Color Atlas and Synopsis of Vascular Disease [1st ed.] 0071749543, 9780071749534, 9780071749541

Table of contents :
Title page......Page 4
DEDICATION......Page 6
CONTENTS......Page 8
Contributors......Page 11
Preface......Page 16
Acknowledgments......Page 18
LOWER EXTREMITIES: MISCELLANEOUS DISEASES......Page 20
AORTIC AND UPPER EXTREMITYARTERIAL DISEASE......Page 52
CAROTID ARTERY OCCLUSIVE DISEASE......Page 106
ANEURYSMAL DISEASE......Page 136
NON-ATHEROSCLEROTIC DISORDERS......Page 178
ARTERIAL & VENOUS VISCERAL DISEASE......Page 200
VENOUS DISEASES......Page 244
LIMB SWELLING......Page 320
VASOSPASTIC AND VASCULITIC DISEASES......Page 360
ENVIRONMENTAL DISEASES......Page 422
LIMB ULCERATIONS......Page 440
Appendix......Page 468
A
......Page 474
B
......Page 476
C
......Page 477
D
......Page 480
E
......Page 481
F
......Page 482
H
......Page 483
K
......Page 484
L
......Page 485
M
......Page 486
P
......Page 488
R
......Page 490
S
......Page 491
T
......Page 493
V
......Page 494
W
......Page 495

Citation preview

AS CO ORA and SY 0 SIS ot

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COLOR ATLAS AND SYNOPSIS OF VASCULAR DISEASES

NOTICE Medicine is an ever-changing science. As new research and clinical experience broaden our knowledge, changes in treatment and drug therapy are required. The authors and the publisher of this work have checked with sources believed to be reliable in their efforts to provide information that is complete and generally in accord with the standards accepted at the time of publication. However, in view of the possibility of human error or changes in medical sciences, neither the authors nor the publisher nor any other party who has been involved in the preparation or pub­ lication of this work warrants that the information contained herein is in every respect accurate or complete, and they disclaim all responsibility for any errors or omissions or for the results obtained from use of the information contained in this work. Readers are encouraged to confirm the information contained herein \v:ith other sources. For example and in particular, readers are advised to check the product information sheet included in the package of each drug they plan to administer to be certain that the information contained in this work is accurate and that changes have not been made in the recommended dose or :in the contraindications for administration. This recommendation :is of particular importance in connection with new or infrequently used drugs.

COLOR ATLAS AND SYNOPSIS OF VASCULAR DISEASES E D ITO RS

Steven M. Dean, DO, FACP, RPVI, F SVM Associate

Professor of Internal Medicine

Dh•ision of Cardiova cular

Medicine

Director, Vascular Medicine Program

The Ohio

Uni

v ersity Wexncr Medical Center Columbus, Ohio

Bhagwan Satiani, MD, MBA, RPVI, FACS Professor of Clinical urgcry D i v i sio n of

Vascular Diseases & Surgery

Departmen t of urgery

Director, FAME Faculty Leadership Institute

,

D i rector Vascular

The O h io

tate University

Lab

Wexncr Medic:� I Center

SERIES EDITOR William T. Abraham, MD, FACP, FACC, FAHA, FESC Professor of Medicine, Phy siology, and Cell B iology 01air of Excellence In Cardiovascular Med icine

D irec tor , Division of Cardiovascular Medicine

Deputy Director, Davis Heart and Lung Research Institute

The Ohio tate University Columbus, Ohio

.I New York

Chi�go Milan

San Francisco

New Delhi

Medical

Athens ingapore

London Madrid ydney Toronto

Me xico

ity

Copyright©

2014 by McGraw-Hill Education. All rights reserved. Except as permitted under the United States Copyright Act of 1976, no part of this publication in any form or by any means, or stored in a database or retrieval system, without the prior written permission of the publisher.

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All trademarks are trademarks of their respective owners. Rather than put a trademark symbol after every occurrence of a tTademarked name, we use names in an editorial fashion only, and to the benefit of the trademark owner, with no intention of infTingement of the trademark. Where such designations appear in this book, they have been printed with initial caps. McGraw-Hill Education eBooks are available at special quantity discounts to use as premiums and sales promotions or for use in corporate tTaining programs. To contact a representative, please visit the Contact Us page at www.mhprofessional.com. TERMS OF USE This is a copyrighted work and McGraw-Hill Education and its licensors reserve all rights in and to the work. Use of this work is subject to these terms. Except as permitted under the Copyright Act of

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DEDICATION I would like to dedicate this book to my wonderful family. First of all, never-ending gratitude is owed to my wife, Jenny and daughter, Annie. They have been incredibly understanding and supportive of all the time I've invested in editing this book as well as my profession.

To my brother, Michael. Thanks as you've made life more fun! Finally, my career in medicine would not have been possible without the initial support of my parents, Merrell and Sherma. !love you all. Steven M. Dean

To my best friend and wife Mira. To the land of the free and home of the brave, the most blessed place on earth for immigrants. Bhagwan Satiani

This page intentionally left blank

CONTENTS Contributors . ... ...... . .. ... ... ... ... ... ... . . . .. . i:x

Prefa.ce .. ... ...... ... ... ... . .. ... . . . ... . .. ...... xv

Acknowledgm nts .

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xvii

vii

PART4 ANEURYSMAL DISEASE 29 Endovascular An urysm Repair

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11 8

30 Endovascular Abdominal Aortic Aneurysm Repair for Ruptured Abdominal Aortic Aneurysm .

LOWER EXTREMITIES: Aortoilia Disease: Oeclwion . . . . . . . . . . . . . . . . . . . . . ...2

Aortoiliac O=lusive Disease: Treatment With Stenting.... ..6 .

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31 Thoracic and Thoracoabdominal Aneurysms:

MISCELLANEOUS DISEASES

Femoral Popliteal Disease: Bypa s .

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.... . . . . .. . . .

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.

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. 122

Open Repair . . . . . . . . . . . . . . . . . . . . . . . .. .. . ... . . 125 32 Thoracic Endovascular Aneurysm Repair ... ... ... ..... 130 33 Femoropopliteal Aneury ms ...................... 133

.I I

34 Arterial Pseudoaneurysms ........................13 7

Femoral Popliteal Disease: PTA and tenting . ... . ...... . 14

35 Mycotic Aneurysmal Disease .. . .. . ... . ............ 141

Tibioperoneal Occlusive Disease ..

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Limb Gangrene and Amput..ltion .

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Extra-Anatomic Bypass Grafts ......................25

yndrome .

36 Vascular Ehlers-Danlo

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37 MarfaJl Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149

38 Loeys Dietz yndrome and Related Disorders

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Blue Toe yndrome (Atheromatous Embolization} . . . . . . . . 29

PART 5 PART 2

NON-ATHEROSCLEROTIC Dl SORDERS 39 Popliteal Artery Entrapment yndrome . . . . . . . . . . . . . . . 160

AORTIC AND UPPER EXTREMITY ARTERIAL DISEASE Atbero derotic Arch V

9

sel Disease .. . . . . . .

10 l110racic Aortic Dissection .

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... . . ...... .

40 Iliac Artery Endofibrosis ... . . . . . ... . . . . .......... 163 .

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... . . ..42

11 Innominate and ubdavian Artery Peripheral

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.3 4

Arterial Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46 ite Complications . . . . . .49

12 Upper Ex'tremity lsch mia: Acces

l11oracic Outlet ynd•·ome and Arterial Aneurysm of

1

Upper Extremity ...............................5 4 4 Arte1ial Thoracic Outlet yndrome .

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15 Vertebrobasilar Insufficiency: ubdavian teal yndrome 1 17

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ubclavian Coronary Steal . . . . .... . .... . . ... . ......67 ongenital Vascular Anomalies of the

Upper Extremity . . . . .. . . . . . . . . .. . .. . . . . . . . . . . . . 71 18 Allen Test and Evaluation of the Palmar Ardl . . . . . .. . . . . . 79 19 Hypothenar Hammer yndrome .

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20 Hand I chemia After Placement of Hemodialysis Access.

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yndrom

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PART6 ARTERIAL & VENOUS VISCERAL DISEASE 43 A therosderoti Renal Artery teno is . . .

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44 Fibromuscular Dysplasia-Associated

.. . . . . . . . . . 182

Renal Artery tenosis . . . . . . . . . . . . . . . . . . . . . . . . . . . 187

45 Acute Mesenteric Ischemia ....................... 193 46 Celiac Axis Compression yndrome . ... .

47

48 Bu dd- hiari Syndrome 49

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hronic Mesenteric Ischemia ...................... 202 .

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picnic Vein Thrombosis .. ... .................... 215

50 Mesenteric Venous Thrombosis .................... 21 9

VENOUS DISEASES Acute uperficial Venous Thrombosis:

CAROTID ARTERY OCCLUSIVE DISEASE 21 Arterio !erotic

41 Klippei-T•·enauna

42 Congenital Arteriovenous Malformations . ..... ...... . . 172

arotid Oeclusi,·e Dis ase: urgical .... . ..88

22 Arteliosclerotic Carotid Oeclusive Disease: tent ... . . . ...92 23 Arteriosclerotic Carotid Oeclusive Disease: Ulcerati,·e . . . . . 97

24 Arterios I rotic Carotid Oeclusive Dis ase: Occlusion

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25 Carotid Artery Fibromuscular Dysplasia . . . .

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26 Carotid Artery Dis

ti011 . .

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27 Carotid Artery Aneurysm . ... .................... I08

28 Carotid Artery Traumatic Injuries . . . . ... . . . . . . . .... . Ill

Evaluation and Treatment . . .

.

.

... .

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.

. . .. . . . . . . ... 2 26

Evaluation and Treatment of Calf Vein Thrombosis . . . . . . . 230

Medical Mmagement of Femoropopliteal Deep Venous Thrombosis

.

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Percutmeous Endovenous Intervention in

Femoropopliteal Deep Venous 11lrombosis

.

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....

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. . 243

May-Thurner yndrome . . ... . ... . .. . ... . ... . .... 251 Paget- cbroetter yndrome ....................... 255

Benign Acute Blue Finger ........................ 259

CONTENTS

viii

Phlegmasia Cerulea Dolens ....................... 263 Panniculitis . . . . .

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tasis Dermatitis .

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Pseudo-Kaposi Sarcoma (Acroangiodermatitis) . . .

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Heparin-, Low-Molecular Weight Heparin-, and Warfarin-Induced kin Necrosis . . ... . . .... .

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Overview of pider, Reticular, and Varicose Veins ....... 2 8

Sclerotherapy ................................. 2 93 Endovenous Laser Ablation for Varicose Veins . .

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condary Lymphedema

Primary and

67 Lower Extremity CeUulitis . .

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68 Leg welling Secondary to Muscle Rupture . .. . . .

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69 Pretibial Myxedema ............................ 316 Uped ma

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82 lhromboangutis Obliterans {Buerger Disease) . . . 83 Giant Cell and Takayasu Arteritis

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84 Behs:et yndrome . . .

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85 Nephrogenic S •sternic Fibro is. ... ... ... ... ... ... .. 398

PART10

86 Frostbite . . .

LIMB SWELLING

7

with Polyangiiti ) .............................. 372

81 Polyarteritis Nodo

E NVIRONMENTAL DISEASES

PARTS

6

80 Churg-Strauss yndrome (Eo inophilic Granulomatosis

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71 Upolymphedema .............................. 3 27 72 Phlebolymphedema

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Fa tiLial Edema . .. . . . .

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89 Erythromelalgia .. .

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76 Acrocyanosis

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75 Uvedo Reticularis and Uvedo Racemo a . ... . .

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78 Granulomatosis with Polyangiitis (Wegener) . . . .

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407

. 410 .

414

90 Erythema AB 1gne ............................. 418

PART11 LIMB ULCERATIO NS 91 Venous tasis Ulceration

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92 Arterial Ulcerations .. .. . . .. . . . . . . .

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. . 435

95 Necrobiosis Lipoidica and Diabetic Dermopathy . . . . . . . . . 440 96 Calciphylaxis . . . . . . Appendix.

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Index .

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77 ANCA- egative maUVe el Vasculitis. . .... . . . . . . . . . 35 79 Microscopic Polyangiitis

.

94 Pyoderma Gangrenosum . .

VASOSPASTIC AND VASCULITIC DISEASES 74 Raynaud Phenomenon .

.

88 Immersion Foot yndromc . . .

93 Neuropathic Ulceration .

PART9

.

87 Pernio.. . . .. . . . . . . . .. . .. . . . .... . . . . . .. . .. .

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455

CONTRIBUTORS Scott P. Albert, MD

John R. Bartholomew, MD, FACC, MSVM

Assistant Professor of Surgery

Professor of Merucine, Cleveland Clinic Lerner College of Merucine

Department of Surgery

Section Head, Vascular Merucine

Division of Hepatobibary Surgery

Robert and Suzanne Tomsich Department of Carruovascular

Upstate Merucal University

Merucine

Syracuse, NY

Cleveland Cbnic

Lana Alghothani, MD

Cleveland, OI-l

Department of Internal Merucine

Joshua A. Beckman, MD, MS

The Ohio State University Wexner Merucal Center

Carruovascular Division Brigham and Women's Hospital

Columbus, OH

Harvard Merucal School

Georgann Anetakis Poulos, MD

Boston, MA

Fellow

Peter M. Bittenbender, MD

Department of Dermatology

Department of Internal Merucine

University of Pittsburgh Medical Center

Division of Carruovascular Merucine

Pittsburgh, PA

The Ohio State University Wexner Merucal Center

A. George Akingba, MD, PhD

Columbus, OI-l

Assistant Professor

Mark Bloomston, MD

Surgery and Biomerucal Engineering Department of Surgery

Associate Professor of Surgery

Division of Vascular Surgery

Department of Surgery

lnruana University School of Medicine

Division of Surgical Oncology

lnruanapobs, IN

The Ohio State University Wexner Merucal Center

Clint Allred, MD

Colun1bus, OI-l

Department of Internal Merucine

Thomas E. Brothers, MD

The Ohio State University Wexner Merucal Center

Department of Surgery

Columbus, OH

Merucal University of South Carobna

Stacy P. Ardoin, MD, MS

Charleston, SC

Division of Rheumatology and Immunology

Nicole C. Bundy, MD, MPH

The Ohio State University Wexner Merucal Center

Division of Rheumatology

Columbus, OI-l

Department of Internal Merucine

W. David Arnold, MD

The Ohio State University Wexner Merucal Center

Assistant Professor

Columbus, OI-l

Departments of Neurology and PM&R

Teresa L. Carman, MD

Division of Neuromuscular Medicine

Department of Medicine

The Ohio State University Wexner Merucal Center

Division of Carruovascular Medicine

Columbus, OI-l

University Hospitals Case Merucal Center

Benjamin J. Aumiller, MS, MD

Assistant Professor of Merucine

Section of Vascular Surgery Inruana University School of Merucine

Case Western Reserve University School of Merucine Cleveland, OH

IU-Methorust Hospital

Marissa J. Carter, MA, PhD

Inruanapobs, IN

President

Faisal Aziz, MD, RVT, RPVI Assistant Professor

Strategic Solutions, Inc. Cody, WY

Department of Surgery

Ana Casanegra, MD

Division of Vascular Surgery

Assistant Professor

Pennsylvania State University College of Merucine

Vascular Merucine Program

Hershey, PA

Carruovascular Section

Shalene Badhan, MD Division of Rheumatology and Immunology The Ohio State University Wexner Merucal Center Columbus, OI-l

Department of Internal Merucine University of Oklal1oma Health Sciences Center Oklahoma City, OK

ix

CONTRIBUTORS

X

Stephen L. Chastain, MD, RVT

Borzoo Farhang, DO, MS

Meilicine Fellow

Clinical Instructor

Greenville Hospital System Vascular Health Alliance

University of Vermont School of Medicine

Greenville, SC

Fletcher Allen Medical Center

Shane Clark, MD

Department of Anesthesiology

Dermatology Resident The Ohio State University Wexner Meilical Center

Fletcher Allen Health Care Burlington, VT

Gahanna, O H

Caroline E. Fife, MD

Mark Crandall, MD

Meilical Director

Department of Internal Memcine Department of Cariliovascular Meilicine

St. Luke's Wound Center The Woodlands, TX

The Ohio State University Wexner Meilical Center

John H. Fish Ill, MD, FSVM, RPVI

Columbus, OH

Aurora Carillovascular Services

Kevin P. Cohoon, DO, MSc

The Vascular Center at St. Luke's Medical Center

Department of Internal Memcine Fellow, Division of Cariliovascular Disease

Milwaukee, WI

Jonathan Forquer, DO

Mayo Clinic

Department of Internal Meilicine

Rochester, M

Division of Cariliovascular Memcine

Anthony J. Comerota, MD, FACS, FACC Director, Jobst Vascular Institute

University of Cincinnati Cincinnati, OH

Toledo, OH

Miriam L. Freimer, MD

Adjunct Professor of Surgery

Associate Professor and Clinical Vice Chair

University of Michigan

Department of

Ann Arbor, Ml

The Ohio State University Wexner Meilical Center

Michael C. Dalsing, MD

eurology

Columbus, 01-1

E. Dale and Susan E. Habegger Professor of Surgery

Nitin Garg, MBSS, MPH

Director of Vascul.ar Surgery

Department of Surgery & Radiology

Division of Vascular Surgery

Division of Vascular Surgery

Department of Surgery

Meilical University of South Carolina

lniliana University School of Medicine

Ralph H. Johnson VA Meilical Center

Inilianapolis, IN

Charleston, SC

Michael Davis, MD, FSVM

Michael R. Go, MD

Assistant Professor of Internal Medicine

Assistant Professor of Surgery

Department of Internal Meilicine

Department of Surgery

Division of Cariliovascular Memcine

Division of Vascular Diseases and Surgery

The Ohio State University Wexner Meilical Center

The Ohio State University Wexner Meilical Center

Columbus, OH

Columbus, 01-1

Steven M. Dean, DO, FACP, RPVI

Christopher J. Goltz, MD

Associate Professor of Clinical Medicine

Vascular Surgery Fellow

The Ohio State University Wexner Meilical Center

Department of Surgery

Columbus, OH

Division of Vascular Surgery

Sapan S. Desai, MD, PhD Assistant Professor

lnmana University School of Medicine lnilianapolis, IN

Department of Surgery

NavYash Gupta, MD, FACS

Duke University Meilical Center

Chief, Division of Vascular Surgery

Durham,

C

Essa M. Essa, MBBS Department of Internal Memcine Division of Cariliovascular Meilicine The Ohio State University Wexner Meilical Center Columbus, 01-1

orthShore University HealthSystem Clinical Associate Professor, Department of Surgery University of Chicago Pritzker School of Meilicine Skokie, IL

CONTRIBUTORS Joseph Habib, MD

Vikram S. Kashyap, MD

Vascular Surgery Fellow

Professor of Surgery

Division of Vascular Diseases and Surgery

Case Western Reserve University School of Medicine

Department of Surgery

Chief, Division of Vascular Surgery and Endovascular Therapy

The Ohio State University Wexner Medical Center

Department of Surgery

Columbus, OH

University Hospitals Case Medical Center

Rula A. Hajj-Ali, MD

Cleveland, OH

Assistant Professor of Medicine

Tanaz A. Kermani, MD, MS

Department of Rheumatic and Immunologic Diseases

Assistant Clinical Professor

Center for Vasculitis Care and Research

Da,�d Geffen School of Medicine

Cleveland Clinic, OH

Department of Medicine

Katya L. Harfmann, MD

Division of Rheumatology

Resident Department of Internal Medicine

University of California Los Angeles, CA

Division of Dermatology

Raghu Kolluri, MD, FACP, FSVM

The Ohio State University College of Medicine

Director, Vascular Medicine and Vascular Laboratories

Columbus, 01-1

Prairie Cardiovascular Consultants

Mounir J. Haurani, MD Assistant Professor of Clinical Surgery

Springfield, IL

Andrew K. Kurklinsky, MD, RPVI, FSVM

Division of Vascular Diseases and Surgery

Assistant Professor of Medicine

Department of Surgery

Division of Cardiovascular Medicine

The Ohio State University Wexner Medical Center

Mayo Clinic

Columbus, 01-1

Jacksonville, FL

Travis Hubbuch, DPM

James Laredo, MD, PhD

Resident, Department of Orthopedic Surgery

Associate Professor

Cleveland Clinic

Department of Surgery

Cleveland, OH

Division of Vascular Surgery

Stephanie Jacks, MD Dermatology Resident

George Washington University Medical Center Washington, DC

The Ohio State University Wexner Medical Center

Byung Boong Lee, MD, PhD

Gahanna, OH

Clinical Professor

Michael R. Jaff, DO

Department of Surgery

Department of Vascular Medicine Division of Cardiovascular Medicine Massachusetts General Hospital

Division of Vascular Surgery George Washington University Medical Center Washington, DC

Boston, MA

Gary W. Lemmon, MD

Wael N. Jarjour, MD, FACP

Professor of Surgery

Associate Professor of Medicine Division of Rheumatology and Immunology The Ohio State University Wexner Medical Center Columbus, 01-1

Benjamin H. Kaffenberger, MD Chief Resident Division of Dermatology The Ohio State University Wexner Medical Center Columbus, 01-1

Department of Surgery Division of Vascular Surgery Indiana University School of Medicine Indianapolis, IN

Maria E. Litzendorf, MD Assistant Professor of Surgery Division of Vascular Diseases and Surgery The Ohio State University Wexner Medical Center Columbus, 01-1

Robert Lookstein, MD, FSIR, FAHA Associate Professor of Radiology and Surgery Chief, Division of Interventional Radiology Mount Sinai Medical Center New York, NY

xi

CONTRIBUTORS

xii

Michael Maier, DPM

Eric Mowatt-Larssen, MD

Department of Cardiovascular Medicine

Assistant Professor

Division of Vascular Medicine

Division of Vascular Surgery

Cleveland Cliruc

Duke University Medical Center

Cleveland, OH

Durham,

C

Ashima Makol, MD

Alan Nadour, MD, FSVM

Division of Rheumatology,

Prairie Cardiovascular Consultants

Mayo Clinic,

Springfield, IL

Rochester, MN

John Anthony O'Dea, MD

Julie E. Mangino, MD

Department of Medicine

Professor of Internal Medicine

Division of Vascular Medicine and Intervention

Medical Director, Department of Clinical Epidemiology

Massachusett' s General Hospital

Division of Infectious Diseases

Boston, MA

The Ohio State University Wexner Medical Center Columbus, OI-l

Angela H. Martin, MD

Luigi Pascarella, MD Department of Surgery Division of Vascular Surgery

General Surgery Resident

Duke University Medical Center

Department of General Surgery

Durham,

Indiana University School of Medicine

David H. Pfizenmaier II, MD, DPM

Indianapolis, IN

Sheryl Mascarenhas, MD

C

Consultant, Vascular Medicine Cardiovascular Diseases

Division of Rheumatology and Immunology

Mayo Clinic

The Ohio State University Wexner Medical Center

Rochester, MN

Columbus, OI-l

Jason Prosek, MD

Ali Mehdi, MD Department of Internal Medicine

Clinical Instructor /House Staff Department of Internal Medicine

Cleveland Clinic, OI-l

Division of Nephrology

Ari J. Mintz, DO

The Ohio State University Wexner Medical Center

Resident

Columbus, OI-l

Department of Internal Medicine

Daniel J. Reddy, MD

Clinical Associate in Medicine

Professor of Surgery, Wayne State University

Tufts University School of Medicine

J. D. Dingell VA Medical Center

Lahey Hospital and Medical Center

Detroit, MI

Burlington, MA

Suman Rathbun, MD

Bruce Mintz, DO, FSVM

Professor

Clinical Associate Professor

Vascular Medicine Program

Internal Medicine

Cardiovascular Section

Rutgers

Department of Internal Medicine

ew Jersey Medical School

Director Vascular Technology Training Program

University of Oklahoma Health Sciences Center

Newark ,

Oklahoma City, OK

J

Rocio Moran, MD, FACMG

Christina Rigelsky, MS, CGC

Medical Director, General Genetics Clinics

Certified Genetic Counselor

Center for Personalized Genetic Healthcare

Genomic Medicine Institute

Genomic Medicine Institute

Lerner Research Institute

Cleveland Cliruc Foundation

Cleveland Cliruc

Cleveland, OI-l

Cleveland, OI-l

Raghu Motaganahalli, MD, FRCS, FACS

Thorn W. Rooke, MD, FSVM

Assistant Professor-Program Director

Department of Vascular Medicine

Department of Surgery

Division of Cardiovascular Diseases

Section of Vascular Surgery

Mayo Clinic

Indiana University School of Medicine

Rochester, MN

Indianapolis, IN

CONTRIBUTORS Irving L. Rosenberg, MD

Sachin Sheth, MD

Department of Internal Merucine

Clinical Fellow

Division of Rheumatology and Immunology

Division of lnterventional Radiology

The Ohio State University Wexner Merucal Center

Mount Sinai Merucal Center

Columbus, OH

New York, NY

Jean M. Ruddy, MD

Lawrence A. Shirley, MD

Department of Surgery

Surgical Oncology Fellow

Merucal University of South Carolina

Department of Surgery

Charleston, SC

Division of Surgical Oncology

Satish K. Sarvepalli, MD, MPH

The Ohio State University Wexner Merucal Center

Fellow

Columbus, 01-1

Department of Internal Merucine

Cynthia K. Shortell, MD, FACS

Division of Infectious Diseases

Department of Surgery

The Ohio State University Wexner Merucal Center

Division of Vascular Surgery

Columbus, OH

Duke University Medical Center

Bhagwan Satiani, MD, MBA, RPVI, FACS

Durham, NC

Professor of Clinical Surgery

Mitchell Silver, DO, FACC, FSVM

Merucal Director, Vascular Laboratories, OSU Heart &

Director, Center for Critical Limb Care

Director Faculty Leadership Institute

Columbus, 01-1

Division of Vascular Diseases & Surgery

Marcus D. Stanbro, DO, FSVM, RPVI

Vascular Center

Department of Surgery

The Ohio State University Wexner Merucal Center Columbus, OH

Adil Sattar, MD

Riverside Methorust Hospital

Assistant Professor of Clinical Surgery Department of Surgery Division of Vascular Surgery Greenville Health System

Department of Internal Merucine

Greem�lle, SC

SIU School of Merucine

Jean Starr, MD, FACS, RPVI

Residency Oflice Springfield, IL

Robert M. Schainfeld, DO, FSVM, FSCAI

Associate Professor of Clinical Surgery Division of Vascular Diseases and Surgery Department of Surgery

Department of Vascular Merucine

The Ohio State University Wexner Merucal Center

Division of Carruovascular Merucine

Columbus, OH

Massachusetts General Hospital

Shankar M. Sundaram, MD, FACS, FCCP

Boston, MA

Han·ison Health Partners

Irina Shakhnovich, MD

Thoracic and Vascular Surgery

Department of Surgery

Bremerton, WA

Division of Vascular Surgery

Axel Thors, DO

Merucal College of Wisconsin Milwaukee, WI

Department of Surgery Division of Vascular Diseases and Surgery

Mohsen Sharifi, MD, FACC, FSCAI, FSVM

The Ohio State University Wexner Merucal Center

Director, Arizona Carruovascular Consultants & Vein Clinic

Columbus, 01-1

Adjunct Professor, A.T.Still University

Mesa, AZ

Aditya M. Sharma, MD, RPVI Assistant Professor of Merucine Carruovascular Division University of Virginia School of Merucine Charlottesville, VA

Nikos Tsekouras, MD Jobst Vascular Institute Toledo, OH

Christopher Valentine, MD Assistant Professor of Clinical Merucine Department of Internal Merucine Division of Nephrology The Ohio State University Wexner Merucal Center Columbus, 01-1

xiii

CONTRIBUTORS

xiv

Marcelo P. Villa-Forte Gomes, MD, FSVM

Kenneth J Warrington, MD

Program Director, Vascular Medicine Fellowship

Associate Professor of Medicine

Department of Cardiovascular Medicine

Department of Medicine

Division of Vascular Medicine

Division of Rheumatology

Cleveland Clinic

Mayo Clinic

Cleveland, OH

Rochester, MN

John C. Wang, MD, MSc

ldo Weinberg, MD

Assistant Professor of Surgery

Department of Vascular Medicine

Case Western Reserve University School of Medicine

Division of Cardiovascular Medicine

Department of Surgery

Massachusetts General Hospital

Division of Vascular Surgery and Endovascular Therapy

Boston, MA

University Hospitals Case Medical Center Cleveland, OH

PREFACE Vascular diseases involving the arterial, venous, and lymphatic sys­

a wide variety of vascular-related topics. The book integrates the fields

tems are becoming increasingly common commensurate with the

of vascular medicine, vascular surgery, and endovascular therapy in an

aging population. Inexplicably, the proper identification and therapy

"easy to learn" fashion. Ninety-six chapters are presented in a unique

of these myriad disease states is unfortunately not typically included

format of succinct, bulletecl teaching points combined with multiple

in the education of neither the medical student nor the resident.

singular photographs of vascular pathology. When appropriate, vivid

Thus, most practicing physicians are not prepared to accurately diag­

arteriographic and ultrasonographic images are included as well. Since the text is clinically oriented, concise, and current, we feel

nose and manage affected patients. Consequently, we have constructed the

Color Atlas and Synopsis if

Vascular Diseases to enable medical students, postgraduate physician trainees, and practicing clinicians to rapidly assimilate information on

that you will not only derive practical and useful knowledge, but also thoroughly enjoy perusing this compilation. Steven M. Dean and Bhagwan Satiani

XV

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ACKNOWLEDGMENTS I'd like to acknowledge the following people who either diTectly or

Series Editor, Dr. Bill Abraham-thanks for allowing me to complete

indiTectly assisted in bringing this book to fruition: My fonner mentors

this project and for your unyielding support!

at the Cleveland Clinic Foundation (Drs Jess Young, Jeff Olin, John

Bartholomew, and William Ruschhaupt) for their incredible teaching of the principles of Vascular Medicine; my co-editor Dr. Bhagwan Satiani-he is both a true scholar and friend; proof editor Sapna Rastogi as she somehow consistently managed to keep this project on time and accurately edited; my good mends and colleagues (Thompson, Baldwin, Vasquez, Olminator, Stanbro, and DTY) at Readership Central for their daily wisdom and unbelievable wit; the

Steven M. Dean Thanks are due to my friend and hard-working co-editor Steven M. Dean, my faculty in the Division of Vascular Diseases and Surgery at Ohio State for contributing inunensely to the atlas, to all the authors who submitted their excellent work on time and to the folks at McGraw-Hill and the very persistent Sapna Rastogi! Bhagwan Satiani

xvii

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LOWER EXTREMITIES: MISCELLANEOUS DISEASES

CHAPTER 1

2

AO RTO I L IAC D I S EAS E : O C C LU S I O N Moun i r J .

H a u ra n i ,

MD

PATI ENT STO RY A 60 -year-old woman with hype rt e n.rnplaining of pain in all the digits of the left hand and noticeable discoloration of the left hand during dialysis. H is symptoms resolved a>mple tel y upon d i soont inui ng dialy is .

DIAG NO S I S A N D TREATMENT COU RS E • Photoplethysmography o f th left hand d igi ts showed d iminished wav

forms in all fingers of the left hand . The waveforms im prov ed

upon compression of the fistula ( Fi gures 20- 1 and 20-2).

• Duple.:< ultrasound examinat ion of the fistula showed reversal of flow in the ra di al artery ( Figures 20-3 to 20- 5 ) . •

Diagnostic arteriogram o f th e left upper extremity was performed

that showed normal arterial vasculatw·e of left upper extremity . elective catheterization of the ulnar artery showed ante grade Bow

Fi stula compression

PPG Gain:

Without

With

Without

With

t

l

t

t

Thumb w/Rstula compression 2 Speed: 5 Amplitude: 5 mm 5th Digit finger with & w/o compress ion

Red PPG 5th digit w/Compresslon Gain: 2 Speed: 5 Amplitude: 7 mm Lt index finger with & w/o compression

F I G U R E 20-1 Plethysmog raphic wavefonns of l eft second and fifth digits with and without compression of arteriovenous fistula showing improve­

ment in perfusion with compression of the fistula.

HAN D ISCH EM IA AFT E R P LAC E M E NT O F H EMOD IALYSI S ACCESS

85

in the u l na r utery , f illing of the pa l m ar ard1, a n d retrograde flow

•

Fistula compression

in the r a di al artery ( F igur 20- 6).

Wrth

The patient was brought to the ope rating room and unde.r

local anesthetic,

the distal radial artery was liga ted .

Without

He had dialysis

after the procedure and had no further symptoms of digit isd1emia during dialysis .

E PI D EM IOLOG Y •

lsd>emic digit pain in a h e m od ial ·sis patient "; th a n arteriovenous

fistula can be a pot ential l y eri ous co mpli cati on . In the ma j o rity

of cases, th arterial " steal " causes pain and discomfort, but in extreme cases , it can lead to tiss ue necrosis and eventual lo

PPG Gain:

of

Thumb w/Fistula compression 2 Speed: 5 Ampl�ude: 1 8 mm

d igi ts .

o f d i t a l hypop rfusion i•che m i yndrom v aries -4 a n wh r from I % Lo 20010 . 1 1 t is more com mon in pati n t wh

With & w/o compression

• P r val

have art riovenous fi t ulae bas d as

n

in wh m th

mpared to th

infl w . 1

inflo w from the bra 1ial

art

ry

radial art ry is used fo1· arterial

F I G U RE 20-2 Plethysmographic waveforms of the right thumb with and w i thout compression of the a rte ri ovenous fistul a showing Improvement i n perfusion with compression of the fistula .

CLI N I CAL PRESE NTATION •

Pa t ie nts " � th a r t r i " nou fi tul a may present emergent ly with acute isd1 mia of upper

xtr mity immediate l y after

fi ula placeme nt . These pati n •

need e m e rge nt ligation of

fistula.

However, the majorit of patients present in a d el ayed fashion.

Most of them ar e symptomatic only during dialysi s . According to

some studies, rev ersal of flow can be noticed in up to 30010 of the pa tie nts with arterio,·enous fistulas; however , intervention is indicated in onl those patients who are s mp tom a tic .

