Clinical condition that occurs due to obstruction of the superior vena cava.
Most common etiology is malignancy; however, there has been an increase in benign causes due to more frequent use of intravascular devices.
Although rarely fatal, may sometimes present as life-threatening upper airway obstruction.
High index of suspicion is required to make the diagnosis in many cases.
Treatment and prognosis depend on underlying etiology.
Superior vena cava (SVC) syndrome is a clinical condition that occurs as a result of obstruction of the SVC, leading to interrupted venous return from the head, thorax, and upper extremities to the right atrium. The increased venous pressure results in edema of the head, neck, and arms, often with cyanosis, plethora, and distended subcutaneous vessels. It can be caused by either intraluminal obstruction of the SVC or extrinsic compression.
History and exam
Key diagnostic factors
- history of smoking, pacemaker, or central venous catheter
- localized edema of the face and upper extremities
- facial plethora
- distended neck veins
- distended chest veins
- hoarseness of voice
- blurred vision
Other diagnostic factors
- weight loss
- chest pain
- mental changes
- skin rash
- laryngeal edema
- multiple pacemaker leads
- central venous catheters/ports
- age >50 years
1st investigations to order
- chest x-ray
- chest CT
- chest MRI
- ultrasound of upper extremities
Investigations to consider
- sputum cytology
- sputum culture
- erythrocyte sedimentation rate
- C-reactive protein
acute airway obstruction
- Cardiac tamponade
- Constrictive pericarditis
- Acute COPD exacerbation
- Antithrombotic therapy for VTE disease: CHEST guideline and expert panel report
- ACR Appropriateness Criteria: upper extremity swelling
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