We experienced a rear case of Crohn's Disease following Hirschsprung disease.
A 27-year-old woman who had undergone a Duhamel-Okamoto's operation for Hirschsprung disease when she was an infant had a right lower abdominal pain. Laparotomy was performed at another hospital with a diagnosis of acute appendicitis. During the operation, stenosis and perforation of the terminal ileum into the abdominal wall were confirmed and the ileocecum was also resected. Pathological study of the resected specimen revealed granuloma. The patient was diagnosed as Crohn's disease and had been managed by ED therapy, but developed abdominal pain and vomiting. She was referred to the department of internal medicine in our hospital. Under a diagnosis of intestinal obstruction, nutrition management with TPN as well as insertion of a long tube was conducted. Symptomatic remissioin was attained, but ileus symptoms recurred when the long tube was removed. So she was transferred to the depertment and underwent a subtotal colectomy and ilostomy because the anal function was poor. The resected specimen revealed a severe adhesion in the sigmoid colon which caused intestinal obstruction. This case of Hirschsprung disease associated with Crohn's disease is very rare. In the best of our knowledge, only another case has been reported in the Japanese literature so far.