TY - JOUR
T1 - A multi-country study of prevalence and early childhood mortality among children with omphalocele.
AU - Nembhard, Wendy N.
AU - Bergman, Jorieke E. H.
AU - Politis, Maria D.
AU - Arteaga-Vázquez, Jazmín
AU - Bermejo-Sánchez, Eva
AU - Canfield, Mark A.
AU - Cragan, Janet D.
AU - Dastgiri, Saeed
AU - de Walle, Hermien E. K.
AU - Feldkamp, Marcia L.
AU - Nance, Amy
AU - Gatt, Miriam
AU - Groisman, Boris
AU - Hurtado-Villa, Paula
AU - Kallén, Kärin
AU - Landau, Danielle
AU - Lelong, Nathalie
AU - Lopez-Camelo, Jorge
AU - Martinez, Laura
AU - Morgan, Margery
AU - Pierini, Anna
AU - Rissmann, Anke
AU - Šípek, Antonin
AU - Szabova, Elena
AU - Tagliabue, Giovanna
AU - Wertelecki, Wladimir
AU - Zarante, Ignacio
AU - Bakker, Marian K.
AU - Kancherla, Vijaya
AU - Mastroiacovo, Pierpaolo
PY - 2020/12/1
Y1 - 2020/12/1
N2 - BACKGROUND: Omphalocele is the second most common abdominal birth defect and often occurs with other structural and genetic defects. The objective of this study was to determine omphalocele prevalence, time trends, and mortality during early childhood, by geographical region, and the presence of associated anomalies. METHODS: We conducted a retrospective study with 23 birth defect surveillance systems in 18 countries who are members of the International Clearinghouse for Birth Defects Surveillance and Research that submitted data on cases ascertained from 2000 through 2012, approximately 16 million pregnancies were surveyed that resulted in live births, stillbirths, or elective terminations of pregnancy for fetal anomalies (ETOPFA) and cases with omphalocele were included. Overall prevalence and mortality rates for specific ages were calculated (day of birth, neonatal, infant, and early childhood). We used Kaplan-Meier estimates with 95CI) to calculate cumulative mortality and joinpoint regression for time trend analyses. RESULTS: The prevalence of omphalocele was 2.6 per 10,000 births (95 2.5, 2.7) and showed no temporal change from 2000-2012 (average annual percent change = -0.19 p = .52). The overall mortality rate was 32.195 30.2, 34.0). Most deaths occurred during the neonatal period and among children with multiple anomalies or syndromic omphalocele. Prevalence and mortality varied by registry type (e.g., hospital- vs. population-based) and inclusion or exclusion of ETOPFA. CONCLUSIONS: The prevalence of omphalocele showed no temporal change from 2000-2012. Approximately one-third of children with omphalocele did not survive early childhood with most deaths occurring in the neonatal period.
AB - BACKGROUND: Omphalocele is the second most common abdominal birth defect and often occurs with other structural and genetic defects. The objective of this study was to determine omphalocele prevalence, time trends, and mortality during early childhood, by geographical region, and the presence of associated anomalies. METHODS: We conducted a retrospective study with 23 birth defect surveillance systems in 18 countries who are members of the International Clearinghouse for Birth Defects Surveillance and Research that submitted data on cases ascertained from 2000 through 2012, approximately 16 million pregnancies were surveyed that resulted in live births, stillbirths, or elective terminations of pregnancy for fetal anomalies (ETOPFA) and cases with omphalocele were included. Overall prevalence and mortality rates for specific ages were calculated (day of birth, neonatal, infant, and early childhood). We used Kaplan-Meier estimates with 95CI) to calculate cumulative mortality and joinpoint regression for time trend analyses. RESULTS: The prevalence of omphalocele was 2.6 per 10,000 births (95 2.5, 2.7) and showed no temporal change from 2000-2012 (average annual percent change = -0.19 p = .52). The overall mortality rate was 32.195 30.2, 34.0). Most deaths occurred during the neonatal period and among children with multiple anomalies or syndromic omphalocele. Prevalence and mortality varied by registry type (e.g., hospital- vs. population-based) and inclusion or exclusion of ETOPFA. CONCLUSIONS: The prevalence of omphalocele showed no temporal change from 2000-2012. Approximately one-third of children with omphalocele did not survive early childhood with most deaths occurring in the neonatal period.
KW - prevalence
KW - mortality
KW - surveillance
KW - omphalocele
KW - registry
U2 - 10.1002/bdr2.1822
DO - 10.1002/bdr2.1822
M3 - Article
VL - 112
SP - 1787
EP - 1801
JO - Birth Defects Research
JF - Birth Defects Research
SN - 2472-1727
IS - 20
ER -