Hand

TREAT M E NT OPT I O N S I

1ern ia due to a post -dial

Hea d

· acces arteriovenous fistula can be

treated in a number of wa y :

• o·

I reva

larizati n with int rval ligati n

In this pe 1• t i n, th art ry i l igated distal t

an d a

bypass i

d n

between th

th art ry. Th basi prin "pie i nativ arte 1 .

th ongm

f fi...-rula

proximal and d i tal po rti ns f

lo

stop th

rev r al of flow in th

F l G U RE 20- 3 Direction of blood flow across the a nastomosis Into the proximal and dista l cephal ic vein (blue a rrows). Reversal of blood fl ow in the artery distal to the anastomosis is shown by the red a rrow.

• Banding In this o pe ration, th diamet r of th anastomo is is narrow with a suture (band), o that th ,. lurne of th blood flowing out of artery is reduce d . •

D isto l arterial Ligation

In cases w i tb easily demonstrable reversal of flow on di agnostic

studies, simple Ligation of artery di tal to the origin of fistula can p re v ent reversal of A o w .

PAT I E NT E D U CATI O N Patients hould b e educated about th sign s and symptoms o f h a nd isd>emia, s o they can make d ini ci an s aware of their

Hand

Head

symptoms .

FIGUR 0- Arterial duplex ultrasound study showi ng the an astomosis (blue a rrow).

CHAPTER 20

86

PATI ENT RESOU RCES •

http: I I en. wikiped.ia. orglwiki iVasa.Uar_a=_steal_syndrome

R E F E R E N CE S I . Duncan H , Fergus n L Faris I . Incide n ce of th e radi al steal

ndrome in pa ti nts with Br scia fi st ula for hemodial ysis : its

clinical ignifican 2. K' un

.J

Vase Sura. 1 9 6 ;4 : 1 44- 1 47 .

K B, chanzer H , F ink! r

dynam i evaluation of mgioa Vase ura. 1 979; 1 3 : 1 70- 1 77 .

du re r.

pro el

non i m·asive evaluation of arteriovenous

r

hen

Hand

hem clialy is.

al . Intraope rative fist u l ae and grafts. Th

te al stud . J Vase Tech . 1 994 ; 1 8 : 1 77- 1 92 .

M,

Ulnar artery

, Haimov M, Bu rrows L. H rna ­

3 . DeMa si RJ , Gregory RT, SorreU K A ,

4 . Morsy A , Kulbaski

Radial artery

F I G URE 20-5 Arterial duplex ultrasound study: antegrade flow I n radial artery (red color). Retrograde flow in ulnar a rt e ry (blue oolor). The anastomosis is indicated by the blue arrow.

, l.siklar H , Lumsden A B . I n "dence

and cha.-acteri stics of patients wit h hand ischemia after a

bemodial sis access prooccl ure . J Sura Res. 1 99 ;74: - I 0.

Retrograde radial

F I G U RE 20-6 Arteriogram showing antegrade flow in the ulnar a rtery and retrograde flow In the radial artery (arrows).

CAR OTI D ARTERY O CCLUSIVE DISEASE

PART 3 CA R OTI D A RT E RY OCC LU S I VE D I S EA S E

88

CHAPTER 2 1

21 ARTE R I O S C L E R OT I C

CA ROT I D O CC LU S I V E : S U R G I CAL

M ichael R . G o , M D

PATI E N T STORY A 6 2 - year-o l d

a ucasian man p re se nt e d I week a fte r an ep i s ode

w here be s po nta neo u sl y dropp ed a cigaret te be was h o ldi n g in his dght h an d . He i mm e d iat e l y no te d a n i n a bi l i ty to grasp objects and n um bn e ss in h is right han d . These symptoms lasted for

2

minutes

and then spontaneously resolved . Current! , his right hand feels

com p l ete l y normal. H e denies othe r symp toms such as a m aurosi s ,

p ara l y sis , p a r esthe si as , s p e ech disturbance , or gait disturbance . His p ast med ical history i s sig nificant for h ype rte nsion , h yper ­

cho les terolemia , and c r a m ping in hi calve when he walks long

distance s . He has a

30 pack- year his tor

of smoking and d e n i es

use of a l c o h o l . A caro t i d d up l e x ultrasoun d s h o ws a left 70% to

99% interna l carot i d arter y ( I CA ) stenosi s . A surgical o ption was e lec ted by the pati e n t ( Figur e s 2 1 - 1 to 2 1 - 3 ) .

E P I D E M I O LOGY t roke ranks third a mong all

behind heart di ease and p r yea r . 1

n n ua l l y , 5 5 ,000 mor over 60% of a l l stroke

caus

f death in t he United

w o rn

n than men

are

aiTe

ed ,

dea t hs occur in wo m e n . Afri

m ricans have t wice the st roke ri k of Cau a i a ns .

A m ri

tate

n er , with 79 5 ,000 strok s o cu rri ng

ns a l s o have an i ncreased i n ci d en

to Cau c a si ans . 2

and

n

l e x ica n

of s tr o k e co m p a r e d

ETIOLOGY A N D PATHOPHYSIOLOGY •

One-third of all strokes a re related t o cervical caroti d di..ease .

tandard risk factors for coronar and s yste m i c a therosc l e ros i s

a p pl y to this patient po p ulation s uch as age , male s ex , family hi sto ry , smoking , hypertension , hyperlipidemia, sed e ntary lifestyle, and

high dietary fa t . •

T h e m e c ha ni m of cervi al carotid t r ke is u s ua l l y emboliza­

tion from d1e cuotid bi furcat i on p l a q ue , but he modynami c ri k s of

co m pr om i s e from stenosi ma y a lso p lay a rol . Th

mbolization a n d h m ody nam i co m p r o m i se i ncrea e w i t h i ncrea i ng I C A teno i

.l

DIAG N O S I S •

When

oti d terr i tory tr k or t ra nsien t i

suspect d , •

car

tid d up l

x

in

an a

emi at tack (TIA is

red it d ,·ascular laboratory to

delin degree o f stenosis is m andatory .

When carotid d up l ex is nondiagnostic, co m p ut e d tomogr aphy (C or mag netic reso nance angi og raphy (MRA) may be used .

F I G U RE 2 1 - 1 (A) Carotid endarterectomy ( C EA) c a n be performed via a neck Incision along the anterio r border of the sternocleidomastoid muscle (red arrow). The i nferior border of the mandible Is Indicated by the bl ue a rrow. (B) The platysma is divi d ed and the sternocleido­ mastoid muscle (blue a rrow) and jugular vein are retracted laterally. The head end of the patient is towards the right side of the photos. Procedure Is being performed on the left ca rotid artery.

89

ARTER I OSCLEROTIC CAROTID OCCLUSIVE: S U RG ICAL

FIG URE 2 1 -2 (A) The vagus is preserved i n the carotid sheath . (B) The hypoglossal nerve is encountered cephal ad i n the dissection (dark blue arrow). The i nternal carotid artery (ICA) is shown by the b lack a rrow.

atheter angi graphy is indi

i nvasive tudies and when

t d i n the

s

t ting of conflict ing non­

rot id artery st nting (CA ) is planned . 4

C li N I CAL F EAT U R ES •

Neurologic examination may reveal motor o r senso

deficits

contralateral to the affected carotid artery. Aphasia , d ysphas ia , or apraxia may be reported .

•

Amaurosis fugax, or sudden romp I te or partial loss of vision in one eye, is

a

result of embolization from the cervical carotid artery to

the ip ilatet-al centra l retinal artery. Up to 70% of st rok e survivors can regain functional i ndependen

; howe•·er, 1 5% to 30% becom

permanent ly d isabled and 200/o will require long-term care. 1 I n patients O\•er 6 5 years

r

f age, 6 months after a stroke 500/o have s m

"dual hemiparesis, 300/o require some a istan

2 6% cann t per r. rm a iviti

with walking ,

of daily l i vi ng independ ntly, 1 9%

have aphasia, and 26% are insti tutionalized . ' •

M

n l i � t i me

s t o f an t.ch mk troke in the U n i t d t a t e i s

$ 1 40 ,04 . In 2007 , th total

dollar . 1

o

t of strok

x

eded 4 0 b i U i n

M AN AG E M E NT •

Carotid endarterectom ( CEA) bas been well established

as

a treat­

ment for cervical carotid disease. R ecently , debate has centered on CEA \'er •

us

CA in d1e management of carotid disease .

AU patients hou ld receive op t i mal med i al therap . A pirin is

indi ated for all pati nts with atherosd eroti •

Warfarin may be ind icated

to treat

ro tid d i s as .

patient who have had strok e

from card ia embolization, but there is no ev idence supporting

the use of h parin and warfarin or dopidogrel to p revent or t reat troke related to cervi •

I carotid disease.

M anagement of hypertension and hypercholesterolemia, smoking

ce

arion , and

dietary and activity modification are all mainstays of

treatment . •

Patients with asymptomatic carotid stenosis greater dun or equal to 600/o gain stroke risk reduction with carotid revascularization .

F I G URE 2 1 -3 (A) The intraoperative carotid clamping is tolerated without neurologic compromise by greater than 85% of patients . The plaque is noted by the green arrow as soon as the ca rotid bifurcation is opened . Carotid endarterectomy (CEA) may be perfo rmed with or without intraoperative shunting (dark blue arrow); i f done without shunting , cerebral monitoring of some kind is indicated to detect i ntolerance of clamping. This can be accompl ished by using regional anesthesia with di rect monitoring of mental status, or with general anesthesia using e l ectroencephalo g ram, somatosensory-evoked potential , or i nternal carotid a rtery (ICA) stump p ressu re monito ri ng . (B) Endarterecto my may be done longitudinally as pictured or using an eversion technique without an effect on outcome, but if longitud ina l endarterectomy is performed, patch an g ioplasty offers better stroke and restenosis rates than primary closure (blue arrow).

PART 3 CA R OTI D A RT E RY OCC LU S IVE D I S EA S E

90

However, that risk reduction m ust be onsidered in l ight o f the

pati 11t ' s l ife expectancy , with intervention reserved for patients 78 with at l east a 5 -year l ife e xpectancy . • •

Curre nt medical therapy may outperform intervention in certain low - risk asymptomatic patients , and there is low absolute risk reduction with intervention for asymptomatic patients . O verall stroke risk in medicall · managed asymptomatic patients is only

2% per year , tbus careful patient selection i n asymptomatic

• Patients considered high risk fo r CEA incl ude those with anatomi ibl e lesions, cervical immobi l i ty , prior neck dis-

ection, trachea t my, contra l ateral cranial nerve inj ury, prior

radiation th rapy, oontralatcr� occlusion , and recurr nt stenosis after CEA . Med i a l comorbidit i s conside include presen

d high ri k for

CEA

of chronic ob tructive pu l monary diseas ,

ew

York Heart Association (NYHA) class I I I or I V heart failure , jec­

tion fraction l ess than 300/0, recent myocardial infarction (MI), and un table angina. •

hort- term risks in center of excellence for both CEA and CA may be equiv�ent for the composite endpoint of any st roke, death ,

or M I . •

Age increases risk ";tb CAS. Carotid tortuosity may present prob­ lems with stent or embolic protection device deployment.

•

Symptomatic patients may be better treated by

CEA ,

especially

when older than 70 years or if male . •

70 years may be offered CAS or CEA with equivalent composite stroke o •· Ml or death rates . In this grou p , CA incur higher stroke change for lower Ml risk, and CEA incurs higher Ml risk in

exchange for lower t roke risk .

Circulation . 20 I I ; 1 2 3 : e I -e209 . 2 . Incidence and Prevalence: 2006 Chan Book on Cardiovascular and Luna

Di�ases.

MD: Nationa l Heart , Lung, and B l ood I nsti ­ Av ai l ab l e at http: / /www . nhlbi . nih.gov /resources

Bethesda ,

3 . K l indorfer D, Panagos P, Pancio l i A. Incidence and short- t rm prognosis a ft er· transient ischemic attack in a population-based

study .

Stroke. 200 5 ; 36 : 720-72 3 .

4 . Ricotta J J , Aburahma A, Ascher E , Eskandari M , Faries P , Lal B K . U pdated Society for Vascular S urge ry guidelines for manage­ ment of extracranial carotid disease : executive summary. J

Sur8 . 20 1 1 ; 54 :

Vase

3 2 - 8 36.

5. Aspl L01d K , tegmayr B, Peltonen M . From the twentieth to the

twenty-first century : a publ ic health perspecti\'e on stroke. l n : Gins­

berg MD, Bogoussla\ ky J , eds. Cerebrovascular Disease Parhophpwlo8Y,

DiaB"""is, and Jl,fanaaement . 6 . Ke l l y - Hayes

M,

Malden, MA: Blackwel l Science; 1 998 .

Beiser A , Kase

C ,

Scaramucci A , D ' Agostino

R B , Wol f PA . The inAuence of gender and age on disabi l ity fo l ­ l owing ischemic stroke: t he Framingham study .

J Stroke Certbrov­

asc Dis. 200 3 ; 1 2 : 1 1 9 - 1 26. 7. Endarterectomy for asy mptomat ic carotid artery tcnosi s . Execu ­ t i ,·e Com m i tt ee for the A ymptomatic Carotid Atherosclerosis

8 . H a l l iday A , Mansfie l d A , Marro j , e t � - M R C A symptomatic Caro tid

u rgery Tria l

(A CST)

Colla borative G roup . Preve n ­

t ion of disa bl i ng and fat al strokes by successful carotid endarterectomy i n p a tients w i thout rece n t neuro logica l symptoms : randomised contro l led t rial .

• Patients O\'er 70 years may be offered CEA ba ed on a lower peripro

tatistics-20 I I upd ate : a report from the A meri can Hea rt

Association tatistics Committee and troke tatistics Committee .

tudy. }MfA . 1 99 5 ; 2 7 3 : 1 42 1 - 1 428 .

In th absence of high risk for CEA criteria, patients aged less than

risk in

I . R oger V , Go A , L l oyd-J o n e D . Heart disease and s t roke

/docs /06a_ip_chtbk . pdf. Accessed May 8, 20 1 3 .

Carotid inten·ention is indicated in patients with symptomatic carotid disease i f the stenosis is greater than 5 0% . 9• 10 cal l y ina

R E F E R E N CES

tute ; 2006 .

carotid disease is paramoun t . •

CHAPTER 21

d ural t ro ke risk (Figure 2 1 - 1 ) . I l - l£

Lancer .

2004; 3 6 3 : 1 49 1 - 1 5 0 2 . 9. Ferguson G G , Eliasziw M, Barr H W . The

orth American symp­

tomatic carotid endarterectomy trial : surgica l results i n 1 4 1 5 patients .

FOLLOW-U P •

R ecurre nt stenosis occurs in 6% t o 1 4% o f patients undergoing 17 CEA . urveil l ance using d uplex ultrasound is re mmended w ithin 30 day after

CEA , and an accepted

long-term regimen is

duplex ultrasound e "ery 6 months for stenoses over 50% and year l y dup l ex for stenoses le

than 50%.

1

Stroke. 1 999 ; 30: 1 7 5 1 - 1 7 58 .

1 0 . Randomised trial of endarterecto my for recently symptom atic carotid ste nosis : final results of the M RC Eu ropean Carotid urgery Tria l ( E C 1).

I I . M ante e V, Timaran endarterectomy rotid endart

r

v

Lancet. 1 998 ; 3 5 1 : 1 379- 1 387 .

,

hi u D. The carotid revascu l ariution

rsu st n t ing t ri a l ( R Sl)-stenting versus

ctomy for carotid disease.

Suoke. 20 1 0;4 1 :

3 1 - 34.

PROV I D E R RESOURCE •

1 2 . Yadav J , Wholey I H , K untz RE . Protected carotid -artery

http : / /www .j •-ascsurg. org/artide/ S074 1 - 5 2 1 4( 1 1 )0 1 6 3 5 - I

stenting versus endarterectomy i n high-risk patients .

/a bstract.

Jl,fed. 2004; 3 5 1 : 1 49 3 - 1 50 1 .

EnolJ

1 3 . Eckstein J , Ringleb PA, Allenberg J . Results of the stent­

PATI E NT R ESO U RCES

protected angioplasty versus carotid endarterectomy ( S PACE)

•

study to treat symptomatic stenoses at 2 years: a multinational ,

•

http: / / www . vascularweb. org/ vascu l ;trhealth /Page Icar t id -endarterectomy .aspx

http: / / www . nind . nih . gov / diso rd r /stroke /carotid

_endarterectomy_backgrounder.htrn

prospective, randomised tri al . Loncet. 200 ; 7 : 8 93-902 . 1 4. Mas J L , Chatellier G , Beyssen B. Endarterectomy versus stent­ ing in patients with symptomatic e\'ere carotid stenosis. N

Med. 2006; 3 5 5 : 1 660- 1 6 7 1 .

Enol j

ARTE R I OSCLEROTIC CAROT I D OCCLU SIVE: S U R G I CAL

1 5 . Mas JL, Chatellier G , Beyssen B . Endarterectomy versus angio­ plasty in patients with symptomatic severe carotid stenosis (EVA - 3 S) trial : results up to 4 years from a randomised, multi­ centre trial . Lancet Neurol. 2008 ; 7 : 8 8 5-892 . 1 6 . Carotid artery stenting compared with endarterectomy in patients with symptomatic carotid stenosis (International Carotid Stenting Study) : an interim analysis of a randomised controlled trial . International Carotid-Stenting Study Investigators. Lancet.

2 0 1 0 ; 375 : 985-997.

91

1 7. Roseborough G , Perler B . Carotid artery disease: endarterec­ tomy. In : Cronenwett J, Johnston K, eds . Vascular Suraery. Philadelphia, PA: Elsevier ; 2 0 1 0 .

PART 3

92

C HAPTER 22

CAROTID ARTERY OCCLUSIVE DISEASE

2 2 A RT E R I O S C L E ROT I C

CA ROT I D OCC LU S I V E : ST E N T

M ichael R . G o , M D

CLI N I CAL FEATU RES •

contralateral to the affected carotid artery. Aphasia, dysphasia , or

1 week after an episode

A 72-year-old Caucasian man presented

apraxia may also be reported.

where he spontaneously dropped a cigarette he was holding in his

•

right hand. He had immediately noted an inability to grasp objects and numbness in his right hand. These symptoms lasted for

to the ipsilateral central retinal artery. •

completely normal . He denied other symptoms such as amaurosis , paralysis, paresthesias, speech disturbance , o r gait disturbance . His rolemia, and cramping in bis calves when he walked long distances.

40 pack-year bistory of smoking and denied use of alcohol. 70% to 99%

A carotid duplex ultrasound examination showed a left internal carotid artery (ICA) stenosis .

•

E P I D E M I O LOGY Stroke ranks third among all causes of death in the United States

behind heart disease and cancer, with 1 per year . •

Annually, over

•

5 5 , 000 m ore women than men are affecte d , and

decision to revascularize should be relatively independent of the method of revascularization, with case-specific nuances contribut­

Am ericans also have an increased incidence of stroke compared

ing more to the decision of CAS versus CEA.

to Caucasian s . 2

•

•

the use of heparin and warfarin or clopidogrel to prevent or treat stroke related to cervical carotid diseas e.

smoking, hypertension, hyperlipidemia, sedentary lifestyle , and

•

high dietary saturated fatty acids and cholesterol . The m e chanism of cervical carotid stroke is usually emboliza­

treatment.

promise from stenosis may also play a rol e . The risks of emboli­

•

60% gain stroke risk reduction vvith carotid revascularization.

However, that risk reduction must b e considered in light of the

ICA stenosi s . 3

patient' s life expectancy, \·vith intervention resen•ed for patients with at least a 5 - year life expectancy . 7• 8

DIAG NOSIS

•

When carotid territory stroke o r transient ischemk attack (TIA) i s

Current medical therapy may outperform intervention in certain low­ risk asymptomatic patients, and there is low absolute risk reduction

suspected, carotid duplex i n a n accredited vascular laboratory t o

\vith intervention for asymptomatic patients. Overall stroke risk in

define degree of stenosis is mandatory.

medically managed asymptomatic patients is only

When carotid duplex is nondiagnostic, computed tomography ( CT)

careful patient selection in asymptomatic carotid disease is paramount.

or magnetic resonance angiography (MRA) may be used. •

Patients with asymptomatic carotid stenosis greater than and equal to

zation and hemodynamic compromise increase with increasing

•

Management of hypertension and hypercholesterolemia, smoking cessation , and dietary and activity modification are all mainstays of

tion from a carotid bifurcation pl aque , but hemodynamic com­

•

Warfarin may be indicated to treat patients who have had stroke from cardiac embolization, but there is no evidence supporting

apply to carotid disease such as age , male sex, family history,

•

All patients should receive optimal medical therapy. Aspirin is indi­ cated for all patients with atherosclerotic carotid disease .

ETIOLOGY A N D PATHOPHYS IOLOGY Standard risk factors for coronary and systemic atherosclerosis

Carotid endarterectomy (CEA) has been well established as a treat­ on CEA versus CAS in the management of carotid disease . The

Am ericans have twice the stroke risk of Caucasians . Mexican

•

$ 1 40 , 048 . In 2 007 , the total cost of stroke exceeded $40 billion . 1

ment for cervical carotid disease. Recently, debate has centered

60% of all stroke deaths occur in women . African

One-third of all strokes are related to cervical carotid disease .

Mean lifetime cost of an ischemic stroke in the United States is

MANAG E M E NT

795 , 000 strokes occurring

•

70% of stroke survivors can regain functional independence; 1 5 % to 30% become permanently disabled and 20% ,viJl 5 require long-term care. In patients over 65 years old , 6 months after stroke SO% have some residual hemiparesis, 30% require some assistance with walking, 26% cannot perform activities of daily living 6 independently, 1 9% have aphasia, and 26% are institutionalized. Up to

however,

past medical history was significant for hypertension , hypercholeste­

•

Amaurosis fugax , or sudden complete or partial loss of vision in one eye, is a result of embolization from the cervical carotid artery

2 minutes

and then spontaneously resolved. After this episode bis right hand felt

He had a

Neurologic examination may reveal motor or sensory deficits

Catheter angiography is indicated in the setting of conflicting non­ invasive studies and when carotid artery stenting (CAS) is planned .

•

4

2% per year, thus

Carotid intervention is indicated in patients ,.vith symptomatic carotid stenosis if greater than demonstrated.

9'10

5 0% diameter reduction is

., I

ARTE R IOSCLE ROTIC CAROT I D OCCLU S IVE: STE NT

•

PART 3 CA ROTI D A RT E RY OCCLU S I VE D I S EASE

Patients considered high risk for EA include those with anatomi­ cally inacces ible I si ns , cervical immobility , p ri or neck d.i.ssec tion, trach ostomy, contrala ral cranial nen·e injury, prior radiation

•

•

therapy, contralateral occlusion, and recurrent tc.nosis aft e r CEA. Medical comorbidities consid red high risk for CEA indude pr ence of chronic obstructive pulmonary disease, ew York Heart Associa­ tion (NYHA) class Ul or IV heart failure, ejection fraction less than 3�o, recent myocardial infarct ion (Ml), and unstable angina.

hort-term risks in centers of excellence for both CEA and CA may be equivalent for the compos ite endpoint of any stroke, death, or Ml .

Center for Medicare and Medicaid Services ( C M S) ap p rov a l for CA

continues to undergo scrutiny , but currentl CA is reimbursed only ·

for

high-risk pa tient with ymptomatic high-grade stenosis. Patients

who are at high risk and sym pto ma ti c with 5�A. to 79"Al steno i

and high-risk

asym ptomatic patients

with over 0% steno is can be

co ve red in the context of a trial. These criteria were put forth in the C rmative years of CA when it was considered more reasonable to

attempt

A in patients with

that these ind.icati

•

ns

some contraindication to C E A . Evidence

will evolve come in the form of recent FDA

app roval of several de,;ces for use in nonhigh-risk patients.

Age alone increas ri k with CA , and this is thought to be relat d to increa d arch calcification and changing arch morphology that can make a cess difficult ( Figu r 2 2- 1 ) . Carotid to11: uosity

F I G U R E 22- 1 Steepness o f th e aortic a rch, which c a n b e defi ned b y the vertical dista nce between the ostium o f the i nnominate a rtery a n d the apex of the g reater curve of the aortic a rch , adds techni ca l difficulty to ca rotid artery stenting (CAS). Calcific a tion of the arch and great vessels Is thought to increase peri procedural stroke ri sk during carotid a rtery stenting. Red arrow i ndicates a pacemaker i n place.

93

�

-

-

�-- - c:; ..: -'l � +!i.

-

-

-

-

- .

I

PART 3

94

CA ROTI D A RT E RY OCCLUS IVE D I S EAS E

•

•

CHAPTER 22

(Figure 22- 2) is also asso iated with age and may present probl ms with stcnt or emboli prot ction device deployment . ymptom�ti patients may be better treated by CEA, esp�ally when older than 70 years or if male . Patients meeting high risk for CEA criteria with symptomatic high-grade stenosis may be offered CA as primary treatment (Figures 2 2 - 3 and 22-4). In the absence of being considered higb risk for CEA, patients aged less tban 70 year may be offered A or CEA w;th e9uivalent composite stroke or Ml or death rates . In this group , CAS incurs higher stroke risk in exchange for lower M1 risk , and CEA incurs higher Ml risk in exchange for lower stroke risk . 1 1 " 1 6

FOLLOW-U P •

urveillance after CA should be done with duple.x as after CEA . 1 7 However, velocity criteria should be altered when interpreting post-CAS degrees of stenosis . Decreased compliance after stenting resul in increased A w velocities through the tented area. In this etting, if tandard velocity riteria are used , false! increas d est i­ mates of in-stent stenosis wil l r ult. 1 8 PATI ENT RESO U RC E •

http: / /www . ninds. rub.gov /disorders /stroke/carotid _end;n-terectomy_backgrounder.htm

R E F E R E NCES I . Roger V , Go A , Uoyd-Jones D . Heart disease and stroke

statistics-2 0 I I update : a report from the American Heart Association tatistia; Committee and troke tatistics Committee . CJrculauon . l0 1 1 ; 1 2 3 :e i S -e209 .

2 . Incidence and Prevalence: 2006 Chart Book o n Cardiovascular and Luna Diseases. Bethesda, MD : ational Heart , Lung, and Bl ood I nsti­ tute ; 2006 . Available at http: I /ww w. nhlbi .nih.gov /r ources /docs/06a_ip_chtbk .pdf. Accessed May 8 , 20 1 3 . 3 . Kleindorfer D , Panagos P , Pancioli A . lnciden

and sbort-term progoo is after t ransient ischemi attack in a populati n-bas d tudy. Suok 200 5 ; 36: 720-72 3 .

4 . Ricotta J J , Abw·ahma A , Ascher Eskandari M Farie P , Lal BK. U pdated Sociel for Vascular urgery guidelin for manag menl of extracranial rotid di ease : executive swmnary. J Vase uro . 20 1 1 ; 54: 32- 36. · ,

5 . Asplund K , tegmayr B, Peltonen M . From the twentieth to the

twenty -IU . t oentury : a public health perspective on stroke. In: ins­ berg MD , Bogousslavsl:y J, ed.s. Cerebrovascular Disease Pathop�vsiololiY, Dwgnosls, and Jlfanaoemenc. Malden, MA: Bbd:well Science; 1 99 . 6. Kdly-Hayes M, Beiser A , Kas C , Scaramucci A, D 'Agostino

RB, Wolf PA. The influence of gender and age on disability fol ­ lowing ischemic stroke: the Framinghan1 study. j Stroke Ctrebrov­ asc Du. 2003; 1 2 : 1 1 9- 1 26 .

7. EndaJ-terectomy for a.symptomati carotid artery stenosis . Execu ­ tive Committee for the Asymptomatic Carotid Atberoscl rosis tudy. }Mf.A . 1 99 5 ; 27 3 : 1 42 1 - 1 42 .

F I G U RE 22-2 I nte rn a l carotid artery (ICA) to rt uosi ty (red arrow) i ncreases the techn ica l difficulty of carotid artery stenti ng (CAS}, posi ng proble ms with stent or embolic protection device delivery. Sten osis is indicated by the blue arrow. This image is from a different patient to demonstrate the diffi cu lty with tortuous vessels.

; ' �

ARTE R IOSCLE ROTIC CAROT I D OCCLUS IVE: STENT

•

-f

PART i

- ••I ' -

'I

CA R OTI D A RT E RY OCC L U S I VE D I S EA S E

FIG U RE 22-3 A different view of the carotid bifurcation a nd the origin o f the I nternal carotid a rtery (ICA) shows the tight stenosis (blue arrow).

F I G U R E 2 2-4 (A) Carotid artery stenting (CAS) requires delivery of a percutaneous sheath across the aortic a rch and into the ipsil ateral common carotid a rtery. A gu ide-wire with an embolic p rotection device is used to cross the lesion; the device is deployed in the distal internal carotid artery (ICA), and then angioplasty and stenting of t h e ICA lesion is performed. Options for cerebral protection a re filters (as depicted in the figure), distal occlusion balloons, and proximal occlusion with flow reversal. Fi lters are mechanical screens placed distal to the lesion and collect debris that is removed after stent deployment . Lesions must be crossed i n order to deliver the filter, but they do offer the advantage of mainta i n i ng cerebral blood flow. Distal occlusion devices occlude the distal i nternal carotid with a balloon. The lesion must be crossed to del iver the balloon, and the ICA i s occluded, making this option less attractive to many cli nicians. The flow reversal proxima l balloon method uses two bal loons, one inflated i n the common carotid and t h e other in t h e external carotid. Reversed fl o w from the i nternal carotid is ma intained b y conti nuous a rteriovenous shunt­ ing from the i nternal carotid to a separate sheath in the fe moral vein. This option allows for cerebral p rotection prior to crossing the lesion, but does require larger sheath access, and not all patients tolerate flow reversal . (B) Completion arteriogram shows the stent in place (red bra cket).

95

96

PART 3

CAROTID ARTERY OCCLUSIVE DISEASE 8 . Halliday A, Mansfield A, Marro J, et al. MRC Asymptomatic Carotid Surgery Trial (ACST) Collaborative Group . Prevention of disabling and fatal strokes by successful carotid endarterectomy in patients without recent neurological symptoms: randonUsed controlled trial . Lancet. 2004 ; 3 63 : 1 49 1 - 1 502 . 9. Ferguson G G , Euasziw M , Barr HW. The North American symp­ tomatic carotid endarterectomy trial : surgical results in 1 4 1 5 patients. Stroke. 1 999 ; 30: 1 75 1 - 1 75 8 . 1 0 . RandonUsed trial o f endarterectomy for recently symptomatic carotid stenosis: final results of the MRC European Carotid Sur­ gery Trial (ECST) . Lancet. 1 998 ; 3 5 1 : 1 379- 1 3 8 7 . 1 1 . Mantese V, Timaran C , Cmu D . The carotid revascularization endarterectomy versus stenting trial (CREST)-stenting versus carotid endarterectomy for carotid diseas e . Stroke. 20 1 0 ; 4 1 : S 3 1 -S34. 1 2 . Yadav JS, Wholey MH, Kuntz RE. Protected carotid-artery stenting versus endarterectomy in high-risk patients . N Enal1 Med. 2004 ; 3 5 1 : 1 49 3 - 1 50 1 . 1 3 . Eckstein J, Ringleb PA, Allenberg J . Results of the stent­ protected angioplasty versus carotid endarterectomy (SPACE) study to treat symptomatic stenoses at 2 years : a multinational, prospective, randonUsed trial. Lancet . 2008 ; 7 : 893 -902 .

C HAPTER 22

1 4. Mas JL, Chatellier G, Beyssen B. Endarterectomy versus stent­ ing in patients with symptomatic severe carotid stenosis. N Enal1

Med. 2006 ; 3 5 5 : 1 660- 1 67 1 . 1 5 . Mas JL, Chatellier G, Beyssen B. Endarterectomy Versus Angio­ plasty in Patients With Symptomatic Severe Carotid Stenosis (EVA - 3 S) trial : results up to 4 years from a randonUsed, multi­ centre trial . Lancet Neural. 2008 ; 7 : 8 8 5 -892 . 1 6 . Carotid artery stenting compared with endarterectomy in patients with symptomatic carotid stenosis (International Carotid Stenting Study) : an interim analysis of a randonUsed controlled trial . International Carotid-Stenting Study Investigators. Lancet . 2 0 1 0 ; 375 : 9 8 5 -997. 1 7. Roseborough G , Perler B . Carotid artery disease: endarterec­ tomy. In : Cronenwett J, Johnston K, eds . Vascular Suraery. Pmladelphia, PA: Elsevier ; 2 0 1 0 . 1 8 . AbuRahma A , Abu-Hal:imah S, Bensenhaver J , Dean L S , Keiffer T, Emmett M. Optimal carotid duplex velocity criteria for defining the severity of carotid in-stent restenosis. 1 Vase Sura. 2008 ; 48 : 5 89-594.

ARTE RIOSCLEROTIC CAROTID OCCLU S IVE D I S EAS E : U LC ERAT IVE

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23 ARTE R I O S C L E R OT I C

CA ROT I D O CC L U S I V E D I S EAS E : U LC E RATI V E

M ichael R . Go, M D

PATI ENT STO RY A 5 8 -year-old woman p re sen t ed I week after a single episode of left ­ eye blindness tbat lasted 30 se con d s .

It spontaneously resolved,

and

sbe had no further visual disturb a nces . he den ies weakness , numb­

ness, par a! ·sis , paresthesias, s pee ch disturbance, or gait disturbance .

A carotid duplex ultrasow1d suggested a le s s than 50% left intemaJ

carotid a r te ry (I CA) steno is , but there was a suggestion of a co m ple x

p l a q ue. Further i m aging ";th computed tomograph · (CI) angio graphy was

performed, and showed

a

l e ft ICA eccentric, u l ce rat i ve co m p l e x

p la q ue not associated w i th a significant steno is.

ETIOLOGY A N D PATHOPHYS IOLOGY • Whi le d egree of carotid steno is is related to

mo r pho l ogy rna !ncr ased

·

al o

pia a rol e .

risk � r n e uro l ogi

vents i

tr

ke risk , plaque

with le

n in patien

organized , soft , echol ucen t , compl ex , or ulcera ted plaque , regard­ le

of th deg 1·

o i s . Plaqu t ba t is echolu

of t

gcneous, and ulcerat d , ond has a hig h l ipid co n te n t m o re WlStablc and prone

•

to ru p tu re with embolizat ion . 1

Intraplaque hemorrhage , or pl a qu e with thin or

caps, may also p r ese nt

a

nt, b e t ro­ may be

co 1·

ru p tured fibrous

higher stroke risk ( Figure 2 3 - 1 ) .

F I G U RE 2 3- 1 An ulcerated complex carotid lesion on angiography (red arrow).

