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- •Lymphedema
- •Foreword
- •Preface I
- •Preface II
- •Contents
- •Contributors
- •Clinical Presentation
- •Lymphedema Staging
- •Diagnosis
- •Therapy
- •Physical and Non-Operative Therapy
- •Operative Therapy
- •Introductory Note
- •Primary Lymphedema
- •Secondary Lymphedema
- •Complications of Lymphedema
- •Conclusions
- •References
- •Embryological Development of the Lymphatic System
- •Lymphedema
- •Lymphangioma
- •Protein-Losing Enteropathy and Intestinal Lymphangiectasia
- •Complex Vascular Malformations
- •Infectious Diseases
- •Lipedema
- •Lymphangioleiomyomatosis
- •References
- •Introduction
- •Molecular Lymphology
- •Work-up
- •Syndromes
- •Chromosomal Aneuploidies and Sporadic Syndromes
- •Conclusion
- •References
- •References
- •Anatomical
- •Functional
- •Lymph Flow Pathways
- •Skin and Subcutaneous Tissue
- •Gut Lymphatics
- •Lung Lymphatics
- •References
- •References
- •References
- •Tissue Fluid
- •Lymph
- •Physiological Observations
- •Proteins in Obstructive Lymphedema
- •Lymph Cytokines in Obstructive Lymphedema
- •References
- •Tissue Fluid Pressure and Flow
- •Pressures in the Normal Limb
- •Pressures in the Lymphedema
- •Normal Tissue Fluid Flow
- •Tissue Fluid Flow in Lymphedema
- •Lymph Pressure and Flow
- •Extrinsic Factors that Propel Lymph
- •Normal Conditions
- •Lymphedema Conditions
- •Intrinsic Factors that Propel Lymph
- •Pressures in Lymphedematous Limbs
- •Lymph Flow in Normal Limbs
- •Lymph Flow in Lymphedematous Limbs
- •General Remarks
- •References
- •Immune processes in lymphatics and nodes
- •Remarks
- •References
- •General Considerations
- •Clinical Diagnosis
- •Associated Disorders
- •When Further Investigation Is Needed
- •References
- •References
- •Conclusion
- •References
- •References
- •Consensus Documents
- •Consensus Documents in the Treatment of Lymphedema
- •International Society of Lymphology
- •International Lymphedema Framework
- •Italian
- •Latin American
- •Australian
- •American Cancer Society
- •National Lymphedema Network
- •Summary
- •Concluding Thought
- •Disclosure
- •References
- •Signs to Look for at Presentation
- •References
- •Introduction
- •Clinical Diagnosis
- •Differential Diagnosis
- •Introduction
- •Differential Diagnosis: Other Reasons for a Swollen Limb
- •Differentiating the Lymphedemas
- •Filarial Lymphedema
- •Malignant Lymphedema
- •Factitious Lymphedema
- •Primary Lymphedema
- •When a Patient Might First Present
- •Risk Factors to Consider at Presentation
- •Laboratory Diagnosis
- •Waist-to-Height Ratio
- •Streeten Test
- •Capillary Fragility Assessment
- •Assessment of Aortic Distensibility and Stiffness in Lipedema
- •Pain Perception Assessment
- •Ultrasound Examination
- •CT and MRI Examination
- •Lymphoscintigraphy and Fluorescent Microlymphography
- •Clinical Management
- •Prognosis
- •References
- •General Considerations
- •When Clinical Examination Should Be Complemented by Imaging
- •Methods to Evaluate Lymph Flow, Lymphatic Vessels, and Lymph Nodes
- •Methods of Evaluating Tissue Changes
- •References
- •Brief Historical Note
- •Materials and Methods
- •Interpretation and Comments
- •Primary Lymphedema
- •Secondary Lymphedema
- •Lymphatic Filariasis
- •Kaposi Sarcoma
- •Klippel–Trenaunay and Other Lymphangiodysplastic/Mixed Syndromes
- •The Future
- •Conclusions
- •References
- •References
- •Introduction
- •Lymphoscintigraphy and/or SPECT-CT Lymphoscintigraphy
- •Lymphoscintigraphy or SPECT-CT Lymphoscintigraphy in Relation to the Clinical Presentation of the “Simple” Lymphedematous Situations
- •In Primary Lower Limb Lymphedemas
- •In Secondary Lymphedemas
- •Lymphoscintigraphy to Demonstrate the Collateralization Pathways
- •Lymphoscintigraphy, Lymphoceles, and Lymphangiomas?
- •X-Ray Computed Tomography?
- •Positron Emission Tomography or Positron Emission Tomography Combined with X-Ray Computed Tomography?
- •Magnetic Resonance Imaging and/or Lymphangio-MRI with Injection of Contrast Enhancement?
