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Hemangioma is the most common type of vascular anomaly (birthmark). It is a benign (noncancerous) tumor of the cells, called endothelial cells, that normally line the blood vessels. In hemangiomas, the endothelial cells multiply at an abnormally rapid rate.
Infantile hemangiomas have a fairly predictable pattern of growth. Most appear during the first weeks of life and grow rapidly (called the proliferative phase) for 6 to 12 months. Then they begin a much slower process of shrinking, or regressing (called the involuting phase), which may take from one to about seven years. Finally, the tumor enters its final, shrunken state (called the involuted phase), after which it will never regrow. Tumor regression is complete in 50% of children by age 5 and in 70% of children by age 7. By the time a child reaches 10 to 12 years of age, involution of the tumor is always complete. Some residual fatty tissue or thin skin may remain after involution.
Rarely hemangioma begins in the womb and presents fully grown at birth, called a congenital hemangioma. There are two forms of congenital hemangioma:
1) rapidly invoulting congenital hemangioma (RICH)
2) non-involuting congenital hemangioma (NICH)
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Vascular malformations are benign (non-cancerous) lesions that are present at birth, but which may not be visible for weeks or months after birth. Unlike hemangiomas, vascular malformations do not have a growth cycle and then regress - they continue to grow slowly throughout life. There are several types of vascular malformations:
- Capillary (port wine stains) - always present at birth as pink or purple skin patches
- Venous - often confused with a hemangioma, these malformations are soft to the touch and the color disappears when compressed. They are most commonly found on the jaw, cheek, tongue and lips
- Lymphatic - formed when excess fluid accumulates within the lymphatic vessels
- Arteriovenous - abnormal connections between arteries and veins, resulting in a high flow, pulsating collections of blood vessels
Mixed - a combination of any of the other four types
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Most infantile hemangiomas are not usually present at birth or are very faint red marks. Shortly after birth, however, they grow rapidly - often faster than the child's growth. Over time, they become smaller (involute) and lighter in color. The process of involution may take several years.
Vascular malformations are present at birth and enlarge proportionately with the growth of the child. They do not involute spontaneously and may become more apparent as the child grows.
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Infantile hemangioma is not inherited. It is believed to be caused by a mutation in a primitive stem cell responsible for developing blood vessels.
Although most vascular malformations are sporadic (occuring by chance). Some are inherited in a family as an autosomal dominant trait. Autosomal dominant means that one gene is necessary to express the condition, and the gene is passed from parent to child with a 50/50 risk for each pregnancy. Males and females are equally affected and there is great variability in expression of the gene. In other words, a parent may unknowingly have had a hemangioma because it faded, but the child is more severely affected. The family may not come to the attention of a geneticist until the birth of the child with a more severe condition. Other relatives with mild expression of the gene are often discovered at that time, confirming autosomal dominant inheritance.
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If a hemangioma or vascular malformation is very large or affects the breathing system (airway or lungs) or another large organ system, it could be life-threatening. If a hemangioma has uncontrollable bleeding, this could also be life-threatening. Large and/or life-threatening lesions should be evaluated by an interdisciplinary team of specialists that includes plastic surgeons, dermatologists, ophthalmologists, radiologists, and other specialists, depending on what organs are involved.
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Treatment for hemangiomas depends upon their size, location, and severity. Treatment is usually not recommended for a small, hemangioma, since it will become smaller (involute) on its own, leaving almost normal skin. However, hemangiomas that cause bleeding problems, feeding or breathing difficulties, growth disturbances, or impairment of vision may require medical or surgical intervention.
Treatment may include the following:
- corticosteroid or other drugs
- surgical removal
- embolization of the blood vessels (injection of material into the blood vessels to block the blood inflow)
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Treatment for vascular malformations depends upon the type. Each type of malformation is treated differently. Laser therapy is usually effective for capillary malformation (port wine stains), which tend to be flat, violet or red patches on the face. Arterial malformations are often treated by embolization (blood flow into malformation is blocked by injecting material near the lesion). Venous malformations are usually treated by direct injection of a sclerosing (clotting) medication which causes clotting of the channels. Most often, a combination of these various treatments is used for effective management of the vascular anomaly.
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Has a vascular anomaly affected you or your child?
Tell us about it . How did you deal with the diagnosis? What types of therapies did you undergo? What support groups did you lean on? Sharing your story will be a great source of comfort to families who are going through similar situations. Thank you!
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 A mother's story
Shortly after our daughter, Alexandra, was born, we noticed a slightly pink area of skin under her eye. We were not too alarmed, since we were so happy to have a healthy baby girl join our family. The excitement of this new arrival far outweighed any concern of the small mark.
A few short weeks after returning home from the hospital, the excitement was quickly replaced by concern as the hemangioma grew rapidly. Because of the close proximity to her eye, our pediatrician suggested that we see a specialist in the Vascular Anomalies Center of Children's Hospital Boston. Our initial concerns were superficial and looking back they are so trivial it is hard to believe they crossed our mind. Are other children going to make fun of her? How will she deal with the questions and the stares? Will I do a photo birth announcement or holiday card? It was not too long before the hemangioma was covering much of her cheek and the underlying swelling had distorted her nose, mouth and eye. In addition, the hemangioma had ulcerated causing profuse bleeding and discomfort for Alexandra.
It obviously didn't take us long to think about the more serious concerns. We were referred to an opthamologist at Children's Hospital and she has helped ease our fears and treat any vision related problems. The other concerns were addressed with the Vascular Anomalies specialist. The option at this point was to put her on steroids. We had held off on this for as long as possible since putting an infant on steroids is not highly recommended. In addition to the steroids she had to wear arm restraints when she slept so that she could not touch her face. Putting her to bed with these always brought a tear to my eye, especially since she would struggle and cry as I put them on but we knew it was for the best.
There were also the social issues that made leaving the house stressful. It was hard not to react to situations that, looking back, were likely harmless. A teenager at a park pointing and saying "What is on her face?" or a friend trying to ease their tension by making a sarcastic remark. It is hard to know if we were oversensitive but I found myself becoming overly protective and filled with anxiety when out in public. I just wanted to have the normal questions like -- is she a good sleeper? How old is she?
Seeing the specialist was always a relief to us, both with our initial concerns and the more serious concerns that quickly followed. He always made time in his schedule for us and returned our calls personally. His calming demeanor and thorough explanations of what to expect in the weeks and years to come has answered all of our questions. We always felt reassured and optimistic after our appointments. Our experience at Children's has been such an incredible one and has helped both our daughter and us deal with the physical and emotional issues that the hemangioma has caused.
Although the hemangioma is still quite large, we do not notice it as we once did. There are still occasional situations that are uncomfortable ? a pharmacist pointing and asking "What is wrong with her?" or the questions from kids "Is that ketchup on her face?". But I react differently now. I can matter-of-factly say that "It is just a birthmark. It will go away" and I know that it is true. |
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Arianna Faro struggled for years with the challenges of living with Klippel-Trenaunay (KT) syndrome, but has come to accept the role it plays in her life.
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Vascular Malformations, Tumors and Hemangiomas
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My daughter, Jenny, had a hemangioma in the diaper area about 14 years ago. I remember that being a problematic time -- there wasn't a lot of information out there about hemangioma back then. I remember having to go to the library and use the old indexes to look up articles about it and to find doctors to contact. 
