BUERGERS DISEASE

(Group 1)

BRIEF DESCRIPTION Buerger's disease (thromboangiitis obliterans) is a rare disease of the arteries and veins in the arms and legs. In Buerger's disease, your blood vessels become inflamed, swell and can become blocked with blood clots (thrombi). This eventually damages or destroys skin tissues and may lead to infection and gangrene. Buerger's disease usually first shows in the hands and feet and may eventually affect larger areas of your arms and legs. Buerger's disease is rare in the United States, but is more common in the Middle East and Far East. Buerger's disease usually affects men younger than 40 years of age, though it's becoming more common in women. Virtually everyone diagnosed with Buerger's disease smokes cigarettes or uses other forms of tobacco, such as chewing tobacco. Quitting all forms of tobacco is the only way to stop Buerger's disease. For those who don't quit, amputation of all or part of a limb may be necessary. The first reported case of thromboangiitis obliterans was described in Germany by von Winiwarter in an 1879 article titled "A strange form of endarteritis and endophlebitis with gangrene of the feet. A little more than a quarter of a century later, in Brookline, NY, Leo Buerger published a detailed description of the disease in which he referred to the clinical presentation of thromboangiitis obliterans as "presenile spontaneous gangrene. The paper discussed the pathological findings in 11 limbs amputated from Jewish patients with the disease. SYMPTOMS Extreme sensitivity to heat and cold Pain in the digits due to ischemia Cyanotic and ruddy Nails beds thicken Peripheral pulses become weaken and thread Skin may have blackish ulcerations Intermittent claudication is a hallmark symptom, identified by cramps in the legs after exercise. Laboratory and diagnostic study findings Doppler ultrasonography findings are diminished or absent compared with those for opposite leg Phlebography (venography) shows an unfilled segment of the vein in an otherwise completely filled vein with its connecting collaterals, this test is generally most indicative in diagnosing venous thrombosis.

CAUSES It isn't clear what triggers Buerger's disease. RISK FACTORS Unknown Autoimmune vasculitis Genetics Men between 20 and 35 years of age Smoking and chewing tobacco PATHOPHYSIOLOGY Smoking is very closely related to Buerger's disease and smoking history is one of the criterion for diagnosing the disease. In general if the patient absolutely abandons smoking the course of the disease will be invariably benign, but if smoking continues any treatment will ultimately be futile. Though "passive smoking" has adverse effect on cardiovascular system, non smokers should never develop the disease. Active smokers can be indentified by measuring levels of continine, the major metabolite of nicotine in urine. Since all smokers do not develop the disease an immunopathogenesis is considered probable. It has been proposed in Japanese that presence of a gene linked to some HLA antigens might control the susceptibility to the disease. Socioeconomic conditions, work environment may also play in etiology as the disease is seen more in out door and manual workers. Hypercoagulable state has been observed in association with the disease. Hepatitis B Virus and rickettssiosis may contribute to pathogenesis, but this role is uncertain. Buergers disease is an inflammatry occlusive disease which involves all layers of medium sized and small arteries of the extremitiles. Involved superficial veins bear a close resemblance to those in the affected artery. Majority of the patients develop critical limb ischemia with trophic lesions are distal to ankle, the anklebrachial doppler index could be normal in early stage. Toe pressures can be measured and if it is less than 30 mm Hg, the healing of ulcers is unlikely. The disease though commences peripherally, may gradually extend proximately occluding the larger arteries. Clinical Presentation : varies with the stage of the disease. The patients may present with foot claudication and later with calf claudication. Gangrene and ulceration may follow the above symptoms, but many times they may occur without previous history of claudication. The stepwise progession of the disease as seen in atherosclerasis may not occur in Buergers disease. Parasthesia, coldness and skin colour changes are common complaints. Dependent rubor and slow venous filling are commonly seen. Gangrene and ulceration usually follow minor trauma and with development of secondary infection they may progress proximally and are associated with intolerable rest pain.

