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Published on: March 5, 2018
Clinical Outcomes of Pediatric Chronic Intestinal Pseudo-Obstruction
Dayoung Ko1, Hee-Beom Yang2, Joong Youn1
1Department of Pediatric Surgery, Seoul National University Children's Hospital, Seoul 03080, Korea.
Insights
Pediatric chronic intestinal pseudo-obstruction (CIPO) often presents early and generalized. Urologic symptoms and disease extent predict poor parenteral nutrition (PN) outcomes, while myopathy predicts mortality in these rare cases.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Motility Disorders
- Rare Diseases
Background:
- Chronic intestinal pseudo-obstruction (CIPO) is a rare condition mimicking intestinal obstruction without mechanical blockage.
- Outcomes and predictors for pediatric CIPO remain poorly understood.
- This study focuses on clinical outcomes and associated factors in primary pediatric CIPO.
Purpose of the Study:
- To analyze clinical outcomes in pediatric CIPO patients.
- To identify predictors for parenteral nutrition (PN) dependence and mortality.
- To establish a better understanding of CIPO's impact on children.
Main Methods:
- Retrospective review of 66 primary CIPO patients diagnosed between 1985 and 2017.
- Evaluation of PN duration, long-term PN use, home PN, and mortality as outcomes.
- Analysis of onset age, urologic symptoms, pathologic type, and involvement extent as predictors.
Main Results:
- Early onset (63.6%) and generalized involvement (83.3%) were common.
- Urologic symptoms (21.2%) and generalized CIPO correlated with poor PN outcomes.
- Myopathy (11 patients) and specific pathologic types predicted mortality (18.2% overall).
Conclusions:
- Pediatric CIPO frequently presents early and generalized, often requiring long-term PN.
- Urologic symptoms and disease extent are key factors influencing PN outcomes.
- Pathologic type, particularly myopathy, is a significant predictor of mortality in pediatric CIPO.
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.
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