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Multi-Institutional Review From the Pediatric Colorectal and Pelvic Learning Consortium of Minor Spinal Cord Dysraphism in the Setting of Anorectal Malformations: Diagnosis, Treatment, and Outcomes. J Pediatr Surg 2023 Aug;58(8):1582-1587

Date

05/30/2023

Pubmed ID

37248140

DOI

10.1016/j.jpedsurg.2023.04.009

Scopus ID

2-s2.0-85160320068 (requires institutional sign-in at Scopus site)

Abstract

BACKGROUND/RATIONALE: Anorectal malformations (ARM) are associated with congenital anomalies of the spine, but the impact of a minor spinal cord dysraphism (mSCD) on fecal continence in the setting of ARM remains unclear.

MATERIALS/METHODS: A retrospective review was performed utilizing data from the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. The patient cohort was reviewed for ARM type, mSCD screening/incidence/neurosurgical intervention and age-based BMP utilization.

RESULTS: 987 patients with ARM were categorized into mild (38%), moderate (32%) or complex (19%). 694 (70%) had normal spinal (NS) status. 271 (27.5%) patients had mSCD. MRI alone (49%) was the most common screening test for mSCD. US screening had a positive predictive value of 86.3% and a negative predictive value of 67.1%. Surgical intervention rates for mSCD ranged between 13% and 77% at a median age of 0.6-5.2 years. 726 (73.6%) patients were prescribed BMP (74.4% NS, 77.5% mSCD). Laxatives were most utilized BMP in all groups <5yo. ≥5yo, enema utilization increased with ARM complexity independent of spine status (with or without neurosurgical intervention). Neurosurgical intervention did not affect BMP utilization at any age or with any ARM when mSCD was identified.

CONCLUSIONS: MSCD influence on bowel function in the setting ARM remains unclear. No significant impact of mSCD was noted on ARM patient bowel management program utilization. Variability exists within PCPLC site with screening and intervention for mSCD in patients with ARM. Future studies with standardized care may be needed to elucidate the true impact of mSCD on long term patient outcomes in ARM patients.

TYPE OF STUDY: Retrospective Comparative Study.

LEVEL OF EVIDENCE: III.

Author List

Garvey EM, Fuller M, Frischer J, Calkins CM, Rentea RM, Ralls M, Wood R, Rollins MD, Avansino J, Reeder RW, Durham MM, Pediatric Colorectal and Pelvic Learning Consortium (PCPLC)

Author

Casey Matthew Calkins MD Professor in the Surgery department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Anorectal Malformations
Child
Child, Preschool
Colorectal Neoplasms
Heart Defects, Congenital
Humans
Infant
Neural Tube Defects
Retrospective Studies
Spinal Cord
Spinal Dysraphism