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Constipation is common in childhood, and most patients can be successfully managed by their primary care provider. However, some patients will require more specialized management either due to an underlying congenital colorectal disorder such as Hirschsprung disease or anorectal malformation or due to severe functional constipation that is refractory to medical management. [ABSTRACT FROM AUTHOR]

As pediatric patients with colorectal diseases grow, it is important to address transition to adult practice. We aim to describe our center's transition process and early outcomes. We developed a standardized process for transition to adult practice. An annual survey is given to parents and caregivers starting at age 12 that assesses knowledge of disease, independence with healthcare tasks, and confidence and interest regarding transition. After multidisciplinary review, those eligible are recommended for transition. Those not referred are provided with tools to help with areas of weakness. Outcomes were analyzed with descriptive and regression analyses (significance at p ≤ 0.05). A total of 116 patients were evaluated, with 80 patients (69.0%) recommended for transition. Median age at survey was 15.5 years [IQR: 13.7–18.1], and those recommended were older (16.6 years [IQR: 14.7–19.4] vs 13.5 years [IQR: 12.5–14.9], p < 0.001)). Primary diagnosis and gender were not associated with recommendation for transition. Overall, a minority (18.1%) were able to complete healthcare tasks; this correlated strongly with transition recommendation (26.3% vs 0.0%, p < 0.0001). On regression controlling for age, diagnosis, knowledge, and confidence, age (aOR 1.98, 95% CI 1.44–2.71) and confidence (aOR 3.78, 95% CI 1.29–11.11) independently predicted transition recommendation. A standardized approach may be effective in transitioning patients from pediatric to adult colorectal surgery practice. Patients who transition are more confident and can perform healthcare tasks independently; however, these skills are not essential prior to a recommendation of transition. III. • A standardized approach to transition to adult practice can be useful in identifying pediatric patients ready for transition to adult practice and successfully transitioning these patients. • Factors that aligned with successful transition recommendation in this sample included older age, ability to perform healthcare tasks independently, and confidence with transition to adult practice. [ABSTRACT FROM AUTHOR]

Antegrade colonic enemas (ACE) can be an effective management option for defecation disorders and improve quality of life. Best practice regarding channel placement is unclear and variation may exist around preferred initial type of channel, age at placement, and underlying diagnoses. We aimed to describe practice patterns and patient characteristics around ACE channel placement. We conducted a multicenter retrospective study of children with an ACE channel cared for at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) from 2017 to 2022. Kruskal–Wallis test was utilized to test the age at surgery by site with significance level of 0.05. 500 patients with ACE channel were included. 293 (58.6%) patients had their ACE procedure at a PCPLC center. The median age at surgery was 7.6 [IQR 5.3–11.0] years for the overall cohort and 8.1 [IQR 5.3–11.5] years for placement at PCPLC centers. For PCPLC centers, median age at placement varied significantly across centers (p = 0.009). 371 (74.2%) patients received Malone appendicostomy, 116 (23.2%) received cecostomy, and 13 (2.6%) received Neo-Malone appendicostomy. Median age of patients by channel type was 7.7 [IQR 5.3–11.0], 7.5 [IQR 5.7–11.0], and 9.8 [IQR 4.2–11.6] years, respectively. The most common indication for cecostomy was idiopathic/refractory constipation (52.6%), whereas anorectal malformation was the most common indication for Malone (47.2%) and Neo-Malone (61.5%). Among ACE channels placed at PCPLC centers, there was variation across institutions in preferred initial channel type. The 4 highest volume centers favored Malone appendicostomy over cecostomy. There is variation in practice of ACE channel placement. At specialty pediatric colorectal centers, age at time of placement and type of channel placed varied across institutions. Further work is needed to better characterize diagnosis- and age-focused patient centered outcomes to clarify recommendations for our patients who benefit from these procedures. Retrospective comparative study. Level III. [ABSTRACT FROM AUTHOR]

Hirschsprung Disease (HD) is a rare cause of functional bowel obstruction in children. Patients are typically diagnosed in the neonatal period and undergo pull-through (PT) soon after diagnosis. The optimal management and post-operative outcomes of children who present in a delayed fashion are unknown. A multi-center retrospective review of children with HD was performed at participating Pediatric Colorectal and Pelvic Learning Consortium sites. Children were stratified by age at diagnosis (neonates <29 days; infants 29 days–12 months; toddler 1 year–5 years and child >5 years). 679 patients with HD from 14 sites were included; Most (69%) were diagnosed in the neonatal period. Age at diagnosis was not associated with differences in 30-day complication rates or need for PT revision. Older age at diagnosis was associated with a greater likelihood of undergoing fecal diversion after PT (neonate 10%, infant 12%, toddler 26%, child 28%, P < 0.001) and a greater need for intervention for constipation or incontinence postoperatively (neonate 56%, infant 62%, toddler 78%, child 69%, P < 0.001). Delayed diagnosis of HD does not impact 30-day post-operative outcomes or need for revision surgery but, delayed diagnosis is associated with increased need for fecal diversion after pull-through. III [ABSTRACT FROM AUTHOR]

Despite surgical advances for complex congenital colorectal conditions, such as anorectal malformation (ARM) and Hirschsprung disease (HD), many adolescents require transfer from specialist pediatric to adult providers for ongoing care. A systematic review of PubMed, MEDLINE and Embase was conducted to identify what is known about the transitional care of patients with ARM and HD (PROSPERO # CRD42022281558). The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) framework guided our reporting of studies that focused on the transition care of 10–30-year-olds with ARM and HD. Eight studies were identified that included patient and parent (n = 188), and/or clinician perspectives (n = 334). Patients and clinicians agreed that transitional care should commence early in adolescence to support transfer to adult care when a suitable level of maturation is reached. There was little evidence from patients that transfer happened in a timely or coordinated manner. Patients felt that clinicians did not always understand the significance of transfer to adult services. No models of transition care were identified. Surgeons ranked ARM and HD as the most common conditions to experience delayed transfer to adult care. Beyond pediatric surgeons, patients also highlighted the importance of general practitioners, transitional care coordinators and peer support groups for successful transition. There is little research focused on transitional care for patients with ARM and HD. Given evidence of delayed transfer and poor experiences, the development of models of transitional care appears essential. • The nature of health issues faced by adolescents with congenital colorectal conditions, and the health professionals who care for them, is complex, but poorly described in the literature. We did not identify any successful models of transitional care for this surgical cohort. [ABSTRACT FROM AUTHOR]

Studies of mental health in adolescents with Hirschsprung disease (HD) are scarce. This cross-sectional study investigates mental health, psychosocial functioning and quality of life in HD adolescents. Adolescents (12–18 years) treated at the Department of pediatric surgery at Oslo University Hospital were invited for participation. Mental health was assessed by interview; Child Assessment Schedule (CAS) and questionnaires; parental Child Behavior Checklist (CBCL) and adolescent Youth Self-Report (YSR). Psychosocial functioning was rated by Child Global Assessment Scale (cGAS). Adolescent Quality of Life was assessed by Pediatric Quality of Life inventory (PedsQL) and chronic family difficulties (CFD) by interview. Medical records were reviewed for somatic history. Thirty-seven adolescents, 28 males, median age 14.3 years, participated. By CAS interview, 8 of 37 (44% of females and 14% of males) fulfilled criteria for psychiatric diagnosis all within emotional and related disorders. Twenty-seven percent had CBCL internalizing scores and 16% had YSR internalizing scores in clinical range indicating emotional problems. By interviewer rated cGAS, 27% were scored in clinical range. By PedsQL 16% reported reduced psychosocial health score. Increased CFD, lower psychosocial functioning and reduced QoL as well as less paternal education were significantly associated with psychiatric diagnosis. Twice as many (4/8) adolescents who either had a stoma or bowel management had a psychiatric diagnosis compared to those who had neither stoma nor bowel management (7/28). Nearly one in four adolescents with HD fulfilled criteria for psychiatric diagnosis. Mental health problems were associated with reduced psychosocial function and reduced QoL. III. [ABSTRACT FROM AUTHOR]

Studies examining functional outcomes and health-related quality of life for patients with congenital surgical anomalies (CSA) are increasingly common. However, the prevalence of mental health disorders in this population has not been determined. The purpose of this review is to summarize the reported prevalence of mental health disorders in children born with gastrointestinal CSA. A systematic review of the literature was conducted on Medline (OVID), EMBASE (OVID), CINAHL (EbscoHost), and Scopus to identify studies reporting mental health diagnoses in children with a gastrointestinal CSA. A meta-analysis of the prevalence of anxiety disorders and depressive disorders was completed. Of 2546 manuscripts, seven met the inclusion criteria. All included manuscripts looked at patients with anorectal malformation (ARM) and/or Hirschsprung disease (HD). No studies assessed other gastrointestinal CSAs. A total of 183 psychiatric diagnoses were reported in 1167 patients. A pooled analysis of ARM patients revealed a 14.0% prevalence of anxiety disorders (N = 208; CI 95 = 4.4–23.4%) and an 18.75% prevalence of depressive disorders (N = 48; CI 95 = 7.7–29.8%). A pooled analysis of HD patients revealed a 15.8% prevalence of anxiety disorders (N = 19; CI 95 = 0.6–32.2%) and a 4.75% prevalence of depressive disorders (N = 758; CI 95 = 3.23–6.26%). There is a paucity of evidence on mental health outcomes of individuals born with gastrointestinal CSA. However, the available evidence suggested a high prevalence of mental health diagnoses in individuals born with ARM or HD with 1 in 7 having an anxiety disorder. Additionally, 1 in 5 ARM patients and 1 in 20 HD patients were diagnosed with a depressive disorder. Systematic review and meta-analysis. IV. • What is currently known about this topic? Limited studies on mental health disorders in children with congenital surgical anomalies have indicated a potential increased prevalence of these disorders. • What new information is contained in this article? Mental health disorders, anxiety, and depression occur more commonly in children born with anorectal malformations and Hirschsprung disease than the general population. [ABSTRACT FROM AUTHOR]

