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Assessment of postoperative bowel function in anorectal malformation (ARM) patients is crucial for benchmarking outcomes. We compared existing bowel function scoring systems in various aspects in patients with ARM. With ethical approval, this was a cross-sectional study involving 5 paediatric surgery referral centres in Malaysia, comparing the Kelly, Japanese Study Group of Anorectal Anomalies (JSGA), Holschneider and Krickenbeck bowel function questionnaires. We recruited patients aged 4–17 years, who had completed definitive surgery & stoma closure (where relevant) > 12 months prior to participation. We standardised outcomes of each scoring system into categories ('good', 'fair', 'poor' and 'very poor') to facilitate comparison. Parents & patients were surveyed and asked to rate the ease of understanding of each questionnaire. The difference in protocol scores rated between parents and patients were compared. Association of each bowel function scoring protocol with type of anomaly was assessed. Statistical significance was p < 0.05. Thirty-nine parents (21 mothers, 18 fathers) and 23 patients were included in this study. Fair agreement was found between Kelly and Krickenbeck protocols (κ = 0.343; p < 0.001), between JSGA constipation and Holschneider protocols (κ = 0.276; p = 0.002); JSGA constipation and Krickenbeck protocols (κ = 0.256; p = 0.004); and between Holschneider and Krickenbeck protocols (κ = 0.273; p = 0.003). Only the Kelly protocol showed significant correlation between parents and patients' answers (ρ = 0.459, p = 0.028). Krickenbeck demonstrated the best negative correlation of patients' scores with ARM types (ρ = −0.401, p = 0.001). The Kelly protocol ranked highest when comparing ease of understanding. All the questionnaires appeared comparable in assessing postoperative faecal continence in ARM patients. The Kelly questionnaire performed best in 3 key areas of assessment. Level III Cross-Sectional Study. What is currently known about this topic? While there are many scoring systems to assess bowel function, there is not much information in regard to differences in applicability of the systems, whether they are easy to understand, are reflective of the types of ARM, and can be adapted to the local setting. What new information is contained in this article? The Kelly questionnaire performed best overall in 3 key areas of assessment: parent-patient inter-rater correlation, association between type of anomaly and bowel function, and ease of use. [ABSTRACT FROM AUTHOR]

Long-term outcomes of cloacal malformations remain unclear. We evaluated postoperative bowel control, bladder function and quality of life in patients under 18 years of age with cloaca. This was a multi-center cross-sectional observational study accomplished by the Nordic Pediatric Surgery Research Consortium. Patients with a cloacal malformation, 4–17 years of age, were eligible. Data including patient characteristics, surgical procedures, and complications were retrieved from case records. Established questionnaires with normative control values evaluating bowel function, bladder function, and health-related quality of life (HRQoL) were sent to the patients and their caregivers. The study was approved by the participating center's Ethics Review Authorities. Twenty-six (67%) of 39 eligible patients with median age 9.5 (range, 4–17) years responded. Twenty-one (81%) patients had a common channel ≤3 cm. Imaging confirmed sacral anomalies in 11 patients and spinal cord abnormalities in nine. Excluding patients with stoma (n = 5), median bowel function score was 12 [7-19], and 5 patients (20%) reported a bowel function score ≥17, approaching normal bowel control level. Bowel management increased proportion of socially continent school-aged children to 52%. Six (23%) patients had a permanent urinary diversion or used clean intermittent catheterization (CIC), while majority (70%) of the remaining patients were urinary continent. The reported HRQoL was comparable to healthy Swedish children. Whilst well-preserved spontaneous bowel control was rare, a majority of patients were dry for urine without any additional procedures. Few patients experienced social problems or negative impact on HRQoL due to bladder or bowel dysfunction. Level IV. • What is currently know about this topic? • Cloacas are complex malformations that require reconstructive surgical procedures and have a negative impact on bowel and bladder function. • What new information is contained in this article? • Most children with cloacal malformations have impaired bowel control, which improves with increasing age. A large proportion of patients need a permanent stoma or use antegrade enemas to stay clean. • The impaired bowel and bladder function has limited impact on health-related quality of life. [ABSTRACT FROM AUTHOR]

The goal of this study was to evaluate outcomes in patients treated with sacral nerve stimulation (SNS) for medically refractory fecal incontinence or severe constipation. We performed a retrospective cohort study of all patients treated with SNS after failed medical management at a single center between 9/1/2015 and 6/30/2022. Demographic and clinical data was extracted from the electronic medical record. Rates of involuntary bowel movements were evaluated using a bowel severity score questionnaire and compared pre- and post-SNS using McNemar and McNemar–Bowker tests. 70 patients underwent SNS placement. The median age was 12.8 years (IQR 8.6–16.0) and 61.4% were male. The most common diagnosis was idiopathic constipation (67.1%), followed by anorectal malformation (15.7%), and others. 43 patients had severity scores recorded both pre- and at least 90 days post-SNS insertion. The rates of daytime and nighttime involuntary bowel movements were significantly different pre-compared to post-SNS placement (p = 0.038 and p = 0.049, respectively). The rate of daytime and nighttime fecal continence increased from 44% to 58.1% and 53.5%–83.7%, respectively. The rate of at least weekly daytime and nighttime fecal incontinence decreased from 48.8% to 18.7% and 34.9%–7.0%, respectively. Minor pain/neurological symptoms occurred in 40% of patients, while 5.7% developed a wound infection. Further surgery for the SNS was required in 40% of patients. SNS placement can be an effective treatment for medically refractory fecal incontinence. Minor complications and the need for further procedures are common, while more serious complications like wound infections are rare. Retrospective Cohort Study. Level 3. [ABSTRACT FROM AUTHOR]

Congenital megarectum (CMR) is sometimes associated with anorectal malformations (ARM), although there is no established therapeutic strategy. This study aims to clarify the clinical features of ARM with CMR, and to demonstrate the effectiveness of a surgical treatment, namely laparoscopic-assisted total resection and endorectal pull-through technique. We conducted a review of the clinical records of the patients with ARM with CMR treated at our institution between January 2003 and December 2020. Seven of 33 cases of ARM (21.2%) were diagnosed with CMR, four males and three females. The types of ARM were 'intermediate' in four, and 'low' in three patients. Five of the seven patients (71.4%) required resection of megarectum for intractable constipation and underwent laparoscopic-assisted total resection and endorectal pull-through technique. Bowel function was improved after resection in all five cases. All five specimens showed hypertrophy of the circular fibers, and three of them showed abnormal location of ganglion cells within the circular muscle fibers. CMR often causes intractable constipation and requires resection of the dilated rectum. Laparoscopic-assisted total resection and endorectal pull-through technique for ARM with CMR considered to be an effective, minimally invasive treatment for intractable constipation. Level Ⅳ. Treatment study. [ABSTRACT FROM AUTHOR]

