Clinical relevance of inflammation on rectal biopsy for Hirschsprung disease: An outcomes analysis.

OBJECTIVES: Inflammation on diagnostic rectal biopsy for children with suspected Hirschsprung disease (HSCR) is reported on pathology, and its significance is unknown. We describe the management and outcomes of a cohort with inflammation on rectal biopsy compared to those without. Specifically, to address the hypothesis that inflammation on diagnostic biopsy is associated with increased complication rates irrespective of intervention type and timing. METHODS: A single institution retrospective review of children with HSCR who underwent biopsy and endorectal pull-through (ERPT) from 2010 to 2020 was performed. The primary outcome was overall complications at 30-days following ERPT. Secondary outcomes included timing and type of operative intervention as well as postoperative enterocolitis diagnosed within 6-months of ERPT. RESULTS: Forty-nine children were identified; inflammation was present on diagnostic biopsy for 17 children. Those with inflammation were more likely to have clinical evidence of enterocolitis at the time of biopsy (p = 0.001) and were more likely to undergo leveling colostomy before ERPT (p = 0.01). Children with inflammation had a higher anastomotic leak rate (p = 0.04). Subgroup analysis of patients with inflammation undergoing primary ERPT versus leveling colostomy demonstrated no significant difference in outcomes following definitive ERPT. CONCLUSIONS: Our study suggests inflammation on diagnostic rectal biopsy for HSCR is associated with increased anastomotic leak rates. While additional prospective studies are indicated, attention to methods of mitigating inflammation and confirming its resolution before definitive pull-through may be of benefit for improving clinical outcomes in patients found with inflammation on diagnostic rectal biopsy.

Fecal continence outcomes and potential disparities for patients with anorectal malformations treated at referral institutions for pediatric colorectal surgery.

PURPOSE: Fecal incontinence is a problem for many patients born with an anorectal malformation (ARM) that can impact quality of life. It is unknown if racial, ethnic, and socioeconomic disparities relate to fecal continence in these children. We sought to examine outcomes and potential disparities in care. METHODS: We performed a multicenter retrospective study of children > 3y with ARM evaluated at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). The primary outcome was fecal continence. We evaluated for associations between fecal continence and race, sex, age, and insurance status. RESULTS: 509 patients with ARM from 11 institutions were included. Overall, 24% reported complete fecal continence, and fecal continence was associated with older age (p < .001). For school-aged children, 27% reported complete continence, while 53% reported none. On univariate analysis, patients with combined private and public insurance showed lower rates of continence when compared to those with private insurance (23 vs. 12%; p = 0.02). Age was associated with continence on univariate and multivariable analyses. CONCLUSION: Rates of complete fecal continence in this population are low. Differences based on payor status may exist. There were no observed disparities related to sex and race. Further investigation is warranted to improve care for this patient population. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Multi-institutional retrospective comparative study.

Venogram before long-interval repeat cannulation for pediatric extracorporeal membrane oxygenation.

It is rare for children to receive more than one course of support with extracorporeal membrane oxygenation, and in those who do undergo multiple episodes, the interval is usually days to weeks between events. Little data exists on re-cannulation years after an initial extracorporeal membrane oxygenation run, and late repeat cannulation can pose unique challenges. We report the case of a 10-year-old male patient with right jugular vein occlusion due to a previous course of extracorporeal membrane oxygenation as a neonate, who was successfully supported via central cannulation. This case demonstrates the importance of adequate imaging of target vasculature prior to attempting re-cannulation of a previously used vessel. Establishing a thoughtful strategy for late repeat cannulation is essential to achieve safe access in unusual and challenging situations.

Imaged-guided and muscle sparing laparoscopic anorectoplasty using real-time magnetic resonance imaging.

