Localized Lymphedema

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 ...