Hemangiomas and Vascular Malformations (eBook)

Foreword 6
Preface 7
Table of Contents 8
Contributors 12
Part I INTRODUCTION 14
1 Angiogenesis 15
Abstract 15
Introduction 15
The Angiogenic Process 16
Angiogenesis Anomalies 19
References 19
2 Historical Background 21
References 23
Part II HEMANGIOMAS 26
3 Hemangiomas of Infancy: Epidemiology 27
Abstract 27
Concepts 27
References 30
4 Classification of Infantile Hemangiomas and Other Congenital Vascular Tumors 32
Abstract 32
Introduction 32
Classification of Congenital Vascular Tumors 32
Stage/Type (“What”) 32
Infantile Hemangioma 32
Prodromal Phase 32
Clinical Symptoms 32
Differential Diagnoses 34
Initial Phase 34
Clinical Symptoms 34
Differential Diagnoses 35
Proliferation Phase 35
Clinical Symptoms 35
Differential Diagnosis 35
Maturation Phase 36
Clinical Symptoms 36
Differential Diagnosis 36
Regression Phase 36
Clinical Symptoms 36
Differential Diagnosis 36
Congenital Hemangioendothelioma (HE) 36
The Rapidly Involuting Congenital Hemangioendothelioma (RICH) 37
The Non-involuting Congenital Hemangioendothelioma (NICH) 38
The “Tufted Angioma” 38
The Kaposi-like Congenital Hemangioendothelioma (KHE) 38
Organ/Number/Localization (“Where”) 39
Organs 39
Number 39
Localization 41
Proliferation Pattern/Complications (“How”) 41
Growth Pattern 41
Complications 42
References 43
5 Diagnosis of Hemangiomas 44
Abstract 44
Introduction 44
Diagnosis of Hemangiomas is Based First on the Patient’s Medical History 44
Physical Examination 45
Imaging 45
References 46
6 Visceral Hemangiomas 47
Abstract 47
Introduction 47
Head and Neck Hemangiomas 47
Brain 47
Thyroid 48
Parotid 48
Thoracic Hemangiomas 49
Mediastinum 49
Lung 49
Pleura 49
Heart 49
Esophagus 50
Thymus 50
Abdominal Hemangiomas 50
Stomach 50
Intestine 50
Liver 50
Treatment 52
Spleen 53
Pancreas 53
Urinary Tract 53
Penis 53
Testicle 53
Female Genital Tract 54
Breast 54
Conclusion 54
References 55
7 Principles of Therapy of Infantile Hemangiomas and Other Congenital Vascular Tumors of the Newborns and Infants 56
Abstract 56
Introduction 56
Indication for Treatment of Congenital Vascular Tumors 57
Spontaneous Course 57
Induction of Regression 57
Local Procedures 58
Physical Procedures 58
Cryotherapy 58
Scarification Techniques 58
Laser Therapy 59
X-Ray Therapy 60
Mechanical Procedures 60
Compression 60
Ligation 60
Embolization 61
Chemical Procedures 61
Sclerotherapy 61
Interstitial Magnesium Seeds 61
Interstitial Corticoid Crystals 61
Imiqiumod (Aldara®) 61
Systemic Procedures 61
Antiproliferative Drugs 61
Antiangiogenesis 61
Removal 62
Early Complications 62
Residuals 62
References 63
8 Medical Treatment of Hemangiomas 64
Abstract 64
Introduction 64
Corticosteroids 64
Vincristine 67
Interferon 67
Cyclophosphamide 68
Bleomycin 69
Perspectives: New Anti-Angiogenic Drugs 69
References 69
9 Laser Therapy of Infantile Hemangiomas and Other Congenital Vascular Tumors of Newborns and Infants 71
Abstract 71
Introduction 71
Laser Therapy 71
Superselective Laser Systems 71
Flash Lamp Pumped Pulsed Dye Laser “FLPD Laser” 72
Frequency Doubled Nd:YAG Laser (“KTP”) 72
Pulsed Nd:YAG Laser 72
Continuous Wave (cw-Nd:YAG) Lasers 74
Transcutaneous Direct Application 75
Transcutaneous Application with Fluid Cooling Cuvette 75
Transcutaneous Application with Continuous Ice Cube Cooling 76
Impression Technique 79
Interstitial Puncture Technique 80
Endoscopy 82
References 83
10 Surgery of Hemangiomas 84
Abstract 84
Introduction 84
Indications and Timing for Surgical Treatment 84
Techniques 85
Postoperative Care 87
Conclusion 87
References 88
11 Facial Hemangiomas 89
