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Sturge Weber Syndrome

Portwine Stain (Capillary Malformation)

AVM (Arteriovenous Malformation)

AVF (Arteriovenous Fistula)

Parkes Weber Syndrome

Sturge-Weber syndrome (also called "encephalotrigeminal angiomatosis") is a congenital disorder characterized by a vascular birthmark and neurological abnormalities. Symptoms of the disorder, which vary widely among patients, may include eye and internal organ irregularities. The most apparent symptom is a facial birthmark or port wine stain which is present at birth and usually involves at least one upper eyelid and the forehead. The stain, varying from light pink to deep purple, is caused by an overabundance of capillaries just beneath the surface of the affected skin. Neurological symptoms include excessive blood vessel growth on the surface of the brain (angiomas). These angiomas are typically located on the posterior or occipital region of the brain and cause seizures, which often start before one year of age and may worsen with age. The convulsions usually appear on the side of the body opposite the port wine stain and vary in severity. A weakening or loss of use of the side of the body opposite the port wine stain (hemiparesis) may also develop. Developmental delay of motor and cognitive skills may occur. Glaucoma (increased pressure within the eye) may be present at birth or develop later. Buphthalmos (enlargement of the coatings of the eye) may also occur in the eye that is affected by the port wine stain. Sturge-Weber syndrome rarely affects other body organs. ©2005 NINDS

Please visit these links for more details on Sturge Weber Syndrome

Sturge Weber Foundation

Sturge Weber Community

What is a Portwine Stain?

A portwine stain or capillary malformation is a vascular birthmark or lesion that is caused by an excess amount of blood flow in the capillaries just beneath the skin. The color of this birthmark is caused by the excess blood flowing through the capillaries and can range from light pink to dark purple. Portwine stains develop in the first few weeks of gestation. There are some studies that correlate the excess capillary growth with a nerve deficiency. Because portwine stains are vascular birthmarks, they are not curable. Pulsed dye laser is the treatment of choice for the management of the birthmark. The laser temporarily shrinks the capillaries, causing a lightening of color. The capillaries usually will eventually grow back; thus the need for repeated laser treatments and maintenence.

Please visit these links for more details about Portwine Stain birthmarks

Birthmarks.com

Vascular Birthmarks Foundation

What is an Arteriovenous Malformation (AVM)?

Arteriovenous malformations (AVMs) are defects of the circulatory system that are generally believed to arise during embryonic or fetal development or soon after birth. They are comprised of snarled tangles of arteries and veins. Arteries carry oxygen-rich blood away from the heart to the body’s cells; veins return oxygen-depleted blood to the lungs and heart. The presence of an AVM disrupts this vital cyclical process. Although AVMs can develop in many different sites, those located in the brain or spinal cord—the two parts of the central nervous system—can have especially widespread effects on the body.

AVMs of the brain or spinal cord (neurological AVMs) are believed to affect approximately 300,000 Americans. They occur in males and females of all racial or ethnic backgrounds at roughly equal rates. ©2005 NINDS

Please visit these links for more information about AVM's

Center for Endovascular Surgery: Arteriovenous Malformation (good diagram)

Nucleus Health Information - Arteriovenous Malformations (2003)

Arterio Venous Malformations of the Brain and Spine

Interactive MRI slices of an AVM patient's brain

What is an Arteriovenous Fistula (AVF)?

An arteriovenous fistula (AVF) is a direct connection between an artery and a vein. The blood bypasses the capillaries and tissues, and returns to the heart. Arterial blood has a higher blood pressure than veins and causes swelling of veins involved in a fistula. AVF's are similar to AVM's in that they both disrupt the circulatory cycle. An AVF is characterized by a single connection between and artery and a vein, whereas an AVM contains multiple arteries and veins. Larger AVF's can cause heart failure.

Arteriovenous Fistula

eMedicine - Vein of Galen Malformation (AVF): Article by Raj D Sheth, MD

Vein of Galen Support Group

What is Parkes Weber Syndrome?

Parkes Weber Syndrome is characterized by a capillary malformation (PWS) that has a underlying high flow vascular malformation (AVM). This syndrome causes hypertrophy (overgrowth) of the affected area. When trying to find information about this syndrome on the web, it is usually combined in an article or web site dedicated to Klippel Trenaunay Syndrome, though the two are different.