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                                                 D18.1 Lymphangioma

Lymphangiomas are uncommon, hamartomatous, congenital malformations of the lymphatic system that involve the skin and subcutaneous tissues. The classification of lymphangiomas lacks a standard clear definition and universal application, in part because of the nature of lymphangiomas, which represent a clinicopathologic continuum. The classification most frequently used divides these lesions into 2 major groups based on the depth and the size of these abnormal lymph vessels. The superficial vesicles are called lymphangioma circumscriptum. The more deep-seated group includes cavernous lymphangioma and cystic hygroma. Many categorize cystic hygroma as a variant of cavernous lymphangioma. Note the image below.

Lymphangiomas, also called lymphatic malformations, are noncancerous, fluid-filled cysts that occur in lymphatic vessels. These vessels contain a substance called lymph, and together they make up the lymphatic system. Lymph helps to properly regulate fluid in body tissue. Lymph also works with the immune system to help fight infection.

It’s estimated that lymphangioma occurs in about 1 in 4,000 births. They can affect nearly any part of the body, but are most common on the head or neck. About 75 percentTrusted Source of lymphangiomas are located in this region. Half of all lymphangiomas in the head or neck are recognized at birth, and 90 percent will be apparent by age 2.

Symptoms of lymphangioma

You can have one lymphangioma localized to a certain area or many throughout the body. Symptoms vary based on the size and location of the lymphangioma. Lymphangiomas may appear as tiny reddish or blue dots. As lymph accumulates, they can create significant and deforming swelling and masses. Depending on where the swelling is located and the tissue or organs affected, serious health problems can occur. For example, a swollen tongue can cause speech and eating difficulties. Lymphangiomas of the eye socket can cause double vision. Lymphangiomas affecting the chest may result in difficulty breathing and chest pain.

Causes of this condition

Lymphangiomas occur when lymph backs up in the lymph vessels surrounding the body’s tissues. That causes pooling and swelling. Experts aren’t exactly sure why this occurs, but they think it might happen when there’s an error in cell division during fetal development. This error can cause a dysfunctional lymphatic system.

Lymphangioma can also sometimes occur in babies born with too many or not enough chromosomes, such as those with Down syndrome and Noonan syndrome. Most with lymphangiomas are born with them. They may not be apparent at birth, but can become more noticeable as these children grow older.

Diagnosing lymphangioma

The diagnosis of lymphangiomas can be made before birth if an ultrasound exam picks up on the abnormality. If a doctor notices one after birth, they may order an MRI scan, CT scan, or ultrasound to confirm the diagnosis, and evaluate the size and impact. If the lymphangioma isn’t visually noticeable at birth, it should become apparent by the time a child is 2 years old.

Treatment for this condition

Your child’s doctor may advise against treatment if symptoms are mild or absent. If the lymphangioma is large, uncomfortable, or impacting your child’s well-being in any way, your doctor is likely to suggest one of several therapies. Treatment will depend on the type, size, and location of the lymphangioma.

Treatment may include:

• Surgery: This involves cutting out the cyst, but it can be difficult if the mass is close to organs and nerves.
• Sclerotherapy: This therapy involves injecting a chemical directly into the mass to shrink and collapse it, and it’s becoming more commonly used. Sclerotherapy has about the same success rate as surgery in treating lymphangiomas, but with fewer complications.
• Laser therapy or radiofrequency ablation: A laser or needle delivers a current to destroy the mass.

It’s nearly impossible to remove or destroy every cell of a lymphangioma, so they tend to grow back. Multiple surgeries or treatments may be needed over time to fully manage this condition. 

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Trusted Sourcerates vary according to the type and location of the lymphangioma, as well as whether it was in one area or in several throughout the body.