Superior vena cava syndrome

Background

• External compression by extrinsic malignant mass causes majority of cases
• Thrombus in SVC from indwelling catheter/pacemaker is increasingly more common as cause
• Infection
• Rarely constitutes an emergency
  • Gradual process; collaterals dilate to compensate for the impaired flow
  • Exception is neurologic abnormalities due to increased ICP, laryngeal edema causing stridor, decreased cardiac output

Risk Factors

• Lung Cancer
• Lymphoma
• Indwelling vascular catheters (increasing incidence)
• Thrombotic coagulopathy
• Goiter
• TB
• Radiation
• Pericardial constriction

Diagnosis

CT chest showing R lung tumor compressing SVC

• CT with IV contrast
  • Recommended imaging modality (assesses patency of the SVC, evaluate etiology mass vs. thrombus)
• CXR
  • Shows mediastinal mass or paranchymal lung mass (10% of pts)

Differential Diagnosis

Facial Swelling

• Buccal space infections
• Dental problems
• Canine space infection
• Facial cellulitis
• Herpes zoster
• Masticator space infections
• Maxillofacial trauma
• Neoplasm
• Parapharyngeal space infection
• Salivary gland diagnoses
  • Parotitis
    • Bilateral parotitis
    • Suppurative parotitis
    • Viral parotitis (Mumps)
  • Ranula
  • Sialoadenitis
  • Sialolithiasis
• Superior vena cava syndrome

Oncologic Emergencies

Related to Local Tumor Effects

• Malignant airway obstruction
• Bone metastases and pathologic fractures
• Malignant spinal cord compression
• Malignant Pericardial Effusion and Tamponade
• Superior vena cava syndrome

Related to Biochemical Derangement

• Hypercalcemia of malignancy
• Hyponatremia due to SIADH
• Adrenal insufficiency
• Tumor lysis syndrome
• Carcinoid syndrome

Related to Hematologic Derangement

• Neutropenic fever
  • Neutropenic enterocolitis (typhlitis)
• Leukostasis and hyperleukocytosis
• Hyperviscosity syndrome
• Thromboembolism

Related to Therapy

• Chemotherapy-induced nausea and vomiting
• Cytokine release syndrome
• Chemotherapeutic drug extravasation
• Differentiation syndrome (retinoic acid syndrome) in APML
• Stem cell transplant complications
• Catheter-related complications
  • Tunnel infection
  • Exit site infection
  • CVC obstruction (intraluminal or catheter tip thrombosis)
  • Catheter-related venous thrombosis
  • Fracture of catheter lumen
  • Oncologic therapy related adverse events

Clinical Features

• Facial swelling
  • Worse in morning, gets better as day progresses
• Cyanosis
• Dyspnea
• Cough
• Arm swelling
• Distended neck/chest wall veins
• Neurologic abnormalities (rare)
  • Visual changes
  • Dizziness
  • Confusion
  • Seizure

Treatment

• Elevate head of bed
• Assess for and treat elevated intracranial pressure
• Corticosteroids and loop diuretics have questionable efficacy and should be held until ordered by admitting team
• Intravascular stent
• If malignancy
  • Mediastinal radiation
• If thrombus
  • Anticoagulation, catheter removal, consider thrombolytics