Lymphatic Malformations (LMs)

About Lymphatic Malformations (LMs)

When lymph vessels are not formed properly, lymph fluid can collect and form cysts. Lymphatics are part of the immune system, carrying clear fluid rich with white blood cells that help remove waste and toxins from the body. LMs can occur anywhere in the body, sometimes in isolation or widespread to a limb or deeper in the muscles and bones. Although LMs can be present at birth, they can also occur in young people or adults. They can grow rapidly or slowly and cause other complications or infections depending on their location. 

Types of lymphatic malformations include:

• Microcystic — clusters of small fluid-filled cysts.
• Macrocystic — larger interconnected fluid-filled cysts.
• Channel Type — central conducting lymphatic anomaly usually affecting the chest
• Generalized Lymphatic Anomaly (GLA) — abnormal lymphatic development in multiple organ systems, commonly affecting the respiratory and abdominal lymphatics
• Kaposiform lymphangiomatosis (KLA) — a very rare lymphatic anomaly that is aggressive and can cause severe symptoms to the lungs and bones and causes pain and bleeding.
• Gorham Stout — abnormal lymphatic vessels invade the bones causing loss of bone and make patients at risk for fractures. 
• Lymphedema — more commonly affecting the legs than arms. This is tissue swelling caused by an accumulation of protein-rich lymphatic fluid not being drained properly through the body's lymphatic system. This swelling can be painful and affect the movement of the limb.

Symptoms

Depending on size and location, symptoms can range. Larger LMs can cause swelling in the area and can compress vital organs. Smaller LMs appear on the skin as translucent or clear or dark blood-filled clusters that can leak lymphatic fluid and bleed. LMs can cause the enlargement of the soft tissues, muscles, or bones. Complications include bleeding and infection due to cellulitis or sepsis.

Diagnosis

Columbia doctors can diagnose lymphatic malformations during the examination. In addition, Columbia doctors may use:

• Ultrasound — to evaluate the cysts if they are near the skin’s surface. Some LMs are detected before birth during a prenatal ultrasound.
• Magnetic Resonance Imaging (MRI) — to help confirm the diagnosis and evaluate the extent of the LM, the number and size of the cysts, and the impact on the surrounding tissues.
• MR lymphangiogram and conventional lymphangiography — to study the central lymphatic flow.

Treatments We Offer

Columbia doctors offer a range of options to treat LMs that includes: 

• Compression therapy — especially for patients with lymphedema.
• Occupational and physical therapy — manual lymphatic drainage, especially for lymphedema.
• Sclerotherapy — a minimally invasive procedure performed by an interventional radiologist that involves draining and injecting the cysts with a medication that will irritate the inside lining, causing it to shrink.
• Medications such as sirolimus.
• Surgery.

Why Choose Columbia?

Here at Columbia, we are a designated and world-renowned Comprehensive Center of Excellence in lymphatic diseases by the Lymphatic Education & Research Network (LE&RN).  We are experts in the research, diagnosis, and treatment of lymphatic malformations and we are leading the way in innovative techniques and therapies. Rest assured, our team works with you to discuss which treatments are the least invasive and most effective for your child.