Mendez A A, Keret D, Robertson W, MacEwen G D
Alfred I. duPont Institute, Wilmington, Delaware.
J Pediatr Orthop. 1989 Sep-Oct;9(5):604-8. doi: 10.1097/01241398-198909010-00019.
A case of Gorham's disease involving the proximal femur of a 12-year-old girl is presented. Although Gorham's disease is rare and has benign histologic characteristics, in this patient it took an aggressive clinical course that resulted in severe morbidity. Diagnosis is difficult to establish; almost all investigative approaches provide only indirect evidence of the pathology. A high index of suspicion and open biopsy performed soon after presentation will establish diagnosis. Treatment modalities may be more effective if applied earlier in the disease process, especially as the osteolytic process appears to escalate after pathologic fractures, which occur frequently in this disorder.
本文报告了一例发生在一名12岁女孩近端股骨的戈勒姆病。尽管戈勒姆病罕见且具有良性组织学特征，但该患者的临床病程呈侵袭性，导致了严重的发病率。诊断很难确立；几乎所有的检查方法都只能提供病理学的间接证据。高度怀疑并在就诊后尽早进行开放活检可确立诊断。如果在疾病过程中更早应用治疗方式可能会更有效，尤其是因为溶骨过程在病理性骨折后似乎会加剧，而病理性骨折在这种疾病中经常发生。
