In patients with Hirschsprung disease (HD), pelvic floor physical therapy (PFPT) is recommended for persistent incontinence or constipation refractory to other treatment, but there are no studies on utilization of PFPT. We aimed to assess clinical and sociodemographic factors associated with successful establishment of PFPT and outcomes following PFPT.We performed a single-institution, retrospective chart review of patients with HD referred to PFPT between 2020 and 2023, involving both exercise and biofeedback. Data were collected on clinical factors, sociodemographics, and symptoms before and after PFPT. Those who "saw PFPT," defined as at least one in-person appointment, were compared to those who did not see PFPT; symptoms were also compared. A p-value of 0.05 was considered significant.There were 83 patients, of which 37 (44.6%) saw PFPT. There were no differences in age, transition zone, prior interventions, or symptoms. Half of the patients who saw PFPT only completed an initial visit; one-fifth completed the series. Most common reason for failure to see PFPT was scheduling issues. Patients who failed to see PFPT had financial stressors (42.5% vs. 16.1%, p = 0.02) and required formal support systems (28.2% vs. 3.3%, p = 0.02). In patients seeing PFPT, incontinence significantly improved (81.1% before vs. 40.5% after, p = 0.001).Although PFPT is recommended in children with HD, those with financial stressors or scheduling issues may have barriers to access. However, those who see PFPT have improved symptoms. This suggests a need for improved accessibility of pediatric PFPT to children with HD, such as integration of PFPT into colorectal clinics.

Hirschsprung disease (HD) contributes significantly to the burden of disease related to conditions amenable to pediatric surgery within the Democratic Republic of Congo (DRC). However, it has received less clinical attention due to limited resources. We aimed at assessing the long-term follow-up outcomes following an innovate resection approach in South Kivu, eastern DRC.

In this case series, the authors elucidate the admissions for HD spanning from 2016 to 2021 at a Pediatric Surgical Center in the DRC. All patients underwent surgical management. Due to the absence of an extemporaneous biopsy, the resection margin was defined by a formula developed by the authors: 5 cm for patients under 5 years, 5 cm plus 1 cm per age from 6-10 years, and 5 cm plus 0.5 cm per age above 10 years. Post-operative mortality and complication rates were equally computed. Descriptive statistics were calculated for continuous variables. Discharge-to-Follow-up data were visualized using time-to-event curves.

Thirty-one patients aged 195 (interquartile range: 18-669) days were included in this study. Thirteen (58.93%) were from mining areas. The patients were surgically managed and follow-up for a year. 6.45% died in the 30-day post-operative period and 19.35% were lost to follow-up. The complaints were recorded from the fourth month following the surgery with most of the patients complained about fecal soiling (58.06%). The overall complaint rate was 80% (95%CI:56.20%-90.69%). The overall rate of incontinence was different compared to those with fecal soiling (p = 0.04). Although lacking statistical significance, the length of stay (LoS) post-surgery predicting an 9% increased incidence of complaints during the follow-up period (adjusted Hazard Ratio: 1.09, 95% CI:0.97-1.23).

HD cause avertable morbidity in South Kivu. These data suggest that the long-term outcome in HD can be mitigated through appropriate surgical management during the operative and intervention aimed at optimizing the LoS.

In this study, we aimed to compare the efficacy of robot-assisted and laparoscopic-assisted modified Soave with that of short muscular cuff anastomosis surgery for classical Hirschsprung disease (HSCR).

Sixty children with HSCR who underwent surgical treatment in our department between January 2021 and December 2023 were retrospectively enrolled. The collected data included operative time, anal dissection time, blood loss, length of hospital stay, postoperative complications, and postoperative defecation control status.

No significant differences were observed in the operative time between the robot and laparoscopic groups (P > 0.05); however, the anal dissection time and intraoperative blood loss in the robot group were significantly lower than those in the laparoscopic group (P < 0.05). Additionally, there was no significant difference in the incidence of enterocolitis and length of hospital stay between the two groups. Significantly more patients presented with anastomotic complications in the laparoscopic group than in the robot group (P < 0.05). The defecation and soiling frequencies in the robot group were significantly lower than those in the laparoscopic group at the follow-up examination (P < 0.05). The postoperative defecation function score in the robot group was better than that in the laparoscopic group (P < 0.05).

Robot-assisted modified Soave with short muscular cuff anastomosis has a shorter anal dissection time, lower incidence of anastomotic complications, and better defecation function in patients with classical Hirschsprung disease.

