BACKGROUND
Cavernous angiomas are clusters of abnormal blood vessels found in the brain, spinal cord, and, rarely, in other areas of the body. There are many names for this condition:

• cavernous angioma
• cavernous hemangioma
• cerebral cavernous malformation (CCM)
• cavernoma

RELEVANT ANATOMY
Figure 1: Drawing showing the mulberry like cavernous malformation.



Figure 2: Photomicrograph showing the blood filled caverns forming the CCM.



Figure 3: MRI showing the old blood products (hemosiderin) surrounding multiple CCMs.

SIGNS and SYMPTOMS of DISEASE
A cavernous angioma may have no symptoms. When symptoms are present, they often depend on the location of the angioma and on the strength of the angioma walls.
A patient with a cavernous malformation can present with:

1. Seizures.
2. Neurological deficits such as weaknesses in arms or legs, vision problems, balance problems, or memory and attention problems.
3. Headaches.

• Angiomas can bleed slowly within the walls of the angioma and remain quite small. A small hemorrhage may not require surgery, but may be reabsorbed by the body. However, continued small hemorrhages in the same cavernous angioma often cause deterioration in function.
• Angiomas can bleed more profusely within the walls of the angioma. This can cause them to grow and put pressure on the surrounding brain tissue.
• Finally, angiomas may bleed through a weak spot in the angioma wall into the surrounding brain tissue. This is called an overt hemorrhage.

DIAGNOSTIC TESTS
The cavernous angiomas are not visible on angiogram and are only inconsistently visible on CAT scans An MRI scan, with and without contrast and with gradient echo sequences, read by an experienced physician remains the best means of diagnosing this illness.

TREATMENT OPTIONS

• Observation: Most cavernous angiomas are observed for change in appearance, recent hemorrhage or clinical symptoms.
• Surgery: Surgery is advocated for cavernous angiomas with recent hemorrhage, those which are expanding in size, and in some cases, those which are causing seizures resistant to medical treatment.
• Radiation: Radiosurgery, by gamma knife, linear accelerator or new shaped beam techniques, is a controversial treatment that has been used on cavernous angiomas too dangerous to reach through traditional surgery.

SURGICAL TECHNIQUE
Cerebral cavernous angiomas are surgically removed using a craniotomy, or opening the skull. This is usually performed under general anesthesia, except in cases where mapping of the brain while awake is needed. Cavernous angiomas in the spine are removed using laminectomy or unroofing of the vertebrae.

Surgery for cavernous angioma has been made safer using the operating microscope (microsurgery) and image guided surgical navigation (also known as computer-assisted or frameless stereotaxy) to reach the cavernous angioma with as little disruption to normal brain or spinal cord as possible.

SURGICAL RISKS
Risks of any surgery, including cavernous angioma, include stroke, paralysis, coma or death, although these complications are rare with modern surgery performed by expert neurosurgeons.

AUTHOR
PASCAL JABBOUR, MD.

RELEVANT TERMS

1. Endothelial: The layer of cells that lines the blood vessels and other organs. Cavernous angiomas are comprised of endothelial cells that lack the support of the outer layers of blood vessel. This makes them leaky and fragile.
2. Hemorrhage: The escape of blood from the vessels.
3. Hemosiderin: An iron deposit which is a blood breakdown product. When a hemosiderin ring is seen around a cavernous angioma on MRI, this means that the cavernous angioma bled some time in the past.
4. Magnetic Resonance Imaging: A method of imaging soft tissues of the body, such as the brain and spinal cord, by applying an external magnetic field. Magnetic resonance images distinguish between hydrogen atoms in different environments and produces a picture of these environments.
5. Craniotomy: A surgical procedure in which the skull is opened in order to access the brain.