The Exstrophy-Epispadias Complex. Research Concepts and Clinical Applications

Transcription

1 The Exstrophy-Epispadias Complex Research Concepts and Clinical Applications

2 The Exstrophy-Epispadias Complex Research Concepts and Clinical Applications Edited by John P. Gearhart Johns Hopkins Medical Institutions Baltimore, Maryland and Ranjiv Mathews SUNY Health Science Center at Syracuse Syracuse, New York Springer Science+ Business Media, LLC

3 LIbrary of Congress Cataloglng-In-Publlcatlon Data The exstrophy-epispadias coeplex : research concepts and clinical applications I edited by John P. Gearhart and Ranjlv Mathews. p. ce. "ProceedIngs of the FIrst InternatIonal Syeposlue on Exstrophy and EpIspadIas. held April In Baltleore. Maryland"--T.p. verso. Includes bibliographical references and Index. ISBN ISBN (ebook) DOI / I. Bladder exstrophy Congresses. 2. EpIspadIas Congresses. I. Gearhart. John P. II. Mathews. Ranjlv. III. InternatIonal Syeposlue on Exstrophy and EpIspadIas (1st: 1997 : Baltleore. Md.) [DNLM: I. Bladder Exstrophy--surgery Congresses. 2. Eplspadlas -surgery Congresses. a. ReconstructIve SurgIcal Procedures Congresses. WJ 500 E R05BO.E '62'00B3--dc21 DllLM/DLC for LIbrary of Congress CIP Cover figure from, Canning, D. A., Gearhart, J. P., Peppas, D. S., and Jeffs, R. D., "The cephalotrigonal reimplant in bladder neck reconstruction for patients with exstrophy or epispadias," Journal of Urology. ISO: 156, Proceedings of the First International Symposium on Exstrophy and Epispadias. held April 9-10, 1997, in Baltimore, Maryland ISBN Springer Science+Business Media New York Originally published by Kluwer Academic/Plenum Publishers in 1999 Softcover reprint of the hardcover 1st edition 1999 ill I A C.I.P. record for this book is available from the Library of Congress. All rights reserved No part of this book may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, microfilming, recording. or otherwise, without written permission from the Publisher

4 DEDICATION This book is dedicated to the following people: Madison Pryor, Ph.D. who taught me that the study of biology is the basis of medicine; to Lonnie W. Howerton, M.D. who taught me that urology is the pinnacle of surgical sub-specialities; to Hiram C. Polk, M.D. who taught me to ask questions, and more importantly to find answers; to Roy Witherington, M.D. who taught me the skills and science of urology; to Mr. J. Herbert Johnson, MB, FRCS, who taught me the craft of pediatric urology; to Robert D. Jeffs, M.D. who taught me the skills and science of reconstructive pediatric urology; and to Patrick C. Walsh, M.D. for his mentorship and constant dedication to discovery and achievement. v

5 PREFACE The undertaking of the treatment of an infant born with bladder exstrophy is one of the most weighty responsibilities that can fall upon the shoulders of the reconstructive surgeon. The modern treatment of a child born with bladder exstrophy began in the mid- 1970's with the widespread application of staged reconstruction. This approach has consistently yielded very good results in several series. However, as in all serious congenital birth defects, there is certainly room for advancement. Issues such as the routine use of osteotomy, timing and type of epispadias repair, combining bladder closure with epispadias repair, the approach to the small bladder, and the management of a failed exstrophy still remain. This National Institute of Health/National Kidney Foundation/Johns Hopkins-sponsored seminar was an attempt to bring experts in the field of pediatric orthopedic surgery, pediatric urology, pediatric surgery, adult urology, and basic science together to share their experiences in an attempt to foster new clinical and basic science research communications between the participants. If these collaborations result, then this first international meeting will have been successful. The editors would like to thank all of the contributors for their timely and complete submissions. John P. Gearhart, M.D. Ranjiv Mathews, M.D. vii

