Glaucoma in Sturge–Weber‐Krabbe syndrome about 10 cases

Abstract

Aims/Purpose: The objective of this study is to evaluate the results of glaucoma treatment in a series of 10 children with Sturge–Weber syndrome.Methods: The objective of this study is to evaluate the results of glaucoma treatment in a series of 10 children with Sturge–Weber syndrome collected between 2010 and 2023 at the paediatric ophthalmology department of the 20 August 1953 hospital of the University Hospital of Casablanca.Results: The mean age at diagnosis was 11 months, with a Sex‐ratio of 1.5. Seven patients had a unilateral facial angioma occupying the V1 territory and three had a bilateral angioma, the cutaneous angioma was extended to the neck and thorax in one patient of these 10 cases. Two patients had a meningeal angioma revealed by hemicorporeal seizures contralateral to the cutaneous angioma. Buphthalmia was found in five cases, dilatation of the episcleral vessels in four patients, predominantly in the temporal area. No cases of choroidal angioma were noted. The average preoperative ocular tone was 18 mm Hg. The average c/d was 0.5. All 10 eyes had trabeculectomy under 5 Fluorouracil. 4 eyes were reoperated. We noted 1 case of vitreous exit intraoperatively. The final ocular tone was <20 mm Hg in eight eyes, four of which had adjuvant hypotonizing treatment with Beta‐blocker and Dorzolamide, after a mean follow‐up of 24 months.Conclusions: The risk of Glaucoma being important, the Sturge–Weber‐Krabbe syndrome must be evoked in front of any facial hemangioma and its existence should not delay the therapy.