TYPES OF HEMANGIOMAS
Infantile Hemangiomas are benign (not cancer) collections of extra blood vessels in the skin and are one of the most common skin growths of the first year of life. They appear most frequently during the first one to four weeks after birth and occur in about 5% of all children. Hemangiomas may look different depending upon how deep they are in the skin. More surface (superficial) hemangiomas tend to be bright red and raised with an uneven surface. Deeper hemangiomas tend to be smooth on the surface, and may be skin-colored or sometimes blue in coloration. Often, both superficial and deep parts will be present (sometimes referred to as a mixed hemangioma). Not infrequently, superficial types of hemangiomas will begin as flat pink areas that rapidly change into raised, bright red growths.
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 they can be associated with problems with the brain or heart 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.
Image Courtesy of Dr. Anthony J. Mancini
Segmental Hemangioma Map Creative Commons HIG © 2023 by Hemangioma Investigator Group is licensed under Attribution 4.0 International. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/
PHACE(S) SYNDROME
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:
P = Posterior fossa abnormalities: abnormal development of the back of the brain.
H = Hemangiomas: large and often patterned on the face and/or scalp.
A = Arterial: abnormal blood vessels of the brain and/or neck.
C = Cardiac: abnormalities of the heart and/or aortic arch (blood vessels coming from the heart)
E = Eye abnormalities.
(S) = Sternal defects and/or supraumbilical raphe: skin changes over the chest or stomach.
Other than the hemangioma, the most common problems seen in PHACE are changes of the blood vessels of the brain, neck or heart. Most children with PHACE only have a couple of the problems, it is very rare for an individual 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.
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.
Image Courtesy of Dr. Anthony Mancini
Image Courtesy of Dr. Anthony Mancini
HEMANGIOMAS
OVER THE LOWER SPINE
Infantile hemangiomas in this area require imaging evaluation for spinal cord abnormalities. The hemangioma may also require treatment if there is ulceration (skin breakdown), extends into the buttock crease (just below this hemangioma)/ around the anus and hemangioma has excessive growth.
Infantile hemangiomas over the skin on the center lower back/buttock area (lumbosacral area) carry a risk for being associated with a spinal cord problem. A “tethered spinal cord” is one such abnormality in which the spinal cord is abnormally stretched and anchored to the spinal canal with reduced mobility. Often, symptoms may be delayed until adulthood, but it can start during childhood. Common symptoms are lower back pain, pain and weakness of the legs, walking problems, and loss of control of the bladder and bowel. If your child has a hemangioma in this location, see your doctor to discuss whether imaging the spine for this possibility may be appropriate.
RELATED HIG RESEARCH
The HIG prospectively evaluated a group of patients with infantile hemangioma (IH) in the midline lumbosacral region for spinal anomalies to determine the risk of spinal anomalies and to make evidence-based recommendations for screening. 35% of the infants with isolated lumbosacral IHs had spinal anomalies. Ulceration of the hemangioma was associated with a higher risk of having spinal anomalies. The presence of additional cutaneous anomalies also was associated with a higher likelihood of finding spinal anomalies.
Conclusions: Infants and children with midline lumbosacral IHs are at increased risk for spinal anomalies. Screening magnetic resonance imaging is recommended for children with these lesions.
Drolet BA, Chamlin SL, Garzon MC, Adams D, Baselga E, Haggstrom AN, Holland KE, Horii KA, Juern A, Lucky AW, Mancini AJ, McCuaig C, Metry DW, Morel KD, Newell BD, Nopper AJ, Powell J, Frieden IJ, A Prospective Study of Spinal Anomalies in Children with Infantile Hemangiomas of the Lumbosacral Skin. J Pediatr 2010 Nov;157(5):789-94.
MULTIPLE HEMANGIOMAS
Some infants have multiple (also referred to as multifocal) hemangiomas. The number of hemangiomas can be quite variable, ranging from a few to hundreds. In patients with numerous hemangiomas, the hemangiomas tend to be smaller in size. It is most common for hemangiomas to appear on the skin, but they can develop in internal organs as well.
The risk of having internal involvement increases with the total number of skin hemangiomas. This risk is uncommon in infants with 4 or fewer hemangiomas, but increases if there are 5 or more hemangiomas. The liver is the most common internal site affected; other internal sites are far less common. If 5 or more skin hemangiomas are present, a liver ultrasound is recommended. If liver hemangiomas are present, thyroid hormone levels should be checked, because infants with liver hemangiomas can also have low thyroid hormone levels.
Other evaluations for internal involvement include a careful history and physical examination. It may include other imaging studies or blood work depending on the individual situation. Like hemangiomas on the skin, internal hemangiomas also grow during infancy and regress (go away) during childhood.
Most internal hemangiomas do not require treatment, but if symptoms are present, the lesions are excessively large, or rapid growth is seen, treatment may be needed to slow growth and prevent complications.
RELATED HIG RESEARCH
Horii KA, Drolet BA, Frieden IJ, Baselga E, Chamlin SL, Haggstrom AN, Holland KE, Mancini AJ, McCuaig CC, Metry DW, Morel KD, Newell BD, Nopper AJ, Powell J, Garzon MC; Hemangioma Investigator Group. Prospective study of the frequency of hepatic hemangiomas in infants with multiple cutaneous infantile hemangiomas. Pediatr Dermatol. 2011 May-Jun;28(3):245-53.