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

Marcia L Feldkamp; Mark A Canfield; Sergey Krikov; David Prieto-Merino; Antonin Šípek Jr; Nathalie LeLong; Emmanuelle Amar; Anke Rissmann; Melinda Csaky-Szunyogh; Giovanna Tagliabue; Anna Pierini; Miriam Gatt; Jorieke E H Bergman; Elena Szabova; Eva Bermejo-Sánchez; David Tucker; Saeed Dastgiri; María Paz Bidondo; Aurora Canessa; Ignacio Zarante; Paula Hurtado-Villa; Laura Martinez; Osvaldo M Mutchinick; Jorge Lopez Camelo; Adriana Benavides-Lara; Mary Ann Thomas; Shiliang Liu; Wendy N Nembhard; Elizabeth B Gray; Amy E Nance; Pierpaolo Mastroiacovo; Lorenzo D Botto

doi:10.1002/bdr2.2306

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

Birth Defects Res. 2024 Feb;116(2):e2306. doi: 10.1002/bdr2.2306.

Authors

Marcia L Feldkamp¹, Mark A Canfield², Sergey Krikov¹, David Prieto-Merino³, Antonin Šípek Jr⁴, Nathalie LeLong⁵, Emmanuelle Amar⁶, Anke Rissmann⁷, Melinda Csaky-Szunyogh⁸, Giovanna Tagliabue⁹, Anna Pierini¹⁰, Miriam Gatt¹¹, Jorieke E H Bergman¹², Elena Szabova¹³, Eva Bermejo-Sánchez¹⁴, David Tucker¹⁵, Saeed Dastgiri¹⁶, María Paz Bidondo¹⁷, Aurora Canessa¹⁸, Ignacio Zarante¹⁹, Paula Hurtado-Villa²⁰, Laura Martinez²¹, Osvaldo M Mutchinick²², Jorge Lopez Camelo²³, Adriana Benavides-Lara²⁴, Mary Ann Thomas²⁵, Shiliang Liu²⁶, Wendy N Nembhard²⁷, Elizabeth B Gray²⁸, Amy E Nance²⁹, Pierpaolo Mastroiacovo³⁰, Lorenzo D Botto³¹

Affiliations

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

Background: Gastroschisis 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.

Methods: We 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.

Results: Overall, 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.

Conclusions: Gastroschisis 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.

Keywords: gastroschisis; geographic region; multinational; prevalence; surveillance.

MeSH terms

Female
Gastroschisis* / epidemiology
Hernia, Umbilical* / epidemiology
Humans
Infant, Newborn
Limb Deformities, Congenital*
Maternal Age
Pregnancy
Prevalence
Stillbirth

Grants and funding

CC999999/ImCDC/Intramural CDC HHS/United States