What is Pemberton's sign associated with?

Pemberton's Sign: Association with Superior Vena Cava Syndrome

Pemberton's sign is primarily associated with superior vena cava syndrome (SVCS), which occurs due to obstruction of blood flow through the superior vena cava. 1 This clinical sign is elicited when a patient raises their arms above their head, resulting in facial plethora, distention of neck veins, and potential respiratory distress.

Clinical Significance and Pathophysiology

Pemberton's sign occurs due to compression of the superior vena cava when the arms are raised, which:

• Exacerbates venous congestion in the head, neck, and upper extremities
• Increases pressure in the jugular veins
• May cause facial flushing, cyanosis, and respiratory distress
• Typically worsens when the patient is in a supine position 2

The sign is positive when these symptoms develop within 30-60 seconds of arm elevation.

Common Causes

1. Malignancy (60% of cases) 1

   • Lung cancer
   • Lymphoma
   • Metastatic disease to mediastinum

2. Non-malignant causes

   • Indwelling central venous catheters and devices 3
   • Mediastinal fibrosis
   • Large retrosternal goiter
   • Aortic aneurysm
   • Thrombosis

Clinical Presentation of Superior Vena Cava Syndrome

Patients with SVCS typically present with:

• Swelling of face and upper extremities
• Dilated neck or chest wall veins 4
• Dyspnea (most common symptom)
• Chest pain
• Cough and hoarseness
• Headache, visual disturbances, and confusion in severe cases 1

Diagnostic Approach

When Pemberton's sign is positive, further evaluation should include:

1. Imaging:

   • CT angiography (first-line imaging with sensitivity 83% and specificity 96%) 5
   • Echocardiography to assess for cardiac involvement
   • Ultrasound of upper extremities if DVT is suspected

2. Laboratory tests:

   • D-dimer testing (if PE is suspected as a complication)
   • Complete blood count

Management Considerations

Management depends on the underlying cause:

1. For malignancy-related SVCS:

   • Radiation therapy for radiosensitive tumors 6
   • Chemotherapy for lymphoma or small cell lung cancer
   • Corticosteroids for lymphoma/leukemia (especially in pediatric cases) 2

2. For thrombosis-related SVCS:

   • Anticoagulation therapy
   • Thrombolytic therapy in severe cases
   • Removal of central venous catheters if they are the cause 3

3. For mechanical obstruction:

   • Endovascular stenting for immediate relief
   • Surgical intervention for certain cases (e.g., goiter)

Clinical Pearls and Pitfalls

• Pearl: Pemberton's sign should be assessed in all patients with unexplained facial or upper extremity edema.
• Pearl: The presence of this sign warrants urgent evaluation, as SVCS can progress to life-threatening airway compromise.
• Pitfall: Failing to recognize this sign may lead to delayed diagnosis of potentially serious underlying conditions.
• Pitfall: Avoid procedures that require the patient to lie flat if SVCS is suspected, as this may worsen symptoms.

Differential Diagnosis

When Pemberton's sign is positive, consider:

• Superior vena cava syndrome
• Large retrosternal goiter
• Mediastinal mass
• Pulmonary embolism with right heart strain
• Cardiac tamponade (which may present with similar venous congestion) 7

Pemberton's sign is a valuable clinical finding that should prompt immediate investigation for superior vena cava obstruction and its underlying causes.