Article

Urgent Endovascular Treatment of a Ruptured Tibioperoneal Pseudoaneurysm in Behcet's Disease

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Abstract

Behçet's disease is an autoimmune, systemic vasculitis disease that is characterized by oral aphthous and genital ulcerations and ocular lesions. Vascular manifestations in the form of aneurysms are the main predictors of mortality and morbidity in such cases. Normally, these aneurysms are located in the pulmonary arteries, the aorta, carotid, subclavia, and the femoropopliteal sector. Open surgery presents a maximum complication rate of 50%, principally in the form of anastomotic pseudoaneurysms. We report the case of a 41-year-old man who was diagnosed with Behçet's disease 3 years before, and attended the emergency department after a 10-day history of pain and an infragenicular swelling edema in the right limb, without any previous record of trauma. An initial vascular exploration revealed a pusatile mass in the infrapopliteal region and absence of the posterior tibial pulse in the right limb. The rest of the exploration did not reveal any alterations. A Doppler ultrasound scan showed a ruptured infrapopliteal pseudoaneurysm. An emergency, selective arteriography of the popliteal artery was performed by using a contralateral femoral access approach, and a ruptured tibioperoneal pseudoaneurysm was embolized with three 5-mm metallic coils (Cook). The patient was discharged after 4 days and in the 2 years since then has remained asymptomatic. Aneurysms of the distal vessels in Behçet's disease are very infrequent and each case should be evaluated on an individual basis. Endovascular treatment is a good therapeutic alternative.

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... Twenty-two true TPTAs have been reported (51%), 1 , 2 , 4-20 while twenty-one false (pseudo) TPTAs were reported (49%). [21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37] Although the literature suggests that false aneurysms are more common in the infra-popliteal region, the same cannot be said for the TPT artery based on case reports. 3 True aneurysms were discovered at an average age of 55 years, 1 , 2 , 4-20 while false aneurysms were discovered at an average age of 49 years. ...
... 3 True aneurysms were discovered at an average age of 55 years, 1 , 2 , 4-20 while false aneurysms were discovered at an average age of 49 years. [21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37] True aneurysms presented more frequently in males (18 cases, 82%) 1 , 2 , 4-6 , 9-11 , 13-18 , 20 than in females (4 cases, 18%) 7 , 8 , 12 , 19 False aneurysms presented more frequently in males (13 cases, 82%) 22 , 24-26 , 29 , 31-37 than in females (3 cases, 18%). 21 , 27 , 28 Accounting for cases that reported a one-dimensional diameter, the average diameter for true aneurysms was 3.58 cm, 1 , 2 , 5 , 8 , 10 , 13 , 17-20 while it was 4.02 cm 22 , 26 , 27 , 29 , 30 , 32 , 34 , 36 for false aneurysms. ...
... For true aneurysms, the following etiologies have been reported: mycotic (8 cases, 36%), 1 , 6 , 9-11 , 14 , 15 atherosclerosis (6 cases, 27%), 4 , 5 , 8 , 12 , 17 idiopathic (5 cases, 23%), 2 , 5 , 7 , 13 , 20 Behcet's disease (2 cases, 9%), 16 , 18 and iatrogenic (1 case, 5%). 19 For false aneurysms, the following etiologies have been reported: mycotic (7 cases, 33%), 22 , 24 , 26 , 29 , 32 , 35 iatrogenic (6 cases, 29%), 27 , 30 , 36 blunt trauma (2 cases, 10%), 21 , 33 penetrating trauma (2 cases, 10%), 23 , 34 Behcet's disease (1 case, 5%), 25 idiopathic (1 case, 5%), 28 unspecified systemic vascular disease (1 case, 5%), 31 and vascular Ehlers-Danlos Syndrome (vEDS) (1 case, 5%). 37 Authors of many case reports included in this review have stated that the majority of false TPTAs are secondary to trauma, infection, or iatrogenic injury. ...
