Hirschsprung-associated enterocolitis (HAEC) is a life-threatening complication of Hirschsprung disease (HD), with significant morbidity and mortality rates. Early diagnosis and treatment are critical, and caregivers play a key role in detecting HAEC, performing colonic irrigations, and seeking timely medical care. This study validates an HAEC action plan designed to assist caregivers in managing HAEC at home.

A caregiver-focused HAEC Action Plan was developed following the National Institute of Health (NIH) 5-step "Clear & Simple" approach. It included patient-specific information, symptom pictographs, and a stepwise HAEC management protocol. Previously validated pictograms were used with the permission of the original institution. Additionally, 6 unvalidated pictograms were used. Readability was assessed using multiple formulas. Caregivers and the community evaluated the newly used pictograms' validity and action plan's comprehension, quality, and usefulness using the Consumer Information Rating Form (CIRF). Understandability and actionability were evaluated by librarians using the Patient Education Materials Assessment Tool. Clinicians validated its suitability using the modified Suitability Assessment of Materials (SAM) instrument.

The 6 new pictograms were validated. Readability was acceptably below the 6th-grade level. Overall CIRF score reached 91.5 % (superior). Understandability, actionability, and suitability were rated at 91 %, 91 %, and 82.5 %, respectively. Suggested improvements included using clearer pictograms, unit adjustments, additional details on irrigation duration, improved text visibility, and translation of the tool into other languages.

The HAEC Action Plan is a clear, user-friendly tool for guiding caregivers in the early recognition and management of HAEC. Once employed in clinical practice, it has the potential to reduce HAEC-associated morbidity and mortality in children with HD.

N/A.

Children with total colonic Hirschsprung disease (TCHD) are a unique group of patients with pre- and postoperative management challenges. This review provides a rational, expert-based approach to diagnosing and managing TCHD.

The guidelines were developed by the Hirschsprung Disease Interest Group members established by the American Pediatric Surgical Association (APSA) Board of Governors. Group discussions, literature review, and expert consensus were used to summarize the current knowledge regarding diagnosis, staged approach, the timing of pull-through, and pre-and postoperative management in children with TCHD.

This paper presents recommendations for managing TCHD before and after reconstruction, including diagnostic criteria, surgical approaches, bowel management, diet, antibiotic prophylaxis, colonic irrigations, and post-surgical considerations.

A clear understanding of the unique challenges posed by TCHD and consensus on its treatment are lacking in the literature. This review standardizes this patient group's pre- and postoperative management.

V.

This study aimed to compare the differences in postoperative complications and long-term bowel function outcomes between patients with rectosigmoid Hirschsprung disease (HD) who underwent transanal endorectal pull-through (TEPT) beyond infancy (age> 1 year of age) and those during infancy (≤ 1 year of age).

All patients with rectosigmoid HD at Beijing Children's Hospital between January 2011 and December 2020 were analyzed retrospectively. They were divided into two groups based on age at TEPT: group A was defined as patients who performed TEPT beyond infancy (age>1 year of age), and group B as patients who performed TEPT during infancy (age ≤ 1 year of age). Clinical details were collected from medical records. Bowel function outcomes were assessed by the Rintala questionnaire (age ≥ 4 years).

A total of 339 patients were included: 216 (63.7%) who operated with TEPT beyond infancy (group A) and 123 (36.3%) during infancy (group B). Regarding postoperative complications, all patients suffering anastomosis leakage following TEPT (7/216, 3.2%) occurred in group A, and the rate of anastomosis leakage in group A was significantly higher than in group B (3.2% vs. 0.0%, p = 0.044). 228 patients (228/327, 69.7%) completed the Rintala questionnaire. There was no significant difference in long-term bowel function outcomes between the two groups.

Compared with patients who performed TEPT during infancy, those beyond infancy are more likely to suffer anastomosis leakage. however, the long-term bowel function outcomes seem comparable.

A retrospective single-center study.

III.

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.

Hirschsprung's disease (HSCR) is a congenital disorder characterized by failure of the neural crest cells to migrate and populate the distal bowel during gestation affecting different lengths of intestine leading to a distal functional obstruction. Surgical treatment is needed to correct HSCR once the diagnosis is confirmed by demonstrating the absence of ganglion cells or aganglionosis of the affected bowel segment. Hirschsprung's disease associated enterocolitis (HAEC) is an inflammatory complication associated with HSCR that can present either in the pre- or postoperative period and associated with increased morbidity and mortality. The pathogenesis of HAEC remains poorly understood, but intestinal dysmotility, dysbiosis and impaired mucosal defense and intestinal barrier function appear to play a significant role. There is no clear definition for HAEC, but the diagnosis is primarily clinical, and treatment is guided based on severity. Here, we aim to provide a comprehensive review of the clinical presentation, etiology, pathophysiology, and current therapeutic options for HAEC.

Hirschsprung's-associated enterocolitis (HAEC) is a common post-operative problem for patients with Hirschsprung disease (HSCR). However, treatment strategies remain variable among providers, institutions, and even nations. The purpose of this study was to identify differences in treatment patterns for HAEC.