Objective: To compare the clinical outcomes between total transanal endorectal pull-through (TTEPT) and laparoscopic-assisted transanal endorectal pull-through (LTEPT) in children with rectosigmoid Hirschsprung's disease. Methods: A retrospective study was conducted to compare patients with rectosigmoid Hirschsprung's disease who underwent TTEPT or LTEPT at Beijing Children's Hospital between January 2016 and June 2021. Clinical details were collected from medical records. Patients' parents completed the Krickenbeck questionnaire to evaluate the long-term bowel function (age >4 years) by telephone. A literature search was conducted by using the National Center for Biotechnology Information (NCBI) PubMed database. We combined data from our data with eligible articles and performed a meta-analysis. Result: From our data, there was no difference in the incidence of postoperative complications or long-term bowel function between the patients undergoing TTEPT and LTEPT. A meta-analysis, including five published articles and our data, was performed with a total of 414 patients (n = 236 with TTEPT and n = 178 with LTEPT). For postoperative complications, there were no significant differences between TTEPT and LTEPT for the incidence of HAEC (odds ratio [OR], 0.90; 95% confidence interval [CI], 0.45-1.80; P = .77) or anastomotic leak (OR, 2.52; 95% CI, 0.40-15.80; P = .32). Regarding bowel function outcomes, the incidence of soiling (OR, 1.77; 95% CI, 0.84-3.71; P = .13) and constipation (OR, 1.20; 95% CI, 0.54-2.64; P = .66) were also similar for the two approaches. Conclusion: There was no significant difference in postoperative complications and bowel functional outcomes in patients with rectosigmoid HD undergoing TTEPT or LTEPT. Levels of Evidence: III.

To explore the role of daily trans-anal irrigation (TAI) before pull-through surgery in preventing Hirschsprung disease-associated enterocolitis (HAEC) and improving other clinical outcomes in short-segment Hirschsprung disease (HSCR).

We enrolled the children with short-segment HSCR who received primary pull-through surgery from February 2012 to August 2019 at our hospital. They were divided into two groups. Daily TAI group: daily TAI once diagnosed until undergoing surgery. Non-daily TAI group: anal dilation or glycerine enema, supplemented with TAI if these were noneffective. HAEC, nutritional status at surgery, postoperative rehabilitation, and long-term defecation function were compared.

A total of 191 children were enrolled, 147 in the Daily TAI group and 44 others. 44 pairs of children were successfully matched by 1:1 propensity score matching. In all, 10 (11.36%) of the 88 children developed HAEC, 5 (5/44, 11.36%) in the Daily TAI group and 5 (5/44, 11.36%) in another, and the difference was not statistically significant (χ2 = 0.000, p = 1.000). Besides, there was no significant difference in the nutritional status at surgery, postoperative rehabilitation indicators, and the defecation function.

For children with short-segment HSCR, preoperative daily TAI had no significant advantage over non-daily TAI in preventing HAEC and improving other clinical outcomes.

For children with short-segment Hirschsprung disease, one or more times trans-anal irrigations (TAI) per day before pull-through surgery may not be necessary. Daily TAI had no significant advantage over non-daily TAI in preventing preoperative and postoperative HAEC. Whether to receive daily TAI before surgery did not affect postoperative recovery and long-term defecation function.

Although patients with Hirschsprung disease are mostly affected by imperfect bowel function and fecal control in the long term, they are also predisposed to lower urinary tract symptoms, impaired sexual functions, infertility, psychosocial issues, and decreased quality of life. Rare, but notable comorbidities, which may manifest after childhood, include inflammatory bowel disease and familial medullary thyroid cancer. Despite frequent occurrence of fecal incontinence and constipation, the overall long-term outlook is quite optimistic as social continence with a good quality of life can be achieved by efficient bowel management also in those affected patients, whose self-coping strategies and medical treatment remain insufficient. Bowel dysfunction and other potential long-term complications are best managed by an interdisciplinary specialized approach not overlooking psychosocial issues, which also helps to correctly identify areas requiring continuing input by adult healthcare for young adult patients after transition. Additional research is needed to unravel the pathophysiological mechanisms of the long-term bowel dysfunction to identify novel therapeutic targets for development of more efficient innovative management strategies and thereby improvement of quality of life.

Patients with Hirschsprung disease (HD) can experience soiling after their pull-through. We evaluated the use of antegrade continence enema (ACE) for this patient population and investigated the timing and factors associated with getting the patient clean.

We performed a single-institution retrospective review (from January 2020 to January 2023) of patients with HD and prior pull-through who had persistent soiling, failed laxative treatment or rectal enemas, and were treated with antegrade enemas. The primary outcome was time to become "clean of stool" which was defined as at least one bowel movement per day, no more than one stool accident per week, and no significant stool burden radiographically. Kaplan-Meier survival analysis was performed, and univariate cox proportional hazard regression was used to assess factors associated with time to continence.

Thirty patients who met the criteria underwent ACE creation at a median age of 6.5 y (interquartile range 5.3-9.8) with a median follow-up time of 11.5 mo (interquartile range 5.6-16.5). Most patients became clean by 4 mo (13 of 20, 65%) with similar results at 1-y follow-up (16 of 21, 76%). The median time to becoming clean was 4.3 mo (95% confidence interval: 1.7-15.0. Patients with hypermotility were more likely to continue to soil at 1 y (80% versus 13%, P = 0.01). There were no additional factors significantly associated with time to cleanliness.

ACE is a useful modality for HD patients with soiling. Most became clean of stool in 4 mo. The presence of hypermotility was associated with a higher incidence of persistent soiling at 1 y.

