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.