Article
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Infra-popliteal artery aneurysms are uncommon. While literature has begun to accumulate on these aneurysms, literature is relatively lacking with respect to the tibioperoneal trunk (TPT). We describe a case of a 71-year-old male who presented with a true, atherosclerotic TPT aneurysm associated with blue toe syndrome (BTS). Our surgical approach involved initial catheter directed thrombolysis followed by excision of the aneurysm with a reversed great saphenous vein (GSV) interposition graft. A review of the literature suggests that this is the first report of its kind. We also present a comprehensive review of the published literature on TPT.
... 9,10 The most related predictor of this disease morbidity and mortality is arterial aneurysm. 11,12 The most common sites of aneurysmal involvement are as follows: Pulmonary arteries, thoracic and infrarenal segments of aorta, carotid, subclavian, femoral and popliteal arteries. Distal vessels involvement and complications are very infrequent. ...
... 17,22,23 Chance of aneurysmal rupture in Behcet' s disease is not always related to the size of the aneurysm; whereas, delay in the treatment has more important role. 11 For this reason, early diagnosis and treatment of pulmonary aneurysm is associated significantly with decreased rate of morbidity and mortality. 24 Hamuryudan et al. reported that the survival rate of patients with pulmonary aneurysm was 62%. 25 ESR and chest X-ray are the best factors for evaluation of pulmonary aneurysm and response to treatment. ...
... 12,26 There are two policy for treatment of Behcet' s disease with aneurysmal manifestations; surgical and medical managements. 11 In acute phase, the surgical operation is not recommended due to the increased rate of thrombosis and suture dehiscence. 10 The cornerstone of its treatment is administration of corticosteroid as a pulse therapy followed by corticosteroid and cyclophosphamide with or without anticoagulant drugs as maintenance treatment. ...
Article
Full-text available
Behcet's disease is a multi-systemic inflammatory disorder with cutaneous acneiform eruptions, orogenital aphthae, uveitis, arthritis and systemic vascular inflammation. One of the rare vascular manifestations is thoraco-abdominal aortic and pulmonary aneurysm that is associated with high risk of morbidity and mortality. We report a 36-year-old man with chronic cough, hemoptysis, significant weight loss, and orogenital ulcers from one year before referral. Initial assessments revealed multiple parahillar nodules in chest X-ray, chronic inflammatory anemia, erythrocyte sedimentation rate more than 100, and positive Human Leukocyte Antigen B5 and B51. Evaluation for infection and malignancies was unremarkable. Open exploratory lung study showed multiple pulsatile nodules in both lungs. AMIGO computed tomogram confirmed multiple right and left pulmonary artery aneurysms and impending to rupture aneurysm at subdivision of inferior mesenteric artery. After beginning of three methylprednisolone and cyclophosphamide pulse doses, the clinical aspect of the patient dramatically improved. Although pulmonary aneurysm is a rare manifestation of Behcet's disease and it is more infrequent in the distal branches, it can be seen in patients presenting with inflammatory disease and respiratory manifestations and with Behcet's disease diagnosis. Corticosteroid pulse-therapy could be considered as the first line of medical treatment in these patients.
... 9,10 The most related predictor of this disease morbidity and mortality is arterial aneurysm. 11,12 The most common sites of aneurysmal involvement are as follows: Pulmonary arteries, thoracic and infrarenal segments of aorta, carotid, subclavian, femoral and popliteal arteries. Distal vessels involvement and complications are very infrequent. ...
... 17,22,23 Chance of aneurysmal rupture in Behcet' s disease is not always related to the size of the aneurysm; whereas, delay in the treatment has more important role. 11 For this reason, early diagnosis and treatment of pulmonary aneurysm is associated significantly with decreased rate of morbidity and mortality. 24 Hamuryudan et al. reported that the survival rate of patients with pulmonary aneurysm was 62%. 25 ESR and chest X-ray are the best factors for evaluation of pulmonary aneurysm and response to treatment. ...
... 12,26 There are two policy for treatment of Behcet' s disease with aneurysmal manifestations; surgical and medical managements. 11 In acute phase, the surgical operation is not recommended due to the increased rate of thrombosis and suture dehiscence. 10 The cornerstone of its treatment is administration of corticosteroid as a pulse therapy followed by corticosteroid and cyclophosphamide with or without anticoagulant drugs as maintenance treatment. ...
