Gastroschisis prevalence patterns in 27 surveillance programs from 24 countries, International Clearinghouse for Birth Defects Surveillance and Research, 1980–2017

Author:

Feldkamp Marcia L.1ORCID,Canfield Mark A.2,Krikov Sergey1,Prieto‐Merino David3,Šípek Antonin4,LeLong Nathalie5,Amar Emmanuelle6,Rissmann Anke7ORCID,Csaky‐Szunyogh Melinda8,Tagliabue Giovanna9,Pierini Anna10,Gatt Miriam11,Bergman Jorieke E. H.12ORCID,Szabova Elena13,Bermejo‐Sánchez Eva14,Tucker David15,Dastgiri Saeed16,Bidondo María Paz17,Canessa Aurora18,Zarante Ignacio19,Hurtado‐Villa Paula20,Martinez Laura21,Mutchinick Osvaldo M.22ORCID,Camelo Jorge Lopez23,Benavides‐Lara Adriana24ORCID,Thomas Mary Ann25,Liu Shiliang26,Nembhard Wendy N.27,Gray Elizabeth B.28,Nance Amy E.29ORCID,Mastroiacovo Pierpaolo30,Botto Lorenzo D.31

Affiliation:

1. Division of Medical Genetics, Department of Pediatrics University of Utah School of Medicine Salt Lake City Utah USA

2. Birth Defects Epidemiology and Surveillance Branch, Texas Department of State Health Services Austin Texas USA

3. Faculty of Medicine Universidad de Alcalá Madrid Spain

4. Czech Republic Department of Medical Genetics Thomayer Hospital Prague Czech Republic

5. Université Paris Cité, Centre of Research in Epidemiology and StatisticS (CRESS), Obstetrical Perinatal and Pediatric Epidemiology Research Team (EPOPé), INSERM, INRA Paris France

6. France REMERA, Registre des malformations en Rhône Alpes, Hospices Civils de Lyon Lyon France

7. Malformation Monitoring Centre Saxony‐Anhalt, Medical Faculty Otto‐von‐Guericke‐University Magdeburg Magdeburg Germany

8. Hungarian Congenital Anomalies Registry and Rare Diseases Centre, National Center for Public Health and Pharmacy Budapest Hungary

9. Lombardy Congenital Anomalies Registry, Cancer Registry Unit, Fondazione IRCCS, Istituto Nazionale dei tumori Milan Italy

10. Unit of Epidemiology of Rare Diseases and Congenital Anomalies Institute of Clinical Physiology, National Research Council and Fondazione Toscana Gabriele Monasterio, Tuscany Registry of Congenital Defects Pisa Italy

11. Malta Congenital Anomalies Registry, Directorate for Health Information and Research Pieta Malta

12. Department of Genetics, University of Groningen University Medical Center Groningen Groningen the Netherlands

13. Faculty of Public Health Slovak Medical University in Bratislava Bratislava Slovak Republic

14. ECEMC (Spanish Collaborative Study of Congenital Malformations), CIAC (Research Center on Congenital Anomalies) Institute of Rare Diseases Research (IIER), Instituto de Salud Carlos III Madrid Spain

15. Congenital Anomaly Register & Information Service for Wales, Public Health Wales, Knowledge Directorate, Singleton Hospital Sketty Lane Swansea UK

16. Health Services Management Research Centre Tabriz University of Medical Sciences Tabriz Iran

17. National Network of Congenital Anomalies of Argentina (RENAC), National Institute of Epidemiology (INE) National Administration of Laboratories and Health Institutes, National Ministry of Health Institutes Buenos Aires Argentina

18. Regional Register Congenital Malformation Maule Health Service (RRMC‐SSM) Maule Chile

19. Instituto de Genética Humana, Pontificia Universidad Javeriana Bogotá Bogotá Colombia

20. Facultad de Ciencias de la Salud, Pontificia Universidad Javeriana Cali Cali Colombia

21. Mexico ReDeCo Monterrey Nuevo Leon Mexico

22. Department of Genetics, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán RYVEMCE, Registry and Epidemiological Surveillance of Congenital Malformations Mexico City Mexico

23. ECLAMC, Center for Medical Education and Clinical Research (CEMIC‐CONICET) Buenos Aires Argentina

24. Costa Rican Birth Defects Register Center (CREC) Costa Rican Institute for Research and Teaching in Nutrition and Health (INCIENSA) Cartago Costa Rica

25. Department of Medical Genetics and Pediatrics Alberta Congenital Anomalies Surveillance System, Alberta Children's Hospital Calgary Alberta Canada

26. Canadian Congenital Anomalies Surveillance System (CCASS), Centre for Surveillance and Applied Research, Public Health Agency of Canada Ottawa Ontario Canada

27. Department of Epidemiology, Fay W. Boozman College of Public Health University of Arkansas for Medical Sciences and Arkansas Reproductive Health Monitoring System, Arkansas Children's Research Institute Little Rock Arkansas USA

28. Metropolitan Atlanta Congenital Defects Program, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention Atlanta Georgia USA

29. Utah Birth Defect Network, Office of Children with Special Care Needs, Division of Family Health, Utah Department of Health and Human Services Salt Lake City Utah USA

30. International Center on Birth Defects, International Clearinghouse for Birth Defects Surveillance and Research Rome Italy

31. Department of Pediatrics The University of Utah Salt Lake City Utah USA

Abstract

AbstractBackgroundGastroschisis is a serious birth defect with midgut prolapse into the amniotic cavity. The objectives of this study were to evaluate the prevalence and time trends of gastroschisis among programs in the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR), focusing on regional variations and maternal age changes in the population.MethodsWe analyzed data on births from 1980 to 2017 from 27 ICBDSR member programs, representing 24 countries and three regions (Europe+ (includes Iran), Latin America, North America). Cases were identified using diagnostic codes (i.e., 756.7, 756.71, or Q79.3). We excluded cases of amniotic band syndrome, limb–body wall defect, and ruptured omphalocele. Programs provided annual counts for gastroschisis cases (live births, stillbirths, and legally permitted pregnancy terminations for fetal anomalies) and source population (live births, stillbirths), by maternal age.ResultsOverall, gastroschisis occurred in 1 of every 3268 births (3.06 per 10,000 births; 95% confidence intervals [CI]: 3.01, 3.11), with marked regional variation. European+ prevalence was 1.49 (95%CI: 1.44, 1.55), Latin American 3.80 (95%CI: 3.69, 3.92) and North American 4.32 (95%CI: 4.22, 4.42). A statistically significant increasing time trend was observed among six European+, four Latin American, and four North American programs. Women <20 years of age had the highest prevalence in all programs except the Slovak Republic.ConclusionsGastroschisis prevalence increased over time in 61% of participating programs, and the highest increase in prevalence was observed among the youngest women. Additional inquiry will help to assess the impact of the changing maternal age proportions in the birth population on gastroschisis prevalence.

Publisher

Wiley

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