Download Superior Vena Cava Syndrome

Sejal Patel, MSIV
Gillian Lieberman, MD
Superior Vena Cava
Syndrome
Sejal Patel, MSIV
University of Massachusetts Medical School
Beth Israel Deaconess Medical Center
Sejal Patel, MSIV
Gillian Lieberman, MD
Objectives
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Case Presentation
SVC Syndrome
Pathogenesis
„ Etiology
„ Clinical Features
„ Menu of Tests
„ Treatment
„ Prognosis
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Sejal Patel, MSIV
Gillian Lieberman, MD
Case Presentation
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37 year old previously healthy female presenting
with cough x 3 weeks, and R neck pain and
swelling
10-15 pack year smoking history
Physical exam:
Mild facial flushing
„ Trace edema in R neck and facial region
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? SVC syndrome – Chest CT with contrast
ordered
Sejal Patel, MSIV
Gillian Lieberman, MD
Patient’s Chest CT
Lung mass
BIDMC PACS
Sejal Patel, MSIV
Gillian Lieberman, MD
Patient’s Chest CT
SVC
Mediastinal mass
BIDMC PACS
Sejal Patel, MSIV
Gillian Lieberman, MD
Patient’s Chest CT
R Pulmonary artery
BIDMC PACS
Sejal Patel, MSIV
Gillian Lieberman, MD
Patient’s Chest CT
Collaterals
BIDMC PACS
Sejal Patel, MSIV
Gillian Lieberman, MD
Hospital Course
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Chest CT:
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RUL spiculated mass compatible with neoplasm
Mediastinal lymphadenopathy causing compression of the
SVC, R main pulmonary artery and R main stem bronchus
Transbronchial biopsy, cervical mediastinoscopy
Lymph node biopsy: poorly differentiated carcinoma
Dx: Unresectable stage IV non-small cell lung cancer
Outpatient chemotherapy (cisplatin and etoposide) and
radiation therapy
Possible SVC stent for symptomatic relief
Sejal Patel, MSIV
Gillian Lieberman, MD
Superior Vena Cava Syndrome
Clinical manifestation resulting from
partial or complete obstruction of the
superior vena cava
Sejal Patel, MSIV
Gillian Lieberman, MD
Pathogenesis
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SVC Obstruction
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Extrinsic compression
„ Benign or malignant process involving R lung, lymph
nodes, mediastinal structures
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Intrinsic or luminal obstruction
„ Neoplastic infiltration, thrombosis
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Collateral Development
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Azygous, internal thoracic, lateral thoracic,
paraspinous, and esophageal venous systems
Sejal Patel, MSIV
Gillian Lieberman, MD
Collateral Circulation
Neckbelow
veinsor at the level of the azygous
Obstruction
vein Æ SVC bypass via superficial venous systems
resulting in clinical evidence of SVC obstruction
Internal
thoracic vein
SVC
Azygous
vein
Obstruction above
the level of the
Hemiazygous
azygous vein Æ direct
vein SVC bypass and
no clinical evidence of SVC obstruction
Paraesophageal
veins
Lateral thoracic vein
IVC
Netter, Atlas of Human Anatomy
Sejal Patel, MSIV
Gillian Lieberman, MD
Etiology
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Malignant - 85%
Lung cancer (75-80%)
„ Lymphoma (8-10%)
„ Thymoma, mediastinal germ cell tumors, metastases
(8-10%)
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Benign - 10-15%
Inflammatory – fibrinosing mediastinitis
(histoplasmosis, tuberculosis), sclerosing cholangitis,
sarcoidosis, postradiation fibrosis
„ Iatrogenic – thrombosis from CV line, pacemaker
electrodes
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Sejal Patel, MSIV
Gillian Lieberman, MD
Clinical Features
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Symptoms
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Depend on the acuity of SVC obstruction and collateral
development
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Facial, neck, and bilateral upper extremity swelling are the most
common presenting symptoms
Dyspnea, orthopnea, hoarseness, and cough suggest airway
obstruction
Head fullness, syncope, and lethargy suggest cerebral edema from
venous congestion
Clinical signs
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Facial plethora, tachypnea, venous distension in the neck and
chest wall
Bending forward or lying down may worsen symptoms
Sejal Patel, MSIV
Gillian Lieberman, MD
SVC Syndrome
Distended
veins
Facial and neck
edema
www.meddean.luc.edu/.../ phyabn/image15.jpg
www.UpToDate.com
Sejal Patel, MSIV
Gillian Lieberman, MD
Differential Diagnosis
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Pericardial tamponade and heart failure
Nephrotic syndrome
Mediastinal masses
Aortic aneurysm
Vasculitis
Infections: Tuberculosis, Histoplasmosis, fungal
Sejal Patel, MSIV
Gillian Lieberman, MD
Diagnostic Tests
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Radiologic
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Chest X-ray
Computerized axial
Tomography (CT)
Magnetic Resonance
Imaging (MRI)
Ultrasonography
Contrast-enhanced
venography
Tc 99m scan
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Histologic
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Procedures
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Sputum/pleural fluid
cytology
Bone marrow biopsy
Lymph node biopsy
Bronchoscopy
Thoracentesis
Thoracotomy
Special Tests
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Increased central venous
