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Esophageal and
Gastric Disorders in
Infancy and Childhood

Holger Till
Mike Thomson
John E. Foker
George W. Holcomb III
Khalid M. Khan
Editors

123
Esophageal and Gastric Disorders
in Infancy and Childhood
Holger Till • Mike Thomson
John E. Foker • George W. Holcomb III
Khalid M. Khan
Editors

Esophageal and Gastric

Disorders in Infancy
and Childhood
Editors
Holger Till George W. Holcomb III
Medical University of Graz Department Surgery, University of
Graz Missouri, Kansas City Children’s
Austria Mercy Hospital
Kansas City, MO
Mike Thomson USA
Sheffield Children’s Hospital
Paediatric Gastroenterology Centre Khalid M. Khan
Sheffield University of Arizona
UK Tucson, AZ
USA
John E. Foker
Division of Cardiothoracic Surgery
Department of Surgery
University of Minnesota Medical School
Minneapolis, MN
USA
Department of Surgery
Boston Children’s Hospital
Harvard Medical School
Boston, MA
USA

ISBN 978-3-642-11201-0    ISBN 978-3-642-11202-7 (eBook)

DOI 10.1007/978-3-642-11202-7

Library of Congress Control Number: 2017933703

© Springer-Verlag Berlin Heidelberg 2017

This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or
part of the material is concerned, specifically the rights of translation, reprinting, reuse of
illustrations, recitation, broadcasting, reproduction on microfilms 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 specific 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, express or implied, with respect to the material
contained herein or for any errors or omissions that may have been made.

Printed on acid-free paper

This Springer imprint is published by Springer Nature

The registered company is Springer-Verlag GmbH Germany
The registered company address is: Heidelberger Platz 3, 14197 Berlin, Germany
To my three marvelous girls Ella, Jess, and Flo for teaching me
the importance of life outside work, to my wonderful wife Kay
for being my best friend, and to my Dad for inspiring me to
follow the medical path.
Mike Thomson
To what’s truly important in life, my family especially the boys
Karl and Dan, and my coeditors especially JEF, it has been a
pleasure to work with you all.
Khalid M. Khan
To my family, who supported me all these years and taught me
never to give up. It was worth it!
Holger Till
Preface

This book owes its existence to the families who brought us their babies with
the difficult problem of esophageal atresia. Other families have similarly
inspired each of the editors and authors of this book in their continuing efforts
to treat the many difficult esophageal and gastric disorders which occur in
childhood. We acknowledge our feelings of great indebtedness to these
patients and families. They offered us the privilege to learn from them.
Originally, the book was only going to be a how-to-do-it surgery book, but it
rapidly grew into a multidisciplinary effort. We quickly realized we needed to
enlist a number of distinguished pediatric gastroenterologists as well as other pedi-
atric surgeons to provide the necessary information. As the result of everyone’s
efforts, this book offers a comprehensive and detailed approach to these complex
patients. We believe this is an informative and detailed resource which will be of
great value for all workers in this field, but we look forward to your judgment.
Of course, the success of this book is dependent on each author’s contri-
bution, and we cannot thank them enough for their scholarly chapters and
their patience. Moreover, each editor dedicated a great deal of time and
energy to review the proofs. Finally, Holger and Mike, sharing the leading
editorship equally, created the connections among the articles and shaped it
into a unified piece of work. Of course many other peopled supported this
process tremendously like Sandra Becker, Mary Merrit and Bharatwaj M.V.
We thank you all.
Last but not least we return the love and energy of our families, who shared
our enthusiasm and gave us strength to accomplish this work.
On behalf of all authors, editors, families, and friends, we dedicate this
book to our patients and the future challenges this field presents.

Yours sincerely,
The Editors

Postscript:
Mike Thomson would like to acknowledge his coeditors in their unstinting
energy and application toward the eventual conclusion of this book. He would
also like to pay tribute to the patience and understanding of his friends as
authors who have contributed to this massive piece of work, without whom
this would not have been even remotely a feasible venture. Thank you so
much for your patience while this long gestation came about. Lastly he would

vii
viii Preface

like to thank most deeply for their support and, at times, bewildering faith in
this project his family – Kay, his lovely friend and wife and partner, Ella, Jess,
and Flo, his wildly amazing and fast-growing up daughters.
Introduction

The outlook for pediatric patients should be for 70 or more good years, a time-
line which leaps well past the traditional therapeutic mileposts of length of
stay (LOS) and even 5- and 10-year outcomes. The subject of this book,
Esophageal and Gastric Disorders of Infancy and Childhood, includes some
of the most serious birth defects as well as such common but potentially severe
problems as gastroesophageal reflux (GER). As will be emphasized, these
lesions and disorders may also have significant later consequences, and there-
fore success beyond childhood will likely depend on the effectiveness and
durability of the early treatment. Clearly, the specific problems, if not well
managed, will stand in the way of realizing 70 or more good years. Although
this long-term goal may be difficult to achieve, the recent and continuing
advances described in these chapters now make it increasingly possible.
The editors have designed this book Esophageal and Gastric Disorders in
Infants and Children to be of value to all workers in this area, even the most
experienced and knowledgeable. To be sure, this is also a lofty goal, and to
accomplish it we have included a wide range of features in this book to
broaden its value. First, both medical and surgical chapters are included and
equally emphasized. Certainly, more and more information is becoming
available in both the medical and surgical fields making it difficult to keep up
in one’s own specialty much less in other areas. The selection of the best
treatment plan, however, requires some understanding of the benefits and
consequences of the various options even though they may be outside of
one’s specialty.
Our experience has also indicated that there is less and less shared under-
standing between pediatricians and surgeons despite protestations from the
“best” centers that this is “not true at our place.” Either medical or surgical
therapy will likely have benefits, especially initially, but limitations may
appear over the long term, increasing the importance of the treatment deci-
sion. This book will provide the practitioner with the information on which to
base these decisions. The organization of the major sections begins with nor-
mal development and function, followed by the biological bases of the dis-
ease problems to provide a background for reviewing the benefits and
consequences of the therapeutic approaches. The biological features are
emphasized throughout the book which we believe will make it easier to
interpret the clinical situations encountered.
This book initially was to be limited to pediatric esophageal problems. As
we began working with the esophageal topics, however, the stomach kept

