Lymphangioma

The lymphatic system plays an important role in the body's immune system because it helps to circulate disease-fighting white blood cells throughout the body. However, these tiny lymphatic vessels can become very problematic if a lymphangioma develops. These benign masses can grow to an excessive size that will require surgical treatment to fix.

Definition & Facts

A lymphangioma is a malformed cyst that occurs somewhere within the lymphatic system. These cysts are benign and non-cancerous. Depending on their size and position, lymphangiomas are divided into a few different categories.

• A capillary lymphangioma happens when only the small, capillary-like lymphatic vessels near the epidermis are affected.
• Cavernous lymphangiomas are cysts made up of dilated lymphatic channels that spread throughout the body's tissue.
• A cystic hygroma is a specific type that occurs when the lymphatic vessels collect an abnormal amount of fluid and become very large.
• The fourth type of lymphangioma is a hemangiolymphangioma that occurs when the lymphatic cyst becomes intertwined with blood vessels.

Lymphangiomas are also categorized into either suprahyoid if they are above the hyoid bone in the neck or infrahyoid if they are below the bone and in the body.

Symptoms & Complaints

Smaller lymphangioma, called lymphangioma circumscriptum, right below the surface of the skin normally just look like a small cluster of blisters that are pinkish or reddish.

A cavernous lymphangioma is deeper beneath the skin, but it is large enough to become visible. The mass of a cavernous lymphangioma has a slightly fleshy feel, and it can be a small bump or a massive bulge. These types of lymphangiomas normally appear on either the neck, tongue, hand, or foot. The color can vary anywhere from pink to deep purple or blue. Cystic lymphangiomas have very similar appearances to cavernous ones, but they feel softer and can appear in the armpit or groin.

Most lymphangiomas are painless, but occasionally, patients report tenderness when pressure is applied. Over time, lymphangiomas very slowly grow in size. Eventually they can become large enough to put pressure on other organs in the body that can cause other health problems. For example, if the lymphangioma is putting pressure on the lungs or airway, a patient can start to have difficulty breathing, and if it is putting pressure on the intestines, digestive issues can occur. 

Causes

Though the mechanisms of what causes a lymphangioma are understood, no one is positive about the underlying reason that a lymphangioma develops. Even if the symptoms of the lymphangioma do not appear for years, a lymphangioma develops during fetal development.

Instead of becoming connected to other vessels and circulating lymph fluid throughout the body, certain lymph sacs can be disconnected. Instead of properly moving fluid around, these disconnected areas build up fluid and white blood cells without being able to move them elsewhere. Eventually this causes a cyst to build up in the affected area.

Most of the time, there does not seem to be a recognizable cause for why the lymphatic system does not develop properly. Researchers believe that a pregnant woman using alcohol or suffering from a viral infection can possibly increase lymphangioma risk in the fetus, but the precise mechanism that alters lymphatic system development is unknown.

However, in some cases, there is a genetic factor to lymphangiomas. Patients with genetic disorders, such as Noonan syndrome, Turner syndrome, or Down syndrome, are 40 percent more likely to have a cystic hygroma.

Diagnosis & Tests

Because the symptoms of lymphangiomas are so distinctive, the diagnostic process is typically straightforward. In many cases, lymphangiomas can even be diagnosed before a woman gives birth. If the lymphangioma is large enough, it can show up on an ultrasound.

After conducting a physical examination, a doctor will generally suspect a lymphangioma if the patient has some sort of large, fleshy mass in one of the common lymphangioma locations.

The diagnosis can then be confirmed through a biopsy. The biopsy will require a doctor to remove some of the fluid from the lesion, and then it can be examined under a microscope to confirm the diagnosis.

If the lymphangioma is very deep under the skin, an ultrasound, magnetic resonance imaging (MRI) scan, or other imaging test can be used to determine the precise shape of the lymphangioma and see if it is affecting other organs.

Treatment & Therapy

Lymphangiomas do not harm the patient or carry an increased cancer risk, so treatment is not always necessary. However, even if the lymphangioma is not putting pressure on any part of the body, many patients choose to get it removed for cosmetic reasons.

Smaller lymphangiomas can quickly be treated with a noninvasive method called sclerotherapy. This procedure injects a sclerosing agent into the abnormal lymph tissue, and this causes them to shrink. Doxycycline, picibanil, and ethanol are all potential sclerosing agents. In many cases, the size of a larger lymphangioma can be decreased just by draining the buildup of lymph fluid, but this is just temporary.

To provide a more permanent treatment, doctors may recommend that the abnormal lymph tissue be removed through surgery. Surgery typically does not carry any substantial risks unless the lymphangioma is in a problematic position. However, it is possible for the lymphangioma to recur if the surgery does not remove all lymph tissues. 

Prevention & Prophylaxis