Lymphangioma

A lymphangioma, or lymphatic malformation, is a rare, noncancerous (benign) fluid-filled bump under your baby’s skin. A buildup of overgrown lymph vessels causes these masses, or cysts, to form.

Lymph vessels (or channels) are part of your lymphatic system. They’re responsible for moving a colorless fluid called lymph through your tissues and bloodstream. If your baby has a blockage in their lymph vessels, fluid can collect and create a cyst.

Most lymphangiomas appear between birth and the age of 2. They usually form on your baby’s head or neck, but can develop anywhere on their body. They can be tiny bumps or large growths. Some larger malformations can cause deformities and may damage nearby vital organs. But most lymphangiomas don’t require treatment.

Types of lymphatic malformations

Healthcare providers classify lymphangiomas based on their size, depth and location. There are three main types.

Macrocystic lymphatic malformation (cystic hygroma)

Macrocystic lymphatic malformations are large, soft masses most often found deep within your baby’s neck, face, armpits or chest. They appear reddish-blue and can grow so large that they obstruct your baby’s airway at birth. Half of all babies born with this type of malformation have a chromosomal disorder, like Down syndrome, Noonan syndrome or Turner syndrome.

Microcystic lymphatic malformation (lymphangioma circumscriptum)

Microcystic lymphatic malformations are smaller, pimple-sized cysts most often found closer to the surface of your baby’s skin. They appear on their arms, legs, chest, armpits and mouth. They may vary in color, ranging from clear to pink to black. This type of malformation may be congenital (present at birth) or appear a few years later (acquired).

Mixed lymphatic malformation

Mixed lymphangiomas are simply a combination of macrocystic lymphatic malformations and microcystic lymphatic malformations.

Symptoms of a lymphangioma

Lymphatic malformations don’t normally cause pain and shouldn’t be itchy. These cysts are almost always noncancerous (benign) and are rarely life-threatening. These cases depend on the size and location of the cyst, especially if it blocks or puts pressure on a vital organ. Lymphangiomas that affect your baby’s:

• Bones: May cause bone loss or bone overgrowth
• Chest: May cause chest pain, shortness of breath (dyspnea) or wheezing
• Eyes: May cause bulging eyes (proptosis) or double vision (diplopia)
• Gastrointestinal tract or pelvis: May cause bladder blockage, constipation or frequent infections
• Mouth or windpipe (trachea): May cause feeding problems, shortness of breath (dyspnea), speech issues or trouble swallowing (dysphagia)

Lymphatic malformations are usually found in only one area of your child’s body, but sometimes, they’re widespread. When this occurs in your child’s bones and soft tissues, it’s called lymphangiomatosis.

Lymphangioma causes

Scientists don’t know the exact cause of lymphatic malformations. But they happen when your child’s lymphatic system doesn’t form properly during fetal development.

Your body has a network of vessels, tissues and organs that carry lymph — which contains white blood cells called lymphocytes — through your bloodstream and tissues. This network is the lymphatic system, and it controls how much fluid is in your body to help your immune system function properly. Like a hose carrying water, your lymphatic system is constantly flowing.

Sometimes, the fluid in a lymph vessel backs up, similar to a kink in a hose. This kink causes a pool of lymph to collect in front of the blockage. As a result, the pool of lymph appears as a liquid-filled bump under your baby’s skin (cyst).

How doctors diagnose lymphangiomas

Depending on the size of the lymphangioma, your healthcare provider may be able to detect it on a prenatal ultrasound.

After your baby is born, your provider will perform a physical exam. They’ll carefully look over the cyst and look for signs of any genetic conditions. They may also ask you questions about your medical history.

Tests that are used

Your healthcare provider may use certain tests to confirm a lymphangioma diagnosis. They can learn more about the size and what caused the growth using the following tests:

• Biopsy
• CT scan
• MRI
• Ultrasound

If your child has signs or symptoms of a genetic disease, they may suggest genetic testing. This looks for gene changes (variants), which can also help them determine treatment.

How do you treat lymphangiomas?

Most of the time, lymphatic malformations don’t need treatment because they’re noncancerous growths that may go away on their own. In this case, your child’s healthcare provider will keep an eye on it and watch for any changes (“watch and wait” approach).

Treatment might be necessary if the cyst is large and blocking a vital organ. The type of lymphangioma treatment will depend on its size and location, and may include:

• Cryotherapy: Healthcare providers can use extreme cold to freeze and remove abnormal tissue.
• Laser therapy: Providers can use certain forms of laser therapy to treat lymphatic malformations.
• Medications: Your baby’s provider may use medications like sirolimus, sildenafil or propranolol to treat a lymphangioma.
• Percutaneous drainage: In some cases, your baby’s provider might make a cut (incision) in the lymphangioma to drain the fluid.
• Radiofrequency ablation: Your baby’s provider might decide to deliver high-frequency radio waves through a small needle into the lymphangioma to destroy it.
• Sclerotherapy: Your baby’s provider can sometimes inject a solution (sclerosing agent) into the lymphatic malformation to cause scarring, eventually shrinking it.
• Surgical removal (excision): Your baby’s provider might choose surgery as the best option to remove as much of the lymphangioma as possible.

Complications of the treatment

Surgical removal of lymphatic malformations is often unsuccessful. They tend to grow back because they start deep beneath the surface of your child’s skin. It can be very difficult to locate and remove all the affected lymphatic cells. Studies have reported that more than 30% of lymphangiomas recur (come back). Cysts that are closer to the surface of your child’s skin have a lower chance of returning because most cells are easy to remove.

There’s also a high risk of infection after surgery that could complicate the healing process. Taking steps to prevent infection by cleaning and caring for the surgical site will lead to the best outcome.

When should my child see their healthcare provider?

Your child should see their healthcare provider if their lymphangioma:

• Becomes infected after surgery (leaks yellow or clear pus)
• Changes color
• Changes shape or size
• Feels warm to the touch
• Prevents your child from moving normally or using part of their body

What questions should I ask my child’s healthcare provider?

Questions you may want to ask your child’s provider include:

• Does my child’s lymphangioma need removal?
• What are the odds that my child’s lymphangioma will return after surgery?
• What should I do if my child injures their lymphangioma?

What can I expect if my child has a lymphatic malformation?

The majority of lymphangiomas aren’t life-threatening and won’t cause any health problems for your child. Rarely, the location and size of a cyst can put pressure on a vital organ, causing problems with the way it functions. In that case, they’ll need treatment. But most lymphangiomas don’t require treatment.

As your child’s caretaker, monitor their lymphatic malformation. Reach out to their healthcare provider if you notice changes to the size, color or location of the cyst, or if it prevents your child from moving normally.

Can lymphatic malformations be prevented?

You can’t prevent lymphangiomas from forming because they’re the result of the abnormal development of your child’s lymphatic system. In some cases, lymphangiomas occur as part of an underlying genetic condition. If you plan on becoming pregnant and want to understand your risk of having a child with a genetic condition, ask your healthcare provider about talking with a genetic counselor.