UITNODIGINGUITNODIGING VoorVoor het bijwonen het bijwonen van devan openbare de openbare verdedigingverdediging van hetvan proefschrift het proefschrift FUNCTIONALFUNCTIONAL DEFECATION DEFECATION DISORDERSDISORDERS IN CHILDRENIN CHILDREN novelnovel insights insights into into epidemiology, epidemiology, evaluationevaluation and and management management doordoor FUNCTIONAL DEFECATION DISORDERS IN CHILDREN DISORDERS DEFECATION FUNCTIONAL IN CHILDREN DISORDERS DEFECATION FUNCTIONAL ILANILAN JASPER JASPER NADER NADER KOPPEN KOPPEN VrijdagVrijdag 6 april 6 april 2018 2018 om 12.00om 12.00 uur uur FUNCTIONALFUNCTIONAL DEFECATION DEFECATION in dein Agnietenkapel de Agnietenkapel OudezijdsOudezijds Voorburgwal Voorburgwal 229-231 229-231 DISORDERSDISORDERS IN IN CHILDREN CHILDREN AmsterdamAmsterdam novelnovel insights insights into into epidemiology, epidemiology, Na afloopNa afloop bent bentu van u vanharte harte uitgenodigd uitgenodigd voorvoor de receptie de receptie ter plaatse. ter plaatse. evaluationevaluation and and management management PARANIMFENPARANIMFEN Dan DanDori Dori 06-1033595406-10335954 MischaMischa Hoogeveen Hoogeveen 06-4153085906-41530859 ILAN J.N. KOPPEN ILAN J.N. KOPPEN ILAN J.N. ILANILAN KOPPEN KOPPEN [email protected]@amc.nl 06-1984066006-19840660 ILANILAN J.N. J.N. KOPPEN KOPPEN 14781-koppen-cover.indd14781-koppen-cover.indd 1 1 19/02/201819/02/2018 12:18 12:18 FUNCTIONAL DEFECATION DISORDERS IN CHILDREN novel insights into epidemiology, evaluation and management Ilan J.N. Koppen 14781-koppen-layout.indd 1 19/02/2018 21:53 Part of the research in this thesis was funded by The Royal Netherlands Academy of Arts and Sciences (Academy Ter Meulen Grant) and the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (Charlotte Anderson Travel Award). The printing of this thesis was financially supported by: Universiteit van Amsterdam, Nederlandse Vereniging voor Gastroenterologie, Chipsoft, Coloplast B.V., Hero Benelux, Ipsen Farmaceutica B.V., Mead Johnson B.V., Medical Measurement Systems B.V., Norgine B.V., Nutricia Early Life Nutrition, Solgar Vitamins Holland, Teva Nederland B.V., Wellspect HealthCare, Winclove B.V., Yakult Nederland B.V. Layout & cover design: Design Your Thesis | www.designyourthesis.com Printing: Ridderprint B.V. | www.ridderprint.nl ISBN: 978-94-6299-907-7 Copyright © 2018 by Ilan J.N. Koppen. All rights reserved. Any unauthorized reprint or use of this material is prohibited. No part of this thesis may be reproduced, stored or transmitted in any form or by any means, without written permission of the author or, when appropriate, of the publishers of the publications. 14781-koppen-layout.indd 2 19/02/2018 21:53 FUNCTIONAL DEFECATION DISORDERS IN CHILDREN novel insights into epidemiology, evaluation and management ACADEMISCH PROEFSCHRIFT ter verkrijging van de graad van doctor aan de Universiteit van Amsterdam op gezag van de Rector Magnificus prof. dr. ir. K.I.J. Maex ten overstaan van een door het College voor Promoties ingestelde commissie, in het openbaar te verdedigen in de Agnietenkapel op vrijdag 6 april 2018, te 12.00 uur door Ilan Jasper Nader Koppen geboren te Amsterdam 14781-koppen-layout.indd 3 19/02/2018 21:53 PROMOTIECOMMISSIE: Promotores: Prof. dr. M.A. Benninga AMC-UvA Prof. dr. C. Di Lorenzo The Ohio State University Overige leden: Dr. D.K. Bosman AMC-UvA Prof. dr. A.J.P.M. Smout AMC-UvA Prof. dr. H.C.P.M. van Weert AMC-UvA Prof. dr. E.H.H.M. Rings Erasmus Universiteit Rotterdam Prof. dr. C.J. Fijnvandraat AMC-UvA Prof. dr. I. de Blaauw Radboud Universiteit Nijmegen Faculteit der Geneeskunde 14781-koppen-layout.indd 4 19/02/2018 21:53 Wonders of the Human Body “How Do It Know?” There’s an intestinal structure that is not very famous But deserves great credit, and that’s the human anus. It practices active, but discrete discrimination In the never-ending process of waste elimination. And due to its location it receives adverse attention In the continuing duty of thoughtful retention. Scientists proclaimed matter’s form in three types of status But already the anus could differentiate: firm, fluid, or flatus But “how do it know” which substance to safely pass When confronted with solid, liquid, or gas? And fortunately “it do know” and doesn’t need to think As it completes each task with a self-satisfied wink! Wallace D. Brown Journal of Pediatric Gastroenterology and Nutrition 1995;20(4):481 Permission for reuse granted by Wolters Kluwer Health, Inc. 14781-koppen-layout.indd 5 19/02/2018 21:53 TABLE OF CONTENTS General introduction and outline of the thesis 9 PART I EPIDEMIOLOGY Chapter 1 Prevalence of functional defecation disorders in children; a 31 systematic review and meta-analysis Chapter 2 Is there an association between functional constipation