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Preoperative undernutrition is prevalent among children with Hirschsprung disease (HSCR). To evaluate whether preoperative nutritional support reduces the incidence of postoperative HSCR-associated enterocolitis (HAEC), we conducted a prospective, multicenter, open-label randomized controlled trial. The trial was initiated on January 1, 2021 and completed on October 31, 2022. It was carried out across seven tertiary hospitals in China. A total of 110 patients admitted for pull-through surgery for HSCR were enrolled. Eligible participants were randomly assigned 1:1 via a centralized web-based randomization system ( www.cbdps.com ), which generated the allocation sequence. Specifically, 55 patients were allocated to the preoperative nutritional support (PNS) group and 55 to the standard medical care (SMC) group. Surgeons and patients were not blinded to group assignment. The primary outcome was the incidence of HAEC at 3 months after surgery. At 3 months postoperatively, the incidence of HAEC in the PNS group was significantly lower compared to the SMC group. Specifically, the HAEC incidence was 7.27% (4/55) for the PNS group versus 29.09% (16/55) for the SMC group. This resulted in an absolute risk reduction of 21.82% (95% confidence interval: −35.64% to −7.99%; p  = 0.003). No adverse events were documented. These results demonstrate that preoperative nutritional support substantially reduces early HAEC incidence in pediatric HSCR patients following pull-through surgery. Trial Registration: ClinicalTrials.gov NCT04598841. Hirschsprung disease, characterized by the absence of ganglion cells leading to functional bowel obstruction, can be treated with surgery. Here, the authors report that preoperative nutritional support in children with Hirschsprung disease reduced the risk of postoperative enterocolitis by comparison to standard of care in a randomized controlled trial.

In this paper, we have compared and analyzed the effect of laparoscopic and open surgical treatments in children with congenital megacolon. To address this, a total of 64 children with congenital megacolon who underwent surgery in the hospital, particularly from April 2014 to December 2020, were selected as the research objects. They were divided into control and observation groups by the random number table method, with 32 cases in each group. The control and observation groups were treated with open surgical and laparoscopic treatments, respectively. The treatment effects of the two groups were compared. The enema time, operation time, blood loss, anal defecation time, and duration of postoperative hospital stay of the observation group were lower than those of the control group. The comparison between the two groups was statistically significant (P<0.05). There was no significant difference in CRP and WBC between the two groups before surgery (P>0.05). The CRP level and WBC of the two groups were both increased after operation, the CRP level of the observation group was lower than that of the control group, the difference was statistically significant (P<0.05), the WBC of the two groups was not statistically significant (P>0.05). The rate of excellent and good defecation in the observation group on the 7th day after surgery was higher than that in the control group, and the difference was statistically significant (P<0.05). There was no significant difference in Krickenbeck scores between the two groups before surgery (P>0.05); 6 months after the surgery, the score of Krickenbeck in both groups increased, and that of the observation group was higher than that of the control group, indicating a difference in the overall score (P<0.05). The total complication rate within 7 days after surgery in the observation group was lower than that in the control group, and the difference was not statistically significant (P>0.05). Laparoscopic treatment of congenital megacolon could improve surgical indicators and reduce stress response in children, improve defecation and anal function, reduce the risk of complications, and promote recovery.

For the surgical treatment of Hirschsprung’s disease, several surgical techniques are used to resect the distal aganglionic colon. Two frequently used techniques are the Duhamel procedure and the transanal endorectal pull-through procedure. During the ‘8th Pediatric Colorectal Course’ in Nijmegen, November 2015, a workshop was organized to share experiences of both techniques by several experts in the field and to discuss (long term) outcomes. Specifically, the objective of the meeting was to discuss the main controversies in relation to the technical execution of both procedures in order to make an initial assessment of the limitations of available evidence for clinical decision-making and to formulate a set of preliminary recommendations for current clinical care and future research.

Background Hirschsprung’s disease (HSCR) is a serious congenital bowel disorder with a prevalence of 1/5000. Currently, there is a lack of systematically developed guidelines to assist clinical decision-making regarding diagnostics and management. Aims This guideline aims to cover the diagnostics and management of rectosigmoid HSCR up to adulthood. It aims to describe the preferred approach of ERNICA, the European Reference Network for rare inherited and congenital digestive disorders. Methods Recommendations within key topics covering the care pathway for rectosigmoid HSCR were developed by an international workgroup of experts from 8 European countries within ERNICA European Reference Network from the disciplines of surgery, medicine, histopathology, microbiology, genetics, and patient organization representatives. Recommendation statements were based on a comprehensive review of the available literature and expert consensus. AGREE II and GRADE approaches were used during development. Evidence levels and levels of agreement are noted. Results Thirty-three statements within 9 key areas were generated. Most recommendations were based on expert opinion. Conclusion In rare or low-prevalence diseases such as HSCR, there remains limited availability of high-quality clinical evidence. Consensus-based guidelines for care are presented.

