BUERGER Diseases
(Thromboangitis obliterans)
PREFACE
Actually, Buerger's disease (thromboangitis obliterans) is a peripheral vascular occlusive disease is more common in Asia than in Western countries. This disease is an idiopathic disease, probably due to an autoimmune disorder of blood vessels,pan-angitis which results ultimately stenosis and occlusion of blood vessels.
The first report thromboangitis obliterans has been described in Germany by von Winiwarter in 1879 in an article titled "A strange form of endarteritis and endophlebitis with gangrene of the feet ". Approximately a quarter of a century later, in Brookline New York, Leo Buerger published a more complete explanation of the disease where it is more focused on the clinical picture of thromboangitis obliterans as " presenile spontaneous gangrene ".
Almost 100% of cases thromboangitis obliterans (sometimes called Tromboarteritis obliterans) or Buerger disease Winiwarter attack at the age of young adult smokers. The disease is widely available in Korea, Japan, Indonesia, India and other countries in South Asia, Southeast Asia and East Asia.
The prevalence of Buerger's disease in the United States has declined over the last half decade, it is certainly due to the declining number of smokers, and also due to better diagnostic criteria. In 1947, the prevalence of this disease in the United States as many as 104 cases of 100 thousand population. The latest data, the prevalence of the disease is estimated at 12.6 - 20% of cases per 100,000 population.
Deaths caused by Buerger's disease is still rare, but in patients with this disease who continue smoking, 43% of patients have to do one or more amputations in 6-7 years later. Recent data, in December 2004 issued by the CDC publication, a total of 2002 deaths were reported in the United States based on the cause of death, month, race and gender (International Classification of Diseases, Tenth Revision, 1992), has reported a total of nine deaths berhubungkan with thromboangitis obliterans, a comparison of men and women is 2:1 and the white and black ethnic groups is 8:1.
ANATOMY OF BLOOD VESSELS DIRECTION
Blood vessels consist of three types: arteries, veins, and capillaries.
A. Arteries
Arteries carry blood from the heart and distributed to various tissues of the body through its branches. Arteries of the smallest, less than 0.1 mm in diameter, called arterioles. Union of the branches of the artery is called anastomosis. There are no valves in the arteries.
And the anatomic arterial blood vessels that branches do not hold a terminal anastomosis with the arterial branches that memperdarahi adjacent areas. End fusngsional arteries are blood vessels that entered its terminal branches of the anastomosis with the terminal branches of adjacent arteries, but the magnitude of the anastomosis is not enough to keep the network alive if one clogged artery.
2. Vena
Veins are blood vessels that drain blood back to jantng; many veins have a pole. The smallest veins are called venules. Smaller vein or its branches, which unite to form larger veins, which are often united with one another to form the plexus of veins. Profunda artery type is often accompanied by two veins each of its sides, and called venae cominantes .
3. Capillary
Capillaries are microscopic vessels that make up the fabric that connects arterioles to venules. In some areas of the body, especially at the ends of the fingers and thumb, there is a direct connection between arteries and veins without capillaries mediated. Relationship like this place called arteriovenous anastomosis .
Figure 1. Anatomy of blood vessels
H ISTOLOGI S TRUKTUR P EMBULUH D DIRECTION GENERAL
Tunica intima . is the layer in direct contact with blood. This layer is formed mainly by endothelial cells.
Tunica media . Layer that sits between the tunica media and adventitia, also called the media layer. This layer is mainly formed by smooth muscle cells and tissue and elastic.
Tunica adventitia . Is the outermost layer is composed of connective tissue.
Figure 2. Histology of blood vessels
DEFINITION
Thromboangitis obliterans or Buerger's disease (TAO) is a chronic occlusive disease of the arteries and veins are small and medium. Especially regarding peripheral vascular inferior and superior extremities. Disease of the arteries and veins are to be segmental in the limbs and rarely on the tools.
Thromboangitis obliterans disease is a disorder that started the occurrence of vascular obstruction in the hands and feet. Blood vessels from constricting or in part is due to obstruction by a clot, thereby reducing inflammation and blood flow to tissues.
