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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

  • No ulceration, varicosities, or stasis pigmentation

  • Episodes of lymphangitis and cellulitis

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 can be helpful, especially in those with premenstrual or seasonal exacerbations

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

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Finnane  A,  et al. Review of the evidence of lymphedema treatment effect. Am J Phys Med Rehabil. 2015 Jun;94(6):483–98.
[PubMed: 25741621]  
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Haghighat  S,  et al. Comparing two treatment methods for post mastectomy lymphedema: complex decongestive therapy alone and in combination with intermittent pneumatic compression. Lymphology. 2010 Mar;43(1):25–33.
[PubMed: 20552817]  
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Torres Lacomba  M,  et al. Effectiveness of early physiotherapy to prevent lymphoedema after surgery for breast cancer: randomised, single blinded, clinical trial. BMJ. 2010 Jan 12;340:b5396.
[PubMed: 20068255]  

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