Definition

Lymphangioma is a benign fluid-filled cyst that occurs in the body's lymphatic vessels. Lymphatic vessels work together to form a system that regulates body tissue fluid. Lymph nodes also work to help the body's defense system fight infection.

The estimated case of lymphangioma is around 1 in 4000 births. Lymphangiomas can occur anywhere, either in the skin or mucous membranes and around 75% of cases occur in the head or neck area. About 50% of cases of lymphangioma in the head or neck are discovered at birth, and 90% of cases are found by the age of 2 years.

Causes

The exact cause of lymphangioma cases is still unknown. Congenital abnormalities of the lymphatic system during fetal development are thought to be the cause of lymphangioma. The blockage of the lymphatic system in the womb causes congenital lymphangiomas found at birth. In addition, lymphangiomas are also often associated with certain genetic disorders such as Trisomy 13, 18, and 21, and certain syndromes such as Down syndrome.

Risk factor

There are no currently known risk factors for lymphangioma, and no particular race is more prevalent in it. However, based on current evidence, the most frequent incidence of lymphangioma cases is found at birth or early in life.

Symptoms

There may be one or many localized lymphangiomas in a particular area in the body. Symptoms may vary depending on the size and location of the lymphangioma. Lymphangiomas may appear as small red or bluish dots. If lymph fluid accumulates, swelling and masses may occur in cases of lymphangioma.

If lymphangioma occurs in important organs or body tissues, the symptoms can be significant and reduce quality of life. If the lymphangioma is on the tongue, there may be difficulty eating and speaking symptoms. Double vision could manifest if the lymphangioma occurs in the eye socket area. Chest pain and breathing difficulty may occur if lymphangioma appears in the chest area.

Diagnosis

The diagnosis of lymphangioma can be established in pregnancy by performing ultrasound (USG) examination to help find any visible abnormalities. If the doctor notices an abnormality after birth, they will usually order other tests, such as an MRI, CT scan, or ultrasound, to confirm the diagnosis and examine its size and impact. If the lymphangioma is not visible at birth, it can sometimes be apparent when the child is 2 years old.

However, in addition to diagnostic tests, the doctor will first conduct a medical interview and physical examination to confirm the diagnosis of lymphangioma. In this interview, the doctor will inquire about the symptoms and timing of the lymphangioma.

A physical examination will help doctor to assess the condition of the lymphangioma in more detail. Lymphangiomas may have red to purple vesicle-like papules or blood-filled blisters on the skin surface. In addition, there may also be swelling due to obstruction of the lymphatic system due to congenital lymphatic disorders, especially in the neck area.

If lymphangioma is thought to exist elsewhere in the body, physical examination can also help to further investigate your symptoms. The doctor will ask you to lie on your back.

Other diagnostic tests, such as a complete blood count, may be considered to determine if the lymphangioma is caused by infections.

Management

Generally, no specific treatment can be used to manage lymphangioma. Doctors tend not to recommend any treatment if there are no symptoms or the symptoms are mild. If the lymphangioma is large, uncomfortable, or reduces quality of life, doctors will suggest several treatments.

The planned management will depend on the type, size, and location of the lymphangioma. Some treatment options include surgery, sclerotherapy, laser therapy, or radiofrequency ablation.

Surgery will remove the lymphangioma cyst but if the mass is located close to organs and nerves, it can be difficult to remove and need to be treated carefully.

Sclerotherapy is the process of injecting certain chemicals directly into the mass with the hope that the mass will shrink and collapse. This treatment is more commonly used and has a similar success rate to surgical treatment but with fewer complications.

Laser therapy, or radiofrequency ablation, is a treatment method that uses a laser and needle to deliver heat and destroy the mass.

Although there are several treatments for lymphangioma, it is almost impossible to remove or destroy the whole cells of lymphangioma and lymphangiomas tend to reappear in the same area. Therefore, it sometimes takes a longer period of time and repeated surgeries or treatments to completely remove the lymphangioma.

The recurrence rate of lymphangioma cases varies depending on the type and location of the lymphangioma.

Complications

A complication that can arise in cases of lymphangioma is lymphangioma circumscriptum. Lymphangioma circumscriptum is associated with minimal bleeding, recurrent cellulitis (skin redness and inflammation with clear margins) and lymph node discharge. Two cases of lymphangiosarcoma originating from lymphangioma circumscriptum were reported. However, in these cases, it was found that there had been prolonged exposure to X-ray treatment on the existing lymphangiomas so radiation therapy should be avoided in patients with lymphangiomas.

Other lymphangiomas can cause feeding difficulties, breathing problems, and serious infections if they occur in the neck area. If they occur on the scrotum (the sac of skin covering the testicles), complications such as scrotal pain and bleeding may occur, although they are rare.

Prevention

The prevention of lymphangioma is currently unknown. However, there are steps that can be taken to help prevent complications that may occur in cases of lymphangioma. Here are the steps:

• Early recognition of lymphangioma that appears on the surface of the skin or other organs by consulting a doctor.
• Perform routine medical check-ups, especially for children who are at risk of having lymphangioma, such as patients with Down syndrome.
• Pregnant women should receive an ultrasound examination from a doctor to find out if there are abnormalities seen in the child being conceived.

When to see a doctor?

If you experience continuous and persistent symptoms and signs, accompanied by progressive symptoms, you should see a surgeon or dermatologist. The doctor will conduct a medical interview, physical examination, and certain diagnostic tests to determine the exact diagnosis and appropriate management for you.

Looking for more information about other diseases? Click here!