Analysis of Sturge Weber Syndrome: A Retrospective Study

ABSTRACT

Background: To review the clinical manifestations and neuroimaging features of patients with Sturge-Weber syndrome(SWS) treated at a tertiary care centre over a 3 year period. Methods:A retrospective study of six patients with SWS (4 males and 2 females) was conducted. Data was collected by reviewing the clinical histories of patients diagnosed with SWS over the last 3 years. Results: All patients had port-wine stain (PWS) involving the eyelid. Glaucoma was the main ocular disease being diagnosed in 2 eyes of three patients (66.66%). Five patients (83.33%) had neurological impairment including seizure, hemiparesis, headache, and delayed development. However, the most common neurological manifestation was epilepsy (83.33%), which could be controlled with antiepileptic drugs. In neurological imaging intracranial abnormalities were demonstrated which included cerebral atrophy (75.0%), cerebral calcification (50.0%), leptomeningeal angioma (25.0%), and enlarged choroidal plexus (25.0%). The ocular complications and intracranial abnormalities were usually ipsilateral to the PWS.. Conclusion: Port-wine stains, glaucoma, and seizure were the most common clinical presentations of Sturge-Weber syndrome detected in this study. Complete ophthalmic and neurological evaluation should be performed at the time of diagnosis