ix
x Introduction

intruding whether through the gastroesophageal junction or into the chest

itself. Function moves downward while damage usually flows upward. The
esophageal and gastric mucosal layers were active, intertwined, could
undergo a variety of unsatisfactory changes with inflammation and injury,
and were difficult to separate. Clearly, these two organs seemed to belong
together from both medical and surgical standpoints.
With the scope of the book determined, the topics were selected to cover
the significant biological, medical, and surgical issues of the pediatric esoph-
agus and stomach. The distinguished contributing authors have been chosen
for their insight into these problems and treatments. The range and depth
provided will not only provide comprehensive current information but also
establish a framework on which to build. Knowledge will continue to move
ahead and a solid conceptual background will be valuable to adding and
understanding future advances.
An insightful presentation of the diseases and treatments is also of real
value because a worldwide consensus and uniform approach to these problems
often does not currently exist. Despite the efforts of many workers in the field
and attempts to provide well-reasoned recommendations, little seems to be
settled in the minds of practitioners. Even for common problems such as GER
and esophageal strictures, we have found individual conclusions about the
severity of the findings and the resulting treatment plans not only vary widely
across the world but even within institutions. The assessment and treatment of
reflux, for example, is surprisingly varied among pediatricians and surgeons,
and not just between them. This is despite the efforts of acknowledged experts
to set detailed guidelines. For GER, the methods and difficulties of diagnosis
and the judgment of its significance, as well as the evaluation of potential treat-
ments, all pose difficulties. This book, by fully presenting the issues, should
improve understanding and lead to more effective therapy.
The goal of 70 years or more also requires the active pursuit of normality.
To reach this goal follow-up evaluations and treatments should be continued
as necessary to be sure normality remains in sight. What will be needed in
follow-up will often depend on an understanding of the longer term effects of
both the abnormalities and the treatments, and this may be provided by well-
designed prospective studies. At present, while evidence-based decision-­
making is certainly a worthwhile goal, it is in limited supply. We anticipate
that the authors of this book will continue to be leaders in developing well-
designed studies. However, in the meantime, practitioners must select and
provide treatment based on the information available. The contents of this
book will be aimed to aid the practitioner in making these decisions.
The biological underpinning of the clinical situations is of particular
importance for pediatric problems. Not only are most congenital defects the
result of developmental abnormalities, but there is also an active background
of continuing normal development, which increases complexity in manage-
ment generally. What might seem to be routine treatments with defined con-
sequences in the adult population may be of far greater significance in the
infant and child. Of course, there are significant biological factors in the treat-
ment of adult patients but active development has ended, leaving the back-
ground reasonably stable.
Introduction xi

In children, therapy directed at a disease process will be superimposed on

continuing normal development and all are inter-related and may be affected.
Whether medical or surgical, any therapy which affects function or anatomy
over a long period will likely have several consequences. The commonly used
acid suppression treatment of reflux esophagitis, for example, may produce
significant effects on the development of the stomach, in particular, the acid-­
producing cells. Other areas of development may also be affected by the
medications and, perhaps, impaired calcification will lead to decreased bone
density. Such considerations become greater the longer the planned therapy.
Common surgical operations, such as fundoplication, may also have sig-
nificant later consequences. A thoracotomy incision which results in the
fusion of ribs and/or the loss of innervation of the serratus anterior muscle
might produce significant problems including scoliosis. Granted, no current
operation is in its final form, but the principles and details that comprise the
surgical goals should be accessible and are some of the additional consider-
ations that must be factored in when selecting therapy. Consequently, each
surgical writer will present not only the indications for the operations along
with the important details that will make for a successful repair but also, as
far as possible, the long-term biological consequences and later problems
associated with them. These issues are present in virtually all therapeutic
choices and have increased importance when carried out against the back-
ground of growth and development. Predictably, the longer view will be more
and more thrust upon us.
In keeping with the long view in treating these patients, it is recognized
that many of these esophageal and gastric disorders may have significant later
consequences. Another feature of this book, therefore, has been carrying the
information on the disease processes and effects of treatment into early adult-
hood. For those caring for children, there almost seems to be an assumption
that life does not go much beyond age 16, a proposition for which there is
little evidence. The teenager is handed off “doing well” to practitioners car-
ing for young adults and, in effect, disappears. The later consequences, how-
ever, of GER and the long-term effects of the other types of treatment used in
childhood, whether medical or surgical, illustrate the importance of the lon-
ger view.
Treatment may require a choice between a relatively easy short-term solu-
tion and one that appears more difficult but which has significant long-term
advantages. This conflict is most often present in surgical approaches. An
operation which produces a desirable short LOS does not necessarily place
the child on a path to 70 or more good years. This goal may be an unwelcome
burden for the surgeon whose peace of mind as well as reputation depends on
a shorter LOS and the occurrence of only “acceptable” complications.
Problems which may occur 20 or 30 years later may not even be considered.
The spread of information and the inevitable increasing emphasis on long-­
term results, however, will make these considerations necessary.
The therapy of difficult lesions and diseases may run the risk of setting in
motion new problems or the substitution of one disease state for another.
Palliative solutions are sometimes necessary but, by definition, bring their
own form of chronic problems, and, with time, the deficiencies will become
xii Introduction

