Hemangiomas
What are they? | What do they look like? | How are they diagnosed? | What are possible complications? | Should my child receive treatment? | How are they managed? | Risks and side effects
What is a hemangioma?
Hemangiomas of infancy are benign (non-cancerous) vascular tumors composed of cells that normally line the blood vessels (endothelial cells). They are the most common tumor of childhood, occurring in up to 10% of infants. Hemangiomas are seen in all racial groups but are more common in Caucasians. They also occur more frequently in girls and low-birth-weight (less than 1,000 grams) premature infants.
Hemangiomas of infancy may be visible at birth or may not be recognized until the first few weeks or even months of life. In general, however, most hemangiomas become evident by 2 to 3 weeks of age. Although they commonly occur on the head (illustrations 1-3) or neck (illustration 4) (60%), they can occur in any region of the body (illustrations 5-6). Some lesions are small and hardly visible, whereas others are large and readily observable (illustration 7). Most hemangiomas appear as single tumors, though 15% present with more than one lesion.
The cause of hemangiomas is not yet understood, but it is known that they are not related to drugs or medications that may have been taken during pregnancy, nor are they related to any known environmental exposures that may have occurred during that time. Although no specific gene abnormality has been identified, in some families multiple family members over several generations have been affected.
Hemangiomas of infancy almost always have a period of growth, which is referred to as their proliferative phase. This is followed by a period of shrinkage, referred to as their involutive phase (illustrations 8-10). Most hemangiomas begin their proliferative (growth) phase shortly after birth. This phase usually lasts for 4 to 6 months, but can be longer or shorter. Each lesion has its own growth timetable. The involutive phase is much slower and can take as long as ten years. It is difficult to predict how long this shrinkage process will last. Lesions that involute slowly, over a long period of time, are much less likely to shrink completely. In some children, even those hemangiomas that do shrink completely may leave residual fatty tissue and telangiectasias (tiny dilated blood vessels) of the skin.
Rarely, hemangiomas are fully grown at birth and do not increase in size (illustration 11). These tumors are called congenital hemangiomas. Unlike hemangiomas of infancy, congenital hemangiomas either involute rapidly or not at all. Rapidly involuting congenital hemangiomas are referred to as RICH. Non-involuting congenital hemangiomas are referred to as NICH.
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What do hemangiomas look like?
We classify hemangiomas as superficial, deep, or combined, depending upon the involvement of the skin and other soft tissues. If the lesion is near the skin surface, it is referred to as a superficial hemangioma. If it grows in the lower layers of the skin or in muscle, it is called a deep lesion. When it has components of both superficial and deep lesions, we refer to it as a combined lesion. Superficial lesions appear as bright red, raised or flat patches on the skin or a textured area that resembles the appearance of a strawberry. Veins radiating from the lesion also may be visible beneath the skin. Deep hemangiomas have a gray to blue hue, but due to their depth they may not be noticed for many weeks or months after birth, although they are growing. This type of lesion is firmer and more rubbery to touch, and its color does not completely disappear when you press on the area. Combined lesions may have a layered combination of red on top and blue below. As hemangiomas involute, they undergo a change in consistency and color, becoming softer and grayer. Eventually, the lesion is replaced internally by fatty tissue. The more visible involved skin is replaced by thin skin that appears more normal.
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How are hemangiomas diagnosed?
In most cases, a diagnosis is made by correlating medical history with physical examination findings. If there is any uncertainty in distinguishing a hemangioma from other lesions or vascular malformations, ultrasonography (a non-invasive diagnostic tool) usually provides a definitive answer. In certain cases, magnetic resonance imaging (MRI) or computed tomography (CT), which are radiological tests, may also be necessary to make a diagnosis and determine the extent of the lesion. If there is any question regarding the possibility of a malignant (cancerous) growth, a biopsy will be performed. This involves removing a small section of tissue from the lesion for microscopic examination by a pathologist. Ultrasonography is performed with the child awake, while MRI and biopsy require general anesthesia. CT is performed under sedation.
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What are the possible complications?
Although complications are uncommon, they can occur. Possible complications include ulcerations (skin breakdown) (illustration 11), obstruction of vital functions such as vision, hearing, or breathing, and distortion of facial features. When there is extensive involvement of the liver, heart failure may occur.