A challenge when repairing imperforate anus is positioning the neo-rectum into the center of the sphincter muscle complex (SMC) with limited muscle injury and scarring. Unfortunately, the path through the components of the SMC are often non-linear. We have used MRI to delineate the complex and guide the needle through the center using standard MRI-guidance (Raschbaum GR et al. J Pediatr Surg 45:220-223, 2010; Thomas TT et al. J Pediatr Surg 35:927-930, 2000). However, asynchronous scanning requires multiple, time-consuming scans to advance the needle in stepwise fashion. Asynchronous scanning also prevents visualizing the needle as it is advanced. We recently integrated software into the MRI operative suite that allows placement of the needle with real-time MRI. We report the feasibility and utility of real-time MRI-assisted laparoscopic assisted anorectoplasty (RT MRI-LAARP). Needle guidance was performed with Siemens Espree 1.5 T MRI with T1 Flash RT Sequence. After needle placement, laparoscopic mobilization, fistula takedown and pull-through was performed using the needle to guide dilation to create a tract to pull-through the neo-rectum. Charts of patients who underwent RT MRI-LAARP were reviewed. Demographics, anatomy, number of needle passes, OR duration and complications are reported. There were five children that underwent RT MRI-LAARP; one was a redo secondary to a retracted rectovestibular fistula. Operative time ranged from 187-505 min. Average hospital stay was 4.0 ± 1.0 days. There were no intraoperative complications although one patient had temporary urinary retention post-op. Muscle sparring laparoscopic anorectoplasty using real-time MRI is feasible and facilitates needle placement through the SMC.

Obtaining central access in challenging pediatric patients.

PURPOSE: Central catheter placement is one of the most commonly performed procedures by pediatric surgeons. Here, we present a case series of patients where central access was obtained at our institution with the utilization of a novel ultrasound-guided technique. This series represents the first of its kind where the native, parent vessels were inaccessible, resulting in a challenging situation for providers. METHODS: A retrospective chart review was performed in pediatric patients (0-17 years) at a tertiary care institution between July 2012 and November 2017 on all central line procedures where ultrasound was utilized to cannulate the brachiocephalic or superior vena cava in face of proximal occlusion. Our group has previous experience utilizing an image-guided in-plane approach to central line placement in the pediatric population. Demographics, operative characteristics, and postoperative complications were reviewed. RESULTS: A total of 11 procedures were included in this case series where the BC (N = 9) or SVC (N = 2) were cannulated for access. Internal jugular vein cannulation was attempted on each patient unless preoperative imaging demonstrated occlusion. The median operative time was 43 ± 23 min. Most procedures were performed on the right sided (63%), with catheters ranging from 4.2F single lumen to 14F double lumen. Since being placed, three (27%) catheters have been removed, with one due to non-use, one due to sepsis, and the final one due to malposition. CONCLUSION: With the continued need for long-term central access in the pediatric population, distal vein occlusion or inaccessibility can prove challenging when attempting to obtain central access. Here, demonstrated a safe alternative technique that provides an additional option in the pediatric surgeon's armamentarium for patients with difficult central access.

Redo pullthrough for Hirschsprung disease.

Pullthrough procedures for Hirschsprung diseases typically have favorable results. However, some children experience long-term postoperative complications comprising stooling disorders, such as intermittent enterocolitis, severe stool retention, intestinal obstruction, as well as incontinence. Reoperative Hirschsprung Disease surgery is complex. This begins with the workup after the initial presentation following primary pullthrough, continues with the definitive surgical correction with redo pullthrough, and ends with long-term follow-up of individuals. The decision tree can be varied with each patient. The operating pediatric surgeon must be able to utilize different operations and treatment options available. While lesser procedures may provide relief in a select population, those with residual aganglionosis or transition zone pathology or mechanical problems will likely require a redo pullthrough. Thus, the diagnostic workup, treatment plan, and definitive surgical care should be coordinated, and executed by an experienced, specialized team at a pediatric referral center.

Bacterial nutrient foraging in a mouse model of enteral nutrient deprivation: insight into the gut origin of sepsis.