The treatment of Hirschsprung disease (HSCR) is surgical resection of aganglionic bowel and subsequent pull-through of ganglionated bowel. Despite many advances since the initial description of the disease and its surgical management more than half a century ago, there remain considerable controversies regarding the history of the surgical technique, the optimal timing of the primary and multistage pull-through, the best treatment for patients with a delayed diagnosis of HSCR, and the management of post pull-through complications such as soiling due to sphincter incompetence, the presence of a transition zone, and the prevention of enterocolitis. The following review will explore each of these controversies.

Hirschsprung disease (HSCR) is a severe congenital disorder affecting 1:5000 live births. HSCR results from the failure of enteric nervous system (ENS) progenitors to fully colonise the gastrointestinal tract during embryonic development. This leads to aganglionosis in the distal bowel, resulting in disrupted motor activity and impaired peristalsis. Currently, the only viable treatment option is surgical resection of the aganglionic bowel. However, patients frequently suffer debilitating, lifelong symptoms, with multiple surgical procedures often necessary. Hence, alternative treatment options are crucial. An attractive strategy involves the transplantation of ENS progenitors generated from human pluripotent stem cells (hPSCs).

ENS progenitors were generated from hPSCs using an accelerated protocol and characterised, in detail, through a combination of single-cell RNA sequencing, protein expression analysis and calcium imaging. We tested ENS progenitors' capacity to integrate and affect functional responses in HSCR colon, after ex vivo transplantation to organotypically cultured patient-derived colonic tissue, using organ bath contractility.

We found that our protocol consistently gives rise to high yields of a cell population exhibiting transcriptional and functional hallmarks of early ENS progenitors. Following transplantation, hPSC-derived ENS progenitors integrate, migrate and form neurons/glia within explanted human HSCR colon samples. Importantly, the transplanted HSCR tissue displayed significantly increased basal contractile activity and increased responses to electrical stimulation compared with control tissue.

Our findings demonstrate, for the first time, the potential of hPSC-derived ENS progenitors to repopulate and increase functional responses in human HSCR patient colonic tissue.

The main advantage of the laparo-assisted transanal endorectal pull-through technique (LA - TERPT) for Hirschsprung Disease (HD) is the respect to the rectal-anal anatomy. Postoperative complications have been observed recently. The present study aims to determine how often these postoperative complications occur in these patients. From January 2009 to December 2018, a retrospective analysis was conducted on 36 children (25 males) with HD who underwent LA-TERPT. Data were collected on the age of diagnosis and surgery, sex, the presence of other pathologies, and cases of enterocolitis. In all cases, anorectal manometry (ARM) was performed to evaluate the anal tone. The median age at diagnosis was 2 months and the mean age at surgery was 5 months. Nine related pathologies were identified: five cases of Down syndrome, one case of hypertrophic stenosis of the pylorus, atresia of the esophagus, polydactyly, and anorectal malformation. A patient with total colonic aganglionosis was identified through laparoscopic serummuscular biopsies. Enterocolitis was diagnosed in 7 cases before and 6 after surgery. At follow-up, the complications recorded were: 5 cases of constipation (treated with fecal softeners), one case of anal stenosis (patient with anorectal malformation), 16 cases of soiling (treated with enemas) and 1 child with fecal incontinence (treated with a transanal irrigation system). The ARM was performed in all 36 cases and showed normal anal tone, except for one case with anal hypotonia. LA-TERPT is an important surgical technique for HD. According to the literature, soiling is the most main complication after HD surgery, probably due to "pseudo-incontinence" with normal anal sphincter tone.

Hirschsprung's disease (HD) is a rare congenital disease that is characterised by the absence of ganglion cells in the myenteric plexus starting in the distal bowel. This results in distal functional obstruction and may lead to complications like enterocolitis. The treatment is surgical and requires the resection of the aganglionic segment, and the pull-through of normal intestine into the anal opening. However, even after successful surgery, patients may continue to have symptoms.

Discuss current surgical techniques and management strategies for patients with postoperative symptoms after surgical correction of Hirschsprung's disease.

A review of the literature was done through PubMed, with a focus on clinical management and approach.

We describe the clinical problems that can occur after surgical correction. These include obstructive symptoms, enterocolitis, or faecal incontinence. A systematic approach for the evaluation of these patients includes the exclusion of anatomic, inflammatory, behavioural or motility related factors. Depending on the severity of the symptoms, the evaluation includes examination under anaesthesia, the performance of contrast studies, endoscopic studies, measurement of anal sphincter function and colonic motility studies. The treatment is focused towards addressing the different pathophysiological mechanisms, and may include medical management, botulinum toxin to the anal sphincter or rarely redo-operation.

Patients with Hirschsprung's disease need to have surgical correction, and their postoperative long-term management is complex given a variety of associated problems that can occur after surgery. A systematic evaluation is necessary to provide appropriate therapy.

Abnormal motility of the residual colon has been reported in post-pull-through Hirschsprung disease (PT-HSCR) patients with persistent defecation problems. We reviewed the role of colonic manometry (CM) in the management of defecation disorders in these patients.

We retrospectively reviewed the medical record of PT-HSCR children who underwent CM for persistent symptoms of abnormal defecation. We reviewed their clinical course and its relation to CM findings.