increasingly apparent. Transplantation, with the need to hold off rejection,

provides a clear example of the problems that predictably result from substi-
tuting one disease for another. Less obvious are other treatments which sig-
nificantly alter anatomy and function, such as interposition grafts for
esophageal atresia (EA), but the consequences may also be unsatisfactory.
Palliation, although accepted and sometimes necessary, should be limited for
the best long-term results.
We have emphasized the biological theme of this book, as is well illus-
trated by the chapters on the recent enlistment of developmental responses to
solve some of the most severe problems. The use of growth induction on the
small esophageal segments for patients with esophageal atresia (EA), for
example, provides a biological solution for the traditionally difficult defect of
long-gap esophageal atresia (LG-EA). At the extreme end of the EA spec-
trum, the entire thoracic esophagus may be missing, making the possibility of
using only the esophagus for the repair apparently impossible. The develop-
mental potential, however, is great and even very small (2–3 mm) esophageal
primordia have been induced to grow into an outwardly normal lower esopha-
gus. This has been a spectacular example of using a biology-based therapy to
solve the problem of a significant congenital defect (Chaps. 22 and 25). Long,
recalcitrant esophageal strictures have also been difficult to treat and typi-
cally have led to gastric or colon interpositions. A variation on the tension-­
induced esophageal growth approach has been used to treat the significant
problem of long strictures by using staged resections with greatly improved
long-term results (Chapter 34). These are the most noteworthy examples of
the biological approach but others will follow.
Another strength of this book is the detailed explanations of the surgical
treatment of some of the most severe problems. Growth induction provides an
excellent solution to LG-EA, but less well known are the technical difficulties
in achieving a good growth response. The details helpful in growth induction
as well as in the surgical treatment of related problems such as the difficult
esophageal anastomosis, tracheomalacia, and re-operative strategies among
other procedures are presented. These descriptions will overcome the space
and page limitations of journal articles and be valuable to surgeons.
A developmental solution requires that the defect is not primarily genetic
in origin and all the information necessary for a catch-up response is present.
Although congenital defects are often loosely thought to be genetic in origin,
the evidence suggests these problems arise primarily from faulty develop-
ment. EA, for example, seems to result from the faulty budding of the trachea
from the primordial esophageal tube and the creation of a gap between the
upper and lower esophageal segments. With the segments separated and the
entire esophagus no longer acted on by the tension of the growing spinal
column, the gap may increase in length. The outwardly wide EA spectrum
predictably begins with a central defect and is determined by the length of the
gap and the presence or absence of residual fistulas into the airway.
Despite the apparent complexity of the EA spectrum, development can be
effectively restarted. Only the signal is missing which is able to trigger the
well-orchestrated three-dimensional growth seen during development.
Catch-up growth can be reliably induced in the small and widely separated
Introduction xiii

esophageal segments by providing the missing signal. The signals themselves

have usually proven to be very straightforward biomechanical stimuli, and for
the esophagus, axial tension will produce effective growth of the smallest
segments. The tension principle of esophageal growth induction, as noted,
has also been extended to acquired problems, such as long recalcitrant
strictures.
When a principle is basic and effective, it is likely to have other general
applications with improved treatment solutions. Although not applicable to
every problem, there are other examples of tissue or organ deficiency where
it has been used with impressive results. For patients with a hypoplastic car-
diac ventricle, induced growth has produced two usable ventricles and has
avoided a palliative solution which brings increasing problems over the suc-
ceeding years. Currently, an intra-abdominal testis is most commonly treated
by dividing the arterial blood supply, losing most, if not all, function. Traction,
however, will stimulate catch-up length and an improved result. Deficiencies
of skin and abdominal wall are commonly treated with tissue expanders to aid
closure. These devices were initially thought to involve only skin stretching,
but basic studies have shown a proliferative response to the tension by the
many cell types involved. Effective induced lengthening of the remaining
intestine in the short gut syndrome would greatly speed the transition from
hyper-alimentation and costly elemental solutions to a more normal diet. For
all of these problems, as with LG-EA, the traditional treatments have been
palliative or otherwise unsatisfactory for the long term. The use of these bio-
logical solutions with the induction of normal development has moved the
outlook for these patients far closer to normal, with fewer adverse conse-
quences and better long-term results.
When we focus on anatomy, embryology, and function dependent on these
abnormalities which are caused by chance, we must remember that medicine
has evolved massively over the last 30 years and that approaches which are
not just reparative in a surgical sense may be beneficial to the quality of life
of an individual affected by such issues. Indeed the advances in medical and
pharmaceutical approaches have been enormous in this time span and should
be and are, emphasized, within this text. For instance, it was only understood
in the early 1990s that Helicobacter pylori was the primary cause of peptic
ulcer disease and not, as previously thought, an overactive vagal nerve stimu-
lated acid production from the stomach. So went Billroth-type operations and
selective vagotomies. Medical approaches recently have therefore challenged
surgery to produce reasons for the traditional approaches, and we see the
obvious advantages now. Similarly, surgical solutions such as open fundopli-
cation have been supplanted by laparoscopic approaches which, in turn, have
been challenged by endoscopic trans-oral approaches – which are receiving a
great deal of attention at present and are promising. Long-term anti-acid med-
ical treatments are generally safe and may be attached to less morbidity than
surgical solutions, and this area has received considerable attention in this
book. So, while medical and surgical approaches are both effective, it must be
identified that a joint approach in pediatrics is mandatory if we are to achieve
a balanced management strategy for many of these complex conditions. We
would never suggest that a pure surgical or medical approach is ideal for an
xiv Introduction

empyema or a subdural hematoma, for example, and this is equally true in

this field involving the upper GI tract. This is what makes this book unique –
as, for the first time, surgeons and pediatric gastroenterologists have come
together to produce the first text on these complex topics which is of a bilat-
eral genre. Pediatric gastroenterologists and surgeons alike do not live and
work in bubbles isolated from one another in the real world but work in
superb harmony (in the best centers) and to the advantage of the families and
children they serve. Long may this continue and we hope that this text will
serve to perfect that partnership as the editorship has also done. When we
look to the future, we can see many options and opportunities that involve
closer collaboration between medical and surgical GI doctors and teams.
They may also involve such specialities as bioengineering, molecular biol-
ogy, genetics, neuroimmunology, neurogenetics, tissue modeling, etc., but
not forgetting the traditional team-orientated approaches involving our col-
leagues in GI physiology, feeding therapy, psychology, dietetics, GI nursing,
stoma therapy, and pharmacology to name only a few. We hope that this mul-
tispecialty book will appeal and educate many within our multidisciplinary
specialty and conjoin medicine and surgery toward a greater theme which is
collaboration and a more effective collateral approach going forward.
As stated at the beginning of the Introduction, the goal in pediatric therapy
must be for 70 or more good years, and this is a stringent requirement. The
problems encountered in pediatric patients are a unique mixture of develop-
mental and genetic defects, which play out on a continuing background of
normal and abnormal development. For these young patients, the often long-­
range consequences of both diseases and treatments must also be kept in
mind. The aim of this book is to clarify as much as possible these complex
events and the therapeutic options. Treatments which enlist normal biological
responses, we believe, will have particular value and, as this approach
becomes better understood and the applications expand in number, the goal
of 70 or more good years will come into view for even the most difficult of
these problems.
Contents