- •Magnetic Resonance Imaging in the Diagnosis of Pathologically Positive Lymph Nodes?
- •Heavily T2-Weighted Imaging or Magnetic Resonance Lymphangiography for Lymphedemas?
- •MRI or MRL in Lymphedemas?
- •MRI and Lymphangiomatosis?
- •MRI and Lymphangiomas?
- •Lymphoscintigraphy and/or MRI?
- •Conclusions
- •References
- •Visual Lymphography and Radiological Lymphography
- •Radiological Lymphography
- •Oil Contrast Lymphography
- •References
- •Microlymphography in Healthy Individuals, in Chronic Venous Disease, and in Lymphedema (Table 23.1)
- •Measurement of Microlymphatic Pressure
- •Lymphatic Vasomotion and Lymphatic Flow Motion
- •References
- •Measurement of Fibrotic Induration
- •Measurement of Fluid Content
- •Measurement of Limb Volume and Circumference
- •Measurement of Functional Status of the Lymphatic System
- •Measurement of the Structural Status of the Lymphatic System and of the Limb
- •Measurement of the Status of the Vascular System
- •Measurement of the Subjective Parameters
- •Treatment Outcomes
- •References
- •General Overview
- •Primary and Secondary Infections
- •Primary Infections
- •Secondary Infections: Dermato-Lymphangio-Adenitis
- •Chronic Dermatolymphangioadenitis
- •Acute DLA
- •Differential Diagnosis of Lymphangitis, Erysipelas and Dermato-Lymphangio-Adenitis
- •Bacteriology of Lower Limb Skin
- •Bacterial Flora of Normal Foot and Calf Skin
- •Bacterial Flora of Normal Leg Lymph
- •Bacterial Flora of Lymphedematous Leg Lymph
- •Sensitivity of Isolates to Antibiotics
- •Prophylaxis of Recurrent DLA
- •Chronic DLA
- •Treatment of Acute DLA Attacks
- •References
- •Introduction
- •Sites of Accumulation of Lymph and Tissue Fluid in Lymphedema
- •Morphological Changes in the Lymphedematous Skin and Subcutis
- •Hydraulic Conditions in the Subcutaneous Tissue
- •Pressures
- •Pressure Gradient Across Skin and Subcutaneous Tissue
- •Conditions for Creating Centripetal Tissue Fluid Flow
- •Manual Massage
- •Indications
- •Advantages and Shortcomings
- •Manual Massage Hydraulics
- •Pneumatic Massage
- •Indications
- •Advantages and Shortcomings
- •Pneumatic Compression Hydraulics
- •Remarks for Users of Compression Devices
- •References
- •Introduction
- •Complete Decongestive Physiotherapy
- •The Use of CDP
- •Long-Term Therapy Results
- •References
- •Introduction
- •Detailed Characterization of MLD According to Dr. E. Vodder
- •Stationary Circle
- •Rotary Stroke
- •Pump Stroke
- •Scoop Technique
- •Additive Manual Techniques
- •Indication and Contraindication
- •References
- •Introduction
- •Investigations
- •References
- •Graduated Compression Garments
- •Multilayered Bandage Compression
- •Intermittent Pneumatic Compression
- •Impact of Compression Therapy upon Lymphedema Outcomes
- •References
- •References
- •Conservative Therapies for Secondary Lymph Edema
- •Contemporary Treatments
- •The Groupings of Contemporary Treatments
- •Methods
- •Pharmacogenomics and Medications Targeting the Lymphatic System
- •Low-Level Scanning and Hand-Held Laser
- •Lymphatic Drainage Massage Delivered by Partners/Carers and Mechanically
- •Mild Exercise (Tai Chi)
- •Moderate Exercise (In and Out of Water)
- •Electro-Stimulation
- •Tissue Manipulation
- •Kinesio-Taping
- •Diet (Mid-Chain Triglycerides) and Abdominal Issues
- •Placebo
- •References
- •Antibiotics
- •Conclusion
- •References
- •Introduction
- •General Considerations
- •Intermittent Pneumatic Compression
- •Compression
- •Use of Elastic Bandages
- •Special Compression Material
- •Medical Compression Stockings
- •Exercise
- •Lymphedema Severity-Adapted Forms of CDP
- •Stage I Lymphedema
- •Stages II and III Lymphedema
- •References
- •Introduction
- •Lymphedema of the Arm
- •Considerations in Manual Lymph Drainage
- •General Considerations for Compression
- •Compression Therapy in the Arms
- •References
- •Introduction
- •Physical Treatment of Lymphedema of the Face and Neck
- •Manual Lymph Drainage (Leduc Method)
- •Description of the Maneuvers
- •Protocol for Manual Treatment of Lymphedema of the Face and Neck
- •Multi-Layered Bandaging Leduc Method
