APSA PaperComplete VACTERL evaluation is needed in newborns with rectoperineal fistula
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.
References (12)
- et al.
Anorectal malformations
Clin Perinatol
(2012) - et al.
Analysis of 1,992 patients with anorectal malformations over the past two decades in Japan. Steering Committee of Japanese Study Group of Anorectal Anomalies
J Pediatr Surg
(1999) - et al.
Immediate and long-term results of surgical management of low imperforate anus in girls
J Pediatr Surg
(1998) - et al.
Anomalies associated with anorectal malformations according to the Krickenbeck anatomic classification
J Pediatr Surg
(2012) - et al.
Associated malformations in patients with anorectal anomalies
Eur J Med Genet
(2007) - et al.
Genetics of Hirschsprung disease and anorectal malformations
Semin Pediatr Surg
(2010)
Cited by (18)
High-grade Vesicoureteral Reflux in Patients With Anorectal Malformation From the ARM-Net Registry: Is Our Screening Sufficient?
2024, Journal of Pediatric SurgeryThe Importance of Screening for Additional Anomalies in Patients with Anorectal Malformations: A Retrospective Cohort Study
2023, Journal of Pediatric SurgeryCitation Excerpt :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.
Screening for VACTERL Anomalies in Children with Anorectal Malformations: Outcomes of a Standardized Approach
2023, Journal of Pediatric SurgeryPerineal cutaneous appendix and anorectal malformation
2023, Anales de PediatriaCorrelation of anorectal malformation complexity and associated urologic abnormalities
2021, Journal of Pediatric SurgeryCitation Excerpt :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.
Preoperative cardiopulmonary evaluation in specific neonatal surgery
2019, Seminars in Pediatric Surgery