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Buerger Disease (Thromboangiitis Obliterans): Differential Diagnoses & Workup

Author: Eric J Hanly, MD, Resident, Department of Surgery, The Johns Hopkins University School of Medicine; Fellow, Department of Surgery, The Johns Hopkins University School of Medicine
Coauthor(s): Ozanan R Meireles, MD, Resident Physician, Department of Surgery, Michigan State University College of Human Medicine; Research Fellow in Minimally Invasive Surgery, Department of Surgery, Johns Hopkins University School of Medicine; E Jerry Cohn Jr, MD, FACS, Vascular Surgeon, The Vein Center at Savannah Vascular Institute
Contributor Information and Disclosures

Updated: May 1, 2009

Differential Diagnoses

Antiphospholipid Antibody Syndrome and Pregnancy
Peripheral Arterial Occlusive Disease
Atherosclerosis
Polyarteritis Nodosa
Diabetes Mellitus, Type 1
Raynaud Phenomenon
Diabetes Mellitus, Type 2
Reflex Sympathetic Dystrophy
Frostbite
Scleroderma
Giant Cell Arteritis
Systemic Lupus Erythematosus
Gout
Takayasu Arteritis
Infrainguinal Occlusive Disease
Thoracic Outlet Obstruction

Other Problems to Be Considered

  • Acrocyanosis
  • Carpal tunnel syndrome
  • Cervical rib
  • Ergotism
  • Juvenile temporal arteritis with eosinophilia
  • Livedo reticularis
  • Metatarsalgia
  • Neuropathy, peripheral
  • Neurotrophic ulcers
  • Orthopedic problem of the foot or arch
  • Trauma
  • Vasculitis, other causes
  • Calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia (CREST) syndrome
  • Systemic lupus erythematosus
  • Rheumatoid vasculitis
  • Mixed connective-tissue disease
  • Antiphospholipid-antibody syndrome
  • Diabetes mellitus
  • Embolic occlusion of small or medium arteries
  • Hyperhomocysteinemia with atherosclerosis
  • Popliteal artery entrapment syndrome
  • Repetitive vibratory equipment use
  • Hypothenar hammer syndrome

Workup

Laboratory Studies

  • No specific laboratory tests confirm or exclude the diagnosis of Buerger disease. The primary goal of a laboratory workup in patients thought to have the disease is to exclude other disease processes in the differential diagnosis. Tests often used as markers for the diagnosis of systemic vasculitis, such as the acute-phase reactants, are negative in TAO. A complete serologic profile must be obtained.
    • CBC count with differential
    • Liver function tests
    • Renal function tests
    • Urinalysis
    • Glucose (fasting)
    • Erythrocyte sedimentation rate
    • C-reactive protein
    • Antinuclear antibody
    • Rheumatoid factor
    • Complement
    • Anticentromere antibody
    • Scl-70 antibody
    • Antiphospholipid antibodies

Imaging Studies

  • Angiography/arteriography
    • Arteriographic abnormalities consistent with Buerger disease are sometimes seen in limbs that are not yet clinically involved; therefore, arteriography of all 4 limbs may be required.
    • The hallmark angiographic findings in patients with Buerger disease are nonatherosclerotic, segmental occlusive lesions of the small- and medium-sized vessels (eg, digital, palmar, plantar, tibial, peroneal, radial, and ulnar arteries) with formation of distinctive small-vessel collaterals around areas of occlusion known as "corkscrew collaterals" (see Image 4). Such arteriographic findings suggest Buerger disease but are not pathognomonic because similar lesions can be observed in patients with scleroderma, CREST syndrome, systemic lupus erythematosus, rheumatoid vasculitis, mixed connective-tissue disease, antiphospholipid-antibody syndrome, and even diabetes mellitus.
  • Echocardiography: Always perform echocardiography in patients thought to have Buerger disease in order to exclude a proximal source of emboli as the cause of distal vessel occlusion.

Other Tests

  • An abnormal Allen test result indicating distal arterial disease and establishing involvement of the upper extremities in addition to the lower extremities helps differentiate thromboangiitis obliterans from atherosclerotic disease.
    • To perform the Allen test, the patient is instructed to make a fist, which exsanguinates the hand and fingers. The examiner's thumbs are then used to occlude the radial and ulnar arteries. The patient then opens the hand, after which the examiner releases the pressure on the ulnar artery while the radial artery remains compressed.
    • Prompt return of color to the hand indicates patency of the ulnar artery (ie, a normal or negative test result). The patency of the radial artery can then be tested by repeating the maneuver but with the pressure on the radial artery released while the ulnar artery remains compressed.
    • Failure of the hand to promptly refill with blood indicates occlusion of the respective artery distal to the wrist (ie, an abnormal or positive test result). While an abnormal result can be present in other types of small-vessel occlusive disease of the hands, a positive Allen test finding in a young smoker with leg ulcerations is highly suggestive of Buerger disease.

