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Published on: April 7, 2015
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.
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.
Abstract:
A 4-year-old child was transferred to the paediatric intensive care unit with acute respiratory failure following 4 days of fever, nausea and vomiting. Chest X-ray on admission had an enlarged cardiac silhouette and transthoracic echo confirmed a large pericardial effusion. An emergent pericardiocentesis was performed at bedside which drained nearly 1000 mL of purulent fluid. Postdrainage course was complicated by acute systolic and diastolic heart failure, thrombocytopenia and acute renal failure. A chest CT and MRI were concerning for a diffuse mediastinal soft-tissue density, so the patient underwent interventional radiology-guided biopsy complicated by haemorrhage requiring mediastinal exploration and subtotal thymectomy. Histopathology revealed changes consistent with kaposiform lymphangiomatosis and MRI demonstrated involvement of the lumbar spine and right hip. Following a course of intravenous antibiotics, the patient was started on sirolimus and prednisolone and ultimately discharged home.
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