Superior Vena Cava Syndrome

Ranjita Pallavi,MD

Internal Medicine

PGY-2

Case Presentation 58 yo female presented with cough productive of yellow sputum, sore

throat and fever with chills for 1 week. She was seen earlier in clinic with similar complaints and given a course of

Zithromax. She was using her Albuterol pump more often. PMHx:

-HIV dx 2007: CD4 284 in 8/2012, VL undetectable in 8/2012, Bactrim, Zithromax ppx dced 6/2012 as CD4 consistently elevated.

-Asthma since childhood, never intubated, non steroid dependent

-Thyrotoxicosis with crisis in 2008, Multinodular goitre-bx benign in 9/2008

-Parotid cyst and masses 9/2008

-Seizure disorder

-Migraine, Chronic Low back pain, Severe OA of knees

-Anxiety, Depression with Psychosis

Case Presentation PSHx:-Partial hysterectomy 1979 for fibroids-Tonsillectomy 1992 Allergies: None Medications at Home:Advair 100/50 BIDAlbuterol prnReyataz 300 mg dailyTruvada 1 tab po dailyAbilify 2 mg dailyParoxetine 30 mg dailyBaclofen 10 mg bidNeurontin 400 mg tidLyrica 100 mg bidKeppra 750 mg bidFerrous SulphateTrazadone 25 prn insomniaDocusate 100 mg daily

Case Presentation Family Hx: Mother with heart disease, Breast ca-died of breast ca, father

with DM, died of complications Social Hx: lives by herself in Harlem in apt with elevator, has HHA, smokes

2 cigarettes/day ( previously ½ PPD X 20 years), No ETOH, no drugs VS in the ER: T 98.9 F HR 110 BP 129/74 RR 20 Sat 94-97% RA, Wt 115

kg( Baseline) Physical Exam: Gen:Obese, AAF, not in acute distress, alert and oriented X3 HEENT: Icteric sclera, No thrush Neck: +symmetrical fullness at base of neck, bilateral cervical and

supraclavicular LAD Lymph: mildly tender submandibular LAD Cardiac: Normal Pulm: Symmetrically reduced breath sounds b/l, no wheezes/rales/rhonchi Abd: soft, non tender, no organomegaly, BS+ Skin: Normal Extr:1+ B/l pitting edema.

Case Presentation Labs: Initial: WBC 9.5, H/H 8.4/24.8 (<- 12/35.3 in 8/2012 baseline), PLT 277 BMP: K 3, CO2 29, BUN/Creat 9/0.8 LFT: ALP 141( elevated since8/2012), T Bili 3.2, D Bili 1.1 Pro BNP 43.3 INR 1.18 Troponins negative CDX: new RML opacification, left hilar fullness, widened mediastinum EKG: Sinus tachycardia, No ST-T wave changes CT PE Protocol :RUL segmental arterial filling defects c/w PE, main PA

normal, heterogenous enhancing mass in right side of thyroid extending into the mediastinum: multinodular goitre vs malignancy, bulky mediastinal and Left hilar LAD.

Positive for PE: patient started on heparin drip CT abdomen non-contrast: cholelithiasis, rest normal

Case PresentationFurther Hospital Course: Patient responded to antibiotic treatment with Ceftriaxone. Since the CT

showed bilateral cervical LAD, she underwent Biopsy of the left cervical anterior LN. She was then later swtiched to Arixtra and discharged home after 10 days of IV antibiotics to follow up as outpatient for results of the biopsy.

She however returned 2 weeks later with persistent symptoms of cough

with yellowish sputum production and worsening sob. She also reported significant weight loss unintentional 10 pounds along with night sweats.

Repeat Chest CT showed worsening clot burden in the previous areas of

PE and new PE in the left upper lobe. Also noted was interval increase in the left hilar mass and mediastinal LAD.

She received an IVC filter and was continued on anticoagulation.

