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January Case of the Month

Clinical History:

64 y/o male with intermittant leg weakness and incontinence. No pain or radicular complaints.

View Diagnosis

Primary Diagnosis

Cavernous malformation of the spinal cord, aka cavernoma.

Secondary Diagnosis

N/A

Diagnosis Discussion

Intramedullary cavernomas of the cord are quite uncommon and account for between 3 and 12% of all vascular lesions of the spinal cord. Little is known of their natural history, although their tendency to produce hemorrhage and episodic symptomatic worsening is recognized. These are very uncommon; estimated incidence is 0.02-4% of the population with 3-5% occuring in the spinal cord. They are congenital and enlarge with time. There is slow flow within these cavernomas with no arterial-venous shunting. Unlike cavernomas in the brain, there is a 70% prediliction for spinal cord cavernomas in females. Can be solitary or multiple. Single lesion is usually sporadic, multiple lesions think of the familial (autosomal dominant) form. Typically present clinically in the fourth decade. Four type of presentations, acute episode with stepwise decline, acute onset with rapid deterioration, acute onset with mild deficit and gradual deterioration and slowly progressive neurologic decline. Acute symptoms attributed to hemorrhage, with back pain and delayed neurologic deterioration. Hemorrhage rate is unknown but is likely less than cerebral cavernomas (0.5 - 1.0% per year). Appearance on imaging is typical. Lobulated mass with a low signal peripheral hemosiderin rim. Variable degree of internal enhancement. When edema is present, it is likely produced by microhemorhage into parenchyma. Treatment is conservative initially. Microsurgical resection may be necessary in instances where neurologic deterioration occurs.

General Anatomic Area:

Thoracic

Secondary Anatomic Area:

conus medullaris

Disease Category:

Vascular

Contributor:

Adam Flanders, MD

Date:

01/01/2005

Difficulty Level:

Easy

Pathology Confirmed?:

No
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ACR Category Number:

N/A

Keywords:

cavernous malformation, OCVM, cavernoma,vascular malformation, occult