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For further information, see CMDT Part 14-19: Lymphedema

KEY FEATURES

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

General Considerations

  • Primary lymphedema

    • Due to congenital developmental abnormalities of lymphatics

    • Obstruction may be in the pelvic or lumbar lymph channels and nodes when the disease is extensive and progressive

  • Secondary lymphedema involves inflammatory or mechanical lymphatic obstruction due to

    • Trauma

    • Regional lymph node resection or irradiation

    • Extensive involvement of regional nodes by malignant disease or filariasis

  • Secondary dilation of the lymphatics occurs in both forms and leads to incompetence of the valve system, which

    • Disrupts the orderly flow along the lymph vessels

    • Results in progressive stasis of a protein-rich fluid

CLINICAL FINDINGS

Symptoms and Signs

  • Episodes of acute and chronic inflammation may be superimposed on the lymphedema

  • Hypertrophy of the limb

  • Markedly thickened and fibrotic skin and subcutaneous tissue

DIAGNOSIS

Imaging Studies

  • T2-weighted MRI 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

TREATMENT

Medications

  • No effective cure; treatment strategies are to control lymphedema and allow normal function

  • Antibiotic therapy (and periods of elevation) for secondary infection should cover Staphylococcus and Streptococcus organisms (Table 32–6)

  • Prophylactic antibiotics have not been shown to be of benefit

  • Diuretic therapy: intermittent courses are rarely helpful

Surgery

  • Amputation for the rare complication of lymphangiosarcoma

Therapeutic Procedures

  • Intermittent elevation of the extremity, especially during the sleeping hours (foot of bed elevated 15–20 degrees by placing pillows beneath the mattress)

  • Constant use of graduated elastic compression stockings

  • Massage toward the trunk, either manually or with pneumatic pressure devices

  • Good hygiene and treatment of any trichophytosis of toes to avoid secondary cellulitis

  • Wound care centers specializing in the care of lymphedema may be helpful

Table 32–6.Examples of empiric choices of antimicrobials for adult outpatient infections (listed in alphabetical order, except for syphilis).

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