TY - JOUR
T1 - Prevalence and mortality among children with anorectal malformation
T2 - A multi-country analysis
AU - Kancherla, Vijaya
AU - Sundar, Manasvi
AU - Lucita, Tandaki
AU - Lux, Anke
AU - Bakker, Marian K.
AU - Bergman, Jorieke E.H.
AU - Bermejo-Sánchez, Eva
AU - Canfield, Mark A.
AU - Dastgiri, Saeed
AU - Feldkamp, Marcia L.
AU - Gatt, Miriam
AU - Groisman, Boris
AU - Hurtado-Villa, Paula
AU - Kallen, Kärin
AU - Landau, Danielle
AU - Lelong, Nathalie
AU - Lopez-Camelo, Jorge
AU - Martinez, Laura Elia
AU - Mastroiacovo, Pierpaolo
AU - Morgan, Margery
AU - Mutchinick, Osvaldo M.
AU - Nance, Amy E.
AU - Nembhard, Wendy N.
AU - Pierini, Anna
AU - Sipek, Antonin
AU - Stallings, Erin B.
AU - Szabova, Elena
AU - Tagliabue, Giovanna
AU - Wertelecki, Wladimir
AU - Zarante, Ignacio
AU - Rissmann, Anke
N1 - Publisher Copyright:
© 2022 The Authors. Birth Defects Research published by Wiley Periodicals LLC.
PY - 2023/2/1
Y1 - 2023/2/1
N2 - Purpose: We examined the total prevalence, trends in prevalence, and age-specific mortality among individuals with anorectal malformation (ARM).Methods: We conducted a retrospective cohort study using data from 24 population- and hospital-based birth defects surveillance programs affiliated with the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR) from 18 countries and for births from 1974 to 2014. We estimated pooled and program-specific total prevalence per 10,000 total births. Poisson regression was used to assess time trends in prevalence from 2001 to 2012 when most programs contributed data. We calculated selected age-specific proportions of deaths, stratified by case status.Results: The pooled total prevalence of ARM was 3.26 per 10,000 total births (95% Confidence Interval = 3.19, 3.32) for birth years 1974–2014. About 60% of cases were multiple or syndromic. Prevalence of multiple, syndromic, and stillborn cases decreased from 2001 to 2012. The first week mortality proportion was 12.5%, 3.2%, 28.3%, and 18.2% among all, isolated, multiple, and syndromic cases, respectively.Conclusions: ARM is relatively rare, with multiple and syndromic cases showing decreasing prevalence during the study period. Mortality is a concern during the first week of life, and especially among multiple and syndromic cases. Our descriptive epidemiological findings increase our understanding of geographic variation in the prevalence of ARM and can be used to plan needed clinical services. Exploring factors influencing prevalence and mortality among individuals with ARM could inform future studies.
AB - Purpose: We examined the total prevalence, trends in prevalence, and age-specific mortality among individuals with anorectal malformation (ARM).Methods: We conducted a retrospective cohort study using data from 24 population- and hospital-based birth defects surveillance programs affiliated with the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR) from 18 countries and for births from 1974 to 2014. We estimated pooled and program-specific total prevalence per 10,000 total births. Poisson regression was used to assess time trends in prevalence from 2001 to 2012 when most programs contributed data. We calculated selected age-specific proportions of deaths, stratified by case status.Results: The pooled total prevalence of ARM was 3.26 per 10,000 total births (95% Confidence Interval = 3.19, 3.32) for birth years 1974–2014. About 60% of cases were multiple or syndromic. Prevalence of multiple, syndromic, and stillborn cases decreased from 2001 to 2012. The first week mortality proportion was 12.5%, 3.2%, 28.3%, and 18.2% among all, isolated, multiple, and syndromic cases, respectively.Conclusions: ARM is relatively rare, with multiple and syndromic cases showing decreasing prevalence during the study period. Mortality is a concern during the first week of life, and especially among multiple and syndromic cases. Our descriptive epidemiological findings increase our understanding of geographic variation in the prevalence of ARM and can be used to plan needed clinical services. Exploring factors influencing prevalence and mortality among individuals with ARM could inform future studies.
KW - anorectal atresia
KW - birth defect
KW - epidemiology
KW - mortality
KW - prevalence
U2 - 10.1002/bdr2.2129
DO - 10.1002/bdr2.2129
M3 - Article
C2 - 36401554
AN - SCOPUS:85142275133
SN - 2472-1727
VL - 115
SP - 390
EP - 404
JO - Birth Defects Research
JF - Birth Defects Research
IS - 3
ER -