DIAGNOSIS • Gross characteristics o f p l a q ue morphology, s u ch as p rese nce o f

ulceration, thrombus, calcification, o r eccentricity , can b e d efined

by standard carotid duplex or angiography. •

Du p l e " ; im aging has the additional capability of identi f}i ng ho mo­

geneous or hete roge neou plaque a n d echogeni or echolucent p l aq u e .

• Intravascu lar u l t raso u nd perform d at •

th

t ime of car tid angiogra­

phy can pr o vi d e even m re detail ab ut pl aque chua tudie o n the use of high- resolution

m p u t ed

t eristi

s.

tomog ra ph y ( CI)

and magn t i resonance ( M R ) i m ag i ng , as weU as £ l uorod oxygl u­ cose

posit J·on emission tomography (FOG-PET) i m aging , to define

what co ns t i tu t e s high - and l ow-risk carotid p l a qu e are ongoing.

• As these imaging t echniques inlp rov e at predicting the behay io r of

carotid plaque, it m;ty become possible to predict which patients ar e

more likely to l stenosis. · "-

benefit from inter•ention for asymptomatic carotid

MA AG E M E N T •

Diag nos is and cli ni ca l features o f ulcerative cervical carotid dise a se

are si mi l ar to those of nonulcerative di sea se . However, rislc of

stroke from these l e s io n s is l e ss well defined .

jJI I

1 1 97

�·

�

PART 3 CA R OTI D A RT E RY OCC LU S IVE D I S EA S E

98

•

A l l pat ie nts should rece ive op ti ma l medical therapy . Aspirin i s ind i­

CHAPTER 23

•

Pat ient! with carotid disease hould also know the signs and s y mp ­

toms of TIA or stroke so that they can seek exped itious tr atmen t .

ted for all patient! with atheroscleroti carotid diseas . •

Warfarin rna

be indicated t o t re a t p a t i e n ts who have ha d stroke

&om cardiac embolization, bu t there i s no evidence s u p p o rting

PATI ENT RESOU RCES

the use of h e p ari n and warfarin or dopidogrel to pre,·ent or

•

treat stroke rel at e d to u lce ra ti v e (or stenotic) cervical carotid

. aspx is a website p rovide d by the

disease . •

M an age me nt of hypertension and hypercholesterolemia,

http: I / www . v ascul arweb . org/ nscul arhcalth / Pages /default ciety fo r Vascular urgery

that contains podcasts and online information abou t carotid dis­

sm ok i n g

ease and other peripheral vascular conditions .

cessation , and clie tary and acti v ity modification are all mainstays of tr e a tm e nt .

me advocate int rvention when patients have had stroke or transi · nt ischemi attack is less than

•

(TIA),

even when th degree of carotid stenosis

50%.

lnt rvenl ion fo r asymplomati

ul erative le ions that p r od uce a

h igh- grade steno is does not d i ffer from nonu l e ra t i ve lesions . •

R E F E R E N CES I . O ' H oll e ran LW, K e nn e l l y M M , M C l ur k c n

N a tura l hi st ory o f asymptomatic carotid 1 9 7; 1 54:6 59-662 .

2.

Horie

N,

plaqu

Intervention for asy mptomat ic co m pl e x or ulcerative l e ions

Morikawa

M,

lshiz aka

Feb 20 1 2 ;43(2) : 3 9 3 - 3 9 .

3 . F igueroa AL, ubramanian

logical fea tures : a comparison between PET activity, plaque mo rphol ­

ogy and his t opathology . Circ Cnrcllovasc lmaelne. Jan 20 1 2 ; 5( 1 ):69-77.

Patients should be informed of the link between atherosclerotic

disease in general an d carotid disease in particular ,vith control lable as

hypertension, diabetes abnormal lipids and

high-fat diet, s m o king , and sed ent a ry lifestyle .

, Cury RC, et al. Distribution of inll am ­

mat io n "�thin carotid atherosclerotic plaques "�th high risk morpho­

PAT I E NT E D U CATI O N

risk facto rs such

, et al. Assessment of carotid

tability based on the dynamic enhancement pattern in

p laqu e components ,vith mult idete tor CT angiography . Srrolu.

without high-grade stenosis is m o re con t roversial but may be

appropriate in selected high st r oke risk patients.

•

M, Jo h nson J M .

plaqu . Am J Sure.

4.

U - K i ng-lm JM, Young V, Gillard JH. Carotid-artery imaging in

the di agnosis and management of patients at risk of stro ke . l.oncel euro/.

2009 ; 8 : 569- 580.

ARTE R I OSCLEROTI C CAROTI D OCCLU SIVE D I S EAS E: OCCLU S I O N

PART 3 CAROTI D ARTERY OCCLUS IVE D I S EAS E

24 A RT E R I O S C L E ROT I C

CA ROT I D O C C L U S I V E D I S EAS E : O CC LU S I O N

M ichael R . G o , M D

PATI E NT STO RY A 5 0-year-old man presented with a 1 -year history of worsening fatigue and bitemporal headaches. He denied focal neurologic symp­ toms, but complained of constant dizziness which was exacerbated when he went from a lying to a standing position . Sometimes he experienced syncopal episodes, and a complete syncope workup thus far had been negative . Carotid duplex ultrasound suggested chronic occlusion of his left internal carotid artery (ICA) , with a 70% to 99% stenosis of his right ICA . Carotid duplex ultrasound also showed elevated velocities in his external carotid arteries (ECAs) bilaterally indicating signifi cant stenosis.

E P I D E M IOLOGY •

Symptomatic ICA occlusion has an incidence of 6 per 1 00,000, though the rate of asymptomatic chronic occlusion is unknown and may be higher. 1

ETIOLOGY A N D PATHOPHYS IOLOGY •

Total ICA occlusion results from thrombosis of or embolization to the cervical carotid in the setting of chronic stenosis. Cardiogenic embolization to a normal carotid bifurcation or carotid dissection may also cause tota l occlusion of the ICA .

•

Acute occlusion may result in a carotid territory stroke.

•

A previously asymptomatic chronic ICA occlusion may become symptomatic related to embolic or hemodynamic issues .

•

Embolism may occur from the ipsilateral ECA via collaterals to the cerebral circulation. It may also occur when there is occult patency of the occluded ICA, which then serves as the source of embolic material .

•

Hemodynamic insufficiency may occur when any condition that interferes with cerebral perfusion such as orthostasis, hypotension, volume depletion, or cardiac failure is superimposed on the carotid occlusion, especially when contralateral carotid disease is signifi cant (Figure 24- 1 ) . 2

CLI N I CAL FEATU RES •

The distinction between hemodynamic and embolic stroke in the setting of chronic ICA occlusion is important.

•

Embolic symptoms are those of classic stroke or transient ischemic attack (TIA) and typically are focal . They may include contralateral motor or sensory deficits or amaurosis .

•

Hemodynamic symptoms may be similar to those of classic stroke or TIA, but may also be less predictable and atypical.

FIGURE 24-1 Occlusion of the intern a l carotid artery (ICA) results i n co l lateral formation via the external carotid artery (ECA).

99

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PART 3

1 00

CHAPT ER 24

CA ROT I D A RT E RY OCCLUSIVE D I S EAS E mptorns such

as

limb shaking, retinal claudi

lion, heacbche

from large pul atile ECA collate rals, syncope , and gcneraliz d 2 fatigue hav aU been reported .

D I AG NOSIS • Carotid duplex ultrasound e.xamination typicaUy shows a high­

resistant signal in the carotid bulb and the very prox:imal iCA (Figure 24- 2 ) . Distally , there are no Doppler ignals audible in the carotid artery. •

Some form of contrast examination such

as

digital subtraction arte­

riography , magnetic re so na n ce arteriography MRA ) , or computed

tomographic Cf) arteriography is generally revill regress spontaneously \·vith reports up to 89% spontaneous closure rate in PSAs less than 3 em . Thus, it may be advisable to treat smal l , nonenlarging PSAs in hemodynamically stable patients with no laboratory evidence of hemorrhage with observation .

Options include o

•

NATU RAL H I STO RY •

A large sheath access (beyond 6 F) in upper extremities should be done with planned surgical exposure of the artery so that the arteriotomy can be repaired under direct vision .

DIAG NOS IS

Symptoms: Painful pulsatile mass is the common presentation . Continued expansion may lead to ecchymosis, cutaneous ischemia, and skin necrosis. Expansion may lead to compression of surrounding femoral nerve, weakness of hip flexion , swelling due to femoral vein compression . Distal embolization and acute limb .ischemia are rare but not uncommon . Rupture can lead to carmovascular collapse and death . Patients can have a large retroperitoneal bleed without any swelJing in the groin due to high femoral arterial puncture.

Femoral arter. i al access should be obtained in the segment of artery that overl ies the femoral head . This 'vill ensure the abil ity to com ­ press the artery against the head of the femur to obtain adequate hemostasis . A high puncture ,.v ill likely result in a large retroperi­ toneal bleed without any groin hematoma. A low puncture usually results in i njury to the profunda femoris artery , superficial femoral artery , and leads to PSA that cann ot be well com pressed.

RISK FACTORS O R ETIO LOGY There is a steamly increasing frequency of PSAs due to increased number of percutaneous interventions. 1 Factors that increase the likelihood for a PSA include

Routine use of duplex ultrasonography or fluoroscopic guidance should be encouraged wh ile obtain i ng arterial access .

PART 4 AN E U RYS MAL D I S EAS E

ARTERIAL PSEU DOAN E U RYSM S

to re onstruct the arterial include •

infection,

system. Compli lions of surgical o·epair

seroma formation, and delayed discharge &om

th hospita l . Death is un usu a l except in

circumstances of shock .

UGC utilizes the ultrasound transducer to visualize the neck PSA

and

apply pressure until it is

occl uded .

procedural suocc ss ranging between 74% and 90% on strating

its cost-effectiveness. 1° Care

of the

tudies have shown

is taken

to

as

well as dem­

PATI ENT RESOU RCE •

be considered . Complications of compression include rupture ,

I . Ahmad F, Turner A , Torrie P, Gibson M . latrogenic femoral

a rte ry pseudoaneurysms-a review of current methods of di agnos is and treatment . Clm Radio/. Dec 200 ; 6 3( 1 2): 1 3 1 0- 1 3 1 6. 2 . Pa tri ck A , A l i FA , arail K F , et al . Femoral pseudoaneurysrns.

Vase Endovascular Sura. 2006;40 ( 2 ) : I 09- 1 1 6 . 3 . Megalopoulos A,

•

UGTI is a minimal ly invasive procedure that involves i nj ecti ng

thrombin into the P A sac with u l tra so und gui dan ce . 10 Idea l ly patients considered for U GTI should have a wel l -visualized neck that should be fairly long , and narro w . However, patients who hn·e a wide neck, short neck , non visualized neck are at a risk for complications of thrombin i njection1 1 and should be carefully selected for this procedure . Using B- mode imaging, a spinal needle ( 2 2 - 2 5 gau ges ) is inserted i nto the P A s ac. While >isualizing

a rev iew

superiority of UGTI over U G C . 14

4. Sloan K, Mofidi R, N agy J, Fl e tt MM, Chakraverty . Endovas­ cular treatment for traumatic popl iteal a rtery pseudoane u­ rysrns after kn e

A po s tinje cti on duplex •

scan h ould

d

monstrate

no flow

in the

sac.

Addi ti onal mod of min im a ll y invasive treatments include endova.s­ cular techniqu es such as coil embolization 15 ( Figure 34- 2 A and B) an d co vered e nt d ep lo yment 16 (Figure 34- 3A and B) . Indications differ depending on the anatomy . Risks o f endovascular approadles include failure of tre at m e nt as well as add iti onal P A formation.

PAT I E NT E D U CATIO N •

If n o int e rv enti o n is p l anne d for a sma l l P A , the patient and fam i ly must be in fo rme d of the a ctivi ty level pemlissible, travel restric­

tions and symptoms and igns of e x p ans ion .

follow - up

A proper plan ult rasound and examination must be outlined .

PROV I D E R RESOURCES •

h tt p : / / w "

•

http: / l www . ncbi .nlm . nih.go,· /pubmed/

.n

bi . nlm . ni h . gov l pu b m d/ 1 0 58 7 38 9 1 9 3706 1 4

for

a rthro p l as ty . Vase Endovascular Sura.

Jun- Jul

2009;4 3 ( 3 ) : 286-290. 5. Barm;lll P , Farber A. Traumat ic pseudoaneurysm of the visceral

ao rtic segment managed using both open sw-gery and endonscu­ lar therapy . Ann Vasc S ura. Aug 20 1 1 ; 2 5{6) : 840. AB, Mi ller J M , Kosinslci A , et al. A prospective of surgically treated groin complications follo,ving percutaneow cardi ac procedures. Am Sura. 1 994;60: 1 3 2 - 1 3 7.

6 . Lumsden

eval uation

7. K at ze nsch lage r R , Ugurl uoglu A , Ahmadi A , et a l . I ncidence of pseudoaneurysm after

diagnost ic and Radio/ow. 1 99 5 ; 1 9 5 :463-466 .

therapeutic angiography.

8. McCann R L , Schwart z LB, Pie pe r K . Vascular compl ications of cardiac catheterization. J Vase Sura. 1 99 1 ; 1 4 : 3 7 5 - 3 8 1 . 9 . K ent KC, McArclle C R , Kennedy B, et al . A p ros pe cti ve study of the clinical outcome of femoral ps c udoan eury s ms

a r terioven o us

fi s tulas induced by a rterial p uncture . J

and Vase Sura.

1 99 3 ; 1 7 : 1 2 5 - 1 3 1 .

1 0. Fellmeth BD, Roberts A C , Bookstein J J , et al. Postangiographic

femoral artery injurie :

ompl ications in lude t he risk of lcin n crosi , femoral vein

thrombosis or , embolization into the art rial ys tem in up to 2% cas

of the

2007 ;40(6): 499- 504.

the needle throughout the procedure (to avoid a=idental

thrombin injection into the arterial system) approximately 1 00 to I 000 U / m L of thrombin is inje ct ed into the sac at approxim ately 0 . 1 mL i ncre ments until th rom bos is is achieved . Color-flow imaging is used while injecting thrombin to demonstrate success­ ful closure of the . ac. veral series have reported success rate between 93% and I 00%. UGTI is the procedure of choice in • 1 pat ients who a re on anticoagulation . 1 2 1 Meta - ana l y is has suggested

iminas , Trel opo u l o s G. Traumati pseudoan­ blunt trauma: case report and literature . Vase Endorascular Sura. Dec 2006- Jan

eurysm of the poplit ea l artery a fte r

especially above the inguinal ligament . PSAs located abo,·e the

inguinal l igament are contraindicated due to the risk of uncontrol­ lable bl eeding. Other contraindications include anastomoti P A, thromho is re ulting in di tal ischemia or iscllemia of the overlying skin , and infection . Long procedure time , patient a nd technician fatigue, patient discomfo rt , and availability of more successful treatment modalities have led to this tedmique falling out of favor.

http : I I en . wiki pedi a. org I wilci I Pseudoa.neurysm

R E FERENCES

avoid compres­

sion of the femoral artery . U G C is successful when P As are less than 2 em . Anticoagulati on bas also been negatively linked t o success of the procedure. II flow is demonstrated in the sac beyond I hour of compression then alternate mode of therapy should

1 39

nonsurgical repair w i th ultJ"asound guided

co mpress i on . Radio/ow. I 99 1 ; 1 78 : 67 1 -67 5 . I I . Cope C,

Zei t

R . Co ag u l a t ion of aneury ms by d i rect percutane­

ous thrombin inj ction.

AJR A m } R�nraenol. 1 986; 1 47: 38 3 - 3 7 .

1 2 . Mo rr ison L, Obra nd DA , t e i nm e t z OK , MontreuH B . Treat ­ m e n t of femoral a rte ry pseudoan urysms ,vith p rcutaneous thrombin injection.

1 3.

Ann Vase Sura . 2000; 1 4 :6 34-639.

heiman RG, Brophy D P . T rea t m e n t

of iatrogenic femoral p se udo an e urys ms with percutaneous thrombin injection : experience in 54 p ati e nt s . Radio/ow. 200 I ;2 1 9 : 1 2 3- I 27.

1 4. Tisi PV, Callam M J . Surgery versus non-surgical treatment for femoral pseudoaneurysms.

Cochrane DaUJbase S_yst Rev.

2006; I : CD00498 1 . 1 5 . Abisi , Chick C, Wi l l iams I , H i l l , Gordon A . Endovascular coil

embolizat ion for large Vase Endovascular Sura.

reports.

l a rsto n WA, Ligush J , et a l . Endovascular repair aneurysm• , pseudoaneurysrns, and art e ri o v no u s Ann Vase Sura. May 1 997; I I ( 3) : 2 56-26 3 .

1 6. Criado E,

peripheral

fi tulas.

aneurysm s : two case o v 2006;40( 5) : 4 1 4-4 1 7 .

femoral false

Oct-

of

1 40

PART 4 AN E U RYS MAL D I SEASE

CHAPTER 34

F I G U R E 34-2 A l a rge pseudoa neurysm a risi ng from superficial femoral artery (A) that was treated with coil embol ization (B).

F I G URE 34-3 A Profunda femoris artery PSA (A) that was treated with combination of coi l embolization and covered stent (B).

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I

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- -�.(�! PART 4

MYCOTIC A N E U RYSMAL D I S EASE

AN E U RYS MAL D I SEASE

35 MYC OT I C A N E U RYS M A L D I S EAS E

Jean Starr, M D, FACS, R PVI

PATI E NT STO RY A 36-year-old ma n was admi tted t o the hospital with sig ns and sym p ­ toms of sep is. He h ad a history of intra,· nous drug abuse as w U as

h patitis B a nd C. He was discovered to hav e a l e ft [. rearm abscess

sit , as well

at an intravenous drug a

as

eptic arthritis of the left

hip with metJ:ucil lin- resistant Sraphylococcus aurcus (MR A) bacte rem i a .

l eft hi p a n d con crva­

He underwent incision a nd dr a in ag of t h

t i ve management of th left foreat-m . a rt

riogram ( CTA)

co m p ut e d tom graphi

abdomen for abdomina l p ai n showed

f th

infla m mati on around th d' tal abd minal

a o r ti tis (Figure 3 5- 1 . A l t ho ugh his

a

rta consistent with

linical cotiJ'S im proved, hi

abdominal p a i n p r · t d and a r pea t CTA I we k I t r ·h wed a an ury m of th d is t a l a 1ta (Figure 3 5 - 2 .

large faI

pt i ns were discus

d a n d he u n d erwe nt su

r pair of the a rta ( Fi gu r

tuaUy on long- �

ful

3 5 - 3 n d 3 5 -4) an wa d '

rm an t ibi ot i

ndous

lar

arg d even ­

F I G U Rr 35- 1 Computed tomographic (CT) sca n wtth periaortic i nfla mmation consistent with aortitis (blue arrow).

E P I DE M I O LOGY •

en t s a rare ti o logy of ao rti aneurysm

Infectious aortitis repr

with one of the largest rev i

ws

revealing tha t 2 . 8% of 67 3 consecu­

th·e abdominal aorti aneurysm (AAA ) patients presented ";th

infectious aort itis

and abdominal •

as

the e tio logy , inducling locations in th thoracic

aortas. 1

The disease is significantly m or e devastating th a n naclitional aneu ­ rysmal disease ";th a large proportion of p atien ts ,_;tJ, a mycotic

a n eurys m ( 1 9%-4 o/o) presenting fo r the hr.>t time wit h rupture . 1 "1

ETIOLOGY OR PATHOPHYSI OLOGY •

The term mycouc i actua l l a misnomer for infectious ao rt itis since most aortic i nfections a re not eco n da · to a fungal pa th oge n .

M a ny organi ms have been im p l icated with

com mon .

thers

in lud

rreprococcu pncumon/ac,

domonas auuamosa, Morsonclla moraani l , almontlla peci

• lnfectio1

s!-9

a ur eus

L istcna monocytoscnrs, Pseu­

It o f seed-

urce or in� ction i n an immunocompr miscd

host . lnfecti ns also o this is a dilferent dis

t

Pasteurella multocida, and

i n native ve els ar most commonly the r

i ng from a r mot

being th mo

cur

se

i n previously placed p ro stheti grail: , but

n t i ty and not the ubject o f this chapter.

DIAG N O S I S

Clinical Feat u res •

The diagnosis f info ctiou s aortiti is o ft e n mad !at , ad ding to the complexities a nd hazards of t re at in g dl elise�.

I G U R E 35-2 Distal aortic mycotic false a neurysm 1 week l ater (bl ue

arrow).

141

PART 4 AN E U RYS MAL D I S EAS E

1 42

•

C HAPTER 35

Patients may be younger and have evidence of remote infection or even a systemic infectious process.

•

The hosts may be immunocompromised from various causes, including cancer.

•

Initial signs and symptoms may be cryptogenic and i nclude vague abdominal or back pai n , nonspecific fevers, elevated white count, and sepsis of unknown etiology . A high index of suspicion is neces­ sary so that treatment can be rendered as early as is feasible. 5

RAD IOGRAP H I C STU D I ES •

•

•

Typically, the most common radiographic study obtained to eval u­ ate a source of abdominal infection is a contrasted CT scan . Early in the disease process, inflammation around a normal sized aorta m ay be the only evidence of aortitis . As the disease progresses, one may see development of an aortic aneurysm and subsequent rapid enlargement (within days to weeks) . The aneurysm may have an u nusual configuration if a pseu­ doaneurysm (PSA) has developed .

MANAG E M E NT OR I NTERVENTION OPTIONS •

Conservative management of this disease process typ ically results in death . o standard operative treatment option for patients with infectious aortitis exists. More traditional approaches include in situ graft replacement with rifampicin-bonded grafting and omental pedicle closure, 3 extra-anatomic bypass with aortic debridement, 4 and in situ graft replacement vvith cryopreservecl arterial homograft. 2

•

FIGURE 35-3 Aortogram demonstrating dista l aortic fa lse aneurysm

(blue a rrow) and i nvolvement of the i l iac vessels.

More modern approaches to complex vascular diseases include endovascular management. Endovascular aneurysm repair for mycotic aneurysmal disease has been reported more frequently as an alternative strategy to open methods with reasonable early (5%) 10 1 1 and late ( 1 1 %) mortality . '

•

The infectious process may involve the visceral segment of the abdominal aorta, usually resulting in worse outcomes. ew tech­ niques and experience with visceral clebranching may offer better 1 options to patients 'Arith a m ore extensive aortic disease process . 2

COMPLICATIONS •

1 In-hospital m ortality ranges from 9% to 2 3% . ' 2 ' 4 Still , a significant 1 number (2 3%) are reported to die soon after discharge. Reinfec­ 1 tion rates during follow-up range from 0% to 8%. • 2 •4

PATIENT EDUCATION AND FOLLOW- U P •

•

Patients may b e advised t o remain o n long-term suppressive anti­ biotic therapy, although recommended l ength of time has not been established . They should be educated as to the warning signs for recurrent infection . Imaging studies every 6 to 1 2 m onths may help to monitor for recurrent infection, PSAs, or other compl i cations, depending on the type of repair.

FIGURE 35-4 Su ccessfu l aortic endog raft placement, reso lving the fa lse aneurysm .

PART 4 AN E U RYS MAL D I S EAS E

MYCOTIC A N E U RYSMAL D I S EASE

PROV I D E R F ESOU RCES •

http: / /radiographics. r na.org/content /2 17/ 1 8 5 3 . full

• http: / /www. researchgate.net/publication/ 593 496_0utoome

_ after_endovascular_stent_graft_treatment_for_mycotic _aortic_aneurysm_a_systematic_re,•iew?ev=pub_cit_i nc PATI E NT- R ESO U RCES •

http: / /en . wilciped ia .org/wiki / Aneurysm

•

http: / / www . fr emd .com /mycotic-U>eurysm/

R E F E R E N CES I . Dubois M, Daenens K, Houthoofd , Peeterrnans WE, Fourneau I.

Treatment of mycotic aneurysms with invol vement of the abdomi­ nal aorta: single-centre experience in 44 consecutive cases . Eur j Vase Endovasc Sura. Oct 20 1 0;40(4) :4 50-456.

2 . Bisdas T , Bredt M , Pichlmaier M , et al Eight-year experience with cryopreserved arterial homografts for the in situ recon­ struction of abdominal aortic infections . J Vase Sura. Aug 2 0 1 0 ; 5 2(2): 3 2 3 - 3 30. 3 . Uchida N , Katayama A , Tamura K, Miwa , Masatsugu K, ueda T. In situ rcpla ment for myooti an urysrns on the thora · and abdominal aorta using rifampicin-bonded grafting and omental pedi le grafting. Ann Thorae Sura. F b 20 1 2 ;93(2):438-442. 4. Yu Y , H ieh HC, Ko PJ , Huang YK, Chu JJ, Lee CH. urgical outcome for mycotic aort ic and iliac anuerysm. World J Sura. J ul 20 1 1 ; 3 5(7): 1 67 1 - 1 678 .

5 . Cartery , Astudillo L, Deelchand A, et a l . Abdominal infectious aortitis caused by Sueptoeoccus pneumomac: a case report and litera­ ture revi w. Ann Vase Sura. Feb 20 1 1 ; 2 5(2) : 266. e9-e l 6 . 6.

B a l A, Schiinleben F, Agaimy A, Gessner A, Lang W. Listeria monoc_rroaenes as a rare cause of mycotic aortic aneurysm. j Vase Sura. Aug 20 1 0; 5 2(2):456-4 5 9.

7. Kwon OY, Lee J , Choi H , Hong HP, Ko YG. Infected abdomi ­ nal aortic aneurysm due to Moraanella moraanu: CT findings . Abdom lmaaina. Feb 20 1 1 ; 36( 1 ) : 8 3-8 5 . 8 . Koelemay M J . Pasteurella mulrocida infection, a rare cause of mycotic abdominal aortic aneurysm. J Vase Sura. Dec 2009; 5 0(6) : 1 496- 1 498 . 9. Dick J , Tiwari A, Menon J, Hamill n G . Abdominal aort ic aoeu­ ry m eoondary to in fection with Pseudomonas oeruatnosa: a rare u of mycoti aneury m . Ann Vase ura. Jul 20 1 0; 24( 5) : 692 . e l -e4 . I 0.

K a n CD, Lee HL, Luo C Y , Yang Y J . The efficacy o f aortic stent grafts in the management of mycotic abdominal aortic aneurysm­ institute case management with systemic Literature comparison . Ann Vase Sura. May 20 1 0; 24(4) : 4 3 3 -440.

I I.

Clough RE, Black A , Lyons OT, et al. Is endovascular repair of mycotic aortic aneurysms a durable treatment option? Eur j Vase Endovase Sura. Apr 2009; 37(4) :407-4 1 2 .

1 2 . oulc M , Javerliat I, Rouanct A , Long A , Lermwiaux P . Visceral debranching and aortic endoprosthesis for a suspected mycoti pseudoaneurysm of the abdominal aorta involving visceral arteries. Ann Vase Sura . Aug 20 I 0; 24(6): 8 2 5 .e 1 3-e 1 6 .

1 43

PART 4 AN EU RYS MAL D I S EASE

1 44

CHAPTER 36

36 VAS C U LA R E H L E RS­

DA N LO S SY N D R O M E

Angela H . Martin, MD Raghu Motaganaha l l i , MD, F RCS, FACS

PATI E N T STORY A 2 3-year- ld woman w>.S seen in a vascular surgery clinic for a pulsatile mass over th calf for a few weeks ' duration. She recollected sustaining

a

min

r

injury while she was horse riding. :he also had a history of easy

brui.sahility . Her brother also has similar history of skin bruising with hypcrmobile joints and

thin skin .

Furlher investigation suggest ed a p

the ant erior tibial artery ( Figur

udoaneurysm arisi ng &om 1e

36- 1 A ) .

was t reat d with ret -

r grade coil embolizat ion of the anteri r tibial artery ( F igur 36- 1 B via a di r

was d

e

·posure of the artery at the ankle . Th arteriotomy site

ed w i t h pi dg ted sut ure s .

period without any

ophor ·is analy

bi psy and collagen el

lar

he did well in the po t perative

mp) jcations . Addit ional investigation w ith kin ·

that �ggested a vas u­

hie•· -Danlos yndromc (ED ) . he was t reated w ith vitamin

suppl m nt and ad vi

d to avoid

ports a nd

cont i nued follow-up.

C

f th n ed for

E PI D E M I O LOCY • • •

Heterog neous group of inherited connective tissue disorders.

ix main forms of ED identilied 'vith several rare variants. 1

Majority arc autosomal dominant inheritance ; orne less com mon forms are autosomal recessive or sex )jnked . 2

• Prevalencc of i / I O,OOO to 1 / 2 5 ,000. •

The vascul ar ( AKA type IV or ecchymotic variant) accounts for

to

J O% of al l c

s of ED

5%

with a prevalence of less than 1 / 1 00,000

overall . 1•4 •

The va

cu lar form has the worst prognosis of all forms of ED with

median age of death at 48 ·e ars . 2

PATH OP H YS IOLOGY de f. ct cause m utation in col lagen synthesi . 2

•

Geneti

•

Vascular ED

is

i ted wid1 t ype I l l pr

due to mutations in the

COL3A l g n ,

r

•

vascular fragility .

l lagen defici nci s

5) without E L = MVPS In the presence of a fa m i l y h istory (5) E L a n d FH of M F S (as d efi n ed above) = M F S (6) Syst (� 7 poi nts) a n d FH o f M F S ( a s defi n ed above) = M FS• (7) Ao (Z � 2 above 20 years old, � 3 b e l ow 20 yea rs) + FH of M F S (as d efi n e d above) = M FS• Scorin g of system i c featu res Wrist a n d t h u m b s i g n-3 (wrist or t h u m b s i g n-1 ) Pectus ca rinatum deformity-2 (pectus excavatum or ch est asymm etry-1 ) H i ndfoot defo rm ity-2 (p l a i n pes p l a n us-1 ) P n e u m othorax-2 D u ra l ectasi a-2 Protrusio a cetab u l i-2 Reduced U S/LS a n d i n creased a rm/h e i g ht a n d no severe sco l i osis-1 Sco l iosis o r thoraco l u m ba r kyphosis-1 Reduced e l bow extension-1 Faci a l featu res (3/5}-1 (do l i chocep ha ly, enophth a l m os, downsl a nt i ng p a l pe b ra l fissu res, m a l a r hypoplasia, retrognathia) S k i n striae-1 Myopia > 3 diopters-1 M itra l va lve pro l a pse (a l l types}-1 Maxi m u m tota l : 20 poi nts; score � 7 i n d i cates system i c i nvolvement A o , aortic d i a m eter at the s i n uses o f Va lsa lva a bove i n d icated Z-score o r a o rtic root d i ssecti o n ; E L, ecto p i a l entis; E LS, ectop i a l e ntis syndrome; FBN 1, fi bri l l i n - 1 m utatio n ; FBN 1 w i t h known A o , FBN 1 m utati o n t h a t has b e e n i dentifi e d i n a n i n d iv i d u a l with

aortic a n e u rysm; FH, fa m i l y h istory; MASS, myo p i a , m itra l valve p ro l a pse, a o rtic root d i l at i o n , skelet a l fi n d i ngs, striae syndrome;

MVPS, m itra l valve p ro l a pse syn drome; Syst, syste m i c score; U S/LS, u pper segme nt/l ower seg m e nt ratio; Z, Z-sco re. •caveat: without d iscri m i nati n g featu res of S h pri ntze n - G o l dberg syn drome, Loeys- D i etz syn d ro m e o r vascu l a r E h l e rs- D a n l o s syn d ro m e .

can negatively affect respiratory function in the most severe cases . Spontaneous pneumothorax occurs in approximately 4.4% of patients with MFS . Recurrence rates of pneumothorax are high. •

Aortic dissection is the most severe, and potentially life threatening, cardiovascular complication of MFS. The underlying pathology that can lead to this potentially catastrophic complication is related to cystic medial degeneration of the aortic wall , which is characterized by fragmented elastic fibers, a decrease in the amount of smooth muscle cells, and the deposition of collagen and mucopolysaccha­ rides between cel.ls of the aortic tunica media. Elastic fiber fragmentation leads to decreased aortic compliance, which results in aortic root dilatation and, in the most severe cases, aortic dissection. Diameter of the sinus of Valsalva greater than 5 em, dilatation rate of more than 1 . 5 mm/y, and a positive family history are the most common risk factors for aortic dissection . These patients require lifelong monitoring of the progression of the aortic dilatation (Figure 3 7-5) . In a significant number of patients,

MAR FAN SYN D R O M E

PART 4 AN E U RYS MAL D I S EAS E

pathology o f the aortic wall results i n aortic valve insufficiency . Laxity of the mitral valve is frequently seen in MFS, resulting in mitral valve prolapse in approximately

50% to 80% of cases. Other

cardiovascular manifestations associated with MFS include left ven­ tricular dilatation and dilatation of the pulmonary and iliac arteries (Figure •

37-6) .

Ectopia lentis (Figures

37- 1 and 37-2) is the most common ocular

abnormality associated with MFS. It develops in utero and can be clinically apparent at the first ophthalmologic visit. Pathophysi­ ologically, ectopia lentis is characterized by the displacement of the lens(es) . Most frequently there is a bilateral displacement of the lenses upward. It is estimated that ectopia len tis is present in approximately

60% of MFS patients . On transnlission electron

microscopy the ciliary zonular filaments appear stretched or

FIGURE 37-1 Ectopia lentis or lens dislocation in M arfan syndrome (M FS). (Photograph courtesy of Robin Vann, MD, and Brian Lutman, CRA.)

interpositioned by disrupted microfibril bundles. It has to be emphasized that ectopia lentis is not pathognomonic for MFS since it is associated with other disorders , which include Ehlers-Danlos syndrome , Weill-Marchesani syndrome, congenital contractural arachnodactyly, sulfite oxidase deficiency, and bomocystinuria. Other less specific ocular disorders associated with MFS include myopia, glaucoma, cataracts, and retinal detachment. •

Lastly, patients with MFS bave a high incidence of pain: Grahame et a! . demonstrated that as many as

70% to 96% of patients report

pain in at least one location in the body . The underlying etiology of the pain is idiopathic and remains to be elucidated .

