What are the other Names for this Condition? (Also known as/Synonyms)

• Cystic Hygroma

What is Lymphangioma? (Definition/Background Information)

• A Lymphangioma is a benign type of tumor that develops spontaneously, due to certain abnormalities in the lymphatic system. The tumor can occur either before birth (congenital, due to genetic factors), or shortly after birth (due to physiological factors)
• There are two types of Lymphangioma tumors:
  • Cystic Lymphangioma: Occurs around the head, neck, and groin region
  • Cavernous Lymphangioma: Occurs around the head, neck, and groin region, and additionally, are found inside the mouth, torso, limbs, and abdomen
• Lymphangiomas are rare and appear as painless, swollen, fluid-filled masses that are not malignant. However, depending on their location, they can cause breathing problems by choking the windpipe, or obstruct the intestines
• Currently, there are no methods to prevent formation of such tumors. However, the prognosis of Lymphangioma is generally good on a surgical excision and removal of the complete tumor

Who gets Lymphangioma? (Age and Sex Distribution)

• A Lymphangioma usually forms before the birth of the child (during the fetal stage), or within two years of birth. However, these tumors can form at any age
• Both males and females may be affected by this tumor condition
• There is no specific information on any racial or ethnic preference

What are the Risk Factors for Lymphangioma? (Predisposing Factors)

The risk factors of Lymphangioma include:

• Genetic disorders, like Turner syndrome, Down syndrome, and Noonan syndrome, are associated with Lymphangiomas
• Any of the Trisomies, such as Trisomy 13 (Patau’s syndrome), Trisomy 18 (Edwards syndrome), and Trisomy 21 (Down syndrome), can increase the risk
• Alcohol abuse
• A viral infection during pregnancy

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Lymphangioma? (Etiology)

• A Lymphangioma tumor develops when the lymphatic system gets obstructed due to various reasons, as the fetus grows
• The obstruction causes fluid to fill-up within the lymphatic channels, which then dilate. The lesion continues to grow after the baby is born to reach great sizes
• The reason for the obstruction of the lymphatic channels may be due to genetic factors, and/or physiological abnormalities that develop in the fetuses, due to various conditions

What are the Signs and Symptoms of Lymphangioma?

The signs and symptoms of Lymphangioma include:

• The typical sign of Lymphangioma is a very prominent, red-to-pink colored, bulging fluid-filled mass of tissue, in the neck or head. This is the most common location of occurrence of the tumor
• The swollen lesions may have a smooth surface, or appear like a collection of bubbles
• There may be an absence of pain at the location, since most tumors are painless

How is Lymphangioma Diagnosed?

A diagnosis of Lymphangioma may involve:

• Through physical exam with evaluation of complete family medical history
• Ultrasonography, angiographic studies of affected region
• Tissue biopsy for Lymphangioma: Amniocentesis or chorionic villus sampling may be performed during pregnancy
• CT scan of the affected region
• Genetic tests and analysis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Lymphangioma?

The possible complications due to Lymphangioma include:

• Infection during the surgical intervention, which may necessitate multiple surgeries
• Growth of the tumor after surgery, due to its incomplete removal
• Adjoining structures, tissues, nerves, and organs, can be damaged during the surgery
• Lymphangioma formations during early stages of pregnancy can complicate the pregnancy and affect normal fetal growth
• Blockage of the food pipe (tracheal or bronchial obstruction), windpipe (esophageal obstruction), or intestine (intestinal obstruction), by the overgrown mass

How is Lymphangioma Treated?

Following are the treatment methods for Lymphangioma:

• Surgical removal of the tissue mass, with reconstructive surgery, if the tumor is located on the face or neck region. The surgery may be performed in stages and is the most effective method for a complete cure
• Combination treatment using chemotherapy and radiation therapy, if the tumor is recurrent, or if surgical treatment is not possible
• Use of steroids and sclerosing injections into the tumor
• Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
• Follow-up care with regular screening and check-ups are important, to watch for any recurrences

How can Lymphangioma be Prevented?

• Current medical research has not established a way of preventing Lymphangiomas
• Genetic counseling and genetic testing could help those individuals having a family medical history of any of the risk factors, planning for a child
• Regular medical screening during pregnancy for individuals with a higher risk

What is the Prognosis of Lymphangioma? (Outcomes/Resolutions)

• Prognosis is generally very good, if no additional complications or any surgical infections develop
• If genetic disorders are present and the lesion develops during the early stages of pregnancy, then the outcome cannot be predicted
• A late development of Lymphangioma during pregnancy, has a better chance at being corrected, after the child is born
• Some tumors resolve spontaneously by themselves, within the gestational period (normally by week 18-20)

Additional and Relevant Useful Information for Lymphangioma:

Mild Lymphangiomas may be treated by aspiration drainage of the fluid. However, this is not a permanent solution and invasive procedures may still have to be performed.