Download - Dr. Boni - Buerger
dr. Bonifacius Lukmanto SpB.
IntroductionNonatherosclerotic Vascular disease
Characterized by the absence or minimal presence of atheromas, segmental vascular inflammation, vasoocclusive phenomenon, and involvement of small- and medium-sized arteries and veins of the upper and lower extremities.
Strongly associated with heavy tobacco use.Progression of the disease is closely linked to continued
use.Typical presentations are rest pain, unremitting ischemic
ulcerations, and gangrene of the digits of hands and feet.Described in Germany by von Winiwarter in an 1879 and
published by an American man called Leo Buerger.
PathophysiologyThe etiology of Buerger disease is unknown.
The disease mechanism underlying Buerger disease remains unclear.
FrequencyThe disease has decreased over the
past half decade in United States.More common in males (male-to-
female ratio, 3:1).Most patients with Buerger disease
are aged 20-45 years.
ClinicalHistory
Diagnosis of Buerger disease is difficult to establish.
Most patients (70-80%) Buerger disease present distal ischemic rest pain and/or ischemic ulcerations on the toes, feet, or fingers.
Progression of the disease lead involvement of more proximal arteries, but involvement of large arteries is unusual.
Patients may also present with claudication of the feet, legs, hands, or arms.
Foot or arch claudication erroneously attributed to an orthopedic problem.
Late in the course of their disease present with foot infections and, occasionally, with florid sepsis.
Clinical
PhysicalPainful ulcerations and/or frank gangrene of
the digits.The hands and feet are usually cool and
mildly edematous.Superficial thrombophlebitis.Paresthesias (numbness, tingling, burning,
hypoesthesia) of the feet and hands and impaired distal pulses.
> 80% of patients present with involvement of 3-4 limbs.
PhysicalPoint-Scoring system by Papa are using the
following criteriaOnset before age 45Tobacco use.Exclusion of atherosclerosis or proximal
source of emboli.Lack of hypercoagulable state.Lack of definable arteritis (ie, progressive
systemic sclerosis, giant cell arteritis).Classic arteriographic findings.Involvement of digital arteries of finger or
toes.
PhysicalSegmental involvement (ie, "skip areas").Corkscrew collaterals.No atherosclerotic changes.Classic histopathologic findings.Inflammatory cellular infiltrate within
thrombus.Intact internal elastic lamina.Involvement of surrounding venous tissues.
Physical
Table 1. Scoring system for the diagnosis of thromboangiitis obliterans (x)
Positive points
Age at onset Less than 30 (+2)/30-40 years (+1)
Foot intermittent claudication
Present (+2)/ by history (+1)
Upper extremity Symptomatic (+2)/ asymptomatic (+1)
Migrating superficial vein thrombosis
Present (+2)/ by history only (+1)
Raynaud Present (+2)/ by history only (+1)
Angiography; biopsy If typical both (+2)/ either(+1)
Table 1. Scoring system for the diagnosis of thromboangiitis obliterans (x)Negative points
Age at onset 45-50 (-1)/more than 50 years (-2)
Sex, smoking Female (-1)/ nonsmoker (-2)
Location Single limb (-1)/no LE involved (-2)
Absent pulses Brachial (-1)/femoral (-2)
Arteriosclerosis, diabetes, hypertension, hyperlipidemia
Discovered after diagnosis 5.1-10 years (-1)/2.1- 5 years later (-2)
Table 1. Scoring system for the diagnosis of thromboangiitis obliterans (x)
Table 2. Sum of points defines the probability of the diagnosis of thromboangiitis obliterans
Number of points Probability of diagnosis
0-1 Diagnostic excluded
2-3 Suspected, low probability
4-5 Probable, medium probability
6 or more Definite, high probability
WorkupLaboratory Studies
No specific laboratory tests confirm or exclude the diagnosis of Buerger disease.
Imaging StudiesAngiography/arteriography.Echocardiography: exclude a proximal source of
emboli as the cause of distal vessel occlusion.Other Tests
An abnormal Allen test.Histologic Findings
Biopsy is rarely needed unless the patient presents with unusual characteristics, such as large-artery involvement, or age older than 45 years.
Angiography/arteriography
TreatmentMedical Care
Absolute discontinuation of tobacco use is the only strategy proven to prevent the progression of Buerger disease.
No forms of therapy are definitive.Surgical Care
Autologous vein bypass of coexistent large-vessel atherosclerotic stenoses in patients with severe ischemia who have an acceptable distal target vessel.
Sympathectomy.