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Internal Medicine

Thromboangiitis obliterans (TAO) “Buerger’s disease”

Thromboangiitis obliterans, also known as Buerger disease, is a recurring progressive inflammation and thrombosis (clotting) of small and medium arteries and veins of the hands and feet.

Cover image: Thromboangiitis Obliterans or Buerger's disease is an arterial vascular disease associated with smoking. Note the blackened tips of middle and pinky fingers. | Image courtesy of Charlie Goldberg, M.D.

Thromboangiitis obliterans, also known as Buerger disease, is a recurring progressive inflammation and thrombosis (clotting) of small and medium arteries and veins of the hands and feet.


Etiology

  • Tobacco
    • Smoking and/or chewing
    • Smoking index (SI) > 300 is a risk factor
      • [No. of cigarettes per day X No. of years of smoking]
    • Pack-years index (PYI) > 40 is a risk factor
      • [No. of years of smoking] X [No. of cigarette packs per year\
  • < 30 years

Pathophysiology

Segmental, progressive, occlusive, inflammatory disease of small & medium-sized vessels with superficial thrombophlebitis.

  1. Thromboembolism due to blood disorders
  2. (Nicotine) → Vasoconstriction → Arterial Occlusion → Necrosis
    • Panarteritis is a common feature
Pathophysiological phases of thromboangiitis obliterans
Pathophysiological phases of thromboangiitis obliterans | Piazza G, Creager MA. Thromboangiitis obliterans. Circulation [Internet]. 2010 Apr 27 [cited 2017 Mar 22];121(16):1858–61. Available from: http://www.ncbi.nlm.nih.gov/pubmed/20421527

Complications:

  • Ulcerations
  • Gangrene
  • Autoamputation

Presentation

  • Intermittent claudication: Progressing to pain, ulceration & gangrene
  • Itching

Clinical subtypes:

  • Type I: Upper limb TAO (rare)
  • Type II: Involves legs & feet
  • Type III: Femoropopliteal involvement
  • Type IV: Aortofemoral involvement
  • Type V: Generalised

Diagnosis

Olin (2000) diagnostic criteria

  1. 20–40 years old and male (although females have been diagnosed)
  2. Current (or recent) history of tobacco use
  3. Presence of distal extremity ischemia (indicated by claudication, pain at rest, ischemic ulcers or gangrene) documented by noninvasive vascular testing such as ultrasound
  4. Exclusion of other autoimmune diseases, hypercoagulable states, and diabetes mellitus by laboratory tests.
  5. Exclusion of a proximal source of emboli by echocardiography and arteriography.
  6. Consistent arteriographic findings in the clinically involved and noninvolved limbs

Allen test

Buerger’s test

  • Elevation pallor: Vascular angle or Buerger’s angle is the angle to which the leg has to be raised before it becomes pale, whilst in supine decubitus
    • Normal limb may not show pallor even at 90°
    • Vascular angle < 20°: Severe ischemia
  • Sunset foot: When patient shifts to sitting position, the leg will revert to the pink colour more slowly than normal and also pass through the normal pinkness to a red-orange colouring (rubor – redness)
  • Rubor of dependency: Foot will return to its normal colour if descended

Imaging:

  • Arterial Doppler & Duplex scan
  • CT Angiography (Transfemoral retrograde angiogram)
    • Narrowing of blood vessels
    • Spiralling of blood vessels

Differential diagnosis:

  • Atherosclerosis (build-up of cholesterol plaques in the arteries)
  • Endocarditis (an infection of the lining of the heart)
  • Other types of vasculitis
  • Severe Raynaud’s phenomenon associated with connective tissue disorders (e.g., lupus or scleroderma)
  • Clotting disorders of the blood

Management

Lifestyle changes

  • Cessation of tobacco

Medical management (Acute cases):

  • Vasodilation:
    • Nifedipine
    • Prostaglandins
    • Hyperbaric oxygen
    • Epidural anaesthesia
  • Antithrombin:
    • Low-dose aspirin
  • Streptokinase
    • Adjuvant

Surgical management (Chronic cases):

  • Debridement of ulcers
  • Sympathectomy
  • Omentoplasty: Surgical procedure in which part of the greater omentum is used to cover or fill a defect, augment arterial or portal venous circulation, absorb effusions, or increase lymphatic drainage
  • Profundoplasty: Surgical enlargement of the junction of the femoral artery and its deep branch, a common operation to relieve narrowing by atherosclerosis at this point
  • Amputation (if required)

Buerger-Allen exercises

Specific exercises intended to improve circulation to the feet and legs
Buerger-Allen exercises
Buerger-Allen exercises. 1, Elevate feet on padded chair or board for 1/2 to 3 minutes. 2, Sit in relaxed position while each foot is flexed and extended then pronated and supinated for 3 minutes. The feet should become entirely pink. If the feet are blue or painful, elevate them and relax as necessary. 3, Lie quietly for 5 minutes, keeping legs warm with a blanket. | From Black and Matassarin-Jacobs, 1997.

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