Surgical Management of Glaucoma in Sturge-Weber Syndrome

Glaucoma is a common feature, with an incidence of 30%-71% in patients with Sturge-Weber syndrome. Many mechanisms of raised intraocular pressure have been described in the past, the most consistent being congenital trabeculodysgenesis, increased episcleral venous pressure and hypersecretion due to ciliary body angioma.

An increased risk of intra and post-operative complications has been noted with glaucoma filtering procedures in these patients, predominantly due to rupture of the fragile vasculature in the choroidal hemangiomas, leading to expulsive choroidal haemorrhage or exudative choroidal detachment (CD) caused by sudden decompression during or after filtering procedures. Prohylactic sclerotomies have been advocated, to be performed prior to ocular decompression, during filtering procedures in order to avoid these complications. The necessity of prophylactic procedures has been questioned. Eibschitz-Tsimhoni et al., in a retrospective study, have reported that none of their 17 patients with SWS who underwent glaucoma filtering surgery without prophylactic posterior sclerotomy developed intraoperative suprachoroidal haemorrhage or choroidal effusion requiring therapeutic intervention.