Case Report: BUERGER’S DISEASE

Deusah Ezrah G Dela Rosa, MD

Department of Surgery, UP- PGH
September 3, 2010

CLINICAL ABSTRACT
Name: Magsombol, Sergio Aranas Case Number: 3595236
Age/Sex: 39 years/M
Date of Admission: 2010-08-05 Date of Discharge 2010-08-30
Primary Service TCVS Consultant: Leoncio Kaw, M.D.
Admitting Diagnosis: Chronic Peripheral arterial disease
T/C Buerger's disease, with left toe gangrene (5th digit)
Final Diagnosis: Buerger's disease with left toe gangrene 5th digit left foot
Operation: Superficial Femoral-Peroneal Artery Bypass (reversed saphenous vein graft, left)
(2010-08-24, Melendres, F A)
Findings: Superficial Femoral Artery good flow; peroneal artery with slow back bleed

History:
4 months PTC, patient accidentally slide down a
concrete pavement and wounded his left small toe which he
noted to have had purulent pus formation. Several days
after, patient experienced intermittent stabbing rest pain,
radiating to leg area and ~ 6/10 in severity; temporary
relieved by intake of Ibuprofen 500mg prn. Patient consulted
with a local hospital and ungectomy was done.
2 months PTC, there was still noted rest pain and
purulent pus formation over the affected area; patient noted
discomfort is usually relieved by a period of rest, but quickly
returns with a mild exercise. Patient sought consult with a
surgeon who prescribed the patient with Co-Amoxiclav
750mg/tab, which the patient took for 1 week 3x a day but
never provided any symptomatic relief. Duplex scan was
done revealing total occlusion of right proximal posterior
tibial artery and mild anterior tibial artery with evidence of
distal collateralization, and left distal superficial artery with reconstitution of flow in the distal
segment. No management was done.
Few days PTC, persistence of symptoms prompted consult at PGH. Hence,
admission

Past Medical History

(-) DM, HPN, CVD, BA
(-) known allergies to drug and medications
(-) illicit drug use/ previous hospitalization

Family Medical History

(+) DM- paternal side
(+) HPN and Heart Disease – maternal side/siblings

Personal and Social History

Works as a part-time painter/mason
(+) smoker ~40 pack years
(+) heavy alcoholic drinker
 Case Report: BUERGER’S DISEASE
Deusah Ezrah G Dela Rosa, MD
Department of Surgery, UP- PGH
September 3, 2010

Review of Systems
(-) weight loss/ anorexia/fever
(-) headache/dizziness/tinnitus/vertigo
(-) cough/colds/dyspnea/orthopnea/hemoptysis
(-) abdominal pain/constipation/diarrhea/melena/hematochezia
(-) dysuria/hematuria/nocturia
(-) polyuria/polyphagia/polydipsia
(-) easy fatigability/easy bruising

Physical Examination
Gen Survey: conscious, coherent, ambulatory, NICRD
Vital Signs: BP: 110/80 HR: 84 RR: 20 TEMP: afebrile
HEENT: AS, PPC, (-) CLAD, (-) NVE, (-) TPC
Chest/Lungs: SCE, (-) retractions, CBS
CVS: AP, DHS, NRRR, (-) murmur
Abdomen: soft, flat, nabs, non-tender
Skin/Extremities: PNB, (-) cyanosis/pallor, (+) weaker pulse over the lower extremities; (r<l);
(+) necrotic gangrenous left small toe; no active purulent discharge.
Neuro exam: E/N

Course in the Wards

On 1st HD, patient was admitted in the ward. Routine diagnostic exams were done
and followed up. Patient was hooked to IVF: PNSS 1L x KVO; DAT with SAP. Patient was
then referred to CVS and SAPOD for clearance.

Laboratory Results
Blood Type: "b" positive
8/6/10: CBC: wbc-11.94/ rbc-4.68/ hgb-135/ hct-0.413/ mcv- 88.2/ mch-28.8/ mchc-327/
Rdw-12.1/ plt ct. - 406/ neut-0.627/ lymph-0.234/ mono-0.098/ eos-0.036/ baso-0.005
8/11/10: PTT: 37.6/ 42.2
8/06/10: URINALYSIS: straw/clear/blood-neg/bili-neg/urobili-normal/ketones-neg/alb
neg/nitrite-neg/gluc-neg/ ph-7.5/sg-1.010/leuc-neg/
8/13/2010: AORTOILIAC AND BILATERAL FEMORAL ANGIOGRAPHY: findings
consistent with Buerger's disease

A concrete diagnosis of THROMBOANGIITIS OBLITERANS (TAO) is often difficult as

it relies heavily on exclusion of other conditions. The commonly followed diagnostic criteria
are outlined below although the criteria tend to differ slightly from author to author. Olin
(2000) proposes the following criteria:

1. Typically between 20–40 years old and male, although recently females have been
diagnosed.

2. Current (or recent) history of tobacco use

 Case Report: BUERGER’S DISEASE
Deusah Ezrah G Dela Rosa, MD
Department of Surgery, UP- PGH
September 3, 2010

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.