PurposeHirschsprung's associated enterocolitis (HAEC) is a complication of Hirschsprung's Disease (HD) with considerable morbidity and mortality. The variability in presentation leads to a wide variety of the reported prevalence pre-and postoperatively. This systematic review aimed to clarify the prevalence of HAEC in short—(S-HD), long (L-HD), TCA and the type of operation used.MethodsA systematic literature-based search for relevant cohorts was performed using Pubmed/Medline, Cochrane Library from its inception to May 2021. Studies reporting on pre-and postoperative enterocolitis, segment length, and surgical procedure (Soave, Swenson, Duhamel) were included. Pooled prevalence and subgroup analysis have been calculated for pre-and postoperative HAEC.Results4738 articles were identified from the literature search, among which 57 studies, including 9744 preoperative and 8568 postoperative patients, were included. The groups were sorted by length of the aganglionic segment for further analysis. The pooled prevalence for preoperative HAEC was 18.3% for all types, 15.2% for S-HD and 26.1% for TCA. The pooled prevalence for postoperative HAEC was in total 18.2% for all segment lengths and used techniques. Subgroup analysis showed no significant difference in the occurrence of postoperative enterocolitis between the three techniques.ConclusionThe prevalence of preoperative HAEC increases with segment length. However, pooled data suggest that the postoperative risk for developing HAEC, independently of the employed method and segment length, is comparable to the preoperative risk.

Abstract Objectives Hirschsprung disease (HD) is a congenital condition defined by the absence of ganglion cells in the distal-most portion of the gastrointestinal tract. Biopsies and resections for HD can be adrenaline inducing for the general surgical pathologist because specimens are infrequent; HD is 1 of only a few neuroanatomic diseases that general surgical pathologists diagnose; numerous preanalytic factors (eg, biopsy adequacy, surgeon sampling protocol, processing artifacts) can affect histologic interpretation; and most importantly, the diagnosis has high stakes. Methods We provide a comprehensive overview of the background, relevant clinical procedures, and pathologic assessment of HD. Grossing and frozen section protocols, an algorithmic approach to diagnosis, and histologic pearls and pitfalls are also discussed. Results Evaluation and recognition of the features of HD have evolved significantly in the past 2 decades with the discovery of the value of calretinin immunohistochemistry in the late 2000s and the recent development of straightforward and reproducible histologic criteria for identification of the HD transition zone. Conclusions These advancements have substantially improved the pathologist’s ability to reliably evaluate for HD. Nonetheless, as with any high-stakes surgical pathology specimen, clear communication with the clinical team is essential.

Background Advances in surgical techniques and perioperative care have improved the short- and mid-term postoperative outcomes of patients with Hirschsprung disease (HD). However, the long-term outcomes of these patients (older than 10 years) have not been fully investigated. The aim of this systematic review is to clarify the prevalence of long-term outcomes and the quality of life of these patients. Methods PubMed, AMED, Cochrane Library, CINAHL and PsycINFO databases were searched from inception to October 2018, following the Meta-analysis of Observational Studies in Epidemiology (MOOSE) guideline. Original studies reporting the outcomes of patients older than ten years with HD were selected and reviewed. The overall prevalence of fecal incontinence, constipation, bowel function score, bladder dysfunction symptoms, and patients’ quality of life were extracted from the included studies and pooled through the random-effects meta-analysis model. The heterogeneity and variation in the pooled estimations were evaluated by Cochrane’s Q test and the I 2 test. The sensitivity analysis was conducted by the sequential omission of individual studies. Publication bias was evaluated by Egger’s linear regression test. The whole procedure was conducted with Stata (version 14). Results In total, 3406 articles were identified from the literature search, among which twelve studies, including 625 patients, were included for analysis. The pooled prevalences of fecal incontinence, constipation, and bladder dysfunction symptoms and good to excellent bowel function scores were 0.20 (95% CI 0.13–0.28), 0.14 (95% CI 0.06–0.25), 0.07 (95% CI 0.04–0.12), and 0.95 (95% CI: 0.91–0.97), respectively; the pooled mean score of gastrointestinal-related quality of life was 118 (95% CI: 112.56–123.44). Conclusions HD patients older than ten years old have an overall high prevalence of fecal incontinence and a low quality of life. Targeted and evidence-based follow-up procedures and transitional care are essential to meet these patients’ long-term care needs. Prospective and multicenter research that focuses on the attributes and predictors of the long-term prognosis of patients with HD are necessary.

