Congenital Esophageal Stenosis

153 Pages·2019·5.152 MB·English

by  Ashraf Ibrahim, Talal Al-Malki

Congenital
Esophageal Stenosis
Ashraf Ibrahim
Talal Al-Malki
123
Congenital Esophageal Stenosis
Ashraf Ibrahim • Talal Al-Malki
Congenital Esophageal
Stenosis
Ashraf Ibrahim Talal Al-Malki
Consultant Pediatric Surgeon Senior Consultant Pediatric and Neonatal
Armed Forces Hospital, Southern Region Surgeon, Alhada Military Hospital
King Fahad Military Hospital Vice President for D&Q, Taif University
Khamis Mushait Taif
Saudi Arabia Saudi Arabia
ISBN 978-3-030-10781-9 ISBN 978-3-030-10782-6 (eBook)
https://doi.org/10.1007/978-3-030-10782-6
Library of Congress Control Number: 2019935127
© Springer Nature Switzerland AG 2019
This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of
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does not imply, even in the absence of a specific statement, that such names are exempt from the relevant
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The publisher, the authors, and the editors are safe to assume that the advice and information in this book
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This Springer imprint is published by the registered company Springer Nature Switzerland AG
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
To my wife Aya for her love, encouragement,
and support
To my daughters Hager and Sarah
To my son Ibrahim, a growing pediatric
surgeon
To my grandsons Yossef and Eyad
And to my granddaughters Jasmine and
Farida
A. Ibrahim
To Fatmah, our first CES patient, who taught
us a lot about CES
Talal Al-Malki
Preface
Congenital esophageal stenosis (CES) is a complex
spectrum of diseases. It is our privilege to go into the
depth of this topic. It is complex due to its different
subtypes, different locations, and variable severity.
Furthermore, almost 60% of CES are associated with
esophageal atresia (EA), which adds to the complex-
ity of the disease. The diagnosis of CES may be chal-
lenging especially in the neonates. A neonate may
not pass the neonatal period without morbidities or
even mortality if CES is not properly diagnosed and
managed. Gastroesophageal reflux disease (GERD),
dysmotility, esophageal stricture, leakage, and recur-
rent tracheoesophageal fistula (TEF) will add to the
difficulty in managing CES. Treatment of isolated
CES seems to be much easier and straightforward
than that associated with EA.
Our aim is to improve the understanding of CES,
its diagnosis, and management. Every effort should
be made to avoid esophageal replacement surgery
and to have a patient free of dysphagia. We aim at a
good quality of life with the patient’s own
esophagus.
The aim of this comprehensive book is to help
trainees and interested surgeons to develop into tal-
ented surgeons in CES management. There are
enough radiographs, photographs, and tables to
facilitate understanding of the topic.
To the best of our knowledge, this is the first book
about CES in the literature. We hope the book will
be an important addition for surgery trainees and
pediatric and general surgeons.
vii
viii Preface
We wish to express our deep gratitude, thankfulness, and appreciation to
Professor Nader Morad (former chief of histopathology, College of Medicine and
Medical Sciences, King Khalid University, Abha, Kingdom of Saudi Arabia), who
was of great help in the histopathological part of this work.
Also, we wish to acknowledge our appreciation and gratitude to Mubarak
M.  Al-Shraim (Department of Pathology, College of Medicine and Medical
Sciences, King Khalid University, Abha, Kingdom of Saudi Arabia) for his close
supervision and valuable suggestions.
Appreciation also goes to Dr. Ahmed. Y. Abouelyazid (assistant professor and
consultant preventive medicine, Mansoura Faculty of Medicine, Egypt) for his
cooperation especially in the statistical work.
Last but not least, the authors are very thankful to Dr. Mohammed Ahmed
Algathradi (consultant radiologist and pediatric radiologist at King Khalid
University, Abha, Maternity and Children Hospital and Armed Forces Hospital,
southern region, Saudi Arabia) for his valuable contribution in the section of exter-
nal compression and vascular rings of the esophagus.
Khamis Mushait, Saudi Arabia Ashraf Ibrahim
Taif, Saudi Arabia Talal Al-Malki
Contents
1 General . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
Introduction and Background . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
Basic Notes About the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
Embryology of the Esophagus and CES . . . . . . . . . . . . . . . . . . . . . . . . . 2
Molecular Biology of Esophageal Atresia . . . . . . . . . . . . . . . . . . . . . . 4
The Upper Esophageal Pouch . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4
Development of CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
Future Directions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
Histology of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
Anatomy of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
Blood Supply of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
Lymphatic Drainage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
Innervation of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
Physiology of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
The Upper Esophageal Sphincter (UES) Function . . . . . . . . . . . . . . . 8
Esophageal Body Peristalsis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8
Lower Esophageal Sphincter (LES) Function . . . . . . . . . . . . . . . . . . . 9
Historical Notes About CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
2 Histology of the Atretic Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
Ultrastructural Changes of the Atretic Esophagus . . . . . . . . . . . . . . . . . . 18
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27
3 Etiology of Motility Disorders in EA and CES . . . . . . . . . . . . . . . . . . 29
Diagnostic Tools to Investigate Dysphagia in EA . . . . . . . . . . . . . . . . . . 33
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36
4 The Spectrum of CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
The Spectrum of CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
CES Associated Congenital Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . 40
