Dr.Ipsita Das

Dr.Prasenjit Kalita , Dr.Ipsita Das

Abstract

Sturge weber syndrome is a congenital, sporadic phacomatoses characterised by hamartomas involving brain, skin and eyes. The vision-threatening features include development of glaucoma, retinal degeneration and detachment following diffuse choroidal hemangioma. A 12 year old mentally retarded boy was referred from neurology department for ophthalmic evaluation. On thorough examination, characteristic facial port wine stain was noted in the left side and ocular examination revealed of loss of vision and epibulbar hemangioma in left eye. Intraocular pressure was found to be raised with anterior chamber angle closure in 2 quadrant in the affected side. USG B scan showed diffuse choroidal hemangioma and retinal detachment with subretinal haemorrhage in the left eye. CE MRI report also supported the findings of USG B scan. The patient was treated conservatively.