... In thoracoabdominal aorta, the abdominal aorta shows more frequent aneurysm development than the thoracic aorta. Unusual aneurysms and pseudoaneurysms like ulnar, celiac, subclavian, left anterior descending, tibioperoneal , iliac, and superior mesenteric artery aneurysm were reported as rare manifestations of BD124125126127128129. Pulmonary artery aneurysm (PAA) with an associated rate of 1%–10% of patients in BD is the most common pulmonary lesion [130]. ...
... In some cases, ligation of affected vessel (mostly peripheral ones) is mandatory even in the cases of pulmonary artery [154, 155]. Endovascular approach is a minimally invasive technique for aneurysm repair and is growingly advised by some authors [125, 134, 156]. In BD, this technique has less complications and better results comparing to atherosclerotic lesions [157]. ...
... Other approach to some kinds of aneurysms is percutaneous embolization of individual aneurysm using substances like n-butyl cyanoacrylate [160] . The most frequently encountered postoperative complication was anastomotic pseudoaneurysm [125, 161]. Percutaneous transluminal stent angioplasty was successfully used by Han et al. in BD complicated with Budd- Chiari syndrome [162]. ...
Article
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Behcet's disease (BD) is the only systemic vasculitis involving both arteries and vein in any sizes. It frequently encounters in rheumatology clinics. It has some major morbidities and even fatal outcomes in some cases. The aim of this paper is to analyze the main concepts on pathophysiology and treatment options in BD, focusing on cardiovascular aspects, thrombosis, and potential future treatment.
... 4 Não existe exame laboratorial específico para a doença de Behçet, mas a presença do gene HLAB-51 é sugestiva da doença. 2,[4][5][6] As artérias mais acometidas com a formação de aneurismas são as artérias pulmonar, femoral, ilíaca, aorta e poplítea. Os principais achados patológicos na parede do aneurisma são: espessamento da adventícia, fibrose, infiltração linfocítica perivascular, diminuição das fibras musculares e elásticas da camada média e aumento das células esponjosas e fibroblastos da camada íntima. ...
... O tratamento cirúrgico na fase aguda da doença é associado a maiores taxas de trombose e deiscência de sutura. 5 Sugere-se que o uso associado de corticoides e imunossupressores, e ...
... 4 There is no specific laboratory test for Behçet's disease, but the presence of the HLAB-51 gene is suggestive of the disease. 2,[4][5][6] The arteries most affected by the formation of aneurysms are the pulmonary, femoral, iliac, aorta, and popliteal arteries. The main pathological findings in the aneurysm wall are: thickening of the adventitia, fibrosis, perivascular lymphocytic infiltration, decrease in muscle and elastic fibers in the media layer, and increase in spongy cells and fibroblasts in the intimal layer. ...
... Surgical treatment in the acute phase of the disease is associated with higher rates of thrombosis and suture dehiscence. 5 It is suggested that the combined use of corticosteroids and immunosuppressants, and possibly the use of antiplatelet agents or anticoagulants, may be effective in reducing complications associated to the procedures. 4 Aneurysms and pseudoaneurysms of the distal arteries are infrequent in these patients and each case must be evaluated individually. ...
... The aorta is the most commonly affected artery followed by the pulmonary artery. Aneurysms occur more frequently in the abdominal aorta than in the thoracic aorta [21][22][23]. ...
... Surgical repair is the standard treatment for aneurysms with dilatation, stenosis, or obstruction, while alternative treatments involve minimally invasive endovascular therapy for aneurysm repair [22,24,25]. [26]. ...
