Contents. 1 Embryological and Anatomical Introduction... 1

Transcription

1 1 Embryological and Anatomical Introduction Preliminary Remarks Leptomeninges Subpial Space Anatomy Relationships of the Subpial Space Pathology Inflammation Tumors Introduction and General Comments Regarding Pediatric Intracranial Arteriovenous Shunts Introduction From Adults to Children Vascular Lesion Types and Disease Groups Nonproliferative Lesions Arteriovenous Lesions Isolated Brain AVMs Cerebral Arteriovenous Fistulas Vein of Galen Aneurysmal Malformations Cerebrofacial Arteriovenous Metameric Syndromes Dural Lesions Telangiectasias The Blue Rubber-Bleb Nevus or Bean Syndrome Venous Malformations (Cavernomas) Venous Angiomas or Developmental Venous Anomalies Cerebrofacial Venous Metameric Syndrome (Sturge-Weber Syndrome) Induced Pial Shunts Spinal Cord AVM General Conclusions on Vascular Lesions Proliferative Lesions PHACE or PHACES Diffuse Angiodysplasia

2 XII 2.4 Classification of CAVMs by Age Group Fetal Age Neonatal Age Infancy After 2 Years Classification by Symptom Group Congestive Cardiac Manifestations Hydrodynamic Disorders Melting-Brain Syndrome Clinical Evaluation Scores Revised Concept of the Congenital Nature of Vascular Malformations Genetics Familial Hemiplegic Migraine Familial Cerebral Aneurysms PKD1 and Bourneville PDK1-PDK Ehlers-Danlos Type IV Multiple Cutaneous Mucous Venous Malformations, Blue Rubber Bleb Nevus Syndrome CADASIL Familial Paragangliomas Familial Cavernomas Neurofibromatosis-1 and Other Collagen Diseases Hemorrhagic Hereditary Telangiectasia or Rendu-Osler-Weber Disease Vascular Remodeling and the Congenital Nature of Arteriovenous Shunts Endothelium as a Sensor and Transducer of Signals Endothelium-Specific Receptor-Coupled Event Endothelium and Mediator-Effector Molecules Involved with Remodeling Role of Matrix Modulators in Vascular Remodeling Clinical Implications of Vascular Remodeling Vein of Galen Aneurysmal Malformation Introduction Historical Landmarks Lesions Clinical Aspects Modern Concept of Vein of Galen Aneurysmal Malformation Vein of Galen Aneurysmal Dilatation Dural Arteriovenous Shunts with Aneurysmal Dilatation of the Vein of Galen Vein of Galen Varix

3 XIII 3.7 Vein of Galen Aneurysmal Malformation Natural History of Vein of Galen Aneurysmal Malformation Cardiac Manifestations Macrocrania and Hydrocephalus Late Natural History of Vein of Galen Aneurysmal Malformation with Patent Sinuses Dural Sinus Occlusion and Supratentorial Pial Congestion and Reflux Dural Sinus Thrombosis and Infratentorial Pial Reflux Spontaneous Thrombosis Objectives and Methods of Treatment General Remarks Neonates Reducing Oxygen Consumption Improving Oxygen Delivery Infants and Children Technical Management General Remarks Follow-Up Complications: Morbidity Overall Mortality Neurological Outcome by Age Group Other Techniques Surgery Transvenous Treatment Radiosurgery Cerebral Arteriovenous Fistulas Definitions and Anatomic Spaces Angioarchitecture Single CAVFs Multiple CAVFs Associated Conditions Hereditary Hemorrhagic Telangiectasia Encephalocraniocutaneous Lipomatosis Presentation Natural History Management

4 XIV 5 Cerebral Arteriovenous Malformations General Remarks Angioarchitecture of Cerebral Arteriovenous Malformations Single Nidus Versus Multifocal Niduses Conditions Associated with CAVMs Conditions Mimicking CAVMs False Pial Arteriovenous Malformations Perinidal Angiogenesis Postischemic Luxury Perfusion Proliferative Angiopathy Induced Pial AV Shunts Secondary to Dural Sinus High-Flow Lesions Angioarchitectural Progression of CAVMs in Children Venous Angiopathy Dural Sinus High Flow Venous Ischemia and Thrombosis Venous Hemorrhage Venous Enlargement Arterial Angiopathy Spontaneous Thrombosis of Arteriovenous Malformations Objectives of Treatment Complete Exclusion Partial Treatment Neonates and Infants Hydrodynamic Disorders Multiple Arteriovenous Malformations Children Rebleeding Technical Management General Remarks Other Techniques Surgery Radiation Therapy Cerebrofacial Arteriovenous Metameric Syndrome Introduction Clinical Manifestations Retinal AVMs and AVMs Along the Optic Nerve and Chiasm Retinal AVMs Optic Nerve and Chiasmatic AVMs