Total parenteral nutrition (TPN) leads to a shift in small intestinal microbiota with a characteristic dominance of Proteobacteria This study examined how metabolomic changes within the small bowel support an altered microbial community in enterally deprived mice. C57BL/6 mice were given TPN or enteral chow. Metabolomic analysis of jejunal contents was performed by liquid chromatography/mass spectrometry (LC/MS). In some experiments, leucine in TPN was partly substituted with [13C]leucine. Additionally, jejunal contents from TPN-dependent and enterally fed mice were gavaged into germ-free mice to reveal whether the TPN phenotype was transferrable. Small bowel contents of TPN mice maintained an amino acid composition similar to that of the TPN solution. Mass spectrometry analysis of small bowel contents of TPN-dependent mice showed increased concentration of 13C compared with fed mice receiving saline enriched with [13C]leucine. [13C]leucine added to the serosal side of Ussing chambers showed rapid permeation across TPN-dependent jejunum, suggesting increased transmucosal passage. Single-cell analysis by fluorescence in situ hybridization (FISH)-NanoSIMS demonstrated uptake of [13C]leucine by TPN-associated bacteria, with preferential uptake by Enterobacteriaceae Gavage of small bowel effluent from TPN mice into germ-free, fed mice resulted in a trend toward the proinflammatory TPN phenotype with loss of epithelial barrier function. TPN dependence leads to increased permeation of TPN-derived nutrients into the small intestinal lumen, where they are predominately utilized by Enterobacteriaceae The altered metabolomic composition of the intestinal lumen during TPN promotes dysbiosis.

Functional Outcomes of Patients Who Underwent Anorectal Malformation Repair Using MRI Guidance.

BACKGROUND: Despite the initiation of minimally invasive laparoscopic techniques, the majority of patients who undergo anorectal malformation repair still experience functional bowel issues in childhood, including constipation and fecal incontinence. In this study, we evaluate the functional outcomes of a procedure in which magnetic resonance imaging guidance is used during initial laparoscopic repair to better locate the epicenter of the sphincter muscle complex and pelvic floor with the goal of more accurate placement of the neoanus and improved functional outcomes. METHODS: A retrospective chart review evaluated demographic, operative, and outcome details for patients who underwent this procedure. A telephone survey was employed to determine levels of social continence using the validated Baylor Continence Scale and to determine what type of bowel management is used. RESULTS: Twenty-six patients were included. Median age at operation was 7 months, and median age at follow-up was 4 years old, with a range of 1-9. Bowel management regimen results revealed that 19 % (n = 5) use no bowel management regimen, 58 % (n = 15) use laxatives only, and 23 % (n = 6) use enemas. Enema use was not associated with different spine or sacral anomalies (p = 0.77). Fifteen patients (58 %) answered the Baylor Continence Scale questions and had a median score of 14. No difference was found in scores when accounting for lesion level (p = 0.43), quality of needle placement (p = 0.46), or quality of sphincter muscles (p = 0.75). CONCLUSIONS: Using MRI guidance in the repair of anorectal malformations shows promise in both the qualitative and quantitative functional outcomes of this complex patient population. LEVEL OF EVIDENCE: Level III.

Outcomes From Colonic Pull-Through for Cloacal Exstrophy Differ by Colon Length: A Multi-Institutional Study.

