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Marfan's Syndrome is a rare connective tissue disease whose surgical treatment presents several challenges. The existing criteria and recommendations on how and when to surgically approach this disease is almost exclusively confined to the root and ascending aorta, in contrast to the variability of indications on the involvement of the remaining segments of the thoracic and abdominal aorta, due to the multiplicity, morphology and topography of these involvements. This case report is related to a 39 year old woman with Marfan's syndrome, with a type B chronic dissection and a segmentar aneurysm of the proximal descending thoracic aorta with 6,1cm of maximum diameter. The clinical and imagiologic evaluation pointed out the absence of any disorder of the heart, root or ascending aorta. The patient underwent surgical management of the dissecting aneurysm through a transthoracic approach to the aneurysm, with fenestration of the distal aorta and exclusion of the aneurism and intercostal arteries. The efficacy and result of the procedure in addition to the rarity of the vascular pathology presented by the patient, even in the context of a Marfan, completely justify the presentation and disclosure of this clinical case.

A 26-year-old woman, complained of repeated urinary infections at the beginning of February 2010. Several urine sample tests identified an Escherichia colli strain, which was treated with antibiotics, according to the antibiogram data. Shortly afterwards, as a result of a back pain, a pyelonephritis was diagnosed, which was also treated with antibiotics. The pain progressively intensified over time and in June 2011 she began to suffer from high and persistent fever, which led to her hospitalisation. The investigation through CT-angiography showed the existence of three aneurysms of the thoracic aorta (one of the aortic arch and two of the descending thoracic aorta) and a large aneurysm of the abdominal aorta, in a contained rupture, surrounding the origin of the renal arteries, of infectious etiology. The patient underwent three sequential and successful surgical operations, using the conventional approach that consisted in the resection and replacement of the aneurysms with "in situ" vascular prosthesis. The main aspects of the pathology, clinical presentation and treatment of this extensive, rare and challenging situation are presented and discussed herein.

Adult congenital aortic coarctation is an entity rarely seen in clinical practice. It is commonly diagnosed and managed in the early stages of life, mean in the neo-natal or young children's ages. Some cases however can be overlooked at this scrutinity and become recognizable at later stages, in adulthood, through symptoms and signs resulting from the deep hemodynamic deregulation caused by the disease in the proximal and distal aortic physiology, requiring often imperative therapeutic repair. In this paper, an extensive revision is made on the main pathologic, clinical and diagnostic features of the disease, culminating in a critical analysis on the contemporary therapeutic methods available, which includes the conventional open surgery and the endovascular intervention, which includes the balloon angioplasty, the stenting and the covered stents.

Purpose: This study was undertaken to assess the biologic behavior of arterial allografts used for vascular reconstruction in patients undergoing immunosuppression therapy because of a previously transplanted organ. Methods: Thirteen patients with a transplanted kidney and receiving azathioprine, cyclosporine, and prednisone therapy, received 16 ABO-compatible arterial allografts to treat vascular complications of the kidney transplantation, popliteal aneurysms, and chronic or critical ischemia of the lower extremities. One aortoiliac, one iliorenal, two iliofemoral, three femorofemoral, one femoroperoneal, and eight femoropopliteal grafts were used in this particular group of patients. The arteries were retrieved from a donor whose diagnosis was cerebral death and, after careful preliminary studies, were stored cold, with use of liquid nitroxide vapor. Results: The patients were monitored up to 45 months, mean 20 months. Graft tolerance was good, there were no signs or symptoms of acute rejection, and there was no perturbation of the immunologic tolerance of the transplanted kidney. During the follow-up period, two grafts occluded. On the basis of the histologic studies, it was not possible to relate the occlusion to rejection. Conclusions: This clinical study, undertaken for the first time in human beings, seems to confirm that immunosuppression modifies host tolerance to arterial allografts, improving their biologic behavior, a fact that was already documented in animal experimentation. (J VASC SURG 1994;20:271-8.)

