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Thromboangiitis Obliterans Clinical Presentation

  • Author: Naiem Nassiri, MD; Chief Editor: Vincent Lopez Rowe, MD  more...
 
Updated: Nov 19, 2015
 

History

Because a firm diagnosis of thromboangiitis obliterans (TAO), or Buerger disease, is difficult to establish, a number of different diagnostic criteria have been proposed.[7] In 1990, Olin et al asserted that the following criteria must be met for the diagnosis to be made with reasonable certainty[8] :

  • Age younger than 45 years
  • Current (or recent) history of tobacco use
  • Presence of distal extremity ischemia (indicated by claudication, pain at rest, ischemic ulcers, or gangrene) documented by noninvasive vascular testing
  • Exclusion of autoimmune diseases, hypercoagulable states, and diabetes mellitus by laboratory tests
  • Exclusion of a proximal source of atheroemboli by echocardiography and arteriography
  • Consistent arteriographic findings in the clinically involved and noninvolved limbs

Most patients with TAO (70-80%) present with distal ischemic rest pain or ischemic ulcerations on the toes, feet, or fingers (see the image below).[9, 10] Progression of the disease may lead to involvement of more proximal arteries, but involvement of large arteries is unusual.

Feet of patient with thromboangiitis obliterans (B Feet of patient with thromboangiitis obliterans (Buerger disease). Note ischemic ulcers on distal portion of left great, second, and fifth toes. Although patient's right foot is normal in gross appearance, angiography demonstrated compromised arterial flow to both feet.

Patients may also present with claudication of the feet, legs, hands, or arms and often describe experiencing the Raynaud phenomenon (a pathologic vasospastic process involving pain, paresthesias, and color changes of the digits of the hands and feet in response to cold or anxiety).

Patients who seek medical attention late in the course of their disease may present with foot infections and, occasionally, with florid sepsis.

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Physical Examination

Patients with TAO can develop painful ulcerations (see the image below) or frank gangrene of the digits. The hands and feet of patients with the disease are usually cool and mildly edematous.

Tobacco smoke stains on male patient's fingers sug Tobacco smoke stains on male patient's fingers suggest diagnosis of thromboangiitis obliterans (Buerger disease). Patient presented with small, painful ulcers on tips of thumb and ring finger.

Superficial thrombophlebitis (often migratory) occurs in almost half of patients with TAO (see the image below). Paresthesias (numbness, tingling, burning, hypoesthesia) of the feet and hands and impaired distal pulses in the presence of normal proximal pulses are usually found in patients with severe disease. More than 80% of patients present with involvement of three or four limbs.

Superficial thrombophlebitis of great toe in patie Superficial thrombophlebitis of great toe in patient with thromboangiitis obliterans (Buerger disease).

In 1996, Papa et al proposed a point-scoring system to support or contest the diagnosis of TAO on the basis of the following criteria[11] :

  • Distal extremity (feet, toes, hands, or fingers) involvement
  • Onset before age 45 years
  • Tobacco use
  • Exclusion of atherosclerosis or proximal source of emboli
  • Absence of a hypercoagulable state
  • Absence of definable arteritis (eg, progressive systemic sclerosis or giant-cell arteritis)
  • Classic arteriographic findings
  • Involvement of digital arteries of finger or toes
  • Segmental 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

The scoring system is applied as indicated in Table 1 and Table 2 below.

Table 1. Scoring System for Diagnosis of Thromboangiitis Obliterans[11] (Open Table in a new window)

Positive Criterion Positive Points
Age at onset < 30 y (+2)



30-40 y (+1)



Foot intermittent claudication Present (+2)



By history only (+1)



Upper extremity Symptomatic (+2)



Asymptomatic (+1)



Migrating superficial thrombophlebitis Present (+2)



By history only (+1)



Raynaud phenomenon Present (+2)



By history only (+1)



Angiography; biopsy If typical, both (+2)



Either(+1)



Negative Criterion Negative Points
Age at onset 45-50 y (−1)



>50 y (−2)



Sex; smoking Female (−1)



Nonsmoker (−2)



Location Single limb (−1)



No lower extremity involved (−2)



Absent pulses Brachial (−1)



Femoral (−2)



Arteriosclerosis, diabetes, hypertension, hyperlipidemia Discovered 5.1-10 y after diagnosis (−1)



Discovered 2.1-5 y later (−2)