• Treating refractory functional constipation requires a collaborative approach by surgeons and gastroenterologists. • The work-up should include a contrast enema, anorectal manometry for assessment of the sphincters, and a colonic motility evaluation. • Antegrade flushes are effective and colon resections can be avoided in most cases, regardless of underlying colonic dysmotility. Management of refractory constipation in children has not been standardized. We propose a protocolized approach which includes a contrast enema, anorectal manometry and exclusion of Hirschsprung disease (HD). For those without HD or with normal sphincters, an assessment of the colonic motility may be needed. The subgroups of dysmotility include (1) slow motility with contractions throughout, (2) segmental dysmotility (usually the sigmoid), or (3) a diffusely inert colon. We offered a Malone appendicostomy in all groups with the hope that this would avoid colonic resection in most cases. Patients with medically refractory constipation were reviewed at a single institution (2020 to 2021). For patients without HD or an anal sphincter problem, assessment of colonic motility using colonic manometry was performed followed by a Malone appendicostomy for antegrade flushes. Of 196 patients evaluated for constipation refractory to medical management, 22 were felt to have a colonic motility cause. These patients underwent colonic manometry and Malone appendicostomy. 13 patients (59%) had a slow colon but with HAPCs throughout, 5 (23%) had segmental dysmotility, and 4 (18%) had a diffuse colonic dysmotility. 19 (86%) responded well to antegrade flushes with 17 reporting no soiling and 2 having occasional accidents. 3 patients (14%) failed flushes and underwent a colon resection within 6-month following Malone procedure. We propose a protocol for medically refractory constipation which provides a collaborative framework to standardize evaluation and management of these patients with antegrade flushes, which aids in avoidance of colonic resection in most cases. Level IV [ABSTRACT FROM AUTHOR]

• 3D High resolution anorectal manometry (3D-HRAM) provides useful measurements that help guide treatment of chronic constipation and faecal incontinence in children. • 3D-HRAM is safe and provides detailed functional morphology of anal sphincters and it is useful to understand underlying pathophysiology of constipation and fecal incontinence in children. • This articles demonstrartes that water perfused 3D-HRAM and conventional manometry (CM) profiles are comparable. Anorectal manometry is a valuable tool for objective assessment of motility motor and sensory function of the anorectum. The aim of this study was to investigate role of water-perfused (WP) three dimensional high-resolution anorectal manometry (3D-HRARM) compared to WP conventional manometry (CM) in the management of chronic idiopathic constipation (CIC) and faecal incontinence (FI) in children. This was a retrospective review of 122 consecutive children, who had WP 8-channel CM or 24 channel 3D-HRARM and endosonography under ketamine anaesthesia from September 2012 to February 2019. All patients had a validated symptom severity score questionnaire ranging from 0 (best) to 65 (worst). Mann-Whitney-U test and Spearman rank test were used and p <0.05 was considered significant. Subjects were divided according to investigation: CM group (n = 75) and 3D-HRAM (n = 47), who were otherwise comparable. Median anal resting pressure was 50 mmHg and rectoanal inhibitory reflex (RAIR) threshold volume was 10mls across the entire cohort. There were no significant differences in resting pressure or RAIR threshold when using conventional or 3D-HRARM. Rectal capacity was significantly higher in the CM group (p = 0.002). Rectal capacity and internal anal sphincter (IAS) thickness positively correlated with symptom severity, duration and patient age. 3D-HRARM provided a more detailed depiction of the anorectal pressure profile. Botulinum toxin was injected into the external anal sphincter (EAS) muscle (n = 75, 61%) and an anterograde colonic enema (ACE) stoma was needed subsequently in 19 (16%) to treat recurrent constipation and soiling symptoms. Rectal biopsy was done in 43(35%) patients, if RAIR was absent or inconclusive. CM and 3D-HRARM are comparable in terms of resting pressure and RAIR threshold. 3D-HRAM is safe and provides detailed functional morphology of anal sphincters and it is useful to understand underlying pathophysiology of constipation and faecal incontinence in children and plan further treatment. Level I [ABSTRACT FROM AUTHOR]

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

To determine the long term outcome of antegrade colonic enema (ACE) stoma for treatment of chronic constipation and soiling in children. We reviewed patients prospectively who underwent formation of ACE stoma after failure of maximum medical treatment of chronic constipation and soiling from September 2008 to October 2020. All patients had anorectal manometry, endosonography and colonic transit study. A validated symptom severity (SS) score questionnaire was used pre operatively and during follow up. The sum of SS score ranged between 0 (best) and 65 (worst). Data was expressed as median(range) and Wilcoxon rank sum test was used and p value < 0.05 was considered significant. 58 patients had formation of ACE stoma, laparoscopic appendicostomy 56, cecostomy 1, and open procedure 1. The main indications for the ACE stoma were chronic functional constipation (FC) 50(86%), anorectal malformations (ARM) 6(11%) and Hirschsprung disease (HD) 2(3%). 41(71%) of patients had previous intrasphincteric botulinum toxin (BT) treatment. Age at operation was 11(range 4–15) years and follow up was 36(3–84) months. 47(81%) patients were continent of stool within three months postoperatively. The preoperative SS score improved from 34/65 (26–47) to 8/65 (4–12) at 12 months and 5/65 (2–11) at 24 months follow up, p ≤ 0.05. complication rate was 15(26%), wound infection 5, granulation tissue 4, stoma prolapse 4, retraction 1, abscess formation and fistula 1. 15 (26%) adolescents had transition of their care to adult services. There is a significant improvement of constipation, soiling and general health of patients following formation of ACE stoma. Adolescents benefits an orderly transition of their care to adult services. Level 2, Therapeutic study. [ABSTRACT FROM AUTHOR]

Recent studies in children with idiopathic rectal prolapse report up to 48% require surgical intervention to manage refractory disease. We sought to examine outcomes of our non-surgical approach to managing rectal prolapse using a bowel management program. A retrospective review was performed for all children with the diagnosis of rectal prolapse between 2011 and 2020. Children with a rectal polyp or hemorrhoid were excluded. 47 children with rectal prolapse were identified (median age at diagnosis of 4 years (IQR 3,7.75); age ≤ 4 years n = 30; age > 4 years n = 17). Associated diagnoses included constipation (n = 45, 96%) and psychiatric diagnoses (n = 7, 14%). Children underwent a bowel management program including stimulant laxatives in 44 (94%) and osmotic laxatives in 2 (4%). Median follow-up time was 181 days (IQR 77, 238). Median time to resolution of rectal prolapse was 9 months (IQR 4, 13) with a maximum time to resolution of 31 months. We compared children ≤ 4 years old (Group A) to those > 4 years old (Group B). Psychiatric diagnoses were less common in Group A (3.5 vs. 38.9%, p = 0.003). Median time to spontaneous resolution was 6.5 months (IQR 3.5, 9.5) in Group A versus 13.5 (IQR 4, 16) months in Group B, p = 0.13. No differences in surgical intervention were identified. Three (6.4%) patients required surgery for prolapse. A bowel management program is an effective treatment for most children with rectal prolapse. This data suggests that surgical intervention is unnecessary in most children. III. [ABSTRACT FROM AUTHOR]

