Lymphatic Malformations

An LM can occur alone or as part of other conditions:

• Lymphangiomatosis: LMs that are widespread in bone and soft tissue
• Gorham-Stout disease: LMs that erode the underlying bone
• Overgrowth: LMs with overgrowth of the adjacent bone and soft tissues
• CLOVES syndrome
• Noonan syndrome
• Turner syndrome
• Klippel-Trenaunay syndrome (KTS)

LMs develop before birth but may not be noticed until later. LMs happen because of genetic changes (called mutations or variants). Most often, the affected gene is PI3KCA.

The abnormal gene is not passed down by parents (not inherited). The mutation starts in 1 cell. Any cells that divide from that cell will continue the mutation. This is called a mosaic mutation.

We use sclerotherapy (SCLAIR-oh-THAIR-uh-pee) to reduce the size of an LM by blocking unneeded veins.

First, an interventional radiologist uses ultrasound guidance to pinpoint the unneeded veins. Then a doctor injects a chemical that blocks blood flow and makes the veins collapse.

Sclerotherapy may cause scarring. In some cases, the LM may not shrink or it may come back.

Read more about sclerotherapy (PDF).

Sometimes surgery to remove or reduce the size of an LM is the best option. We consider surgery based on your family’s wishes and whether the LM is:

• Causing symptoms like pain or bleeding
• Making it hard to see, breathe or move
• Affecting other important functions
• Disfiguring

Surgery may not be possible if the LM is widespread or surrounds important body parts and organs.

Some children need multiple surgeries over a period of time. Your child may have surgery as well as other types of treatment.

Surgery causes scarring and may damage the body parts that the LM affects. Years after the surgery, lymph vessels may return at the site of a scar.

We use facial nerve mapping to make surgery safer when removing LMs in the head. Before surgery, we use small electric pulses to map the exact location of the branches of the facial nerves. During the procedure, real-time feedback about nerve activity helps guide surgeons. The technique means shorter surgery time, with smaller cuts and near-zero risk of nerve injury.

Our doctors were the first to use medical-grade Super Glue to make it safer and easier to remove the tangle of extra veins. This is called glue embolization surgery (GES).

• Before the procedure, we give your child medicine so they sleep without pain (general anesthesia).
• Watching on flat screens that show your child’s LM in real time, an interventional radiologist places the needle in exactly the right spot. This assures that only the abnormal vessels are injected with glue.
• The glue hardens the LM without harming the tissue around it.
• Using a low-dose CT scan, we make a 3-D image of the LM to guide surgeons during the delicate procedure.
• Surgeons remove the LM, including the glue inside.

We use surgical laser therapy to treat some LMs, often with other types of treatment. Most children need multiple treatments spread over many months.

Before laser surgery, we give your child medicine so they sleep without pain during the treatment (general anesthesia).

Laser therapy may cause some tissue scarring or changes in the color of the skin in the area. These scars and color changes can sometimes be improved by cosmetic procedures.

LMs can cause swelling (lymphedema) that hurts, increases the risk for infection or limits motion. To ease pain and help your child be more active, a certified lymphedema therapist will create a custom treatment plan for your child.

We use complete decongestive therapy (CDT). There are 4 parts to CDT:

• Skin care
• Gentle massage to improve flow of lymph (manual lymph drainage)
• Compression therapy
• Exercise

Read more.

LMs are sometimes managed with medicines. Some medicines treat symptoms like pain or bleeding. Other medicines treat LMs directly. Your healthcare team will watch your child closely while they are taking these medicines. Your doctor will discuss the benefits and risks of each medicine.

Medicines that help manage LMs include:

• Antibiotics.
• Corticosteroids.
• Aspirin.
• Rapamycin (sirolimus). Our clinic was one of the first to offer sirolimus to patients.

For some children, genetic testing helps doctors identify the exact genetic cause of their LM. Such a precise diagnosis may expand treatment options to include drugs that target the overactive biological pathway causing the malformation. If this is the case for your child, our doctors will talk with you about the options and any research studies (clinical trials) that might be a good match.

LMs that leak fluid into the skin may cause infection (cellulitis). If cellulitis keeps coming back, it can:

• Cause pain
• Change how the skin and body looks
• Lead to a serious infection

For a serious infection, your child will need antibiotics. We may also treat them with steroid therapy. Corticosteroids can lower inflammation in the LM.

• We have treated hundreds of children with LMs. Doctors with less experience might confuse an LM for a different type of vascular anomaly, leading to the wrong treatment.
• Our Vascular Anomalies team brings together experts with many different skills and experiences. These include otolaryngologists, dermatologists, plastic surgeons, general surgeons, ophthalmologists, geneticists and interventional radiologists.
• Our physician-scientists help set national standards for care of young people with vascular anomalies. We provide the most advanced treatments in our region.

• A correct diagnosis is the first step toward improving your child's condition. We have the expertise to accurately diagnose your child’s LM. If tests are needed to confirm it, we have a broad range of options, such as genetic testing and imaging studies. We will work with you to decide the best treatment option for your child.
• Our physician-scientists have created a gene panel to test patients’ tissue and body fluids for the genetic changes known to cause vascular anomalies. We can test DNA in tissue sent to us from doctors across the nation. No other children’s hospital has a gene panel certified for clinical use with vascular malformations. This certification means doctors can make treatment decisions based on the results.
• The gene panel helps us identify the exact genetic cause of an LM in some children. A precise diagnosis may expand your child’s treatment options to include drugs that target the overactive biological pathway causing the malformation. These targeted therapies may be newly developed or already approved for other diseases.
• Our genetic testing expertise helps us create new therapies that we offer in research studies called clinical trials.
• Learn more about vascular anomalies research at Seattle Children’s.

• Until Seattle Children’s pioneered glue embolization surgery (GES), many children with vascular malformations endured repeated, painful injections or multiple surgeries. To make it safer and easier to remove an LM in a single surgery, our team first injects medical-grade Super Glue.
• For children who need surgery to remove an LM in the face, we use facial nerve mapping. This greatly reduces the risk of nerve injury and scarring.

• We schedule visits so your child sees all the specialists they need in as few days as possible. In some cases, we can consult via video phone calls with doctors or patients.
• Your child’s team will work together — and with you — to make a treatment plan that fits your child’s unique needs. Combining our skills helps make sure your child gets the very best care.
• As long as needed, our team keeps a watch on your child’s condition. We are always here to answer your questions and connect you to community resources.

• Children do not react to illness, injury, pain and medicine in the same way as adults. They need – and deserve – care designed just for them. Our experts focus on how treatments today affect growing bodies in the future. We provide the best and safest treatment for your child, based on our years of experience and the newest research.
• At Seattle Children's, we work with many children and families from around the Northwest and beyond. Whether you live nearby or far away, we can help with financial counseling, schooling, housing, transportation, interpreter services and spiritual care. Read about our services for patients and families.