The lifelong impact of Hirschsprung disease (HD) upon children and their families is increasingly well recognized. Parental psychosocial wellbeing and family functioning are determinants of psychological and health-related outcomes in children with chronic conditions. We performed a cross-sectional cohort study to evaluate the psychosocial functioning of parents/caregivers of children with HD, beyond early childhood. Parents/caregivers of children with HD, aged 4–14 years, managed at a tertiary pediatric surgical center were surveyed. Parent psychosocial outcomes, including adjustment to illness and family response, were assessed using four validated measures: Family Management Measure (FaMM); Parent Experience of Child Illness (PECI); Patient Reported Outcomes Measurement Information System (PROMISR) anxiety; and PROMISR depression. The Pediatric Quality of Life Inventory (PedsQL) was administered to assess child quality of life (proxy-report). Forty parents (mean age 38.7 ± 5.6 years) of children with HD (mean age 8.0 ± 2.5) participated. Parents expressed greater long-term uncertainty (PECI) and poorer perceived condition management ability (FaMM) than comparator chronic disease cohorts. Other scores for parental adjustment to their child's condition (PECI) and family response (FaMM) were comparable to reference cohorts. Symptoms of anxiety and depression were prevalent in our cohort (52.5 % and 42.5 % respectively); however, the proportion with moderate - severe PROMISR anxiety (χ 2 = 2.50, p = 0.114) and depression (χ 2 = 0.156, p = 0.693) scores did not significantly differ from the expected population distribution. Proxy-reported child quality of life (PedsQL) was significantly reduced relative to healthy children (p = 0.0003), but comparable to those with physical health problems with special healthcare needs (p = 0.624). Parents of children with HD experience long-term uncertainty and have poorer perceived condition management ability than parents of children with other chronic childhood illnesses. This work highlights the importance of targeted parental education and support beyond primary surgical management, and provides a benchmark for this cohort, against which subsequent intervention-based studies may be assessed. II. [ABSTRACT FROM AUTHOR]

Hirschsprung Disease (HD) is typically diagnosed in the neonatal period. A small subset of patients have a prolonged course of abdominal distention and constipation prior to diagnosis. Late HD is defined as having been diagnosed at greater than or equal to one year of age. The literature is limited and offers conflicting data on the implications of a late diagnosis. We aim to investigate the presentation, operative approach, and functional outcomes of a large cohort of patients with a late HD diagnosis. All patients with a late diagnosis of HD (after 1 year of age) at our institution between 1997 and 2021 were included. Twenty-eight patients were diagnosed with HD at a median age of 3.4 years. Chronic constipation, failure to thrive, and enterocolitis occurred in 100 %, 31 %, and 14 %, respectively. All patients underwent contrast enema and biopsies during their workup, identifying primarily rectosigmoid disease (n = 27) and total colonic aganglionosis (n = 1). Surgical intervention was performed in 27 patients, with 4 patients (15 %) needing a stoma (3 with plan for staged pull-through, 1 long-term stoma) and 23 patients (85 %) undergoing a single-stage pull-through. Postoperative complications included Hirschsprung-associated enterocolitis (n = 5), ostomy prolapse and revision (n = 2), abdominal distention requiring ileostomy creation (n = 2), redo pull-through (n = 2), retroperitoneal hematoma (n = 1), and cecostomy tube placement (n = 1). At a median follow-up of 5.4 years, 83 % of eligible patients achieved fecal continence with 43 % needing laxatives for persistent constipation. Recognizing a late presentation of HD requires a high index of suspicion. Patients with a late diagnosis did not experience an increased rate of permanent stoma, complications, or redo surgery compared to rates reported for the larger HD population. Similar long-term functional outcomes were achieved compared to the larger HD population. IV. • Late diagnosis of Hirschsprung Disease is marked by a long-standing history of constipation with most patients having rectosigmoid disease. • Immediate post-operative and long-term functional outcomes in patients with a late diagnosis of Hirschsprung Disease are comparable to outcomes in the larger population with Hirschsprung Disease. [ABSTRACT FROM AUTHOR]

Total colonic aganglionosis (TCA) is a rare variant of Hirschsprung disease (HD) where the colon and portion of distal ileum lack ganglion cells. Most pediatric use either a straight ileoanal (Swenson or Yancey-Soave) or a short Duhamel pull-through for TCA. There are no large studies comparing these techniques. We aimed to compare short-and medium-term outcomes between these approaches. A retrospective review was performed among children with TCA from 2001 to 2019 undergoing a primary Duhamel or Swenson pull-through across three large children's hospitals. Patients undergoing redo and patients with greater than 30 % small bowel aganglionosis were excluded. We gathered data on demographics, operative approach, and outcomes at one, two, and three years. Continuous variables were analyzed with t-tests and categorical variables with Chi square or Fisher's tests. There were 54 patients, with 26 (48 %) undergoing Duhamel and 28 (52 %) undergoing Swenson pull-through. There were no differences in sex, age, medical comorbidities, or operative details, including age at pull-through, laparoscopic vs open, length of involved small bowel, and operative time. Length of stay and post-operative complications were not different. Three years after pull-through, patients undergoing Duhamel had fewer stools per day (1-3 stools 69.6 % vs 14.3 %, p = 0.003) and were less likely to be prescribed fiber supplementation (4.2 % vs 43.8 %, p = 0.003). There were no differences in irrigations, botulinum toxin administration, loperamide, or HD admissions. Both Duhamel and straight pull-throughs are safe for treatment of TCA, with acceptable short- and medium-term outcomes. Further studies on patient-reported outcomes are necessary to examine long-term differences. III. • Total colonic aganglionosis (TCA) is difficult to manage operatively. Multiple approaches, including Swenson and Duhamel pull-throughs, are all acceptable options but have not been well studied in comparison to one another. • We performed an analysis of short- and medium-term outcomes following Swenson and Duhamel pull-throughs on children with TCA. • Both Duhamel and Swenson pull-throughs were safe with minimal incidence of post-operative complications. • At three years post-procedure, there was a statistically significant trend towards lower number of stools in patients with a Duhamel pull-through compared to those with a Swenson pull-through; otherwise, there were no clinically significant differences between populations. [ABSTRACT FROM AUTHOR]

A proximal resection margin greater than 5 cm from the intra-operative histologically determined transition zone has been deemed necessary to minimize the risk of transition zone pull-through. This extended resection may require the sacrifice of vascular supply and even further bowel resection. The impact of extended proximal resection margin on post-operative complications and functional outcomes is unclear. A retrospective chart review of patients who underwent primary pull-through for Hirschsprung disease at a single institution between January 2008 and December 2022 was performed. An adequate proximal margin was defined by a circumferential normally ganglionated ring and absence of hypertrophic nerves. The extended margin was defined as the total length of proximal colon with normal ganglion cells and without hypertrophic nerves. Fecal incontinence severity was assessed with the Pediatric Fecal Incontinence Severity Score (PFISS). Eighty seven patients met criteria for inclusion. Median age at primary pull-through was 17 days (IQR 10–92 days), 55% (n = 48) of patients had an extended proximal margin (EPM) ≤ 5 cm, and 45% (n = 39) had an EPM > 5 cm. An EPM ≤5 cm was not associated with increased rates of Hirschsprung associated enterocolitis (≤5 cm 43%, >5 cm 39%, P = 0.701), diversion post pull-through (≤5 cm 10%, >5 cm 5%, P = 0.367) or reoperation for transition zone pull-through (≤5 cm 3%, >5 cm 0%, P = 0.112). EPM ≤5 cm had more frequent involuntary daytime bowel movements (P = 0.041) and more frequent voluntary bowel movements (P = 0.035). There were no differences in other measures of fecal incontinence severity. Shorter proximal extended margins beyond the adequate ganglionated margin do not significantly impact post-operative complication rates and have an unclear effect on fecal incontinence. Case Control. Level III. [ABSTRACT FROM AUTHOR]

Adults with Hirschsprung disease (HD) and anorectal malformations (ARM) may experience persisting and new somatic and psychosocial problems. Patient education programs (PEPs) may improve self-management in patients with chronic illnesses. The aim of this study was to explore HD and ARM adults' experiences with and evaluation of a group-based PEP. We also looked at factors that might influence the attendance rate. Non-intellectually impaired HD and ARM adults were invited to attend a diagnosis specific PEP at a pelvic floor interdisciplinary center. Eight health care professionals lectured. Aspects of the PEP were graded anonymously in a patient reported experience measure (PREM). Ethical approval was obtained. 17% (21/125) of invited adults (10HD, 11ARM) attended four PEPs. 19/21 (90%) PREMs were returned. Participants found meeting peers and sharing experiences especially valuable in addition to improved disease knowledge. Lectures by the pediatric and colorectal surgeons, stoma nurse, and sexologist were rated highest by the participants. The majority reported that the PEP would be helpful in managing everyday life. All participants recommended PEP in adolescence. Factors such as gender and travel distance did not affect attendance rate, but participants were older than non-participants, median 37 versus 24 years (p = 0.01). Attendance rates were low among HD and ARM adults invited to a PEP, but participants were overall highly satisfied. Peer support, mutual learning, and increased disease knowledge were seen as invaluable assets of the PEP. A web-based PEP was discouraged, while physical PEPs for both adults and adolescents were encouraged. III. [ABSTRACT FROM AUTHOR]

Transition of care (TOC; from childhood into adulthood) of patients with anorectal malformations (ARM) and Hirschsprung disease (HD) ensures continuation of care for these patients. The aim of this international study was to assess the current status of TOC and adult care (AC) programs for patients with ARM and HD. A survey was developed by members of EUPSA, ERN eUROGEN, and ERNICA, including patient representatives (ePAGs), comprising of four domains: general information, general questions about transition to adulthood, and disease-specific questions regarding TOC and AC programs. Recruitment of centres was done by the ERNs and EUPSA, using mailing lists and social media accounts. Only descriptive statistics were reported. In total, 82 centres from 21 different countries entered the survey. Approximately half of them were ERN network members. Seventy-two centres (87.8%) had a self-reported area of expertise for both ARM and HD. Specific TOC programs were installed in 44% of the centres and AC programs in 31% of these centres. When comparing centres, wide variation was observed in the content of the programs. Despite the awareness of the importance of TOC and AC programs, these programs were installed in less than 50% of the participating centres. Various transition and AC programs were applied, with considerable heterogeneity in implementation, content and responsible caregivers involved. Sharing best practice examples and taking into account local and National Health Care Programs might lead to a better continuation of care in the future. III. [ABSTRACT FROM AUTHOR]