Article
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Background Behcet’s disease (BD) is a systemic vasculitis characterized by oral and genital aphthosis, and ocular and skin lesions. The disease is involved in vascular, gastrointestinal, and central nervous systems. Vasculitis may exacerbate fatal problems, such as anastomotic pseudoaneurysms. If the mesenteric vessels are involved, severe abdominal symptoms such as intestinal obstruction may occur. Case presentation This case report describes a young female patient who suffered from BD with recurrent abdominal aortic pseudoaneurysms, as well as deep venous thrombosis and subsequent complications of incomplete intestinal obstruction. This patient first underwent stent grafting, which was followed by rupture of two newly formed anastomotic pseudoaneurysms within six months. Emergency open surgical repair (OSR) was then performed on the ruptured pseudoaneurysms. Thrombosis and incomplete ileus occurred five months after surgery. This case was unique due to the presence of incomplete intestinal obstruction being the possible main complaint for a patient with Behcet’s disease, and it is the first ever case to be reported. Conclusion Intestinal obstruction may present as the possible main complaint in BD. Careful and attentive strategy should be carried out to prevent fatal outcomes.
... There are only a few reports on treating ruptured PAA secondary to BD in the literature. [13][14][15][16] All of these cases have undergone emergent ET with the exception of 1 case in which a ruptured popliteal artery aneurysm was diagnosed. 14 This case was also treated with immunosuppressives and closely followed for 5 days prior to ET. ...
Article
Full-text available
Behcet’s disease (BD) is a vasculitic condition that affects arteries and veins of all sizes. Arterial pseudoaneurysm is a rare but life-threatening complication of BD. We reported a 41-year-old man with 20 years history of BD and 5 years history of bilateral blindness. The patient presented with sudden onset of pain and swelling in his left groin and was diagnosed with the ruptured pseudoaneurysm of the left common femoral artery (CFA), which extended into the origins of superficial and deep femoral arteries. The patient was in the active phase of BD; therefore, he was initially treated with corticosteroids and cyclophosphamide pulse therapies and subsequently underwent stent graft placement. Post stenting angiography and postprocedural ultrasonography confirmed the successful exclusion of pseudoaneurysm without any evident complication. Four years of follow-up was uneventful.
... Behcet's disease (BD) frequently occurs in our daily clinical work. BD patients usually need intensive care in the late-stage of the disease because of frequently occurred cardiovascular events [1][2][3]. However, potential medical interventions to patients with BD still remain unclear. ...
... The most affected sites reported for arterial aneurysm were the aorta, pulmonary, femoral, popliteal and carotid arteries [2]. Arterial aneurysms were also associated with high CRP and ESR levels [18,19]. Also in a study by Bartlett et al., it was recommended that BD patients during exacerbation, especially men, should be screened for an abdominal aortic aneurysm. ...
Article
Full-text available
Background: Behçet’s disease (BD) is a variable vessel vasculitis and vascular involvement is one of its life threatening manifestations. Arterial involvement frequently occurs with male predominance with pseudoaneurysms being the most common presentation. Immunosuppressive therapy is the mainstay of treatment in vascular involvement. Case presentation: The case we report here is a 40 year old Iraqi BD patient with manifestations of recurrent oral and genital ulcers, bilateral anterior uveitis, and deep vein thrombosis. The pathergy test was positive. The HLA-B51 was negative, erythrocyte sedimentation rate 102 mm/1st h and C-reactive protein was 48 mg/L. After discontinuation of his medications for about 9 months, the disease presented with leg pain and swelling that was diagnosed as huge left superficial femoral artery pseudoaneurysm by Doppler ultrasonography. CT angiography revealed a 90 × 88 × 70 mm pseudoaneurysm with partial mural thrombosis. He was scheduled for emergency surgery due to severe intractable pain. he received a pulse of methylprednisolone 1 g/day for 3 days and then surgery was done in the form of exclusion, repair and femorofemoral bypass were done. Post-operatively, the patient had an uneventful course; distal pulses became palpable, pain and swelling subsided. Post-operation, prednisolone 1 mg/kg was continued and he received cyclophosphamide 750 mg intravenously. His blood homocysteine level was higher than normal 23.8 μmol/L. He was discharged with a high dose of steroid and monthly cyclophosphamide treatment. Conclusion: Arterial pseudoaneurysm is life-threatening in BD and should be kept in mind to prevent major complications. Vascular involvement in BD patients is probably associated with hyperhomocysteinemia.
