Cerebral cavernous venous malformation
Updates to Article Attributes
Cerebral cavernous venous malformations is a common cerebral vascular malformation usually with characteristic appearances on MRI.
Cavernous malformations are found throughout the body. This article focuses on cerebral cavernous venous malformations. For a general discussion please refer to the general article on cavernous venous malformation
Terminology
Many alternative terms have been used over the years including cavernous hemangioma, cerebral cavernous malformation or simply cavernoma. As these lesions are not neoplastic, the term 'hemangioma' and 'cavernoma' should be avoided.
Additionally is important to note that according to newer nomenclature (ISSVA classification of vascular anomalies) these lesions are merely known as slow flow venous malformations. Having said that, it is probably helpful in reports to include the word 'cavernous' as this term is ubiquitous in the literature and most familiar to many clinicians.
Epidemiology
Most patients who present symptomatically do so at 40-60 years of age. Most patients have single lesions. Multiple lesions may be familial and screening of family members may be indicated (see familial multiple cavernous malformation syndrome). Additionally, they are commonly seen after therapeutic irradiation of the brain, along with capillary telangiectasias 3.
Clinical presentation
The majority of lesions remain asymptomatic throughout life and are found incidentally. Presentation due to haemorrhage is with a seizure or focal neurological deficit. The risk of haemorrhage is 1% per year for familial cases, and somewhat less for sporadic lesions.
Pathology
Histologically cavernous malformations are composed of a "mulberry-like" cluster of dilated thin-walled capillaries, with surrounding haemosiderin 3. Unlike AVMs there is no normal brain between the interstices of these lesions.
On occasion they are intimately associated with a developmental venous anomaly (DVA), in which case they are known as mixed vascular malformations.
Radiographic features
Cerebral cavernomas tend to be supratentorial (~ 80% cases) but can be found anywhere including the brainstem. They are usually solitary, although up to 1/3rd of patients with sporadic lesions have more than one 2.
CT
Unless large, these lesions are difficult to see on CT. They do not enhance. If large then a region of hyperdensity can be seen. If there has been a recent bleed then it is more conspicuous and may be surrounded by a mantle of oedema.
MRI
MRI is the modality of choice, demonstrating a characteristic “popcorn” or "berry" appearance with a rim of signal loss due to haemosiderin, which demonstrates prominent blooming on susceptibility weighted sequences.
T1 and T2 signal is varied internally depending on the age of the blood produces and small fluid fluid levels may be evident.
Gradient Echo or T2* sequences are able to delineate these lesions better than T1 or T2 weighted images. In patients with familial or multiple cavernous angiomas GRE T2* sequences are very important in identifying the number of lesions missed by conventional Spin echo sequences.
Susceptibility imaging (SWI) may have sensitivity equal to that of GRE in detecting these capillary telagiectasiae in brain. SWI is also highly sensitive in detecting calcification as compared to T1 and T2 images.6
If a recent bleed has occurred then surrounding oedema may be present.
The lesions generally do not enhance, although enhancement is possible.7
Angiography / DSA
Cavernous malformations are angiographically occult and do not demonstrate arteriovenous shunting.
Treatment and prognosis
Many cavernous malformations are asymptomatic and can be treated conservatively. Symptoms can relate to mass effect, epileptic activity or repeated haemorrhage. Symptomatic lesions should, when possible, be resected and complete resection is curative 9.
Differential diagnosis
The differential when cavernomas are numerous is that of other causes of cerebral microhaemorrhages, including 2:
- cerebral amyloid angiopathy - usually numerous small foci
- chronic hypertensive encephalopathy - more common in the basal ganglia
- diffuse axonal injury (DAI)
- cerebral vasculitis
- radiation vasculopathy
- haemorrhagic metastases
- Parry-Romberg syndrome 2
Larger lesions can mimic:
- haemorrhagic cerebral metastases
- haemorrhagic primary brain tumours (e.g. ependymoma, GBM)
Calcified lesions, such as old neurocysticercosis, or other infections (e.g. tuberculoma) should also be considered.
