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Hemangioma: the most common benign tumor of infancy and Children

 

Both of these children have been diagnosed with a hemangioma.

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   According to the medical literature, of the nearly 4 million children born in the United States each year, 10 percent or 400,000 will be born with a vascular anomaly.  Ninety percent of these birthmarks will disappear by age 1.  The remaining 40,000 children will be diagnosed with a significant vascular anomaly and will require medical intervention by a physician experienced in the diagnosis and treatment of a hemangioma.(1)

Most hemangiomas will develop over the first few days-weeks of life. It is reported that 70% of all hemangiomas will become apparent within the first 4 weeks of life. The remaining 30% are visible at birth. These two types of hemangioma are classified into two groups; Congenital Hemangioma and Infantile Hemangioma.  

A Congenital Hemangioma (CH) is fully formed at birth. These lesions may actually be visible by ultrasound during pregnancy.

An Infantile Hemangioma (IH) often first appears as a small light red or blue flat patch. They are rarely fully formed at birth. Infantile hemangiomas develop over the first weeks of life. Hemangioma is more common in females with reports ranging from 2.5-5 times predominance in females. They occur more often in Caucasians and are less likely in Asian infants. (for the purpose of this article hemangioma refers to IH)

Infantile Hemangiomas occur more often in premature infants and have a higher incidence in multiple gestation births. (twins) Premature and infants weighing less then 2.2 pounds, have an incidence of hemangioma of nearly 3 times more then full term infants. There is an statistically significant finding that hemagnioma occurs more often in mothers with advance maternal age, had complicatiosn of placenta previa and pre-eclampsia and multiple gestations.  This data provides interesting trends and leads to questions that will direct research in the future however; there is no known cause for a hemangioma .

When a hemangioma first appears it is often small and can be either bluish or reddish. It may be a small raised spot or a flat patch.  This first appearance is called a "precursory lesion".   Most hemangiomas develop during the first few weeks; they are rarely full grown at birth .  Those hemangiomas that develop in the first weeks of life are called Infantile Hemangioma .   Hemangiomas that are fully developed at birth are called Congenital Hemangioma.  Hemangiomas are classified as either superficial, deep or combined superficial & deep.  Traditionally hemangiomas were called strawberry birthmarks or cavernous hemangioma today physicians experienced in the diagnosis and treatment of vascular lesions prefer the superficial or deep classifications.  "Superficial" hemangiomas are flat and red, those that are deep beneath the skin and appear blue in color are called "Deep" hemangiomas. Hemangiomas often have both superficial and deep components; they may be called "Combined Superficial & Deep" .  Recently there has been a newly described classification of hemangioma listed, "Segmental" , this type of hemangioma is characterized by a large flat or slightly raised Hemangioma often seen on the face. Segmental Hemangiomas typically grow rapidly and are more difficult to treat.    The classification of a hemangioma is based on the published work of Dr. John Mulliken of Boston Children's Hospital in the 1980's.  The classification system continues to evolve as the understanding of hemangioma deepens. 

Infantile Hemangiomas will typically grow for 9-12 months; some may grow up to about 18 months  This is called the "growth phase" of a hemangioma development. After completing the growth phase a hemangioma will plateau and then enter into a spontaneous regression period. This is call "involution . "   Some hemangiomas involute slowly and others involute rapidly.   Those that involute rapidly often will leave little to no evidence that a hemangioma was ever present.  Hemangiomas that involute slowly, taking >5 years are more likely to leave residual scaring in the forms of fibro fatty tissue, small blood vessels and other scarring.  While all Hemangiomas eventually involute the result is not always cosmetically acceptable. Evaluation by an experienced physician can help to determine if a hemangioma should be treated or to be allowed resolve naturally. 

Most hemangiomas do not result in significant complications for the patient. However; those that are considered complicated hemangiomas can have endangering complications including, pain, ulceration, infection, structural abnormalities, and interference with breathing, vision and hearing. According to the American Academy of Dermatology, hemangiomas of infancy should be evaluated for these complications and treated accordingly. The treatment for complicated hemangiomas include; observation, medical managment, laser, and surgical excision. The medications used to treat hemagnioma include steroid injections, oral steroids, and chemotherapy agents like interferon and vincritine. (NOTE- Interferon has been associated with a risk of neruotoxicity, spastic diplaigia in infants, careful considerations of the risks vs benefits must be made before using interferon in the treatment of hemangioma.)

