Clinical Presentation and Management Outcomes of Coexistent Congenital Glaucoma and Retinopathy of Prematurity

Abstract

Purpose: The purpose of this study was to report presentation and outcomes of coexisting congenital glaucoma with retinopathy of prematurity (ROP) in “Indian Twin cities ROP study (ITCROPS).” Methods: Children with ROP and congenital glaucoma were identified from ITCROPS (prospective digital database) between 1997 and 2016. The presentation, interventions, and outcomes for glaucoma and ROP were evaluated. Results: Out of 15,000 premature-infants database, 3000 children had ROP, of these 87 eyes of 64 premature children had glaucoma. Five eyes (5.7%) of 3 children in the entire ROP cohort had coexisting congenital glaucoma (before any ROP intervention), 3 eyes of 2 children had primary congenital glaucoma and 2 eyes of one child had glaucoma with microspherophakia. The mean age at surgery was 2.7±0.6 months. At presentation, all eyes had corneal edema, mean corneal diameter was 10.3±0.75 mm and IOP was 20.4±1.67 mm Hg. Three eyes had stage-2 ROP and two eyes had stage-3 ROP. Four eyes received intravitreal Bevacizumab. All eyes underwent combined trabeculotomy with trabeculectomy. Post glaucoma surgery, 2 eyes underwent laser for ROP and 1 eye with stage-2 ROP was observed with no treatment. ROP regressed without any sequalae in all 5 eyes. Two eyes with microspherophakia and one eye with lens coloboma underwent lensectomy. One eye postlensectomy needed Ahmed glaucoma valve implant for IOP control. The mean IOP at last follow-up was 13.6±1.67 mm Hg and all eyes needed topical antiglaucoma medications for IOP control. Conclusions: Congenital glaucoma coexisting with ROP is a medical emergency. Intravitreal therapy can help tide over the vision-threatening fulminant phase of ROP while awaiting the effect of glaucoma surgery on corneal clarity. The need for medications even after glaucoma surgery suggests severe glaucoma phenotype. Close coordination between subspecialties is needed for best outcomes.

Management outcomes of secondary glaucoma due to retinopathy of prematurity: A 19-year prospective study at a tertiary eye care Institute. The Indian Twin cities ROP Screening (ITCROPS) database report number 8

“…Of these, 87 eyes of 57 children were diagnosed with glaucoma. Five eyes of 3 children had primary congenital glaucoma with ROP, the details of which are already published [18]. Excluding the 5 eyes with Primary Congenital Glaucoma (PCG), we included 82 eyes of 54 children with ROP and secondary glaucoma in this study.…”

“…The glaucoma in eyes with ROP was classified into congenital and secondary glaucomas based on the presentation [18]. Diagnosis of congenital glaucoma was based on elevated IOP at birth with corneal changes suggestive of congenital glaucoma (corneal edema, Haabs' striae, enlarged corneal diameter) prior to any intervention for ROP.…”

“…If the IOP remained persistently high or corneal edema persisted or if there were other signs of progression like the enlargement in corneal diameter/ Habb's striae or increase in disc cupping, surgical intervention was planned and performed. Our choice of glaucoma surgery and the technique of combined trabeculotomy and trabeculectomy are as described in our earlier publication [18].…”

“…Overall, 39% of eyes had ambulatory vision in our series, and we noted better visual outcomes in eyes with less severe ROP compared to those with advanced stages of ROP. It would be important to closely monitor these children with a multidisciplinary approach with protocols of vision assessment, refraction, glaucoma evaluation and retinal examination at every visit, besides close collaboration with early intervention specialists, low vision and rehabilitation specialists [15,18].…”

Approach to childhood glaucoma: A review

“…IOP elevation may also follow laser therapy for threshold ROP. 70 Topical anti-glaucoma therapy and surgery in the form of peripheral iridectomy for pupillary block from the mainstay of treatment. Lensectomy may be useful in angle closure mechanisms, however, GDD's or ciliary body ablation can also be considered in cases of chronic angle closure that have failed medical or primary procedures.…”