Pediatric chronic intestinal pseudo-obstruction is a rare, serious, and intractable disease: A report of a nationwide survey in Japan

Mitsuru Muto, Hiroshi Matsufuji, Takeshi Tomomasa, Atsushi Nakajima, Hisayoshi Kawahara, Shinobu Ida, Kosuke Ushijima, Akio Kubota, Sotaro Mushiake, Tomoaki Taguchi

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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.

Original languageEnglish
Pages (from-to)1799-1803
Number of pages5
JournalJournal of Pediatric Surgery
Volume49
Issue number12
DOIs
Publication statusPublished - Dec 1 2014

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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    Muto, M., Matsufuji, H., Tomomasa, T., Nakajima, A., Kawahara, H., Ida, S., Ushijima, K., Kubota, A., Mushiake, S., & Taguchi, T. (2014). Pediatric chronic intestinal pseudo-obstruction is a rare, serious, and intractable disease: A report of a nationwide survey in Japan. Journal of Pediatric Surgery, 49(12), 1799-1803. https://doi.org/10.1016/j.jpedsurg.2014.09.025