Outcomes of Management of Glaucoma in Phacomatosis Pigmentovascularis Over the Last Three Decades: A Single-Center Experience.

Purpose: To report the clinical outcomes of glaucoma management in patients with phacomatosis pigmentovascularis (PPV) treated over a period of 3 decades.
Design: Retrospective cohort study.
Participants: Fifty-five eyes of 38 patients (21 unilateral and 17 bilateral) with glaucoma in PPV managed at one institution between January 1990 and December 2019 with a minimum follow-up of 1 year.
Methods: Medical records of children with glaucoma in PPV were reviewed, and demographic and clinical data were collected. Surgical interventions included primary combined trabeculotomy-trabeculectomy (CTT), trabeculectomy with mitomycin C (MMC), and transscleral cyclophotocoagulation (TSCPC). Complete success was defined as intraocular pressure (IOP) ≥ 6 and ≤ 16 mmHg without medications and qualified success as IOP ≤ 16 mmHg with the use of up to 2 medications.
Main Outcome Measures: Intraocular pressure, best-corrected visual acuity (BCVA), corneal clarity, antiglaucoma medications at preoperative and postoperative visits (last visit), and complications.
Results: Median age was 4 months (range, 0.2-252 months) at the time of glaucoma surgery. Thirty-nine eyes (74%) had primary CTT, 10 eyes (19%) had trabeculectomy with MMC, and 4 eyes (7%) with advanced glaucoma had TSCPC. Two eyes (3.6%) received medical treatment. Preoperative IOP reduced from a mean of 25.7 ± 8.4 mmHg on 0.8 ± 0.6 medications to 14.6 ± 5.2 mmHg on 0.4 ± 0.5 medications (P < 0.0001) at last follow-up after surgery (77.7 ± 56.5 months). Thirty-seven eyes (67.3%) required medications preoperatively, and 22 eyes (40%) required medications at the last follow-up. Of 25 patients with available BCVA at last follow-up, 9 (36%) had ≥ 20/60; of these, 6 had > 20/40. Four of 10 eyes that underwent trabeculectomy with MMC developed retinal detachment and were managed surgically; however, all of these eyes had poor visual outcomes. There was no incidence of bleb leakage, bleb-related infection, or endophthalmitis.
Conclusions: Combined trabeculotomy-trabeculectomy is safe and effective as a primary procedure for management of glaucoma in PPV. Trabeculectomy augmented with MMC as a second procedure was associated with a higher rate of complications.
(Copyright © 2021 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)