Axenfeld-Rieger综合征继发青光眼15例临床分析

目的:探讨Axenfeld-Rieger综合征继发青光眼患者的临床表现及其治疗。方法:回顾性病例研究。收集我院2003-01/2016-01就诊的Axenfeld-Rieger综合征继发青光眼患者15例26眼的临床资料,并进行回顾性分析。结果:患者年龄跨度为3月龄~43岁,中位数年龄为11岁。15例患者中11例(73%)为双眼患病,4例(27%)为单眼患病。临床表现方面,有20眼(77%)可见虹膜萎缩或缺损;17眼(65%)有不同程度的虹膜前粘;14眼(54%)可见瞳孔变形或多瞳;14眼(54%)可见后胚胎环;11眼(42%)有角膜大小的异常。全部患眼房角检查均可见虹膜根部附着偏前,以及不同程度的房角粘连。根据资料,全部患者中仅2例具有家族性遗传史;5例除眼部异常外还伴有牙齿、上颌骨、心血管等全身其他系统发育异常。有20眼进行了抗青光眼手术治疗,手术方式包括小梁切开术、小梁切除术、青光眼减压阀植入术等。术后随访到了9例患者(11只术眼),平均随访59mo,随访时眼压均低于术前眼压(t=2.4185,P=0.0362),其中7只术眼眼压≤21mmHg,远期手术成功率64%。结论:Axenfeld-Rieger综合征继发青光眼的临床表现多种多样,患者可同时伴有全身其他系统发育异常,该病治疗棘手,抗青光眼手术能够有效降压,改善临床症状。

A clinical analysis on 15 cases of Axenfeld-Rieger syndrome associated with secondary glaucoma

AIM:To investigate the clinical features of Axenfeld-Rieger syndrome associated with secondary glaucoma and its surgical treatment.METHODS:It was a retrospective case series study.The general clinical data and related ocular manifestations in 15 patients(26 eyes)with Axenfeld-Rieger syndrome associated with secondary glaucoma in our hospital from January 2003 to January 2016 were collected and analyzed retrospectively.RESULTS:Age of the patients ranged from 3-month to 43 years old,and the median age was 11 years old.There were of all 11 patients(73%)had bilateral glaucoma and 4(27%)had unilateral glaucoma.In the aspect of clinical features,hypoplasia or deficiency of the iris was found in 20 eyes(77%),peripheral iridocorneal adhesions was found in 17 eyes(65%)in various extent,and pupil distortion or multiple-pupil was found in 14 eyes(54%);Also posterior embryotoxon was found in 14 eyes(54%)as well;And 11 eyes(42%)had anomalies in corneal size.That root of iris is located at a more anterior position than that of a normal eye was found in all affected eyes by gonioscopy,coupled with different extent of angle synechiae-closure.According to the data,in 15 patients only two had a family history of ARS,while 5 had systematic abnormalities,eyes excepted,in dental,maxillary,cardiovascular,et al.Anti-glaucoma surgeries including trabeculotomy,trabeculectomy,glaucoma valve implantation were performed in 20 eyes.Nine patients(11 eyes)were followed up for 59mo on average.Intraocular pressure was significantly lower than pre-operation(t=2.4185,P=0.0362),and intraocular pressure in 7 followed-up eyes was controlled≤21mmHg.The long-term success rate of surgery was 64%.CONCLUSION:The clinical features of Axenfeld-Rieger syndrome associated with secondary glaucoma were diverse.The patients might concomitant with other systematic dysplasia.Although it was tough to treat,surgery for glaucoma could reduce the intraocular pressure effectively.