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Compared to bilateral choanal atresia\/stenosis, unilateral choanal atresia\/stenosis is generally diagnosed later and might be under\u2010ascertained in birth defect registries. Data from the population\u2010based Texas Birth Defects Registry and Texas vital records, 1999\u20132018, were used to assess the prevalence of choanal atresia\/stenosis. Poisson regression models were used to evaluate associations with infant and maternal characteristics in two analytic groups: isolated choanal atresia\/stenosis (<jats:italic>n<\/jats:italic>\u2009=\u2009286) and isolated, bilateral choanal atresia\/stenosis (<jats:italic>n<\/jats:italic>\u2009=\u2009105). The overall prevalence of choanal atresia\/stenosis was 0.92\/10,000, and the prevalence of isolated choanal atresia\/stenosis was 0.37\/10,000 livebirths. Variables associated with choanal atresia\/stenosis in one or both analytic groups included infant sex, pregnancy plurality, maternal race\/ethnicity, maternal age, and maternal residence on the Texas\u2013Mexico border. In general, adjusted prevalence ratios estimated from the two analytic groups were in the same direction but tended to be stronger in the analyses restricted to isolated, bilateral defects. Epidemiologic studies of isolated choanal atresia\/stenosis should consider focusing on cases with bilateral defects, and prioritizing analyses of environmental, social, and structural factors that could account for the association with maternal residence on the Texas\u2013Mexico border.<\/jats:p>","DOI":"10.1002\/ajmg.a.63549","type":"journal-article","created":{"date-parts":[[2024,2,7]],"date-time":"2024-02-07T06:52:34Z","timestamp":1707288754000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Prevalence and descriptive epidemiology of choanal atresia and stenosis in Texas, 1999\u20132018"],"prefix":"10.1002","volume":"194","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6406-4247","authenticated-orcid":false,"given":"Renata H.","family":"Benjamin","sequence":"first","affiliation":[{"name":"Department of Epidemiology UTHealth School of Public Health  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa K.","family":"Marengo","sequence":"additional","affiliation":[{"name":"Birth Defects Epidemiology and Surveillance Branch Texas Department of State Health Services  Austin Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9025-3276","authenticated-orcid":false,"given":"Angela E.","family":"Scheuerle","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Genetics and Metabolism University of Texas Southwestern Medical Center  Dallas Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5874-4155","authenticated-orcid":false,"given":"A. 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