Difference between revisions of "Lymphedema"

(Created page with "==Background== * Lymphedema is due to abnormal accumulation of interstitial fluid and fibroadipose tissue. * Occurs with lymphatic load exceeds capacity in the lymphatic syste...")
 
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* Feeling of heaviness, tightness, or discomfort
 
* Feeling of heaviness, tightness, or discomfort
 
* May be pitting at onset
 
* May be pitting at onset
* Dermal thickening with skin becoming dry, firm, and hyperkeratotic  
+
* Severe cases have dermal thickening with skin becoming dry, firm, and hyperkeratotic  
 
** Occurs due to cutaneous fibrosis and adipose deposition.
 
** 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.
 
* Stemmer Sign - positive if unable to pinch and lift skin at the base of second toe or finger.
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* Acute deep vein thrombosis
 
* Acute deep vein thrombosis
 
* Post-thrombotic Syndrome
 
* Post-thrombotic Syndrome
 +
* Hypoalbuminemia
 +
* Congestive Heart Failure
 
* Limb Hypertrophy
 
* Limb Hypertrophy
 
** Hypertrophy of soft tissue or bone (Klippel-Trenaunay syndrome)  
 
** Hypertrophy of soft tissue or bone (Klippel-Trenaunay syndrome)  
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==Evaluation==
 
==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==
 
==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==
 
==Disposition==

Revision as of 03:22, 2 October 2017

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
    • Dermatitis

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