Glaucoma In Sturge Weber Syndrome : A Case Report

The aim of this report is to describe the case of a child with Sturge Weber syndrome with ocular involvement manifesting as unilateral open-angle glaucoma.

20^th August Hospital, Ibn Rochd University Hospital Casablanca, Morocco

We report the case of a 10-years-old girl, with a history of unilateral facial port-wine stain since birth, and progressive seizures treated with carbamazepine, and diagnosed as Sturge Weber syndrome. The patient was addressed to our clinic for an ophthalmological consult with a complaint of progressive unilateral visual loss. Upon examination, there was a large hemifacial naevus flammeus mildly hypertrophic, and extending from the frontal to the mandibular area. Ophthalmic examination showed a visual acuity of 20/50 in the ipsilateral eye (right eye), compared to 20/20 in the left eye and a cup/disk ratio of 8/10 in the right eye. Intraocular pressure was elevated in the right eye : 31 mmHg versus 17 mmHg in the left eye.

The main aim of the treatment was to control intraocular pressure (IOP) and prevent the progressive optic nerve damage and visual field loss. The patient was prescribed prostaglandin eye drops in order to lower IOP, and a dermatology consult was scheduled to treat the naevus with laser esthetically. Topical treatment was ineffective in controlling eye pressure, so a trabeculectomy was indicated.

Glaucoma is one of the most common ocular complications associated with Sturge Weber syndrome, and represents the worst prognostic factor for visual function. It is important to perform a full ophthalmologic examination in these patients as most glaucoma cases are congenital, but it can also occur in young children and adults, and its management can be quite challenging despite medical or surgical treatment.