Hirschsprung's disease in the newborn: experience in Zaria, Nigeria

Abstract

In a 10-year retrospective review of 15 newborns aged < or = 42 days presenting with Hirschsprung's disease, there were 12 boys and three girls aged 4-42 days (median 18 days). Twelve babies presented with complete intestinal obstruction. In 12 babies, there was a history of delayed passage of meconium (after 2-6 days). One baby each developed caecal and sigmoid perforation. Barium enemas in three babies without complete intestinal obstruction suggested Hirschsprung's disease in two. Following resuscitation, the two infants who had perforated had caecostomy and sigmoid repair with right transverse colostomy, respectively. One infant had ileostomy for total colonic aganglionosis associated with ileal atresia. All the others had initial diversion colostomy. Rectal biopsies confirmed Hirschsprung's disease in all the babies. The ileum was injured during colostomy in one case, requiring repair. Postoperative anastomotic leakage occurred in the infant with ileal injury and colostomy necrosis occurred in another infant. Five babies (33%) died, three from overwhelming infection (caecal perforation, sigmoid perforation, ileal injury), one from hypokalaemia (ileostomy) and one from an unidentified cause. Few cases of Hirschsprung's disease present in the newborn period in our environment and, when they do, they usually present with complete intestinal obstruction with high morbidity and mortality.