Case Presentation Results of the Left Cervical LN FNA were consistent with poorly

differentiated metastatic ca. She later underwent Left Cervical LN excision biopsy which confirmed the previous finding. The repeat CT also showed worsening infiltrates in both lungs and patient was then treated for HCAP. In view of her HIV status, she was also started on Bactrim for suspected PCP and Zithromax to cover for atypicals. These were later discontinued due to negative workup.

Patient then had worsening sob, became increasingly dyspnoeic and

orthopnoiec when she was transferred to MHC for further management.

On initial evaluation, patient was noted with facial edema, b/l UE edema R> L and collateral veins on upper chest. No stridor.

Repeat Chest and Neck CT were done, however in view of AKI, without contrast. It showed: b/l cervical and supraclavicular LAD with necrotic nodes left hilar mass and left hilar LAD with marked narrowing of theleft upper and lower lobe bronchi, bulky right hilar and mediastinal LAD causing significant narrowing of the trachea,b/l retropectoral and axillary LAD,enlarged Pulmonary arteries, moderate pleural effusions.

Patient was taken to Lincoln Hospital for RT. Received 1 session. However expired next day. Patient not resuscitated as she was DNR/DNI.

Introduction

SVC Syndrome : A medical entity where compression of SVC by various causes brings clinical symptoms and signs of facial, upper body edema, formation of collateral circulations, and causes cyanosis and dyspnea.

Affects 15,000 people in the US every year.

Symptoms develop over a period of 2 weeks in approx. a third of patients, and over longer periods in other cases.

Anatomy and Physiology

n engl j med 356;18 www.nejm.org may 3, 2007

Etiologies

n engl j med 356;18 www.nejm.org may 3, 2007

Clinical Features

n engl j med 356;18 www.nejm.org may 3, 2007

Imaging CDX: Mediastinal widening, Pleural Effusions CT Chest with Contrast Venography MRI PET Tissue Biopsy Bronchoscopy Transthoracic Needle Biopsy Sputum Cytology Thoracentesis Mediastinoscopy and Mediastinotomy

Venographic Classification of SVC Syndrome

AJR 148:259-262, February 1987STANFORD ET AL.

Venographic Classification of SVC Syndrome-Contd.

STANFORD ET AL. AJR 148:259-262, February 1987

CT Diagnosis of Superior Vena Cava Syndrome: Importance of

Collateral Vessels It was believed at that time that CT diagnosis of obstruction of the superior

vena cava (SVC) or its major tributaries required at least two findings: One was lack of (or decreased) opacification of central venous structures

distal to the site of obstruction.

(This may be associated with a visible, obstructing lesion or intraluminal filling defects.)

The other CT finding was opacification of collateral venous vessels. The fulfillment of either criterion alone was insufficient for an accurate CT diagnosis of venous obstruction.

Results of their study: The presence of collateral vessels, regardless of the number and location of the vessels shown on CT scans, was highly accurate as a predictor of superior vena cave syndrome, with a sensitivity of 96% and a specificity of 92%.

The most common site of venous obstruction seen on CT scans was the SVC (n = 41), followed by the brachiocephalic vein (n = 20) and the jugular vein (n = 2).

KIM ET AL. AJA:161, September1993

CT Diagnosis of Superior Vena Cava Syndrome: Importance of

Collateral Vessels-Contd.

KIM ET AL. AJA:161, September1993

Clinical and Radiological Grading of

Superior Vena Cava Obstruction

Respiration 2008;76:69–75Plekker et al

Clinical and Radiological Grading of Superior Vena Cava Obstruction-

Contd.

Respiration 2008;76:69–75Plekker et al

Clinical and Radiological Grading of Superior Vena Cava

Obstruction-Contd.

Respiration 2008;76:69–75Plekker et al

Clinical and Radiological Grading of Superior Vena Cava

Obstruction-Contd. Results of their study: Thirty-four cases of SVCO were evaluated: 8 (23.5%) were

clinicallymild,16 (47%) moderate and 10 (29.5%) severe. Lung cancer was the underlying histological diagnosis in 94% of

cases. Radiologically,53% had complete SVCO. A well-developed collateral system was found in 14 (41%). A scoring system subtracting a ‘collateral score’ from an ‘obstruction

score’ showed a significant correlation with the clinical score (r = 0.75, p <0.01).

