| Name: | |
| Caption: | A small hemangioma of infancy |
| Diseasesdb: | 30033 |
| Icd10: | |
| Icd9: | |
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| Medlineplus: | 001459 |
| Emedicinesubj: | derm |
| Emedicinetopic: | 201 |
| Meshid: | D006391 |
Before considering the hemangioma it is important to understand that there have been recent changes in the terminology used to define vascular anomalies (abnormal lumps made up of blood vessels). The term hemangioma was originally used to describe any vascular tumour both present around birth or appearing later in life. Mulliken et al separated these conditions into a family of self involuting tumours (growing lesions that eventually disappear) from the family of malformations (enlarged or abormal vessels present at birth and essentially permanent. The importance of this separation is that allows us to differentiate early in life between lesions that will resolve versus those that are permanent. Examples of permanent malformations include Port-wine stains (capillary vascular malformation) and masses of abnormal swollen veins (venous malformations).Hemangiomas and Vascular Malformations in Infants and Children: A Classification Based on Endothelial Characteristics. Unfortunately many textbooks and dictionaries are not up to date creating great confusion.
A hemangioma is a benign self involuting tumour of endothelial cells (the cells that line blood vessels) Haemangiomas of infancy They are connected to the circulatory system and filled with blood. The appearance depends location. If they are on the surface of the skin the look like a ripe strawberry, if they are just under the skin the 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. They are formed either during gestation or most commonly they are not present at birth but appear during the first few weeks of life. They are often misdiagnosed, initially, as a scratch or bruise but the diagnosis 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 childhood tumor occurring in approximately ten percent of Caucasians, and are less prevalent in other races. Females are three to five times more likely to have hemangiomas than males. They are also more common in twin pregnancies. Approximately eighty percent are located on the face and neck, with the next most prevalent location being the liver. Although hemangiomas are benign, some serious complications can occur.
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 the Stanford Children's Surgical Laboratory revealed that localized soft tissue hypoxia coupled with increased circulating estrogen after birth may be the stimulus.[1] There is also a hypothesis presented by researchers at Harvard and the University of Arkansas that maternal placenta embolizes to the fetal dermis during gestation resulting in hemangiomagenesis,[2][3] yet Duke researchers conducted genetic analyses of small nucleotide polymorphisms in hemangioma tissue compared to the mother's DNA that contradicted this notion.[4] 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.
The vast majority of hemangiomas are not associated with complications. Hemangiomas may break down on the surface to form ulcers. If the ulceration is deep, significant bleeding may rarely occur. Ulceration on the diaper area can be painful and problematic.
If a hemangioma 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 large hemangiomas 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 psychosocial complications: 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, as distortion can be difficult to treat surgically.
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. The mainstay of treatment is oral corticosteroid therapy. Other drugs such as interferon or vincristine are sometimes considered if the corticosteroids do not work. If this fails, surgical removal often becomes necessary. Blockage of the airway will often require a tracheostomy to be performed (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. Pulsed dye laser can be useful for very early flat superficial lesions if they appear in cosmetically significant areas or for those lesions that leave residual surface blood vessels in the case of incomplete resolution. Unfortunately raised lesions or lesions under the skin do not respond to laser.
Ulceration will usually heal with topical medication and special dressings under medical supervision. Sometimes pulsed dye laser can be used to accelerate healing.
Hemangiomas go through three stages of development and decay: