Biomedical subjects
Publications and source records attributed to H Tüzün.
A young man was admitted to hospital with dyspnea, malaise, chest pain and night sweating. Investigative studies revealed a cystic mass lesion originating from the heart. Surgical exploration of the tumor showed that it was unresectable and pathology of the biopsy material was primitive neuroectodermal tumor. Medical literature concerning this unusual type of tumor is reviewed.
One of the severe complications of Behçet's syndrome is pulmonary arterial involvement. The authors report a patient with Behçet's 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.
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 Behçet's 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.
Two patients with Behçet's 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.
Pulmonary arterial involvement is an important complication of Behçet's syndrome (BS). Among 2179 patients with BS, 24 (1.1%) were diagnosed as having pulmonary arterial aneurysms (PAAs). Haemoptysis was the presenting symptom in all but one. All were male. The mean age at the time of the diagnosis of PAA was 30 +/- 11 S.D. yr (range 17-59 yr). Their mean disease duration was 5 +/- 4 yr (range 3 months-16 yr). There was a high prevalence of thrombophlebitis (21/24, 88%). Histopathological examination showed pulmonary vasculitis involving all layers of pulmonary arteries and veins. Twelve patients (50%) died after a mean of 9.5 +/- 11 S.D. months (range 1-36 months) after the onset of haemoptysis. The mean duration of follow-up of the remaining 12 patients was 25.5 +/- 24 S.D. months (range 1-78 months). The treatment consisted mainly of pulsed or oral cyclophosphamide alone or with prednisolone. As is true with other severe manifestations of Behçet's syndrome, PAAs are more common among males. They are associated with a prevalence of thrombophlebitis and there is high mortality despite treatment.
PURPOSE: To describe findings at radiologic evaluation of pulmonary arterial involvement in Behçet disease. MATERIALS AND METHODS: Between 1978 and 1993, 31 pulmonary arterial aneurysms were diagnosed in 15 consecutive male patients, aged 21-56 years (mean, 32 years 5 months), with known Behçet disease. The patients were assessed at chest radiography (n = 15), computed tomography (n = 14), angiography (n = 10), and magnetic resonance (MR) imaging (n = 2). RESULTS: Three patients had single and 12 had multiple pulmonary aneurysms. In six patients, aneurysms were associated with pulmonary artery occlusion. Follow-up chest radiography revealed five subjects with progression leading to fatal rupture of aneurysms and two subjects with regression of the aneurysms and cessation of their symptoms. CONCLUSION: Intravenous digital subtraction angiography was the procedure of choice in the documentation of pulmonary arterial involvement, and MR imaging was most helpful in the presumptive diagnosis of occluded aneurysms. The appearance of pulmonary aneurysms represents a poor prognosis in patients with Behçet disease, but there is a chance that the aneurysms will not rupture.
We report a case of Behçet's disease with pulmonary and splenic involvement. The radiologic presentation was quite different from the modes defined in the literature. A large, anteriorly located, well circumscribed ovoid mass was the x-ray film image of a large area of infarction with hemorrhage. The involved pulmonary artery showed signs of vasculitis with thrombus formation. To our knowledge, this mode of presentation is different from the cases reported in the literature.
In this report, 14 patients with Behçet's disease with a total of 16 aneurysms are presented. The most common site of aneurysm formation was the femoral artery. Patients with Behçet's disease and aneurysm formation carry the highest morbidity and mortality rates among all those with the disease. The results of reconstructive surgery in the treatment of aneurysms are discouraging, with many anastomotic aneurysms and grafts occluding. In this study, it was possible to ligate major arteries such as the common iliac, superficial femoral and popliteal without limb loss. It is suggested that ligature either at initial surgery or reoperation should be performed to avoid complications of reconstructive surgery. Adjuvant immunotherapy is required to control relapses responsible for new aneurysm formation.
Buerger's 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 Buerger's 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.
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