Welcome to part 2 of our Lymphatic System series, on Lymphatic Malformations, by the
Vascular Anomalies Center at Children’s Hospital Colorado.
Click here to watch part 1, which explains how the Lymphatic System works, or check out
the description below.
A Lymphatic Malformation is when the Lymphatic System doesn’t form correctly, causing parts
of the body to swell.
Since it occurs before birth, it’s a “congenital,” or “primary” condition, and is usually
visible by early childhood, or more rarely, later in life.
It can vary greatly between people, and can change appearance over time.
To many, this swelling can be emotionally distressing, socially isolating, and difficult
to understand.
When we form in the womb, our cells read our DNA, or “blueprints,” to build everything
in our bodies, including the blood and lymphatic systems, the two mass transit systems of our
bodies.
If the blueprint is misread, or contains errors, the lymphatics gets built incorrectly, and
are “mal-formed.”
This is why it’s called a “lymphatic mal-form-ation.”
Terms like “cystic hygroma” and “lymphangioma,” while still widely used, are outdated and
incorrect because they suggest some relation to cancer.
A lymphatic malformation is not cancer, and does not become cancerous.
There are many kinds of lymphatic malformations: First, “cystic lymphatic malformations”
are lymphatics that were built incorrectly.
The lymph piles up like a traffic jam, forming a group of bubbles, which we call “cysts.”
They can be “microcystic,” meaning “tiny bubbles,” “macrocystic” meaning “big
bubbles”, or “mixed.”
The microcystic type form in clusters, and are very small and sponge-like.
If near the skin, they may appear as small blisters, called “blebs.”
The macrocystic type are larger than about 2cm, and can become much larger.
They look like soft, smooth, translucent bubbles under the skin, and may tint the skin slightly
blue.
They can be painful, and put pressure on other parts of the body.
Both types can form anywhere in the body, but most often form near major lymphatic channels,
especially near the neck and armpits.
CCLA’s, or “Central Conducting Lymphatic Anomalies,” are when the central lymphatics,
the main central highway that transports chyle from the intestines, is malformed.
Much larger traffic jams form, and the body tries to relieve the pressure by building
new routes anywhere it can, even taking side-streets.
These new routes don’t reach the heart, however, and the lymph pools up again, and
some pools may leak into the chest or abdomen.
When this happens, fats, proteins, immune cells, and other important passengers can’t
get to their destinations, which, over time, starts to cause health problems throughout
the body.
In the chest, this is called a “chylothorax,” which can cause a cough, discomfort in the
chest, and difficulty breathing.
In the abdomen, it’s called “Chylous Ascites.”
The abdomen can become very large, full, and painful.
Lymphatic malformations may not become visible until later in life, and many things can change
their appearance.
For example, when an injury occurs, immune cells are sent to the affected area.
If the injury gets infected, even more immune cells are sent.
If a cyst is nearby, it can swell, or even fill up with blood, changing its appearance.
The infection itself can also cause redness, swelling, and pain.
A cyst may also expand slowly with age, or more rapidly during puberty.
There are many ways to treat a Lymphatic malformation, depending on its cause: First, the cyst may
be observed over time to see if it deflates on its own.
Next, "sclerotherapy" can help macrocystic lymphatic malformations shrink or collapse.
While asleep under general anesthesia, the cyst is partially deflated, and a medication
is injected that irritates it, causing scar tissue to form.
This irritation lasts for about 2-3 days, and usually swells for 1-2 weeks.
Over about a month, the cyst starts to shrink as the scar tissue grows.
Multiple treatments may be needed depending on the extent of the malformation.
If the cyst is limited to a small enough area, part of it can be removed by surgery.
This option depends on many factors, like how nearby organs would be affected by the
surgery.
Unless the malformation is limited to a small area, sclerotherapy and surgery will not completely
repair it, and flare-ups of swelling can still happen.
These symptoms can be managed, however, but need long term monitoring.
Finally, medications may help slow the flow of lymph, and soften the cyst.
This helps organize the traffic jams, allowing them to drain.
This is an active field of research, and places like Children's Hospital Colorado are helping
future medications become more targeted, personalized, and effective.
Call to Action Our team is ready to work with you to best understand and treat your lymphatic
malformation.
To learn more, contact the Vascular Anomalies Center by visiting our website below.