Nikhil Thapar1,2, Efstratios Saliakellis1, Marc A Benninga3, Osvaldo Borrelli1, Joe Curry4, Christophe Faure5, Roberto De Giorgio6, Girish Gupte7, Charles H Knowles8, Annamaria Staiano9, Yvan Vandenplas10, Carlo Di Lorenzo11. 1. Neurogastroenterology and Motility Unit, Department of Paediatric Gastroenterology, Great Ormond Street Hospital NHS Foundation Trust. 2. Stem Cells and Regenerative Medicine, UCL Great Ormond Street Institute of Child Health, London, United Kingdom. 3. Emma Children's Hospital, Academic Medical Centre, Amsterdam, the Netherlands. 4. Neonatal and Paediatric Surgery Department, Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom. 5. Division of Pediatric Gastroenterology, Sainte-Justine Hospital, Université de Montréal, Montréal, Quebac, Canada. 6. Department of Medical and Surgical Sciences, Centro di Ricerca Biomedica Applicata (C.R.B.A.), St Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy. 7. Liver Unit, Birmingham Children's Hospital NHS Foundation Trust, Steelhouse Lane, Birmingham. 8. Barts and the London School of Medicine and Dentistry, Queen Mary University, London, UK. 9. Department of Translational Medical Science, Section of Pediatrics, University of Naples "Federico II," Naples, Italy. 10. Department of Paediatrics, UZ Brussel, Vrije Unversiteit Brussel, Brussels, Belgium. 11. Division of Pediatric Gastroenterology, Nationwide Children's Hospital, Columbus, OH.
Abstract
OBJECTIVES: Chronic intestinal pseudo-obstructive (CIPO) conditions are considered the most severe disorders of gut motility. They continue to present significant challenges in clinical care despite considerable recent progress in our understanding of pathophysiology, resulting in unacceptable levels of morbidity and mortality. Major contributors to the disappointing lack of progress in paediatric CIPO include a dearth of clarity and uniformity across all aspects of clinical care from definition and diagnosis to management. In order to assist medical care providers in identifying, evaluating, and managing children with CIPO, experts in this condition within the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition as well as selected external experts, were charged with the task of developing a uniform document of evidence- and consensus-based recommendations. METHODS: Ten clinically relevant questions addressing terminology, diagnostic, therapeutic, and prognostic topics were formulated. A systematic literature search was performed from inception to June 2017 using a number of established electronic databases as well as repositories. The approach of the Grading of Recommendations Assessment, Development and Evaluation (GRADE) was applied to evaluate outcome measures for the research questions. Levels of evidence and quality of evidence were assessed using the classification system of the Oxford Centre for Evidence-Based Medicine (diagnosis) and the GRADE system (treatment). Each of the recommendations were discussed, finalized, and voted upon using the nominal voting technique to obtain consensus. RESULTS: This evidence- and consensus-based position paper provides recommendations specifically for chronic intestinal pseudo-obstruction in infants and children. It proposes these be termed paediatric intestinal pseudo-obstructive (PIPO) disorders to distinguish them from adult onset CIPO. The manuscript provides guidance on the diagnosis, evaluation, and treatment of children with PIPO in an effort to standardise the quality of clinical care and improve short- and long-term outcomes. Key recommendations include the development of specific diagnostic criteria for PIPO, red flags to alert clinicians to the diagnosis and guidance on the use of available investigative modalities. The group advocates early collaboration with expert centres where structured diagnosis and management is guided by a multi-disciplinary team, and include targeted nutritional, medical, and surgical interventions as well as transition to adult services. CONCLUSIONS: This document is intended to be used in daily practice from the time of first presentation and definitive diagnosis PIPO through to the complex management and treatment interventions such as intestinal transplantation. Significant challenges remain to be addressed through collaborative clinical and research interactions.
OBJECTIVES: Chronic intestinal pseudo-obstructive (CIPO) conditions are considered the most severe disorders of gut motility. They continue to present significant challenges in clinical care despite considerable recent progress in our understanding of pathophysiology, resulting in unacceptable levels of morbidity and mortality. Major contributors to the disappointing lack of progress in paediatric CIPO include a dearth of clarity and uniformity across all aspects of clinical care from definition and diagnosis to management. In order to assist medical care providers in identifying, evaluating, and managing children with CIPO, experts in this condition within the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition as well as selected external experts, were charged with the task of developing a uniform document of evidence- and consensus-based recommendations. METHODS: Ten clinically relevant questions addressing terminology, diagnostic, therapeutic, and prognostic topics were formulated. A systematic literature search was performed from inception to June 2017 using a number of established electronic databases as well as repositories. The approach of the Grading of Recommendations Assessment, Development and Evaluation (GRADE) was applied to evaluate outcome measures for the research questions. Levels of evidence and quality of evidence were assessed using the classification system of the Oxford Centre for Evidence-Based Medicine (diagnosis) and the GRADE system (treatment). Each of the recommendations were discussed, finalized, and voted upon using the nominal voting technique to obtain consensus. RESULTS: This evidence- and consensus-based position paper provides recommendations specifically for chronic intestinal pseudo-obstruction in infants and children. It proposes these be termed paediatric intestinal pseudo-obstructive (PIPO) disorders to distinguish them from adult onset CIPO. The manuscript provides guidance on the diagnosis, evaluation, and treatment of children with PIPO in an effort to standardise the quality of clinical care and improve short- and long-term outcomes. Key recommendations include the development of specific diagnostic criteria for PIPO, red flags to alert clinicians to the diagnosis and guidance on the use of available investigative modalities. The group advocates early collaboration with expert centres where structured diagnosis and management is guided by a multi-disciplinary team, and include targeted nutritional, medical, and surgical interventions as well as transition to adult services. CONCLUSIONS: This document is intended to be used in daily practice from the time of first presentation and definitive diagnosis PIPO through to the complex management and treatment interventions such as intestinal transplantation. Significant challenges remain to be addressed through collaborative clinical and research interactions.
Authors: Lusine Ambartsumyan; Julie Khlevner; Samuel Nurko; Rachel Rosen; Ajay Kaul; John E Pandolfino; Elyanne Ratcliffe; Desale Yacob; B U K Li; Jaya Punati; Manu Sood; Satish S C Rao; Marc A Levitt; Jose T Cocjin; Leonel Rodriguez; Alejandro Flores; John M Rosen; Jaime Belkind-Gerson; Miguel Saps; Jose M Garza; John E Fortunato; Rose L Schroedl; Laurie A Keefer; Joel Friedlander; Robert O Heuckeroth; Meenakshi Rao; Khalil El-Chammas; Karla Vaz; Bruno P Chumpitazi; Rina Sanghavi; Sravan K R Matta; Tanaz Danialifar; Carlo Di Lorenzo; Anil Darbari Journal: J Pediatr Gastroenterol Nutr Date: 2020-08 Impact factor: 2.839
Authors: Giovanni Di Nardo; Federica Viscogliosi; Francesco Esposito; Vincenzo Stanghellini; Maria Pia Villa; Pasquale Parisi; Alessia Morlando; Girolamo Caló; Roberto De Giorgio Journal: J Neurogastroenterol Motil Date: 2019-10-30 Impact factor: 4.924