II. Epidemiology

  1. Incidence: Primary Lymphedema occurs 1 in 10,000

III. Pathophysiology

  1. Lymphatic System typically is responsible for 10-20% fluid return (remaining 80-90% is venous return)
  2. Lymphedema is accumulation of Protein-rich fluid in extracellular space

IV. 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

V. 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. Responds to limb elevation and compression
    5. Involves distal extremity (e.g. dorsal foot)
      1. See Stemmer's Sign (pathognomonic for Lymphedema)
  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

VI. Differential Diagnosis: Acute Conditions critical to exclude (e.g. emergency department)

VII. Differential Diagnosis: Subacute and Chronic Conditions

  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

VIII. Complications

  1. Recurrent Bacterial Cellulitis (Gram Positive Bacteria)
  2. Refractory Late Edema (Non-Pitting Edema)
  3. Pain and decreased extremity range of motion
  4. Severe Lymphedema (acute swelling >80%)
    1. Requires hospitalization and possible surgical intervention
  5. Lymphagiosarcoma (Stewart-Treves Syndrome)
    1. Upper extremity soft tissue malignancy complicating chronic upper extremity edema
    2. Rare complication of Breast Cancer Management with Lymph Node dissection (<0.45% of cases)
    3. Presents as arm blue-purple Macule or Papule (or as bulla or Cellulitis)
    4. Biopsy suspected lesions

IX. Management: General

  1. Indicated in aggressive decongestive lymphatic therapy
  2. Compression (Prevents late edema or Brawny Edema)
    1. Precautions
      1. Contraindicated in limb Arterial Insufficiency (except for low resting pressure wraps)
      2. Confirm adequate padding
      3. Observe for friction sites (risk of open sores)
    2. Wraps (active edema reduction)
      1. High resting pressure (ACE Wrap, Tubigrip)
        1. Absolutely contraindicated in Peripheral Vascular Disease
      2. Mult-layer compression
        1. Multiple-layer: Cotton layer, ace wrap, firm wrap)
        2. Most effective compression for Wound Healing
        3. Combines high and low resting pressure
      3. Low resting pressure (e.g. Rosidahl, Lymphedema wrap)
        1. These wraps do not require a resting force (rely on Muscle movement for return)
        2. Ineffective, if calf Muscle is not functional to assist return (use high resting pressure instead)
        3. Typically safe to use in Peripheral Arterial Disease
    3. Elastic Support Garments or Compression Stockings (built-in pressure gradient)
      1. Used for maintenance only (not for acute, active edema reduction)
      2. May initiate Compression Stockings 40 mmHg or greater after edema improves with wraps
      3. 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 (performed by Lymphedema specialists)
      1. Massage fluid proximally
      2. Ezzo (2015) Cochrane Database Syst Rev (5):CD003475 +PMID: 25994425 [PubMed]
  3. Limb Elevation
    1. Elevate above heart level for 30 minutes three times daily
  4. Exercise
    1. Promotes surrounding Muscle activity to promote lymphatic flow and fluid return
  5. Good skin hygiene (prevent infection)
    1. Keep web spaces between digits dry
    2. Apply Antifungal powder
    3. Use Skin Lubricants (Moisturizers)
  6. Avoid local injury or Trauma
    1. Avoid walking barefoot (especially outdoors)
  7. Medications
    1. Benzopyrones (Not available in U.S)
    2. Topical coumarin (Not available in U.S.)
    3. Avoid Diuretics (minimal to no effect)
  8. Observe closely for Cellulitis

X. 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 Lymphovenous bypass of obstructed lymph channels
    1. Reduces limb circumference >1.9 cm
    2. Fallahian (2022) Ann Plast Surg 88(2):195-9 +PMID: 34398594 [PubMed]

XI. Resources

  1. National Lymphedema Network Position Papers
    1. http://www.lymphnet.org/lymphedemaFAQs/positionPapers.htm

XII. References

  1. Novotny (2017) Wound Care Update, Park Nicollet Conference, St Louis Park, MN (attended 9/15/2017)
  2. Shelby (2015) Crit Dec Emerg Med 29(6): 2-8
  3. Sabiston (1997) Surgery, Saunders, p.1574-7
  4. Grada (2017) J Am Acad Dermatol 77(6):995-1006 +PMID: 29132859 [PubMed]
  5. Rockson (2001) Am J Med 110:288-95 [PubMed]

Images: Related links to external sites (from Bing)

Related Studies