Cerebral cavernous venous malformation
Cerebral cavernous venous malformations, commonly known as cavernous hemangioma or cavernoma, are common cerebral vascular malformations, 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 and links to cavernomas in other locations, please refer to the general article on cavernous venous malformation.
Many alternative terms have been used over the years including cavernous hemangioma, cerebral cavernous malformation or simply cavernoma. As these lesions are not neoplastic, it has been argued that the terms 'hemangioma' and 'cavernoma' should be avoided. Additionally, it 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 all 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.
For brevity, the term cavernous malformation is used in the remainder of this article.
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, cavernous malformations, along with capillary telangiectasias, are commonly seen following cerebral radiotherapy 3.
The majority of lesions remain asymptomatic throughout life and are found incidentally. Presentation due to hemorrhage may cause a headache, seizure or focal neurological deficit. The risk of hemorrhage is 1% per year for familial cases and somewhat less for sporadic lesions.
Histologically cavernous malformations are composed of a "mulberry-like" cluster of hyalinized dilated thin-walled capillaries, with surrounding hemosiderin 3. These vessels are thrombosed to varying degrees. 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 one-third 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 edema.
MRI is the modality of choice, demonstrating a characteristic “popcorn” or "berry" appearance with a rim of signal loss due to hemosiderin, which demonstrates prominent blooming on susceptibility weighted sequences.
T1 and T2 signal is varied internally depending on the age of the blood products 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 weighted imaging (SWI) may have sensitivity equal to that of GRE in detecting these capillary telangiectasias in the 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 edema may be present.
The lesions generally do not enhance, although enhancement is possible 7.
Cavernous malformations can be grouped into four types based on MRI appearances using the Zabramski classification 11.
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 hemorrhage. Symptomatic lesions should, when possible, be resected and complete resection is curative 9.
The differential, when cavernous venous malformations are numerous, is that of other causes of cerebral microhemorrhages, 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-induced vasculopathy
- hemorrhagic metastases
- Parry-Romberg syndrome 2
Larger lesions can mimic:
- 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
- 11. Zabramski JM, Wascher TM, Spetzler RF et-al. The natural history of familial cavernous malformations: results of an ongoing study. J. Neurosurg. 1994;80 (3): 422-32. doi:10.3171/jns.1994.80.3.0422 - Pubmed citation
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