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Hirschsprung's disease and intestinal neuronal dysplasia--a frequent association with implications for the
P P Schmittenbecher1, P Sacher, D Cholewa
1Department of Pediatric Surgery, St. Hedwig's Hospital, Regensburg, Germany.
Insights
Hirschsprung-associated intestinal neuronal dysplasia (HaIND) affects 40% of Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Developmental Biology
Background:
- Hirschsprung's disease (HD) is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
- Intestinal neuronal dysplasia (IND) is a hypothesized developmental anomaly of the enteric nervous system.
- The association between HD and IND (HaIND) requires further investigation.
Purpose of the Study:
- To prospectively analyze the association between HD and IND.
- To determine the clinical implications of HaIND on surgical management and postoperative outcomes.
Main Methods:
- Prospective analysis of 106 newly diagnosed HD cases.
- Evaluation of 10 pediatric surgical departments in Central Europe.
- Assessment of IND presence, dissemination, and location in relation to HD.
Main Results:
- HaIND was identified in 40% of HD cases, with IND being disseminated in one-third and localized in two-thirds.
- Enterostomy sites were in pathologically innervated bowel in 72% of HaIND cases.
- Associated IND, especially disseminated forms, correlated with delayed defecation restoration, persistent constipation, and increased need for secondary interventions.
Conclusions:
- HaIND is a significant finding in a substantial proportion of HD patients.
- The presence and extent of IND influence postoperative outcomes, particularly in cases of disseminated HaIND.
- Early identification and consideration of HaIND are crucial for optimizing surgical strategies and patient management.
Abstract:
Between 1991 and 1993, 106 newly diagnosed cases of Hirschsprung's disease (HD) were prospectively analyzed for the association of HD and intestinal neuronal dysplasia (IND) at ten pediatric surgical departments in central Europe. Hirschsprung-associated IND (HaIND) was found in 40% of cases. IND was disseminated in one-third and localized in two-thirds of the patients. Initial clinical symptoms were related to the length of the aganglionic segment, but not to the presence of HaIND. An enterostomy performed in 72 cases (67.9%) was located in a segment of pathologically innervated bowel in 50% of all cases, but in 72% of cases of HaIND. The proximal margin of the resected bowel showed pathological innervation in 44% of cases. Supplemental biopsies from the intestine (apart from diagnostic suction biopsies and biopsies at the enterostomy site) led to the first identification or definition of length of associated IND in 17.9% of cases. Postoperatively, the presence of long-segment aganglionosis or associated IND implied a delay in the restoration or normal defecation. Persistent constipation was found in 40% of patients with associated disseminated IND at follow-up at 6 months, compared to 20.6% in patients with isolated HD. These children needed secondary interventions more often than patients with associated localized IND or isolated HD. HaIND thus has clinical implications for the postoperative course if IND is disseminated.