Cerebral cavernous malformation
Cerebral cavernous haemangioma (also known as a cerebral cavernous malformation or simply cavernoma) 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 haemangiomas. For a general discussion please refer to the general article on cavernous haemangiomas.
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.
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.
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.
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.
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 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.
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.
- 1. Bergui M, Bradac GB. Uncommon symptomatic cerebral vascular malformations. AJNR Am J Neuroradiol. 1997;18 (4): 779-83. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 2. Blitstein MK, Tung GA. MRI of cerebral microhemorrhages. AJR Am J Roentgenol. 2007;189 (3): 720-5. doi:10.2214/AJR.07.2249 - Pubmed citation
- 3. Jain R, Robertson PL, Gandhi D et-al. Radiation-induced cavernomas of the brain. AJNR Am J Neuroradiol. 2005;26 (5): 1158-62. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 4. Brunereau L, Labauge P, Tournier-Lasserve E et-al. Familial form of intracranial cavernous angioma: MR imaging findings in 51 families. French Society of Neurosurgery. Radiology. 2000;214 (1): 209-16. Radiology (full text) - Pubmed citation
- 5. Vilanova JC, Barceló J, Smirniotopoulos JG et-al. Hemangioma from head to toe: MR imaging with pathologic correlation. Radiographics. 24 (2): 367-85. doi:10.1148/rg.242035079 - Pubmed citation
- 6. Zhu WZ, Qi JP, Zhan CJ et-al. Magnetic resonance susceptibility weighted imaging in detecting intracranial calcification and hemorrhage. Chin. Med. J. 2008;121 (20): 2021-5. Chin. Med. J. (link) - Pubmed citation
- 7. Pinker K, Stavrou I, Knosp E et-al. Are cerebral cavernomas truly nonenhancing lesions and thereby distinguishable from arteriovenous malformations? MRI findings and histopathological correlation. Magn Reson Imaging. 2006;24 (5): 631-7. doi:10.1016/j.mri.2005.10.037 - Pubmed citation
- 8. Vogler R, Castillo M. Dural cavernous angioma: MR features. AJNR Am J Neuroradiol. 1995;16 (4): 773-5. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 9. Sekhar L, de Oliveira E. Cranial microsurgery. Thieme. (1999) ISBN:0865776989. Read it at Google Books - Find it at Amazon
- 10. Kondziolka D, Monaco EA, Lunsford LD. Cavernous malformations and hemorrhage risk. Prog Neurol Surg. 2013;27: 141-6. doi:10.1159/000341774 - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Cerebral cavernous malformation||✗|
|Cavernous haemangioma of brain||✗|
|Cerebral cavernous haemangioma||✗|
|Cerebral cavernous hemangioma||✗|
|Cavernous hemangioma of brain||✗|
|Cerebral cavernous malformations||✗|
|Cerebral cavernous haemangiomas||✗|
|Cerebral cavernous hemangiomas||✗|
|Cavernous hemangiomas of brain||✗|
|Cavernous hemangiomas of the brain||✗|
|Cavernous haemangiomas of brain||✗|
|Cavernous haemangiomas of the brain||✗|