Part I Introduction

1 The Biology of Defects, Disease, and Treatments���������������������������� 3

John E. Foker
Part II The Esophagus

2 The Genetics and Molecular Biology

of Oesophageal Development������������������������������������������������������������ 9
Stephen P. Robertson and Spencer W. Beasley
3 Swallowing and the Upper Esophageal Sphincter������������������������ 29
Robert E. Kramer
4 Esophageal Motility ������������������������������������������������������������������������ 41
Hayat M. Mousa and Rodrigo Machado
5 Vascular, Neurological and Functional Development
of the Oesophagus���������������������������������������������������������������������������� 73
Udo Rolle and Alan J. Burns
Part III Esophageal Atresia Spectrum

6 The Spectrum of Esophageal Atresia �������������������������������������������� 79

John E. Foker
7 Esophageal Atresia and Tracheoesophageal Fistula:
The Clinical Spectrum, Diagnosis, and Evaluation���������������������� 91
Justin D. Klein and Russell W. Jennings
8 Oesophageal Atresia Associations������������������������������������������������ 107
Lewis Spitz
9 Congenital Esophageal Stenosis Associated
with Esophageal Atresia���������������������������������������������������������������� 113
Ashraf H.M. Ibrahim and Talal A. Al Malki
10 Choanal Atresia, Esophageal Atresia,
Facial Anomalies, and Dysautonomia������������������������������������������ 125
Francesco Cozzi and Denis A. Cozzi

xv
xvi Contents

11 Skeletal Anomalies Associated with Esophageal Atresia ���������� 135

Jonathan Nubla Sembrano, Walter H. Truong,
Charles Gerald Tan Ledonio, and David Wayne Polly Jr.
Part IV Repair of Shorter Gap EA

12 History of the Treatment of Esophageal Atresia������������������������ 157

John E. Foker
13 Preoperative Evaluation���������������������������������������������������������������� 163
H. Till
14 Thoracotomy Incisions������������������������������������������������������������������ 167
John E. Foker and Adrian Bianchi
15 The Current Repair Techniques of Short-Gap
EA/TEF ������������������������������������������������������������������������������������������ 171
H. Till and M. Hoellwarth
16 Thoracoscopic Repair of Esophageal Atresia
and Tracheoesophageal Fistula���������������������������������������������������� 179
George W. Holcomb III
17 Repair of Other Congenital Esophageal Anomalies ������������������ 189
Shawn D. St. Peter
18 Evaluation and Repair
of Laryngotracheoesophageal Clefts�������������������������������������������� 197
Katherine K. Hamming and Frank L. Rimell
19 Postoperative Management of Routine Esophageal
Atresia Cases���������������������������������������������������������������������������������� 203
Christopher G. Turner and Russell W. Jennings
Part V Repair of Long-Gap EA: The Difficult End
of the Spectrum

20 The Long-Gap Esophageal Atresia Problem������������������������������ 213

John E. Foker
21 Delayed Primary Anastomosis in the Management
of Long-Gap Esophageal Atresia�������������������������������������������������� 221
Prem Puri and Florian Friedmacher
22 Surgical Methods to Increase Esophageal Length
in Long (Wide)-Gap Esophageal Atresia with
and Without Tracheoesophageal Fistula�������������������������������������� 229
Sigmund H. Ein
23 Thoracoscopic Repair of Pure Esophageal Atresia�������������������� 243
Benjamin E. Padilla and Marcelo Martinez-Ferro
Contents xvii

Part VI The Growth Procedure for Long Gap EA

24 Growth Induction (the Foker Procedure)

and a Flexible Approach for the Repair of Long-Gap
Esophageal Atresia������������������������������������������������������������������������ 259
John E. Foker
25 Thoracoscopic Elongation of the Esophagus in Long-Gap
Esophageal Atresia������������������������������������������������������������������������ 285
David C. van der Zee
26 Perioperative Management of the Esophageal
Growth Procedure�������������������������������������������������������������������������� 295
Michael Sweeney
27 The Growth Potential (Form and Function):
The International Esophageal Growth Experience�������������������� 303
Khalid M. Khan
Part VII Interposition Grafts to Establish Continuity

28 Gastric Transposition in Infants and Children �������������������������� 313

Robert A. Cowles and Arnold G. Coran
29 The Gastric Tube���������������������������������������������������������������������������� 321
Gabriel O. Ionescu, Simona Gavrilescu, and Gabriel Aprodu
30 Colon (Including Ileum) Interposition ���������������������������������������� 347
Mohammed Abdel-Lalif Ayad, Khaled Mohamed El-Asmer,
and Alaa Fayez Hamza
31 Esophageal Replacement with Jejunum in Children������������������ 361
Klaas(N) M.A. Bax
32 Oesophagus Tissue Engineering: Future Options
in Oesophageal Replacement Through Regenerative
Medicine������������������������������������������������������������������������������������������ 371
Amulya K. Saxena
Part VIII After EA-Repair: Complications and Post-­repair Issues

33 Overview: The Post-repair Issues and the Active

Pursuit of Normalcy���������������������������������������������������������������������� 389
John E. Foker
34 Evaluation After EA Repair: Endoscopy,
Ultrasound, and Function ������������������������������������������������������������ 401
Khalid M. Khan
35 Complications Following Pediatric Esophageal Surgery ���������� 415
Sigmund H. Ein
xviii Contents

36 The Biology of Stricture Formation After Esophageal

Atresia Repair�������������������������������������������������������������������������������� 441
John E. Foker
37 Strictures: Bougienage and Balloon Dilation������������������������������ 449
Khalid M. Khan
38 The Dynamic Stent in the Treatment of Oesophageal
Strictures���������������������������������������������������������������������������������������� 459
Luigi Dall’Oglio
39 Mitomycin C Application on Caustic Esophageal Strictures���� 467
Khaled Mohamed El-Asmer, Mohammed Abdel-Lalif Ayad,
and Alaa Fayez Hamza
40 Reoperations After Esophageal Atresia Repair
(for Significant Leaks, Recurrent Fistulas, Strictures,
Residual Tracheal Pouches, Large Diverticula,
Partially Intrathoracic Stomachs, and Failed Repairs)�������������� 471
John E. Foker
41 Growth Induction to Treat Long Esophageal Strictures������������ 489
Tara C. Kendall Krosch and John E. Foker
42 The Esophagogastric Junction, Reflux,
and Esophageal Atresia ���������������������������������������������������������������� 497
Khalid M. Khan
43 Management of Gastroesophageal Reflux
After Esophageal Atresia Repair�������������������������������������������������� 511
Janine N. Pettiford and Daniel J. Ostlie
44 The Stomach and Esophageal Atresia Repair���������������������������� 519
Khalid M. Khan
45 Learning to Eat After Esophageal Atresia Repair:
In Infancy and Childhood ������������������������������������������������������������ 527
James Brudney
Part IX The Airway: Nerves, Malacia and Life-­Threatening
Events

