Vascular Myelopathies - Vascular Malformations of the Spinal Cord: Presentation and Endovascular Surgical Management

Louis P. Caragine, Jr., MD, PhD, Van V. Halbach, MD, Perry P. Ng, MD, Christopher F. Dowd, MD


Semin Neurol. 2002;22(2) 

In This Article

Metameric Angiomatosis (Cobb's Syndrome) and Disseminated Angiomatosis (Osler-Weber-Rendu Syndrome)

In cases of metameric angiomatosis there is a well-defined spinal AVM with involvement of the dura, vertebrae, muscular wall, skin, and even viscera. Cobb's syndrome presents in a number of ways: (1) flat, metameric angiomas involving the thoracic spine and chest or cervical or lumbar spine and extremities; (2) flat, metameric angiomas associated with subarachnoid hemorrhage; and (3) superficial arteriovenous angiomatosis of the trunk or extremities associated with subarachnoid hemorrhage.[22] It is difficult to achieve complete cure. Embolization, surgery, and percutaneous vertebroplasty may be used in combination or alone, depending on the symptomatology.

In disseminated angiomatosis, vascular telangiectasias may involve the skin of the face and/or hands or mucous membranes of the respiratory and/or gastrointestinal tract. Often the main presenting feature is epistaxis. Pulmonary angiomatosis is less common but may present with septic emboli, paradoxical emboli, or pulmonary fistula. Capillary telangiectasias, arteriovenous fistulas, AVMs, capillary angiomas, and intercranial aneurysms may also be found in the brain or spinal cord, or both.