BACKGROUND: Cloacal exstrophy (CE) represents a rare sub-group of anorectal malformations. Traditionally managed with a permanent colostomy, colonic pull-through (PT) has emerged to allow cleanliness without a life-long stoma. We sought to understand outcomes of PT in a large multi-center CE population. METHODS: We performed a retrospective study involving eleven pediatric hospitals. We gathered data on demographics, outcomes, and anatomical factors including colon length. Continuous variables were analyzed with Wilcoxon rank-sum tests and categorial variables with Fisher's exact tests. RESULTS: There were 98 patients, of which the majority (n = 70, 71.4 %) never underwent PT. There were no differences in exstrophy type, demographics, or associated anomalies. Median age at PT was 1.3 years (IQR 0.3-3.7). Of the cohort that continue to use their PT, the majority (n = 16, 69.6 %) are not clean. In total, 7.1 % (n = 7) of the cohort is clean with a PT, and only one patient is continent. Clean patients have a longer colon length than those who are not clean or opt for re-do ostomy (64.0 cm [IQR 46.0-82.0] vs 26.5 cm [IQR 11.6-41.2], p = 0.005). CONCLUSION: Overall, we demonstrate that most children born with CE will keep their stoma. Only a small percentage who elect to undergo colonic PT are clean for stool. Greater colon length correlates with success. This suggests that multiple factors, including colon length, are important when considering PT in a child with CE. LEVEL OF EVIDENCE: III.

Comparison of the PCPLC Database to NSQIP-P: A Patient Matched Comparison of Surgical Complications Following Repair of Anorectal Malformation.

BACKGROUND: Anorectal malformations (ARM) are rare and heterogenous which creates a challenge in conducting research and offering recommendations for best practice. The Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) was formed in 2016 to address this challenge and created a shared national data registry to collect information about pediatric colorectal patients. There has been no external validation of the data collected. We sought to evaluate the database by performing a patient matched analysis comparing 30-day outcomes identified in the PCPLC registry with the NSQIP-P database for patients undergoing surgical repair of ARM. METHODS: Patients captured in the PCPLC database from 2016 to 2021 at institutions also participating in NSQIP-P who underwent ARM repair younger than 12 months old were reviewed for 30-day complications. These patients were matched to their NSQIP-P record using their hospital identification number, and records were compared for concordance in identified complications. RESULTS: A total of 591 patient records met inclusion criteria in the PCPLC database. Of these, 180 patients were also reviewed by NSQIP-P. One hundred and fifty-six patient records had no complications recorded. Twenty-four patient records had a complication listed in one or both databases. There was a 91 % concordance rate between databases. When excluding complications not tracked in the PCPLC registry, this agreement improved to 93 %. CONCLUSION: Including all patients evaluated for this subpopulation, a 91 % concordance rate was observed when comparing PCPLC collected complications to NSQIP-P. Future efforts can focus on further validating the data within the PCPLC for other patient populations. LEVEL OF EVIDENCE: V.

Glucagon-like peptide 2 increases efficacy of distraction enterogenesis.

BACKGROUND: Application of distractive forces to small bowel induces intestinal growth, or enterogenesis. This emerging area of research may provide treatment for short bowel syndrome. Glucagon-like peptide 2(GLP-2) has also been reported to induce small bowel growth after bowel resection. We hypothesized that exogenous GLP-2 will result in enhanced distraction-induced enterogenesis. METHODS: Distraction-induced model was performed in 10-wk-old C57BL/6 mice using osmotic forces with high molecular weight polyethylene glycol (PEG)-stretch. Four groups were studied: Control group (PEG-/GLP-2-); PEG-stretch (PEG+/GLP-2-); GLP-2 control (PEG-/GLP-2+); and GLP-2 stretch (PEG+/GLP-2+). GLP-2 was given via subcutaneous osmotic pump over the 5 d of experiment. Morphology was measured by histomicrography. Epithelial cell (EC) proliferation was measured with proliferating cell nuclear antigen immunofluorescent staining. Total intestinal growth and blood vessel volume was assessed with Micro computed tomography volumetry. Vascular endothelial growth factor, fibroblast growth factor 1 and 2, and platelet-derived growth factor were measured by reverse-transcriptase polymerase chain reaction. RESULTS: EC proliferation increased significantly in all groups compared with controls, but was greatest in the GLP-2 stretch group. Diameter and length significantly increased in the PEG-stretch and GLP-2 stretch groups. Moreover, there was statistically greater diameter, crypt depth and EC proliferation in the GLP-2 stretch versus PEG-stretch groups. GLP-2 stretch vessel volume was greater than all other groups and was significantly increased compared with controls. The relative expression of platelet-derived growth factor increased significantly in the PEG-stretch group versus the Control group. CONCLUSIONS: GLP-2 had an additive effect on EC proliferation, tissue growth, histomorphology, and vascularization. We also demonstrated a unique action of GLP-2, the enhancement of intestinal vascularization. The combination of enterogenesis and GLP-2 may yield an improved approach to treat short bowel syndrome.

