pathology: lymphedema

yogabook / pathologie / lymphedema

lymphedema

Definition of

Accumulation of fluid in the interstitium caused by mechanical insufficiency (deficiency, reflux or obstruction) of the lymphatic system. All parts of the body can be affected by lymphoedema. Lymphoedema is divided into stages:

  1. Stage 0: latency stage, asymptomatic
  2. Stage 1: reversible stage, protein-rich doughy-soft edema with only a few, small, localized fibrosclerotic tissue changes. Pressing leaves a dent, elevation noticeably reduces the edema. Positive Stemmer’s sign
  3. Stage 2: spontaneously irreversible stage, further fibrosclerotic changes, proliferation of the fat cells; hardly compressible edema, no recognizable improvement by elevation
  4. Stage 3: Elephantiasis, extensive fibrosclerotic changes with extensive fatty tissue proliferation, body part swollen beyond recognition of the anatomical structures, severely restricted flexibility; tendency to eczema, vesicles, fistulas, poorly healing wounds

According to etiology, a distinction is made between

  1. Rare primary form: Malformation or absence of lymph nodes; usually whole body regions are affected. The whole body, however, is not compatible with life (usually: death of the embryo, otherwise postnatal). Also in Milroy disease and Meige disease (Nonne-Milroy-Meige syndrome)
  2. common secondary form with multiple causes, see below.

Lymphoedema leads to an inflammatory reaction in which monocytes, fibroblasts and adipocytes migrate and fat and collagen are deposited („protein fibrosis“).

Cause

  1. very rare: congenital
  2. Tumor diseases
  3. Traumas
  4. Lymphangitides
  5. RA
  6. Chronic venous insufficiency (CVI)
  7. Diabetes mellitus
  8. iatrogenic: surgery, radiological irradiation, lymph node resection for tumor therapy, vein removal for bypass surgery

Diagnosis

  1. Lymphography/lymphoscintigraphy
  2. Sono
  3. Tests and signs: Stemmer’s sign, thumb test (indentation with temporary retention of a dent, possibly no longer present from stage 2)

Symptoms

  1. local tissue swelling
  2. usually painless, but there is a dull feeling of heaviness
  3. Spread from distal to proximal
  4. Increased subcutaneous fatty tissue, lifting of the normal body contours, box toes

Complications

  1. Increased risk of secondary infections (erysipelas, mycoses)
  2. Movement restrictions

Therapy

  1. Manual or mechanical lymphatic drainage
  2. Compression clothing / bandage
  3. Exercise therapy in compression garments
  4. OP: super microsurgery
  5. Surgery: microsurgical lymphaticovenous anastomoses
  6. Avoidance of injuries, injections, blood sampling, mosquito bites, sunburns in the affected area
  7. Prophylactic against inflammation: good skin care
  8. NO diuretics!
  9. For severe movement restrictions: Resection of fatty tissue

DD

  1. Lipedema
  2. Obesity
  3. Myxedema
  4. if unilateral: phlebothrombosis
  5. if unilateral: lymphogenous metastatic carcinoma
  6. Chronic venous insufficiency