Handbuch der inneren Medizin

Begriindet yon L. Mohr und R. Staehelin

Herausgegeben yon

H. Schwiegk

Dritter Band: Verdauungsorgane Fiinfte, Yollig neu bearbeitete und erweiterte Auflage

Teil1 Esophagus

Springer-Verlag Berlin Heidelberg New York 1974

Diseases of the Esophagus

By

G. Vantrappen and J. Hellemans

With Contributions of

C. Debray' W. Deloof· V.J. Desmet· D.A.W. Edwards' J. Fevery G. Fransen' K. Geboes . J. De Groote' E. Hafter . A. Haney

P. Heitmann' P. Housset . J. Janssens' A. Lacquet H.P. Lazar' B. T. Le Roux . H. Monges . J. G. Pearson' W. Pelemans

E. Ponette . J. Pringot . J. A. Rinaldo' J. De Schryver E. C. Texter' G. N. Tytgat . P. Valembois . F. ViI ardell . B. S. Wolf

With 358 partly coloured Figures

Springer-Verlag Berlin Heidelberg New York 1974

GASTON R. VANTRAPPEN, M.D., Agg. H.D. Professor of Medicine

JAN J. HELLEMANs, M.D., Agg. H.D. Professor of Medicine

Department of Medical Research and Department of Medicine, Akademisch Ziekenhuis St. RafaeI, University of Leuven, B·3000 Leuven

A special US edition is available under the title G. VANTRAPPEN J. HELLEMANS, Diseases of the Esophagus

ISBN 978-3-642-86431-5 ISBN 978-3-642-86429-2 (eBook) DOI 10.1007/978-3-642-86429-2

Library of Congress Cataloging in Publication Data Diseases of the Esophagus

(Verdauungsorgane. T. 1) (Handbuch der inneren Medizin, Bd. 3. T. 1) Bibliography: p.

Esophagus-Diseases. II. V ANTRAPPEN, G., ed. III. HELLEMANS, J., ed. IV. Series: Handbuch der inneren Medizin. Bd. 3. T. 1.

RC41.H342 Bd. 3, T. 1 [RC815.7] 616'.026 74-13240

This work is subject to copyright. AII rights are reserved, whether the whole or part of the material is concerned, specifically those of translation, reprinting, re-use of illustrations, broadcasting, reproduction by photocopying

machine or similar means, and storage in data banks.

Under § 54 of the German Copyright Law where copies are made for other than private use, a fee is payahle to the publisher, the amount of the fee to be determined by agreement with the publisher.

© by Springer-Verlag, Berlin'Heidelberg 1974. Softcover reprint ofthe hardcover 1 st edition 1974

The use of registered names, trademarks, 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.

Typesetting,: Universitlitsdruckerei H. Stiirtz AG, Wiirzburg

List of Contributors

DEBRAY, c., M.D., Professeur, Medecin des Hopitaux, Chef du Service de Gastro­enterologie, Hopital Bichat, Paris, France

DELOOF, W., M.D., Department of Medicine, Akademisch Ziekenhuis St. Rafael, University of Leuven, 3000 Leuven, Belgium

DESMET, V. J., M.D., Professor of Pathology, Department of Medical Research and Department of Pathology, Akademisch Ziekenhuis St. Rafael, University of Leuven, 3000 Leuven, Belgium

EDWARDS, D. A. W., M.D., F.R.C.P., MRC Department of Clinical Research, University College, Hospital Medical School, University Street London WC1E 6JJ, Great Britain

FEVERY, J., M.D., Associate Professor of Medicine, Department of Medical Research and Department of Medicine, Akademisch Ziekenhuis St. Rafael, University of Leuven, 3000 Leuven, Belgium

FRANSEN, G., M.D., Department of Surgery, Akademisch Ziekenhuis St. Rafael, University of Leuven, 3000 Leuven, Belgium

GEBOES, K., M.D., Department of Medical Research and Department of Medicine, Akademisch Ziekenhuis St. Rafael, University of Leuven, 3000 Leuven, Bel­gium

GROOTE, De, J., M.D., Professor of Medicine, Department of Medical Research and Department of Medicine, Akademisch Ziekenhuis St. Rafael, University of Leuven, 3000 Leuven, Belgium

HAFTER, E., M.D., Spezialarzt fUr innere Medizin F.M.H., Magen-Darmkrank­heiten, T6distrasse 36, CH-8002 Zurich, Switzerland

HANCY, A., M.D., 27, Bd. d'Athenes, F-1300 Marseille, France

HEITMANN, P., M. D., Krankenanstalten Duren, Abteilung fUrinnere Krankheiten, 516 Duren, Germany

HELLEMANS, J., M.D. Agg. H.O., Professor of Medicine, Department of Medical Research, University of Leuven, Leuven, Belgium; Chief Geriatric Unit, De­partment of Medicine, Akademisch Ziekenhuis St. Rafael, 3000 Leuven, Bel­gium

HOUSSET, P., M.D., Attache de consultation it L'Hopital Bichat, Membre de la Societe Nationale Francaise de Gastro-Enterologie, Paris, France

JANSSENS, J., M.D., Aangesteld Navorser NFWO, Department of Medical Research and Department of Medicine, Akademisch Ziekenhuis St. Rafael, University of Leuven, 3000 Leuven, Belgium

LACQUET, A., M.D., Professor of Surgery, Department of Surgery and Department of Surgical Pathology, Akademisch Ziekenhuis St. Rafael, University of Leuven, 3000 Leuven, Belgium

VI List of Contributors

LAZAR, H. P., M.D., 700 North Michigan, Chicago, Ill, 60611, USA

LE Roux, B. T., Ch.M., F.R.C.S.E., Professor of Thoracic Surgery, Thoracic Unit, Department of Surgery, Wentworth Hospital, P. B. Jacobs, Durban, Natal, South Africa

MONGES, H., M.D., Professeur, Medecin des Hopitaux, Clinique des maladies de l'appareil digestif et de la nutrition, Hopital Nord, Marseille 15, France

PEARSON, J. G., M.D., Director of Radiotherapy, Provincial Cancer Hospitals Board, Dr. W. W. Cross Cancer Institute, 11560 University avenue, Edmonton, Alberta, T6G 1Z2, Canada

PELEMANS, W., M.D., Research Fellow, Department of Medical Research and Department of Medicine, Akademisch Ziekenhuis St. Rafael, University of Leuven, 3000 Leuven, Belgium

PONETTE, E., M.D., Assistant Professor of Radiology, Department of Radiology, Akademisch Ziekenhuis St. Rafael, University of Leuven, 3000 Leuven, Bel­gium

PRINGOT, J., M.D., Assistant Professor of Radiology, Department of Radiology, Cliniques Universitaires, U.C.L., 3000 Leuven, Belgium

RINALDO, J. A., Jr., M.D., Medical Director, Providence Hospital, 16001 W. Nine Mile Rd., Southfield, Michigan 48075, USA

SCHRYVER, DE, J., M.D., Wilhelmina Kinderziekenhuis, Nieuwe Gracht 137, Utrecht, The Netherlands

TEXTER, E. C., Jr., M.D., Professor of Medicine, Physiology and Biophysics, School of Medicine, Assistant Dean, School of Health, Related Professions, Associate Chief of Staff for Education, 300 E Roosevelt Rd., Little Rock, AR 72206, USA

TYTGAT, G. N., M.D., Lektor in Gastroenterologie, Akademisch Ziekenhuis bij de Universiteit van Amsterdam, Wilhelmina Gasthuis, Amsterdam, The Netherlands

V ALEMBOIS, P., M.D., Aangesteld Navorser N.F.W.O., Consultant in Surgery, Department of Medical Research and Department of Surgery, Akademisch Ziekenhuis St. Rafael, University of Leuven, 3000 Leuven, Belgium

V ANTRAPPEN, G., M.D. Agg. H.O., Professor and Chairman, Department of Medi­cal Research, University of Leuven, Leuven, Belgium; Chief Gastrointestinal Unit, Department of Medicine, Akademisch Ziekenhuis St. Rafael, 3000 Leu­yen, Belgium

VILARDELL,F., M.D.,D. Sc.Med., Director, School of Gastroenterology, Universidad Autonoma de Barcelona, Hospital Santa Cruz y San Pablo, Barcelona 13, Spain

WOLF, B. S., M.D., Chairman and Professor of Radiology, The Mount Sinai School of Medicine, 11 East 100th street, New York 10029, USA

Preface

This book aims to be a synthesis of our current knowledge about the normal and pathological esophagus. Although a number of excellent monographs on limited aspects of esophageal pathology are available, a recent handbook treating the whole of esophageal physiology and pathology is lacking. We attempted to present the collected material in such a way that even the neophyte in the field would not get lost in the wealth of data. For this reason we have included a number of illustrations such as classical radiological and endoscopic images, manometric tracings and uncomplicated graphs, which may seem superfluous for specialists but will be helpful to the reader who wants to be initiated in the subject. At the same time we tried to be fairly complete so as to make available to the esophageal specialist a book of references, to which he can readily turn when faced with rare diseases or unusual physiological or pathophysiological pheno­mena. In order to achieve both aims the authors often give their own point of view when faced with controversal topics, while classical as well as more recent features and concepts are mentioned and diverging opinions discussed.

