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Recurrent hemorrhagic pericardial effusion in a child due to diffuse lymphangiohemangiomatosis: a case report

Shyam S Kothari1, Sanjiv Sharma, Kinjal Bhatt

  • 1Department of Cardiology, All-India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India. kothariss@vsnl.com.

Journal of Medical Case Reports
|February 23, 2010
PubMed

Insights

Diffuse lymphangiohemangiomatosis is a rare cause of recurrent hemorrhagic pericardial effusion in children. This condition involves widespread lymphatic and blood vessel malformations, often leading to fatal respiratory failure despite treatment.

Area of Science:

  • Pediatric Cardiology
  • Pediatric Oncology
  • Medical Genetics

Background:

  • Recurrent hemorrhagic pericardial effusion in children without a clear cause is uncommon.
  • This case highlights a rare pediatric presentation of a complex vascular malformation disorder.

Purpose of the Study:

  • To report a case of diffuse lymphangiohemangiomatosis presenting as recurrent hemorrhagic pericardial effusion in a child.
  • To emphasize the importance of considering this rare diagnosis in similar clinical scenarios.

Main Methods:

  • Case report of a 4-year-old Indian girl with recurrent hemorrhagic pericardial effusion.
  • Diagnostic workup included imaging for pulmonary involvement, mediastinal mass, and bone lesions.
  • Confirmation via pericardiectomy and lung biopsy.

Main Results:

  • The patient was diagnosed with diffuse lymphangiohemangiomatosis.
  • Partial improvement was noted with thalidomide and radiotherapy.
  • The patient ultimately succumbed to progressive respiratory failure.

Conclusions:

  • Diffuse lymphangiohemangiomatosis should be included in the differential diagnosis for pediatric hemorrhagic pericardial effusion of unknown etiology.
  • This condition carries a poor prognosis, often complicated by respiratory compromise.
Abstract

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