Lymphedema is a chronic, progressive swelling of tissue with protein-rich fluid as a consequence of developmental (primary lymphedema) or acquired (secondary lymphedema) disruption of the lymphatic system. Extremities are most commonly affected, followed by genitalia.
Most cases are secondary to nematode infection (filariasis), malignancy, or cancer-related treatment.
Typically presents with painless unilateral limb swelling; pitting edema is present in early disease, whereas nonpitting edema is a sensitive but nonspecific finding in advanced disease.
Diagnosis is usually made on clinical grounds and confirmed by lymphoscintigraphy.
First-line treatment involves compression, ranging from static garments to complex massage and pneumatic compression devices. Surgical procedures are reserved for patients refractory to conservative measures and/or with significant morbidity.
There is no cure. Successful care requires a long-term, collaborative approach between patient and providers.
The progressive swelling of a body part, usually an extremity, following developmental (primary lymphedema) or acquired (secondary lymphedema) disruption of the lymphatic system resulting in lymph (a protein-rich fluid) accumulating in the interstitial space. The extremities are most commonly involved, followed by the genitalia.
History and exam
Key diagnostic factors
- history of malignancy
- history of travel to endemic filariasis area
- history of previous surgery
- history of radiation therapy
- painless unilateral swelling of extremity or genitalia
- involvement of distal extremity
- positive Stemmer sign
Other diagnostic factors
- history of penetrating trauma to the axilla or groin
- limb heaviness and/or weakness
- nonpitting edema
- skin changes
- family history of lymphedema
- breast cancer treatment
- nematode infection (filariasis)
- surgery near axillary or inguinal lymph nodes
- advanced tumor, nodes, and metastasis (TNM) stage
- radiation therapy
- curvilinear scars
- Family history of lymphedema
- genetic syndrome
1st investigations to order
Investigations to consider
- MRI scan of affected extremity
- CT scan of affected extremity
- ultrasound of affected area
- blood smear for filariasis
- genetic testing
- Chronic venous insufficiency
- Deep vein thrombosis (DVT)
- The diagnosis and treatment of peripheral lymphedema
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