Spinal cord cavernous malformation
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- updated 11 May 2022:
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Spinal cord cavernous malformations, also known as 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 a general discussion of the pathology refer to the main article cerebral cavernous malformation.
Epidemiology
Spinal cord cavernomas are rare, representing ~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
Common presenting symptoms include pain, weakness and paresthesiasparaesthesias. 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 micro haemorrhages 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.
Site
More than half of spinal cavernomas are located in the thoracic cord. The second most common location is the cervical cord 4.
Radiographic features
CT
- often occult 3
Angiography
- often occult 3
MRI
Minimal cord expansion or oedema unless there has been recent haemorrhage.
- 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 echo (GE): there can be hypointense “blooming” on gradient echo sequences (haemosiderin)
- T1 C+ (Gd): may demonstrate minimal enhancement on post-contrast images
Treatment and prognosis
Early surgical resection should be considered for all symptomatic patients before repeated haemorrhage or enlargement occur 4.
-<p><strong>Spinal cord cavernous malformations</strong>, also known as <strong>spinal cavernomas,</strong> are vascular malformations that occur within the <a href="/articles/spinal-cord">spinal cord</a>.</p><p>This article specifically relates to spinal cord cavernomas. For a discussion of cerebral cavernomas and 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 ~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>Common presenting symptoms include pain, weakness and paresthesias. Four patterns of clinical presentation have been described <sup>4</sup>:</p><ul>-<li>discrete episodes of neurological deterioration with varying degrees of recovery between episodes</li>-<li>slow progression of neurological decline</li>-<li>acute onset of symptoms with rapid decline</li>-<li>acute onset of mild symptoms with subsequent gradual decline lasting weeks to months</li>-</ul><p>Episodes of haemorrhage have been proposed as the mechanism underlying acute episodes of neurological deterioration. Progressive myelopathy may be caused by micro haemorrhages 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><h5>Site</h5><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><h4>Radiographic features</h4><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><p>Minimal cord expansion or oedema unless there has been recent haemorrhage. </p><ul>-<li>rounded regions of heterogeneous signal intensity on T1 and T2 weighted images due to blood products of varying ages (“popcorn appearance”)</li>-<li>low signal intensity rim on T2 weighted images (haemosiderin)</li>-<li>-<strong>gradient echo (GE):</strong> there can be hypointense “blooming” on gradient echo sequences (haemosiderin)</li>-<li>-<strong>T1 C+ (Gd):</strong> may demonstrate minimal enhancement on post-contrast images</li>- +<p><strong>Spinal cord cavernous malformations</strong>, also known as <strong>spinal cavernomas,</strong> are vascular malformations that occur within the <a href="/articles/spinal-cord">spinal cord</a>.</p><p>This article specifically relates to spinal cord cavernomas. For a discussion of cerebral cavernomas and 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 ~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>Common presenting symptoms include pain, weakness and paraesthesias. Four patterns of clinical presentation have been described <sup>4</sup>:</p><ul>
- +<li>discrete episodes of neurological deterioration with varying degrees of recovery between episodes</li>
- +<li>slow progression of neurological decline</li>
- +<li>acute onset of symptoms with rapid decline</li>
- +<li>acute onset of mild symptoms with subsequent gradual decline lasting weeks to months</li>
- +</ul><p>Episodes of haemorrhage have been proposed as the mechanism underlying acute episodes of neurological deterioration. Progressive myelopathy may be caused by micro haemorrhages 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><h5>Site</h5><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><h4>Radiographic features</h4><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><p>Minimal cord expansion or oedema unless there has been recent haemorrhage. </p><ul>
- +<li>rounded regions of heterogeneous signal intensity on T1 and T2 weighted images due to blood products of varying ages (“popcorn appearance”)</li>
- +<li>low signal intensity rim on T2 weighted images (haemosiderin)</li>
- +<li>
- +<strong>gradient echo (GE):</strong> there can be hypointense “blooming” on gradient echo sequences (haemosiderin)</li>
- +<li>
- +<strong>T1 C+ (Gd):</strong> may demonstrate minimal enhancement on post-contrast images</li>
Images Changes:
Image 8 MRI (T2) ( create )
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