Lymphedema

Epidemiology

Incidence: Primary Lymphedema occurs 1 in 10,000

Pathophysiology

Lymphatic System typically is responsible for 10-20% fluid return (remaining 80-90% is venous return)
Lymphedema is accumulation of Protein-rich fluid in extracellular space

Types

Primary Lymphedema
1. Congenital Lymphedema
  1. Lymphedema Congenita
  2. Milroy's Disease (Hereditary)
2. Pubertal onset of edema
  1. Lymphedema Praecox (Most common primary Lymphedema)
3. Middle age onset of edema
  1. Lymphedema tarda (associated with injury)
Secondary Lymphedema (acquired lymphatic obstruction)
1. Tumor obstruction of Regional Lymph Nodes
  1. Prostate Cancer
  2. Lymphoma
2. Surgical excision or radiation to regional nodes
  1. Breast Cancer with axillary node dissection
3. Infection of regional Lymph Nodes
  1. Filariasis (Wuchereria bancrofti)
  2. Tuberculosis

Signs

Early edema (Protein-rich fluid accumulation)
1. Soft "puffy" extremity swelling
2. Maximal increase increase in girth in first year
3. Easily pits
4. Responds to limb elevation and compression
5. Involves distal extremity (e.g. dorsal foot)
  1. See Stemmer's Sign (pathognomonic for Lymphedema)
Late edema (inflammatory fibrosis)
1. Woody, firm Non-Pitting Edema (Brawny Edema)
2. Skin thickened and hyperkeratotic
3. Refractory to limb elevation and compression

Differential Diagnosis
Acute Conditions critical to exclude (e.g. emergency department)

Differential Diagnosis
Subacute and Chronic Conditions

See Edema
Chronic Venous Insufficiency
Postphlebitic Syndrome
Myxedema (Hypothyroidism)
Lipedema
1. Seen in women with onset after Puberty
2. Increased subcutaneous fat between Pelvis and ankle
Malignant Lymphedema
1. Rapid, painful cancer-related edema begins centrally
Distinguishing characteristics of early edema
1. Subcutaneous fibrosis (peau d'orange)
2. Stemmer Sign (skin does not tent on dorsal digits)
3. Preferential swelling of foot dorsum
4. Involved extremity of squared-off digits

Complications

Recurrent Bacterial Cellulitis (Gram Positive Bacteria)
Refractory Late Edema (Non-Pitting Edema)
Pain and decreased extremity range of motion
Severe Lymphedema (acute swelling >80%)
1. Requires hospitalization and possible surgical intervention
Lymphagiosarcoma (Stewart-Treves Syndrome)
1. Upper extremity soft tissue malignancy complicating chronic upper extremity edema
2. Rare complication of Breast Cancer Management with Lymph Node dissection (<0.45% of cases)
3. Presents as arm blue-purple Macule or Papule (or as bulla or Cellulitis)
4. Biopsy suspected lesions

Management
General

Indicated in aggressive decongestive lymphatic therapy
Compression (Prevents late edema or Brawny Edema)
1. Precautions
  1. Contraindicated in limb Arterial Insufficiency (except for low resting pressure wraps)
  2. Confirm adequate padding
  3. Observe for friction sites (risk of open sores)
2. Wraps (active edema reduction)
  1. High resting pressure (ACE Wrap, Tubigrip)
    1. Absolutely contraindicated in Peripheral Vascular Disease
  2. Mult-layer compression
    1. Multiple-layer: Cotton layer, ace wrap, firm wrap)
    2. Most effective compression for Wound Healing
    3. Combines high and low resting pressure
  3. Low resting pressure (e.g. Rosidahl, Lymphedema wrap)
    1. These wraps do not require a resting force (rely on Muscle movement for return)
    2. Ineffective, if calf Muscle is not functional to assist return (use high resting pressure instead)
    3. Typically safe to use in Peripheral Arterial Disease
3. Elastic Support Garments or Compression Stockings (built-in pressure gradient)
  1. Used for maintenance only (not for acute, active edema reduction)
  2. May initiate Compression Stockings 40 mmHg or greater after edema improves with wraps
  3. Replace every 3-6 months with loss of elasticity
4. Mechanical Pneumatic Pressure Device
  1. Indicated for severe edema
  2. Applied at night or 2-3 times per week
5. Special Massage Techniques (performed by Lymphedema specialists)
  1. Massage fluid proximally
  2. Ezzo (2015) Cochrane Database Syst Rev (5):CD003475 +PMID: 25994425 [PubMed]
Limb Elevation
1. Elevate above heart level for 30 minutes three times daily
Exercise
1. Promotes surrounding Muscle activity to promote lymphatic flow and fluid return
Good skin hygiene (prevent infection)
1. Keep web spaces between digits dry
2. Apply Antifungal powder
3. Use Skin Lubricants (Moisturizers)
Avoid local injury or Trauma
1. Avoid walking barefoot (especially outdoors)
Medications
1. Benzopyrones (Not available in U.S)
2. Topical coumarin (Not available in U.S.)
3. Avoid Diuretics (minimal to no effect)
Observe closely for Cellulitis

Management
Surgery

Excision of hypertrophic fibrotic subcutaneous tissue
1. Indicated for elephantiasis
2. Types of procedures
  1. Charles Operation
  2. Kondoleon Procedure
Lymphatic pedicle transfer
1. Supplies alternative lymph drainage
2. No proven efficacy
Microvascular Lymphovenous bypass of obstructed lymph channels
1. Reduces limb circumference >1.9 cm
2. Fallahian (2022) Ann Plast Surg 88(2):195-9 +PMID: 34398594 [PubMed]

Resources

National Lymphedema Network Position Papers
1. http://www.lymphnet.org/lymphedemaFAQs/positionPapers.htm

References

Novotny (2017) Wound Care Update, Park Nicollet Conference, St Louis Park, MN (attended 9/15/2017)
Shelby (2015) Crit Dec Emerg Med 29(6): 2-8
Sabiston (1997) Surgery, Saunders, p.1574-7
Grada (2017) J Am Acad Dermatol 77(6):995-1006 +PMID: 29132859 [PubMed]
Rockson (2001) Am J Med 110:288-95 [PubMed]