Cavernous Angioma, also known as cerebral cavernous malformation (CCM), cavernous haemangioma, and cavernoma, is a vascular disorder of the central nervous system that may appear either sporadically or exhibit autosomal dominant inheritance. 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.

This disease is characterized by grossly dilated blood vessels with a single layer of endothelium and an absence of neuronal tissue within the lesions. These thinly-walled vessels resemble sinusoidal cavities filled with stagnant blood. Blood vessels in patients with CCM can range from a few millimeters to several centimeters in diameter. CCM lesions commonly resemble raspberries in external structure.

Many patients live their whole life without knowing they have a cerebral cavernous malformation. Other patients can have severe symptoms like seizures, headaches, paralysis, bleeding in the brain (cerebral hemorrhage), and even death. The nature and severity of the symptoms depend on the lesion's location in the brain. Approximately 70% of these lesions occur in the supratentorial region of the brain; the remaining 30% occur in the infratentorial region.

Symptoms and Diagnosis

Clinical symptoms of this disease include recurrent headaches, focal neurological deficits, hemorrahagic stroke, and seizures, but CCM can also be asymptomatic. Diagnosis is most commonly made by magnetic resonance imaging MRI, but not all MRI exams are created equal. It's paramount that the patient request a gradient-echo MRI (aka T2-Flair) in order to unmask small or punctate lesions which may otherwise remain undetected. Sometimes quiescent CCMs can be revealed as incidental findings during MRI exams ordered for other reasons.

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 discernable via angiogram in the same location as in the MRI, then an arteriovenous malformation (AVM) becomes the primary concern.

CCMs & Venous Angiomas

Not infrequently a CCM is accompanied by a venous angioma, also known as a developmental venous anomaly (DVA). These lesions appear either as enhancing linear blood vessels or caput medusae--a radial orientation of small vessels that resemble the hair of Medusa from Greek Mythology. These lesions are thought to represent developmental anomalies of normal venous drainage. These lesions should not be removed, as reports of venous infarcts have been reported. When found in association with a CCM that needs resection, great care should be taken not to disrupt the angioma.

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Facial nerve hemangioma - Imaging Clinic - Bell's palsy
From Ear, Nose & Throat Journal, 11/1/03 by Enrique Palacios

Bell's palsy accounts for almost 50% of all cases of facial nerve paralysis. (1) Tumors are involved in only 5% of cases of facial paralysis, and the most common tumors are acoustic and facial neuromas. (2) Facial nerve hemangiomas account for only 0.7% of all temporal bone tumors. (1-3) Facial nerve hemangiomas arise from vascular plexuses distributed along the facial nerve paths in the geniculate ganglion region, in the mastoid segment of the facial nerve near the origin of the chorda tympani, and in the internal auditory canal around Scarpa's ganglion. (2)

Microscopically, facial nerve hemangiomas are seen as large vascular spaces lined with thin endothelium surrounded by thick walls filled with uniform fibrous tissue. (1,2) The characteristic honeycomb appearance seen on computed tomography (CT) (figure, A) is the result of erosion of the temporal bone and calcification of the collagen-containing walls between the vascular channels. The presence of bony channels, indistinct tumor margins, and intratumor bony spicules led to the term ossifying hemangiomas. (3)

[FIGURE A OMITTED]

High-resolution CT and enhanced magnetic resonance imaging (MRI) (figure, B) together are the most sensitive diagnostic modalities available to demonstrate facial nerve hemangiomas. (1)

[FIGURE B OMITTED]

Facial nerve hemangiomas and facial nerve neuromas yield similar clinical findings. Characteristically, severe nerve dysfunction is seen in patients with small hemangiomas; tinnitus and dizziness are seldom present. Conversely, symptomatic facial nerve neuromas are relatively large. Facial nerve hemangiomas and neuromas can be differentiated by their histologic appearance and by the presence or absence of the ossifying characteristics of facial nerve hemangiomas. (2) Facial nerve hemangiomas should also be distinguished from meningiomas, congenital cholesteatomas, metastatic carcinomas, and other vascular tumors that involve the temporal bone.

Surgical removal is the definitive treatment. In view of the extrinsic growth pattern of these tumors, surgical removal should be performed early to preserve facial nerve function. (4) Tumors in the geniculate ganglion region usually lead to an intense perineural reaction or direct nerve infiltration. Therefore, the opportunities to preserve an intact nerve following surgery are limited. Treatment by total excision and facial nerve grafting can provide a permanent cure and restore facial function. (2,5)

References

(1.) Salib RJ, Tziambazis E, McDermott AL. et al. The crucial role of imaging in detection of facial nerve haemangiomas. J Laryngol Otol 2001;115:510-13.

(2.) Escada P, Capucho C, Silva JM, el al. Cavernous haemangioma of the facial nerve. J Laryngol Otol 1997:111:858-61.

(3.) Friedman O. Neff BA, Willcox TO, et al. Temporal bone hemangiomas involving the facial nerve. Otol Neurotol 2002;23:760-6.

(4.) Alobid I, Gaston F, Morello A, et al. Cavernous haemangioma of the internal auditory canal. Acta Otolaryngol 2002:122:501-3.

(5.) Pulec JL. Facial nerve angioma. Ear Nose Throat J 1996:75: 225-38.

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