Precis: Glaucoma associated with Sturge-Weber syndrome (SWS) often requires surgical intervention. Our study shows that trabeculectomy is efficacious in treating this condition.
Purpose: The purpose of this study was to describe the surgical outcomes of glaucoma associated with SWS in children presenting to the tertiary Paediatric Ophthalmology Department at The Children's Hospital at Westmead.
Materials and methods: A retrospective study of patients with SWS referred to the Department of Ophthalmology at The Children's Hospital at Westmead between 2003 and 2016 with at least 2 years of follow-up were identified, and information was collected from the clinical notes of all subjects.
Results: A total of 27 patients with SWS were evaluated for glaucoma in which 8 were excluded due to inadequate follow-up. In total, 19 patients with SWS were included in this study in which glaucoma was diagnosed in 15 patients and 19 eyes, of which 13 eyes required glaucoma surgery. A total of 21 surgical procedures were performed with a median follow-up of 85 months. A primary trabeculotomy was performed in 5 eyes of which 4 required re-do trabeculotomy, and 3 of these eyes underwent a Baerveldt tube (BVT) shunt as a third procedure. One eye with a primary trabeculotomy underwent a BVT as a secondary procedure. A BVT was inserted in a total of 6 eyes in which it was a primary procedure in 2 eyes. Of the 6 eyes undergoing a BVT insertion, 5 achieved success (2 complete and 3 qualified), and 1 failed. One case underwent intraluminal stent removal. Six eyes underwent a primary trabeculectomy and needed no further surgical intervention. In the trabeculectomy group, 4 eyes achieved complete success and 2 eyes achieved qualified success.
Conclusions: Glaucoma affects a significant proportion of patients with SWS and is associated with the presence of an ipsilateral port-wine stain in most cases. In our study, trabeculectomy was the most efficacious procedure for controlling intraocular pressure and reducing the burden of ongoing treatment in SWS-associated glaucoma.