Introduction: Composite long-term outcomes of cloacal malformations remain unclear. We aimed to evaluate bowel and bladder control, sexual function, reproductive health, and quality of life in adulthood., Methods: A Nordic multi-center cross-sectional observational study evaluating the outcome of adult patients with cloacal malformations was performed. Patient characteristics were retrieved from case records. Established questionnaires were sent to the patients to evaluate bowel- bladder- and sexual function and quality of life., Results: Thirty-four of 48 (70%) eligible patients with median age 28 years (18-45) responded. Eight (24%) patients had a common channel >3 cm. Imaging-confirmed spinal and sacral abnormalities were present in 4 and 17 patients respectively. 85% (n = 29/34, four patients with permanent urinary diversions after bladder neck closure excluded) reported no urinary leakage without physical activity or urge to urinate. Three patients had a permanent enterostomy. 42 % (n = 11/26) reported acceptable bowel function according to Bowel Function Score (with eventual ongoing bowel management). The median Profile of Female Sexual Function Score was below normative values. Quality of life was comparable to the reference population, but thirty percent scored within the distress domains. Eight patients (24%) had been pregnant (15 pregnancies), resulting in seven live births, but the need of assisted reproduction techniques (40%, n = 6/15) and miscarriage (40%, n = 6/15) was common., Conclusions: Adequate spontaneous bowel control was rare, while most patients were dry for urine without additional procedures. Cloacal malformation also have a negative impact on sexual function health related quality of life and reproductive health. Long-term follow-up is crucial, not only regarding bowel and bladder function, but also for sexual and reproductive function, which may be important negative factors for health-related quality of life., Level of Evidence: Level IV., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: An option for medically refractory fecal incontinence and/or constipation is the antegrade continence enema (ACE). We investigated ACE usage and its perceptions, including whether patients were able to discontinue use of the appendicostomy/cecostomy tube., Methods: Patients who underwent appendicostomy creation or cecostomy tube placement at two institutions between 2012 and 2021 were reviewed. Patients or parents/guardians were contacted for completion of a survey. Summary statistics for clinical data were tabulated and associations were evaluated with chi-square analysis., Results: A total of 165 patients were included, including 92 (55.8%) males. Eighty-two (49.7%) surveys were completed. Most patients (51.5%) presented with fecal incontinence; 38 (23.3%) presented with constipation. More patients had a primary underlying diagnosis of anorectal malformation (39.4%), followed by functional constipation (21.2%), Hirschsprung disease (18.8%), and spinal malformation (17.6%). Thirty-six (21.8%) patients discontinued flushes by time of contact, with switch to laxatives being the most common reason (19%), followed by appendicostomy stricture/obstruction/closure (17%), switch to ileostomy/colostomy (17%), and patient preference (14%). There was no difference in patients' ability to stop using flushes based on underlying diagnosis (p = 0.31). The majority (84.1%) of respondents were "very likely" to recommend antegrade enemas to other children with similar diagnosis and 76.8% reported being "very satisfied" that the operation was done., Conclusions: There remains a high degree of satisfaction with antegrade continence enemas for children with constipation and fecal incontinence; some children may be able to stop using antegrade enemas with varied mechanisms including patient/family weaning versus with assistance and laxative trials., Type of Study: Retrospective cohort study., Level of Evidence: III., Competing Interests: Conflicts of interest The authors have no conflicts of interest to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Despite optimal conservative and medical treatment, some children with functional constipation (FC) continue to experience symptoms. Antegrade continence enema (ACE) surgery has been suggested as the primary surgical option after less invasive pharmacological and non-pharmacological interventions have not been effective. The purpose of this systematic review was to assess the outcomes of ACE for children with FC., Methods: Electronic databases were searched (inception-March 2024) for studies evaluating ACE surgery performed in children with FC. The primary outcome was treatment success (as defined in the original manuscript), including at least defecation frequency and/or fecal incontinence frequency. Secondary outcomes were cessation of ACE, complications, health-related quality of life (HRQoL) and patient/parent satisfaction. Quality of evidence was evaluated based on tools from the New-Ottawa Scale and Joanna Bridge Institute., Results: Thirteen studies were included, representing 477 children with FC treated with either an appendicostomy or a cecostomy. Reported treatment success rates varied widely, ranging from 32% to 100%. ACE treatment was stopped in 15% due to treatment success and in 8% due to treatment failure, leading to more invasive surgery. Complication rates ranged from 6% to 100%, requiring surgical intervention in 0% to 34%. An improvement in HRQoL following ACE treatment was reported in all three studies that assessed HRQoL. The two studies assessing patient/parent satisfaction, reported high satisfaction rates., Conclusion: Reported treatment success and complication rates following ACE surgery for children with FC vary widely. This systematic review highlights the necessity for uniform definitions and treatment guidelines for ACE surgery in children with FC., Level of Evidence: III., Competing Interests: Conflict of interest None., (Copyright © 2024. Published by Elsevier Inc.)

Background: Chronic constipation and fecal incontinence are devastating problems for patients with anorectal malformations, Hirschsprung's disease, idiopathic constipation, and spina bifida/spinal cord injuries. Finding the proper regimen allows these patients to be out of diapers and free of stool accidents. A typical bowel management regimen consists of daily laxatives or enemas; because these products are sold over the counter, insurance companies do not cover them. This study reviews the cost of bowel regimens and analyzes their economic impact on the families we treat., Methods: A retrospective review of patients undergoing bowel management between January 2016 and September 2023 was done. The mean annual income of families was calculated using their zip codes., Results: Upon review, 430 patients met inclusion criteria; 167 were on laxatives, and 263 were using enemas. There was significant variation in the cost of medications based on the distributors they were bought from and the dose of the medication in their regimen. The cost of laxatives ranged from $15.70 to $2938.10 annually. The cost of enemas containing glycerin ranged from $29.20 to $4380.00 annually. In comparison, diapers/incontinence briefs ranged from $131.40 to $4343.50 annually. The median annual income of patients across 41 states ranged from $32,192 to $225,119., Conclusions: Our findings emphasize the importance of proper counseling families regarding medication costs in the market, intending to promote long-term treatment adherence. They also serve as a data source to advocate for improved insurance coverage of the medications required to manage these chronic conditions successfully., Level of Evidence: IV., Competing Interests: Conflict of interest The authors have no conflict of interests or competing interests to declare., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Children with severe constipation and a neurodiverse disorder (Autism and/or developmental delay) represent a challenging bowel management group. Treatment outcomes with laxative or enema therapy remain limited and are often complicated by patient/caregiver compliance. We hypothesized that children with neurodiverse disorders and severe constipation would benefit from a bowel management program (BMP) that includes early use of antegrade enemas. Children requiring BMP in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry with diagnosis of constipation and/or constipation with pseudo-incontinence were reviewed. Those with Hirschsprung disease and/or Anorectal Malformation were excluded. BMP needs in patients with a neurodiverse diagnosis were compared to those without to evaluate differences in BMP's. 372 patients requiring a BMP were identified. 95 patients (58 autism spectrum, 54 developmental delay) were neurodiverse, and 277 patients were not. Neurodiverse patients had a higher prevalence of enema therapy 62.1% (59/95) vs. 54.9% (152/273) and use of antegrade enema therapy 33.7% (32/95) vs. 21.2% (58/273), p = 0.126. Neurodiverse patients were older 37.9% (36/95) > 12 years vs. 23.1% (63/273), p = 0.001 and 47.6% (10/21) were changed from laxative to enema therapy over time. 80% (8/10) of those changed from laxatives to enemas used antegrade therapy. 67.3% (35/52) of neurodiverse patients followed over time were on enema therapy at the most recent visit with 80% (28/35) requiring antegrade therapy. A large portion of patients with a neurodiverse disorder who fail laxative therapy use antegrade enemas to achieve effective bowel management. Early consideration of an antegrade conduit may simplify treatment in this group of children. III. [ABSTRACT FROM AUTHOR]