The objective of this study was to evaluate the results of endovascular treatment of the superficial femoral artery and to identify some possible predictors of poor prognosis.All patients with femoropopliteal artery occlusive disease submitted to endovascular treatment in the internment section of the first author, between July 2007 to September 2010, were reviewed. Intention to treat analysis was performed. Patient's demographics and angiographic anatomic characteristics (results were standardized according to TransAtlantic Inter-Society Consensus II) were analyzed. Kaplan-Meier survival analyses were performed to assess time-dependent outcomes. We performed a univariate and bivariate analysis in order to determine factors temporally associated with a poor prognosis of endoluminal therapy. Results were evaluated in view of absence of re-stenosis, absence of re-intervention, patency and overall limb salvage rate.A total of 34 patients corresponding to 36 lower limbs were treated during this period (mean age 65±12 years; males: 80%). Seventy-five percent of patients presented with chronic lower limb ischemia stage IV according to the Leriche-Fontaine classification, while the remaining 25% presented with chronic lower limb ischemia stage III. The lesions classified according to the TASC II document included 9 lesions TASC II B (25%), 20 lesions TASC II C (55%) and 7 lesions TASC II D (19%). Runoff at the tibial level was 1.8±0.6 patent vessels. The success rate was 94%. Mean follow-up was 13.5 months (varying 1-31 months) from the date of initial intervention. Five lower limbs (14%) presented with re-stenosis. Primary patency rates were 91% at 1 month and 77% at 6 months. Limb salvage rate was 88% at 12 months. Subgroup analysis revealed that primary patency rates were highly dependent on patient gender, lesion type and infra-popliteal runoff patent vessels.Endovascular treatment of femoropopliteal sector is associated with high technical success rate. The best results were obtained in male patients with segmental, stenotic lesions and with a runoff of at least two vessels.

The clinical case of a 37 years old female with the previous diagnosis of human immunodeficiency virus infection (HIV), under medical control, is reported, in whom an extensive and large thoracoabdominal aortic aneurysm was diagnosed (type II of the Stanley Crawford's classification), completely asymptomatic. The patient underwent surgical management, consisting in the resection of the aneurysm and prosthetic replacement, utilizing the "simplified technique", that was once again extremely well succeeded in this most demanding situation. The clinical and pathological features of this infection and the potential involvement in the pathogenesis of this uncommon aneurysm are discussed, according to the data published in the literature. Furthermore, the main aspects of the surgical management are described and discussed, facing the diverse technical alternatives nowadays used in clinical practice.

Vascular Ehlers-Danlos syndrome is a rare genetic and hereditary disorder that affects and weakens the structure and sustentability of medium and large size arteries, causing severe clinical complications, often fatal, such as is the case of spontaneous ruptures, aneurysms or arterial dissections. Concomitantly, spontaneous intestinal perforations may occur, as well as uterine ruptures, following a pregnancy and during the peripartum period. The authors report the clinical cases of three young females of the same family, with the diagnosis of vascular Ehlers-Danlos syndrome, coursing with severe involvement of the aorta (ascending aortic anerysm, dissection of the thoracoabdominal aorta), who underwent successful surgical management and were evaluated from 6 to 18 years after the operations. To complete the clinical presentation of the cases, the authors review the most recent literature and discuss the main features of this entity, namely its genetic origin, the arterial morphology and structural alterations, its clinical expression, the diagnosis and the challenges faced by the therapeutical approach, wether it would be the conventional arterial reconstructive surgery or the most recently introduced endovascular intervention.

The clinical case of a 13 years old female is reported, with the diagnosis of congenital coarctation of the abdominal aorta, with involvement of renal and visceral arteries, causing arterial hypertension and a systolic bruit in the auscultation of the epigastric and umbilical areas. Conventional angiography disclosed a stenotic process of the upper abdominal aorta, with concomitant ostial stenosis of the renal and digestive arteries, and post-stenotic dilatation of the celiac axis and superior mesenteric artery. The physical development of the patient, close to the adult pattern, allowed the performance of a complex revascularization procedure,accomplished in May 1984, consisting in the implantation of an aorto-aortic bypass graft, through the diaphragm, followed by visceral and renal revascularization, by means of independent prosthesis taken from the aortic bypass. The post-operative course was uneventful and blood pressure returned immediately to normal levels. Reviewed recently, the patient was found in good clinical condition,asymptomatic and with normal blood pressure, developing a normal family, social and Professional life. An Angio-CT evaluation, performed in February 2010, almost 26 years after the operation, revealed the complex revascularization procedure patent and working in excellent condition. To our knowledge, this seems to be the longest follow up of such a procedure employed successfully in a very rare clinical entity, thus justifying its presentation and divulgation.

The celiac axis compression syndrome caused by the median arcuate ligament of the diaphragm is an exceedingly rare clinical entity, coursing with postprandial abdominal pain, nausea, vomiting and weight loss. Despite the original description made some decades ago, its existence is still challenged by some authors, due to a weak clinicopathological correlation and inconstant results obtained with its surgical management. The authors report the case of a 35 years old female with the clinical presentation above described, who underwent an exhaustive investigation in gastroenterology, leading to the diagnosis, made by CT-scans and conventional angiography, of celiac axis compression syndrome by the median arcuate ligament of the diaphragm. A definitive surgical repair was performed, consisting in the ligament division, followed by resection and replacement of the celiac axis, by a 6 mms PTFE grafts. The post-operative course was uneventful and reviewed one month later, the patient was found in excellent condition and completely free of symptoms. Pathological studies of the celiac axis disclosed significant proliferative and degenerative lesions of the arterial wall, consequence of the compression mechanism, a fact that has not been reported in previous similar publications. The main feature of this unique clinical entity and its surgical management are discussed, based on a review of the most recent publications of the literature.

The clinical case of a 39 years old female is reported, with the diagnosis of tumor of the right kidney extending into the infra-hepatic vena cava, assuming the shape of a floating thrombus. The patient underwent right radical nephrectomy, followed by resection of the intra caval tumor. Both the procedure and post operative course were uneventfull. Histopathological evaluation of the specimens, associated to specific imunohistochemistry studies, confirmed the diagnosis of angiomyolipoma of the kidney. A review of the literature concluded that this is the 27th case published of a kidney angiomyolipoma extending into the inferior vena cava, thus justifying its presentation and divulgation.

The authors report the clinical case of a 34 years old male, with a germ cell tumor of the testis, who developed large retroperitoneal metastasis, with compression and later on invasion of the inferior vena cava. The patient underwent chemotherapy followed by resection of the original tumor and of the retroperitoneal metastasis and the vena cava was replaced by a vascular prosthesis. The post operative course was uneventful and one year later he was found in excellent condition. The rarity of the situation as well as of its surgical management justifies the presentation and the discussion of the case, based on the most recent data of the literature dedicated to the topic.

Paradoxical embolism (EP) occurs when a venous thrombus passes into the arterial circulation, most commonly through an intracardiac shunt. A patent foramen ovale (FOP) is present in 25-35%of people at all ages. It is now possible to detect FOP by contrast echocardiography and it has been shown that there exists quite frequently a spontaneous transient right-to-left shunt during systole and, moreover, a right-to-left shunt during Valsalva maneuver and coughing. Acute or chronic pulmonary hypertension are also causes of shunt. This phenomenon has been associated with catastrophic outcomes such as stroke, visceral infarction and ischemic limb. We report the case of a 31-year old man with multiple paradoxical thromboemboli into his right kidney, spleen, small bowel and left brachial artery. Paradoxical embolism was suspected and confirmed by transoesophageal contrast echocardiography, disclosing FOP with right-to-left shunt. Successful percutaneous closure of patent foramen ovale with Amplatzer(R) PFO occluder 18 mm allowed subsequent oral antiaggregation with acetilsalicilic acid 150 mg/day and clopidogrel 75 mg/day. A new case gives the opportunity to review mechanisms, diagnosis and therapeutic issues. Treatment strategies for FOP in recurrent PE include antiplatelet agents, anticoagulants, surgical closure, or percutaneous closure devices. The completion of ongoing, randomized clinical trials comparing percutaneous closure devices with medical management is urgently needed to clarify if the risks of invasive endovascular device placement are outweighed by a long-term reduction in recurrent vascular events.