La boratory Stu d ies •

ln addition to a detailed history and physical examination, other diagnostic modalities including E C G , echocardiography, x-ray, and MRI are utilized to detect major and minor criteria conditions . Which imaging modality is used depends on the presenting symp­ toms. For example , a pelvis x-ray can identify protrusio acetabuli , while an MRI scan of the lumbar spine is an imaging modality of choice to detect dural ectasia. Since the management of MFS

FI GU R E 37-2 This fig u re demonstrates u n i latera l (right eye) ectopic

lens that is displ aced supero-l atera l ly (most common displ acement location) in a 57 years old female patient with M a rfan syndrome. The genetic m utations respo nsible for Marfan syndrome presu mably cause more severe derangements in composition of the microfibrils than those found in ectopia lentis that is not associated with Marfan syndrome which consequently leads to m ore p rofo u n d lens d ispl acement. (Photograph courtesy of Robin Vann, MD and Brian Lutman, CRA.)

exceeds the level of expertise of any single medical specialty, con­ sultations with ophthalmology and cardiology specialists is critical for the proper assessment of the respective ocular and cardiovascu­ lar manifestations. •

It has to be emphasized that family history is not always positive , and approximately one out of four MFS cases are the result of new mutations . Thus, genetic testing for FBN1 mutations are used to confirm the diagnosis, save that FBNl mutations may also cause other Marfan-like disorders and that as many as

9% to 34% of

MFS patients bave no detectible FBN1 mutations . The inability to detect a genetic mutation in

FBN1

does not absolutely exclude the

diagnosis. Currently, molecular diagnosis modalities are character­ ized witb low sensitivity and specificity.

Differential Diagnosis •

Despite distinct clinical , genetic, and pathophysiologic findings, MFS can be confused with other disorders that involve skeletal, cardiac, or ophthalmologic manifestations , especially in patients without a family history positive for MFS . In these patients clinical follow-up may be the only way to differentiate MFS from some of the other disorders. The differential diagnosis of MFS includes

FIGURE 37-3 A positive wrist sign is exempl ified when thumb and fi ngers overlap when encircl ing the contra l atera l wrist. ( Photograph

courtesy of Rocio Moran, MD.)

1 51

PART 4 AN E U RYS MAL D I S EAS E

1 52

o

C HAPTER 37

Homocystinuria is characterized by several overlapping skeletal and ocular features with MFS, and mitral valve prolapse . However, the aortic enlargement is typically not seen in them . It is an auto­ somal recessive disorder and is characterized by elevated urinary homocysteine, mental retardation, a predisposition to throm­

o

boembolic events, and high prevalence of coronary artery disease. Ehlers-Danlos syndrome type IV (the vascular type) includes skin laxity, scarring, easy bruising, as well as arterial dilatation and dissection.

o

MASS phenotype (an acronym for mitral valve prolapse , aortic root diameter at upper limits of normal for body size , striae of the skin , and skeletal disorders) is a familial disorder that includes features si.m.ilar to MFS with the aortic enlargement that

o

is usually milder and nonprogressive . Beals syndrome (congenital contractural arachnodactyly) is an autosomal dominant disorder characterized by multiple joint contractures, significant scoliosis, abnormal pinnae, and muscular

o

FIGURE 37-4 A positive thumb sign is present if the phalanx of the thumb extends beyond the u l na r border. (Photograph courtesy of

Rocio Moran, MO.)

hypoplasia in addition to a marfanoid appearance . Familial thoracic aortic aneurysm and dissection is inherited as an autosomal dominant trait and is characterized by progression at a faster rate than other aortic disorders , and frequently it does not

o

show typical systemic manifestations of MFS . Congenital bicuspid aortic valve disease and associated aortopa­ thy is a cardiovascular disorder with similar histopathologic find­ ings to MFS yet differs as the dilatation of the ascending aorta is

o

often seen at the mid ascending aortic section rather than at the aortic sinuses . The bicuspid aortic valve may function normally . Loeys-Dietz syndrome is another autosomal dominant connec­ tive tissue disorder characterized by arterial tortuosity and aneu­ rysms, craniofacial malformations, and skeletal abnormalities , with an increased risk of dissection throughout the arterial tree including small-size arteries. Additional features include hyperte­ lorism without ectopia lentis and a broad or bifid uvula.

MANAG E M ENT o In addition to genetic counseling and evaluation, patient care in

MFS should focus on the treatment of presenting symptoms and prophylactic measures to maintain and improve cardiovascular health. Serial echocardiograms performed yearly or more fre­ quently and regular computed tomography (CT) surveillance are necessary for detecting and monitoring dilatation of the aorta.

Beta-blockers are indicated in all MFS patients (including pediatric patients) to decrease the risk of aortic dissection by reducing ejec­ tion fraction and controlling heart rate. In a randomized prospec­ tive trial that involved 70 MFS patients, Shores et al . demonstrated that prophylactic treatment with beta-blockers was effective in slowing the rate of aortic dilatation and reducing the risk of aortic 5 dissection. Jn the same study, data from the Kaplan-Meier survival analysis showed that prophylactic beta-blocker treatment was asso­ ciated with improved survival of MFS patients . The beta-blocker dose should be adjusted for age and weight with a goal to maintain a heart rate at 60 to 70 and 1 00 beats /min in patients at rest and after submaximal exercise, respectively. Calcium antagonists and angiotensin-converting enzyme inhibitor drugs are an alternative in patients with beta-blocker intolerance . o Early prophylactic surgical repair of aortic root dilatation is recom­

mended when the diameter at the aortic sinuses of tl1e ascending

FI GU R E 37-5 CT-30 scan demonstrati ng a massive thoracoabdominal aortic aneurysm (diameter 8.6 em) i n a patient with Marfa n syndrome (M FS). =

MAR FAN SYN D R O M E

aorta reaches

PART 4 AN E U RYS MAL D I S EAS E

6

5 cm . In patients with a diameter o f the ascending 5 em , surgical repair is recommended based on

aorta less than

other indications such as family history of aortic dissection, rapid progression of aortic dilatation (defined as aortic root growth of

>2

mm

per year or

5% per year) , symptomatic aortic valve regur­

gitation and progressive left ventricular dilatation or dysfunction, and the feasibility of a valve-sparing operation. The rationale for surgical intervention is based on data showing that the outcomes of prophylactic aortic root surgery are superior to those of emergent surgery. Impact of the preservation of the aortic valve during aortic root surgery on indications for the surgical intervention is contro­ versial and is a matter of debate . •

The incidence of aneurysmal dilatation and dissection of the descending thoracic and abdominal aorta is significantly lower when compared to the ascending aorta and aortic root in MFS, and the majority of surgical procedures on the descending thoracic aorta are performed for expansion of a chronic dissection . The initial management of dissection of the descending thoracic aorta is pharmacologic therapy. Hagan et al . demonstrated that surgical mortality exceeds that for medical management by a factor of

3 .7

Moreover, connective tissue disease, such as MFS, is considered a contraindication for stent-graft insertion in acute dissection by most surgeons. In nonacute settings a maximum descending aortic diameter greater than

6.5

em

or rate of growth

0 . 5 cm / 6 mo is 37-7) . The risk

considered as an indication for intervention (Figure

of dissection in pregnant patients increases with an aortic diameter greater

than 4 em. Thus, frequent cardiovascular monitoring is

FIGURE 37-6 M i l d d i l ation of the i nfra ren al abdom i n a l aorta, an a n e u rysm of the left common i l iac artery (2 em i n diameter), a n d mild d i l atation of the right common iliac a rte ry i n a patient with M a rfa n syndrome (M FS) w h o u nderwent S t . Jude a o rt i c va lve replacement with an ascen ding aortic graft.

necessary for such patients . •

Progressive mitral valve regurgitation that requires mitral valve repair or replacement occurs in up to

50% of MFS patients.

Symptomatic mitral valve prolapse and regurgitation or progressive left ventricular dilatation and / or dysfunction are an indication for 8 surgical intervention However, most patients with MFS will not require mitral valve surgery. When feasible , the mitral valve should be repaired rather than replaced in patients undergoing concomi­ tant aortic valve-sparing root replacement . In patients where the aortic valve is being replaced with a mechanical valve , mitral valve replacement rather than repair should be considered . •

Surgical interventions for spinal deformities in MFS patients (ie , spinal fusion) are associated with high complication rate s . Data from a retrospective study showed that the rate of fixation failure was

2 1 % in MFS and that dural tear , infection, and pseudarthrosis 8%, 1 0%, and 1 0% of MFS patients ,

complications found in

respectively. 9 Failure of fixation is a common complication in MFS patients because of the thin laminae, thin pedicles, and osteopenia. Because of these properties, some authors suggested that the

number of fixation points should be maximized . •

When the protrusion of the medial wall of the acetabulum into the pelvic cavity results in symptomatic osteoarthritis in older patients , total hip arthroplasty is recommended .

on structural bone graft­

ing of the medial wall cavity is frequently performed . 1 0 Younger patients

(e r E M , et a l . The International

Registry of Acute Aortic Dissection ( IRA D) : new i nsights into an

old disease . }MIA. 2000; 2 8 3 : 897-90 3 . 8 . Bhudia

, Troughton R, Lam B, et al. Mitral valve surgery in

843 -848 .

patients after aortic surgery ) . Biannual evaluation i reconu ne oded in some MF

patients with more complex comorbidi t ies.

A nnual ophthal mologic examination, incl uding screening for detachment of retina, glaucoma, myopia, and cataracts , is recom­ mended for a l l MF

patients regardless of prese nt i ng symptoms.

1 994; 3 30 :

1 3 3 5 - 1 34 1 .

ed>ocardiography, computerized tomographic angiography, mag­ patients (especia l ly in

20 I 0;4 7:

P O , Weiss A P , Pyeritz RE . Th e cervical Spine. 1 997; 2 2 : 98 3 -9 9.

the adult Marfan synd rome patient .

netic resonance inlaging angiography, or s t andard aortography of

revised Ghent

pine in Marfan sy ndrome .

vascular examination that includes transthoracic two-dimensional

the entire aorta is recomme nded for aU MF

The Mtd Gtn(L .

476-4 5 .

7. Hagan

A l l patients mth M F

N Enai J Med.

3 . Loeys B L , Dietz HC, Braverman A C , et al .

Thorac Sura.

FOLLOW-U P

•

caused by a recurre nt de novo missense mutation in the fibrillin

gene .

2. Hol lister DW, Godfrey M , Sakai L Y , Pyeritz RE. Immunohisto­

to the autosomal dominant pattern of inheritance . Ultrasonographic

•

I . Dietz H C , Cutting G R , P eritz RE, et al. Marfim syndrome

Genetic counseling should be offered to patients who plan to start a 50% of aU offspring will inherit the genetic mutation for MF due

•

REFERENCES

9. Jones KB, Erkula G,

ponse l ler P D , Dormans J P .

ity correction i n Marfan syndrome. 1 0. Van de Velde

Ann Thorac Sur9. 2006;

Spine.

, Fi l l man R , Yandow

I:

pine deform­

2002 ;2 7 : 2 00 3 - 20 1 2 .

. Protrusio a etabuli i n

Marfan syndrome : history , diagnosis, a n d t reatment . } &ne }oint

Sura Am.

2006 ;

: 6 39-646 .

LOEYS D I ETZ SYNDROM E A N D RELATE D D I SOR D E RS

PART 4

AN E U RYS MAL DI S E AS E

38 LO EYS D I ETZ SY N D R O M E AN D R E LAT E D DI SO RDERS

Recio M oran, M D , FACM G Christina Rigelsky, M S , CGC

PATI E NT STO RY A 26-year-old wo man was referred for evaluation of a possible con­ nective tissue disorder . be first came to medical atten tion in early

childhood when she was diagnosed ";th a pectus excavatum and joint

bypermobility . Additional history induded mitral valve prol apse ,

inguinal and umbilical hernias,

as

well as scoliosis .

he carried the diag­

nosis of bypermobile Eblers-Danlo synd rom e (EDS) fo r many years

y et had normal co ll age n tudies for ED type I V . At the age of 1 9 years, mitral valve repair for severe

mitral valve regurgitation was performed.

ix years later, she presented to the emergency room for evaluat ion of

sudd en -onse t tachycardia and cheo;t pai n. Ecbocardiogram revealed a clilated ao.-ra , and computed tomographic

(Cl) scan sho wed bilateral

subclavian artery aneury ms. The physical ex amin a tion was n ot ab l e for

normal height , weight , and body mass index (BMI). Marfan syndrom

assc

span

ment revealed a systemic score of 6 w i th points for increased arm

to

he ight ratio, pectus excavatum, posith·e wrist sign , mitral valve

prolapse, and bindfoot deformity . Additional physical examination find­

i ngs were notable for a bifid uvula and normal palate. A transforming

growth factor beta c.

(TGF- � ) spectrum

disorder was suspected and a

1 3 6 3T> A mutation in the TGF/3Rl gene consistent with the diagnosis

of Loeys Dietz syndrome ( LD ) was confirmed on sequencing.

E P I DE M I OLOGY •

The prevalence of LDS is unknown . There is no enrichment in any pa r t i cular ethnic or racial gro u p or gen der .

ETIO LOGY A N D PATHOPHY5 10LOG •

Due to po i nt mutations a nd ( rarely) deletions in the TGF/3R l , TGF/3R2, TGF/32, or SMADJ genes .

• •

A utosoma l dom inant disord r .

Highly variable disorder even ,vithin famiues with inco mp lete

penctr.nce. •

M utations in other genes involved in the T G F - P signaling pathway

a re likely to eme rge

as

disease contributing .

fLJ N I LAL F EATU R E LD

is a disorde •· o f connective tissue characterized by: 1

Vascu lar •

Dil at atio n or dissection o f th e aorta most commonly thoracic

( Figure

3 -I)

•

Other arterial aneurysms and dissections ( Figure

•

A rteri al to.-t uosit-y

38- 1 )

F I G URE 38-1 Sa g ittal plane image of a LOS associated dilated aortic root and large left subdavlan artery aneurysm.

1 55

PART 4 AN E U RYS MAL D I S EAS E

1 56

C HAPTER 38

Skeletal o Pectus excavatum or pectus carinatum o Scoliosis

o Joint laxity

o Aradmodactyly

o Talipes equinovarus or hindfoot deformity o Osteoarthritis (SMAD3)

o Cervical spine instability

Cran iofacia l o Ocular hypertelorism

o Bifid uvula or cleft palate (Figure 3 8 - 2 ) o Craniosynostosis

FIGURE 38-2 Wide, bifid uvu l a cha racteristic of Loeys Dietz syndrome

(LDS)

Cutaneous o Translucent s ki n (Figure 3 8 - 3 ) o Easy bruising o Dystrophic

I MAG I N G o I f diagnosis is confirmed o r highly suspected , patients should

undergo imaging from head to pelvis to investigate for additional

arterial disease .

o Cen�cal spine films should be obtained to evaluate for instability.

H I STOPATHOLOGY o Histologic examination of aortic tissue reveals fragmentation of

elastic fibers , loss of elastin content, and accumulation of amor­

phous matrix components in the aortic media .

o Findings of cystic medial necrosis do not distinguish LOS from other causes of aortic aneurysm .

FIGURE 38-3 Thin, tra nslucent skin with large scars i n a patient with

DI FFER ENTIAL D IAGNOSIS o EDS type IV has many overlapping features with LO S . The diag­ nosis of EDS type IV is confirmed by abnormal type Til collagen

biosynthesis and / or identification of a disease-causing mutation in

COL3A l .

Inheritance is autosomal dominant .

o Marfan syndrome also shares many overlapping features with LOS.

Cardinal manifestations involve the ocular, skeletal , and cardiovas­

cular systems with new: revised clinical criteria to help aid in the diagnosis . Ocular findings play a more prominent role in Marfan syndrome than LOS . Molecular testing of FBNl can help aid the clinical diagnosi s . 2

o Arterial tortuosity syndrome is a rare autosomal recessive connec­

tive tissue disorder characterized by severe tortuosity, stenosis , and aneurysms of the aorta and middle-sized arterie s . Skeletal and skin involvement is common . The underlying genetic defect is loss-of­ function mutations in

SLC2A 1 0. 3

o Familial aneurysms and dissections should be considered in patients with aneurysms and / or dissections of the thoracic aorta involv-

ing either the ascending or descending aorta. While isolated aortic

Loeys Dietz syndrome (LDS).

PART 4

LO EYS D I ETZ SYN DROM E AND RELATED D I SORDERS

disease docs o ccu r , additional fmdings include abdominal aortic aneurysms, oerebral and pe rip h e r al artery aneurysms , patent du • tus arteriosus, bicusp id aortic v a l ve , livedo reticularis (a p urp l ish

skin discoloration in a lace y p attern t yp ical l y on the e.xtremities), iris Aocculi asymp to m at i c , ru ptur ed p a p i lla ry e p i the lial cysts ) , and ea r ly - o nse t occlusive vascular diseases. M u ta tion s in the following genes have been associated with this di agnos is : TGFBRl , TGFBRI ,

MYH I I , ACTA2, M YLK, SM.ADJ!

1 57

AN E U RYS MAL D I S E AS E •

Activit y and oth r restri cti o n s should includ th avoi d an

tact spo rt s ,

of con­

competitive sport s , and isometric e. x e rc ise .

PATI ENT E D U CATI O N Patients s uspected o f LD should undergo ge net i c co unse l i ng . Genetic counseling p ro,·ides p atients and their families infor mation

on

the nature of the disorder and inheritance to help them make

informed medical and personal decisions .

M AN AG E M ENT

PATIENT A N D P ROV I D E R RESOU RCES

Evaluation and t r atment for patients w i t h LDS is aimed a t

• Loeys Dietz ynd ro me Foundation : http : / / www . loc ysdietz . org/

e

tabl ishi.ng the extent o f disease and t reatment of m ani fes ta t io n s. 5

Recommendations •

in lude

Ecbocardiography with a tt en ti o n

• N a tio n a l M arfa n Foun d a t io n : h t tp : / /w ww . marfan . org / marfan /

to absolute a o r t ic size and

Z-scores. M agn e t i

resonance angiography (MRA) o r CT scan from head to p e l vi s to id e ntify arterial aneurysms and arterial tortuosit y

distal to the thoracic aorta . o

•

The interval of fol low-u p imag ing \\�l l depe nd on the clinical

fmdings but usually r. Seattle, WA; 1 99 3 .

2 . Loeys B L , D ie t z HC, B ra v e r m an A C , ct al . The •·evised ghe nt nosology for th e marfan syndrome. } Mcd Gene!. 20 1 0;47 :476-48 5 . 3 . Callewaert BL, Willaert A , Kerstjens- Frederikse W , et al .

A rterial tort uo si t y s y nd ro m e : clinical and molec ul a r findin g in 1 2 newl y identified fa m ili es . Hum Mutnt . 2008 ; 29: 1 50- 1 58 .

4.

l i le�cz O M , Re gala do E . Thoracic aortic aneurysms an d aortic dis ections . I n : Pagan RA , Bird TO, Dolan CR, te phe ns K , A d a m M P , eds. Genermew•. Seattle, WA ; 1 993 .

5 . W illi a m s J A , Loeys B L ,

wakanma LU , et al . Early surgi cal expe·

rience w i t h loeys-dietz: a new s yndrome of a ggr essive thoracic aortic an e ury sm disease . Ann Thoroc Surg. 2007 ; 8 3 : 7 5 7- 7 6 3 ; discussion 578 5 -790.

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NON­ ATH ER OSCL ER OTIC DISOR D E RS

1 60

PART S N O N -ATH E ROSCLEROTIC D ISORD E RS

CHAPTER 39

39 PO P L I T EA L A RT E RY E N T RA P M E N T SYN D RO M E

B h agwan Sati a n i , M D , M BA, R PV I , FACS

o Examination can show arterial compression elicited by maneuvers

such as plantar flexion and dorsiflexion of the feet.

A 2 8-year-old male athlete presented with increasing difficulty running, resulting in severe bilateral calf cramping. He consulted \v:ith orthopedics and neurosurgery with no definite etiology identified . Vascnlar surgery was consulted with a provisional diagnosis of a compartment syndrome . On physical examination he had normal pulses at rest. Diminution of dorsalis pedis and posterior tibial pulses was appreciated on both plantar flexion and dorsiflexion . Duplex ultrasound examination showed normal signals at rest, obliteration of arterial signals on plantar flexion, and reactive hyperemia on a neutral foot position.

PATHOP HYSIOLOGY Popliteal entrapment syndrome consists of a group of conditions where vascular and/ or neurologic compression symptoms result due to com­ pression of the popliteal artery, vein, or nerve. The compression is due to a congenital anomaly where the popliteal artery becomes functionally occluded by passing medial to and under the medial head of the gastrocnemius muscle or a slip of that muscle, \'lith consequent compres­ sion of the artery. o Relationship of muscle and artery in the popliteal fossa resulting in

extrinsic arterial compression .

o Repetitive insult to the popliteal artery can cause arterial damage

and lead to aneurysm, thromboembolism, and arterial thrombosis.

Although there are several classifications, the four types of embryologic entrapment in a commonly used classification system are shown in Figure 39- 1 . 1 • 2

confirmed by noninvasive studies.

o Duplex ultrasound can be diagnostic, although some authors have

reported false-positive studies in athletes.

5

o A baseline duplex ultrasound examination is performed with

the foot in the neutral position . Normal triphasic Doppler signals are documented (Figure 3 9 - 2 ) . While the Doppler probe is insonating the popliteal artery, the foot is dorsillexed and plantar flexed to assess Doppler signals in the distal popliteal artery . Typically , the arterial signals are severely attenuated or absent during one of these maneuvers (Figure 3 9 - 3 ) . After documenting occlusion of the artery , once the provocative maneuver is released the arterial signals resume , often with reactive hyperemia (Figure 3 9 -4) .

o A contrast arteriogram shows deviation of the popliteal artery from

the middle of the popliteal fossa (Figure 3 9 - 5 ) . The artery often appears narrowed, although this is due to extrinsic compression not intrinsic pathology. Long-standing compression can result in post-stenotic dilatation, aneurysm formation, or even occlusion of the artery.

o Computed tomography (CT) or computed angiography and

magnetic resonance angiography (MRA) can be diagnostic and may replace contrast arteriography.

D I F F E R E NTIAL DIAG NOS IS o Unstable plaque or thrombosis .

The mean age of patients is 32 years . 3 About one-third of patients have bilateral entrapment.

o The diagnosis is made by history and physical examination and

o Arterial occlusive disease .

CLI N I CAL FEATU RES o Patients are more commonly male.

DIAG N OSIS

o Cystic adventitial disease.

4

o History of leg pain or aching and tiredness; cramping on walking

or exercise (intermittent claudication) , which is relieved by rest, is the most common presenting symptom . Some swelling may also be described .

o A number of patients may remain completely asymptomatic until

the artery is either severely narrowed or completely thrombosed .

o Occasionally patients may present \·vith acute arterial ischemia due

to thrombosis of the popliteal artery from long-standing entrap­ ment, intimal damage, and eventual thrombosis . The tip-off is the young age of the patient, no evidence of arteriosclerosis elsewhere, and the prior history of claudication as described above .

o Compartment syndrome.

o Arterial embolism: acute limb ischemia due to thrombosis of the

popliteal entrapment.

o Sometimes there is a functional popliteal entrapment syndrome

where there is no anatomic abnormality but a functional entrap­ ment due to gastrocnemius hypertrophy and resulting arterial compression. l110 s e \'lith functional entrapment are younger, more commonly females, highly trained athletes, and have normal nonin­ vasive tests .

o Another problem is that up to 5 6% of asymptomatic indi,�duals can

exhibit evidence of popliteal artery occlusion by duplex scanning \'lith provocative maneuvers. 3

POPLITEAL ARTE RY E NTRAPM E N T SYN DROME

PART 5 N O N -ATH E ROSC LE R OTI C D I SORD E R S Type I I

Type I

Popliteal artery

t h e gastrocnemius

Type I l l

Type IV

Popliteal vein Accessory slip of the gastrocnemius

Medial head of the gastrocnemius

FIGURE

39-1 Types of p opl itea l artery entrapm ent.

Type 1 shows l ocati o n of the medial head of gastrocnemius muscle, wh ich is attached more l atera ll y than Is n o rma l , with resu lting p opl i tea l artery entrapme n t Type 2 shows an a b n o rma l course of the pop l itea l artery, with entrapment. Type 3 shows l ocat i o n of an anoma lous muscle band with abnormal attachment, with resu l ti n g popl iteal a rtery entrapment. T y p e 4 shows p r i m itive position of the distal popliteal a rtery pos terior to t h e pop l iteus muscle. {Repri nted from P i l l a i J. A cu rrent I nterpretation of pop l itea l vascu l a r entrapment. J Vase Surg, 2008 ; 48:561 -565, with permission from E lsevier.)

M AN AG E M ENT •

A definitive diagno is resonan

art riograph

angiography . Th

s

n o rm a l , p la nt a r

r

po plit I

art

n be ,

studi

or

t ablished b art riography , ma gn tic

metim

s

t ypical ly sh

a computed tomographi w

dorsiflexion is performed with tress

contrast study repeated to demon•trate th cases,

m e di a l d viation of the

ry, occlusion , or a post:-st n ti dilatation. lfth artery is

the artery

m ay be

po iti nal abn rmalities . I n severe

occluded .

• The surgical approach is e it he r a medial or posterior calf inci s i o n i n

to access the artery and resc t t h musculotendinous abnor­ mality in addition to either repairing or replacing the artery when necc sary . Results arc xcellent when the condi tion is treated early before progression to throm is or I of collaterals .

o rd e r

•

Endovascular stenting of the popliteal arte ry has occasi onally bee n per­

fanned for relief of the entrapment. However, recurrence bas been the

no rm ,

and surgical intervention is the preferred treatment option. '

F I G URE 39-2 Duplex ultrasound ex a m i n ation at rest showing normal D oppl er signals.

161

PART S N O N -ATH E R OSC LE R OT I C D I SO R D E R S

1 62

CHAPTER 39

PAT I E NT EDU CATION Complications after urgical release "�th or wi tho ut arterial repair the usual surgical compl ications

(besid

indude

uch as wot.md problems)

• Comparunent ·ndrome •

Lymph leaks thrombosis of the r

rte.-ial •

Venous

pair

thrombosis

eurologic side effects : superficial or deep nerve r

Lat

injury

currence

Patients

as

must be ad,�scd on the possibility of these compl icat i o ns immediate and late fol low-up and the chance of the cont ral­ limb being symptomatic later o n .

well

ateral

as

FOLLOW-U P Although recurrence of thi s condition is

rare ,

clinical

F I G URE 39-3 Duplex ultrasound examination indicating cessation of a rterial signals on dorsifl exion of the foot, confi rmi ng popliteal a rtery e ntrap m ent.

fol low -up is

r=o mmended . Athletic activity can be resumed once wound healing

has occurred and tone

•

back to

phy si ca l

nonnal .

therap · has returned

musde strength

http: / I e n . wikipcdia .org/ wiki/ Popliteal_artery_entrapm yndrom

and

nt

_

• http : / / " vw .liv strong.com /article / 1 3 5 79-popl.iteal -a.rtcry -entrapment -sym ptoms /

R E F E R E NCES I . Pillai J . A current interpretation of popliteal ,·ascular entrapment .

J

l 'asc Sure. 200 ;48 : 6 1 - 6 5 .

2 . Delaney TA , Gonzalez LL . Occlusi n o f popliteal arte m uscle

3.

inha

F IGURE 39-4 Duplex ultrasound examination showing cessat ion of arte­ rial signals with planta r flexion and resumption of normal arterial signals in the popliteal artery on releasing the foot from plantar flexion.

due to

entrapment . Sure•IJ · 1 97 1 ; 69 : 97- 1 0 1 .

,

n J , H It PJ , Tb mps n M M , ftus I M , RJ . Popliteal ntrapment ynd rom . } l'osc ure.

H ught

Hinchl iO:

20 1 2 ; 5 5 : 2 5 2-262 . 4.

UrgJtl

I mis N , Eli

, Agg labs J,

and surgi I appr ch of p

pli t al

art

, Georgi u

re trospe ctive study. Jlasc Ht'tlirh RJ.ri tlfonae. 2008;4:

5 . Tu rn ipseed WD. Po plit ea l 200 1 ; 3 5 : 9 1 0-9 1 5 .

. Diagnosis

ry entrapment synd rom :

3-

a

.

entnpment syndrome. } Jlosc ure.

6 . Di Marzo L , Cavalluo A , O ' Donnell D , higematsu H ,

Levien LJ , Rich N . Endo,·ascular stenting for poplit I ,·ascular en­ Sure. 20 1 0;24( 8 } : 1 1 3 5 .e l - 3 .

n·apment is not recommended. Ann Vase

FIGURE 39-5 Arteriogram showing bilatera l po pl itea l artery deviation from the midline in the popliteal fossa with na rrow in g due to en trap m ent.

PART S N O N -ATH E ROSCLEROTI C DISORDERS

I LIAC ARTERY E N DO F I B RO S I S

1 63

40 I LI AC A RT E RY

E N DO F I B RO S I S

J e a n Sta rr, M D, FACS, R PVI

Exercise pressures Rest

A 47-year-old woman presented t o the outpatient vascular cbnic with a 1 -year history of left leg pain that began with cramping in the left calf area, progressed up to the thigh, and now is in the buttock and hip region. Even walking a short distance had become significantly uncomfortable, and the pain had progressively gotten worse. She denied any rest pain or nonhealing ulcers . She was an avid runner and was training for a marathon when her symptoms began and now had stopped exercising. She was initially told she had a muscular strain and to bmit activities, but she found the discomfort unbearable , even with walking. She was a bfelong nonsmoker and had no significant past medical history . Physical examination showed a 5-ft, 1 -in, 1 05 -lb female with a diminished left femoral pulse with no palpable left pedal pulses and normal contralateral pulses . oninvasive Doppler testing showed an ankle-brachial index (ABI) on the right of 1 . 2 , which did not change with exercise . The ABI on the left at rest was 0. 7, which indicated moderate disease , and this further dropped to 0 . 45 with exercise (Figure 40- 1 ) . The left femoral waveform was monophasic (Figure 40- 2 ) . Duplex ultrasound imaging confirmed a left external iliac occlusion (Figure 40- 3 ) . The options were discussed with her, including conservative management, open surgery, and endovascular methods . She opted to undergo angiography (Figures 40-4 and 40-5 ) with concomitant left external ibac stenting. The occlusion required antegrade and retrograde approaches in order to successfully cross the lesion (Figure 40-6) and place a stent (Figure 40-7). Her postprocedural ABI returned to normal (Figure 40-8 ) .

E P I D E M IOLOGY o Iliac endofibrosis was first described in cycbsts in 1 986 by

Chevaber. 1 The true incidence of iliac artery endofibrosis is not known, and few cases have been reported .

o It has been described in young, high-performance athletes, typically

runners and cyclists, with few, if any, other risk factors for vascular disease.

ETIOLOGY AND PATHOPHYS IOLOGY o The external iliac artery hes distally and posteriorly in the peh�s.

Endofibrosis has been postulated to arise from repeated trauma from a hypertrophied psoas muscle where it apposes the external iliac artery during hip flexion . 1 Proximally, the external iliac artery is fixed by the common ihac bifurcation, and distally it adheres to the inguinal bgament structures. It is in this relatively fixed segment

2

R Ankle (DP): L Ankle (DP): L Brachial:

1 58 1 02 131

1 74 62 1 38

R AB I : L AB I :

1.21 0.78

1.26 0.45

3

4

5

6

7

8

9

Exercise pressures L Ankle (DP)

- L Brachial

10

I

200

8" 1 80 � 1 60 E

.§.. �

:::> "' "'

�

Cl. .2

B

"' >(J)

-4

1 40 120 1 00 80 60

----

----.

40 Rest Minutes FI GU R E 40-1 N o n i nvasive exercise test demonstrati ng drop in left

ankle-brach i a l index (AB I ) after exercise.

PART S N O N -ATH E ROSC LE R OT I C D I SO R D E R S

1 64

CHAPT ER 40

Doppler R) Femoral

L) Femoral

Segmental B P index

I Gain: 23%

Segment-brachial

Brac hial

1 28

I

Gain: 5 1 %

131

L) Popliteal

Gain: 5 1 o/o

R) Post Tibial

L) Post Tibial

Gain: 51 %

Gain: 5 1 %

R) Dors. Pedis

L) Dors. Pedis

Gain: 49%

Gain: 5 1 o/o

1.21

An kle-brac hial i ndex

0.78

FIGURE 40-2 Dim inished left femora l and distal waveforms.

that external forces may

tion,

as

a wel l

th r

theori

arte ry ,

art · rial

r

s

compr

im·

ulting in

sid e

cause ws

el mechanical stre

sion, r esu l ti ng

lve high A

w

in luminal

an l

elonga­

rates through this egment of

ndothel ial damage ,

branch

-

steno is. 2

as

well as kinlcing d ue

tethering th artery in place. J

morp hologic d1aracteristics of endofibro­ it from atherosclerot ic lesion , including a l ow e r 1 of calcification4 and intimal 1bro is.

o The re tends to be eli tin

is

to

dill"e rentiate

inciden

e

CLI N I CAL F EATU R ES o Claudi

fatigu ,

ti n is classic al l y charact rized

cramp,

or dis

able, usually occurring pro

m fort, which

one

is

a.s

th onset of mus

e

repr ducible and p r e d i ·­

levd distal to an art rial occlusive

FIG U R£ 4 -3 Duplex imaging of left iliac a rtery occlusion

(yellow arrow).

I LIAC ARTERY E N DO F I B RO S I S

•

PART S N O N -ATH E ROSCLEROTI C D ISORDERS

When claudication i s secondary t o iliac artery endofibrosis, patients often do not possess otber stigmata of vascular disease and often present witb proximal tbigh muscle fatigue or weakness.

•

Initially, tbese high-performance athletes may be told tbey have muscular strains or sprains or even compartment syndrom e . Physical tberapy may be prescribed , which will obviously n o t rem­ edy tbe underlying pathology or symptomatology.

D I F F E R E NTIAL DIAG NOSIS •

Claudication secondary t o tbe more common atherosclerosis proc­ ess and pseudoclauclication due to spinal stenosis should be consid­ ered , altbough unusual in tbis unique athletic patient group.

•

Claudication is typically worked up by first obtaining noninvasive studies in tbe vascular laboratory. Tbis may include physiologic stuclies to assess quantitative blood How, ABl measurement,5 arte­ rial duplex imaging, or any combination of tbese.