-<p><strong>Cerebral cavernous venous malformations</strong> is a common <a href="/articles/cerebral-vascular-malformations">cerebral vascular malformation</a> usually with characteristic appearances on MRI. </p><p>Cavernous malformations are found throughout the body. This article focuses on cerebral cavernous venous malformations. For a general discussion please refer to the general article on <a href="/articles/cavernous-venous-malformation-1">cavernous venous malformation</a></p><h4>Terminology </h4><p><span style="font-size:13px; line-height:1.6em">Many alternative terms have been used over the years including </span><strong style="font-size:13px; line-height:1.6em">cavernous hemangioma</strong><span style="font-size:13px; line-height:1.6em">, </span><strong style="font-size:13px; line-height:1.6em">cerebral cavernous malformation</strong><span style="font-size:13px; line-height:1.6em"> or simply </span><strong style="font-size:13px; line-height:1.6em">cavernoma. </strong><span style="font-size:13px; line-height:1.6em">As these lesions are not neoplastic, the term 'hemangioma' and 'cavernoma' should be avoided. </span></p><p>Additionally is important to note that according to newer nomenclature (<a href="/articles/issva-classification-of-vascular-anomalies">ISSVA classification of vascular anomalies</a>) these lesions are merely known as slow flow venous malformations. Having said that, it is probably helpful in reports to include the word 'cavernous' as this term is ubiquitous in the literature and most familiar to many clinicians. </p><h4>Epidemiology</h4><p>Most patients who present symptomatically do so at 40-60 years of age. Most patients have single lesions. Multiple lesions may be familial and screening of family members may be indicated (see <a href="/articles/familial-multiple-cavernous-malformation-syndrome">familial multiple cavernous malformation syndrome</a>). Additionally, they are commonly seen after therapeutic irradiation of the brain, along with <a href="/articles/capillary-telangiectasia-cns">capillary telangiectasias</a> <sup>3</sup>.</p><h4>Clinical presentation</h4><p>The majority of lesions remain asymptomatic throughout life and are found incidentally. Presentation due to haemorrhage is with a seizure or focal neurological deficit. The risk of haemorrhage is 1% per year for familial cases, and somewhat less for sporadic lesions.</p><h4>Pathology</h4><p>Histologically cavernous malformations are composed of a "mulberry-like" cluster of dilated thin-walled capillaries, with surrounding haemosiderin <sup>3</sup>. Unlike <a href="/articles/arteriovenous-malformations">AVMs </a>there is no normal brain between the interstices of these lesions. </p><p>On occasion they are intimately associated with a <a href="/articles/developmental-venous-anomaly">developmental venous anomaly (DVA)</a>, in which case they are known as <a href="/articles/mixed-vascular-malformation">mixed vascular malformations</a>.</p><h4>Radiographic features</h4><p>Cerebral cavernomas tend to be supratentorial (~ 80% cases) but can be found anywhere including the brainstem. They are usually solitary, although up to 1/3<sup>rd</sup> of patients with sporadic lesions have more than one <sup>2</sup>.</p><h5>CT</h5><p>Unless large, these lesions are difficult to see on CT. They do not enhance. If large then a region of hyperdensity can be seen. If there has been a recent bleed then it is more conspicuous and may be surrounded by a mantle of oedema. </p><h5>MRI</h5><p>MRI is the modality of choice, demonstrating a characteristic “popcorn” or "berry" appearance with a rim of signal loss due to haemosiderin, which demonstrates prominent blooming on susceptibility weighted sequences. </p><p>T1 and T2 signal is varied internally depending on the age of the blood produces and small fluid fluid levels may be evident.</p><p>Gradient Echo or T2* sequences are able to delineate these lesions better than T1 or T2 weighted images. In patients with familial or multiple cavernous angiomas GRE T2* sequences are very important in identifying the number of lesions missed by conventional Spin echo sequences.</p><p>Susceptibility imaging (SWI) may have sensitivity equal to that of GRE in detecting these capillary telagiectasiae in brain. SWI is also highly sensitive in detecting calcification as compared to T1 and T2 images.<sup>6</sup></p><p>If a recent bleed has occurred then surrounding oedema may be present.</p><p>The lesions generally do not enhance, although enhancement is possible.