Management of Life-threatening Refractory Chylothorax and Chylopericardium due to Lymphangiomatosis

Lymphangiomatosis is a rare disorder. Chylous effusion and osteolytic lesions are diagnostic clues. Radiographic findings can be suggestive of the disease, but the final confirmatory diagnosis is made histologically. We report a case of an infant who presented with life-threatening refractory chylothorax and chylopericardium due to diffuse pulmonary lymphangiomatosis, leading to critical condition with challenging management. With multidisciplinary approach, the child improved and was discharged home with satisfactory outcome.