A questionnaire was distributed to members of the International Pediatric Endoscopic Group (IPEG) community that focused on HSCR and HAEC management strategies. Questionnaire responses were collected via the Research Electronic Data Capture (RedCap).

178 responses were obtained: 30% from North America, 20% South America, 20% Europe, 26% Asia, and 4% from Australia. 37% had a dedicated pediatric colorectal center. After diagnosis, 53% send patients home with irrigations, while 29% perform a primary PT before discharge; the type of PT varied between Soave (50%), Swenson (25%) and Duhamel (13%). Only 29 respondents (17%) stated their institution had guidelines for HAEC management; however, inpatient treatments were fairly consistent: 95% performed rectal irrigations, 93% obtained an abdominal radiograph, and 72% held feeds; 55% taught families irrigations before discharge. Utilization of Botulinum (BT) injections was mixed: 36% never utilized BT injections, 33% only used BT if irrigations were not tolerated, and 16% only injected BT for recurrent episodes. Preventative HAEC measures were also varied and included anal dilations (44%), prophylactic antibiotics (34%), probiotics (29%), and routine home irrigations (22%).

There is wide variation of care in managing enterocolitis episodes in patients with Hirschsprung disease. Further research leading to consensus guidelines and standardization practices can help improve the care for these patients.

V TYPE OF STUDY: Treatment study/ survey.

Patients with Hirschsprung disease (HD) can struggle with persistent obstructive symptoms even after a successful pull-through. These symptoms lead to stasis of stool and can result in Hirschsprung associated enterocolitis (HAEC). Recurrent episodes of HAEC warrant further workup; if there are no signs of mechanical obstruction or an aganglionic pull-through, the use of botulinum toxin injections to the internal anal sphincter has been utilized to relieve these symptoms. In this review, we describe the variations in botulinum toxin injection use and describe ongoing studies to prevent obstructive symptoms and Hirschsprung-associated enterocolitis (HAEC). Botulinum toxin injection utilization has been described for obstructive symptoms after HD pull-through, in the setting of active HAEC, and has been proposed to be part of the treatment algorithm for prevention of HAEC after pull-through. Dosing utilized for the injections, along with the complications, are also described. Prospective, multi-institutional trials are needed to identify the effectiveness of botulinum toxin injections in the outpatient/prophylactic setting as current data suggest some benefits in preventing future obstructive symptoms; however, other studies have conflicting results.

Stasis from obstruction at the level of the internal anal sphincter (IAS) can lead to Hirschsprung-associated enterocolitis (HAEC) and may be improved by botulinum toxin (BT) injections. Our aim was to determine if BT injection during HAEC episodes decreased the number of recurrent HAEC episodes and/or increased the interval between readmissions.

A retrospective review was performed of patients admitted for HAEC from January 2010 to December 2019. Demographics and outcomes of patients who received BT were compared to patients who did not receive BT during their hospital stay.

A total of 120 episodes of HAEC occurred in 40 patients; 30 patients (75%) were male, 7 (18%) had Trisomy 21 and 10 (25%) had long-segment disease. On multivariate analysis, patients who received BT during their inpatient HAEC episode had a longer median time between readmissions (p = 0.04) and trending toward an association with fewer readmissions prior to a follow-up clinic visit (p = 0.08).

The use of BT in HD patients hospitalized for HAEC is associated with an increased time between recurrent HAEC episodes and trended toward fewer recurrent episodes. The use of BT should be considered in the management of patients admitted with HAEC.

Botulinum toxin (BT) injections may play a role in preventing Hirschsprung associated enterocolitis (HAEC) episodes related to internal anal sphincter (IAS dysfunction). Our aim was to determine the association of outpatient BT injections for early obstructive symptoms on the development of HAEC.

A retrospective review of children who underwent definitive surgery for Hirschsprung disease (HSCR) from July 2010 - July 2020 was performed. The timing from pull-through to first HAEC episode and to first BT injection was recorded. Primary analysis focused on the rate of HAEC episodes and timing between episodes in patients who did and did not receive BT injections.

Eighty patients were included. Sixty patients (75%) were male, 15 (19%) were diagnosed with trisomy 21, and 58 (72.5%) had short-segment disease. The median time to pull-through was 150 days (IQR 16, 132). Eight patients (10%) had neither an episode of HAEC or BT injections and were not included in further analysis. Forty-six patients (64%) experienced at least one episode of HAEC, while 64 patients (89%) had at least one outpatient BT injection. Compared to patients who never received BT injections (n = 9) and those who developed HAEC prior to BT injections (n = 35), significantly fewer patients who received BT injections first (n = 28) developed enterocolitis (P < 0.001), with no patient developing more than one HAEC episode.

Outpatient BT is associated with decreased episodes of HAEC and increased interval between HAEC episodes requiring inpatient treatment. Scheduling outpatient BT injections to manage obstructive symptoms may be beneficial after pull-through for HSCR.

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.