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伴有玻璃体视网膜病变的遗传性前部巨眼症:一项手术挑战。

Hereditary anterior megalophthalmos with posterior vitreoretinopathy: a surgical challenge.

作者信息

Rao Aparna, Videkar Chetan

机构信息

Department of Glaucoma, LV Prasad Eye Institute, Bhubaneswar, Odisha, India.

出版信息

BMJ Case Rep. 2014 Mar 14;2014:bcr2013202212. doi: 10.1136/bcr-2013-202212.

Abstract

Anterior megalophthalmos is an inherited condition characterised by enlargement of the anterior segment with associated glaucoma and vitreoretinopathy. These associations make surgical management very challenging. A 12-year-old boy, diagnosed and operated elsewhere for congenital glaucoma in both eyes, presented with raised intraocular pressure. Investigations revealed enlarged corneal diameter, increased anterior chamber depth with thick, clear cornea and no Haab striae while fundus evaluation revealed posteriorly dislocated cataractous lens with total rhegmatogenous retinal detachment in both eyes. The surgical challenges in management are highlighted in this case where appropriate investigations can aid in the selection of appropriate surgeries with good visual outcome.

摘要

前部巨眼症是一种遗传性疾病,其特征为眼前节增大,并伴有青光眼和玻璃体视网膜病变。这些关联使得手术治疗极具挑战性。一名12岁男孩,曾在其他地方被诊断为双眼先天性青光眼并接受手术,现出现眼压升高。检查发现角膜直径增大、前房深度增加、角膜厚且透明,无哈布条纹,而眼底检查显示双眼晶状体后脱位并伴有完全性孔源性视网膜脱离。本病例突出了治疗中的手术挑战,在此病例中,适当的检查有助于选择合适的手术,从而获得良好的视觉效果。

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