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晶状体半脱位合并 Parry-Romberg 综合征:病例报告。

Lens subluxation combined with parry-romberg syndrome: case report.

机构信息

Department of Ophthalmology and Eye Research Institute, Eye and ENT Hospital, Fudan University, 200031, Shanghai, China.

NHC Key Laboratory of Myopia (Fudan University), Key Laboratory of Myopia, Chinese Academy of Medical Sciences, 200031, Shanghai, China.

出版信息

BMC Ophthalmol. 2024 May 13;24(1):210. doi: 10.1186/s12886-024-03457-y.

Abstract

BACKGROUND

Parry-Romberg syndrome (PRS) is a rare progressive degenerative disorder of unknown etiology. Here we report a rare case of PRS combined with lens subluxation in Eye and ENT hospital of Fudan University, Shanghai. To our knowledge, it is the first reported case of PRS combined with lens subluxation that has been managed surgically with phacoemulsification and CTR placement and IOL implantation in Shanghai.

CASE PRESENTATION

A 60-year-old woman was referred for "right visual blur for 2 years" and had persistent right facial paralysis of unknown etiology since the age 12. She had right facial muscle atrophy and paralysis. Eye examination also showed the right eyelid pseudoptosis, enophthalmos, age-related cataract combined with lens subluxation existed in the right eye. The patient was diagnosed as age-related cataract and lens subluxation in the right eye and progressive hemifacial atrophy (Parry-Romberg syndrome). We conducted a combined phacoemulsification, IOL and CTR implantation and pupilloplasty surgery for the patient under general anesthesia and the postoperative UCVA was 20/30 and remained for 1 year's follow up.

CONCLUSIONS

Here we reported a rare case of PHA combined with lens subluxation in China. After appropriate eye surgery, the patient achieved satisfying vision result in the right eye.

摘要

背景

Parry-Romberg 综合征(PRS)是一种病因不明的罕见进行性退行性疾病。本文报告了复旦大学眼耳鼻喉科医院收治的一例 PRS 合并晶状体半脱位的罕见病例。据我们所知,这是上海首例经超声乳化白内障吸除术联合后房型人工晶状体植入术和房角支撑型巩膜扣带术治疗 PRS 合并晶状体半脱位的病例。

病例介绍

一名 60 岁女性因“右眼视力模糊 2 年”就诊,12 岁时不明原因出现持续性右侧面瘫,目前右侧面部肌肉萎缩伴瘫痪。眼部检查发现右眼假性上睑下垂、眼球内陷、年龄相关性白内障合并晶状体半脱位。患者被诊断为右眼年龄相关性白内障和晶状体半脱位及进行性半侧颜面萎缩(Parry-Romberg 综合征)。我们在全麻下为患者行白内障超声乳化吸除术、人工晶状体和房角支撑型巩膜扣带术联合瞳孔成形术,术后右眼最佳矫正视力(UCVA)为 20/30,并随访 1 年。

结论

本文报告了中国首例 PRS 合并晶状体半脱位的病例。经适当的眼部手术治疗后,患者右眼视力获得满意的结果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e21/11089757/375ab64e87aa/12886_2024_3457_Fig1_HTML.jpg

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