Thirty PT-HSCR patients underwent CM, of which five were diagnosed with transition zone pull-through and were excluded. Of the remaining 25 patients, 16 had colonic dysmotility, 8 had normal CM, and one had colonic hypermotility. In patients with dysmotility, five responded to ongoing medical management, three required surgical intervention (ileostomy), three remained symptomatic with medical management but not yet received surgical intervention, and five were lost to follow-up. In patients with normal CM, four responded to ongoing medical therapy, two required additional surgery (antegrade enema procedure), and two were lost to follow-up. The patient with hypermotility improved with adding loperamide.

Colonic dysmotility can occur in PT-HSCR patients with persistent defecation problems. CM was helpful in delineating the degree of colonic neuromuscular dysfunction. CM results were used in conjunction with other clinical data to determine optimal management. Our findings support that medical management should first be optimized before consideration of colonic manometry and surgical interventions.

In recent decades, the survival of children with congenital anomalies and paediatric cancer has improved dramatically such that there has been a steady shift towards understanding their lifelong health outcomes. Paediatric surgeons will actively manage such conditions in childhood and adolescence, however, adult surgeons must later care for these 'grown-ups' in adulthood. This article aims to highlight some of those rare disorders encountered by paediatric surgeons requiring long-term follow-up, their management in childhood and their survivorship impact, in order that the adult specialist may be better equipped with skills and knowledge to manage these patients into adulthood.

A comprehensive literature review was performed to identify relevant publications. Research studies, review articles and guidelines were sought, focusing on the paediatric management and long-term outcomes of surgical conditions of childhood. The article has been written for adult surgeon readership.

This article describes the aforementioned conditions, their management in childhood and their lifelong implications, including: oesophageal atresia, tracheo-oesophageal fistula, malrotation, short bowel syndrome, duodenal atresia, gastroschisis, exomphalos, choledochal malformations, biliary atresia, Hirschsprung disease, anorectal malformations, congenital diaphragmatic hernia, congenital lung lesions and paediatric cancer.

The increasing survivorship of children affected by surgical conditions will translate into a growing population of adults with lifelong conditions and specialist healthcare needs. The importance of transition from childhood to adulthood is becoming realized. It is hoped that this timely review will enthuse the readership to offer care for such vulnerable patients, and to collaborate with paediatric surgeons in providing successful and seamless transitional care.

Cell replacement in aganglionic intestines is a promising, yet merely experimental tool for the therapy of congenital dysganglionosis of the enteric nervous system like Hirschsprung disease. While the injection of single cells or neurospheres to a defined and very restricted location is trivial, the translation to the clinical application, where large aganglionic or hypoganglionic areas need to be colonized (hundreds of square centimetres), afford a homogeneous distribution of multiple neurospheres all over the affected tissue areas. Reaching the entire aganglionic area in vivo is critical for the restoration of peristaltic function. The latter mainly depends on an intact nervous system that extends throughout the organ. Intra-arterial injection is a common method in cell therapy and may be the key to delivering cells or neurospheres into the capillary bed of the colon with area-wide distribution. We describe an experimental method for monitoring the distribution of a defined number of neurospheres into porcine recta ex vivo, immediately after intra-arterial injection. We designed this method to localize grafting sites of single neurospheres in precise biopsies which can further be examined in explant cultures. The isolated perfused porcine rectum allowed us to continuously monitor the perfusion pressure. A blockage of too many capillaries would lead to an ischaemic situation and an increase of perfusion pressure. Since we could demonstrate that the area-wide delivery of neurospheres did not alter the overall vascular resistance, we showed that the delivery does not significantly impair the local circulation.

Applying evidence-based guidelines can enhance the quality of patient care. While robust guideline development methodology ensures credibility and validity, methodological variations can impact guideline quality. Besides methodological rigor, effective implementation is crucial for achieving improved health outcomes. This review provides an overview of recent literature pertaining to the development and implementation of guidelines in pediatric surgery. Literature was reviewed to provide an overview of sound guideline development methodologies and approaches to promote effective guideline implementation. Challenges specific to pediatric surgery were highlighted. A search was performed to identify published guidelines relevant to pediatric surgery from 2018 to June 2023, and their quality was collectively appraised using the AGREE II instrument. High-quality guideline development can be promoted by using methodologically sound tools such as the Guidelines 2.0 checklist, the GRADE system, and the AGREE II instrument. While implementation can be promoted during guideline development and post-publication, its effectiveness may be influenced by various factors. Challenges pertinent to pediatric surgery, such as limited evidence and difficulties with outcome selection and heterogeneity, may impact guideline quality and effective implementation. Fifteen guidelines were identified and collectively appraised as suboptimal, with a mean overall AGREE II score of 58%, with applicability being the lowest scoring domain. There are identified challenges and barriers to the development and effective implementation of high-quality guidelines in pediatric surgery. It is valuable to prioritize the identification of adapted, innovative methodological strategies and the use of implementation science to understand and achieve effective guideline implementation.

The aim of this study was to develop a nomogram for predicting the probability of postoperative soiling in patients aged greater than 1 year operated for Hirschsprung disease (HSCR).

