Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5514_Библиотеки_им_академика_М_И_Перельмана.pdf
Bladder
Exstrophy and
Epispadias
ACompendium
Aseem R. Shukla · Rakesh S. Joshi
Editors-in-Chief
Joao Luiz Pippi Salle · Pramod P. Reddy
Dana A. Weiss · Paul A. Merguerian
Jaishri Ramji
Associate Editors
Bladder Exstrophy and Epispadias
Aseem R. Shukla • Rakesh S. Joshi
Editors-in-Chief
Joao Luiz PippiSalle • Pramod P. Reddy
Dana A. Weiss • Paul A. Merguerian
Jaishri Ramji
Associate Editors
Bladder Exstrophy
and Epispadias
A Compendium
Editors-in-Chief
AseemR.Shukla, MD, MHCI
Division of Urology
Children’s Hospital of Philadelphia
Philadelphia,PA,USA
Perelman School of Medicine
University of Pennsylvania
Philadelphia,PA,USA
RakeshS.Joshi, MCh, DNB
Department of Paediatric Surgery
B. J. Medical College and Civil Hospital
Ahmedabad,Gujarat,India
Associate Editors
Joao Luiz PippiSalle, MD
Division of Urology
SickKids Hospital and Co-Director of the
Center for Pelvic Reconstruction
Pramod P. Reddy, MD
Burnet Campus
Cincinnati Children's
Cincinnati, OH, USA
Toronto, Canada
Paul A. Merguerian, MD
Dana A. Weiss, MD
Division of Urology
Childrens Hospital of Philadelphia
Division of Urology
Seattle Children's Hospital
Seattle, WA, USA
Philadelphia, PA, USA
Jaishri Ramji, MCh
Department of Paediatric Surgery
Ahmedabad Civil Hospital
Ahmedabad, India
ISBN 978-3-031-91237-5 ISBN 978-3-031-91238-2 (eBook)
https://doi.org/10.1007/978-3-031-91238-2
© The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature
Switzerland AG 2025
This work is subject to copyright. All rights are solely and exclusively licensed by the Publisher, whether
the whole or part of the material is concerned, specically the rights of translation, reprinting, reuse of
illustrations, recitation, broadcasting, reproduction on microlms or in any other physical way, and
transmission or information storage and retrieval, electronic adaptation, computer software, or by similar
or dissimilar methodology now known or hereafter developed.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication
does not imply, even in the absence of a specic statement, that such names are exempt from the relevant
protective laws and regulations and therefore free for general use.
The publisher, the authors and the editors are safe to assume that the advice and information in this book
are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the
editors give a warranty, expressed or implied, with respect to the material contained herein or for any
errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional
claims in published maps and institutional afliations.
This Springer imprint is published by the registered company Springer Nature Switzerland AG
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
If disposing of this product, please recycle the paper.
To the courageous children affected by
bladder exstrophy and epispadias, whose
indefatigable spirit, determination, and hope
compel us to do better by them each day.
To Douglas Canning, Richard Grady, and
Atul Thakre, our mentors lost too soon,
whose exemplary lives of selessness, grit,
and love inspire us every day to be better.
Foreword
My fascination with congenital anomalies dates back to my time as a bright-eyed
medical student and the correlation of how a seemingly small embryological miscue
could lead to a major anatomical defect. The excitement of restoring form and function as I rotated on the surgical services propelled me to a career in urology and
perhaps no clearer example between embryological maldevelopment and eventual
anatomic anomaly exists than with the exstrophy-epispadias complex.
Driven to pursue reconstructive pediatric urology, I was fortunate to learn from
Sir Herbert Johnston, one-half of the duo that founded the specialty of pediatric
urology in the United Kingdom, at Alder Hey Children’s Hospital in Liverpool,
England, the rst center to successfully close a child with cloacal exstrophy, and
have him survive, in 1965. After completing additional training in pediatric surgical
techniques, I truly believed I would return to Georgia and begin practice in pediatric
urology; however, as fate—or a serendipitous plan by Robert Jeffs and Patrick
C.Walsh—my plan and subsequent career was rerouted to Baltimore, MD.
