Purpose: The aim of this study was to assess real-world educational outcomes and developmental disorders in patients with a history of Hirschsprung disease compared to an age-matched control group.
Methods: With ethics approval (H2016:014) a retrospective cohort study of all children diagnosed with Hirschsprung disease at a single centre from 1992 to 2017 was performed. A 10:1 date-of-birth matched control cohort was constructed from a population-based directory. The educational outcomes were compared using the following measures: Early Developmental Instrument, Grades 3, 7, and 8 assessments, Grade 9 completion, Grade 9 performance, and high school graduation. Fisher's exact tests were used to compare the odds of failure between cases to controls. Only children who reached 4 years of age were included.
Results: A total of 75 cases with Hirschsprung disease patients were identified. Patients with Hirschsprung disease were at increased risk of failing to meet expectations on the Early Development Instrument. After entering elementary school, Hirschsprung patients were at no greater risk than their peers of failing to meet expectations on standardized testing or failing to graduate from high school.
Conclusion: Using real-world measures of academic success as a surrogate for neurodevelopmental status, our study demonstrates that patients with a history of Hirschsprung disease demonstrated poor neurodevelopmental performance in pre-school, but the educational achievements of patients did not differ from controls once they started school. These promising data can be used to mitigate preconceived notions that patients with Hirschsprung disease require special education, which may be isolating and psychosocially damaging.
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http://dx.doi.org/10.1016/j.jpedsurg.2021.12.040 | 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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