Hirschsprung-associated Enterocolitis Research Articles

Considering Hirschsprung-associated enterocolitis in infants dehydration: Misdiagnosis risk and management of an underestimated lethal disease.

Considering Hirschsprung-associated enterocolitis in infants dehydration: Misdiagnosis risk and management of an underestimated lethal disease.

Impact of Colorectal Nurse Specialist supervised parental administration of rectal washouts on Hirschsprung's disease outcomes: a retrospective review.

To highlight the utility of Colorectal Nurse Specialist (CNS) supervised parental administration of rectal washouts in the management of Hirschsprung's disease (HD). Retrospective case note review of HD patients treated at a tertiary children's hospital in United Kingdom from January 2011 to December 2022. Data collected included demographics, complications, enterocolitis, obstructive symptoms and stomas. Primary pull-through (PT) is done 8-12weeks after birth. Parental expertise in performing rectal washouts at home is ensured by our CNS team before and after PT. PT was completed in 69 of 74 HD patients. Rectal washouts were attempted on 63 patients before PT. Failure of rectal washout efficacy necessitated a stoma in four patients (6.4%). Of the 65 patients who had PT and stoma closed, three (4.5%) required a further stoma over a mean follow-up period of 57 months (Range 7-144 months). Two of these had intractable diarrhoea due to Total Colonic Aganglionosis (TCA). One patient (1.5%) had unmanageable obstructive symptoms requiring re-diversion. Hirschsprung-associated enterocolitis (HAEC) requiring hospital admission occurred in 14 patients (21%). Our stoma rates are lower compared to recent UK data. This could potentially be due to emphasis on parental ability to perform effective rectal washouts at home under CNS supervision.

Clinical characteristics and influence ofpostoperative Hirschsprung-associated enterocolitis: retrospective study at a tertiary children's hospital.

To explore the influence of postoperative Hirschsprung-associated enterocolitis (post-HAEC) on long-term outcomes and to identify risk factors of post-HAEC. The medical records of 304 eligible patients diagnosed with Hirschsprung's disease (HSCR) were reviewed. We analyzed the clinical characteristics of post-HAEC and its influence on long-term outcomes. Furthermore, risk factors for early and recurrent HAEC were identified separately. The overall incidence of post-HAEC was 29.9% (91/304). We categorized early HAEC as occurring within postoperative 3months (n = 39) and recurrent HAEC as occurring ≥ 3 episodes within postoperative 6months (n = 25). Patients with early HAEC were more likely to experience worse nutritional status, defecation function, and quality of life compared to those with late or no episodes (P < 0.05). Similarly, the adverse influences of recurrent HAEC on these outcomes were also significant (P < 0.05). The risk factors for early HAEC included preoperative undernutrition, long-segment HSCR, and postoperative Grade 3-4 complications within 30days. For recurrent HAEC, risk factors were preoperative malnutrition, non-parental caregivers, long-segment HSCR, and postoperative Grade 3-4 complications within 30days. Classification of post-HAEC based on the first episode time and frequency was necessary. The earlier or more frequent episodes of post-HAEC have detrimental influences on long-term outcomes. Furthermore, risk factors for early and recurrent HAEC were different.

Crohn’s disease in patients with Hirschsprung disease and Hirschsprung-associated enterocolitis: A review

In the last decade, the incidence of inflammatory bowel disease in children has been increasing. Some studies have reported the development of Crohn’s disease in patients with Hirschsprung disease and Hirschsprung-associated enterocolitis. Suggestions have been made about the possible causes of the development of Crohn’s-like lesions after intestinal resection in early childhood. This study aimed to summarize information about Hirschsprung-associated Crohn’s disease and Crohn’s-like lesions after surgical treatment of Hirschsprung disease. The search was carried out in scientific databases of eLibrary, PubMed, ScienceDirect, and Google Scholar. The search time interval was 2000–2023. Fourteen articles dedicated to Hirschsprung-associated Crohn’s disease were identified. Common pathogenesis factors in inflammatory bowel diseases and Hirschsprung-associated enterocolitis were identified, such as microbiota disturbance and chronic inflammation. Predisposing factors for pathological immune response to dysbiosis and incidence of enterocolitis included enteric nervous system dysfunction, impaired neuroimmune regulatory mechanisms, general immunological disturbance, and “immaturity” and abnormality of the barrier function of the intestinal mucosa. The “classic form” of Crohn’s disease may not develop in patients with Hirschsprung disease and recurrent attacks of enterocolitis but Crohn’s-like lesions. Persistent functional or organic obstruction after surgical treatment of Hirschsprung disease enhances the negative effect of predisposing factors, leading to chronic inflammation of the intestinal mucosa. Delayed occurrence of Hirschsprung-associated enterocolitis is a predisposing factor for the development of chronic inflammation. When Hirschsprung-associated enterocolitis occurs repeatedly, endoscopic assessment should be performed for the exclusion of Crohn’s disease and chronic inflammatory changes in the anastomosis area for treatment selection.

