A review of the reported cases of chronic intestinal pseudo-obstruction in Japan and an investigation of proposed new diagnostic criteria.

Intestinal pseudo-obstruction is a clinical syndrome in which the clinical symptoms of intestinal obstruction appear without mechanical obstruction of the intestine. We searched for articles from Japana Centra Revuo Medicina for the period 1983-2009 using the keywords 'chronic' and 'intestinal pseudo-obstruction'. 124 articles were identified, and of these 121 cases were investigated using our diagnostic criteria. The patients were between 0 (just after birth) and 84 years of age, indicating that chronic intestinal pseudo-obstruction (CIP) can occur at any age. The mean age was 43.6 years and the median age was 47 years. Forty-nine patients were male and 72 were female, showing a slight tendency towards female predominance. Five cases (4.2%) had a definitive family history. Of the identified causes of secondary CIP, systemic sclerosis was the most common. Abdominal bloating was the most common initial symptom, seen in 90 (81%) patients. Patients having poor intestinal peristalsis with stagnation of the contents of the small intestines causing fatty stools and bacterial overgrowth complained of diarrhea. The interval between the initial symptoms and diagnosis ranged from 0 to 60 years, with a mean and median interval of 7.3 and 2 years, respectively. In case reports of CIP in Japan, the sensitivity of our diagnostic criteria was found to be 85.9%, indicating that the criteria are useful. For improvement in the rate of recognition of CIP and practical application of the diagnostic criteria in Japan, it is important to conduct further studies.