Clinical impact of intestinal neuronal malformations: a prospective study in 141 patients

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

“…These children required long-term bouginage and other secondary interventions such as sphincteromyectomy or redo of an enterostomy more often than children with isolated HD or localized HaIND. Ure et al [27] reported that a secondary resection was necessary signi®cantly more often in patients with HaIND. Moore et al [16] noted obstructive symptoms in 14%, one-half of them in patients with HaIND.…”

Interstitial cells of Cajal in human gut and gastrointestinal disease

“…light on the pathophysiological mechanisms and they are in addition of little predictive value for the clinical outcome (Cord-Udy et al, 1997; Koletzko et al, 1993;Ure et al, 1994Ure et al, , 1997. Besides structural abnormalities of the ENS, a low number of (vasoactive intestinal polypeptide) VIP-ir nerve fibers (together with normal distribution of NOS nerve fibers) (Cortesini et al, 1995) and a decreased number of (substance P) SP-ir nerve fibers (Hutson et al, 1996) have been reported in constipated children whereas VIP, SP, and galanin immunoreactive nerve fibers may be increased in constipated adults (Sjolund et al, 1997).…”

An approach to minimize postoperative enterocolitis in Hirschsprung's disease

“…Intestinal neuronal dysplasia has been found in the proximal ganglionic colon of 20% to 75% of patients with HD [1,2,5,6,8,22]. The current series positioned in the upper half (58%) of this range probably because of thorough adoption of the methodology required for the diagnosis of HD and allied disorders; histochemical characterization excluded the risk of confusing IND with the histologic changes of the transitional zone.…”

“…Even in the absence of mortality, postoperative EC is consequently a major cause of morbidity that justifies further investigation to clarify its pathophysiology and effective prevention. Research have addressed patients' characteristics, mechanical factors, type of technique, mucin production, gut immunity, mucosal genes, bacterial and viral agents, neuroendocrine cells, and retention of a residual aganglionic segment or transitional zone but have not demonstrated an unequivocal pathogenesis [17,20,[24][25][26][27][28][29][30][31]; a coexisting proximal dysplastic colon has been correlated with unsatisfactory outcome after HD surgery, suggesting a pathogenic relationship or an additive effect of both malformations [5,6].…”

“…Despite intensive research, the pathophysiology of this complication has not been fully elucidated; because type B intestinal neuronal dysplasia (IND), a well-established cause of abnormal colonic motility, 0022 is frequently found in the ganglionic pulled-through colon, it seems attractive to implicate coexisting IND in the pathogenesis of postoperative EC [1][2][3][4][5][6][7][8].…”