The Robison D. Harley, MD Childhood Glaucoma Research Network International Pediatric Glaucoma Registry: The First 872 Cases

Purpose: To report on epidemiologic data from an international, centralized pediatric glaucoma database of 872 patients, focusing on genetic and clinically significant factors.

Design: Database study utilizing retrospective analysis.

Subjects: Eight hundred seventy-two children, both female and male, were included in the database. After accounting for database coding errors, data from 865 patients with pediatric glaucoma were analyzed. Number of eyes analyzed fluctuated for each variable.

Methods: The registry is an open access, no charge, Research Electronic Data Capture database. Participating clinical centers input data with local Institutional Review Board approval and subsequently have access for research purposes. We retrospectively reviewed 872 patients, comparing demographics, family history, country, disease presentation, and Childhood Glaucoma Research Network diagnoses. Analyses for each variable were conducted in SPSS Software v.28.0. Chi-square analyses were performed for nominal data, and ordinal and continuous data were analyzed using Mann-Whitney test, analysis of variance, or Kruskal-Wallis tests with multiple comparisons.

Main outcome measures: Childhood Glaucoma Research Network glaucoma type and markers of clinical severity by country (laterality, cup-to-disc ratio [CTD], corneal diameter, opacification, edema; visual acuity [VA], intraocular pressure, Haab striae, axial length).

Results: Twenty clinical sites from 10 countries entered data. Centers in the USA, India, and Iran input the most data. In the USA, open-angle glaucoma following cataract surgery was most common, while in India and Iran it was primary congenital glaucoma neonatal onset. Bilateral disease was more frequent in India and Iran compared to the USA (X² = 50.6, P < 0.001). Clinical measures of severity were typically worse in India compared to the USA. This included increased CTD (X² = 24.0, P = 0.002), increased corneal diameter (X² = 8.9, P = 0.01), presence of corneal opacification (X² = 10.7, P = 0.001), presence of corneal edema (X² = 11.7, P < 0.001), and worse VA (U = 873.5, P < 0.001). Intraocular pressure and presence of Haab striae were not associated with country (P > 0.05), while axial length was increased in the USA by an average of 1.04 mm (U = 5787, P = 0.002).

Conclusions: This registry has potential to advance our understanding of pediatric glaucoma. Differences in family history, disease presentation, and glaucoma type suggest unique country phenotypes. Registry expansion may allow for insight into best practices for pediatric glaucoma.

Financial disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.