Superior Vena Cava

Superior Vena Cava

Syndrome
Syndrome
Sejal
Sejal

Patel, MSIV
Patel, MSIV
University of Massachusetts Medical School
University of Massachusetts Medical School
Beth Israel Deaconess Medical Center
Beth Israel Deaconess Medical Center
Sejal Patel, MSIV
Gillian Lieberman, MD
Sejal Patel, MSIV
Gillian Lieberman, MD
Objectives
Objectives

Case Presentation
Case Presentation

SVC Syndrome
SVC Syndrome

Pathogenesis
Pathogenesis

Etiology
Etiology

Clinical Features
Clinical Features

Menu of Tests
Menu of Tests

Treatment
Treatment

Prognosis
Prognosis
Sejal Patel, MSIV
Gillian Lieberman, MD
Case Presentation
Case Presentation

37 year old previously healthy female presenting

37 year old previously healthy female presenting
with cough x 3 weeks, and R neck pain and
with cough x 3 weeks, and R neck pain and
swelling
swelling

10
10
-
-
15 pack year smoking history
15 pack year smoking history

Physical exam:
Physical exam:

Mild facial flushing
Mild facial flushing

Trace edema in R neck and facial region
Trace edema in R neck and facial region

? SVC syndrome
? SVC syndrome

Chest CT with contrast

Chest CT with contrast
ordered
ordered
Sejal Patel, MSIV
Gillian Lieberman, MD
Patients Chest CT
Patients Chest CT
Lung mass
BIDMC PACS
Sejal Patel, MSIV
Gillian Lieberman, MD
Patients Chest CT
Patients Chest CT
SVC
Mediastinal mass
BIDMC PACS
Sejal Patel, MSIV
Gillian Lieberman, MD
Patients Chest CT
Patients Chest CT
R Pulmonary artery
BIDMC PACS
Sejal Patel, MSIV
Gillian Lieberman, MD
Patients Chest CT
Patients Chest CT
Collaterals
BIDMC PACS
Sejal Patel, MSIV
Gillian Lieberman, MD
Hospital Course
Hospital Course

Chest CT:
Chest CT:

RUL
RUL
spiculated
spiculated
mass compatible with neoplasm
mass compatible with neoplasm

Mediastinal
Mediastinal
lymphadenopathy
lymphadenopathy
causing compression of the
causing compression of the
SVC, R main pulmonary artery and R main stem bronchus
SVC, R main pulmonary artery and R main stem bronchus

Transbronchial
Transbronchial
biopsy, cervical
biopsy, cervical
mediastinoscopy
mediastinoscopy

Lymph node biopsy: poorly differentiated carcinoma
Lymph node biopsy: poorly differentiated carcinoma

Dx
Dx
:
:
Unresectable
Unresectable
stage IV non
stage IV non
-
-
small cell lung cancer
small cell lung cancer

Outpatient chemotherapy (
Outpatient chemotherapy (
cisplatin
cisplatin
and
and
etoposide
etoposide
) and
) and
radiation therapy
radiation therapy

Possible SVC stent for symptomatic relief
Possible SVC stent for symptomatic relief
Sejal Patel, MSIV
Gillian Lieberman, MD
Superior Vena Cava Syndrome
Superior Vena Cava Syndrome
Clinical manifestation resulting from
Clinical manifestation resulting from
partial or complete obstruction of the
partial or complete obstruction of the
superior vena cava
superior vena cava
Sejal Patel, MSIV
Gillian Lieberman, MD
Pathogenesis
Pathogenesis

SVC Obstruction
SVC Obstruction

Extrinsic compression
Extrinsic compression

Benign or malignant process involving R lung, lymph
Benign or malignant process involving R lung, lymph
nodes,
nodes,
mediastinal
mediastinal
structures
structures