I arrived at Johns Hopkins on February 1, 1985, and was fortunate to benet
from the knowledge and mentorship Bob (a pioneering surgeon and a founding
father of pediatric urology in North America) offered in complex congenital genitourinary disorders. Though care for exstrophy at Johns Hopkins dated back to the
1920s with Hugh Hampton Young, Bob was the rst to really establish a dedicated
unit of excellence at Johns Hopkins specically, and only, designated for children
with exstrophy or epispadias. While he had established his multi-staged approach in
the 1970s, with this now high-volume center, we worked to rene it and always
sought to improve it. In 1996, I was fortunate, and humbled, to succeed him as
Director of Pediatric Urology.
This second addition of The Exstrophy-Epispadias Complex, a book I originally
edited with Dr. Ranjiv Mathews back in 1999, is more than a collection of authors
and articles; it is the representation of many individuals’ tireless work. Though my
life’s work has been the treatment and correction of the anatomical insult in exstrophy, I very much view the congenital spectrum with the same bright-eyed awe as I
vii
viii
Foreword
did during medical school each time I assuage a young couple’s anxiety regarding a
prenatal diagnosis. We have collectively gained much ground, but the work remains.
Professor and Chief,
Jeffs Division of Pediatric Urology
The Brady Urological Institute,
Johns Hopkins Hospital
Baltimore, MD, USA
JohnP.Gearhart, MD, FAAP, FACS (Ed) (Hon)
Foreword
I had the good fortune to be Dr. Hendren’s second Fellow in Pediatric Urology. It
was the period when Dr. Hendren was Chief of Pediatric Surgery at the MGH (later
he was to become Chief at Boston Children’s). He was a master surgeon and his
teachings focused on the complex reconstruction of urologic malformations and
form the basics of complete primary repair of exstrophy. Dr. W.Leadbetter (of ureteral reimplant) appreciated the surgical talent of Dr. Hendren and supported his
active involvement in the training of pediatric urologic residents which included
training Pediatric Urology Fellows. As Chief, of Pediatric Surgery, Dr. Hendren did
it all, megaureter repairs, hypospadias, ureteral reimplants, and exstrophy closures
(many as one stage procedures). Dr. Hendren trained only three Pediatric Urology
Fellows at the MGH, but many after returning to Boston Children’s.
I was fortunate to be one of those fellows and lifelong interest in bladder exstrophy was ignited. My observation of this anomaly has conrmed for me that bladder
exstrophy presents on a spectrum, and whether a baby presents with cloacal exstrophy, bladder exstrophy, or penopubic epispadias depends on the timing of the premature rupture of the infra-umbilical membrane in relation to the descent of the
urorectal septum.
While my preference developed for the complete primary repair of exstrophy, as
surgeons we can only try to establish “normal anatomy” and anticipate that “normal” physiology will follow. We must recognize that likelihood of “normal” function depends in large part on where the exstrophy anomaly sits on the spectrum
between cloacal exstrophy and epispadias.
I am pleased that this book dedicated to bladder exstrophy is bringing together
today’s surgeons that are committed to taking the baton and continuing to make
strides in understanding, caring for, and advocating for children affected by exstrophy. I do hope that it is informative for the reader, and ideally, will ignite a passion
in caregivers as Dr. Hendren did in me in those early days of my career in Boston.
Children’s Hospital of Wisconsin
Medical College of Wisconsin
Milwaukee, WI, USA
MichaelE.Mitchell, MD
ix
Preface
Every book has an origin story, and this book’s antecedents beg the reader’s indulgence in sharing some recollections.
Nearly two decades ago, we met for the rst time in an ofce that sat astride a
small patient holding area, directly across the entrance to a solitary operating room
that held two operating tables arrayed in parallel. We stood in a ward among the
labyrinthine corridors of one of the largest hospitals in Asia, the Civil HospitalAhmedabad, a safety-net and tertiary hospital serving the 60 million inhabitants of
India’s state of Gujarat. Aseem, visiting from Minneapolis, was accompanied by the
already well-known bladder exstrophy expert surgeon from Seattle, Richard Grady.
Across the table sat Rakesh, a pediatric surgeon at Civil Hospital, and a partner to
the host that day, Atul Thakre, who had recently completed a pediatric urology fellowship and was embarking on a practice at Civil Hospital.
That patient holding area across from the ofce reverberated with the giggles and
cries of a dozen children with bladder exstrophy at various stages—primary, failed,
and complex variants—in the laps of their bewildered parents, looking expectantly
for a solution to an anomaly they could not understand, but would bear out of their
love for their child.
