Blood Vessel Disorders

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Overview

Blood vessel proliferations (hemangiomas) are the most common benign tumors in infants and children. About 10% of infants have them, and they are usually present at birth or within the first 2 or 3 months of life. Hemangiomas undergo a rapid phase of growth followed by a slow period of clearing up or disappearing (called involution). Most hemangiomas are solitary tumors that appear most commonly on the head and neck, but they may also appear on the trunk and extremities.

Causes

Hormones most likely influence the rapid development and proliferation of blood vessels that result in a hemangioma. The exact mechanism of enlargement and ultimate involution is not entirely understood. Female infants are three times more likely to have a hemangioma. Hemangiomas are seen in almost 25% of premature infants who have a birth weight of less than 2 pounds.

Signs and Symptoms

Hemangiomas most commonly appear during the first month of life. The initial phase of rapid proliferation typically lasts less than a year. During this time, the hemangioma can grow surprisingly large. Over the next few years, the tumor begins to involute and completely regresses by the time the child is 5 - 10 years of age. The lesion may heal with no perceptible scar, or it may leave a gray, fibrous residue with various small blood vessels. The skin may appear loose or slack.

Hemangiomas, sometimes called strawberry hemangiomas, can be deep or superficial. A deep hemangioma is a blood vessel proliferation that occurs in the deeper layers of the skin. It can be flat or slightly raised and may appear bluish or dusky, and blood vessels may be visible. Superficial hemangiomas are bright pink or red in color and protrude from the surface. Some hemangiomas are both superficial and deep.

Multiple hemangiomas occur in 20% of cases. There may be a few or numerous small lesions appearing on the skin. They may involve both the skin and the internal organs. Involution of multiple hemangiomas occurs more rapidly than involution of solitary lesions and is completed by the time the child has reached 1 to 2 years of age.

Complications

Complications include ulceration, bleeding, and infection with rapidly growing hemangiomas. A hemangioma around the eye can prevent an infant from opening it, permanently endangering the child's vision and requiring aggressive treatment. Similarly, when a hemangioma blocks the oral or nasal passages, or the genital or anal areas, treatment is critical.

Rare complications

Rarely, there have been associations with abnormalities of underlying bone structure and with a disorder of blood clotting, which is likely caused by blood clotting cells (platelets) collecting in the hemangioma, reducing the presence of platelets elsewhere in the body. In these cases, treatment of the clotting disorder and the hemangioma should be initiated rapidly, because this condition can be life-threatening. Hemangiomas on the liver can put stress on the organ, resulting in increased blood flow to the heart and potential heart failure.

Diagnosis

Hemangiomas are usually diagnosed based on the patient's clinical history and presentation. A biopsy of the lesion can be done, as well as an ultrasound examination. Magnetic resonance imaging (MRI) is the most useful examination because it provides a picture of the hemangioma and adjacent structures.

Treatment

Because most hemangiomas regress completely on their own, there is usually no treatment. The final result of involution is often more cosmetically acceptable than if the hemangioma has been removed surgically. The decision to treat a hemangioma is based on several factors including location, size, and complications. The risks and benefits of treatment should be weighed carefully. Despite parental pressure, treatment is reserved for hemangiomas that are life-threatening, that distort or obstruct important structures, and that ulcerate.

Oral corticosteroids are first-line therapy for hemangiomas that require treatment. They are administered at high doses for 4 to 6 weeks and are gradually tapered off. If the medication is discontinued too soon, the hemangioma can regrow. Not all hemangiomas respond to this therapy, about one-third respond rapidly, but the rest respond minimally or not at all.

Corticosteroids can be injected directly into the tumor, usually once a month for several months. This may help resolve ulcerated hemangiomas.

Interferon-alpha is a possible treatment for a hemangioma that does not respond to other therapies. It is reserved for those with a life-threatening lesion that obstructs a vital structure. Side effects can be experienced with this medication, and they should be thoroughly explained to, and understood by the child and parents before using this therapy.

Lasers can be used to treat hemangiomas, but their effectiveness has not been established. Furthermore, laser treatment is not effective for the deeper components of lesions. Laser therapy can successfully treat residual blood vessels after involution and is helpful in reducing the pain and healing time of ulcerated lesions.

Hemangiomas can be surgically removed (excised), although surgery is reserved for critical cases and for those in which the surgical scar is more cosmetically acceptable than that left by natural regression.

Prevention

There is no known method for preventing hemangiomas. Infections in ulcerated lesions can be avoided with meticulous care.

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