Gorham's syndrome: a usually fatal cause of pleural effusion treated successfully with radiotherapy

• Published in: Thorax Vol. 51; no. 12; pp. 1275 - 1276
• Main Authors: McNeil, K. D., Fong, K. M., Walker, Q. J., Jessup, P., Zimmerman, P. V.
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and British Thoracic Society 01-12-1996; BMJ; BMJ Publishing Group LTD
• Subjects: Adult; Biological and medical sciences; Diseases of the osteoarticular system; Humans; Male; Medical sciences; Miscellaneous. Osteoarticular involvement in other diseases; Osteolysis, Essential - complications; Osteolysis, Essential - radiotherapy; Pleural Effusion - complications; Pleural Effusion - radiotherapy; Case study; Human; Treatment; Effusion; Respiratory disease; Gorham idiopathic osteolysis; Diseases of the osteoarticular system; Complication; Bone disease; Rib(bone); Radiotherapy; Pleura
• ISSN: 0040-6376; 1468-3296
• DOI: 10.1136/thx.51.12.1275

A 21 year old man presented with a right sided pleural effusion. Destruction of the 11th and 12th right ribs and adjacent vertebral bodies was noted on computed tomographic scanning. An open rib biopsy revealed the histopathological changes of Gorham's syndrome. In view of the progressive vertebral destruction and inevitable spinal cord compromise, he was treated with high dose radiotherapy. The process was arrested and he remains well with no signs of recurrence after four years. Pleural effusion and vertebral destruction complicating Gorham's syndrome carry a poor prognosis but, in this case, high dose radiotherapy has been effective in controlling both the effusion and the progressive bony destruction.