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Anorectal Malformations Anorect A l m A lform One of the most alluring aspects of the pediatric surgery practice is the long-term follow up of patients with A tions AnorectAl congenital anomalies. It is a good opportunity –and an obligation as well- to actually see the results of your work and to see how the children develop, not only physically but A Multidisciplinary Approach mAlformAtions also psychosocially, and to see how they finally participate in society. Therewith presents the unique opportunity to combine research of short- term outcomes with that of long-term outcomes, even into adulthood. A Multidisciplinary In this thesis, we aimed to optimize care of anorectal malformation patients with a multidisciplinary approach, and pioneered in the long- term follow up of this patient group. Approach Desiree van den Hondel Desiree van den Hondel ANORECTAL MALFORMATIONS A Multidisciplinary Approach Desiree van den Hondel ANORECTAL MALFORMATIONS A Multidisciplinary Approach The printing of this thesis was financially supported by the department of pediatric sur- gery, Erasmus MC - Sophia Children’s Hospital, Rotterdam ANORECTALE MALFORMATIES Een Multidiscipilnaire Benadering PROEFSCHRIFT ter verkrijging van de graad doctor aan de Erasmus Universiteit Rotterdam op gezag van de Rector Magnificus Prof. dr. H.A.P. Pols en volgens besluit van het college voor Promoties. ISBN: 978 94 6169 729 5 De openbare verdediging zal plaatsvinden op Cover: Optima Grafische Communicatie, Rotterdam, the Netherlands woensdag 14 oktober 2015 om 9.30 uur door Lay-out: Desiree van den Hondel Desiree van den Hondel Printed by: Optima Grafische Communicatie, Rotterdam, the Netherlands geboren te Dordrecht © Desiree van den Hondel 2015 All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photoco- pying, recording, or otherwise, without the prior permission in writing from the proprie- tor(s). Promotiecommissie Contents Promotor: Prof.dr. R.M.H. Wijnen Preface and outline of the thesis p7 Overige leden: Prof.dr. D. Tibboel Prof.dr. J.C. Escher Part 1: Neonatal care Prof.dr. L.W.E. van Heurn Chapter 1: Colostomy complications p29 Copromotoren: Dr. H. IJsselstijn Chapter 2: Relevance of upper limb anomalies in syndromal disorders p49 Dr. C.E.J. Sloots Chapter 3: Screening and treatment of tethered cord syndrome p69 Part 2: Long-term outcome Chapter 4: Growth and development until 5 years of age p87 Chapter 5: Neuropsychological functioning at school age p105 Chapter 6: Quality of life in cloacal malformation patients p125 Chapter 7: Sexuality after colorectal surgery p139 Part 3: Discussion and summary Chapter 8: General discussion and recommendations p163 Chapter 9: Summary / Nederlandse samenvatting p191 Part 4: Appendices List of publications p206 Curriculum vitae p207 PhD portfolio p208 Paranimfen: Sanne Hofstede Kitty Snoek Dankwoord p210 Preface and outline of the thesis 10 | P R E F A C E P R E F A C E | 11 Congenital malformations of the anorectum occur in 1 to 3 in every 5000 live borns.1,2 As medical care improved, so did survival. With the introduction of colostomy by Littré In the Netherlands, each year approximately 50 to 60 children are born with an anorec- in 1710 also patients with more severe anorectal malformations had a -slight- chance tal malformation.3 This may not seem much, but these malformations constitute a con- for survival.8 In the 18th century, the German anatomist and surgeon Heister, wrote siderable part of everyday pediatric surgery practice. Anorectal malformations consist a treatise ‘About how should the anus be opened when it is imperforate’ in 1749.7 He of a wide spectrum of anomalies with different functional results, and are therefore recommended to check all newborns for anorectal malformations. He described the fascinating both to treat and to study. operative technique, placing the infant on the knees of an assistant and making two incisions, as if draining an abscess. This technique is remarkably similar to the oper- ative technique described by Sabuncuoglu two centuries before. The present-day Historical background techniques were developed in the 20th century by Dr Browne (V-Y cutback procedure, 1951) and Dr Peña and DeVries (posterior sagittal anorectoplasty, 1982). These tech- The first description of anorectal malformations goes back to the second century AD, niques will be described later. when Soranus, a Greek physician who practiced in Alexandria and subsequently in Rome, was one of the first to describe anorectal malformations in detail.4 Where new- borns with congenital anomalies usually were left to death, Soranus attempted to treat Embryology and definitions neonates with an anorectal malformation by dividing a thin anal membrane.5 Paul of