Bowel management programs are efficacious in pediatric patients with fecal incontinence or intractable constipation unresponsive to standard treatment. No studies have been done examining outcomes in adults. The objective of this study was to assess continence and quality of life outcomes in adults who have underwent bowel management program. A retrospective review of patients 16 or older at the time they underwent a bowel management program for fecal incontinence or constipation was performed. Data collected included intake and follow-up stool and urinary continence, patient-reported outcomes measures (Cleveland Clinic Constipation Score, Baylor Continence Scale, Vancouver Symptom Score for Dysfunctional Elimination), and an age-adjusted health-related quality of life measure. The cohort included 38 patients with a median age of 19 years (range: 16-55) when they underwent our program. 50% of patients were female and the majority (33, 87%) were White. The most common diagnosis was anorectal malformation (16, 42%) followed by functional constipation (10, 27%). Stool continence rates improved after undergoing the program (52.7% prior to 87.6% at follow-up, p<0.01). There was significant improvement in the Baylor Continence Scale, Cleveland Clinic Constipation Score, and PedsQL (p<0.05). Adult patients who underwent a bowel management program for severe fecal incontinence or constipation show significant improvement in stool continence rates, patient-reported outcomes measures, and quality of life. A bowel management program (in-person or via telemedicine) is a feasible treatment strategy for adult patients who fail standard management of fecal incontinence or constipation and should be offered when appropriate. Level of evidence: III. [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]

BackgroundSacral nerve stimulation (SNS) can be beneficial for children with constipation, but no studies have focused on children with constipation severe enough to require antegrade continence enemas (ACEs). Our objective was to evaluate the efficacy of SNS in children with constipation treated with ACE.MethodsUsing a prospective patient registry, we identified patients

• What is currently known about this topic? Patients with organic chronic constipation and fecal incontinence (CCFI) have improved Quality of Life (QoL) following appendicostomy or cecostomy creation and antegrade colonic enema (ACE) therapy. • What new information is contained in this article? Patients with functional CCFI have improved QoL following cecostomy creation and ACE therapy, and the QoL improvements are comparable to patients with refractory CCFI due to an organic cause. We compared patient- and family-reported overall and stool-related quality of life (QoL) before and after an antegrade continence enema (ACE) procedure (cecostomy tube insertion) for refractory chronic constipation or fecal incontinence (CCFI). We hypothesized that patients with functional diagnoses experience similar improvements in QoL compared to those with organic diagnoses. This is a cross-sectional study of patients undergoing cecostomy tube insertion for CCFI at a tertiary pediatric hospital from 2012 to 2019. Patients and/or primary caregivers completed validated stooling and overall QoL surveys based on three time points: before surgery, three months after surgery, and at the time of survey / date of last follow-up. Repeated measures analyses compared scores over time between subjects and within the diagnostic groups. The response rate was 65% (22/34 patients, 12 organic and 10 functional diagnoses). Mean age was 8.3 years and 32% of the participants were female. Organic diagnoses were: spina bifida (6), anorectal malformation (5), and Hirschsprung Disease (1). There was substantial improvement in stool-related and overall QoL at three months post-ACE procedure (both p<0.001) for all patients; both scores continued to improve significantly until the date of last follow-up (median 4.1 years, IQR 2.3–5.6, p<0.001). There was no statistically significant difference in scores between patients with organic and functional diagnoses. Caregivers perceive a significant, sustainable improvement in stooling habits and QoL following ACE therapy. The improvement is comparable between patients with a functional diagnosis and those with an underlying organic reason for their CCFI. [Display omitted] [ABSTRACT FROM AUTHOR]

An appendicostomy (ACE) is a surgical option for antegrade enemas in children with severe constipation and/or fecal incontinence who have failed medical management. In 2019, we initiated an expedited post-operative protocol and sought to examine our short-term outcomes compared with our historical cohort. A retrospective review was performed of all children undergoing ACE between 2017 and 2020. Children were excluded if they underwent an associated procedure (e.g. colon resection). Patients were divided into two cohorts: historical cohort (2017–2018, Group A) and the expedited protocol (2019 to present, Group B). The primary outcome was length of stay. 30 patients met inclusion (Group A = 16, Group B = 14). The most common indications for ACE were constipation (50%) and constipation or fecal incontinence associated with anorectal malformation (43%). Group B experienced a decreased length of stay (1 vs 3 days, P = 0.001) without differences in 30-day surgical site infection (7.1% vs 18.8%, p = 0.61) or unplanned visit (15.4% vs 18.8%, p = 1.0). Group B had a higher prevalence of MiniACE® button placed through the appendix vs. Malone (42.8% vs 12.5%, p = 0.10). Our expedited post-op protocol decreased length of stay without other significant adverse clinical sequelae. Retrospective Comparative Study, Level III. [ABSTRACT FROM AUTHOR]

Many patients with anorectal malformations (ARM) need a bowel management program (BMP) to manage lifelong problems of fecal incontinence or severe constipation. We aimed to evaluate the sustainability of the results in such a program. A single-institution retrospective review was performed in children with ARM who attended our BMP (2015–2019). Standardized definitions and validated tools were used to assess fecal continence (Baylor Continence Scale), constipation (Cleveland Constipation Scoring System), urinary symptoms (Vancouver Symptoms Score), and the Pediatric Quality of Life (PedsQL) and health-related quality of life (HRQOL) at the start of BMP and 1-year after completion of the program. 222 patients with ARM at a median age of 6.7 (IQR, 4.9–10.1) years were identified. All (100%) soiled at intake with 149 (67.1%) patients being treated with rectal or antegrade enemas and 73 (32.9%) with oral laxatives. At 1 year 150 (70.4%) were clean, 72.7% were on enemas and 27.3% were on laxatives (p = 0.08). 109 out of 148 (73.6%) patients were clean on enemas. A further 41 out of 66 (62.1%) patients were continent on laxatives with voluntary bowel movements and clean. In the group that was clean, there was improvement in Baylor Continence Scale (25 vs. 13.0, p < 0.000000002), Vancouver (11 vs. 6, p = 0.0110) scores, and clinically relevant improvement in the total PedsQL HRQL (78–85) and the PedsQL HRQL physical function (86–92) and psychosocial domain (77–82). There was no improvement in Cleveland (10 vs. 9, p = 0.31) score. An intensive BMP offers significant benefits in the treatment of fecal incontinence in ARM. It appears to also improve urinary incontinence and urinary voiding as well as the patient's quality of life. These changes are sustainable over at least one year. [ABSTRACT FROM AUTHOR]

