lymphedema

Etiology

• primary
  • congenital: Milroy's disease
  • lymphedema praecox:
    • Meige's disease
    • onset at puberty
  • lymphedema tarda: begins after age 35
  • associated disorders
    • Turner syndrome
    • Noonan syndrome
    • lymphangiectasia
    • lymphangiomyomatosis
• secondary
  • obesity
  • recurrent lymphangitis
    • most frequently due to Streptococci
    • common
  • filariasis: most common cause world wide
  • tuberculosis
  • neoplasm
    • prostate cancer
    • lymphoma
  • surgery
    • upper extremity lymphedema after mastectomy
    • gynecologic surgery for gynecologic cancer (20-40%)
  • radiation
  • infrequent causes
    • tuberculosis
    • contact dermatitis
    • lymphogranuloma venereum
    • rheumatoid arthritis
    • pregnancy
    • factitious - following application of tourniquets

Epidemiology

• primary lymphedema:
  • prevalence: 1/10,000
  • women affected more frequently than men

Pathology

• primary:
  • agenesis, hypoplasia or obstruction of lymph vessels
• secondary:
  • obstruction of previously normal lymphatic channels
• progressive fibrosis of cutaneous & subcutaneous tissue (lipodermatosclerosis)
• hyperkeratosis
• papillomatosis

Clinical manifestations

• generally painless
• edema of extremity of cosmetic concern
  • edema may begin in foot & progress superiorly to involve the entire leg
  • initially pitting edema
  • tissues become indurated & fibrotic with chronic lymphedema & edema may become non-pitting
• chronic, dull heavy sensation of the leg

Radiology

• abdominal & pelvic ultrasound to detect obstructing lesions
• CT of the abdomen to detect obstructing lesions
• lymphoscintigraphy or lymphangiography
  • rarely indicated
  • in primary lymphedema, lymphatic channels are absent, hypoplastic or ectatic
  • in secondary lymphedema, lymphatic channels are generally dilated & it may be possible to determine the level of obstruction

Complications

• excess risk for cutaneous angiosarcoma* with upper extremity lymphedema after mastectomy
• excess risk of non-melanoma skin cancer* with lower extremity lymphedema (RR=2.7)

* excess risk for skin cancer in affected extremity

Differential diagnosis

• deep vein thrombosis
• chronic venous insufficiency (venous stasis)

Management

• foot care to prevent recurrent lymphangitis
  • skin hygiene
  • emollients to prevent dryness
  • treat fungal infections of the foot aggressively
• prophylactic antibiotics are often helpful
• encourage physical activity
• frequent elevation of legs
• graduated compression hose
• intermittent pneumatic compression devices
• diuretics are contraindicated because they may cause intravascular volume depletion & electrolyte imbalances & will do little for the lymphedema
• microsurgical lymph-venous anastomotic procedures
• surgical resection of localized mass of lymphedema (abdomen, thigh, upper arms) may be of some success

More general terms

• lymphatic disease

More specific terms

• hereditary lymphedema
• hereditary lymphedema 2 (Meige lymphedema)
• lymphedema & ptosis
• lymphedema-distichiasis syndrome

Additional terms

• deep vein thrombosis (DVT)
• hypotrichosis-lymphedema-telangiectasia syndrome
• lymphatic system
• lymphedema-hypoparathyroidism syndrome
• venous stasis; venous hypertension

Patient information

lymphedema patient information