Lymphedema & Lymphangitis

• • 2 primary diseases: Lymphedema and lymphangitis

Lymphedema

• • Little known about fluid dynamics of lymphatic system

  • Lymph propulsion occurs from lymphatic smooth muscle contractions

  • 2-4 L/d drain into subclavian vein daily

  • Mechanism for lymphedema: Impaired flow out of extremity

  • Primary disease

  • -Abnormal lymphatic development

    -Classified by age: Congenital (< 1 year old), familial (Milroy disease), lymphedema praecox (adolescence, unilateral), lymphedema tarda (> 35 years old)

  • Secondary disease

  • -Disease that causes obstruction to lymphatic system

    -Most common cause: Surgical excision, also radiation or axillary, inguinal areas

    -Less common causes: Bacterial, fungal infections, trauma

    -Developing countries: Filariasis (Wuchereria bancrofti)

    -Chronic lymphedema can result in lymphangiosarcoma (Stewart-Treeves syndrome)

Lymphangitis

• • Caused by hemolytic strep or staph infection in area of cellulites near open wound

  • Multiple long red streaks seen coursing toward lymph nodes

  • Can lead to sepsis and death if untreated

Epidemiology

Lymphedema

• • Lymphedema praecox: 3.5:1 female predominance

Symptoms and Signs

Lymphedema

• • Slowly progressive and painless

  • In early stages, edema is pitting; with time, fibrosis occurs and edema becomes nonpitting

  • Centered around ankle, pronounced around dorsum of foot and toes

  • Thickened skin, hyperkeratosis

  • Chronic eczematous dermatitis

  • History often defines cause

  • Painless edema in adolescent girl with family history = primary lymphedema

  • History of surgery, radiation, parasite infection suggests secondary cause

Lymphangitis

• • Pain at wound site, often red streaks along lymphatics toward lymph nodes

  • Regional lymph node enlargement

  • High fevers, sepsis

Laboratory Findings

Lymphangitis

• • Elevated WBC count

  • Blood and wound cultures

Imaging Findings

Lymphedema

• • Venous duplex: Exclude venous insufficiency

  • Lymphangiography: Rarely used because can damage lymphatics

  • Lymphoscintigraphy: May confirm diagnosis if ambiguous

  • CT/MRI: Can diagnose unknown malignancy as a secondary cause

Rule Out

• • Congestive heart failure, chronic renal insufficiency, chronic liver insufficiency

  • Congenital vascular malformations

  • Chronic venous insufficiency

  • Reflex sympathetic dystrophy

Lymphangitis

• • Superficial thrombophlebitis

  • Cat scratch fever

  • Cellulitis

• • Physical exam

  • Duplex US

  • CBC

  • Lymphoscintigraphy (rarely)

Lymphedema

• • No cure, goal of therapy to reduce complications

  • Pneumatic compression is first-line therapy

  • Leg elevation, manual lymphatic drainage massage, low-stretch wrapping

  • Skin care to prevent infection

  • Moisturizing lotions to prevent cracks

  • Rarely, surgical reduction of lumb bulk

  • -Sistrunk procedure: Staged excision of subcutaneous tissue

    -Thompson procedure: Lymphatic reconstruction, using omental free flaps to stimulate new lymphatic channels