 

Table 2. Numerical Scores Defining Probability of Diagnosis of Thromboangiitis Obliterans (Open Table in a new window)

No. of Points Probability of Diagnosis of Thromboangiitis Obliterans
0-1 Diagnosis excluded
2-3 Diagnosis suspected (low probability)
4-5 Diagnosis probable (medium probability)
≥6 Diagnosis definite (high probability)

 

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Complications

Potential complications of TAO include the following:

  • Ulcerations
  • Gangrene
  • Infection
  • Need for amputation
  • Rare occlusion of coronary, renal, splenic, or mesenteric arteries
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Contributor Information and Disclosures
Author

Naiem Nassiri, MD RPVI, Instructor in Vascular Surgery, Rutgers Robert Wood Johnson Medical School; Founder and Director, Vascular Anomalies and Malformations Program, Vascular Center of New Jersey and Bristol Myers Squibb Children’s Hospital Center for Advanced Surgery

Naiem Nassiri, MD is a member of the following medical societies: American College of Surgeons, Society for Vascular Surgery, American Venous Forum, Society for Clinical Vascular Surgery, International Society for the Study of Vascular Anomalies

Disclosure: Nothing to disclose.

Chief Editor

Vincent Lopez Rowe, MD Professor of Surgery, Program Director, Vascular Surgery Residency, Department of Surgery, Division of Vascular Surgery, Keck School of Medicine of the University of Southern California

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, Society for Vascular Surgery, Vascular and Endovascular Surgery Society, Society for Clinical Vascular Surgery, Pacific Coast Surgical Association, Western Vascular Society

Disclosure: Nothing to disclose.

Acknowledgements

Matthew Carpenter, MD Program Director, Department of Internal Medicine, Department of Internal Medicine, Keesler Medical Center; Assistant Clinical Professor, Uniformed Services University of the Health Sciences, F Edward Hebert School of Medicine

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.

Eric J Hanly, MD 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.

Ozanan R Meireles, MD Instructor in Surgery, Department of Surgery, Massachusetts General Hospital, Harvard Medical School

Ozanan R Meireles, MD is a member of the following medical societies: American College of Surgeons, American Society for Gastrointestinal Endoscopy, and Society of American Gastrointestinal and Endoscopic Surgeons

Disclosure: Nothing to disclose.

Brian D Peyton, MD Chief of Vascular and General Surgery, Keesler Medical Center; Assistant Professor, Department of Surgery, Associate Program Director, Department of General Surgery, Uniformed Services University of the Health Sciences, F Edward Hebert School of Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References
  1. von Winiwarter F. Ueber eine eigenthumliche Form von Endarteriitis und Endophlebitis mit Gangran des Fusses. Arch Klin Chir. 1879. 23:202-26.

  2. Buerger L. Thrombo-angiitis obliterans: a study of the vascular lesions leading to presenile spontaneous gangrene. Am J Med Sci. 1908. 136:567-80.

  3. Espinoza LR. Buerger's disease: thromboangiitis obliterans 100 years after the initial description. Am J Med Sci. 2009 Apr. 337(4):285-6. [Medline].

  4. Malecki R, Zdrojowy K, Adamiec R. Thromboangiitis obliterans in the 21st century-A new face of disease. Atherosclerosis. 2009 Feb 12. [Medline].

  5. Salimi J, Tavakkoli H, Salimzadeh A, Ghadimi H, Habibi G, Masoumi AA. Clinical characteristics of Buerger's disease in Iran. J Coll Physicians Surg Pak. 2008 Aug. 18(8):502-5. [Medline].

  6. ICD10Data.com. 2014 ICD-10-CM Diagnosis Code I73.1. Available at http://www.icd10data.com/ICD10CM/Codes/I00-I99/I70-I79/I73-/I73.1. Accessed: May 2, 2014.

  7. Abyshov NS, Zakirdzhaev EA, Aliev ZM. [Modern aspects of diagnostics and treatment for thromboangiitis obliterans]. Khirurgiia (Mosk). 2009. 75-9. [Medline].

  8. Olin JW, Young JR, Graor RA, Ruschhaupt WF, Bartholomew JR. The changing clinical spectrum of thromboangiitis obliterans (Buerger's disease). Circulation. 1990 Nov. 82(5 Suppl):IV3-8. [Medline].

