Dr.Romi Singh, S19143
INTRODUCTION: Sturge Weber Syndrome (SWS) or encephalotrigeminal angiomatosis is a neurocutaneous disorder with angiomas involving leptomeninges and skin of face known as port wine stain (PWS). Glaucoma is the most common ocular presentation of SWS.
MATERIALS: A 6yr old girl came to our OPD with diminision of vision and white spot in left eye since 1-1/2 yrs. She had history of epileptic episodes 2 yrs back. She had a PWS over her left face since birth not changing in size. Her vision in right eye was 6/6 and that in left eye was PL. On examination of anterior segment, she had a mature cataract in her left eye with shallow AC and normal IOP. B-Scan USG revealed a choroidal mass with vitreous haemorrhage. Her paediatric opinion confirmed Sturge-Weber Syndrome. She was operated for left eye cataract extraction under GA.
CONCLUSION: Sturge Weber Syndrome with cataract is rare presentation with few cases reported in literature.
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