“Vascular anomalies” is a general term often used to describe a number of different abnormalities that occur in children or young adults. Appearing as a cosmetic blemish or mass, possibly gaining attention due to interfering with the area’s normal functions with pain, swelling, or bleeding, vascular malformations are often the result from abnormal development in blood vessels, lymph vessels, veins, and/or arteries.
A true vascular malformation is usually present at birth, grows proportionally with the child, does not go through a rapid growth phase in infancy, and does not disappear. Vascular malformations may become apparent later in life as blood flow increases through abnormal connections between arteries and veins. Vascular malformations may also be the result of fluid or blood accumulating in poorly formed veins or lymphatic channels. Vascular malformations may cause cosmetic deformities of the head and neck and interfere with normal function in these areas. They also may occur in other areas of the body and may cause pain, swelling, and bleeding.
Because of the complexity of many vascular anomalies, treatment requires a collaborative approach and input from numerous specialties, including pediatrics, pediatric orthopedics, plastic surgery, and endovascular surgery. Understanding that no two patients are exactly alike and that each person’s symptoms are unique, our physicians in the Cerebrovascular Center at Mount Sinai have specific training, skills, and expertise to treat simple and complicated cases. Depending on the condition’s severity, treatment options will vary.
Diagnosing Vascular Malformations
Vascular malformations are possibly influenced by hormonal changes occurring during puberty and pregnancy and may also be the result of fluid or blood accumulating in poorly formed veins or lymphatic channels. Usually present at birth and growing proportionally with the child, true vascular malformations do not go through a rapid growth phase in infancy and do not disappear. Vascular malformations may become apparent later in life as blood flow increases through abnormal connections between arteries and veins. With the use of MRIs, malformations deep in the body may be diagnosed.
Improving Appearance and Alleviating Symptoms
Unlike hemangiomas, most vascular malformations require treatment. While not all vascular malformations can be completely eradicated, modern treatment techniques can improve the appearance of the patient and alleviate the symptoms of pain, swelling, and bleeding. Many children and adults with vascular malformations achieve high levels of performance at school, in sports, and in their careers.
Because vascular malformations are rare and complex, optimal results are achieved at centers specializing in their diagnosis and treatment. Each patient with a vascular malformation is unique and requires the attention of professionals with specific training, skill, and experience in this field and access to the most advanced equipment and materials.
Treatment Options for Vascular Malformations
The pediatric cerebrovascular team provides the latest and most advanced therapies for treating all categories of vascular malformations.
Most importantly, our multidisciplinary team uses thoughtful consideration in the staging of treatment, often employing many stages and treatment combinations carefully slated for the right problem, symptom, location, and age of the patient.
These treatment modalities include:
- Embolization closes the abnormal blood vessels from the inside using “glues” or particles. At Mount Sinai, we use a minimally invasive technique called embolization. Although this technique can close the entire abnormality in some patients, for others, the malformation may need to be surgically removed or treated with a special type of radiation (radiosurgery). In these cases, embolization prior to either of these approaches can shrink the malformation and reduce the risk of bleeding if surgical removal is required.
- Laser treatment can be effective for treating superficial venous malformations or the superficial component of a deep lesion. Our physicians have extensive experience in navigating the very narrow therapeutic range that is unique to each patient. The lasers we use are capable of effectively reducing the bluish discoloration of the lining of the lip and mouth and the skin. By using a cooling device together with the laser, the skin is spared and it is possible to treat the vascular malformation through intact skin. In a recent innovation, our center often combines laser treatment with surgery and sclerotherapy to successfully treat compound venous malformations.
In lymphatic malformations, lasers are primarily used to treat the small blisters that appear on the surface of the tongue and lining of the mouth. Since the lymphatic malformation may involve the entire thickness of the tongue, this treatment may not be curative, but it significantly improves the quality of life of the patient (resolving leaking of blood-stained fluid and the inability to eat a wide range of foods). The treatment is administered under general anesthetic and is usually done every few years. After a patient heals, most or all of the blisters disappear.
- Sclerotherapy is often used in treating venous malformations and lymphatic malformations. A substance is injected through the skin into the abnormal vessel of the malformation. The substance can be one of many that are able to irritate the wall of the vessel, which results in the formation of a blood clot. The blood clot will effectively stop the flow of blood through the vessel and the inflammation of the inner wall will destroy the vessel, which the body replaces with scar tissue (sclerosis).
Surgical removal of a venous malformation can be staged (done in stages over a few months) or, if possible, in one operation. Our physicians have recently perfected a treatment, whereby the lesion is first treated with sclerotherapy and then, within 24 to 48 hours, is surgically removed. The addition of sclerotherapy in this way clots the lesion off, thereby reducing the risk of severe blood loss during surgery. This technique reduces the number of treatments necessary, since the area that undergoes sclerotherapy is being removed. The results of this combined treatment have been excellent and we have been able to remove lesions that were previously considered inoperable. In addition, our experience shows, more and more lymphatic malformations are treated only with sclerotherapy, avoiding large disfiguration scars, than with surgery. With respect to microcystic (small cysts) lesions, recurrence rates after surgery are usually high and multiple procedures are usually necessary. The use of sclerotherapy using our gravity technique has revolutionized the treatment of microcystic lesions.
- Surgery can be an option alone or after embolization for vascular malformations. When the combination is elected, embolization is followed 24 to 48 hours by surgical resection of the nidus. If the condition is extensive, it is important to resect only enough tissue to prevent recurrence, but avoid disfigurement. In appropriate cases, angiography is repeated after surgery and, if needed, embolization may be done for more long-term stability.
Combined Vascular Malformations
Many patients have vascular malformations that are made up of different types of abnormally formed vascular channels. One of the most common forms is Klippel-Trenaunay syndrome (also called CLVM because it involves malformations of the capillaries, lymphatics, and veins). Patients with this condition present in different ways, depending upon the type and severity of their anomalies. Treatment often requires input from numerous specialties, including pediatrics, pediatric orthopedics, plastic surgery, and endovascular surgery. When symptoms are related to malformations of the veins, endovenous laser and venous embolization or sclerotherapy can be effective.