Hirschsprung's disease (HSCR) is often associated with other congenital and chromosomal defects. This study aimed to describe the prevalence of congenital heart disease (CHD) and congenital urogenital lesions in children with HSCR, with and without Trisomy 21 and other associated anomalies, to guide appropriate screening. The Pediatric Health Information System was queried for patients with HSCR who underwent surgical treatment between 2016 and 2021. The prevalence of CHD, congenital urogenital lesions, Trisomy 21 and other congenital syndromes were calculated. Multivariable regression modeling was used to identify predictors of postoperative intensive care unit (ICU) admission and postoperative length of stay (LOS). Of 2021 HSCR patients at 47 children's hospitals, 264 (13.1%) had CHD, 244 (12.1%) had Trisomy 21, and 103 (5.1%) had a congenital urogenital lesion. The prevalence of CHD (49.6 vs. 8.1%, P < 0.001) and of undergoing a cardiac intervention with associated CHD (40.5 vs. 23.1%, P = 0.002) were higher in patients with Trisomy 21 compared to those without. CHD was associated with an increased likelihood of postoperative ICU admission (OR: 1.6, 95% CI: 1.1, 2.2) and greater postoperative LOS (IRR: 2.6, 95% CI: 2.6, 2.7), irrespective of Trisomy 21 diagnosis. The prevalence of CHD among HSCR patients (13.1%) was higher than previously reported, and CHD patients required more resource-intensive care after pull-through. While Trisomy 21 was associated with higher rates of CHD and cardiac intervention, 8.1% of HSCR patients without Trisomy 21 had CHD. Screening echocardiogram should be considered in all children diagnosed with HSCR, as CHD may influence perioperative risk stratification. However, screening renal ultrasound may have limited utility given the low prevalence of urogenital lesions in this population. Level III. [ABSTRACT FROM AUTHOR]

Transition zone pull-through (TZPT) is incomplete removal of the aganglionic bowel/transition zone (TZ) in patients with Hirschsprung disease (HD). Evidence on which treatment generates the best long-term outcomes is lacking. The aim of this study was to compare the long-term occurrence of Hirschsprung associated enterocolitis (HAEC), requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively to patients with TZPT treated with redo surgery to non-TZPT patients. We retrospectively studied patients with TZPT operated between 2000 and 2021. TZPT patients were matched to two control patients with complete removal of the aganglionic/hypoganglionic bowel. Functional outcomes and quality of life was assessed using Hirschsprung/Anorectal Malformation Quality of Life questionnaire and items of Groningen Defecation & Continence together with occurrence of Hirschsprung associated enterocolitis (HAEC) and requirement of interventions. Scores between the groups were compared using One-Way ANOVA. The follow-up duration lasted from time at operation until follow-up. Fifteen TZPT-patients (six treated conservatively, nine receiving redo surgery) were matched with 30 control-patients. Median duration of follow-up was 76 months (range 12–260). No significant differences between groups were found in the occurrence of HAEC (p = 0.65), laxatives use (p = 0.33), rectal irrigation use (p = 0.11), botulinum toxin injections (p = 0.06), functional outcomes (p = 0.67) and quality of life (p = 0.63). Our findings suggest that there are no differences in the long-term occurrence of HAEC, requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively or with redo surgery and non-TZPT patients. Therefore, we suggest to consider conservative treatment in case of TZPT. [ABSTRACT FROM AUTHOR]

The Coronavirus Disease 2019 pandemic provided a natural experiment to study the effect of social distancing on the risk of developing Hirschsprung's Associated Enterocolitis (HAEC). Using the Pediatric Health Information System (PHIS), a retrospective cohort study of children (<18 years) with Hirschsprung's Disease (HSCR) across 47 United States children's hospitals was performed. The primary outcome was HAEC admissions per 10,000 patient-days. The exposure (COVID-19) was defined as April 2020–December 2021. The unexposed (historical control) period was April 2018–December 2019. Secondary outcomes included sepsis, bowel perforation, intensive care unit (ICU) admission, mortality, and length of stay. Overall, we included 5707 patients with HSCR during the study period. There were 984 and 834 HAEC admissions during the pre-pandemic and pandemic periods, respectively (2.6 vs. 1.9 HAEC admissions per 10,000 patient-days, incident rate ratio [95% confidence interval]: 0.74 [0.67, 0.81], p < 0.001). Compared to pre-pandemic, those with HAEC during the pandemic were younger (median [IQR]: 566 [162, 1430] days pandemic vs. 746 [259, 1609] days pre-pandemic, p < 0.001) and more likely to live in the lowest quartile of median household income zip codes (24% pandemic vs. 19% pre-pandemic, p = 0.02). There were no significant differences in rates of sepsis (6.1% pandemic vs. 6.1% pre-pandemic, p > 0.9), bowel perforation (1.3% pandemic vs. 1.2% pre-pandemic, p = 0.8), ICU admissions (9.6% pandemic vs. 12% pre-pandemic, p = 0.2), mortality (0.5% pandemic vs. 0.6% pre-pandemic, p = 0.8), or length of stay (median [interquartile range]: 4 [(Pastor et al., 2009; Gosain and Brinkman, 2015) 2 , 11 2,11 days pandemic vs. 5 [(Pastor et al., 2009; Tang et al., 2020) 2 , 10 2,10 days pre-pandemic, p = 0.4). The COVID-19 pandemic was associated with significantly decreased incidence of HAEC admissions across US children's hospitals. Possible etiologies such as social distancing should be explored. II. • COVID-19 provided a natural experiment to study the effects of social distancing on Hirschsprung's-associated enterocolitis. • There were significantly fewer admissions for Hirschsprung's-associated enterocolitis without evidence of increased severity. • Communicable infections may contribute to the pathogenesis of Hirschsprung's-associated enterocolitis. [ABSTRACT FROM AUTHOR]

Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality amongst patients with Hirschsprung disease (HD); rectal Botulinum toxin (Botox) has been reported a possible prevention strategy. We aimed to evaluate our institution's historic cohort of HD patients, first to determine our incidence of HAEC and second to begin assessing the effect of Botox on HAEC incidence. Patients with HD seen at our institution between 2005 and 2019 were reviewed. Incidence of HD and frequencies of HAEC and Botox injections were tallied. Associations between initial Botox treatment or transition zone and HAEC incidence were evaluated. We reviewed 221 patients; 200 were included for analysis. One hundred thirteen (56.5%) patients underwent primary pull-through at a median age of 24 days (IQR 91). Eighty-seven (43.5%) patients with initial ostomy had their intestinal continuity reestablished at a median of 318 days (IQR 595). Ninety-four (49.5%) experienced at least one episode of HAEC and 62 (66%) experienced multiple episodes of HAEC. Nineteen (9.6%) patients had total colonic HD and had an increased total incidence of HAEC compared to patients without total colonic HD (89% vs 44%, p < 0.001). Six (2.9%) patients received Botox injections at the time of pull-through or ostomy takedown; one experienced an episode of HAEC (versus 50.7% of the patients who were confirmed to have not received Botox injections at their surgery, p = 0.102). Further prospective study on Botox's effect on Hirschsprung-associated enterocolitis is required and is the next step in our investigation. Level III. [ABSTRACT FROM AUTHOR]

This study sought to characterize sexual function and fecal incontinence related quality of life (QOL) outcomes for adult males with anorectal malformation (ARM) or Hirschsprung's Disease (HD). We conducted a cross-sectional survey study of male patients ≥18 years with ARM or HD. Patients were identified from our institutional database, contacted and consented by telephone, and sent a REDCap survey via email. The International Index of Erectile Function (IIEF-5) and Male Sexual Health Questionnaire (MSHQ) evaluated erectile dysfunction (ED) and ejaculatory dysfunction (EjD), respectively. The Cleveland Clinic Incontinence Score (CCIS) and the Fecal Incontinence Quality of Life Scale (FIQLS) assessed fecal incontinence-related outcomes. A linear regression analysis of IIEF-5 scores compared to CCIS scores was used to evaluate for an association between ED and incontinence. Of 63 patients contacted, 48 completed the survey. The median age for respondents was 22.5 years (IQR 20–25). There were 19 patients with HD and 29 patients with ARM. On the IIEF-5 survey, 35.3% report some level of ED. On the MSHQ-EjD survey, the median score was 14 out of 15 (IQR 10.75–15), indicating few EjD concerns. The median CCIS was 5 (IQR 2.25–7.75) and the median FIQL scores ranged from 2.7 to 3.5 depending on the domain assessed, demonstrating some QOL challenges secondary to fecal incontinence. On linear regression analysis, IIEF-5 and CCIS scores were weakly associated (B = −0.55, p = 0.045). Male adult patients with ARM or HD may have ongoing concerns with sexual function and fecal incontinence. Level 4. Cross-Sectional Survey Study. [ABSTRACT FROM AUTHOR]

