ESSENTIALS OF DIAGNOSIS
Painless persistent edema of one or both lower extremities, primarily in young women.
Pitting edema without ulceration, varicosities, or stasis pigmentation.
Lymphangitis and cellulitis may occur.
The primary form of lymphedema is caused by congenital hypo- or hyperplastic proximal or distal lymphatics. The obstruction may be in the pelvic or lumbar lymph channels and nodes when the disease is extensive and progressive. The secondary form of lymphedema involves inflammatory or mechanical lymphatic obstruction from trauma, regional lymph node resection or irradiation, or extensive involvement of regional nodes by malignant disease or filariasis. Lymphedema may occur following surgical removal of the lymph nodes in the groin or axillae. Secondary dilation of the lymphatics that occurs in both forms leads to incompetence of the valve system, disrupts the orderly flow along the lymph vessels, and results in progressive stasis of a protein-rich fluid. Episodes of acute and chronic inflammation may be superimposed, with further stasis and secondary fibrosis.
Hypertrophy of the limb results, with markedly thickened and fibrotic skin and subcutaneous tissue (Figure 12–4) in very advanced cases (eFigures 12–18 and 12–19).
Lymphedema with a dorsal pedal hump and exaggerated skin folds near the ankle. (Used, with permission, from Dean SM, Satiani B, Abraham WT. Color Atlas and Synopsis of Vascular Diseases. McGraw-Hill, 2014.)
Lymphedema of entire left leg. (Reproduced, with permission, from Orkin M, Maibach HI, Dahl MV [editors]. Dermatology. Originally published by Appleton & Lange. Copyright © 1991 by The McGraw-Hill Companies, Inc.)
Mossy foot with lymphatic excrescences. (Reproduced, with permission, from Orkin M, Maibach HI, Dahl MV [editors]. Dermatology. Originally published by Appleton & Lange. Copyright © 1991 by The McGraw-Hill Companies, Inc.)
T2-weighted MRI has been used to identify lymphatics and proximal obstructing masses. Lymphangiography and radioactive isotope studies may identify focal defects in lymph flow but are of little value in planning therapy.
There is no effective cure for lymphedema; treatment strategies can control the lymphedema and allow normal function. Most patients can be treated with some of the following measures: (1) Aid the flow of lymph out of the extremity through intermittent elevation, especially during the sleeping hours (foot of bed elevated 15–20 degrees, achieved by placing pillows beneath the mattress); constant use of graduated elastic compression stockings; and massage toward the trunk—either by hand or by means of pneumatic pressure devices designed to milk edema out of an extremity. Wound care centers specializing in the care ...