Lymphangioma refers to a rare, congenital condition that usually does not pose a health threat unless it occurs in a fetus (fetal cystic hygroma).
Lymphangiomas occur when a blockage in the lymphic system leads to fluid accumulation, and they typically appear as a fluid-filled, yellowish or reddish bubble or series of bubbles beneath the skin.
This condition may affect people of all ages, but the vast majority of cases are diagnosed before age five. Lymphangiomas may affect any part of the body, but they are most commonly seen on the neck or groin.
Since they have no chance of becoming malignant, lymphangiomas are usually treated for cosmetic reasons only. In cases where they emerge early in the process of fetal development, however, cystic lymphangiomas can result in fetal death due to excess fluid accumulation.
Signs and Symptoms
While all lymphangiomas involve a malformation of the lymphic system, they come in three distinct types, each of which has a slightly different appearance and different implications.
Lymphangioma circumscriptum consists of one or a cluster of translucent, bubble-like structures of varying sizes on the surface of the skin.
They are typically pink to dark red in color. While lymphangioma circumscriptum can be found on skin’s surface, the other two types of lymphangiomas occur deeper under the skin.
Cavernous lymphanioma and cystic lymphangioma (also called cystic hygroma, familial nuchal bleb, fetal cystic hygroma (FCH), or hygroma colli) do not affect the skinís surface like lymphangioma circumscriptum does, but instead result in a bulge or thickening of the skin. Cavernous lymphaniomas vary widely in size, ranging from as small as a centimeter in diameter to several centimeters wide.
In some cases, they may even affect an entire extremity such as a hand or foot. Although they are usually painless, the patient may feel mild pain when pressure is exerted on the area.
Cystic lymphangiomas share many commonalities with cavernous lymphaniomas, and some doctors consider them to be too similar to merit separate categories. However, cystic lymphangiomas usually have a softer consistency than cavernous lymphaniomas, and this term is typically the one that is applied to lymphangiomas that develop in fetuses.
Learn more about the types and symptoms of Lymphangioma.
Causes and Risk Factors
The direct cause of lymphangioma is a blockage of the lymphatic system as a fetus develops, although symptoms may not become visible until after the baby is born. This blockage, in turn, is thought to be caused by a number of factors, including maternal alcohol use and viral infections during pregnancy.
Cases of cystic lymphangioma that emerge during the first two trimesters of pregnancy may be associated with genetic disorders like Turnerís syndrome, Noonan syndrome, and chromosome abnormalities such as trisomies 13, 18, and 21.
The prognosis for cases of lymphangioma circumscriptum and cavernous lymphangioma is generally excellent. There is a minor risk of infection, but this can be treated easily, and the tumor can be removed at any time so long as no infection is present.
The prognosis is also good for fetuses that are diagnosed with cystic lymphangioma late in the pregnancy. However, this condition is more dangerous when it develops early in a pregnancy, and is especially worrisome when a genetic disorder is present. When prenatal cases of cystic lymphangioma resolve themselves quickly (by 18-20 weeks of gestation), the prognosis is still moderate to good, but cases that are not resolved by this stage have a poor prognosis.
Diagnosis and Treatment
Cases of lymphangioma are diagnosed by inspection. In prenatal cases, cystic lymphangioma is diagnosed using ultrasound technology. When fetal cystic lymphangioma is diagnosed, amniocentesis or CVS procedures may be conducted to check for associated genetic disorders. Draining lymphangiomas of fluid provides only temporary relief, so they are typically treated using surgical resection.