Lymphedema

1. Epidemiology
   1. Incidence: Primary Lymphedema occurs 1 in 10,000
2. Types
   1. Primary Lymphedema
      1. Congenital Lymphedema
         1. Lymphedema Congenita
         2. Milroy's Disease (Hereditary)
      2. Pubertal onset of edema
         1. Lymphedema Praecox (Most common primary Lymphedema)
      3. Middle age onset of edema
         1. Lymphedema tarda (associated with injury)
   2. Secondary Lymphedema (acquired lymphatic obstruction)
      1. Tumor obstruction of Regional lymph nodes
         1. Prostate Cancer
         2. Lymphoma
      2. Surgical excision or radiation to regional nodes
         1. Breast Cancer with axillary node dissection
      3. Infection of regional lymph nodes
         1. Filariasis (Wuchereria bancrofti)
         2. Tuberculosis
3. Signs
   1. Early edema (protein-rich fluid accumulation)
      1. Soft "puffy" extremity swelling
      2. Maximal increase increase in girth in first year
      3. Easily pits
      4. Involves distal extremity (e.g. dorsal foot)
      5. Responds to limb elevation and compression
   2. Late edema (inflammatory fibrosis)
      1. Woody, firm Non-Pitting edema (Brawny edema)
      2. Skin thickened and hyperkeratotic
      3. Refractory to limb elevation and compression
4. Differential Diagnosis
   1. See Edema
   2. Chronic Venous Insufficiency
   3. Postphlebitic syndrome
   4. Myxedema (Hypothyroidism)
   5. Lipedema
      1. Seen in women with onset after Puberty
      2. Increased subcutaneous fat between Pelvis and ankle
   6. Malignant Lymphedema
      1. Rapid, painful cancer-related edema begins centrally
   7. Distinguishing characteristics of early edema
      1. Subcutaneous fibrosis (peau d'orange)
      2. Stemmer Sign (skin does not tent on dorsal digits)
      3. Preferential swelling of foot dorsum
      4. Involved extremity of squared-off digits
5. Complications
   1. Recurrent Bacterial Cellulitis (Gram Positive Bacteria)
   2. Refractory Late Edema (Non-Pitting edema)
6. Management: General
   1. Compression (Prevents late edema or Brawny edema)
      1. Contraindicated in limb Arterial Insufficiency
      2. Aggressive decongestive lymphatic therapy
      3. Elastic support garments (built-in pressure gradient)
         1. Replace every 3-6 months with loss of elasticity
      4. Mechanical Pneumatic Pressure Device
         1. Indicated for severe edema
         2. Applied at night or 2-3 times per week
      5. Special Massage Techniques
         1. Massage fluid proximally
   2. Limb Elevation
      1. Elevate above heart level for 30 minutes tid
   3. Good skin hygiene
      1. Keep web spaces between digits dry
      2. Apply Antifungal powder
   4. Avoid local injury or trauma
      1. Avoid walking barefoot (especially outdoors)
   5. Medications
      1. Benzopyrones (Not available in U.S)
      2. Topical coumarin (Not available in U.S.)
   6. Observe closely for Cellulitis
7. Management: Surgery
   1. Excision of hypertrophic fibrotic subcutaneous tissue
      1. Indicated for elephantiasis
      2. Types of procedures
         1. Charles Operation
         2. Kondoleon Procedure
   2. Lymphatic pedicle transfer
      1. Supplies alternative lymph drainage
      2. No proven efficacy
   3. Microvascular bypass of obstructed lymph channels
      1. Experimental
8. References
   1. Sabiston (1997) Surgery, Saunders, p.1574-7
   2. Rockson (2001) Am J Med 110:288-95