Hirschsprung's Disease in Adults

ABSTRACT

Hirschsprung's disease is a disorder caused by the absence of ganglion cells in the colon and rectum. It has an incidence of 1 in 5000 births, the majority diagnosed and treated in the neonatal period due to symptoms of intestinal obstruction. Persistence of Hirschsprung's disease into adulthood is very rare. In such patients, prolonged periods of constipation are a common problem. For the diagnosis, a colon study and anorectal manometry are performed, and the presence of the disease is confirmed by an excisional biopsy proving the absence of the ganglion cell in Auerbach and Meissner's plexus. Although various surgical procedures have been performed, there is no obvious optimal choice for treatment of Hirschsprung's disease in adolescents and adults. We experienced two cases of Hirschsprung's disease, confirmed by a rectal biopsy, in 20-year patients. Prior to a definitive operation, a sigmoid loop colostomy was performed due to severe dilatation of the left colon and rectum. Six months later, one patient was treated using Duhamel's procedure, and the other by using a proctosigmoidectomy and coloanal anastomosis. No postoperative complications were observed, and the patients had bowel movements three to four times a day. Despite its infrequent incidence, adult Hirschsprung's disease should be suspected in patients who have had lifelong constipation. Several successful surgical treatments have been used for treatment of patients with adult Hirschsprung's disease. In our cases, the functional results of Duhamels' procedure and of a proctosigmoidectomy with coloanal anastomosis were satisfactory.