para>Symptoms may be exacerbated by lying down or bending forward. ‚
PHYSICAL EXAM
- Accessory venous drainage: venous distention of neck and chest wall
- Facial edema
- Plethora of face (excess RBCs)
- Cyanosis, stridor
- Horner syndrome
- Swelling of arms
- Confusion, obtundation
DIFFERENTIAL DIAGNOSIS
- SVC blood clot
- Syphilitic or aortic aneurysm
- Tuberculosis mediastinitis
- Fungal infections
- Congestive heart failure
- Cushing disease/syndrome
DIAGNOSTIC TESTS & INTERPRETATION
Initial Tests (lab, imaging)
- Increased cervical venous pressure; usually 20 to 40 mm Hg (if performed) (1)[C]
- Sputum cytology for presence of malignant cells
- Radiologic studies to scan thorax for mediastinal masses, mediastinal widening, associated pleural effusion, lobar collapse, or cardiomegaly
- Chest x-ray, contrast-enhanced CT, or MRI (if unable to receive contrast) is usually adequate to establish diagnosis.
- Doppler US to rule out thrombosis
- Venography (if stent or surgery planned)
Follow-Up Tests & Special Considerations
Severity of symptoms is important in determining urgency of intervention. The following grading system has been proposed; see algorithm for management (2)[C]. ‚
- Grade 0: asymptomatic: radiographic SVC obstruction in the absence of symptoms
- Grade 1: mild: edema in head or neck (vascular distention), cyanosis
- Grade 2: moderate: edema in head or neck with functional impairment (dysphagia, cough, impairment of head movements, visual disturbances)
- Grade 3: severe: mild or moderate cerebral edema (headache, dizziness) or laryngeal edema or diminished cardiac reserve
- Grade 4: life-threatening: significant cerebral edema (confusion, obtundation) or severe laryngeal edema or significant hemodynamic compromise
- Grade 5: fatal: death
Diagnostic Procedures/Other
- Percutaneous needle biopsy used to establish histologic diagnosis should be done prior to initiation of therapy.
- Open biopsy may be necessary; however, these patients are at increased risk for cardiorespiratory compromise under general anesthesia.
- Bronchoscopy, thoracentesis, thoracotomy, lymph node biopsy as indicated.
Test Interpretation
Sputum cytology, thoracentesis, bone marrow biopsy, lymph node biopsy, bronchoscopy, or thoracotomy to confirm malignant cells. ‚
TREATMENT
GENERAL MEASURES
- Goal: Remove compression, relieve symptoms, and prevent complications.
- Radiotherapy mainly in non " “small cell lung cancer and non-Hodgkin lymphoma; start within 24 hours.
- Neoadjuvant chemoradiotherapy followed by resection (Pancoast tumors)
- Remove central venous catheter if cause of thrombosis.
- Benign causes usually respond to medical therapy, including diuretics, upright positioning, and fluid restriction, until adequate collateral circulation is established and clinical regression is noted.
MEDICATION
- Supportive therapy
- Corticosteroids (3)[C]
- Can be effective in steroid-sensitive malignancies such as lymphomas or thymoma
- Commonly prescribed, although role in acute SVC syndrome management is not clear
- May interfere with diagnosis of suspected lymphoma if administered prior to diagnostic procedures
- Most commonly referenced steroid is dexamethasone 4 mg q6h.
- Possible adverse effects: hyperglycemia, peptic ulcer, adrenal suppression, immunosuppression
- Possible interactions with medications metabolized by or that inhibit or induce CYP3A4 and P-glycoprotein
- Diuretics
- Loop diuretic use has not been validated but may provide symptomatic relief.
- Possible adverse effects: hypokalemia, hypomagnesemia, hyperuricemia, tinnitus
- Caution with sulfa allergy (except ethacrynic acid)
- Anticoagulation
- Patients with extensive thrombosis in conjunction with stenotic SVC obstruction may benefit from local catheter-directed thrombolysis (4)[C].
- Patients with central venous thrombosis should receive 3 to 6 months of anticoagulation to reduce the risk of pulmonary embolism
- Benefit of low-molecular-weight heparin over oral anticoagulation is unknown.
