Cavernous Hemangioma - Causes, Symptoms, Diagnosis, and Treatment

What Is a Cavernous Hemangioma?

Cavernous hemangioma is a benign, abnormal mass of tangled thin-walled capillaries of the brain or spinal cord that form during development. The thin walls of the capillaries make hemangioma prone to bleeding. The blood-filled spaces separated with connective tissue gives cavernous hemangioma looks like a raspberry. The blood within the hemangioma is usually slow-moving. The slowly progressing tumor of the endothelial cells (cells found in the inside lining of blood vessels or the heart) is also called cerebral cavernous malformation. They often occur in multiples and almost always on one side of the brain. The clusters can vary in size from less than a quarter of an inch to three to four inches.

A cavernous hemangioma can involve every organ of the body and is grouped into four types following their histopathologic characteristics - capillary malformation or telangiectasias, cavernous malformation (a type of vascular malformation that consists of abnormally enlarged blood vessels or caverns that are filled with blood), venous malformation (type of vascular malformation that involves abnormal veins), and arteriovenous shunting malformations.

What Causes Cavernous Hemangioma?

Cavernous hemangiomas are genetic and caused by mutation of the genes. However, it is observed to be more common in people descendants of the original Hispanic population, European-American descendants, or Ashkenazi Jewish descent. A cavernous hemangioma can be in two forms

• Familial - This is when it has been inherited from either of the parents and involves genetic mutation of dominant genes, like CCM1, CCM2, and CCM3.

• Sporadic - This is a more common form. It develops when the malformation occurs without any inherited gene. It may be caused by acquired genetic changes in particular cells and does not pass between generations.

Behaviors, like smoking cigarettes, can increase inflammation and increase the risk of hemorrhage.

What Are the Symptoms of a Cavernous Hemangioma?

Cavernous hemangiomas are not cancerous and usually seen in ages between 20 to 30 years. They are well-defined and visible and account for about 8 to 15 percent of all intracranial and spinal vascular malformations. Approximately 25 percent of individuals with cavernous hemangioma never experience any serious symptoms. The symptoms of cavernous hemangioma may differ depending on the size, and location of the hemangioma including

• Blurred or double vision.

• Seizures (sudden uncontrolled electrical interruption in the brain).

• Slurred speech.

• Bleeding.

• Coordination problems.

• Memory issues.

• Tremors.

• Numbness.

• Severe headaches.

• Poor concentration.

• Weakness.

• Tingling or numbness in the hands and legs.

Cavernous hemangioma usually occurs in the brain but can develop in the spinal cord, skin, retina, liver, adrenal glands, and gastrointestinal tract.

What Are the Potential Complications of a Cavernous Hemangioma?

Untreated cerebral hemangiomas can result in serious complications, like when it develops in areas responsible for subconscious functions, such as the cerebellum (part of the brain that controls coordination and movement-related motor skills).

• Bleeding - It is the most common complication of a cavernous hemangioma that is more likely to occur in people with previously diagnosed hemorrhage. Severe bleeding can be life-threatening and lead to long-lasting problems. The risk of bleeding is documented more with cavernous hemangioma associated with atypical venous drainage. Also the more cavernous, the greater the chance of hemorrhage.

• Neurological Problems - Cavernous hemangioma along the spine can cause spinal compression leading to vision loss.

• Stroke - It is associated with a blocked artery and may cause permanent disability or death.

How to Diagnose Cavernous Hemangioma?

The majority of lesions remain asymptomatic throughout life while some present with headaches, seizures, or focal neurological deficits due to hemorrhage. The physician will start with a physical examination followed by a detailed family history. Cavernous hemangiomas can be diagnosed by

• Genetic Testing - It involves examining the DNA (deoxyribonucleic acid) for mutations or variants.

• Magnetic Imaging Resonance - Radiological studies play a pivotal role in diagnosing cavernous hemangioma with MRI as the modality of choice. The specialized magnetic resonance imaging (MRI) technique known as susceptibility-weighted imaging is used to identify small areas of bleeding.

• Orbital Echography - It examines soft tissue structures surrounding the eye. The diagnostic tool involves non-irradiating examination using ultrasound.

• Tonometry - The test is performed to measure intraocular pressure.

How to Differentiate Cavernous Hemangioma and Other Vascular Malformations?

Cavernous hemangiomas are not true vascular tumors, but rather slow-or venous malformations. They are present at birth as non-proliferating vascular birthmarks composed of anomalous ectatic venous channels and are incorrectly called cavernous angiomas. The arteriovenous malformations, on the other hand, are high-flow lesions with rapid blood movement.

How Is Cavernous Hemangioma Treated?

A cavernous hemangioma can be treated, depending on the location of the malformation. If the hemangioma is causing discomfort, interfering with bodily functions, or growing, treatment options may include:

• Neurosurgery - Symptomatic cavernous malformation can be surgically removed by a procedure known as craniotomy (an operation where a small hole is made in the skull to remove the affected area). Surgery is also recommended to remove cavernous malformations from other parts, such as the eyes or spinal cord if they cause problems.

• Stereotactic Radiosurgery - A single concentrated dose of radiation is aimed directly at the cavernous causing it to thicken and scar. However, the effectiveness of this is unknown and considered only if the position of the cavernous hemangioma makes neurosurgery too difficult or dangerous.

• Embolization - This involves injecting a substance into the blood vessels feeding the hemangioma to block blood flow and shrink the tumor.

• Medications - Symptoms, like headaches and seizures, can be controlled with medications. The use of beta blockers can stop further growth of the hemangioma and reduce the size and redness, especially in children.

Conclusion

The diagnosis and management of cavernous hemangioma are best done with an interprofessional team including a radiologist, neurologist, neurosurgeon, and pathologist. The involvement of a geneticist can help to evaluate for the hereditary form of the malformation. A rehabilitation specialist can help to improve the functioning of the affected area. A counselor or a mental health professional can help one to manage emotional difficulties that may interfere with daily activities. Asymptomatic lesions may be observed while symptomatic lesions may need surgery. Medications can be used to manage individual symptoms, like headaches and seizures.