Aegina (625-690 AD) further refined the treatment by dilating the anus with bougies Anorectal malformations are characterized by absence of the anal orifice within the and applying wine and salve after the incision.6 Thereafter, there was stagnation in sphincter complex. In most cases, an ‘ectopic opening’ or fistula is present and the pel- medical care until the fifteenth century, when Sabuncuoglu from Turkey emphasized vic floor is hypoplastic.9 the difference between high and low malformations, referring to the amount of tissue between the skin and the rectum. Fig. 1 shows an illustration of Sabuncuoglu of the In normal embryonic development, the digestive tract is formed from the foregut, the incision for anorectal malformations.7 midgut, and the hindgut. The foregut differentiates into the esophagus, stomach, du- odenum, liver, gall bladder, pancreas, and spleen, while the midgut differentiates into duodenum, jejunum, ileum, cecum with appendix, ascending and transverse colon. Fig. 1: Puncture of anal membrane in the 15th century The hindgut differentiates into the distal third of the transverse colon, descending and sigmoid colon, rectum and anus.10 The hindgut terminates in the endodermally lined cloaca, which is caudally in direct contact with the ectoderm, forming the ‘cloacal membrane’.10 The cloacal membrane reaches dorsally into the tail groove, which is the future anal orifice. During the sixth and seventh weeks, the cloaca is divided into the urogenital sinus and the anorectal tract by a septum of mesodermal tissue, called the urorectal fold. In this process the cloacal membrane is thought to play an important role for orientation, although exact biomechanisms are unclear (Fig. 2a).11 In abnormal development as seen in anorectal malformations, the dorsal cloaca and dorsal part of the cloacal membrane seem to be missing (Fig. 2b).12 As a consequence, the cloacal membrane does not extend to the tail groove. Now, when the anorectal Illustration from Sabuncuoglu (Figure from Yesildag et al.©7) fold develops, which should divide the cloaca into the urogenital sinus and anorec- 12 | P R E F A C E P R E F A C E | 13 Fig. 2: Normal and abnormal development of the hindgut tal tract, a wide variety of anomalies can occur. Molecular mechanisms are still under Wolan duct Wolan duct A B research, but there is evidence that mutations of sonic hedgehog (SHH) and its down- Kidney Kidney stream molecules Gli-2 and Gli-310,13 as well as Wnt inhibitory factor 114 and FGF sign- aling pathways15 have been described to play a role in the pathogenesis of anorectal malformations. Allantois Ureter Allantois Ureter Several classification systems for anorectal malformations have been proposed. The Urorectal fold Urorectal fold Urogenital sinus Urogenital sinus classification system used nowadays, the Krickenbeck classification5 (Table 1) was de- veloped in 2005 and had two main purposes: first to determine what operative tech- Hindgut Cloaca Hindgut Cloaca (anorectal tract) (anorectal tract) nique would be required for the patient, and second to be able to compare long-term follow up studies more precisely. Cloacal Dorsal cloaca membrane missing The most common types of anorectal malformations (Fig. 3) seen in male patients are Tailgroove Dorsal part cloacal perineal fistula (40%) and rectourethral fistula (31%), and in female patients are vestib- (future anal orice) membrane missing ular fistula (57%), perineal fistula (27%) and cloacal malformation (11%).16 The differ- This figure is based on Kluth et al.©12. Schematic drawing of a normal (A) and an abnormal (B) cloaca. In the abnormal embryo, the cloacal membrane is too short because it does not extend to the region of the tail groove (dark grey area). As a consequence, ent types of malformations require different operative techniques and have different the dorsal cloaca is missing. These drawings were based on scanning electron microscopy in 80 mice embryos (SD-mice or prognoses. ‘Danforth’s short tail mice’). Fig. 3: Most prevalent types of anorectal malformations A B In almost all patients, the anorectal malformation is detected postnatally. Only few cases of antenatally detected anorectal malformations have been reported.17-19 These cases were detected by ultrasonography at the end of the first trimester, when liquid Bladder Bladder Rectum Rectum contents of the dilated distal colon were be detected. This may have been the result of swallowed amniotic fluid or urine that had passed the fistula. Urethral stula Urethra Urethra Table 1: Krickenbeck classification for anorectal malformations Perineal Sphincter Sphincter stula complex complex Major clinical groups Rare / regional variants C D E Perineal fistula Pouch colon atresia
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