Kaposiform lymphangiomatosis presenting with a Group A Streptococcus pericardial effusion

Taylor Koerner1, Mehul D Patel2, Vinay Pai3

  • 1Pediatrics, The University of Texas Health Science Center at Houston, Houston, Texas, USA.

BMJ Case Reports
|March 2, 2022
PubMed

Insights

A child with kaposiform lymphangiomatosis presented with respiratory failure due to a large pericardial effusion. Treatment involved drainage, antibiotics, and medications like sirolimus, leading to recovery.

Area of Science:

  • Pediatric Critical Care Medicine
  • Cardiology
  • Oncology

Background:

  • Kaposiform lymphangiomatosis (KLA) is a rare vascular tumor.
  • KLA can present with life-threatening complications.

Observation:

  • A 4-year-old child presented with acute respiratory failure and a large pericardial effusion.
  • Initial management included pericardiocentesis, revealing purulent fluid.

Findings:

  • Post-drainage complications included heart failure, thrombocytopenia, and renal failure.
  • Histopathology confirmed kaposiform lymphangiomatosis with multi-site involvement.
  • Treatment involved antibiotics, sirolimus, and prednisolone.

Implications:

  • This case highlights the diverse and severe presentations of KLA in children.
  • Multidisciplinary management is crucial for improving outcomes in KLA.
  • Early diagnosis and targeted therapy can lead to successful recovery.

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