Purpose: To report the clinical features, management, and treatment outcomes of glaucoma in microspherophakia.
Methods: Medical records of 159 eyes of 80 subjects with microspherophakia were reviewed. The clinical features at presentation, presence of glaucoma, methods of treatment, and their outcomes were noted. Glaucoma was diagnosed based on intraocular pressure (IOP)≥22 mm Hg on 2 different occasions and/or glaucomatous optic disc damage. Angle closure was defined as occludable angles >270 degrees with or without presence of peripheral anterior synechiae.
Results: Glaucoma was diagnosed in 81 eyes (51%). The mean age of subjects was 20±13 years, mean refractive error was -13.5±5.5, the mean IOP was 27.7±11.1 mm Hg. IOP≥22 mm Hg was present in 84% of eyes, disc damage in 59% of the eyes, 75% eyes had angle closure, and 25% had open angle on gonioscopy. Subluxation of crystalline lens was seen in 53 eyes and 14 eyes had dislocation of the lens; systemic associations were present in 21 subjects (3 Marfan syndrome, 18 Weill-Marchesani syndrome). Nine eyes out of 51 and 2 out of 16 eyes responded to medical treatment and laser iridotomy, respectively. Of the 48 eyes that required surgical intervention, 24 eyes underwent trabeculectomy. Complete success probability of trabeculectomy was 86% [95% confidence interval (CI), 63%-95%] at 6 months, 77% (95% CI, 53%-90%) at 1 year, which was maintained till 7 years, and reduced to 61% (95% CI, 26%-84%) at 8 years. Nearly 20% of eyes at presentation and 30% of the eyes at last follow-up were blind due to glaucoma.
Conclusions: More than half of the eyes with microspherophakia in this series presented with glaucoma; angle closure was the predominant form of glaucoma. Blindness due to glaucoma in microspherophakia was 20% to 30%.