Pediatric Lymphatic Malformations

A lymphatic malformation is a lymphatic vessel that isn't formed correctly. The vessel traps the lymph fluid and causes cysts to form. Your child may have one or more of these cysts.

Lymphatic vessels are part of the lymphatic system. This is part of the immune system. It helps fight infection and other disease. It also helps keep the fluid balance in the body. It does this by emptying extra fluid into blood vessels. This system includes: 

• Lymphocytes. White blood cells that fight infection and disease.
• Lymph. White blood cells that contain fluid.
• Lymph vessels. Thin tubes that carry lymph fluid all over the body.
• Lymph nodes. These are bean-shaped glands. They are found in the armpit, groin, neck, chest, stomach, and other parts of the body.
• Other organs or body tissues. For example, these are bone marrow, spleen, thymus and tonsils. Other organs, such as the digestive tract, also contain lymphatic tissue.

Some lymphatic malformations affect nearby tissue. This causes problems and keeps the tissue from working as it should. For example, a malformation in the chest can cause breathing problems. These can be life-threatening.

A lymphatic malformation is a problem that your child is born with (congenital). This means that the issue happened during pregnancy, when your baby was forming. When the lymphatic vessels formed, they may have become blocked and enlarged. This could cause lymphatic fluid to build up.

This condition is more common in babies of older mothers. Babies with certain chromosome problems also have a higher risk. These can include Down syndrome and Turner syndrome. 

Most of the time, your child’s healthcare provider can spot symptoms at birth. If there aren’t any symptoms at birth, they often start before your baby is 2 years old.

Symptoms can be a bit different for each child. Symptoms depend on the size of the malformation and where it is. They can include:

• A soft, smooth lump or mass. This is most often on the neck. It can also be on your child’s head, mouth, tongue, eye, chest, stomach, arms, legs, or scrotum and penis.
• A lump or mass that gets larger quickly. This may be because of bleeding or an infection.
• Swelling, pain, bleeding and infection. Signs of infection can include redness, warmth, pain, swelling and drainage.
• A malformation in the chest may cause trouble breathing and swallowing.
• A malformation in the eye may cause trouble seeing.

The symptoms of this condition may look like symptoms of other health problems. Make sure your child sees their healthcare provider for a diagnosis.

A healthcare provider may first see this condition in your baby during an ultrasound in pregnancy.

You may also need an MRI test during pregnancy. An MRI can show a lymphatic malformation. MRIs use large magnets, radio waves and a computer to make pictures.

After birth, your baby’s healthcare provider may diagnose a malformation during an exam. Your baby may also need the following tests:

• CT scan. A CT scan shows detailed X-ray images of any part of the body. This test will show if other organs are connected to the malformation.
• MRI. An MRI may also be used after birth. An MRI is more detailed than a CT scan.

Treatment will depend on your child’s symptoms, age and general health. It will also depend on how severe the condition is.

Your baby's healthcare provider may refer your baby to a specialist for treatment. Your baby may need to see an ear, nose and throat doctor (ENT or otolaryngologist) or a surgeon. Your child may also need to see a doctor who specializes in conditions with imaging procedures (interventional radiologist).

Watching

Your child’s healthcare provider may watch (monitor) the malformation. They will look for signs of infection, bleeding or increases in size.

Medicines

If your child has an infection, they will need antibiotics.

Surgery

Your child may need surgery to cut out (excise) small and some large cysts. The cysts may be partly or fully removed.

Sclerotherapy

Your child may get shots (injections) into the cysts which can destroy them. Repeat treatments may be needed.

Laser therapy or radiofrequency ablation

This treatment will destroy cysts with a laser or radio waves. These are used on small cysts or cysts on the skin or mouth. Several treatments may be needed or used together with other treatments.

An untreated lymphatic malformation may cause problems. It can quickly grow in size, become infected or bleed. Even if your child’s malformation is treated, it may come back. 

Other complications depend on the location and size of the malformation. They can include:

• Large cysts in the neck or chest may get in the way of breathing and swallowing. This can be life-threatening.
• Bleeding
• Infection
• Malformed soft tissue and bone
• Surgery may harm the nearby tissue or cause bleeding or other problems
• Sclerotherapy may cause problems depending on the medicine used

If your child has this condition, their healthcare provider may watch the malformation for changes. Your child may need follow-up care after surgery or other treatments. Make sure you go to all appointments.

Your child may need special dental care. They may also need to be careful not to injure the affected area. Talk with your child’s provider about how to manage the condition.

Call your child's healthcare provider if your child has trouble breathing or swallowing. If this happens suddenly, call 911 or go to the emergency room.

You should also call your child’s healthcare provider if the malformation changes in size, bleeds or looks infected. Signs of infection include redness, warmth, swelling, pain and drainage.