McNeil K D, Fong K M, Walker Q J, Jessup P, Zimmerman P V
Department of Thoracic Medicine, Prince Charles Hospital, Chermside, Brisbane, Australia.
Thorax. 1996 Dec;51(12):1275-6. 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.
一名21岁男性出现右侧胸腔积液。计算机断层扫描显示右侧第11和12肋骨及相邻椎体破坏。开放性肋骨活检显示为戈勒姆病综合征的组织病理学改变。鉴于椎体进行性破坏及不可避免的脊髓受压,对其进行了高剂量放疗。病情得到控制,四年后他情况良好,无复发迹象。戈勒姆病综合征合并胸腔积液和椎体破坏预后较差,但在此病例中,高剂量放疗有效控制了积液和进行性骨质破坏。