Abstract 89
Face 89
Diagnosis 89
Treatment 90
Eye 91
Nasal Tip 91
Filtrum and Lips 92
Parotid 92
Segmental Forms and PHACES 92
Rapid Involuting Hemangioma/Non Involuting Hemangioma 93
Kaposiform Hemangioendothelioma/ Tufted Angioma 93
Psycho-Social Impact 93
References 94
12 Anogenital Hemangiomas 96
Abstract 96
Diagnosis 96
Complications 97
Prevention and Therapy 97
References 99
Part III VASCULAR MALFORMATIONS 100
13 Genetic Aspects of Vascular Malformations 101
Abstract 101
Introduction 101
Capillary Malformation (CM) 102
Capillary Malformation-Arteriovenous Malformation (CM-AVM) 103
Cerebral Cavernous Malformation (CCM) 103
Venous Anomalies 104
Venous Malformation (VM) 104
Cutaneomucosal Venous Malformation (VMCM) 104
Glomuvenous Malformation (GVM) 105
Lymphatic Malformations (LM) 106
Lymphedema 106
Arteriovenous Malformation (AVM) 107
Hereditary Hemorrhagic Telangiectasia (HHT) 107
PTEN Hamartoma Tumor Syndrome (PHTS) 107
Conclusion 108
References 108
14 Epidemiology of Vascular Malformations 111
Abstract 111
Incidence of Vascular Malformations 111
Distribution of Vascular Malformations 111
Distribution by Gender 111
Time of Appearance 112
Body Location of VMs 112
References 112
15 Classification of Vascular Malformations 113
Abstract 113
Introduction 113
References 116
16 Non Invasive Diagnostics of Congenital Vascular Malformations 117
Abstract 117
Introduction 117
Functional Tests 117
Morphofunctional Tests 117
Imaging 119
References 122
17 Nuclear Medicine in Diagnostics of Vascular Malformations 123
Abstract 123
Introduction 123
Whole Body Blood Pool Scintigraphy (WBBPS) 123
Lymphoscintigraphy 127
Transarterial Lung Perfusion Scintigraphy (TLPS) (or “Microspheres” Study) 131
References 134
18 Invasive Diagnostics of Congenital Vascular Malformations 136
Abstract 136
Introduction 136
Clinical Signs 136
Plain Film 136
Morphologic and Functional Aspects 136
Phlebography 142
Arteriography 142
Lymphography 142
References 144
19 Principles of Treatment of Vascular Malformations 145
Abstract 145
Truncular CVM 145
Extratrucular CVM 146
Support Therapy 151
Pain Therapy 151
Edema Therapy 151
Deep Venous Thrombosis Prevention 151
References 151
20 Interventional Therapy in Arteriovenous Congenital Malformations 152
Abstract 152
Introduction 152
Embolizing Materials 152
Catheter Devices 153
Strategies and Tactics 155
Special Interventional Techniques 155
Combined Therapy 159
References 160
21 Diagnosis and Management of Soft Tissue Vascular Malformations with Ethanol 162
Abstract 162
Introduction 162
Concepts in Patient Management 163
Endovascular Therapy of Vascular Malformations 164
Arteriovenous Malformations 164
Arteriovenous Fistulae 165
Venous Malformations 165
Lymphatic Malformations 166
Intralipomatous Vascular Malformations 167
Conclusion 167
References 167
22 Sclerotherapy in Vascular Malformations 169
Abstract 169
Introduction 169
Indications 169
Sclerosing Agents 170
Technique of Classic Sclerotherapy 170
Sclerotherapy with Microfoam 171
Technique of Treatment with Microfoam in Vascular Malformations 171
Sclerotherapy of Lymphatic Malformations with OK-432 177
References 177
23 Laser Therapy of Vascular Malformations 178
Abstract 178
Introduction 178
Capillary Malformations 179
Port-Wine Stains (PWS) 179
Capillary-Lymphatic Malformation 180
Hyperkeratotic Capillary Malformation 180
Port-Wine Stains with Associated Vascular Malformations (Neurocutaneous Syndromes or Phakomatoses) 181
Nonfading Telangiectasias 181
Cutis Marmorata Telangiectatica Congenita (Van Lohuizen Syndrome) 181
