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[小眼症及其致病分类]

[Microphthalmos and its pathogenic classification].

作者信息

Majima A

机构信息

Department of Ophthalmology, Nagoya City University Medical School, Aich-ken, Japan.

出版信息

Nippon Ganka Gakkai Zasshi. 1994 Dec;98(12):1180-200.

PMID:7832065
Abstract

Congenital microphthalmos is a common malformation encountered clinically. Microphthalmos in adults is here defined as eyes whose axial length is below 20.4 mm in males and 20.1 mm in females; in under 14-year-old children, it is eyes at least 3 square root of 2/3 below the mean for age-similar controls. Experimental animals with hereditary microphthalmos have been widely investigated, and many environmental factors given to pregnant animals frequently induce microphthalmos. In both clinical and experimental microphthalmos, there are conspicuous variations in size, and various kinds of ocular and systemic complications. Recently, fetal alcohol syndrome produced by alcohol intake during pregnancy has been reported. In this syndrome, microphthalmos is one of the important symptoms. Experimentally, microphthalmos also developed at a high incidence among mouse fetuses whose mothers were given ethanol during pregnancy. The present investigator established a preliminary etiological classification of microphthalmos in 1984. In this paper summing up newly obtained results, the relationship to neural crest cells and histochemical changes of glycosaminoglycan molecular species, the author presents a final pathogenic classification of microphthalmos, which consists of developmental disturbance of the optic vesicle, malformation of the optic cup, mesenchymal dysgenesis of the anterior ocular segment, maldevelopment of the lens, maldevelopment of the vitreous, faulty closure of the embryonic fissure and developmental disturbance of the wall of eyeball.

摘要

先天性小眼畸形是临床上常见的一种畸形。本文将成年人的小眼畸形定义为男性眼轴长度低于20.4毫米、女性低于20.1毫米的眼睛;对于14岁以下儿童,是指至少比年龄相仿的对照均值低3√2/3的眼睛。遗传性小眼畸形的实验动物已得到广泛研究,给怀孕动物施加的许多环境因素常常会诱发小眼畸形。在临床和实验性小眼畸形中,眼睛大小存在显著差异,并且有各种眼部和全身并发症。最近,有报道称孕期饮酒会导致胎儿酒精综合征。在这种综合征中,小眼畸形是重要症状之一。在实验中,孕期给母鼠喂食乙醇,其后代小鼠胎儿也有很高的小眼畸形发生率。本研究者在1984年建立了小眼畸形的初步病因分类。在本文总结新获得的结果时,作者阐述了小眼畸形与神经嵴细胞的关系以及糖胺聚糖分子种类的组织化学变化,提出了小眼畸形的最终致病分类,包括视泡发育障碍、视杯畸形、眼前节间充质发育异常、晶状体发育不良、玻璃体发育不良、胚胎裂闭合不全以及眼球壁发育障碍。

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