Bedah - Buerger's Disease

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    BUERGERS DISEASE

    Satria Pandu Persada Isma

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    Background

    Also known as thromboangiitis obliterans

    Nonatherosclerotic, segmental, inflammatory,vasoocclusive disease

    Affects the small- and medium-sized arteries and

    veins of the upper and lower extremities

    Strongly associated with heavy tobacco use.

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    Patophysiology

    Etiology is unknown

    Exposure to tobacco is essential for bothinitiation and progression of the disease

    Immunologic phenomenon that leads to

    vasodysfunction and inflammatory thrombi

    Prevalence of HLA-A9, HLA-A54, and HLA-B5 is

    observed.

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    Frequency

    12.6-20 cases per 100,000 population (US)

    More common in males (M:F ratio 3:1)

    Most patients are aged 20-45

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    History

    Age younger than 45 years

    Current (or recent) history of tobacco use

    Presence of distal extremity ischemia

    Exclusion of autoimmune diseases,hypercoagulable states, and diabetes mellitus

    Exclusion of a proximal source of emboli

    Consistent arteriographic findings

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    History (contd.)

    70-80%present with distal ischemic rest painand/or ischemic ulcerations on the toes, feet, orfingers

    Involvement of large arteries is unusual

    May also present with claudication of the feet,legs, hands, or arms and often describe theRaynaud phenomenon

    May present with foot infections

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    http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=/websites/emedicine/med/images/Large/2959Feet_Final.jpg&template=izoom2http://www.emedicine.com/cgi-bin/foxweb.exe/makezoom@/em/makezoom?picture=/websites/emedicine/med/images/Large/2960Foot_Final.jpg&template=izoom2
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    Physical Exam

    Develop painful ulcerations and/or frankgangrene of the digits

    Hands and feet are usually cool and mildly

    edematous

    Superficial thrombophlebitis (often migratory)

    Paresthesias

    Impaired distal pulses 80% percent of patients present with

    involvement of 3-4 limbs.

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    Physical Exam (contd.)

    Determine the colour

    Vascular angle

    Capillary filling timeVenous filling

    Pressure areas

    Allens test

    Temperature

    Cappilary refilling

    Feel all the pulsesAuscultate

    Check all the

    nerves

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    Differentials

    Raynaud phenomenon

    Systemic lupuserythematosus

    Antiphospholipid-antibody syndrome

    Diabetes mellitus

    Atherosclerosis

    Carpal tunnel syndrome

    Peripheral neuropathy

    Neurotrophic ulcers

    Trauma

    Vasculitis, other causes

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    Laboratory Workup

    No specific laboratory tests confirm orexclude the diagnosis of Buerger disease

    Primary goal of a laboratory workup inpatients thought to have the disease is toexclude other disease processes

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    Imaging Studies

    Angiography/arteriography

    nonatherosclerotic, segmental occlusivelesions of the small- and medium-sized

    vessels

    corkscrew collaterals

    Echocardiography

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    Morphology

    Segmental acute & chronic vasculitis withsecondary spread to contiguous veins andnerves

    Inflammation permeates arterial wallsaccompanied by thrombosis of the lumen

    Characteristically the thrombus containsmicroabscesses marked by a central focusof neutrophils surrounded bygranulomatous inflammation

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    Medical Care

    Absolute discontinuation of tobacco use isthe only strategy proven

    Use of thrombolytic therapy remainsinconclusive

    Intravenous iloprost ?

    Use of well-fitting protective footwear

    Avoidance of cold environments

    Avoidance of drugs that lead tovasoconstriction

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    Surgical Treatment

    Surgical revascularization for Buergerdisease is usually not feasible

    Autologous vein bypass

    Sympathectomy

    Amputation

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    Diet & Activity

    No dietary restrictions are needed

    Encourage cardiovascular exercise, activityshould be restricted by symptoms only

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    Complications

    Ulcerations

    Gangrene

    Need for amputation

    Rare occlusion of cerebral, coronary,renal, splenic, or mesenteric arteries

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    Prognosis

    Among patients with who quit smoking,94% avoid amputation

    Patients who continue smoking there is a43% chance that an amputation will berequired sometime during a 7-8 year

    period

    Mortality is rare

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    GRAZIE