Surgical Management of Glaucoma in Sturge-Weber Syndrome

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Sturge-Weber syndrome (SWS) or encephalo-trigeminal hemangiomatosis is a sporadic, mesodermal phakomatosis characterised by hemangiomas involving many parts of the body, particularly ocular, intracranial and facial cutaneous structures [1-3]. Glaucoma is a common feature, with an incidence of 30%-71% in patients with Sturge-Weber syndrome [4-6]. 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 [7,8]. 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 aіer filtering procedures [9-13]. Prohylactic sclerotomies have been advocated, to be performed prior to ocular decompression, during filtering procedures in order to avoid these complications. Нe necessity of prophylactic procedures has been questioned. Eibschitz-Tsimhoni et al.[4], 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 eوٴusion requiring therapeutic intervention [4]. Нe aim of our study was to evaluate the surgical outcomes in terms of intraocular pressure control, maintenance of visual acuity and complications of glaucoma surgeries, in eyes with Sturge-Weber syndrome and to evaluate risk factors leading to surgical failure.

Institutional review board and ethics committee clearance for analysis of charts was obtained before commencing the study. A retrospective chart review of all consecutive cases diagnosed with Sturge-Weber syndrome, who underwent glaucoma surgery from January 2003 to December 2013 was performed. Нe diagnosis of SWS was made clinically on the basis of typical ocular and systemic features. Patients without a minimum follow-up of 2 months were excluded. Нe data obtained included age, gender, ocular and systemic features of SWS, visual acuity, baseline intra ocular pressure (IOP), Goldmann applanation tonometry, gonioscopy (Posner four mirror, Ocular instruments.inc. Bellevue, WA), ultra sound pachymetry (Pachette 2, DGH tech. Inc. Shermans Dale, PA), details of medical treatment for glaucoma as well as details of the glaucoma surgery. Postoperative data collected included visual acuity, IOP, number of IOP lowering medications and details of post-operative complications, including their management at post-op 1 week, 6 weeks and final follow up visit.

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