Vigorita V J, Magitsky S, Bryk E
Department of Orthopaedic Surgery, St Vincent's Catholic Medical Center, New York, NY, USA.
Clin Orthop Relat Res. 2006 Oct;451:267-73. doi: 10.1097/01.blo.0000223990.49400.7e.
We present the case report of a 35-year-old man with Gorham's disease (disappearing bone disease, massive osteolysis) with initial clinical findings of small bowel lymphangiomatosis and multicentric osteolysis. The patient, who otherwise was healthy, had a chylothorax develop and he died 9 months later of thoracic and pulmonary complications. An autopsy revealed absence of the proximal thoracic duct and significant lymphangiectatic abnormalities of the pleural, peritoneal, diaphragmatic, splenic, and small bowel tissue with lymphangiomatous masses in the thoracic and mediastinal regions. The findings suggest a subtype of Gorham's disease characterized by a dysplastic lymphatic system. Osteolysis was correlated anatomically with lymphangiectatic tissue, suggesting mediation of osteoclastic resorption via local lymphatic tissue factors.
我们报告了一例35岁男性戈谢病（消失性骨病，大量骨质溶解）病例，其最初临床表现为小肠淋巴管瘤病和多中心骨质溶解。该患者原本身体健康，随后出现乳糜胸，9个月后因胸部和肺部并发症死亡。尸检显示胸段近端淋巴管缺如，胸膜、腹膜、膈肌、脾脏和小肠组织存在明显的淋巴管扩张异常，胸部和纵隔区域有淋巴管瘤样肿块。这些发现提示戈谢病的一种亚型，其特征为发育异常的淋巴系统。骨质溶解在解剖学上与淋巴管扩张组织相关，提示破骨细胞吸收是通过局部淋巴组织因子介导的。
