Clinical characteristics and surgical effect observation of congenital aniridia combined with cataract

Objective: To analyze the clinical manifestations, surgical approaches and postoperative prognosis for the cases of congenital aniridia combined with cataract. Methods: In this retrospective case series, 26 patients diagnosed with congenital aniridia combined with cataract were collected from Zhongshan Ophthalmic Center from February 2002 to August 2016. The Clinical data were collected to analyze the clinical features, surgical approaches and postoperative prognosis. T-test was used for statistical analysis. Results: Twenty-six bilateral congenital aniridia patients were included in the case series, with 50% male cases. The average age for the first visit was (8.72±8.06) years old. Hereditary patients constitutes 30.8% (8/26) of the total number. The proportions for bilateral and unilateral cataracts were 88.5% (23/26) and 11.5% (3/26) respectively, and 49 eyes suffering from both congenital aniridia and cataract were therefore included in the final analysis. The most common morphology cataract subtypes were lamellar cataract (24.5%, 12/49), posterior subcapsular cataract (22.4%, 11/49), and total cataract (18.4%,9/49). The observed ocular comorbidities included nystagmus (36.7%, 18/49), vitreous opacity (28.6%, 14/49), foveal hypoplasia (20.4%, 10/49), ametropia (12.2%, 6/49), exotropia (12.2%, 6/49), congenital glaucoma (12.2%, 6/49), esotropia (4.1%, 2/49), congenital ptosis (4.1%, 2/49), lens ectopia (4.1%, 2/49), scleral staphyloma (2.0%, 1/49) and pigmentary degeneration of retina (2.0%, 1/49). 30.6% (15/49) eyes were performed the cataract extraction surgery. The percentage of postoperative best corrected visual acuity (BCVA) for ≥0.1 and ≥0.3 were 93.3% (14/15) and 20.0% (3/15) respectively. Evaluated ocular pressure (33.3%, 5/15), severe posterior capsular opacification (PCO) (13.3%, 2/15) and choroidal hemorrhage combined with choroidal detachment (6.7%, 1/15) were detected as the postoperative complications. Followed by cataract extraction, 80.0% (12/15) eyes were sequentially performed the intraocular lens implantation, while, 20.0% (3/12) eyes remained aphakia due to ocular comorbidities. Conclusions: Congenital aniridia combined with cataract are rare diseases, calling for the precious retrospective researches. This disorder tended to affect both eyes and occurred hereditary. The clinical courses of the cases presented progressive features. Ocular comorbidities were the crucial factors to influence the surgical approaches and postoperative prognosis. (Chin J Ophthalmol, 2017, 53: 821-827).