LYMPHEDEMA

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LYMPHEDEMA

• Eden Wheeler, M. D.
• ROCKHILL ORTHOPAEDICS
• Physical Medicine and Rehabilitation

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What is Lymphedema ?

• Swelling of a body part, usually a limb, due to
  accumulation of lymph fluid from obstructed flow
  of the lymphatics
• Primary lymphedema is due to a congenital
  deformity of the lymphatic system
• Lymphedema congenita present at birth
• Lymphedema praecox develops in childhood or
  adolescence
• Lymphedema tarda develops in adulthood

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Secondary Lymphedema

• Secondary lymphedema usually results from a
  trauma to the lymphatic system
• Surgery
• Radiation therapy
• Tumor compression/obstruction
• Traumatic injury
• Infection
• Lymphoproliferative disease
• Tumor/cancer involvement

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What is the lymphatic system ?

• Retrieves proteins filtered out of the
  circulatory system by capillaries and returns
  them to the venous system for circulation
• The lymphatic system originates as lymph
  capillaries in the spaces between cells
• The capillaries join to form larger vessels
  called the lymphatics

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Lymphatic System (continued)

• The lymphatics are joined together by lymph nodes
  which act as filters and empty into the right
  lymphatic duct or the thoracic duct
• Lymphatics
• smaller diameter and thinner walls than veins or
  arteries
• contain valves like veins to assist with lymph
  flow
• also are aided by skeletal muscle contractions
  and pulsatile movements of surrounding blood
  vessels to assist with lymph flow

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How does it occur ?

• Decreased lymph flow from lymphatic damage
  results in increased protein concentration in the
  interstitial fluid
• With increased protein concentration, fluid is
  shifted into the interstitial space due to
  oncotic pressure
• With fluid accumulation in the interstitial
  space, swelling/edema occurs in that body part

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How does it occur ? (continued)

• Lymphatic damage and fluid stasis also leads to
  scar tissue/fibrosis development with collagen
  and fibrin deposition to vessel walls, therefore
  further blocking lymph flow
• Fluid stasis increases susceptibility to
  infection with cycle initiated for further
  lymphatic damage

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How frequently does it occur ?

• Affects 1 of the US population over 2 million
  people usually from breast cancer treatment
• Post-mastectomy 25.5
• Post-mastectomy with axillary lymph node
  dissection and radiation therapy 38.3
• Further increased with obesity and/or infection

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How frequently does it occur ? (continued)

• May also see with
• lymphoma
• prostate cancer
• melanoma
• Hodgkins disease
• ovarian cancer
• Cushings disease
• Systemic lupus erythmatosus
• Even more common in third world countries due to
  prevalence of parasitic infections

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Types of lymphedema

• Acute
• mild, transient form
• occurring within days of surgery
• resolving within first weeks
• Painful
• occurs 4 to 6 weeks after surgery
• frequently associated with phlebitis or
  lymphangitis
• Erysipeloid
• occurs with chronic form after local trauma

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Types of lymphedema (continued)

• Chronic
• more insidious and painless form
• occurs 18 to 24 months after surgery after
  gradual development of fibrous tissues
• Dependent
• occurs with fluid overloading conditions such a
  renal failure or cardiovascular diseases
• more involving dependent or inactive extremities

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Physiologic consequencesfrom lymphedema
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What is seen clinically ?

• Edema of the involved anatomy
• Pain can, but not always may be, present
• more common with infection
• Usually no skin ulcerations are seen
• if present, more consistent with chronic venous
  insufficiency
• If erythema, must consider infection
• Thickening, hardening or doughiness of the
  subcutaneous tissue
• Seldom are skin changes seen

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Are diagnostic tools appropriate ?

• Lymphangiography has been used in past to
  evaluate, however
• rarely is it required due to strong correlation
  with history and exam for diagnosis
• anatomical information gained has no impact on
  treatment
• can frequently exacerbate the condition
• If clinical suspicion, venogram or venous doppler
  can rule out presence of thrombosis
• Best tools remain detailed history and exam

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What happens if not treated ?

