- Swelling of a body part due to an abnormality in regional lymphatic drainage
- Results in increased interstitial volume secondary to the accumulation of tissue (lymphatic) fluid
- Most common in the lower limb (80%) but also can occur in the arms, face, trunk, and external genitalia
- Predominant sex: Female > Male.
- Predominant age: Any age
- 13% of breast cancer patients treated with surgery; 42% of those treated with surgery and radiation therapy
- Estimated to be between 1/6,000 and 1/300 live births; Milroy disease presents at birth.
- Meige disease develops during puberty.
- 120 million people worldwide are affected with filariasis.
- 3 million–5 million people are affected by secondary lymphedema in the US.
- Filariasis: Most common cause worldwide
- Prior trauma
- Infection of affected limb
- History of prior surgical or radiation therapy for malignancy
- Long history of venous insufficiency
- Milroy disease: Autosomal dominant; diagnosed either at birth or the 1st year of life
- Lymphedema praecox has onset between the ages of 1 and 35 years.
- Lymphedema tarda occurs >35 years.
Treatment of congestive heart failure (CHF), venous insufficiency
- Postoperative: Gradual failure of distal lymphatics, which have to “pump” lymph at a greater pressure through damaged proximal ducts
- Risk is higher with postoperative radiation because radiation reduces regrowth of ducts owing to fibrous scarring.
- Trauma; recurrent infection; malignancy, including metastatic disease
- Developing countries: Most common cause is filariasis (Wucheria bancrofti).
Commonly Associated Conditions
Recent surgery: Vein stripping can significantly exacerbate mild lymphedema (1)[B].
- 1st symptom: Painless swelling
- Feeling of heaviness in the limb, especially at the end of the day and in hot weather
- Initial: Pitting edema, can spread proximally
- Later: Nonpitting; after 1st year, does not spread proximally/distally but spreads radially
- Hyperkeratosis (thicker skin)
- Papillomatosis (rough skin)
- Increase in skin turgor
- Positive Stemmer sign (inability to pinch the skin of the dorsum of the second toe between the thumb and forefinger): Exclude heart failure.
Diagnostic Tests & Interpretation
- Lack of response to elevation or diuretic therapy may indicate a lymphatic insufficiency (2)[B].
- Diuretics increase excretion of salt and water thereby decreasing plasma volume, venous capillary pressure, and filtration. Diuretics improve filtration edema but don’t improve lymph drainage over the long-term.
Initial lab tests
- Comprehensive chemistry panel: Evaluate for hepatic or renal impairment.
- Urinalysis: Protein-losing nephropathy
- Ultrasound: Evaluate for acute/chronic deep vein thrombosis (DVT). Gives information about soft-tissue changes but does not tell about truncal anatomy of the lymphatics (1)[B].
- Duplex ultrasound: Lymphedema causes gradual impedance of venous return that aggravates the edema; 82% of patients with unexplained limb edema were diagnosed using a combination of duplex ultrasound and lymphoscintigram (3)[A].
Follow-Up & Special Considerations
- Lymphangiogram: Direct cannulation of lymphatics through the skin; risk for infection, local inflammation; not used commonly (3)[C]
- Lymphoscintigram: Radiolabeled protein technetium-99m-labeled colloid:
- Measures lymphatic function, lymph movement, lymph drainage, and response to treatment
- Sensitivity 73–97%; specificity 100%
- Best to use 1-h and delayed images together (3)[A]
- CT scan: Calf skin thickening, thickening of the subcutaneous compartment, increased fat density, thickened perimuscular aponeurosis; typical honeycomb appearance (3)[B]
- MRI: Circumferential edema, increased volume of subcutaneous tissue, honeycomb pattern above the fascia between the muscle and subcutis; cannot differentiate primary from secondary lymphedema (3)[B]
CHF, renal failure, hypoalbuminemia, protein-losing nephropathy, lipidemia, DVT, chronic venous disease, postoperative complications following ipsilateral surgery, cellulitis, Baker cyst, idiopathic edema
- Micronized purified flavonoid fraction [Daflon 500 mg] is effective in decreasing venous stasis and idiopathic cyclic edema, chronic venous insufficiency, and postmastectomy lymphedema. It also reduces capillary permeability and the inflammatory component (4)[C].