Evaluation of Post-neonatal Intensive Care Unit Home Irrigations Prior to Pull-through: Implications for Hirschsprung Disease Management

BackgroundPull-through procedures for Hirschsprung disease (HD) can be performed during the Neonatal Intensive Care Unit (NICU) stay or delayed until discharge following home irrigations. This study assesses the safety of a delayed pull-through as an alternative to neonatal reconstruction in infants with successful abdomen decompression with home irrigations based on Hirschsprung-associated enterocolitis (HAEC) development. MethodsA single-institution retrospective review of neonates with HD who underwent delayed or neonatal pull-through from July 2018–July 2022. Endpoints included post-pull-through HAEC incidence, recurrence at an 18-month follow-up, time to the first HAEC episode, NICU length of stay (LOS), and HAEC-related LOS. ResultsTwenty-four neonates were included. Eighteen were discharged from the NICU with home irrigations. Of these, 3 (28%) developed enterocolitis preoperatively, 12 (67%) underwent a delayed pull-through. NICU LOS in the delayed cohort was 3 times shorter than in the neonatal (6 vs. 18 days, p < 0.01). The incidence of enterocolitis (82% vs. 80%), time to the first episode (43 vs. 57 days), and HAEC-related LOS (median of 3 days) were similar. ConclusionsDelayed HD pull-through is a viable neonatal reconstruction alternative that reduces NICU stay without increasing the risk of postoperative HAEC development. Type of StudyOriginal Research Article. Level of EvidenceIII.

Rates of Hirschsprung-Associated Enterocolitis Decrease With Increasing Child Opportunity Index

BackgroundHirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in patients with Hirschsprung disease (HD). There is a correlation between social determinants of health (SDOH) and outcomes in children with HD. The Child Opportunity Index (COI) is a publicly available dataset that stratifies patients by address into levels of opportunity. We aimed to understand if a relationship exists between COI and HAEC. MethodsA single-institution, IRB-approved, retrospective cohort study was performed of children with HD. Census tract information was used to obtain COI scores, which were stratified into categories (very low, low, medium, high, very high). Subgroups with and without history of HAEC were compared. ResultsThe cohort had 100 patients, of which 93 had a COI score. There were 27 patients (29.0%) with HAEC. There were no differences in demographics or clinical factors, including length of aganglionic colon, operative approach, and age at pull-through. As child opportunity score increased from very low to very high, there was a statistically significant decrease in the incidence of HAEC (p = 0.04). ConclusionWe demonstrate a significant association between increasing opportunity and decreasing incidence of HAEC. This suggests an opportunity for targeted intervention in populations with low opportunity. Level of EvidenceIII. IRB NumberIRB14-00232.

Comparison between Anal Dilatation Protocols Following an Endorectal Pull-through for Hirschsprung Disease

Objective: The purpose of this study was to compare the mechanical obstruction rate following a transanal endorectal pull-through (TERPT) in patients with Hirschsprung disease, between regular anal dilatation (AD) and selective anal dilatation (NAD) which meant that dilatation was only performed when an obstructive symptom occurred. Materials and Methods: A retrospective chart review of patients with Hirschsprung disease who underwent TERPT/abdominal assisted TERPT at Siriraj Hospital between January 2009 and December 2021 was carried out. It was the surgeon’s preference that the dilatation protocols between the 2 groups (AD or NAD) were assigned. Mechanical obstructions included evidence of stricture, a clinical symptom of constipation, presence of Hirschsprung-associated enterocolitis (HAEC), and/or requirement of re-operation. Results: In total, 132 patients were included in this study, including 55 cases in the AD group (41.7%) and 77 cases in the NAD group (58.3%). Postoperative mechanical obstructions occurred in 84 patients (63.6%). Among the mechanical obstructions, there were 35 strictures (26.5%), 26 constipation (19.7%), 40 HAECs (30.3%), and 6 reoperation (4.5%). The mechanical obstruction rates in the AD [33/55 (60.0%)] and NAD [51/77 (66.2%)] groups were not significantly different (p = 0.582). The AD group was of a significantly younger age (p = 0.022) and lower body weight (p = 0.048) than the NAD group; however, a younger age and lower body weight were not significantly related with any of the obstructive complications. AD had a rate of anastomosis/cuff stricture [13/55 (23.6%)] similar to NAD [22/77 (28.6%)] (p = 0.665).