To present the long-term follow-up outcomes of patients with intestinal neuronal dysplasia type B (IND-B) managed either conservatively or surgically. We conducted an ambispective, observational, longitudinal, and comparative study. Clinical data were reviewed at the start of treatment. After a minimum period of five years, the patients participated in semi-structured interviews in which the bowel function score (BFS) was applied to assess intestinal function, a proposed intestinal symptom index (ISI) to assess clinical symptoms, and a classification of clinical prognosis to assess treatment success. Comparisons between the two types of treatment were performed by evaluating pre- and post-treatment criteria. Fifty patients diagnosed with IND-B were included in the study. Thirty-eight patients underwent surgical treatment (26 elective surgical treatment for primary colorectal resection and 12 emergency colostomies for intestinal obstruction or enterocolitis). Twelve patients were managed conservatively. With the exception of the patients who required an emergency operation (n = 12), the two groups were composed of patients with severe constipation who had similar clinical and functional characteristics at the time of IND-B diagnosis. A better clinical response was observed in patients submitted to conservative treatment, with a greater increase in the BFS (16.5 [-4/+18] versus 4 [-15/+17]; p = 0.001), indicating better bowel function and a more pronounced drop in ISI (-6 [-7/-4] versus -4 [-6/+1]; p = 0.015), suggesting fewer symptoms. The percentage of patients who had a successful treatment was higher in the group treated conservatively (72.7% versus 42.3%; p = 0.03). Conservative management showed better long-term outcomes than surgical management in children with IND-B. [ABSTRACT FROM AUTHOR]

• Olive oil enemas are a safe and effective remedy for chronic constipation. • Olive oil enemas followed by glycerin enemas are useful for fecal disimpaction. • Olive oil enemas are effective in patients with various underlying disorders requiring bowel management. Few reports have determined the efficacy of olive oil enemas for severe constipation. Here, we review our experience with olive oil enemas in children with severe chronic constipation. In our outpatient pediatric surgery department, the charts of 118 patients prescribed with olive oil enemas between January 2010 and November 2019 were retrospectively reviewed. A 1–2 ml/kg olive oil enema was given either alone or followed several hours later by a glycerin enema. Ratings included "very effective (VE)," "effective (E)," "limited (L)," "ineffective (I)," and "unknown (U)." One hundred and fifteen (97.5%) patients were able to use olive oil enemas at home. Forty-nine had functional constipation; 43 had anorectal malformation; 40 had Hirschsprung disease; 12 had spina bifida; and 10 had other maladies. Used as an enema, olive oil was effective in treating fecal impaction in 77.6% of patients; as a lubricant, it was effective in treating 76.9% of patients. Efficacy for fecal disimpaction was similar among patients with different underlying disorders. Olive oil enemas are useful for more than three-quarters of children with severe chronic constipation. Further study is warranted to add olive oil enemas as an adjunctive treatment in the management of severe constipation. [ABSTRACT FROM AUTHOR]

Enemas have become a common practice for treating fecal incontinence and severe constipation. Several patients receiving enemas complained of severe, colicky, abdominal pain during enema administration and complained that the duration for fluid to pass was progressively increasing. Contrast studies showed a startling picture of severe right colon dilatation and a spastic, narrow, left colon. An investigation was started to seek the origin and possible management of this condition. Medical and radiologic records were reviewed retrospectively, with emphasis on the type and ingredients of enemas used, the duration the patients had been receiving enemas, and their original diagnosis. A literature review was done on previous reports of this condition and publications related to long-term use of enemas. This series included 22 patients (average age, 19.6 years; range, 8–54) with fecal incontinence due to anorectal malformations (10 cases), myelomeningocele (5), cloaca (2), severe colonic dysmotility (2), Hirschsprung's disease (2), and sacrococcygeal teratoma (1). The average duration of enema use was 13.7 years (range, 4–45). The composition of the enemas included saline/glycerin (six cases), only saline solution (five), saline/glycerin/soap (four), plain water (three), and one case each of molasses/milk, saline/glycerin/soap/phosphate, saline/phosphate, and only phosphate. The enemas were performed in an antegrade fashion in 21 cases and rectally in 1. All patients had a dilated right colon and a narrow, spastic, left, transverse, and descending colon. Four patients underwent colonoscopy, colonic manometry, and mucosal biopsies, which did not help in explaining the etiology of the problem. In the literature, 43 reports mentioned a "long-term follow-up" for the administration of enemas, but we could not find a description of symptoms, such as in our cases. An intriguing and, to our knowledge, previously unreported complication of chronic enema use is presented. We call attention to an overly concerning complication and report our findings in the hope that they will aid and stimulate more investigations into this condition. Several hypotheses to explain the cause are presented, as well as potential treatment options. [ABSTRACT FROM AUTHOR]

Children undergoing repair of an anorectal malformation (ARM) may have persistent soiling and/or constipation postoperatively. An anatomic reason should be sought; one of the causes for these problems that may require reoperation is mislocation of the rectum and anus outside of the muscle complex. We reviewed our population of children who underwent re-do anorectoplasty surgery between 2014 and 2019. Indications for surgery and outcomes were recorded. Twelve patients had a lateral mislocation and underwent reoperation. There were no immediate complications in this subgroup. 9 of 10 patients are clean, and 4 are now able to have voluntary bowel movements. For patients who are found to have a significant lateral mislocation, we describe a new surgical technique that replaces the rectum and neo-anus directly in the midline through the muscle complex which may improve functional outcome. [ABSTRACT FROM AUTHOR]

Anal fissure (AF) in children is usually treated with laxatives and/or topical agents such as calcium channel blockers. We hypothesize that owing to the superior efficacy of Polyethylene glycol (PEG) in treating constipation in children, adding diltiazem (DTZ) might not improve healing of AF. Children ≤ 14 years with anal fissure presented to the pediatric surgery clinic between November 2014 and March 2016 were recruited. Randomization was performed to either PEG with DTZ or PEG with placebo. Study personnel, patients, and their families were blinded. Primary outcome was resolution of symptoms. Secondary outcomes were constipation and treatment complications at 12-week follow up. 48 patients were randomized: 24 to PEG + DTZ and 24 to PEG + placebo. Both groups were similar in their baseline characteristics. At week 12, majority of patients' symptoms have improved without significant difference between groups; painful defecation at week 12: 20.8% and 8.3% (p-value 0.41), blood per rectum at week 12: 4.2% and 8.3% (p value 0.58) in the DTZ and placebo groups, respectively. Additionally, there was similar improvement in constipation in both groups. PEG alone was associated with similar improvement in anal fissure symptoms in children compared to PEG and topical diltiazem combined. I [ABSTRACT FROM AUTHOR]

