A lymphatic malformation is a clump of lymph
vessels that form a growing, jumbled, spongy cluster.
Lymphatic malformations are masses (unusual growths), but they are
(not cancerous).
What Happens in Lymphatic Malformations?
The lymphatic (lim-FAT-ik) system is a network of tubes that collects lymph (LIMF).
This clear, pale yellow fluid drains out of blood vessels all over the body. The lymphatic system
returns the lymph to the bloodstream. Lymphatic malformations grow because the lymph
in their vessels can't drain out well. This makes them swell, which is known as a
flare.
Lymphatic malformations also grow because they contain fragile malformed veins
in their walls that bleed easily. The lymphatic malformation can fill with blood.
The pooling lymph or blood stretches the vessels and cysts, making the lymphatic malformation
grow larger. If it's close to the skin surface, it can look bruised.
As a lymphatic malformation grows, it may put pressure on nearby body parts, such
as the eyes, trachea (windpipe), or blood vessels. Lymphatic malformations may form
inside skin, fat,
, 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 lymphatic malformations:
microcystic lymphatic malformations are spongy and have small
vessels and tiny cysts.
macrocystic lymphatic malformations (also 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?
A lymphatic malformation usually appears as a growing, spongy-feeling lump. A child
can have more than one lymphatic malformation, but they are usually in the same area
of the body.
The skin over lymphatic malformations often has small bubbles, called vesicles
(VESS-ih-kuls). These look like tiny blisters. The fluid in them starts out clear
and colorless, but will turn dark red if blood leaks into it. Less commonly, the vesicles
are filled with milky fluid called chyle (KYE-ul).
A child with a lymphatic malformation may have:
overgrowth and swelling in affected areas (lips, tongue, jaws, cheeks, arms, legs,
fingers, or toes)
pain and swelling of the lymphatic malformation
a tendency to injure the lymphatic malformation, 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 lymphatic malformations may cause problems with:
speaking
breathing
swallowing
feeding
Orbit (eye socket) lymphatic malformations may cause:
double vision
bulging of the eye
Chest lymphatic malformations may cause:
wheezing
chest pain or pressure
trouble breathing
narrowing of the airways to the lungs
Lymphatic malformations in the gastrointestinal tract (gut) or pelvis can cause:
Lymphatic malformations form very early in pregnancy, but doctors don't know why.
Most lymphatic malformations 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 lymphatic malformations do have genetic
mutations identified. But they're also thought to happen randomly and are not inherited.
Lymphatic malformations are more common in children with:
an overgrowth
(genetic disorders that cause an unusual increase in the size of the body
or a body part)
Noonan syndrome
How Are Lymphatic Malformations Diagnosed?
Lymphatic malformations may be seen on a prenatal (before birth) ultrasound
scan. A lymphatic malformation not found earlier might be found when the baby is born.
Most significant lymphatic malformations are seen by age 2, after lymph fluid has
built up and stretched the vessels of the lymphatic malformation or there is bleeding
into it. But some aren't found until the teen years or later.
To be sure the lump is a lymphatic malformation and not another kind of
, doctors will do an exam and order imaging tests such as:
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 lymphatic malformation's type and location and consider
the child's age, health, and other medical problems. They'll decide on a treatment
that has the fewest side effects and risks, and offers the best results.
Things to know about treatment options:
Most lymphatic malformations that appear suddenly will decrease in size and pain
without treatment, but very rarely go away on their own.
Incision (cutting into) and draining the lymph from a lymphatic malformation can
temporarily reduce its size. This is usually only done for diagnosis or to treat an
infection.
Sclerotherapy (injection of a medicine into the lymphatic
malformation) makes the lymph vessels collapse and scar together, shrinking the lymphatic
malformation.
Surgical removal is sometimes the best choice when the lymphatic malformation 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 lymphatic malformations in the skin or mouth.
Radiofrequency ablation is sometimes used to treat lymphatic malformations in
the tongue. A needle inserted into the lymphatic malformation sends out radio waves
that ablate (destroy) the malformation.
Medicines are often used for large lymphatic malformations. These are low-risk,
generally well-tolerated, and shrink most lymphatic malformations over time.
Antibiotic treatment and drainage of the lymphatic malformation may be needed if
it gets infected, which is rare.
What Else Should I Know?
Lymphatic malformations can grow back, so kids might need to have more than one
treatment.
Kids may feel shy or embarrassed about how a lymphatic malformation looks. Be sure
to support your child emotionally. It can help to find a local support group where
other families can talk about what works for them. Ask your child's care team for
recommendations. You also can look online at:
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