Our knowledge about the structure and function of the normal and the patho­logical esophagus has increased considerably during the last few years. Our insight into the local and central innervation of the esophagus has improved; the behavior of the gastroesophageal sphincter has greatly been clarified and different types of esophageal motor responses have been recognized which, each in its own way, may be influenced pharmacologically. Therefore we thought it useful to thoroughly discuss the basic data, i.e. anatomy, histology, electron microscopy and physiology. Many diseases cause disturbances of esophageal mechanisms. The section on physiology was written to elucidate these mechanisms, starting from the normal motility. The examination of the esophagus has been improved by the refinement of the traditional methods, such as radiology, endoscopy and by the recent introduction of new techniques, such as electromyography, the acid infusion test and pH- and PD-measurements. Although the clinical and diagnostic value of these procedures has not yet been fully established, clinical correlations are discussed. In the chapter on diagnotic procedures, however, the stress is laid on procedures rather than on diagnosis, in order to minimize overlap with the chapters in which the diseases themselves are discussed.

There are many esophageal diseases in which the disorders or lesions of the esophagus are but a component of a more general condition. Motor disturbances may be found in collagen diseases, anatomical anomalies in various congenital malformations, mucosal lesions in several cutaneous diseases, esophageal varices in a series of extraesophageal conditions, etc. It is obviously unnecessary to give a detailed description of all these larger entities; for instance, we deemed it superfluous to elaborate on causes, symptoms and treatment of acute and chronic alcoholic intoxication because it may produce motility disturbances. Other conditions, such as systemic sclerosis, Chagas' disease and portal hypertension are discussed more thoroughly since their cause, evolution or treatment are important for the understanding of the esophageal component. In this discussion special prominence has been given to involvement of other parts of the gastroin­testinal tract.

VIn Preface

The necessity of subdividing the text in chapters and sections sometimes entails artificial classifications. For instance, hiatal hernia with gastroesophageal reflux, reflux esophagitis and peptic stenosis of the gullet are treated in three different sections, although they may be considered one coherent entity, which might equally well have been discussed under a single heading.

This book does not offer detailed descriptions of surgical interventions but surgery is treated to the extent that it may interest the non-surgeon.

We wish to express our gratitude to all those who have helped us so much in the preparation of this book. First of all we wish to mention the contributors, especially those not belonging to the Leuven group. Their competence and personal experience have greatly enhanced the value of this work. These, and the authors of our university, who have shown so much forbearance, will forgive us for the strict demands we imposed on them.

Our thanks are due also to our collegues of the Sint-Rafael and Saint-Pierre University Hospitals, who have allowed us to draw on the work of their depart­ments: at Sint-Rafael Prof. J. VANDENBROUCKE, head of the department of internal medicine, Prof. A. BAERT, head of the department of radiology, Prof. H. DEGREEF, head of the department of dermatology, and Dr. L. BROECKAERT of the section of gastroenterology; at Saint-Pierre, Prof. P. BODART, head of the department of radiology, Prof. P. J. KESTENS, head of the department of surgery and Prof. C. DIVE and Dr. R. FIASSE of the section of gastroenterology.

Dr. G. Tops (UFSIA) has ably assisted us in the translation of many texts into English.

Mr. RUMMENS carried out the photographic work on the pictures provided by the Leuven group. We also gratefully acknowledge the help of the Gevaert Company, Mortsel. We are particularly grateful for the secretarial assistance of Mrs. M. VANDERVEKEN, R. VERBIST and M. RUMBAUT, who have done a great job in the preparation of the manuscript. And last but not least our thanks are due to the Springer Verlag. The spirit of cooperation and the courtesy of Mr. BERGSTEDT has been a great help throughout the preparation of this book.

Leuven, October 1974 G. V ANTRAPPEN J. HELLEMANs

Contents

Chapter 1

Basic Data

Anatomy and Embryology. By G. FRANSEN and P. V ALEMBOIS. With 6 Figures 1. 1.1. 1.1.1. 1.1.2. 1.1.3. 1.2. 1.3. 1.4. 1.4.1. 1.4.2. 1.4.3. 1.5. 1.6. 1.6.1. 1.6.2. 1.7. 1.8. 1.8.1. 1.8.2. 2. References

Anatomy ..... . Topographic Anatomy The Cervical Esophagus The Thoracic Esophagus The Abdominal Esophagus The Musculature of the Esophagus Proper The Pharyngoesophageal Junction The Esophagogastric Junction . The Gastroesophageal Sphincter The Phrenoesophageal Membrane The Diaphragmatic Hiatus Arterial Blood Supply Venous Drainage . . Intrinsic Veins . . . Extrinsic Veins Lymphatic Drainage Nerve Supply (Innervation) Parasympathetic Innervation Sympathetic Innervation Embryology

1 1 1 1 3 3 4 6 6 6 7 7 9 9

10 10 11 12 12 13 13 15

Histology and Electron Microscopy. By V. J. DESMET and G. N. TYTGAT. With 8 Figures 17 1. Mucous Membrane (Tunica Mucosa) 17 1.1. Epithelium. . . . . 17 1.1.1. Squamous Epithelium 17 1.1.1.1. Histology..... 17 1.1.1.2. Cell Kinetics 19 1.1.1.3. Histochemistry 19 1.1.1.4. Electron Microscopy 21 1.1.2. Non.squamous Epithelial Structures, Cardia-type Glands 24 1.1.2.1. Histology..... 24 1.1.2.2. Electron Microscopy 27 1.2. Lamina Propria 27 1.3. Muscularis Mucosae 29 2. Tunica Submucosa 29 3. Tunica Muscularis 30 4. Angio-architecture 35 5. Innervation . . . 35 6. Adventitia, Elastic-muscular System, Serosa 37 References . . . . . . . . . . . . . . . . . . . . 38

Physiology. By J. HELLEMANS and G. VANTRAPPEN. With 20 Figures 40 1. The Functions of the Esophagus . 40 2. The Esophagus at Rest . . . . . . . . . . . . . . . 40 2.1. The Pharyngoesophageal Sphincter . . . . . . . . . . 40 2.1.1. Location..................... 40 2.1.2. The Pressure Profile of the Pharyngoesophageal Sphincter 41 2.1.3. Manometric Measurements of the Resting Pressure in the Pharyngoesophageal

Sphincter . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42

x

2.1.4. 2.1.5. 2.2. 2.2.1. 2.3. 2.3.1. 2.3.2. 2.3.3.

2.3.4. 2.3.5. 2.3.5.1. 2.3.5.2. 2.3.5.3.

2.3.5.4. 3. 3.1. 3.1.1. 3.1.2. 3.1.3. 3.1.3.1. 3.1.3.2. 3.1.3.3. 3.1.4. 3.1.4.1. 3.1.4.2. 3.1.4.3. 3.1.5. 3.2. 3.2.1.

3.2.1.1. 3.2.1.2. 3.2.1.3. 3.2.1.4. 3.2.2. 3.2.2.1. 3.2.2.2. 3.2.2.3. 3.2.2.4. 3.2.3. 3.3. 3.3.1. 3.3.1.1. 3.3.1.1.1. 3.3.1.1.2. 3.3.1.2. 3.3.1.3. 3.3.1.4. 3.3.1.5. 3.3.1.6. 3.3.1.7. 3.3.2. 3.3.2.1. 3.3.2.2. 3.3.2.3. 3.3.2.4. 3.3.2.4.1. 3.3.2.4.2. 4. 4.1. 4.2. 4.3. 4.3.1.

