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. What is Known: • Paediatric
intestinal pseudo-obstruction is a rare, heterogeneous disorder that confers a high rate of morbidity and mortality • Complications of paediatric
intestinal pseudo-obstruction include
chronic pain, small intestine bacterial overgrowth and malrotation. Other complications can occur related to its management, such as line
infections with
parenteral nutrition or cardiac side effects of prokinetic medications What is New: • Progress in medical and surgical
therapy in recent years has led to improved patient outcomes • Enteral autonomy has been reported in most patients at as early as 1 month post-
transplantation.