... Son zamanlarda Behçet hastalığının tedavisinde bazı daha az invazif teknikler artan sıklıkta kullanılmaya başlanmıştır. 8 Anevrizma tedavisinde endovasküler girişimler, perkütan embolizasyon teknikleri, perkütan translüminal stent anjiyoplasti gibi yöntemlerin başarıyla uygulanabileceği ve komplikasyon oranlarının anlamlı derecede azaltılabileceği bildirilmiştir. 9 BEHÇET HASTALIĞINDA KALP VE DAMAR TUTULUMU: RADYOLOJİK GÖRÜNTÜLEME YÖNTEMLERİ Ayşe Gül KUNT ve ark. ...
Article
Purpose Popliteal artery aneurysms are the most common peripheral arterial aneurysms. One of their etiologies is Behçet's disease. Discussions about the best way to treat popliteal artery aneurysms in BD are still ongoing in the literature. We report a case of a patient that presented with a ruptured right popliteal artery aneurysm that had a successful endovascular treatment and was followed up for 36 months. Case report Our patient was a 59-year-old male with a medical history of hypertension, diabetes mellitus and Behçet's disease. He presented with acute limb ischemia of the right lower limb. Doppler ultrasound showed a ruptured large aneurysm of 7,2 × 5,1 cm in size in the right popliteal fossa, compressing the popliteal artery. Digital subtraction angiography revealed a ruptured saccular aneurysm with a diameter of 7 cm of the popliteal artery. After heparin was administered intravenously, a Fluency™ 6 mm × 40mm covered stent-graft was inserted. Final angiography showed a well positioned stent-graft excluding the aneurysm without any endoleak. The below-the-knee arteries were all patent. The symptoms of acute limb ischemia gradually disappeared and a surgical evacuation of the hematoma was performed five days after by internal popliteal approach. He was treated with Aspirin, Clopidogrel, Prednisolone and Azathioprine and followed up for 36 months without recurrence of symptoms. Conclusions Endovascular stent-graft insertion in popliteal artery aneurysms in Behcet's disease might be a safe alternative to surgical intervention. Immunosuppressive therapy should precede the surgical or the endovascular intervention and should be continued after the procedure to avoid postoperative early and late complications.
Article
Résumé L’atteinte artérielle au cours de la maladie de Behçet est rare et la localisation au niveau du tronc tibio-péronier est exceptionnelle. Nous rapportons le cas d’un jeune suivi pour maladie de Behçet et chez qui l’évolution vers l’angiobehçet a été inhabituelle. Le traitement médical et l’abstention thérapeutique ont été décidés suivant un faisceau d’argument.
Article
Objectives Endovascular treatment of peripheral arterial aneurysms (PAA) in patients with Behcet's Disease is controversial. We report a single institution experience of primary stent-graft repair of PAA in Behcet’s patients. Methods Behcet’s patients who had endovascular stent-graft repair of PAA at a single center between January 2012 and December 2018 were identified. Demographics, past medical history, cardiovascular risk profile, and perioperative details were analyzed. Results Twenty-two patients (mean age 38±7, 100% males) were included. The distribution of PAAs was: external iliac artery (EIA) in two (9%) patients, common femoral artery (CFA) in four (18%) patients, superficial femoral artery (SFA) in 15 (68%) patients, and above knee popliteal artery (PA) in one (5%) patient. Pseudoaneurysm was diagnosed in five (23%) patients. We used antegrade access in the majority of patients 16 (73%). Technical success was achieved in 21 (95%) patients. The mean hospital stay was 3±2 days. There were no mortalities or serious complications, and five (23%) patients experienced access site complications. The mean follow up was 23±11 months. The early primary and assisted primary patency rate was 91%. However, follow-up CTA detected stent-graft occlusion in six patients (27%). Conclusion Stent-graft for PAA in patients with Behcet’s had high early patency rate and minimally morbidity. However, the complications and re-intervention rates were high during the follow up with low surgical conversion.
