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  • • 2 primary diseases: Lymphedema and lymphangitis


  • • 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)


  • • 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



  • • Lymphedema praecox: 3.5:1 female predominance

Symptoms and Signs


  • • 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


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

    • Regional lymph node enlargement

    • High fevers, sepsis

Laboratory Findings


  • • Elevated WBC count

    • Blood and wound cultures

Imaging Findings


  • 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


  • • Superficial thrombophlebitis

    • Cat scratch fever

    • Cellulitis

  • • Physical exam

    • Duplex US

    • CBC

    • Lymphoscintigraphy (rarely)


  • • 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

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