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McCune-Albright Syndrome: A Case Report
McCune-Albright症候群：病例報告
摘要
一個九歲女孩，在跌倒後發生左大腿急性劇烈疼痛，有X光發現在左側大腿骨頸部有一具有硬化性邊緣的骨溶性病變，骨幹部有一很長的螺旋型骨折。此外在左側經骨的骨幹有一大的骨溶性病變。身體其他部位的骨頭並沒有任何病變。手術治療骨折處並將附近的骨髓送病理分析，病理報告是纖維性發育不良。病患理學檢查可見右側腰部皮膚有咖啡色斑點乳房已發育並有陰毛。在過往史方面，她從一歲開始便有陰道出血。因此臨床診斷為McCune-Albright症狀群。我們建議性早熟的女性必需接受長時問的追蹤檢查以及早發現骨頭可能的纖維性發育不良及其併發症，而病人也必需儘量避免受傷以減少骨折的發生率。
並列摘要
A nine-year-old girl suffered from swelling and acute severe pain in her left thigh one day after a fall. The patient had had vaginal bleeding since she was one year old as well as some cafe-au-lait spots in her right flank area. Developed breast and pubic hairs were also noted as well as low serum gonadotropin levels. Bilateral ovarian cysts had been noted when she was 3 years old. After the accident, frontal and lateral radiographs of left femur showed an eccentric osteolytic lesion with sclerotic border over the medial aspect of the left femoral neck and a long spiral fracture over the left femoral diaphysis. Another osteolytic lesion with endosteal erosion over the diaphysis of the left tibia was also noted. No other definite osseous lesion was found on bone survey films. Open reduction and internal fixation (ORIF) with plate and screws were performed for the fracture. Curettage of the adjacent bone marrow and biopsy of the lesions at femur neck and tibia were also performed during the operation. The pathologic report of these lesions were fibrous dysplasia. Indications are suggested that females with precocious puberty should be followed over a period of years for the possible appearance of the fibrous dysplasia of bone and complication of abnormal bone. As far as possible the patient should be careful to avoid even minor trauma to reduce fracture incidence due to the fragility of the abnormal bone.