Contents

Respiratory Pressure Variations 43 Elasticity or Tonic Contraction 43 The Esophagus Proper 45 The Resting Pressure . . . . . 45 The Lower Esophageal Sphincter (L.E.S.) (Gastroesophageal Sphincter) 45 The Position of the Sphincter in Relation to the Squamocolumnar Junction 46 The Pressure Profile of the Sphincter. The Pressure Inversion Point (P.I.P.) 46 Manometric Measurements of the Resting Pressure in the Lower Esophageal Sphincter . . . . . . . . . . . . . . . 48 Other Measurements of Sphincter Strength 50 Variations of the Sphincteric Pressure 51 The Intrinsic Sphincteric Properties 51 Hormonal Control of the Sphincter Strength 53 The Response of the Lower Esophageal Sphincter to Increased Intragastric Pressure ....................... 56 Nervous Control of the Lower Esophageal Sphincter Pressure 57 Bolus Transport. Primary Peristalsis 59 Mechanical Activity 59 Oral Transport . . . . . . . . . . 59 Pharyngeal Transport. . . . . . . 62 Electromyographic Studies of Deglutition 63 Leading Complex . . . . . . . 63 Constrictor Musculature . . . . 64 Variations in Ancillary Activity 65 Esophageal Transport . . . . . 65 The Peristaltic Progression 65 Longitudinal and Circular Musculature 65 Striated and Smooth Muscles 67 Transport through the Gastroesophageal Sphincter 68 Intraluminal Pressure Variations . . . . . . . . 69 The Deglutition Complex in the Pharynx and in the Pharyngoesophageal Sphincter . . . . . . . . . . . . 69 Relaxion of the High Pressure Zone 69 The e-(Elevation) Wave . . . . . . 71 The t-(Tongue) Wave . . . . . . . 71 The p-(Peristaltic) Wave ..... 71 The Deglutition Complex in the Esophagus 71 The Initial Negative Deflexion 72 The First Positive Wave 72 The Second Positive Wave 73 The Peristaltic Contraction 73 The Deglutition Complex in the Gastroesophageal Sphincter 74 Innervation . . . . . . . . . . . . . . . . 75 Innervation of Oropharyngeal Phase . . . . . 75 The Deglutition Center in the Rhombencephalon 75 The Existence of a Deglutition Center 75 Localization of the Deglutition Center 75 The Peripheral Afferent System 76 Elementary Reflexes Versus Swallowing 77 The Central Afferent System ..... 78 The Cortical Control of the Deglutition Center 78 Interference with Other Centers 80 Efferent Pathways. Motoneurons . . 81 Innervation of the Esophageal Phase 82 Central Nervous Centers 82 Afferent Pathways . . . . . . 82 Efferent Pathways . . . . . . 84 The Intramural Nervous System 85 The Motor Neurons. . . . . . 85 Local Nerve Fibers . . . . . . 85 Reflex Responses of the Esophagus 86 Secondary Peristalsis . . . . . . . 86 Esophageal Propulsive Force (E.P.F.) 87 On- and off-Response; Duration Response 87 The "On" Response (Circular Muscle) 87

Contents

4.3.2. The "Off" Response (Circular Muscle) 4.3.3. The Duration Response (Longitudinal Muscle) 4.4. Inhibition . . . . . . . . . . . . . . . 4.4.1. Deglutitive Inhibition ........ . 4.4.2. Inhibition by Distension ....... . 4.5. Relaxion of the Gastroesophageal Sphincter 4.6. Other Reflexes . . . . 5. Retrograde Transport . 5.1. Rumination-Eructation 5.2. Retching and Vomiting 5.3. Belching References . . . . . . . . . . .

Chapter 2

Diagnostic Procedures

History and Symptoms of Esophageal Disease. By D. A. W. EDWARDS. 1. 1.1. 1.1.1. 1.1.1.1. 1.1.1.2. 1.1.1.3. 1.1.2. 1.1.2.1. 1.1.2.2. 1.1.2.3. 1.1.2.4. 1.1.2.5. 1.1.3. 1.1.3.1. 1.1.3.2. 1.1.3.3. 1.2. 1.2.1. 1.2.2. 1.2.2.1. 1.2.2.2. 1.2.3. 2. 2.1. 2.1.1. 2.1.2. 2.1.3. 2.2.

Symptoms and Syndromes Symptoms and their Significance The Sensation of Obstruction The Site of the Obstruction The Site of the Receptors . The Sensation of "Choking" Regurgitation . . . . . . The Volume of Regurgitate The Taste ....... . The Content . . . . . . . The Timing ...... . The Association with Position Pain ..... The Distribution The Character . . . . . . . The Timing ....... . Syndromes ....... . "Spill-Over" and "Spill-Into" Disease Obstruction Syndromes . . . . . . . The Stricture Syndrome ..... . The Achalasia Syndrome . . . . . . The So-Called "Functional" Dysphagias History ..... . Pharyngeal Problems Pouch ..... . Stricture or Web .. Muscular and Neural Esophageal Problems

XI

88 89 89 89 91 91 91 92 92 92 93 93

With 5 Figures 103 103 104 104 104 105 105 105 105 106 106 106 106 107 107 107 107 108 108 109 109 110 111 112 113 113 114 114 114

Radiological Examination of the Esophagus. By J. PRINGOT and E. PONETTE. With 72 Figures ............ 119 1. The Radiological Technique . . 119 1.1. Equipment. . . . . . . . . 119 1.1.1. The Standard Diagnostic X-Ray Unit 119 1.1.2. The Image Intensifier . . . . . . . . 122 1.1.3. Television Fluoroscopy . . . . . . . 125 1.1.4. Spot Filming ........... 126 1.2. The Recording Techniques Used with Image Intensifiers 127 1.2.1. Photofluorography............. 127 1.2.2. Cinefluorography . . . . . . . . . . . . . . 128 1.3. Techniques of Recording the Television Images 131 1.3.1. Cinefluorography . . . . . 131 1.3.2. Video Recording . . . . . 131 2. Contrast Materials . . . . 133 2.1. Positive Contrast Materials 133 2.1.1. Barium Sulfate ..... 133 2.1.2. Iodinated Contrast Materials . 135

XII Contents

2.1.3. Tantalum Powder .. . . . 137 2.2. Negative Contrast Materials . 137 3. The Radiological Examination 137 3.1. General Principles . . . . . 137 3.2. Normal Radiological Anatomy and Physiology 138 3.2.1. Hypopharynx and Cervical Esophagus 138 3.2.1.1. Radiological Landmarks ......... 138 3.2.1.2. Hypopharynx and Cervical Esophagus during Swallowing 141 3.2.1.3. Hypopharynx and Cervical Esophagus after Swallowing 146 3.2.1.4. The Modified Val salva Maneuver 147 3.2.2. Thoracic Esophagus 147 3.2.2.1. Normal Motor Activity . . 148 3.2.2.2. Normal Esophagogram 150 3.2.3. Esophagogastric Region . . 154 3.3. Preparation of the Patient . 156 3.4. Radiological Examination without Contrast Material 157 3.4.1. The Air Esophagogram . . . . . . . . . . . . . 157 3.4.2. Mediastinal Widening . . . . . . . . . . . . . . 159 3.4.3. Mediastinal Calcifications . . . . . . . . . . . . 161 3.4.4. Signs Related to Esophageal Perforation or Rupture 163 3.4.5. Retention of Contrast Material . . . . . . . . . 165 3.5. Radiological Examination with Contrast Material 165 3.5.1. Basic Technique of Examination 165 3.5.2. Abnormal Images ...... 166 3.5.2.1. Filling Defects and Impressions 166 3.5.2.1.1. Malignant Tumors . . . . . . 166 3.5.2.1.2. Intraluminal Defects . . . . . 171 3.5.2.1.3. Intramural Extramucosal Defects 173 3.5.2.1.4. Impressions . . . 175 3.5.2.2. Narrowings 178 3.5.2.2.1. Circular Tumors . 178 3.5.2.2.2. Benign Strictures 179 3.5.2.2.3. Rings...... 180 3.5.2.3. Changes in Mucosal Pattern 180 3.5.2.4. Crater-like Images 180 3.5.2.5. Diverticula, Perforations, Fistulas 184 3.6. Special Procedures . . . . . . . 184 3.6.1. Examination of the Pharyngoesophageal Region 184 3.6.1.1. Examination without Contrast Material 184 3.6.1.1.1. Technique of Examination 184 3.6.1.1.2. Abnormal Images ........ 184 3.6.1.2. Examination with Contrast Material 186 3.6.1.2.1. Single Films of the Swallowing Act . 187 3.6.1.2.2. Single Films after Swallowing . . . 187 3.6.1.2.3. Rapid Photofluorography of the Swallowing Act 187 3.6.1.2.4. Cinefluorography of the Swallowing Act . . . . 187 3.6.1.2.5. Contrast Laryngopharyngography . . . . . . 187 3.6.1.3. Abnormal Images of the Opacified Pharyngoesophageal Region 190 3.6.1.3.1. Cricopharyngeal Indentation 190 3.6.1.3.2. Filling Defects . . . . 190 3.6.1.3.3. Extrinsic Compression 190 3.6.1.3.4. Narrowing...... 190 3.6.1.3.5. Obstruction . . . . . 191 3.6.1.3.6. Dilatation of the Pharynx 191 3.6.1.3.7. Diverticula and Pouches . . 191 3.6.1.3.8. Motility Disorders of the Pharyngoesophageal Region 191 3.6.2. The Demonstration of Esophageal Varices . . . . . 192 3.6.3. Pharmacoradiography............. 194 4. The Radiological Examination of the Esophagus in Children 194 References . . . . . . . . . . . . . . . . . . . . . . . . . . . 198

Esophagoseopy. By P. HoussET, A. SIMOENS and CR. DEBRAY. With 16 Figures 204 1. Introduction . . . . . . . . . . . . . . . . . . . . . . 204 2. Common Recommendations for All Endoscopic Examinations 204 3. Equipment and Techniques . . . . . . . . . . . . . . . 204

