Large Hemangiomas on the Face
Hemangiomas on the face are considered large when they are more than 5 cm (about 2 inches) in size. When hemangiomas are large or follow certain patterns on the face, there is a chance of other associated problems such as those seen in PHACE syndrome (see below). Large hemangiomas of the lower half of the face (in a “beard” location) may be associated with other hemangiomas in the airway that can sometimes affect breathing. It is recommended that your child see his/her doctor as soon as possible for large hemangiomas on the face, or for hemangiomas located around the eye, on the nose, or lips, even if smaller in size, since treatment may be needed.
PHACE syndrome refers to the uncommon association of large hemangiomas of the face and birth abnormalities of the brain or brain blood vessels, heart or heart blood vessels or, rarely, eyes. Sometimes there can also be changes of the skin over the middle part of the chest (sternum) or stomach above the belly button. These abnormalities are there at birth. The diagnosis of PHACE is made by physical examination, imaging (special pictures) or the head, neck and heart, and an eye examination. If you are concerned that your child may have PHACE syndrome, it is important that your child is seen by a physician familiar with the diagnosis, evaluation and treatment of hemangiomas and PHACE syndrome.
The cause of PHACE syndrome is not known. It affects girls much more often than boys. It does not appear to run in families. PHACE is an acronym that stands for the following:
Other than the hemangioma, the most common problems seen in PHACE are changes of the blood vessels of the brain, neck or heart. It is very rare for a child to have every possible problem. While many children with PHACE are otherwise normal, some may be at risk for neurological problems such as migraine-like headaches, seizures and developmental delay including speech delay. PHACE has been reported in association with stroke but this is very rare.
PHACE was only recently described in 1996. Since then, there have been significant advances in the understanding and recognition of PHACE. The Hemangioma Investigator Group (HIG) has been very involved in PHACE research and currently has several active studies. It is hopeful that these research efforts will lead to the cause of and prevention of PHACE and improved treatments for those children affected.
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.
Related HIG Research: