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For further information, see CMDT Part 12-18: 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

  • No 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 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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Chen  K  et al. Surgical management of postmastectomy lymphedema and review of the literature. Ann Plast Surg. 2021;86:S173.
[PubMed: 33346539]  
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Finnane  A  et al. Review of the evidence of lymphedema treatment effect. Am J Phys Med Rehabil. 2015;94:483.
[PubMed: 25741621]  
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Schaverien  MV  et al. New and emerging treatments for lymphedema. Semin Plast Surg. 2018;32:48.
[PubMed: 29636654]  

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