• A cycle of fibrosis, stasis and protein
  accumulation begins with progression and
  worsening of edema
• Increased incidence of infection
• Elephantiasis may develop in final stages
• In severe cases, rare complication of
  lymphangiosarcoma may occur

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When is treatment appropriate ?

• ALWAYS in the presence of painless swelling,
  especially if greater than 2 cm
• If swelling occurs after the 6 week period
  following completion of treatment
• ALWAYS with swelling in the months or years after
  treatment, but first must rule out cancer
  recurrence or metastatic disease

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What are goals of treatment ?

• Improve cosmesis
• Preserve skin integrity
• Soften subcutaneous tissues
• Avoid infection or lymphangitis
• Decrease limb size
• Avoid contracture of the involved limb
• Most importantly patient and family education

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What are treatment options ?

• Medications
• Antibiotics
• should always have on hand to begin treatment
  with first signs of infection
• should cover Gram positive organisms with most
  common pathogen Group A Strep
• Diuretics
• can decrease water and sodium in the interstitial
  fluid, but have no impact on protein status
• risks usually outweigh benefits
• Benzopyrones / Coumarin
• stimulate proteolysis
• not available in United States or FDA approved

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What are treatment options ?

• Mechanical interventions
• elevation
• protection of limb
• manual lymph drainage/massage
• compression garments to reduce and control edema
• exercise, especially of distal musculature, to
  facilitate lymph flow
• Surgery Not curative
• Excisional Charles / Homans procedure
• debulking of the area to remove excess tissue to
  decrease volume
• Physiological
• drainage of the area via lymph to lymph or lymph
  to venous anastomosis

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What can Physiatry provide for treatment ?

• Education regarding factors contributing to
  lymphedema and therefore increased compliance
• Early treatment for initial or recurrent
  infection
• Emphasize proper care of the extremity
• Determination of appropriate therapy and/or
  long-term compression needs

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What are measures to be taken for protection ?

• Make every attempt to avoid
• cuts scratches insect bites
• hangnails punctures burns
• Do not irritate or handle these injuries if they
  occur
• No blood pressures or venipunctures in involved
  limb
• Liberal use of moisturizer to avoid dryness or
  cracking

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Measures to take (continued)

• Wear protective garments for appropriate
  activities
• gardening or pruning
• dishwashing
• baking/cooking
• sewing
• Immediate contact with physician if presence of
  infection
• Carry heavy parcels or purses on the opposite
  limb
• Sunscreen or long-sleeved garments for sun
  exposure
• Sleep with limb elevated

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Measures to take (continued)

• Jewelry should not be worn on affected limb
• Insect repellent for high risk exposure
• Utilize electric razor for shaving
• Check water temperature with other limb or
  thermometer before immersion
• Avoid heavy lifting or repetitive movements with
  involved limb
• Avoid tight-fitting garments to affected limb
• Avoid climate temperature extremes

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What types of compression are available ?

• Compression stocking
• can be used for treatment and maintenance
• 2 pairs are appropriate to maximize hygiene
• last from 4 to 6 months
• can be difficult to don or doff and keep in
  position
• Compressive wrapping
• better flexibility for specific problem areas and
  for specific patients
• patients and families can learn technique
• allow greater activity level than pumps
• can be time consuming to don

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Available compression (continued)

• Compression pumps
• not adequate for primary therapy
• do not address proximal edema
• high cost with decreased compliance
• less convenient for associated exercise or
  mobility
• variable protocols dependent on brand and type
  but ranges
• single chamber/uniform/intermittent compression
  30-60 mm Hg
• multi-chamber/differential/sequential
  compression60-120 mm Hg
• Reid sleeve
• markets as being less expensive, less cumbersome
  and more effective than traditional pump