Cavernous Malformations

What is cavernous malformation?

Cavernous hemangioma or cavernous malformations are an abnormal build up of blood vessels within internal organs or the skin. These malformations arise from very small vessels that separate the arterial system from the venous system. Cavernous hemangiomas are well-defined and clearly visible lesions that can reach a significant size. These malformations account for about 8% to 15% of all the intracranial and spinal vascular malformations. They can cause hemorrhage; and, sometimes can be life threatening if they arise in a critical area of an internal organ.

Causes of cavernous malformations:

The causes of cavernous malformations are not yet fully known. Some researchers and studies have shown the importance of estrogen signaling in the rapid growth of hemangiomas.

About one third of hemangiomas are present at the time of birth. The other two thirds arise in the initial months life.

They can be in the upper layers of skin, deep within the skin, or mixture of both. They can also grow within internal organs.

Symptoms of cavernous malformations:

The symptoms and appearance of cavernous malformations usually depend on their location. If they are on the surface of skin, they appear as a ripe strawberry. That's why they are also known as strawberry nevus. If these malformations are under the skin, they appear as a bluish swelling. If they grow in an internal organ (such as the liver, larynx, or brain) the symptoms will be related to those that would occur when that organ fails to properly function.

Diagnosis of cavernous malformations:

Usually cavernous malformations in the skin are diagnosed by via physical examination because they are clearly visible.

In case of deep or complicated lesions, computerized tomography (CT) scans or magnetic resonance imaging (MRI) scans might be used.

CT and MRI scans may also be used to diagnose the cavernous malformations if they occur within internal organs.

Treatment options:

Any (or a combination) of the following options may be used to treat a cavernous hemangioma. The type of treatment will depend on its location, size and its affect on a patient's quality of life.

• No Surgery: If the lesion is in a difficult area or small in size, a doctor may choose to leave the malformation alone. The same is true if the risk/benefit ratio does not favor treatment. In most cases, a doctor will then keep observing the malformation with a series of periodic CT or MRI scans.
• Surgical Removal: If a cavernous hemangioma is large and easily accessible, a doctor may choose to remove it surgically. Once a cavernous hemangioma is removed, the lesion is cured completely. Difficulties arise when there are multiple hemangiomas or if they occur deep inside an internal organ. Cavernous hemangiomas present significant difficulties for surgeons when they are within the brain.
• Radiation Therapy: Radiation therapy has not yet proven to be effective for the treatment of cavernous hemangiomas. However, some physicians do use focus beam radiation (such as gamma knife) even though the long-term results of radiation therapy have not been shown.