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What is Lympatic Malformation Including Cystic Hygroma?

A lymphatic malformation [lim-FAT-ik MAL-for-MAY-shun] is a rare condition where a fluid-filled mass grows in the lymphatic system. The malformations are lymphatic tissue filled with fluid (cyst). It is possible for a child to have one or more of these cysts.

These malformations can be found anywhere in your child’s body (except for the brain), although they are most often found in the head and the neck. Some lymphatic malformations can be very small, while others can be very large. 

Most of the time, this mass is not cancer. However, they can still be harmful as they take up space in your child’s body and can affect how other body systems function. Sometimes they can also change the shape of the affected area.

The fluid will gather in spaces in the lymphatic system. These spaces are created when the lymphatic system first develops. Doctors can detect it by the time a child is 2 years old, although most of the time they are found at birth. 

Most of the time, a lymphatic malformation will only affect one area of your child’s body. In more rare cases, they can affect more than one area. These malformations often grow larger as your child grows, but can also get smaller without any reason. Puberty can also make a lymphatic malformation can grow faster. Other things that can make a malformation grow faster include infections, traumas, or bleeding in the affected area. 

The lymphatic system is a part of the immune system and helps the body get rid of illness and disease. The lymphatic system is a series of tubes, or channels, through the body. These channels transport a thin, water-like fluid called lymph [LIMF] from all over the body to the bloodstream. As the lymph passes through the body, it moves through lymph nodes, which are placed in certain places throughout the body, such as the neck, armpits, groin, and behind the knees.

Symptoms

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 child is 2 years of age. If a malformation is not diagnosed at birth, they are usually found because they have compressed or shifted some of the nearby organs or other body structures.

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

  • A soft, smooth lump or mass. This is most often found on the neck. It can also be on your child’s head, mouth, tongue, eye, chest, stomach, arms, legs, scrotum, or 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.
  • Trouble breathing and swallowing.
  • Trouble seeing.
There are other conditions that have similar symptoms to a lymphatic malformation. It is a good idea to have your child see a doctor if you notice these symptoms. If your child has not already been diagnosed with the condition, it is important to rule out other conditions, as they can sometimes be more serious.

When to See a Doctor

See your child’s doctor if your child has any symptoms of a lymphatic malformation. If it goes untreated, it may cause problems. It can quickly get larger, become infected, or bleed. Even if your child’s malformation is treated, it may come back.

There are other conditions that have similar symptoms to a lymphatic malformation. It is a good idea to have your child see a doctor if you notice these symptoms and your child has not already been diagnosed with the condition to rule out these other possibilities, as they can sometimes be more serious.

Call your child's health care 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.

Causes

Although the exact cause of lymphatic malformation is not known, it seems that 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. 

Diagnosis and Tests

A lymphatic malformation can be diagnosed during pregnancy with:

  • An ultrasound. A healthcare provider may first see this condition in your baby during an ultrasound.
  • An MRI test. MRIs use large magnets, radio waves, and a computer to make pictures of inside the body.

After birth, a lymphatic malformation can be diagnosed with:

  • An exam. Your baby’s healthcare provider may diagnose a malformation during an exam.
  • A CT scan. A CT scan shows detailed images of any part of the body. This test will show if other organs are connected to the malformation.
  • An MRIAn MRI may also be used after birth. An MRI is more detailed than a CT scan.

Treatments

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

Specific treatments consist of:

  • Watching. Your child’s healthcare provider may watch 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 excise (cut out) small and some large cysts. The cysts may be partly or fully removed.
  • Sclerotherapy. Your child may get shots (injections) into the cysts. These can destroy them.
  • Laser therapy or radiofrequency ablation. These tests destroy cysts with a laser or radio waves. These are used on small cysts or cysts in the mouth.
Your child may need follow-up care after surgery or other treatments. Make sure you go to all appointments.

Prevention

As there is currently no known cause of lymphatic malformations, there are no known ways to prevent them from happening.

A lymphatic malformation is a rare condition where a fluid-filled mass grows in the lymphatic system. These malformations can be found anywhere in the body (except for the brain), although they will usually affect the head and the neck. Some lymphatic malformations can be very small, while others can be very large.