Generalised lymphangiomatosis with chylothorax

Abstract

A 9 month old boy presented with acute respiratory distress and was found to have a left pleural effusion. The chylous nature of the effusion, multiple bony lytic lesions, and spleinic cysts lead to the diagnosis of congenital lymphangiomatosis with chylothorax. Surgical intervention including pleurectomy was required after unsuccessful conservative management.