Aortic mural thrombosis is a rare situation in clinical practice. Its pathogeny is ignored in the majority of cases and clinical presentation is variable, according to the location of the thrombus and the multiple and diverse forms of embolism that can cause. For these reasons, clinical diagnosis may be extremally difficult. The authors report the cases of two women aged 52 and 41 years old respectivelly admitted in the Emergency Department with abdominal and lumbar pain, due splenic embolic infarctions, a diagnosis made by CT-Scans. The computed tomography disclosed also the presence of mural thrombus in the celiac aorta, one of them as a floating thrombus, which were regarded as the source of the embolism. Supraceliac aortic thrombectomy was performed, associated to splenectomy in both cases. Post operative course was uneventfull and reviewed 10 months and 9 years after the operation, they were found in pretty good condition, with no evidence of recurrence of the disease.

In the management of penetrating carotid injuries, the surgeon must act in an emergency way to prevent active bleeding and to restore cerebral blood flow. Preservation of the carotid circulation decreases morby-mortality in these patients. Controversial diagnostic and therapeutic approach remain when no signs of bleeding or neurological ischemia are evident. The authors report the case of a male patient submitted to an emergency operation to repair a internal carotid thrombosis consequence of a penetrating gunshot injury at the area II of the neck. We reviewed the literature related to this condition and the different methods of treatment that can be employed.

The clinical case of a 44-year old female is reported, whose chief complaints were arterial hypertension and disabling intermittent claudication of both legs. Angiography disclosed a segmental occlusion of the para-renal aorta, associated to an occlusion of superior mesenteric and left renal arteries and a critical stenosis of the right renal artery, probably of degenerative etiology ("coral reef"), but not comproved by pathological studies. The patient underwent a complex revascularization procedure, consisting in the implantation of a bypass graft from the supraceliac aorta to both common femoral arteries through a retropancreatic way, followed by superior mesenteric and both renal arteries revascularization by means of independent PTFE grafts attached to the main bypass graft. Post-operative course was uneventful and blood pressure returned to normal values. An angio-CT control study revealed the integrity of the revascularization procedure and the recovery of the left kidney functionality, whose artery was completely occluded. Te etiology, topography and surgical management of this most demanding situation are subjected to an analysis and discussion.

The clinical case of a 34 years old male is reported, who was referred to our clinic complaining of disabling claudication of the lower extremities. No risk factors for atherosclerotic disease could be identified. Six years previously he sustained a severe automobile crush injury, resulting in cranio-encephalic and abdominal trauma, and fractures of the left knee and both legs. Appropriate medical and surgical care were delivered and he recovered completely, with no restrictions. Six months before the observation, he started to complain of intermittent claudication of both legs. An angio-CT evaluation disclosed the diagnosis of a chronic dissection of the abdominal aorta of post traumatic etiology, causing significant restriction of blood flow to the lower extremities. The patient underwent surgical management consisting of the resection and prosthetic replacement of the abdominal aorta. The post operative course was uneventful and he became asymptomatic. A control angio-CT study revealed the graft working in excellent condition. The main features of this clinical entity are analyzed and discussed based on a clinical revision of the bibliography on the subject.

