Capillary Malformation - Children's Hospital Boston

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My Child Has:

Capillary Malformation

Programs that treat this condition

Vascular Anomalies Center

Sturge-Weber Syndrome Clinic

What is a capillary malformation (CM)?

CM is a flat, sharply demarcated, red-pink vascular stain of the skin. It is sometimes referred to as a "port-wine stain." CM in the face can be confused with a very common vascular birthmark in fair-skinned infants that is located on the forehead, eyelids, nose, or upper lip (called "angel kiss"), or on the back of the neck (called "stork bite"). The difference is that a CM persists whereas the common stains typically fade by one year of age. Viewed through a microscope, a CM is characterized by dilated (widened) capillaries to venous-sized channels in the upper portion of the skin. The walls of the abnormal vessels are very thin and there is a deficiency of the nerves that normally surround the vessels. These vessels slowly enlarge as the child grows older, probably explaining why the color of the stain can darken and the skin can become pebbly.

How common are CMs?

The incidence in Caucasian children is 0.3% of newborns. Sometimes, the CM is hidden because the baby's skin is bright red or if the baby is anemic.

What causes a CM?

The precise cause is unknown. Investigators believe CMs arise because of abnormal formation of the tiny vessels of the skin early in the life of the embryo, after the larger blood vessels are in place. No known food, medication, or activity during pregnancy can cause a CM.

Where do CMs occur?

CM can be anywhere on the body, most commonly in the head/neck region. They can also be present in several areas in the same child.

When should a vascular anomalies specialist be consulted?

If the CM is a problem because of appearance, a laser specialist should be contacted. Most CMs are harmless cutaneous (skin) birthmarks, but some signal an underlying abnormality. If the CM covers part of the forehead and upper eyelid, the infant should be evaluated for the presence of vascular abnormalities of the eye and brain--called Sturge-Weber syndrome. CM overlying the spinal column can be associated with a spinal vascular abnormality (Cobb syndrome). CM can overlie a deeper vascular abnormality involving arteries, veins, or lymphatics, and often there is overgrowth of the involved tissues.

What are the most common complications of CM?

A facial CM can darken in color and the skin can thicken with aging. Tiny vascular growths called "pyogenic granuloma" (which bleed easily) can appear at any age. CM can also be associated with overgrowth of the underlying soft tissues or bones.

How are CMs diagnosed?

These vascular stains are easily diagnosed by sight. They are not easily demonstrated by standard radiographic techniques.

How are CMs treated?

The treatment is "pulsed-dye" laser (PDL). Multiple sessions are necessary and sometimes laser therapy must be repeated. Some physicians prefer to treat the child under a general anesthetic; others use a local anesthetic cream. The overall response rate is 70-80% lightening of the stain. The outcome is better in the facial area than in the trunk or limbs. There is controversy about whether the results are better if laser treatment begins in infancy or childhood.

Laser treatments may minimize the tendency of the skin to thicken and darken in color. Surgical procedures can be useful if there is overgrowth of soft tissue or enlargement of skeletal structures. In older patients, if the skin is very thick and laser is unsuccessful, surgical replacement with skin grafts may be indicated.

What research is being done on CMs?

CMs can be examined for growth factors and receptors that are involved in the formation and repair of these tiny blood vessels. Although such studies help researchers better understand these vascular birthmarks, they are currently not beneficial to an individual child because the abnormality has already occurred. The study of genes involved in formation of blood vessels is more exciting because it has the potential to lead to the development of new treatments. One mutation in a gene called RA5AI causes a type of hereditary CM, sometimes in combination with AVM (CM-AVM).

Telangiectasias:

Hereditary Hemorrhagic Telangiectasia (HHT) - also called Rendu-Osler-Weber syndrome - is a genetic disorder of the blood vessels. It occurs in approximately 1 in 5,000 people and affects males and females from all racial and ethnic groups.

HHT causes abnormalities of the blood vessels. While most blood vessels in a person with HHT are normal, they have a small number that have a specific type of abnormality. A person with HHT has a tendency to form blood vessels that lack the capillaries between an artery and vein. A blood vessel that is abnormal in this way is called telangiectasia if it involves small blood vessels. If it involves larger blood vessels, we call it arteriovenous malformation (AVM).

The abnormality causes bleeding, typically during and after puberty and usually affects skin and mucous membranes (mouth, nose, and gastrointestinal tract). Nosebleeds (epistaxis) are one of the most common types of this bleeding. In HHT, AVMs may occur in the lungs, liver, and brain.

Antibiotic prophylaxis is recommended before dental and surgical procedures for all patients with pulmonary AVMs to prevent potential life-threatening conditions.

More information on HHT from the HHT Foundation International

Cutis marmorata telangiectatica congenita (CMTC), which literally means "marbeled skin," presents as a distinctive deep purple, net-like vascular staining pattern that is present at birth. This vascular birthmark usually involves the trunk and extremities. Ulceration of the depressed purple areas can occur, as can undergrowth of the affected limb and tissue beneath the skin. Almost all infants with this condition show improvement during the first year of life. In time, widening of the veins becomes more prominent and persists into adulthood, along with atrophy (thinning) and staining of the skin.



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