3.1. 3.1.1. 3.1.2. 3.1.3. 3.2. 3.2.1. 3.2.2. 3.2.3. 3.2.3.1. 3.2.3.2. 3.2.3.3. 3.2.3.4. 4. 5. 5.1. 5.1.1. 5.1.2. 5.2. 5.2.1. 5.2.2. 5.2.3. 5.2.4. References

Contents

Rigid Esophagoscopy . . . . . . . . . . . . . . . . The Esophagoscope of Segal and Dubois de Montreynaud The Eder-Hufford Esophagoscope Results of Rigid Esophagoscopy Flexible Esophagoscopy . . Principle of the Fiberscopes . . Instruments ........ . Examination Technique . . . . Preparation and Positioning of the Patient Insertion of the Apparatus and Exploration Associate Maneuvers . . . . . . Advantages and Drawbacks ... Indications and Contraindications Accidents and Incidents Accidents . Perforation Hemorrhage Incidents .. . Failure to Pass Pharyngoesophageal Sphincter Respiratory Spasm Pain Bleeding

Exfoliative Cytology of the Esophagus. By F. VILARDELL. With 9 Figures 1. Introduction . . . . . . . . . . . . . . 2. Esophageal Cytological Techniques . . . . 2.1. Techniques Combined with Esophagoscopy 2.2. Abrasive Techniques 2.3. Lavage Techniques . . . . . . . . 2.4. Combined Techniques . . . . . . . 3. An Appraisal of Cytologic Techniques 4. Normal Cytology of the Esophagus . 5. Criteria for Malignancy in Esophageal Cytology 6. Cyto-Histological Correlations . . 7. Indications of Esophageal Cytology . . . . . . 8. Results of Esophageal Cytology .. . . . . . 9. Diagnostic Errors ............ . 10. Cytology Compared with Other Diagnostic Techniques 11. Fluorescent Techniques in Esophageal Cytology 12. Cytology in Beningn Conditions of the Esophagus 13. Effects of Roentgen Therapy on Esophageal Cells 14. Cytology in the Early Diagnosis of Esophageal Cancer References . . . . . . . . . . . . . . . . . . . . . . . . .

XIII

205 205 206 206 207 207 207 209 209 210 213 214 215 216 216 216 216 216 216 217 217 217 217

218 218 218 218 219 220 220 221 221 221 225 225 225 226 228 228 229 230 231 232

The Manometric Examination of the Esophagus. By W. PELEMANS and E. C. TEXTER, JR. With 6 Figures .............. 235 1. The Balloon-Kymographic Method . . . . . . . . . 235 2. Intraluminal Pressure Measurements . . . . . . . . 236 3. Interpretation of Intraluminal Pressure Measurements 238 4. The Perfused Catheter System . . . . . . . . . . . 238 5. The Technique of Intraluminal Pressure Measurements 243 References . . . . . . . . . . . . . . . . . . . . . . . . . 243

pH Measurements. By W. PELEMANS and G. V ANTRAPPEN. With 2 Figures 1. pH Measurements in vitro . . . 2. pH Measurements in vivo . . . 3. The Pull-trough Technique 4. Fixed Location of pH Electrode 5. Interpretation of Results 6. The Acid Clearing Test . . . 7. Protracted pH Measurements References . . . . . . . . . . . . . .

246 246 246 247 248 249 250 251 251

XIV Contents

PD Measurements. By J. JANSSENS and G. VANTRAPPEN. With 1 Figure 1. History ............. . 2. The Origin of the Potential Difference 3. Techniques of PD Measurements . . 4. PD Measurements in the Esophagus References . . . . . . . . . . . . . . . . . .

The Acid Infusion Test. By W. PELEMANS and G. VANTRAPPEN 1. Procedure . . . . . . 2. Hazards of the Test 3. The Mechanism of Pain References . . . . . . . . . . .

Pharmacological Tests. By J. JANSSENS and J. HELLEMANs. With 2 Figures References . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

Electromyography of the Esophagus. By J. HELLEMANs, G. VANTRAPPEN and J. JANS-

253 253 253 255 257 259

262 262 263 263 265

266 269

SENS. With 14 Figures 270 1. Introduction. . . . . . . . . 270 2. Registration Method . . . . . . 272 3. The Electromyographic Tracings . 273 3.1. The Pharyngoesophageal Sphincter 273 3.2. The Striated Muscle Segment of the Esophagus . . 274 3.3. The Transitional Zone between Striated and Smooth Muscles 275 3.4. The Smooth Muscle Segment of the Esophagus 276 3.5. The Gastroesophageal Sphincter . . . . 279 4. The Deglutitive Inhibition. . . . . . . 280 5. Electromyography in Esophageal Diseases 282 References . . . . . . . . . . . . . . . . . . 284

Chapter 3

Motility Disturbances of the Esophagus

Achalasia. By G. VANTRAPPEN and J. HELLEMANs. With 27 Figures 1. 2. 2.1. 2.2. 2.3. 3. 4. 4.1. 4.2. 4.2.1. 4.2.1.1. 4.2.1.2. 4.2.1.3. 4.2.1.4. 4.2.2. 5. 5.1. 5.2. 6. 6.1. 6.1.1. 6.1.2. 6.1.2.1. 6.1.2.2. 6.1.2.3. 6.1.2.4. 6.2. 6.2.1. 6.2.2.

Definition . . . Incidence ............. . Age Distribution . . . . . . . . . . . Sex Distribution . . . . . . . . . . . Geographical Distribution and Incidence Achalasia in Animals . . . . Etiology and Pathogenesis. . Genetic Factors . . . . • . Neuromuscular Abnormalities Anatomical Lesions . . . . . The Nuclei of the Brainstem . Vagal Nerve Fibres ..... The Intramural Nerve Plexus Smooth Muscle Pharmacological Defects Clinical Features . . . Symptoms and Signs . Vigorous Achalasia . . Technical Examinations Radiology ..... . Plain Chest Film . . . Radiological Examination with Contrast Medium Minimal Achalasia Mild Achalasia . . Moderate Achalasia ............ . Severe Achalasia . . . . . . . . . . . . . . Manometry ............... . Resting Pressures in the Gastroesophageal Sphincter Resting Pressure in the Esophagus . . . . . . . .

287 287 288 288 288 288 289 289 289 290 290 290 290 291 291 292 293 293 296 297 297 297 300 300 301 302 303 304 304 305

Contents xv

6.2.3. The Deglutitive Response in the Gastroesophageal Sphincter . 305 6.2.4. Deglutitive Responses in the Esophagus 307 6.3. Mecholyl Test . . . . . 308 604. Cytological Examination 308 6.5. Endoscopic Examination 308 7. Differential Diagnosis . . 309 7.1. Other Causes of Megaesophagus 309 7.1.1. Chagas' Disease .. . . . 309 7.1.1.1. Acute Phase . . . . . . . 309 7.1.1.2. Chronic Chagas' Syndrome 310 7.1.1.3. Megacolon........ 310 7.1.1.4. Megaesophagus ..... 311 7.1.2. Esophageal Cancer . . . . 312 7.1.3. Rare Causes of Esophageal Dilatation 313 7.2. Other Causes of Aperistalsis 313 7.3. Achalasia-like Disorders . . 314 704. Diffuse Spasm . . . . . . 316 7.5. Post-Vagotomy Dysphagia 316 7.6. Differential Diagnosis in Children. 316 8. Complications and Associated Diseases 317 8.1. Carcinoma. . . . . . . 317 8.1.1. Incidence....... 317 8.1.2. Age and Sex Distribution 318 8.1.3. Pathology . . . . . 318 8.1.4. Pathogenesis.... 318 8.1.5. Signs and Symptoms 318 8.1.6. Treatment..... 318 8.2. Esophagitis. . . . 319 8.3. Bronchopulmonary Complications 319 8.4. Perforation 319 8.5. Associated Conditions 320 9. Natural History 321 10. Treatment 321 10.1. Medical and Psychiatric Therapy 321 10.2. Dilatations 321 10.2.1. Types of Dilators. . 322 10.2.1.1. Mechanical System . 322 10.2.1.2. Hydrostatic System 322 10.2.1.3. Pneumatic System . 323 10.2.2. Preventive Measures 324 10.2.3. Results...... 325 10.2.3.1. Immediate Results . 325 10.2.3.2. Immediate Complications 325 10.2.3.3. Late Results . . . . . . 327 10.3. Surgical Treatment . . . 331 10.3.1. Techniques......... . . . . . . . . 331 10.3.1.1. Operations Based on the Theory of "Idiopathic Dilatation" . . . . . . . 331 10.3.1.2. Operations Based on the Theory of Disturbance in Esophageal Innervation 331 10.3.1.3. Operations Aimed at Decreasing the Resistance at the Cardia 332 10.3.1.4. Operations to Replace Part of the Esophagus by Intestine 333 10.3.2. Results and Complications of Heller's Myotomy 334 10.3.2.1. Results.............. 334 10.3.2.2. Complications........... 336 lOA. Sphincteric Pressures after Treatment. 338 10.5. Myotomy or Forceful Dilatation? 339 References . . . . . . . . . . . . . . . . . . 341

Diffuse Esophageal Spasm. By G. VANTRAPPEN and J. HELLEMANs. With 3 Figures 355 1. Definition 355 2. Incidence . 355 3. Pathology . 356 4. Symptoms . 356 5. Technical Examinations 357 5.1. Radiology . . . . . . 357 5.2. Manometric Examinations 358

XVI Contents

6. Related Conditions . . . . . . . . . . . . . . . . 6.1. Idiopathic Muscular Hypertrophy of Lower Esophagus 6.2. The Hypertensive Sphincter 7. Diagnosis . . . . 8. Treatment .... 8.1. Medical Treatment 8.2. Dilatation . . . . 8.3. Surgical Treatment References . . . . . . .