Intrinsic or luminal obstruction
Intrinsic or luminal obstruction

Neoplastic
Neoplastic
infiltration, thrombosis
infiltration, thrombosis

Collateral Development
Collateral Development

Azygous
Azygous
, internal thoracic, lateral thoracic,
, internal thoracic, lateral thoracic,
paraspinous
paraspinous
, and esophageal venous systems
, and esophageal venous systems
Sejal Patel, MSIV
Gillian Lieberman, MD
Collateral Circulation
Collateral Circulation
SVC
Azygous
vein
IVC
Internal
thoracic vein
Lateral thoracic vein
Paraesophageal
veins
Netter, Atlas of Human Anatomy
Hemiazygous
vein
Neck veins
Obstruction above the level of the
azygous vein direct SVC bypass and
no clinical evidence of SVC obstruction
Obstruction below or at the level of the azygous
vein SVC bypass via superficial venous systems
resulting in clinical evidence of SVC obstruction
Sejal Patel, MSIV
Gillian Lieberman, MD
Etiology
Etiology

Malignant
Malignant
-
-
85%
85%

Lung cancer (75
Lung cancer (75
-
-
80%)
80%)

Lymphoma (8
Lymphoma (8
-
-
10%)
10%)

Thymoma
Thymoma
,
,
mediastinal
mediastinal
germ cell tumors, metastases
germ cell tumors, metastases
(8
(8
-
-
10%)
10%)

Benign
Benign
-
-
10
10
-
-
15%
15%

Inflammatory
Inflammatory

fibrinosing
fibrinosing
mediastinitis
mediastinitis
(
(
histoplasmosis
histoplasmosis
, tuberculosis),
, tuberculosis),
sclerosing
sclerosing
cholangitis
cholangitis
,
,
sarcoidosis
sarcoidosis
,
,
postradiation
postradiation
fibrosis
fibrosis

Iatrogenic
Iatrogenic

thrombosis from CV line, pacemaker

thrombosis from CV line, pacemaker
electrodes
electrodes
Sejal Patel, MSIV
Gillian Lieberman, MD
Clinical Features
Clinical Features

Symptoms
Symptoms

Depend on the acuity of SVC obstruction and collateral
Depend on the acuity of SVC obstruction and collateral
development
development
Facial, neck, and bilateral upper extremity swelling are the mos Facial, neck, and bilateral upper extremity swelling are the most t
common presenting symptoms common presenting symptoms
Dyspnea Dyspnea, , orthopnea orthopnea, hoarseness, and cough suggest airway , hoarseness, and cough suggest airway
obstruction obstruction
Head fullness, syncope, and lethargy suggest cerebral edema from Head fullness, syncope, and lethargy suggest cerebral edema from
venous congestion venous congestion

Clinical signs
Clinical signs

Facial plethora,
Facial plethora,
tachypnea
tachypnea
, venous distension in the neck and
, venous distension in the neck and
chest wall
chest wall

Bending forward or lying down may worsen symptoms
Bending forward or lying down may worsen symptoms
Sejal Patel, MSIV
Gillian Lieberman, MD
SVC Syndrome
SVC Syndrome
www.meddean.luc.edu/.../ phyabn/image15.jpg www.UpToDate.com
Facial and neck
edema
Distended
veins
Sejal Patel, MSIV
Gillian Lieberman, MD
Differential Diagnosis
Differential Diagnosis

Pericardial
Pericardial
tamponade
tamponade
and heart failure
and heart failure

Nephrotic
Nephrotic
syndrome
syndrome

Mediastinal
Mediastinal
masses
masses

Aortic aneurysm
Aortic aneurysm

Vasculitis
Vasculitis

Infections: Tuberculosis,
Infections: Tuberculosis,
Histoplasmosis
Histoplasmosis
, fungal
, fungal
Sejal Patel, MSIV
Gillian Lieberman, MD
Diagnostic Tests
Diagnostic Tests

