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To describe current bowel management program (BMP) strategies in anorectal malformation (ARM) patients based on patient-level predictors using data from a multi-institutional consortium.Patient bowel function and BMP were reviewed from Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) data. The PCPLC is comprised of multidisciplinary specialists researching colorectal and pelvic disorders. Seven US institutions submitted de-identified clinical data on ARM patients into a centralized patient registry.The primary ARM of 624 patients was categorized into Mild (45.2%), Moderate (40.4%) or Complex (14.2%) anomaly classifications. Patient-specific BMP were examined based on age and on the presence of spinal cord/sacral anomalies. 418 (67%) enrolled patients were prescribed BMP (5 yo 56.4%; ≥5-12 yo 86.7%; ≥12 81.5%). Constipation was the primary chief complaint (80.2%). Forty percent of patients on a BMP were toilet trained and approximately one-half (48.5%) reported daytime stool accidents. Secondary surgical interventions for antegrade continence enemas (ACE) were examined; 14.5% of patients employed ACE strategies and utilization increased with age and varied based on anatomic anomalies.This is the first report on BMP strategies for patients with ARM from the Pediatric Colorectal and Pelvic Learning Consortium. Individual patient characteristics are explored for their impact on bowel management strategy utilization.IV.

Author(s): Greenberg, Benjamin M; Casper, Theron Charles; Mar, Soe S; Ness, Jayne M; Plumb, Patricia; Liang, Shannon; Goyal, Manu; Weinstock-Guttman, Bianca; Rodriguez, Moses; Aaen, Gregory S; Belman, Anita; Barcellos, Lisa F; Rose, John W; Gorman, Mark P; Benson, Leslie A; Candee, Meghan; Chitnis, Tanuja; Harris, Yolanda C; Kahn, Ilana L; Roalstad, Shelly; Hart, Janace; Lotze, Timothy E; Rensel, Mary; Rubin, Jennifer P; Schreiner, Teri L; Tillema, Jan-Mendelt; Waldman, Amy Tara; Krupp, Lauren; Graves, Jennifer; Drake, Kaylea; Waubant, Emmanuelle | Abstract: Background and objectiveThe objective of this study was to determine whether family members of patients with pediatric multiple sclerosis (MS) have an increased prevalence of autoimmune conditions compared with controls.MethodsData collected during a pediatric MS case-control study of risk factors included information about various autoimmune diseases in family members. The frequency of these disorders was compared between cases and controls.ResultsThere was an increased rate of autoimmune diseases among family members of pediatric MS cases compared with controls with first-degree history of MS excluded (OR = 2.27, 95% CI 1.71-3.01, p l 0.001). There was an increased rate of MS among second-degree relatives of pediatric MS cases compared with controls (OR = 3.47, 95% CI 1.36-8.86, p = 0.009). The OR for MS was 2.64 when restricted to maternal relatives and 6.37 when restricted to paternal relatives.DiscussionThe increased rates of autoimmune disorders, including thyroid disorders and MS among families of patients with pediatric MS, suggest shared genetic factors among families with children diagnosed with pediatric MS.

Introduction The current understanding of Hirschsprung-associated enterocolitis (HAEC) is based mainly on single-center, retrospective studies. The aims of this study are to determine risk factors for postoperative HAEC using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) database. Materials and Methods We performed a multicenter, retrospective, case–control study of children with Hirschsprung disease (HD) who had undergone a pull-through procedure and were evaluated at a PCPLC member site between February 2017 and March 2020. The cohort with a history of postoperative HAEC was compared with that without postoperative episodes of HAEC to determine relevant associations with postoperative HAEC. Results One-hundred forty of 299 (46.8%) patients enrolled had a history of postoperative HAEC. Patients with a rectosigmoid transition zone had a lower association with postoperative HAEC as compared with those with a more proximal transition zone (odds ratio [OR]: 0.46, 95% confidence interval [CI]: 0.26, 0.84, p Conclusion Patients with a rectosigmoid transition zone have less postoperative HAEC compared with patients with a more proximal transition zone. Multi-institutional collection of clinical information in patients with HD may allow for the identification of additional risk factors for HAEC and afford the opportunity to improve care.