The lymphatic system helps to eliminate toxic or waste materials from the body. Lymphangomia is caused by a disorder with the lymphatic system.
Lymphangiomas are the result of a congenital condition and are usually apparent at birth, or at least by the time a person is 2 years old.
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What is a lymphangioma?
A lymphangioma can affect any part of the body but usually occurs on the head, neck, or mouth. The swelling is made up of one or many fluid-filled sacs that are caused by a problem with the lymphatic system.
The lymphatic system is part of the immune system and is made up of a network of tubes known as lymph vessels. These vessels transport a fluid called lymph around the body and into the bloodstream.
Lymph nodes positioned around the lymphatic system help rid the body of infection and inflammation.
Causes and risk factors
Some complications of lymphangioma include difficulty or pain when swallowing, and breathing problems.
Lymphangiomas are caused by abnormal development of the lymphatic system, but exactly why this happens is unknown.
A lymphangioma is described as a "somatic mutation," meaning that it affects the genes but is not an inherited condition.
Lymphangiomas can also occur as part of another condition, including:
Lymphatic malformations can occur in both males and females of any race. They are a rare condition affecting around 1 in 4,000 newborns.
In general, lymphangiomas do not cause any medical problems. However, because of their prominence on the face and neck, they can affect a person's appearance.
Also, more serious complications can occur, including:
- breathing problems when swellings in the neck press on the airway
- difficulty swallowing or speaking
- inflammation or cellulitis
- double vision if the eye socket is affected
- wheezing and chest pain if the chest is affected
Lymphangiomas usually occur in one localized area. Occasionally, they can be widespread throughout the body.
While the swelling will often be present at birth, it may be too small to see at first. In these cases, the lymphatic malformation grows as the infant grows.
The appearance of lymphangiomas can vary from small patches to large swellings, depending on how much fluid they contain.
There are three types of lymphatic malformations:
- Macrocystic: A large, fluid-filled pocket or pockets under the skin. The skin appears red or bluish. Macrocystic lymphatic malformations are more than 2 centimeters (cm) in diameter and usually occur on the neck. They can also affect the chest, armpit, or groin.
- Microcystic: A group of small, fluid-filled sacs that can occur anywhere on the body. The skin is red or bluish, and the mass grows in proportion with the child.
- Mixed: A combination of macrocystic and microcystic lymphatic malformations.
Once a baby is born, a lymphangioma can be identified during a physical examination. It will usually appear as a soft, ill-defined fluid-filled mass that moves around under the skin when pressure is applied.
The use of ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) scans can also help identify the mass.
Surgery is a possible treatment option. If the lymphatic mass is close to nerves or muscle, it can make the surgery more complicated.
Treatment for lymphangiomas varies case by case, and it often involves a team of specialists working together to decide the best course of action.
The location, type, and symptoms of the mass will all contribute towards deciding what treatment to use. If the lymphangioma is not causing specific problems, either medically or regarding appearance, then often no treatment is needed.
When treatment is needed, the most common types are:
- Surgery: Surgical removal can be a difficult procedure if the lymphatic malformation has traveled into the nerves and muscles.
- Sclerotherapy: A solution is injected into the swelling to cause it to shrink or collapse.
- Radiofrequency ablation: A high frequency current delivered via a needle destroys abnormal tissue.
- Dermabrasion: A skin resurfacing technique can be used to treat facial scarring.
- Percutaneous drainage: An incision is made in the lymphatic malformation, and the fluid is drained.
- Drug treatment: Commonly associated with treating cancer, the drug sirolimus has been shown to shrink lymphatic malformations. Clinical trials are still determining its effectiveness, however.
Unfortunately, in many cases of lymphangiomas, they can recur after removal.
Further treatment may also be necessary if the swelling has affected a person's breathing, eating, or speaking.
Outlook and complications
In general, the long-term outlook for people with lymphangiomas is good.
However, if left untreated, they can have a significant impact on a person's quality of life. This impact includes complications such as disfigurement, which can be particularly distressing in cases of lymphatic malformations on the face.
Body parts or organs located near the lymphangioma can also be affected.
Simple masses that are completely removed generally do not recur. However, more complex masses that are completely removed recur in 10 to 27 percent of cases.
Between 50 and 100 percent of people who have a complex mass only partly removed will experience recurrence.