According to a study published in the Journal of the American Association for Pediatric Ophthalmology and Strabismus, glaucoma drainage devices (GDDs) are an effective treatment for Sturge-Weber syndrome (SWS)-associated glaucoma but have a high rate of complications.
The investigators evaluated GDD implantation for refractory pediatric glaucoma associated with SWS (or encephalofacial angiomatosis), a rare congenital neurooculocutaneous disorder associated with a facial port-wine birthmark and ipsilateral intracranial leptomeningeal angioma, seizures, and cognitive impairment.
They retrospectively reviewed medical records of consecutive children (age <18 years) with SWS-associated glaucoma at Duke Eye Center who were treated by a single surgeon using GDDs over 20 years (July 1, 1996, to January 1, 2016).
The main outcome measure was successful treatment with GDD. GDD treatment success was defined as the absence of any of 3 indications of treatment failure: intraocular pressure (IOP) of >21 mm Hg on 2 consecutive visits despite maximal medical therapy, additional IOP-lowering surgery, and sight-threatening complications. Kaplan-Meier analysis at 1, 3, and 5 years was used to assess GDD treatment success.
Overall, 22 eyes of 22 children (64% girls), with a median age of 0.73 years (range, 0.06-13.2) at glaucoma diagnosis, were treated with GDDs. The children’s median age at GDD surgery was 4.8 years (range, 0.6-13.3). More than half of patients (68%) had a prior glaucoma surgery on the same eye. Patients received follow up for a mean duration of 2.8±1.5 years.
GDD treatment success was 91% (95% confidence interval, 68-98) at 1 year, 75% (50-89) at 3 years, and 52% (24-73) at 5 years. Treatment failure was reported in 36% of cases (8 eyes).
Postoperative complications were reported in 50% of eyes, with 23% of eyes having had more than one complication. Reported complications included the need for tube revision (n=5), mild cataract (n=4), choroidal effusion with or without serous retinal detachment (n=4), and inflammation (n=2). Severe vision-threatening complications occurred in 3 eyes (n=1 each: possible infection requiring GDD removal, persistent hypotony, and cilioretinal artery occlusion).
Limitations of the study included possible selection bias given the retrospective design, a short minimum follow-up duration (6 months), the small sample size, and outcomes for 3 different GDD devices (Baerveldt 250 mm2, Baerveldt 350 mm2, and Ahmed S2/FP7).
“GDDs are an effective treatment for SWS-associated glaucoma but have a high rate of complications,” researchers report. “We report several severe complications that prompted surgical modifications for increased safety when implanting GDDs in eyes with SWS-associated glaucoma.”
Glaser TS, Meekins LC, Freedman SF. Outcomes and lessons learned from two decades’ experience with glaucoma drainage device implantation for refractory Sturge Weber-associated childhood glaucoma. J AAPOS. Published online October 13, 2021. doi:10.1016/j.jaapos.2021.05.019