Primary congenital glaucoma (PCG) occurs worldwide and has a broad range of ocular manifestations. It poses a therapeutic challenge to the ophthalmologist. A proper diagnostic evaluation under anesthesia is advisable for all children who do not cooperate for an office examination. Medical therapy only serves as a supportive role, and surgical intervention remains the principal therapeutic modality. Angle incision surgery such as goniotomy or trabeculotomy ab externo is the preferred choice of surgery in the Caucasian population. Primary combined trabeculotomy-trabeculectomy with or without antifibrotic therapy is the preferred choice in certain regions such as India and the Middle East where the disease usually presents with severe forms of corneal edema along with megalocornea. In refractory cases, trabeculectomy with antifibrotic therapy or glaucoma drainage devices are available options in the armamentarium. Cycloablative procedures should be reserved for eyes with poor visual potential. Myopia is common among children with PCG, and appropriate optical refractive correction in the form of glasses or contact lenses should be provided. Amblyopia therapy should be instituted to ensure overall visual development in the early developmental years. Low-vision rehabilitation services should be provided to children with vision impairment. Long-term follow-up is mandatory and carers of children with PCG should be counseled and educated about this need. Regardless of the visual outcomes, clinicians should emphasize the need for education of these children during the clinic visit. The overall goal of the management should be to improve the overall quality of life of the children with PCG and their carers.

OBJECTIVE: To evaluate the long-term outcomes of glaucoma management in patients with congenital ectropion uveae (CEU) over a period of three decades at a single large referral tertiary eye care center.
METHODS: Retrospective chart review of all patients with CEU treated surgically from 1990 to 2019 was performed. Primary combined trabeculotomy-trabeculectomy (CTT), trabeculectomy with and without mitomycin-C (MMC) (0.2 mg/mL for 1 min) and transscleral cyclophotocoagulation (TSCPC) were performed. Intraocular pressure (IOP) ≥6 and ≤16 mmHg without medications was considered as complete success and IOP≤ 16 mmHg with the use of upto 2 medications as qualified success.
RESULTS: A total of 26 eyes of 21 patients were identified with a median age of 7 years (range, 6 days to 19 years) at the time of glaucoma surgery. Median follow-up was 51.1 months (range, 7-244.6 months). Primary CTT was performed in 17 eyes (65%), trabeculectomy in 5 eyes (19%) with application of MMC in 2 eyes, and 3 eyes (12%) underwent TSCPC. One painful blind eye (4%) underwent evisceration. Mean IOP reduced from 30.8 ± 7.6 mmHg on a mean of 1.3 ± 0.8 glaucoma medications preoperatively to a mean IOP of 15.2 ± 5.9 mmHg (P < 0.0001) on a mean of 0.2 ± 0.5 medications postoperatively at final follow-up (P = 0.0009). Complete success was achieved in 20 eyes, and qualified success in 2 eyes.
CONCLUSIONS: CTT is a safe and efficacious primary procedure for management of early-onset glaucoma in CEU. Trabeculectomy with or without adjuvant MMC is a viable second line of treatment in late-onset glaucoma with CEU for IOP control.

To report the clinical outcomes of glaucoma management in patients with phacomatosis pigmentovascularis (PPV) treated over a period of 3 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 2 medications.
Intraocular pressure, best-corrected visual acuity (BCVA), corneal clarity, antiglaucoma medications at preoperative and postoperative visits (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 (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.
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.