46 Apparent Life-Threatening Event (ALTE) in Infants

with Esophageal Atresia���������������������������������������������������������������� 537
Francesco Cozzi and Denis A. Cozzi
47 New Insights and Applications in the Treatment
of Nerve Injuries���������������������������������������������������������������������������� 549
Alison K. Snyder-Warwick, Andrew Yee,
and Susan E. Mackinnon
48 Tracheomalacia and the Effective Aortopexy ���������������������������� 571
John E. Foker, Abby C. Meyer, and Frank Rimell
49 Thoracoscopic Aortopexy�������������������������������������������������������������� 581
Klaas(N) M.A. Bax
Contents xix

Part X Further Out from EA Repair: Functional Results

and the Quality of Life

50 Long-Term Results: Prognosis, Developmental

Milestones, and Quality of Life After Surgery
for Esophageal Atresia������������������������������������������������������������������ 597
Daniel C. Aronson
51 Outcomes of Oesophageal Atresia Beyond Childhood:
Helsinki Experience ���������������������������������������������������������������������� 603
Saara J. Sistonen, Mikko P. Pakarinen, and Risto J. Rintala
52 The Minnesota Experience������������������������������������������������������������ 615
Khalid M. Khan
53 Long-Term Follow-Up and Quality of Life After
Gastric Transposition�������������������������������������������������������������������� 623
Lewis Spitz and Lorraine Ludman
54 The Follow-Up of the Gastric Tube���������������������������������������������� 631
Gabriel O. Ionescu, Simona Gavrilescu,
and Sandu Gabriel Aprodu
55 Long-Term Follow-Up After Gastric Reconstruction
of the Esophagus���������������������������������������������������������������������������� 645
Sarah E. Billmeier, David I. Soybel,
and Michael T. Jaklitsch
56 Late Follow-Up of Colon Interpositions�������������������������������������� 663
Terry Lynn Buchmiller and William Hardy Hendren III
57 The Long-Term Follow-Up from the Parents’
and Patient’s Perspective�������������������������������������������������������������� 675
J. Trompelt
Part XI Acquired Esophageal Problems

58 Esophageal Injuries and Foreign Bodies ������������������������������������ 695

Filippo Torroni, Paola De Angelis, and Luigi Dall’Oglio
59 Caustic Ingestions�������������������������������������������������������������������������� 701
Mário César Vieira and Paulo Fernando Souto Bittencourt
60 Esophagitis: Causes Other Than Reflux�������������������������������������� 713
Mike Thomson
61 Eosinophilic Esophagitis (North America)���������������������������������� 723
Aileen Har and Sandeep K. Gupta
62 Eosinophilic Esophagitis (Europe) ���������������������������������������������� 739
Juan A. Tovar, Ana Lourdes Luis, and Cristina Riñón
63 Effect of Systemic Illness, Medication, Radiation,
and Infection on the Esophagus���������������������������������������������������� 749
Seema Mehta and Ryan W. Himes
xx Contents

64 Oesophageal Varices���������������������������������������������������������������������� 765

P.J. McKiernan
65 Laparoscopic Heller-Dor Procedure for the Treatment
of Esophageal Achalasia���������������������������������������������������������������� 775
Girolamo Mattioli, Alessio Pini Prato, Valentina Rossi,
Stefano Avanzini, Giovanni Montobbio,
and Vincenzo Jasonni
66 Esophageal Tumors in Childhood and Adolescence:
Benign and Malignant ������������������������������������������������������������������ 781
Till-Martin Theilen and Michael La Quaglia
67 Epidermolysis Bullosa: Epidemiology, Diagnosis,
Complications, and Treatment������������������������������������������������������ 801
Richard G. Azizkhan and Ahmed Mami
Part XII The Gastroesophageal Junction

68 Lower Esophageal Sphincter: Normal Structure

and Function���������������������������������������������������������������������������������� 817
Osvaldo Borrelli and Nikhil Thapar
69 Gastroesophageal Junction: The Mucosa – Anatomy
and Cell Types�������������������������������������������������������������������������������� 823
Marta C. Cohen
70 Epidemiology of Gastroesophageal Reflux Disease ������������������ 829
Michael A. Manfredi
71 The Genetics of GER and GERD ������������������������������������������������ 835
Isabel Filges and Raoul I. Furlano
72 Pathophysiology of Gastro-­oesophageal Reflux Disease������������ 845
Silvia Salvatore and Geoffrey Davidson
73 The Oesophageal Mucosa: To Barrett’s and Beyond –
The Genesis of Oesophageal Injury and Cellular
Mutations���������������������������������������������������������������������������������������� 855
Haider N, Day A and Spencer W. Beasley
Part XIII Assessment of GE Reflux

74 GERD: History and Examination������������������������������������������������ 871

Gigi Veereman-Wauters
75 GER: The Place of pH Testing������������������������������������������������������ 875
Yvan Vandenplas
76 pH and Impedance Measurements in Infants
and Children���������������������������������������������������������������������������������� 879
Yvan Vandenplas
77 Assessment of GE Reflux: Esophageal Motility Studies������������ 897
Samuel Nurko
Contents xxi

78 Esophageal Intraluminal Impedance ����������������������������������������   907

Tobias G. Wenzl
79 Endoscopy with Biopsy for Esophagitis ������������������������������������   913
Mike Thomson
80 Barium Contrast Radiography and Scintigraphy��������������������   925
Rossella Turco, Dario Ummarino, and Annamaria Staiano
81 Assessment of GERD: Ultrasound ��������������������������������������������   935
Ahmed Sarkhy
82 Ear, Lung, and Esophageal Fluid Evaluation
in GER Diagnosis ������������������������������������������������������������������������   939
David J. Rawat
Part XIV Treatment Approaches for GERD: Lifestyle Changes

