Superior Mediastinum Syndrome

Clinical Features

Superior vena cava syndrome:

• Swelling, plethora, and cyanosis of the face, neck, and upper extremities.
• Suffusion of the conjunctiva.
• Engorgement of collateral veins.
• Altered mental status.

Superior mediastinal syndrome:

• Respiratory symptoms: cough, hoarseness, dyspnoea, orthopnoea, wheezing, and stridor.
• Supine position worsens symptoms.
• Dysphagia.
• Chest pain.
• Altered mental status and syncope.

Diagnosis

BEWARE – inappropriately requested investigations may result in worsening respiratory compromise, rapid clinical deterioration and cardio-respiratory arrest!

CXR include PA/AP and lateral to establish size of mass and whether the mass is anterior or posterior.

Chest CT Only perform if it is safe for the patient. This test is not mandatory.

If significant mass on CXR and/or significant respiratory symptoms: Avoid sedation or general anesthesia, this may precipitate respiratory failure leading to death

If respiratory symptoms deteriorate on lying supine, un-sedated chest CT in supine position should be avoided. This may also precipitate acute respiratory failure. If patient can tolerate lying prone or lateral without worsening of symptoms, CT chest may be considered in these positions.

Chest USS If unable to perform chest CT, consider discussing with radiologist for chest/mediastinal ultrasound scan to assess mediastinal mass.

Blood tests Baseline blood investigations (FBC, blood film, U&E, Phosphate, Urate, LDH, AFP & HCG) and urinary catecholamines should be performed as indicated.

Management

• Establishing a tissue diagnosis may not be possible and patients may need empiric treatment as a life-saving measure. First-line treatment in emergent situations is high-dose steroids, although they may confound the diagnosis. Prednisolone 60 mg/m²/day (2 mg/kg/day) or methylprednisolone 48 mg/m²/day (1.6 mg/kg/day) divided into two daily doses should be employed. This will treat hematologic malignancies and decrease airway edema. The patient should undergo biopsy as soon as the mass shrinks and the patient is stable.
• If poor response to steroids, chemotherapy such as vincristine, cyclophosphamide with or without an anthracycline can be added. Tumor-specific chemotherapy should be instituted after a biopsy has been obtained.
• If a solid tumor not responsive to steroids or chemotherapy, emergent radiation can be performed.
• For symptomatic venous thrombosis with no evidence of hemorrhage, anticoagulation can be initiated using systemic or low-molecular-weight heparin (LMWH):
  • Unfractionated heparin can be started with a 75 U/kg bolus followed by 18 U/kg/h (for children) to 28 U/kg/h (infants) continuous infusion. Titrate to a goal-activated partial thromboplastin time of 60-85 s or anti-Xa level of 0.3-0.7 U/ml.
  • LMWH 1 mg/kg every 12 h. Titrate to a goal anti-Xa level of 0.5-1 U/ml.

References

• Wilson, L.D., Detterbeck, F.C., Yahalom, J., 2007. Clinical practice. Superior vena cava syndrome with malignant causes. N. Engl. J. Med. 356 (18), 1862_1869.
• Philip Lanzkowsky (2022). Lanzkowsky’s Manual of Pediatric Hematology and Oncology. 7^th edition. London, UK: Elsevier
• Paediatric Haematology & Oncology: Supportive Care Protocols. A collaborative publication from Great Ormond Street Hospital for Children NHS Foundation Trust, The Royal Marsden NHS Foundation Trust, University College London Hospitals NHS Foundation Trust.
• Philip A. Pizzo, David G. Poplack (2021). Principles and Practice of Pediatric Oncology. 8th Edition. Lippincott Williams & Wilkins

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