Abstract

Vision loss in Sturge-Weber syndrome (SWS), a rare congenital disorder, is primarily due to glaucoma. We reviewed the data of all consecutive SWS-associated glaucoma cases in patients who had undergone combined trabeculotomy-trabeculectomy (CTT) at a tertiary glaucoma facility between January 1993 and December 2015. We analyzed the preoperative and postoperative intraocular pressure (IOP), corneal clarity, visual acuity, success rate, need for repeat surgery, and number of topical antiglaucoma medications needed at last follow-up. Twenty-six eyes of 20 patients with SWS (surgical age 0.7-96 months; mean 18.64 ± 29.74 months) had undergone primary CTT. The mean preoperative IOP was 32.76 ± 7.86 mm Hg (range 22-54 mm Hg) with medication (mean 3.11 ± 1.17; range 1-5). At the last follow-up (61-288 months); mean SD 134.73 ± 67.77 months), two eyes had IOP <6 mm Hg. Twenty-four eyes analyzed had an IOP of 13.63 ± 6.11 (mean ± SD; range 9-41) mm Hg. All these had an IOP <15 mm Hg at last follow-up except one, which had an IOP of 41 mm Hg. There was a mean reduction of 54.62% ± 31.33% in IOP from baseline. The antiglaucoma medication score at last follow-up visit was 0-3. No eye achieved predefined complete success or modified complete success. A total of 41.7% (10/24) of eyes attained both qualified and modified qualified success. Eleven eyes needed repeat surgeries. No intraoperative complications were noted. Visual acuity was below 6/60 in four eyes. Combined trabeculotomy-trabeculectomy showed promising results as a treatment for SWS-associated glaucoma in children. Long-term visual and surgical outcomes are encouraging.

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