Common Childhood Conditions
What Does a Lymphatic Malformation (LM) Look Like?
Macrocystic lymphatic malformation on a newborn.
An LM is often present at birth, most commonly in the neck and face area.
LMs do not involve blood vessels. Instead, they involve the lymphatic, or body fluid, system. They result from a blockage or defect of the lymphatic vessels as they are forming.
Often called cystic hygroma or lymphangioma, LMs are sometimes misdiagnosed as hemangiomas or vein malformations.
The lymphatic system is a collection and transfer system for fluid in the body's tissue, and is part of the immune system.
The lymph nodes are also part of this same system.
When the lymphatic channels are blocked, this results in excess fluid build-up in both the tissue and the vessels. When this is close to the surface of the skin, you can see a prominent enlargement of the area.
If the face is involved, it will swell and become disfigured. If it occurs in the mouth or tongue, it can interfere with eating and breathing. These lesions may occur anywhere, but are often found in the head and neck.
What Causes an LM?
The exact cause of the formation of these lesions is unknown. The lymphatic system formation error is believed to occur as a baby is forming during the early embryonic period. No genetic defect (faulty gene) has been detected at this time.
Will an LM Change?
Because of the nature of the lymphatic circulatory system, certain issues are unique to LMs.
When your child gets ill from a virus or bacteria, the symptoms get worse and the malformation can swell rapidly.
The lymphatic system responds to the bacteria or virus by increasing the chylous (ki-lus) flow (a milky fluid consisting of lymph and fat extracted during digestion and passed to the bloodstream through the thoracic duct). This causes swelling in the affected area. After your child recovers, the swelling slowly goes down.
LMs can increase or grow with the child.
I Think I've Seen an LM on My Child. What Should I Do Now?
Referral to a specialty center such as Children's is crucial to ensure proper treatment by a team of medical specialists. These specialists will include surgeons, interventional radiologists (doctors who specialize in diagnosing and treating with MRIs, CT scans and ultrasound), dermatologists, and nurse practitioners who are experienced with the special needs of children with this condition.
How Will Seattle Children's Diagnose a LM?
These masses are first evaluated by physical exam and then by imaging (X-rays). Magnetic resonance imaging (MRI) or CT scans give the best information on the extent and location of the cyst.
How Will Seattle Children's Treat an LM?
Lymphatic malformation on the lateral face.
Since these lesions are part of the immune system which fights infection, the masses are highly prone to infection. This is the main cause for concern and the reason prompt treatment in young children is necessary. Bleeding can occur in the LM, causing pain and swelling.
Because the blood vessel system and lymph system run so close together, any suspected infection of an LM must be treated right away with oral or even intravenous (in the vein) antibiotics. This is done to avoid the risk of sepsis (infection in the blood).
Families need to learn the warning signs and symptoms of infection and the importance of careful hygiene practices, including careful mouth and teeth care.
Two methods most often used to treat LMs are:
- Sclerotherapy - using medications to reduce swelling and infection. Sclerosing agents are injected into certain large cysts to reduce their size. This means that the cyst is not removed, but "scars" down on itself so that growth stops. This procedure may be done by an interventional radiologist or a surgeon.
- Surgery - removal of the LM by cutting it out surgically. This is often done through several surgeries over a period of time to reduce the risk of complications. If the airway is involved, the LM is removed as soon as it is diagnosed. Sometimes, a tracheotomy may be needed to keep the airway open.
In addition, steps are taken to reduce swelling of the tissue around the area involved with the LM and to prevent infection.
Sometimes, immune defects are detected in children affected with this condition. If this is the case, immunology testing will be done.