Roka Yam Bahadur, Paudel Gunraj, Khatri Bidur, Munakomi Sunil
B.P.Koirala Institute of Health Sciences, Department of Surgery, Dharan, Nepal.
Turk Neurosurg. 2010 Oct;20(4):508-11. doi: 10.5137/1019-5149.JTN.2387-09.2.
The McCune-Albright syndrome was described as a syndrome of polyostotic fibrous dysplasia, café au lait skin pigmentation, and autonomous endocrine hyperfunction in 1937. We report a 17-year girl with early menarche and accelerated growth for the past three years. The endocrinological examination showed slight rise of growth hormone with other hormones in normal range. The CT showed an expansive bony lesion over the left parietal area. The bone mass was excised with bone cement cranioplasty performed for the defect. Histology confirmed it was fibrous dysplasia. Although uncommon, this syndrome must be kept in mind in cases with bony abnormalities and extensive endocrinological workup done and treatment given for best results.
1937年,McCune - Albright综合征被描述为一种伴有多骨纤维发育不良、牛奶咖啡斑皮肤色素沉着和自主性内分泌功能亢进的综合征。我们报告一名17岁女孩,初潮早且在过去三年中生长加速。内分泌检查显示生长激素略有升高,其他激素水平正常。CT显示左顶叶区域有一个膨胀性骨病变。切除骨块后用骨水泥颅骨成形术修复缺损。组织学证实为纤维发育不良。尽管这种综合征不常见,但对于有骨异常的病例,必须进行全面的内分泌检查并给予治疗,以获得最佳效果。