AUTHORS - Natasha Nassar1, Emanuele Leoncini2, Emmanuelle Amar3, Jazmín Arteaga-Vázquez4, Marian K. Bakker5, Carol Bower6, Mark A. Canfield7, Eduardo E. Castilla8,9,10, Guido Cocchi11, Adolfo Correa12,13, Melinda Csáky-Szunyogh14, Marcia L. Feldkamp15,16, Babak Khoshnood17, Danielle Landau18, Nathalie Lelong17, Jorge S. López-Camelo8,9, R. Brian Lowry19, Robert McDonnell20, Paul Merlob21, Julia Métneki14, Margery Morgan22, Osvaldo M. Mutchinick4, Miland N. Palmer16, Anke Rissmann23, Csaba Siffel12, Antonin Sìpek24, Elena Szabova25, David Tucker22, and Pierpaolo Mastroiacovo2 - AFFILIATIONS - Mastroiacovo21Population Perinatal Health Research, Kolling Institute of Medical Research, University of Sydney, Australia 2Center of the International Clearinghouse for Birth Defects Surveillance and Research, Rome, Italy 3Rhone-Alps Registry of Birth Defects REMERA, Lyon, France 4Instituto Nacional de Ciencias Médicas y Nutrición “Salvador Zubirán”, Departamento de Genética, Registro y Vigilancia Epidemiológica de Malformaciones Congénitas, Mexico City, Mexico 5 EUROCAT Northern Netherlands, Department of Genetics, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands 6Western Australian Register of Developmental Anomalies, Perth, Australia 7Birth Defects Epidemiology and Surveillance Branch, Texas Department of State Health Services, Austin, Texas 8Instituto Nacional de Genética Médica Populacional, Rio de Janeiro, Brazil 9Estudio Colaborativo Latino Americano de Malformaciones Congenitas at Centro de Educación Médica e Investigación Clínica, Buenos Aires, Argentina 10Estudio Colaborativo Latino Americano de Malformaciones Congenitas at Laboratório de Epidemiologia de Malformações Congênitas, Instituto Oswaldo Cruz, Fundação Oswaldo Cruz, Rio de Janeiro, Brazil 11Indagine Malformazioni congenite Emilia Romagna Registry, Department of Pediatrics, Bologna University, Bologna, Italy 12Metropolitan Atlanta Congenital Defects Program, Division of Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia 13Department of Medicine, University of Mississippi Medical Center, Jackson, Mississippi 14Department of Hungarian Congenital Abnormality Registry and Surveillance, National Center for Healthcare Audit and Inspection, Budapest, Hungary 15Division of Medical Genetics, University of Utah Health Sciences Center, Salt Lake City, Utah 16Utah Birth Defect Network, Utah Department of Health, Salt Lake City, Utah 17Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche S953, Epidemiological Research on Perinatal Health and Women’s and Children’s Health, Hôpital Cochin and UPMC University, Paris, France 18Department of Neonatology, Soroka University Medical Center, Beer-Sheba, Israel 19Alberta Congenital Anomalies Surveillance System, Alberta Health and Wellness, Calgary, Canada 20Dublin EUROCAT Registry, Health Service Executive, Dublin, Ireland 21Department of Neonatology, Rabin Medical Center, Beilinson Campus, Petah Tikva, Israel 22Congenital Anomaly Register for Wales, Singleton Hospital, Swansea, Wales, United Kingdom 23Malformation Monitoring Centre Saxony-Anhalt, Otto-von-Guericke University Magdeburg, Germany 24National Registry of Congenital Anomalies of the Czech Republic, Department of Medical Genetics, Thomayer University Hospital, Prague, Czech Republic 25Slovak Teratologic Information Centre, Slovak Medical University, Bratislava, Slovak Republic.BACKGROUND—The prevalence of esophageal atresia (EA) has been shown to vary across different geographical settings. Investigation of geographical differences may provide an insight into the underlying etiology of EA. METHODS—The study population comprised infants diagnosed with EA during 1998 to 2007 from 18 of the 46 birth defects surveillance programs, members of the International Clearinghouse for Birth Defects Surveillance and Research. Total prevalence per 10,000 births for EA was defined as the total number of cases in live births, stillbirths, and elective termination of pregnancy for fetal anomaly (ETOPFA) divided by the total number of all births in the population. RESULTS—Among the participating programs, a total of 2943 cases of EA were diagnosed with an average prevalence of 2.44 (95% confidence interval [CI], 2.35–2.53) per 10,000 births, ranging between 1.77 and 3.68 per 10,000 births. Of all infants diagnosed with EA, 2761 (93.8%) were live births, 82 (2.8%) stillbirths, 89 (3.0%) ETOPFA, and 11 (0.4%) had unknown outcomes. The majority of cases (2020, 68.6%), had a reported EA with fistula, 749 (25.5%) were without fistula, and 174 (5.9%) were registered with an unspecified code. CONCLUSIONS—On average, EA affected 1 in 4099 births (95% CI, 1 in 3954–4251 births) with prevalence varying across different geographical settings, but relatively consistent over time and comparable between surveillance programs. Findings suggest that differences in the prevalence observed among programs are likely to be attributable to variability in population ethnic compositions or issues in reporting or registration procedures of EA, rather than a real risk occurrence difference.2030-01-01