Lymphedema– Causes, Symptoms, Diagnosis, Treatment and Ongoing care

Basics

Description

  • 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

Epidemiology

Incidence

  • 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.

Prevalence

  • 120 million people worldwide are affected with filariasis.
  • 3 million–5 million people are affected by secondary lymphedema in the US.

Risk Factors

  • Filariasis: Most common cause worldwide
  • Mastectomy
  • Prior trauma
  • Infection of affected limb
  • History of prior surgical or radiation therapy for malignancy
  • Long history of venous insufficiency
  • Obesity

Genetics

  • 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.

General Prevention

Treatment of congestive heart failure (CHF), venous insufficiency

Pathophysiology

  • 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.

Etiology

Secondary lymphedema:

  • Trauma; recurrent infection; malignancy, including metastatic disease
  • Developing countries: Most common cause is filariasis (Wucheria bancrofti).

Commonly Associated Conditions

Venous disease

Pressure, Lymphatic system, congestive heart failure, lymphatic drainage, recurrent infection, breast cancer, malignancy,

Diagnosis

History

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

Physical Exam

  • 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.

Lab

Initial lab tests

  • Comprehensive chemistry panel: Evaluate for hepatic or renal impairment.
  • Urinalysis: Protein-losing nephropathy

Imaging

Initial approach

  • 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]

Differential Diagnosis

CHF, renal failure, hypoalbuminemia, protein-losing nephropathy, lipidemia, DVT, chronic venous disease, postoperative complications following ipsilateral surgery, cellulitis, Baker cyst, idiopathic edema

Treatment

Medication

  • 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]

Additional Treatment

General Measures

  • 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

Additional Therapies

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  • 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].

Surgery/Other Procedures

  • 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

In-Patient Considerations

Initial Stabilization

  • May admit to specialized rehabilitation unit for combination treatment in patients with heart failure or severe pulmonary disease
  • IV antibiotics for infection

Admission Criteria

Systemic signs of infection

IV Fluids

Not used unless needed for sepsis

Nursing

  • Leg elevation
  • Encourage patient mobilization/exercise.
  • Patient education for bandaging/wound care

Discharge Criteria

  • Resolution of signs/symptoms of infection (e.g., elevated white blood cell count, fever, abnormal vital signs)
  • Clinical improvement in wound appearance

Ongoing Care

Follow-Up Recommendations

Lymphedema will return in several days if patient stops wearing compression garments during the day and bandaging at night.

Patient Monitoring

  • Daily visit to therapist for acute treatment
  • Monthly visits for maintenance care

Diet

Low sodium

Patient Education

  • Use compression garments, especially when exercising.
  • Avoid affected limb(s) being dependant for long period of time: Patient should perform daily skin examination.

Prognosis

Good with daily care

Complications

  • 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.

References

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.

Codes

ICD9

457.1 Other lymphedema

Snomed

234097001 lymphedema (disorder)

Clinical Pearls

  • 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

 

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