•

Exercise testing may help unmask underlying occlusive clisease in tbe patient who presents witb a normal resting ABl, which may especially occur in patients witb iliac artery endofibrosis who do 67 not yet have an occlusion. •

•

Color duplex imaging may help to characterize

kinks and intravas­

cular lesions in tbe external iliac artery, especially witb tbe use of provocative maneuvers . 8 •

Contrast studies are usually next in lin e . Tbis may involve com­ puted tomographic (CT) angiography, magnetic resonance (MR)

FIGURE 40-4 Angiogram reve a l i n g left i l iac occl usion, reconstitution of l eft femoral, and large left pelvic co l latera l bed (b lue a rrow) .

angiography, or catbeter-based angiography . Each has its own inherent advantages and disadvantages . •

Altbough CT and MR are noninvasive, catbeter-based angiography offers tbe advantage of potentially treating tbe disease at tbe same time by minimally invasive means . Angiographic findings may range from subtle stenosis to complete occlusion , witb possible intraluminal thrombosis . 9 Typically, tbere may be no otber signs of vascular occlusive disease , altbough tbere may be evidence of con­ tralateral disease in tbe same location.

MANAG E M E NT OR I NTERVE NTION OPTIO N S •

T h e ideal treatment for iliac artery endofibrosis would include alleviating the obstruction , as well as eliminating the inciting cause . Most likely , the latter is not feasible unless the same activities that caused the vessel injury (running , cycling) were avoided .

•

T reatrnent aimed at relieving tbe obstruction includes iliofemoral bypass (for longer segment of clisease) , iliac endarterectomy witb patching, shortening of tbe arterial segment (for hemodynamic kiulcing) , and endovascular approaches witb balloon angioplasty and 3 10 1 stenting. • • 1

•

Open surgical approaches typically require a retroperitoneal and possible groin incision , wbich may not be desirable in endurance athletes .

•

Endovascular procedures offer a minimally invasive means to improve arterial How, tbereby returning an athlete to endurance activities more expediently. Good results have been obtained from

FIGURE 40-5 Left extern a l i l iac occl usion (blue arrow).

1 65

PART S N O N -ATH E R OSC LE R OTI C D I SO R D E R S

1 66

CHAPTER 40

t h surgiel approaches; however, few l ong - t e r m results exist after endova cular m an ag ment . CO M PLICATIONS • Nontreatment results in continued s rmptorns and is typicall not

tolerated by this high-performance group of patients . The natural history of untreated iliac artery endofibrosis is unknown.

•

urgicaJ and endovascular in te rv en ti on can result in typica l revas­ cularization compl icati ons of graft stenosis or occlusion, infection , nerve injury, and pseudoaneurysm fonnation, among others.

PAT I E NT E D U CATI O N A N D FOLLOW- U P • Pati n ts should be ad vis d to avoid th e activ itie t h a t

initial arterial injury , but this m a y no t be p ala t ab l e t o

used th most athletes.

I

• Follow-up should be a dv i sed every 6 to 1 2 months to assess for

recunence of symptoms and graft patency .

'�PATI ENT'RES.OU RCES" • http : / / wv.-w .livestrong.com /artide / I 8 3 5 79- popliteal-artery -entrapment -symptoms/

• http: / /cyclingtips. com .au/ 20 1 1 / 1 1 /

..-ci -induced-arterial

-endofibrosis I

F IG U RE 40-6 Lesion crossing {blue arrow).

R E F E R E NCES I.

he,·alier J M , Enon B, Walder J , et al. End fibrosis f the

iliac artery in bicycle ra pathologiel state . Ann Vase Surg.

external

2.

rs:

N ov

unrecognized 1 9 6; I 3 ) : 2 97 - 3 0 3 .

an

Scavee V, tainicr L, D ltombc T, et al . External iliac artery endofibrosis : a new possible predisposing factor. } l'asc Surg . J ul 200 3 ; 3 ( 1 ): 1 0- 1 82 .

R ehman A, Brndbury A W . External iliac endofibrosis in endurance athletes: a novel case in an endurance runner and a re,·iew of the literature. Eur J Vase Endomsc Surg. Dec

3 . Ford SJ ,

2 00 3 ; 26(6):629-634.

4 . Vink

A , Bender MH, chep G , et al. Histopathological

com parison between endofibro is of the high-performance cycl i t

and athero lerosis in the external ilia artery . J Vase Surg. Dec 200 ;48(6 : 1 4 5 8 - 1 46 3 . 5 . Ta y l or A J , G org KP. Ankle to brachial pressure ind x in n rmal ubje and train d cy list with exer ise-indu ed I g pain . Mtd ci Sports Exue. N o v 200 1 ; 3 3( 1 1 ) : 1 862 - 1 67.

6. Fernandez-Garcia B, Alvarez Fernandez J, Vega Garda F, et a!. Diagnosing extemal ilia endofibrosis by pos t e x ercise ankle to arm index in cyclists. M�d Sd Sports E=c. Feb 2002 ; 34( 2 ) : 222 - 22 7 . 7. Abraham P, Bickert ,

ieUe B, hevalier J M , aumet J L . Pressure measurements at rest and after heavy exercise to d e te ct moderate arterial lesions in athletes . j Vase Sura. Apr 200 1 ; 3 3 (4): 7 2 1 -72 7 .

MH, Schmikli SL, Wijn PF . Color D oppler used to detect kinking and intravascular lesions in the iliac arteries in endurance athletes ";th claud i ca ti on . fur J Uluasound. Dec 200 1 ; 1 4( 2 - 3 ): 1 29- 1 40 .

. Schep G, Be n d er

FIG U RE 40-7 Post-stent placement (blue a rrow at proximal stent).

PART 5 N O N -ATH E ROSC LE R OTI C D I SORD E R S

I LIAC ARTERY E N DOFI B ROSIS

r---- Segmental BP -----, Segment-brachial index Brachial

1 32

R) Post Tibial

L) Post Tibial

Gain: 50%

Gain: SO%

R) Oors. Pedis

L) Do rs. Ped.

Gain: 50%

Gain: SO% 1.20

Ankle-brac hlal l ndex

F I G U R E 40-8 Postprocedure a n kle-brachial i ndex (ABI).

9. Kral CA , Han DC, Edwar ds WD , p i t te ll PC, Tazelaar H D ,

Cherry K J Jr. Obstructive temal iliac arteri opathy i n avid bicyclists : new and ,·ariable h is to p atho logic features in fou r women . J Vruc Sura. ep 2002 ; 36( 3 ) : 565- 570.

1 0. Bucci F, Ottaviani N , Plagnol P. Acute thrombosis of

external iliac arte ry secondary to e nd o fibrosis . Ann Vase Sura.

Jul 20 1 1 ; 2 5 ( 5 ) : 698 .e 5 -7 .

I I . Maree AO, Ashequl I s l a m M ,

nuderl M , et al External iliac artery endofibrosis in an amateur runn e r: hemodynamic, angiographic, histopathological evaluation and percutaneous revascularizat:ion . Vase Med. Aug 2007; 1 2( 3) : 2 0 3 -206. .

1.09

1 67

PART S N O N -ATH E R OSC LE R OT I C D I S O R D E R S

1 68

CHAPTER 4 1

41 K L I P P E L-T R E N A U N AY SYN D RO M E

Maria E. Litzendorf, M D

PATI ENT STO RY nt d w ith painful right -leg varicosities. h was actively involved in s •vera! ports , and noticed increasingly painful varicos i ti es in her posterior -latenl thigh and extending distally down A 1 6- year-old girl pre

to her foot . Shortly after h r birth purple-red superficial discoloration

along h

r right leg extending up to her !lank that abruptly tenninated prior to cro ing midline was identified. everal right limb- hortening p ro edures were required . Initial management included gradient compr ssion hose with inlprovement in her pain. he pr 1ted several years later w ith worsening symptoms and underwent resecti n of her symptomati vein dust r foil wed by targeted

sclerotherapy a typi al

everal months post peratively . Figure 4 1 - 1 ill ustra t

of

synd rome ( KT ) . While gen rally benign, KT can entations that benefit from urgery , derotl1erapy ,

Klippel-Tr naunay hav

unusual p

and I or thermal ablation treat ment .

E P I D E M IOLOGY Rare condi t ion , sp radi in pat tern , but may

o 0

curs

in aU

hav

• A ffects males and femal 2 births

M

l

inberit an e

no dear genet i

orial inbel"itance pattern

etbni groups equall /

o Lack of large tudi 0

nat ure ,

multifa

s,

equa l ly

but inciden

post ul ated at I

in I 00,000

live

rnmonly diagnosed in dlildh od

ETIOLOGY A N D PATHOPHYSIOLOGY o Un I ar and th p a rt i al

ntrover ial et iology.

persist

de r mal cl velopm

a

re u lt

Comp ling t heories i nclude of a n embryologi vascular ystcm , meso­ ntal abnorma liti , and vmous hyp !"tension as

n

of vascular agenesis, atr

ia, or hypoplasia p

ibly related

to cornpre si n of the d cp venous system by abnormal muscl

or

fibrovascular cord . J.. l

o May be associ a ted with increased maternal age, increased paternal

age , and increased num ber of pregnancies . Vascular malformations

are

found at increased rates in family

members

of patients with

KT 2

D I AG NOSIS o Classic triad described by Klippel and T renaunay in 1 900 consists

of capillary malformations, varicose veins or venous malformations, and osseomuscular linlb h pertrophy .

o Cuta neous appearance of c�pillary malformations (previously referred

to as a port- \v;n

stain ) that a re usuall

present

at or soon

after birth . Limb hypertrophy involve both bone and soft

tis

u , and in

rare circumsta.nces involves a truncal locati n . 6 A lthough it ma

'

F I G U RE 4 1 -1 C har a cte r i sti c KTS appe a ra nce of u n i lat era l capilla ry malformations (formerly known as port-wine stain) with va ricose veins and a ss oc i a t ed limb hypertrophy. (Photograph courtesy of Steven M. Dean , DO, FACP, RPVI.)

KLI P P E L-TR ENAU NAY SYN D RO M E

PART S N O N -ATH E ROSCLEROTI C D ISORDERS

b e evident early, limb hypertrophy i s often recognized after the affected individual begins walking. •

Lymphatic abnormalities can often be present and range from hypoplasia to aplasia . Lymphedema can exacerbate limb hypertro­ phy and venous congestion .

•

A related syndrome , Parkes- Weber syndrome (PWS) , is often confused with KTS, and is characterized by the presence of arterio­ venous fistulae or arteriovenous malformations in addition to the constellation of clinical findings classically described with KTS.

•

Diagnosis is made clinically, with the presence of at least two of the three main features.

CLI N I CAL FEATU RES •

Capillary malformations-The most common clinical findings are typically present at birth and have a flat, patchy appearance with a red to purple color . When found in the truncal location, there is generally a sharp border that fails to cross the midline (Figure 4 1 -2) . They may or may not blanch with pressure, and are most frequendy seen on the same extremity affected with hyper­ trophy. Moreover, although most commonly at the superficial level , these malformations can also be seen in the subcutaneous tissues. 7 While some of these lesions do progress , and are especially

FIGURE 41 -2 Truncal ca p i l l a ry ma lformations with typical red-purple, flat appeara n ce that fa i l to cross m i d l i n e . (Photograph courtesy of

Steven M. Dean, DO, FACP, RPVI.)

aggravated during puberty and pregnancy, others may fade with capillary thrombosis . 6 •

Limb hypertrophy, the least common clinical finding, occurs in approximately 67% of patients with KTS. The lower extremities are predominantly affected, with the upper extremities accounting for only about 5% of cases6 (Figures 4 1 -3 and 4 1 -4) . Although hypertrophy of the affected limb is the classic finding with KTS, there are reports of both equal or shorter limb size on the affected side. In addition, the bony overgrowth

can

involve one or more

bones on the affected extremity, and can be seen in conjunction with syndactyly and polydactyly. 8 The limb discrepancy is usually greater than 2 em and accompanied by soft tissue and muscle hypertrophy as well as increased skin thickness that may be due in

part to lymphatic involvement . Although growth rate is unpredict­ able , parents can be reassured that the syndrome is not character­ ized by progressive involvement of additional limbs . Venous malformations-Patients characteristically present with painful, large , and extensive varicosities. There are variable manifestations of venous involvement including complete agen­ esis of the deep venous system , hypoplasia, aneurysmal dilata­ tion , venous reflux , and persistence of embryonic structures. Up to 80% of patients will have a persistent lateral marginal

vein , also known as the "vein of Servelle . " 6 This vein originates in the lateral foot and courses proximally along the lateral leg for a variable distance , draining most commonly into either the internal iliac vein or a branch of the profunda femoris vein . It is usually thick walled, superficial , and incompetent along its entire length . Persistent sciatic veins can provide a main source of outflow in patients with a hypoplastic deep syste m . The venous component of KTS can also involve the intra-abdominal organ s . Manifestations vary and include rectal bleeding, hema­ turia, and esophageal variceal bleeding . Cerebrospinal involve­ ment, though extremely rare , can also occur .

FIGURE 41 -3 Lower extremity KTS with capillary malformation, as well as underlying pedal lymphovenous malformation via magnetic resonance imaging (M Rl). (Photograph courtesy of Steven M. Dean, DO, FACP,

RPVI )

1 69

1 70

PART S N O N -ATH E ROSCLEROTIC D ISORD E RS

C HAPTE R 4 1

D I F FER ENTIAL D IAG NOSIS o Most commonly confused with PWS .

o The presence of a high-flow arteriovenous fistula in addition to a

capillary malformation with limb hypertrophy characterizes PWS.

o Although low-flow arteriovenous malformations have recently been

described in patients with KTS using advanced imaging techniques, the presence of an easily palpable thrill or prominent pulsation portends a more serious clinical course associated \vith PWS .

o While KTS is often managed nonoperatively, \vith control of

symptoms using graclient compression and management of associated lymphedema, patients with PWS can require frequent interventions due to the high-flow nature of their malformations .

MANAG E M E NT o Capillary malformations-Generally, do not require any specific

treatment as they rarely become symptomatic. If bleeding or ulceration occurs and surgical excision is undertaken, wound 6 complications occur at higher rates. Pulse dye lasers can be used to decrease the appearance of capillary malformations but may require multiple treatments for optimal results .

o Limb hypertrophy-If the limb cliscrepancy is less than 2

em ,

treatment consists o f a contralateral shoe insert . Discrepancies of greater than 2 em can result in significant gait disturbances that merit surgery. Epiphysiodesis is undertaken to prevent tl1e irreversible sequelae of limb discrepancy including permanent gait disturbance and scoliosis .

o Venous malformations-T h e initial treatment of varicosities

associated with KTS is leg elevation and gradient compression stocldngs . If symptoms cannot be alleviated with these meas­ ures , then both sclerotherapy and operative resection or abla­ tion of the affected veins can be safely undertaken . Prior to any intervention , imaging to delineate the presence or absence of the deep system in conjunction with the presence of any persist­ ent or anomalous embryonic structures is paramount. Duplex ultrasound , magnetic resonance imaging (MRI) , and venography aid in planning any intervention. Surgery is reserved for highly symptomatic patients . Only after confirmation of adequate outflow veins should ligation be considered. Stripping of the lateral marginal vein as well as selective ligation of varicosities can be performed with good results . 8 Most authors advocate use of a tourniquet during surgery . Endovenous ablation including radio frequency and laser has been reported in selected case s . Sclerotherapy is a low-risk treatment that can be u s e d either alone or in conjunction with these other modalities to treat symptomatic varicosities in patients with KTS .

o Lymphedema-The goal of lymphedema management with KTS

mimics lymphedema management in general , and is centered on control of local manifestations and prevention of infections . Manual lymphatic massage , graduated compression stockings , intermittent pneumatic compression , leg elevation , and atten­ tion to skin hygiene all play a role in the management of lymph­ edema. Operative debulking procedures can be undertaken in severe case s , and lymphatic reconstructions have been reported in some centers .

FIGURE 41 -4 KTS affecting the u p p e r extremity with capi l l a ry

m a lformation extending dista l ly to involve the hand and digits. (Photograph courtesy of Steven M. Dean, DO, FACP, RPV/.)

KLIPPEL-TR E NAU NAY SYN DROM E

FOLLOW-U P

3 . Servelle M . Klippel and Trenaw1ay syndrome : 7 6 operated cases.

• Patient follow-up i s cLctated b y the severity o f symptoms and inter­ ventions t hat are planned . Patients who e

PART 5 N O N -ATH E ROSC LE R OTI C D I SORD E R S

mptoms are oontrol led

with oompres ion alone are reassured, as most patients with KT have a benign ourse .

Ann

Sura . Mar 1 9 5 ; 20 I ( 3 ): 365 - 37 3 .

4 . Bourde

. C lassification d es syndromes de Klippel-Trenaunay

de Parkes- Weber d 'a pres les donees angiographiques . Ann Radio/

(Paru). 1 974; 1 7(2 ) : 1 5 3 - 1 60 .

5 . Basken;lle P A , Ackroyd J , Browse Klippel-Trenaunay s mdrom e . Ann

PAT I E NT RESO U RCES

Nov 1 98 5 ; 202 ( 5 ) :

624-6 2 7 .

• htt p : / / k- t . org/

• htt p : / / www . ninds . nih .god disorders /kllppel_trenaunay /klippel_trenaunay .hbn

• http: 1 / gbr. nl m . nih .gov /concLtion / ldippel -trenaunay -syndrome

6 . Capraro

P,

Fisher J , Hammond D C , Grossman JA. Klippel ­

T renaunay syndrome .

2 0 5 2 -2060. 7. Gloviczlci P, Hollier

Plan

Reconsrr

Sura .

L, Telander R, et a! .

May 2002; I 09(6):

urgical implications of

K lippel and Trenaunay syndrome . Ann Sura. Mar 1 98 3 ; 1 97( 3) :

3 5 3 - 362 .

R E F E R E NCES

8 . G loviczk.i P, Driscoll OJ . Klippei-Trenaunay syndrome: current

I . Berry A, Peterson C , Mize W , et a l . Kl ippel -Trenaunay syndrome . An1 ) Med Gener. 1 99 ;79(4) : 3 1 9- 32 6 .

2 . Lorda- an chez I , Prieto L, Rodriguez- Pinill a

L . The etiology o f the

S ura .

E, Martinez- Frias ML.

Ina-eased parental age and number of pregnancies in K lippel­

Trenaunay- Weber syndrome. Ann Hum Gena. 1 998 ; 6 2 ( pt 3): 2 3 5 - 2 39.

rnanag men t .

Phleboloav . 2007 ; 2 2(6): 29 1 -298.

171

PART 5 N O N -ATH E ROSCLE ROTI C D I SO R D E R S

1 72

CHAPTER 42

42 CO N G E N I TAL

ARTE R I OV E N O U S MALFO R M AT I O N S

James Laredo, M D , PhD Byung Boong Lee , M D, P h D

PATI ENT STO RY A 3 5 -year-old Caucasian man with a pulsating painful flanlc mass was referred for further e\·aluation and treatment . The mass had been

present for over 1 5 years and had enlarged over the past year along the right lower back and fla.nl in an area underlying a birth mark (Figure 42- 1 . Examination confinned a I S - cm tender mas with

o,·erlying erythematous skin changes. A thrill wa palpable over the mass, and a cont illuous brujt was preseut on auscultation .

The lesion was confirmed to be a localized arteriovenous malfor­

mation ( AVM) w ith mmwal in,·olvement of the surroundmg tissues a demonstrated on abdominal computed tomography (Cl) ·cannmg with angiography including three-rumen ional ( l=igure 42- 2

( 3D)

reconstruction

.

onventional angiography ( Figure

42 - 3 )

confinned the presence

of an extensive A VM lesion with multiple feedmg art ries . The nus­

sively rulated venous outflow due to the fistulous lesion pi'Odudng a hemodynamicaUy ad,·an

d condition.

Direct pw1ct u re , transvenous embolization utiJjzing 0 . 0 3 5 Bentsen wires was performed preoperatively for subse q uent open resection of the lesion to red uce intraoperative bleeding.

om­

p l e te excision of the lesion filled with the coils was pe rformed sa.fel · ( F i gure 42-4) leaving a mill er residual lesion that was treated w i th conventional sclerotherapy F igure 42 - 5 ) . Follow-up

CT angiography and 3D reconstruction demonstrated an exceUent resul t ( Figure 4 2 - 6 ) .

E P I D E M I O LOGY

Congenital Vascu l a r M a lformation (Arte riovenous M a lformation Subtype) • Congenital vascular malformations ( CVMs are inborn vascular

defects that present at birth and may become clinically evident later in life .

•

The AVM

is the least

common

all dirucaUy sigruli ant •

VM, representing I 00/o to 1 5% of

C Ms .

The majority o f CVMs ( 5%-90%) a re either a venous malforma­

tion (VM) 1 or a lymphatic malformation ( LM ) .

2

Hema ngioma • The neonatal or infantile hemangioma is n o t a vascular m alforma­ tion but a vascular tun1or.

•

•

Hemangioma is a vascular tumor that originates from endothelial cells and is present in the early neonatal period . Hemangiomas occur in approximat ly

I%

to 2% and I 0° 'o of white

infant at birth wd u age I yeu, respectively.

F I G U R E 42 -1 An a rteri011 e nous malformation presenting as a pulsating back or flank m a ss. A 35-year-old Caucasian m an shown with a pulsating mass on his back and right flank. The mass had been present for over 1 5 years and had enlarged over the past yea r . The mass was assodated with p a i n and developed In an area underlying a birth mark.

CON G E N ITAL ARTE RIOVENOUS MALFORMATIONS

PART 5 N O N -ATH E R OSC LE R OTI C D I SOR D E R S

F I G U RE 42-2 Abdominal computed tomography (CT) demonstrating a n arteriovenous ma lformation (AVM) o f the right back and flank. The AVM lesion was confi ned to the superfidal tissues. Conventional CT sca n n i n g with i ntravenous oontrast (A) with th ree-dimensional (3D) reconstruction (B) was performed.

F I G URE 42-3 Angiography of an arteriovenous malformation (AVM) of the right back and flank. Note the extensive AVM lesion with multipl e feeding a rteries and massi vely di lated venous outflow.

1 73

PART S N O N -ATH E ROSCLEROTIC D ISORD E RS

1 74

•

Hemangiomas occur most commonly in white infants , with an i nci­ dence rate 1 0 to 1 2 times that of black and Asian infants.

•

Females are affected more often than m ales by a ratio of 3: 1 .

C HAPTE R 42

ETIOLOGY A N D PATHOPHYS IOLOGY Congenit a l Vascu l a r M a lformation (Arteriovenous M a lformation S u btype) •

CVMs are inborn vascular defects that present at birth and continue to grow at a rate proportional to the growth rate of the body .

•

The AVM is a congenital anomaly of the vascular system where the anatomic defect produces shunting of arterial. blood into the venous system .

•

The vast majority of the AVMs exist alone as i ndependent lesions, but occasionally occur with other CVM lesions.

•

AVM itsel f is further subgrouped into two different types, based on the embryological stage where developmental arrest has occurred : extratruncular and trun cular lesions. 3

The A VM type most often associated with unpredictable biological behavior is the extratruncular lesion that has unique, pathologic, embryological characteristics. Its prol iferative potential is derived from the mesenchymal cells of origin where developmental arrest has occurred at an earl ier stage of i n utero organogenesis. In contrast, the other type of A VM , the truncular form , does not have a mesenchymal cell origin or characteristics . Both forms are clas­ sified based on the Hamburg classification (Table 42 - 1 ) . I n addition to its u nique embryological characteristics, A VM exhibits complicated hemodynam ics. Both truncular and extratrun­ cular A VM lesions are associated with altered cardiovascular hemo­ dynamics occurring centrally, peripherally, and locally, i nvolving the arterial , venous , and lymphatic systems. These characteristics make the AVM the most hemodynam i cally complex type of CVM . The A VM also carries a high rate of progression and significant destructive potential of the primary lesion and its secondary effects. 4

FIGURE 42-4 Surgical resection of an a rteriovenous m a lformation (AVM) of the right back and flank. D i rect puncture, tra nsvenous emboli­ zation uti lizing 0.035 Bentsen wires was performed prior to open resec­ tion of the lesion to reduce i ntraope rative bleeding. The Bentsen wi res a re seen protruding from the lesion.

The AVM , especially the infiltrating extratruncular-type lesion , is the most dangerous, prim itive CVM that is associated with high rates of recurrence and potentially life-threatening and limb-threatening complications. Proper treatment of AVMs requires accurate diagnosis and precise classification of its embryological subtype (as either truncular or extratruncular) , and determ ination of its hemodynamic status.

Extratruncu l a r AVM Lesion This type of A VM develops during an early stage of embryogenesis . It persists as an embryonic tissue remnant fol l o w i ng developmen­ tal arrest that occurs d u ring the reticular stage of embryogenesis, prior to m aturation of the vascular system i n to vascular trunks . E xtratru ncu]ar lesions m ai ntain the origi nal reticular network, resul ting in AV shunting w i th n o capill ary check val ve syste m . The n i d u s of the l esion retain s its n o n fistulous condition ( i n contrast to the truncular lesion and the fistulous condition ) . T h e extratruncular lesion produces a significant hemodynami c alteration t o both the arte rial a n d venous syste m s , resulting i n shunting of arterial b l o o d into the v e n o u s system that a l s o h a s a m echankal i mpact on the surro u n d i ng tissues and organs.

FI GU R E 42-5 Postoperative angiography after surgical resection of a n a rteriove nous m a lformation (AVM) o f the right b a c k and flank. Note t h e minor residual lesion, w h i c h w a s treated with conventi onal sclerotherapy.

CO N G EN ITAL ARTE RIOVE N O U S MALFO R M ATI O N S

PART S N O N -ATH E ROSCLEROTI C D ISORDERS

FIGURE 42-6 Fol l ow-up abdominal computed tomography (CT) with three-dimensional (3D) reconstruction after combined endovascular and open surgical treatment of a n arteriovenous ma lformation (AVM) of the right back and flank. Note the exce llent result with no sign ificant resi dual lesion.

TABLE 42-1 H a m b u rg Classification of Congenital Vascu l a r M a lformations•· b

M a i n cl assificati o n based on its predom i n a nt vascu l a r component • Predo m i n a ntly a rterial defects • Predom i n a ntly venous defects • Predom i n a ntly a rteriovenous (AV) s h u nt i n g defects • Predo m i n a ntly lymphatic defects • Predom i n a ntly capi l l a ry m a lformation • Co m b i ned vascu l a r defects S u bclassification based on the e m b ryo l og i ca l stage of the defect • Extratru ncu l a r forms-deve lopmental arrest at the e a r l i er stages of e m b ryo n a l l ife • D iffuse, i nfi ltrating • Lim ited, l oca l ized • T runcu l a r forms-deve lopmenta l arrest at the l ater sta ges of e m b ryo n a l l ife • Ap lasia o r obstru ction • Hypop lasia, a p l asia, hyperp lasia • Sten osis, m e m brane, con g e n ital s p u r • D i l atation • Loca l ized (a n e u rysm) • D iffuse (ectasia) • B o t h extratru n c u l a r a n d tru n c u l a r fo rms may exist togeth e r i n the s a m e vascu l a r m a lformation; m ay be com b i n ed w i t h oth e r various ma lform ations (eg, ca p i l l a ry, a rteri a l , A V s h u nt i n g , ve n o u s , h e m o ly m p h atic, a n d/o r lym p h atic); a n d/or m a y exist with hemangioma. b B ased o n t h e consensus on t h e co n g e n it a l vasc u l a r m a lformation (CVM) cl assificati o n t h ro u g h the i nternati o n a l works h o p i n H a m b u rg, G e rma ny, 1 988, w h i c h w a s u p h e l d b y s u bsequently fo u n ded I nternati o n a l Soci ety for Vascu l a r A n o m a ly ( I S SVA) .

1 75

PART S N O N -ATH E ROSCLEROTIC D ISORD E RS

1 76

Furthermore, the lesion retains its ability to proliferate and its m esenchymal cell characteristics derived from mesodermal cells (angioblasts) . Lesions proli ferate in response to stimulation (eg, trauma, surgery, hormone , menarche , pregnancy) , resulting in an increase in their size, extent, and severity. Suboptimal treatment of extratruncular AVMs often results in lesion recurrence .

Truncu l a r AVM Lesion This type of A VM develops during a later stage of embryogenesis compared with the extratruncular form . It is the result of a defective arterial system that develops duri ng arterial trunk formation (truncal stage). The truncal stage of fetal development occurs after the reticular stage of vascular development and results in the formation of a mature lesion . Truncular A VMs no longer possesses mesenchymal cell (angioblast) characteristics and carry no risk of recurrence. However, these lesions often have more serious hemodynamic consequences to the vascular system compared with the extratruncular form . Truncu­ l ar A VMs persist as fistulous lesions with a direct connection between an artery and vein , with no defined nidus. This fistulous lesion produces significantly more serious hemodynamic problems: cardiac failure, arterial insufficiency (eg, gangrene) , and / or chronic venous insufficiency.

Hemangioma •

The hemangioma is a self-limited vascular tumor.

•

The growth cycle of a hemangioma is characterized by a prolifera­ tion phase of early rapid growth, followed by an i nvol utional phase of slow regression that usually occurs before the age of 5 to 1 0 years in the m ajority of cases .

•

Most hemangiomas are small and pose only minor cl inical problems before they involute and become clinically silent.

•

Up to 20% of hemangiomas require treatment. Indications for treatment incl ude aggressive growth , proximity to vital structures, or comp}jcations such as ulceration, bleeding, or h igh-output 5 cardiac failure.

DIAG NO S I S CVMs commonly occur a s mixed lesions presenting with AVM , VM, LM , and / or congenital malformation components . Therefore, the evaluation of any suspected A VM shoul.d proceed in a logical, step-wise manner, bearing in mind that the proposed A VM lesion may actually prove to be a m ixed CVM lesion . Diagnosis of a suspected AVM requires specific evaluation and confirmation as A V M . As a general r u l e , th e extent a n d severity of a n y C V M affecting the vascular system (anatomically and hemodynam ically) usually determines the type of cl inical manifestations observed . The history and physical examination should be followed by diagnostic imaging in order to distinguish the A VM from the various CVMs ( eg, duplex ultrasonography and magnetic resonance imaging [MRJ] ) . Most AVMs occur as single lesions. When A VMs present with additional elements derived from other CVMs (eg, VM, LM), they are classified as hemolymphatic malforma­ tion (HLMs) . These mixed lesions are considered to be an extended form of Khppel-Trenaunay syndrome (KTS) , which is also known as

C HAPTE R 42

Parkes-Weber syndrome (PWS) . In this situation, the initial prioril)' of investigation should be to confirm the presence of an A VM .

I MAG I N G Initial diagnostic i m aging should b e performed with a combination o f basel ine noninvasive t o less-i nvasive tests . More specific diagnostic procedures then follow for precise and detailed assessment of the AVM as a whole in order to define its embryological subtype as either extratruncular or truncular . The most common initial studies include •

Duplex ultrasonography (arterial and venous)

•

MRI with T l - and T2 -weighted imaging

•

CT angiography w ith contrast enhancement, with 3D CT reconstruction

•

Whole body blood pool scintigraphy (WBBPS)

•

Transarterial lung perfusion scintigraphy (TLPS)

The final diagnosis should be confirmed with an invasive study to further define the lesion and plan proper treatment. These studies include •

Selective and superselective angiography

•

Percutaneous direct puncture arteriography

•

Percutaneous direct punct=e phlebography

In addition to the assessment of the primary A VM les.ion , assess­ ment of the secondary impact on the nonvascular organ systems, especially to the m usculoskeletal system , is warranted . Early detec­ tion of vascular-bone syndrome with long bone length discrepancy is essential for appropriate management. Duplex ultrasonographic evaluation allows hemodynamic assess­ ment of the arterial and venous components involved with the AVM directly and indirectly. Duplex ultrasound is extremely valuable for the cl inical follow- up and remains the first-choice study among the various noninvasive tests available for CVM eval uation in addition to MRJ. MRI remains the major diagnostic study for the entire group of CVMs . MRJ allows assessment of lesion extent, severity , and anatomic relationship to the surrounding tissues or structures or organs. MRJ of the AVM lesion is usually followed up with CT angiography as confirmatory study. A mong the many newly developed noninvasive to less-invasive tests for the evaluation of CVMs, TLPS has a unique role in deter­ mining the degree of arteriovenous shunting that occurs w ithin an extremity AVM lesion . TLPS is able to detect and assess a micro-AV shunting lesion . These types of lesions are notoriously difficult to diagnose and detect with conventional arteriography and can be easily m issed . M icro-AVMs frequently occur in the combined form of CVM , the HLM (eg, PWS) . Misdiagnosis of this potentially limb­ threatening lesion can be avoided with TLPS alone . TLPS also pro­ vides quantitative measurement of the shunting status during therapy . TLPS may replace the substantial role of traditional arteriography as a follow -up assessment tool for extremity A VMs .

PART S N O N -ATH E ROSCLEROTI C DISORDERS

CO N G EN ITAL ARTE RIOVE N O U S MALFO R M ATI O N S

MANAGEM ENT New Con cept of M u ltidisci p l i n a ry Approach The A VM remains the most challenging malformation among the various CVMs due to its effects on the card iovascular system and its hemodynamic consequences . The clinical manifestations associated with the A VM are dependent on lesion location, where centrally and peripherally located lesions m ay produce cardiac failure and arte­

TABLE 42-2 I n d ications for Treatment of Arteri ovenous Ma lformations • • •

• •

rial or venous insufficiency , respectively . In addition, l ocal effects of A VMs may i ncl ude ulceration and gangrene. Indications for treat­ ment are listed in Table 42- 2 . Because o f the virulent n ature o f the AVM , all AVM lesions will eventually progress to the point of anatomic, physiol ogic, and hemo­ dynamic deterioration , resulting in a potentially life-threatening or limb-threatening lesion . Therefore , i deally, an early aggressive approach to all A VM lesions, with either macro- or m icro-AV shunt­

• •

• •

•

i ng, is favored .