Anorectal malformations (ARMs) are common congenital anomalies cared for at Mulago Pediatric Surgery Unit (PSU), similar to other sites in the region. All patients undergo staged repairs and complete treatment at older ages compared to high-income countries (HICs). This is the first study to examine long-term bowel function in our patients and compare with HICs. A retrospective cohort study was conducted of all children 3–12 years old with ARMs who had repair between January 2012 and June 2017 and who completed surgical repair at least 6 months prior to the study. Bowel function was measured using the fecal continence scoring system derived from Rintala and Lindahl (1995). As in prior studies, patients were classified by score into four categories: Poor (6–9); Fair (9–11); Good (12–17); and Normal (18–20). Median follow up was 2 years post stoma closure. Long-term bowel function was Normal/Good in 65% (C.I 27, 45), and Fair/Poor 35% (C.I 55, 73), with soiling in 49% (C.I 40, 59), constipation in 23% (C.I 16, 32); and incontinence in 39% (C.I 30, 39). There was no statistically significant association between bowel function and multiple demographic, social, and clinical factors. Median age at completion of treatment (stoma reversal) was 2.3 years old, and median duration of colostomy (interval between stoma placement and takedown) was 1.8 years. Despite definitive repair at older age and almost two years of living with a stoma, our patients achieve fair long-term bowel function. Nonetheless, improved follow up and timely management of complications may improve outcomes soiling, incontinence and constipation. Level II. [ABSTRACT FROM AUTHOR]

We sought to examine the long-term clinical success rates of a bowel management program (BMP) for children with severe constipation or fecal incontinence. A single center review was conducted of children (≤ 18 years) enrolled in a BMP and followed in a colorectal specialty clinic (2011–2017). All patients who completed an initial week of the BMP were included. Patients enrolled in a BMP after 2018 were excluded. Success was defined as no accidents and < 2 stool smears per week. A total of 285 patients were reviewed. BMP was initiated at a median age of 7 years (9 months-17 years). Primary diagnoses included functional constipation (112), anorectal malformation (ARM) (104), Hirschsprung Disease (HD) (41), rectal prolapse (14), spina bifida (6), fecal incontinence (3) and other (5; 4 sacral coccygeal teratomas and a GSW to the buttocks). Initial bowel regimen included large volume enema in 54% and high dose stimulant laxative in 46%. The initial Bowel Management Week (BMW) was successful in 233 (87% of adherent patients) patients with 17 (6%) non-adherent. One hundred twenty-two patients had follow-up at 12 months (72% success amongst adherent patients, 7% of patient non-adherent) and 98 patients had follow-up at 24 months (78% success amongst adherent patients, 10% of patients non-adherent). 21/154 (14%) patients started on enemas were later successfully transitioned to laxatives and 13/132 (10%) patients started on laxatives subsequently required enemas in order to stay clean. Clinic phone contact occurred outside of scheduled visits for adjustment to the BMP in 44% of patients. 33% of patients had surgery to aid bowel management (antegrade colonic enema (ACE) = 81, resection + ACE = 13, diverting stoma = 4). Median follow up was 2.5 years (5 weeks–7 years). Children who follow a structured BMP with readily available personnel to provide outpatient assistance can experience successful treatment of severe constipation or fecal incontinence long-term. A multi-institutional collaboration is necessary to identify factors which predict failure of a BMP and non-adherence. Single-center retrospective chart review. 3 [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate the impact of implementing a Bowel Management Program (BMP) on the quality of life (QOL) in children with pseudoincontinence. Children aged 2.5–13 years with pseudoincontinence were included. Evaluations were performed before and after BMP implementation. Perceptions from parents about QOL were assessed by a QOL questionnaire, and the severity of fecal incontinence (FI) was assessed using the fecal incontinence index (FII) of the Wexner scale. 115 children (mean age of 7.54 ± 2.48) were studied over a 3–12 month period (mean duration 4.36 months). QOL improved from 2.45 ± 1.57 to 14.36 ± 1.37, P˂0.05. There was a significant improvement of the FII: 18.65 ± 1.25 versus 0.13 ± 0.39, P˂ 0.05. There was a significant inverse correlation between the final scores of QOL (14.36 ± 1.37) and FII (0.13 ± 0.39) after implementation of the BMP (r = − 0.53; P ˂ 0.05). This is the largest case series examining QOL in pseudoincontinent children. It demonstrates that BMP significantly improves the QOL of these children in the short and midterm. In addition, it is feasible to apply the FII in assessing incontinence in children. To our knowledge, this is the first study of its kind in our region. Level IV. [ABSTRACT FROM AUTHOR]

The purpose of this study was to describe the outcomes of colonic resection for constipation in children. Three different types of resection are compared: pan-proctocolectomy with ileoanal pouch anastomosis (IPAA), total colectomy with ileorectal anastomosis (IR), and segmental resections and anastomosis (SR). All colonic resections were prospectively recorded and their outcomes tabulated. Outcomes were classified as Good: anal defecation with no soiling; Intermediate: anal defecation with occasional soiling or need for ACE; Poor: a permanent stoma. All complications were also recorded. 22 children underwent colonic resection over a 20 year period. Mean follow up was 40 months. 18 had prior antegrade continent enema (ACE), and 12 had a prior stoma. 13 underwent IPAA, 6 IR, and 3 SR. 10 (45%) had a good outcome, 4 (18%) had an intermediate outcome, and 8 (36%) had a poor outcome. Resection restored 9/12 (75%) of children with a preexisting stoma to anal defecation. No procedure produced better outcome than the others in terms of results or complications. There may be a role for colonic resection in selected constipated children, but parents should be warned that there remains a significant possibility of a permanent stoma. Our study suggests that around two-fifths will be left with a permanent stoma. Level IV. [ABSTRACT FROM AUTHOR]

