[Management of childhood glaucoma associated with Sturge-Weber syndrome]

Abstract

Purpose: Sturge-Weber syndrome is a rare congenital disorder consisting of cutaneous, leptomeningeal and ocular vascular malformations. Glaucoma occurs in 30-70 % of patients. Treatment is challenging due to a high risk of severe complications such as expulsive haemorrhage, massive choroidal effusion and serous retinal detachment. Ocular findings and the occurrence of complications under different treatment modalities have been reviewed.

Methods: A retrospective analysis of a case series of 5 children (5 eyes/mean age 5.6 years) with secondary glaucoma associated with Sturge-Weber syndrome was undertaken. Ocular findings, treatment modalities, intraocular pressure and complications were assessed.

Results: Facial port-wine nevus, i.e., nevus flammeus and dilated episcleral vessels were present in all cases. Diffuse choroidal haemangiomas were seen in four eyes. Glaucoma surgery was performed in 4 children, one child was treated with antiglaucomatous medication alone. Reversible uveal effusion and subluxation of the lens appeared postoperatively in one eye, persistent serous retinal detachment occurred 3 years after surgery in another eye (with an intraocular pressure of 10 mmHg). Both complications were found in eyes with diffuse choroidal haemangioma.

Conclusion: Management of glaucoma associated with Sturge-Weber syndrome is difficult and controversial. Medical treatment often does not decrease intraocular pressure sufficiently. When planning surgical intervention an increased risk of severe complications has to be considered, especially in the presence of diffuse choroidal haemangioma.