This study was undertaken to assess retrospectively the epidemiology and risk factors for amputation of combined vasculo-orthopaedic traumatic lesions, during a 18-year period, from March 1987 to May 2005, comprising the review of the clinical charts of 149 patients. The series includes a predominance of male patients (84%) with an average age of 34 years. Eighty-five per cent of the lesions resulted from traffic accidents (49% velocipedes, 39% automobiles, 12% trampling), 10% were consequence of falls and 5% resulted from agriculture activities. Orthopaedic lesions include 83 fractures of upper limbs (22 open), 123 of lower limbs (67 open), 21 scapulo-thoracic dissociations and 27 knee dislocations. Vascular lesions include 8 subclavian, 20 axillary, 25 braquial, 10 radial, 6 ulnar and 2 diverse, in the upper limbs; and in lower limbs 4 iliac, 27 femoral, 52 popliteal, 5 tibio-peroneal trunk, 5 anterior tibial, 12 posterior tibial and 12 peroneal. Overall mortality was 3.3% and the amputation rate was 17%. Knee dislocations were responsible for one third of amputations, followed by fractures of bone legs. The association of femur to bone legs fractures from one side and of the knee to bone legs fractures from the other, coursed with high levels of amputation, together with lacerations and crushing of the limbs. The prompt diagnosis of the lesions and the immediate, hierarchic and multidisciplinary approach were considered also as relevant prognostic factors in the management of these most demanding conditions.

From 1980 to 2005, eight patients, four male and four female, age range between 33 to 86 years (average 55.2) with the diagnosis of primary aneurysm of the carotid bifurcation, underwent surgical management. Patients with postendarterectomy pseudo-aneurysms (also rotulated as "secondary") were excluded from the study, due to different pathogenic, clinical and therapeutic features. Four aneurysms were degenerative by nature, occurring in the older patients of the series. Some other etiologies include arterial fibrodysplasia, brucellosis, Behçet's disease and penetrating wound of the neck. All patients underwent the surgical resection of the aneurysm, followed by reestablishment of the carotid continuity in 6 cases; two patient had a definite ligation of the internal and external carotid artery, respectively. There was no operative mortality nor significant morbidity. From 1 to 25 years after the operation, three patients died for different reasons, two were lost for follow up and the remaining three are found alive and well. The main features of clinical presentation, etiology, diagnosis and surgical management of this entity are presented and discussed and compared with similar experiences reported in the international literature.

Two clinical cases of an unusual complication of the renal transplantation are reported, consisting in the infection of the arterial wall by fungi of the species Candida Albicans and Aspergillus, which caused acute ruptures of the external iliac artery and hypovolemic shock. The main features of the pathogenesis of the infection, its diagnosis, management and prevention are subjected to analysis and discussion.

The surgical management of the aortic prosthesis infection still remains an enormous challenge for the vascular surgeon and a critical issue for the patient's integrity and life. Several techniques for its management have been devised and employed, along the years, but none of them revealed itself as totally satisfactory. This stimulates the creation of new alternatives. We present the clinical case of an infected aortic prosthesis in a 41 year old man, complicated by duodenal and ureteral erosion, in whom the autologous superficial femoral artery was employed successfully in the treatment of this most demanding situation.

The thrombotic occlusion of one renal artery may become completely asymptomatic, due to the functionality of the contralateral kidney. However, in rare circumstances, such is the case of individuals with a solitary kidney, a situation of anuria and acute renal failure may constitute the main presentation of the condition. The authors report the clinical case of a 43 year old male patient, with the previous diagnosis of an infrarenal aortic occlusion and a single left kidney, who developed a thrombotic occlusion of the renal artery, with anuria and acute renal failure. The patient underwent an emergency revascularization procedure, consisting in the implantation of a prosthetic bypass graft from the superceliac aorta to the renal artery, with immediate recovery of the diuresis and renal function. Seventeen months later as a consequence of an anastomotic hyperplasia, an occlusion of the bypass graft occurred, again with anuria and acute renal failure. The patient was reoperated on and due to the inadequacy of both saphenous veins to be used as the material of choice for the revascularization procedure, a redundant segment of the left colic artery (Riolan's arcade) was removed and used as an interposition graft, from the middle colic artery to the renal artery, followed by an immediate restoration of diuresis and renal function. The singular and recurrent character of this clinical condition and the utilization of an original, eventually unique and well succeeded revascularization procedure, prompted its presentation and divulgation.