My patient’s salient features, after satisfying 4 out of the 6 criteria cited by Olin, highly
correlates with the signs and symptoms of Thromboangiitis Obliterans (also known as
Buerger's disease) which is a recurring progressive inflammation and thrombosis (clotting) of
small and medium arteries and veins of the hands and feet. It is strongly associated with use
of tobacco products, primarily from smoking, but also from smokeless tobacco. However, the
association of Buerger’s disease with tobacco use, particularly cigarette smoking, cannot be
overemphasized. Most patients with Buerger’s are regular smokers, but some cases occur in
patients who only smoke “moderately”; others have been reported in users of smokeless
tobacco.

While the etiology of Buerger disease is unknown, exposure to tobacco is essential for
both initiation and progression of the disease. The notion that the condition is linked to
tobacco exposure is supported by the fact that the disease is more common in countries with
heavy use of tobacco and is perhaps most common among natives of Bangladesh who
smoke a specific type of cigarettes, homemade from raw tobacco, called "bidi." While the
overwhelming majority of patients with Buerger disease smoke, a few cases have been
reported in nonsmokers that have been attributed to the use of chewing tobacco; it has been
postulated that Buerger’s disease is an “autoimmune” reaction (one in which the body’s
immune system attacks the body’s own tissues) triggered by some constituent of tobacco.

The disease mechanism underlying Buerger disease remains unclear, but a few
observations have led investigators to implicate an immunologic phenomenon that leads to
vasodysfunction and inflammatory thrombi. Patients with the disease show hypersensitivity to
intradermally injected tobacco extracts, have increased cellular sensitivity to types I and III
collagen, have elevated serum anti–endothelial cell antibody titers, and have impaired
peripheral vasculature endothelium-dependent vasorelaxation. Increased prevalence of HLA-
 Case Report: BUERGER’S DISEASE
Deusah Ezrah G Dela Rosa, MD
Department of Surgery, UP- PGH
September 3, 2010

A9, HLA-A54, and HLA-B5 is observed in these patients, which suggests a genetic
component to the disease.

Death from Buerger disease is rare, but in patients with the disease who continue to
smoke, 43% require 1 or more amputations in 7.6 years. Most recently, in a December 2004
CDC publication, the 2002 deaths report in the United States divided by cause of death,
month, race, and sex (based on the International Classification of Diseases, Tenth Revision,
1992), reported a total of 9 deaths related to TAO, depicting male to female gender ratio of
2:1 and white to black ethnicity ratio of 8:1.

Symptoms and complications of Buerger's

disease are due to inflammation of the arteries
and veins of the hands and/or feet and
development of blood clots. This creates
blockages in the blood vessels, which result in
an insufficient supply of blood, oxygen, and
nutrients to cells and tissues in the hands and/or
feet.

The initial symptoms of Buerger’s Disease often

include claudication (pain induced by insufficient
blood flow during exercise) in the feet and/or hands, or pain in these areas at rest. The pain
typically begins in the extremities but may radiate to other (more central) parts of the body.
Other signs and symptoms of this disease may include numbness and/or tingling in the limbs
and Raynaud’s phenomenon (a condition in which the distal extremities — fingers, toes,
hands, feet — turn white upon exposure to cold). Skin ulcerations and gangrene of the digits
(fingers and toes) are common in Buerger’s disease. Pain may be very intense in the affected
regions, as seen in my patient.

Despite the severity of ischemia (lack of blood flow) to the distal extremities that occurs
in Buerger’s, the disease does not involve other organs, unlike many other forms of
vasculitis. Even as ulcers and gangrene develop in the digits, organs such as the lung,
kidneys, brain, and gastrointestinal (GI) tract remain
unaffected. The reasons for the confinement to the
extremities and sparing of other organs are not known.

Buerger's is not immediately fatal, but it is life-

shortening. Amputation is common and major amputations (of
 Case Report: BUERGER’S DISEASE
Deusah Ezrah G Dela Rosa, MD
Department of Surgery, UP- PGH
September 3, 2010

limbs rather than fingers/toes) are almost twice as common in patients who continue to
smoke. Death rate has not been consistently shown as higher in patients who do not cease
smoking but for this and other health concerns quitting is highly recommended. Female
patients tend to show much higher longevity rates than men.

Buerger’s disease can be mimicked by a wide variety of other diseases that cause
diminished blood flow to the extremities. These other disorders must be ruled out with an
aggressive evaluation, because their treatments differ substantially from that of Buerger’s
disease. For Buerger’s there is no treatment known to be effective.

Diseases with which Buerger’s disease may be confused include 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, and others.

Angiograms of the upper and lower

extremities, which have been done on
my patient, are helpful in making the
diagnosis of Buerger’s disease. In the
proper clinical setting, certain
angiographic findings are diagnostic of
Buerger’s. These findings include a
“corkscrew” appearance of arteries
that result from vascular damage,
particularly the arteries in the region of
the wrists and ankles. Angiograms
may also show occlusions (blockages)
or stenosis (narrowings) in multiple
areas of both the arms and legs.

