基于临床病例观察永存胎儿眼内血管的发生  

Looking upon genesis of persistent fetal vasculature on account of clinical observation

作　　者：董白霞[1,2] 洪颖[1] 陈慧瑾[1] 马志中[1] 

机构地区：[1]北京大学第三医院眼科教育部重点实验室,北京100191 [2]河北医科大学第二医院眼科

出　　处：《中国实用眼科杂志》2016年第11期1169-1177,共9页Chinese Journal of Practical Ophthalmology

摘　　要：目的通过2例永存胎儿血管（Persistent Fetal Vasculature，PFV）的系统观察进一步理解眼的胚胎发育过程和争议问题。方法典型病例回顾性研究。自2011年2月至2012年1月在北京大学第三医院眼科中心应用术前、术中详细照片记录、录像回放比对、组织病理学观察及文献复习方法对2例典型的PFV病例进行研究，综合分析正常眼的胚胎发育过程，从临床观察有力证据出发探讨现存的争议问题。结果2例典型的PFV眼部体征绝大多数符合Goldberg的系统描述。由虹膜色素上皮（经过瞳孔领）绕过虹膜的基质与角膜内壁发生粘连而形成了先天虹膜囊肿，而且跨越了前房空间，从而为角膜内皮来源于神经外胚叶提供了有力的直接观察依据。扇形网络构象更倾向支持永存瞳孔膜由晶体前血管膜残余形成。但这些网络结构表面均包裹由神经嵴细胞来源的虹膜基质组织。结论角膜内皮与虹膜色素上皮层在胚胎时期有密切联系。角膜内皮和Descemet膜来源于神经外胚叶可能性大。角膜内皮的发生采取由中央向周边的进行方式；来自周围的神经嵴细胞是沿着视杯前面与（发生中）角膜内皮之间的间隙进入发生虹膜基质，能更合理地解释眼前节的发生过程。瞳孔残膜的形成可能是胚胎血管残余与神经嵴细胞相互诱导所形成。Objective To understand fetal development in normal eyes and to illustrate involved controversies through a series of observations in 2 typical persistent fetal vasculature （PFV）. Meth- ods From February 2011 to January 2012 at Peking University Third Hospital, image data prior to surgery and series of photographs during the procedures were carefully collected and reviewed com- paratively with surgical video record. Novel tissue samples obtained from surgeries were observed his- topathologically. With literature review, comprehensive analysis was carried out to explore current con- troversies and illustrate the process of embryonic development. Results Majority of the abnormal signs were concomitant with those that Goldberg described. Pigment epithelium layer of iris detoured over stroma through pupil to adhere with the back surface of cornea, which manifested as an iris cyst. Trace of fetal development supplied a hard evidence that corneal endothelium was originated from neuroectodermal cells. Network profile of persistent pupillary membrane tended to be formed by anterior tunica vasculosa lentis while the remnants were coated by iris stromal tissues which were believed to be originated from neural crest cells. Conclusions Closed relation between corneal endothelium and pigment epithelium of iris at early stage of fetal development is evidenced by clinical observations. Corneal endothelium and descemet＇s membrane are most possibly originated from neuroectodermal and spread with the manner migrated from center to periphery. Neural crest cells go forward to center from periphery via the staggered gap between developing endothelium and ante- rior surface of optic cup to form stroma of iris. Mechanism of persistent pupillary membrane forma- tion could be a result of mutualism between anterior tunica vasculosa lentis and neural crest cells.

关 键 词：永存胎儿血管 角膜内皮 瞳孔残膜 胚胎发育 

分 类 号：R771.02[医药卫生—眼科]