Lymphedema

Revision as of 03:29, 2 October 2017 by Stokunaga (talk | contribs) (Secondary)

Background

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

Etiology

Primary

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

Secondary

  • Cancer and cancer treatment (Lymphadenectomy, Radiotherapy)
    • Breast Cancer - most common
    • Sarcoma
    • Lower Extremity Melanoma
    • Gynecologic Cancer
    • Genitourinary Cancer
    • Head and neck cancer
  • Infection
    • Lymphatic Filariasis
    • Tuberculosis
    • Recurrent Skin Infections (eg. Cellulitis, Erysipelas)
    • Lymphadenitis
  • Obesity
  • Inflammatory disorders
    • Dermatitis
    • Sarcoidosis
    • Arthritis
      • Rheumatoid Arthritis
      • Psoriatic Arthritis
      • Juvenile Idiopathic Arthritis

Clinical Features

  • Slowly progressive swelling
  • Feeling of heaviness, tightness, or discomfort
  • May be pitting at onset
  • 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

  • Venous Insufficiency
  • Acute deep vein thrombosis
  • Post-thrombotic Syndrome
  • Hypoalbuminemia
  • Congestive Heart Failure
  • Limb Hypertrophy
    • Hypertrophy of soft tissue or bone (Klippel-Trenaunay syndrome)
    • Overgrowth of body part (Proteus Syndrome)
  • Myxedema
  • Lipedema
  • Tumor

Evaluation

  • Diagnosis is primarily made clinically. See clinical features above.
  • Additional studies
    • Duplex Ultrasound
    • Lymphoscintigraphy
    • Computed Tomography
    • Magnetic Resonance Imaging/Lymphography
    • Indocyanine Green (ICG) Lymphangiography
    • Genetic Testing

Clinical Staging (by International Society of Lymphology)

  • Stage 0 - Asymptomatic, swelling not evident despite impaired lymphatic transport. May have feeling of heaviness in limb.
  • Stage I (mild) - Soft edema +/- pitting, no sign of dermal fibrosis, fluid accumulation subsides with limb elevation within 24 hours.
  • Stage II (moderate) - Some dermal fibrosis present, not reversible with limb elevation alone.
  • Stage III (severe) - Lymphostatic elephantiasis, skin changes such as fat deposits, acanthosis, warty overgrowths.

Management

  • 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

See Also

External Links

References