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Lymphatic Malformations

What is a lymphatic malformation?

A lymphatic malformation is a mass in the head or neck that results from an abnormal formation of lymphatic vessels. Lymphatic vessels are small canals that lie near blood vessels and help carry tissue fluids from within the body to the lymph nodes and back to the bloodstream.

There are two main types of lymphatic malformations:

Nearly all cystic hygromas occur in the neck, although some lymphangiomas can occur in the mouth, cheek, and tissues surrounding the ear, as well as other parts of the body. Lymphatic malformations are present at birth, but they are sometimes not detected until the child is older. Some lymphatic malformations can spread into surrounding tissues and affect the proper development of the area. Lymphatic malformations may also enlarge and become infected following an upper respiratory infection.

Lymphatic malformations are sometimes seen in children with certain chromosome abnormalities and genetic conditions, including Down syndrome and Turner syndrome.

What causes a lymphatic malformation?

A lymphatic malformation is a congenital (present from birth) defect that occurs during early embryonic development when the lymphatic vessels do not properly form. The vessels may become blocked and enlarged as lymphatic fluid collects in the vessels, forming a mass or a cyst.

What are the symptoms of a lymphatic malformation?

An internal lymphatic malformation in the mouth or cheek may not be noticed until it becomes infected and enlarges. Cystic hygromas can grow very large and may affect breathing and swallowing. The following are the most common symptoms of a lymphatic malformation. However, each child may experience symptoms differently. Symptoms may include:

The symptoms of a lymphatic malformation may resemble other neck masses or medical problems. Always consult your child's physician for a diagnosis.

Illustration of transabdominal fetal ultrasound

How is a lymphatic malformation diagnosed?

During pregnancy, a fetal ultrasound may detect some large lymphatic malformations. Ultrasound is a diagnostic tool used to evaluate organs and structures inside the body with high-frequency sound waves. After birth, diagnosis of a lymphatic malformation is generally determined by a physical examination. In addition to a complete medical history and physical examination, diagnostic procedures for a lymphatic malformation may include the following:

Treatment of lymphatic malformation:

Specific treatment of lymphatic malformation will be determined by your child's physician based on:

Treatment may include:

A small percentage of lymphangiomas can regrow and are often detectable within a year after surgery. Regrowth is more likely with larger and more complicated malformations.

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