PHACE Syndrome

PHACE Syndrome

Most infantile hemangiomas are not serious.

However, children with certain types of facial hemangiomas may have other related issues.

PHACE syndrome may be suspected in infants with large hemangiomas on the face, head, and / or neck.

What is PHACE syndrome?

PHACE syndrome is an association between large infantile hemangiomas of the face, head and / or neck and developmental defects of the eyes, heart, major arteries and brain. The cause of PHACE syndrome is unknown.

P = Posterior fossa (This refers to possible abnormal structures in the brain, particularly the cerebellum.)
H = Hemangioma
A = Arterial (This refers to possible abnormal arteries in the brain, neck or chest.)
C = Cardiac (This refers to possible heart problems or abnormalities of the large blood vessels connected to the heart.)
E = Eyes (This refers to possible eye or vision issues.)

Infants with PHACE syndrome may have one or more of these problems, in addition to the hemangioma. Rarely, children may have an abnormal sternum (breastbone) or hypothyroidism (low thyroid hormone).

Hemangiomas may be small or not visible at birth. They grow quickly and can be seen during the first days to weeks of life. In children with PHACE syndrome, hemangiomas usually cover a large area of the face, head or neck. They can appear as one lesion or a patch of multiple lesions. Other problems may be visible at birth such as a breastbone deformity.

Signs and symptoms vary. Severity of symptoms and complications also varies.

If a hemangioma looks suspicious for PHACE syndrome, additional testing is needed. These tests may include:

Other tests may be needed.

As it grows, the hemangioma can lead to skin breakdown (ulcerations), change facial features and / or cause issues with breathing, eyesight, or hearing.

Other complications depend on what structures other than the skin are involved. For example, developmental delay, seizures, headaches, and / or abnormal muscle tone could occur if the brain is involved.

Usually the hemangioma(s) in PHACE syndrome require medication by mouth or on the skin. These medications are used to stop hemangioma growth. They also treat and / or prevent complications. Surgical treatments could also be needed.
PHACE syndrome needs evaluation and treatment by a team of experts. Routine follow up is important.

Last Updated 02/2020

Reviewed by Kiersten Ricci, MD

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