Rendu-Osler-Weber Syndrome (Hereditary Hemorrhagic Telangiectasia) 182
Other Telangiectasies 183
Lymphatic Malformations 183
Truncular Lymphatic Malformation Cystic Hygromas 184
Extratruncular Lymphatic Malformation 184
Intraluminal (Intracystic) Technique 184
Microcystic (Solid) Lymphatic Malformation “ITT” 185
Bare Fiber Contact Vaporization 185
Endoscopic Coagulation 185
Venous Malformations 186
Truncular Venous Malformation 186
Extratruncular Venous Malformation 186
Soft Tissue Phlebectasias 186
Cutaneous/Subcutaneous Malformation 187
Mucous Membrane Affection 188
Glomuvenous Malformation (“Glomangioma”) 188
Arterial Malformations 188
Truncular Arterio-Venous Fistula 188
Combined Truncular/Extratruncular Arterio-Venous Malformation 189
References 190
24 The Combined Treatment of Arteriovenous Malformations 191
Abstract 191
Introduction 191
Non-Surgical Methods of Treatment 191
Surgical Methods of Treatment 192
Preoperative Embolization 192
Intraoperative Embolization 194
Postoperative Embolization 194
References 200
25 Multidisciplinary Surgical Treatment of Vascular Malformations 201
Abstract 201
Introduction 201
Muscle 201
Joints 202
Intraosseous 202
Hand 202
Skin 202
Head and Neck 203
Pelvis 204
Conclusion 204
Suggested Readings 204
26 Treatment of Arterial Malformations 205
Abstract 205
Introduction 205
Aneurysms 205
Stenosis, Hypoplasia and Aplasia 206
Arterial Course Anomalies 207
References 208
27 Treatment of Arteriovenous Malformations 210
Abstract 210
Introduction 210
Therapeutic Options 210
Treatment Strategy 210
Type of AVM 210
Symptoms 213
Evolution 215
Conclusion 215
References 216
28 Treatment of Venous Malformations 217
Abstract 217
Introduction 217
Conservative Treatment 217
Sclerotherapy 217
Classic Sclerosis 217
Foam Sclerosis 218
Absolute Alcohol Sclerosis 218
Endovascular Catheter Embolization 219
Laser Treatment 220
Surgery 220
Truncular Forms 220
Treatment of Venous Malformations 222
Extratruncular Forms 222
References 223
29 Treatment of Lymphatic Malformations 225
Abstract 225
Introduction 225
General Principles and Guidelines 225
Treatment Modalities 234
OK-432 Sclerotherapy 234
Ethanol Sclerotherapy 236
Post Sclerotherapy Care of the Lesion/Patient 236
Surgical/Excisional Therapy 237
Clinical Experiences – Predominant (Truncular and Extratruncular) LM Lesions 239
Extratruncular Lesion: Lymphangioma 239
Truncular Lesion: Primary Lymphedema 239
Management of LM Belonging to HLM 239
Clinical Experiences – HLM lesion 242
Prospects 242
Conclusion 242
References 243
30 Treatment of Vascular Malformations in Newborns and Infants 245
Abstract 245
Introduction 245
Indication for Therapy and Treatments 246
Arterial Malformations 246
Truncular Forms 246
Coarctation of the Aorta Descendens 246
Indications for Therapy 246
Techniques 246
Arterial Aneurysms 247
Indications for Therapy 247
Techniques 247
Extratruncular Forms 248
Port-Wine Stains (PWS), Salmon Patch – Nevus Flammeus 248
Nevus Flammeus Neonatorum (Nevus Teleangiectaticus Medialis) 248
Indication for Therapy 248
Techniques 249
Venous Malformations 249
Truncular forms 249
Aplasia or Obstruction, Dilatation of Deep Veins 249
Indications for Therapy 250
Techniques 250
Treatment of Discrepancy in the Length of Lower Limbs 250
Elongation of Limbs with the Ilizarov Technique 251
Obstruction of Inferior Vena Cava – “Cava Membrane Disease” (Budd-Chiari Syndrome) 252
Indications for Therapy 252
Techniques 252
Venous Aneurysms and Aneurysms of Vena Jugularis Interna 252
Indications for Therapy 252
Techniques 252
Extratruncular Forms: Infiltrating and Limited Venous Malformations 253