• What is known? • Hirschsprung-associated enterocolitis is a common cause of morbidity and mortality. • Social determinants of health can affect health outcomes. • What is new? • There are several social determinants that are associated with a higher risk of enterocolitis, such as child protective services involvement, maternal drug use, and missed clinic appointments. Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in patients with Hirschsprung disease (HD). The objective of this study was to examine the association of social determinants of health (SDOH) with HAEC. A review of patients who underwent primary pull through for HD at our institution from 2014 to 2021 was performed. Clinical, surgical, and SDOH data were collected. HAEC was defined by an international scoring system. Categorical variables were analyzed via Fisher's exact tests and continuous variables with Mood's median tests. One hundred patients were identified with 29 patients (29%) having at least one episode of HAEC during a median follow-up of 31 months (IQR: 11.7–55.7). Children who utilized public transportation for clinic visits, had one or more missed appointments, had any reported safety concerns, were involved with Child Protective Services, had parents/guardians who were not married, lived with people other than their immediate family, or had mothers who reported drug use or lack of prenatal care were found to have a higher likelihood of developing HAEC (p <0.04 for all). Age at HD diagnosis, age at pull through, operative approach, length of aganglionic colon, and Trisomy 21 were not significant predictors of HAEC. In our series of 100 patients undergoing primary pull through, there was a significant correlation of HAEC with several social determinants of health elements while anatomical and clinical factors were not associated with HAEC. Attention to social determinants of health and identifying high-risk patients may serve to prevent morbidity and mortality from HAEC. III [ABSTRACT FROM AUTHOR]

Intraoperative resection level in patients with Hirschsprung disease (HD) is determined by contrast enema, surgeon's intraoperative judgement and full thickness biopsy (FTB) identifying ganglia. This study aims to evaluate diagnostic accuracy of contrast enema and FTB in determination of resection level and whether this can be improved by measuring submucosal nerve fiber diameter. We retrospectively analyzed contrast enema and intraoperative FTBs obtained in our center, determining diagnostic accuracy for level of resection. Gold standard was pathological examination of resection specimen. Secondly, we matched transition zone pull-through (TZPT) patients with non-TZPT patients, based on age and length of resected bowel, to blindly compare nerve fibers diameters between two groups using group comparison. From 2000–2021, 209 patients underwent HD surgery of whom 180 patients (138 males; median age at surgery: 13 weeks) with 18 TZPTs (10%) were included. Positive predictive value of contrast enema was 65.1%. No caliber change was found in patients with total colon aganglionosis (TCA). Negative predictive value of surgeon's intraoperative judgement and FTB in determining resection level was 79.0% and 90.0% (91.2% single-stage, 84.4% two-stage surgery) respectively. Mean nerve fiber diameter in TZPT was 25.01 µm (SD= 5.63) and in non-TZPT 24.35 µm (SD= 6.75) (p = 0.813). Determination of resection level with combination of contrast enema, surgeon's intraoperative judgement and FTB results in sufficient diagnostic accuracy in patients with HD. If no caliber change is seen with contrast enema, TCA should be considered. Resection level or transition zone cannot be determined by assessment of submucosal nerve fiber diameter in FTB. clinical research paper. [ABSTRACT FROM AUTHOR]

Debate exists on whether patients with Hirschsprung Disease (HD) should undergo immediate resection during their newborn hospitalization or undergo a staged procedure. This study sought to compare postoperative outcomes among newborns receiving immediate versus staged surgery for rectosigmoid HD. The Nationwide Readmission Database was queried (2016–2018) for newborns with HD who underwent surgical resection during their newborn hospitalization (immediate) versus planned readmission (staged). Those who did not receive rectal biopsy or had long-segment or total colonic HD were excluded. A propensity score-matched analysis (PSMA) of patients receiving either surgery was constructed utilizing >70 comorbidities. Outcomes were analyzed using standard statistical tests. 1,048 newborns with HD were identified (56% immediate vs. 44% staged). Staged resection was associated with higher total hospitalization cost ($56,642 vs. $50,166 immediate), p = 0.014. After PSMA, the staged cohort was more likely to require home healthcare at discharge and experience unplanned readmission (40% vs. 23%). These patients experienced more gastrointestinal complications (40% vs. 22%) on readmission, especially Hirschsprung-associated enterocolitis (35% vs. 20%). Newborns receiving staged procedures for HD experience higher rates of unplanned readmission complications and incur higher hospitalization costs. This information should be utilized to defray healthcare utilization costs for newborns with HD. Retrospective Comparative. III. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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 4 • What is currently known about this topic? HD-IBD is rare with approximately 2% incidence based on previous studies. • What new information is contained in this article? - Risk factors for HD-IBD may include long segment disease, trisomy 21, and previous episodes of HAEC. - HD-IBD can be found in patients younger than 5 years old but often occurs many years after pullthrough surgery. - Biological agents are the most effective pharmacological treatment for HD-IBD. - Almost 30% of HD-IBD required surgical treatment for IBD. [ABSTRACT FROM AUTHOR]

Hirschsprung Disease (HD) is a common congenital intestinal disorder. While aganglionosis most commonly affects the rectosigmoid colon (rectosigmoid HD), outcomes for patients in which aganglionosis extends to more proximal segments (long-segment HD) remain understudied. This study sought to compare postoperative outcomes among newborns with rectosigmoid and long-segment HD. The Nationwide Readmission Database was queried from 2016 to 2018 for newborns with HD. Newborns were stratified into those with rectosigmoid or long-segment HD. Those who received no rectal biopsy or pull-through procedure during their newborn hospitalization were excluded. A propensity score-matched analysis (PSMA) of newborns with either type of HD was constructed utilizing 17 covariates including demographics, comorbidities, and congenital-perinatal conditions. There were 1280 newborns identified with HD (82% rectosigmoid HD, 18% long-segment HD). Patients with rectosigmoid HD had higher rates of laparoscopic resections (35% vs. 12%) and less frequently received a concomitant ostomy (14% vs. 84%), both p < 0.001. Patients with long-segment HD were more likely to have a delayed diagnosis (12% vs. 5%) and require multiple bowel operations (19% vs. 4%), both p < 0.001. They experienced higher rates of complications, including small bowel obstructions (10% vs. 1%), infections (45% vs. 20%), and Hirschsprung-associated enterocolitis (11% vs. 5%), all p < 0.001. After PSMA, newborns with long-segment HD were found to have a longer length of stay and higher hospitalization costs. Newborns with long-segment HD experience significant delays in diagnosis, surgery, and complications compared to those with rectosigmoid HD. This information should be utilized to improve healthcare delivery for this patient population. Retrospective comparative study. III. • Aganglionosis in Hirschsprung Disease (HD) most commonly affects the rectosigmoid colon. Outcomes for patients with long-segment HD remain understudied. • Newborns with long-segment HD (LSDH) experience delays in diagnosis and surgery compared to those with rectosigmoid HD. • Newborns with LSHD experience higher complications and utilize more healthcare resources than those with rectosigmoid HD. [ABSTRACT FROM AUTHOR]

Hirschsprung disease (HSCR) is a complex genetic disease characterized by the absence of ganglion cells in the intestines, leading to a functional obstruction in infants. At least 24 genes have been identified for the pathogenesis of HSCR. They contributed to approximately 72% of HSCR cases. We aimed to elucidate further the genetic basis of HSCR in Indonesia using the whole-exome sequencing (WES) approach. WES was performed in 39 sporadic non-syndromic HSCR patients and 16 non-HSCR subjects as controls. Variants presented in controls were excluded, followed by in silico prediction tools and population allele frequency databases to select rare variants. We determined the minor allele frequency (MAF) using gnomAD (MAF <0.1%). We involved 24 (61.5%) males and 15 (38.5%) females. Most patients (62%) had short-segment aganglionosis and underwent the Duhamel procedure (41%). We identified several candidate novel variants in HSCR-related genes, including UBR4, GDNF, and ECE1. Moreover, we also identified some novel candidate genes, including a possible compound heterozygous variant in the MUTYH gene: the first variant, a known protein-truncating variant associated with colorectal cancer (CRC), p.Glu452Ter and the second variant is novel, p.Ala39Val. Moreover, the type of variants was not associated with the aganglionosis type. We identified several novel genes and variants, including the variant associated with CRC, that might contribute to the pathogenesis of HSCR. No genotype–phenotype associations were noted. Our study further confirms the complex network involved in enteric nervous system development and HSCR pathogenesis. Level III. [ABSTRACT FROM AUTHOR]

Concurrent esophageal atresia with tracheoesophageal fistula and Hirschsprung disease is a rare combination of congenital anomalies, with only four cases reported to date in full-term neonates. A 4-day-old full-term neonate from rural Ethiopia was brought to our pediatric surgical unit with symptoms of respiratory distress and excessive frothy secretions. Chest exam revealed intercostal and subcostal retractions along with crepitation in the right posterior chest. The abdomen was distended, and a positive blast sign was noted during the rectal exam. Echocardiography showed a small patent ductus arteriosus. We attempted to pass an orogastric tube, but it was not possible. Chest and abdominal X-rays showed air in the stomach and the orogastric tube in the upper esophagus, suspicious for an esophageal atresia with a tracheoesophageal fistula. The patient had persistent thrombocytopenia, requiring multiple platelet transfusions, and was treated for aspiration pneumonia before he was deemed stable to undergo an operation. He received daily maintenance fluids with electrolytes for nutritional support and underwent daily rectal irrigations. On day of life 17 he was taken to the operating room. We found and ligated a distal tracheoesophageal fistula and did an end-to-end esophageal anastomosis via right thoracotomy. Because of the ongoing need for daily rectal irrigations, we also did a full thickness transanal rectal biopsy and confirmed the diagnosis of Hirschsprung disease. Postoperatively, the patient had no complications and has since started breastfeeding. He continues to receive rectal stimulation and irrigation and is on the wait list for his pull-through operation. A high index of suspicion for Hirschsprung disease is crucial in patients with esophageal atresia and tracheoesophageal fistula, delayed meconium passage and lack of spontaneous bowel function. [ABSTRACT FROM AUTHOR]