The external iliac artery has physical characteristics, in particular, caliber and length, similar to those of the renal artery and would thus seem to be an ideal substitute for it. However, there are no studies in the literature designed to confirm this possibility. The present study was designed to demonstrate the feasibility of use of the external iliac artery as a substitute for the renal artery in surgical revascularization of renal artery fibrodysplasia and to assess the long-term biologic behavior of the external iliac artery so used.Twelve patients (11 female, 1 male), ages 1 to 43 years (mean, 24.4 years), with severe hypertension underwent aortorenal bypass grafting with use of the external iliac artery to treat preocclusive stenotic lesions (n = 7), complete occlusion (n = 3), or aneurysm (n = 2) of the renal artery. It was considered unnecessary to reestablish circulation in the donor limb in 2 patients (young children), and transposition of the ipsilateral internal iliac artery was performed in 9 patients; in the remaining patient circulation was reestablished with insertion of an iliofemoral prosthesis.There were no operative deaths. In 1 patient nephrectomy was necessary because of occlusion of the graft as a consequence of technical complications. In 8 patients there was immediate normalization of blood pressure without aid of medication, and in the remaining 3 patients an antihypertensive drug was prescribed. Two patients were lost to follow-up; the other 9 were assessed with angiography at a mean of 8.8 years after the operation. All grafts were found to be in excellent condition, with no evidence of dilatation, kinking, twisting, anastomotic fibroplasia, or wall irregularities.On the basis of this experience, the external iliac artery is confirmed as a substitute for the renal artery, and the excellent long-term results would seem to make it particularly useful in young patients with long life expectancy, such as those with renal artery fibrodysplasia.

Favorable long-term results have been reported after femorofemoral bypass in the treatment of iliofemoral occlusive disease. Functional alterations in the donor artery, occurring after the implantation of the graft, have been considered as having an arresting effect on the progress of the occlusive disease, which explains the favorable long-term results. However, until now, the intrinsic mechanism of this phenomenon has remained unexplained. Little attention has been devoted to the evaluation of the natural history of the donor artery. To achieve this purpose, 18 patients previously operated on for femorofemoral bypass, with a normal patent graft, were submitted to angiographic evaluation, from 8 to 57 months after the operation (mean 22 months). The angiograms were compared with the preoperative films to assess the progress of the occlusive disease in the donor artery. A slight but significant dilatation of the proximal donor arterial segment was documented in every case, affecting the external iliac and common femoral arteries. The pathogenesis of this phenomenon is discussed. It may be related to decreased peripheral resistance and increased blood flow occurring in the donor arterial segment, reported after the implantation of the shunt. This dilatation of the donor artery may have a compensatory effect for local atherosclerotic stenosis, thereby explaining the favorably long duration of the femorofemoral bypasses, which has been emphasized since the early days of extraanatomic revascularization. A similar phenomenon may occur in the vessels of the upper extremity, after axillofemoral revascularization. (J VASC SURG 1988;8:106-11.)

Aortobifemoral bypass remains the standard therapy for aortoiliac occlusive disease. Extraanatomic revascularization has been considered as an acceptable alternative and provides reasonable long-term results. Because some of the causes for its failure are related to the prosthetic material used, a technique that uses autologous material and is applicable in patients with unilateral common iliac lesions was developed. Iliofemoral crossover transposition is a femorofemoral bypass procedure in which the external iliac artery is used as a graft. The procedure is easily performed and avoids the use of prosthetic materials, minimizing the risks of their related complications. Because its biologic behavior, including hemodynamic performance, has proven to be excellent, satisfactory long-term results can be anticipated. (J Vasc Surg 2002;36:404-7.)