Lymphedema: Difference between revisions

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==Background==
==Background==
* Lymphedema is due to abnormal accumulation of interstitial fluid and fibroadipose tissue.
* Lymphedema is swelling due to abnormal accumulation of interstitial protein rich fluid and fibroadipose tissue.
* Occurs with lymphatic load exceeds capacity in the lymphatic system
* Occurs when 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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** Head and neck cancer
** Head and neck cancer
* Infection
* Infection
** Lymphatic Filariasis
** [[Lymphatic filariasis]]
** Tuberculosis
** [[Tuberculosis]]
** 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
** Sarcoidosis
** [[Sarcoidosis]]
** Arthritis
** [[Arthritis]]
*** Rheumatoid Arthritis
*** [[Rheumatoid arthritis]]
*** Psoriatic Arthritis
*** [[Psoriatic arthritis]]
*** Juvenile Idiopathic Arthritis
*** [[Juvenile Idiopathic Arthritis]]
** Dermatitis


==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  
* 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.
===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)||
*Soft edema +/- pitting
*Fluid accumulation subsides with limb elevation within 24 hours
*no sign of dermal fibrosis
||[[File:1lymphedema.png|70px]]
|-
| Stage II (moderate)
||
*Pitting present
*NOT reversible with limb elevation alone
*Some dermal fibrosis present
||[[File:2lymphedema.png|70px]][[File:3lymphedema.png|70px]]
|-
| Stage III (severe)||
*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}}
* Acute deep vein thrombosis
* Post-thrombotic Syndrome
* Limb Hypertrophy
** Hypertrophy of soft tissue or bone (Klippel-Trenaunay syndrome)
** Overgrowth of body part (Proteus Syndrome)
* Myxedema
* Lipedema
* Tumor


==Evaluation==
==Evaluation==
===Workup===
* Rule out alternative diagnoses (e.g. duplexes to rule out DVT if indicated)
* Additional non-ED studies may include:
** [[ultrasound: DVT|Duplex Ultrasound]]
** Lymphoscintigraphy
** Computed Tomography
** Magnetic Resonance Imaging/Lymphography
** Indocyanine Green (ICG) Lymphangiography
** Genetic Testing


===Diagnosis===
* Diagnosis is primarily made clinically.


==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>==
 
[[File:Lymphedema Compression sleeve November 2106 Second Skin 016.jpg|thumb|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==
==Disposition==
 
*Discharge if uncomplicated


==See Also==
==See Also==
 
*[[Unilateral leg swelling]]


==External Links==
==External Links==
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==References==
==References==
<references/>
<references/>
[[Category:FEN]]

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
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