Development of a novel approach to safely couple the intestine to a distraction-induced device for intestinal growth: use of reconstructive tissue matrix.

BACKGROUND: Distraction-induced intestinal growth may be a novel treatment for short bowel syndrome. Longitudinal, distractive tension created by the application of force creates a significant challenge: to produce adequate force, yet not cause perforation at the fixation points. This paper describes our development of a coupling strategy to allow for successful bowel lengthening. METHODS: A curvilinear hydraulic device was implanted in an isolated Roux limb of small bowel in young Yorkshire pigs. Bowel was lengthened over a 2-week period. Study groups included: Group 1: Twelve silk transmural anchoring sutures into an engineered-coupling ring at the ends of each device. Group 2: Addition of felt pledgets to the coupling rings on the serosal surface of the small bowel. Group 3: Extraluminal use of either thin AlloDerm(®), thick AlloDerm(®), or Strattice™ mesh to anchor the device. RESULTS: Group 1 (suture-only) resulted in a gradual pulling through the suture with increasing tension and no lengthening. Felt pledgets eroded in a similar fashion, causing abdominal sepsis. Thin AlloDerm(®) failed to prevent erosion; however, it protected against gross contamination. Animals in which either thick AlloDerm(®) or Strattice™ mesh was used survived complication free to the study endpoint. Both thick AlloDerm(®) and Strattice™ prevented erosion and perforation allowing for an average of 10.85 cm expansion. CONCLUSION: This study demonstrates the use of either thick AlloDerm(®) or Strattice™ reconstructive tissue matrix which allows for safe and effective coupling. Further, we suggest this approach could be an adjunct to esophageal lengthening procedures.

Mesenteric neovascularization with distraction-induced intestinal growth: enterogenesis.

BACKGROUND: Distraction-induced enterogenesis, whereby the intestine lengthens with application of linear forces, is an emerging area which may provide a unique treatment for short bowel syndrome. With an increase in overall tissue mass, there is an increase in oxygen and nutrient demand. We hypothesized that a neovascularization within the mesentery is necessary to support the growing small bowel. METHODS: A curvilinear hydraulic device was used to induce growth within the small bowel of Yorkshire pigs, and the intestine was harvested after 14 days. High-resolution gross pictures were recorded of the mesentery at implantation and at harvest, and CT imaging of the bowel and mesentery was performed at harvest after dye injection. RESULTS: After 2 weeks of distraction, an average of 72.5 % (8.7 cm) bowel lengthening was achieved. Gross images of the mesentery between major vessels showed a blossoming of the microvasculature and this was confirmed by CT imaging with 3D reconstruction. Mesenteric sample taken from the distracted segment had a fourfold increase in the volume of microvasculature versus controls. CONCLUSION: Enterogenesis results not only in increased bowel length, but also significant increase in the mesenteric microvascularity. Presumably, this sustains the lengthened segment after application of longitudinal forces.

Ultrasound-Guided Enteropexy for Repair of Ostomy Prolapse.

