Presentation
Presents with left-sided retro-orbital headache, photophobia and decreased sensation of the left side of the face.
Patient Data
A 2cm typical cavernous malformation with complete haemosiderin ring and a mulberry internal structure situated at the junction of the posterior temporal, parietal and occipital lobes. It shows a peripheral haemosiderin staining and demonstrates lobulated internal areas of T1 hyper intensity with minor internal areas of enhancement. No evidence of a venous angioma, and no evidence of enlarged adjacent vessels.
There is a small development venous anomaly in the left frontal lobe.
The small focus of signal susceptibility in the left pons is unchanged and consistent with a small cavernoma.
Rest of the brain is unremarkable; aside from afore mentioned lesions, cerebral parenchymal signal intensity and architecture are normal, with no other lesions identifiable. Normal flow voids are identified from the major arteries at the base of the brain. Ventricles and sulci are normal.
CONCLUSION: Typical cavernous malformation of left cerebral hemisphere and left pontine area as described with no evidence of an associated venous angioma or arteriovenous malformation.
The patient has remained under radiological surveillance, with stable appearances of the left-sided cavernous malformation.
Case Discussion
Vascular malformations are commonly divided into four categories: arteriovenous malformation, venous angioma, cavernous angioma/malformation, and capillary telangiectasias 1,2. This case is as example of a cerebral cavernous malformation.
A cavernous malformation or angioma is a hamartomatous "berry-like" collection of vascular spaces lined by thin walls devoid of smooth muscle 3. The characteristic feature is that it contains almost no intervening brain parenchyma among the abnormally enlarged, thin-wall endovascular channels 1. Surrounding brain parenchyma is often gliotic and haemosiderin-stained, and may contain small low-flow feeding arteries and draining veins 3. They appear to grow by a process of cavern proliferation in the setting of repetitive lesional haemorrhages (haemorrhagic proliferative dysangiogenesis) 4.
The peak incidence occurs between 40–60 years of age. Most patients have a single lesion which remains clinically silent and usually presents as an incidental finding on neuroimaging. When symptomatic, patients commonly present with seizures and progressive neurological deficits 1,4.
The radiological appearance of cavernous malformations is variable on CT and MRI. On pre-contrast CT, the lesion commonly appears 1:
- hyperdense, but mixed hyperdense and isodense lesions have been described
- mass effect is frequently present and occasionally secondary to haematoma, is frequently present
Contrast administration may improve the delineation of the lesion with faint enhancement1-4.
High-field MR imaging is considered the radiographic “golden standard” for the diagnosis and evaluation of cavernous malformations 2. This demonstrates characteristic “popcorn” appearance of the lesion, with a low signal intensity of the rim due to haemosiderin deposition 1-4.
Incidental lesions are usually managed conservatively. Symptomatic cavernous malformations can be surgically excised 1-4. However, the potential risks and benefits of surgery for each patient must be diligently discussed prior to removal of these benign malformations.
Case courtesy of Dr. Frank Gaillard.
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