- 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
- Congenital Lymphedema (6-12%) - before age 2
- Lymphedema Precox (77-94%) - at onset of puberty
- Lymphedema Tarda (11%) - after age 35
- Cancer and cancer treatment (Lymphadenectomy, Radiotherapy) 
- Breast Cancer - most common
- Lower Extremity Melanoma
- Gynecologic Cancer
- Genitourinary Cancer
- Head and neck cancer
- Inflammatory disorders
- 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.
- Venous insufufficiency
- Deep venous thrombosis (uncomplicated)
- Leg or foot infection
- Compartment syndrome
- Limb hypertrophy
- Hypertrophy of soft tissue or bone (Klippel-Trenaunay syndrome)
- Overgrowth of body part (Proteus Syndrome)
- Post-thrombotic Syndrome
- Causes of bilateral pedal edema
- Diagnosis is primarily made clinically. See clinical features above.
- Rule out alternative diagnoses (e.g. duplexes to rule out DVT if indicated)
- Additional non-ED studies may include:
- Duplex Ultrasound
- Computed Tomography
- Magnetic Resonance Imaging/Lymphography
- Indocyanine Green (ICG) Lymphangiography
- Genetic Testing
Clinical Staging (by International Society of Lymphology) 
- 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.
|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|
|Stage II (moderate)||*Pitting present *Some dermal fibrosis present *Not reversible with limb elevation alone|
|Stage III (severe)||*Lymphostatic elephantiasis (pitting may be abscent) *Skin changes such as fat deposits, acanthosis, warty overgrowths.|
- 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
- Discharge if uncomplicated
- 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
- 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