Difference between revisions of "Lymphedema"

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** Recurrent Skin Infections (eg. [[cellulitis]], [[erysipelas]])
 
** Recurrent Skin Infections (eg. [[cellulitis]], [[erysipelas]])
 
** [[Lymphadenitis]]
 
** [[Lymphadenitis]]
* Obesity
+
* [[the obese patient|Obesity]]
 
* Inflammatory disorders
 
* Inflammatory disorders
 
** Dermatitis
 
** Dermatitis
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*** [[Rheumatoid arthritis]]
 
*** [[Rheumatoid arthritis]]
 
*** [[Psoriatic arthritis]]
 
*** [[Psoriatic arthritis]]
*** Juvenile Idiopathic Arthritis
+
*** [[Juvenile Idiopathic Arthritis]]
  
 
==Clinical Features==
 
==Clinical Features==
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** 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.
 +
 +
===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>===
 +
{| {{table}}
 +
| align="center" style="background:#f0f0f0;"|'''Stage'''
 +
| align="center" style="background:#f0f0f0;"|'''Signs & Symptoms'''
 +
| align="center" style="background:#f0f0f0;"|'''Image'''
 +
|-
 +
| Stage 0 (asymptmatic)||
 +
*Generally asymptomatic, although may have feeling of heaviness in limb
 +
*Swelling not evident on exam
 +
||
 +
|-
 +
| Stage I (mild)||
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*Soft edema +/- pitting
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*Fluid accumulation subsides with limb elevation within 24 hours
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*no sign of dermal fibrosis
 +
||[[File:1lymphedema.png|70px]]
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|-
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| Stage II (moderate)
 +
||
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*Pitting present
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*NOT reversible with limb elevation alone
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*Some dermal fibrosis present
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||[[File:2lymphedema.png|70px]][[File:3lymphedema.png|70px]]
 +
|-
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| Stage III (severe)||
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*Lymphostatic elephantiasis (pitting may be absent)
 +
*Skin changes such as fat deposits, acanthosis, warty overgrowths
 +
||[[File:4lymphedema.png|70px]]
 +
|}
  
 
==Differential Diagnosis==
 
==Differential Diagnosis==
* Venous Insufficiency
+
{{Unilateral leg swelling DDX}}
* [[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==
 
==Evaluation==
* Diagnosis is primarily made clinically. See clinical features above.
+
===Workup===
 
* Rule out alternative diagnoses (e.g. duplexes to rule out DVT if indicated)
 
* Rule out alternative diagnoses (e.g. duplexes to rule out DVT if indicated)
 
* Additional non-ED studies may include:
 
* Additional non-ED studies may include:
** Duplex Ultrasound
+
** [[ultrasound: DVT|Duplex Ultrasound]]
 
** Lymphoscintigraphy
 
** Lymphoscintigraphy
 
** Computed Tomography  
 
** Computed Tomography  
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** Genetic Testing
 
** Genetic Testing
  
===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>===
+
===Diagnosis===
* Stage 0 - Asymptomatic, swelling not evident despite impaired lymphatic transport. May have feeling of heaviness in limb.
+
* Diagnosis is primarily made clinically.  
* 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<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>==
 
==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>==
 +
[[File:Lymphedema Compression sleeve November 2106 Second Skin 016.jpg|thumb|Example of compression therapy: lymphedema compression sleeve on mannequin]]
 
* General Measures  
 
* General Measures  
 
** Self-monitoring - for size, sensation, color, temperature, skin condition
 
** Self-monitoring - for size, sensation, color, temperature, skin condition
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==See Also==
 
==See Also==
 
+
*[[Unilateral leg swelling]]
  
 
==External Links==
 
==External Links==

Latest revision as of 16:22, 29 September 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.

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
1lymphedema.png
Stage II (moderate)
  • Pitting present
  • NOT reversible with limb elevation alone
  • Some dermal fibrosis present
2lymphedema.png3lymphedema.png
Stage III (severe)
  • Lymphostatic elephantiasis (pitting may be absent)
  • Skin changes such as fat deposits, acanthosis, warty overgrowths
4lymphedema.png

Differential Diagnosis

Unilateral leg swelling

Differential Diagnosis of Pedal Edema

Evaluation

Workup

  • 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

Diagnosis

  • Diagnosis is primarily made clinically.

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