FP831 : Bilateral glaucoma- a rare case of overlapping Sturge-Weber syndrome and Klippel-Trenaunay syndrome.

Dr.PALLABI RAY CHAUDHURI, P21387

Sturge-Weber syndrome (SWS) is characterized by meningo-facial angiomas with cerebral calcification. Klippel-Trenaunay syndrome (KTS) is another very rare type of phakomatosis with cutaneous angiomas ,varicose vain, enlargement of soft tissue or bone. Overlap between SWS and KTS is very rare.

We report a case of 6 years old boy presented with dimness of vision having a past history of recurrent seizure since 2 years of age and was found to have elevated intraocular pressure and glaucomatous optic disc changes in both eyes. He also have port-wine stain over face both sides , left side of chest and hand with hypertrophy of left hand and leg, venous prominence over abdominal wall and chest.CT scan shows left sided angiomatosis in brain and vena caval anomalous connections in abdomen. Usually SWS presented as unilateral port-wine stain and ipsilateral glaucoma but this case reveals a very rare overlap between SWS and KTS presented with bilateral port-wine stain and bilateral glaucoma.