5 XV Cerebral AVMs Facial AVMs, Nasal AVMs, and Mandibular AVMs Investigation for CAMS Patients CAMS and Angiogenic Activity Dural Arteriovenous Shunts Introduction Classification Age Groups Disease Groups Dural Sinus Malformations DSM with Giant Pouches Fetal and Postnatal Changes of Sinuses DSM of the Jugular Bulb Infantile Dural Arteriovenous Shunts (AVS) Adult Type of Dural Arteriovenous Shunts in Children Other Dural Shunts Vein of Galen Aneurysmal Malformation Dural Supply to Pial Cerebral Arteriovenous Malformations Proliferative Angiopathic Disease Systemic Disorders Recurrence in Intradural AVS and Secondary Transdural Supply General Remarks on Treatment Venous Anomalies and Malformations Developmental Venous Anomalies Single Abnormalities Associated Features Associated Cavernomas Segmental and Nonsegmental Cerebro-orbito-facial Venous Lesions Sturge-Weber Syndrome From SWS to Cerebrofacial Venous Metameric Syndrome Orbitofacial Venous Lesions Complex Pseudo-metameric Cerebrofacial Venous Syndrome Blue Rubber Bleb Nevus (Bean Syndrome) The Association of BRBN with DVA Cerebral Venous Malformations in BRBN BRBN and HHT

6 XVI 9 Craniopagus and Cranial Midline Epidural Venous Anomalies Introduction Postulated Relationships Between the Superior Sagittal Sinus and Adjacent Structures Ladan and Laleh s Angiographic Anatomy Technical Remarks and Functional Testing Discussion on Surgical Management Cerebral Venous Thrombosis Introduction Pathophysiology and Risk Factors Imaging Symptoms Treatment Outcome Hemangiomas Introduction Pathogenesis Histological Findings Clinical Presentation of Hemangiomas Diagnosis Complications in Hemangiomas Management of Hemangiomas Pharmacological Therapy of Hemangiomas Corticosteroids Interferon-Alpha 2a Vincristine Aminocaproic Acid Other Treatments Laser Treatment of Hemangiomas Endovascular Treatment of Hemangiomas Arterial Embolization Intralesional Embolization Noninvoluting Capillary Hemangiomas Subglottic Hemangiomas Periorbital Hemangiomas

7 XVII Oral Hemangiomas Salivary Gland Hemangioma Bone Hemangiomas Associated Anomalies Psychological Impact Kaposiform Hemangioendothelioma and Consumption Coagulopathy, the Kasabach-Merritt Syndrome Phenomena PHACES Introduction Clinical Aspects Posterior Fossa Abnormalities Hemangiomas Arterial Anomalies Coarctation and Congenital Heart Disease Eye Abnormalities Sternal Cleft Stenotic Arterial Disease PHACES, a Congenital Malformation and a Proliferative Disease Cervicofacial Vascular Malformations Introduction Traditionally Postulated Embryogenesis Diagnostic and Pretherapeutic Evaluation Clinical Diagnosis of a Vascular Malformation Arteriovenous Shunts Soft Tissue AVMs Intramuscular AVMs Cutaneous AVMs Intra-osseous AVMs Mandibular and Maxillary AVMs Signs and Symptoms Metameric Cerebrofacial AVMs Intra-osseous Slow-Flow Malformations Arteriolar-Capillary Malformations Capillary Venous Malformations Venous Vascular Malformations

8 XVIII Complex Cerebrofacial Venous Syndromes (CVMS or Sturge-Weber Syndrome) Lymphatic Malformations Mixed Vascular Malformations Multifocal AVMs False Maxillofacial Vascular Malformations Idiopathic Facial Vascular (Venous) Dilatations Facial Venous Dilatation Associated with Intracranial Vascular Lesions Parachordal Arteriovenous Fistulas (Extracranial and Extraspinal Arteriovenous Fistulas) Introduction Specific Clinical Features Topographic Approach Branchial Arteriovenous Shunts Maxillary Artery/Vein Arteriovenous Fistulas Ascending Pharyngeal-Internal Jugular Arteriovenous Fistulas Vertebro-vertebral Arteriovenous Fistulas Paraspinal Arteriovenous Fistulas Technical Management of High-Flow Fistulas Spinal Cord Arteriovenous Malformations Introduction Classification Natural History and Clinical Aspects Neonatal and Infants Children Over 2 Years of Age Diagnosis Angioarchitecture Treatment Therapeutic Abstention Embolization Results

9 XIX 16 Vascular Trauma and Epistaxis Introduction Traumatic Carotid-Cavernous Fistulas Post-traumatic Sinus Thrombosis Traumatic Dissections Intracranial Arterial Aneurysms Iatrogenic Injury Traumatic Insult of Vascular Malformation Epistaxis Technical Remarks Intracranial Aneurysms in Children Introduction Incidence Presentation Etiology Traumatic Aneurysms Infectious Aneurysms Saccular Aneurysms Dissecting Aneurysms Location Therapeutic Strategies Arterial Ischemic Stroke Introduction Epidemiology Pathophysiology Clinical Presentation Imaging of Arterial Stroke in Children Outcome and Prognosis Etiology Cardiac Disorders Acute Regressive Cerebral Arteriopathy Dissections Moyamoya Disease

10 XX Hematological Disorders and Coagulopathies Metabolic Disorders Proliferative Angiopathy PHACE Hereditary Hemorrhagic Telangiectasia, or Rendu-Osler-Weber Disease Spinal Cord Strokes Treatment and Management References Subject Index