ARM in ARM? Investigating the co-occurrence of anorectal malformations and labioscrotal anomalies

Anorectal malformations (ARMs) are known to be associated with other defects, however there is no evidence addressing the co-occurrence of ARMs with anomalies of structures derived from the labioscrotal folds. We performed a systematic review (Prospero: CRD420251013945) aiming at investigating correlations and patterns between ARMs and labioscrotal anomalies (LSAs), when presenting simultaneously. Our search identified 1642 articles. After abstract screening, 344 full texts were assessed and 144 met the inclusion criteria. In total, these reported on 319 individual cases with both ARM and LSA. Individuals with complex ARMs had proportionally the most LSAs associated with their ARM - 58.4% of these were associated with only a single LSA. For 'high' and 'low' ARMs, 66.0% and 77.0% of cases were associated with a single LSA respectively. As data reporting prevalence of each type of ARM lacks information about LSAs, the potential to predict the presence of LSAs according to type of ARM is limited. Nevertheless, this review demonstrates a frequent association of ARMs with LSAs, that has not been previously described. This evidence highlights the importance of early screening and timely management of LSAs in patients with any type of ARM, with close collaboration between paediatric surgeons, urologists and other clinicians.