Birth prevalence of anorectal malformations in England and 5-year survival: a national birth cohort study

Abstract

ObjectiveTo determine the birth prevalence, maternal risk factors and 5-year survival for isolated and complex anorectal malformations.DesignNational birth cohort using hospital admission data and death records.SettingAll National Health Service England hospitals.PatientsLive-born singletons delivered from 2002 through 2018, with evidence in the first year of life of a diagnosis of an anorectal malformation and repair during a hospital admission, or anorectal malformation recorded on the death certificate. Cases were further classified as isolated or complex depending on the presence of additional anomalies.Main outcome measuresBirth prevalence of anorectal malformations per 10 000 live births, risk ratios for isolated and complex anorectal malformation by maternal, infant and birth characteristics, and 5-year survival.ResultsWe identified 3325 infants with anorectal malformations among 9 474 147 live-born singletons; 61.7% (n=2050) of cases were complex. Birth prevalence was 3.5 per 10 000 live births (95% CI 3.4 to 3.6). Complex anorectal malformations were associated with maternal age extremes after accounting for other sociodemographic factors. Compared with maternal ages 25–34 years, the risk of complex anorectal malformations was 31% higher for ≥35 years (95% CI 17 to 48) and 13% higher for ≤24 years (95% CI 0 to 27). Among 2376 anorectal malformation cases (n=1450 complex) born from 2002 through 2014, 5-year survival was lower for complex (86.9%; 95% CI 85.1% to 88.5%) than isolated anorectal malformations (98.2%; 95% CI 97.1% to 98.9%). Preterm infants with complex anorectal malformations had the lowest survival (73.4%; 95% CI 68.1% to 78.0%).ConclusionsDifferences in maternal risk factors for isolated and complex anorectal malformations may reflect different underlying mechanisms for occurrence. Five-year survival is high but lowest for preterm children with complex anorectal malformations.

Malformaciones anorrectales en pediatría

“…• Consumo de altas dosis de cafeína • Consumo de medicamentos para el asma Su incidencia en las malformaciones anorrectales ha sido determinada a través del uso de métodos retrospectivos y técnicas de muestreo sistemático (5,(10)(11)(12).…”

“…La supervivencia a cinco años es alta, pero más baja para los niños prematuros con malformaciones anorrectales complejas. En la mayoría de los casos, las muertes por una ARM se dan durante la primera semana de vida (3,12). El acceso a la información y la atención clínica juegan un papel muy importante en el pronóstico del paciente, pues estos factores pueden influir significativamente en la mortalidad asociada a esta enfermedad (6).…”

Clinical application of middle descending colon-double lumen ostomy with distal stoma narrowing in the treatment of anorectal malformation

“…Anorectal malformations (ARMs) are the most common congenital anomaly of the digestive tract, with an incidence of approximately 3.5 in 10,000 live-births based on a national birth cohort study in England [1]. According to the Krickenbeck diagnosis classification, which combines the advantages of the Wingspread classification with the classification of Pe˜na, ARMs are classified into perineal fistulas, rectourethral fistulas, rectovesical fistulas, vestibular fistulas, cloacal malformations, patients with no fistula, and anal stenosis [2].…”

Anorectal Malformations and Late-Term Problems

“… 1 Its incidence is 2-3.5:10 000, and it is slightly more common among boys than girls. 2 , 3 The most common variant is atresia with rectourethral fistula in boys and atresia with rectovestibular fistula in girls. 2 Five percent of the patients have anal atresia without fistula, and this variant is mainly associated with trisomy 21.…”

“…While trisomy 21 is seen in half of these patients, over 90% of patients with anal atresia diagnosed with trisomy 21 have atresia without fistula. 3 , 4 …”