Incidence of CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45
ix
x Contents
5 Diagnosis of CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
Diagnosis of CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
Upper Gastrointestinal Contrast Studies (UGI) . . . . . . . . . . . . . . . . . . . . 51
Normal Anatomy and Physiology of the Esophagus . . . . . . . . . . . . . . 52
Technique of UGI Study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52
Evaluating the Oral and Pharyngeal Phase . . . . . . . . . . . . . . . . . . . . . 52
Esophageal Phase Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53
Disorders of GEJ . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54
Anatomical and Functional Causes for GER in EA/TEF Patients . . . . . . 55
Outside Impressions of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . 55
Double Aortic Arch . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55
Left Sided Aortic Arch with an Aberrant Right Subclavian Artery . . . 57
Right Aortic Arch (RAA) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 57
Pulmonary Artery Sling . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60
6 Treatment of CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63
Dilatation of CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63
Surgery for CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65
Indications of Anti-reflux Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66
Gastrostomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67
Indications for Gastrostomy Tube (GT) Insertion . . . . . . . . . . . . . . . . . . 67
Gastrostomy Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68
Role of Gastrostomy in CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 70
The Antireflux Surgery in Pediatrics . . . . . . . . . . . . . . . . . . . . . . . . . . . . 71
Complications of the Anti-reflux Surgery . . . . . . . . . . . . . . . . . . . . . . . . 75
Fundoplication and CES Associated with EA . . . . . . . . . . . . . . . . . . . . . 78
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79
7 Anastomotic Stricture After EA Repair and Role of CES . . . . . . . . . 85
Diagnosis of Anastomotic Stricture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85
The Anastomotic Stricture Index (SI) . . . . . . . . . . . . . . . . . . . . . . . . . 85
The Esophageal Anastomotic Stricture Index (EASI) . . . . . . . . . . . . . 86
Time for First Assessment of Suspected AS . . . . . . . . . . . . . . . . . . . . 87
Risk Factors for Anastomotic Stricture (AS) . . . . . . . . . . . . . . . . . . . . 87
CES as a Risk Factor for Anastomotic and Distal
Esophageal Stricture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88
Treatment of Anastomotic Stricture (AS) . . . . . . . . . . . . . . . . . . . . . . . . 88
Dilatation of Esophageal Stricture . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90
Bougie or Balloon Dilators . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90
Indwelling Esophageal Balloon for Benign Stricture . . . . . . . . . . . . . 92
Adjuncts to Dilatation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 92
The Technique of Balloon Dilatation We Practice . . . . . . . . . . . . . . . . 94
Anastomotic Stricture in Non-CES Group has
a Better Response and Effectiveness to Dilatation
than CES Associated with EA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98
Contents xi
Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100
8 What Should Be Done if Dilatations with Adjuncts Fail? . . . . . . . . . 103
Endoscopic Electrocautery Incisional Therapy (EIT) . . . . . . . . . . . . . . . 103
Esophageal Stents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104
Managing Complete Loss of the Patency of the Esophageal Lumen . . . 108
The Refractory and the Recurrent Esophageal Stricture . . . . . . . . . . . . . 108
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110
9 Experience with Balloon Dilatation . . . . . . . . . . . . . . . . . . . . . . . . . . . 113
Experience with Balloon Dilatation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113
Dilatation for Cases of EA/TEF . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113
Dilatation for CES Associated with EA . . . . . . . . . . . . . . . . . . . . . . . . 114
Dilatation for Isolated CES Involving the GEJ . . . . . . . . . . . . . . . . . . 115
Dilatations of Isolated CES Proximal to GEJ . . . . . . . . . . . . . . . . . . . 116
Dilatations for Cricopharyngeal Achalasia . . . . . . . . . . . . . . . . . . . . . 118
Dilatation for Post Corrosive Stricture . . . . . . . . . . . . . . . . . . . . . . . . . 119
Key Points for Managing Esophageal Strictures . . . . . . . . . . . . . . . . . 120
Diagnostic Challenges of Isolated CES Involving the GEJ . . . . . . . . . . . 121
CES Wrongly Diagnosed as Cardiac Achalasia . . . . . . . . . . . . . . . . . . 121
Is it Cardiac Achalasia or CES? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 122
Stricture Due to Gastroesophageal Reflux Disease (GERD) . . . . . . . . 123
Cardiac Achalasia in Neonates and Infancy and Young Children . . . . 124
CES Involving the GEJ . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 126
Timed Barium Esophagogram . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 128
Surgical Management of Unresponsive Stricture . . . . . . . . . . . . . . . . 129
Surgical Resection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130
10 Congenital Membranous Disease of the Esophagus (MD) . . . . . . . . . 133
Types of MD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 133
The Non-Perforated Type of MD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 133
Congenital Perforated MD of the Esophagus . . . . . . . . . . . . . . . . . . . 134
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 134
11 Congenital Esophageal Stenosis in Adults . . . . . . . . . . . . . . . . . . . . . . 137
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 140
12 Future Directions for CES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141
The POEM for Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141
Esophageal Stents for Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 144
Summary and Conclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 145
Recommendations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 147