Lymphangiomas, also called lymphatic malformations, are noncancerous, fluid-filled cysts that occur in lymphatic vessels. These vessels contain a substance called lymph, and together they make up the lymphatic system. Lymph helps to properly regulate fluid in body tissue. Lymph also works with the immune system to help fight infection.
It’s estimated that lymphangioma occurs in about 1 in 4,000 births. They can affect nearly any part of the body, but are most common on the head or neck. About 75 percent of lymphangiomas are located in this region. Half of all lymphangiomas in the head or neck are recognized at birth, and 90 percent will be apparent by age 2.
You can have one lymphangioma localized to a certain area or many throughout the body. Symptoms vary based on the size and location of the lymphangioma. Lymphangiomas may appear as tiny reddish or blue dots. As lymph accumulates, they can create significant and deforming swelling and masses.
Depending on where the swelling is located and the tissue or organs affected, serious health problems can occur. For example, a swollen tongue can cause speech and eating difficulties. Lymphangiomas of the eye socket can cause double vision. Lymphangiomas affecting the chest may result in difficulty breathing and chest pain.
Lymphangiomas occur when lymph backs up in the lymph vessels surrounding the body’s tissues. That causes pooling and swelling. Experts aren’t exactly sure why this occurs, but they think it might happen when there’s an error in cell division during fetal development. This error can cause a dysfunctional lymphatic system.
Lymphangioma can also sometimes occur in babies born with too many or not enough chromosomes, such as those with Down syndrome and Noonan syndrome. Most with lymphangiomas are born with them. They may not be apparent at birth, but can become more noticeable as these children grow older.
There are several types of lymphangioma. Types are mostly defined by their size.
These are lymphangiomas that are bigger than 2 centimeters (cm) with defined borders. They also may be called cystic hygromas or cavernous lymphangiomas. They sometimes appear as bluish-reddish, spongy masses.
These lymphangiomas are smaller than 2 cm and have undefined borders. They may grow in clusters and can appear as tiny blisters. They also may be referred to as lymphangioma circumscriptum and capillary lymphangiomas.
Combined or mixed
As the name implies, this is a combination of the two other types of lymphangioma.
The diagnosis of lymphangiomas can be made before birth if an ultrasound exam picks up on the abnormality. If a doctor notices one after birth, they may order an MRI scan, CT scan, or ultrasound to confirm the diagnosis, and evaluate the size and impact. If the lymphangioma isn’t visually noticeable at birth, it should become apparent by the time a child is 2 years old.
Your child’s doctor may advise against treatment if symptoms are mild or absent. If the lymphangioma is large, uncomfortable, or impacting your child’s well-being in any way, your doctor is likely to suggest one of several therapies. Treatment will depend on the type, size, and location of the lymphangioma.
Treatment may include:
- Surgery: This involves cutting out the cyst, but it can be difficult if the mass is close to organs and nerves.
- Sclerotherapy: This therapy involves injecting a chemical directly into the mass to shrink and collapse it, and it’s becoming more commonly used. Sclerotherapy has about the same success rate as surgery in treating lymphangiomas, but with fewer complications.
- Laser therapy or radiofrequency ablation: A laser or needle delivers a current to destroy the mass.
It’s nearly impossible to remove or destroy every cell of a lymphangioma, so they tend to grow back. Multiple surgeries or treatments may be needed over time to fully manage this condition.
Recurrence rates vary according to the type and location of the lymphangioma, as well as whether it was in one area or in several throughout the body.
Many lymphangiomas cause no health problems at all. They aren’t cancerous, and they don’t increase risk for developing cancer. When a lymphangioma does need treatment, the therapies are very successful. Many of these cysts and masses can be removed or reduced in size. Overall, people generally recover without any problems. Repeat treatments may needed, however, since lymphangiomas do tend to recur.