To report the clinical outcomes of glaucoma management in patients with phacomatosis pigmentovascularis (PPV) treated over a period of three decades.
Retrospective cohort study.
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.
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 two medications.
IOP, best-corrected visual acuity (BCVA), corneal clarity, antiglaucoma medications at preoperative and postoperative visit (last visit), and complications.
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 (P20/40. 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.
Combined trabeculotomy-trabeculectomy is safe and effective as a primary procedure for management of glaucoma in PPV. MMC-augmented trabeculectomy as a second procedure was associated with higher rate of complications.

Copyright © 2021. Published by Elsevier Inc.