Outcomes of Microcatheter–Assisted Trabeculotomy for Glaucoma Associated With Sturge–Weber Syndrome and Phakomatosis Pigmentovascularis

Abstract

To evaluate mid-term efficacy and safety of ab externo Microcatheter-assisted trabeculotomy (MAT) for early-onset glaucoma associated with Sturge-Weber syndrome (SWS) and phakomatosis pigmentovascularis (PPV). Retrospective, non-comparative, interventional case series. Medical records of consecutive SWS- or PPV-associated glaucoma patients who had undergone ab externo MAT between August 2017 and April 2020 at Beijing Children's Hospital were reviewed. Success was defined as an intraocular pressure (IOP) of <21 mmHg with (qualified success) or without (complete success) the use of antiglaucoma medication. Overall, 13 eyes (12 patients) with SWS and 9 eyes (8 patients) with PPV were included, with a mean age of 12.8 ± 15.8 months at the time of surgery and a mean follow-up time of 39.5 ±10.4 months. Both the SWS (26.5 ± 5.3 mmHg at baseline vs 16.5 ± 5.0 mmHg at the last visit; P<.001) and PPV (29.2 ± 7.5 mmHg vs 23.4 ± 4.7 mmHg; P=.014) subsets achieved a statistically significant fall in IOP following surgery. The Kaplan-Meier survival rate of complete (qualified) success after 42months was 76.2% (87.5%) and 22.2% (40.0%) for eyes with SWS and PPV, respectively. Complications were minimal. Phakomatosis pigmentovascularis was associated with worse surgical outcomes. Ab externo MAT is an effective and safe treatment for early-onset glaucoma associated with SWS, but a gradual increase in IOP over time was noted in some patients. Ab externo MAT has limited efficacy for early-onset glaucoma associated with PPV in the mid-term.