Complete VACTERL evaluation is needed in newborns with rectoperineal fistula

Given that a rectoperineal fistula is developmentally the most mature lesion in the spectrum of anorectal malformations, it is not clear whether it merits a complete VACTERL evaluation. We sought to determine if the same evaluation is required to rule out associated anomalies in newborns with rectoperineal fistula as those with more complex anorectal malformations.

We performed a retrospective review of the pediatric colorectal center database at our tertiary care children's hospital from 2000 to 2012. Patients with anorectal malformations were categorized as rectoperineal fistula or "other" using the Krickenbeck classification. Records were reviewed to identify associated anomalies.

308 patients (156 males) were treated at our institution during the time period (rectoperineal fistula=102). Thirty-five (34%) patients with a perineal fistula had at least one associated anomaly. The most common anomalies were cardiac lesions (29% excluding PFO and PDA), genitourinary (20.6%), and malformations of the spine (15.7%). The overall occurrence of anomalies was lower than the "other" group.

Our review demonstrates that newborns with a rectoperineal fistula frequently have associated anomalies and should undergo an evaluation similar to more complex lesions. These findings illustrate the importance of a structured approach to the evaluation of even the most straightforward lesions.