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一例伴有牵牛花碟异常和早产儿视网膜病变样视网膜病变的先天性永存原始玻璃体增生症。

A case of anterior persistent hyperplastic primary vitreous associated with morning glory disc anomaly and retinopathy of prematurity like retinopathy in a term-born child.

机构信息

Department of Ophthalmology, West China Hospital, Sichuan University, No.37, Guoxue xiang, Chengdu, 610041, Sichuan, China.

出版信息

BMC Ophthalmol. 2021 Dec 28;21(1):455. doi: 10.1186/s12886-021-02200-1.

Abstract

BACKGROUND

Association of morning glory disc anomaly (MGDA) with persistent hyperplastic primary vitreous (PHPV) has been reported earlier. Retinopathy of prematurity (ROP) like retinopathy in preterm babies with optic disc anomalies has also been published. Our case is unique in terms of presence MGDA, PHPV, unilateral ROP like retinopathy in a term infant with normal birth weight.

CASE PRESENTATION

A 5-month-old girl, born at term with a birth weight of 3750 g, presented with anterior PHPV, MGDA and ROP like retinopathy. In order to prevent retinal detachment, she received 360 degree barrage laser photocoagulation at the edge of the optic disc excavation of the left eye. In the follow-up a month later, laser scars were found in her left fundus without other complications.

CONCLUSION

PHPV and MGDA with ROP like retinopathy in term and normal weight baby is rare. The peripheral avascular retinal area, caused by the dragging of the defected optic disc, might have been more vulnerable to the oxygen change after birth which resulted in ROP like retinopathy. High sensitivity to oxygen results in a series of changes such as upregulation of VEGF and IGF-1 may cause ROP-like retinopathy.

摘要

背景

先前已有研究报道,牵牛花综合征(MGDA)与永存性原始玻璃体增生症(PHPV)之间存在关联。早产儿视盘异常的早产儿视网膜病变(ROP)也曾有过报道。我们的病例在足月出生、体重正常的婴儿中出现 MGDA、PHPV、单侧 ROP 样视网膜病变,具有独特性。

病例介绍

一名 5 月龄女婴,足月出生,体重 3750g,表现为前 PHVP、MGDA 和 ROP 样视网膜病变。为防止视网膜脱离,左眼行视盘凹陷边缘 360 度格栅状激光光凝。一个月后随访时,发现左眼眼底有激光瘢痕,无其他并发症。

结论

足月出生、体重正常的婴儿中出现 PHPV 和 MGDA 伴 ROP 样视网膜病变较为罕见。缺损视盘牵拉所致的周边无血管视网膜区域可能在出生后对氧变化更为敏感,从而导致 ROP 样视网膜病变。对氧的高敏感性导致一系列变化,如 VEGF 和 IGF-1 的上调,可能导致 ROP 样视网膜病变。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/edf2/8715584/d5d77e2c7200/12886_2021_2200_Fig1_HTML.jpg

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