Problematic hemangiomas
Certain patterns of hemangioma presentation and certain locations are more problematic and more likely to cause complications. Children with these hemangiomas should be evaluated by a multidisciplinary team of pediatric specialists with special expertise in treating vascular anomalies.
Multiple skin hemangiomas often are associated with hemangiomas of internal organs, including the liver, lungs, and intestines. Rarely, even the brain is affected.
Hemangiomas on the face that present in a distribution resembling a beard can be associated with airway involvement.
Some hemangiomas over the face and neck are associated with PHACES syndrome. This syndrome involves coexisting abnormalities of the posterior brain, the great vessels of the heart, the eye, and chest wall.
Hemangiomas located over the lower back and spine can be associated with spinal defects.
Hemangiomas involving the genital area can be associated with pelvic and urinary system abnormalities.
Extensive liver hemangiomas can be associated with heart failure and hypothyroidism (low thyroid hormone).
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Should my child receive treatment for a hemangioma?
Most hemangiomas involute completely and do not require treatment. Nevertheless, all children with significant hemangiomas should undergo a thorough evaluation and continued observation. Decisions as to whether or not to treat and when to treat must be made on an individual basis, with consideration given not only to the physical functioning of the child, but also to prevention of permanent disfigurement and the emotional impact of the lesion.
In a small percentage of patients in whom hemangiomas disappear completely, residual fatty tissue or superficial skin telangiectasias remain. To improve appearance, these patients may require surgery and/or laser therapy some time during childhood.
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How are hemangiomas managed?
Treatment is dependent upon both the stage of growth (proliferation vs. involution) of the lesion, the presence of complications, and emotional needs and considerations. Observation, laser therapy, drug therapy, and surgical removal are the four most common management options. In some patients, a combination of several of these treatments may be used over a period of time.
- Observation. In most cases, observation is the best course to follow, especially when the hemangioma is involuting and your child's functioning is not impaired.
- Laser therapy. Laser therapy is used to treat ulcerated hemangiomas and superficial residual skin telangiectasias. This treatment option has not proven to be effective during the early growth phase of hemangiomas and can cause significant scarring.
- Drug therapy. A class of steroid medications called corticosteroids may be used to slow the growth and decrease the size of the lesion as much as possible until the growth phase is over. This medication may be applied topically (placing ointment on the skin), taken orally, or given by injection. When there is a dramatic response to treatment, the hemangioma begins to shrink and appear pale in two to three weeks.
In patients with massive or potentially life-threatening hemangiomas, one of several approaches is used. In some children, vincristine (a drug that has been used to treat cancerous growths) is sometimes given. In other children, interferon (an anti-viral drug that has been found to decrease vascular growths) may be used. - Surgery. Surgery may be advised for selected patients, including: (1) children with painful and ulcerated hemangiomas that do not heal; (2) children whose lesions interfere with breathing, vision, or other vital functions; (3) those who have hemangiomas that do not completely involute; and (4) those in whom hemangiomas have resulted in unacceptable scarring or deformity.
Ulcerated hemangiomas may require aggressive wound care, treatment with topical antibiotics, laser therapy, and/or surgical removal.
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What are the risks and side effects of treatment?
While each of therapies mentioned above can be effective, each also involves certain risks or side effects.
- Laser therapy. Early intervention with laser therapy may result in some scarring and changes in skin pigmentation. These can often (but not always) be corrected by later cosmetic procedures.
- Drug therapy. The most common side effects of oral corticosteroids are irritability, swelling of the face, stomach irritation, and slowing of growth. All of these side effects disappear when the medication is stopped. Because of increased susceptibility to infection while on steroids, live-virus vaccines should be avoided during steroid treatment.
Vincristine can cause constipation, abnormalities in body salt levels, and neurologic symptoms.
Interferon, which is used only when there are no other options, may result in fever, flu-like complaints, and agitation. It may also result in more serious neurologic problems such as spasticity. - Surgery. Depending on the location and extent of the hemangioma, complications such as bleeding and infection may occur. Although surgical removal of a hemangioma results in significant improvement of appearance, there may be some scarring. When scarring is severe, it may be improved by subsequent scar revision surgery.
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Rev. 12/04, 6/07
