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 | * 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 | ** [[Lymphatic filariasis]] | ||
** Tuberculosis | ** [[Tuberculosis]] | ||
** Recurrent Skin Infections (eg. | ** Recurrent Skin Infections (eg. [[cellulitis]], [[erysipelas]]) | ||
** Lymphadenitis | ** [[Lymphadenitis]] | ||
* Obesity | * [[the obese patient|Obesity]] | ||
* Inflammatory disorders | * Inflammatory disorders | ||
** Dermatitis | ** Dermatitis | ||
** Sarcoidosis | ** [[Sarcoidosis]] | ||
** Arthritis | ** [[Arthritis]] | ||
*** Rheumatoid | *** [[Rheumatoid arthritis]] | ||
*** Psoriatic | *** [[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 | ||
* 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. | ||
* 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== | ||
{{Unilateral leg swelling DDX}} | |||
==Evaluation== | ==Evaluation== | ||
* | ===Workup=== | ||
* Additional studies | * Rule out alternative diagnoses (e.g. duplexes to rule out DVT if indicated) | ||
** Duplex Ultrasound | * Additional non-ED studies may include: | ||
** [[ultrasound: DVT|Duplex Ultrasound]] | |||
** Lymphoscintigraphy | ** Lymphoscintigraphy | ||
** Computed Tomography | ** Computed Tomography | ||
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** Genetic Testing | ** 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>== | ||
* General Measures | [[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 | ** 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 | ||
==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
- 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
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]
Differential Diagnosis
Unilateral leg swelling
- Gravitational
- Venous stasis
- Thrombophlebitis
- Lymphedema
- Medications
- Deep venous thrombosis (uncomplicated)
- Leg or foot infection
- Fracture
- Compartment syndrome
- Limb hypertrophy
- Hypertrophy of soft tissue or bone (Klippel-Trenaunay syndrome)
- Overgrowth of body part (Proteus Syndrome)
- Lipedema
- Tumor
- Post-thrombotic Syndrome
- Causes of bilateral 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]
- 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
- ↑ Szuba A, Rockson SG. Lymphedema: classification, diagnosis and therapy. Vasc Med 1998; 3:145-56
- ↑ 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
- ↑ 3.0 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
- ↑ Rockson SG. Lymphedema. Am J Med 2001; 110:288-95