The authors retrospectively analyzed HSCR patients with surgical therapy over 1 year of age from January 2000 and December 2019 at our department. Eligible patients were randomly categorized into the training and validation set at a ratio of 7:3. By integrating the least absolute shrinkage and selection operator [LASSO] and multivariable logistic regression analysis, crucial variables were determined for establishment of the nomogram. And, the performance of nomogram was evaluated by C-index, area under the receiver operating characteristic curve, calibration curves, and decision curve analysis. Meanwhile, a validation set was used to further assess the model.

This study enrolled 601 cases, and 97 patients suffered from soiling. Three risk factors, including surgical history, length of removed bowel, and surgical procedures were identified as predictive factors for soiling occurrence. The C-index was 0.871 (95% CI: 0.821-0.921) in the training set and 0.878 (95% CI: 0.811-0.945) in the validation set, respectively. And, the AUC was found to be 0.896 (95% CI: 0.855-0.929) in the training set and 0.866 (95% CI: 0.767-0.920) in the validation set. Additionally, the calibration curves displayed a favorable agreement between the nomogram model and actual observations. The decision curve analysis revealed that employing the nomogram to predict the risk of soiling occurrence would be advantageous if the threshold was between 1 and 73% in the training set and 3-69% in the validation set.

This study represents the first efforts to develop and validate a model capable of predicting the postoperative risk of soiling in patients aged greater than 1 year operated for HSCR. This model may assist clinicians in determining the individual risk of soiling subsequent to HSCR surgery, aiding in personalized patient care and management.

Hirschsprung disease (HSCR) is a rare congenital intestinal disease that occurs in 1 in 5,000 live births. HSCR is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. Most patients present during the neonatal period with the first meconium passage delayed beyond 24 h, abdominal distension and vomiting. Syndromes associated with HSCR include trisomy 21, Mowat-Wilson syndrome, congenital central hypoventilation syndrome, Shah-Waardenburg syndrome and cartilage-hair hypoplasia. Multiple putative genes are involved in familial and isolated HSCR, of which the most common are the RET proto-oncogene and EDNRB. Diagnosis consists of visualization of a transition zone on contrast enema and confirmation via rectal biopsy. HSCR is typically managed by surgical removal of the aganglionic bowel and reconstruction of the intestinal tract by connecting the normally innervated bowel down to the anus while preserving normal sphincter function. Several procedures, namely Swenson, Soave and Duhamel procedures, can be undertaken and may include a laparoscopically assisted approach. Short-term and long-term comorbidities include persistent obstructive symptoms, enterocolitis and soiling. Continued research and innovation to better understand disease mechanisms holds promise for developing novel techniques for diagnosis and therapy, and improving outcomes in patients.

After an initial pull-though, patients with Hirschsprung disease (HD) can present with obstructive symptoms, Hirschsprung-associated enterocolitis (HAEC), failure to thrive, or fecal soiling. This current review focuses on algorithms for evaluation and treatment in children with HD as a part of a manuscript series on updates in bowel management. In constipated patients, anatomic causes of obstruction should be excluded. Once anatomy is confirmed to be normal, laxatives, fiber, osmotic laxatives, or mechanical management can be utilized. Botulinum toxin injections are performed in all patients with HD before age five because of the nonrelaxing sphincters that they learn to overcome with increased age. Children with a patulous anus due to iatrogenic damage of the anal sphincters are offered sphincter reconstruction. Hypermotility is managed with antidiarrheals and small-volume enemas. Family education is crucial for the early detection of HAEC and for performing at-home rectal irrigations.

During "bowel management week," abdominal radiographs are used to monitor the amount and location of stool. A radiologist familiar with the treatment plan can provide an improved interpretation. The goal of this paper is to standardize the radiological reports during a bowel management week.

We saw 744 patients during bowel management week from May 2016 until March 2023. Diagnosis included: anorectal malformation (397), idiopathic constipation (180), Hirschsprung disease (89), and spina bifida (78). Laxatives were the treatment for 51% of patients, and 49% received enemas. Characteristic radiographs were selected for each treatment group for a proposed reading standardization.

When the stool is visualized, it is crucial to report its location. Having a contrast enema helps with the correct interpretation of the colonic anatomy. It is also essential to always compare the amount of stool with the radiograph from the previous day to determine if there is an increase or decrease in stool. Examples of radiographs are shown to guide the use of the preferred proposed terminology.

Providing information regarding which treatment modality the patient is receiving and stating that a patient is on a bowel management week treatment is crucial for the radiologist to provide adequate interpretation. The radiologist must be familiar with the treatment goals and purpose of the daily radiograph.

Children with Hirschsprung disease have postoperative long-term sequelae in defecation that contribute to morbidity and mortality and significantly impact their quality of life. Pediatric patients experience ongoing long-term defecation concerns, which can include fecal incontinence (FI) and postoperative obstructive symptoms, such as constipation and Hirschsprung-associated enterocolitis. The American Pediatric Surgical Association has developed guidelines for management of these postoperative obstructive symptoms and FI. However, the evaluation and management of patients with postoperative defecation problems varies among different pediatric gastroenterology centers. This position paper from the Neurogastroenterology & Motility Committee of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition reviews the current evidence and provides suggestions for the evaluation and management of postoperative patients with Hirschsprung disease who present with persistent defecation problems.

