Lymphatic Malformations

What are lymphatic malformations?

Lymphatic malformations are lymphatic vessels that do not form properly. They form before a baby is born and grow into a cluster of non-cancerous (benign) spongy masses. Lymphatic malformations usually grow just under the skin, but can also form in the bones or other organs. Although they are most often found in the head and neck area, they can grow anywhere in the body.

The lymphatic system is part of the immune system. Lymphatic vessels are filled with fluid that they carry throughout the body. Lymphatic malformations occur when the lymph moves slowly or does not drain well. This causes the fluid to pool and swell, which can put pressure on nearby organs or body parts. They can also contain veins and may fill with blood.

Lymphatic malformations are groups into two main types:

• Microcystic lymphatic malformations have small cysts and look sponge-like.
• Macrocystic lymphatic malformations have large cysts that are may be filled with fluid or blood.

What are the signs and symptoms of lymphatic malformations?

The symptoms of lymphatic malformations can vary depending on the size of the malformation and where it is in the body. Some common symptoms in children include:

• Swelling in the neck or armpit
• A soft lump or mass, most often on the neck, but can appear almost anywhere on the body
• Blood blisters on the skin
• Enlargement of a body part, such as a cheek, lip, ear, toe or tongue
• Problems with breathing, eating or speaking

Children with lymphatic malformations have a higher risk for skin infections. They may also have bleeding and pain in the area.

What causes lymphatic malformations?

Lymphatic malformations develop early in pregnancy. Experts do not know what causes them to grow. Lymphatic malformations are associated with a mutation in the PIK3CA gene, for which we now have medical therapies available, and they can be diagnosed through a simple biopsy.

How are lymphatic malformations treated?

Treatment for lymphatic malformations can depend on where the malformation is located and whether it is causing symptoms. Some children may not need any treatment. Your care team at Arkansas Children's is experienced at treating all kinds of lymphatic malformations and will work with you to create the best treatment plan for your child. Treatment options may include:

• Sclerotherapy, a procedure that injects a medicine into the malformation to make it shrink
• Medications to help to reduce pain and reduce the size of the lymphatic vessels
• Surgery to remove small malformations or to remove extra tissue