Hirschsprung's disease patients diagnosed at over 15 years of age: An analysis of a Japanese nationwide survey

Purpose: Hirschsprung's disease (HD) is usually diagnosed in patients who are under 1 year of age, however, there are still several reports of adult HD cases. We herein analyzed the data of HD patients collected over 30 years according to a nationwide survey in Japan. Methods: The data of HD patients over 15 years of age were thus selected in three phases, namely from 1978 to 1982, from 1988 to 1992, and from 1998 to 2002. A total of 27 patients (0.7%) out of 3,852 were thus analyzed. Results: The male/female was 15/11. The age at diagnosis was as follows: 10 patients were teenagers (37.0%), 14 patients were in their 20s (51.9%), 1 patient was in his 40s (3.7%), and 2 patients were in their 50s (7.4%). The extent of aganglionosis was as follows; the lower rectum: 12 patients (44.4%), the sigmoid colon: 14 patients (51.9%), and the descending colon 1 patient. As a definitive operation, the Duhamel's procedure including Z-shaped anastomosis was performed on 14 patients (51.9%), Swenson's procedure on 5 patients (18.5%), Soave's procedure on 2 patients (7.4%), and Myectomies on 2 patients (7.4%). No mortalities were reported among these cases. Conclusions: Twenty-seven HD patients diagnosed over 15 years of age were analyzed. The number of patient diagnosed over 15 years of age has decreased over time. However, further attention is still required in adult patients who are present with persistent intestinal obstruction of unknown etiology.