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Early indirect laser photocoagulation to induce regression of retinal vascular abnormalities in incontinentia pigmenti

机译:早期间接激光光凝诱导色素性尿失禁的视网膜血管异常消退

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摘要

An 18-day-old female infant with a diagnosis of incontinentia pigmenti, which had been confirmed by biopsy findings from a hyperpig-mented bullous skin rush on the anterior surface of both lower extremities (Fig. 1), was referred to our clinic for retinal evaluation. She weighed 3220 g at birth and had been born at 41 weeks gestation. Initial retinal examination with scleral depression of the right eye revealed a pale optic nerve, and markedly dilated and tortuous retinal vessels with an abrupt termination within the posterior pole, extending three disc areas temporally and inferiorly, two disc areas nasally and one disc area superiorly to the disc (Fig. 2A). The peripheral retina beyond the anastomotic ring was completely avascular. These findings resembled those of zone 1 aggressive posterior retinopathy of prematurity. Retinal examination of the left eye revealed a normal disc and macula with mild dilatation of the retinal veins at the posterior pole (Fig. 2B). These vessels terminated beyond the major vascular arcades. There appeared to be vascular anastomoses throughout 360 degof the retina. The peripheral retina was avascular and demonstrated an ischaemic appearance with haemorrhages. There was no evidence of retinal detachment in either eye.
机译:经两个下肢前表面色素沉着的大疱性皮肤色素沉着的活检发现,已确诊患有色素失禁的18岁女婴(图1),已转诊至我们的诊所视网膜评估。她出生时重3220克,出生于妊娠41周。最初的视网膜检查伴右眼巩膜下陷显示视神经苍白,视网膜血管明显扩张和曲折,后极内突然终止,在时间上和下方延伸三个椎间盘区域,向后延伸两个椎间盘区域,一个椎间盘上方光盘(图2A)。吻合环以外的周围视网膜完全无血管。这些发现与1区早产型侵袭性后视网膜病变相似。左眼的视网膜检查显示椎间盘和黄斑正常,后极视网膜静脉轻度扩张(图2B)。这些血管终止于主要的血管拱廊之外。整个视网膜360度出现血管吻合。周围视网膜无血管,并表现出缺血性外观和出血。两只眼睛都没有视网膜脱离的迹象。

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