Progression of the disease may lead to 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 and often describe the Raynaud phenomenon of sensitivity of the
hands and fingers to cold. Foot or arch claudication may be erroneously attributed to an
orthopedic problem. Patients who seek medical attention late in the course of their disease
may present with foot infections and, occasionally, with florid sepsis. Although classic
Buerger disease affects the vessels of the extremities, a few cases of aortic, cerebral,
 Case Report: BUERGER’S DISEASE
Deusah Ezrah G Dela Rosa, MD
Department of Surgery, UP- PGH
September 3, 2010

coronary, iliac, mesenteric, pulmonary, and renal thromboangiitis obliterans have been
reported.
More recently a point-scoring system has been proposed by Papa to support or contest
the diagnosis of TAO using the following criteria (Table 1 & 2).

o Distal extremity (feet, toes, hands, fingers) involvement

o Onset before age 45
o Tobacco use
o Exclusion of atherosclerosis or proximal source of emboli
o Lack of hypercoagulable state
o Lack of definable arteritis (i.e., progressive systemic sclerosis, giant cell
arteritis)
o Classic arteriographic findings
o Involvement of digital arteries of finger or toes
o Segmental involvement (i.e., "skip areas")
o Corkscrew collaterals
o No atherosclerotic changes
o Classic histopathologic findings
o Inflammatory cellular infiltrate within thrombus
o Intact internal elastic lamina
o Involvement of surrounding venous tissues

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)

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)

 Case Report: BUERGER’S DISEASE
Deusah Ezrah G Dela Rosa, MD
Department of Surgery, UP- PGH
September 3, 2010

Arteriosclerosis, diabetes, hypertension, Discovered after diagnosis 5.1-10 years (-1)/2.1-

hyperlipidemia 5 years later (-2)

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

Smoking cessation has shown to slow the progression of the disease and decrease the
severity of amputation in most patients, but does not halt the progression. The patient must
abstain from the use of tobacco in any form for the rest of his life. He must also avoid
extremes in temperature and any injury from poorly fitted shoes. Fungus infections of the feet
should be treated, for they will always aggravate the condition. Complete rest in bed over a
long enough period may allow
ulcerated areas to heal.
These special exercises should also
be carried out as part of the patient’s
general care: While he is lying down,
elevate the legs about ten or fifteen
degrees and keep them there for ten
seconds, then lower them over the
edge of the bed for three minutes.
Rest for two minutes with the lying
legs flat, then repeat at least ten
times, after which allow the patient to
rest. The patient must do this at least
four times a day
 Case Report: BUERGER’S DISEASE
Deusah Ezrah G Dela Rosa, MD
Department of Surgery, UP- PGH
September 3, 2010

Vascular surgery, which has been the case for our patient, can sometimes be helpful in
treating limbs with poor perfusion secondary to this disease. The use of vascular growth
factor and stem cell injections has been showing promise in clinical studies. Streptokinase
has been proposed as adjuvant therapy in some cases. In addition Limaprost, an oral PGE1
derivative, has shown efficacy in one published randomized trial.
References

1. Joyce JW (1990). "Buerger's disease (thromboangiitis obliterans)". Rheum Dis Clin North Am 16 (2): 463–70. PMID 2189162.

2. Olin JW (Sep 2000). "Thromboangiitis obliterans (Buerger's disease)". N Engl J Med 343 (12): 864–

9. doi:10.1056/NEJM200009213431207. PMID 10995867.

3. Hussein EA, el Dorri A (1993). "Intra-arterial streptokinase as adjuvant therapy for complicated Buerger's disease: early

trials". International surgery 78 (1): 54–8. PMID 8473086.

4. Spine: 15 January 2009, Volume 34, Issue 2, pp 115–120 doi: 10.1097/BRS.0b013e31818f924d "Randomized Trial The Efficacy

of Prostaglandin E1 Derivative in Patients With Lumbar Spinal Stenosis," Matsudaira, Ko; Seichi, Atsushi; Kunogi, Junichi; Yamazaki,

Takashi; Kobayashi, Atsuki; Anamizu, Yorito; Kishimoto, Junji; Hoshi, Kazuto; Takeshita, Katsushi; Nakamura,

Kozo http://journals.lww.com/spinejournal/Abstract/2009/01150/The_Efficacy_of_Prostaglandin_E1_Derivative_in.4.aspx

5. von Winiwarter F (1879). "Ueber eine eigenthumliche Form von Endarteriitis und Endophlebitis mit Gangran des Fusses". Arch

Klin Chir 23: 202–26.

6. Buerger L (1908). "Thrombo-angiitis obliterans: a study of the vascular lesions leading to presenile spontaneous

gangrene" ([dead link]). Am J Med Sci 136: 567–80.doi:10.1097/00000441-190810000-00011.

7. T. Aktoz, M. Kaplan, O. Yalcın, I. H. Atakan, O. Inci,; “Penile and scrotal involvement in Buerger's disease" (p 401-403)

published online: Nov 25 2008 8:07PM DOI: 10.1111/j.1439-0272.2008.00859.