H e m orrh age H i gh-output heart fa i l u re Seco n d a ry a rte ri a l isch e m i c com p l ications Secondary comp l i cations of chro n i c venous hypertension Lesions l o cated at l ife-th reate n i n g reg i o n (eg, proxi m ity to the a i rway), o r l ocated at the reg i o n th reate n i n g vita l fu n ctions ( e g , seeing, eati n g , hearing, o r breath i n g) Disab l i n g p a i n F u n cti o n a l i m p a i rment Cosmetica l ly severe deform ity Vascu l a r-bone syn drome Lesions l ocated at the reg i o n with potenti a l ly h i g h r i s k o f com p l i cation (eg, h e m a rthrosis)

A VM lesion recurrence rates following currently available conven­ tional (surgical or nonsurgical) treatment are m uch higher than those associated with other CVMs, making recurrence a hallmark of thi s CVM subtype. One o f the major reasons for such dismal results over the l ast several decades was a flawed approach to the A VM , combined with a l ack of a thorough understanding of the fundamental embryo­ l ogical characteristics among extratru ncular lesions . The old strategy where the goal was to shut off the feeding artery , willie leaving the n i dus of the lesion i ntact, resulted in stimulating a more aggressive neovascular response by the surviving primitive lesion , ultimately making the condition worse . Tills often ill planned and i mproper treatment strategy (eg, incom­ plete resectio n , l igation of the feeding arteries) only stimulates the A VM lesion to transform from a dormant state to a proliferative state, resulting i n massive growth with uncontrollable complications. Aggressive control of the lesion nidus is therefore essential in order to prevent recurren ce and eventual deterioration of the AVM lesion . A controlled, aggressive approach m ust be e xercised in the management of AVMs where the benefit of treatment must always exceed the risk of the associated morbidity . Only i n situations where treatment morbiclity and mortal ity are exceedi ngly h igh should a pall i ative approach be considered . In reality, not every AVM can be treated or should be treated by virtue of its simple existence . Although A VMs can be far more serious than any other CVMs and associ ated with dismal long-term outcomes, careful assessment of the treatment strategy and an accurate assessment of the ri sk-to-benefit ratio prior to initiating treatment are essential for success, along with clearly defined treatment goals and realistic expectations . A relatively new multiclisciplinary team approach to the treatment of A VMs resulted in significant improvements in the workup, cliagnosis, and treatment of these lesions. 6 Reductions in morbidity, mortality, and recurrence rates associated with the treatment of A VMs have been reported w i th the m ultidiscipli nary approach . A fully i n tegrated specialty team for advanced diagnosis and treatment of A VMs will pro­ vide the full spectrum of endovascular and surgical therapy. The multi­ disciplinary team approach 'vill allow maximum coordination among the various CVM-rel ated specialists (Table 42- 3 ) .

TABLE 42-3 M u ltidisci p l i n a ry Team for the Contemporary M a n age­

ment of the Arteri ovenous Ma lformation •

Vascu l a r Su rgery

•

Pediatric S u rgery

•

S u rg e ry

•

Pl astic a n d Reconstructive

•

Ortho p e d i c S u rg e ry

•

O ra l - M axi l l a ry-H ead a n d N eck Surgery

• •

N e u rosurgery Anesthesiology

•

Pathol ogy

•

C a rd i ovascu l a r M e d i c i n e

•

• •

Physical M e d i c i n e and Re h a b i l itat i o n G e n e ra l M e d i c i n e Pedi atrics

•

l nterventi o n a l R a d i o l ogy

•

N uc l e a r M e d i c i n e

•

• • •

D i a g n ostic Ra d i o l ogy Dermato l ogy N e u rol ogy Psyc h i atry

1 77

1 78

PART S N O N -ATH E ROSCLEROTIC D ISORD E RS Vascu l a r a n d Endovascu l a r Therapy Surgical resection has long been the gold standard for the treatment of AVMs despite high rates of complication , morbidity, and recurrence. Complete eradication of the nidus of A VM is required to achieve an effective cure . Surgical resection has long been the only means of eradicating the AVM nidus . Incomplete resection in many cases is unavoidable due to the prohibitively high morbidity associated with radical surgical therapy. The role of surgical resection changed w:itb the development of endovascular therapy over the last several years. Open surgical resec­ tion outcomes have significantly improved with the use of adj unctive endovascular therapy . Preoperative em bolo/ sclerotherapy bas been shown to improve the safety and efficacy of subsequent surgical resec­ tion, resulting in significantly reduced morbidity and mortality (eg, intraoperative bleeding) . Postoperative supplemental endovascular therapy has also been shown to be of benefit i n the surgical treatment of AVMs. Endovascular therapy with various embolization and scleroth e rapy modalities is a fully accepted th e rapeutic option in the treatment of AVM lesions. Endovascular therapy alone has been shown to be ben­ eficial in patients with prohibitively h igh surgical risks and in patients with surgically inaccessible lesions. It remains the treatment of choice for A VM lesions that extend beyond the deep fascia and involve mus­ cle , tendon , and bone-the diffuse infiltrating type of extratruncular form of AVMs . Precise delivery of the embolosderosants directly into the nidus of the A VM lesion ( extratruncular form) is required for successful endovascular therapy . The outdated approach where the goal of treat­ ment was embolization of the AVM feeding arteries is no longer via­ ble and should be condemned . A combination-type therapy approach where all three routes of del ivery (transarterial , trans venous, and direct puncture) are util ized in order to obliterate the AVM lesion nidus is most efficacious. Multisession endovascular therapy is preferred and allows admi n­ istration of the minimally effective volume of embolization and scle­ rotherapy agents during each session in order to reduce the risk of associated acute and chronic morbidity . The most commonly utilized treatment agents include absolute ethanol , onyx, N-butyl cyanoacr­ ylate (NBCA) , coils, and contour particles (eg, lvalon) .

Eth a n o l Scl erotherapy Absolute ethanol is associated with significant morbidity . To m ini­ m ize inj u ry to surrounding tissues, ethanol m ay be effectively dil uted to 60% when u sed to treat superficial AVMs that carry a high risk of skin necrosis and AVM lesions in d ose proxim ity to nerves . Administration of smaller vol umes . i n d ivided doses also minim izes the risk of surrounding tissue inj ury. The residual etha­ nol m ay be drained before removal of needles. lf locahzed swel l ing and tissue reaction is severe , light compression m ay be appl ied for 5 to 1 0 m i nutes after treatment. Direct compression of the vein draining the AVM during treatment may prevent early drainage during ethanol injection . Appropriate precautions for the potential risk of cardiopulmonary complications during ethanol sclerotherapy should be made with the m inimal possible amount of ethanol allowed ( 1 mg/kg of body weight in maximum per session) .

C HAPTE R 42

The importance of close monitoring of the cardiopulmonary-vascular system during the ethanol sclerotherapy cannot be overemphasized ; appropriate or immediate handling of the increased pulmonary pressure when the ethanol should reach to the pulmonary bed is absol utely mandatory. Pulmonary hypertension is a potentially fatal morbidit)' by chemical toxicity of the ethanol , and pulmonary spasm , can lead to cardiopul­ monary arrest. Special precaution to the increased risk of skin necrosis should be exercised via arterial puncture; do not allow the ethanol to reflux to the arterial side .

Coil a n d G l ue Embolotherapy A major liability of coil embolotherapy is it only exerts mechanical effects to block flow to induce thrombosis and does not have any direct effect on the endothelium to induce permanent damage; therefore, recanalization of the nidus will ultimately recur. Therefore , it is desirabl e to have further control of the nidus w ith additional permanent therapy via absol ute ethanol . Although the fi stulous AVM, which often requires coils, is a mostly truncular lesion with lack of evolutional potential . Before i njecting ethanol into a high-Row drai ning vein , coil embolization should be undertaken with one or more of the follow­ ing modalities : platinum spiral coil , tornado coil , and / or detachable coil(s) . Glue (NBCA) embol ization also should be considered with 30% to 50% concentration to minimize foreign body reaction, and its use should be l i m ited to surgical cand idates, if possible ; try to puncture as close as possible to the A V -connection nidus to m ake a gl ue cast from the nidus to proximal draining vein .

PATI ENT EDUCATION Patients with AVM and other CVMs can experience recurrence of their lesion . The likel ihood of a recurrence is directly related to the lesion type and extent of treatment. Patients are instructed to report any new l esions and symptoms to the doctor with regular foUow-up and ongoing monitoring and imaging as indicated .

FOLLOW- U P After treatment, patients should b e reassessed a t 3-month intervals o r more frequently, i f needed for the flrst year, and then a t yearly inter­ vals as appropriate , to monitor for symptoms associated with les.ion recurrence .

CONCLUS I O N A rteriovenous malformation is a potentially l i fe- and l imb-threatening lesion . This is especially true with the fistulous truncular form , due to its unique embryologic and hemodynamic characteristics . A multidisciplinary team and early, aggressive approach to the treatment of A VMs utilizing fully integrated endovascular and surgi­ cal therapy can achieve effective control of AVM lesions. As 'v:ith the treatment of any CVM , careful assessment of the risks and benefits associated with the AVM and proposed treatment should serve to guide subsequent therapy.

CON G E N ITAL ARTERIOVENOUS MALFORMATIONS

PROV I D E R R ESOURCES •

http : I /emedicine. medscap e . com / article / 1 1 60 1 67-oven;ew

•

htt p : / / www. phleboly mp hology . org/ 20 1 1 / I I / congenit..l ­ arteriovenous- malformations-what-are-the- pcry ectives/

PATIENT RESOU R200 cm/s + poststenotic turbulence Occl u sion

>3. 5

No flow can be detected in the ren a l artery

•psv, peak systo l i c vel ocity.

bTh e RAR can not b e used if the a o rtic PSV i s greater t h a n

1 00 c m / s o r l ess t h a n 4 0 cm/s .

1 83

1 84

PART 6 ARTE RIOVE N O US VISC E RAL D I S EAS E

C HAPTER 43

Angiogra phic Featu res o Catheter-based contrast angiography is the gold standard for the diagnosis of ARAS (Figure 43-4) .

o Intravascular ultrasound and translesional pressure gradients may aid in the assessment of the hemodynamic significance of a renal

artery stenosis.

o Some measurements acquired during angiography may assist in pre­ dicting the clinical benefit of renal artery revascularization includ­

ing baseline translesional pressure gradient, hyperemic systolic gradient of the stenosis of greater than or equal to

fractional-flow reserve, and renal frame count. 5

2 1 mm 1-lg,

D I F F E RENTIAL D IAG NOSIS o Fibromuscular dysplasia ( F M D ) is a noninflammatory , nonathero­ sclerotic arterial disease that most commonly occurs in women aged

20 to 60 years . The renal arterie s are affected in 75% of

patient s . 6 Differentiating features from ARAS are younger age at presentation and imaging characteristics including a more distal involvement and a typical , often beaded appearance of the renal artery (Figure 4 3 - S A) . When indicate d , percutaneous translumi­ nal angioplasty has proven to be successful in patients with FMD ( Figure 4 3 - S B) .

o T akayasu arteritis i s a large vessel arteritis . Diagnostic criteria

include young age, intermittent claudication of more than one extremity (classically the upper extremity), a decreased brachial

pulse, an interbrachial systolic blood pressure difference greater than I 0 mm 1-lg, a bruit over a subclavian artery, and imaging evidence of narrowing of the aorta or one of its large branches . Inflammatory markers are elevated in the active stages of disease . While typically affecting the aortic arch and the great vessels , T akayasu arteritis may also result in a mid-aortic variant that can manifest with renovascular

HTN.

o Segmental arterial mediolysis (SAM) is a noninflammatory, non­ atherosclerotic condition with a predilection to visceral arteries.

Lesions appear as strands of arterial narrowing on imaging (often CTA or MRA) . SAM may be asymptomatic and thus incidentally discovered or manifest with abdominal or flank pain.

MANAG E M E NT o Optimal pharmacologic management of atherosclerotic risk factors is the backbone of the treatment of ARAS. Tbis includes lipid­

lowering therapy, tobacco cessation , glycemic control in diabetes mellitus, antiplatelet medications (despite a lack of direct evidence of benefit in patients with ARAS) , and blood pressure control as per Joint

ational Committee VII goals . 7

o Current guidelines assigned a class lla recommendation for renal

artery revascularization for patients with hemodynamically signifi­ cant RAS and accelerated , resistant, or malignant hypertension,

HTN with an unexplained unilateral small kidney, and 1-!TN with intolerance to medication . Other indications included progressive chronic kidney disease over the past 3 to

6 months with bilateral

renal artery stenosis or a renal artery stenosis to a sohtary function­ ing kidney, unstable angina, recurrent unexplained congestive heart '

failure , or sudden unexplained pulmonary edema.

FIGURE 434 Arteriographic image of atherosclerotic ren a l artery stenosis (ARAS) i nvolvi ng left aorto-ostial segment.

ATH E ROSC LEROTI C R E NAL ARTERY STEN OSIS

PART 6 A RT E R I OV E N O U S V I S C E RA L D I S EASE

F I G U RES 43-5 Arteriographic images o f intimal fib ropla sia variant o f fib romuscu l a r dysplasia (FMD) in the mid/distal renal artery before (A)/after (B) percuta neous transluminal angioplasty.

•

The mechanism u n d

rlying ARA is often bulky atheromatous

ao rto re nal p l aqu , thus pe rcuta ne ou s transhnninal a ngio pl a sty does not prov id e durable pa ten cy . 8 E n do vascu l a r rena l artery stent

p l oc m n t ,

on th'

other hand, offers an acute procedural success

rate of up t o 98%. •

Pa t i e nt

lect ion fo r

is problematic, as all

inteo-venlion .

9· 10

ndova cular rena l artery

patients do not r

stent

pia

ment

spond favorably to

PATI E NT ED UCATION

ARA can often b e man aged medically; howe,·er , s o m e p a ti en ts may b e n e fi t from intervention. Pa t i en t s with dear i n d i ca t i o ns a s outl i ned earlier shoul d be offered en d o va scu l a r renal art e ry st nt p l a cem e nt . However, other pa t i e n t s shou ld be informed of th therapeutic o p t i ns a n d about cu rr e n t gaps i n the ab i li t y to pr diet r pon se t trea t m e nt of A RA , and a joint decision should be m a de regarding the method of treatment .

FOLLOW-U P •

FoUow -up o f patie n ts w i th asse ss me n t s and imag ing

ARA

sh

uld

incorporate bo th clinical

studies .

•

Renal a rtery d up l ex ul traso u nd is often the mo t u ful tool surveillance of ARA , whether revascularizat:ion ha been performed or n ot .

•

The natu ra l history o f A R A

for

is i nco m p l t ly und rstood ; there­

fore , surveillance protoco l s are often

dict a ted

by

local

pra t ic . 2 lf

s t e no s i s is 600/o in a renal artery s u p pl yi ng a nonnal - sized kidney

and th co n t ra la te ra l

nation is performed i n

nal art ry is wid I pa t nt , a repe at exami­ 6 months, and if w1changed , a nn ua l renal

1 85

PART 6 A RT E R I OVE N O U S V I SCE RA L D I S EASE

1 86

artery duplex ultrasonography i s reasonable. Factors that may prompt more frequent testing include deterioration in blood pres­ sure control , progression of renal dysfunction, or renal atrophy. •

There are currently no prospective studies evaluating the surveillance of stented renal arteries. The criteria for in-stent restenosis have been proposed (fable 4 3 - 2 ) . A reasonable protocol for poststent duplex ultrasonography is within 30 days of the procedure, after 6 and 1 2 months, and then annually if there is no suspicion of reste.nosis . PROV I D E R RESOURCE •

http: / /emedicine. medscape. com /arti le/ 24502 3-overview

CHAPTER 43

TABLE 43-2 Proposed Criteria for Stented Renal Arteries

Degree of Stenosi s

PSV"

0% to 59%

i­ cion is required for progressive vis era I ischemia leading to bowel necro is , as the degree of isd1emia cannot be reliably predicted by physical examination, laboratory testing, or current imaging tests.

imilarly, early operative intervention to resect gangrenous bowel rests on a high index of suspicion, along with a planned second­ look operation.

at

PAT I E NT E D U CATI O N

NOM I •

30%

2

CELIAC AXI S COM PRESSION SYN DROM E

PART 6 A RT E R I OV E N O U S V I SCE RA L D I S EASE

46 C E L I AC AX I S

CO M P R E S S I O N SYN D RO M E

Joseph Habi b, M D Bhagwan Satiani, MD, M BA, R PVI , FACS

PAT I E NT STORY A

2 2 - year-ol.d Caucasian woman

presented

to the

vasc

ul ar clinic with

intense postprandial abdominal pain that oCCWTed 20 to 30 min ut e s

after e a ting . he also r e layed a history of food fear al o ng with occa ­ sional nausea and ,·omiting. he had experienced about a 2 5 -lb weight loss over the last 6 m o nths . Her past m.,dical his to ry was not significant for any chronic illnesses or conditions induding absence of a psy­ chiatric or drug abuse history . be stated that he bad undergone an e x ten s iv e gasLTO in te s tin a l wo r l..-u p including u p pe r and low er endos­ copy and a right upper quadrant ultrasound, but no diagnooi · bad been e tabli.shed . On examination th patient appeared thin, in no acute di

tre

s. Her abdomen

was soft , no nte nd e r ,

nondistended .

On

auscultation, an pigastric bruit that increased \\;th expiration was

fou nd . A co m p uted

to mog r ap hi angiogram (CTA) was obtain e d

that compression of the celia axis b the median a rcua te ligam en t of the diaphragm and poststenotic d ila t a ti o n of the

d em o nstr a te d extrinsic

celiac artery ( Fi gure 46 - 1 ) .

Thi s patient

has th e typical

presentation o f celiac axis co mpression

sy ndro m e ( CAC ), also !mown as median arcuate ligament

s yndrome.

E PI D E M I O LOGY •

A

significant num ber of i ndi,-iduals ha ve narrowing of the celiac

axis

by the median

arcuate l igame nt

of the di a phragm , and full

expira ti o n seems to exacerbate this compression . It is still uncertain whether chronic intestinal ischemia can den· lo p from com pre io n of th celia

axis a lo n e .

• Usually occur in young, thin females. H

eve1·e com pre ·

n

that per ists during inspintion occurs in

approximately I% of patients. 3

• Between 1 3% and 50% of patients may have some degree of

com pres ion and "-' 70% with peak systol i c velocity (PSV) of 445 cms· 1 and e levated end d i asto l i c velocity (EDV) of 68 cms· 1 .

PART 6 ARTERIOVE N OUS VISCERAL D I S EAS E

FIGURE 47-2 Dup lex u ltrasound scan with color flow Doppler. Superior mesenteric artery (SMA) stenosis > 70% with peak systol i c velocity (PSV) o f 407 cms· 1 and elevated end diastolic velocity (EDV) of 46 cms· 1 .

intervention. Angiographic information is crucial when considering open revascularization options . o Oblique or lateral projections are required for viewing the

mesenteric arteries due to their ventral origin from the abdominal aorta (Figure 47 -6) .

o Other clues to significant CMI are presence of prominent compen­

satory collateral circulation between the CA and the SMA (arc of BUhler, arc of Barkow), and the SMA and the IMA (arc of Riolan or meandering mesenteric of Moskowitz) (Figure 47-7 A, B) .

TREATM ENT o Open surgical revascularization should be offered to patients with

symptomatic CMI who have acceptable risk profiles .

o Percutaneous revascularization with mesenteric angioplasty and/ or

stenting appears to be an attractive option, especially for patients

with CMI who are typically malnourished. However, due to its slightly higher symptomatic recurrence rate , endovascular therapy should be offered to high-risk, older patients who are otherwise unfit for open surgery .5

M esenteric Bypass o Open revascularization can be performed in antegrade or retro­

grade fashion.

o There is controversy about the number of mesenteric arteries that

should be revascularized . Primary treatment aim is to revascularize the most symptomatic mesenteric vascular bed and potential sec­ ondary optimization of other diseased arteries .

Anteg rade Bypass o Aortoceliac and / or SMA bypass can be achieved transabdomi­

nally via a midline laparotomy , or through bilateral subcostal incision s .

o The supraceliac aorta is accessed b y entering the lesser sac, mobi­

lization of the left liver lobe, and incising the left diaphragmatic

F I G U R E 47-3 D u p l ex u ltraso u n d scan with color flow Doppler.

I nfe rior m esenteric artery ( I M A) stenosis > 70% with peak systolic vel ocity (P SV) of 385 cms· 1 .

203

204

PA RT 6

ART E R I OVE N O U S V I S CE RA L D I S EASE

F I G URE 47-4A Computed tomogra p hic ( CT) scan with co ntrast o f a patient with heavy tobacco use and chronic mesenteric Ischemia (CM I); axial image of celiac artery (CA) stenosis a nd ostial calcific plaque (small wh i te arrow).

F I G U R E 47-4C Computed tomographic (CT) scan sagitta l i mage of celiac a rtery (CA) and superior mesenteric artery (SMA) stenosis, and ocduded Inferior mesenteric a rtery (IMA) (white arrow) with distal reconstiMion.

CHAPTER 47

FIGURE 47-4 8 Axial image of superior mesenteric artery (SMA) stenosis and ostial calci fi c plaque (sm a l l white arrow).

F I G U RE 47-40 Three-dimensional reconstruction computed tomo­ graphic angiogram (CTA) image showing heavy ostial calcification of the celiac artery (CA), superior mesenteric a rtery (SMA), and occluded nonvisu a l i zation of the i nferior mesenteric artery (I MA), right renal artery (RRA), left ren a l artery (LRA). Note paucity of collateralization.

CHRON I C M E S E NTERIC I S C H EMIA

PART 6 A RT E R I OVE N O U S V I S C E RA L D I S EA S E

crura . 11>e to mach i s retract d ge n t l y caudad m d esophagus t o the pati nt's left . "llu· e to four inches of th supmc l i a aort.l an be isol at ed for creati n of the proximal anastomo is. •

Within the lesser sac, the CA and its proximal branches (hepatic

and spleruc) are isolated for the celia.c anastomosis. Simultaneous MA bypass can

be achieved with bi furca ted prosthetic (Dacron or

polytetrailuoroethylene [ l!fFE I) grafts (Figure 4 7- S A , B or crea­ tion of pantaloon

great saphenous vein grafts .

are used if ther e is intraperitoneal con­ of bacteria from bowel co n t e n ts from either p erfo r a ti o n or intestinal resection of nonviable bowel.

• Nonsynthetic graft conduits taminati n

• The M A is i

!at d at th ba.s of the small bowel mesenl ry aft r

taking down th l igament f Tr itt . The byp

tunn led

r tropancreat ically ( Figur

to the MA is

limb

47- C) .

• Th p at i e n t is fully h parinized pri r to

damping and creation of the a n as t o mo

a

s.

rti or arterial

M a nni t ol

and furo­

emide may be given intn p r at i v ly C r renal prot el i n d uri ng

eros -damp. I mpaired cardia fun i n and h a "rcumferential pra el ia aorti lcih ati n a1•e contra.indicat ions is to ant gr�de m s nt ri byp.us. R trograde mes nt ri byp r�m m nd d r. r these pati nts.

temp rary aorti

Retrograde Bypass • A 3 - to 4-crn MA

gm nt is i

at the root of th small bowel m byp

can

n d ui t

lated at i m t proximal p rtion entery a de ribed earlier. The

be constructed using prostheti

nfiguralinn or a a l o ng

grafts either in a short­

F I G U RE 47-S A Co m p uted to m ographi c ccn scan with contrast o f a p at ient with chronic m ese n te ric ischemia (CMI) a n d re mote histo ry of aortobife moral byp as s . Sagittal i mage of severe superior mesenteric artery (S M A) ostia l stenosis and heavy "spi l l-over" aortic a therosc le­ ro t ic pl a q ue (black a rrow). The celiac artery (CA) is occluded (sm a l l wh ite arrow) and t h e i nfe rior mesenteric a rt e ry (I MA) is not v isu a l ized. Note scaphoid abdomen associated with weig h t loss . (Image courtesy of Henry Baele, MD.)

- loop ( Fig ure 47-9A , B).

• The most important technical aspect is avoidance of graft kinking when the bowel (and •

dosure of la parot om y . The distal anast o mos i s

MA) i returned to its anatomic position on

on th e M A is constructed first.

Proximal

anastomosis can be from the distal infrarenal aorta, or proximal right or l e ft common iliac arteries. Th· decision on choice of inflow depends

•

on the Ia · of t he graft and absence of ci rcumfe re ntia l calcification .

to th CA is p bl malic. I f th A rC

n

gat iv

Homan sign.Th re is

low r extr mity pu

no cya.nosis in the extremit:i

are int act and symm trical. Acute d

, and dist:ol p v nous

DV1) is suspected . Th quantitativ D-dim level is 1 200 mg/c!L, and a venous d upl ultrasound reveals acut DVT invoh·­ ing the mmon femoral (Figur 5 3- 1 ), femoral , and poplit eal veiru .

thrombosis

F I G U R E SJ-1 Transverse view of gray-scal e ultrasound imaging of the right upper th igh, depi cting the common femoral artery (CFA) and common femora l vein (CFV). Panel A shows the ultrasound image of the vascular structures without compression. Panel B shows a noncompressible CFV (arrow) due to acute thrombus when pressure is applied to the skin directly above the vei n by the operator

235

CHAPTER 53

236

E P I D E M IOLOGY o Venous thromboemboHc disease , including acute DVT and acute pulmonary emboHsm

(PE) , is the third most common cardiovascu­

lar disease in the United States .

o Approximately two-thirds of all venous thromboembolk events (VTE) are related to hospitalization .

o It is estimated that over a milHon cases of VTE are diagnosed each year in the United States alone .

o The absolute risk of DVT or PE in the population (all ages) is estimated at I % to 3% per year .

o The incidence of DVT or PE increases with age . The estimated age­ associated incidence of VTE increases approximately from

I case per I 00,000 person-years (I I I 00,000) in teenagers (estimated absolute risk of O . OO I % per year) to I / 1 00 person­ years over the age of 7 5 (estimated absolute risk of I % per year) . 1

ETIOLOGY A N D RISK FACTO RS o Acute DVT or PE is a multifactorial disease . Tbe greater the

number of risk factors present, the more Hkely a patient is to develop acute DVT .

o In addition to increasing risk with age, risk factors for DVT or PE can be classified as situational, acquired , or inherited (Table 5 3 - I ) . 1 -3

o Situational risk factors can be defined as transient clinical circum­

stances that increase the risk of VTE while they are present and for a short period (from a few weeks to a few months) after they have

resolved .

o Acquired risk factors consist of medical conditions that interfere

with normal hemostasis or plasma viscosity. Due to their relapsing or remitting cHnical course , these conditions tend to increase the

risk of VTE periodically . For example , a patient with a

TABLE 53-1 Risk Factors for Venous Thromboembolic Disease

Situational

Acquired

Inherited

•

S u rgery

•

Cancer

•

Factor V Lei den

•

Pro l onged i m mobi l ity (incl u d i n g l onghaul fl i g hts)

•

Anti p h ospho l i p i d a ntibodies

•

Hyperhomocyste i n e m i a

•

B o ny fractu res or l e g casts

•

Proth rom b i n gene G 202 1 0A m utation

Vascu l itis

•

Antithro m b i n deficiency

•

Pregn a n cy

•

Syste m i c l upus e rythematosus

•

Prote i n C deficiency

•

Ora l contraceptives

•

I nfl a m m atory bowel d i sease

•

Prote i n S deficiency

•

Ho rmone rep l a cement thera py

•

N e p h rotic syn drome

•

Factor VI I I excess

•

Adj uvant h o r m o n a l therapy for breast cancer

• •

Myeloprol iferative d isorders

•

Factor IX excess

Chemotherapy

•

M o n o c l o n a l g a m mopath ies

•

Factor XI excess

• •

Obesity

•

Hyperviscosity synd ro m es

•

•

Centra l venous catheters

•

Pa roxys m a l n octu rn a l h e m og l ob i n u ri a

Other p o i nt m utati ons in the genes that encode fo r factor V a n d pro­ thro m b i n genes

•

Heparin-ind u ced thrombocytopenia ( H ID

•

Factor VI I I excess

M ED I CAL MANAG E M ENT OF F E M O ROPOP LITEAL D E E P VENOUS T H R O M B O S I S

vasculitis (such a s granulomatosis with polyangiitis) bas a signifi­ cantly increased risk of VTE during a flare of disease activity, but the risk is reduced when the disease is in rerrtission. • Inherited risk factors represent congerutal thrombophilias, that

is, genetic mutations and polymorphisms that increase the risk of thrombosis by causing specific changes in the debcate balance of normal hemostasis that ultimately result in greater thrombin generation and cbrucal thrombosis .

237

• A concomitant, underlying acute DVT is present in as many as

30% of patients with a clirllcal diagnosis of acute superficial throm­ bophlebitis in the lower extremities .

•

• Venous duplex ultrasonography is the method of choice for objec­

tive diagnosis of an acute femoropopUteal DVT, with sensitivity and specificity greater than 95% to 99%. Duplex impUes the use of both gray-scale imaging ultrasound combined with analysis of blood

PATHOPHYS I O LOGY

flow by Doppler . The single most accurate and reUable sonographic criterion to diagnose acute DVT is lack of vein compressibility (Figure 5 3 - 1 , panel B) . If a vein is free of thrombus, it will become completely compressible when external pressure is applied directly over the vein (Figure 5 3 - 2 , panel B). This sonograpbic maneuver is performed and observed by interrogation of lower extremity veins using gray-scale ultrasound in transverse view (Figure 5 3 - 3 ) .

• Rudolph Virchow was a 24-year-old pathologist when he postu­

lated that thrombus formation was influenced by the presence of three conditions (triad) : venous stasis, vascular injury, and changes in the blood itself (ie, hypercoagulabiUty of blood) . Those condi­ tions, alone or in combination, were invariably present in patients who had DVT diagnosed during autopsy.

• Modern understanding of the multifactorial pathophysiology of

DVT suggests that, in any given individual, one or several cUrucal risk factors may cause one or more of the conditions postulated in Virchow triad, hence leading to abnormal thrombus formation . For example , a patient with cancer may have venous stasis (from direct tumor invasion of a vein or by extrinsic venous compression resulting in venous obstruction) , vascular injury (by direct invasion of a vein by tumor itself or metastasis) , and bypercoagulability due to cancer cell-mediated synthesis of prothrombotic proteins or by

increasing plasma tissue factor levels . • Most acute DVT results from thrombus formation that begins

in venous valves and/ or in calf veins, followed by cranial propagation . 4

•

Acute DVT is defined as proximal if it involves the veins at or above the knee level, that is , popbteal , femoral, common femoral , and/ or iliac veins . Acute DVT is defined as distal if it involves the calf veins (peroneal , posterior tibial , soleal , gastrocnemius­ Figure 5 3 - 3 ) .

• Approximately 40% t o 5 0% o f patients with symptomatic, proxi­

mal lower extremity acute DVT without any chest symptoms have evidence of (asymptomatic) PE by pulmonary angiography or ventilation-perfusion scintilography (V /Q scan) .5

• Of all patients with symptomatic acute PE, between 40% and

70% will have a concomitant acute DVT (that may be symptomatic or not) by venous duplex ultrasound of the lower extremities. Two-thirds of these DVTs will be proximal DVTs , while one-third will be isolated calf DVTs 6

DIAG NOSIS • Cllnical diagnosis of acute DVT is insensitive and nonspecific.5

• The severity of signs and symptoms does not necessarily correlate

with the extension or location of the acute DVT .

• The Homan sign was originally described as calf discomfort eUcited

by passive dorsiflexion of the ankle of a limb with suspected DVT .

It has poor sensitivity (mp le te blood co unt (CBC was within normal lim.its .

The p at i ent was diagnosed with cl ass i c stasis dermatitis, a condition com m onl y misdiagnosed as bi l atera l lower extremity cdlulitis .

E P I D E M I OLOGY • The p re val e nce is estimated to •

light female prep nd ranee

be grea ter than

I o/o of the popul ation . '

report e d . 1

Prevalen e increase with age .

o

st i m at e d pr v a l ence of 6%

SO ye ar

.2

to 7% in patients older tha n

ETIOLOGY AND PATHOPHYSIOLOGY ta si de rm a titis l i ke l y occurs secondary t o the chronic

inflam ma tion and microangiopathy that result from chroni venous insufficiency. 1 o

Disrup tion in

function of the venous v a l ve s ys t e m in the dee p and

perforating venous tributaries of the l egs causes backflow i nto the

F I G URE 60-1 Lower extremity edema and pretibial erythema consistent with stasis dermatitts. This is commonly misdiagnosed as bilateral lower extremity cellulitis.

superficial venous syste m , yielding venous h yp erten sion . '

•

Valvular dysfunction results from increas i ng age or can be the

result of specific events, including de ep vein thrombosis , o

pregnancy, surgery, or history of a lower e -xtr e m i ty inju ry . 1 of blood in the mi cro ­

Venous hypertension decreases the flow

vasculature and allows for an increase in the capiUary perm e abil­ ity, resu l t ing in the extravasation of erythrocytes and th e

of fl u id a nd pl asma protei n s i nt o to microangio p a thy . 1

•

passage

the tissu e , oonse9uently leading

Proteins , most co m monly fibrin, are deposited perh·ascularly , fo r m ing a h ·aline culT a nd inhibiting o x ygen diffus ion . 1 •2

•

Decreased blood flow causes an u pregu lat io n of interceUular

adh ion molecule- ! I AM- I ) and vascul ar cell adhesion mole­ •

cule (V AM- I ) , thus activating neutrophils aod macro phages. 1

.1

Activated ne utrophil re l ease inflam m ato ry mediators, &ee

radicals , and p r o teases , causing pericap i l la ry inflammation. 1

DIAG N O S I S

Cl inical Feat u res of Stasis Dermat iti s • Often first manifests as lower e.-.ctremit

edema .

caling, xerosis , and p ruritus are often pre sen t

distal medial calf ( F igure 60-2 . Less often, affect the lateral calf.

within the mid and similar m anifest a tions F I G URE 60-2 Scaling, xerosis, and pruritus a re often present In the medial lower extremity. (Photograph courtesy of Dr. Steven M. Dean.)

C HAPTER 60

276

•

Hemosiderin deposition often leads to the classic brown d iscolora­ tion seen in stasis dermatitis (Figure 60- 3 ) .

•

Erythema, scaling, and diff u se dermatitis can result i n a weepy appearance with oozing and crusting (Figure 60-4) .

•

•

When stasis dermatitis is present chronically, fibrosis of the underlying tissues progresses to lipodermatoscl erosis . A constricting band forms around the distal cal f causing the classic "inverted wine bottle appearance" of the lower extremity (Figure 60-5 ) . Chronic stasis dermatitis ca n also present with violaceous papules, plaques, and nodules mimicking Kaposi sarcoma. This entity is known as pseudo-Kaposi sarcoma or acroangiodermatitis. 2

Typica l Distribution •

•

•

Most evident in the medial supramall eolar region, the site of major communicating veins and th e refore the region where rnicroangio­ 1 pathy is most intense. Patches of dermatitis occur commonly over dilated varicose veins . 1 Can occur unilaterall y when the lower extremity has been inj u red via trauma or surgery.

