[Generalized lymphangiomatosis with chylopericardium].

A case of a child with lymphangiomatosis and chylopericardium is reported. Diagnosis of chylopericardium was performed at 11 months with ascendant lymphography which evidenced thoracic duct agenesia. At the age of 3 years he underwent operation of pleuro pericardial window. He was also treated with low fat medium chain triglyceride diet, and temporary clinical improvement occurred. The patient died at 13 years of age because of extensive lymphangiomatosis to abdomen, lungs and bones.