6 CONTENTS 1. The Embryology and Epidemiology of Bladder Exstrophy.... Bruce Slaughenhoupt 2. Prenatal Diagnosis of Bladder and Cloacal Exstrophy and Related Conditions 5 Roger C. Sanders 3. Anatomy and Biomechanics of the Pelvis in Exstrophy Mihir M. Jani, Paul D. Sponseller, John P. Gearhart, A. Valdevit, and E.Y.-S. Chao 4. Detrusor Morphology and Innervation in Bladder Exstrophy: A Preliminary Investigation J. A. Gosling, R. Mathews, and J.P. Gearhart 5. The Response of the Bladder Smooth Muscle to Increased Bladder Neck Resistance Edward J. Macarak, Stephen Zderic, Howard M. C. Snyder, Douglas Canning, and Pamela S. Howard 6. Muscarinic Cholinergic Receptors in Bladder Exstrophy: Pharmacological Observations Supporting Functional Closure and Subsequent Bladder Neck Reconstruction Ellen Shapiro, Robert D. Jeffs, John P. Gearhart, and Herbert Lepor 7. A Quantitative Histological Analysis of the Bladder in Classical Bladder Exstrophy in Various Stages of Reconstruction Utilizing Color Morphometry Dennis S. Peppas, Marie-Blanche Tchetgen, Benjamin R. Lee, Robert D. Jeffs, and John P. Gearhart 8. Analysis of Smooth Muscle and Collagen Subtypes in the Normal Newborn and Bladder Exstrophy Patient Benjamin R. Lee, Elizabeth J. Perlman, Alan W. Partin, Robert D. Jeffs, and John P. Gearhart ix

7 X Contents 9. A Large Animal Model of Bladder Exstrophy: Observations on Bladder Smooth Muscle and Collagen Content and Function Bruce L. Slaughenhoupt and John P. Gearhart 10. Tissue Engineering Techniques For Closure of Bladder Exstrophy: An Experimental Animal Model Anthony Atala 11. Surgical Principles in the Primary Closure of Bladder Exstrophy John P. Gearhart and Ranjiv Mathews 12. Anterior and Posterior Innominate Osteotomy in Exstrophy Repair Paul D. Sponseller, John P. Gearhart, Mihir M. Jani, and Robert D. Jeffs 13. Bladder Exstrophy Closure without Osteotomy in Philadelphia Douglas A. Canning, Thomas Lanchoney, Simone McKitty, Stephen Zderic, Howard M. Snyder, III, and John W. Duckett, Jr. 14. Bilateral Anterior Pubic Osteotomy in Bladder Exstrophy Closure Peter Frey The Failed Exstrophy Closure: Strategies for Management--the Johns Hopkins Experience John P. Gearhart and Ranjiv Mathews 16. Applications of Ureterosigmoidostomy in Bladder Exstrophy Raimund Stein, Margit Fisch, and Rudolf Hohenfellner 17. Cystoplasty in Bladder Exstrophy: Indications and Techniques Steven G. Docimo 18. Bladder Exstrophy: Therapeutic Alternatives Sami Arap and Amilcar M. Giron Ill 19. Penile Length in Adulthood after Exstrophy Reconstruction Richard I. Silver, Andrew Yang, Jacob Ben-Chaim, Robert D. Jeffs, and John P. Gearhart 20. Complete Penile Disassembly for Epispadias Repair Michael E. Mitchell The Cantweii-Ransley Epispadias Repair in Exstrophy/Epispadias: Lessons Learned John P. Gearhart, Christopher Sciortino, Jacob Ben-Chaim, Dennis S. Peppas, and Robert D. Jeffs 22. The Impact ofurodynamics on Surgery for Incontinence in Children with Exstrophy Stuart B. Bauer

8 Contents xi 23. The Use of Sub-Mucosal Collagen in the Small Bladder Associated with Bladder Exstrophy Paolo Caione and Alberto Lais 24. Review of Long-Term Results of Staged Functional Reconstruction and Bladder Neck Plasty in Bladder Exstrophy Patients Followed from Birth at the Johns Hopkins Hospital Yegappan Lakshmanan, John P. Gearhart, and Robert D. Jeffs 25. Psychological Aspects of Bladder Exstrophy: Vulnerabilities, Risks, and Protective Factors from a Longitudinal Perspective William G. Reiner 26. The Outcome of Patients with Classic Bladder Exstrophy in Adult Life Jacob Ben-Chaim, Robert D. Jeffs, William G. Reiner, and John P. Gearhart 27. Long Term Results of Bladder Exstrophy: The London Experience C. R. J. Woodhouse 28. Long Term Results of Bladder Exstrophy: The Mainz Experience Raimund Stein, Margit Fisch, and Rudolf Hohenfellner Semen Analysis and Transrectal Ultrasound Findings in Men with Bladder Exstrophy Terry W. Hensle, Erik T. Goluboff, and Harry Fisch 30. Anatomy of Cloacal Exstrophy: The Surgical Implications Douglas A. Husmann and David R. Vandersteen Current Urologic Management of Cloacal Exstrophy: Experience with 11 Patients Edwin A. Smith, John R. Woodard, Bruce H. Broecker, Rafael Gosalbez, Jr., and Richard R. Ricketts 32. Indications and Types of Bladder Augmentation in Cloacal Exstrophy Michael E. Mitchell Cloacal Exstrophy: Experience with 41 Cases from W. Hardy Hendren, Robert E. Gross, and Dennis P. Lund Abstracts 237 Index