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

• Hypertrophy of the limb

• Markedly thickened and fibrotic skin and subcutaneous tissue

Diagnosis

Imaging Studies

• Lymphangiography and radioactive isotope studies may identify focal defects in lymph flow but are of little value in planning therapy

• T₂-weighted MRI has been used to identify lymphatics and proximal obstructing masses

Treatment

Medications

• No effective cure

• Antibiotic therapy for secondary infection should cover Staphylococcus and Streptococcus organisms

• 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)

• Constant use of graduated elastic compression stockings

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

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

Outcome

Complications

• Secondary infection

Prognosis

• Dictated by associated conditions and avoidance of recurrent cellulitis

• Good with aggressive treatment

References