF VASCULAR SURGERYJuly 2009132 Roseborough
Most experts consider CACS to cause symptomsthrough intestinal ischemia as a consequence of a stealsyndrome, where blood flow is diverted away from thesmall-bowel circulation to the CA circulation. This findingcan be demonstrated angiographically with selective cathe-terization of the superior mesenteric artery, where blood isshunted from the superior mesenteric artery circulation tothe hepatic circulation during expiration when the CA iscompressed (Fig 6).
Another source of controversy occurs because celiaccompression can be seen in individuals who are completelyasymptomatic: some degree of celiac compression can bedemonstrated angiographically in 12% to 49% of asymp-tomatic individuals.39-42 However, most coronary and in-frainguinal occlusive lesions are asymptomatic. In an anal-ysis of 674 patients from the Framingham Study, Murabitoet al43 found that only 18% of patients with objectivefindings of infrainguinal occlusive disease reported symp-toms of claudication. Yet, no one would consider the highincidence of asymptomatic femoral artery occlusions todiscount femoral artery occlusive disease as a cause of lowerextremity claudication. Liver transplant surgeons ignorethis diagnosis at their peril; it is thought to be clinicallysignificant in 4% to 10% of patients undergoing liver trans-plantation and can lead to hepatic artery thrombosis andgraft loss if not treated.4,44
Further confusion stems from the “neurogenic the-ory” of CACS, which suggests that abdominal pain ismediated by the celiac ganglion. This theory arises fromtwo publications, each consisting of only two case re-ports.45,46 In their publication, Snyder et al45 observedin two patients that the CA was constricted by fibers ofthe celiac ganglion rather than the diaphragm. That doesnot mean that the pathophysiology is unrelated to re-stricted blood flow in the CA; in fact, the authors specif-ically state, “the unilateral integrity of the splanchnicnerves and celiac ganglion was maintained during thedissection, such that the small amount of autonomictissue transected did not constitute a ganglionecto-my.”45 In the two patients reported by Carey et al,46 theCA was not compressed by nerve fibers but by the MAL.However, they included the results of sympathectomiesof the celiac ganglion that they performed in a smallseries of dogs, in which they found that sympathectomyincreased CA blood flow.46 It has been recognizedfor �100 years that sympathectomy can increase bloodflow, and their results do nothing to prove that neuro-logic dysfunction of the celiac ganglion plays any role inthe pathophysiology of CACS. There is no evidence fromeither of these articles that CACS is due to anythingother than compression of the CA, be it by nerve fibers orfibers of the diaphragm. No one else has espoused theneurogenic theory in almost 40 years, and this theoryshould be disregarded. However, wide dissection of theCA is indicated, with complete neurolysis of any nerve
fibers that may be constricting the CA.
CONCLUSIONS
The experience at Johns Hopkins with a unique surgi-cal series of 15 patients suggests that the CA can be decom-pressed laparoscopically with minimal morbidity, althoughthere is potential for significant vascular injury. Intrinsicdisease of the CA may be occult and should be sought forand corrected when indicated. Surgical or percutaneoustechniques can be applied in these patients, but stentingshould not be attempted without decompression of the CAfirst. These results lend further credibility to this controver-sial but debilitating syndrome, and we hope that in thefuture more patients with this diagnosis are offered thistreatment as a result of our and subsequently reportedexperience.
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