Book contents
- Uncommon Causes of Stroke
- Uncommon Causes of Stroke
- Copyright page
- Contents
- List of Contributors
- Preface
- PART I: INFECTIOUS AND INFLAMMATORY CONDITIONS
- PART II: HEREDITARY AND GENETIC CONDITIONS AND MALFORMATIONS
- 16 PULMONARY ARTERIOVENOUS MALFORMATIONS
- 17 HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER–WEBER–RENDU DISEASE)
- 18 CEREBRAL AUTOSOMAL DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY (CADASIL)
- 19 CEREBROVASCULAR COMPLICATIONS OF FABRY’S DISEASE
- 20 MARFAN’S SYNDROME
- 21 PSEUDOXANTHOMA ELASTICUM
- 22 EHLERS-DANLOS SYNDROME
- 23 PROGERIA
- 24 MELAS AND OTHER MITOCHONDRIAL DISORDERS
- 25 STURGE-WEBER SYNDROME
- 26 VON HIPPEL-LINDAU DISEASE
- 27 ANEURYSMS
- 28 ARTERIOVENOUS MALFORMATIONS OF THE BRAIN
- 29 CEREBRAL CAVERNOUS MALFORMATIONS AND DEVELOPMENTAL VENOUS ANOMALIES
- 30 CEREBROVASCULAR MANIFESTATIONS OF NEUROFIBROMATOSIS
- 31 MENKES DISEASE (KINKY HAIR DISEASE)
- 32 WYBURN-MASON SYNDROME
- PART III: VASCULAR CONDITIONS OF THE EYES, EARS, AND BRAIN
- PART IV: DISORDERS INVOLVING ABNORMAL COAGULATION
- PART V: SYSTEMIC DISORDERS THAT ALSO INVOLVE THE CEREBROVASCULAR SYSTEM
- PART VI: NONINFLAMMATORY DISORDERS OF THE ARTERIAL WALL
- PART VII: VENOUS OCCLUSIVE CONDITIONS
- PART VIII: VASOSPASTIC CONDITIONS AND OTHER MISCELLANEOUS VASCULOPATHIES
- PART IX: OTHER MISCELLANEOUS CONDITIONS
- Index
32 - WYBURN-MASON SYNDROME
from PART II: - HEREDITARY AND GENETIC CONDITIONS AND MALFORMATIONS
Published online by Cambridge University Press: 06 January 2010
Book contents
- Uncommon Causes of Stroke
- Uncommon Causes of Stroke
- Copyright page
- Contents
- List of Contributors
- Preface
- PART I: INFECTIOUS AND INFLAMMATORY CONDITIONS
- PART II: HEREDITARY AND GENETIC CONDITIONS AND MALFORMATIONS
- 16 PULMONARY ARTERIOVENOUS MALFORMATIONS
- 17 HEREDITARY HEMORRHAGIC TELANGIECTASIA (OSLER–WEBER–RENDU DISEASE)
- 18 CEREBRAL AUTOSOMAL DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY (CADASIL)
- 19 CEREBROVASCULAR COMPLICATIONS OF FABRY’S DISEASE
- 20 MARFAN’S SYNDROME
- 21 PSEUDOXANTHOMA ELASTICUM
- 22 EHLERS-DANLOS SYNDROME
- 23 PROGERIA
- 24 MELAS AND OTHER MITOCHONDRIAL DISORDERS
- 25 STURGE-WEBER SYNDROME
- 26 VON HIPPEL-LINDAU DISEASE
- 27 ANEURYSMS
- 28 ARTERIOVENOUS MALFORMATIONS OF THE BRAIN
- 29 CEREBRAL CAVERNOUS MALFORMATIONS AND DEVELOPMENTAL VENOUS ANOMALIES
- 30 CEREBROVASCULAR MANIFESTATIONS OF NEUROFIBROMATOSIS
- 31 MENKES DISEASE (KINKY HAIR DISEASE)
- 32 WYBURN-MASON SYNDROME
- PART III: VASCULAR CONDITIONS OF THE EYES, EARS, AND BRAIN
- PART IV: DISORDERS INVOLVING ABNORMAL COAGULATION
- PART V: SYSTEMIC DISORDERS THAT ALSO INVOLVE THE CEREBROVASCULAR SYSTEM
- PART VI: NONINFLAMMATORY DISORDERS OF THE ARTERIAL WALL
- PART VII: VENOUS OCCLUSIVE CONDITIONS
- PART VIII: VASOSPASTIC CONDITIONS AND OTHER MISCELLANEOUS VASCULOPATHIES
- PART IX: OTHER MISCELLANEOUS CONDITIONS
- Index
Summary
Wyburn-Mason syndrome (WMS), also known as the Bonnet-Dechaume-Blancsyndrome, is a rare non hereditary phakomatosis characterized by congenital retinal, orbital, and brainstem (usually midbrain) arteriovenous malformations (AVMs), and, less frequently, facial AVMs. With the increased availability of noninvasive brain imaging, intracranial AVMs are detected. Recognition of the association between the retinal and intracranial lesions is important because it allows early identification of the associated intracranial and facial AVMs. This chapter discusses the historical features, pathophysiology, and treatment options for WMS. Retinal AVMs usually do not grow or bleed, and are usually not responsible for significant visual loss. Due to their size and location, WMS AVMs are usually not amenable to surgical removal or radiosurgery. Embolization carries an increased risk because the lesions share a blood supply with vital brainstem structures. Therefore, patients are usually left untreated until the AVMs bleed, at which time heroic measures may be necessary.
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- Uncommon Causes of Stroke , pp. 231 - 234Publisher: Cambridge University PressPrint publication year: 2008