Abstract
Chronic intestinal pseudo-obstruction (CIPO) is an extremely rare condition with symptoms of recurrent intestinal obstruction without any lesions. The outcomes of pediatric CIPO and predictors for the outcomes have not yet been well established. We analyzed the clinical outcomes and associated factors for the outcomes of pediatric CIPO. We retrospectively reviewed 66 primary CIPO patients diagnosed between January 1985 and December 2017. We evaluated parenteral nutrition (PN) factors such as PN duration, PN use over 6 months, home PN, and mortality as outcomes. We selected onset age, presence of urologic symptoms, pathologic type, and involvement extent as predictors. The early-onset CIPO was found in 63.6%, and 21.2% of the patients presenting with urologic symptoms. Of the 66 patients, 47 and 11 had neuropathy and myopathy, respectively. The generalized involvement type accounted for 83.3% of the cases. At the last follow-up, 24.2% of the patients required home PN management. The mean duration of PN was 11.8 ± 21.0 months. The overall mortality rate of primary CIPO was 18.2%. PN factors were predicted by the urologic symptoms and extent of involvement. However, mortality was predicted by pathologic type. The onset age was not significantly associated with the outcomes. CIPO with urologic symptoms and generalized CIPO had poor PN outcomes. Myopathy is suggested as a predictor of mortality in children with primary CIPO.
Highlights
Chronic intestinal pseudo-obstruction (CIPO) was first reported in 1958, and pediatric intestinal pseudo-obstruction (PIPO) was first reported in a case series of 11 children in1977 [1]
In the case of primary CIPO, neuropathy, myopathy or mesenchymopathy are shown as an abnormality in the enteric nervous system, not as a symptom of pseudo-obstruction caused by an underlying disease
Clinical outcomes were evaluated using mortality and parenteral nutrition (PN) factors, which included PN duration, PN use over 6 months, and need for home PN
Summary
Chronic intestinal pseudo-obstruction (CIPO) was first reported in 1958, and pediatric intestinal pseudo-obstruction (PIPO) was first reported in a case series of 11 children in1977 [1]. Chronic intestinal pseudo-obstruction (CIPO) was first reported in 1958, and pediatric intestinal pseudo-obstruction (PIPO) was first reported in a case series of 11 children in. When the patients show severe obstructive symptoms without any mechanical obstruction, we can suspect the possibility of CIPO. The diagnostic criteria for CIPOs varied in previous studies [2,3]. CIPO is divided into primary and secondary CIPOs. In the case of primary CIPO, neuropathy, myopathy or mesenchymopathy are shown as an abnormality in the enteric nervous system, not as a symptom of pseudo-obstruction caused by an underlying disease. Pathogenesis of enteropathy is still not clearly established [4]
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