862 Successful Surgical Management of Chylothorax Secondary to Gorham-Stout Disease

Abstract

Abstract Introduction Gorham-Scout Disease (GSD) is a rare skeletal disorder of unknown aetiology characterised by progressive osteolysis and excessive lymphovascular proliferation. Chylothorax is a severe and life-threatening complication of GSD that confers a poor prognosis. Literature review reveals only one previous case of successful surgical management for chylothorax secondary to GSD. Case presentation A teenage asylum seeker presented with a large left pleural effusion on a background of chronic left flank collection secondary to lymphovascular malformation. Cytological analysis of the fluid drained from chest drain insertion confirmed a chylothorax. A posterior rib excision biopsy helped to confirm the diagnosis of GSD. She had VATS ligation of the thoracic duct and talc pleurodesis, however, due to recollection and ongoing symptomatic discomfort, she subsequently underwent left thoracotomy, decortication of lung and pleurectomy. These surgical interventions helped to stabilise the chylothorax with ongoing management for GSD related to radiotherapy and pain control via the administration of bisphosphonates. Conclusions GSD is a rare disease that can develop a number of severe complications including a chylothorax. Management via surgical and radiotherapeutic techniques can provide long term remission, however, other modalities may be needed in order to optimise analgesic requirements.