The purpose of this study was to assess bowel function and quality of life (QoL) in patients with Hirschsprung's disease (HD) and identify patients who have 'failed' treatment. A review of a single-center HD cohort treated with pull-through surgery from 2004 to 2017 was completed. Bowel function of patients five years and above and QoL of all patients were assessed using validated questionnaires. Patients who 'failed' treatment were defined as above five years with one or more of: a) long-term stoma, b) needing an antegrade continence enema or transanal irrigation, c) severe soiling, or d) severe constipation. Statistical analysis was performed with P < 0.05 deemed significant. Data are given as mean [range]. Seventy-one patients presented with HD within the study period. Mean follow-up was 5.4 years [0.7–13.3]. Of 38 eligible patients, bowel function was assessed in 24 patients (nine had a stoma, five lost to follow-up). The mean incontinence score was 17 [0–28)], and the mean constipation score was 17 [5–25]. Incontinence and constipation scores were worse than healthy controls (P < 0.001 and P = 0.001, respectively) and did not improve with age. Fifty-six patients had QoL assessed with no difference between our cohort (81 [25–100]) and healthy controls (81 [unknown]); (P = 0.85). Thirty-three patients were assessed for 'failure' (bowel function score n = 24; stoma n = 9). Thirty of 33 (91%) children older than five years can be considered to have 'failed' treatment. Patients have worse bowel function than healthy children, which does not improve with age. QoL is comparable to healthy controls. A significant proportion of patients have poor outcomes and have 'failed' treatment. Level III. [ABSTRACT FROM AUTHOR]

Retained transition zone is a leading cause of obstructive symptoms after pull-through operation in Hirschsprung's disease. We aimed to evaluate the extent of the histological transition zone in patients with Hirschsprung's disease. We performed an observational study. DAB + immunohistochemistry for Protein Gene Product 9.5 was used to evaluate the neuronal networks in serial sections of pull-through specimens obtained from children with Hirschsprung's disease (n = 12). Reference ranges for ganglion size/density and nerve trunk diameter were statistically determined using healthy controls obtained from colostomy specimens from children with anorectal malformations (n = 8). The transition zone was defined as ganglionic bowel exhibiting ganglion hypoplasia, hypertrophic nerve trunks, or partial circumference aganglionosis. The mean submucosal nerve trunk diameter in controls was 19.56 μm +/− 3.87 μm. The median age at pull-through for Hirschsprung's disease was 5 months (3–14 months). The median length of the transition zone across the population was 8 cm (4–22 cm). Median transition zone extent was significantly longer in patients with long-segment aganglionosis (n = 6) compared to rectosigmoid aganglionosis (n = 6, 13 cm vs 6 cm, p = 0.041). Due to the age of the patients enrolled, long-term follow-up of bowel function is not yet available. Our data suggest that, in children with rectosigmoid Hirschsprung's disease, the transition zone can extend for up to 13 cm. In children with long-segment disease, a longer transition zone is possible. Extended resection at a minimum 5 cm beyond the most distal ganglionic intra-operative biopsy and intra-operative histological examination of the proximal resection margin are required to minimize transition zone pull-through. 2. [ABSTRACT FROM AUTHOR]

Botulinum toxin (botox) is a commonly used treatment for functional anorectal and colonic disorders. Although generally regarded as safe, complications associated with botox injection into the anal sphincters in children with severe defecation disorders are not well described. We aimed to review our institutional experience and the existing literature to better understand the safety of this practice. We performed a retrospective review of pediatric patients undergoing botox administration into the anal sphincter for treatment of a variety of defecation disorders between 2014 and 2018. Additionally, we performed a review of all published literature reporting complications from botox injection in this patient population. 881 patients ranging from 5 weeks to 19.7 years underwent a total of 1332 botox injections including our institution (332 patients/526 injections) and the reviewed series (549 patients/806 injections). Overall, complications were seen after 9 (0.7%) injections and included urinary incontinence (n = 5), pelvic muscle paresis (n = 2), perianal abscess (n = 1), pruritis ani (n = 1), and rectal prolapse (n = 1). Patient age, weight, and diagnosis were not associated with an increased rate of complication in our institutional experience. All complications were self-limited and did not require intervention. There were no episodes of systemic botulinum toxicity. Botox injection into the anal sphincters is accepted practice in children with Hirschsprung disease, severe functional constipation, and internal anal sphincter achalasia and appears to be safe from this review. The precise dosing and age at which complications are more likely to arise could not be ascertained and require further study. IV Retrospective cohort study. [ABSTRACT FROM AUTHOR]

Many management options exist for the treatment of refractory rectal prolapse (RP) in children. Our goal was to characterize current practice patterns among active members of APSA. A 23-item questionnaire assessed the management of full-thickness RP for healthy children who have failed medical management. The survey was approved by our IRB and by the APSA Outcomes committee. 236 surgeons participated. The respondents were geographically dispersed (44 states, 5 provinces). 32% of respondents had twenty or more years of clinical experience. 71% evaluated 1–5 RP patients in the last 2 years, while 5% evaluated > 10. 71% performed 0–1 procedure (operation or local therapy [LT]) for RP over 2 years. 59% would treat a 2-year-old patient differently than a 6-year-old with the same presentation, and were more likely to offer up-front surgery to a 6-year-old (26% vs 15%, p = 0.04), less likely to continue medical management indefinitely (2% vs 7%, p = 0.01), and more likely to perform resection with rectopexy (30% vs. 15%, p = 0.01). 71% perform LT as an initial intervention: injection sclerotherapy (59%), anal encirclement (8%), and sclerotherapy + anal encirclement (5%). 70% consider LT a failure after 1–3 attempts. If LT fails, surgical management consists of transabdominal rectopexy (46%), perineal proctectomy or proctosigmoidectomy (22%), transabdominal sigmoidectomy + rectopexy (22%), and posterior sagittal rectopexy (9%). There is wide variability in the surgical management of pediatric rectal prolapse. This suggests a need for development of processes to identify best practices and optimize outcomes for this condition. [ABSTRACT FROM AUTHOR]

Chronic constipation in children is associated with primary megarectum. This study investigated outcomes of surgical treatment of idiopathic megarectum in children. This retrospective comparative study included 52 children with idiopathic megarectum (mean age, 9.4 ± 1.7 years) treated from 2007 to 2016. Patients were divided into 2 groups. Group 1 included 23 patients who underwent a Soave pull-through operation. Group 2 included 29 children who underwent laparoscopic low anterior resection with endorectal stapled anastomosis using laparoscopic ultrasound guidance. All patients had clinical and laboratory evaluations with anorectal manometry, colonoscopy and contrast enema studies. Six patients (26.1%) in Group 1 and one (3.5%) in Group 2 experienced anastomosis leakage requiring colostomy (χ2 = 3.867, P = 0.049). In long-term follow-up, 3 children (13.1%) in Group 1 and 2 (6.9%) in Group 2 had ongoing constipation; this difference was not significant. Frequent loose stools with soiling were significantly more common in Group 1 (14 patients; 60.9%) than in Group 2 (4 patients; 13.8%) (χ2 = 10.566, P = 0.001). Our experience shows that laparoscopic video-assisted low anterior resection of the colon with endorectal stapled anastomosis under laparoscopic ultrasound guidance to determine the level of colon resection is the better operation then Soave for children with idiopathic megarectum and chronic constipation. This approach provides good functional results and reduces complications. Treatment study. Level III, retrospective comparative study. [ABSTRACT FROM AUTHOR]

