[Enterocolitis associated with Hirschsprung's Disease. Experience in a pediatric teaching hospital]

Hirschsprung's associated enterocolitis (HAEC) is the most serious and potentially fatal complication of the disease, with a significant morbidity and mortality. The effect of HAEC varies remarkably among the publications since the lack of consensus and the absence of established diagnostic criteria. Patients diagnosed with Hirschsprung's disease (HD) were selected and treated between January 2009 and December 2012 in Fundación HOMI, finding 41 cases of HD, 6 of them with total aganglionosis. 76% of the patients had HAEC, 18 of them during the neonatal period and 6 of them after the final pull-through; 23% presented recurrent enterocolitis episodes. HAEC was the first clinical manifestation in 25 (61%) of the patients with HD. From HD patients limited to the rectum and sigmoid 67% had enterocolitis unlike TCA (total colonic agangliniosis) cases in which all presented HAEC episodes. In our setting, HAEC remains to be an unknown complication; this revision demonstrates its high incidence and mortality.