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KMID : 0356720030190040254
Journal of the Korean Society of Coloproctology
2003 Volume.19 No. 4 p.254 ~ p.259
Hirschsprung¡¯s Disease in Adults
Park In-Ja

Yu Chang-Sik
Yoo Sang-Hwa
Lee Kang-Hong
Kim Hee-Cheol
Kim Jin-Cheon
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.
KEYWORD
Adult Hirschsprung¡¯s disease, Surgical treatment
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