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Purpose: To report incidence rates of primary congenital glaucoma in Korea and evaluate comorbidity and mortality from2001 to 2015. Methods: This study is a nationwide and retrospective population-based study. We used claims data from the Korean NationalHealth Insurance Service database between 2001 and 2015. Data for all patients diagnosed with primary congenitalglaucoma were retrieved using the Korean Electronic Data Interchange and Korean Standard Classification of Diseases-6codes. Results: The number of patients with primary congenital glaucoma between 2001 and 2015 was 776, of which 437 weremale (56.31%) and 339 were female (43.69%). The annual prevalence demonstrated a general decreasing trend since 2011,but this was not significant. Over the total survey period, the incidence rate was 11.0 per 100,000 births, with 12.0 casesamong males and 10.0 among females. The incidence according to age was 518 (68.78%) patients at age 0, 112 (13.66%) at 1year, 70 (8.39%) at 2 years, and 76 (9.17%) at 3 years. Of the 776 patients diagnosed with primary congenital glaucoma in thestudy population, 27 died. The observed mortality per 100,000 people is about 10 times higher than that of the general infantand child population under the age of 4 years. Visual impairment was the most common accompanying disability, followed bybrain lesion. Conclusions: Our study’s estimates of the nationwide population-based incidence of primary congenital glaucoma in a Koreanpopulation will expand our understanding of the disease and allow healthcare systems to plan for primary congenitalglaucoma.
