Fatal Pulmonary Lymphangiectasia Manifesting After Repeated Surgeries for Intractable Chylopericardium and Chylothorax in a 20-Month-Old Girl

A 20-month-old girl was admitted to our hospital because of progressive dyspnea on exertion and a 10-month history of failure to thrive. She had bilateral hearing impairment. The family history was unremarkable. The patient had a hemangioma on the temporal portion of the head and no edema on the legs or face. A chest radiograph (Figure 1A) showed enlargement of the cardiac silhouette with pulmonary congestion. Cardiac ultrasound and computed tomographic scanning of the chest (Figure 1B) revealed copious pericardial effusion with no structural heart diseases. Diagnostic pericardiocentesis produced 150 mL of milky fluid (Figure 1C), which confirmed a diagnosis of chylopericardium because of positive Sudan III staining, a high triglyceride concentration, and a predominance of lymphocytes (>95%). Despite a medium-chain triglyceride-enriched diet and total parenteral nutrition for more than 1 month after pericardiocentesis, the massive pericardial effusion persisted, which prompted surgical management including the clipping of the thoracic duct and creation of a left pericardial …