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

Am J Ophthalmol. 2023 Apr:248:51-59. doi: 10.1016/j.ajo.2022.12.005. Epub 2022 Dec 10.

Abstract

Purpose: 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).

Design: Retrospective, non-comparative, interventional case series.

Methods: 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.

Results: 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 42 months 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.

Conclusions: 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Child, Preschool
  • Follow-Up Studies
  • Glaucoma* / complications
  • Glaucoma* / surgery
  • Humans
  • Infant
  • Intraocular Pressure
  • Neurocutaneous Syndromes* / complications
  • Neurocutaneous Syndromes* / surgery
  • Retrospective Studies
  • Sturge-Weber Syndrome* / complications
  • Sturge-Weber Syndrome* / diagnosis
  • Sturge-Weber Syndrome* / surgery
  • Trabeculectomy* / adverse effects
  • Treatment Outcome

Supplementary concepts

  • Early-Onset Glaucoma
  • Phacomatosis pigmentovascularis