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Hemangioma Information

Large Hemangiomas on the Face

Hemangiomas on the face are considered large when they are >5cm (about 2 inches) in size. When they are large or follow certain patterns, it may mean there is a chance that they are associated with other problems such as PHACE syndrome (see below). You should also see your doctor right away about hemangiomas in certain locations such as around the eye, on the nose, or on the lips or lower part of the face to determine if treatment might be needed.

PHACE Syndrome

Some hemangiomas, particularly larger hemangiomas located on the face, can have other associated problems. PHACE syndrome, first described in 1996, refers to the association of large infantile hemangiomas (usually face or neck) and birth defects affecting the brain, heart, eyes, and sternum (the middle part of the chest) or abdominal wall above the belly button. The diagnosis of PHACE is made by physical examination, Magnetic resonance (MRI and MRA) imaging of the head, cardiac exam using echocardiography, and an eye examination. If you are concerned that your child has PHACE syndrome, it is important to see a physician who specializes in the care of children with hemangiomas who can help to coordinate these evaluations.

The cause of PHACE syndrome is not known. It affects girls nine times more often than boys. It does not appear to run in families. PHACE is an acronym referring to the most common associated birth defects as outlined below.

P = Posterior fossa abnormalities. These structural abnormalities of the back of the brain are present at birth and do not develop after the baby is born. 



H = Hemangiomas. These tend to involve large segments of the head and/or neck.

A = Arterial (blood vessel) abnormalities of the neck and brain.

C = Cardiac (heart) and aortic arch (blood vessels coming from the heart) abnormalities

E = Eye abnormalities

Children with PHACE syndrome may be at risk for other neurological problems including migraine headaches, seizures, developmental delay, speech delay, and very rarely strokes. They may also have endocrine (hormone) abnormalities such as growth hormone deficiency, pituitary underdevelopment or low thyroid.

Over the last decade, there have been significant advances in the understanding and recognition of PHACE. The Hemangioma Investigator Group (HIG) has several ongoing research studies concerning this condition. These and other such research efforts may lead to improved diagnostic protocols, earlier detection of associated problems, and identification of the most effective and safest treatment.

face

Figure 1 in:
Haggstrom A N et al. Pediatrics 2006;117:698-703.
Used with permission from the American Academy of Pediatrics.



Courtesy of Dr. Anthony J. Mancini

 

Large hemangiomas on the Lower Part of the Face

Babies with large hemangiomas on the lower part of the face, also called the "beard distribution" are associated with hemangiomas in the airway that may affect breathing and require treatment.

This infant girl had an extensive hemangioma of the “beard area”, with associated hemangiomas in the airway.  She was treated with oral steroids, with marked improvement in the skin hemangiomas and resolution of the airway hemangiomas.