Lymphatic Malformations

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Lymphatic malformations are sometimes referred to as “lymphangiomas" and are composed of malformed, dilated channels filled with lymphatic fluid. The lymphatic system is an extension of the vascular (blood) system. Its function is to remove waste and excess fluid from the soft tissues of the body. Most lymphatic malformations are present at birth, or identified within the first two years of life. However, some may not be evident until adolescence or early adulthood.

Lymphatic Malformations Over Time

Lymphatic malformations tend to grow deep beneath the surface, usually between the overlying skin and the underlying musculature. They may cause asymmetry, distortion, and bony hypertrophy (overgrowth) in the areas where they occur. A lymphatic malformation of an extremity is frequently associated with lymphedema (swelling of the limb). When they occur closer to the skin, lymphatic malformations may manifest as vesicles (blisters) that are clear or bluish.

Lymphatic malformations do not shrink. In fact, they expand or contract depending on the flow of lymphatic fluid, the presence of inflammation, or bleeding inside the dilated channels. Infection, trauma, or bleeding in these channels can result in rapid growth of the lesion.

Treatment for Lymphatic Malformations

Lymphatic malformations can be "microcystic" (small cysts), "macrocystic" (large cysts), or a combination of both. As treatment for each type is different, the proper diagnosis is essential. Compression garments are often the first line of therapy to control filling of dilated lymphatic channels and swelling of extremities. Keeping the area of the lymphatic malformation clean is also extremely important, particularly for lesions involving the feet and legs. Keeping these areas clean and dry helps reduce the risk of skin breakdown and infection.

Sclerotherapy is the process of instilling a caustic material (usually a type of concentrated alcohol) into the dilated channels in an effort to cause scarring and collapse of the vessel walls. This is performed by the interventional radiologist under general anesthesia and is well-described for macrocystic lymphatic malformations. Often, the lesion will shrink in size, but will not completely disappear. Sometimes, multiple sessions are required to effectively treat an area of lymphatic malformation.

The only way to truly eradicate lymphatic malformations is to remove them surgically. However, surgery is not always possible or the preferred choice. Surgical excision is usually limited to symptomatic areas of the lesion and may even be dangerous in certain areas of the body where vital structures reside. It is also important to know that lymphatic channels can re-expand even after complete excision. This may result from residual lymphatic malformation despite a suspected complete excision.

Incisions in tissues overlying lymphatic malformations may be subject to postoperative healing problems, given the compromised vascularity. Infection, hematoma (blood collection), and prolonged fluid drainage (oozing from the wound) are frequently encountered. For lymphatic malformations involving the extremities, periodic monitoring as the child grows for changes in volume or circumference of the extremity in addition to length discrepancies. Once again, compression garments and physical therapy may be helpful to alleviate these symptoms.

If the lymphatic malformation has a superficial component on the surface of the skin (lymphangioma circumscriptum), this may be treated with dermabrasion (scraping) or excision and grafting (surgical removal of the affected skin with coverage using a partial thickness piece of skin from another part of the body).

Complications of Lymphatic Malformations

Sudden enlargement of a lymphatic malformation is likely the result of bleeding or an infection (cellulitis). If there is an infection involving an area that contains a lymphatic malformation, antibiotic therapy must be initiated. Cellulitis can be differentiated from bleeding most often by symptoms of redness and heat over the lymphatic malformation. If you suspect cellulitis, you should see your family doctor immediately. Bleeding into a lymphatic malformation can be painful. Administration of pain medication, rest, and time will often treat this problem effectively.