Article
Purpose: Arterial involvement of Behcet disease is often found in the form of a rapidly expanding aneurysm. We report a case of successful treatment of a ruptured popliteal artery aneurysm with a stent-graft insertion. Case report: A 55-year-old male patient was admitted because of pain and swelling in the right leg that had persisted for 15 days. Computed tomography (CT) angiographyshowed a contained rupture of a large right popliteal artery aneurysm. Laboratory tests showed elevated erythrocyte sedimentation rate (ESR) and C-reactive protein level. The patient had a history of recurrent oral and genital ulceration, folliculitis, and erythema nodosum. He was diagnosed as having Behcet disease with arterial involvement. After 5 days of immunosuppressant medications, the ESR decreased. A 7 mm × 10 cm stent-graft was inserted in the right popliteal artery, and completion angiography showed the successfully excluded aneurysm. His symptoms were gradually relieved. The stent-graft was patent on CT angiography 15 months after the procedure. He has been followed up for 16 months with dual antiplatelet agents and immunosuppressive medications. Conclusion: Endovascular therapy for peripheral aneurysms in patients with Behcet disease is safe and minimally invasive. Careful followup to monitor disease progression is necessary.
Article
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Only a few cases of infrapopliteal aneurysms are reported in the literature. These are commonly associated with trauma, infection, and iatrogenic injuries and mostly present as pseudoaneurysms. We report the case of a 44-year-old man with Behçet disease and an 8-cm tibioperoneal trunk aneurysm and discuss the management options of these aneurysms.
Article
True aneurysms of the tibioperoneal trunk are rare. Given the scarcity of reports, the clinical presentation and treatment is not well defined. This is a case report of a 50-year-old male patient presenting with severe lower extremity swelling and compartment syndrome with neurological compromise secondary a tibioperoneal trunk aneurysm. He was also noted to have discrete ipsilateral popliteal and dorsalis pedis artery aneurysms. Given the location and size of the aneurysm, the severe leg swelling, and venous hypertension, aneurysmorrhaphy or aneurysm sac excision with arterial reconstruction was prohibitively dangerous. Thus, following fasciotomies, a hybrid repair utilizing a saphenous vein superficial femoral to anterior tibial artery bypass along with coil embolization of the aneurysm sac was performed. The patient recovered full function of his leg and follow-up CT angiogram demonstrated thrombosis and regression of the aneurysm sac with a patent bypass.
Article
Pseudoaneurysms remain a rare complication of knee and leg trauma. They may go unnoticed and manifest late after the initial trauma; they are often associated with arteriovenous fistula. No standard treatment is recognized. We report a case of post-traumatic pseudoaneurysm of the inferior articular artery of the knee, treated by coil embolization and a case of post-traumatic pseudoaneurysm of the anterior tibial artery associated with an arteriovenous fistula, treated with coil embolization and decompression surgery. Follow-up in both patients was satisfactory. Copyright © 2015 Elsevier Masson SAS. All rights reserved.
Article
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Article
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Behçet's syndrome is a multisystemic disease characterized by relapsing uveitis, oral and genital ulcerations, and vascular system involvement. The vascular involvement is seen as venous occlusion, arterial occlusion, and aneurysm formation in this disease, and the surgical treatment of a Behçet's aneurysm has technical difficulties. In this report, we suggest that the huge popliteal artery aneurysm in Behçet's syndrome can be treated by ligation of the popliteal artery. A 58-year-old male patient was admitted to our clinic because of an infrapopliteal great mass at the left leg. Color Doppler ultrasonography and arteriography revealed a 71 × 54 mm aneurysmal dilatation at the distal popliteal artery. Surgery did not reveal any suitable arterial formation for bypass to the distal area of the popliteal artery and tibial arteries. For this reason, we applied ligation of aneurysmal dilatation at the distal popliteal artery. The patient tolerated the operation well and had no signs of ischemia during the postoperative period. In conclusion, especially in aneurysm of arteries such as the popliteal artery, which has critical importance for maintaining distal perfusion, ligation may be a treatment method if there are no other alternatives.