Background: Enterostomies provide fecal diversion for numerous conditions, but anatomical complications-prolapse, stricture, and retraction-occur in up to 25%. Given up to 76% of these complications require surgical intervention, effective minimally invasive repair techniques for their management are needed. This article describes a new technique for prolapse repair utilizing image-guided surgery for incisionless repair of ostomy prolapse. Methods: To perform the procedure, the prolapsed bowel is reduced and evaluated for feasibility for ultrasound repair. Under direct ultrasound guidance sutures are used to pexy the bowel loop to the overlying fascia. Sutures are tied with knots and sutures buried below the skin to securely tack the bowel to the abdominal wall. Results: Four patients aged 2-10 years underwent ultrasound-guided enteropexy for repair of major prolapse of an end ileostomy (2 patients), loop colostomy, and end colostomy. All patients remained free of major prolapse for 3-10 months after the procedure, 2 of whom have progressed to ostomy takedown without complications. Conclusions: Ultrasound-guided enteropexy is a noninvasive effective way to manage ostomy prolapse.

Thoracoscopic Patch Repair of Congenital Diaphragmatic Hernia: Can Smaller Incisions Treat Larger Defects?

INTRODUCTION: Thoracoscopic CDH repair is increasingly performed for Type A and small Type B defects that are amenable to primary repair. However, the thoracoscopic approach is controversial for larger defects necessitating a patch due to technical complexity, intraoperative acidosis, and recurrence risk. We aim to compare clinical outcomes between thoracoscopic and open patch repair of Type B/C defects, using a standardized technique. METHODS: This is a single-center retrospective review of thoracoscopic and open CDH patch repairs January 2017-December 2021. We excluded primary repairs, Type D hernias, repairs on ECMO, recurrent repairs. Various preoperative, intraoperative, and postoperative variables were compared. Primary outcome was recurrence rate. Secondary outcomes included intraoperative pH and pCO2, operative time, and complication rates. RESULTS: Twenty-nine patients met inclusion criteria (open = 13, thoracoscopic = 16). The open cohort had lower o/e total fetal lung volume (29 vs 41.2%, p = 0.042), higher preoperative peak inspiratory pressures (24 vs 20 cm H2O, p = 0.007), were more frequently Type C defects (92.3 vs 31%, p = 0.002) and had liver "up" in left-sided hernias (46 vs 0%, p < 0.0001). Intraoperatively, median lowest pH and highest pCO2 did not differ; neither did overall median pH or pCO2. Operative times were similar (153 vs 194 min, p = 0.113). No difference in recurrence rates was identified, however postoperative complications were higher in the open group. There were no mortalities. CONCLUSIONS: Although we demonstrate higher disease severity of patients undergoing open repair, thoracoscopic patch repair for Type B/C defects is safe and effective in patients with favorable physiologic status, alleviating concerns for intraoperative acidosis, operative length, and risk of recurrence. LEVEL OF EVIDENCE: II.

Do preoperative factors predict success of antegrade continence enemas in children?

INTRODUCTION: Antegrade continent enemas (ACE) procedures are one treatment option in children with medically refractory constipation or encopresis and predicting success is difficult. We hypothesize that there are preoperative factors that can be identified to help with patient selection and family counseling. METHODS: We conducted a retrospective study of children who underwent a cecostomy or appendicostomy for an ACE program between 2015 and 2021. Underlying diagnosis, pre-operative bowel regimen and imaging were analyzed. Patients were reviewed for success at 3-, 6- and 12-months post-procedure. Data was analyzed with Fisher's Exact, Kruskal-Wallis and logistic regression where applicable with significance defined as p < 0.05. RESULTS: Forty-three children were identified; 28 were male, 15 were female, mean age at time of operation was 8 years old. 76% were considered successful at 3-months, 86% at 6-months, and 87% at 12-months post- procedure. Univariate analysis showed that a pre-ACE retrograde enema program predicted success at 3-months (94% vs. 64% p = 0.03) but no difference at 6- or 12-months. At one year after ACE procedure there was a significant reduction in number of enteral medications (2 to 0, p < 0.01) and 94% of patients were on one or fewer at one year follow-up. Age, gender, weight at time of operation, contrast enema, anorectal manometry and colonic transit time results were not predictive of outcomes. CONCLUSION: In this study, we characterized expected time to success in our population as well as identified use of a pre-operative retrograde enema program as a potential predictor of success at 3-months in children undergoing an ACE procedure. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Prognosis study.

