EPOS
European Paediatric Ophthalmological Society
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Surgical treatment of glaucoma associated with Sturge Weber syndrome
edelson catherine1, dureau pascal1, audren francois1, metge florence1, denion eric1, caputo georges11fondation ophtalmologique A. de Rothschild
Introduction: Sturge-Weber syndrome is characterized by naevus flammeus of the face and ipsilateral angioma of the meninges responsible for epilepsy. Ocular manifestations consist in a choroidal angioma and glaucoma due to an episcleral haemangioma decreasing filtration. The aim of this study was to evaluate the outcome of glaucoma treatment in pediatric patients with Sturge- Weber syndrome. Methods: This retrospective case series analysis included all patients, operated for glaucoma associated with Sturge -Weber syndrome between 2004 and 2007. The surgical technique, the control of intra-ocular pressure (IOP), the need for additional medical treatment, and the surgical complications were analysed Results: Ten eyes of eight patients were operated. Median age at surgery was 11 months. All children presented with facial angioma and three with meningeal angioma. Deep sclerectomy was performed in 9 eyes and cyclodiode in 1 eye. Two eyes were retreated with cyclodiode and received anti-glaucoma medication, in the eye primarily treated with cyclodiode a second session was performed. One eye had a retinal detachment due to choroidal hemangioma. After 23.8 months of follow-up, mean final IOP was <20mmHg in 9 eyes (81.8%), of those 4 eyes (36.3%) needed anti-glaucoma medication. Conclusion: In Sturge Weber syndrome,treatment is difficult with an important rate of failure. Cyclodiode is inconstantly efficient and needs several procedures while hypotony induced by filtering surgery is responsible for retinal detachment or choroidal effusion. Deep sclerotomy is associated with a lower retinal risk. The use of antimetabolites has to be evaluated in this surgery. Functional prognosis is correlated to the control of IOP, but also to the cerebral lesions and the uni or bilaterality of the condition. Management of glaucoma associated with Sturge-Weber has to be specified, results being often transient.