Figure 3. Buerger's Disease
E TIOLOGI
The cause is not clear, but there is usually no familial factors and nothing to do with Diabetes Mellitus. This disease most commonly heavy smokers who started smoking at a young age, sometimes at school age. Smoking cessation provide improvements to the disease.
Although the cause of Buerger's disease is unknown, a close connection with the use of tobacco can not be denied. use and impact of tobacco plays an important role in initiating and developing the disease. Almost the same with other autoimmune diseases, thromboangitis obliterans may have a genetic predisposition without a direct cause gene mutations. Most researchers suspect that the disease is an immune-mediated endarteritis immune system.
P ATOGENESIS
Buerger's disease is actually spread the mechanism is unclear, but several studies have indicated an implication that started the phenomenon of immunological dysfunction of the blood vessels and the area around the thrombus. Patients with this disease show hypersensitivity to intradermal injection of extracts of tobacco, increasing highly sensitive cells on collagen type I and III, increased serum antibody titers of anti-endothelial cells, and endothelial damage vasorelaksasi bound peripheral vasculature. Increased prevalence of HLA-A9, HLA-A54, and HLA-B5 are monitored in these patients, who allegedly has a genetic disease.
Ischemia caused by blood vessels (especially the inferior extremity), pathological changes will occur: (a) muscle to atrophy or fibrosis experience, (b) the bones have osteoporosis and if gangrene arise then there is destruction of bone that develop into osteomyelitis, (c) and contractures occur atrophy, (d) skin atrophy, (e) perineural and perivascular fibrosis, (f) ulceration and gangrene which started from the fingertips.
M ANIFESTASI CLINICAL
Thromboangitis clinical picture is mainly caused by ischemia obliterans. Symptoms (symptoms) are most often is a major pain and a variety of levels. Fontaine grouping can not be used here because the pain occurs it breaks. The pain increases at night and the cold, and will decrease when the limb in a dependent state. Attacks can also be paroksimal pain and often a picture similar to Raynaud's disease. In the situation further, when it has no ulcers or gangrene, the pain is severe and persistent.
Earliest manifestations may claudication (pain when walking) is pathognomonic for the arch Buerger disease. Claudication distance is a reflection of the distal arterial occlusive disease of the plantar artery or tibioperonea. Progressive ischemic rest pain and could arise about not only your toes, but also the fingers and toes affected may show signs (sign) cyanosis or rubor, if dependent. Frequent inflammation of the nail fold and consequently paronychia. Small skin infarcts can occur, especially phalang distal pulp can progress to gangrene or ulceration of chronic pain.
Sign (sign) and symptoms (symptom) of this disease include itching and ignorant of the legs and Raynaud's phenomenon (a condition in which the distal extremities: fingers, heels, hands, legs, become white when exposed to cold temperatures). Ulcers and gangrene in the toes are common in Buerger's disease (Figure 4). Pain may be felt in the affected area.
Figure 4. Buerger's Disease Clinical
Skin changes as in other chronic arterial occlusion disease is less obvious. At first light paled skin just looks mainly at your fingertips. In phase looks further vasoconstriction which was signed (sign) i with a mixture of pale-reddish-cyanotic when gets cold stimuli. In contrast with Raynaud's disease, ischemic attack here is usually unilateral. At the touch, the skin often feels cold. In addition, arterial pulsation is low or missing a sign (sign) of physical importance.
Migrant superficial thrombophlebitis can occur months or years before symptoms appear to (symptom) occlusion Buerger disease. The acute phase showed reddish skin, a little pain, and palpable venous channels that hardens as long as a few millimeters to centimeters under the skin.This disorder often appears in several places on the extremities and lasts for several weeks. After that the former looks bumpy. Sign (sign) this does not occur in occlusive arterial disease, it is almost pathognomonic for thromboangitis obliterans.
Clinical symptoms (Symptoms) thromboangitis obliterans is actually quite diverse. Ulcers and gangrene occur at a more advanced stage and is often preceded by edema and triggered by the trauma. Area of ischemia is often demarcated the extent of the toe nails. These boundaries will blur when there is secondary infection ranging from redness to the sign (sign) cellulitis.
