Lymphedema

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

• 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)
  • 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
  • Dermatitis
  • Sarcoidosis
  • Arthritis
    • Rheumatoid arthritis
    • Psoriatic arthritis
    • Juvenile Idiopathic Arthritis

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.

Differential Diagnosis

Unilateral leg swelling

Differential Diagnosis of Pedal Edema

• Gravitational
• Venous stasis
• Thrombophlebitis
• Lymphedema
• Medications
  • NSAIDS
  • Birth control
  • Steroids
• Deep venous thrombosis (uncomplicated)
  • Phlegmasia alba dolens
  • Phlegmasia cerulea dolens
• Leg or foot infection
  • Cellulitis
  • Necrotizing fasciitis
  • Septic Joint
• 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
  • CHF
  • Pretibial myxedema
  • Renal failure
  • Liver failure
  • Hypoalbuminemia

Evaluation

• 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
  • Lymphoscintigraphy
  • 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 absent) Skin changes such as fat deposits, acanthosis, warty overgrowths

Management

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

• Unilateral leg swelling