Prevalence and mortality among children with anorectal malformation: A multi‐country analysis

Abstract

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.

Treatment of Anorectal Malformations in German Hospitals: Analysis of National Hospital Discharge Data from 2016 to 2021

“…In total, 1,726 newborn cases with ARM in the 6 years correspond to an incidence of 1:2,730 (4.71 million live births in the time period 12 ), which is comparable to the incidence that was reported by Kancherla et al 3 ARMs are rarely accessible to prenatal diagnostics. 13 Therefore, a decentralized distribution at the time of birth in 388 institutions with a median (P25-P75) of 1 (1-2) is plausible.…”

“…1,2 Among these, anorectal malformations (ARMs) are the most common at a rate of 1:2,800 live births. 3 ARMs are a spectrum of different anomalies of the anorectum with the essential commonality of a mislocated opening of the anus. In most cases, the urogenital tract is also affected, and ARM is commonly associated with multiple congenital malformations of other organs.…”

Benchmarks for Pediatric Surgical Registries: Recommendations for the Assessment and Grading of Complications

“…38 A wide spectrum of diseases is covered in the registry and several analyses of pediatric surgical diseases, including foregut, midgut, and hindgut atresias, have been published over the years. [39][40][41] The EUROCAT registry reports primarily address the perinatal mortality of children with congenital anomalies. Garne et al were the only researchers to conduct an analysis of surgical complications in children with gastrointestinal malformations in Denmark using the EUROCAT data.…”

Bowel function, urinary tract function, and health-related quality of life in males with anorectal malformations

“…Congenital anorectal malformations (ARM) are one of many congenital anomalies among newborns. The birth prevalence of ARM in Sweden is approximately 1:3000 [ 1 , 2 ]. There is a small predominance of male infants born with ARM with a sex ratio of 1.3:1 [ 1 ].…”