Lymphedema: Difference between revisions

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==Background==
==Background==
* Lymphedema is due to abnormal accumulation of interstitial fluid and fibroadipose tissue.
* Lymphedema is due to abnormal accumulation of interstitial protein rich fluid and fibroadipose tissue.
* Occurs with lymphatic load exceeds capacity in the lymphatic system
* Occurs with lymphatic load exceeds capacity in the lymphatic system


===Etiology===
===Etiology===
====Primary====
====Primary<ref>Szuba A, Rockson SG. Lymphedema: classification, diagnosis and therapy. Vasc Med 1998; 3:145-56</ref>====
* Congenital Lymphedema (6-12%) - before age 2
* Congenital Lymphedema (6-12%) - before age 2
* Lymphedema Precox (77-94%) - at onset of puberty
* Lymphedema Precox (77-94%) - at onset of puberty
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====Secondary====
====Secondary====
* Cancer and cancer treatment (Lymphadenectomy, Radiotherapy)
* Cancer and cancer treatment (Lymphadenectomy, Radiotherapy) <ref>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 </ref>
** Breast Cancer - most common
** Breast Cancer - most common
** Sarcoma
** Sarcoma
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** Genetic Testing
** Genetic Testing


===Clinical Staging (by International Society of Lymphology)===
===Clinical Staging (by International Society of Lymphology) <ref name="ISL">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</ref>===
* Stage 0 - Asymptomatic, swelling not evident despite impaired lymphatic transport. May have feeling of heaviness in limb.
* 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 I (mild) - Soft edema +/- pitting, no sign of dermal fibrosis, fluid accumulation subsides with limb elevation within 24 hours.
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* Stage III (severe) - Lymphostatic elephantiasis, skin changes such as fat deposits, acanthosis, warty overgrowths.
* Stage III (severe) - Lymphostatic elephantiasis, skin changes such as fat deposits, acanthosis, warty overgrowths.


==Management==
==Management<ref>Rockson SG. Lymphedema. Am J Med 2001; 110:288-95</ref><ref name="ISL">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</ref>==
* General Measures
* General Measures  
** Self-monitoring - for size, sensation, color, temperature, skin condition
** Self-monitoring - for size, sensation, color, temperature, skin condition
** Limb elevation
** Limb elevation
** Diet and exercise - maintain ideal body weight. Recommended to use compression garments during exercise.
** Diet and exercise - maintain ideal body weight. Recommended to use compression garments during exercise.
** Avoid skin infection/injury
** Avoid skin infection/injury
* Compression Therapy - bandaging, garments, intermittent pneumatic compression
* Compression Therapy - bandaging, garments, intermittent pneumatic compression  
* Physiotherapy - manual lymphatic drainage
* Physiotherapy - manual lymphatic drainage  
** Contraindicated in presence of cellulitis, neoplasm, DVT, moderate-severe heart failure
** Contraindicated in presence of cellulitis, neoplasm, DVT, moderate-severe heart failure
* Surgical Referral
* Surgical Referral

Revision as of 03:13, 5 October 2017

Background

  • Lymphedema is due to abnormal accumulation of interstitial protein rich fluid and fibroadipose tissue.
  • Occurs with 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

  • Cancer and cancer treatment (Lymphadenectomy, Radiotherapy) [2]
    • 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) [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

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
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