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McCune-Albright综合征中的视神经病变:积极治疗的指征

Optic neuropathy in McCune-Albright syndrome: an indication for aggressive treatment.

作者信息

Bocca G, de Vries J, Cruysberg J R, Boers G H, Monnens L A

机构信息

Department of Paediatrics, University Hospital Nijmegen St Radboud, The Netherlands.

出版信息

Acta Paediatr. 1998 May;87(5):599-600. doi: 10.1080/08035259850158371.

Abstract

McCune-Albright syndrome consists of the triad polyostotic fibrous dysplasia, café-au-lait spots and autonomous hyperfunction of one or more endocrine systems. The most frequent neurological complication of craniofacial fibrous dysplasia is visual loss. We describe a 17-y-old boy with McCune-Albright syndrome and acute loss of vision in the left eye caused by encroachment of the left optic nerve by fibrous dysplastic lesions. Neurosurgical intervention improved left eye vision. Aggressive intervention is indicated in cases of acute visual loss in patients with craniofacial fibrous dysplasia. This is supported by a review of other reported cases.

摘要

McCune-Albright综合征由多骨型纤维性发育不良、咖啡斑和一个或多个内分泌系统的自主性功能亢进三联征组成。颅面骨纤维性发育不良最常见的神经并发症是视力丧失。我们描述了一名17岁男孩,患有McCune-Albright综合征,因纤维发育异常病变侵犯左侧视神经导致左眼急性视力丧失。神经外科干预改善了左眼视力。对于颅面骨纤维性发育不良患者出现急性视力丧失的情况,应采取积极干预措施。其他报道病例的综述支持了这一点。

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