Hasan Tüzün

Management of aneurysms in Behçet's syndrome: An analysis of 24 patients

BACKGROUND The surgical therapy of Behçet aneurysms is often unsuccessful, resulting in graft occlusions, anastomoses, and/or new aneurysms. METHODS Twenty-nine aneurysms were documented in 24 Behçets patients during a period of 19 years. All patients were male, ranging in age from 20 to 53 years (mean, 35 +/- 7.3 years). The mean duration of disease was 9 +/- 5 years. There were nine abdominal aorta, four iliac, three common femoral, five superficial femoral, four popliteal, one subclavian, one carotid, and one posterior tibial artery aneurysm. In addition, in one patient an aneurysm developed from the arterialized venous conduit that had been inserted for a common femoral artery aneurysm elsewhere. Five patients were already under immunosuppressive therapy for ocular problems at the time of diagnosis. Fifteen patients received immunosuppressive therapy after operation. We performed one abdominal aneurysmorrhaphy, two iliac artery PTFE graft interpositions, two aortobiliac bypasses (PTFE), six aortic tube graft (three PTFE, three Dacron) interpositions, one avrtofemoral bypass (PTFE), two iliofemoral bypasses (PTFE), two superficial femoral artery graft (PTFE) interpositions, and three popliteal graft interpositions (one PTFE, two vein graft). Also as an initial procedure one carotid, one subclavian, four superficial femoral, one popliteal, and one posterior tibial artery were ligated. RESULTS Nineteen patients were followed up for a mean duration of 47.3 +/- 27 months (range, 1 to 108 months). The patient with a subclavian aneurysm died of massive bleeding on postoperative day 15. Four patients were lost to follow-up. In the abdominal aortic aneurysm group one patient died of gastrointestinal bleeding 4 years after the operation. Another patient from the same group died 5 years after operation without any vascular disease. In the common femoral artery group the patient with an occluded iliofemoral graft died of an exsanguinating pulmonary artery aneurysm in the first year after operation. Overall, there were five anastomotic aneurysms. In addition, after the initial operation two iliofemoral, one aortofemoral, and one popliteal interposition graft were occluded without disabling ischemia. CONCLUSIONS Aneurysms limited to the extremities could be ligated without disabling ischemia. Abdominal aortic aneurysms could be treated with tube graft insertion, giving satisfactory results. Patients could tolerate graft occlusion without major ischemia.

Pulmonary Artery Involvement and Associated Lung Disease in Behçet Disease: A Series of 47 Patients

AbstractPulmonary artery aneurysms (PAAs) are well known causes of mortality and morbidity in Behçet disease (BD). However, pulmonary artery involvement in BD is not limited to PAA; the other main type of pulmonary artery involvement is pulmonary artery thrombus (PAT), with or without associated PAA. In addition, other types of lung disease like nodules and cavities in the lung parenchyma are frequently associated with pulmonary artery involvement, and can be misinterpreted as being due to infection. We surveyed the clinical, radiologic, and laboratory characteristics and outcome of 47 BD patients with pulmonary artery involvement and the associated findings, all seen and followed at a single dedicated tertiary care center.We identified 47 (41 male, 6 female) patients in whom pulmonary artery involvement was diagnosed, who were registered in the multidisciplinary clinic at Cerrahpasa Medical Faculty between January 2000 and December 2007. Mean age at diagnosis was 29 ± 8 years, and mean disease duration to the onset of pulmonary artery involvement was 3.6 ± 4.8 years. Hemoptysis was the most common presenting symptom (79%) followed by cough, fever, dyspnea, and pleuritic chest pain. Thirty-four of 47 patients (72%) presented with PAA, including 8 with associated PAT. The remaining 13 patients (28%) had isolated PAT. Patients with isolated PAT in general have clinical features similar to patients with PAA. However, hemoptysis was less frequent and voluminous in patients with isolated PAT. Most (91%) of the patients had active disease outside the lungs when they presented with pulmonary artery involvement.Forty (85%) patients had nodules and 6 (13%) had cavities when first seen. Peripheral venous thrombosis was present in 36 of 47 (77%) patients, and intracardiac thrombi in 12 of the 36 (33%) patients. Nodules, cavities, and intracardiac thrombi were mainly present in the acute stages of pulmonary artery involvement.Pulmonary artery involvement is usually multiple, and involves mostly descending branches of the pulmonary artery. Pulmonary artery involvement may disappear, but arterial stenosis or occlusions usually develop at the same location. After a mean follow-up of 7 years, 12 of 47 (26%) patients were dead; patients with larger aneurysms were more likely to die. Sixteen of 47 (34%) patients were symptom free, and the remaining 40% had mild dyspnea (13/47) and/or small bouts of hemoptysis (8/47).Pulmonary artery pressure may be elevated, and may indicate a poor prognosis. Mediastinal lymphadenopathy and mild pleural and pericardial effusions may also be observed. Corticosteroids and immunosuppressive agents are the mainstays of treatment; however, refractory cases may require embolization, lobectomy, cavitectomy, and decortication.

