Hirschsprung's disease and intestinal neuronal dysplasia - a frequent association with implications for the postoperative course

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.

Transanal one-stage endorectal pull-through for Hirschsprung’s disease: a comparison with the staged procedures

“…The obstruction and/or enterocolitis after Hirschsprung's surgery depends on the type of operation performed, the residual internal sphincter function, the presence of stricture at anastomotic site, the incomplete resection of aganglionic bowel and presence of neuronal intestinal dysplasia in upstream bowel which is present in approximately 20% of cases. In a recent publication, the HD-associated intestinal neuronal dysplasia was found in 40% of the cases [15]. In our study, postoperative enterocolitis was found in 4.8% of TOSPET group and 23.8% of Swenson group (p \ 0.05).…”

Persistent obstructive symptoms after surgery for Hirschsprung’s disease: Development of a diagnostic and therapeutic algorithm

“…There is evidence that many children with Hirschsprung's disease have associated motility disorders proximal to the aganglionic bowel, including associated IND 15,16 and gastroesophageal reflux. 17 Unfortunately, there still is disagreement among pathologists and clinicians regarding the precise histologic criteria for making the diagnosis of IND, the frequency with which it occurs in children with Hirschsprung's disease and the correlation between the findings of IND and the clinical occurrence of abnormal motility.…”

Laparoscopic extensive colectomy with transanal Soave pull-through for intestinal neuronal dysplasia in 17 children

“…The 5.8% soiling rate in this study was within the 4%-17% range reported in literature. [3,9,12] Patients with subtotal colectomy have frequent bowel movements after surgery, and 40 or more bowel movements a day at most, but it can recover to 2-3 times per day after about 6 months to 1 year for most cases due to preservation of the ileocecal valve and the cecum. Long-term amelioration of this condition has been reported.…”

“…Clinical experience [9,10] has indicated that IND is one of the most common causes of chronic retractable constipation. Although there is controversy over the surgical treatment in patients with IND, [11] aggressive surgical management should be adopted because IND has an unpredictable clinical course, and that its management should be dependent on the conditions of each patient.…”