Hirschsprung's disease: 8 years experience in a Nigerian teaching hospital

Background: Hirschsprung's disease (HD) is a common cause of intestinal obstruction in children. Despite increased understanding of the disease and several techniques of treatment, significant complications continued to be associated with its management.
Objective: To study the outcome of management of HD in the University Teaching Hospital, Ilorin, Nigeria.
Materials and Methods: The clinical records of all children managed for HD between January 1998 and December 2005 were retrospectively reviewed. Demographic data, modes of presentation, methods of diagnosis, age at colostomy formation, age and type of definitive operations were noted from patients' records. Complications associated with colostomy, pre- and postoperative complications, final outcome and duration of follow-up were also noted.
Results: Twenty-one patients (20 males, one female) managed for HD were reviewed. Eight (38%) were diagnosed in the neonatal period, the mean age at diagnosis being 22 months. Fourteen patients had completed the three stages of the operation, one is awaiting colostomy closure and two patients are waiting for definitive operations. Out of the 15 patients who had definitive surgery, nine had Swenson's pull-through operations while six had Duhamel operations. At presentation, six patients had enterocolitis, one of whom had spontaneous colonic perforation, two patients had sepsis. Colostomy-related complications recorded included sixteen patients with dermatitis, nine with colostomy prolapse, one patient with colostomy diarrhea and one with enterocolitis and sepsis. Following definitive surgery, three patients had wound infection, one partial intestinal obstruction, one postDuhamel hemorrhage and two enterocolitis. Complications after colostomy closure included two wound infections and one severe enterocolitis. There were five deaths (23.8%)-two from sepsis, two from enterocolitis and one from an adverse drug reaction. One of the children who had enterocotitis preoperatively died of enterocolitis after colostomy. Range of follow-up was six weeks- to eight years.
Conclusions: Many complications from HD resulted from late presentation to our unit. Colostomy caused unacceptable morbidity. Most deaths were due to sepsis and enterocolitis. The Swenson's and Duhamel operations gave satisfactory results. Early presentation and improved facilities in our pediatric surgical unit will reduce morbidity and mortality.