Surgical therapy of pulmonary arterial aneurysms in Behçet's syndrome.

Two patients with Behçets syndrome were operated for exsanguinating pulmonary artery aneurysm into a bronchus. Lower lobectomy was performed in the first patient. He died of hypoxia and sepsis on the 8th postoperative day. Pneumonectomy was undertaken in the second patient, who is well 30 months after the operation.

Surgical Treatment of Buerger's Disease: Experience with 216 Patients

Buergers disease (thromboangiitis obliterans) is a rare peripheral vascular disease that usually affects young male smokers. It is characterized by multiple occlusions distal to the knee and elbow. Although rarely encountered in this condition, major arterial occlusions can be corrected by arterial revascularization. The hospital records of 216 patients (214 men and 2 women) with Buergers disease were reviewed retrospectively, 142 (66%) between 1 and 120 (mean(s.d.) 72.8(24)) months after diagnosis. Of these patients 21 had had an arterial revascularization: 183 lumbar and 20 thoracic sympathectomies had been performed. In 30 of the patients a total of 29 minor and four major amputations had been performed. In appropriate cases arterial reconstruction provides better healing of ischaemic lesions. Although vasomotor tone is usually normalized in 2 weeks to 6 months after sympathectomy, the temporary increase in blood flow is often sufficient to heal ischaemic lesions during this period.

Management and prognosis of nonpulmonary large arterial disease in patients with Behçet disease

OBJECTIVE The purpose of this study was to evaluate and report our treatment policies in the management of nonpulmonary arterial aneurysms in Behçet disease and to assess the prognosis in a cohort of 25 patients diagnosed between 1996 and 2007 by formally reassessing their outcome at the present time. METHODS We identified 25 patients (24 men/1 woman) with Behçet disease with nonpulmonary aneurysms (n = 23) or occlusions (n = 2) between 1996 and 2007. All patients fulfilled the International Study Group Criteria for Behçet disease. Aneurysms were demonstrated with contrast-enhanced computed tomography (CT) or magnetic resonance angiography (MRA) after first-line ultrasonography. Standard surgical procedures were carried out in 22 patients. One patient with a nonruptured saccular aortic aneurysm and 2 patients with carotid aneurysms were managed only medically. For the patients with aneurysms located in the aortic bifurcation, we preferred aorto-bi-iliac bypasses; for the six extremity aneurysms, we were able to ligate the arteries; and for the other 10 extremity aneurysms we used polytetrafluoroethylene (PTFE) grafts for bypass procedures. All patients received immunosuppression with cyclophosphamide and corticosteroids before the operation and were continued in the postoperative period. All patients were examined between January and December 2010 paying special attention for new and anastomotic aneurysms and graft patency. RESULTS There was one death and 1 patient was lost to follow-up. The remaining 23 patients (92%) were under follow-up after a mean of 7.4 ± 2.9 years after their operation. Four PTFE grafts (40%) inserted for extremity aneurysms occluded with no disabling consequences. Also, 6 patients who were treated with ligation postoperatively began to complain of mild to moderate claudication. In 2 patients, aneurysms recurred at the anastomotic site, whereas in 3 patients, new aneurysms developed at other sites. CONCLUSION The surgical management of large, nonpulmonary arterial disease of Behçet disease is currently quite satisfactory. When the false aneurysm is in the infrarenal aorta, aorto-bi-iliac bypass is the preferred surgical intervention. Extremity aneurysms can be treated with synthetic graft insertion. In selected cases, ligation can give satisfactory results; however, postoperative claudication is common. In some patients with small intact saccular aneurysms, surgery may not be necessary. Patients must be prescribed immunosuppressive therapy with cyclophosphamide and corticosteroids before and after the surgical intervention in order to avoid Behçet disease activation.

Surgical treatment of Buerger's disease.

We aimed to evaluate the characteristics of 198 new patients with Buergers disease treated surgically in the last decade. We also compared these results with our former series reported in 1993. The records of patients with Buergers disease who were enrolled in an ongoing investigational protocol between 1991 and 2001 were reviewed. Sympathectomy was carried out in 161 patients and revascularization in 19 patients. The cumulative secondary patency rate was 57.9% for bypass grafts at a mean follow-up of 5.4 years. Clinical outcome following sympathectomy was considered improved in 52.3% of patients, stable in 27.8%, and worse in 19.8%. Seven major and 36 minor amputations were performed, with a limb salvage rate of 95.6%. The aggressiveness of the disease has increased compared with previous series, parallel to the expansion of cigarette consumption. Bypass surgery should be considered for patients with severe ischemia who have target vessels. Sympathectomy still has a role to improve distal flow.