Figure 5 is an image of the finger Buerger's disease patients who had happened to gangrene. This condition is extremely painful and wherein a time required amputation in these areas.
Figure 5. The fingertip on Buerger's Disease
Typical course of the disease, which gradually gets worse. intermittent disease develops, stage by stage, growing phalanx after phalanx, finger by finger. Arrival of a new attack and which ones would be affected finger can not be predicted. Buerger Morbus is probably about a foot or hand, maybe both. Patients are usually terrible fatigue and sleep disturbed by pain due to ischemia.
K RITERIA D IAGNOSIS
Definitive diagnosis is often difficult obliterans thromboangitis disease if the condition is very severe disease. There are several criteria that can be used as criteria for diagnosis, although these criteria sometimes differ from one author to another.
Some of the things below can be used as the basis for the diagnosis of Buerger's disease:
A. The existence of the sign (sign) arterial insufficiency
2. Generally, young adult men
3. Heavy smokers
4. The existence of gangrene which are difficult to recover
5. Migratory history of thrombophlebitis
6. There was no sign (sign) atherosclerosis elsewhere
7. Lower extremities are usually affected
8. Definitive diagnosis by anatomic pathology
The majority of patients (70-80%) that Buerger's disease of the distal ischemic pain at rest or ischemic ulcers on the heel, foot or toes.
Figure 6. legs of patients with Buerger disease. ischemic ulcers on the toes first, second and fifth.Although this patient's right leg seemed normal, with angiography obstructed blood flow seen on both legs.
Figure 7. Tromboplebitis superficial toe in patients with Buerger's disease.
Buerger's disease also should be suspected in patients with one or more sign (sign) the following clinical:
a. Finger ischemic pain in the upper and lower extremities in young adult males with heavy smoking history.
b. Claudication distance
c. Recurrent superficial thrombophlebitis
d. Raynaud's syndrome
DIFFERENTIAL DIAGNOSIS
Buerger's disease should be differentiated from chronic atherosclerotic arterial occlusive disease. This last situation is rarely the upper extremities. Atherosclerotic occlusive disease of diabetes occur in the same distribution as thromboangitis obliterans, but usually prevents the development of neuropathy accompanying leg claudication.
SUPPORTIVE EXAMINATION
There are no specific laboratory tests to diagnose Buerger's disease. Unlike other vasculitis diseases, acute phase reactions (such as erythrocyte sediment rate and C reactive protein level) Buerger's disease patients is normal.
The recommended test for diagnosing the causes of vasculitis which includes a complete blood examination; liver function tests; determination of serum creatinine concentration, increased blood sugar levels and sediment rate, antinuclear antibody testing, rheumatoid factor, the sign (sign)-sign (sign) on the serological CREST (calcinosis cutis, Raynaud phenomenon, sklerodaktili and telangiektasis) syndrome and scleroderma, and screening for hypercoagulable, this screening includes examining antiphospholipid antibodies and homocysteine in patients with Buerger's highly recommended.
Angiogram in the upper and lower extremities can be helpful in diagnosing Buerger's disease. At angiografii was found an image of " Corkscrew "of the arteries that occurs due to vascular damage, the small arteries in the wrist and leg. Angiography also demonstrated occlusion (bottlenecks) or stenosis (stiffness) in various regions of the hands and feet.
Figure 8. To the left is a normal angiogram. Right image is an abnormal angiogram of the hand arteries is indicated by the typical picture of "Corkscrew" on the arm. Change is occurring on a small part of the blood vessels under the right arm in the picture (ulna artery distribution).
Decrease in blood flow (ischemia) on the hand can be seen on the angiogram. This situation will memgawali ulcers on the hands and pain.
Figure 9. the abnormal angiogram of the hand
Although ischemic (berkurangannya blood flow) in Buerger's disease continue to occur in the distal extremities that occurs, the disease is not spread to other organs, unlike other vasculitis diseases. When there is ulceration and gangrene in the fingers, just as other organs of lung, kidney, brain, and gastrointestinal tract are not affected. Cause this happens is unknown.
With Doppler examination can also help in diagnosing this disease, that is by knowing the velocity of blood flow in blood vessels.
