Lymph is a clear fluid that is carried through very small channels throughout the body (much like blood is carried through blood vessels). Blocking the flow of this fluid results in the build up of lymph in the surrounding tissues. It can occur any where on the body but commonly appears most notably in either the arms or the legs and is called lymphedema. It can be a primary or secondary disorder characterized by the accumulation of lymph in soft tissues, caused by inflammation, obstruction, or removal of lymph channels.
Lymphedema may be primary or secondary. The primary type can be present from birth (congenital lymphedema) or may occur during puberty mostly in women(lymphedema praecox), or less frequently later in life (lymphedema tarda).
Primary lymphedema is a result of missing lymphatic channels to carry the lymph. It occurs less often in men. The patient complains of swelling of the foot, leg, or entire extremity. On examination, the edema is diffuse, causes a typical mound on the dorsum (back part) of the foot or hand, and is only partially pitting (compressible). There are usually no skin changes in the acute period and the veins are not distended. When lymphedema is due to infection, the response to antistreptococcal antibiotics is rapid. If antibiotics are given for only a brief period (1 week), relapses are common. In chronic cases the swollen area can be very tender and the skin appears to be very thickened.
Secondary lymphedema is often a result of infection. In this setting, the onset is explosive, with chills, high fever, toxicity, and a red, hot, swollen leg. Red (Lymphangitic)streaks may be seen in the skin over a large area, and lymph nodes in the groin are usually enlarged and tender. These features may distinguish it from acute infection in the veins or thrombophlebitis (venous inflammation).
Secondary lymphedema in older persons may be due to a tumor or mass in the pelvis or groin. Obliteration of lymphatic tissue by excision or radiation therapy is another cause. In these cases, the swelling of the extremity may occur over a period of weeks or months.
Lymphedema is aggravated by prolonged standing, pregnancy, obesity, warm weather, and the menstrual period.
Lymphedema of the lower extremities begins with mild swelling of the foot and gradually extends to the entire limb. The edema is usually painless and initially may be pitting (compressible). Over time the skin over the edema becomes brawny and non-pitting. There may also be pain from the swelling. There is no evidence of ulceration or varicose veins.
The diagnosis is often made clinically. Lymphangiography using an injection into the lymphatic channels in the web spaces between the toes or fingers is rarely performed because the material used to outline the channels can cause more damage to the lymphatics and make the problem worse. Radioactive isotope studies (lymphosynctigraphy) utilize a radioactive isotope injection into a vein and carries far less risk to damaging the lymphatic channel and can provide the same information about the anatomy.
Swelling can be treated by elevation or periodic compression using a device composed of air bladders that intermittently squeeze the extremity, and then application of a firm support bandage or compression garment to be worn while the patient is up. More recently sequential massage by a trained therapist has been utilized with better results (Complex Decongestive Therapy (CDT). The affected area is massage in the direction of the lymphatic flow to encourage drainage. This is done daily and the patient or family member or care giver can be instructed in how to perform this once a standard response has been achieved. Once the limb is decreased in size, compression garments are again used to maintain the smaller size of the limb.
The foot of the bed at home can be elevated 15 to 20 degrees by placing pillows beneath the mattress. Occasionally, diuretics (“water pills”) may be prescribed but their routine use is somewhat controversial. Foot and skin hygiene is necessary to prevent infection and drying of the skin.
Treatment of other symptoms and signs is symptomatic and supportive. Patients report having fewer symptoms when they avoid Constricting clothing and salty or spicy foods.
Surgery is rarely performed to remove hypertrophied lymph channels or obstructed lymph channels and generally involves a resection of a large amount of skin and the tissue underneath. Surgery to reconnect lymph channels (anastomosis) has been attempted using a microscope and very fine suture or by placement of other lymph bearing tissue (such as the fat within the abdomen, or omentum) to be used as a wick to help drain the fluid from the extremity.
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