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。

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.