Pleuropulmonary and skeletal lymphangiomatosis with chylothorax and chylopericardium

Congenital lymphangiomatosis of lung and bone, with or without chylothorax, is a rare but often fatal systemic lymphatic malformation. In those who survive infancy and early childhood, parietal pleurectomy with excision of lymphatic lakes and ligation of the thoracic duct can be successful. Two patients with lymphangiomatosis are described, 1 with chylothorax and chylopericardium with generalized skeletal lesions and the other with pleuropulmonary lesions and chylothorax. Both were successfully treated with parietal pleurectomy, excision of lymphatic lakes, and ligation of lymphatics, including the thoracic duct. To our knowledge, the triad of generalized skeletal lymphangiomatosis, chylopericardium, and chylothorax has not been previously reported.