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儿童眼眶纤维发育不良。临床特征与治疗

Fibrous dysplasia of the orbit in childhood. Clinical features and management.

作者信息

Moore A T, Buncic J R, Munro I R

出版信息

Ophthalmology. 1985 Jan;92(1):12-20. doi: 10.1016/s0161-6420(85)34071-x.

DOI:10.1016/s0161-6420(85)34071-x
PMID:3974991
Abstract

In 16 children with orbitocranial fibrous dysplasia confirmed histopathologically, facial asymmetry, orbital dystopia, and unilateral proptosis were the main presenting problems. Five children had radiological evidence of narrowing of the optic canal but only two developed optic atrophy and visual loss. One patient had persistent epiphora due to involvement of the bony nasolacrimal duct. Sixteen patients were treated by excision of the dysplastic bone and 15 had immediate craniofacial reconstruction. A good cosmetic and functional result was achieved in the majority of cases although transient vertical diplopia and minor degrees of ptosis occurred postoperatively in about a third. Fibrous dysplasia of the orbit is progressive in childhood and is best managed by radical surgery and reconstruction in a specialized craniofacial unit.

摘要

在16例经组织病理学确诊为眶颅纤维发育不良的儿童中,面部不对称、眼眶移位和单侧眼球突出是主要的临床表现。5例儿童有视神经管狭窄的影像学证据,但只有2例出现视神经萎缩和视力丧失。1例患者因骨性鼻泪管受累而持续溢泪。16例患者接受了发育异常骨切除术,15例患者立即进行了颅面重建。尽管约三分之一的患者术后出现短暂性垂直复视和轻度上睑下垂,但大多数病例都取得了良好的美容和功能效果。眼眶纤维发育不良在儿童期呈进行性发展,最好在专业的颅面外科单位进行根治性手术和重建治疗。

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Fibrous dysplasia of the orbit in childhood. Clinical features and management.儿童眼眶纤维发育不良。临床特征与治疗
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