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

• Lymphangioma of Mouth
• Lymphangioma of Oral Cavity

What is Oral Lymphangioma? (Definition/Background Information)

• Oral Lymphangioma is a benign tumor of the lymphatic system, which mostly occur in the oral cavity (mouth). A majority of the tumors are diagnosed before a child reaches 2 years of age
• Researchers believe that an Oral Lymphangioma occurs as an abnormality during embryological growth (while in the womb). The risk factors for the condition are generally unknown, but certain congenital disorders, such as Turner syndrome, Trisomy 13, Trisomy 18, and Noonan syndrome, are thought to influence its development
• Some tumors are known to affect speech and create eating difficulties in children. Many Oral Lymphangiomas also develop secondary infections that necessitate immediate treatment
• The healthcare provider may chose to monitor the growth of small-sized tumors, while large tumors that cause significant signs and symptoms may be treated or surgically removed
• Currently, there are no methods to prevent formation of Oral Lymphangiomas. However, the prognosis is generally good on surgical excision and a complete removal of the tumor

Who gets Oral Lymphangioma? (Age and Sex Distribution)

• A majority of the Oral Lymphangiomas, over 50% of them, are discovered (or diagnosed) at birth. By age 2 years, over 90% of these tumors are discovered
• Both males and females are affected
• Among all racial and ethnic groups, Oral Lymphangiomas are more common in Caucasians

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

In many individuals, no risk factors have been identified. However, in some, the risk factors for Oral Lymphangioma may include:

• Congenital disorders that include Turner syndrome, Down syndrome, and Noonan syndrome
• Chromosomal disorders such as Trisomy 13 (Patau’s syndrome) and Trisomy 18 (Edwards syndrome)
• Fetal alcohol syndrome
• 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 Oral Lymphangioma? (Etiology)

Oral Lymphangiomas are thought to develop due to obstruction of the lymphatic system, 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
• The obstruction may occur due to genetic factors and/or physiological abnormalities that develop in the fetuses

Many researchers believe that an Oral Lymphangioma is a malformation during the embryological development, rather than a true tumor.

What are the Signs and Symptoms of Oral Lymphangioma?

The signs and symptoms of Oral Lymphangioma include:

• The presence of a 1-2 cm sized mass in the mouth or on the lips
• The swollen lesions may have a smooth surface, or appear like a collection of bubbles
• Over 75% of the tumors occur in the oral cavity; the remaining occur in the head and neck region
• The most common site is the dorsum of tongue; however, the lymphangioma can also occur in the floor of the mouth and on the lips
• Most tumors may present no pain (painless tumors), but some tumors are known to bleed
• In children, the tumor can affect speech, chewing and swallowing of food

How is Oral Lymphangioma Diagnosed?

A diagnosis of Oral Lymphangioma may involve:

• Thorough physical exam with evaluation of complete medical history (including family history)
• Ultrasonography, angiographic studies of the affected region
• CT scan of the oral cavity
• Genetic tests and analysis
• Tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

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 Oral Lymphangioma?

The complications due to Oral Lymphangioma may include:

• Almost 80% of the tumors are known to develop secondary infections. When this happens, the tumor has to be aggressively treated, which may include hospitalization
• Once infected, it can become large in size causing difficulty in breathing; the affected babies may even require an emergency tracheostomy (tube insertion into the windpipe to restore breathing)
• Infection following a surgical procedure, due to which additional surgeries may be required
• Recurrence of the tumor following its (incomplete) removal
• Blockage of the food pipe or windpipe may take place due to large-sized tumors

Sometimes, severe bleeding can be fatal in infants.

How is Oral Lymphangioma Treated?

Oral Lymphangiomas are treated using the following methods:

• A ‘wait and watch’ approach may be adopted for small-sized tumors, while large-sized tumors may be surgically removed
• Sclerotherapy to inject medications into the tumor may cause them to collapse. If this is successful, then a surgery can be avoided
• Laser surgical excision
• Surgical removal of the lymphangioma with reconstructive surgery (if required). The surgery may be performed in stages and can result in a cure
• In case of complications, such as superimposed infections, urgent treatment and care with hospitalization of the child may be necessary
• In case of speech defects, the assistance/consultation of a speech pathologist may be necessary

Follow-up care with regular screening and check-ups are important, to watch-out for any recurrences.

How can Oral Lymphangioma be Prevented?

Current medical research has not established a way of preventing Oral Lymphangiomas.

• Maintain proper oral hygiene to prevent secondary infections from aggravating the condition
• Genetic counseling and genetic testing can help those individuals having a family medical history or any of the risk factors, and planning for a child
• Regular medical screening during pregnancy for women who are at risk

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

• The prognosis of Oral Lymphangioma is generally very good with suitable treatment, if no additional complications or surgical infections develop
• Some tumors are known to disappear on their own
• Underlying genetic disorders or syndromes may adversely affect the prognosis

Additional and Relevant Useful Information for Oral 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.