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Lymphedema
Byung-Boong Lee John Bergan Stanley G. Rockson
Editors
A Concise Compendium of Theory and Practice
Editors
Byung-Boong Lee, MD Department of Surgery Division of Vascular Surgery George Washington University School of Medicine Washington, DC USA
John Bergan, MD Vein Institute of La Jolla University of California La Jolla, CA USA
Stanley G. Rockson, MD Division of Cardiovascular Medicine Stanford University School of Medicine Falk Cardiovascular Research Center Stanford, CA USA
ISBN 978-0-85729-566-8 e-ISBN 978-0-85729-567-5 DOI 10.1007/978-0-85729-567-5 Springer London Dordrecht Heidelberg New York
British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library
Library of Congress Control Number: 2011928401
© Springer-Verlag London Limited 2011 Whilst we have made considerable efforts to contact all holders of copyright material contained in this book, we may have failed to locate some of them. Should holders wish to contact the Publisher, we will be happy to come to some arrangement with them. Apart from any fair dealing for the purposes of research or private study, or criticism or review, as permitted under the Copyright, Designs and Patents Act 1988, this publication may only be repro­duced, stored or transmitted, in any form or by any means, with the prior permission in writing of the publishers, or in the case of reprographic reproduction in accordance with the terms of licenses issued by the Copyright Licensing Agency. Enquiries concerning reproduction outside those terms should be sent to the publishers. 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 laws and regulations and there­fore free for general use. Product liability: The publisher can give no guarantee for information about drug dosage and applica­tion thereof contained in this book. In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature.
Cover design: SPI Publisher Services
Printed on acid-free paper
Springer is part of Springer Science+Business Media (www.springer.com)

Foreword

The entire field of vascular diseases has experienced phenomenal progress during the past few decades. Multiple books and journal publications are silent witnesses to the dramatic advances in the diagnosis and management of arterial and venous diseases. Endovascular prosthesis, bare and covered stents, and complex endolumi­nal techniques dealing with the devastating sequelae of atherosclerosis have dra­matically changed our approach to abdominal and thoracic aneurysms, obstructive coronary and carotid lesions, as well as arterial occlusion of the extremities. The same technological revolution has reached the field of venous diseases. The tradi­tional “long and short” saphenous stripping for varicose veins that many of us per­formed during decades of vascular surgery practice, have been replaced by foam sclerotherapy, radiofrequency, and laser catheters. Dermatologists, interventional radiologists, cardiologists, and other non-surgical specialists have entered the endovenous therapy field en masse. Prestigious societies devoted to the study and care of patients with venous diseases have sprouted worldwide for the benefit of the millions of patients suffering from diseases of the veins. The majority of papers published in vascular journals deal with arterial topics, a few on venous diseases, and even fewer on lymphatic disorders.
The recently published third edition of The Handbook of Venous Disorders of the American Venous Forum dedicates only 42 of its 744 pages to lymphatic diseases. The prestigious Journal of Vascular Surgery publishes annually a relatively small number of papers on the lymphatic system.
If venous diseases are the Cinderella of the vascular field, as the late Michael Hume, former President of the American Venous Forum called them, then lym­phatic malformations are Cinderella’s poor cousin.
There has been, however, a revival of Cinderella’s cousin and this book is a wel­come update of the significant progress observed in the lymphatic field during the past 20 years. The monumental work of early pioneers in the field of lymphatic circulation, such as Servelle, Kinmonth, Casley-Smith, Olszewski and Nielubowicz, Földi, and many others, has established the basis and served as a stepping-stone for many of the subjects covered by recognized specialists in this book. One of the most important obstacles in the study of the lymphatics has been its visualization. On the trail of visual lymphography using intradermal injections of Patent Blue (alphazurine)
v
vi Foreword
to visualize cutaneous and deeper lymphatics, followed the technique of oil lym­phography and lymphadenography, as described by Kinmonth. These techniques were a useful tool in the study of the lymphatic vasculature and served as a basis for a working classification of lymphedemas. The tedious and time-consuming lym­phography has been replaced by new nuclear medicine imaging techniques, radio­nuclide lymphoscintigraphy, multislice CT, magnetic resonance imaging, and computerized axial tomography. These techniques have contributed to guiding the clinician in the process of establishing a rational diagnosis and dictating appropriate treatment. Advances in diagnosis have been followed by an array of therapeutic techniques. Many of them are described in this book by their original authors. Because of my long-time interest in the subject, I have had personal experience in some of the diagnostic and surgical techniques described in this book, such as oil lymphography, lymphoscintigraphy, lymphovenous anastomosis, and debulking procedures for massive lymphedema. I consider, however, that at present, a program of complex manual decongestive technique associated with properly applied inter­mittent pneumatic compression and followed by well-supervised compression ther­apy in a compliant patient may achieve long-lasting edema control without the need for surgery. The role of surgery, as described in surgical books and texts on vascular surgery, has been relegated to a few cases of severe fibro-lymphedema and to recon­structive plastic surgery to excise and remodel the redundant skin folds resulting from a good lymphatic manual decompression program of the extremities.
The field of genetics has found fertile ground in the lymphatic vasculature. I am certain that as genetic research on the phenotypes of different lymphedema condi­tions advances, our thoughts and concepts on the nature and classification of some primary lymphedemas will change. The same will occur in those cases of lym­phedemas associated with other vascular and nonvascular anomalies. We are on the threshold of a true revolution in our understanding of the Kinmonth lymphedemas for the benefit of our own understanding of the disease, and hopefully for the benefit of many unfortunate patients suffering from the disabling pathophysiology of the lymphatic circulation.
Bethesda, USA J. Lionel Villavicencio