<sup>7</sup></p><h5>Angiography / DSA</h5><p>Cavernous malformations are angiographically occult and do not demonstrate arteriovenous shunting. </p><h4>Treatment and prognosis</h4><p>Many cavernous malformations are asymptomatic and can be treated conservatively. Symptoms can relate to mass effect, epileptic activity or repeated haemorrhage. Symptomatic lesions should, when possible, be resected and complete resection is curative <sup>9</sup>. </p><h4>Differential diagnosis</h4><p>The differential when cavernomas are numerous is that of other causes of <a href="/articles/cerebral-microhaemorrhage">cerebral microhaemorrhages</a>, including <sup>2</sup>:</p><ul>- +<p><strong>Cerebral cavernous venous malformations</strong> is a common <a href="/articles/cerebral-vascular-malformations">cerebral vascular malformation</a> usually with characteristic appearances on MRI. </p><p>Cavernous malformations are found throughout the body. This article focuses on cerebral cavernous venous malformations. For a general discussion please refer to the general article on <a href="/articles/cavernous-venous-malformation-1">cavernous venous malformation</a></p><h4>Terminology </h4><p>Many alternative terms have been used over the years including <strong>cavernous hemangioma</strong>, <strong>cerebral cavernous malformation</strong> or simply <strong>cavernoma. </strong>As these lesions are not neoplastic, the term 'hemangioma' and 'cavernoma' should be avoided. </p><p>Additionally is important to note that according to newer nomenclature (<a href="/articles/issva-classification-of-vascular-anomalies">ISSVA classification of vascular anomalies</a>) these lesions are merely known as slow flow venous malformations. Having said that, it is probably helpful in reports to include the word 'cavernous' as this term is ubiquitous in the literature and most familiar to many clinicians. </p><h4>Epidemiology</h4><p>Most patients who present symptomatically do so at 40-60 years of age. Most patients have single lesions. Multiple lesions may be familial and screening of family members may be indicated (see <a href="/articles/familial-multiple-cavernous-malformation-syndrome">familial multiple cavernous malformation syndrome</a>). Additionally, they are commonly seen after therapeutic irradiation of the brain, along with <a href="/articles/capillary-telangiectasia-cns">capillary telangiectasias</a> <sup>3</sup>.</p><h4>Clinical presentation</h4><p>The majority of lesions remain asymptomatic throughout life and are found incidentally. Presentation due to haemorrhage is with a seizure or focal neurological deficit. The risk of haemorrhage is 1% per year for familial cases, and somewhat less for sporadic lesions.</p><h4>Pathology</h4><p>Histologically cavernous malformations are composed of a "mulberry-like" cluster of dilated thin-walled capillaries, with surrounding haemosiderin <sup>3</sup>. Unlike <a href="/articles/arteriovenous-malformations">AVMs </a>there is no normal brain between the interstices of these lesions. </p><p>On occasion they are intimately associated with a <a href="/articles/developmental-venous-anomaly">developmental venous anomaly (DVA)</a>, in which case they are known as <a href="/articles/mixed-vascular-malformation">mixed vascular malformations</a>.</p><h4>Radiographic features</h4><p>Cerebral cavernomas tend to be supratentorial (~ 80% cases) but can be found anywhere including the brainstem. They are usually solitary, although up to 1/3<sup>rd</sup> of patients with sporadic lesions have more than one <sup>2</sup>.</p><h5>CT</h5><p>Unless large, these lesions are difficult to see on CT. They do not enhance. If large then a region of hyperdensity can be seen. If there has been a recent bleed then it is more conspicuous and may be surrounded by a mantle of oedema. </p><h5>MRI</h5><p>MRI is the modality of choice, demonstrating a characteristic “popcorn” or "berry" appearance with a rim of signal loss due to haemosiderin, which demonstrates prominent blooming on susceptibility weighted sequences. </p><p>T1 and T2 signal is varied internally depending on the age of the blood produces and small fluid fluid levels may be evident.</p><p>Gradient Echo or T2* sequences are able to delineate these lesions better than T1 or T2 weighted images. In patients with familial or multiple cavernous angiomas GRE T2* sequences are very important in identifying the number of lesions missed by conventional Spin echo sequences.</p><p>Susceptibility imaging (SWI) may have sensitivity equal to that of GRE in detecting these capillary telagiectasiae in brain. SWI is also highly sensitive in detecting calcification as compared to T1 and T2 images.<sup>6</sup></p><p>If a recent bleed has occurred then surrounding oedema may be present.</p><p>The lesions generally do not enhance, although enhancement is possible.<sup>7</sup></p><h5>Angiography / DSA</h5><p>Cavernous malformations are angiographically occult and do not demonstrate arteriovenous shunting. </p><h4>Treatment and prognosis</h4><p>Many cavernous malformations are asymptomatic and can be treated conservatively. Symptoms can relate to mass effect, epileptic activity or repeated haemorrhage. Symptomatic lesions should, when possible, be resected and complete resection is curative <sup>9</sup>. </p><h4>Differential diagnosis</h4><p>The differential when cavernomas are numerous is that of other causes of <a href="/articles/cerebral-microhaemorrhage">cerebral microhaemorrhages</a>, including <sup>2</sup>:</p><ul>
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