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  • Title: Pediatric chronic intestinal pseudo-obstruction is a rare, serious, and intractable disease: a report of a nationwide survey in Japan.
    Author: Muto M, Matsufuji H, Tomomasa T, Nakajima A, Kawahara H, Ida S, Ushijima K, Kubota A, Mushiake S, Taguchi T.
    Journal: J Pediatr Surg; 2014 Dec; 49(12):1799-803. PubMed ID: 25487487.
    Abstract:
    BACKGROUND/PURPOSE: A nationwide survey was conducted to identify the clinical presentation of pediatric chronic intestinal pseudo-obstruction (CIPO) in Japan. METHODS: Data were collected via a questionnaire, ensuring patient anonymity, from facilities that treat pediatric gastrointestinal diseases in Japan. RESULTS: Ninety-two responses were collected from forty-seven facilities. Sixty-two patients (28 males, 34 females) met formal diagnostic criteria for CIPO. The estimated pediatric prevalence was 3.7 in 1 million individuals. More than half the children (56.5%) developed CIPO in the neonatal period. Full-thickness intestinal specimens were available for histopathology assessment in forty-five patients (72.6%). Forty-one (91.1%) had no pathological abnormalities and were considered to be idiopathic. Patients were treated according to the local protocol of each facility. Forty-one patients (66.1%) had restricted oral intake of ordinary diets, and twenty-nine (46.8%) depended on parenteral nutrition. No therapeutic intervention, including medication and surgery, successfully improved oral food intake or obstructive symptoms. Only three patients (4.8%) died from enteritis or sepsis. CONCLUSIONS: In Japan, pediatric CIPO is a rare, serious, and intractable disease. The prognosis with respect to survival is good, but unsatisfactory because of the need for prolonged parenteral nutrition and associated potential for restricted quality of life.
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