Efficacy of topical brinzolamide in children with retinal dystrophies

ABSTRACT

Background: Inherited retinal dystrophies are a leading cause of irreversible blindness in children in the United States. Topical carbonic anhydrase inhibitors have improved central vision and cystoid macular edema in patients with retinal dystrophies, but few studies have assessed their efficacy in children.

Materials and Methods: A retrospective chart review was performed with Institutional Review Board approval to identify pediatric patients with inherited retinal dystrophies who received topical brinzolamide at a single university center between 2008 and 2015. Serial visual acuity and central macular thicknesses were compared to assess the efficacy of brinzolamide.

Results: Seven subjects were identified who met the inclusion criteria. Four had juvenile X-linked retinoschisis, two had retinitis pigmentosa, and one had Leber congenital amaurosis. All were prescribed brinzolamide thrice daily; however, one patient was completely non-compliant. Four of the six treated patients exhibited a mild decrease in central macular thickness in both eyes during the study with all six treated patients having significantly improved vision at the first endpoint, 33.2 ± 8.2 months after treatment initiation. For treated patients, average visual acuity (LogMAR) ± standard error of the mean improved from 0.5 ± 0.04 pre-treatment to 0.3 ± 0.1 at the second endpoint, 50.2 ± 7.3 months after treatment initiation.

Conclusions: Mild anatomic improvement of macular cysts was seen in pediatric patients using brinzolamide. Visual acuity improvement occurred even without significant reduction in macular cysts. Further studies are needed to determine whether the beneficial effects of carbonic anhydrase inhibitors are sustained in children with inherited retinal degenerations.

KEYWORDS:

• Brinzolamide/carbonic anhydrase inhibitors
• inherited retinal dystrophy
• Leber congenital amaurosis (LCA)
• Retinitis pigmentosa (RP)
• X-linked juvenile retinoschisis (XLRS)

Acknowledgments

The John and Marcia Carver Nonprofit Genetic Testing Laboratory, The Chakraborty Family Foundation, and Research to Prevent Blindness provided support for this study. This project was made possible by funding through the NIH Training Grant (T35 HL 7485-35) at the University of Iowa Carver College of Medicine and the University of Iowa Institute for Vision Research.

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.

Supplementary Material

Supplemental data for this article can be accessed at https://doi.org/10.1080/13816810.2019.1660381.

Additional information

Funding

This work was supported by the University of Iowa Institute for Vision Research Endowment; the Chakraborty Family Foundation; the Ronald Keech Professorship; the Research to Prevent Blindness; and the National Institutes of Health under Training Grant [T35 HL 7485-35] (Chen). The Carver Laboratory, University of Iowa, performed molecular genetic testing.