Spinal cord cavernous malformation
Updates to Article Attributes
Spinal cavernous malformations (spinal cavernomas) are vascular malformations that occur within the spinal cord.
This article specifically relates to spinal cord cavernomas. For a discussion of cerebral cavernomas and for a general discussion of the pathology refer to the main articlecerebral cavernous malformation.
Epidemiology
Spinal cord cavernomas are rare, representing approximately 5% of intramedullary lesions in adults and 1% of intramedullary lesions in children 3.
Peak presentation is during the fourth decade, which is similar to the peak incidence of cerebral cavernomas 4. Females are more commonly affected than males.
Clinical presentation
Minimal cord expansion or oedema unless there has been recent haemorrhage.
Common presenting symptoms include pain, weakness and paraesthesias.
Four patterns of clinical presentation have been described 4:
- discrete episodes of neurological deterioration with varying degrees of recovery between episodes
- slow progression of neurological decline
- acute onset of symptoms with rapid decline
- acute onset of mild symptoms with subsequent gradual decline lasting weeks to months
Episodes of haemorrhage have been proposed as the mechanism underlying acute episodes of neurological deterioration. Progressive myelopathy may be caused by microhaemorrhages and gliosis.
Pathology
Like intracranial cavernomas, spinal cavernomas consist of blood-filled endothelial-lined spaces lined by thickened, hyalinised walls that lack elastic fibres and smooth muscle 5.
Radiographic features
More than half of spinal cavernomas are located in the thoracic cord. The second most common location is the cervical cord 4.
CT
Often
-
often occult 3
.
Angiography
Often
-
often occult 3
.
MRI
- rounded regions of heterogeneous signal intensity on T1 and T2 weighted images due to blood products of varying ages (“popcorn appearance”)
- low signal intensity rim on T2 weighted images (haemosiderin)
-
gradient
echoecho (GE): there can be hypointense “blooming” on gradient echo sequences (haemosiderin) - T1 C+ (Gd): may demonstrate minimal enhancement on post-contrast images
There is minimal cord expansion or oedema unless there has been recent haemorrhage
Treatment and prognosis
Early surgical resection should be considered for all symptomatic patients, before repeated haemorrhage or enlargement occur 4.
-<p><strong>Spinal cavernous malformations </strong>(<strong>spinal cavernomas</strong>) are vascular malformations that occur within the spinal cord.</p><p>This article specifically relates to spinal cord cavernomas. For a discussion of cerebral cavernomas and for a general discussion of the pathology refer to the main article <a href="/articles/cerebral-cavernous-venous-malformation">cerebral cavernous malformation.</a></p><h4>Epidemiology</h4><p>Spinal cord cavernomas are rare, representing approximately 5% of intramedullary lesions in adults and 1% of intramedullary lesions in children <sup>3</sup>.</p><p>Peak presentation is during the fourth decade, which is similar to the peak incidence of cerebral cavernomas <sup>4</sup>. Females are more commonly affected than males.</p><h4>Clinical presentation</h4><p>Minimal cord expansion or oedema unless there has been recent haemorrhage.</p><p>Common presenting symptoms include pain, weakness and paraesthesias.</p><p>Four patterns of clinical presentation have been described <sup>4</sup>:</p><ul>- +<p><strong>Spinal cavernous malformations </strong>(<strong>spinal cavernomas</strong>) are vascular malformations that occur within the spinal cord.</p><p>This article specifically relates to spinal cord cavernomas. For a discussion of cerebral cavernomas and for a general discussion of the pathology refer to the main article <a href="/articles/cerebral-cavernous-venous-malformation">cerebral cavernous malformation.</a></p><h4>Epidemiology</h4><p>Spinal cord cavernomas are rare, representing approximately 5% of intramedullary lesions in adults and 1% of intramedullary lesions in children <sup>3</sup>.</p><p>Peak presentation is during the fourth decade, which is similar to the peak incidence of cerebral cavernomas <sup>4</sup>. Females are more commonly affected than males.</p><h4>Clinical presentation</h4><p>Minimal cord expansion or oedema unless there has been recent haemorrhage.</p><p>Common presenting symptoms include pain, weakness and paraesthesias.</p><p>Four patterns of clinical presentation have been described <sup>4</sup>:</p><ul>
-</ul><p>Episodes of haemorrhage have been proposed as the mechanism underlying acute episodes of neurological deterioration. Progressive myelopathy may be caused by microhaemorrhages and gliosis.</p><h4>Pathology</h4><p>Like intracranial cavernomas, spinal cavernomas consist of blood-filled endothelial-lined spaces lined by thickened, hyalinised walls that lack elastic fibres and smooth muscle <sup>5</sup>.</p><h4>Radiographic features</h4><p>More than half of spinal cavernomas are located in the thoracic cord. The second most common location is the cervical cord <sup>4</sup>.</p><h5>CT</h5><p>Often occult <sup>3</sup>.</p><h5>Angiography</h5><p>Often occult <sup>3</sup>.</p><h5>MRI</h5><ul>- +</ul><p>Episodes of haemorrhage have been proposed as the mechanism underlying acute episodes of neurological deterioration. Progressive myelopathy may be caused by microhaemorrhages and gliosis.</p><h4>Pathology</h4><p>Like intracranial cavernomas, spinal cavernomas consist of blood-filled endothelial-lined spaces lined by thickened, hyalinised walls that lack elastic fibres and smooth muscle <sup>5</sup>.</p><h4>Radiographic features</h4><p>More than half of spinal cavernomas are located in the thoracic cord. The second most common location is the cervical cord <sup>4</sup>.</p><h5>CT</h5><ul><li>often occult <sup>3</sup>
- +</li></ul><h5>Angiography</h5><ul><li>often occult <sup>3</sup>
- +</li></ul><h5>MRI</h5><ul>
-<strong>gradient echo (GE):</strong> there can be hypointense “blooming” on gradient echo sequences (haemosiderin)</li>- +<strong>gradient echo (GE):</strong> there can be hypointense “blooming” on gradient echo sequences (haemosiderin)</li>
Image 3 MRI (T2) ( create )
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