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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Isolated swollen extremity secondary to lymph vessel damage from surgery, radiation, disease, or trauma, or as a primary developmental abnormality

  • No evidence of heart failure, renal disease, malabsorption, or other edema-producing conditions

GENERAL CONSIDERATIONS

  • Secondary lymphatic obstruction:

    • – Usually caused by damage to the lymphatic vessels or enlarged lymph nodes, particularly from neoplasm

    • – More common in older persons in whom diseases that obstruct the lymph vessels are more common

  • Primary lymphedema:

    • – A developmental abnormality that occurs in younger individuals

    • – Much less common than secondary lymphedema

  • Lymphedema praecox (most common form of primary lymphedema):

    • – More common in women (10:1)

    • – Often starts after menarche or the first pregnancy

    • – Often runs in families

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Painless swelling of an extremity extending to the digits

PHYSICAL EXAM FINDINGS

  • Nonpitting edema of an extremity

  • Thickened skin in affected limb with peau d’orange appearance

  • Stemmer sign: inability to tent the skin at the base of the affected digits

  • Yellow nail syndrome

DIFFERENTIAL DIAGNOSIS

  • Angioneurotic edema

  • Infectious lymphangitis

  • Venous insufficiency

  • Thrombophlebitis

  • Other causes of edema such as heart failure

DIAGNOSTIC EVALUATION

IMAGING STUDIES

  • Duplex Doppler echocardiography demonstrates normal arteries and veins in the affected limb

  • Lymphoscintigraphy shows:

    • – Reduced deep lymphatic vessels and prominent superficial lymphatics in lymphedema praecox

    • – Generally reduced lymphatics after radiation or in lymphangitis

    • – Obstructed dilated lymphatics with enlarged lymph nodes, in filariasis or after surgery (eg, mastectomy)

  • MRI or CT scan can demonstrate the presence of edema within the epifascial plane, along with cutaneous thickening

  • Contrast MRI is a promising technique for identifying the lymphatic vessels

  • Bioelectric impedance analysis is an emerging diagnostic technique that can quantify the amount of extracellular fluid in the extremities

DIAGNOSTIC PROCEDURES

  • Venography to exclude venous disease

TREATMENT

CARDIOLOGY REFERRAL

  • Suspected heart failure

HOSPITALIZATION CRITERIA

  • Thrombophlebitis

  • Heart failure

  • Suspected neoplasm

MEDICATIONS

  • Physiotherapy and massage

  • Elevation of the limb

THERAPEUTIC PROCEDURES

  • Compressive stockings

SURGERY

  • Lymph node transplantation

  • Lymphovenous shunt

  • In extreme cases, surgery to reduce the amount of subcutaneous tissue can be considered (Charles operation)

DIET AND ACTIVITY

  • A low-sodium diet may help reduce swelling

  • Exercise can help reduce swelling

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

  • Resolution of problem

  • After surgery

FOLLOW-UP

  • Yearly in stable patients

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