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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

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:

Larger lesions can mimic:  

Calcified lesions, such as old neurocysticercosis, or other infections (e.g. tuberculoma) should also be considered. 

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