Congenital nonprogressive hemangioma
What is a congenital nonprogressive hemangioma?
A congenital hemangioma is a vascular lesion that is present and fully grown at birth. It may be diagnosed on a prenatal ultrasound. The terms noninvoluting congenital hemangioma (NICH) and rapidly involuting congenital hemangioma (RICH) were first used in 2000. Until that time, these lesions were considered to be a subtype of infantile hemangioma. We now understand that these vascular lesions look very different under the microscope and behave very differently than infantile hemangiomas. Congenital hemangiomas are most often found in the skin and are usually superficial (close to the surface of the skin). They can also be found in the liver. Ultrasound shows they are made up of thin-walled low- to high-flow blood vessels. Unlike infantile hemangiomas they do not grow after birth, and some may even shrink. The prevalence is equal in males and females.Congenital hemangiomas are much less common than infantile hemangiomas.
What does a congenital hemangioma look like?
Congenital hemangiomas are usually round or oval. They are pink to blue in color with pallor (pale colored skin) around them.The involved area is raised above the skin.They feel warmer to the touch than the rest of the body. Congenital hemangiomas most often appear on the head, neck or limbs of the body.
What are the types of congenital hemangiomas?
Congenital hemangiomas can be broken down into two categories.
- Noninvoluting congenital hemangioma.
- Rapidly involuting congenital hemangioma.
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Noninvoluting congential hemangioma |
Noninvoluting congenital hemangiomas are present at birth and grow proportionately as the child grows. They do not go through a regression phase. They may be surgically removed. If the NICH is large, it may need embolization prior to surgery to help control the bleeding. Embolization is a procedure done by a radiologist to help decrease the blood flow to a vascular lesion. It is done before surgery to lessen blood loss during surgery.
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Rapidly Involuting Congenital Hemangioma |
Rapidly involuting congenital hemangiomas are also present at birth. However, they go through a rapid regression phase and may be completely gone by the time the child is 12 to 18 months old. Surgery may be necessary even after regression of the tumor because of loose skin or scarring.
What causes a congenital hemangioma?
The cause of a congenital hemangioma is not known. Children's Hospital of Wisconsin is doing research to investigate causes of vascular malformations and tumors.
Treatment Options:
Treatment options vary depending on the size and location of the lesion. Observation is appropriate for those lesions that are showing regression. Currently there are no oral medications to treat congenital hemangiomas. Oral steroids, although effective on infantile hemangioma, do not work for congenital hemangiomas. Surgical resection is the best option if there are complications such as skin breakdown and bleeding. A cardiology consult may be necessary if the lesion is very large and has significant blood flow through it.
Complications:
Complications are skin breakdown, bleeding or possibly cardiac problems because of the large amount of blood flow to the lesion.
Differences between congenital hemangioma and infantile hemangioma:
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Congenital hemangioma |
Infantile hemangioma |
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Present at birth. |
Visible between 2 weeks and 4 months of age. |
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Growth is complete at birth or may grow proportionately as the child grows. |
Grow rapidly for approximately 6 to 12 months (average is around 8 months). |
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Equal prevalence in males and females. |
Female prevalence 5:1. |
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Less common but not rare. |
Common (4-5 percent incidence in newborns). |
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Rapid or no involution (shrinking). |
Slow involution that takes months or years. |
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