Recent advances in the management of Hirschsprung's disease

BACKGROUND: Major advances have occurred in the management of Hirschsprung's disease since Swenson described his definitive operation in 1948. These advances have occurred in the following areas: genetics, neurophysiology, definitive management in the newborn, total colonic aganglionosis (TCA), Hirschsprung's-associated enterocolitis (HAEC), intestinal neuronal dysplasia (IND), and laparoscopic and perineal approaches for definitive pull-through and redo pull-through operations. METHODS: This paper will focus on the definitive management of the newborn, TCA, and HAEC, areas in which we have had considerable experience at our institution. RESULTS: We have treated almost 90 newborns with the definitive pull-through with minimum morbidity. We have managed 25 patients with TCA, of whom 5 had total intestinal involvement and died. The remaining 20 have undergone a total colectomy and endorectal pull-through (ERPT), with zero mortality and a very acceptable stooling pattern and continence rate. Our experience with more than 350 patients with Hirschsprung's disease over the past 25 years has demonstrated an incidence of HAEC of between 20% and 30%. During this period, we have performed 19 redo pull-through operations, the majority of which were ERPTs, with results comparable with those seen with a primary pull-through operation. CONCLUSIONS: The major advances that have occurred in the management of Hirschsprung's disease include the definitive management of the newborn, our understanding of Hirschsprung's-associated enterocolitis and the treatment of this entity, and the recent successful management of the very complex form of this disease, total colonic aganglionosis.