Radiologic
Radiologic

Chest X
Chest X
-
-
ray
ray

Computerized axial
Computerized axial
Tomography (CT)
Tomography (CT)

Magnetic Resonance
Magnetic Resonance
Imaging (MRI)
Imaging (MRI)

Ultrasonography
Ultrasonography

Contrast
Contrast
-
-
enhanced
enhanced
venography
venography

Tc
Tc
99m scan
99m scan

Histologic
Histologic

Sputum/pleural fluid
Sputum/pleural fluid
cytology
cytology

Bone marrow biopsy
Bone marrow biopsy

Lymph node biopsy
Lymph node biopsy

Procedures
Procedures

Bronchoscopy
Bronchoscopy

Thoracentesis
Thoracentesis

Thoracotomy
Thoracotomy

Special Tests
Special Tests

Increased central venous
Increased central venous
pressure( 20
pressure( 20
-
-
50 mmHg)
50 mmHg)
Sejal Patel, MSIV
Gillian Lieberman, MD
Chest X
Chest X
-
-
ray
ray

Mediastinal
Mediastinal
widening
widening

Venous collaterals
Venous collaterals

Large
Large
azygous
azygous
vein
vein

Dilated L superior
Dilated L superior
intercostal
intercostal
vein (aortic
vein (aortic
nipple)
nipple)

Mediastinal/hilar
Mediastinal/hilar
masses
masses

Pleural effusion
Pleural effusion

Calcifications
Calcifications
Mediastinal
widening
www.embbs.com/aem/photo/sob-xr.jpg
Sejal Patel, MSIV
Gillian Lieberman, MD
Computed Tomography
Computed Tomography

Mediastinal
Mediastinal
mass
mass

Pulmonary lesion
Pulmonary lesion

SVC obstruction
SVC obstruction

Hilar
Hilar
adenopathy
adenopathy

Pleural effusion
Pleural effusion
www.UpToDate.com
SVC
obstruction
Collaterals
CT image with contrast
Sejal Patel, MSIV
Gillian Lieberman, MD
Magnetic Resonance Imaging
Magnetic Resonance Imaging

Excellent anatomic
Excellent anatomic
visualization
visualization

Useful if
Useful if
contraindication to
contraindication to
IV contrast
IV contrast
Paratracheal
mass
Gradient echo T1-weighted MRI
www.UpToDate.com
Sejal Patel, MSIV
Gillian Lieberman, MD
Ultrasonography
Ultrasonography

SVC
SVC

cannot be directly imaged due to a lack of adequate
cannot be directly imaged due to a lack of adequate
acoustic window
acoustic window

patency can be indirectly determined with normal
patency can be indirectly determined with normal
waveforms in the
waveforms in the
brachiocephalic
brachiocephalic
and
and
subclavian
subclavian
veins
veins

Exclusion of thrombus in the upper extremity,

Exclusion of thrombus in the upper extremity,
axillary
axillary
,
,
subclavian
subclavian
, and
, and
brachiocephalic
brachiocephalic
veins
veins
Sejal Patel, MSIV
Gillian Lieberman, MD
Ultrasonography
Ultrasonography
www.emedicine.com
Patient with SVC syndrome Patient status post SVC stent
Venous pulsatility
Respiratory phasicity
Sejal Patel, MSIV
Gillian Lieberman, MD
Venography
Venography

Most conclusive
Most conclusive
diagnostic tool
diagnostic tool

Defines SVC obstruction
Defines SVC obstruction
and collateral circulation
and collateral circulation

Identifies thrombus
Identifies thrombus
Extrinsic
compression
of SVC
www.emedicine.com
Venogram: Pt with SVC syndrome
Sejal Patel, MSIV
Gillian Lieberman, MD
Histology
Histology

Histoplasmosis
Histoplasmosis

Malignancy
Malignancy
Small cell lung cancer
H. capsulatum
www.muhealth.org/.../ thoracic/img/cellsmall.jpg www.med.cmu.ac.th/student/ patho/Kamthorn/
Sejal Patel, MSIV
Gillian Lieberman, MD
Treatment
Treatment