Hirschsprung associated enterocolitis (HAEC) is a challenging problem in a subset of children with Hirschsprung disease (HD). In refractory cases, fecal diversion may be required. The aim of this study was to characterize patients who require fecal diversion for HAEC management and examine their long-term outcomes. A retrospective review of prospectively collected data within the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry was performed. All children with a history of a corrective procedure for HD and documented post-operative HAEC were included. Our primary outcome was diversion to manage HAEC after pull-through and secondary outcomes included incidence of recurrent HAEC, fecal continence following ostomy closure, and bowel management needs at last follow-up. 951 patients were identified with a history of HD and 852 had undergone pull-through. 339/852 (39.8 %) had documented HAEC and 75/339 (22.1 %) required an ostomy, 54 (72 %) of which underwent reversal at a median of 239.5 days. After reversal, 10/54 (18.5 %) required repeat diversion for recurrent HAEC. 30/75 (40 %) had their pull-through revised. Median age at last follow-up was 5.3 [3.4, 9] years. At time of last follow-up, 40.3 % were toilet-trained for stool and 42.9 % required long term enema-based therapy. Fecal diversion for refractory HAEC was common in our study. Slightly more than half of the patients underwent successful closure of their stoma during over 4.5 years of follow-up, while less than half were toilet-trained for stool at their last visit. These children may represent a unique cohort that warrants further investigation. Level VI. • Fecal diversion is an important clinical tool for the management of refractory Hirschsprung Associated Enterocolitis. • Patients who require diversion have a high burden of long-term bowel management needs. [ABSTRACT FROM AUTHOR]

It is challenging to distinguish between patients with Hirschsprung disease (HD) and patients with other causes of defecation problems based on clinical presentation in patients older than six months. Pathological examination of the rectal biopsy is the gold standard for the diagnosis of HD. The aim of this study was to gain insight into 1) the prevalence and severity of complications following rectal biopsy, 2) the final diagnoses of patients referred for biopsy, and 3) clinical factors associated with HD in patients older than six months. Children suspected of HD above the age of six months referred for biopsies were analyzed retrospectively. Severity of complications of rectal suction biopsy (RSB) and full thickness biopsy (FTB) were assessed using Clavien-Madadi (CM) grading. Factors associated with HD were tested using multivariate logistic regression analysis. From 2000 to 2022, 234 children older than six months of age underwent biopsies because of defecation problems (median age of 47.2 months (IQR = 17.2–87.2)). Of these, 130 out of 234 children underwent RSB and 112 out of 234 children underwent FTB. One patient (0.4 %) developed a complication following RSB (CM1-A): fever without evident cause. Two patients (0.9 %) had ongoing rectal bleeding following FTB. One patient did not require an intervention (CM1-A), the other received rectal spongostan to stop the bleeding and erythrocyte transfusion (CM2). The most frequent final diagnoses were functional constipation (n = 179, 76.5 %), HD (n = 25, 10.6 %) and food intolerance (n = 5, 2.1 %). Associated factors for HD were distended abdomen (OR 5.41, CI 2.05–14.31), vomiting (OR 4.14, CI 1.64–11.85) and no abdominal pain (OR 0.14, CI 0.03–0.65) (model R2 = 0.278). In children older than six months suspected of HD, presenting with distended abdomen, vomiting and no abdominal pain, we advise obtaining a rectal biopsy in case other causes of constipation are ruled out, because a rectal biopsy is a safe procedure with only minor complications. The most common diagnosis in patients older than six months of age referred for rectal biopsy was functional constipation. Level III. [ABSTRACT FROM AUTHOR]

Enterostomy takedown is common in neonates with Hirschsprung's disease, anorectal malformations, or necrotizing enterocolitis. Stapled bowel anastomosis has become routine in adults, but size of up to 12 mm diameter precludes performing enterostomy takedown in young infants using regular intestinal staplers. After the introduction of miniature (5 mm diameter) staplers, we increasingly used them for enterostomy takedown. This study compares enterostomy takedown using the miniature stapler (MS) to the conventional hand-sewn (HS) technique. Retrospective review of all children <3 years of age undergoing enterostomy closure at our institution from 2008 to 2023 were retrospectively reviewed. Demographics, operative times, complications, and outcomes were compared between those who underwent the procedure using MS versus HS technique. Data are quoted as median (range). A total of 102 patients were enrolled, including MS (n = 26) and HS (n = 76) anastomoses. There were no statistical differences in age, sex, or indication for enterostomy. Enterostomy takedown using MS was faster [82.5 (44–218) versus 147 (52–381) minutes, p < 0.001) and associated with earlier commencement of feedings [2 (1–6) versus 4 (1–24) days, p = 0.001], as well as shorter length-of-stay [6 (2–20) versus 17 (3–52) days, p < 0.001), compared to the HS technique. This is the first study that systematically evaluates the novel 5 mm ministapler for enterostomy takedown in young children. Its use was associated with quicker operative times, earlier feeding and shorter hospital stay. These findings are especially relevant in children with co-morbidities who do not tolerate longer anesthesia times. Randomized, controlled trials should be performed to prospectively confirm these findings. Level III, retrospective comparative study. [ABSTRACT FROM AUTHOR]

Hirschsprung's (HSCR) disease, also known as aganglionic megacolon, or congenital intestinal aganglionosis affects roughly 1 out of every 5000 newborns. It is a birth defect characterized by the partial or complete loss of ganglion cells in the myenteric and submucosal plexus of the distal intestine which leads to ineffective peristalsis, constipation, and obstruction. Clinical assessment and radiological observations might imply HSCR disease, but definitive diagnosis requires biopsy interpretation and confirmation of ganglion cell loss. The difficulty in identifying immature ganglion cells added to the variability in interpreting immunohistochemical markers of ganglion cells warrants the search for new markers. Our recent research identified Beta-1,3-N-acetylglucosaminyltransferase (B3GNT6) as a potential candidate, as it consistently stains the cytoplasm of ganglion cells. To evaluate its utility, we conducted a preliminary assessment of B3GNT6 expression in nineteen gastrointestinal tissue samples and observed cytoplasmic staining in ganglion cells across all samples. This consistent staining pattern suggests B3GNT6 could serve as a reliable marker for diagnosing Hirschsprung's disease. This article serves as a preliminary evaluation of B3GNT6 as a ganglion cell immunohistochemical marker, highlighting its potential significance while acknowledging the need for further validation in larger, more diverse cohorts. [ABSTRACT FROM AUTHOR]

Immunohistochemistry (IHC) markers are employed to improve the diagnostic yield when testing for Hirschsprung disease (HSCR). Yet, a superior test has not been identified. We aimed to determine the diagnostic test accuracy (DTA) of IHC markers. We conducted database search for studies reporting IHC staining on rectal biopsy investigating for HSCR. We constructed 2 × 2 contingency tables, and calculated DTA estimates in pooled and paired testing using random-effect model meta-analysis. Twenty eight IHC markers from 107 studies were used to investigate for HSCR in 10891 children. In pooled analysis; calretinin sensitivity and specificity were superior to acetylcholinesterase, S100, and peripherin [98 % (CI; 0.95–0.99) & 99 % (CI; 0.97–0.99)], [94 % (CI; 0.86–0.97) & 99 % (CI; 0.96–0.99)], [92 % (CI; 0.85–0.96) & 97 % (CI; 0.89–0.99)] and [91.7 % (CI; 0.54–0.98) & 94.8 % (CI; 0.59–0.99)], respectively. In paired analysis calretinin diagnostic odds ratio was superior to hematoxylin and eosin (H&E), acetylcholinesterase and S100: [3349 (PI; 551.3–22667.2) vs 345.3 (PI; 54.9–2394.2)], [300.9 (PI; 13.3–4146.9) vs 34.6 (PI; 2.2–363.9)] and [696.9 (PI; 91.2–3401.7) vs 196.9 (PI; 29.8–890.5)], respectively. In biopsies labelled inadequate for H&E testing, calretinin specificity to rule out HSCR reached 92 % (CI; 0.288–0.998). IHC provides additional diagnostic value over H&E. Calretinin appears to be, currently, a superior IHC marker. The available literature is of variable quality, cautious interpretation of the findings should be considered. III. • What is currently known about this topic? Many immunohistochemistry markers have been used to improve the diagnostic accuracy when testing for Hirschsprung disease on rectal biopsy. • What new information is contained in this article? We report the staining characteristic and diagnostic accuracy of 28 distinct Immunohistochemistry markers used in the diagnosis of Hirschsprung disease. Calretinin demonstrate the best diagnostic test accuracy parameters, and seems superior to rule out Hirschsprung disease in inadequate rectal suction biopsy. [ABSTRACT FROM AUTHOR]

Colonic atresia (CA) is associated with Hirschsprung disease (HD) in up to 10% of cases. Therefore, some surgeons elect to complete proximal diversion at the initial operation. We sought to better define the incidence of concurrent HD and evaluate practice patterns regarding diversion for CA. The Pediatric Health Information System (PHIS) database was used to identify patients with CA from 2013 to 2022. Patients with small bowel atresia, anorectal malformation, gastroschisis, or first operation after 14 days of age were excluded. Index and subsequent operations were defined. Complications, time to enterostomy closure, and unplanned operations were evaluated. HD was diagnosed in 8 (9.5%) patients and 7 of these were initially diverted. Diverted and anastomosed patients were demographically similar. In the 58 (69%) patients initially diverted, 19 (33%) had an ileostomy. Continuity was restored with an ileo-colic anastomosis in 63% of diverted and 27% of primarily anastomosed patients. Of those initially managed with a colostomy, 53% ultimately had a colo-colonic anastomosis. Patients with primary anastomoses had fewer operations and received more days of parenteral nutrition. Other outcomes did not vary. In a large population of infants with CA, 9.5% had concurrent HD. Almost 70% of CA patients underwent initial diversion and only one with HD had a primary anastomosis. Patients managed with a primary anastomosis were substantially more likely to retain the proximal colonic segment, but had a similar incidence of complications. When intraoperative colonic biopsies are obtained, primary anastomosis is a safe and effective strategy for CA. 3. [ABSTRACT FROM AUTHOR]

