Lymphangiomas are malformations of the lymphatic system characterized by lesions that are thin-walled cysts; these cysts can be macroscopic, as in a cystic hygroma, or microscopic. The lymphatic system is the network of vessels responsible for returning to the venous system excess fluid from tissues as well as the lymph nodes that filter this fluid for signs of pathogens. These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and involve the head and neck. These malformations are either congenital or acquired. Congenital lymphangiomas are often associated with chromosomal abnormalities such as Turner syndrome, although they can also exist in isolation. Lymphangiomas are commonly diagnosed before birth using fetal ultrasonography. Acquired lymphangiomas may result from trauma, inflammation, or lymphatic obstruction. Most lymphangiomas are benign lesions that result only in a soft, slow-growing, "doughy" mass. Since they have no chance of becoming malignant, lymphangiomas are usually treated for cosmetic reasons only. Rarely, impingement upon critical organs may result in complications, such as respiratory distress when a lymphangioma compresses the airway. Treatment includes aspiration, surgical excision, laser and radiofrequency ablation, and sclerotherapy. Signs and symptoms
There are three distinct types of lymphangioma, each with their own symptoms. They are distinguished by the depth and the size of abnormal lymph vessels, but all involve a malformation of the lymphic system. Lymphangioma circumscriptum can be found on the skins surface, and the other two types of lymphangiomas occur deeper under the skin.
Cavernous lymphangiomas
Composed of dilated lymphatic channels, cavernous lymphangiomas characteristically invade surrounding tissues. Cystic hygromas
Cystic hygromas are large, macrocystic lymphangiomas filled with straw-colored, protein-rich fluid. Hemangiolymphangioma
As suggested by their name, hemangiolymphangiomas are lymphangiomas with a vascular component.Lymphangiomas may also be classified into microcystic, macrocystic, and mixed subtypes, according to the size of their cysts. Microcystic lymphangiomas
Microcystic lymphangiomas are composed of cysts, each of which measures less than 2 cm3 in volume. Macrocystic lymphangiomas
Macrocystic lymphangiomas contain cysts measuring more than 2 cm3 in volume. Mixed lymphangiomas
Lymphangiomas of the mixed type contain both microcystic and macrocystic components.Finally, lymphangiomas may be described in stages, which vary by location and extent of disease. In particular, stage depends on whether lymphangiomas are present above or superior to the hyoid bone (suprahyoid), below or inferior to the hyoid bone (infrahyoid), and whether the lymphangiomas are on one side of the body (unilateral) or both (bilateral). Stage I
Unilateral infrahyoid. Stage II
Unilateral suprahyoid. Stage III
Unilateral suprahyoid and infrahyoid. Stage IV
Bilateral suprahyoid. Stage V
Bilateral suprahyoid and infrahyoid. Treatment
Treatment for cystic hygroma involves the removal of the abnormal tissue; however complete removal may be impossible without removing other normal areas. Surgical removal of the tumor is the typical treatment provided, with the understanding that additional removal procedures will most likely be required as the lymphangioma grows. Most patients need at least two procedures done for the removal process to be achieved. Recurrence is possible but unlikely for those lesions able to be removed completely via excisional surgery.