Histologic Findings

Olin contends that a biopsy is rarely needed unless the patient presents with unusual characteristics, such as large-artery involvement, or age older than 45 years.

  • In its acute phase, Buerger disease is characterized by highly cellular, segmental, occlusive, inflammatory thrombi, with minimal inflammation in the walls of affected blood vessels. Secondary spread from the affected small- and medium-sized arteries to contiguous veins and nerves is often observed. Microscopically, the polymorphonuclear leukocyte–predominant inflammatory cellular aggregate may form microabscesses and multinucleated giant cells.
  • In the subacute phase, intraluminal thrombosis progressively organizes, but it may defer to vascular recanalization.
  • The end-stage phase of the disease is characterized by mature thrombus and vascular fibrosis.
  • In all 3 stages, the integrity of the normal structure of the vessel wall, including the internal elastic lamina, is maintained. This distinguishes thromboangiitis obliterans from arteriosclerosis and from other types of systemic vasculitis, in which disruption of the internal elastic lamina and the media can be extensive.

More on Buerger Disease (Thromboangiitis Obliterans)

Overview: Buerger Disease (Thromboangiitis Obliterans)
Differential Diagnoses & Workup: Buerger Disease (Thromboangiitis Obliterans)
Treatment & Medication: Buerger Disease (Thromboangiitis Obliterans)
Follow-up: Buerger Disease (Thromboangiitis Obliterans)
Multimedia: Buerger Disease (Thromboangiitis Obliterans)
References
Further Reading
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References

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Further Reading

Clinical guidelines

Counseling to prevent tobacco use and tobacco-caused disease: recommendation statement.
 United States Preventive Services Task Force - Independent Expert Panel.  1996 (revised 2003 Nov).  13 pages.  NGC:003268

Chronic wounds of the lower extremity.
American Society of Plastic Surgeons - Medical Specialty Society.  2007 May.  21 pages.  NGC:005966

VA/DoD Clinical practice guideline for rehabilitation of lower limb amputation.
Department of Defense - Federal Government Agency [U.S.]
Department of Veterans Affairs - Federal Government Agency [U.S.]
Veterans Health Administration - Federal Government Agency [U.S.].  2007 Aug.  163 pages.  NGC:006060

Clinical trial

 TACT-NAGOYA: Therapeutic Angiogenesis Using Cell Transplantation

Related eMedicine topics
Digital Amputations

Atherosclerosis

Vascular Occlusive Syndromes of the Upper Extremity

Vascular Ulcers

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Keywords

Buerger disease, Buerger's disease, thromboangiitis obliterans, TAO, endarteritis, endophlebitis, vasoocclusive disease, presenile spontaneous gangrene, corkscrew collaterals, bidi, cigarette smoking, tobacco use, secondhand smoke, smoking cessation, distal ischemic rest pain, ischemic ulcerations, amputation

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Contributor Information and Disclosures

Author

Eric J Hanly, MD, Resident, Department of Surgery, The Johns Hopkins University School of Medicine; Fellow, Department of Surgery, The Johns Hopkins University School of Medicine
Eric J Hanly, MD is a member of the following medical societies: American Medical Association, Association of Military Surgeons of the US, MedChi, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Ozanan R Meireles, MD, Resident Physician, Department of Surgery, Michigan State University College of Human Medicine; Research Fellow in Minimally Invasive Surgery, Department of Surgery, Johns Hopkins University School of Medicine
Ozanan R Meireles, MD is a member of the following medical societies: American College of Surgeons and Society of American Gastrointestinal and Endoscopic Surgeons
Disclosure: Nothing to disclose.

E Jerry Cohn Jr, MD, FACS, Vascular Surgeon, The Vein Center at Savannah Vascular Institute
E Jerry Cohn Jr, MD, FACS is a member of the following medical societies: American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Richard M Stillman, MD, FACS, Honorary Medical Staff, Northwest Medical Center; Former Chief of Staff and Medical Director, Wound Healing Center, Department of Surgery, Northwest Medical Center
Richard M Stillman, MD, FACS is a member of the following medical societies: American College of Angiology, American College of Surgeons, Association for Academic Surgery, and Society of University Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Vincent Lopez Rowe, MD, Assistant Professor of Surgery, Department of Surgery, Division of Vascular Surgery, University of Southern California Medical Center
Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, Association for Academic Surgery, Peripheral Vascular Surgery Society, Society for Clinical Vascular Surgery, and Society for Vascular Surgery
Disclosure: Nothing to disclose.

CME Editor

Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy
Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences
Disclosure: Nothing to disclose.

Chief Editor

William H Pearce, MD, Chief, Division of Vascular Surgery, Violet and Charles Baldwin Professor of Vascular Surgery, Department of Surgery, Northwestern University School of Medicine
William H Pearce, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, American Surgical Association, Association for Academic Surgery, Association of VA Surgeons, Central Surgical Association, New York Academy of Sciences, Society for Vascular Surgery, Society of Critical Care Medicine, Society of University Surgeons, and Western Surgical Association
Disclosure: Nothing to disclose.

 
 
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