hirschsprung's enterocolitis

AIMS: Hirschsprung'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 been poorly explored as predictor for postoperative HAEC (post-HAEC). This study aimed to identify potential predictive factors for post-HAEC, with a particular focus on histopathological findings. METHODS: Data from Hirschsprung patients treated in a single Italian centre between 2010 and 2022 with a follow-up >6 months were collected...

BACKGROUND & AIMS: RET tyrosine kinase is necessary for enteric nervous system development. Loss-of-function RET mutations cause Hirschsprung disease (HSCR), in which infants are born with aganglionic bowel. Despite surgical correction, patients with HSCR often experience chronic defecatory dysfunction and enterocolitis, suggesting that RET is important after development. To test this hypothesis, we determined the location of postnatal RET and its significance in gastrointestinal (GI) motility...

PURPOSE: Hirschsprung 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...

Hirschsprung's disease (HD) is a rare condition that affects newborns and is characterized by the lack of ganglion cells in the colon. Typical symptoms include difficulty passing stool, vomiting, and trouble feeding. Various surgical methods are available to manage the condition. The aim of the study is to investigate and compare the post-surgical outcomes of different surgical techniques used in the treatment of HD. A thorough literature search was conducted using various electronic databases to identify relevant studies to be referred to...

OBJECTIVE: Hirschsprung disease in newborns can be a potentially life-threatening condition, with risks for complications such as Hirschsprung-associated enterocolitis. Accessing health information in a readable format for complex diseases demonstrates an important outlet for families to address concerns. While it is important to seek out information from trusted providers, many individuals seek out ways to educate themselves further by using common search engines and turning to the internet...

UNLABELLED: Objective: To compare the Duhamel and transanal endorectal pull-through (TERPT) procedures in the treatment of children with Hirschsprung's disease. METHODS: Studies comparing the Duhamel and TERPT procedures were included until 22 July 2023. R software (version 4.3.0) was used to perform the meta-analysis. RESULTS: Ten studies with a sum of 496 patients were included. The length of postoperative hospital stay and incidence of postoperative constipation were longer and higher after the Duhamel procedure than the TERPT procedure ( p < 0...

BACKGROUND: Inflammatory Bowel Diseases (IBD) are known to occur in association with Hirschsprung disease (HSCR). Most of cases are represented by Crohn Disease (CD) occurring in patients with Total Colonic Aganglionosis (TCSA) with an estimated prevalence of around 2%. Based on these considerations and on a number of provisional data belonging to our Center for Digestive Diseases, we developed a unicentric cross-sectional observational study aimed at describing phenotype, genotype, pathology and metagenomics of all patients with TCSA and Crohn-like lesions...

No abstract text is available yet for this article.

Introduction The variation in standardized, well-defined parameters in Hirschsprung's disease (HSCR) research hinders overarching comparisons and complicates evaluations of care quality across healthcare settings. This review addresses the significant variability observed in these parameters as reported in recent publications. The goal is to compile a list of commonly described baseline characteristics, process and outcome measures, and to investigate disparities in their utilization and definitions. Materials and methods A systematic review of literature on the primary care process for HSCR was performed according to PRISMA guidelines...

INTRODUCTION: A proximal resection margin greater than 5 cm from the intra-operative histologically determined transition zone has been deemed necessary to minimize the risk of transition zone pull-through. This extended resection may require the sacrifice of vascular supply and even further bowel resection. The impact of extended proximal resection margin on post-operative complications and functional outcomes is unclear. METHODS: A retrospective chart review of patients who underwent primary pull-through for Hirschsprung disease at a single institution between January 2008 and December 2022 was performed...

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...

INTRODUCTION: In many resource-limited settings, patients with Hirschsprung's Disease (HD) undergo initial diverting colostomy, followed by pull-through, and finally, colostomy closure. This approach allows for decompression of dilated and thickened bowel and improved patient nutritional status. However, this three-stage approach prolongs treatment duration, with significant stoma morbidity, costs, and impact on quality of life. Our aim was to determine whether pull-through for HD can safely be performed with simultaneous stoma closure, reducing treatment approach from three to two stages...

BACKGROUND: Hirschsprung disease (HD) is characterized by absent neuronal innervation of the distal colonic bowel wall and is surgically treated by removing the affected bowel segment via pull-through surgery (PT). Incomplete removal of the affected segment is called transition zone anastomosis (TZA). The current systematic review aims to provide a comprehensive overview of the prevalence and clinical impact of TZA. METHODS: Pubmed, Embase, Cinahl, and Web of Sciences were searched (last search: October 2020), and studies describing histopathological examination for TZA in patients with HD were included...

BACKGROUND: There 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. METHODS: All 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...

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...

No abstract text is available yet for this article.

OBJECTIVE: To analyze the effect of laparoscopic Soave combined with Deloyers turnover on the efficacy and prognosis of children with congenital Hirschsprung's disease, and to explore an effective and safe operation, so as to provide a reference for clinical development of treatment plan and promote the faster recovery of children. METHODS: A total of 80 children with Hirschsprung's disease admitted to our hospital from July 2021 to June 2022 were selected and included in the traditional group and minimally invasive group according to different surgical procedures, with 40 cases in each group...

OBJECTIVE: Little is known about intestinal anastomotic leakage and stenosis in young children (≤3 years of age). The purpose of this study is to answer the following questions: (1) what is the incidence of anastomotic stenosis and leakage in infants? (2) which surgical diseases entail the highest incidence of anastomotic stenosis and leakage? (3) what are perioperative factors associated with anastomotic stenosis and leakage? METHODS: Patients who underwent an intestinal anastomosis during primary abdominal surgery in our tertiary referral centre between 1998 and 2018 were retrospectively included...

INTRODUCTION: Rural children have worse health outcomes compared to urban children. One mechanism for this finding may be decreased access to specialized care at children's hospitals. The objective of this study was to evaluate the hospital types where complex surgical care in infants is performed nationally. METHODS: This study examined infants (<1 y old) in the Kids' Inpatient Database from 2009 to 2019 who underwent surgery for one of the following conditions: esophageal atresia, gastroschisis, omphalocele, Hirschsprung disease, anorectal malformation, pyloric stenosis, small bowel atresia, congenital diaphragmatic hernia, and necrotizing enterocolitis...

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...