Lymphedema
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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