To investigate whether Leukotriene B4 receptor 2 (BLT-2), an upstream regulator of tight junction protein (TJP) Claudin-4, and TJPs could be etiologic factors in Hirschsprung-associated enterocolitis (HAEC) after pull-through (PT) for Hirschsprung disease (HD). Normoganglionic colon (HD-N) and aganglionic rectum (HD-A) specimens from rectal/rectosigmoid (R/RS) or descending/transverse (D/T) HD were assessed using quantitative polymerase chain reaction (qPCR) for Occludin, TJP-1, TJP-2, Junctional adhesion molecule (JAM)-1, JAM-2, Claudin-1, Claudin-3, Claudin-4, and BLT-2 and immunoblotting for Claudin-4 using fresh specimens obtained intraoperatively (2021–2024; n = 17; R/RS = 15 and D/T = 2). Claudin-4 immunohistochemistry was also evaluated quantitatively using preserved (n = 29; R/RS = 20 and D/T = 9; 2009–2021) and fresh HD specimens for comparison with anorectal malformation patients having colostomy closure as controls (n = 42) and between HD-A versus HD-N, R/RS versus D/T, and HAEC (+) versus HAEC (−). Technically inadequate or transitional zone PT were excluded. Subjects were 123 PT cases. Mean ages at PT/colostomy closure (years) were R/RS: 2.7 ± 2.9, D/T: 1.6 ± 2.2, and controls: 1.4 ± 0.7. Postoperative HAEC occurred 18 times in 14 PT cases (grade I = 5, grade II = 13). Post-PT HAEC was significantly more frequent in D/T (50.0% versus 6.4%; p < 0.001); Claudin-4 was significantly lower in HD-N from post-PT HAEC cases, especially D/T (p < 0.05) on immunohistochemistry. Claudin-4 was significantly lower in HD-N/HD-A compared with controls on immunoblotting (p < 0.05) and immunohistochemistry (p < 0.001). qPCR showed TJP-1, TJP-2, JAM-1, JAM-2, Claudin-4, and BLT-2 were significantly lower in HD-N/HD-A compared with controls. Lower Claudin-4 and BLT2 in post-PT HAEC HD-N (especially D/T) suggests generalized epithelial barrier derangement with possible etiologic implications for HAEC. Ⅱ [ABSTRACT FROM AUTHOR]

• Soiling after Hirschsprung disease pull-through may be related to sphincter damage which currently has no optimal treatment. • A new technique of anal sphincter reconstruction showed improvement of bowel control in these patients at one year. • Non-sedate 3D-anorectal manometry showed improvement of squeeze function and ability to complete close the anal sphincters. • In patients without Down syndrome, improvement in Baylor Continence Scale was demonstrated (4.5 vs. 0.75). In patients with Hirschsprung disease (HSCR), soiling may be related to anal sphincter damage following the initial pull-through. No optimal treatment has been developed for such patients, although enemas (rectal or antegrade) have been applied with some success. We present the one-year outcomes of a new technique for anal sphincter reconstruction. All patients with HSCR referred from other institutions for post pull-through soiling were studied. Seven patients with patulous sphincters underwent sphincter reconstruction. Six had a full preoperative evaluation and were included in the study. Their 12-month outcomes were assessed. All six patients had soiling without voluntary bowel movements (VBMs). One patient was clean on Malone flushes when referred. Three underwent pre- and post-reconstruction non-sedated three-dimensional anorectal manometry, and objectively were able to close their sphincters following the reconstruction. All patients without Down syndrome (4 of 6) showed improvement in the abbreviated Baylor Continence Scale (4.5 vs. 0.75). One patient has achieved total bowel control without antegrade flushes, three now have VBMs which they did not have before but have occasional accidents and use antegrade flushes intermittently. They reported higher productivity, the ability to participate in sports and be away from home with confidence in their regimen. Two of 6 patients have Down syndrome and required a redo pull-through for other indications and underwent empiric sphincter reconstruction. For these two patients we do not have an outcomes assessment. A new technique for sphincter reconstruction shows promising results in improvement of bowel control at one year. IV [ABSTRACT FROM AUTHOR]

• Knowledge about sexual function and the presence of lower urinary tract symptoms in Hirschsprung patients is very limited. • Such knowledge is very scarce after specifically minimally invasive endorectal pull-through. • Few studies have explored Hirschsprung males' ability to ejaculate, and none has reported adolescents' ability to ejaculate. • Minimally invasive endorectal pull-through seems to preserve erection and ejaculation in adolescent males. • Lower urinary tract symptoms are rare after minimally invasive endorectal pull-through in adolescent males. Effects of colorectal resection on sexual function and the lower urinary tract are inadequately studied in patients with Hirschsprung disease (HD). This study aimed to increase existing knowledge on sexual function and lower urinary tract symptoms (LUTS) in HD male adolescents operated with minimally invasive endorectal pull-through (ERPT). Non-syndromic male adolescents (12–18 years) operated with ERPT were invited to participate in this single-center cross-sectional study which included a semi-structured interview on sexual function and LUTS, a questionnaire recording LUTS, and a urodynamic study. Uroflowmetry curves were eligible for evaluation if the voided volume was >50% of expected bladder capacity. Ethical approval and informed consent were obtained. Of 37 eligible male adolescents, 35 (95%) with a median age of 14.9 (12.0 -18.3) years were included. 94% had rectosigmoid aganglionosis, and 97% underwent a minimally invasive ERPT. 34 (97%) visited the outpatient clinic. The ability to get erections and to ejaculate was assessed in 25/35 (71%) adolescents. 25/25 (100%) could get an erection, and of adolescents ≥15 years 14/15 (93%) could ejaculate. 32 (91%) returned the questionnaire and underwent urodynamic studies. 15/32 (47%) reported LUTS, but most had only sporadic LUTS. 31/32 (97%) were highly satisfied with their bladder function. Sporadic urinary incontinence was reported by 4/32 (13%), but none reported social problems due to this. Of the eligible 28/32 (88%) uroflowmetry curves, 15/28 (54%) were normal. Minimally invasive ERPT seems to preserve sexual function and does not induce lower urinary tract symptoms in adolescent HD males. III. [ABSTRACT FROM AUTHOR]

• Some bowel dysfunction remains after pullthrough in the majority of patients with hirschsprung disease (HD). • Recto-anal inhibitory reflex (RAIR) is present in 37.5% patients after pullthrough. • Anal resting pressures (ARP) and reappearance of RAIR do not predict bowel continence or control during follow-up. Anorectal manometry may be useful to objectively evaluate anorectal function following definitive pullthrough for Hirschsprung Disease (HD) but there is little published data. Our study aims to investigate anorectal manometry findings and their association with bowel function. This was a prospective observational study. Convenience sampling method was used to recruit all HD patients who had definitive pullthrough from January 2019 to December2020 in our institution. High-resolution anorectal manometry (HRAM) was used to record anal resting pressure (ARP), length of high-pressure zone (HPZ), and presence/absence of recto-anal inhibitory reflex (RAIR). The Paediatric Incontinence/Constipation Scoring System (PICSS) was scored for all participants. PICSS is a validated questionnaire with scores mapped to an age-specific normogram to denote constipation, incontinence, and their combinations. Non-parametric and chi-square tests at significance p <0.05 were conducted to examine the relationship between PICSS categories and manometry findings. Ethical approval was obtained. There were 32 participants (30 boys). Median age at participation was 26.5 months (range: 13.8–156). Twenty-four (75%) had transanal pullthrough, 8(25%) underwent Duhamel procedure. PICSS scored 10(31.3%) as normal, 8(25%) as constipation, 10(31.3%) as incontinent, and 4(12.5%) as mixed. RAIR was present in 12 patients (37.5%). HPZ, maximum ARP, mean ARP were comparable across all PICSS groups without statistically significant differences. Presence of RAIR was not significantly associated with any PICSS groups (p = 0.13). Bowel function does not appear to be significantly associated with HRAM findings after definitive pullthrough for HD, but our study is limited by small sample size. RAIR was present in 37.5% patients after pullthrough. Level II [ABSTRACT FROM AUTHOR]

• Rectal washouts with primary pull-through (PT) is the preferred management for Hirschsprung's disease. • Colostomies are classically preferred in three-stage PT. • Nearly 50% of our cohort required a stoma before PT. • 22% required a stoma post-PT and were more likely to need a long-term stoma. To review the role of stomas in the initial and long-term management of Hirschsprung disease (HD). Patients treated for HD at our institution between January 2004 and August 2021 were identified. Data were collected regarding: demographics, indication/bowel location/type of stomas performed and outcomes, pull-through (PT) procedure, and follow-up duration. Ninety-five patients (78 male) were identified including one early unrelated death. Forty-four of 94 (47%) required a stoma before PT procedure. Of these 44, 38 (86%) had ileostomies and the remaining six (14%) colostomies; one ileostomy remains long-term. The commonest indication for initial stomas was washout failure (41%). Ninety-one patients had undergone primary PT or secondary PT with stoma closure at the time of the study. A further new stoma was required after primary PT or three-stage management in 20/91 (22%). The commonest indications were constipation/soiling (25%) and anastomotic leak (20%). Seven out of 20 (35%) were performed within 30 days of a previous procedure and all were closed; three patients required further long-term stomas. Thirteen (65%) required a stoma >30 days, nine remain long-term. Surgical revision of stomas was required in 14/56 (25%) – prolapse and retraction being the commonest indications. Overall, 56/94 (60%) patients required stomas (pre- and/or post-PT) to manage their condition and 13/94 (14%) have a long-term stoma in place. Mean follow-up was 7.8 years (0.5 - 17.6). Stomas remain an integral part of HD management both initially (47%) and long-term (14%); they carry a considerable associated morbidity. Ileostomy is preferred for initial management. Level III [ABSTRACT FROM AUTHOR]