Conclusions: Clinical severity of SVCO depends upon the degree of SVCO and is

ameliorated by collateral formation. The novel clinical scoring system can predict the underlying CT

features in SVCO and may be valuable in the bedside assessment of SVCO severity.

Respiration 2008;76:69–75Plekker et al

Grading System for SVC Syndrome

Treatment Algorithm for Malignant Causes

Radiation Therapy

Effective modality for malignancy related SVCO.

Relative Contraindications to RT:

Previous RT in same area

Certain Connective Tissue Disorders like Scleroderma

Known radioresistant tumor types eg Sarcoma

Response rates in literature clinical: Significant discordance with objective

response rates measured by imaging.

RT treatment can vary based on tumor histology and intent of treatment.

RT involves CT Based simulation for designing RT fields: should

encompass gross tumor volume and involved nodal regions and shield

normal organs particularly lungs and esophagus.

Field size may be altered during treatment course.

Monitor for progression of radioresistant tumors requiring alternative treatment.

Occasionally, symptom worsening may be due to thrombus.

Radiation Therapy-Contd.

As per a systematic review done by Rowell and Gleeson: RT provided relief

in ¾ ths of SVCO in SCLC and 2/3 rds of SVCO in NSCLC

Rapidity of response ranges from 7-15 days, may be seen as early as 72 hours.

Chemotherapy and radiotherapy are effective in relieving SVCO in a

proportion of patients whilst stent insertion may provide relief in a

higher proportion and more rapidly.

The effectiveness of steroids and the optimal timing of stent insertion

(whether at diagnosis or following failure of other modalities) remain

uncertain.

Radiation Therapy In SCLC chemotherapy and/or radiotherapy relieved SVCO in 77%.

17% of those treated had a recurrence of SVCO.

In NSCLC, 60% had relief of SVCO following chemotherapy and/or radiotherapy

19% of those treated had a recurrence of SVCO.

Insertion of an SVC stent relieved SVCO in 95%.

11% of those treated had further SVCO but recanalization was possible in the majority resulting in a long-term patency rate of 92%.

Morbidity following stent insertion was greater if thrombolytics were administered.

Chemotherapy Lymphomas, SCLS, Germ Cell Tumors: Highly chemosensitive.

RT may be used but poorer long term results; used for failed

chemotherapy.

Chemotherapy can relieve SVCO symptoms in 80% of NHL and

77% of SCLC.

Response rates similar to RT: 7-15 days.

Addition of chemotherapy to RT: No significant benefit.

Endovascular Stenting Relief may be immediate but most often between 24-72 hours.

Useful for:

○ Patients without a tissue diagnosis

○ Previously treated with RT

○ Known Chemotherapy and RT resistant tumors.

Stent placement needs to be followed up by other

treatment modalities.

Use of thrombolytics.

Prophylactic anticoagulation advocated.

Comparison of outcomes limited due to absence of randomized

controlled trials: Reasons for the same include:

○ One treatment more easily available than the other.

○ Clinical reason to favor one modality over another.

Comparison of RT, Stent Insertion and Chemotherapy

Benign SVC Syndrome Most commonly due to chronic hemodialysis catheters and post

transvenous Pacemaker implantation. Stenting for benign disease not recommended due to longer life

expectancy, lack of long term follow up and possibility of stent fracture,

migration or thrombosis. Surgery is effective. PTFE good prosthetic device with good short term patency rates: may be

related to intimal irregularity and stenosis making it prone to thrombosis. Autogenous vein grafts have a higher patency rate. Femoral, Subclavian and Jugular veins have been used: disadvantage of

impaired venous return at the site of harvesting. Spiral vein graft created from saphenous vein to form a venous conduit is

the preferred graft now. Disadvantages: long suture line: more thrombogenic, more time consuming

to construct. Another autologous technique: Pericardial tube graft replacement.

Thank You

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