Journal
EUROPEAN JOURNAL OF PEDIATRICS
Volume 181, Issue 7, Pages 2619-2632Publisher
SPRINGER
DOI: 10.1007/s00431-021-04365-9
Keywords
Chronic pseudo-obstruction; Intestinal dysmotility; Paediatric; Children
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Funding
- CAUL
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Paediatric intestinal pseudo-obstruction (PIPO) is a rare and challenging disorder to diagnose and treat, with management mainly relying on parenteral nutrition and intestinal transplantation, which may lead to serious complications in the long term.
Paediatric intestinal pseudo-obstruction (PIPO) encompasses a group of rare disorders in which patients present with the clinical features of bowel obstruction in the absence of mechanical occlusion. The management of PIPO presents a challenge as evidence remains limited on available medical and surgical therapy. Parenteral nutrition is often the mainstay of therapy. Long-term therapy may culminate in life-threatening complications including intestinal failure-related liver disease, central line thrombosis and sepsis. Intestinal transplantation remains the only definitive cure in PIPO but is a complex and resource-limited solution associated with its own morbidity and mortality. We conducted a scoping review to present a contemporary summary of the epidemiology, aetiology, pathophysiology, diagnosis, management and complications of PIPO. Conclusion: PIPO represents a rare disorder that is difficult to diagnose and challenging to treat, with significant morbitity and mortality. The only known cure is intestinal transplantation.
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