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Spinal Cavernous Malformations
By Jack Hoch, reviewed by Issam Awad, MD
 

Cavernous malformations are not limited solely to the brain. While not as common, spinal cavernous malformations do comprise a small, but important subset of those afflicted with the disease.

The composition of spinal cavernous malformations is the same as those found in the brain: thinly walled bubbled-shaped cavernous vessels which are underdeveloped and have little elasticity. There is very little, if any, intervening nerve tissue between the bubble-shaped caverns. Very small supplying arteries and draining veins may connect to the cavernous malformation.

Other characteristics:

- The MRI appearance of spinal cavernous malformations is generally the same as its cerebral counterparts. These lesions are still not visible to angiography.

- They can be located in vertebral elements, nerve roots, or epidural tissues, but by far the largest number are located in the spinal cord substance itself (intramedullary).

- Spinal cavernous malformations account for 5 to 12% of spinal vascular malformations.[1]

- They are slightly more common in women.

- Like their cerebral counterparts, spinal cavernous malformations tend to become symptomatic around the 3rd to 5th decade of life.

- An unspecified percentage of cavernous malformations remain asymptomatic.

- Symptomatic lesions may present with acute, episodic, or progressive deficits in functioning that correspond to the region of the spine affected by the cavernous malformation.

- In most cases, symptoms result from hemorrhage. Because the spinal cord is a small structure with many sensory and motor fibers crowded near a cavernous malformation, the risks of hemorrhage from a spinal lesion are high, often leading to serious disability or total paralysis.

- Pain is normally overshadowed by motor symptoms and is also present in about one half of cases.[2]

- Generally, surgery is recommended for symptomatic lesions.[2] Surgery is also considered for larger single lesions that are accessible to the surface of the spinal cord, or exophytic (bulging out from it).

- Radiosurgery offers no advantages versus having no treatment. This is analogous to brainstem cavernous malformations.[2]

- Surgery removing only part of the cavernous malformation frequently leads to a return of the cavernous malformation. There is up to a 66% potential re-bleeding rate.[3]

- Spinal operations are less problematic than brainstem ones.

- Most frequent complications from surgery ("acute surgical deterioration") are due to 'posterior column manipulation'(25% of patients) normally resulting in transient deficits. The posterior column is the section of the spinal cord that is closest to the skin. A portion of the posterior column runs the entire length of the spinal cord. The posterior column contains sensory fibers that conduct the position sense (of where joints are). Surgeons often must operate near this section of the spinal cord to reach a cavernous malformation.

- Approximately 5% of operations result in permanent deficits. (1988 thru 1997 studies: 47 patients of which 33 showed improvement, 12 had transient deficits, 2 with permanent deficits, 10 had subtotal resections, no deaths).[3]

- One study purports that intramedullary spinal cavernous malformations (IMSC- those lesions found within the spinal cord itself) may be suggestive of multiple cavernous malformations elsewhere in the “neuraxis” (spine and brain). Nearly 50% of patients in this particular study were found to harbor multiple cavernous malformations after initial IMSC diagnosis and additional MRI of the neuraxis.[4]

 

References
 

[1] Jellenger K: Vascular malformations of the central nervous system: a morphological overview. Neurosurg Rev 9:177-216, 1986.

[2] McCormick PC, Michelsen WJ, Post KD, et al: Cavernous malformations of the spinal cord. Neurosurgery 23:459-463, 1988.

[3] Connolly ES, McCormick PC: Intramedullary Vascular Malformations: Type II Glomus Aerteriovenous malformations and Cavernous malformations. Spinal Vascular Malformations 10:135-146, 1999.

[4] Vishteh AG, Zambramski JM, Spetzler RF: Patients with spinal cord cavernous malformations are at an increased risk for multiple neuraxis cavernous malformations. Neurosurgery, Vol.45, Nș 1, July 1999.