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Vascular Malformations

Overview

A vascular malformation is a condition in which blood channels grow abnormally. They may be found in big arteries and veins, smaller channels known as arterioles and venules, tiny capillaries, and/or lymphatic tubes that transport lymphatic fluid and WBC outside the arteries and veins.

Vascular abnormalities are uncommon, accounting for less than 1% of the population in some cases. Many forms of vascular malformations don't create symptoms until they're activated by situations like puberty, pregnancy, significant surgery, trauma, or illness. If you have one, it most likely developed before you were born. Vascular malformations are caused by the improper growth and expansion of a single kind of vessel or a group of vessels. Depending on the type of vessel involved, abnormalities might get bigger and become troublesome over time. They might be benign and small or can be quite dangerous, even life-threatening.

Types

The easiest way to classify vascular abnormalities is by the type of channels involved and how blood flows through them.

Capillary Malformation

Capillary malformations impair the capillaries in the dermis, resulting in a macular, pink, or purple tint that appears at birth and lasts the entire life. Capillary malformations, which affect 0.5 percent of people, were called "Port-wine stain" earlier.

Venous Malformations 

Venous abnormalities are present from birth, the same as other vascular defects. They are the most prevalent type of vascular abnormality, affecting 1% to 4% of the population. Clinically, they present as blue, soft, spongy lesions on the head, limbs, or trunk.

Lymphatic malformations

Lymphatic malformations are lymphatic fluid-filled vascular tubes, pouches, or vesicles with a single endothelial cell layer, with 75 percent of cases occurring in the cervicofacial area. The quantity of lymphatic fluid present and the amount of bleeding or inflammation causes lymphatic abnormalities to expand and shrink. As a result of intralesional hemorrhage, these lesions are typically visible at birth and look like tiny, crimson tent nodules.

 Arteriovenous malformations

This shunt is normally present from birth, but it isn't noticeable until late adolescence. Somewhat compressible and pulsatile with a detectable thrill, arteriovenous malformations can be seen. The most prevalent location for this sort of lesion is intracranial, and it can grow in response to specific triggers such as trauma or adolescence. AVMs can arise in soft tissues or skeletal bones and usually accompany recurrent bleeding episodes rather than pain.

Causes

All of these characteristics are present from birth and become visible at various times. When coupled with Hereditary Hemorrhagic Telangiectasia, the pulmonary arteriovenous malformation is transmitted genetically. A lot of research is also being conducted on the genetics of various abnormalities. The majority of them are only recognized as something that happens during the formation of the arteries, veins, and/or lymph vessels, for no known reason.

Risk Factors And Epidemiology

A family history of AVMs might raise your risk. The majority of AVMs, however, are not inherited. AVM is more common in people with certain genetic disorders. Osler-Weber-Rendu syndrome is one of them.

The current estimates for new AVM detection are 1 per 100,000 people per year. The population incidence is around 10 per 100,000, implying that there are approximately 30,000 people in the United States.

Signs And Symptoms

The following signs and symptoms may occur alongside bleeding;

·         Neurological function deteriorates with time.

·         Headaches

·         Vomiting and nausea

·         Seizures

·         Consciousness impairment

·         Muscle weakness

·         Loss of balance in one portion of the body and may lead to movement issues.

·         Chest pain

Diagnosis

Doctors frequently prescribe imaging scans to aid with diagnosis, such as:

Brain Angiography

A contrast agent, which is injected into an artery, is used in this test. The dye enhances the appearance of blood vessels on X-rays by highlighting their structure.

CT scan

It employs X-rays to generate pictures of the head, brain, or spinal cord and can detect bleeding.

Heart catheterization

An imaging technique that includes the injection of dye into the blood arteries of the heart to highlight them on X-rays.

Magnetic resonance imaging

This scan employs strong magnetic and radio signals to produce comprehensive pictures of the tissues. Tiny differences in these cells can be detected using an MRI.

Magnetic resonance angiography

An MRA records the pattern, as well as the distance and speed, of a moving object.

Differential Diagnosis

There are many conditions that can present as vascular malformations, such as:

·         Anterior Circulation Stroke

·         Cardioembolic Stroke

·         Cavernous Sinus Syndromes

·         Cerebral Amyloid Angiopathy

·         Dissection Syndromes

·         Subarachnoid Hemorrhage

·         Cerebral Aneurysms

·         Cerebral Venous Thrombosis

·         Chronic Paroxysmal Hemi crania

·         Cluster Headache

Treatment

Your doctor will perform regular imaging and clinical examinations to monitor an AVM for changes or complications. AVMs that cause bleeding or other symptoms must be treated.

Medications

Seizures, headaches, and back discomfort can all be managed with medication.

Procedures

Surgery is the most common therapy for AVM. If you're at a high risk of bleeding, your doctor may suggest surgery. The operation has the potential to eradicate the AVM totally. This therapy is frequently employed when AVM is in a region where surgeons may remove it without inflicting severe harm to the brain structures.

Endovascular embolization

Endovascular embolization is a procedure in which a catheter is threaded into the arteries and into the AVM. Then, to restrict blood flow, a chemical is administered to seal sections of the AVM. This may also be done before major surgery or radiation treatment to lessen the chance of complications.

Stereotactic radiosurgery

AVMs are sometimes treated using stereotactic radiosurgery. The blood vessels are damaged and the blood flow to the AVM is cut off using powerful, highly concentrated beams of radiation.

Prognosis

They could go away on their own if you don't treat them. Vascular abnormalities progress slowly over time. They don't get any smaller. They generally need medical help. Although most individuals with the illness live reasonably normal lives, they are always in danger of tangles bursting and bleeding into the brain, resulting in a stroke. Every year, around one out of every hundred patients, has a stroke.

Lifestyle Modifications

Inquire with your physician about the location and size of your AVM and the implications of these factors for your treatment choices. AVM complications, including bleeding and stroke, can create emotional as well as practical concerns. Relaxation therapy and talking therapy are beneficial in such instances. Not just for patients and family members but also for treating physicians, vascular abnormalities are a cause of significant concern and anxiety. The importance of an accurate diagnosis and a comprehensive approach to therapy can indeed be stressed.

Our clinical experts continually monitor the health and medical content posted on CURA4U, and we update our blogs and articles when new information becomes available. Last reviewed by Dr.Saad Zia on June 02, 2023.

References

Vascular Malformations | Children's Hospital of Philadelphia (chop.edu)

https://www.chop.edu/conditions-diseases/vascular-malformations#:~:text=Vascular%20malformations%20are%20a%20type,combinations%20of%20these%20blood%20vessels.

Vascular Malformations and Hemangiomas (nationwidechildrens.org)

https://www.nationwidechildrens.org/conditions/health-library/vascular-malformations-and-hemangiomas