and 61 excessive bodyweight in children? Chapter 3 Functional defecation disorders and excessive body weight: a 73 systematic review Chapter 4 Attention deficit hyperactivity disorder and functional defecation 97 disorders in children PART II EVALUATION Chapter 5 Using the Bristol Stool Scale and parental report of stool 115 consistency as part of the Rome III criteria for functional constipation in infants and toddlers Chapter 6 The Brussels Infant and Toddler Stool Scale ('BITSS'): a study on 129 inter-rater reliability Chapter 7 Characterizing colonic motility in children with chronic intractable 149 constipation: a look beyond high-amplitude propagating sequences Chapter 8 Impaired postprandial colonic response in the presence of 175 coordinated propagating colonic contractions suggests an extrinsic neuropathy in children and adolescents with intractable functional constipation Chapter 9 Segmental colonic dilation is associated with premature 193 termination of high-amplitude propagating contractions in children with intractable functional constipation 14781-koppen-layout.indd 6 19/02/2018 21:53 PART III MEDICAL MANAGEMENT Chapter 10 Awareness and implementation of the 2014 ESPGHAN/ 213 NASPGHAN guideline for childhood functional constipation Chapter 11 Adherence to polyethylene glycol treatment in children with 227 functional constipation is associated with parental illness perceptions, satisfaction with treatment and perceived treatment convenience Chapter 12 Is there a role for pre-, pro- and synbiotics in the treatment of 247 functional constipation in children? A systematic review Chapter 13 Transanal irrigation in the treatment of children with intractable 265 functional constipation PART IV SURGICAL MANAGEMENT Chapter 14 Surgical decision-making in the management of children with 279 intractable functional constipation: what are we doing and are we doing it right? Chapter 15 Sacral nerve stimulation for constipation and fecal incontinence 299 in children: long-term outcomes, patient benefit, and parent satisfaction Chapter 16 Surgical management of children with intractable functional 315 constipation; experience of a single tertiary children's hospital Summary and discussion 335 Nederlandse samenvatting en discussie 347 Appendices Contributing authors 359 List of publications 363 PhD portfolio 369 Dankwoord 373 About the author 379 14781-koppen-layout.indd 7 19/02/2018 21:53 14781-koppen-layout.indd 8 19/02/2018 21:53 GENERAL INTRODUCTION AND OUTLINE OF THE THESIS Parts of this introduction have been published as: Childhood constipation: finally something is moving! Ilan J.N. Koppen, Carlo Di Lorenzo, Miguel Saps, Phil G. Dinning, Desale Yacob, Marc A. Levitt, Marc A. Benninga Expert Review of Gastroenterology & Hepatology 2016;10:141-55 & Chapter: Functional Constipation in Children Ilan J.N. Koppen, Marc A. Benninga In: Faure C., Thapar N., Di Lorenzo C. (eds) Pediatric Neurogastroenterology pp.445-458 (2017) Springer, New York. 14781-koppen-layout.indd 9 19/02/2018 21:53 14781-koppen-layout.indd 10 19/02/2018 21:53 General introduction and outline of the thesis PHYSIOLOGY OF DEFECATION The dynamics of defecation are complex and rely on intricate processes involving the autonomic and somatic nervous system, pelvic floor muscles, and internal and external anal sphincters. After passing the stomach, duodenum, jejunum and ileum, chyme is transported from the terminal ileum into the caecum. Subsequently, luminal contents are moved distally through the colon while gradual desiccation and mixing occurs, resulting in progressively solid fecal material.1 The propulsion of feces is achieved by complex colonic motor patterns. Several different colonic motor patterns have been described, with the most well recognized being high-amplitude propagating contractions2,3, which are associated with the mass movement of colonic contents and spontaneous defecation in healthy adults.4,5 Anterograde propagation of feces through the colon leads to filling of the rectum, which induces relaxation of the internal anal sphincter, allowing feces to travel further down the anal canal; this reflex is known as the recto-anal inhibitory reflex. Subsequently, sensory stimuli triggered by rectal distension and by the contact between fecal material and the mucosa of the proximal part of the anal canal result in an urge to defecate. At this point, voluntary contraction of the external anal sphincter and pelvic floor muscles can postpone defecation, by moving the fecal load higher up in the rectum, until place and time are appropriate for defection. When defecation is initiated, voluntary relaxation of the external anal sphincter and the pelvic floor musculature (i.e. the puborectalis muscle and levator ani) allows