A complete resection of the lesion is mandatory due to the increased risk of hemorrhage and seizures due to remnant tissue. Selection of a procedure depends on the location and severity at the presentation, which includes intractable seizures, progressive neurological deterioration, one severe hemorrhage in a non-eloquent region of the brain, or at least 2 severe hemorrhages in eloquent regions of the brain. Surgical resection is the most preferred form of intervention for CCM. Cavernous angiomas are a rare cause of an isolated non-aneurysmal SAH, and very few cases have been reported. Gross and Du summarized the risk factors for hemorrhage in these patients, which included prior hemorrhage from a cerebral cavernous angioma, female sex, and brainstem location of the lesion. There are studies that try to explain the natural course of hemorrhage occurring in patients with CCM.
Supratentorial lesions have been found to present more commonly with seizures, whereas infratentorial malformations present with focal neurological deficits. When these lesions become symptomatic, it can have a wide array of clinical presentation that includes seizures, hemorrhage, headache, and focal neurological deficits. They can be supratentorial, which affects the cerebral cortex, and infratentorial, affecting the brainstem or the cerebellum however, most of them are supratentorial lesions. ĬCMs can have varied presentations depending on the location of the lesion. Mixed vascular malformation – coexistence of vascular malformations and the most commonly encountered form is a CCM that occurs in association with a DVA. CCM – vascular spaces of varying sizes, lined by a single layer of endothelin with no distinct features of arteries or veins. DVAs – most commonly encountered malformation involving the intracranial venous system, which are also known as venous angioma, cerebral venous medullary malformation, or cerebral venous malformation. Arteriovenous malformations – clusters of abnormal arteries and veins with an increased risk of intracranial bleed, more common in young adults. Capillary telangiectasia – dilated thin-walled capillaries due to the absence of smooth muscle or elastic fibers. Moyamoya disease – chronic occlusive cerebrovascular disease resulting in progressive stenosis of the internal carotid artery and its branches. Aneurysms – abnormal saccular outpouchings of the cerebral arteries. Intracranial or cerebral vascular malformations are a group of vascular lesions with varying hemodynamic or structural properties (Fig. Conclusion: Individual CCM or DVA lesions have a benign course however, when they coexist in the same individual, the hemorrhagic risk is increased, which prompts for rapid diagnosis and treatment. Asymptomatic lesions are treated through a conservative approach, while clinically symptomatic lesions need surgical management. Mixed lesions, on the other hand, are best diagnosed with magnetic resonance imaging, which has also been effective in detecting specific abnormalities. CCMs are angiographically occult lesion, and cerebral digital subtraction angiography is the gold standard for the diagnosis of DVA. Mixed CCM with DVA has a higher risk of hemorrhage. DVAs are benign lesions by nature however, venous infarction can occur in a few patients due to acute thrombosis. CCM can have a wide array of clinical presentations like hemorrhage, seizures, or focal neurological deficits or can also be an incidental finding on brain imaging. Summary: The search yielded 1,249 results for “Cerebral cavernous malformations,” 271 results for “Developmental venous anomaly,” and 5 results for “Mixed Cerebral cavernous malformations with Developmental venous anomaly.” DVA is the most common intracranial vascular malformation, followed by CCM. All studies in the English language in the past 10 years were analyzed descriptively for this review. A PubMed search using the keywords “Cerebral cavernous malformations, Developmental venous anomaly, Mixed Cerebral cavernous malformations with Developmental venous anomaly” was done. In this paper, we aim to give a comprehensive review of CCM, DVA, and their coexistence as mixed lesions. The most common mixed form is the coexistence of CCM with an associated developmental venous anomaly (DVA). Background: Cerebral cavernous malformations (CCMs) are intracranial vascular malformations that can exist as a single lesion or mixed vascular lesions.
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