Lymphangiomas, also known as lymphatic malformations, are congenital (since birth) malformations of the skin and tissues under the skin. These are often recognized at birth or within the first two years of life. They make up about five percent of all benign tumors in infants and children.
While lymphangiomas most often appear on the head and neck, they can occur anywhere in the skin and mucous membranes. They have been found in the intestines and pancreas.
When visible at birth, lymphangiomas are usually soft and spongy. In older children, they develop more rapidly and can be soft or firm. These masses are not tender and can have a warty appearance.
There are five main types of lymphangiomas:
- Capillary Lymphangioma: This presents as nodules or gray-pink masses on the skin. The spaces in between the lesions are filled with fluid and lack blood cells. This type most often presents on the head, neck and connective tissues of the soft internal organs.
- Cystic Lymphangioma: This is the most common type of lymphangioma and presents as large cyst-like cavities filled with a straw-colored fluid. Cystic lymphangiomas frequently occur alongside congenital disorders like Turner Syndrome, Down Syndrome and Fetal Alcohol Syndrome. This type rarely presents in adults.
- Cavernous Lymphangioma: This type of lymphangioma can occur anywhere in the body, including the tongue. The skin appears red or bluish, rubbery, and inflamed. Clear fluid can be seen within the vessels in infants.
- Lymphangioma Circumscriptum: This presents as a cluster of small abscesses filled with lymph fluid, ranging in colors from pink, dark red, brown, or black. It generally appears around puberty and is most common on the shoulders, neck, armpits, limbs, and mouth.
- Hemangiolymphangioma: Presenting in both the lymphatic system and blood vessels, this type of lymphangioma can invade muscle, bone and underlying tissues. The result can be serious deformity.
Most prominently, lymphangioma can usually be identified by the distinctive appearance on the skin. But when internal lymphangiomas occur, symptoms may include:
- Fever and chills
- Blood-filled cysts behind the eye
Confirming the diagnosis of lymphangioma usually requires a biopsy of the fluid be taken from the cystic lesions. Prenatal ultrasound can also be used to evaluate lesions that are detected prior to birth.
In most cases, lymphangiomas do not present a serious health threat. Still, patients (or the parents of patients) may want to seek treatment for cosmetic reasons. And in severe cases, where minor bleeding or lymph fluid leaking has occurred, medical treatment may be deemed necessary. Those options include:
- Surgical Removal: Multiple surgeries may be required in order to get all the lesions, but when all lesions are removed completely, they generally do not recur.
- Intralesional Sclerotherapy: The least invasive and most effective form of treatment is considered to be intralesional sclerotherapy, a procedure performed by interventional neuroradiologists. During this non-invasive procedure, the physician injects various agents into the lymphatic vessel in order to shrink the lymphangioma.
- Dye Laser: Laser therapy is used to destroy the lymphangioma. The risk with this technique is the development of port-wine stains and other vascular (blood vessel) lesions.