Morbidity associated with total colon Hirschsprung's disease

Abstract

Increased awareness of total colon Hirschsprung's disease (TCH) has led to improvement in patient care and a decrease in the associated mortality. Morbidity has remained high and necessitates close follow-up to prevent the development of life-threatening complications. We retrospectively reviewed the records of 20 children with TCH treated at this institution since 1961. Follow-up averaged 40.4 months (range, 1 to 150). All patients were diverted with an ileostomy or jejunostomy. Eight children had a Swenson pullthrough, three had a Martin procedure, and eight had a Kimura procedure. One child with multiple anomalies died after his ileostomy. There were no postoperative deaths. Complications following ileostomy and pull-through procedures included excessive fluid losses, wound infections, stoma problems, and bouts of enterocolitis. Three children had no reported complications. Seventeen complications were reported in eight children with the Kimura procedure, 10 in the three children with the Martin procedure, and 22 in the eight children with the Swenson procedure. These complications resulted in an average of 4.4 admissions per patient (range, 1 to 11) with an average length of stay of 96 days per patient (range, 10 to 598). Twelve patients required an average of 63 days of total parenteral nutrition and eight needed 328 days (range, 23 to 867) of supplemental nasogastric feedings. Ten patients required at-home rectal irrigations and seven patients required rectal dilations. Nearly half of all patients were kept on antimotility agents long term. Since 1985 we have performed the Kumura procedure for all children with TCH and have seen a decrease in morbidity. In the last five patients we have performed the right colon patch at the time of the ileostomy to reduce morbidity from excessive fluid losses while the patient is waiting for a pull-through procedure.