• Largest series comparing immediate with delayed surgery. • Immediate surgery does not lead to an increase in complications. • Immediate surgery does not lead to an increase in revisions. • Immediate surgery does not lead to an increased need for subsequent stomas. Definitive surgery for Hirschsprung disease (HD) is typically deferred to allow rectal washouts. In contrast, we have performed pull through on the next available operating list following diagnostic biopsy. Our aims are to compare outcomes of surgery without planned decompression vs. surgery where the timing of pull through was deferred. A consecutive series undergoing pull-through. Timing of surgery was at surgeon's discretion and classified as either no planned decompression surgery or other. Outcomes were i) complications graded >3b Clavien-Dindo (CD) within 30d, or ii) a need for a revisional pull through or iii) a post-pull-through stoma. We excluded total colonic aganglionosis. 156 children (116 boys) underwent pull-through. 71 had surgery without planned decompression. The indications for timing in the other 85 were: planned after home washouts (n = 28), planned after stoma (n = 29), diagnostic error (n = 19), prematurity (n = 3), late presentation (n = 2) and other (n = 4). The mean age at surgery in the surgery without planned decompression group was 18 days (range 1–49 days) vs. 310 days (range 14–4084 days). 5/72 (7%) undergoing surgery without planned decompression experienced a complication of ≥CD 3b, compared to 7/85 (8%) of delayed surgery (p = 0.8). 3/71 (4%) of the surgery without planned decompression group required a stoma following pull through compared to 11/83 (13%) of the delayed group (p = 0.059). 5/71 (7%) of the surgery without planned decompression group required revisional pull through compared to 13/85 (15%) of the delayed surgery group (p = 0.1). Surgery without planned rectal decompression for HD leads to no increase in significant complications, requirement for stoma or revision. Early definitive surgery is safe, and the use of pre-operative stoma or rectal washouts is not always necessary. III [ABSTRACT FROM AUTHOR]

• What is currently known about this topic?. • Most data comparing hirschsprung's interventions is level III-V evidence. This is not robust enough to compare the merits of these interventions. • What new information is contained in this article?. • This study provides high-quality level II evidence comparing outcomes for children treated using different rectal dissection techniques. This can be used to counsel parents and improve clinical decision making. This prospective cohort study compared primary-school-aged outcomes between children with Hirschsprung disease (HD) following Soave, Duhamel or Swenson procedures. Children with histologically proven HD were identified in British/Irish paediatric surgical centers (01/10/2010-30/09/2012). Parent/clinician outcomes were collected when children were 5–8 years old and combined with management/early outcomes data. Propensity score/covariate adjusted multiple-event-Cox and multivariable logistic regression analyses were used. 277 (91%) of 305 children underwent a pull-through (53% Soave, 37% Duhamel, 9% Swenson). Based upon 259 children (94%) with complete operative data, unplanned reoperation rates (95% CI) per-person year of follow-up were 0.11 (0.08–0.13), 0.34 (0.29–0.40) and 1.06 (0.86–1.31) in the Soave/Duhamel/Swenson groups respectively. Adjusted Hazard Ratios for unplanned reoperation compared with the Soave were 1.50 (95% CI 0.66-3.44, p = 0.335) and 7.57 (95% CI 3.39-16.93, p < 0.001) for the Duhamel/Swenson respectively. Of 217 post-pull-through children with 5–8 year follow-up, 62%, 55%, and 62% in Soave/Duhamel/Swenson groups reported faecal incontinence. In comparison to Soave, Duhamel was associated with lower risk of faecal incontinence (aOR 0.34,95%CI 0.13-0.89, p = 0.028). Of 191 children without a stoma, 42%, 59% and 30% in Soave/Duhamel/Swenson groups required assistance to maintain bowel movements; compared to Soave, the Duhamel group were more likely to require assistance (aOR 2.61,95% CI 1.03–6.60, p = 0.043). Compared with Soave, Swenson was associated with increased risk of unplanned reoperation, whilst Duhamel was associated with reduced risk of faecal incontinence, but increased risk of constipation at 5–8 years of age. The risk profiles described can be used to inform consent discussions between surgeons and parents. Level II [ABSTRACT FROM AUTHOR]

• Current knowledge. Surgery at an age of three to six months is preferred for all patients with HD. Bridging time awaiting surgery is necessary, mostly performing transanal irrigation (TAI). • New information. Bowel perforations occur most frequently while awaiting surgery, which can be highly dangerous especially in patients with a total colon aganglionosis (TCA). Patients with a TCA have a significantly higher risk at developing a complication while awaiting surgery. In patients with a (suspected) TCA we advise to refrain TAI and not to postpone surgery. Patients with Hirschsprung disease (HD) mostly undergo surgery around the age of three to six months. While awaiting surgery, therapy to treat the obstruction such as transanal irrigation (TAI) or laxatives is applied. The aim of this study was to gain insight in the prevalence and severity of complications occurring while awaiting surgery and to identify patient characteristics associated with the development of these complications. This study retrospectively analyzed data of patients with HD operated in our center between 2000 and 2021. Complications emerging while awaiting surgery were graded using Clavien-Dindo (CD). Patient characteristics as predictor of a complication were tested using logistic regression analysis. Twenty-two of 132 (17%) included patients (preoperative treatment: 94% TAI; 2% laxatives; 2% other therapy) developed 45 complications while awaiting surgery, including predominantly major complications (91%). Bowel perforation occurred most frequently (n = 9, 7%) wherefrom six caused by TAI (5%), including three patients with total colon aganglionosis (TCA) (2%) counting one life-threatening and one lethal perforation. The other perforations were caused by meconium ileus (n = 2) and Hirschsprung associated enterocolitis (HAEC) (n = 1). Other frequent complications were: sepsis (5%), ileus (4%) and persistent obstruction (4%). Predictive factor for developing complication was TCA (OR 9.905, CI 2.994–32.772, p < 0.001). We found a complication rate of 17% in patients while awaiting surgery, reporting bowel perforation most frequently. We found this complication in patients with TCA being highly dangerous causing one life-threatening and one lethal perforation. Therefore, we advise in patients with (suspected) TCA to limit the time awaiting surgery. level III. [ABSTRACT FROM AUTHOR]

Malone antegrade continence enemas (MACE) provide a conduit in which the patient can achieve improved continence, be clean of stool, and gain independence in maintaining bowel function. The Mini-ACE® is a low-profile balloon button that is used to facilitate the administration of antegrade enemas. We sought to describe our practice and short-term outcomes. This work is a retrospective review of the Mini-ACE® appendicostomy button from April 2019 to March 2021, with follow-up concluding in October 2021. Patient demographics, colorectal diagnoses, and outcomes were examined. Forty-three patients underwent Mini-ACE® placement; 22 (51%) were male. The average age at Mini-ACE® insertion was 9.2 years (range 3–20 years). The most common diagnoses were functional constipation in 19 (44%), anorectal malformation in 15 (35%), and Hirschsprung disease in 3 (7%), spinal differences 3 (7%). There were no intra-operative complications, but 5 (12%) required prolapse resection. The median length of stay was two days (IQR 1, 4). Patients achieved self-catheterization at 4.5 [3,7] months from MACE creation, with 38 children (88%) reporting excellent success in remaining clean of stool. The Mini-ACE® appears to be a safe and low-profile option for antegrade continence enema access. Further research is needed directly comparing complications and patient satisfaction rates between different MACE devices and overall quality of life. Level IV. [ABSTRACT FROM AUTHOR]

Children with colorectal diseases such as anorectal malformations (ARM), Hirschsprung disease (HD), and functional constipation (FC) undergo bowel management programs (BMPs) to achieve cleanliness. While patient outcomes, such as cleanliness and quality of life, are well understood, patient experience, such as relationships, ability to participate in sports, and independence and self-confidence is less well understood. We aimed to assess the relationship between BMP and patient experience. A cross-sectional survey was administered to 295 patients ≥3 years old with ARM, HD, and FC completing BMP. The survey contains 22 questions regarding patient-reported experience measures (PREMs) and 11 regarding patient-reported outcomes measures (PROMs). Each was graded on a Likert scale, with higher scores meaning better experience. Scores were compared by demographics and clinical characteristics and logistic regression was performed controlling for clinically significant variables. A p-value of ≤0.05 was significant. There were 205 eligible respondents (69.5%) with a median age of 8.9 years [IQR: 6.1–12.4]. ARM was most common (51.2%) and most achieved cleanliness on BMP (69.3%). There were no differences in experience scores by age, diagnosis, or bowel regimen. Patients that were clean had significantly higher PREM scores (67.7 [IQR: 64.0–83.0] vs. 64.8 [IQR: 55.0–70.1], p = 0.0002) and PROM scores (36.8 [IQR: 33.0–41.0] vs. 34.0 [31.0–38.5], p = 0.005). On regression analysis, cleanliness remained a strongly significant predictor of positive experience scores (β 7.37, SE 1.86, p < 0.0001). Achieving cleanliness was associated with positive patient experience of bowel management programs. This finding suggests that achieving cleanliness, regardless of regimen, may allow patients the best functional and experiential outcomes. [ABSTRACT FROM AUTHOR]

Despite significant progress in our understanding of the etiology and pathophysiology of Hirschsprung disease (HSCR), early and accurate diagnosis and operative management can be challenging. Moreover, long-term morbidity following surgery, including fecal incontinence, constipation, and Hirschsprung-associated enterocolitis (HAEC), remains problematic. Recent advances applying state-of-the art imaging for visualization of the enteric nervous system and utilizing neuronal stem cells to replace the missing enteric neurons and glial cells offer the possibility of a promising new future for patients with HSCR. In this review, we summarize recent research advances that may one day offer novel approaches for the diagnosis and management of this disease. [ABSTRACT FROM AUTHOR]

