Intestinal neuronal dysplasia as a cause of surgical failure in Hirschsprung's disease: A new modality for surgical management

Two hundred fifteen cases of Hirschsprung's disease (HD) had follow-up after definitive surgical treatment, which had been performed between September 1983 and August 1994. Of these, 20 (9.3%) had recurrence of obstructive symptoms that was unresponsive to conservative treatment. Nine cases were improved by posterior anorectal myectomy (PARM). The other 11 patients, who did not have improvement after PARM, had a re-pull-through. Reoperation consisted of excision of the descending and left transverse colon with pull-through of the right colon, regardless of the histology of the colon. The blood supply was based on the ileocolic vessels, with or without division of the right colic artery. To provide a smooth course for these blood vessels, without kinking, and to obtain adequate length of the right colon with less dissection, the entire bowel was derotated clockwise. Re-pull-through was then performed from the left side of the abdomen. The excised bowel from both operations was examined, using H&E staining, for possible evidence of intestinal neuronal dysplasia (IND) or acquired aganglionosis. All had ganglion cells in the remaining colon and at the distal limit of the pull-through. Histological signs of IND were present in all patients in the previously pulled-through colon. Apart from one mentally retarded child, all had satisfactory bowel movements in a 10 to 54-month follow-up period. IND should be considered a cause of surgical failure in HD. Subtotal colectomy and pull-through of the right colon after clockwise derotation of the entire bowel appears to be effective when symptoms persist after conservative therapy or PARM.