P257 Clinical characteristics and outcome of Hirschsprung's disease-associated inflammatory bowel disease: experience from 33 years of practice in a single institution

Abstract Background Hirschsprung-associated Inflammatory Bowel Disease (H-IBD) is a very rare condition, occurring in approximately 2% of individuals with Hirschsprung's disease. The etiology remains unclear. In a recent study by Wang et al., nine genes were identified as consistently overexpressed in patients with both Crohn's disease and Hirschsprung's disease. Notably, all of them possess proinflammatory properties (IL1B, IL10, CXCL10, ICAM1, EGR1, FCGR3A, S100A12, S100A9, and FPR1). This suggests that chronic inflammation in HD patients may be involved in the progression to IBD. However, due to its low prevalence, there is currently a lack of knowledge about its course and response to treatment. In this study, we aimed to describe our initial experience with H-IBD by analyzing 378 patients operated on for HD at our institution. Methods We retrospectively reviewed all patients who underwent operation for HD from January 1989 to December 2021 in a single institution. Of 378 patients, 13 patients were suspected of H-IBD. The diagnosis of H-IBD was based on the criteria described in Sutthatarn et al. We analyzed patient characteristics and outcome. Results Of the 13 patients, 8 (61.5%) were boys. All patients had a final diagnosis of total colonic aganglonosis (TCA, long segment HD). One patient was accompanied by an imperforate anus and this patient got colonic segmental resection. Duhamel was performed in 4 patients, endorectal pull-through in 5 patients, and Swenson in 1 patient. Seven patients underwent a re-do pull-through operation. H-IBD was diagnosed by bloody stools in 4 patients, diarrhea in 5 patients, repeated Hirschsprung-associated enterocolitis (HAEC) in 3 patients and iron-deficiency anemia in 1 patient. Seven patients started treatment, and all but one with recurrent flare-ups improved. Immunologics were used in 2 patients, and the remaining patients were maintained with steroids, mesalazine, and antibiotics. Additional surgeries for H-IBD were not performed in all patients. Conclusion In our experience, H-IBD was diagnosed in 13 of the 46 children operated on with TCA. (28.3%) Progression to H-IBD should be suspected in patients with recurrent postoperative HAECs and poor response to antibiotic therapy. In addition, in patients older than 5 years of age who require stoma formation due to diarrhea or hematochezia, it may be helpful to screen for the possibility of H-IBD and initiate appropriate therapy.

The Effect of the Ganglionic Segment Inflammatory Response to Postoperative Enterocolitis in Hirschsprung Disease

Introduction We examined the relationship between proinflammatory cytokines that occur in the inflammatory reaction in the intestine in Hirschsprung disease (HD) and Hirschsprung-associated enterocolitis (HAEC). Methods Thirty cases (M:27, F:3) operated on due to HD. The cases were divided into three groups: group 1 with pre and post operative EC, group 2 with post-operative, and group 3 with pre-operative EC. The intestinal segments were evaluated by immunohistochemistry for interleukin 1 beta (IL-1ß), tumor necrosis factor-alpha (TNF-α), and interleukin 6 (IL-6). Results IL-1β staining was significantly higher in the ganglionic zone of groups with enterocolitis compared to the control group (p = 0.012). TNF-α staining in the transitional zone of Group 3 and IL-1β staining in the ganglionic zone of Group 1 was significantly higher than the control group (p = 0.030, p = 0.020). Conclusion In our study, older age at diagnosis and more than 20% IL-1ß staining in the ganglionic segment were found to be risk factors for HAEC. It is noteworthy that the increase in IL-1ß can be associated with HAEC.

Severe colonic distention and malnutrition in a 6-week-old infant: a case of Hirschsprung-associated enterocolitis

Severe colonic distention and malnutrition in a 6-week-old infant: a case of Hirschsprung-associated enterocolitis

Postoperative Hirschsprung’s associated enterocolitis (HAEC): transition zone as putative histopathological predictive factor

AimsHirschsprung’s-associated enterocolitis (HAEC) is the most severe complication of Hirschsprung disease (HD), and its pathogenesis is still unknown. Length of transition zone (TZ) interposed between aganglionic and normal bowel has...

