CVL malformations are deep vascular malformations, characterized by soft compressible deep-tissue swelling, which, at times, is associated with varicosities, arteriovenous shunts, and nevus flammeus-like changes.
Causes
Although the exact cause of lymphatic malformations is unknown, they are thought to be caused by errors in the formation and development of lymphatics during fetal development. The cause is not related to any known drug or medication that may have been taken during pregnancy or to any environmental exposure that may have occurred during that time.
Symptoms
An internal lymphatic malformation in the mouth or cheek may not be noticed until it becomes infected and enlarges. Cystic hygromas can grow very large and may affect breathing and swallowing. The following are the most common symptoms of a lymphatic malformation. However, each child may experience symptoms differently.
Cervicofacial lymphatic malformations (lymphatic birthmarks) may be associated with the overgrowth of the mandibular body. Lymphatic malformations of the floor of the mouth and tongue are usually characterized by vesicles, swelling, and bleeding
Diagnosis
Clinical diagnosis, at times confirmed by arteriography.
Treatment
There is no satisfactory treatment except compression. In larger lesions-if organ function is compromised-surgical procedures, intravascular coagulation. High-dose systemic glucocorticoids, interferon-α, may be effective.
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