Case 343: Kaposiform Lymphangiomatosis

A 17-year-old boy presented to the outpatient department with left-sided breast enlargement for 2 years. He also had multiple long bone fractures after trivial trauma over the past 8 years, along with intermittent episodes of streaky hemoptysis. He was treated with bisphosphonates, with marginal relief of symptoms. At the index presentation, as part of preanesthetic checkup for elective surgery for gynecomastia, laboratory investigations revealed thrombocytopenia, with a platelet count of 100 000/µL (reference range, 150 000-400 000/µL), and a normal hormonal profile. Chest radiography was performed, followed by diagnostic thoracentesis, which yielded minimal hemorrhagic fluid. Subsequently, contrast-enhanced CT and contrast-enhanced MRI were performed. Echocardiography showed diffuse pericardial thickening.