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马凡综合征复发性自发性巩膜破裂

Recurrent spontaneous scleral rupture in Marfan's syndrome.

作者信息

Turaga Kiranmaye, Senthil Sirisha, Jalali Subhadra

机构信息

Department of Glaucoma, L V Prasad Eye Institute, Hyderabad, Andhra Pradesh, India.

Department of Retina and Vitreous Services, LV Prasad Eye Institute, Hyderabad, Telangana, India.

出版信息

BMJ Case Rep. 2016 May 19;2016:bcr2016214764. doi: 10.1136/bcr-2016-214764.

Abstract

The ocular manifestations of Marfan's syndrome (MS) range from ectopia lentis, microspherophakia, myopia, glaucoma and retinal detachment. Spontaneous scleral rupture is a rare complication and recurrent scleral perforation is extremely rare. We report a rare case of a 26-year-old male with MS who had sequential recurrent spontaneous scleral rupture which required surgical repair. He suffered from a similar problem 4 years later in both eyes in a different location, with overlying thin cystic blebs and hypotony maculopathy. Surgical repair with preserved scleral donor patch graft and conjunctival autograft in one eye, and conjunctival advancement in the other eye was performed. This helped stabilise the eyes, and resulted in complete visual recovery in both eyes.

摘要

马凡综合征(MS)的眼部表现包括晶状体异位、小球形晶状体、近视、青光眼和视网膜脱离。自发性巩膜破裂是一种罕见的并发症,而复发性巩膜穿孔极为罕见。我们报告一例罕见病例,一名26岁患有MS的男性先后出现复发性自发性巩膜破裂,需要手术修复。4年后,他双眼在不同部位出现类似问题,伴有上方薄的囊性泡和黄斑部低眼压病变。对一只眼进行了保留巩膜供体补片移植和自体结膜移植的手术修复,另一只眼进行了结膜推进术。这有助于稳定眼球,并使双眼视力完全恢复。

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Recurrent spontaneous scleral rupture in Marfan's syndrome.马凡综合征复发性自发性巩膜破裂
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引用本文的文献

本文引用的文献

1
Current concepts of ocular manifestations in Marfan syndrome.马凡综合征眼部表现的当前概念。
Surv Ophthalmol. 2006 Nov-Dec;51(6):561-75. doi: 10.1016/j.survophthal.2006.08.008.
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Revised diagnostic criteria for the Marfan syndrome.马凡综合征的修订诊断标准。
Am J Med Genet. 1996 Apr 24;62(4):417-26. doi: 10.1002/(SICI)1096-8628(19960424)62:4<417::AID-AJMG15>3.0.CO;2-R.

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