Late Diagnosis of Hirschsprung Disease: Clinical Presentation and Long-Term Functional Outcomes

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

Comparation of robotic-assisted surgery and laparoscopic‑assisted surgery in children with Hirschsprung’s disease: a single-centered retrospective study

BackgroundThere are few studies comparing robotic-assisted surgery (RAS) and laparoscopic-assisted surgery (LAS) in Hirschsprung’s disease (HSCR). This study aimed to compare intraoperative and postoperative outcomes between RAS and LAS performed during the same period.MethodsAll consecutive 75 patients with pathologically diagnosed as HSCR who underwent Swenson pull-through surgery from April 2020 to Nov 2022, were included. Patients were divided into RAS group and LAS group and a retrospective analysis was performed based on clinical indexes and prognosis.ResultsA total of 75 patients were included, among which, 31 patients received RAS and 44 received LAS. The RAS and LAS groups had similar ages, sex, weight, postoperative hospital stays, and fasting times. Compared with LAS, blood loss (p = 0.002) and the incidence of Hirschsprung-associated enterocolitis (p = 0.046) were significantly lower in the RAS group. The first onset of Hirschsprung-associated enterocolitis in patients younger than 3 months occurred significantly earlier (p = 0.043). Two patients experienced anastomotic leakage in the LAS group and one patient experienced incisional hernia in the RAS group. The cost of RAS was significantly higher than that of LAS (p < 0.0001).ConclusionsRAS is a safe and effective alternative for HSCR children, and a delaying primary surgery until later in infancy (> 3 months) may improve outcomes.

Long-term functional outcomes and multidisciplinary management after ileorectal Duhamel pull-through for total colonic aganglionosis- 20-year experience in a tertiary surgical center.

Introduction Management of patients with total colonic aganglionosis (TCA) is challenging for paediatric surgeons. The purpose of this study was to review our institution's 20-year experience regarding long-term outcomes and multidisciplinary team management of these patients after an Ileorectal Duhamel pull-through procedure. Materials and Methods Retrospective review was conducted for all patients diagnosed with TCA in our tertiary institution. Data was collected on demographics, clinical presentation, complications, need for additional surgery and long-term effects on bowel function. Results Of a total of 202 patients with Hirschsprung's disease (HSCR), 13 were diagnosed with TCA (6.4 %). Clinical presentation was variable. 11 presented in neonatal period with distal bowel obstruction and 2 presented with constipation in early infancy. Ileorectal Duhamel pull-through was performed in all patients. Median follow up was 13 years. 11 are toilet trained, of whom 5 are fully continent. 6 continue to have problems with bowel continence or constipation. 1 developed recurrent episode of Hirschsprung's associated enterocolitis (HAEC). 2 patients had stoma re-established. Patients experiencing difficulties in bowel function are jointly managed by a multidisciplinary team consisting of surgeons, gastroenterologists, paediatric psychologists, and clinical nurse specialists. Conclusions TCA can be associated with significant long-term morbidity. Nearly half of the patients in this series have ongoing problems with bowel continence requiring a permanent stoma in some. Diligent follow-up coupled with inputs of a multidisciplinary team has greatly helped manage these complex patients in our institution.

Should we perform a Hirschsprung redo pull-through on patients with retained transition zone?

BackgroundIn Patients with Hirschsprung Disease (HSCR) suffering from obstructive symptoms after their pull-through surgery, a transition zone pull-through (TZPT) was suggested as a causing factor. Current guidelines recommend a reoperation resecting the transition zone in these patients. This present study reports the clinical outcomes in patients with a confirmed TZPT. MethodsA retrospective review of patients who underwent a primary transanal pull-through for recto-sigmoid HSCR at the Children's Hospital Colorado from January 2010 until June 2020 was performed. We included patients with a TZPT confirmed on the definitive histopathological report of the proximal margin's circumference obtained at the time of their pull-through surgery and follow-up at the Children's Hospital Colorado. Demographic data were collected. The primary outcome of interest was fecal control, episodes of Hirschsprung-associated enterocolitis (HAEC), the necessity of bowel management (with either laxatives or enemas), and a redo pull-through. ResultsSix patients had a TZPT. Two patients suffered episodes of HAEC. One patient is fecal incontinent. None of them underwent redo pull-through surgery. Medical treatment was successful in all with obstructive symptoms. ConclusionPatients who underwent a pull-through surgery for HSCR with retained transition zone may suffer from obstructive symptoms, which can be managed conservatively with medical treatment. A TZPT is not necessarily associated with persistent obstructive symptoms. Level of evidence/type of studyLevel III, retrospective.

State of the Art Bowel Management for Pediatric Colorectal Problems: Hirschsprung Disease.

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.

Intervention effects and related mechanisms of glycyrrhizic acid on zebrafish with Hirschsprung-associated enterocolitis.

