- Lymphedema is due to abnormal accumulation of interstitial fluid and fibroadipose tissue.
- Occurs with 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
- Lymphatic Filariasis
- Recurrent Skin Infections (eg. Cellulitis, Erysipelas)
- Inflammatory disorders
- Rheumatoid Arthritis
- Psoriatic Arthritis
- Juvenile Idiopathic Arthritis
- Slowly progressive swelling
- Feeling of heaviness, tightness, or discomfort
- May be pitting at onset
- 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 Insufficiency
- Acute deep vein thrombosis
- Post-thrombotic Syndrome
- Congestive Heart Failure
- Limb Hypertrophy
- Hypertrophy of soft tissue or bone (Klippel-Trenaunay syndrome)
- Overgrowth of body part (Proteus Syndrome)
- Diagnosis is primarily made clinically. See clinical features above.
- Additional studies
- 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.
- 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