Medical management
Medical management
Thrombolytics Thrombolytics for selected cases of acute thrombosis for selected cases of acute thrombosis
Anticoagulants to prevent clot propagation Anticoagulants to prevent clot propagation
Diuretics and corticosteroids for laryngeal and cerebral edema Diuretics and corticosteroids for laryngeal and cerebral edema

Radiation and chemotherapy
Radiation and chemotherapy
Non Non- -Hodgkins Hodgkins lymphomas, germ cell lymphomas, germ cell neoplasms neoplasms, limited , limited- -stage small cell stage small cell
lung carcinoma lung carcinoma - - responsive to chemotherapy responsive to chemotherapy
Radiation Radiation - - 80 80- -90% relieved of SVC syndrome 90% relieved of SVC syndrome

Surgical treatment
Surgical treatment
Bypass of obstructed SVC Bypass of obstructed SVC
Mostly a palliative tool, reserved for patients with advanced Mostly a palliative tool, reserved for patients with advanced intrathoracic intrathoracic
disease disease

Endovascular treatment
Endovascular treatment
Minimally invasive Minimally invasive
Thrombolysis Thrombolysis, angioplasty, and stent placement , angioplasty, and stent placement
80 80- -90% procedural success rates 90% procedural success rates
Sejal Patel, MSIV
Gillian Lieberman, MD
Patent SVC
Endovascular Treatment
Endovascular Treatment
Balloon
deployment
SVC
occlusion
www.emedicine.com
SVC syndrome
Stent mounted on a balloon Status post SVC stent
L superior
intercostal
drainage
Sejal Patel, MSIV
Gillian Lieberman, MD
Prognosis
Prognosis

Benign disease
Benign disease

life expectancy unchanged

life expectancy unchanged

Malignant obstruction of SVC

Malignant obstruction of SVC

Untreated: ~30 days life expectancy
Untreated: ~30 days life expectancy

Treated: < 7 month life expectancy
Treated: < 7 month life expectancy

20% 1
20% 1
-
-
year survival for lung cancer
year survival for lung cancer
NSCLC NSCLC - - poor prognosis, palliative care + radiation poor prognosis, palliative care + radiation tx tx

50% 2
50% 2
-
-
year survival for lymphoma
year survival for lymphoma
Sejal Patel, MSIV
Gillian Lieberman, MD
Summary
Summary

SVC syndrome results from extrinsic or intrinsic
SVC syndrome results from extrinsic or intrinsic
obstruction of the SVC
obstruction of the SVC

Clinical presentation depends on the acuity of the
Clinical presentation depends on the acuity of the
obstruction and adequate collateral development
obstruction and adequate collateral development

Majority of the SVC syndrome cases are caused by a
Majority of the SVC syndrome cases are caused by a
malignant process
malignant process

Variety of radiologic tests are available for diagnosis
Variety of radiologic tests are available for diagnosis

Important to obtain a
Important to obtain a
histologic
histologic
diagnosis to guide
diagnosis to guide
treatment and determine prognosis
treatment and determine prognosis
Sejal Patel, MSIV
Gillian Lieberman, MD
Acknowledgements
Acknowledgements

Dr. Phillip
Dr. Phillip
Boiselle
Boiselle

Dr.
Dr.
Hiroto
Hiroto
Hatabu
Hatabu

Dr. Paul
Dr. Paul
Spirn
Spirn

Dr.
Dr.
Vandana
Vandana
Dialani
Dialani

The Radiology Residents

The Radiology Residents

Dr. Gillian Lieberman

Dr. Gillian Lieberman

Pamela
Pamela
Lepkowski
Lepkowski

Larry Barbaras
Larry Barbaras
Sejal Patel, MSIV
Gillian Lieberman, MD
References
References
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