Paediatric intestinal pseudo-obstruction: a scoping review

Abstract

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

Enteric Neuromyopathies: Highlights on Genetic Mechanisms Underlying Chronic Intestinal Pseudo-Obstruction

“…After the acute sub-obstructive phases, during the inter-crisis period, enteral or parenteral nutrition is often required to prevent extensive weight loss and malnutrition [ 6 ]. In addition to adult cases, children can also be affected by intestinal pseudo-obstruction (hence, pediatric intestinal pseudo-obstruction—PIPO), presenting a clinical picture conferring high morbidity and mortality risks [ 7 , 8 ]. As in adult patients, the frequent manifestations and complications observed in PIPO involve chronic pain, small intestine bacterial overgrowth (related to gut dysfunction), and the occurrence of mid-gut or colonic (sigmoid) volvulus.…”

Pediatric Intestinal Pseudo-Obstruction: An International Survey on Diagnostic and Management Strategies in the European Reference Network for Rare Inherited and Congenital Anomalies Intestinal Failure Teams

“…PIPO is a severe form of rare intestinal dysmotility disorders leading to IF and is related to significant morbidity and mortality ( 10 ). The rarity of the disease, a wide variety of subtypes and heterogeneity within PIPO patient group makes the management even more challenging ( 2 , 7 ). There is an existing guideline on PIPO management, including basis for multidisciplinary nutritional, medical, and surgical management while at the same time there is still lack of evidence ( 2 ).…”

“…management is optimized IF treatment while avoiding complications and unnecessary operations (5,7). Furthermore, timely consideration for intestinal transplantation, the only curative treatment option for the majority, should be part of PIPO management strategies (2,5,7,8).…”

“…The treatment of PIPO is challenging, associated with significant life-long morbidity and mortality and requires specialized multidisciplinary management ( 2 , 6 ). The goal of PIPO management is optimized IF treatment while avoiding complications and unnecessary operations ( 5 , 7 ). Furthermore, timely consideration for intestinal transplantation, the only curative treatment option for the majority, should be part of PIPO management strategies ( 2 , 5 , 7 , 8 ).…”

“…The goal of PIPO management is optimized IF treatment while avoiding complications and unnecessary operations ( 5 , 7 ). Furthermore, timely consideration for intestinal transplantation, the only curative treatment option for the majority, should be part of PIPO management strategies ( 2 , 5 , 7 , 8 ). In an European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) recommendation paper ( 2 ), specific diagnostic criteria and structured management approaches have been presented for PIPO while evidence behind the guidelines is still scarce.…”