Cobb Syndrome

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Clinical Cobb Syndrome

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Synonym

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Cutaneomeningospinal angiomatosis

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Inheritance

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Sporadic

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Prenatal

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None

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Incidence

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Rare; spinal arteriovenous malformations (AVMs) more common without skin involvement, M=F

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Age at Presentation

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Birth; neurologic complications develop in early adulthood

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Pathogenesis

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Unknown

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Clinical

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Skin
Posterior thoracic/lumbar/limb vascular lesion in a dermatornal distribution a corresponding segment of spinal cord

Central Nervous System
Fast flow vascular malformation within the intramedullary (most common) spinal cord with secondary compression/anoxia secondary pain, weakness, muscle atro¬phy, and sensation loss below the level of compression; bladder and sphincter disfunction if malformation extensive; subarachnoid hemorrhage; malformation rna. involve vertebral body

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D/Dx

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Capillary malformation overlying a meningoencephalocele, spinal dysraphism, tethered spinal cord

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Lab

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MRI, MRA
Spinal angiography

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Management

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Referral to neurologist complete neurologic examination Referral to neurosurgeon extirpation of lesion, embolization

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Prognosis

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Dependent on degree of symptomatology prior to intervention may be irreversible