83 Feeding Changes and Positioning Therapy for Infants������������   957

Ahmed Sarkhy and Mike Thomson
84 Lifestyle Changes in Children and Adolescents������������������������   963
Donald J.S. Cameron
Part XV Pharmacologic Therapies

85 Pharmacological Reflux Therapies��������������������������������������������   971

Anil Darbari, Sona Sehgal, Nidhi Rawal, and Rachel Imhoff
86 Mucosal Protective Agent: Sucralfate
in the Treatment of Gastroesophageal Reflux Disease
in Children������������������������������������������������������������������������������������   979
M. Smits and Marc A. Benninga
87 Antacids and Alginates in the Treatment
of Gastroesophageal Reflux Disease������������������������������������������   983
R.E. van der Pol and Marc A. Benninga
88 Histamine-2 Receptor Antagonist in the Treatment
of Gastroesophageal Reflux Disease������������������������������������������   987
Herbert M. van Wering and Marc A. Benninga
89 Proton Pump Inhibitors��������������������������������������������������������������   995
Licia Pensabene and Geoffrey Davidson
90 Prokinetic Therapy���������������������������������������������������������������������� 1015
Gigi Veereman-­Wauters
Part XVI Surgical Therapies

91 Gastroesophageal Reflux: Issues from a Surgeon’s

Perspective������������������������������������������������������������������������������������ 1019
Ma Pilar Abad Calvo and J. Boix Ochoa
92 The Spectrum of Surgical Anti-­reflux Procedures:
Which Operations Work? ���������������������������������������������������������� 1063
E.M. Kiely
xxii Contents

93 Fundoplication in Infants and Children������������������������������������ 1069

Oliver J. Muensterer
94 Reoperative MIS Fundoplication ���������������������������������������������� 1085
Oliver J. Muensterer, Carroll M. Harmon,
and Keith E. Georgeson
95 Endoscopic Approaches to the Treatment of GERD���������������� 1093
Mike Thomson
96 Repair of Hiatus Hernia�������������������������������������������������������������� 1105
Balgopal Eradi and Richard J. Stewart
97 Endoscopic Treatment of Benign Esophageal Strictures
with Removable or Biodegradable Stents���������������������������������� 1119
Yvan Vandenplas, Bruno Hauser, Thierry Devreker,
Daniel Urbain, and Hendrik Reynaert
98 Oesophagogastric Dissociation: When Is It Relevant?������������ 1127
A. Bianchi and A. Morabito
99 Gastrostomy Feeding and Gastroesophageal Reflux���������������� 1133
Peter B. Sullivan
Part XVII Evaluation and Management of the Pediatric
Patient with Suspected GERD

100 Regurgitation in Infants�������������������������������������������������������������� 1141

O. Kirmemis
101 Recurrent Regurgitation and Vomiting in Children���������������� 1149
Prithviraj Rao and Mike Thomson
102 Reflux Esophagitis and the Child with Heartburn ������������������ 1161
Mike Thomson
103 Food Refusal, Dysphagia, and Odynophagia���������������������������� 1167
Amy Tsai, Jose Garza, and Ajay Kaul
104 The Child with Apnoea or ALTE ���������������������������������������������� 1187
M.T. Rawat
105 Children with Pulmonary Disorders������������������������������������������ 1201
Mark L. Everard and Kostas Priftis
106 Gastroesophageal Reflux Disease (GERD)
and Dental Erosion (DE) ������������������������������������������������������������ 1211
Hanaa Halaby and Mike Thomson
Part XVIII Children at High Risk for GERD

107 The Child with Neuromotor Impairment���������������������������������� 1217

Christopher D.C. Rittey
Contents xxiii

108 The Obese Child and Reflux ������������������������������������������������������ 1229

Peter Michael Gillett
109 Children at High Risk for GERD: The Premature
Infant�������������������������������������������������������������������������������������������� 1239
Taher Omari
Part XIX The Stomach

110 Embryology of the Stomach�������������������������������������������������������� 1253

Mike Thomson
111 Normal and Pathologic Mucosa�������������������������������������������������� 1263
Marta C. Cohen
Part XX Evaluation

112 Upper GI Endoscopy in the Diagnosis of Gastropathy������������ 1275

Mike Thomson
113 Radiology of the Stomach in Infants and Children������������������ 1295
Tara L. Holm and Charles A. Dietz Jr.
114 Gastric Motility and Electrogastrography (EGG)�������������������� 1313
Alberto Ravelli
Part XXI Anatomical Gastropathology

115 Anatomical Gastropathology������������������������������������������������������ 1325

Basil Bekdash and Sean S. Marven
116 Congenital Gastric Anomalies���������������������������������������������������� 1337
Brice A. Antao and Victoria A. Lane
117 Congenital Pyloric Stenosis, Webs and Strictures�������������������� 1353
H. Till
118 Gastric Volvulus �������������������������������������������������������������������������� 1355
Tamara Caldaro, Filippo Torroni, Erminia Romeo,
Giovanni Federici di Abriola, and Luigi Dall’Oglio
Part XXII Mucosa-Related Gastropathology

119 Helicobacter-Related Gastritis and Ulceration

and Investigation of Helicobacter pylori ������������������������������������ 1363
Priya Narula
120 Non-Helicobacter pylori Gastritis, Ulceration,
and Drug-Related Gastropathies������������������������������������������������ 1375
Arun Nanjundaraje Urs
121 Long-Term Effects of Achlorhydria on the Stomach
(Helicobacter pylori and PPI Therapy)�������������������������������������� 1387
Marta Tavares and Jorge Amil Dias
xxiv Contents

122 Gastric Bleeding and Perforation ���������������������������������������������� 1397

Jürgen Schleef
123 Ménétrier’s Disease���������������������������������������������������������������������� 1407
Ann Matthai
124 Inflammatory Bowel Disease and the Stomach������������������������ 1411
Robert Heuschkel
125 Autoimmune Gastropathy���������������������������������������������������������� 1417
Ed Giles and Nicholas Croft
126 Systemic Disease Affecting the Stomach������������������������������������ 1425
Sue Protheroe
127 Mucosa-Related Gastropathology: The Upper
Gastrointestinal Tract and the Microbiome������������������������������ 1447
Doron D. Kahana and Timothy Van Natta
Part XXIII Functional Gastropathology