Cutaneous, Subcutaneous and Muscular Infiltrating Venous Malformations (“Glomovenous Malformations”) 253
Indication for Therapy 253
Techniques 253
Limited Venous Malformation 255
Indication for Therapy 255
Techniques 255
Lymphatic Malformations 255
Truncular Forms 255
Lymphoedema – Aplasia or Obstruction (Hypoplasia, Stenosis) of Lymphatic Vessels 255
Indications for Therapy 255
Techniques 255
Soft Lymphoedema – Dilatation of the Lymphatics 256
Indications for Therapy 256
Techniques 256
Chylous Ascites 257
Indications for Therapy 258
Techniques 258
Congenital Chylothorax 258
Indications for Therapy 259
Techniques 259
Extratruncular Forms: Infiltrating and Limited Lymphatic Malformations 259
Lymphangiomas 259
Indications for Therapy 259
Techniques 259
Cystic Hygroma 259
Indications for Therapy 259
Techniques 259
Arteriovenous Malformations 262
Truncular Forms: Deep and Superficial AV Fistulae 262
Ductus Arteriosus Botalli (Deep, Direct (Central) AV Shunt) 262
Indications for Therapy 262
Techniques 262
Superficial, Indirect (Peripheral) AV Shunts (Parkes-Weber Syndrome) 262
Indication for Therapy 262
Techniques 262
Extratruncular Forms: Infiltrating and Limited AV Fistulae 263
Infiltrating Multiplex AV, Microcommunications (Parkes-Weber Syndrome) 263
Indication for Therapy 263
Techniques 263
Limited Tumour Form AV Malformation 263
Indications for Therapy 263
Techniques 263
Combined Vascular Malformations 265
Indication for Therapy 265
Techniques 265
References 266
Part IV TREATMENT PROBLEMS ACCORDING TO SPECIFIC LOCALIZATIONS 267
31 Introductory Remarks 268
32 Management of Head and Neck Vascular Malformations: an Overview 269
Abstract 269
Introduction 269
What is a Vascular Malformation? 269
Vascular Malformation and Vascular Tumors 270
Considerations in Head and Neck Vascular Malformation Treatment 270
Specific HNVM 272
Lymphatic Malformations 272
Venous Malformations 274
Arteriovenous Malformations 274
Capillary Malformations 275
References 276
33 Surgical Treatment of Vascular Malformations in the Hand 278
Abstract 278
Introduction 278
General Principles of CVM Surgery in the Hand 278
Skin Incisions 278
Skin Sparing Technique 279
Use of a Tourniquet 279
Microscope 280
Tissue Involvement: Clinical Pictures and Surgical Treatment 280
Skin Involvement 280
Bone Involvement 280
Nerves 280
Muscles 282
Tendons 282
Conclusion 282
References 283
34 Management of Vascular Malformations in the Thorax Wall 284
Abstract 284
Introduction 284
Venous Malformations 284
Lymphatic Malformations 285
Arteriovenous Malformations 286
References 287
35 Joint Involvement in Patients with Vascular Malformations. Destructive Angiodysplastic Arthritis 289
Abstract 289
Introduction 289
Classification and Therapeutic Approach of Joint Pathologies 290
Destructive Angiodysplastic Arthritis – Stage 1 290
Destructive Angiodysplastic Arthritis – Stage 2 290
Destructive Angiodysplastic Arthritis – Stage 3 290
Conclusion 291
References 293
36 Surgical Approach to Congenital Vascular Malformations (CVM) in the Foot 294
Abstract 294
Arteries 294
Veins 296
Lymphatic Vessels 298
Compartments of the Foot 298
Observations 300
References 306
37 Diagnosis and Management of Vascular Malformations of Bone 307
Abstract 307
Introduction 307
Etiology 307
High-Flow Lesions of Bone 308
Venous Malformations of Bone 309
References 311
38 The Role of Syndromes 313
Abstract 313
Introduction 313
Capillary Malformations 313
Lymphatic Malformations 314
Venous Malformations 314
Arterio-Vascular Malformations (AVMs) 314
Arterial Malformations 314
References 314
Conclusions 316
Subject Index 317