Vascular Tumors / Malformations

One in every ten children is born with a vascular anomaly such as a hemangioma or a vascular malformation. At Ark-La-Tex Craniofacial and Cleft Center, a comprehensive, multi-disciplinary program has been developed to diagnose and treat children and adults with all types of vascular anomalies. Clinical team members work within this team-approach providing state-of-the-art care appropriate for each individual child.

Our team members are skilled at treating a wide variety of conditions including: Hemangiomas, Vascular Malformations, Venular Malformations or Port wine stains, Venous Malformations, Lymphatic Malformations, Arteriovenous Malformations, Klippel-Trenaunay Syndrome (KTS), and Sturge-Weber Syndrome

Treatment:
The Center's physicians use the latest advances in diagnostic, medical, laser, and surgical technologies to customize a treatment plan for each child, including:

• Propanolol Therapy
• Angiography and Embolization
• Local and Systemic Steroid Therapy
• Interferon Therapy
• Laser Therapy
• Sclerotherapy
• Multidisciplinary Surgical Resection

The most common vascular birthmark is a hemangioma, which is a type of benign (non-cancerous) vascular tumor seen in infants. Hemangiomas typically appear within a few days to weeks of the infant’s life and then grow rapidly. This is known as the “proliferative phase” and usually lasts for six months to a year. Then the tumor enters into the “involuting phase” which is the slow shrinking of the hemangioma. Most hemangiomas regress, or get smaller, without any intervention. This phase can last 10 years. Hemangiomas may leave behind scarred or saggy skin when they involute. If the hemangioma is not significantly smaller and less noticeable by the time the child is of school age, treatment may be considered to reduce psychological impact.

Hemangiomas represent a localized growth of abnormal endothelial cells. These are the cells that normally line the inside of blood vessels. Hemangiomas can vary in appearance depending on location. If they are on the surface of the skin, they can have a bright red “strawberry” textured appearance. If they are under the skin, they often look like a bruise or bluish swelling. Children with three or more hemangiomas need to have an ultrasound evaluation to look for any internal lesions. Large hemangiomas should prompt a search for other types of problems. They can be associated with vascular malformation syndromes such as PHACE, Klippel-Trenaunay, or Sturge-Weber syndromes. If children with multiple hemangiomas or large hemangiomas are not growing and gaining weight as expected, or are not reaching their developmental milestones, they should be seen by a multidisciplinary team equipped to treat vascular anomalies.  

There are a few special types of hemangiomas which are different than described above.  In some cases, a fully formed lesion is present at birth.  These “congenital hemangiomas” may not have a growth phase, and may involute rapidly.  Some congenital hemangiomas will never involute and require surgery.  Any hemangioma that does not follow the typical growth pattern should be evaluated by the craniofacial team. Small localized hemangiomas can often be treated with surgery.  Medications such as corticosteroids, beta blockers, or chemotherapeutic agents are used for complicated or large lesions.

Vascular malformations are the other type of vascular birthmark, and are entirely different from hemangiomas. They can occur anywhere in the body. They can occur in isolation or as part of another disease. Typically, the malformation grows in proportion with the child and may not be noticed for months or years. Often they cause little to no problems. Sometimes the mass may start growing out of proportion, occasionally from a triggering factor like trauma, puberty, or pregnancy, though often no triggering factor is identified. Some malformations can however grow rapidly, so if a vascular malformation is suspected that child should be referred to the appropriate medical specialist for evauation. 

Vascular malformations can be broken down into several sub-groups depending on what type of vessel is involved.  Vessel types include: venous malformations, lymphatic malformations, arterial malformations, capillary malformations, or a combination of these types.

Venous malformations are one of the most common vascular anomalies. A VM is an abnormal formation of tangled, dilated veins somewhere within the body. These abnormal veins often lack smooth muscle along the vessel wall, causing them to enlarge and rupture easily. The center of the tangled veins is called the nidus.  There are a few options for treating VMs depending on the size, location, and type of symptoms the person is having. Usually, VMs are not treated unless there is a perceived risk, like possible rupture, or if the person is symptomatic. The two most common methods of treatment are surgery and sclerotherapy. Surgery is the gold standard. Complete removal of the VMs’ nidus is required to achieve an effective cure. Depending on the mass’ size and location, this can be very challenging if not nearly impossible by surgery alone. Sclerotherapy uses a chemical solution to cause destruction of the blood vessel walls and rapid thrombosis, or clotting of the nidus. Typically, sclerotherapy involves a simple injection through a small needle into the mass but it takes several treatments before it is effective.

Arteriovenous malformations (AVMs) are abnormal connections between arteries and veins. They are believed to be caused by a mistake in the formation of the normal arterial-capillary-venous connections that occurs during early pregnancy. The cause of most AVMs is not known.  In childhood, the blush of an AVM can be mistaken for hemangioma. AVM becomes obvious as signs of fast blood flow appear. The skin may become a darker red or purple color, veins nearby may enlarge, a mass may appear beneath the birthmark, a pulse may be felt, and the area may be warm.There are some genetic syndromes which are associated with AVMs.  One is called hereditary hemorrhagic telangiectasia (HHT or Rendu-Osler-Weber syndrome). Affected children usually present with telangiectasia of the skin and mucous membranes (mouth and gastrointestinal tract). AVMs can also occur in the lungs, liver, and brain. Parkes Weber syndrome involves an AVM of a limb with overgrowth of that limb. The affected limb usually has a capillary malformation as well. When the amount of blood passing through the AVM is very large, the heart is affected because it has to handle more blood flow (high-output cardiac failure). AVM are treated techniques such as embolization, sclerotherapy, surgery, or a combination of these techniques.

Lymphatic malformations (previously known as cystic hygromas) can be seen on ultrasound before the baby is born.  It is critical that you arrange for your baby to be evaluated at a medical center with a vascular birthmark team who can work with your obstetrician to ensure a safe delivery and newborn period. Treatment involves sclerotherapy and/or surgery.

Capillary malformations (previously known as port wine strains) are flat and pink in the newborn period.  Laser therapy can be very effective in young children to fade the lesion.

Each type of malformation is treated differently and even two malformations of the same type need an individualized treatment plan depending on the size, location and symptoms. The treatment plan must be specialized for each patient and usually required coordination by a number of different specialists on our team.