128 Duodeno-Gastric Reflux

and Duodeno-Gastro-­Oesophageal Reflux�������������������������������� 1465
Rok Orel
129 Gastric Pacing������������������������������������������������������������������������������ 1481
Marc Christopher Winslet
130 Dermatology and the Oesophagus���������������������������������������������� 1487
Sue Protheroe

Erratum���������������������������������������������������������������������������������������������������� E1

Index������������������������������������������������������������������������������������������������������ 1501
 Part I
Introduction
 The Biology of Defects, Disease,
and Treatments
John E. Foker
The origins of this book began with the success in
growing the smallest esophageal segments to nor-
mal size in patients with esophageal atresia (EA).
It had become clear that the growth procedure
effectively tapped into the considerable develop-
mental potential of even the most rudimentary
blind ends of the esophagus allowing the benefits
of a true primary repair to be realized across the
full EA spectrum [1]. Although congenital defects
are often loosely considered to be primarily
genetic mistakes, the evidence suggests that many
of these problems arise from faulty development
and, therefore, are potentially reversible. A tiny
esophageal primordium poses an obvious obsta-
cle to a primary esophageal repair; however, only
the signal is required to effectively restart the
well-orchestrated and complex three-dimensional
organ development. The signal for the growth and
development of organs and tissue is often a bio-
mechanical stimulus, and in the case of long gap
EA, axial tension provides it [1, 2].
This biological approach also opened up related
questions which needed answers. The questions
J.E. Foker, MD, PhD
Robert and Sharon Kaster Professor of Surgery,
Division of Cardiothoracic Surgery, Department of
Surgery, University of Minnesota Medical School,
Mayo Mail Code Box 207, 420 Delaware Street SE,
Minneapolis, MN 55455, USA
Visiting Professor, Department of Surgery,
Boston Children’s Hospital, Harvard Medical School,
Boston, MA, USA
e-mail: foker001@umn.edu
© Springer-Verlag Berlin Heidelberg 2017
H. Till et al. (eds.), Esophageal and Gastric Disorders in Infancy and Childhood,
DOI 10.1007/978-3-642-11202-7_1
1
included the quality of the esophagus recently sub-
jected to catch up growth, the characteristics of the
gastroesophageal (GE) junction, as well as the uni-
versal debates which surround the treatment of GE
reflux (GER). Because strictures frequently devel-
oped at the anastomotic site, this response also
needed improved understanding. All of these
issues can be viewed as biological questions, and
the answers will be important to designing effec-
tive therapy.
What had started as essentially a surgical
monograph on EA was greatly expanded to con-
tain chapters about all of the disorders of the
esophagus and stomach in childhood. The book
now features the wide variety of medical
approaches to these problems as well as provid-
ing expanded surgical techniques which empha-
size the underlying biological principles. As a
result of this comprehensive approach, the possi-
ble obstacles to unfavorable long-term outcomes
may be recognized and overcome. For pediatric
caregivers, long-term outcomes leap well beyond
the 5- or 10-year results common in the adult
world to a goal of 70 good years, making the
active pursuit of normalcy necessary.
The editors have designed this book on esopha-
geal and gastric disorders in infants and children to
be of value to all workers in this area, even the
most experienced and knowledgeable. To be sure,
this is a lofty goal, and to accomplish it, we have
greatly increased the breadth and depth of this
book. Certainly, more and more information is
becoming available in each field making it difficult
3
4 J.E. Foker
to keep up in one’s own specialty much less in
other areas. The selection of the best treatment
plan, however, requires some understanding of the
benefits and consequences of the various options
even though they may be outside of one’s spe-
cialty. Our experience has also indicated that there
is less and less shared understanding between
pediatricians and surgeons despite protestations
from the “best” centers that this is “not true at our
place”. A medical or a surgical therapy will likely
have benefits, especially initially, but limitations
may appear over the long term, increasing the
importance of the treatment decision.
An insightful presentation of diseases and treat-
ments is also of real value because, currently, a
world-wide consensus and uniform approach to
these problems often does not exist. Even for com-
mon problems such as GER and despite the efforts
of many workers in the field and attempts to provide
well-reasoned recommendations, little seems to be
settled in the minds of practitioners. The assessment
and treatment of GER is surprisingly varied among
pediatricians and surgeons, as well as between
them. Esophageal strictures also remain unsettled,
and we have found that individual conclusions
about the severity of the findings and the resulting
treatment plans vary widely not only across the
world but even within institutions. The chapters in
this book also reveal that disagreements may exist
even among those knowledgeable and experienced.
Understanding should be improved, however, by
fully presenting the issues which will help practitio-
ners achieve more logical treatment plans.
The goal of 70 years requires an active pursuit
of normalcy. For the goal to be reached, follow-­up
evaluations and treatments are often required to be
sure normalcy does not slip away. Well-­designed
clinical studies with longer follow-up will be
expected and, eventually, demanded by families as
well as practitioners. At present, while evidence-
based decision-making is certainly a worthwhile
goal, it is in limited supply. We anticipate that the
authors of this book will continue to be leaders in
developing well-designed studies which will con-
tribute to the evidence base; in the meantime, how-
ever, practitioners must select and provide treatment
based on the information available. The contents of
this book will aid making these decisions.
The biological underpinning of the clinical
situations is of particular importance for pediatric
problems. The developmental abnormalities of
many congenital defects exist against an active
background of continuing normal development,
which greatly increases the complexity. Whether
medical or surgical, any therapy which affects
development over a long period will likely have
consequences. The commonly used acid suppres-
sion treatment of reflux esophagitis, for example,
may produce significant effects on the develop-
ment of the acid-producing cells. Other effects
may be more distant, and the proton pump inhibi-
tors, for example, may alter calcium metabolism
and slowly lead to decreased bone density. Such
considerations become greater the longer the
planned therapy. What might seem to be routine
treatments with defined consequences in the
adult population may be of far greater signifi-
cance in the developing infant and child.
Surgical treatments may also have significant
later consequences. A thoracotomy incision
which results in the fusion of ribs and/or the loss
of innervation of the serratus anterior muscle pre-
dictably leads to significant chest wall problems
which may include scoliosis. Certainly, the vari-