  9. Motukuru V, Suresh KR, Vivekanand V, Raj S, Girija KR. Therapeutic angiogenesis in Buerger's disease (thromboangiitis obliterans) patients with critical limb ischemia by autologous transplantation of bone marrow mononuclear cells. J Vasc Surg. 2008 Dec. 48(6 Suppl):53S-60S; discussion 60S. [Medline].

  10. Kulkarni S, Kulkarni G, Shyam AK, Kulkarni M, Kulkarni R, Kulkarni V. Management of thromboangiitis obliterans using distraction osteogenesis: A retrospective study. Indian J Orthop. 2011 Sep. 45(5):459-64. [Medline]. [Full Text].

  11. Papa MZ, Rabi I, Adar R. A point scoring system for the clinical diagnosis of Buerger's disease. Eur J Vasc Endovasc Surg. 1996 Apr. 11(3):335-9. [Medline].

  12. Graziani L, Morelli L, Parini F, Franceschini L, Spano P, Calza S, et al. Clinical Outcome After Extended Endovascular Recanalization in Buerger's Disease in 20 Consecutive Cases. Ann Vasc Surg. 2012 Apr. 26(3):387-95. [Medline].

  13. Lawrence PF, Lund OI, Jimenez JC, Muttalib R. Substitution of smokeless tobacco for cigarettes in Buerger's disease does not prevent limb loss. J Vasc Surg. 2008 Jul. 48(1):210-2. [Medline].

  14. Melillo E, Grigoratos C, De Sanctis F, Spontoni P, Nuti M, Dell'Omodarme M, et al. Noninvasive Transcutaneous Monitoring in Long-Term Follow-Up of Patients With Thromboangiitis Obliterans Treated With Intravenous Iloprost. Angiology. 2014 Jul 8. [Medline].

  15. Tavakoli H, Salimi J, Rashidi A. Reply: "Treatment-of-choice for Buerger's disease (thromboangiitis obliterans): still an unresolved issue". Clin Rheumatol. 2008 Jun. 27(6):813. [Medline].

  16. Saito S, Nishikawa K, Obata H, Goto F. Autologous bone marrow transplantation and hyperbaric oxygen therapy for patients with thromboangiitis obliterans. Angiology. 2007 Aug-Sep. 58(4):429-34. [Medline].

 
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Feet of patient with thromboangiitis obliterans (Buerger disease). Note ischemic ulcers on distal portion of left great, second, and fifth toes. Although patient's right foot is normal in gross appearance, angiography demonstrated compromised arterial flow to both feet.
Superficial thrombophlebitis of great toe in patient with thromboangiitis obliterans (Buerger disease).
Tobacco smoke stains on male patient's fingers suggest diagnosis of thromboangiitis obliterans (Buerger disease). Patient presented with small, painful ulcers on tips of thumb and ring finger.
Lower-extremity arteriogram of peroneal and tibial arteries of patient with thromboangiitis obliterans (Buerger disease) demonstrates classic findings of multiple small and medium-sized arterial occlusions with formation of compensatory "corkscrew collaterals."
Table 1. Scoring System for Diagnosis of Thromboangiitis Obliterans [11]
Positive Criterion Positive Points
Age at onset < 30 y (+2)



30-40 y (+1)



Foot intermittent claudication Present (+2)



By history only (+1)



Upper extremity Symptomatic (+2)



Asymptomatic (+1)



Migrating superficial thrombophlebitis Present (+2)



By history only (+1)



Raynaud phenomenon Present (+2)



By history only (+1)



Angiography; biopsy If typical, both (+2)



Either(+1)



Negative Criterion Negative Points
Age at onset 45-50 y (−1)



>50 y (−2)



Sex; smoking Female (−1)



Nonsmoker (−2)



Location Single limb (−1)



No lower extremity involved (−2)



Absent pulses Brachial (−1)



Femoral (−2)



Arteriosclerosis, diabetes, hypertension, hyperlipidemia Discovered 5.1-10 y after diagnosis (−1)



Discovered 2.1-5 y later (−2)



Table 2. Numerical Scores Defining Probability of Diagnosis of Thromboangiitis Obliterans
No. of Points Probability of Diagnosis of Thromboangiitis Obliterans
0-1 Diagnosis excluded
2-3 Diagnosis suspected (low probability)
4-5 Diagnosis probable (medium probability)
≥6 Diagnosis definite (high probability)
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