- Growth and dietary effects on gastrointestinal symptoms in children with Hirschsprung's disease. [Journal Article]Pediatr Surg Int. 2026 Sep 26; 42(1).PS
- CONCLUSIONS: Diet-related gastrointestinal complaints are reported frequently in children with Hirschsprung's disease, regardless of aganglionic extent. The findings highlight the need to assess growth and dietary factors in follow-up care and support further interventional studies on dietary strategies.
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- Subtypes of Enteric Neurons in Hirschsprung Disease: A Comparison of Fully Ganglionic and Transitional Zone. [Journal Article]Neurogastroenterol Motil. 2026 Sep; 38(9):e70444.NM
- CONCLUSIONS: Neurochemical subtype composition of enteric neurons is largely preserved between transitional and fully ganglionic bowel in HD. Within the limitations of this study, we did not detect major differences in neuronal subtype composition between transitional and fully ganglionic bowel that could explain persistent postoperative bowel dysfunction.
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- Appendix tip biopsy for Total colonic aganglionosis: technical aspects and diagnostic accuracy in a prospective cohort of 107 pediatric patients. [Journal Article]
- CONCLUSIONS: Appendix tip biopsy is an accurate and tissue-sparing adjunct for diagnosing total colonic aganglionosis. When combined with appropriate histopathological assessment, it provides rapid diagnostic information while preserving the proximal appendix for potential future reconstructive procedures. These findings support its incorporation into the diagnostic pathway for children with suspected TCA.
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- Hirschsprung's disease and a rare mutation of the PIGO gene: Mabry syndrome. [Case Reports]Turk J Pediatr. 2026 Sep 11; 68(4):689-694.TJ
- CONCLUSIONS: It should be remembered that Mabry syndrome can be accompanied by gastrointestinal anomalies, primarily HD. This case highlights the increased phenotypic variability associated with PIGO mutations and demonstrates the importance of genetic assessment in atypical neurodevelopmental conditions.
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- Penile agenesis (aphallia): A rare case report with associated congenital anomalies. [Case Reports]
- Penile agenesis (aphallia) is an exceptionally rare congenital anomaly with an estimated incidence of one in 10 to 30 million live births. We report a full-term male neonate with complete penile agenesis, a normally developed scrotum with bilaterally palpable testes, and passage of urine through the anus. Investigations revealed a rectovesical fistula, horseshoe kidneys with mild pelvicalyceal di…
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- Surgical management of hirschsprung disease: results of a worldwide survey. [Journal Article]
- CONCLUSIONS: A global survey of pediatric surgeons identified variation in perioperative management of patients with HD, with 25% of patients experiencing surgical complications. Opportunities for quality improvement and educational efforts to improve outcomes and surgical disparities were detected.
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- The long-term follow-up outcomes and transition rates across seven representative pediatric congenital surgical conditions: disease severity drives follow-up retention. [Journal Article]
- CONCLUSIONS: Follow-up attrition was paradoxically the highest among patients with favorable postoperative outcomes. Targeted transition programs focusing on ARM and HD populations are urgently needed.
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- Surgical management of fecal incontinence following anorectal malformation repair. [Journal Article]Pediatr Surg Int. 2026 Sep 15; 42(1).PS
- CONCLUSIONS: Surgical correction of residual anatomical defects was associated with partial improvement in bowel function in selected children with fecal incontinence after ARM repair, but normal continence was uncommon. Malone appendicostomy or structured bowel management may be preferable in patients with poor sphincteric or neuromuscular reserve.
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- Persistent non-toxic megacolon in ulcerative colitis: a case report of broad disruption and scar replacement of the muscularis propria. [Journal Article]
- Ulcerative colitis-associated colonic dilatation is clinically important because it may progress to perforation or toxic megacolon. However, surgical decision-making is difficult when systemic toxicity is absent. An 83-year-old woman with long-standing pancolitis-type ulcerative colitis was admitted with worsening bloody diarrhea and abdominal pain. Despite high-dose corticosteroids and inflixima…
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- Research progress on continuous nursing care for children after radical surgery for Hirschsprung's disease. [Review]
- Hirschsprung's disease is a common congenital gastrointestinal malformation in children, for which radical surgery remains the primary treatment modality. Postoperatively, patients are prone to complications such as Hirschsprung-associated enterocolitis, defecation dysfunction, anal stenosis, and psychological or behavioral abnormalities, all of which significantly impair growth, development, and…
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- Long-Term Stoma-Free Outcomes After Diverting Stoma Closure Following Radiation-Based Neoadjuvant Treatment for Rectal Cancer. [Journal Article]
- CONCLUSIONS: Approximately 90% of patients were stoma-free at the final follow-up. Delayed re-stoma creation was most commonly attributable to structural anastomotic complications. Long-term stoma-free status should be considered a clinically meaningful endpoint beyond stoma closure alone.
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- Long-term bowel functional outcomes after surgery for Hirschsprung disease: a comparative study of Duhamel and transanal endorectal pull-through. [Journal Article]
- CONCLUSIONS: The Duhamel group had higher bowel function scores during childhood than the TAEPT group. However, these differences diminished with age, suggesting functional maturation. Understanding procedure-specific functional characteristics may contribute to individualized bowel management and improved long-term quality of life in HD.
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- Macrophage-enteric nervous system crosstalk in Hirschsprung disease and associated enterocolitis: a focused narrative review with clinical implications. [Review]
- Hirschsprung disease (HSCR) results from incomplete distal bowel colonization by enteric neural crest-derived cells. Macrophage-enteric nervous system (ENS) interactions may influence postnatal neuromuscular function and Hirschsprung-associated enterocolitis (HAEC), but direct evidence that macrophages initiate embryonic aganglionosis is limited. This focused narrative review therefore treats pos…
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- Gastrointestinal symptoms and association to food items in children with hirschsprung disease: a multinational patient-reported outcome study from Latin America and Spain. [Multicenter Study]
- CONCLUSIONS: This first multinational Spanish speaking cohort survey demonstrates an association between food items and gastrointestinal symptoms in children with HD. Diet-related gastrointestinal symptoms are common in children with HD and increase with disease extent. Most families identified food items causing symptoms themselves. These findings highlight the need for individualized nutritional counselling as part of multidisciplinary long-term follow-up. Soiling was the main reported symptom in this Hispanic/Latino cohort. These findings emphasize the need for individualized dietary guidance supported by dietitian advice to support bowel function, growth, and quality of life, while preventing nutritional deficiencies.
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- Advancing hirschsprung disease diagnosis: a systematic review of the development and application of artificial intelligence in histopathological analysis. [Systematic Review]
- Hirschsprung's disease (HD) is characterised by absence of ganglion cells in the distal large intestine, requiring accurate histopathological diagnosis. Conventional diagnostic methods are time-consuming, subjective, and demand specialised expertise. While artificial intelligence (AI) shows promise for improving diagnostic capacity, its clinical utility requires rigorous evaluation. Following PRI…
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