Hirschsprung disease, like most surgically correctable congenital anomalies, is now survivable well into adulthood. Patients are, therefore, presenting later in life with problems that were previously infrequently encountered by colorectal surgeons or gastroenterologists. The goals of our review are to describe the current state of care transition for this patient population, identify the specific needs for these patients, and propose guidelines which could inform a future model of transition from the pediatric to adult setting. This is a review of the current state of care transition and long-term outcomes for patients with Hirschsprung disease. Although these patients report a generally good quality of life, the majority suffer from some degree of lifelong gastrointestinal complication, regardless of the type of operative repair. A more formalized transition of care will provide a guide for pediatric surgeons and patients, alleviate colorectal surgeon and gastroenterologist concerns, and provide better long-term care for these patients.
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http://dx.doi.org/10.1097/MPG.0000000000002385 | DOI Listing |
Pathol Res Pract
December 2024
University of Alabama at Birmingham, Department of Pathology, United States. Electronic address:
Hirschsprung's (HSCR) disease, also known as aganglionic megacolon, or congenital intestinal aganglionosis affects roughly 1 out of every 5000 newborns. It is a birth defect characterized by the partial or complete loss of ganglion cells in the myenteric and submucosal plexus of the distal intestine which leads to ineffective peristalsis, constipation, and obstruction. Clinical assessment and radiological observations might imply HSCR disease, but definitive diagnosis requires biopsy interpretation and confirmation of ganglion cell loss.
View Article and Find Full Text PDFGastroenterology
December 2024
Department of Clinical Genetics, Erasmus University Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands; Department of Pediatric Surgery, Erasmus University Medical Center - Sophia Children's Hospital, Rotterdam, The Netherlands. Electronic address:
Background And Aims: The enteric nervous system (ENS), comprised of neurons and glia, regulates intestinal motility. Hirschsprung disease (HSCR) results from defects in ENS formation, yet while neuronal aspects have been extensively studied, enteric glia remain disregarded. This study aimed to explore enteric glia diversity in health and disease.
View Article and Find Full Text PDFAnn Surg
December 2024
Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden.
Objective: The aim was to assess fertility, sexual function and sexual quality of life in males with Hirschsprung's disease (HSCR) in the Nordic countries with a cross-sectional study using self-reported validated questionnaires.
Summary Background Data: Data on fertility and sexual function in males with HSCR are limited.
Methods: This multi-center study targeted all males born between 1970-2003 who underwent pull-through surgery at a pediatric surgery center in Sweden, Denmark, Norway, or Finland.
Front Nutr
December 2024
Department of Urology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Structural Birth Defect and Reconstruction, Chongqing, China.
Background: Nutritional problem after surgery for Hirschprung's disease (HSCR) was not optimistic. This study aimed to analyze the risk factors of postoperative undernutrition for patients with HSCR and establish a scoring system for predicting postoperative undernutrition.
Methods: Retrospective review of 341 patients with HSCR who received Laparoscopic-assisted pull-through surgery in a tertiary-level pediatric hospital was conducted with assessments of clinical data.
Eur J Pediatr Surg
December 2024
Department of Pediatric Surgery, University of Leipzig, Germany, Leipzig, Germany.
Introduction Pediatric robotic colorectal surgery has rapidly evolved, offering enhanced precision and safety for treating complex conditions such as Hirschsprung disease (HSCR), anorectal malformations (ARM), and inflammatory bowel disease (IBD). This review analyzes recent trends, outcomes, and complications in robotic colorectal procedures for pediatric patients. Materials and Methods A systematic review was performed using PubMed, yielding 1,112 articles related to pediatric robotic colorectal surgery.
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