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Children with Hirschsprung's Disease and Syndromes with Cognitive Dysfunction: Manifestations, Treatment, and
Josefine Hedbys1, Johan Hasserius1, Christina Granéli1,2
1Department of Clinical Sciences, Lund University, Lund, Sweden.
Insights
Children with Hirschsprung
Area of Science:
- Pediatric Surgery
- Developmental Pediatrics
- Gastroenterology
Background:
- Hirschsprung's disease (HD) is a congenital condition affecting the large intestine.
- Cognitive dysfunction (CD) can impact the presentation and outcomes of pediatric conditions.
- Understanding these differences is crucial for tailored patient management.
Purpose of the Study:
- To compare initial symptoms, treatments, and bowel function in children with Hirschsprung's disease (HD) with and without cognitive dysfunction (CD).
Main Methods:
- Retrospective chart review of children with HD undergoing transanal endorectal pull-through.
- Analysis of patient characteristics, diagnosis, and treatment data.
- Cross-sectional, patient-reported follow-up for bowel symptoms in children over 4 years old without a colostomy.
Main Results:
- Children with HD and CD had lower birth weight, less vomiting, and a longer delay to surgical consultation.
- At follow-up, children with HD and CD demonstrated significantly poorer bowel function, including reduced ability to sense the urge to defecate and hold back stool.
- 12 of 53 (23%) children with HD had co-occurring CD.
Conclusions:
- Hirschsprung's disease (HD) in children with cognitive dysfunction (CD) presents with distinct initial symptoms and leads to poorer bowel function outcomes.
- A delay in seeking initial surgical consultation was observed in HD patients with CD.
- Special attention is warranted for HD children with CD in clinical practice and research.
Abstract:
Introduction To assess differences in initial symptoms, treatments, and bowel function between children with Hirschsprung's disease (HD) with or without a cognitive dysfunction (CD). Materials and Methods The study included children with HD who underwent transanal endorectal pull-through. A retrospective chart review was performed to collect data on patient characteristics, diagnosis, and treatment. Data on bowel symptoms in children older than 4 years without a colostomy were compiled during a cross-sectional, patient-reported follow-up. Results Fifty-three children with HD were included; of these, 12 (23%) had CD. The median birth weight was lower, frequency of vomiting as the presenting symptom was lower, and time until the first contact with a pediatric surgeon was higher in children with CD than in those without (3,295 vs. 3,623 g, p = 0.013; 28 vs. 66%, p = 0.02; and 4 days vs. 1 day, p = 0.048, respectively). At follow-up, 5 (15%) of 33 children aged over 4 years had CD. More children without CD had some ability to hold back defecation and sense the urge to defecate than those with CD ( p = 0.002 and p = 0.001, respectively). Conclusion HD children who have CD present with different initial symptoms, have a delay in the first consultation with a pediatric surgeon, and experience poorer bowel function outcomes than HD children without CD. Therefore, HD children with CD should receive special attention in both clinical practice and research.