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HE MANGIO MA

A hemangioma of infancy is a benign self-involuting tumor (swelling or growth) of endothelial

cells, the cells that line blood vessels. It usually appears during the first weeks of life andresolves by age 10. In infancy, it is the most common tumor. The word "hemangioma" comes

from the Greek haema- (), "blood"; angeio (), "vessel"; -oma (-), "tumor".

T ERMINOLOGY 

The terminology used to define, describe and categorize vascular anomalies, abnormal lumps

made up of blood vessels, has changed. The term hemangioma was originally used to describeany vascular tumor-like structure, whether it was present at or around birth or appeared later inlife. Mulliken et al. categorized these conditions into two families: a family of self-involuting

tumors, growing lesions that eventually disappear, and another family of malformations,enlarged or abnormal vessels present at birth and essentially permanent. The importance of this

distinction is that it makes it possible for early-in-life differentiation between lesions that willresolve versus those that are permanent. Examples of permanent malformations include port-

wine stains (capillary vascular malformation) and masses of abnormal swollen veins (venousmalformations) 

PRESENT AT ION

Hemangiomas are connected to the circulatory system and filled with blood. The appearancedepends on location. If they are on the surface of the skin, they are reminiscent of a ripe

strawberry (hence, they are sometimes referred to as "strawberry hemangiomas"); however, if they are just under the skin they present as a bluish swelling. Sometimes they grow in internal

organs such as the liver or larynx. In most cases, hemangiomas will disappear over time. Someare formed during gestation; the most common are not congenital, but appear during the first few

weeks of life. They are often initially misdiagnosed as a scratch or bruise; but the correctdiagnosis becomes obvious with further growth. Typically, at the earliest phase in a superficial

lesion, one will see a bluish red area with obvious blood vessels and surrounding pallor.

Sometimes they present as a flat red or pink area. Hemangiomas are the most common childhoodtumor, occurring in approximately ten percent of Caucasians, and are less prevalent in other ethnicities. Females are three to five times as likely to have hemangiomas as males.

Hemangiomas are also more common in twin pregnancies. Approximately 80% are located onthe face and neck, with the next most prevalent location being the liver.

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CAUSES

The cause of hemangioma is currently unknown; however, several studies have suggested the

importance of estrogen signaling in hemangioma proliferation. In 2007, a paper from theStanford Children's Surgical Laboratory revealed that localized soft tissue hypoxia coupled withincreased circulating estrogen after birth may be the stimulus.There is also a hypothesis

 presented by researchers at Harvard and the University of Arkansas that maternal placentaembolizes to the fetal dermis during gestation resulting in hemangiomagenesis,. However,

researchers at Duke University conducted genetic analyses of small nucleotide polymorphisms inhemangioma tissue compared to the mother's DNA that contradicted this hypothesis. More

research is required in order to fully understand the explosive nature of hemangioma growth,

which will hopefully yield targeted therapeutics to treat its most complicated presentations.

COMPLICAT IONS

The vast majority of hemangiomas are not associated with complications. Hemangiomas may break down on the surface, called ulceration. If the ulceration is deep, significant bleeding may

occur in rare occasions. Ulceration on the diaper area can be painful and problematic. If ahemangioma develops in the larynx, breathing can be compromised. A hemangioma can grow

and block one of the eyes, causing an occlusion amblyopia. Very rarely, extremely largehemangiomas can cause high-output heart failure due to the amount of blood that must be

 pumped to excess blood vessels. Lesions adjacent to bone can also cause erosion of the bone.

The most frequent complaints about hemangiomas, however, stem from psychosocialcomplications: the condition can affect a person's appearance and can provoke attention and

malicious reactions from others. Particular problems occur if the lip or nose is involved, asdistortion can be difficult to treat surgically. The potential for psychological injury develops

from school age onward. It is therefore important to consider treatment prior to school if adequate spontaneous improvement has not occurred.

Children with large segmental hemangiomas of the head and neck can be associated with adisorder called PHACES Syndrome

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T REAT  MENT  

Most hemangiomas disappear without treatment, leaving minimal or no visible marks. Large

hemangiomas can leave visible skin changes secondary to severe stretching of the skin or damage to surface texture. When hemangiomas interfere with vision, breathing, or threaten

significant cosmetic injury, they are usually treated.

Until recently, the mainstay of treatment was oral corticosteroid therapy, but there are nowalternative treatments. A published letter reported that the beta-blocker propranolol reduced

severe hemangiomas in infants. The topically applied beta blocker solution / gel Timolol, is also being trialled for small facial hemangiomas that do not justify systemic treatment. Other 

treatments that have been used include interferoN or vincristine. They may be considered if first-line therapy fails.

Surgical removal is sometimes indicated, particularly if there has been delay in commencing

treatment and structural changes have become irreversible. Surgery may also be necessary tocorrect distortion of facial features, again in the case of inadequate or failed early medical

intervention. Blockage of the airway will often require a tracheostomy to be performed, whichinvolves the insertion of an external airway through the front of the neck into the trachea below

the level of the obstruction.

Smaller raised lesions are sometimes treated with injection of corticosteroid directly into the

lesion. A pulsed dye laser can be useful for very early, flat, superficial lesions, if they appear incosmetically significant areas or for those lesions that leave residual surface blood vessels in the

case of incomplete resolution. Sometimes a pulsed dye laser can be used to accelerate healing.Unfortunately, raised lesions or lesions under the skin do not respond to laser treatment.

Ulceration will usually heal with topical medication and special dressings under medicalsupervision.

PROGNOSIS

Hemangiomas go through three stages of development and decay:

1.  In the proliferation stage, a hemangioma grows very quickly. This stage can last up totwelve months.

2.  In the rest stage, there is very little change in a hemangioma's appearance. This usuallylasts until the infant is one to two years old.

3.  In the involution phase, a hemangioma finally begins to diminish in size. 50% of lesionswill have disappeared by 5 years of age, and the vast majority will have gone by 10 years

of age.