颅骨纤维结构不良的临床特征及治疗

Clinical features and treatments of fibrous dysplasia of the skull

目的总结颅骨纤维结构不良的临床特征及治疗情况,以便更好地诊治本病。方法回顾性总结我院收治的39例颅骨纤维结构不良者的临床资料。将获得的数据及随访结果进行统计学检验。结果39例中发病高峰年龄为8～18岁,平均21.26岁;病变位于额前颅底者26例,此部位病变常出现视力下降。视力下降与病变部位、病变生长方式、病程长短有关。手术效果与病变切除程度、术前视力有关。结论本病应早期诊断;视神经管减压可阻止视力的进行性下降;手术可以改善外形并消除头痛。

Objective To summarize the clinical features and treatments of fibrous dysplasia of the skull to improve the diagnosis and treatments. Methods Information of all the 39 patients with definite diagnosis and hospitalized to our hospital from Nov 1, 1997 to Feb 1, 2004 were collected and analyzed. Results The peak age of onset was 8～18 years old, average 21.26 years; the most common diseased region was fronto-anterior skull base （26/39）, and was always accompanied with visual acuity degression, The visual acuity degression was associated with diseased region, vegetution style and length of the course. Operation outcome was associated with pro-operation, visual acuity and the resected extent of the affected region. Conclusions Earlier diagnosis is essential. Decompression of optic canal can prevent the vision loss process and operation can improve the outline appearance and abolish headaches.