pressure( 20-50 mmHg)
Sejal Patel, MSIV
Gillian Lieberman, MD
Chest X-ray
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Mediastinal widening
Venous collaterals
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Large azygous vein
Dilated L superior
intercostal vein (aortic
nipple)
Mediastinal/hilar masses
Pleural effusion
Calcifications
Mediastinal
widening
www.embbs.com/aem/photo/sob-xr.jpg
Sejal Patel, MSIV
Gillian Lieberman, MD
Computed Tomography
CT image with contrast
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Mediastinal mass
Pulmonary lesion
SVC obstruction
Hilar adenopathy
Pleural effusion
SVC
obstruction
Collaterals
www.UpToDate.com
Sejal Patel, MSIV
Gillian Lieberman, MD
Magnetic Resonance Imaging
Gradient echo T1-weighted MRI
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Excellent anatomic
visualization
Useful if
contraindication to
IV contrast
Paratracheal
mass
www.UpToDate.com
Sejal Patel, MSIV
Gillian Lieberman, MD
Ultrasonography
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SVC
cannot be directly imaged due to a lack of adequate
acoustic window
„ patency can be indirectly determined with normal
waveforms in the brachiocephalic and subclavian
veins
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Exclusion of thrombus in the upper extremity,
axillary, subclavian, and brachiocephalic veins
Sejal Patel, MSIV
Gillian Lieberman, MD
Ultrasonography
Patient with SVC syndrome
Patient status post SVC stent
Venous pulsatility
Respiratory phasicity
www.emedicine.com
Sejal Patel, MSIV
Gillian Lieberman, MD
Venography
Venogram: Pt with SVC syndrome
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Most conclusive
diagnostic tool
Defines SVC obstruction
and collateral circulation
Identifies thrombus
Extrinsic
compression
of SVC
www.emedicine.com
Sejal Patel, MSIV
Gillian Lieberman, MD
Histology
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Malignancy
Small cell lung cancer
www.muhealth.org/.../ thoracic/img/cellsmall.jpg
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Histoplasmosis
H. capsulatum
www.med.cmu.ac.th/student/ patho/Kamthorn/
Sejal Patel, MSIV
Gillian Lieberman, MD
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Medical management
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Non-Hodgkins lymphomas, germ cell neoplasms, limited-stage small cell
lung carcinoma - responsive to chemotherapy
Radiation - 80-90% relieved of SVC syndrome
Surgical treatment
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Thrombolytics for selected cases of acute thrombosis
Anticoagulants to prevent clot propagation
Diuretics and corticosteroids for laryngeal and cerebral edema
Radiation and chemotherapy
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Treatment
Bypass of obstructed SVC
Mostly a palliative tool, reserved for patients with advanced intrathoracic
disease
Endovascular treatment
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Minimally invasive
Thrombolysis, angioplasty, and stent placement
80-90% procedural success rates
Sejal Patel, MSIV
Gillian Lieberman, MD
Endovascular Treatment
SVC syndrome
L superior
intercostal
drainage
SVC
occlusion
Stent mounted on a balloon
Balloon
deployment
Status post SVC stent
Patent SVC
www.emedicine.com
Sejal Patel, MSIV
Gillian Lieberman, MD
Prognosis
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Benign disease – life expectancy unchanged
Malignant obstruction of SVC
Untreated: ~30 days life expectancy
„ Treated: < 7 month life expectancy
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„ 20% 1-year survival for lung cancer
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NSCLC - poor prognosis, palliative care + radiation tx
„ 50% 2-year survival for lymphoma
Sejal Patel, MSIV
Gillian Lieberman, MD
Summary
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SVC syndrome results from extrinsic or intrinsic
obstruction of the SVC
Clinical presentation depends on the acuity of the
obstruction and adequate collateral development
Majority of the SVC syndrome cases are caused by a
malignant process
Variety of radiologic tests are available for diagnosis
Important to obtain a histologic diagnosis to guide
treatment and determine prognosis
Sejal Patel, MSIV
Gillian Lieberman, MD
Acknowledgements
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Dr. Phillip Boiselle
Dr. Hiroto Hatabu
Dr. Paul Spirn
Dr. Vandana Dialani
The Radiology Residents
Dr. Gillian Lieberman
Pamela Lepkowski
Larry Barbaras
Sejal Patel, MSIV
Gillian Lieberman, MD
References
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Parish JM, Marschke Rf, Dines DE, Lee RE. Etiologic consideration in
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Markman M. Diagnosis and management of superior vena cava syndrome.
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Schindler N, Vogelzang RL. Superior vena cava syndrome. Experience with
endovascular stents and surgical therapy. Surg Clin North Am 1999; 79:683.
Baker GL, Barnes HJ. Superior Vena Cava Syndrome: Etiology, diagnosis and
treatment. American Journal of Critical Care. 1992; 1:54-64.
Pierson DJ. Disorders of the pleura, mediastinum, and diapragm. In Harrison’s
principles of Internal Medicine, 12th Edition. New York: McGraw-Hill; 1991:1115.
Drews RE. Superior vena cava syndrome. UpToDate 13.2 2004.
Kallab AM. Superior vena cava syndrome. Emedicine 2005.
Cumming ML. Superior vena cava syndrome. Emedicine 2003.