On histopathologic examination, early lesions showed vascular occlusion by thrombus-containing PMN and mikroabses; thickening of blood vessel walls are diffuse. LCsi which usually shows up with recanalization lymphocyte infiltration.
Depiction of the modern methods, such as computerize tomography (CT) and magnetic resonance imaging (MRI) in diagnosis and differential diagnosis of Buerger's disease still can be a primary reference. In patients with leg ulcers are suspected thromboangitis obliterans, Allen test should be conducted to determine the blood circulation in hands and feet.
THERAPY (TREATMENT)
Therapy (treatment) of medical patients with Buerger's disease should begin an intensive effort to convince patients to quit smoking. If the patient successfully quit smoking, the disease will stop at the affected area during treatment (treatment) is given. Unfortunately, most patients are not able to stop smoking and there is always a progression of the disease. To do dilated blood vessels (dilation) with vasodilator drugs, such Ronitol given lifetime. Local wound care, including compressing the affected finger and use of proteolytic enzymes could be useful. Antibiotics are indicated for secondary infections.
Therapy (treatment) surgery for patients with Buerger include debridement of necrotic tissue or gangrenosa conservative, conservative amputation with a maximum length of protection for the fingers or extremities, and sometimes lumbar sympathectomy for palms or fingers, although sometimes rare simpatetomi bermanf a at.
Artery revascularization in these patients is also not possible until there is healing on the sick. The advantage of direct surgery (bypass) in the distal artery msih also become controversial because of the high graft failure rate. However, if the patient has Beb e few distal ischemic arteries, bypass surgery with the use of autologous vein should be considered.
Figure 10. Bypass artery
Sympathectomy can be done to reduce spasma arteries in patients with Buerger disease.Through sympathectomy can relieve pain in certain areas and wound healing ulcers in patients with Buerger's disease, but for long periods of time benefits could not be ascertained.
Lumbar sympathectomy performed by means of at least three pieces of raised sympathetic ganglion, namely Th12, L1 and L2. With this effect will be eliminated and vasoconstriction of blood vessels that are still elastic will be widened so that the foot or hand feels warmer.
Therapy (treatment) recent surgery for patients with Buerger disease (ie in patients who continue to consume tobacco) is a leg amputation without healing ulcers, gangrene is progressive, or persistent pain, and sympathectomy and other treatment failed. Hidarilah amputation if possible, but, if necessary, do the operation by way of saving the leg as much as possible.
Some of the following businesses is essential to prevent complications of Buerger's disease:
· Use footwear that protects the foot to avoid the trauma and injuries as well as heat or other chemicals.
· Take care early and aggressively in the Lula-extremist wound to avoid infection
· Avoidance of cold environment
· Avoid medications that can trigger vasoconstriction
P ROGNOSIS
Pada pasien yang berhenti merokok, 94% pasien tidak perlu mengalami amputasi, apalagi pada pasien yang berhenti merokok sebelum terjadi gangren, angka kejadian amputasi mendekati 0%. Hal ini tentunya sangat berbeda sekali dengan pasien yang tetap merokok, sekitar 43% dari mereka berpeluang harus diamputasi selama periode waktu 7 sampai 8 tahun kemudian, bahkan pada mereka harus dilakukan multiple amputasi. Pada pasien ini selain umumnya dibutuhkan amputasi tungkai, pasien juga terus merasakan klaudikasi (nyeri pada saat berjalan) atau fenomena raynaud’s walaupun sudah benar-benar berhenti mengkonsumi tembakau.
REFERENCES
1. Sjamsuhidajat.R, Wim de Jong, Textbook Surgical Sciences, Issue 2, EGC Medical Book Publishers, London, 2005.
2. Schwartz, Digest Principles of Surgery, Medical Book Publishers EGC, Jakarta, 2000.
3. Reksoprodjo Soelarto, Lecture set of Surgery Faculty of Medicine, University of Indonesia Cipto Mangunkusumo Hospital, Jakarta, 1994.
4. Doherty GM. Current Surgical Diagnosis and Treatment . USA: McGraw Hill.2006.
10. http://doctorology.net/?p=172
11. http://www.kalbe.co.id/files/cdk/files/07/Misdiagnosis
No comments:
Post a Comment