Post-Vagotomy Dysphagia. By D. A. W. EDWARDS. With 1 Figure 1. Definition . 2. Incidence . . . . . . . . 3. Symptoms. . . . . . . . 4. Etiology and Pathogenesis 5. Treatment References . . . . . . . . . . . .

Presby esophagus. By J. HELLEMANS and G. VANTRAPPEN. With 5 Figures 1. Pharynx ....... . 2. Esophagus ....... . 3. Gastroesophageal Sphincter 4. Nature of the Lesions References . . . . . . . . . . . . .

Esophageal Motility in Neonatal Infants. By J. DE SCHRYVER and J. HELLEMANS 1. Introduction . . . . 2. Normal Motility . . . 2.1. Mouth and Pharynx 2.2. Esophagus. . . . . . 2.2.1. The Esophagus at Rest 2.2.1.1. Pharyngoesophageal Sphincter 2.2.1.2. Esophageal Body . . . . . . 2.2.1.3. Gastroesophageal Sphincter . 2.2.2. Deglutition........ 2.2.2.1. Pharyngoesophageal Sphincter 2.2.2.2. Esophageal Body 2.2.2.3. Gastroesophageal Sphincter . References

Motor Disorders Due to Collagen Diseases. By J. HELLEMANS and G. VANTRAPPEN. With 2 Figures ...... . 1. Progressive Systemic Sclerosis . . . . . . . . . . . . 1.1. Definition and General Data . . . . . . . . . . . . . 1.2. Esophageal Involvement in Progressive Systemic Sclerosis 1.2.1. Incidence....... 1.2.2. Pathology . . . . . . . 1.2.3. Radiological Examination 1.2.4. Manometric Examination 1.2.5. Differential Diagnosis . . 1.2.6. Treatment . . . . . . . 2. Systemic Lupus Erythematosus 3. Polymyositis-Dermatomyositis 4. Related Syndromes . . . . . . References . . . . . . . . . . . . . . .

Motor Disorders Due to Muscle Disorders. By G. V ANTRAPPEN and J. HELLEMANS. With 3 Figures .............. . 1. Myotonic Dystrophy (Steinert's Disease) 1.1. Definition and General Data . . . . . . 1.2. Esophageal Involvement ....... . 2. Ocular Myopathy and Oculopharyngeal Myopathy 2.1. Definition and General Data . . . . . . . . . .

361 361 362 362 364 364 364 364 365

367 367 367 367 367 370 370

372 372 372 375 377 377

379 379 379 379 379 379 379 380 380 381 381 381 381 381

383 383 383 385 385 386 387 387 388 389 389 389 390 390

394 394 394 394 397 397

Contents XVII

2.2. Pharyngoesophageal Involvement . 3. Myasthenia Gravis ...... . 3.1. Definition and General Data . . . 3.2. Pharyngoesophageal Involvement 4. Endocrine Disorders of Muscle .. References . . . . . . . . . . . . . . . .

Motor Disorders Due to Lesions of the Central Nervous System. By J. HELLEMANs and

397 398 398 398 399 400

G. V ANTRAPPEN ...... 402 1. Brainstem Lesions . . . . . 402 2. Poliomyelitis. . . . . . . . 402 3. Motor Neuron Disease 403 4. Extrapyramidal Disturbances 404 5. Stiff-man Syndrome 404 6. Dysautonomia . . . . . . . 404 References . . . . . . . . . . . . . . 404

Motor Disorders Due to Peripheral Nerve Lesions. By J. HELLEMANs and G. VANTRAPPEN 407 1. Motor Disorders Associated with Diabetes ....... 407 2. Motor Disturbances Associated with Alcoholic Neuropathy ....... 408 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 408

Emotional Disorders of the Esophagus. By H. MONGES and A. HANey. With 6 Figures 409 1. Globus Hystericus . . . . 409 1.1. Definition and Generalities 409 1.2. Clinical Findings 409 1.3. Diagnosis . . . . . 409 1.4. Interpretation . . . 410 1.5. Treatment. . . . . 410 2. Esophageal Belching 410 2.1. Definition . . . 410 2.2. Etiology. . . . 410 2.3. Clinical Findings 410 2.4. Pathophysiology 411 2.5. Treatment 413 3. Eructation or Belching 414 3.1. Definition . . . . . . 414 3.2. Mechanism of Eructation 414 3.3. Treatment. . . . . . . 415 4. Merycism or Rumination 415 4.1. Merycism in Infants 415 4.1.1. Clinical Data. . . . 416 4.1.2. Interpretation.. . 416 4.1.3. Treatment..... 417 4.2. Merycism in Adults 417 4.2.1. Clinical Data. 417 4.2.2. Mechanism 418 4.2.3. Treatment.. 419 References . . . . . . . 421

The Pathophysiologieal Basis 01 Gastroesophageal and Intestinoesophageal Reflux. By P. HEITMANN ................................. 422 1. The Gastroesophageal Closing Mechanism . . . . . . . . . . . . . . . 422 2. Conditions Associated with Gastroesophageal or Intestinoesophageal Reflux 424 2.1. Sliding Hiatal Hernia ........................ 424 2.2. Gastroesophageal Incompetence in the Absence of Demonstrable Hiatal

Hernia . . . . . . . . . . . . . . . . . . . . . . . . . . 425 2.3. Scleroderma with Esophageal Involvement and Related Conditions 425 2.4. Following Myotomy for Achalasia of the Esophagus . 425 2.5. Vagotomy. . . . . . . . . 425 2.6. Pregnancy. . . . . . . . . 426 2.7. Gastric Operations . . . . . 426 2.8. Prolonged Gastric Intubation 426 References . . . . . . . . . . . . . . 427

XVIII Contents

Chapter 4 Tumors of the Esophagus

Benign Tumors and Cysts of the Esophagus. By G. V ANTRAPPEN and J. PRINGOT. With 6 Figures ..... . . 431 1. Classification. . . . . . . . 431 2. Incidence . . . . . . . . . 432 3. Intramural Tumors and Cysts 433 3.1. Leiomyoma. . . . . 433 3.1.1. Age and Sex Incidence 433 3.1.2. Pathology . . . . 433 3.1.3. Symptoms. . . . 436 3.1.4. Radiographic Signs 436 3.1.5. Esophagoscopy 438 3.1.6. Diagnosis.. 439 3.1.7. Complications. 441 3.1.8. Treatment... 441 3.2. Cysts . . . . . 441 4. Intraluminal Tumors 443 4.1. Fibrovascular Polyp 443 4.2. Papillomas 444 4.3. Adenomas . . 444 5. Hemangiomas 444 References . . . . . . . 445

Malignant Tumors of the Esophagus. By J. G. PEARSON and B. T. LE Roux. With 20 Figures .......... 447 1. Esophageal Carcinoma 447 1.1. Incidence and Etiology 447 1.2. Pathology . . . . . . 448 1.2.1. Squamous Cell Carcinoma 449 1.2.1.1. Site......... 449 1.2.1.2. Size......... 449 1.2.1.3. Macroscopic Appearance. 449 1.2.1.4. Microscopic Appearance 449 1.2.1.5. Direct Spread . . . . 451 1.2.1.6. Lymphatic Metastases 454 1.2.1.7. Blood·Borne Metastases 455 1.2.2. Adenocarcinoma.... 456 1.2.3. Mixed Squamous and Adenocarcinoma 458 1.3. Clinical Features, Natural History, Symptoms and Signs 458 1.4. Investigations . . . 460 1.4.1. Radiology . . . . . . . . . 460 1.4.2. Endoscopy........ 462 1.4.3. Esophageal Cytology . . . . 463 1.4.4. Cervical Lymph Node Biopsy 463 1.4.5. Mediastinoscopy, Pneumomediastinography and Laparotomy 463 1.4.6. Other Investigations . . . . . . . . . . . . . . . . . . 464 1.5. Prognosis . . . . . . . . . . . . . . . . . . . . . . . 464 1.5.1. Factors Influencing Prognosis . . . . . . . . . . . . . . . . . 464 1.5.1.1. The Balance between the Aggressiveness of the Tumor and the Resistance of

1.5.1.2. 1.5.1.3. 1.5.1.4. 1.5.1.5. 1.5.1.6. 1.5.1.7. 1.6 1.6.1. 1.6.2. 1.6.3. 1.6.3.1. 1.6.3.2. 1.6.3.3.

the Patient 464 Treatment 465 Sex. . . . • . . . . . 465 S~. . . . . . . . . . 400 Histology . . . . . . . 466 Condition of the Patient . 466 The Community in which the Patient Lives 466 Treatment. . . . . . . . . . . . . . 466 Comparison of Surgery and Radiotherapy 467 Anatomical Classification . . . . . 470 Radiation Therapy . . . . . . . . 470 Squamous Carcinoma . . . . . . . 470 The Level of the Esophageal Cancer 477 Adenocarcinoma . . . . . . . . . 477

Contents XIX

1.6.4. 1.6.4.1. 1.6.4.1.1. 1.6.1.1.2. 1.6.4.1.3. 1.6.4.1.4. 1.6.4.1.5. 1.6.5. 1.6.6. 1.6.7. 1.6.8. 1.6.9. 1.6.9.1. 1.6.9.2. 1.6.9.3.

