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Anorectal Malformation (ARM) and Hirschsprung's Disease (HD) are common causes of congenital intestinal obstruction in children. Simultaneous occurrence of both conditions is rare. Few have been reported in Europe and Asia, but we have no knowledge so far of such report from Nigeria. We present two patients managed in our centre to highlight the challenges of management of this uncommon coexistence. The first patient was a 5-year-old girl who was referred to us with intestinal obstructive symptoms despite an apparently adequate sized ectopic anus. She had colostomy and rectal biopsy, which confirmed HD. She had corrective surgery performed through a posterior sagittal approach. She did well post operatively. The second patient was a 3-year-old girl who presented with features of intestinal obstruction, had laparatomy and was also referred to us. It was observed in the referral hospital during laparatomy, to have features of HD and rectal atresia intraoperatively. She had colostomy done and rectal biopsy performed at the same time, which confirmed the diagnosis of HD. She had simultaneous correction of both conditions through a posterior sagittal approach. She was in good condition at follow up. It was therefore recommended that a high index of suspicion of HD, should be entertained while managing patients with anorectal malformation.

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.

This is a retrospective study of 136 histologically confirmed cases of Hirschsprung's disease (HD) seen and treated in a single centre over 2 consecutive decades spanning the years from 1972 to 1991. Forty-six cases were seen in the 1st 10 years and 90 in the 2nd. Certain factors were considered to be responsible for the observed improvement during the 2nd decade; these included improvements in the mode of bowel preparation, the use of more effective antimicrobial agents with a wider spectrum of activity for prevention and treatment of established infections, and improvements in the pre-operative nutritional status of the patients and in the staging of the operations. Morbidity was minimal. Mortality dropped from 28.3% in the 1st decade to 5.6% in the 2nd. Causes of mortality in the 1st decade were pneumonia (8), aspiration of gastric contents (2), undernutrition (2) and haemorrhage from a dislodged clamp several days following a Duhamel procedure (1). In the early part of the 2nd decade, three patients died from anastomotic dehiscence following the Swenson procedure and two from aspiration pneumonia.