Bilateral chylothorax complicating massive osteolysis (Gorham's syndrome)

Abstract

Chylothorax may rarely occur in osteolysis. A fatal case of bilateral chylothorax complicating massive osteolysis is described and the pathogenesis and management are discussed. (Thorax 1996;51:1277-1278 Keywords: chylothorax, massive osteolysis, Gorham's syndrome. His respiratory compromise necessitated repeated aspirations and, subsequently, bilateral intercostal tube drainage. Gastrointestinal rest and total parenteral hyperalimentation were promptly instituted. Lymphangiography showed normal lymph channels in the inguinal and lumbar areas, but areas ofminimal contrast leakage were noted in the upper and lower part of the left thoracic cavity; however, the thoracic duct was not visualised on the 24 and 48 hour films. Subsequent chest radiographs showed progressive resorption of the left first to third ribs, left transverse processes of the first to third thoracic vertebrae, part of the body of the seventh cervical vertebra, spinous process of the left scapula, and the right first and second ribs ( figure). Division of MedicineDespite maximal nutritional support his condition deteriorated. Chest tube drainage persisted at a rate of approximately 1500 ml/day. By the third week a diagnosis of massive osteolysis was considered and open thoracotomy with a view to thoracic duct ligation was planned. However, the patient rapidly de- teriorated and died the following day. Necroscopic examination of the affected ribs showed enlargement of the marrow spaces which were completely replaced by numerous lymphatic vessels.

Gorham-Stout Syndrome with Chylothorax in a Six-Year-Old Boy

“…Treatment modalities in GSS with chylothorax include chesttube drainage, pleurectomy, pleurodesis, thoracic duct ligation, radiation therapy, administration of corticosteroids, alpha-2b interferon and biphosphanates (clodronate, pamidronate) [3,4,6,[8][9][10]. In the present case, thoracic duct ligation and pleurectomy was done to relieve the symptoms associated with chylothorax.…”

“…Clodranate was used because of its anti-osteoclastic activity [3,4,9]. Bilateral chylothorax as a complication of Gorham syndrome is almost always fatal, with death usually occurring from malnutrition, lymphopenia, and superimposed infection [3,8]. The present case poorly responsed to mentioned therapies and died due to respiratory insufficiency and sepsis 4 months after the diagnosis.…”

Chylothorax in Gorham's Disease

“…However, the Gorham's disease should be included in differential diagnosis of chylothorax, especially when it is associated with osteolytic lesion. There have been about 30 cases of chylothorax complicating Gorham's disease in the English literature (4,8,9).…”

Pleural Effusion in Gorham-Stout Syndrome: A Case Report and a Literature Review

“…Most of the time pleural effusion in GSS requests several thoracentesis or a chest drainage inserction by the reason of quick relapses. Even several liters of chylous liquid can be produced in a few days conditioning malnutrition [9,12,26,27,30,32]. Frequently this complication necessitates a rapid parenteral nutritional support and human albumin replacement.…”