Abstract Purpose Children failing medical management for severe constipation and/or fecal incontinence may undergo surgical intervention for antegrade enema administration. We present a modification of the laparoscopic-assisted percutaneous endoscopic cecostomy (LAPEC) procedure that allows primary placement of a skin-level device. Methods A single-institution retrospective review was performed from 2009 to 2015. In the modified technique the colonoscope is advanced to the cecum, cecal suspension sutures are placed under laparoscopic visualization, and percutaneous needle puncture of the cecum is performed under direct laparoscopic and endoscopic visualization. A skin-level cecostomy tube is then placed over a guide wire. Patient characteristics and 30-day results were analyzed by Fisher's exact test. Results Fifty-two patients underwent attempted LAPEC. Successful LAPEC using both laparoscopic and endoscopic guidance was achieved in 46 (88.5%). A MIC-KEY device was placed in 38. Corflo PEG tube placement was necessary in 14 due to high BMI (mean 28.4). Colonoscopy failed to reach the cecum in 6 and laparoscopy alone was utilized to achieve successful tube placement. Cecostomy site infections occurred in 3 (5.8%), only in those undergoing PEG placement using a pull technique (p < 0.05). Conclusion Primary placement of a skin-level device was successful in the majority of patients undergoing cecostomy tube placement for bowel management utilizing antegrade colonic enemas. This technique avoids a second anesthesia for tube conversion. Visualization via colonoscopy with the use of cecal suspension sutures is recommended. High BMI necessitates initial placement of a PEG tube and complications exclusively occurred in this group. Type of Study Clinical. Level of Evidence IV Case series study. [ABSTRACT FROM AUTHOR]

Abstract Background Appendicostomy and cecostomy are two approaches for antegrade enema access for children with severe constipation or fecal incontinence as adjuncts to a mechanical bowel management program. Each technique is associated with a unique set of complications. The purpose of our study was to report the rates of various complications associated with antegrade enema access techniques to help guide which option a clinician offers to their patients. Methods We reviewed all patients in our Center who received an appendicostomy or cecostomy from 2014 to 2017 who were participants in our bowel management program. Results 204 patients underwent an antegrade access procedure (150 appendicostomies and 54 cecostomies). Skin-level leakage (3% vs. 22%) and wound infections (7% vs. 28%) occurred less frequently in patients with appendicostomy compared to cecostomy. Nineteen (13%) appendicostomies required revision for stenosis, 4 (3%) for mucosal prolapse, and 1 (1%) for leakage. The rates of stenosis (33 vs. 12%) and wound infection (13 vs. 6%) were higher in patients who received a neoappendicostomy compared to an in situ appendicostomy. Intervention was needed in 19 (35%) cecostomy patients, 15 (28%) for an inability to flush or a dislodged tube, and 5 for major complications including intraperitoneal spillage in 4 (7%) and 1 (2%) for a tube misplaced in the ileum, all occurring in patients with a percutaneously placed cecostomy. One appendicostomy (1%) patient required laparoscopic revision after the appendicostomy detached from the skin. Conclusion Patients had a lower rate of minor and major complications after appendicostomy compared to cecostomy. The unique complication profile of each technique should be considered for patients needing these procedures as an adjunct to their care for constipation or fecal incontinence. Type of study Retrospective comparative study. Level of evidence Level III. [ABSTRACT FROM AUTHOR]

Abstract Background Postoperative outcomes for Hirschsprung disease (HD) remain variable, with many patients affected by constipation and/or fecal incontinence. The long-term impact upon quality of life (QoL) for HD patients is unclear. We measured long-term QoL outcomes in adolescents with HD using validated questionnaires. Methods Patients with HD, managed at a large tertiary pediatric institution between 1997 and 2004, were identified. Patients and/or their proxy completed validated questionnaires. Results were compared with published healthy population controls. Two questionnaires assessed QoL: Pediatric Quality of Life Inventory (PedsQL) and Fecal Incontinence and Constipation Quality of Life (FICQOL). Three measures assessed functional outcomes: Baylor Continence Scale, Cleveland Clinic Constipation Scoring System, and Vancouver Dysfunctional Elimination Symptom Survey. Results Interviews were completed for 58 (70% response rate) patients [M:F, 49:9; median age, 14.5 years (11.1–18.7)]. No significant differences were found in general QoL scores between patients and healthy controls (84.84 versus 81.49, p = 0.28). Disease-specific questionnaires revealed reduced QoL in patients and families, with 17% of parents reporting the bowel dysfunction stopped their child from socializing and 47% of parents experiencing some degree of anxiety/depression regarding their child's bowel condition. Fecal incontinence (r = − 0.59, p < 0.01), constipation (r = − 0.36, p = 0.01), and dysfunctional elimination (r = − 0.59, p < 0.01) all negatively correlated with QoL scores. Conclusions In this study, generic QoL in the adolescent HD population was comparable to healthy populations. However, children with HD have ongoing bowel dysfunction which negatively impacts upon their QoL. Level of evidence Prognosis study:– level II (prospective cohort study). [ABSTRACT FROM AUTHOR]

Purpose To evaluate bowel function in patients with anorectal malformations (ARM) comparing existing scoring systems. Methods Parents of ARM patients treated at our institution were asked to fill in Holschneider, Kricknebeck, and Rintala questionnaires. Scores obtained from the questionnaires were expressed per cent and analyzed depending on the age and type of ARM according to Krickenbeck classification. Patients younger than 3 years of age or with developmental delay were excluded. Results Eighty patients (42 males: 52%) were included. Median age was 7.6 years (range 3–22). Twenty eight patients (35%) had perineal fistula, 13 (16%) bulbar, 7 (9%) prostatic, 5 (6%) rectobladder neck, 15 (19%) vestibular, 7 (9%) had a cloaca and 5 (6%) imperforate anus without fistula. Using Holschneider, Krickenbeck, and Rintala, average scores were respectively 72, 71 and 73 (p = 0.4 with ANOVA). Using the three questionnaires patients with perineal fistula scored 82, 76 and 84 respectively (p = 0.003), with bulbar 70, 71, 73 (p = 0.8), with prostatic 52,69,59 (p = 0.06), with bladder neck 56, 80, 57 (p = 0.004), with vestibular 75,67,75 (p = 0.02), with cloaca 64, 67, 65 (p = 0.9), and with imperforate anus without fistula 61,49, 53 (p = 0.12). Patients from 3 to 6 years of age scored 74,72 and 76 (p = 0.37), from 7 to 12: 70,71 and 71 (p = 0.87), and older than twelve: 74,66 e 73 (p = 0.08). Conclusion The scores obtained using Holschneider, Rintala, and Krickenbeck questionnaires are significantly lower with increasing severity of the ARM. For each type of ARM there are some differences in the results obtained using the three questionnaires. In general, Krickenbeck and Peña questionnaires tend to give lower scores in patients with ARMs that have good prognosis, and higher scores for ARMs with poor prognosis. Age is not significantly related to the score obtained. Level of evidence III Type of study Diagnostic study [ABSTRACT FROM AUTHOR]