Arterio Venous Malformation (AVM)

Oleh: Khodijah Yahya Dwi Prasetyo N. M. Abdul Basith Wildan Syamsuddin F. Syamsudduha

Pembimbing: dr. Sri Lilijanti W, Sp.A

Overview
Arteriovenous Malformation (AVM) are abnormal connections between arteries and veins that are congenital (present at birth).
They don't have the normal network of tiny vessels (capillaries) that normally connect arteries and veins.

AVMs are more common in the brain than in other regions.

The cause of AVMs is unknown.

What is an AVM?
NORMALLY: Blood flows from the heart through large arteries to all areas of the body.
The arteries branch and get smaller until they become a capillary.

The blood travels from the capillary bed back to the heart through veins

The capilarry bed is where the blood exchange oxygen and nutrients with the body tissue and pick up waste.

What is an AVM?
IN AVM: Arteries connect directly to veins without capillary bed in between.
This create a problem called a high pressure shunt or fistula. Veins are not able to handle the pressure of the blood coming directly from the arteries. The veins stretch and enlarge as they try to accept the extra blood. The weakened blood vessels can rupture and bleed and also more likely to develop an aneurysms.

How common are AVM?

AVM are relatively rare birth defect.

They occur approximately once in every 500 births.

What causes AVM?

They are believed to be caused by a mistake in the formation of the normal connections between arteries, veins and capillaries that occurs during early embryonic life.

Unknown

No known food, medication, or activity during pregnancy can cause an AVM.

What are the symptoms of AVM?

AVM develop before birth. Usually an AVM is seen as an innocent stain on your childs skin at birth; often they begin to enlarge in childhood and adolescence. Intracranial (within the head) AVM have no visible symptoms. However, they may cause headaches or sudden bleeding into the brain.

AVM outside the brain appear more slowly with color changes, then bleeding and persistent pain.

Cont..
AVM can occur in any organ in the body. The occur most frequently in the parts of the body below (in order of frequency):

In time, if the blood flow through an AVM is excessive, your childs heart can become overworked.

Head Limbs Trunk Internal organs

Cont..

In infancy and childhood, the blush of an AVM can be mistaken for hemangioma (the most common tumor in babies).

The AVM becomes obvious as signs of fast blood flow appear. The skin becomes a darker red or purple color, nearby veins enlarge a mass appears beneath the stain, and there is local warmth.

What problems are associated with AVM?

Hereditary hemorrhagic telangiectasia (HHT)
A fairly common familiar vascular disorder. Children with HHT can develop AVMs in the lungs, brain, and gastrointestinal track.

Parkes Weber syndrome

In this disorder, several AVMs plus overgrowth of a limb combine to put extra strain on the heart.

Our research collaborators have identified a gene that is changed in about one half of the patients with this syndrome.

Wyburn-Mason syndrome (BonnetDechaume-Blanc syndrome)

A rare combination of a vascular abnormality in the retina and an AVM in the brain.

Its sometimes accompanied by a facial capillary stain and/or overgrowth on one side of the body.

Cobb syndrome
The combination of a cavernous malformation (fragile arteries/veins) of the skin with an AVM of the spinal cord.

How are AVM diagnosed?

Your childs doctor may be able to tell with a physical examination of your child. Around the area of the AVM, the skin is warm and, in time, the pulsation of blood flow can be felt.

After the exam, your childs doctor will probably want to do one or more of the following procedures:
Angiography The most precise test to see the details of the AVM. Your childs doctor will inject a contrast dye that can be seen by x-ray fluoroscopy into the AVM via a flexible tube that is usually inserted in your childs groin.

Ultrasonography (USG) To detect the rapid flow of blood through an AVM.

Magnetic resonance imaging (MRI) This test gives the most information about the extent of the anomaly.

How are AVM treated?

Treatment often depends on where your childs AVM is located. Intracranial AVMs
Radiation Embolization (injection of material into the center of the anomaly via a catheter) Surgery

AVMs in other parts of the body

Embolization Sclerotherapy (direct injection of an irritating solution) Surgery

Wherever possible, surgery provides the best treatment.