Cavernous angioma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Edzel Lorraine Co, DMD, MD
Overview
Cavernous angioma, is a vascular disorder of the central nervous system that may appear either sporadically or exhibit autosomal dominant inheritance. Cavernous malformations are cavernomas and cavernous angiomas. They are vascular malformations composed of closely approximated endothelial-lined sinusoidal collections without significant amounts of interspersed neural tissue (lack of intervening neural tissue is the only histopathologic characteristic that distinguishes these lesions from capillary telangiectasias). Cavernous malformations are typically discrete multilobulated lesions that contain hemorrhage in various stages of evolution. Nearly all cavernous malformations show evidence of recent and remote hemorrhage. Cavernous malformations vary from several millimeters to several centimeters (usually <3 cm) in diameter.
Historical Perspective
Cavernous angioma, also known as cavernous hemangioma, cavernoma, and cerebral cavernous malformation, is a vascular malformation with still an unclear history of its first clinical discovery.
Pathophysiology
Developmental venous anomaly (DVA) can cause a chronic increase in intracranial pressure which can form microhemorrhages around DVA, leading to the development and growth of cavernous angioma. There are two patterns of cavernous angioma, the sporadic and the familial pattern.
Causes
There are no established causes for cavernous angioma.
Differentiating Cavernous angioma from other Diseases
Cavernous angioma appears very similar on MR with calcified neoplastic and hemorrhagic lesions, most especially renal cell carcinoma, melanoma, pleomorphic xanthoastrocytomas, and oligodendrogliomas.
Epidemiology and Demographics
Cavernous angioma is the second most common form of intravascular malformation next to the developmental venous anomaly (DVA). The incidence in the general population is between 0.1–0.5%, and clinical symptoms typically appear between 30 to 50 years of age. Once thought to be strictly congenital, these vascular lesions have been found to occur de novo.
Risk Factors
Family history increases the risk of having cavernous angioma. Genetic testing is recommended for the pathogenic variants of cavernous angioma (KRIT1, MGC4607, and PDCD10).
Natural History, Complications, and Prognosis
Cavernous angioma is usually a benign course since it is a low-flow and low-pressure lesion. Patients with cavernous angioma have variable signs and symptoms, with seizure as the most predominant symptom, followed by hemorrhage and focal neurologic deficit.
Diagnosis
History and Symptoms
Clinical symptoms of this disease include recurrent headaches, focal neurological deficits, hemorrhagic stroke, and seizures, but CCM can also be asymptomatic.
Physical Examination
CT scan
CT scan is not usually used as a primary modality to diagnose cavernous angiomas due to inablility to detect smaller lesions despite the ability to detect lesions complicated by calcification or hemorrhage.
MRI
Diagnosis is most commonly made accidentally by routine magnetic resonance imaging (MRI) screening, but not all MRI exams are created equal. It is paramount that the patient request a gradient-echo sequence in order to unmask small or punctate lesions which may otherwise remain undetected. These lesions are also more conspicuous on FLAIR imaging compared to standard T2 weighing. FLAIR imaging is different from Gradient sequences, rather, it is similar to T2 weighing but suppresses free-flowing fluid signal. Sometimes quiescent CCMs can be revealed as incidental findings during MRI exams ordered for other reasons.
MRA
Sometimes the lesion appearance imaged by MRI remains inconclusive. Consequently neurosurgeons will order a cerebral angiogram or magnetic resonance angiogram (MRA). Since CCMs are low flow lesions (they are hooked into the venous side of the circulatory system), they will be angiographically occult (invisible). If a lesion is discernible via angiogram in the same location as in the MRI, then an arteriovenous malformation (AVM) becomes the primary concern.
Other Imaging Findings
Other Diagnostic Studies
Treatment
Surgery
Surgery is the mainstay treatment for cavernous angioma. Complete surgical resection should be done to prevent seizures and hemorrhage due to remnant tissue.
Cost-effectiveness of therapy
Futures of Investigational Therapies
Several insights on development of cavernous angioma exist. Some drugs have been identified to be able to reduce risk of hemorrhage, and possible development of new lesions. Advances in biomarkers are now being studied more.