The psychological implications of facial disfigurement should not be ignored in the management of facial hemangioma. In a culture where “fitting in” seems to be more important then ever, Parents should consider that children become socially interactive at age 2-3 years of age. Children are teased by their peers for wearing the “wrong” colored socks, imagine the implications for the child with a facial difference resulting from a hemangioma. Many physicians will advocate treatment with the goal of restoring a more normal appearance to the facial difference in time for school. Since many children are in preschool at very early ages families may want to discuss earlier intervention to account for earlier school settings. Some families seek resolution surgically at a very young age due to the individual family dynamics. Early intervention by an experienced physician may minimized the disfigurement and decrease the risk of psycho/social concerns, these issues need to be discussed in the context of the overall long term benefits to the child.

Large faical hemangiomas described as difuse, segmental or regional may be associated with PHACES a rare but complicated disease where the patient may have varied complications including cardiac, eye, brain anomalies. 

In rare instances “hemangiomas” have been associated with life-threatening complications. Usually these lesions are very large, greater the 5cm X 5 cm of surface area. This type of lesion may interfere with eating, breathing, vision, hearing and speech. In fact these lesions are not actually hemangioma but are called Kaposi Form Hemangioma Endothelioma and Tufted Angioma. These two types of vascular anomaly are also associated with complications leading to clotting abnormalities, (thrombocytopenia or Kasa Bach Merritt Syndrome) and congestive heart failure. Although these lesions are not actually a hemangioma they are often first diagnosed as one. Children with more then 4 hemangioma of the skin are at risk for internal hemangioma of the liver, airway, brain or digestive track. Internal hemangioma or visceral lesions are often difficult to detect. There is not a full understanding of the correlation between hemangioma of the skin and internal or structural hemangioma. A child with an internal hemangioma may show signs of jaundice, blood in the stool, croupy cough or difficulty breathing (stridor). Ultrasound or MRI may be performed to rule out internal hemangioma in children with multiple skin lesions or other symptoms.

Tufted Angioma

& KHE

The treatment of a hemangioma can be complicated. It is the position of NOVA that all patients diagnosed with a hemangioma or vascular malformation be evaluated by a physician experienced in the management of vascular anomalies. NOVA can assist you in finding a Vascular Anomaly Treatment Center or private physician that accurately diagnose and treat vascular anomalies.



NOTES:
1.   According to the the most recent data complied by the Hemangioma Investigative Group and presented at the April 2005 workshop: Infantile Hemangioma Current Knowledge & Future Directtions, National Institute of Health, Bethesda, MD the actual incidence of infantile hemangioma ranges from 4-10% based on referral to a medical specialist.  It is thought that the variation in numbers is biased based on the reporting by referral. 

2.  Hemangioma and Vascular Malformations may be associated with other serious diseases and syndromes. It is important to have all vascular anomalies evaluated by a physician.

 

Common

Questions

about Hemangioma

References

Mulliken, Glowacki J (1982) Hemangioma and Vascular Malformations in infants and children.  A classification based on endothelial characteristic.  Plast Reconstr. Surg 69:412-422

Haggstrom, Drolet, Baselga, Chamlin, Garzon, Horii, Lucky, Mancini, Metry, Newell, Nopper, Frieden, Prospectiv Study of infantile hemagniomas:dermgraphic, prenatal and perinatal characteristics.  J Pediatric.  2007 Mar;150(3):2911-4

Frieden, Enjolras, Esterly, Vascular Birthmarks and Other Abnormalities of Blood Vessels and Lymphatics, Chapter 20pp 834-862,

 

NOVA PROVIDES THIS INFORMATION TO FAMILIES AS A RESOURCE. IT IS NOT INTENDED TO ENGAGE IN THE PRACTICE OF MEDICINE OR TO REPLACE THE PHYSICIAN. NOVA DOES NOT CLAIM TO HAVE MEDICAL KNOWLEDGE. NOVA DOES NOT ENDORSE ANY PARTICULAR PHYSICIAN, TREATING FACILITY OR TREATMENT PROTOCAL. IN ALL CASES NOVA AND ITS BOARD OF DIRECTORS RECOMMENDS THAT YOU SEEK THE OPINION OF A PHYSICIAN EXPERIENCED IN THE MANAGEMENT OF HEMANGIOMAS AND VASCULAR MALFORMATIONS. 


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Articles & Reviews

Congenital & Infantile Hemangioma

Diaper Area Hemangioma

Kasabach Merritt Phenomenon

Caring for the Chronically Ill Child

Ulceration: pdf file

A Parents Guide- .pdf file

 

Treatment

Hemangioma Treatment

 

Vascular Anomaly Treatment Centers

 

Research

Hemangioma Research

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 
 

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lUpdated July 2007

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