- •Stimulation of Muscular Activity
- •Compression Garment
- •Education in Precautions to Apply to Avoid Exacerbation of Symptoms
- •Education in Self-Treatment
- •An Example of Self-Treatment of Head and Neck Lymphedema
- •Rehabilitation to Address Functional Impairments
- •Quality of Life
- •References
- •Introduction
- •Anatomy
- •Etiology
- •Diagnosis
- •Clinical Course
- •Treatment
- •Surgical
- •References
- •References
- •Lymphovenous Microsurgical Shunts in Lower Limbs
- •Lympho-Venous Shunts (1966–2010)
- •Pre- and Post-operative Pharmacological Treatment
- •Postoperative Physiotherapy
- •Postoperative Evaluation Criteria
- •Objective Indirect Methods for the Evaluation of the Function of the Lympho-Venous Shunt
- •Direct Methods for Evaluation of Function of Lympho-Venous Shunt
- •Factors Adversely Affecting the Patency of Lymph-Venous Shunts
- •Local
- •Distant
- •Factors Affecting Evaluation of Clinical Results
- •Results in General
- •References
- •Principles
- •Indications
- •Microsurgical Reconstructions
- •Lymphovenous Anastomosis
- •Lymph Node-to-Vein Anastomosis
- •Technique
- •Results
- •Lymph Vessel-to-Vein Anastomosis
- •Microsurgical Technique
- •Results
- •Lymphatic Grafting
- •Technique
- •Results
- •Lymph Node Transplantation
- •Technique
- •Results
- •Problems with Microvascular Lymphatic Reconstructions
- •Conclusions
- •References
- •General Considerations
- •Clinical Experience and Surgical Techniques
- •Results and Final Considerations
- •References
- •Introduction
- •Correlation With the Pathophysiology of Lymphedemas
- •Experimental Basis
- •Indications for Lymphatic Reconstruction Using Lymphatic Grafts
- •Operative Technique
- •Post-operative Procedures
- •Results
- •References
- •NodoVenal Shunt
- •Indications
- •Surgical Techniques
- •End-to-End Anastomosis
- •End-to-Side Anastomosis
- •Contraindications
- •Complications
- •References
- •Introduction
- •Secondary Lymphedema
- •Lymphedema of the Arm: Upper Extremity
- •Indication for Node Grafting
- •Operative Technique
- •Results
- •Plexopathy
- •Breast Reconstruction Combined with Lymphedema Treatment
- •Lymphedema of the Leg: Lower Extremity
- •Operative Technique
- •Results
- •Primary Lymphedema
- •Indications
- •Operative Technique
- •Results
- •Conclusion
- •References
- •Clinical Experiences (Personal)
- •Conclusion
- •References
- •References
- •Introduction
- •The Morphological Changes in Advanced Lymphedema
- •Indications for Debulking
- •Bacteriology of Skin and Deep Tissues
- •Surgical Technique
- •References
- •References
- •Clinical Experience
- •Conclusion
- •References
- •Excess Subcutaneous Adiposity and Chronic Lymphedema
- •The Outcome of Liposuction
- •How to Perform Liposuction for Lymphedema
- •Surgical Technique
- •Postoperative Care
- •Controlled Compression Therapy
- •Volume Measurements
- •When to Use Liposuction to Treat Lymphedema
- •Summary
- •Key Points
- •References
- •Extratruncular Lymphatic Malformation Lesions
- •Truncular Lymphatic Malformation Lesions
- •Clinical Evaluation
- •Clinical Management
- •Conservative (Physical) Therapy
- •Surgical Therapy: Reconstructive Surgery
- •Surgical Therapy: Ablative/Excisional Surgery
- •Liposuction: Circumferential Suction-Assisted Lipectomy
- •Prospect: Primary Lymphedema as Lymphatic Malformation
- •Conclusion
- •References
- •References
- •Diagnosis
- •Management
- •General Considerations
- •References
- •Medical Therapies for Chylorrhea
- •References
- •Introduction
- •Drainage Procedures
- •Image-Guided Approaches
- •Open Surgical Approaches
- •Treatment of Cutaneous Chylorrhea and Chylorrhagia
- •Treatment of Chylothorax
- •Treatment of Chylous Ascites
- •Summary
- •References
- •References
- •Morphology
- •Life Cycle
- •Pathology
- •Gross Pathology
- •Changes Attributed to Filariae
- •Changes Ascribed to Bacterial Infections
- •Immunology
- •References
- •Manifestations


Lymphedema


Byung-Boong Lee
John Bergan
Stanley G. Rockson
Editors
Lymphedema
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
ix
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