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.

Bowel dysfunction has been proven to be the most typical complication of long-segment Hirschsprung disease (LSHD). Additionally, bowel dysfunction in preschool and early childhood should be properly assessed, because it may result in persistent bowel dysfunction, social issues, and depression in adolescence and adulthood. This study evaluated bowel function during preschool and early childhood in patients with LSHD. A cross-sectional bowel function score (BFS) questionnaire survey was conducted on 270 infants with short-segment Hirschsprung disease (SSHD) and LSHD who underwent pull-through (PT) between January 2014 and December 2019 at the Children's Hospital of Nanjing Medical University. One hundred ninety patients who consented to the survey were asked to complete the questionnaire and were divided into two groups: the LSHD group (n = 42) and the SSHD group (n = 148). Bowel function outcomes were assessed by using a questionnaire. The total BFS score in the LSHD group was significantly lower than that in the SSHD group (15.0 [14.0, 17.0] vs. 18.0 [16.0, 19.0], p < 0.05) and did not improve with age (p > 0.05). Independent BFS items with lower scores were discovered in LSHD than in SSHD, including the capacity to hold back defecation, feeling/reporting the urge to defecate, frequency, soiling, and accidents (all p < 0.05). After subgroup analysis of follow-up age (3-5 years, 5 ~ 7 years, and > 7 years), there was no difference between the score of ability to hold back defecation over 7 years old and feeling/reporting the urge to defecate over 5 years old (all p > 0.05). The frequency and soiling scores were lower in the LSHD group than in the SSHD group over 5 years of age (all p < 0.05). Fortunately, there was no difference in constipation scores and social problems between the LSHD and SSHD groups in every sub-follow-up group (all p > 0.05).  Conclusion: Overall, bowel function at preschool and early childhood age was poorer in LSHD than in SSHD and did not improve with age. However, the ability to hold back defecation and feeling/reporting the urge to defecate with age in LSHD may be similar to those in SSHD. Simultaneously, the frequency, soiling, and number of accidents were consistently worse in the LSHD group.  Trial registration: This study was retrospectively registered in the ClinicalTrials database.gov (NCT05461924) in August 2022. What is Known: • Bowel function in SSHD is satisfactory or near-normal, and the general consensus is that the longer the aganglionic segment, the worse the bowel function after surgery. • Bowel functional outcomes of LSHD were not defined, and the limited reports of bowel functional outcomes compared with SSHD were extensively varied. Bowel dysfunction at preschool and early childhood may lead to persistent bowel dysfunction, social problems, and depression in adolescence or adulthood. What is New: • Bowel function in preschool and early childhood in patients with LSHD has not been defined and should be thoroughly assessed in a larger group of patients with precise definitions of incontinence or soiling. • The present study was primarily designed to evaluate bowel function at preschool and early childhood age in LSHD compared with SSHD in a relatively large number of HD cases using BFS.

Robotic colorectal surgery allows adult and pediatric surgeons to overcome the technical limitations of laparoscopic surgery. It also provides improved ergonomics in the field of surgery. Robotic surgery has several advantages in colorectal operations that require complex minimally invasive skills including anorectal malformations, Hirschsprung disease, and inflammatory bowel disease. In this section, we discuss the key aspects of colorectal surgery where robotic instrumentation seems ideal.

Some guidelines for management of Hirschsprung's disease (HSCR, HD) have been developed, but their quality is vague. This study will systematically assess the quality of guidelines and analyze the key recommendations and the best evidence for guidelines.

Applicable guidelines were retrieved using a systematic search of databases. The Appraisal of Guidelines for Research and Evaluation II (AGREE II) tool was used to assess the quality of the guidelines. Then, the recommendations and evidence for the included guidelines were extracted and compared.

A total of nine guidelines were included in this study, and only one had an overall standardized score of more than 60%, indicating that it is worthy of recommendation. The problems identified included ambiguous and low-quality evidence; obvious distributional heterogeneity among the recommendations; a lack of in-depth discussion on the interpretation of staging, diagnostic methods, conservative treatment, and surgical staging of disease.

The quality of guidelines varies widely, and there is a lack of high-quality professional opinions and supporting evidence for the main recommendations. At present, only comprehensive guidelines can be considered high-quality and there is still room for improvement.

After operative intervention for Hirschsprung disease (HD) a child should thrive, be fecally continent, and avoid recurrent episodes of abdominal distention and enterocolitis. This is unfortunately not the case for a significant number of patients who struggle following their pull-through procedure. Many clinicians are puzzled by these outcomes as they can occur in patients who they believe have had a technically satisfactory described operation. This review presents an organized approach to the evaluation and treatment of the post HD pull-through patient who is not doing well. Patients with HD who have problems after their initial operation can have: (1) fecal incontinence, (2) obstructive symptoms, and (3) recurrent episodes of enterocolitis (a more severe subset of obstructive symptoms). After employing a systematic diagnostic approach, successful treatments can be implemented in almost every case. Patients may need medical management (behavioral interventions, dietary changes, laxatives, or mechanical emptying of the colon), a reoperation when a specific anatomic or pathologic cause is identified, or botulinum toxin when non-relaxing sphincters are the cause of the obstructive symptoms or recurrent enterocolitis.