- Benzopyrenes (coumarin): Reduces edema fluid by increasing the number of macrophages and enhancing proteolysis resulting in the removal of protein, increasing softness in the limbs, and decreasing elevated skin temperature.
- Decreases symptoms and signs and decreases instances of secondary infection
- Some reports of hepatotoxicity (4)[C]
- Elevation of affected limb: May be difficult for some patients to comply
- Prevent disease progression.
- Achieve mechanical reduction and maintenance of limb size.
- Alleviate symptoms.
- Prevent skin infection.
Issues for Referral
- Refer to physical therapist with lymphedema training for manual decongestive therapy.
- Provide education for patient/family for self-administration of therapy in future.
- Education for family about bandaging
- Fitting for compression garments
- Exercise: Lymph flow occurs as a result of inspiratory reduction in the intrathoracic pressure associated with inspiration. Best results are achieved with combination of flexibility, strength, and aerobic training (3)[B].
- Compression with custom-made elastic stocking (minimum pressure 40 mm Hg):
- Protection against external incidental trauma
- Decreases the intrinsic trauma on the skin owing to chronically increased interstitial pressures, which cause stretch of the skin and subcutaneous tissues
- No data on preference of custom made versus prefabricated
- Replace every 3–6 months or when starting to lose elasticity (1)[B].
- Multilayer bandaging: Inner layer of tubular stockinette followed by foam and padding to protect the joint flexures and to even out the contours of the limb so that pressure is distributed evenly; outer layer of at least 2 short-stretch extensible bandages; more effective than hosiery alone (1)[B]
- Pneumatic pumps: Development of high pressure up to 150 mm Hg; can reduce limb girth by 37–68.6%; wear a compression stocking when not using pump; high risk of genital edema; no metastasis in limb owing to risk of spread (1)[B]
Complementary and Alternative Medicine
Heat therapy: Hot water immersion, microwave, and electromagnetic irradiation may be helpful (1)[C].
- Debulking procedures (Charles procedure): Radical excision of subcutaneous tissue with primary or staged skin grafting:
- Men had less improvement than women.
- Main risk is infection and necrosis of the skin graft.
- Bypass procedures: Creation of lymphatic–venous anastomosis: Reserved for highly refractory cases only
- May admit to specialized rehabilitation unit for combination treatment in patients with heart failure or severe pulmonary disease
- IV antibiotics for infection
Systemic signs of infection
Not used unless needed for sepsis
- Leg elevation
- Encourage patient mobilization/exercise.
- Patient education for bandaging/wound care
- Resolution of signs/symptoms of infection (e.g., elevated white blood cell count, fever, abnormal vital signs)
- Clinical improvement in wound appearance
Lymphedema will return in several days if patient stops wearing compression garments during the day and bandaging at night.
- Daily visit to therapist for acute treatment
- Monthly visits for maintenance care
- Use compression garments, especially when exercising.
- Avoid affected limb(s) being dependant for long period of time: Patient should perform daily skin examination.
Good with daily care
- Infection (local versus systemic): Common
- Risk of wound formation (venous wounds/abrasions) that are difficult to heal: Common
- Lymphangiosarcoma: Found in lymphedematous arms of patients following radical mastectomy; also in patients with Milroy disease; treatment is radiotherapy with surgery, reserved for patients with discrete nonmetastatic disease.
1. Warren A, et al. Lymphedema: A comprehensive review. Ann Plastic Surg. 2007;59(4):464–72.
2. Mortimer P. “implications of the Lymphatic System in CVI-Associated Edema.” Angiology. The Journal of Vascular Diseases 2000;51(1):3–7.
3. Brennan MJ, Miller LT. Overview of treatment options and review of the current role and use of compression garments, intermittent pumps, and exercise in the management of lymphedema. Cancer.1998;83:2821–7.
4. Tiwari A, Cheng KS, Button M, et al. Differential diagnosis, investigation, and current treatment of lower limb lymphedema. Arch Surg. 2003;138:152–61.
457.1 Other lymphedema
234097001 lymphedema (disorder)
- Use short-stretch bandages for wrapping (not ACE wraps).
- Heat/whirlpool typically makes the wounds/lymphedema worse, not better.
- Patients with lymphedema are at much higher risk for infection than patients with only venous insufficiency