Surgical Treatment. . . . . . . . . . . . . . . 477 Complications of Esophagogastrectomy . . . . . . 479 Disruption or Leakage at the Site of the Anastomosis 479 Hemorrhage from Aortic Erosion . . . . . . . 479 Death from Pulmonary Infection. . . . . . . 480 Pulmonary Embolism and Myocardial Infarction 480 The Pleural Complications . . . . . . . . 480 Preoperative and Postoperative Irradiation 480 Intubation ............ 481 Cancer Chemotherapy. . . . . . . . . . 481 Treatment of Esophagotracheal Fistula . . 482 Results of Treatment . . . . . . . . . . 483 Results of Surgical Management . . . . . . . . . . . . . . . .. 483 Results of Management of Squamous Esophageal Cancer by Irradiation . . 484 Results of Management of Squamous Esophageal Cancer by Irradiation and Surgery . . . . . . . . . . . . . 484

2. Sarcoma. . . . . . . . . . . . 484 3. Pseudosarcoma and Carcinosarcoma 485 4. Malignant Melanoma . . . . . . . 486 5. Metastatic Tumors . . . . . . . . . . 486 6. Other Rare Malignant Tumors of the Esophagus 486 6.1. Carcinoid Tumor . . . . . 486 6.2. Paget's Disease. . . . . . . . . . 486 6.3. Hodgkin's Disease . . . . . . . . 486 6.4. Granular Cell Myoblastoma . . . . 487 6.5. Verrucous Squamous Cell Carcinoma 487 Acknowledgements 487 References . . . . . . . . . . . . . 487

Chapter 5

Inflammatory Lesions of the Esophagus

Reflux Esophagitis. By B. S. WOLF and H. P. LAzAR. With 12 Figures 1. Introduction . . . . . 2. General Considerations 2.1. Hiatal Hernia 2.2. Esophagitis. 2.3. Reflux 2.4. Heartburn. . 2.5. Pathogenesis . 3. Clinical Features . 4. Roentgen Features 5. Endoscopy 6. Manometry . . . . 7. Diagnosis and Differential Diagnosis 8. Therapy ........... . 9. Other Types of Reflux Esophagitis . References . . . . . . . . . . . . . . . . .

Lower Esophagus Lined with Columnar Epithelium. By P. HEITMANN. 1. Historical Aspects, Definition, Incidence and Distribution 2. Pathologic Anatomy 3. Pathophysiology . . 4. Symptomatology . . 5. Diagnosis . . . . . 6. Differential Diagnosis 7. Treatment References . . . . . . . . . .

493 493 493 494 497 499 501 502 504 505 514 515 515 517 519 521

With 12 Figures 525 525 526 529 531 532 535 536 537

Caustic Lesions of the Esophagus. By W. PELEMANS and J. HELLEMANs. With 8 Figures 539 1. Incidence . . 539 2. Etiology. . . 539 3. Pathogenesis. . . . . . . . . . . . . . . . . . . . . . . . . . . . 539

xx Contents

4. Pathology . . . . . . . . . . . . . 4.1. The Acute Necrotic Phase . . . . . . 4.2. The Ulceration and Granulation Phase 4.3. The Phase of Cicatrization and Stricture Formation. 5. Clinical Features 6. Diagnosis 7. Treatment References . . . . .

Acute Infectious Disease. By W. PELEMANS and G. VANTRAPPEN 1. Suppurative Esophagitis ....... . 2. Esophagitis Secondary to Infectious Disease . . . . References . . . . . . . . . . . . . . . . . . . . . . . .

Tuberculosis of the Esophagus. By W. PELEMANS and J. HELLEMANS 1. Incidence . . 2. Pathogenesis. . 3. Pathology ... 4. Clinical Features 5. Diagnosis . . 6. Complications 7. Prognosis 8. Treatment. . References . . . . . . .

Syphilis of the Esophagus. By W. PELEMANS and G. VANTRAPPEN 1. Incidence . . . 2. Pathology . . . . . . 3. Clinical Features . . . 4. Technical Examination 5. Diagnosis . . 6. Complications 7. Treatment References . . . . . . .

Esophageal Mycoses. By W. PELEMANS and G. VANTRAPPEN. With 2 Figures 1. 1.1. 1.2. 1.3. 1.4. 1.5. 1.6. 1.7. 1.8. 1.9. 1.10. 2. 2.1. 2.2. 2.3. 2.4. References

Monilial Esophagitis History ... Incidence .. Etiology ... Pathogenesis. Clinical Features Technical Examination Diagnosis and Differential Diagnosis Complications . . . . . . . . . . Prognosis ........... . Treatment ........... . Other Mycotic Infections of the Esophagus Actinomycosis Mucormycosis Histoplasmosis Blastomycosis

Granulomatous Esophagitis. By W. PELEMANS and G. V ANTRAPPEN 1. Crohn's Disease of the Esophagus. 2. Sarcoidosis of the Esophagus . References . . . . . . . . . . . . .

Chapter 6

Esophageal Webs and Rings. By J. A. RINALDO. With 5 Figures 1. Introduction . . . . . 2. Upper Esophageal Web ............ .

541 541 541 541 541 542 546 549

551 551 551 552

553 553 553 553 553 554 554 554 554 555

556 556 556 556 556 557 557 557 557

558 558 558 558 559 559 560 561 563 563 564 564 564 565 565 565 565 566

568 568 569 569

571 571 572

Definition . . . . . . . Incidence ...... . Etiology and Pathogenesis Pathology .... Clinical Features . Technical Features Diagnosis .... Differential Diagnosis Complications and Prognosis Treatment ..... . Middle Esophageal Web Incidence .. . Etiology .... . Clinical Features . Technical Features Diagnosis ....

Contents

2.1. 2.2. 2.3. 2.4. 2.5. 2.6. 2.7. 2.8. 2.9. 2.10. 3. 3.1. 3.2. 3.3. 3.4. 3.5. 3.6. 4. 4.1. 4.2. 4.3. 4.4. 5. 5.1. 5.2. 5.3. 5.4. 5.5. 5.6. 5.7. 5.8. 5.9. 5.10. 5.11.

Prognosis and Treatment Lower Esophageal Web . Incidence and Etiology . Clinical and Technical Features Diagnosis ..... . Comment ..... . Squamocolumnar Ring Definition . . . . . . Incidence ..... . Etiology and Pathogenesis Pathology .... Clinical Features . Technical Features Diagnosis .... Differential Diagnosis Complications and Prognosis Treatment Comment

References . . . . .

Chapter 7

Esophageal Diverticula. By G. VANTRAPPEN and W. DELOoF. With 12 Figures

1. Lateral Pharyngeal Diverticula and Pouches . . . . . . . 2. The Hypopharyngeal Diverticulum (Zenker's Diverticulum) 2.1. Incidence . . . . . . . . . . . 2.2. Pathophysiology and Pathogenesis 2.3. Pathology . . . . . . . . . . . 2.4. Symptoms. . . . . . . . . . . 2.5. Diagnosis . . . . . . . . . . . 2.6. Complications of Untreated Diverticula 2.7. Evolution and Prognosis 2.8. Treatment. . . . . . . 3. Esophageal Diverticula . 3.1. Midesophageal Diverticula 3.1.1. Pathogenesis and Pathology 3.1.2. Symptoms.. 3.1.3. Complications.... 3.1.4. Diagnosis...... 3.1.5. Treatment...... 3.2. Epiphrenic Diverticula 3.2.1. Incidence 3.2.2. Pathology . 3.2.3. Etiology.. 3.2.4. Symptoms . 3.2.5. Diagnosis. 3.2.6. Complications

XXI

572 573 573 574 574 575 576 576 577 577 577 577 578 578 579 579 579 579 579 580 581 581 581 581 581 583 584 584 584 586 587 587 587 587 588

591

591 593 593 594 596 596 597 598 601 601 603 605 605 605 606 606 606 606 606 607 607 608 608 609

XXII Contents

3.2.7. Treatment...... 3.3. Subphrenic Diverticula References . . . . . . . . . . .

Chapter 8

Congenital Anomalies of the Esophagus

Esophageal Atresia. By G. FRANSEN and A. LACQUET. With 5 Figures 1. History ...... . 2. Incidence . . . . . . 3. Embryology . . . . . 4. Pathological Anatomy 5. Pathophysiology 5.1. Hydramnios .... 5.2. Prematurity . . . . 5.3. Pneumonia 6. Associated Anomalies 7. Diagnostic Procedures . 7.1. Nasogastric Intubation 7.2. Radiological Examinations 8. Treatment ...... . 8.1. Principles of Management . 8.2. Preoperative Management . 8.3. Operative Management .. 9. Anastomotic Complications 9.1. Leaks ......... . 9.2. Strictures . . . . . . . . 9.3. Recurrent Trachoesophageal Fistula 9.4. Neurogenic Dysfunction 10. Trends in Survival . . . . . . . . References . . . . . . . . . . . . . . . . .

