Central nervous system cavernous hemangioma

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Central nervous system cavernous hemangioma (also known as cavernous malformation, cavernoma, or cavernous angioma) is a type of vascular malformation found in the central nervous system (CNS). These lesions are composed of clusters of dilated blood vessels that form a characteristic "mulberry-like" appearance. They can occur anywhere in the CNS, including the brain and spinal cord.

Epidemiology

Cavernous hemangiomas are relatively rare, with an estimated prevalence of 0.4-0.8% in the general population. They can occur sporadically or be inherited in an autosomal dominant pattern, often associated with mutations in the CCM1, CCM2, or CCM3 genes.

Pathophysiology

Cavernous hemangiomas are composed of thin-walled, dilated capillaries with little or no intervening brain parenchyma. These lesions are prone to hemorrhage, which can lead to various neurological symptoms depending on their location. The exact mechanism of hemorrhage is not well understood, but it is believed to be related to the structural weakness of the vessel walls.

Clinical Presentation

The clinical presentation of CNS cavernous hemangiomas can vary widely. Some individuals remain asymptomatic, while others may experience symptoms such as:

• Seizures
• Headache
• Focal neurological deficits
• Intracerebral hemorrhage

Diagnosis

The diagnosis of cavernous hemangiomas is typically made using magnetic resonance imaging (MRI). MRI is the preferred imaging modality due to its high sensitivity and specificity for detecting these lesions. Gradient echo and susceptibility-weighted imaging sequences are particularly useful in identifying the characteristic "popcorn-like" appearance of cavernous hemangiomas.

Treatment

The management of CNS cavernous hemangiomas depends on the symptoms and the location of the lesion. Treatment options include:

• Observation and regular follow-up with MRI for asymptomatic or minimally symptomatic lesions.
• Surgical resection for symptomatic lesions causing significant neurological deficits or recurrent hemorrhage.
• Stereotactic radiosurgery as an alternative for lesions that are not amenable to surgical resection.

Prognosis

The prognosis for individuals with CNS cavernous hemangiomas varies. Asymptomatic lesions may remain stable for years, while symptomatic lesions may require intervention. The risk of hemorrhage is a significant concern, particularly for lesions located in critical areas of the brain or spinal cord.

See also

• Vascular malformation
• Intracerebral hemorrhage
• Magnetic resonance imaging
• Seizure