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To develop evidence-based European League Against Rheumatism (EULAR) recommendations for the management of Behçet disease (BD) supplemented where necessary by expert opinion. The multidisciplinary expert committee, a task force of the EULAR Standing Committee for Clinical Affairs (ESCCA), consisted of nine rheumatologists (one who was also a clinical epidemiologist and one also a Rehabilitation Medicine doctor), three ophthalmologists, one internist, one dermatologist and one neurologist, representing six European countries plus Tunisia and Korea. A patient representative was also present. Problem areas and related keywords for systematic literature research were identified. Systematic literature research was performed using Medline and the Cochrane Library databases from 1966 through to December 2006. A total of 40 initial statements were generated based on the systematic literature research. These yielded the final recommendations developed from two blind Delphi rounds of voting. Nine recommendations were developed for the management of different aspects of BD. The strength of each recommendation was determined by the level of evidence and the experts' opinions. The level of agreement for each recommendation was determined using a visual analogue scale for the whole committee and for each individual aspect by the subgroups, who consider themselves experts in that field of BD. There was excellent concordance between the level of agreement of the whole group and the "experts in the field". Recommendations related to the eye, skin-mucosa disease and arthritis are mainly evidence based, but recommendations on vascular disease, neurological and gastrointestinal involvement are based largely on expert opinion and uncontrolled evidence from open trials and observational studies. The need for further properly designed controlled clinical trials is apparent.
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Behçet's disease is a multisystemic disorder characterized by recurrent ulcers of the mouth and genitalia and relapsing iritis. Four types of vascular lesion are recognized in Behçet's disease: arterial occlusions, aneurysms, venous occlusions, and variceal development. The incidence of vascular involvement reported in the literature ranges from 7% to 29%. The aim of this study was to determine the rate of vascular involvement in Behçet's disease at our hospital between 1983 and 1992. Of 1200 patients with Behçet's disease, 173 (14.4%) had venous manifestations and 19 (1.6%) had arterial manifestations (in some patients more than one organ was involved). In the group of patients with venous manifestations, there were 154 (12.8%) with venous thrombosis, 17 (1.4%) with vena cava superior syndrome, 5 (0.4%) with inferior vena cava syndrome, 5 (0.4%) with varices, 2 with upper extremity venous thrombosis, 1 with internal jugular vein thrombosis, 1 with cavernous sinus thrombosis, and 1 with hepatic vein thrombosis. In the arterial manifestation group there were 7 femoral, 3 abdominal, 3 popliteal, 2 iliac, 2 pulmonary, 1 axillary, and 1 carotid artery aneurysm as well as 3 arterial occlusions. We concluded that vascular surgeons dealing with young adults should bear Behçet's disease, an uncommon clinical entity, in mind.
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Traditionally, bypass grafts are at a high risk for thrombosis or anastomotic degeneration in patients with Behçet's disease. We report the successful deployment of a vein-covered stent across the neck of a ruptured peripheral arterial aneurysm, via a remote site access, with intermediate-term follow-up. Covered stents may represent an attractive alternative to open surgical bypass for the management of aneurysms in patients with Behçet's disease.
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To evaluate the feasibility, efficacy, and outcome of endovascular therapy combined with immunosuppression for the treatment of arterial pseudoaneurysms due to Behçet's disease. Eleven pseudoaneurysms (3 in the abdominal aorta, 3 in the subclavian artery, and individual lesions in the brachiocephalic artery, renal artery, common iliac artery, common carotid artery, and the descending thoracic aorta) in 9 patients with Behçet's disease were treated with 10 stent-grafts and 1 self-expanding stent. All patients with elevated erythrocyte sedimentation rate (ESR) were treated with immunosuppressive agents (azathioprine, prednisolone) before and after the procedure. Results: Endovascular treatment was successful in all cases, without major adverse events. The ESR was reduced from 42.7+/-18.0 mm/h initially to 18.6+/-12.6 mm/h after immunosuppressive therapy prior to endovascular repair. During follow-up (mean 24.1+/-14.0 months, range 6-43), 8 of 11 lesions showed complete resolution. One stent-graft to treat a postsurgical recurrent carotid artery pseudoaneurysm was occluded, and an abdominal aortic pseudoaneurysm recurred; both patients stopped their medications. ESR during follow-up was 7.9+/-4.5 mm/h. Endovascular treatment for pseudoaneurysms due to Behçet's disease is feasible and effective when disease activity is strictly controlled with immunosuppressive therapy.