Beholding that gaggle of children that day in 2009—more than we might see at
an American children’s hospital in an entire year—Richard and Aseem, joined in
ensuing years by pediatric anesthesiologist Anjana Kundu, Cincinnati’s Pramod
P. Reddy, Civil Hospital’s own Jaishri Ramji, Toronto’s Joao Luiz Pippi Salle,
Seattle’s Paul A. Merguerian, and Philadelphia’s Douglas Canning and Dana Weiss,
each a co-editor of this compendium, committed to returning every year to work
together at Civil Hospital to surgically repair, research, and teach bladder exstrophy
and epispadias surgery. The care for these children is possible only because of the
expert faculty, residents, administration, nurses, and entire tireless staff of Civil
Hospital for welcoming, nurturing, and hosting the annual gathering. Our growing
cohort of surgeons, anesthesiologists, nurses, advocates, and affected families soon
took on an appellation: the International Bladder Exstrophy Collaborative (IBEC).
As IBEC grew—the cohort now includes close to 300 children with bladder
exstrophy and epispadias—and our research endeavors began grappling with fundamental questions about technique and outcomes with dozens of pediatric urologists
from around the world descending on Ahmedabad to immerse in the annual IBEC
workshop, the contours of this book were contemplated.
xi
xii
Preface
The venerable authority on bladder exstrophy, Dr. John Gearhart last edited a
book dedicated to bladder exstrophy in 1999, which he published with Springer
Nature. We are honored that this successor book to Dr. Gearhart’s pioneering effort
is also hosted by the same Springer Nature in making this present effort a reality. We
sincerely hope that this book conveys the advances in bladder exstrophy embryology,
radiological diagnosis, and genetics and comprehensively shares—with vivid videos—the nuances of the modern staged repair, complete primary repair, and radical
soft tissue mobilization (Kelly repair) approach to repairing bladder exstrophy and
penopubic epispadias. We also explore long-term concerns of exstrophy such as
incontinence, cosmesis, sexual function, and most importantly, how our efforts, as
physicians, impact a patient’s sense of self and satisfaction. This book also offers the
perspective on the critical role of nursing and also that of caregivers and advocates.
A book comes to fruition only with a legion of support. We are indebted rstly to
the courageous children and caregivers affected by the rare and devastating anomaly
of bladder exstrophy and epispadias. It is their hopes, aspirations, and indefatigable
smiles in the face of immense adversity that inspire us as every day as practitioners,
and whose wellness is the North Star to this effort.
We are grateful to Dr. Michael Mitchell and Dr. John Gearhart, living legends
who have transformed the lives of children with bladder exstrophy not only as surgeons but as dogged perfectionists and students of their discipline, spending careers
articulating their chosen approaches to exstrophy surgery while setting the highest
bar in elucidating outcomes with honesty and transparency. We are honored to have
their reections as a preface to this book. We are profoundly thankful to our coeditors—each of whom is a surgical leader of IBEC—for sharing in this undertaking, and every chapter author and co-author without whose exhaustive effort and
willingness to share their precious time and expertise, this book would not exist.
Our deep appreciation to the efforts of our editors with Springer Nature, Priyanka
Dey, and Paige Ripperger, whose daily nudges became a xture of our inbox to
ensure completion of this undertaking.
To Suhag, Parth, and Nimai: thank you for your love. And thank you for your
encouragement, patience, and endurance in the face of another weekend of, “Can’t
help around the house, have this book to nish!”
To Rima, Vidhi, and Dev: thank you for your unconditional love and everlasting
support despite extended working hours balancing the responsibilities of a pediatric
surgery department and Medical Superintendent duties at Civil Hospital.
Atul Thakre, Richard Grady, and Doug Canning wrote the rst chapters of the
IBEC story, but fate determined that they would not write the epilogue. Their joyful
spirit animates and suffuses the totality of this book—every written word, each chapter and video—and they continue to gently guide our hands whenever we embark on
the care of an affected child. Doug’s dictum—start with the child and go out from
there, is our IBEC motto. This book is dedicated to their eternal spirit. Om Sadgati.
With appreciation,
Philadelphia, PA, USA AseemR.Shukla
Ahmedabad, Gujarat, India RakeshS.Joshi