Outcomes and lessons learned from two decades' experience with glaucoma drainage device implantation for refractory Sturge Weber-associated childhood glaucoma.

PURPOSE

To evaluate glaucoma drainage device (GDD) implantation for refractory pediatric glaucoma associated with Sturge-Weber syndrome (SWS).

METHODS

The medical records of consecutive children with SWS-associated glaucoma at a single center who were treated by a single surgeon using GDDs over 20 years were reviewed retrospectively. The main outcome measure was GDD treatment success, defined as absence of any of the following indications of treatment failure: (1) intraocular pressure (IOP) of >21 mm Hg on two consecutive visits despite maximal medical therapy, (2) additional IOP-lowering surgery, and (3) sight-threatening complications.

RESULTS

A total of 22 eyes of 22 children were included. The median age at glaucoma diagnosis was 0.73 years (range, 0.06-13.2), and the median age at GDD surgery was 4.8 years (range, 0.6-13.3). Most eyes (14 [68%]) had prior glaucoma surgery. Mean follow-up was 2.8 ± 1.5 years. Success (95% confidence interval) by Kaplan-Meier analysis for GDD surgery at 1, 3, and 5 years was 91% (68-98), 75% (50-89), and 52% (24-73), respectively. Failure occurred in 8 eyes (36%). Complications were common, occurring in 50% of eyes, with 23% of eyes having more than one complication. Severe vision-threatening complications (n = 3) included one case each of possible infection requiring GDD removal, persistent hypotony, and cilioretinal artery occlusion.

CONCLUSIONS

GDDs are an effective treatment for SWS-associated glaucoma but have a high rate of complications. We report several severe complications that prompted surgical modifications for increased safety when implanting GDDs in eyes with SWS-associated glaucoma.