Pediatric Intestinal Pseudo-Obstruction: Progress and Challenges

Abstract

BackgroundChronic intestinal pseudo-obstruction is a rare disorder and represents the most severe form of gastrointestinal dysmotility with significant morbidity and mortality. Emerging research shows considerable differences between the adult and pediatric population with intestinal pseudo-obstruction and the term Pediatric Intestinal Pseudo-Obstruction (PIPO) was recently proposed.PurposeThe aim of this article is to provide pediatric gastroenterologists and pediatricians with an up to date review of the etiology and underlining pathophysiology, clinical features, diagnostic and management approaches currently available for PIPO and to discuss future perspectives for the diagnosis and management of this rare disease.

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS): challenges in diagnosis and management

“…The available limited supportive therapeutic options for MMHIS are prokinetic drugs (cisapride and prucalopride), cholinergics (bethanechol, neostigmine, pyridostigmine), macrolides (erythromycin, azithromycin), clavulanate-amoxicillin, octreotide, dopamine-2 receptor antagonists (domperidone and metoclopramide) and gastrointestinal hormones (gastrin, cholecystokinin and secretin) 12. But none of them is curative.…”

Editorial: eosinófilos en dispepsia funcional, mucoprotectores, motilidad del intestino delgado en el SII y estudios manométricos en pediatría

“…Todo esto contribuye a que esté menos difundida, limitándose a centros de mayores complejidades para estudiar los trastornos de la motilidad gástrica e intestinal. Sin embargo, en la pseudoobstrucción intestinal crónica, la manometría antroduodenal es el estudio con mayor capacidad discriminativa; permite confirmar el diagnóstico, aclarar la fisiopatología y optimizar el manejo clínico 46 . Las recomendaciones actuales para la pseudoobstrucción pediátrica de la European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) indican realizar manometría antroduodenal en todo paciente pediátrico en quien se sospeche este diagnóstico 47 .…”

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

“…In PIPO, diagnostic challenges are common from the initial diagnosis to throughout life appearing recurrent ileus like episodes which often lead to delay in diagnostics, multiple operations, increased morbidity, and poor quality of life ( 5 ). 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 ).…”