Kaposiform lymphangiomatosis presenting with a Group A pericardial effusion.

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.