Chylothorax associated with massive osteolysis (Gorham's syndrome)

Abstract

We report a 15-year-old boy with bilateral chylothorax complicating Gorham's syndrome. Thoracic duct ligation failed to prevent fluid reaccumulation. The patient died of lymphocytopenia. Autopsy revealed vascular proliferation in the parietal and visceral pleura as well as in the ribs, which seemed to cause persistent chylothorax responsible for the poor prognosis of this patient. We reviewed treatments provided to 22 patients reported in the literature with Gorham's syndrome and chylothorax.