Primitive Neuroectodermal Tumor of the Pancreas

Primitive neuroectodermal tumor (PNET) of the pancreas is extremely rare. Although the diagnosis of PNET is suggested by the light microscopic appearance of the tumor, it should be confirmed by the immunohistochemical evaluation of the c-myc expression and if possible, further determination of the particular chromosome translocation, t(11;22)(q24,q12). In this report, we present a male patient with pancreatic PPNET who had been followed up for 50 months. The related literature is also reviewed. In our case, the pathologic diagnosis was based on the positive immunoreactivity for CD99 in many of the tumor cells. The complementary cytogenetic studies were not possible in the private setting of the patient’s treatment. The patient was 31 years old when first operated. Within 4 months of the first operation he had local recurrence. In the third year of his follow-up he had been discovered to have pulmonary metastases and another metastatic tumor in his lung was diagnosed the year after. The metastatic foci were primarily treated by surgical resections. He had chemotherapy after each resection of pulmonary metastatic foci. After 50 months of the initial surgical intervention, he succumbed to widespread thoracic and bone metastases. Because of the extreme rarity of PPNET in the pancreas, and its rather protracted course, we think our case may further contribute to the ever expanding database for this particular entity.

Pulmonary artery involvement in Behçet׳s syndrome: Effects of anti-Tnf treatment

OBJECTIVES Anti-TNF agents are being increasingly used in patients with Behçet׳s syndrome (BS) when conventional immunosuppressives fail. However, experience with anti-TNF treatment on pulmonary artery involvement (PAI) of BS is limited. METHODS A chart review revealed 13 patients with PAI (all men) treated with anti-TNF agents (12 infliximab and 1 adalimumab) following an inadequate response to immunosuppressives for 12.2 ± 9.5 SD months and 2 male patients who developed PAI while receiving infliximab for large vein thrombosis for 10 months and for parenchymal central nervous system involvement for 2 years, respectively. RESULTS The first patient developing PAI while receiving infliximab responded to cyclophosphamide and prednisolone but the second died with hemoptysis within 1 month. At the end of the survey, 6 of the 13 patients with PAI were continuing these agents for 25.5 ± 16.2 SD months with good response, 4 stopped anti-TNF treatment after a mean of 23 ± 9.8 SD months after achieving clinical and radiologic response and 1 patient with good response went to another center after receiving infliximab for 10 months and the remaining 2 experienced serious infections (lung tuberculosis and aspergillosis) necessitating early withdrawal. Two patients relapsed within 3 years after stopping anti-TNF agents and concomitant azathioprine. One developed mesenteric vein thrombosis necessitating bowel resection and the second developed new PAI that was controlled with cyclophosphamide and prednisolone after short courses of infliximab, adalimumab, and canakinumab. CONCLUSION Anti-TNF treatment seems to be effective for refractory PAI of BS but may not prevent its development. Relapses can be seen after withdrawal. Caution is required for their serious adverse effects.

A Case of Rapidly Progressive Pulmonary Aneurysm as a Rare Complication of Behçet's Syndrome A Case Report

One of the severe complications of Behçets syndrome is pulmonary arterial involvement. The authors report a patient with Behçets syndrome who had the complications of multiple pulmonary arterial aneurysms presenting with hemoptysis and right ventricular thrombus. He underwent emergency surgery because of failure of intensive immunotherapy and died 2 months after the surgery.

Clinical and Ultrasonographic Evaluation of Lower-extremity Vein Thrombosis in Behcet Syndrome: An Observational Study.

AbstractVascular involvement can be seen in up to 40% of patients with Behcet syndrome (BS), the lower-extremity vein thrombosis (LEVT) being the most common type. The aim of the current study was to compare venous Doppler findings and clinical features between BS patients with LEVT and control patients diagnosed as having LEVT due to other causes.All consecutive 78 patients (71 men, 7 women; mean age 38.6 ± 10.3 years) with LEVT due to BS and 50 control patients (29 men, 21 women; mean age 42.0 ± 12.5 years) who had LEVT due to other causes, or idiopathic, were studied with the help of a Doppler ultrasonography after a detailed clinical examination. Patterns of venous disease were identified by cluster analyses. Clinical features of chronic venous disease were assessed using 2 classification systems. Venous claudication was also assessed.Patients with BS were more likely to be men, had significantly earlier age of onset of thrombosis, and were treated mainly with immunosuppressives and less frequently with anticoagulants. Furthermore, they had significantly more bilateral involvement, less complete recanalization, and more frequent collateral formation. While control patients had a disorganized pattern of venous involvement, BS patients had a contiguous and symmetric pattern, involving all deep and superficial veins of the lower extremities, with less affinity for crural veins. Clinical assessment, as measured by the 2 classification systems, also indicated a more severe disease among the BS patients. In line, 51% of the BS patients suffered from severe post-thrombotic syndrome (PTS) and 32% from venous claudication, whereas these were present in 8% and 12%, respectively, among the controls. Among BS patients, a longer duration of thrombosis, bilateral femoral vein involvement, and using no anticoagulation along with immunosuppressive treatment when first diagnosed were found to be associated independently with severe PTS.Lower-extremity vein thrombosis associated with BS, when compared to LEVT due to other causes, had distinctive demographic and ultrasonographic characteristics, and had clinically a more severe disease course.