先天性瞳孔-虹膜-晶状体膜伴前房角发育不良  

Congenital pupillary-iris-lens membrane with goniodysgenesis

作　　者：杨雪莹 陈长征[1] Yang Xueying;Chen Changzheng(Department of Ophthalmology,Renmin Hospital of Wuhan University,Wuhan 430060,China)

机构地区：[1]武汉大学人民医院眼科中心,430060

出　　处：《国际眼科纵览》2021年第5期415-419,共5页International Review of Ophthalmology

摘　　要：先天性瞳孔-虹膜-晶状体膜伴前房角发育不良(congenital pupillary-iris-lens membrane with goniodysgenesis,CPILMG)是一种罕见的眼前节疾病,典型表现为针尖大小的瞳孔、持续生长的瞳孔膜和前房角发育不全,常单眼发病,无家族遗传性。患者通常伴有遮蔽瞳孔区的白膜和虹膜膨隆,易出现形觉剥夺性弱视和闭角型青光眼。其主要治疗方式为手术治疗,少部分深前房和瞳孔膜能自发消退的患者可随访观察。目前关于CPILMG多为病例报道,临床上易与Axenfeld-Rieger综合征和永存胎儿血管系统等疾病混淆,详尽了解CPILMG的临床表现和鉴别诊断,将有助于明确临床诊疗评估。未来需要大样本研究深入对CPLIMG疾病发展的不同时期对应的发病机制和治疗方式进行探索,进一步提高对此病的认识。Congenital pupillary-iris-lens membrane with goniodysgenesis(CPILMG)is a rare anterior segment disease,typically manifested as a pinpoint-sized pupil,continuously growing pupil membrane and goniodysgenesis.CPILMG usually has monocular disease and has no family heredity.Patients usually have albuginea and irisbulging that cover the pupil area,and are prone to have deprivation amblyopia and angle-closure glaucoma.The main treatment for CPILMG is surgical treatment.A small number of patients who has deep anterior chamber and spontaneous regression of pupil membrane can be followed up for observation.Present studies about CPILMG mainly focus on some case reports.It is easy to be misdiagnosed clinically with Axenfeld-Rieger syndrome(ARS)and persistent fetal vasculature(PFV).Learn more about the linical manifestations and differentiation of CPILMG will be helpful in clinical diagnosis and treatment.Research with larger sample size are expected to further investigate the different stages of CPLIMG and its developing process,its corresponding pathogenesis and treatment methods,so as to improve the understanding of the disease.

关 键 词：先天性眼部疾病/鉴别诊断 瞳孔膜 

分 类 号：R779.6[医药卫生—眼科]