Dr.GARIYASHEE LAHKAR

Dr.Jawahar Jyoti Kuli, Dr. BIKRAM DAM, Dr.Utkarsh Rai

Abstract

Sturge-Weber syndrome (SWS) belongs to a group of disorders; Phakomatoses, caused by GNAQ gene mutation. An 8year old boy, k/c/o SWS (Type1 Roach scale) with H/O trabeculotomy at 6 months of age, presented with headache and diminution of vision in R/E for 1 month. On examination, port-wine stain was noted on right side of face and neck. Ocular melanosis was seen in R/E with aberrant episcleral vessels and corneal oedema. IOP was 42mmHg OD and 22mmHg OS(GAT). Gonioscopy showed open angles and fundus examination revealed choroidal haemangioma in R/E. CT brain detected calcification in fronto-parietal lobe while leptomeningeal angioma was suggested by MRI Brain. Echocardiography confirmed VSD. Posterior sclerotomy (prophylactic) and Trabeculectomy was done under β-blocker cover, to prevent choroidal haemorrhage and effusion, showing suitable results providing impetus to study the role of Posterior sclerotomy, Trabeculectomy with β-blocker in repeated attacks of childhood glaucoma in SWS.