Glaucoma Procedures Safe for Patients With Phacomatosis Pigmentovascularis

An ophthalmologist performs an intraocular pressure test to a baby at the Beijing Tongren Hospital on October 8, 2014 on the eve of the 14th World Sight Day. Beijing Tongren Hospital is a famous institution in the medical world which specialises in ophtalmology and otolaryngology. AFP PHOTO / FRED DUFOUR (Photo credit should read FRED DUFOUR/AFP via Getty Images)
Three decades of data show support for a combined trabeculotomy-trabeculectomy approach.

A combined trabeculotomy-trabeculectomy is safe and effective as a primary procedure for managing glaucoma in patients with phacomatosis pigmentovascularis (PPV), according to investigators who gathered nearly 3 decades worth of data. However, trabeculectomy augmented with mitomycin C (MMC) as a second procedure was associated with a higher rate of complications, according to the study published in Ophthalmology Glaucoma.

This retrospective cohort study included 55 eyes of 38 patients (21 unilateral and 17 bilateral) with glaucoma and PPV managed at a single institution between January 1990 and December 2019 with a minimum follow-up of 1 year. Surgical interventions included primary combined trabeculotomy-trabeculectomy (CTT), trabeculectomy with MMC, and transscleral cyclophotocoagulation (TSCPC). Complete success was defined as intraocular pressure (IOP) ≥6 and ≤16 mm Hg without medications and qualified success as IOP ≤16 mm Hg with the use of up to 2 medications. The median age was 4 months (range, 0.2-252 months) at the time of glaucoma surgery. In the group, 39 eyes (74%) had primary CTT, 10 eyes (19%) had trabeculectomy with MMC, and 4 eyes (7%) with advanced glaucoma had TSCPC. In addition, 2 eyes (3.6%) received medical treatment. 

Researchers found the preoperative IOP reduced from a mean of 25.7±8.4 mm Hg on 0.8±0.6 medications to 14.6±5.2 mm Hg on 0.4±0.5 medications (P <.0001) at last follow-up after surgery (77.7±56.5 months). Of the group, 37 eyes (67.3%) required medications preoperatively, and 22 eyes (40%) required medications at the last follow-up. Of 25 patients with available best-corrected visual acuity (BCVA) at last follow-up, 9 (36%) had ≥20/60. Of these, 6 had >20/40. Of the 10 eyes that underwent trabeculectomy with MMC, 4 developed retinal detachment and were managed surgically; however, these eyes had poor visual outcomes. There was no incidence of bleb leakage, bleb-related infection, or endophthalmitis, according to the researchers.

The researchers note several limitations, including a nonrandomized retrospective design and the variability in the timing of the surgery that depended on the patient’s age at presentation. Also, Snellen visual acuity was available for only two thirds of the patients, and some patients were lost to follow-up or had <1 year of follow-up. 

Investigators report that primary CTT is safe and effective in patients with glaucoma in PPV and that the cohort achieved prolonged IOP control and satisfactory visual outcomes. For patients with bilateral affliction, simultaneous bilateral CTT can be performed. Although trabeculectomy with MMC resulted in good IOP control, it was associated with a higher incidence of postoperative complications.


Mandal AK, Kodavati K, Gothwal VK. Outcomes of management of glaucoma in phacomatosis pigmentovascularis over the last three decades: a single-center experience. Ophthalmol Glaucoma. Published online June 27 2021. doi:10.1016/j.ogla.2021.06.003