La boratory Stud ies •

•

•

•

Clinical diagnosis based on appearance and history of chronic venous insufftciency .

FIGURE 60-3 Hemosiderin deposition often leads to the cl assic b rown d iscoloration seen in stasis dermatitis. (Photograph courtesy of

Dr. Steven M. Dean.)

If suspecting cellulitis o r sepsis, appropriate blood work i s warranted (CBC with differential , blood cultures , wound cultures) . If stasis dermatitis is secondary to venous thrombosis, cons.ider an evaluation for an underlying hypercoagulable state. A venous duplex ultrasound is criti.cal in order to assess for acute and / or remote deep venous thrombosis. Importantly, this exam i­ nation can identify the anatomic location of venous reflux, which may facilitate subsequent corrective intervention .

CO M P LICATIONS •

•

•

Allergic contact dermatitis affects 58% to 86% of patients with venous ulcers. 3 o This can be secondary to the use of topical therapies, including creams, antibiotics, and antiseptics. 3 Topical antibiotics such as neomycin and bacitracin were the culprits in superi mposed aller­ gic contact dermatitis in a sensitized patient (Figure 60-6) . Stasis dermatitis can lead to secondary dissemination , with patches of eczema arising in symmetric and distant distribution patterns . 1 This is known as autosensitization dermatitis or ld reaction . 1 A coexisting irritant dermatitis secondary to wound or venous ulcer secretions can occur . 1

DIFFER ENTIAL D IAG NOSIS •

Asteatotic eczema--also known as eczema craquele. This condi tion involves xerotic skin with superficial cracks resembling a "dried river bed . "

FIGURE 60-4 Erythema, sca ling, and diffuse derm atitis can lead to a weepy appearance with oozing and crusting. (Photograph courtesy

of Dr. Steven M. Dean.)

STAS I S D E RMATIT I S

•

Atopic dermatitis-typically the patient has a history significant for eczema, asthma, or seasonal allergies .

•

Allergic contact dermatitis-associated with stasis dermatitis since venous leg ulcer patients often have contact sensitivities to medica­ m ents used in the management of the ulcers. CommonJy reported allergens include topical antibiotics, fragrances, Balsam of Peru , 3 lanolin alcohol , topical corticosteroids, and rubber accelerators .

•

•

•

Irritant contact dermatitis--often due to exudate from venous 1 ulcers that cause inflammation and irritation of the sk:in . Pretibial myxedema-i nfiltrative plaques of the pretibial region often associated with Graves disease. Cellulitis-often unilateral . May be accompanied by constitutional symptoms such as fever. White blood cell count may be elevated . Stasis dermatitis can be seen along with cellulitis (Figure 60-7) .

•

Psoriasis-sharply demarcated erythematous plaques with charac­ teristic scale . Patient t)'Pically presents with plaques at com mon sites such as scalp, extensor surfaces of extrem ities, umbilicus, and lower back .

•

Mycosis fungoides-sk:in biopsy for histologic assessment can be helpful in the appropriate clinical situation .

•

277

Deep venous thrombosis.

MANAGE M ENT •

•

Underlying varicosities and saphenous vein insufficiency should be addressed , w:ith treatment aimed at improving venous return and decreasing edema. o Compression therapy, incl u d ing Unna boots and various other pressure wraps or compression stockings. o Leg elevation above heart level several times per day. o Lifestyle changes, including exercise and weight loss. o Surgical l igation or thermal ablation of incompetent communi­ cating veins may be indicated if stasis dermatitis persists despite correcting saphenous vein reflux.

FIGURE 60-5 The classic " i nverted wine bottle appearance" of l i podermatosclerosis. (Photograph courtesy of Dr. Steven M. Dean.)

The eczematous dermatitis and pruritic component can be tl"eated 14 with mid-potency topical corticosteroids. '

PATIENT ED UCATION Patients should be educated about the underlying cause of stasis dermatitis, as well as the necessary treatments for the underlying venous insufficiency. •

Encourage daily use of compression devices as prescribed .

FO LLOW- U P •

•

Patients with long-standing b u t stable stasis dermatitis can manage the condition on an outpatient basis. A cute exacerbations should be closely observed by a physician to prevent the formation of nonheal i ng ulcers and infection .

FIGURE 60-6 Stasis dermatitis along with a l l ergic contact dermatitis in a patient previously sensitized to topical neo myci n . ( Photograph

courtesy of Dr. Steven M. Dean.)

CHAPTER (:JJ

278

P ROVI D E R R ESOURCES • •

http: / / emed icme . medscape . com / artide / 1 0 4

1 3-overvie'

http: I I dermnetnz. org/ denn a titis/ venous-eczema . html

.a

m / d rmatitis/ susisdcrnnt itis px

• http: / / w''

v.

l 2) for two conse cu t i ve days.

H E PA R I N- A N D LOW-M OLECU LAR WE I G HT H E PAR I N-I N DUCED S K I N N EC R OS I S E P I DE M IO LOGY •

e vere anticoagulant-induced skin r actions that can develop fol­ lowing subcutaneous injection of hepa ri n (U FH) or any of the

low-molecular weight heparin (LMWH) preparations. I- S I t can also

14 7

•

be e n fo l l o w i ng int ravenous UFH administ ration. • •

More commonly seen in patients receiving U FH. than LMWH. It bas been reported in most of the LMWH preparations ind ud­

ing dalteparin, enoxaparin, tinzaparin, teddparin , certopa rin, and nadroparin _4

•

I n ci de nce likely under

timated due to either under-recognition

•

An u nco mmon mani�

a t ion of H IT ,

•

Most p a t i nt· ( 50%-75%) w ill not devel p thrombocytop nia

and / or under-reporti ng.

d

pite the pr

antibodi •

n e

f b parin-d pend

t a n t ibod i

are p rese n t they are a mark r for H IT . 2 ·4•

1

. However , if

Only 1 0% to 20% of patients who develop HIT ant ibodies du ring subcutaneous administration of UFH or L M W H \viii de v d op

skin le ion s . 2

FIGURE 62- 1 This patient, described in the patient story, developed hepa rin-I nduced thrombocytopenia (H ID fol lowing unfra ctionated hepari n (UFH) admi nis t ra tio n for an acute pulmonary em bol is m . She was started on warfarin prior to correction of her platelet co unt and developed violaceous discoloration of bo th the breasts that prog ressed to full-th ickness ski n necrosi s and eve ntual skin grafting. (With perm ission from the Archives of Internal Medicine. 2004; 1 64:66-70.)

CHAPTER 62

284

ETIOLOGY AN D PATHOPHYSIOLOGY o Generally develops in conjunction with I-ITT, an immunologic reac­

tion triggered by the binding of heparin to PF 4 resulting in the formation of heparin-dependent antibodies that can cause thrombo­ 1 cytopenia and venous and/ or arterial thrombosis. • 2 •4•5

o Patients often have other comorbid conditions such as hyperten­

sion, diabetes mellitus and obesity, underlying malignancy, or a 1 connective tissue disease.

DIAGNOSIS C l inical Featu res o Consider in any patient who develops a skin reaction while

receiving subcutaneous UFH or LMWH or intravenous UFH (Figure 6 2 - 2 ) .

o Lesions appear suddenly and vary from tender, local erythematous

plaques to painful sharply delineated necrotic lesions with a cuff of erythema or \�olaceous discoloration surrounding the necrosis 5 •6 (Figure 6 2 - 3 ) . 2 •

FIGURE 62-2 Heparin-ind uced skin necrosis ( H I S N ) seen i n a n elderly fem a l e at the site of a su bcutaneous heparin i njecti on. N ote the erythematous border surrounding the centra l a rea of ischemia.

o Lesions may be multiple .

o Skin manifestations have also been reported with other unusual

presentations of HlT such as adrenal hemorrhagic infarction and severe acute anaphylactoid reaction. 2 • 5

Typica l Distribution o Most commonly found at the injection site , although they may be

found at distal sites, especially regions with extensive subcutaneous fat tissue such as the buttocks, t:llighs , breasts, or abdomen . 4

LABORATORY STU D I ES o Thrombocytopenia, defined as a platelet count less than

I SO X I 09 / L

o r 50% o r greater drop from preadrninistration levels i n patients recei�ng UFH or LMWH who are considered at risk for HIT .

o Obtain heparin antibodies, an immunologic study (PF 4-heparin

immunoassay) , and / or a functional test (serotonin release assay) .

o Majority of patients with heparin-induced skin necrosis ( HJSN) will

have serologic confirmation of I-ITT .

o Skin biopsy demonstrates extensive focal epidermal necrosis with

marked neutrophil infiltration and extensive fibrin deposition vvithin the capillaries and venules of the dermis '�thout any 1 e�dence of vasculitis .

DIFFER ENTIAL D IAG NOSIS o

A number of conditions can mimic HIS

(Table 62-1 ) . 7

o Important to recognize that HIS

is similar in appearance to warfarin-induced skin necrosis (WISN) , although several factors 3 help differentiate these two : o Timing-HIS generally develops between days 5 and 1 4, while WISN classically develops earlier between days 3 and 7 .

o

o

There i s n o immunologic basis for WISN . WISN does not induce thrombocytopenia, platelet aggregation, or arteriovenous thrombosis.

FIGURE 62-3 An exa mple of a heparin-induced skin reaction that did not resu lt i n necrosis. Although most com monly fou n d at the subcuta neous heparin i nj ection site, these m u ltiple purpuric papules were fou n d in the neck region of a patient receiving i ntravenous hepa ri n .

H EPAR I N -, LOW-MO LECU LAR W E I G HT H E PA R I N-, AN D WAR FARI N - I N DU C E D SKI N N ECRO S I S

MANAGE M ENT

TABLE 62-1 . The Differential Diagnosis for Anticoa g u l a nt-I n duced

o Instruct patients, nurses, and physicians to look for early cutane­ ous signs of intolerance (tender areas of erythema at the UFH or LMWH injection site) or anywhere on the skin.

o Particular attention should be made to any patient complaining of pain or erythema at UFH or LMWH injection sites.

o Immediate discontinuation of UFI-1 or LMWH is mandatory once the diagnosis of HIS

285

is suspecte d . Converting to LMWH for

patients receiving UFH is not recommended because of cross­ 1 2 reactivity benveen these hvo anticoagulants . •

o Treatment using a direct thrombin inhibitor (DTI) such as lep­

irudin, bivalirudin, or argatroban or the low-molecular weight heparinoid , danaparoid (no longer available

in the United States) is

Skin Reactions 7

Wa rfa r i n - i n d uced ski n necrosis (WI S N ) H e p a r i n - i n d u ced skin necrosis (H I S N) Low-m o l e cu l a r weight hepari n (LMWH) s k i n necrosis P u rp u ra fu l m i n a ns Pyoderma g a n g renosum Ecthyma g a n g renosum N ecrotiz i n g fasciitis I n fective endoca rd itis

recommended .

Athero e m b o l i s m

Avoid warfarin if the patient has HIT, until the platelet count has 9 recovered to 1 5 0 X I 0 / L or greater. 8

Pressu re u l ce rs

o Small necrotic lesions should be treated conservatively \vith topical

Calciphylaxis

agents. Large necrotic lesions usually require surgical debridement

Disse m i n ated i ntravascu l a r coa g u l ation ( D I C)

with or without skin grafting.

Cryofibrinogenemia Anti phosp h o l i p i d syndrome System i c vascu l itis (cryog l o b u l i ne m i a , system i c l u pus e rythematosus)

E P I D E M I OLOGY o Reported in patients taking vitamin K antagonists including any of the following preparations: warfarin , bishydroxycoumarin ,

phenprocournon, and acenocoumarol .

o Estimated to occur in 0 . 0 1 % to 0 . 1 % of patients taking warfarin .

o Women are four times more likely to develop than men .

9

o Predominantly seen in areas of increased subcutaneous fat including the breasts , buttocks, and thighs.

1 0• 1 1

ETIOLOGY A N D PATHOPHYS I O LOGY o Warfarin can rarely cause WISN during its initial period of admin­ istration by decreasing the functional activity and synthesis of the vitamin K-dependent proteins II, VII , IX, and X and the natural anticoagulant proteins C and S. Depletion of protein C (which has a shorter half-life than the procoagulant factors I I , IX, and X) may result in a transient hypercoagulable state that can cause thrombosis 1 2 14 of cutaneous vessels and WISN . "

o Patients with protein C deficiency are at greater risk of developing WISN , although it has been reported

in patients \·vith a lupus anti­

coagulant, antithrombin, and protein S deficiencies and in patients who receive large loading doses of warfarin .

o Also associated in HIT, especially when warfarin has been initiated

before the platelet count has recovered and in patients who develop 1 14 a supratherapeutic INR during induction of warfarin therapy. 3 •

o Most commonly develops in individuals being treated for a deep vein thrombosis or pulmonary embolism .

CHAPTER 62

286

DIAG N OS I S

•

Protcin C concentrates have been used in patients with protein deficiency. Pre,·ention of the progression of the kin lesions and/ or regression has been reported . 1 ·9• 1 2

•

lf HIT is diagnosed, a DTI should

C l i nical Featu res •

Mainly aft cts obese, middle-aged wom n.

•

Initial presentation consists of a painful pressure- like sensation or paresthesias follow d by a petechial rash that progre s to an ery­ thematous macule and blue-bla k ecchymosis . This rapidly evolves to hemorrhagic bullae and full -thickness skin necrosis. 9 ' 1 1

• Characteristically •

presents as single lesions o=...rring wulaterall y ; howe\'er, up to one-third of patients will have multiple lesions. 9• 1 4

malJ necrotic lesions are usually managed conservati,·ely with topical agents. Howe\'er, large necrotic lesions may require surgi ­ cal debridement with or without skin grafti ng, and reconstructive surgery or amputation may be necessary .

Usually occurs within the first 3 to 7 days of initiating warfarin therapy .

Typical Distribution •

•

WI N typically 0=.1r s in areas of extensive subcutaneous fat such as the breasts, buttocks , abdomen , and thighs. It may be seen in other areas su ch as the face , arms, calves, back , and on the penis. 9 , 1 2 In th ett ing of H IT , may also present as venous limb gangren , a cond ition defin d as progre sion f a deep vei n thrombo is t limb necro is d spite palpable or Doppl r-identifiable arterial pul e . 1 3

PAT I E NT E D U CAT I O N •

Evaluate protein C activity levels on the acute e'•ent ha subsided and / or the patient is off warfarin . May also consid r checking for the presence of a lupus anticoagulant , protcin , or antithrombin levels if protein C levels are normal . Determine the I R, check the platelet count, and measure anti-PF 4 or heparin antibodies especially if the I N R is supra therapeutic.

•

Biopsies display cutaneous tissue necrosis , thrombosis of dermal veins, subcutaneous capillaries, venules, and deep veins with diffuse fat necrosis and interstitial hemorrhage . 1 2

D I F F E R E NTIAL DIAG NOS I S •

Th differential diagnoses are the same (fable 62 - 1 ) .

• Reas ur the patient that th se conditions are usual ly

as

those for HI N 7

AwaJ·ene · of this unusual advCJ·se rea ion to warfarin i extremely crucial as early recognition and management an be limb and /or lifesaving, especially in the patient with H IT .

• Clini ci ans

and other health care givers must be attentive t o patients complaining of localized slcin discomfort or pain, especially in areas of extensive subcutaneous fat such as the breasts , thighs, or buttocks .

•

Immediate cessation of warfarin therapy and reversal of its effects with fresh fi·ozcn plasma and ''itamin K is mandatory.8• 1 1· 1 4

•

Use of an alternate anticoagulant such as U F H or LMWH is recom­ mend d until the necrotic lesion heals, unl s H IT is suspected or diagnosed . 1 ,9, 1 0, 1 2

•

If-l imited if

identified early.

•

Patients should be educated to avoid UFH or LMWH preparations in the future and to inform all of their physicians of the complica­ tions they have experienced with warfarin, U FH, or LM W H .

F O LLOW-U P •

Routine follow-up i s necessary until the skin lesions have healed . Future recommendations regarding the use of anticoagulant therapy is essential .

-P ROV I D E R R E S O U R C E S ======= •

http: I I dermnetnz. org/ re.1ctions/ warfa.rin- necrosis.hunl

•

http : / /www ,ncbi . nl m .nih.gov /pubmed/ 22 3 1 5 270

PAT I E N T R E S O U RCE

M A N AG E M E NT •

In tru t patients about th potential for advers skin rea ions when as warfa1;n ,

u ing U F H , LMWH , or vitamin K antagonists sud1

LABORATOR Y STU DI ES •

be initi.ated and warfarin should not be started until the patient improves clinically and the platelet count recm·ers to 1 50 X 1 09 /L or greater. 8• 14· 1 5 Once initiated, modest doses of warfarin should be used, avoiding an overshoot of the I R, and it should be overlapped with a DTI for a minimum of 5 days.8' 14

Warfarin can be reintroduced but it should be used with extreme caution and initiated only at low doses ( 1 ·2 mg/d) in combination with a parenteral anticoagulant over an extended period of time up to 1 4 days. 1

•

http: I /www . realself. com / answ r/ what-coumadin-induced­ kin-necrosi - I 0-things-you-need-know

R E F ER E N CE S I.

Ha.renberg, J , HolTmann U , Huhle G , Winkler M , Bayerl C . Cutaneous reactions t o anticoagulants . Recognition and managemen t . Am J Cltn Dermarol. 200 1 · 2(2) : 69-7 5 .

2 . Warkentin TE, Roberts RS, Hirsh J , Kelton J G . Heparin-induced slcin lesions and other unusual sequdae of the heparin-induced thrombocytopenia syndrome: a nested cohort study. Chest . 200 5 ; 1 27( 5 ) : 1 8 57- 1 86 1 . 3 . White PW, add JR, ensel RE. Thrombotic complications of heparin therapy: including six cases of heparin-induced skin necrosis . Ann Sura . 1 979; 1 90( 5 ) : 5 9 5-60 . 4. Handschin AE, Trentz 0, Kock H J , Wanner G A . Low molecular weight heparin -induced skin n crosis-;1 systemati review. Lanaenbecu Arch Sura . 200 5 ; 390( 3) : 249-2 54 .

H EPAR I N -, LOW-MO LECU LAR W E I G HT H E PA R I N-, AN D WAR FARI N - I N DU C E D SKI N N ECRO S I S

5 . Warkentin TE . Heparin-induced skin lesions . Brj Haematol. 1 996;92(2) :494-497. 6 . Shelley WB, Sayen JJ . Heparin necrosis: an anticoagulant­ induced cutaneous infarct . ] Am Acad Dermatol. 1 98 2 ; 7(5) : 674-677. 7 . Denton MD, Mauiyyedi S, Bazari I-1 . Heparin-induced skin necrosis in a patient with end-stage renal failure and functional protein S deficiency . Am} Nephrol. 2001 ; 2 1 : 2 89-2 9 3 . 8 . Bartholomew J R . Transition t o an oral anticoagulant i n patients with heparin-mduced thrombocytoperna. Chest. 2005 ; 1 27(suppl 2): S27- S34. 9 . Howard-Thompson A , Usery JB, Lobo BL, Finch CK. Heparm­ induced thrombocytopenia complicated by warfarm-induced skin necrosis . Am J Health Syst Pharm. 2008 ;65 : 1 1 44- 1 1 47 . 1 0 . Chan YC, Valenti D , Mansfield A O , Stansby G . Warfarin induced skin necrosis. Brj Sura. 2000; 87(3) : 2 66-272 . 1 1 . Cole MS, Minifee PK, Wolma FJ . Coumarin necrosis-a review of the literature . Suraery. 1 988 ( 1 03) : 27 1 -277.

287

1 2 . Chacon G , Nguyen T, Khan A , Sinha A , Maddirala S . Warfarm­ induced skin necrosis mimicking calciphylaxis: a case report and review of the literature . ] Druas Dermatol. 2 0 1 0 : 9(7) : 859-86 3 . 1 3 . Warkentin T E , Elavathil LJ , Hayward C P , Johnston M A , Russett JI, Kelton JG. The pathogenesis of venous limb gangrene associ­ ated with heparin-induced thrombocytopenia . Ann intern Med. 1 997; 1 27(9): 804-8 1 2 . 1 4. Srinivasan AF, Rice L , Bartholomew JR, et al . Warfarin-induced skin necrosis and venous limb gangrene in the setting of heparin-induced thrombocytopenia. Arch intern Med. 2 004; 1 64( 1 ) : 66-70. 1 5 . Warkentin TE, Gremacher A , Koster A , Lincofi AM. Treatment and prevention of heparin-induced thrombocytopenia. American ColJege of Chest Physicians evidence-based clinical practice guidelmes (8th edition) . Chest. 2008 ; 1 3 3 : S340-S380.

CHAPTER 63

288

6

OV E RV I EW O F S P I D E R , R ET I C U LAR , AN D VAR I C O S E V E I N S

Sapan S. Desai , MD, PhD Eric Mowatt- Larssen, M D

PATI E NT STO RY A 44-year-old man presents

with a S-year history of progressi.-e right

lower extremity pain, discomfort , swelling and prominent ( Figure 63. I . His sy mp toms occur daily and a re ing,

calf veins worse with st a nd ­

during his job as a nurse working 1 2 - hour shifts in

particul arl y

Overnight rus s ymp t o ms improve. He history , including that of venous thromboembolism. He is interested in an in te rven t io n to im p ro v e ru quality of life. Phy si ca l examination is n o ta b l e only for large medial calf varicose veins and mild pitting edema aroWld the ankle.

the emergency department .

has no other known past m ed i ca l

CLI N I CAL F EAT U R ES •

Patients h.ve symptoms that range from asymptomati

to severe

pain or discomfort. Patients may also present with lower

sw e l l i ng , a distal calf rash, or a l g ul e r . 1

tr mity

• Pain or discomfort i th most common symptom of chroni

venous eli eas

.

TI1e symptoms are usually d scribed as a s,

-amping, fatigue , hca,in

it chi ng , or throbbing. The

1in

s,

pain

and d iscomfort are oft n located u varicosity sit s, at th m dial extr mity (for great saphenous vein reflu.x-Figure 6 3 -2) r posterolateral calf ( for smal l saphenous v in reflux ) . •

Pain, discomfort , and / or swelling are usually worse with dependence ( eg, standing) and

im p ro ve with extremity elc,·ation

or compression . welling and skin ch ange s tend to start around the ankle, graduaUy progress proxin1all · over year ( Rgure 6 3 - 3 ) . •

Leg ulcers tend

to

(Figure 63-4).

occur around t h e

• Phy ical findings aJ·e de cs

pider

indi

at

v ins

m re ad van

( Figu r

or lateral foot

ibed using th CEAP (Clini al -Et iology­

nal my- Pathophy i logy

das

ankle o r medial

but can

las (fabl

6 3 - 1 ). H ig h r CEAP

d ven us diseas . 2

6 3 - 5 and

3-

are often, b u t not al way ,

asymptomat ic.

e vein ( Figure 6 3 -7 ) , reticular veins (Figure 6 3 d o not p rotrude &om the skin surfa e .

Unlike varico

typicall y

E P I D E M IOLOGY A N D R I S K F ACTORS •

20% of adults

•

S% of adults have

have varicose veins . v

Prevalence increases with age. 1

enous skin changes.

• I % of adults h ave or have ha d a venous ulcer .

FIGURE 63-1 This pa ti ent has cla.sslc varicose vei ns . Va ricosities are blue, subcutaneous, tortuous vei ns over 3 mm in diameter.

OVE RVI EW OF S P I D E R, R ETI CU LAR, AND VAR I CO S E VEI N S

•

Risk factors for varicose veins are advanced age, positive family history, female gender, and multiparity .

•

Risk factors for chronic venous insufficiency are advanced age, positive family history, and obesity .

289

PATHOPHYS I O LOGY •

•

•

•

•

Lower extremity veins have bicuspid one-way valves to keep blood 4 flowing toward the heart during dependence (eg, standing) . Reflux is abnormal retrograde flow of blood in the thigh and calf. It represents valve failure and is defined as an abnormally l ong valve closure time (0.5 second for most of the superficial lower extrem­ ity veins, except 1 second for femoropopl iteal system). Reflux i s the most common cause of chronic venous clisease . Reflux spreads to proximal or clistal segments over time. It is caused by vein wall and/ or valve weakness . Veins can also become obstTucted from chronic deep vein throm­ bosis (DVT) or anatomic obstruction ( eg, May-Thurner syndrome or tumor compression) . Chronic venous obstruction also causes chronic venous clisease . Combined reflux and obstruction also occu r , and usually result in worse signs and symptoms than eith e r process alone.

•

Chronic venous disease at all CEAP classes is commonly caused by saphenous reflux. More advanced venous disease (ie, CEAP classes C 3-C6) tend to involve perforator and deep reflux as well .

•

Patients in CEAP classes C3 to C6 have chronic venous insufficiency. These patients have ambulatory venous hypertension (A VH) : inabi.lit:y to lower venous pressure when the lower extremit:y is dependent. Over time, A VH leads to edema, skin changes, and ulcers .

•

The constant effect of A VH through the effects of gravity on a refluxing vein system leads to the accumulation of blood in the dependent extremities . This eventually leads to capillary outflow of fluid, contributing to the edema in the lower leg. The increased pressure stretches the vein wall , producing the red blood cell leakage, resulting in hyperpigmentation, and chronic skin inflammation.

FIGURE 63-2 Large p rotuberant varicose vein a l ong the anterome­

dial thigh and medial calf. This represents a tributa ry of an underlying incompetent great saphenous vei n .

DIAG NOSIS •

•

•

Duplex ultrasound is the most important initial test since it accu­ rately identifies venous reflux or obslTuction . The saphenous, perforator, and deep systems are interrogated . A "rule out DVT' venous study is not sufficient to d iagnose and lTeat chronic venous disease. Management of chronic venous disease is based on the map 5 where reflux begins and ends. Duplex ultrasound helps to identify the cause for venous disease , along with delineating the anatomy of the venous system . Duplex ultrasound uses a combination of color fl ow imaging and Doppler signals to determi ne the flow vectors and thereby identify sites of refl ux. The cliffe r ential cliagnosis for the patient with extremity pain o r discom fort includes peripheral arterial disease, neuropathy (often lumbar racliculopatby) , muscle pai n , and compartment syndrome.

FIGURE 63-3 Hyperpigmentation along t h e a nteromedial c a l f with surrounding spider and sma l l va ricose veins.

C HAPTER 6 3

290

• Causes o f l eg u l cer incl u d e p eri p h eral arterial d isease, neurop athy

(esp eciall y d iab etes me ll itus) , ch roni c mech anical com p ression (as in p arap l eg ia), an d sk in cancer. one o f th e com mon skin ch anges seen are sp eci fi c for ch ronic venous d isease . Dermato l o gy consu l t or furth er work up sh ou ld b e consi d ere d in p atients with sk in ch anges with out signi fi cant vein d iagnosis . •

Patients with un:l l atera l l eg e d ema may have l ym ph e d ema or p roxi­ ma l venous ob struction . Patients with b i l ateral l ower extremity e d ema m ay al so h ave congestive h eart fai l ure, l i p e d ema, k i d ney d isease, l iver fai l u re , or p roximal venous ob struction .

• Patients with pel vic con gestion s y n d rome can p resent w i th p roxi­

ma l thi gh varicosities . Th e y o ften h ave ch ronic p eh�c p ain and / or p el vic varicosities. Diagnosis o f ovarian or o th er p e l vic vein re fl ux can be mad e b y transvag inal u l trasoun d , conventiona l venograph y , mag netic resonance venog raphy (MRV), or com p uterize d tomog raph y venograph y (CTV) , d e p en d in g on th e ca pab i l ities o f th e faci l ity . •

Patients "�tb venous-typ e sy m p toms out o f p rop ortion to fi n d ings on infrain guinal u l trasoun d or h istory o f DVT may h ave i l iocava l ob struction from anatomic ob struction (May -Th urner) , th rom­ b otic ob struction, or tumor . Imagi ng to ch eck for th ese d iag noses

FIGURE 63-4 Severe venous stasis with a combi nation o f hyperpig­ mentation, lipodermatosclerosis, eczematous crusti ng, and small stasis u l ceration. TABLE 63-1 . CEAP Cl asses

CEAP Class

Physical Finding

Definition

co

None

None

C1

Spider vei ns

D i l ated i ntraderm a l ven u l es l ess th a n 1 mm i n d i a m eter (Figures 63-5 and 63-6)

C1

R eticu l a r ve i n s

D i l ated blue subderm a l vei n 1 to 3 m m i n d i a m eter, usu a l ly tortu ous ( F i g u re 63-7)

C2

Va ricose vei n s

D i l ated subcuta n eo u s ve i n g reater than 3 mm i n d i a m eter, usua l l y tortuous (Fi g u res 63- 1 , 63-2, a n d 63-8)

C3

Edema

I n crease in skin a n d su bcutaneous fl u i d vo l u m e , usua l ly i n dents with pressu re

C4a

Eczem a

Erythematous dermatitis, ca n prog ress to weep i n g, b l isteri ng, or sca l i n g

C4a

Hyperp i g m e ntati on

Loca l ized brown s k i n disco l o rati on ( F i g u res 63-3 a n d 63-4)

C4b

Atro p h i e b l a n ch e

Loca l ized, ci rcu l a r, wh ite, a n d atro p h i c s k i n a reas s u rrou nded by d i l ated capi l l a ries a n d sometimes hyperp i g m e ntatio n

C4b

Lipodermatoscl e rosis

Loca l ized chro n i c i nfl a m m ation a n d fi brosis o f s k i n a n d su bcuta neous tissues, someti mes associated with contract u re or scarri n g ( F i g u re 63-4)

cs

H e a l e d venous u l cer

C6

Active veno u s u l cer

F u l l -thickness skin defect (Figure 63-4)

Ada pted fro m E k l of B , Rutherford RB, JJ Bergan et a l . By consensus, the patient C EAP c l ass i s the h i g h est c l a ss physical fi n d i n g . F o r exa m p l e , a patient with s p i d e r vei n s , varicose ve i n s , a n d l i p od e rmatos c l e rosis is c l ass C4b .

OVE RVI EW O F S P I D E R , R ETICULAR, A N D VAR ICOSE VEINS

in l u d int ravascular ultra

291

und , t ransvaginal u lt rasound , oonven­

t ional ''enography , MR V, or

TV, depending on th capabiliti

th fa ility . '

s

of

TREATM ENT • Compression therapy is used for patients w i th any Je,·el of Yenous reflux . Compression improves venous hemad rnamics and quality

of life for patients with chronic venous disease , reduces edema and

skin discoloration, and impro,·es the venous ulcer healing rate. 7

The main contraindication to compression use is peripheral arterial

d isease , especially with ankle-bradual index ( A B I ) less than 0 . 5 .

Commercial] y available oompression stockings are most often used . • Inelastic compr

ion , such as Unna boots , is also used in patien

with active ulcers . Compression is also used after most other \'ein t reatments, including urgery, thermal ablation, and clerotherap . •

is corr

Reflux

d in dte following ord r: aph nou ,

p rforat or, d

or

telangiectasi as.

main oontraindicat ion t t reatm nt of refilL'
3 -4 mm in diameter) ,

•

D I F F E RE NTIAL DIAG N OS I S •

Dermatologic conditions with o r without pigmentation can b e

•

Other options include laser and intense-pulsed-light (IPL) therapy.

•

Although the use of liquid or foam sclerotherapy in the treatment of the a>dal GSVs or SSV s has been controversial, an increasing number of studies have demonstrated its safety. Ultrasound-guided

mistaken for small varicose veins . •

foam sclerotherapy for primary varicose veins or recurrent veins has shown good results with minimal complications, although follow-up sclerotherapy sessions may be required.

Capillary malformations associated with congenital venous malfor­ mations can also be confused with reticular and spider veins .

•

Cellulitis .

•

Stasis dermatitis.

•

Osler-Weber-Rendu syndrome or hereditary hemorrhagic telangiectasia.

I NVESTIGATIONS •

Laboratory tests do not contribute anything to the management of

CO M PLICATIONS •

•

MANAG E M E NT •

•

•

Sclerotherapy using liquids such as sodium tetradecyl sulfate

•

Recent progress has been made in using foam sclerotherapy (air mixed with detergent solution to create foam) for sclerosing larger varicosities, recurrent veins , and even venous malformations.

•

The best results are obtained in patients with competent large superficial a>dal veins (GSVs or SSVs) . A recent Cochrane review supports the role of sclerotherapy for small veins or recurrent veins after surgery. 2

•

•

•

Magnification is essential in accurately injecting intraluminally and

•

No anesthesia is required for sclerotherapy. Occasionally topical anesthesia is utilized for laser ablation of spider veins.

avoiding extravasation, which can be painful and cause skin necrosis .

Compulsive wearing of stockings post-therapy has been tradition­ ally emphasized to decrease the incidence of hyperpigrnentation or matting, which is an appearance of new small capillaries around the site of treatment.

•

Patients must be informed prior to treatment with sclerotherapy or laser of the need for more than one treatment to successfull y oblit­ erate the veins.

FOLLOW- U P •

Following sclerotherapy all patients must be followed u p for inspection of the local area and detection of possible superficial or deep venous thrombosis and rarely systemic side effects .

• •

Compliance 'vith stockings is repeatedly emphasized . Since hyperpigmentation occurs in 1 0% to 30% of patients , patients are instructed to avoid heavy sun exposure while the injected areas are healing s In addition, evacuation of microthrom­ bus inside the occluded and sclerosed veins is advised to reduce the chances of hyperpigmentation . Patients must therefore return, often more than once , as part of their treatment.

Foam sclerotherapy has been successfully utilizing various agents with obliteration of the GSV in 8 8 % . 3 A small tuberculin syringe with a 30-gauge needle is used for reticular and spider veins.

Patients must also be warned about unusual side effects such as transient neurologic symptoms including transient ischemic attacks, migraine-like headaches, or visual disturbances.

(Sotradecol) or polidocanol have been used for many years to oblit­ erate spider veins (< 3 mm diameter) or reticular veins . Injection of these liquids leads to endothelial destruction and then fibrosis and reabsorption, leading to elimination of the superficial veins .

Pulmonary embolism is extremely rare .

PATIENT ED UCATION •

Sclerotherapy is used for superficial cutaneous or subdermal vari­ cose veins, residual or recurring varicose veins following surgery, and spider or reticular veins.

