I. Epidemiology

  1. Incidence: Primary Lymphedema occurs 1 in 10,000

II. Types

  1. 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)
  2. 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

III. Signs

  1. 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)
  2. Late edema (inflammatory fibrosis)
    1. Woody, firm non-Pitting Edema (Brawny Edema)
    2. Skin thickened and hyperkeratotic
    3. Refractory to limb elevation and compression

IV. Differential Diagnosis

  1. See Edema
  2. Chronic Venous Insufficiency
  3. Postphlebitic syndrome
  4. Myxedema (Hypothyroidism)
  5. Lipedema
    1. Seen in women with onset after Puberty
    2. Increased subcutaneous fat between Pelvis and ankle
  6. Malignant Lymphedema
    1. Rapid, painful cancer-related edema begins centrally
  7. 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

V. Complications

  1. Recurrent Bacterial Cellulitis (Gram Positive Bacteria)
  2. Refractory Late Edema (Non-Pitting Edema)

VI. Management: General

  1. Compression (Prevents late edema or Brawny Edema)
    1. Contraindicated in limb Arterial Insufficiency
    2. Aggressive decongestive lymphatic therapy
    3. Elastic support garments (built-in pressure gradient)
      1. 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
      1. Massage fluid proximally
  2. Limb Elevation
    1. Elevate above heart level for 30 minutes tid
  3. Good skin hygiene
    1. Keep web spaces between digits dry
    2. Apply Antifungal powder
  4. Avoid local injury or trauma
    1. Avoid walking barefoot (especially outdoors)
  5. Medications
    1. Benzopyrones (Not available in U.S)
    2. Topical coumarin (Not available in U.S.)
  6. Observe closely for Cellulitis

VII. Management: Surgery

  1. Excision of hypertrophic fibrotic subcutaneous tissue
    1. Indicated for elephantiasis
    2. Types of procedures
      1. Charles Operation
      2. Kondoleon Procedure
  2. Lymphatic pedicle transfer
    1. Supplies alternative lymph drainage
    2. No proven efficacy
  3. Microvascular bypass of obstructed lymph channels
    1. Experimental

VIII. Resources

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

IX. References

  1. Sabiston (1997) Surgery, Saunders, p.1574-7
  2. Rockson (2001) Am J Med 110:288-95

Images: Related links to external sites (from Google)

Ontology: Lymphedema (C0024236)

Definition (NCI) Excess fluid collection in tissues, causing swelling. It is the result of obstruction of lymphatic vessels or lymph nodes.
Definition (NCI) A disorder characterized by excessive fluid collection in tissues that causes swelling.
Definition (CHV) Edema due to obstruction of lymph vessels or disorders of the lymph nodes
Definition (CHV) Edema due to obstruction of lymph vessels or disorders of the lymph nodes
Definition (CHV) Edema due to obstruction of lymph vessels or disorders of the lymph nodes
Definition (CHV) Edema due to obstruction of lymph vessels or disorders of the lymph nodes
Definition (CHV) Edema due to obstruction of lymph vessels or disorders of the lymph nodes
Definition (MEDLINEPLUS)

The lymphatic system is a network of tissues and organs. It is made up mainly of lymph vessels, lymph nodes and lymph. Lymph vessels, which are different from blood vessels, carry fluid called lymph throughout your body.

The lymphatic system collects excess fluid and proteins from body tissues and carries them back to the bloodstream. Edema, or swelling, may happen when there is an increase in the amount of fluid or because of a blockage in the lymphatic system. The accumulation of lymph is called lymphedema.

Causes of lymphedema include

  • Infection
  • Cancer
  • Scar tissue from radiation therapy or surgical removal of lymph nodes
  • Inherited conditions in which lymph nodes or vessels are absent or abnormal

Treatment includes physical methods, such as compression stockings, and medicines.

NIH: National Cancer Institute

Definition (NCI) A condition in which extra lymph fluid builds up in tissues and causes swelling. It may occur in an arm or leg if lymph vessels are blocked, damaged, or removed by surgery.
Definition (MSH) Edema due to obstruction of lymph vessels or disorders of the lymph nodes.
Definition (CSP) edema due to obstruction of lymph vessels or disorders of the lymph nodes.
Concepts Disease or Syndrome (T047)
MSH D008209
SnomedCT 234097001, 30213001, 155485008, 195486001, 266334008
English Edema, lymphatic, Lymphedema, acquired, Lymphedema, Lymphedemas, LYMPH EDEMA, LYMPHEDEMA, LYMPHOEDEMA, Lymphedema, NOS, Lymphoedema, NOS, Lymphedema NOS, lymphedema (diagnosis), lymphedema, Lymphoedema NOS, Lymphedema [Disease/Finding], lymphedemas, oedema lymphatic, lymphatic oedema, lymphatic edema, Lymphatic Oedema, Lymphatic Edema, Lymphoedemas, Acquired lymphedema, Lymphatic edema, Lymphatic oedema, Lymphoedema, Acquired lymphoedema, Lymphatic edema (morphologic abnormality), Lymphedema (disorder), edema; lymphatic obstruction, edema; lymphatic, lymphatic obstruction; edema, lymphatic; edema, lymphoedema
French LYMPHOEDEME, Lymphoedème SAI, Lymphoedèmes, Lymphoedème
Portuguese LINFEDEMA, Linfedema NE, Linfedemas, Linfoedema, Linfedema
Spanish LINFEDEMA, Linfedema NEOM, Lymphoedema, Lymphedema, Lymphedema NOS, Lymphoedema NOS, edema linfático (anomalía morfológica), edema linfático, linfedema (trastorno), linfedema adquirido, linfedema, Linfedemas, Linfoedema, Linfedema
German LYMPHOEDEM, Lymphoedem NNB, Lymphoedem, Lymphoedeme, Lymphödem
Italian Linfedemi, Linfedema NAS, Linfedema
Dutch lymfoedeem NAO, lymfatisch; oedeem, lymfatische obstructie; oedeem, oedeem; lymfatische obstructie, oedeem; lymfatisch, lymfoedeem, lymfoedemen, Lymfoedeem, Oedeem, lymf-
Japanese リンパ浮腫, リンパ浮腫NOS, リンパフシュ, リンパフシュNOS
Swedish Lymfödem
Czech lymfedém, Lymfedémy, Lymfedém, Lymfedém NOS
Finnish Lymfedeema
Russian LIMFEDEMA, MILROIA BOLEZN', LIMFANGIEKTATICHESKII OTEK, LIMFATICHESKII OTEK, MEIDZHA BOLEZN', TROFEDEMA, TROFICHESKII OTEK, ЛИМФАНГИЭКТАТИЧЕСКИЙ ОТЕК, ЛИМФАТИЧЕСКИЙ ОТЕК, ЛИМФЕДЕМА, МЕЙДЖА БОЛЕЗНЬ, МИЛРОЯ БОЛЕЗНЬ, ТРОФЕДЕМА, ТРОФИЧЕСКИЙ ОТЕК
Croatian LIMFEDEM
Polish Choroba Milroya, Obrzęk limfatyczny
Hungarian Lymphoedema k.m.n., Lymphoedemak, Lymphoedema, Nyirok eredetű vizenyő