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. 2021 May 28;10(11):2376.
doi: 10.3390/jcm10112376.

Clinical Outcomes of Pediatric Chronic Intestinal Pseudo-Obstruction

Dayoung Ko  1 , Hee-Beom Yang  2 , Joong Youn  1 , Hyun-Young Kim  1   3
Affiliations

Clinical Outcomes of Pediatric Chronic Intestinal Pseudo-Obstruction

Dayoung Ko et al. J Clin Med. .

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.

Keywords: chronic intestinal pseudo-obstruction; myopathy; neuropathy; parenteral nutrition; pediatrics.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Diagnostic flow.
Figure 2
Figure 2
Pathologic specimens in chronic intestinal pseudo-obstruction patients were shown. Hematoxylin and eosin staining shows a hypoganglionosis. Arrow indicated the ganglion in the myenteric plexus (a); other slide demonstrates an immature ganglion cell (black triangle) (b); additional muscle layer was identified (circle) (c); muscle cells in the inner circular muscle layer show vacuolization (circle) (d).
Figure 3
Figure 3
Overall survival was shown in this figure. Overall survival rate was 81.8%, and the median follow-up period was 35 months.
See this image and copyright information in PMC

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