The prevention and treatment of Hirschsprung-associated enterocolitis (HAEC) is a serious challenge in pediatric surgery. Exploring the mechanism of HAEC is conducive to the prevention of this disease. To explore the possible mechanism of glycyrrhizic acid (GA) and its therapeutic effect on HAEC. We developed a model of enteritis induced by trinitrobenzenesulfonic acid (TNBS) in zebrafish, and treated it with different concentrations of GA. We analyzed the effect of GA on the phenotype and inflammation of zebrafish. After treatment with TNBS, the area of the intestinal lumen in zebrafish was significantly increased, but the number of goblet cells in the intestinal lumen was significantly reduced, but these did not increase the mortality of zebrafish, indicating that the zebrafish enteritis model was successfully developed. Different concentrations of GA protected zebrafish with enteritis. In particular, high concentrations of GA were important for the prevention and control of HAEC because it significantly reduced the intestinal luminal area, increased the number of goblet cells in the intestinal lumen, and reduced the levels of interleukin (IL)-1β and IL-8. GA significantly reduced the intestinal luminal area, increased the number of intestinal goblet cells, and decreased IL-1β and IL-8 in zebrafish, and is important for prevention and control of HAEC.

Histopathology of Ganglion Cells in the Proximal Resected Bowel Correlates With the Clinical Outcome in Hirschsprung Disease: A Pilot Study

IntroductionIntraoperative leveling biopsy by identifying ganglion cells is crucial to determine surgical margin during surgery for Hirschsprung disease (HSCR). The anastomosis should be performed at least 5 cm proximal to the ganglionic segment to prevent transition zone pull-through. However, the length of the transition zone could be much longer than expected and the histological evaluation of the entire circumference of the proximal margin is recommended, which is time-consuming and not applicable for leveling biopsy. We found that the histopathologic features of ganglion cells varied in the examined bowel specimens and demonstrated a pattern similar to immature and degenerated neuron cells. We assumed that the histopathologic features of ganglion cells in the proximal resected bowel were associated with the clinical outcome and might guide the leveling biopsy. In this study, we described a histopathologic grade of ganglion cells based on the degree of maturity and degeneration. We assessed the correlation between the histopathological grade of ganglion cells in the proximal surgical margin and clinical outcome. MethodsThree hundred fifty seven patients with HSCR treated between 2013 and 2020 were included. The ganglion cells were divided into six grades based on the histopathologic features in frozen sections. Medical records and detailed histopathologic results of intraoperative frozen sections were reviewed. Follow-up data were collected to evaluate clinical outcomes. The pediatric incontinence and constipation scoring system was used to predict bowel function. ResultsThe histopathologic results of proximal resected bowel from 357 HSCR patients were presented as follows: Grade I in 52 patients (14.6%), Grade II in 186 patients (52.1%), Grade III in 107 patients (30.0%), and Grade IV in 12 patients (3.4%). The median follow-up time was 46.8 mo (13.0-97.6 mo). The histopathologic grade of ganglion cells from the proximal resected margin was significantly related to postoperative constipation problems and the incidence of Hirschsprung-associated enterocolitis. The results from the pediatric incontinence and constipation scoring system indicated a positive correlation between better postoperative bowel function and lower histopathologic grade of ganglion cells. ConclusionsThis pilot study showed an association between the histopathologic features of ganglion cells in the proximal surgical margin and the clinical outcome. It may provide additional information for intraoperative pathologic consultation in leveling biopsy to prevent insufficient resection of the affected colon. A prospective study is warranted to validate these findings before clinical application.

Hirschsprung-Associated Enterocolitis at a Referral Institution: A Retrospective Review

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

Neuroimmune regulation in Hirschsprung's disease associated enterocolitis.

Neuroimmune pathways are important part of the regulation of inflammatory response. Nerve cells regulate the functions of various immune cells through neurotransmitters, and then participate in the inflammatory immune response. Hirschsprung's disease (HD) is a congenital abnormal development of intestinal neurons, and Hirschsprung-associated enterocolitis (HAEC) is a common complication, which seriously affects the quality of life and even endangers the lives of children. Neuroimmune regulation mediates the occurrence and development of enteritis, which is an important mechanism. However, there is a lack of review on the role of Neuroimmune regulation in enterocolitis associated with Hirschsprung's disease. Therefore, this paper summarizes the characteristics of the interaction between intestinal nerve cells and immune cells, reviews the neuroimmune regulation mechanism of Hirschsprung's disease associated enterocolitis (HAEC), and looks forward to the potential clinical application value.