Glaucoma

Drainage Devices Effective for Sturge-Weber Glaucoma, But Complication Rate Is High

Lauren Dembeck, PhD
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I Wouldn't Leave The House - But I Accept My Birthmark Now
ALBERTA, CANADA – MARCH 14, 2020 – DESPITE being born with a large birthmark on the left side of her face, this mum-of-two is now proving her high school bullies wrong. Chelsey Peat, 34, from Canada was born with a rare condition known as Sturge Weber syndrome. The neurological disorder means she has a large port wine stain birthmark covering the left side of her face and has a glaucoma in her eye. Chelsey’s condition has led to her undergoing multiple surgeries. This has included brain surgery, where surgeons removed the occipital lobe so Chelsey would stop having seizures as a child. Doctors told her parents that she had 50% chance of living, and 50% chance of mental instability if she did survive. Chelsey told Truly: “I beat all the statistics.” But Chelsey continued to face difficulties in her childhood, as she was targeted for her physical differences. “I didn’t really have that social life; I didn’t have that date for dances.” She has struggled with her mental health because of these experiences. “There were times when I didn’t want to leave the house”, she said. In the video Chelsey goes for her first ever solo photoshoot. It’s something she’s always wanted to do but found it difficult to build up enough self-confidence in the past. Chelsey is an active member of Sturge Weber support groups on social media. “Being an ambassador for people with facial differences has given me the strength to do this,” she said. Now Chelsey has a different attitude towards those who fixate on her differences: “If you’re going to stare, either ask for my autograph or take the damn picture.” PHOTOGRAPH BY Barcroft Studios / Future Publishing – NOTE: This Photo Can Only Be Used Within Context With The Information Provided In The Metadata (Photo credit should read Barcroft Studios / Future Publishing/Barcroft Media via Getty Images)
Researchers review two decades of experience with the treatment approach to childhood glaucoma.

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. 

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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.”

Reference

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

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