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Superior vena cava syndrome.

L S Arya1, Sunil Narain, Sanjay Tomar

  • 1Department of Pediatrics, All India Institute of Medical Sciences, New Delhi. lsarya@rediffmail.com

Indian Journal of Pediatrics
|May 22, 2002
PubMed
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Superior vena cava syndrome (SVCS) in Indian children is frequently caused by hematological malignancies like T-cell acute lymphoblastic leukemia. Early diagnosis and treatment interventions lead to achievable long-term survival without disease.

Area of Science:

  • Pediatric Oncology
  • Hematology
  • Thoracic Medicine

Background:

  • Superior vena cava syndrome (SVCS) is a rare but serious condition in children.
  • Hematological malignancies are a significant cause of SVCS in pediatric populations.
  • Understanding the clinical profile of pediatric SVCS is crucial for timely diagnosis and management.

Purpose of the Study:

  • To investigate the clinical characteristics of children diagnosed with SVCS.
  • To identify the underlying etiologies of SVCS in pediatric patients.
  • To evaluate the outcomes of diagnostic and therapeutic interventions for pediatric SVCS.

Main Methods:

  • Retrospective analysis of case records for children presenting with SVCS over a 10-year period.
  • Identification of underlying hematological malignancies in affected children.

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  • Assessment of treatment modalities and patient follow-up.

    • Main Results:

    • Twenty-one pediatric cases of SVCS were identified, predominantly in males (20:1 ratio).
    • Hematological malignancies, particularly T-cell acute lymphoblastic leukemia (57%) and non-Hodgkin's lymphoma (33%), were the primary causes.
    • No patients required radiotherapy or urgent thoracotomy; 11 survivors had a median follow-up of 6.5 years.

    Conclusions:

    • T-cell acute lymphoblastic leukemia and lymphoblastic lymphoma are common causes of SVCS in Indian children.
    • Diagnostic interventions under local anesthesia and prebiopsy corticosteroid use are effective.
    • Long-term survival without disease is achievable in pediatric SVCS patients with appropriate management.