Hirschsprung disease (HD) is a complex surgical and medical problem that appears to have varied health and social outcomes with the age and neurodevelopmental state of patients. In general, long-term outcomes are thought to be good for the majority of patients despite recognized problems with constipation and/or fecal incontinence. However, there are no universally accepted pathways regarding post-operative bowel management programs nor clearly defined follow-up pathways making the current outcome measures difficult to interpret. Further, other factors that may influence outcome including age at the time of procedure and procedure type continue to lack consensus. Improved support of children in resource limited environments and during periods of transition into the adult medical care environment are needed to improve outcome.  Recent proliferation of multidisciplinary care teams and consortia may help to better understand outcomes and address current knowledge gaps. Continuing these collaborations will be imperative to continuing improvements in care which may ultimately impact outcome.

Functional and motility gastrointestinal disorders are the most common complaints to the pediatric gastroenterologist. Disorders affecting the small intestine carry a significant morbidity and mortality due to the severe limitation of therapeutic interventions available and the complications associated with such interventions. Congenital colorectal disorders are rare but also carry significant morbidity and poor quality of life plus the social stigma associated with its complications. In this review, we summarize the clinical presentation, diagnostic evaluations, and the therapeutic interventions available for the most common and severe gastrointestinal functional and motility disorders of the small bowel, colon, and anorectum.

Ideally, after operative intervention, a child born with Hirschsprung disease (HD) should thrive, achieve fecal continence, and avoid recurrent episodes of abdominal distention and enterocolitis. However, a significant number of patients continue to struggle following their pull-through procedure. The purpose of this review is to present an organized and practical approach to the evaluation and management of the symptomatic patient post pull-through operation for HD.

Children diagnosed with HD who are not doing well after their initial operation can be categorized in three distinct groups: (1) those that have fecal incontinence, (2) those with obstructive symptoms, and (3) those with recurrent episodes of enterocolitis. It is important to have a systematic diagnostic approach for these patients based on a comprehensive protocol. All three of these patient groups can be treated with a combination of either medical management, reoperation when a specific anatomic or pathologic etiology is identified, or botulinum toxin for non-relaxing sphincters contributing to the obstructive symptoms or recurrent enterocolitis. For patients not doing well after their initial pull-through, a systematic workup should be employed to determine the etiology. Once identified, a multidisciplinary and organized approach to management of the symptomatic patients can alleviate most post pull-through symptoms.

Hirschsprung disease (HSCR) and Paediatric Intestinal Pseudo-obstruction (PIPO) comprise two of the most recognized and severe disorders of gastrointestinal (GI) motility. HSCR is a developmental disorder of the enteric nervous system invariably affecting the large intestine, whereas the majority of PIPO conditions represent congenital disorders of one or more components of the neuromusculature and more diffusely affect the GI tract. Histopathology is deemed the gold standard for the diagnosis of HSCR and, arguably, of PIPO, but, other diagnostic modalities such as manometric and genetic studies have seen recent advances that may increase their utility. Especially for PIPO, management is multidisciplinary and best performed in specialist referral centres. Surgery remains the only viable treatment for HSCR and appears essential to optimize and sustain feeding and viability of intestinal function in PIPO patients. Novel therapies such as neural stem cell transplants show promise for the future.

Hirschsprung's disease is a congenital disorder requiring surgery. Most children operated on for Hirschsprung's disease experience postoperative bowel dysmotility. Although various food is known to influence bowel motility, evidence of diet's role and dietary guidelines in treatment of bowel dysfunction in Hirschsprung's disease is lacking. The aim was to explore parental experiences of dietary effects on bowel function in children with Hirschsprung's disease.

A qualitative study including three focus groups with ten parents of children with Hirschsprung's disease at a national Hirschsprung's disease centre. Data were analysed through content analysis.

Parents emphasised diet as a strong influencer on their child's bowel function in Hirschsprung's disease. They expressed great concerns about their responsibility and strived hard to explore and adjust dietary habits to control the child's bowel function. Families' daily and social lives were influenced by the child's diet and bowel function. The parents desired dietary support and guidelines to improve their confidence in self-treatment of Hirschsprung's disease.

Dietary habits play a key role in parental self-treatment of bowel function in their children with Hirschsprung's disease. Dietary guidelines for patients with Hirschsprung's disease are anticipated.

Hirschsprung's disease (HD) is a congenital disorder, characterized by aganglionosis in the distal part of the gastrointestinal tract. Despite complete surgical resection of the aganglionic segment, both constipation and fecal incontinence persist in a considerable number of patients with limited treatment options. There is growing evidence for structural abnormalities in the ganglionic bowel proximal to the aganglionosis in both humans and animals with HD, which may play a role in persistent bowel dysfunction. These abnormalities include: (1) Histopathological abnormalities of enteric neural cells; (2) Imbalanced expression of neurotransmitters and neuroproteins; (3) Abnormal expression of enteric pacemaker cells; (4) Abnormalities of smooth muscle cells; and (5) Abnormalities within the extracellular matrix. Hence, a better understanding of these previously unrecognized neuropathological abnormalities may improve follow-up and treatment in patients with HD suffering from persistent bowel dysfunction following surgical correction. In the long term, further combination of clinical and neuropathological data will hopefully enable a translational step towards more individual treatment for HD.