ous interposition grafts for long gap EA often
have increasing problems with time, and these
should be considered when selecting the initial
therapy. Granted, no current operation is in its
final form, but the principles and details that
comprise the surgical indications and goals as
well as the long-term outcomes should be acces-
sible and part of the therapeutic considerations.
Because operations in children are carried out
against the background of growth and develop-
ment, the longer view will be more and more
thrust upon surgeons.
For those caring for children, there almost
seems to be an assumption that life does not go
much beyond age 16; a proposition for which
there is little evidence. The teenager is handed off
“doing well” to practitioners caring for young
adults and, in effect, disappears from the pediatric
world. The long-term consequences of GER, for
example, and the later effects of other t­herapies
used in childhood, whether medical or surgical,
illustrate the importance of the longer view.
1 The Biology of Defects, Disease, and Treatments
Treatment unfortunately may require a choice
between a relatively easy short-term solution and
one that appears more difficult initially but which
has significant long-term advantages. This con-
flict is most often present in surgical approaches.
An operation which produces a desirable short
length of stay (LOS) does not necessarily place
the child on a path to 70 good years. The latter
goal may be an unwelcome burden for the sur-
geon whose peace of mind as well as reputation
depends on a shorter LOS and the occurrence of
only well-recognized and “acceptable” compli-
cations. Currently, problems which may occur 20
or 30 years later might not even be considered.
The spread of information and the inevitable
increasing emphasis on long-term results, how-
ever, will make these considerations necessary.
The therapy of difficult lesions and diseases
may also run the risk of setting in motion new prob-
lems or the substitution of one disease state for
another. Palliative solutions are sometimes neces-
sary but, by definition, bring their own chronic
issues, and, with time, the deficiencies will become
more important. Transplantation, with the need to
hold off rejection, provides a clear example of the
problems that predictably result from substituting
one chronic disease for another. Interposition grafts
for EA, as mentioned, significantly alter anatomy
and function and the later consequences are often
unsatisfactory. Palliation, although sometimes nec-
essary, will likely be less frequently used.
This book also provides detailed explanations,
which are not readily available elsewhere, of the
surgical treatment of some of the most severe
problems. Growth induction provides a good
solution to the difficult problem of LG-EA, but
there are technical demands in achieving a good
end result. The details helpful in growth induc-
tion as well as in the surgical treatment of related
problems are presented. As our practice has
shown, a number of complications can result
even from routine EA/TEF repairs as well as
from other esophageal and gastric operations.
Consequently, the surgical topics include the dif-
ficult esophageal anastomosis, the long stricture,
the recurrent TEF, a large diverticulum, the
­partial intrathoracic stomach, significant tracheo-
malacia, and various re-operative strategies.
5
Pediatric surgeons will recognize the biological
solutions to many of these problems are a depar-
ture from current approaches. Because the objec-
tives are more nearly normal anatomy and
function, however, they should be considered.
The surgical descriptions will overcome the
space and page limitations of journal articles and
should be helpful.
When a principle is basic and effective, it is
likely to have other general applications with
improved treatment solutions. Although not
applicable to every problem, there are other
examples of tissue or organ deficiency where
growth induction has been used with impres-
sive results. Deficiencies of skin and abdominal
wall are commonly treated with tissue expand-
ers to aid closure. These devices were initially
thought to involve only skin stretching, but
basic studies have shown a proliferative
response to the tension by the many cell types
involved [3]. For patients with a hypoplastic
cardiac ventricle, increased flow across the AV
valves induced growth and produced two usable
ventricles [4]. Longitudinal tension has induced
growth of the small intestine experimentally
[5]. Traction has lengthened intra-abdominal
testes and allowed a normal location [5]. For
these problems of organ deficiencies, as previ-
ously with LG-EA, the traditional treatments
have been palliative or otherwise unsatisfactory
for the long term. The use of biological solu-
tions and the induction of normal development
have moved the outlook for these patients far
closer to normal.
Of additional biological interest is that in
these applications of growth induction, the stimu-
lus is variable which may be important to a maxi-
mum response. Although the biomechanical
growth signal is increased at intervals, the
strength wanes as growth occurs. The importance
of variable signal strength to maximize growth
has been more rigorously demonstrated in cell
culture experiments [6]. Interestingly enough,
this important observation was made first,
although not fully recognized in the clinical treat-
ment of LG-EA.
As stated at the beginning of the Introduction,
the goal in pediatric therapy must be for 70 or
6 J.E. Foker
more good years, and this is a stringent require-
ment. The problems encountered in pediatric
patients are a complex mixture of developmen-
tal and genetic defects, which play out with a
continuing background of normal and abnormal
development. The aim of this book is to clarify
as much as possible these complex interactions
to guide the therapeutic options. Treatments
which enlist normal biological responses, we
believe, will have particular value [7]. As this
approach becomes better understood and the
applications expand in number, the goal of 70
good years will come into view for even the
more difficult of these problems.
References
1. Foker JE, Kendall Krosch TC, Catton K, Munro F, Khan
KM. Long gap esophageal atresia treated by growth
induction: the biological potential and early follow-up
results. Semin Pediatr Surg. 2009;18(1):23–9.
2. Mammoto T, Ingber DE. Mechanical control of
tissue and organ development. Development.
2010;137:1407–20.
3. Olenius M, Dalsgaard C, Wickman M. Mitotic activity
in expanded human skin. Plast Reconstr Surg.
1993;91:213.
4. Foker JE, Berry JM, Vinocur JM, Harvey BA, Pyles
LA. Two-ventricle repairs in the unbalanced AV canal
defect spectrum with mid-term follow-up. J Thorac
Cardiovasc Surg. 2013;146:854–60.
5. Safford SD, Freemerman AJ, Safford KM, et al.
Longitudinal mechanical tension induces growth in
the small bowel of juvenile rats. Gut. 2005;
54(8):1085–90.
6. Shehata SM. Laparoscopically assisted gradual con-
trolled traction on the testicular vessels: a new concept
in the management of abdominal testis. A preliminary
report. Eur J Pediatr Surg. 2008;18:402–6.
7. Syedain ZH, Weiberg JS, Tranquillo RT. Cyclic dis-
tension of fibrin-based tissue constructs: evidence of
adaptation during growth of engineered connective tis-
sue. Proc Natl Acad Sci. 2008;105:6537–42.
 Part II
The Esophagus
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