A Pilot Study: Transcriptional Profiling, Functional Analysis, and Organoid Modeling of Intestinal Mucosa in Hirschsprung Disease.

BACKGROUND: Hirschsprung disease (HSCR) is a congenital colonic aganglionosis. Many HSCR patients develop enterocolitis despite surgical resection. The pathophysiology of this inflammatory process is poorly understood. We compared transcriptional profiles and function of ganglionic and aganglionic tissue in HSCR patients. METHODS: RNA sequencing was performed on mucosal tissues from HSCR patients (n = 6) and controls (n = 3). Function of matched ganglionic and aganglionic regions were investigated utilizing organoids generated from these tissues. RESULTS: Transcriptional differences observed in ganglionic and aganglionic regions of HSCR patients included upregulation of genes involving inflammation, cell differentiation and proliferation as well as decreased expression of genes encoding mucins compared to controls. Organoids derived from ganglionic and aganglionic regions of HSCR patients were similar in epithelial cell differentiation, epithelial barrier formation and response to stimulation with bacterial metabolites and pro-inflammatory cytokines. CONCLUSIONS: Despite normal ganglionic structure, the section of colon adjacent to the aganglionic region in HSCR patients has perturbed gene expression which resembles the aganglionic segment. Transcriptional and functional changes in colonic epithelium are persevered in the ganglionic colon used for pull-through surgery. This may explain persistence of enterocolitis despite surgical excision of aganglionic colon and subsequent endorectal pull-through performed with ganglionic colon during correction of HSCR. LEVEL OF EVIDENCE: N/A.

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.

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.

Fecal continence disparities in patients with idiopathic constipation treated at referral institutions for pediatric colorectal surgery.

PURPOSE: Fecal continence is a concern for many patients with idiopathic constipation and can significantly impact quality of life. It is unknown whether racial, ethnic, and socioeconomic disparities are seen in fecal continence within the idiopathic constipation population. We aimed to evaluate fecal continence and associated demographic characteristics in children with idiopathic constipation referred for surgical evaluation. METHODS: A multicenter retrospective study of children with idiopathic constipation was performed at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). All patients >3y of age with idiopathic constipation diagnosis were included. The primary outcome was fecal continence, categorized as complete (no accidents ever), daytime (no accidents during the day), partial (occasional incontinence day/night), and none (incontinent). We evaluated for associations between fecal continence and race, sex, age, insurance status, and other patient-level factors, employing Kruskal-Wallis and trend tests. RESULTS: 458 patients with idiopathic constipation from 12 sites were included. The median age of diagnosis was 4.1 years. Only 25% of patients referred for surgical evaluation were completely continent. Age at the visit was significantly associated with fecal continence level (p = 0.002). In addition, patients with public and mixed public and private insurance had lower levels of continence (p<0.001). Patients with developmental delay were also more likely to have lower continence levels (p = 0.009) while diagnoses such as anxiety, ADD/ADHD, autism, depression, obsessive-compulsive disorder were not associated. Approximately 30% of patients had an ACE operation (antegrade continence enema) at a median age of 9.2 years at operation. Black patients were significantly less likely to undergo ACE operation (p = 0.016) when compared to white patients. CONCLUSION: We observed data that suggest differences in fecal incontinence rates based on payor status. Further investigation is needed to characterize these potential areas of disparate care. LEVEL OF EVIDENCE: Level III.

Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group.

BACKGROUND/PURPOSE: A small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients. METHODS: Retrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale. RESULTS: There were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD. CONCLUSION: More than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment. LEVEL OF EVIDENCE: Level 4.