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Lymphatic Malformations

What Are Lymphatic Malformations?

The lymphatic (lim-FAT-ick) system is a network of tubes that collects lymph — a clear, pale yellow fluid that seeps out of blood vessels — from all over the body and returns it to the bloodstream.

A lymphatic malformation (LM) is a clump of that form a growing, jumbled, spongy cluster. Lymphatic malformations are also known as lymphangiomas (lim-FAN-jee-oh-muhs).

What Happens in Lymphatic Malformations?

Lymphatic malformations are masses (abnormal growths), but they are not cancers. LMs grow because the lymph in their vessels cannot drain out well. This makes them swell, which is known as a flare.

LMs also grow because they contain fragile malformed veins in their walls that bleed easily. The LM can fill with blood and, if close to the skin surface, look bruised. The pooling lymph or blood stretches the vessels and cysts, making the LM grow larger.

As an LM grows, it may put pressure on nearby body parts, such as the eyes, trachea (windpipe), or blood vessels. LMs may form inside skin, fat, connective tissue, joints, organs, or bones. They can form anywhere in the body but the brain, and are most common in the head and neck area.

There are two main types of LMs: microcystic and macrocystic. The spongy microcystic type has small vessels and tiny cysts. Macrocystic LMs, also sometimes called cystic hygromas or lymphangiomas, have large, stretched vessels and cysts filled with lymph, blood from internal bleeding, or both.

What Are the Signs & Symptoms of Lymphatic Malformations?

An LM usually appears as a growing, spongy-feeling lump. A child can have more than one LM, but they are usually in the same area of the body.

The skin over LMs often has small bubbles, called vesicles, that look like tiny blisters. The fluid in them is initially clear and colorless, but will turn dark red if blood leaks into it. Less commonly, the vesicles are filled with milky fluid (called chyle).

A child with an LM may have:

  • overgrowth and swelling in affected areas (lips, tongue, jaws, cheeks, arms, legs, fingers, or toes)
  • pain and swelling of the LM
  • a tendency to injure the LM, causing bleeding, which can happen with very mild trauma or even with no known trauma

Some symptoms are related to the area of the body affected:

Tongue, windpipe, and mouth LMs may cause problems with:

  • speaking
  • breathing
  • swallowing
  • feeding 

Orbit (eye socket) LMs may cause:

  • double vision
  • bulging of the eye

Chest LMs may cause:

  • wheezing
  • chest pain or pressure
  • trouble breathing
  • narrowing of the airways to the lungs 

LMs in the gastrointestinal tract (gut) or pelvis can cause:

  • bleeding from the rectum
  • constipation (trouble pooping)
  • bladder obstruction (trouble peeing)
  • infections
  • protein loss due to poor absorption and loss of lymph into the gut

LMs of the genitourinary tract (kidney and bladder) can cause:

  • blood in the pee
  • pain when peeing

LMs in bones can cause:

  • bone overgrowth
  • bone loss
  • rarely, a fracture

What Causes Lymphatic Malformations?

LMs form very early in pregnancy, but doctors don't know why. Most LMs are thought to happen at random during the baby's development. They're not caused by anything a woman did or didn't do during her pregnancy.

Some of the more severe LMs do have genetic mutations identified, but are also thought to happen randomly and are not inherited.

Who Gets Lymphatic Malformations?

LMs are more common in children with:

  • Down syndrome
  • Turner syndrome
  • an overgrowth syndrome (genetic disorders that cause an abnormal increase in the size of the body or a body part)
  • Noonan syndrome

How Are Lymphatic Malformations Diagnosed?

LMs may be seen on a prenatal (before birth) ultrasound scan. An LM not found earlier might be detected when the baby is born. Most significant LMs are apparent by age 2, after lymph fluid has built up and stretched the vessels of the LM or there is bleeding into the LM. But some LMs aren't found until the teen years or later.

To be sure the lump is an LM and not another kind of tumor, doctors will do a physical exam and order imaging tests such as:

  • ultrasound scan
  • X-ray
  • computed tomography (CT or CAT) scan
  • magnetic resonance imaging (MRI) scan

How Are Lymphatic Malformations Treated?

Pediatric specialists often work together as a team to provide the best treatment for a child's LM. The location of the lymphatic malformation and the body parts it's pressing on determine which specialists are needed for the team. The doctors involved may include:

  • pediatricians
  • vascular and interventional radiologists
  • hematologists/oncologists 
  • pathologists
  • genetic specialists
  • orthopedic surgeons
  • pediatric medical specialists (pulmonology, cardiology, etc.)
  • pediatric surgeons
  • neurosurgeons
  • ophthalmologists (eye surgeons)
  • otolaryngologists (ear, nose, and throat surgeons)
  • speech pathologists

The team will study the LM's type and location and consider the child's age, health, and other medical problems. They'll decide on a treatment with the fewest side effects and risks, and with the best outcomes.

Things to know about treatment options:

  • Most suddenly appearing LMs will decrease in size and pain without treatment, but very rarely resolve by themselves.
  • Incision (cutting into) and draining the lymph from an LM can temporarily reduce its size. This is usually only done for diagnosis or to treat an infection.
  • Sclerotherapy (injection of a medicine into the LM) causes the lymph vessels to collapse and scar together, so the LM shrinks.
  • Surgical removal is sometimes the best choice when the LM affects organs or interferes with speech, swallowing, or appearance. Minimally invasive surgery may be used, so the surgical scars are very small.
  • Laser therapy may be used for LMs in the skin or mouth.
  • Radiofrequency ablation is sometimes used to treat LMs in the tongue. A needle inserted into the LM sends out radio waves that ablate (destroy) the malformation.
  • Medicines are more commonly being used for large LMs. These are low risk, generally well tolerated, and shrink most LMs over time.

Antibiotic treatment and drainage of the LM may be needed if an LM becomes infected, which is rare.

Looking Ahead

Lymphatic malformations can grow back, so kids might need to have more than one treatment.

Kids may feel shy or embarrassed about how an LM looks. So it's very important to support your child emotionally. It can help to find a local or online support group where other families can talk about what works for them. Ask your child's care team for recommendations. Some kids and teens might benefit from talking to a therapist if they're feeling sad or upset about an LM or its treatment.

Date reviewed: April 2018
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