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小角膜、后部大圆锥角膜、永存原始玻璃体增生症、脉络膜视网膜缺损(MPPC)综合征:玻璃体切除术后病例系列

Microcornea, posterior megalolenticonus, persistent fetal vasculature, chorioretinal coloboma (MPPC) syndrome: Case series post vitrectomy.

作者信息

Rothfield Lindsay D, Cernichiaro-Espinosa Linda A, Alabiad Chrisfouad R, McKeown Craig A, Tran Kimberly, Chang Ta C, Berrocal Audina M

机构信息

University of Miami Miller School of Medicine, 1600, NW 10th Ave, Miami, FL, United States.

Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, 900 NW 17th St, Miami, FL, United States.

出版信息

Am J Ophthalmol Case Rep. 2019 Jan 25;14:5-9. doi: 10.1016/j.ajoc.2019.01.005. eCollection 2019 Jun.

DOI:10.1016/j.ajoc.2019.01.005
PMID:30766938
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6360246/
Abstract

PURPOSE

MPPC syndrome has been described as a syndrome that presents with chorioretinal coloboma, posterior megalolenticonus, persistent fetal vasculature, and chorioretinal coloboma. The purpose of our study is to report three patients who present with a variation of MPPC syndrome who each underwent pars plana vitrectomy, pars plana lensectomy, and amblyopic management. Clinical characteristics, ancillary test findings, and post-surgical functional results are compared to what is reported in the literature.

METHODS

Retrospective review of medical records of patients who presented with microcornea, persistent fetal vasculature, chorioretinal coloboma, and microphthalmia who underwent surgical correction at Bascom Palmer Eye Institute.

RESULTS

3 patients (6 eyes) were included, two males and one female. All patients were born full term, vaginally, and had no family history of genetic abnormalities affecting the eye. All patients had color fundus images, fluorescein angiography (FA) and echography. Four eyes underwent surgery. Following surgical intervention, patients demonstrated improved visual acuity, and improved functional status.

CONCLUSION AND IMPORTANCE

Patients with microcornea, PFV, chorioretinal coloboma, and microphthalmos, can benefit from surgical intervention when functional decline or media opacities are noted. Though difficult to assess accurate visual acuity and visual improvement pre-operatively and post-operatively, it is evident that our patients demonstrated improvement in functionality and vision following surgical intervention.

摘要

目的

MPPC综合征被描述为一种伴有脉络膜视网膜缺损、后部巨大圆锥晶状体、永存原始玻璃体增生症和脉络膜视网膜缺损的综合征。我们研究的目的是报告3例表现为MPPC综合征变异型的患者,他们均接受了玻璃体切割术、晶状体切除术和平视弱视治疗。将临床特征、辅助检查结果和术后功能结果与文献报道进行比较。

方法

回顾性分析在巴斯科姆·帕尔默眼科研究所接受手术矫正的患有小角膜、永存原始玻璃体增生症、脉络膜视网膜缺损和小眼球的患者的病历。

结果

纳入3例患者(6只眼),2例男性,1例女性。所有患者均足月顺产,且无影响眼睛的遗传异常家族史。所有患者均进行了彩色眼底图像、荧光素血管造影(FA)和超声检查。4只眼接受了手术。手术干预后,患者视力提高,功能状态改善。

结论及意义

患有小角膜、永存原始玻璃体增生症、脉络膜视网膜缺损和小眼球的患者,当出现功能下降或介质混浊时,可从手术干预中获益。尽管术前和术后难以准确评估视力和视力改善情况,但显然我们的患者在手术干预后功能和视力得到了改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8881/6360246/8253296692b9/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8881/6360246/701e47fa143e/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8881/6360246/8253296692b9/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8881/6360246/701e47fa143e/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8881/6360246/8253296692b9/gr2.jpg

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