Isolated Trachoesophageal Fistula. By A. LACQUET and G. FRANSEN References . . . . . . .

Bronchoesophageal Fistula. By G. FRANSEN and A. LACQUET References . . . . . . . . . . . . . . . . . . . . . . .

Cleft Larynx. Laryngotracheoesophageal Cleft. Persistent Esophagotrachea. By A. LACQUET

610 610 611

615 615 615 616 617 619 619 619 620 620 622 622 623 625 625 626 627 629 629 630 631 631 633 634

639 641

643 644

and G. FRANSEN . . . . . . . . . . . . . . . . . . . . . . . 646 References . . . . . . . . . . . . . . . . . . . . . . . . . .

Vascular Rings. By G. FRANSEN and W. PELEMANS. With 7 Figures Vascular Anomalies Causing Tracheoesophageal Symptoms. 1. Introduction . . . . . 2. Clinical Picture. . . . . . . 2.1. Respiratory Symptoms . . . . . . . . . . 2.2. Feeding Problems ........... . 3. Different Types of Anomalies . . . . . . . 3.1. Aberrant Right Subclavian Artery (A. lusoria) 3.2. Double Aortic Arch. . . . . . . . . . . . 3.3. Right.sided Aortic Arch . . . . . . . . . . . . . . . . 3.4. Left·sided Aortic Arch with Right·sided Descending Aorta . 3.5. Cervical Aortic Arch . . . . . 3.6. Aberrant Left Pulmonary Artery References . . . . . . . . . . . . .

Chapter 9

Mechanical Lesions of the Esophagus

Traumatic Lesions of the Esophagus. By J. JANSSENS and P. VALEMBOIS 1. Pathogenesis . . . 1.1. Blunt Trauma . . . . . . . . . . . . . . . . . . . . .

648

649 649 649 649 649 650 650 650 653 655 657 657 658 658

661 661 661

1.2. 1.3. 2. 3. 4.

Contents

Penetrating Wounds ............ . Mechanical Agents Acting from Inside the Lumen Symptoms. Diagnosis Treatment

XXIII

662 662 662 663 663

References . . . . . 663

Foreign Bodies in the Esophagus. By J. JANSSENS and P. VALEMBOIS 665

1. Incidence . . . . . . . . 665 2. Pathogenesis and Pathology 665 3. Clinical Features 667 4. Diagnosis . . . . . . . . 669 5. Treatment . . . . . . . . 670 6. Complications . . . . . . 672 7. Esophageal Obstruction from Meat ImpaGtion 672 References . . . . . . . . . . . . . . . . . . . . . 673

Spontaneous Rupture of the Esophagus. (Boerhaave's Syndrome.) By J. JANSSENS and P. VALEMBOIS. With 1 Figure 675

1. Definition . . . . . . 675 2. History and Incidence 675 3. Pathogenesis 675 4. Pathology . . . . . . 676 5. Clinical Features . . . 677 6. Technical Examinations 678 7. Diagnosis . . . . . . 679 8. Prognosis . . . . . . 679 9. Treatment. . . . . 680 10. Esophageal Rupture in Children 680 References . . . . . . . . . . . . . . . 681

Iatrogenic Perforations of the Esophagus. By J. JANSSENS and G. VANTRAPPEN 683

1. Definition . . . . . . . 683 2. Incidence . . . . . . . 683 3. Etiology and Pathogenesis 684 4. Symptoms and Diagnosis 685 5. Treatment 686 References . . . . . . . . . . . . 687

Mallory-Weiss Syndrome. By J. JANSSENS and P. VALEMBOIS 689

1. Definition 689 2. History . . . . 689 3. Incidence . . . 689 4. Pathogenesis 690 5. Clinical Features 691 6. Diagnosis 691 7. Pathology 692 8. Treatment 692 References . . . . . 692

Intramural Rupture and Bleeding. J. JANSSENS and G. VANTRAPPEN 694

1. Etiology. . . . . . 694 2. Pathogenesis . . . . 694 3. Symptoms. . . . . 694 4. Radiological Features and Diagnosis 695 5. Treatment 695 References . . . . . . . . . . . . . . . . . 696

XXIV Contents

Chapter 10

Esophageal Varices. By J. FEVERY and J. DE GROOTE. With 8 Figures 697 1. 2. 3. 4. 4.1. 4.1.1. 4.1.1.1. 4.1.1.1.1. 4.1.1.1.2. 4.1.1.1.3. 4.1.1.2. 4.1.1.2.1.

4.1.1.2.2. 4.1.1.2.3. 4.1.1.2.4. 4.1.1.2.5. 4.1.1.2.6. 4.1.1.2.7. 4.1.1.2.8. 4.1.2. 4.1.2.1. 4.1.2.2. 4.1.2.3.

4.1.2.4. 4.1.3. 4.1.3.1. 4.1.3.1.1. 4.1.3.1.2. 4.1.3.1.3. 4.1.3.1.4. 4.1.3.1.5. 4.1.3.2. 4.1.3.2.1. 4.1.3.2.2. 4.1.3.2.3. 4.2. 4.2.1. 4.2.2. 4.2.3. 4.2.4. 4.3. 5. 5.1. 5.2. 5.3. 6. 6.1. 6.1.1. 6.1.2. 6.1.3. 6.1.4. 6.1.5. 6.1.6. 6.2. 7. 7.1. 7.1.1. 7.1.2. 7.1.3. 7.1.3.1.

Definition . . . . . . . . . 697 Anatomy and Histopathology 697 Incidence . . . . . . . . . 699 Etiology and Pathogenesis. . 699 Esophageal Varices Secondary to "Portal Hypertension" 699 Presinusoidal Causes 699 Prehepatic Pathology . . . . . . . . 699 Forward Flow Group . . . . . . . . 699 Portal Vein Thrombosis or Compression 701 Splenic Vein Thrombosis .. . . . . 702 Presinusoidal Intrahepatic Disorders 702 Hepatic Schistosomiasis (Schistosoma Mansoni, Hematobium, Japonicum) (Mendes, 1965) . . . . . . . . . . . . . . . . . . 702 Myeloproliferative Disease, Myelofibrosis ...... 703 Granulomatous Diseases. . . . . . . . . . . . . . 703 Congenital Hepatic Fibrosis (Fibroangioadenomatosis) . 703 Polycystic Liver Disease 703 Liver Metastases 703 Wilson's Disease . 704 Acute Hepatitis 704 Sinusoidal Causes . 704 Acute Hepatitis 704 Fatty Liver . . . . . ........ 704 Early Stage of Chronic, Non-Suppurative, Destructive Cholangitis (Primary Biliary Cirrhosis) . . . . . . . . . . . . . . . . . 704 Kupffer Cell Hypertrophy with Perisinusoidal Fibrosis 704 Postsinusoidal Portal Hypertension . . . . . . . . 705 Postsinusoidal Intrahepatic Causes . . . . . . . . 705 Liver Cirrhosis . . . . . . . . . . . . . . . . . 705 Liver Cirrhosis Associated with Metabolic Disorders . 706 Alcoholic Hepatitis . . . . . . . . . . . . . 708 Partial Nodular Transformation . . . . . . . 708 Veno-Occlusive Disease . . . . . . . . . . . 708 Extrahepatic Postsinusoidal Portal Hypertension 708 Budd-Chiari Syndrome . . . . . . . . . . . 708 Congestive Heart Failure . . . . . . . . . . 709 Obstruction of Inferior Vena Cava . . . . . . 709 Esophageal Varices without Portal Hypertension . 709 Obstruction of Vena Cava Superior (Downhill Varices) 709 Carcinoma at the Gastroesophageal Junction. . . . . 709 Idiopathic Esophageal Varices . . . . . . . . . .. ........ 709 Esophageal Varices in Patients with Liver Disease, but Normal Portal Pressure 710 Unclassified Miscellaneous Causes. 710 Clinical Features: Bleeding 711 Incidence . . . . . . . . . 711 Pathogenesis of the Bleeding 711 Clinical Symptoms . . . . . 712 Diagnosis . . . . . . . . . 712 Diagnosis of Esophageal Varices 712 Barium Swallow . 712 Esophagoscopy. . . . . . . . 714 Splenoportography . . . . . . 715 Umbilical Vein Portography . . . . . . . 717 Ammonia Tolerance; Indirect Test for Esophageal Varices 718 Evaluation of the Different Techniques Used. . 719 Diagnosis of Bleeding from Esophageal Varices. 719 Therapy. . . . . . . . . . . . . . . . . . 720 Medical Management of Bleeding Varices 720 Blood Transfusion . . . . . . . . . . . .. . 720 Prevention of Hepatic Encephalopathy (in Cirrhotics) 720 Arrest of Further Bleeding . 720 Drugs. . . . . . . . . . . . . . . . . . . . . 720

