Tawara Akihiko, Itou Ken, Kubota Toshiaki, Harada Yukinori, Tou Norihiko, Hirose Naofumi
Department of Ophthalmology, School of Medicine, University of Occupational and Environmental Health, Japan.
J Glaucoma. 2005 Oct;14(5):409-13. doi: 10.1097/01.ijg.0000176931.29477.6e.
To demonstrate that developmental glaucoma (instead of the term goniodysgenetic glaucoma is used in this paper), defined as glaucoma with goniodysgenesis resulting from a fetal maldevelopment of the iridocorneal angle, develops in association with congenital microcoria.
Three subjects descended from a family with autosomal dominant congenital microcoria and goniodysgenesis were followed up for more than 25 years.
The extended family consisted of 3 generations including 8 males and 10 females. In the second generation, 2 of 7 subjects who presented with a history of congenital microcoria had late-onset goniodysgenetic glaucoma. In the third generation, all 3 descendants of the second generation subjects with congenital microcoria had congenital microcoria with goniodysgenesis. Two of these subjects developed late-onset goniodysgenetic glaucoma in both eyes during the 25-years follow-up period. They were both treated with a trabeculectomy in both eyes to control the glaucoma. Histologically, the iridocorneal angle tissues from the patients showed thick juxtacanalicular connective tissue with accumulations of a basement membrane-like extracellular matrix.
Congenital microcoria are considered to be frequently associated with the incidence of late-onset goniodysgenetic glaucoma.
证实发育性青光眼（本文使用该术语而非房角发育异常性青光眼），即因虹膜角膜角胎儿发育异常导致房角发育异常的青光眼，与先天性小角膜相关。
对3名来自常染色体显性遗传性先天性小角膜和房角发育异常家族的受试者进行了超过25年的随访。
这个大家庭包括3代人，有8名男性和10名女性。在第二代中，7名有先天性小角膜病史的受试者中有2人患迟发性房角发育异常性青光眼。在第三代中，第二代有先天性小角膜的受试者的所有3名后代均有先天性小角膜伴房角发育异常。在25年的随访期间，其中2名受试者双眼均发生迟发性房角发育异常性青光眼。他们均接受了双眼小梁切除术以控制青光眼。组织学上，患者的虹膜角膜角组织显示近管周结缔组织增厚，伴有基底膜样细胞外基质积聚。
先天性小角膜被认为常与迟发性房角发育异常性青光眼的发病相关。
