Lymphatic malformations
A lymphatic malformation is a mass in the head or neck that results from an abnormal formation of lymphatic vessels. Other names for these malformations are lymphangiomas or cystic hygromas. 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.
Nearly all these malformations occur in the neck although some can occur in the mouth, cheek, and tissues surrounding the ear, as well as other parts of the body. Over half of lymphatic malformations are congenital (present at birth), and most are detected by age 2. 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.
Symptoms
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:
- A mass or lump in the mouth, cheek, or tongue beneath the mucous membrane that lines the area
- A large, fluid-filled mass to the lower side and back of the neck under the platysma muscle (a thin, flat muscle that extends from the upper chest to the jaw)
The symptoms of a lymphatic malformation may resemble other neck masses or medical problems. Always consult your child's physician for a diagnosis.
The diagnosis
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:
- Transillumination, a method of examination by the passage of light through tissues to assist in diagnosis. The light transmission changes with different tissues.
- Computed tomography scan (also called a CT or CAT scan) to determine if other organs are connected to the malformation. A CT scan is a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
- Magnetic resonance imaging (MRI), a noninvasive diagnostic technique that produces computerized images of internal body tissues and is based on nuclear magnetic resonance of atoms within the body induced by the application of radio waves.
Treatment
Specific treatment of lymphadenopathy will be determined by your child's physician based on:
- Your child's age, overall health, and medical history
- Extent of the condition
- Your child's tolerance for specific medications, procedures, or therapies
- Expectations for the course of the condition
- Your opinion or preference
Treatment may include:
- Observation of the malformation (to watch for growth or changes)
- Antibiotic medications (to treat infection)
- Incision and drainage of the lesion
- Surgery (to remove the lymphatic malformation)
A small percentage of lymphangiomas in the neck can regrow and are often detectable within a year after surgery. Those that occur above the hyoid bone in the oral cavity or on the face can have recurrance rates up to 80 percent and may require subsequent surgeries.