Contents

7.1.3.2. Gastric Cooling. . . . . . . . . . . 7.1.3.3. Balloon Tamponade ....... . 7.1.3.4. Intraarterial Infusion . . . . . . . . 7.2. Surgical Management of Bleeding Esophageal Varices 7.2.1. Emergency Surgical Treatment. . . . . . . . . . . 7.2.2. Elective Surgical Treatment in Liver Cirrhosis with Bleeding Varices 7.2.3. Prophylactic Shunt Operations in Liver Cirrhosis 7.2.4. Prophylactic Operations in Extrahepatic Block . 7.3. Thoracic Duct Drainage . . . . . . . . . 7.4. Transumbilical Portal Decompression . . . 7.5. Sclerosing Injections for Esophageal Varices References . . . . . . . . . . . . . . . . . .

Chapter 11

Hiatus Hernia. By E. HAFTER. With 28 Figures 1. Definition and Classification . . . . . 2. Incidence . . . . . . . . . . . . . 3. Anatomical Basis, Etiology and Pathogenesis 4. Pathological Anatomy 5. Clinical Features . . . . 5.1. Symptoms ...... . 5.2. Hiatus Hernia in Infants 5.3. Hiatus Hernia during Pregnancy 5.4. Postoperative Hernias. . 5.5. Traumatic Hiatus Hernias 5.6. Signs ........ . 6. Technical Features . . . 6.1. Radiological Examination 6.1.1. Technique of X-ray Examination 6.1.2. Radiological Demonstration of Reflux. 6.1.3. Interpretation of X-ray Findings 6.1.4. Paraesophageal Hernia 6.1.5. Mixed Hernias ... 6.1.6. Invagination.... 6.1.7. Note on Terminology 6.2. Endoscopy 6.2.1. Esophagoscopy . 6.2.2. Gastroscopy . . 6.2.3. Peritoneoscopy . 6.3. Manometry 6.4. pH Measurements 6.5. Acid Infusion Test 7. Diagnosis . . . . 8. Complications . . 9. Associated Diseases 10. Evolution and Prognosis 11. Therapy. . . . . 11.1. Medical Treatment 11.2. Surgical Treatment References . . . . . . . . .

Chapter 12

xxv

721 721 721 722 722 727 727 727 728 728 728 728

741 741 742 743 746 747 747 749 749 750 750 750 751 751 751 754 757 763 764 765 765 765 766 768 768 768 769 769 769 771 773 774 775 775 776 779

Resection and Reconstruction of the Esophagus. By P. VALEMBOIS and G. VANTRAPPEN. With 5 Figures . . . . . . . . . . . . 783 1. Resection of the Esophagus . . . 783 1.1. Postoperative Complications . . . 784 2. Replacement of the Esophagus . . 784 2.1. Mortality and Morbidity of the Esophageal Substitution. 788 2.1.1. Necrosis of the Esophageal Substitute. 789 2.1.2. Leakage at the Anastomosis 790 2.1.3. Other Complications 791 2.2. Functional Results 791 References . . . . . . . . . . 792

XXVI Contents

Chapter 13

Etiology and Non-Surgical Treatment of Organic Esophageal Stenosis. By G. V ANTRAP· PEN, J. HELLEMANS and K. GEBOES. With 7 Figures 795 1. Etiology. . . . . . . . . 795 2. Techniques of Dilatation 797 3. Indications for Dilatations . 803 3.1. Benign Strictures. 803 3.2. Malignant Stenoses 804 References . . . . . . . . . 805

Chapter 14

Acquired Esophageal Fistula. By K. GEBOES and P. VALEMBOIS. With 3 Figures 807

1. Esophagorespiratory Fistula . . . . . . . . 807 1.1. Etiology. . . . . . . . . . . . . . . . . 807 1.1.1. Malignant Esophageal Fistula . . . . . . . 807 1.1.2. Esophagorespiratory Fistula of Benign Origin 807 1.1.2.1. Traumatic Fistula . . . . . . . . . . . . 807 1.1.2.2. Fistula Secondary to Esophageal Diverticulum 809 1.1.2.3. Infectious Fistula. . . . . . . . . . . . '. 809 1.1.2.4. Esophageal Fistula and Bronchopulmonary Sequestration 810 1.2. Location of the Fistula 811 1.3. Symptoms. . . . . . 811 1.4. Diagnosis . . . . . . 812 1.5. Treatment. . . . . . 813 2. Aortoesophageal Fistula 814 2.1. Benign Aortoesophageal Fistula 814 2.2. Malignant Aortoesophageal Fistula 814 2.3. Other Fistulas between the Esophagus and the Cardiovascular System 815 2.3.1. Fistula between the Esophagus and the Pericardial Cavity . 815 2.3.2. Kommerell's Diverticulum . . . . . . . . . 815 2.3.3. Esophagocardiac Fistula. . . . . . . . . . 816 2.3.4. Fistula between Larger Veins and Esophagus 816 3. Esophagocavitary Fistulas . 816 3.1. Etiology and Pathogenesis. 816 3.2. Localization 817 3.3. Symptoms. . . . . . . . 817 3.4. Diagnosis . . . . . . . . 818 3.5. Treatment . . . . . . . . 818 4. External Esophageal Fistula 818 References . . . . . . . . . . . . 819

Chapter 15

The Esophagus in Cutaneous Diseases. By K. GEBOES and J. JANSSENS. With 2 Figures 823 1. Bullous Dermatoses. . . . . . . . 823 1.1. Pemphigus. . . . . . . . . . . . . . . . . . 823 1.2. Bullous Pemphigoid (Parapemphigus) . . . . . . . 823 1.3. Benign Mucosal Pemphigoid (Cicatricial Pemphigoid) 824 1.4. Epidermolysis Bullosa Dystrophica . . . . . . . . 824 1.5. Toxic Epidermal Necrolysis (Lyell's Disease, Ritter's Disease) 826 1.6. The Stevens-Johnson Syndrome . . . . . . . . . . . . . 827 1.7. Recurrent Aphtae ......................... 827 1.8. Esophagitis Superficial is Dissecans. Benign or Idiopathic Esophageal Casts 827 2. Disorders of Keratinization . . . . . 828 2.1. Keratosis Follicularis, Darier's Disease 828 2.2. Keratoderma. . . . . . . . 829 2.3. Acanthosis Nigricans . . . . . . . . 829 3. Hypertrophic Osteoarthropathy 830 4. Dermatomyositis . . . . . . . . . . 830 5. Leukoplakia and Other Yellow-White Spots on the Esophageal Mucosa 830 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 831

Contents XXVII

Chapter 16

Miscellaneons and Rare Diseases. By K. GEBOES and W. PELEMANS. With 2 Figures 835 1. Idiopathic Retroperitoneal Fibrosis . 835 2. Amyloidosis . . . . . . . . . . . 835 3. Lupus Erythematosus Disseminatus 835 4. Intramural Diverticulosis 836 5. Pneumatosis Cystoides . . . . . . 837 6. Eosinophilic Granuloma . . . . . . 838 7. Extensive Necrosis of the Esophagus 838 8. Esophagogastric Invagination. Transmigration of the Esophageal Mucosa 838 9. Cervical Osteophytes. External Compression 838 10. Ulcerative Colitis 839 11. Pancreatitis . 839 12. Amebic Abcess 839 13. Worms 839 13.1. Hydatid Cyst 839 13.2. Spirocerca Lupus 839 14. Ectopic Tissues 839 14.1. Primary Melanosis of the Esophagus 839 14.2. Sebaceous Glands 840 14.3. Liver Tissue . . . . . . . 840 14.4. Pancreatic Tissue. . . . . 840 14.5. Heterotopic Gastric Mucosa 840 14.6. Tracheobronchial Remnants 840 14.7. Aberrant Origin of Right Main Bronchus 841 14.8. Thyroid Tissue 841 References . . 841

Subject Index 845