- Appropriate anticoagulation or use of dual-antiplatelet therapy following SVC stent placement is unknown.
- Chemotherapy
- Dependent on etiology/diagnosis
- Treatment of choice for small cell lung cancer and lymphomas
- Targeting agents have been proposed for specific tumor types, although not validated.
ISSUES FOR REFERRAL
Appropriate referral and follow-up is dependent on the cause. ‚
SURGERY/OTHER PROCEDURES
- Percutaneous stenting (for immediate relief) (5)[A],(6)[B]
- Tissue confirmation, especially for lymphomas that require tumor architecture to determine treatment
- SVC reconstruction for benign processes may be considered but is rarely done.
INPATIENT CONSIDERATIONS
Admission Criteria/Initial Stabilization
- Evidence of acute edema of the head or neck
- Signs or symptoms of airway compromise
- Inpatient, intensive care as clinically indicated
- Institute supportive therapy
- Bed rest
- Elevate head
- Oxygen
- Steroids (see "Medication " ¯)
- Diuretics (see "Medication " ¯)
Nursing
Elevate head of patient bed. ‚
Discharge Criteria
Discharge may be appropriate when cause of SVC has been treated and patient is clinically stable. ‚
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
- Bed rest, elevate patient 's head, and limit bending down to decrease the hydrostatic pressure.
- SVC syndrome is often associated with terminal illness; discuss advance directives.
Patient Monitoring
- Severity of clinical symptoms
- If malignant, monitor response to radiotherapy or chemotherapy.
- If infectious, monitor for evaluation of antimicrobial treatment.
- DIET as tolerated; possibly salt restriction to reduce edema
PROGNOSIS
- High probability of initial response
- Linked to cause
- Lung cancer: 1-year survival 20%
- Lymphoma: 2-year survival 50%
- Neoplastic cases: 85% improvement in 3 weeks with radiation therapy, but symptoms usually recur
COMPLICATIONS
Dependent on the underlying disease ‚
REFERENCES
11 Wilson ‚ LD, Detterbeck ‚ FC, Yahalom ‚ J. Clinical practice. Superior vena cava syndrome with malignant causes. N Engl J Med. 2007;356(18):1862 " “1869.22 Yu ‚ JB, Wilson ‚ LD, Detterbeck ‚ FC. Superior vena cava syndrome " ”a proposed classification system and algorithm for management. J Thorac Oncol. 2008;3(8):811 " “814.33 Wan ‚ JF, Bezjak ‚ A. Superior vena cava syndrome. Hematol Oncol Clin North Am. 2010;24(3):501 " “513.44 Lepper ‚ PM, Ott ‚ SR, Hoppe ‚ H, et al. Superior vena cava syndrome in thoracic malignancies. Respir Care. 2011;56(5):653 " “666.55 Rowell ‚ NP, Gleeson ‚ FV. Steroids, radiotherapy, chemotherapy and stents for superior vena caval obstruction in carcinoma of the bronchus: a systematic review. Clin Oncol (R Coll Radiol). 2002;14(5):338 " “351.66 Hochrein ‚ J, Bashore ‚ TM, O 'Laughlin ‚ MP, et al. Percutaneous stenting of superior vena cava syndrome: a case report and review of the literature. Am J Med. 1998;104(1):78 " “84.
ADDITIONAL READING
- Rice ‚ TW, Rodriguez ‚ RM, Light ‚ RW. The superior vena cava syndrome: clinical characteristics and evolving etiology. Medicine (Baltimore). 2006;85(1):37 " “42.
CODES
ICD10
I87.1 Compression of vein ‚
ICD9
459.2 Compression of vein ‚
SNOMED
Superior vena cava syndrome (disorder) ‚
CLINICAL PEARLS
- Lung cancer is the leading cause of SVC syndrome; other malignant causes include lymphoma and metastatic breast and prostate cancer.
- Percutaneous stenting can provide immediate relief.
- Current treatment is disease specific; therefore, pathologic confirmation is vital (3)[C].
- Chemotherapy is the treatment of choice for small cell lung cancer and lymphomas.
- Radiotherapy is useful in non " “small cell lung cancer and non-Hodgkin lymphoma.