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Superior Vena Cava Syndrome

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Superior Vena Cava Syndrome

PHYSICAL EXAM

para>Symptoms may be exacerbated by lying down or bending forward. ‚

Initial Tests (lab, imaging)

Severity of symptoms is important in determining urgency of intervention. The following grading system has been proposed; see algorithm for management (2)[C]. ‚

Sputum cytology, thoracentesis, bone marrow biopsy, lymph node biopsy, bronchoscopy, or thoracotomy to confirm malignant cells. ‚

Admission Criteria/Initial Stabilization

Discharge may be appropriate when cause of SVC has been treated and patient is clinically stable. ‚

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.

I87.1 Compression of vein ‚

  • 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

  • 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 cardiomegalyChest 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)
  • Chest x-ray, contrast-enhanced CT, or MRI (if unable to receive contrast) is usually adequate to establish diagnosis.
  • 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
  • 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.

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 therapyCorticosteroids (3)[C]Can be effective in steroid-sensitive malignancies such as lymphomas or thymomaCommonly prescribed, although role in acute SVC syndrome management is not clearMay interfere with diagnosis of suspected lymphoma if administered prior to diagnostic proceduresMost commonly referenced steroid is dexamethasone 4 mg q6h.Possible adverse effects: hyperglycemia, peptic ulcer, adrenal suppression, immunosuppressionPossible interactions with medications metabolized by or that inhibit or induce CYP3A4 and P-glycoproteinDiureticsLoop diuretic use has not been validated but may provide symptomatic relief.Possible adverse effects: hypokalemia, hypomagnesemia, hyperuricemia, tinnitusCaution with sulfa allergy (except ethacrynic acid)
  • AnticoagulationPatients 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 embolismBenefit of low-molecular-weight heparin over oral anticoagulation is unknown.Appropriate anticoagulation or use of dual-antiplatelet therapy following SVC stent placement is unknown.
  • ChemotherapyDependent on etiology/diagnosisTreatment of choice for small cell lung cancer and lymphomasTargeting agents have been proposed for specific tumor types, although not validated.
  • Corticosteroids (3)[C]Can be effective in steroid-sensitive malignancies such as lymphomas or thymomaCommonly prescribed, although role in acute SVC syndrome management is not clearMay interfere with diagnosis of suspected lymphoma if administered prior to diagnostic proceduresMost commonly referenced steroid is dexamethasone 4 mg q6h.Possible adverse effects: hyperglycemia, peptic ulcer, adrenal suppression, immunosuppressionPossible interactions with medications metabolized by or that inhibit or induce CYP3A4 and P-glycoprotein
  • DiureticsLoop diuretic use has not been validated but may provide symptomatic relief.Possible adverse effects: hypokalemia, hypomagnesemia, hyperuricemia, tinnitusCaution with sulfa allergy (except ethacrynic acid)
  • 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
  • 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)
  • 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 embolismBenefit of low-molecular-weight heparin over oral anticoagulation is unknown.
  • Appropriate anticoagulation or use of dual-antiplatelet therapy following SVC stent placement is unknown.
  • Benefit of low-molecular-weight heparin over oral anticoagulation is unknown.
  • 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

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

  • Evidence of acute edema of the head or neck
  • Signs or symptoms of airway compromise
  • Inpatient, intensive care as clinically indicated
  • Institute supportive therapyBed restElevate headOxygen
  • Steroids (see "Medication " ¯)
  • Diuretics (see "Medication " ¯)
  • Bed rest
  • Elevate head
  • Oxygen

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.
  • 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 causeLung cancer: 1-year survival 20%Lymphoma: 2-year survival 50%
  • Neoplastic cases: 85% improvement in 3 weeks with radiation therapy, but symptoms usually recur
  • Lung cancer: 1-year survival 20%
  • Lymphoma: 2-year survival 50%

COMPLICATIONS

REFERENCES

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

ICD9

SNOMED

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.