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We report a case of Behcet's disease complicated by four arterial aneurysms successfully treated by coil embolisation and stent placement. Percutaneous endovascular repair offers a safe alternative to surgical management of this serious condition.
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We present our experience with surgical treatment of arterial complications in Behcet disease (vasculo-Behcet disease), and the long-term results and pitfalls of surgical treatment. Between January 1990 and January 2003, 20 consecutive patients underwent surgery to treat vasculo-Behcet disease. Most patients (17 of 20) were men, with mean age of 38.4 years. Thirty-four operations were performed in 20 patients. The operative mortality rate was 5.8% (2 patients). There were 17 emergency operations, 6 because of ruptured primary abdominal aneurysms. There were five others with critical limb ischemia, resulting in 3 amputations. All patients were followed up postoperatively on average for 44 months (range, 6 months-14 years). Two additional patients were lost to follow-up. After the initial operation 10-year survival rate was 30%, 10-year complication-free survival rate was 13%, and 5-year repeat operation-free survival rate was 26%. Although surgical intervention should be postponed until active inflammation has subsided, often this is not possible, because of the emergent nature of these problems. Most arterial complications of vasculo-Behcet disease present with a pseudoaneurysm rupture or with impending rupture. An aggressive surgical approach can be life-saving in such instances, and should be undertaken regardless of long-term complications, which are more common when the operation is performed in the presence of active inflammation. Early and late results can be improved by individualizing, selecting a disease-free area for reconstruction, and eliminating use of autologous graft material.
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Behcet's disease is a rare multisystemic chronic autoimmune disorder characterized by a classic triad of urogenital ulcerations, chronic eye inflammation, and skin lesions. We report on a case of a spontaneous pseudoaneurysm of the superficial femoral artery caused by Behcet's disease that was treated with an endovascular stent-graft followed by percutaneous drainage. We emphasize the use of percutaneous drainage of the pseudoaneurysm to decrease compression on the stent-graft and native vessel.
Article
Behcet's disease was first defined by Hulusi Behcetin 1937 as a multisystemic disorder with characteristic pathologic findings including recurrent orogenitalulcers, vascular disorders, and ocular and cutaneous lesions. The disease is mostly encountered at the third and fourth decades of life and especially prevalent in Mediterranean and Far East Asia.Behcet's disease may have both venous and arterial manifestations. The arterial form is rare but it is bore with its manner and to consider recurrent surgical treatments. In this retrospective study, we reviewed the literature related to Behcet's disease and discussed in respect to vascular involvement and the prognosis of surgical treatment.Thirty-one consecutive patients with vasculo-Behcet's disease visiting the departments of cardiovascular surgery, dermatology, and internal medicine in Medical Faculty of Istanbul have been reviewed retrospectively. All available clinical,pathologic, and postoperative data were reviewed and analyzed. Venous system pathologies were present in 15 patients. The pathologies in the remaining patients were related to the arterial tree. Ten patients with aneurysms and/or pseudoaneurysms and six patients with occlusive lesions have undergone surgical treatment. Pseudoaneurysms occurred in six patients at least twice or more. Vascular manifestations are the main predictors of mortality and morbidity in Behcet's disease. Arterial complications may lead to difficult surgical problems.Any invasive methods to arterial system may cause pseudoaneurysms. Repairs by using autogenous veins or synthetic grafts may lead to anastomotic false aneurysms. Surgical treatment should not to be applied in the acute phase of the disease and systemic therapy including colchicine, aspirin,and corticosteroids should be considered for all patients.
Vascular involvements in Behçet’s disease: 8 year-audit.
  • Kuzu M.A.
  • Ozalsan C.
  • Koksoy C.
  • Gurler A.
  • Tuzuner A.
Kuzu MA, Ozalsan C, Koksoy C, Gurler A, Tuzuner A. Vascular involvements in Behç's disease: 8 year-audit.