Recurrent superficial thrombophlebitis marked by redness and tenderness over the affected vain, can occur in arm, leg or the foot. The symptoms usually disappear over 2-3 weeks, leaving behind blackish-brown pigmentation. "Phlebitis migrans" is characteristic of Buergers disease, but is often missed both by the patient and the doctor. Of 255 patients treated by shionaya from an institution in India 98% were males. The major presenting symptoms were. 1. Parasthesia, Coldness, Cyanosis - 37% 2. Gangrene or Ulcer - 18% 3. Foot Claudication - 15% 4. Calf Claudication - 16% 5. Rest pain - 10% 6. Thrombophlebitis - 3% On continuous follow up-72% develop Ulcer/Gangrene, 42% develop phlebitis migrans and about 90% eventually have upper extremity involvement. In the above series 83% had 3 or 4 limb involvement and 17% had 2 limb involvement and NONE had a single limb involvement. In our experience with about 80 patients with Buergers disease, we have not seen a single patient without ulcer or gangrene! The criteria for diagnosis of Burgers disease include (1) History of smoking (2) Onset before the age of 50 years (3) Infrapopliteal arterial occlusive disease (4) Either upper limb involvement or phlebitis migrans (5) Absence of atherosclerotic risk factors other than smoking. Arteriographic findings serve as supporting evidence and will be discussed later.
(Inflammation occurs, and the vessels are prone to spasms and constriction. Inflammatory lesions appear in healthy isolated segments of normal vessels walls, which often occlude blood flow. Scarring, fibrosis, and thrombophlebitis occur, which develops into adhering of the vessels and nerves. Soft tissue and skin cells experience hypoxia, which leads to anoxia and tissue necrosis. Nail beds thicken, and peripheral pulses become weak and thread. As Buergers disease progresses, pain occurs due to tissue death. Skin sloughs, ulcers form, and the extremity is at risk for gangrene.)

DIAGNOSTIC TEST While no tests can confirm whether you have Buerger's disease, your doctor will likely order tests to rule out other more common conditions or confirm suspicion of Buerger's disease brought on by your signs and symptoms. Tests may include:

Blood tests Blood tests to look for certain substances can rule out other conditions that may cause similar signs and symptoms. For instance, blood tests can help rule out scleroderma, lupus, bloodclotting disorders and diabetes, along with other diseases and conditions. The Allen's test Your doctor may perform a simple test called the Allen's test to check blood flow through the arteries carrying blood to your hands. In the Allen's test, you make a tight fist, which forces the blood out of your hand. Your doctor presses on the arteries at each side of your wrist to slow the flow of blood back into your hand, making your hand lose its normal color. Next, you open your hand and your doctor releases the pressure on one artery, then the other. How quickly the color returns to your hand may give a general indication about the health of your arteries. Slow blood flow into your hand may indicate a problem, such as Buerger's disease. Angiogram An angiogram, also called an arteriogram, helps doctors see the condition of your arteries. A special dye is injected into an artery, after which you undergo X-rays or other imaging tests. The dye helps to delineate any artery blockages that show up on the images. Your doctor may order angiograms of both your arms and your legs even if you don't have signs and symptoms of Buerger's disease in all of your limbs. Buerger's disease almost always affects more than one limb, so even though you may not have signs and symptoms in your other limbs, this test may detect early signs of vessel damage. Duplex Scanning : is useful in initial evaluation of the patient. Since these patient may have multiple "skip" lesions and also have significant distal disease, arteriogram is a must before any interventional therapy is planned. Arteriogram remains the "gold standard" for evaluation of arterial occlusive disease. Digital subtraction angiography is better in evaluation of distal occlusions. As mentioned above multisegmental occlusion of distal limb arteries are characteratic of Buergers disease. There is usually an extensive network of collaterals and these have a "corkscrew" or "root like oppearance". About 60% of patents have occulusion of infrapopliteal arteries, about 32% have involvement of femoral popliteal segment and about 8% have aortoiliac disease. Arterial thromboses can extend proximally in aortic lesions causing infrarenal occlusion of the aorta. Involvement of other arteries like visceral vessels, coronary arteries are extremely rare and hence these patients have a normal life span. NURSING RESPONSIBILITIES Patient teaching, instruct the patient to do the following several times a day: Lie flat on a bed with both legs elevated above the level of the heart for two to three minutes.

 Next sit on the edge of the bed with the legs dependent for three minutes Then exercise the feet and toes by moving them up, down, inward, then outward. Lastly, return to the first position and hold for five minutes. Provide for ulcer debridement and healing Remove dead or damaged material from the wound, using wet-to-dry dressing with saline solution and coarse-mesh gauze filled with cotton. Use whirlpool therapy to debride the ulcer bed. Consider using an enzymatic debrider to aid removal of debris. Provide additional intervention to promote venous return and healing, maximize comfort and provide client education for measures to prevent venous stasis ulcer. Administer medications which may include antibiotics. The patient is encouraged to make the lifestyle changes necessitated by the onset of a chronic disease, including pain management and modifications in diet, activity, and hygiene (skin care). The nurse assists the patient in developing and implementing a plan to stop using tobacco. NURSING DIAGNOSIS
Ineffective peripheral tissue perfusion related Pain related to diminished oxygen flow to Fear and anxiety related to actual or potential serious complications. to the impaired affected circulation. extremity.