Purpose Accurate histological diagnosis in Hirschsprung disease (HD) is challenging, due to its complexity and potential for errors. In this study, we present an artificial intelligence (AI)-based method designed to identify ganglionic cells and hypertrophic nerves in HD histology. Methods Formalin-fixed samples were used and an expert pathologist and a surgeon annotated these slides on a web-based platform, identifying ganglionic cells and nerves. Images were partitioned into square sections, augmented through data manipulation techniques and used to develop two distinct U-net models: one for detecting ganglionic cells and normal nerves; the other to recognise hypertrophic nerves. Results The study included 108 annotated samples, resulting in 19,600 images after data augmentation and manually segmentation. Subsequently, 17,655 slides without target elements were excluded. The algorithm was trained using 1945 slides (930 for model 1 and 1015 for model 2) with 1556 slides used for training the supervised network and 389 for validation. The accuracy of model 1 was found to be 92.32%, while model 2 achieved an accuracy of 91.5%. Conclusion The AI-based U-net technique demonstrates robustness in detecting ganglion cells and nerves in HD. The deep learning approach has the potential to standardise and streamline HD diagnosis, benefiting patients and aiding in training of pathologists.

ObjectivesTo describe clinical characteristics and preoperative management of a national cohort of infants with Hirschsprung's disease (HD).DesignPopulation-based cohort study of all live-born infants with HD born in the UK and Ireland from October 2010 to September 2012.SettingAll 28 paediatric surgical centres in the UK and Ireland.Participants305 infants presenting before 6 months of age with histologically proven HD.Main outcome measuresIncidence, clinical characteristics including gestational age, birth weight, gender, associated anomalies; age and clinical features at presentation; and use of rectal washouts or stoma.ResultsThe incidence of HD in the UK and Ireland was 1.8 per 10 000 live births (95% CI 1.5 to 1.9). Male to female ratio was 3.3:1. An associated anomaly was identified in 23% (69), with 15% (47) having a recognisable syndrome. The proportion of infants who presented and were diagnosed in the neonatal period was 91.5% (279) and 83.9% (256), respectively. 23.9% (73) and 44.2% (135) passed meconium within 24 and 48 hours of birth. 81% (246) first presented to a hospital without tertiary paediatric surgical services, necessitating interhospital transfer. Initial colonic decompression was by rectal washouts in 86.2% (263) and by defunctioning stoma in 12.8% (39). Subsequently, 27.4% (72) of infants failed management with rectal washouts and required a delayed stoma, resulting in 36.4% (111) of infants having a stoma.ConclusionsIn this population-based cohort, presentation outside the neonatal period was rare. Nearly half of the infants with HD passed meconium within 48 hours of birth and over one third were managed with a stoma.

Purpose Surgical procedures for bowel aganglionosis require accurate identification of enteric ganglion cells and cholinergic nerve hypertrophy. Current intraoperative mapping through frozen section histopathology-histochemistry is time-consuming and demands skilled interpretation. This study explores an electrochemical sensor for objective, rapid intraoperative mapping of the aganglionic bowel segment via tissue acetylcholine (ACh) detection. Methods An electrochemical biosensor was developed using nanostructured electrodes functionalized with acetylcholinesterase(AChE) and choline oxidase (ChOx) enzyme hybrids. Electrochemical analyses were conducted on homogenized intestinal biopsies obtained intraoperatively from ten patients, including eight diagnosed with Hirschsprung Disease (HSCR) and two with total colonic aganglionosis (TCA). Biopsy samples representing ganglionic, transitional, and aganglionic bowel segments were evaluated. The sensor quantified tissue ACh levels, a surrogate marker of cholinergic neuronal hypertrophy, by measuring the generated electrical current. Results The electrochemical analysis demonstrated significantly higher current levels in the aganglionic segments compared to ganglionic segments in 87.5% (7 out of 8) of HSCR patients. The mean peak currents observed in HSCR cases were 2.62 µA in aganglionic segments, 3.66 µA in transition segments, and 2.04 µA in ganglionic segments. In contrast, electrochemical patterns in patients diagnosed with TCA were atypical; ileal tissue samples from these patients generally yielded higher current measurements than colonic samples across all zones examined. Additionally, there was a progressive increase in tissue current values correlating positively with patient age. Conclusion The electrochemical sensor effectively differentiated aganglionic from ganglionic zones in HSCR, suggesting its potential as a quick, objective tool for intraoperative bowel levelling. Further validation in larger cohorts is necessary to confirm the clinical utility.