Lymphedema

Revision as of 19:47, 26 March 2019 by Rossdonaldson1 (talk | contribs) (Clinical Staging (by International Society of Lymphology) International Society of Lymphology. The diagnosis and treatment of peripheral lymphedema: 2013 Consensus Document of the International Society of Lymphology. Lymphology 2013; 46:1-11)

Background

  • Lymphedema is swelling due to abnormal accumulation of interstitial protein rich fluid and fibroadipose tissue.
  • Occurs when lymphatic load exceeds capacity in the lymphatic system

Etiology

Primary[1]

  • Congenital Lymphedema (6-12%) - before age 2
  • Lymphedema Precox (77-94%) - at onset of puberty
  • Lymphedema Tarda (11%) - after age 35

Secondary

Clinical Features

  • Slowly progressive swelling, may be pitting at onset
  • Feeling of heaviness, tightness, or discomfort
  • Severe cases have dermal thickening with skin becoming dry, firm, and hyperkeratotic
    • Occurs due to cutaneous fibrosis and adipose deposition.
  • Stemmer Sign - positive if unable to pinch and lift skin at the base of second toe or finger.

Differential Diagnosis

Unilateral leg swelling

Differential Diagnosis of Pedal Edema

Evaluation

  • Diagnosis is primarily made clinically. See clinical features above.
  • Rule out alternative diagnoses (e.g. duplexes to rule out DVT if indicated)
  • Additional non-ED studies may include:
    • Duplex Ultrasound
    • Lymphoscintigraphy
    • Computed Tomography
    • Magnetic Resonance Imaging/Lymphography
    • Indocyanine Green (ICG) Lymphangiography
    • Genetic Testing

Clinical Staging (by International Society of Lymphology) [3]

Stage Signs & Symptoms Image
Stage 0 (asymptmatic)
  • Generally asymptomatic, although may have feeling of heaviness in limb
  • Swelling not evident on exam
Stage I (mild)
  • Soft edema +/- pitting
  • fluid accumulation subsides with limb elevation within 24 hours.
  • no sign of dermal fibrosis
Stage II (moderate)
  • Pitting present
  • Some dermal fibrosis present
  • Not reversible with limb elevation alone
Stage III (severe)
  • Lymphostatic elephantiasis (pitting may be absent)
  • Skin changes such as fat deposits, acanthosis, warty overgrowths

Management[4][3]

Example of compression therapy: lymphedema compression sleeve on mannequin
  • General Measures
    • Self-monitoring - for size, sensation, color, temperature, skin condition
    • Limb elevation
    • Diet and exercise - maintain ideal body weight. Recommended to use compression garments during exercise.
    • Avoid skin infection/injury
  • Compression Therapy - bandaging, garments, intermittent pneumatic compression
  • Physiotherapy - manual lymphatic drainage
    • Contraindicated in presence of cellulitis, neoplasm, DVT, moderate-severe heart failure
  • Surgical Referral

Disposition

  • Discharge if uncomplicated

See Also

External Links

References

  1. Szuba A, Rockson SG. Lymphedema: classification, diagnosis and therapy. Vasc Med 1998; 3:145-56
  2. Cormier JN, Askew RL, Mungovan KS, et al. Lymphedema beyond breast cancer: a systematic review and meta-analysis of cancer-related secondary lymphedema. Cancer 2010; 116:5138-49
  3. 3.0 3.1 International Society of Lymphology. The diagnosis and treatment of peripheral lymphedema: 2013 Consensus Document of the International Society of Lymphology. Lymphology 2013; 46:1-11
  4. Rockson SG. Lymphedema. Am J Med 2001; 110:288-95