Neurologic complications such as transient ischemic attacks or strokes are rare. Visual disturbances are often described with foam sclerotherapy. Occasional headaches or even migraine with an aura following treatment also occur. The overall incidence of all neuro­ logic complications is reported to be around 0 . 9%. 4

Doppler tests are also not usually indicated unless there is suspicion of saphenous vein or deep vein reflux . lf there are large feeding veins or large branches originating from the great or small saphen­ ous veins (GSVs or SSVs) , a duplex ultrasound scan is needed to locate incompetence of valves in these large a>dal veins . In the presence of reflux in these a>dal veins, sclerotherapy of spider or reticular veins will fail or temporary success will be followed by recurrence .

Besides unsuccessful obliteration of the veins, common side effects include hyperpigmentation , skin necrosis, matting, ulceration, and rarely anaphylactic or allergic reactions .

spider and reticular veins . •

Ultrasonograpmc guidance may prove useful when sclerotherapy is used for larger subdermal veins that are noted to feed the superfi­ cial spider veins.

protrude above the skin surface , and compress easily vvith slight manual pressure .

•

In general , hyperpigmentation resolves in 6 to 1 2 months . 6 Superfi­ cial veins greater than 1 mm are more likely to leave pigmentation marks than small spider or deeper varicose veins.

SCLEROT H E RAPY

PROVI D E R RESOURCES

o htt p : I I onlinelibrary . wiley .com/ doi/ I 0. 1 002

/ 1 46 5 1 5 8 . 000 1 732 . pub2 /abstract; jse ionid = F62 3 C8 F72 5 B9E48E895 1 0 04C I 004 054.d04t0 3 .

0

http : I / www . bidmc. org /Centers-and- De partments / De p artments / Cardiovascular-Institute/ Your- Cardiovascular­ Health / - /media /Files /Centersand De p artments /Cardia V ascular"/o201nstitute / W omens%20Event%2020 1 2 I AIMahameed .ashx

PAT I E NT R ESO U RCE 0

htt p : I I www . webmd .com/ skin- p roblcms-and- tr�tments I COmlctic- p rocedures-s p ider-veins

R E F E R E NCES

I. A gu 0, H amilton G , Baker OM, Dashwood MR. Endothelin rece p tors in the aet iology and p atho ph ysiology of varicose vei ns . Eur J Vase Endo•·asc. 2002 ;2 3(2): 1 6 5 - 1 7 1 . 2 . T.si PV, Beverley C, Rees A . injection sclerothera py for varicose veins. Editorial Grou p : Cochrane Peripheral Vascular Disease

295

Grou p . Published onlin : 2 1 J an 2009. Available at: http : I I onlinclibrary . wile y .com/doi/ 1 0. 1 002 / 1 46 5 1 8 5 8 . CDOO I 7 32 . p ub2/abstract ;jsessionid= F62 3 C F72 5B9E4 E895 I D 04C I 004 054 .d04t03 . Accessed December 2 5 , 20 1 1 . 3 . mith PC. Chronic venous disease treated by ultrasound guided foam sclerotherap y. Eur J Vase Endovasc Sura. Nov 2006; 3 2 ( 5 ) : 577-5 3. 4 . Bradbury AW, Bate G , Pang K , Dan·all KA, Adam DJ . Ultrasound-guided foam sclerotherapy is a safe and dinicaU y effective treatment for su perficial venous reflux . J Vase Sure . 201 0; 5 2 : 93 9-94 5 . 5 . arvananthan T, Sh pherd A C , Willenberg T, Davi s A H . Neurological com p licati n s o f deroth ra py for varicose veins . } Vase Sura. 201 2 ; 5 5 : 243 - 2 5 1 . 6. Guex J -j , Allaert F-A , Gillet , J - L , Chlier F. I mmediate and midterm comp licati ns of scleroth ra py report of a p ros pe ctive multi-center reg istry of 1 2 , 1 7 3 s lcrothera py sion.s . Dumatol Sura . 200 5 ; 3 1 : 1 2 3- 1 28 .

296

CHAPTER 65

E N D OV E N O U S LAS E R AB LAT I O N FO R VAR I C O S E V E I N S Bhagwan Satia n i , M D , M BA. RPVI , FACS

PATI ENT STO RY A 3 5- year-o l d woman mother of three, pre s ented "ith a p rogre ss i v e increase in the size o f the v ein on the inside of her thigh d uri ng her pregnancies . h e described aching, fatigue , and hea,iness as the d a y w o re on and experi en ced some relief with e l e v ati o n . be initially rel ated some relief with p rescr i ption stodci ngs but st e ad y w o rse ni ng m·er th last year. She found it difficul t to stand at work or during regular chores at ho me . Both her parents had intervention for varico ,e veins. Her physical examination showed a very l arge great s a phe n o us vein (G V) &om t he mid calf to the gro i n wi th some te nd e r ness a lo ng the cow· e o f the ,·ein w i tho u t any palpabl th rom bus . o ulceration or p ig men ta t ion was noted at th ankle . H r d u p l e x venous ult rasound exa mi n atio n howe d no d ee p or uperficial venous thrombo i but a large-diameter and ig nili can t reAu.. x (retrograde Aow d uri ng a Val aha maneuv r) d own t o th mid-calf ( F ig ure 65- 1 ) . Aher di scus ing various o p tions sh e elected for ablation o f h e r G

FIGURE 65- 1 Duplex ultrasound venous examinatton demonstrati ng reflux (retrograde flow) i n the great saphenous vein (GSV).

i th a laser ( Fi gu re 6 5 - 2 ) .

w

PATHOPHYSIOLOGY • The p re v a l e n ce of varicose veins in the Western population is a bout

20"/o. I

• The bicusp id venous valves a re c r uci a l in p romot ing unidirectional now

•

u d al to p ro x i m a l , w i th th m st important

bd ng lose to the a p h nofemoral junction.

sa ph nous valve

1osl varicose veins p rob ab ly have a m u l tifa ori al etiol ogy stru ural weakne is a l ikely cause.

a l t ho ugh an intri nsic

• Varico e veins

on a

can

also occur foil

o nge n i tal basi

"'ing dee p

venous thrombosis or

.

CLI N I CA L F EAT U R E S • Common

m p to m o f vari vein include pain, h avin aching , bu rn i ng , fatigue, th ro b bi ng , it hine s, and oc si nally

restl

legs

ndrome,

• Physical e x a mina ti o n sh uld mainly focus on th extr mi ty

pat ie nt standing as well sup i n e . Th I cati o n , ize, and g n e ra l eli tribution o f th va ri os v ins ar no t e d and

involved with th

prcfcubl mark d on a d i ag ram .

• Pa lp ation

as

wcU

as

a uscul t.tt io n is al so recommended to detect

p u lsa til i ty , thrill.. , bruits, o r any tenderness.

F I G URE 6 5-2 Preoperative photograph of the enlarged va ricose veins (left image) and a postoperative photograph (right Image) follow ng endovenous laser ablation and p h lebectomies.

E N DOVE N O U S LAS E R A B LATI O N FOR VAR I CO S E VEI N S

• Signs of chronic disease such as ulceration , skin changes, pigmenta­ tion , eczema, temperature changes , and edema should be noted.

297

• Surgical stripping of the GSV or small saphenous vein (SSV) with or without removal of the branches (phlebectomy) has been the most common procedure to treat varicose veins for decades.

• A record of palpable pulses is mad e .

• Liquid sclerotherapy or foam sclerotherapy is also an option in

• The traditional named tests such a s Trendelenburg test or Perthes test are now rarely performed, and their utility today is

some patients. • The recent option of an endovenous approach using radiofrequency

questionable .

ablation (RFA) or laser thermal ablation has become widespread as a less invasive alternative. 2

D IAG N OSTIC EVALUATION • Duplex venous scanning is performed to identify reflux o r retro­ grade flow in the GSV, and to document the vein size and extent of

Most patients subjected to ablation will have had a trial of nonopera­

the reflux . • Normally, Doppler evaluation of the GSV during a Valsalva maneu­ ver results in little retrograde flow through the first valve in a caudal direction . However, in patients with varicose veins due to saphenous vein incompetence , retrograde reflux is easily detecte d . Any reflux lasting longer than 0 . 5 ms and ideally greater than

I N D ICATIONS

I

ms is said to be diagnostic of valvular incompetence. In addition, measurement of the vein diameter and extent of the reflux is also

tive regimen including compression stockings for longer than 3 months and sometimes much longer. There are no medications that are scientifically proven to benefit patients with large, symptomatic varicose veins.

PROC E D U R E • Endovenous ablation using either laser o r R F A i s relatively recent

important. A very large-sized saphenous vein, certainly greater

as a less invasive option for the treatment of large axial varicose

than 20 mm in diameter, may lead to a high failure rate with abla­

veins .

tion, although recent developments in technology may allow larger veins to be ablated . • Venography or other imaging studies are generally not necessary.

• The procedure is performed under ultrasound guidance with per­ cutaneous entry into the below-lmee GSV (Figure 6 5 - 3 ) . A sheath and then a guidewire is threaded upwards near the saphena­ femoral junction and positioned about 2 em distal to the junction

LABORATORY STU D I ES • Since most ablation procedures are performed either un der local

(Figures 65-4 and 65 - 5 ) . • Tumescent anesthesia i s administered and with duplex ultrasound

anesthesia or with light sedation , routine laboratory tests are not

imaging, the RFA or laser catheter is slowly pulled back, heating

needed .

and ablating the vein (Figure 6 5 - 6 ) .

• If regional or general anesthesia is necessary for specific reasons ,

• The patient is discharged home with compression stockings o r

then hospital or ambulatory care policies will dictate tests such as a

bandaging with instructions to ambulate frequently and elevate the

complete blood cell count or electrolyte panel .

extremity when sitting.

D I F F E R E NTIAL DIAG N OS I S

R E S U LTS

• The patient' s unilateral symptoms o f venous insufficiency are fairly typical in this age group. Other diagnoses to consider include rare

• The short-term efficacy and safety of endovenous ablation with a 3 laser or radiofrequency has been established.

arterial conditions causing intermittent claudication and neurologic

• Many randomized controlled trials as well as observational reports

conditions. • The main differential diagnosis to be considered is varicosities due to a secondary cause such as deep venous obstruction. A previous history of deep venous thrombosis (DVT) , pregnancies, and a family history of thrombotic disorders are relevant. A duplex ultrasound examination should eliminate this cause of varicose veins . • Congenital causes of varicose veins are easily ruled out on history taking by the time of appearance of varicosities.

MANAG E M ENT • Most patients with symptomatic varicose veins are treated success­ fully, although not cured , with compression stockings. • Intervention is usually only indicated for symptoms not relieved by stockings or complications of varicose veins such as ulceration or bleeding.

and a meta-analysis is available to assist clinicians in determining the best option for patients in need of intervention. Early success rates showing occlusion of the GSV range from 9 3 % to 1 00% .

4

• In a recent randomized report comparing 1 00 ablative procedures witl1 traditional high ligation and stripping of the GSV showed no difference in symptom relief or quality of life. Hematomas occurred 5 more frequently in the surgical cohort. However, two GSVs were found completely recanalized and five were partially recanalized after laser ablation compared to none in the surgical group . • Another similar randomized clinical trial showed no difference in pain score between the open surgical stripping versus ablation, but less bruising and edema after the latter . 6 • It is also generally agreed that although the efficacy and disease­ specific quality of life is the sam e , an earlier return to normal activity is reported after laser ablation. 7

CHAPT E R 65

298

o

In general n

w

techniques

of minim a l ly

irwash•e tripping are co m ­

parable to ablation procedure with a sl;ght increase i n pain and a minimal mer

in return t

work time .

o However, ablation procedures

can

almost always be performed in

the outpatient office under local anesthesia with or ";thout

sedation with great patient satisfaction .

PAT I E NT EDUCAT ION o The pathophysiology of varicose ,·eins and the benign nature of the problem must be explained along with signs and symptoms of

complications resulting &om long-standing varico e veins.

o Th ben licial clli t of prop r graduated and the import a n

of

mpl ian

education .

com pre

is an

entia! part of patien t tr

o FoU wing ablati n pro edw·e , ambulati n i prevent venous ta is and sub

ion stocking

F IG U R E 6 5 -3 T he entry site is shown below the knee after remova l of the sheath and laser catheter prior to pladng an adhesive bandage (different patient).

ngly advised to

qu nt DVT .

uperlicial venous thrombosis of the ablated

V or

occurs

S

oc a.sionaUy, and the patient must be forewarned about sy mpt ms

of pain and swell;ng in the area associ a ted with it .

FOLLOW-U P Although major complications such as acute DVT following ablation are rare (< 1 °/o) , er -thema, saphenous neuralgia, early and late hyper­ pigmentation , edema, and occasionall y superficial venous thrombosis

of tributaries does occur . Therefore , outpatients must be warned of

symptoms of DYT. Repeat duplex ultrasound studies are performed i n the first or second year to confirm closure of the

G V or

if recur­

rent symptoms occur.

o

http: / / www , ncbi . nlm . nih . godpubm.,d/ 20 347 541

o

http: / l www . ncbi . nlm . nih .gov /pubmed! 1 6 1 7 5 5 3

o http: I I www . w ebmd .

Tip ol laser/sheath positioned prior to activation

m I kin-prohle rru -and-trea t me nts

/news/20 1 1 09 1 9/. urgery-v -lascr-treatment-for-\"artco

- veiru

R E F E R E NCES I . M urad M H ,

F I G URE 65-4 Photograph demo nstrating a sheath in place and the laser catheter p laced on the outside prior to i nsertion (different pati e nt) .

oto -Yglesias f , Zumaeta -Carcia

M,

et al.

A

� L..!!!.l

systematic review and meta-anal ·is of the treatments of vari ose veins. }

Vasc Surg.

20 1 1 ; 5 3 : 549- 6 5 .

1 . Gillespie DL Cloviczki M L , Lohr J M ,

et

al.

The care o f patients

with varicose veins and as odated chrome ,·enous diseases:

clinical practice guidel;nes of the Society for Vascular urgery and the American Venous Forum . } Vase

Sura.

2 0 1 1 ; 5 3 : 2 - 54 8 .

3 . R asmussen L H , Bjoern L , Lawaerts M , Blem ings A , Lawaertz B , Eklof B . Randomiz d t ri a l comparing cndo\'enou laser ablation of the great saphenous v in "�th high ligation and stripping in patient with ''•ricose veins: short -term results . } Vase 3 08 - 3 1 5 .

Sura. 2007;46:

Rrst valve

Common femoral vein

F I G URE 65-5 I ntraoperative duplex u ltrasou nd p i cture demonstrating the ti p of the l aser catheter (red arrow), the epigastri c vein, the com­ mon femoral vei n, and the first valve at the great saphenous vein (GSV) and femoral vei n junction (purple arrows).

E N DOVE N O U S lAS E R A B LATI O N FOR VAR I CO S E VEI N S

4 . Min RJ , Kl:rilnam N , Zimmet SE. Endovenous EVL treatment

299

Post endo-venous laser

of saphenous vein reflux : long-term results. J Vase lnterv Radio!. 2003 ; 1 4 : 99 1 -996. 5. Christenson JT, Gueddi S , Gemayel G , Bounameaux H . Prospec­ tive randomized trial comparing endovenous laser ablation and surgery for treatment of primary great saphenous varicose veins with a 2-year follow-up . j Vase Sura. 2 0 1 0 ; 5 2 (5) : 1 2 3 4- 1 241 . 6. Medeiros CAF, Luccas G C . Comparison of endovenous treatment 'vith an 8 1 0 nm laser versus conventional stripping of the great saphenous vein in patients with primary varicose veins . Dermatol

Sura. 2 005 ; 3 1 : 1 68 5 - 1 694. 7. Darwood RJ, Theivacumar N , Dellagrammaticas D , Mavor AID , Gough MJ . Randomized clinical trial comparing endovenous laser ablation with surgery for the treatment of primary great saphenous varicose veins. Brj Sura. 2008 ;95 : 294- 30 1 .

One day after procedure FIGURE 65� Poste ndovenous laser a b l ation d u p l ex u ltrasound pict u re showing the saphenofemoral j u n ction with the g reat saphenous vein (GSV) ablated.

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LI M B SWE LLING

C HAPTER 66

302

66 P R I M A RY A N D

S ECO N DA RY LYM P H E D E M A

Ana Casa n e g ra , M D S u m a n Rath b u n , M D

An 1 8- year-old woman seeks an opinion about swelling in her right leg . She first noticed it 1 year ago when her pants seemed to be tighter on that leg . She bas no pain or discomfort; the edema does not improve after nocturnal rest . On physical examination, the left leg is normal, and on the right the edema involves the toes, which look squared, along with nonpitting edema in the foot. The skin appears thickened , and there is a 4-cm difference in diameter between the legs measured in the calf. She has no adenopathy , and the abdominal examination is unremarkable . A diagnosis of lymphedema tarda is rendered.

DIAG NOSIS H istory o Lymphedema may present as unilateral, painless swelling with

minimal change with elevation.

o The swelling usually starts distally and is more noticeable in fingers

or toes.

o Recurrent cellulitis is a common complication.

E P I D E M I OLOGY o Lymphedema is an accumulation o f lymphatic fluid in the interstitial

tissues, most commonl y in the limbs , resulting in edema.

o Incidence: primary lymphedema-1 : 6000 to 1 : 1 0,000; secondary

lymphedema-varies among different populations; it can be as high as 1•2 30% in breast cancer patients after surgery and radiation therapy . 2 o Primary lymphedema is more prevalent in females, 2 . 5 t o 1 0 : 1 . o Lymphedema can affect patients from birth (congenital

l ymphedema) to advanced age .

o Lymphedema rarely leads t o death, but i s a source o f morbidity and

affects body image .

o The natural history varies fi·om minimal swelling to severe

deformities in the limb.

subtropical regions of Asia, Africa, and Central and South America) , 3 malignancy (involving l ymph nodes or lymphatics) . trauma (surgical damage or resection of lymphatics) , chronic venous insufficiency (increased interstitial fluid), inflammatory diseases, recurrent bacterial infections , and chronic edema. 1 •2

1

ETIOLOGY A N D PATHOPHYS I O LOGY o Lymphatic vessels collect and drain the excessive interstitial fluid

that escapes the capillary circulation . Lymphedema is the accumula­ tion of this protein-rich l ymphatic fluid in the tissues .

o Lymphedema occurs when the production of lymphatic fluid

exceeds the transportation capacity of the lymphatic vessels.

o It can affect multiple areas , but most commonly involves the upper

or lower extremities.

o In primary lymphedema there is an inherited defect in the

lymphatic vessels that manifests at birth to the early 40s . Lymphe­ dema can be the only manifestation, or it can be part of a syndrome ( eg , yellow nail syndrome , lymphedema-distichiasis syndrome , 2 Turner syndrome) .

o Secondary lymphedema is more common , caused by destruction

or damage of the lym phatics or increased production of l ymphatic fluid . Common causes include filarial infection (in tropical and

o History of trauma, surgery , radiation therapy , and malignancy

assists in confirming the diagnosis of secondary lymphedema.

o Congenital l ymphedema is evident at birth or within the first

2 years of life; lymphedema praecox most commonly presents at 1 puberty and lymphedema tarda after age 3 5 . • 4

o Family history of l ymphedema may be present .

Physica l Exa m i n ation o Most of the time the diagnosis is clinical, with no need for

confirmatory imag ing tests .

2

o Edema may be pitting at early stages, but is nonpitting as the

2 disease becomes chronic. •4

o Squared-off toes are characteristic of primary lymphedema; the

toes have a blunt, squared appearance (Figure 66- 1 ) . 1 • 2•4 •5

o Dorsal hump may occur, which presents as noticeable edema

involving the dorsum of the foot (Figure 66-2 ) .

o Stemmer si gn is the inability t o tent the skin a t the base o f the

second toe .

1 '2'4'6

o Peau d' orange is a late-stage sign (Figure 66- 3 ) ; dilated hair

2 follicles with minimal pitting in the skin, cutaneous fibrosis. • 5

o Other skin involvement: hyperkeratosis (Figure 66-4) , papillomatosis

(Figures 66-5 and 66-6) .

'

o In infectious cellulitis (Figure 66-7) , the skin appears erythema­

tous, with increased temperature and lymphangitic streaks toward the lymph nodes . 4

o Assess the area in bel:\veen toes for tinea pedis, as it is a risk factor

for cellulitis .

'

o Surgical scars, masses , and other skin lesions can help with the

diagnosis of secondary lymphedema.

P R I MARY A N D SECON DARY LYM PH E D E MA

FIGURE 66-1 Squaring of the toes, in creased skin fo lds, and

303

FIGURE 66-3 Classical " peau d'orange" or "skin of a n orange " appearance signifies excessive cuta neous lymphatic fl u i d .

hyperkeratosis consistent with lymphedema.

FIGURE 66-2 Dorsal pedal hump of lymphedema. Characte ristic exa ggerated skin fo lds at the ankle a re also i l l u strated.

F I G U R E 6 6 -4 Lym phedema man ifestations: hyperkeratosis o f the ski n . The edema affects t h e toes and foot predom inantly. N ote the ma rks of the e l astic wrapping on the dorsum of the foot.

C HAPTER 66

304

Diagnostic Tests o Lymphangiography, wruch involves the injection of iodinated

contrast into cannulated lymphatics, is rarely used due to risk of worsening lymphedema.

o Lymphoscintigram (Figure 66-8) : diagnostic method of choice .

This nuclear scan foiJows the progression of a radiolaheled contrast

injected in the subcutaneous tissues between toes or fingers. Diagnosis of lymphedema is suggested by delayed transport or backflow of the tracer, asymmetric or absent visualization of the 7 vessels or nodes, and other abnormal patterns. 2 •

o Duplex ultrasound can be useful to rule out other causes of edema such as venous insufficiency and deep venous thrombosis (DVT) .

o Computed tomography (CT) can help diagnose causes for

secondary lymphedema and assess volume discrepancies. Changes

present in lymphedema are skin thickening, honeycomb pattern, and subcutaneous edema . 8

o Magnetic resonance imaging (MRI) lymphography is promising but not widely available. Conventional MRI can show changes in the

FIGURE 66-5 Advanced skin chan ges in the calf of a patient with

lymp hedema. The papulonod u l a r and hyperkeratotic appearance is a l so known as elephantiasis nostras verrucosa .

skin and subdermis . 9

DIFFER ENTIAL D IAG NOSIS o Chronic venous disease and post-thrombotic syndrome: edema,

usually pitting; pain with prolonged standing; hyperpigmentation of the skin; varicose veins . 1

o DVT presents acutely and needs to be excluded with duplex ultrasound .

o Lipedema affects mostly females; it is an abnormal bilateral

deposition of subcutaneous fat. It spares the feet and involves

calves . Stemmer sign is negative , and there are no skin lesions . 1

o Myxedema can be localized or generalized. Usually bilateral , it is associated with generalized skin and adnexa changes such as thin hair, brittle nails, rough skin, and yellowish discoloration . 1

o Systemic causes of edema (congestive heart failure, renal failure,

volume overload , hypoalbuminemia) : bilateral soft pitting edema

with no skin changes , predominantly affecting the ankles and respecting the feet.

MANAG E M E NT o Reduction of edema : Patients need to be referred to a physical

therapist (PT) with training in manual lymphatic drainage (MLD ) , a special technique to reduce edema. The PT will also apply

multilayer wraps between sessions to reduce edema and avoid 5 reaccumulation of lymphatic fluid. 1 •

o Maintenance: Compression stockings up to 40 to 60 mm Hg or

sleeves 20 to 30 mm Hg need to be worn daily. Milder compres­

sion overnight and inelastic underwear are sometimes required. The compression garments need to be renewed regularly (every 4 3-6 months) as they lose elasticity. 1 • • 5

o Intermittent pneumatic compression : May be a helpful adjuvant for maintenance treatment. 1 '

4

o Exercise is advised such as swimming, as it improves lymph flow. 4 o Diuretics are generaiJy not indicated . 2

FIGURE 66-6 A long-sta nding primary lym phedema patient with elep hantiasis. The normal conto u r of the d ista l ca lves and m a l l eo l i have been lost consistent with a n " e lephanti n e " ankle. Other cha racteristic features include acral swe l l i n g with exaggerated skin creases, hyperkeratosis, and a papulonod u l a r eruption in the background of p rofound leg swe l l i n g .

P R I MARY AND SECON DARY LYM PH EDEMA

urgery is not usually re

• •

305

nun ended . 1 •1

Complications Llulit:is: Streptoa>ccus and Staphylococcus are the most common agents. ellulitis should be promptly treated with empidc antibiotics to prevent worsening of lymphedema. Recurrent cellulitis may need chronic antibiotic suppressive therapy. 1 •4 o Cancer: Uncommonly lymphedema rna undergo malignant degeneration (angiosarcoma or lymphangiosarcoma . o

·

PAT I E NT E D U CATI O N •

Compliance with the treatment is critical in tbis disease, as it requires lifelong management ";tl, a significant commitment from the pati nt.

•

The patients or a family member should learn to p

•

kin r·e : Patients sh uld avoid wounds and cut and use prot lion for the alt d limb: glove for gard n work, avoidan e f walking barefoot . kin m isturiz.ing is advised, and pedodi e.. x ami­ nati n of the t e webs, with prompt l r atment of tinea p dis.

rfo nn

MLD.

,

FIGURE 66-7 I nfectious cel lulitis in a patient with lymphedema .

ellulitis prevention : Patients should recognize the clinical igns and symptoms and promptly contact their physician while starting empirical antibiotics . FOLLOW- U P •

Initial frequent follow- up is helpful u ntil the edema i s wel l control­ led, and to reinforce patient education , p roper use of compression garments, and skin care .

•

Long-tenn fol low-up can be early if sy mpt ms are well controlled and more often if complications ari . PATI ENT A N D P ROV I D E R RESOU RCES •

http: / / www . lymphnet .org

•

http: / /www. lymphedemaresources . org

•

http: / / w, v. ncer.go,·/canccr·topic /pdq/support i,·c re /lymphedema / Patient/page I

•

http: / / w,vw.ncbi . nlm.nih.go,· /puhmedbealth!PMH0002 1 06/

•

http: I I www . mayodmic.com /health! I ymphedema/ DS00609

•

http: / /lymphaticresearch . org/ main . php?content=home

R E F E R E N CE S I . Rockson G . Lymph dcma . Am] Mtd. 200 1 ; 1 1 0(4): 28 - 29 5 . 2 . Rockson

. Diagnosis and management of lymphati vascular disease. ] Am Coli Cordial. 200 ; 5 2 ( 1 0): 799- 06.

3. Taylor MJ , Hoerauf A, Bo kari M . Lymphatic filariasis and

onchocerciasis. Lancet . 20 1 0; 376(9747): 1 1 7 5- 1 1 5 .

4 . Kerchn r K , F l i.sch r A , Yo ipovitch G . Lower

tremity lymphedema update : pathophysiology, diagnosis and treatment guidelines. J Am Acad Dermatol. 200 ; 59(2) : 3 24- 3 3 1 .

5 . Rockson G. ur-rent con pts and future dir tions in t h diagnosi a n d management of lymphati vascular d i s ase. Vase Mcd. 20 1 0; 1 5( 3): 2 2 3- 2 3 1 .

F I G U RE 66-8 Lymphosd ntigram: posterio r v i ew at 4 h ours shows dermal backflow in the ri g h t leg and absent lymphatic c h a n nels and lymph nodes on the ri gh t l eg .

C HAPTER 66

306

6 . Stemmer R. [A clllll cal symptom for the early and differential diagnosis of lymphedema] . Vasa . 1 976 ; 5 ( 3) : 2 6 1 -262 . 7. Jensen MR, Simonsen L, Karlsmark T, Billow J. Lymphoedema of the lower extremities-background , pathophysiology and diag­ nostic considerations. Clin Physiol Funct Imaging. 2 0 1 0 ; 3 0(6) : 389-398 .

8 . Monn:in-Delhom ED, Gallix BP, Achard C, Bruel JM, Janbon C . High resolution unenbanced computed tomography i n patients with swollen legs . Lympholo8Y. 2002 ; 3 5(3) : 1 2 1 - 1 2 8 . 9 . Astrom K G , Abdsaleb S, Brenning G C , Ahlstrom KH . MR imaging of primary, secondary, and mixed forms of lymphedema. Acta Radio]. 200 1 ;42(4) :409-4 1 6 .

LOWE R EXTR E M ITY CELLULITIS

307

LOW E R EXT R E M I TY CELLU LITI S Satish K. Sarvepa l l i , M D , M P H J u l i e E . M a n g i no, M D

PAT I E N T STO RY A 4 2 -year-old ma n with m orbid obe s i t y , diabetes melli t us , oong stive

h art fa i lure , and venous insufficiency pre

nted with fever , pain and

swe ll ing of his l e ft lower e x t remity ( L LE). He r ported trivial t n.uma to his LLE after bumping into a table progres tuaU

the I

d

from mild

red o

2

weeks prior; the affected area

to an open ulcer at the ankle. It c ven-

develop d increased rednes , warmth, a nd pain

ft ankle to t he knee. At admi· ion, he had an

tending &om

pen ulcer with

purulent drainage along with xcoriati n of th superficial layer of

th

n

kin ( Figure 67- 1 ). Given the pu rulent nature rn for m thici.ll in -resi tnnt

tart d

n intra\· nous van

Staphylococcus

f the

ourtus

ived approprial wound

m, cin and re

car . Aft r in itia l i m p rovement , he was witch d to to

mplet

l l u(jtis and

( M R A), b wa ral

lindamycin

a l t al of 1 0 da y of therapy . On a 2 - we k follow - up

Uulitis had r·e

l ved .

E P I D E M I O LOGY ding infection of the skin in v o l vin g th

Cel lulitis is a rapidly spr

d eper dermis and th.e ubcutaneou t i ss u . 1 • 2 It extends d cper than erysipelas, 1 which is in the difl'erential diagnosi s .

• A common i nfectio n seen b y both hospital-based a n d p rim ary care physicians . 4 •

Contributes to more than 600, 000 hospitalizations each yea r .

• A nnual office visits for cel lul it i s and cutaneous ab from 4- . 6 m illi n to 9 . 6 rnillion in 2 00 5 .

•

5

ce

4

incr ased

A I sian w ith exudate and purulen t drainage , without an underly­ , is defined

ing drainable abscc nantly due to

oureus . �. 6

as

purultnt cellulitis; it is pr ed o m i ­

• Lesi ns w ithout e."ese antibodies are yet to be determined. Targets include oral mucosal antigens, endothe­ lial cells, T -cell costimulatory molecule, cytotoxic T lymphocyte antigen-4 (CTLA-4) , and oxidized low-density lipoprotein, among others .

•

Anti-Saccharomyces cerevisiae antibodies have also been reported in

ETIOLOGY A N D PATHOPHYS I O LOGY Genetics • Genetics of BS are mostly uncertain; however, it is a polygenic

BS patients, particularly in patients with intestinal involvement .

disorder. • Genetic factors are suggested by the fact that immigrants of

endemic areas to areas of low prevalence show higher disease prev­ alence than the general population of the area .

Endothelial Dysfu nction a n d Coag u l ation Abnorm a l ities • Endotl1elial dysfunction is a hallmark of BS with reduced flow­

mediated dilation. This promotes vascular inflammation and pro­ vides a prothrombotic milieu in BS.

• Human leukocyte antigen (HLA)-B5 1 allele expression signifies an

increased risk of developing BS. HLA-B5 1 phenotype appears to be more prevalent in the familial cases of BS, and its contribution is actually limited to 20% of BS cases . • Other non-HLA genes have also been reported in the pathogenicity

of the disease including intracellular adhesion molecule (ICAM)- 1 gene, interleukin (IL) - 1 gene, endothelin nitric oxide synthase (eNOS) gene, vascular endothelial growth factor (VEGF) gene, manganese superoxide dismutase gene , and cytochrome P450. Mutations in the Mediterranean fever gene (MEFV) encoding the protein pyrin have been implicated as well .

• Nitric oxide (NO) evokes endothelial dysfunction, ,.vith increased

NO concentrations reported in the serum of BS patients as well as their synovium and aqueous humor . can occur in up to 2 5 % of BS patients; although some evidence exists for defects in the hemostasis or coagulation or fibrinolysis cascade, it is currently believed that the thrombotic manifestations of BS are secondary to vascular damage resulting from inflammation and endothelial dysfunction rather than a gener­ alized hypercoagulable state.

• Thrombotic events

•

I nfectious Factors or Mo lecu l a r Mimicry • Many studies point to a possible role for an infectious process in the

pathogenicity of BS.

DIAG N OSIS •

The cliagnosis o f B S remains a challenge given the protean manifes­ tations, and the multiple diseases, which mimic this syndrome as well as the lack of specific cliagnostic tests.

•

The cliagnosis of BS should be based on collective information from the pattern of clinical involvement, laboratory findings, tissue histology, and imaging.

• It is theorized that bacterial or viral antigens share some homology

in terms of sequences and thus cross-react \vith the heat shock family of proteins (HSP) , resulting in a cell-mediated and humoral immune response. • Specific pathogens studied include

Streptococcus species, Helicobacter pylori , herpes simplex virus, and parvovirus B 1 9 .

Cel l u l a r I m m un ity and Cytokines 2 • Immune system dysfunction is the hallmark of BS; this includes

both the innate as well as the acquired immune systems , both the cell-mediated and the humoral components.

CLI N I CAL M AN I FESTATI ONS3 M u cocutaneous M a n ifestations • Recurrent oral ulcers: Present in almost all BS patients. This is the

earliest sign of clisease and may precede other symptoms by years; most common sites are the gingival and buccal mucosa, tongues, and lips.

• As noted earlier, l-ISP and bacterial or viral antigens lead to stimu­

lation of T lymphocytes and oligoclonal expansion; these autoreac­ tive T cells appear to have a critical role in the pathogenic cascade of the disease .

Table 84- 1 summarizes the major clisturbances in the immune or hemostatic or coagulation and fibrinolytic systems in BS patients.

•

Genital ulcers : Occur in 5 7% to 93% of patients, commonly heal with scarring.

• T helper 1 (Tb 1 ) polarization of the immune response occurs in BS;

•

this seems to be integral to the mechanistic cascade that leads to the inflammatory state of BS. These TI-l l -primed cells overproduce

Most common site is the scrotum in males; vulvar, vaginal, and cervical lesions occur in females .

•

Cutaneous lesions : Occur i n 3 8 % t o 90% o f patients.

B E H