APSA Paper
Complete VACTERL evaluation is needed in newborns with rectoperineal fistula

https://doi.org/10.1016/j.jpedsurg.2013.09.039Get rights and content

Abstract

Purpose

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.

Methods

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.

Results

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.

Conclusion

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.

Section snippets

Methods

We performed a retrospective review of the pediatric colorectal center database at our tertiary care children’s hospital from January 2000 to September 2012. This database was created with the institution of a formal Pediatric Colorectal Clinic in 2011. Patients were categorized based on the Krickenbeck classification [9] after thorough review of the operative and imaging reports. Patients in whom the exact lesion could not be determined from the reports or who were repaired at another

Results

308 patients with ARMs (51% male) were treated at our institution during the time period (rectoperineal fistula = 102). Specific types of ARMs are demonstrated in Table 1. Sixty-three patients with rectoperineal fistula were male (61.7%). Rectoperineal fistula was encountered in 40% of males with ARMs and 26% of females. The specific associated anomalies identified in our cohort according to organ system are listed in Table 2. Patent foramen ovale (n = 31) and patent ductus arteriosus (n = 24) were

Discussion

It is generally accepted that patients with complex ARMs require a thorough evaluation for associated anomalies but in patients with rectoperineal fistulas, this work- up may be variably performed. This is perhaps due to the fact that occasionally these patients present in a delayed fashion with constipation and the assumption is made that a benign lesion does not require an extensive work-up for associated anomalies. We identified a deficiency in the work-up of patients with rectoperineal

Conclusion

Our review demonstrates that newborns with a rectoperineal fistula frequently have associated anomalies and should undergo a thorough evaluation similar to those with more complex lesions. These associated malformations may be even more common than demonstrated in our series given the significant number of patients who were incompletely evaluated. These findings illustrate the importance of a structured approach to the evaluation of even the most straightforward lesions.

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    This could be explained by the thorough screening that was performed in patients with complex as well as more simple types of ARM. In our opinion, and in accordance with a previous study, routine screening is necessary for all ARM patients to early detect potential (life-threatening) anomalies and thus improve short- and long-term (functional) outcomes [29]. In addition, routine screening is of great importance to prevent missing additional anomalies with potential severe consequences in the future.

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    Previous studies suggest that an association exists between severity of ARM subtype and the rate of urologic anomalies, although this remains incompletely understood [3,4,9]. For this reason, there remains no consensus on the utility of screening patients with “minor” anomalies, such as perineal fistula, for associated urologic anomalies [13,14]. The purpose of this study was to utilize a large, single institutional database of children with ARM to better evaluate the relationship between ARM severity and associated urologic diagnoses and to assess the incidence of urologic abnormalities in children with ARM to determine if the severity of ARM is correlated with a greater incidence of urologic abnormalities.

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