Abstract

Sturge Weber syndrome is a rare phakomatoses characterised by vascular hamartomas affecting the leptomeninges, and cutaneous and ocular structures. Glaucoma results from angle anomalies, overproduction
from ciliary body haemangioma, or reduction in aqueous outflow secondary to increased episcleral venous pressure. The presence of a choroidal haemangioma in an eye with advanced glaucoma adds to the complicated postoperative recovery. This report is of a 9-year-old girl with a right-sided facial naevus flammeus who was referred for advanced glaucoma in the right eye. The eye was buphthalmic, her intraocular pressure was 25 mm Hg while using 4 topical antiglaucoma medications, and the visual acuity was 6/36. The optic disc had advanced cupping with a pale neuroretinal rim. Her visual field was severely constricted with fixation splitting. She underwent Ahmed valve implantation with controlled filtration via nylon stenting of the tube. Her postoperative recovery was complicated, and she needed further ligation of the tube to reduce drainage. Six weeks later, her intraocular pressure was controlled at 14 mm Hg without any antiglaucoma medication after removal of the nylon stent. Managing an eye with advanced glaucoma in the presence of a choroidal haemangioma needs meticulous controlled drainage to prevent detrimental postoperative complications.