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.
Epidemiology and Demographics
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.
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.
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.