Preface I

Management of chronic swelling of the limbs is a continuing challenge to patients and clinicians. In recent years, there has been progress in diagnoses and treatment of this disabling condition. The goal of this present volume is to collect between two covers the best guidelines for treatment, followed by detailed descriptions of the most effective tests and modern treatments for the disabling condition best called chronic lymphedema.
Chronic lymphedema is a common condition that can be easily recognized and effectively treated according to the guidelines collected in this volume. Several national and international societies are primarily concerned with this condition, and they have periodic meetings that make exchange of useful information possible. Clinical experience has promoted the development of guidelines and statements of principle, which guide diagnosis and treatment of this chronic condition.
Because of the nature of the abnormalities that cause lymphedema, there is no established cure. There are, however, many methods that, when used in their aggre­gate, can correct and ameliorate limb swelling. These methods provide guidelines and prescription for effective treatment. The stated objectives of this volume are to describe the guidelines for thorough diagnosis and effective treatment of patients with disabling lymphedema.
Each of the presenters who write in this volume comes from a different back­ground and draws on different wells of knowledge about lymphedema. Their aggre­gate experience is enormous; it has accumulated over many years through the mechanisms of international, local, and regional scientific meetings. This book states clearly the thoughts and varied experiences of friends and colleagues, all of whom have established canonical treatment principles for a variety of manifesta­tions of this deadly condition.
The authors have hoped to assemble discussions of the many types of lym­phedema. In each discussion displayed in the chapter below, it is hoped that new ideas will develop, which, in the aggregate, will improve accuracy of diagnosis and effectiveness of medical and surgical care.
John Bergan, MD
vii

Preface II

It is truly fortunate that, as we enter the twenty-first century, the fields of lymphatic biology and medicine are experiencing a highly anticipated renaissance. This much­needed emphasis upon the study of the lymphatic system is predicted to have a transformative impact upon our understanding of physiology, health, and disease.
Inexplicably, the lymphatic system has been the subject of passive neglect for centuries of medical development. This is, indeed, paradoxical, considering that such a very important component of the human circulation plays an equally impor­tant role in the normal functioning of the immune apparatus.
Awareness of the importance of lymphatic mechanisms to the continuum of human biology and disease is growing. This “lymphatic continuum” now easily encompasses cardiovascular disease, obesity, autoimmune disease, respiratory and other forms of chronic inflammation, and chronic transplant rejection, among many other expressions of human pathology.
Lymphedema is a central manifestation of both peripheral and visceral diseases of the lymphatic circulation. Any pathological condition of the lymphatic vascula­ture, whether superficial or internal, regional, or systemic, is predominated by the appearance of the characteristic type of tissue edema that occurs when lymphatic dysfunction supervenes. While there is a broad spectrum of lymphatic vascular dis­eases, the most common diagnosis in lymphatic medicine is, of course, lymphedema. This patient population is large and, historically, under-served by the medical community.
At last, after decades and centuries of relative neglect, these patients are increas­ingly receiving attention. It is very timely, and gratifying, that there is now a clinical need for a comprehensive textbook that addresses the problem of lymphedema, and it is equally gratifying to acknowledge that this compendium has called upon the expertise of so many authorities to contribute their collective wisdom.
I am especially honored to collaborate with such an inspiring group of colleagues and, in particular, to have had the privilege to work so closely with my esteemed co-editors, Drs. John Bergan and Byung-Boong Lee.
It is an honor to dedicate this volume to the current and future well-being of our patients with lymphedema.
Stanley G. Rockson, MD
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