The majority of children who undergo operative management for Hirschsprung disease have favorable results. A subset of patients, however, have long-term dysfunctional stooling, characterized by either frequent soiling or obstructive symptoms. The evaluation and management of a child with poor function after pull-through for Hirschsprung disease should be conducted by an experienced multidisciplinary team. A systematic workup is focused on detecting pathologic and anatomic causes of pull-through dysfunction. This includes an exam under anesthesia, pathologic confirmation including a repeat biopsy, and a contrast enema, with additional studies depending on the suspected etiology. Obstructive symptoms may be due to technique-specific types of mechanical obstruction, histopathologic obstruction, or dysmotility-each of which may benefit from reoperative surgery. The causes of soiling symptoms include loss of the dentate line and damage to the anal sphincter, which generally do not benefit from revision of the pull-through, and pseudo-incontinence, which may reveal underlying obstruction. A thorough understanding of the types of complications associated with various pull-through techniques aids in the evaluation of a child with postoperative dysfunction. Treatment is specifically tailored to the patient, guided by the etiology of the patient's symptoms, with options ranging from bowel management to redo pull-through procedure. This review details the workup and management of patients with complications after pull-through, with a focus on the perioperative management and technical considerations for those who require reoperation.

Hirschsprung disease is a relatively uncommon disorder of the developing gastrointestinal tract that requires surgical intervention to restore long-term function. While readmission for Hirschsprung-related complications is a known concern in these patients, we sought to identify patient-level factors associated with a prolonged hospital stay, increased costs at the time of a pull-through operation, as well as the risk for all-cause inpatient readmission after surgery. We hypothesized that higher level of care requirement during the operative stay, age at operation, and length of stay (LOS) would portend increased readmissions and disease-related complications such as Hirschsprung-associated enterocolitis.

Data was obtained from the Pediatric Health Information System database on all Hirschprung patients who underwent a pull-through operation between 2004 and 2019. Regression analyses were performed on this cohort of 3345 patients. Multivariable regression models were utilized to analyze the key outcome variables of postoperative LOS and adjusted charges.

Post-operative LOS was significantly increased by the presence of a surgical complication, congenital/genetic defect, or neurologic/neuromuscular defect. Increased LOS was also seen in Black patients. The cost of pull-through operations was significantly higher in patients admitted to the NICU and ICU during index hospitalization, with a cost increase of approximately $75,000 and $57,000 respectively. Presence of a surgical complication, comorbid congenital/genetic defect, and need for mechanical ventilation were associated with higher odds of inpatient readmission.

The management of patients with Hirschsprung disease is longitudinal and complex. Identification of key patient metrics can aid clinicians in developing targeted care and education strategies to minimize readmission and excessive hospital charges.

Soiling and fecal incontinence are troubling complications which can occur after a pull-through for Hirschsprung disease. They can usually be improved with proper medical management, but in some cases are the result of an anatomic defect related to overstretching of the sphincters and/or damage of the anal canal. For such patients the treatment of this true fecal incontinence is limited to a structured bowel management program with ante- or retrograde enemas to achieve social continence. Herein we report two such patients with overstretched sphincters and loss of the dentate line after an initial pull-through and describe a sphincter tightening technique to improve bowel control.

Purpose: Despite the fact that the laparoscopic-assisted endorectal pull-through (LAEPT) for Hirschsprung's disease (HD) was introduced over 20 years ago, published outcomes in older children and adolescent are lacking. To address this, we studied the long-term results of LATEP for rectosigmoid HD in patients above 5 years of age. Materials and Methods: A retrospective review was conducted on all nonsyndromic patients above 5 years of age and who underwent one-stage LAEPT for rectosigmoid HD between January 2002 and December 2017. Late Hirschsprung-associated enterocolitis (HAEC) was defined as HAEC occurring 1 year after the pull-through operation. Postoperative bowel function was assessed using the Krickenbeck classification. Results: Forty-one patients (37 males and 4 females) were included in this study with a median follow-up of 9.0 years. The median age at surgery was 55 days. Two patients had anastomotic leakage. No patient had late HAEC, rectal prolapse, anastomotic stricture, or intestinal obstruction. According to the Krickenbeck classification, all patients had voluntary bowel movements without constipation. Overall, 65.8% of patients had no soiling. However, on subgroup analysis, only 45.4% of patients younger than 11 years of age had no soiling compared with 89.5% in patients at or older than 11 years of age (P = .003). Conclusions: Our results showed that LAEPT for rectosigmoid HD was a safe procedure. Nearly 90% of patients had normal bowel function by puberty. Further studies are needed to address the problem of soiling in patients younger than 11 years of age.