Ocular findings in Sturge-Weber syndrome

We reviewed the rare ocular findings of Sturge-Weber syndrome (SWS) and the results of implantation of the Ahmed valve in cases associated with glaucoma.

Seven patients (range 18 to 52 years) diagnosed as SWS were reviewed as clinical findings over two years. An anterior chamber maintainer was placed in patients with glaucoma to maintain stable intraocular pressure and minimize the risk of intraocular hemorrhage due to sudden pressure changes during surgery, when an Ahmed valve is implanted.

Episcleral venous vessels were prominent in all cases. Diffuse choroidal hemangiomas were seen in three cases. Nevus of Ota was observed in only one case. Three cases had juvenile glaucoma. One also had buphthalmos. No intraocular hemorrhage or choroidal effusion was observed intraoperatively in valve-implant patients. The surgical treatment of the patients with glaucoma gave favorable outcomes.

Rare ocular findings such as choroidal hemangioma and nevus of Ota are sometimes seen in SWS. Drainage valve implantation, with an anterior chamber maintainer, is a good choice for treatment when surgery is done in cases with glaucoma. This method may reduce the risk of intraoperative suprachoroidal effusion and expulsive hemorrhage by stabilizing intraocular pressure within normal limits during the surgery.