• Health related quality of life (HRQOL) in children with Hirschsprung disease (HD) are equivocal; and data on parent-child agreement remained limited. • Parent-child agreement on HRQOL for children with HD was poor to moderate, highlighting the importance of the multi informant approach for a comprehensive evaluation. Health-related quality of life (HRQOL) is an important outcome among children with Hirschsprung Disease (HD), but there are challenges in interpreting findings in previous studies owing to the choice of a comparator group and informant. We compared parent-proxy versus child self-report HRQOL in children with HD to children with functional constipation (FC) and examined predictors of HRQOL. Data of 126 children (5–18 years, 60.3% male, HD: n = 52, FC: n = 74) were acquired from the Pediatric Colorectal and Pelvic Learning Consortium. Demographics, clinical variables, HRQOL (Pediatric Quality of Life Inventory parent-proxy; child self-report) and functional outcomes (Baylor Continence Scale, Cleveland Clinic Constipation Scoring System) were collected. Parent and child HRQOL was similar for both cohorts, with higher scores on physical functioning and lower scores on emotional and school functioning. For children with HD, demographics and clinical variables did not predict HRQOL in multivariable regression models. For children with FC, greater severity of constipation predicted lower HRQOL (parent-proxy: B = -2.14, p < 0.001; child: B = -1.75, p = 0.001). Parent-child agreement on HRQOL scores was poor to moderate in the HD group (intraclass correlations (ICC)=0.38–0.74), but moderate to excellent in the FC group (ICC=0.63–0.84). Furthermore, parents of children with FC and ≤10 years overestimated children's HRQOL (proportional OR 4.59 (1.63, 13.85); p = 0.004). Clinical symptoms and demographic factors did not predict HRQOL among children with HD, highlighting the need to examine other biopsychosocial factors to understand long term HRQOL. Low parent-child HRQOL agreement in children with HD demonstrates the importance of obtaining parent and child perspectives. III. Prognosis study. [ABSTRACT FROM AUTHOR]

Patients with Hirschsprung disease (HD) are at risk of Hirschsprung associated enterocolitis (HAEC) following pull-through. The purpose of this study was to determine if routine Botulinum toxin (BT) injected one-month post pull-through decreases the incidence of HAEC. We reviewed patients who underwent a primary (not redo) pull-through operation for HD between April 2014 to December 2019. Over the most recent 18 months, BT was administered routinely one-month post-pull-through procedure; these patients were compared to the prior group that did not receive routine BT. A HAEC episode was defined as one that required initiation of treatment for obstructive symptoms in the inpatient or outpatient setting with antibiotics and irrigations. Categorical variables were compared using the nonparametric chi-square test or Fisher's exact test. Continuous variables were compared using the two-tailed Student's t -test. P-value <0.05 was determined to be statistically significant. A total of 70 patients underwent Swenson pull-through during the study period (52% male). There were no statistically significant differences in demographics in the BT vs. non-BT group. Routine post-pull-through BT was given in 28 patients and did not significantly change HAEC incidence compared to the non-BT group (12/28, 43% vs. 16/42, 38%. P = 0.691). Of note, the BT group patients developed HAEC significantly sooner than the patients in the non-BT group (37.5 days vs. 253 days, p = 0.029). More patients in the BT group (n = 18, 64%) required at least one subsequent BT injection compared to the patients in the non-BT group (n = 11, 26%. P = 0.001). We conclude that routine postoperative botulinum toxin injection given one month postoperatively from Swenson pull-through did not change the incidence of HAEC. A prospective controlled study is necessary to confirm these findings. [ABSTRACT FROM AUTHOR]

Transanal endorectal pull-through (TERPT) is a common surgical procedure in Hirschsprung disease (HD). Aim of this study was to gain insight in the prevalence and severity of postoperative complications within 30-days after TERPT and to identify patient and perioperative characteristics, associated with the development of short-term postoperative complications. This study retrospectively analyzed data of children with HD and treated with TERPT in our center between 2005 and 2020. Complications emerging within 30-days after surgery were assessed using Clavien-Dindo (CD). Patient and perioperative characteristic as predictor of a complication were tested using (multivariable) logistic regression analysis. Twenty-two of 106 (21%) included patients (17 transanal only; 77 laparoscopic-assisted; 12 laparotomy-assisted) developed 35 complications, including two patients (1.8%) that deceased. We suspect postoperative rectal irrigation leading to perforation as cause of death in both patients. Six patients (6%) had a minor (CD<3) and 16 patients (15%) a major (CD≥3) complication. Anastomotic leakage (n = 4, 11%), abdominal abscess (n = 3, 9%) and anastomotic stricture (n = 3,9%) occurred most frequently. Predictive factors for developing a complication were older age at time of surgery (OR 1.03 1.00–1.01, p = 0.041), laparotomy-assisted surgery (OR 12.65, CI 1.712–93.07, p = 0.013) and long-segment HD (OR 4.09 CI 1.09–15.39, p = 0.037). We found a CD-graded short-term postoperative complication rate of 21% following TERPT, reporting anastomotic complications most frequently. In patients at risk a diverting stoma should be considered. We suspect postoperative rectal irrigation being the cause of two lethal perforations. Therefore, we recommend to place a rectal transanastomotic tube in all patients receiving TERPT. Level III. [ABSTRACT FROM AUTHOR]

• The presence of a normal number of immature ganglion cells indicates immaturity of ganglia. • A palisading arrangement of ganglion cells is a new pathological feature of immaturity of ganglia, which disappears once ganglion cells mature. Immaturity of ganglia (IG), an allied disorder of Hirschsprung disease (AD-HSCR), develops as neonatal ileus, but the dysmotility spontaneously resolves after several months. The diagnosis of IG using HE staining is often difficult. We herein report a new pathological finding of IG called the 'palisading-like pattern', which may be helpful for improving the diagnostic accuracy. Cases of IG that were managed over the past 28 years were retrospectively reviewed. We investigated the clinical course and pathological findings for Hematoxylin-Eosin (HE) staining. The conventional diagnostic criteria for IG were (1) a normal or slightly increased number of ganglion cells and (2) ganglion cells with small nuclei. Among the 155 cases, 28 were diagnosed with IG, and 10 were retrospectively confirmed by HE staining. A palisading-like pattern was confirmed at the time of the initial ileostomy (median age, 2.5 days), and the palisading-like pattern had completely disappeared by the time of stoma closure (median age, 215 days) in all 10 cases. A palisading-like pattern is not present in other diseases. Even if immunostaining data are not available for a further analysis, the detection of a palisading-like pattern on HE staining makes an accurate diagnosis possible. LEVEL IV. [ABSTRACT FROM AUTHOR]

Botulinum toxin (BT) is used to treat pediatric patients with Hirschsprung disease (HD) with obstructive symptoms. We aimed to characterize use of BT in HD patients across pediatric colorectal surgery referral centers. A multicenter retrospective study of BT use in children (0–18y) with HD was performed using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) between 2017 and 2021. Sites with <10 HD patients recorded were excluded. Patterns were evaluated using Fisher's exact, Wilcoxon rank-sum, Kruskal-Wallis, and Cochran-Armitage trend test. 494 patients at 8 centers were included. 118 (23.9%) received at least one BT injection. Among patients who required redo pullthrough procedures, 53.1% received BT compared to 22.7% of patients who only underwent one pullthrough (p <0.001). Age at pullthrough was also significantly associated (p = 0.021). A lower proportion of Hispanic patients received BT (9.6% vs. 26.3%; p = 0.006). Percentage of HD patients receiving BT varied significantly across sites (p <0.001). Use of BT in patients with HD varies widely with greater use in patients who underwent redo surgery and in those who underwent pullthrough at an older age. Hispanic patients received less BT. These findings highlight the need to develop consensus guidelines and for further study on timing of injections and potential disparities in care. III [ABSTRACT FROM AUTHOR]

• National cohort of pediatric patients with Hirschsprung disease used to compare those with and without Down syndrome. • Patients with Down syndrome have delays in diagnosis and poorer outcomes than those with Hirschsprung disease alone. • Earlier suspicion of Hirschsprung disease in patients with Down syndrome may improve outcomes. Down syndrome (DS) is the most common abnormality associated with Hirschsprung disease (HD). It has been suggested patients with HD and DS have worse outcomes, however the literature is controversial. The Kids' Inpatient Database (KID) from 2003 to 2012 was used to identify newborns with HD. Demographics, hospital characteristics, and outcomes were compared among patients with and without DS using standard statistical tests. There were 481 patients identified with HD, of which 45 (9%) had DS. Patients with DS were older at the time of first rectal biopsy (6 [3–11] days vs. 4 [3–6] days, p = 0.012). There were no differences in operative versus non-operative management in patients with and without DS (p = 0.706). Hospital length of stay was longer in the DS cohort (22 [13–33] days vs. 15 [10–24] days, p = 0.019), and patients with DS were more likely to have a concomitant diagnosis of wound infection (<12% vs. 3%, p = 0.002) and necrotizing enterocolitis (<14% vs. 5%, p = 0.018). The mortality rate for patients with DS was four times higher than those without DS (< 5% vs. < 0.8%, p = 0.018). In this nationwide cohort of patients with Hirschsprung disease, those with Down syndrome experienced delays in diagnosis and worse outcomes. Level III. Treatment study, retrospective comparative study. [ABSTRACT FROM AUTHOR]

Hirschsprung's-associated enterocolitis (HAEC) is a common post-operative problem for patients with Hirschsprung disease (HSCR). However, treatment strategies remain variable among providers, institutions, and even nations. The purpose of this study was to identify differences in treatment patterns for HAEC. A questionnaire was distributed to members of the International Pediatric Endoscopic Group (IPEG) community that focused on HSCR and HAEC management strategies. Questionnaire responses were collected via the Research Electronic Data Capture (RedCap). 178 responses were obtained: 30% from North America, 20% South America, 20% Europe, 26% Asia, and 4% from Australia. 37% had a dedicated pediatric colorectal center. After diagnosis, 53% send patients home with irrigations, while 29% perform a primary PT before discharge; the type of PT varied between Soave (50%), Swenson (25%) and Duhamel (13%). Only 29 respondents (17%) stated their institution had guidelines for HAEC management; however, inpatient treatments were fairly consistent: 95% performed rectal irrigations, 93% obtained an abdominal radiograph, and 72% held feeds; 55% taught families irrigations before discharge. Utilization of Botulinum (BT) injections was mixed: 36% never utilized BT injections, 33% only used BT if irrigations were not tolerated, and 16% only injected BT for recurrent episodes. Preventative HAEC measures were also varied and included anal dilations (44%), prophylactic antibiotics (34%), probiotics (29%), and routine home irrigations (22%). There is wide variation of care in managing enterocolitis episodes in patients with Hirschsprung disease. Further research leading to consensus guidelines and standardization practices can help improve the care for these patients. V Treatment study/ survey [ABSTRACT FROM AUTHOR]