Difference between revisions of "Lymphedema"

(Background)
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==Background==
 
==Background==
* Lymphedema is due to abnormal accumulation of interstitial protein rich fluid and fibroadipose tissue.
+
* 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
 
* Occurs when lymphatic load exceeds capacity in the lymphatic system
  
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** Head and neck cancer
 
** Head and neck cancer
 
* Infection
 
* Infection
** Lymphatic Filariasis
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** [[Lymphatic Filariasis]]
** Tuberculosis
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** [[Tuberculosis]]
** Recurrent Skin Infections (eg. Cellulitis, Erysipelas)
+
** Recurrent Skin Infections (eg. [[cellulitis]], [[erysipelas]])
** Lymphadenitis
+
** [[Lymphadenitis
* Obesity
+
* Obesity]]
 
* Inflammatory disorders
 
* Inflammatory disorders
 
** Dermatitis
 
** Dermatitis
** Sarcoidosis
+
** [[Sarcoidosis]]
** Arthritis
+
** [[Arthritis]]
*** Rheumatoid Arthritis
+
*** [[Rheumatoid arthritis]]
*** Psoriatic Arthritis
+
*** [[Psoriatic arthritis]]
 
*** Juvenile Idiopathic Arthritis
 
*** Juvenile Idiopathic Arthritis
  
 
==Clinical Features==
 
==Clinical Features==
* Slowly progressive swelling  
+
* Slowly progressive swelling, may be pitting at onset
 
* Feeling of heaviness, tightness, or discomfort
 
* Feeling of heaviness, tightness, or discomfort
* May be pitting at onset
 
 
* Severe cases have 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.
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==Differential Diagnosis==
 
==Differential Diagnosis==
 
* Venous Insufficiency
 
* Venous Insufficiency
* Acute deep vein thrombosis
+
* [[DVT]]
 
* Post-thrombotic Syndrome
 
* Post-thrombotic Syndrome
 
* Hypoalbuminemia
 
* Hypoalbuminemia
* Congestive Heart Failure
+
* [[CHF]]
 
* 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.
 
* Diagnosis is primarily made clinically. See clinical features above.
* Additional studies
+
* Rule out alternative diagnoses (e.g. duplexes to rule out DVT if indicated)
 +
* Additional non-ED studies may include:
 
** Duplex Ultrasound
 
** Duplex Ultrasound
 
** Lymphoscintigraphy
 
** Lymphoscintigraphy
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==Disposition==
 
==Disposition==
 
+
*Discharge if uncomplicated
  
 
==See Also==
 
==See Also==

Revision as of 01:54, 27 January 2019

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

  • Venous Insufficiency
  • DVT
  • Post-thrombotic Syndrome
  • Hypoalbuminemia
  • CHF
  • 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.
  • 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 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[4][3]

  • 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