1. Outcomes of Management of Glaucoma in Phacomatosis Pigmentovascularis Over the Last Three Decades
- Author
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Krishnapriya Kodavati, Anil K Mandal, and Vijaya K. Gothwal
- Subjects
Intraocular pressure ,medicine.medical_specialty ,Visual acuity ,genetic structures ,medicine.medical_treatment ,Sturge–Weber syndrome ,Glaucoma ,01 natural sciences ,03 medical and health sciences ,0302 clinical medicine ,Endophthalmitis ,Glaucoma surgery ,Medicine ,Trabeculectomy ,0101 mathematics ,business.industry ,010102 general mathematics ,Retrospective cohort study ,General Medicine ,medicine.disease ,eye diseases ,Surgery ,030221 ophthalmology & optometry ,sense organs ,medicine.symptom ,business - Abstract
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 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.
- Published
- 2022
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