Successful Management of Chronic Chylothorax Secondary to Gorham-Stout Disease

Abstract

Gorham-Scout disease (GSD) is a rare skeletal disorder of unknown etiology characterized by progressive osteolysis and excessive lymphovascular proliferation. Chylothorax is a life-threatening complication. A teenager presented with a left pleural effusion on a background of chronic flank collection secondary to lymphovascular malformation. Cytology of the fluid confirmed a chylothorax and rib excision biopsy confirmed GSD. The patient underwent thoracotomy, decortication, and pleurectomy, which helped to stabilize the chylothorax; ongoing management involved radiotherapy and bisphosphonates. GSD is a rare disease that can develop a number of severe complications including chylothorax. Management via surgical and radiotherapeutic techniques can provide long-term remission.