[Abdominal angina due to fibromuscular dysplasia of the celiac axis. Surgical management]. / Angina abdominal por displasia fibromuscular do tronco celíaco. Tratamento cirúrgico.

The clinical case of a 27-year old man with the diagnosis of chronic mesenteric ischemia ("abdominal angina") is reported, whose chief complaints were severe postprandial pain and remarkable weight loss, for the last 4 months. Following na inconclusive observation in gastroenterology, he underwent an angiographic-CT examination, that disclosed a critical stenosis at the origin of the celiac axis. The remaining digestive vessels, superior and inferior mesenteric arteries, were found free of lesions. The patient was submitted to a revascularization procedure, consisting in the celiac axis resection and its replacement by a prosthetic graft, arising from the supraceliac aorta. The post-operative course was uneventfull, followed by a complete remission of the pain and a progressive weight gain. The histopathological study of the removed artery revealed the diagnosis of arterial fibrodysplasia, a very rare entity in clinical practice, of unknown etiology, affecting predominantely young people and in a decreasing order of frequency the renals, the internal carotids and the external iliac arteries. The localization of the fibrodysplastic disease to the celiac áxis seems to be a unique case, never reported before in the literature, thus justifying its publication and dissemination.

[Conventional open surgery of a splenic artery aneurysm following failure of endovascular management]. / Tratamento cirúrgico convencional de um aneurisma da artéria esplénica após fracasso de intervenção endovascular.

The clinical case of a 73 years old man is reported, complaining for a long time of a persistent abdominal pain in the left upper quadrants, without irradiation and no identified causative factor. These symptoms affected notoriously his quality of life, becoming disabling for his social and professional activities. The investigation by means of CT-scans disclosed a spheric mass, partially calcified, having 44x41mms of maximum size, located between the pancreatic tail and the splenic hilum. A subsequent angio-CT confirmed the diagnosis of a large splenic artery aneurysm and the patient underwent the attempt of an endovascular exclusion by means of an endoprosthesis, which could not be successfully accomplished due to the multiple kinkings and coilings of the splenic artery. Several coils were then used to occlude the aneurismal sac. Contrarily to the expectations, the pain did not disappear after the procedure, becoming even worse, and an open surgical approach was then advised, in another hospital institution, which he promptly accepted. The operation consisted in the resection of the aneurysm and an associated splenectomy, due to multiple infarcts identified in the spleen, consequence of the previous endovascular management. The post operative course was uneventful and he was discharged on day 3. One and four months later he was found in excellent condition and totally pain-free, having resumed his social and professional activities. Based on this clinical case, the authors intend to put an emphasis on this constraint of the endovascular management of peripheral arterial aneurysms, namely on those causing external compression syndromes, painful or others, which can not be relieved by the endovascular exclusion of the aneurysm, as it happened with this patient, thus justifying its presentation and dissemination.

Arg120stop nonsense mutation in the RP2 gene: mutational hotspot and germ line mosaicism?

Mutations in the RP2 gene account for up to 20% of X-linked recessive retinitis pigmentosa (RP). Arg120stop is to date the most frequently reported mutation found in RP2. Mutation screening was performed during the course of a large screening program of retinal degenerative disorders (RDDs) in South Africa using exon 1 and 2 of RP2 in 20 unrelated families with an X-linked mode of retinal degenerative inheritance. Direct sequencing analysis revealed a C-->T transition at position 358 in the proband in a family of German origin. Subsequent analysis revealed that this Arg120stop mutation cosegregated with the disease in an additional affected family member. The nonsense mutation, Arg120stop, could not however, be detected in the somatic cells of the obligate carrier female. This, the first report of a germ line mutation for a family with RP, has many implications for genetic counseling of retinal degeneration (RD). To avoid inaccurate risk assessment for RP due to epigenetic events, such as the rare occurrence of germ line mosaicism, genetic counseling in families with XLRP should always be guided by molecular testing.

[A rare case of Marfan syndrome with involvement of the thoracoabdominal aorta--its surgical treatment]. / Um caso raro de síndrome de Marfan com envolvimento da aorta toracoabdominal--tratamento cirúrgico.

A 49-year-old woman with classic manifestations of Marfan's syndrome, having previously been submitted to two surgical procedures for ascending aorta and aortic valve replacement, was admitted with diagnosis of a large dissecting thoracoabdominal aortic aneurysm, with indication for surgical repair. The patient underwent a successful reconstruction of the aorta by means of the "simplified technique" introduced by ourselves in 1984, as an alternative to the conventional S. Crawford's "inclusion technique", for the management of thoracoabdominal aortic aneurysms. Assessed two years after the operation, the patient was found to be in excellent clinical condition and a control angiography showed the vascular prosthesis and its side branches working properly. This unusual aortic complication of Marfan's syndrome and the usefulness of the unique and original technique employed to replace the thoracoabdominal aorta justify the presentation of this clinical case.

The use of arterial allografts for vascular reconstruction in patients receiving immunosuppression for organ transplantation.

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.

[Distal arteritis of the legs: de-obstruction of the popliteal trifurcation]. / Artérite distale des membres inférieurs: désobstruction de la trifurcation poplitée.

Conventional arteriograms for critical leg and foot ischemia frequently do not opacify the popliteal artery and its branches of division. We have studied the patency of the popliteal artery on amputated limb and we have seen that in a great number of cases, it was possible to retrieve fresh clots from the popliteal artery in order to obtain a optimum outflow and to perform a femoro-popliteal bypass. Since the completion of this study, we systematically explore the popliteal artery surgically in case of critical leg and foot ischemia even in the cases where arteriogram failed to demonstrate a patent artery. This aggressive surgical management resulted in a 78.5% five year limb salvage rate.

[The surgical treatment of renal artery fibrodysplasia. The immediate and late results]. / Tratamento cirúrgico da fibrodisplasia da artéria renal. Resultados imediatos e tardios.

Between November 1977 and April 1992, thirty patients carrying several forms of renal artery fibrodysplasia underwent surgical treatment. The series is composed of thirteen male and seventeen female, aged between 1 and 51 years (mean age 32). All patients had arterial hypertension, which was not relieved by medical therapy, consisting in the administration of an average of 2.9 antihypertensive drugs per patients. Bilateral renal artery lesions were diagnosed in ten patients (33.3%). The remaining twenty patients (66.6%) had unilateral lesions, one of which involved a congenital single kidney, making a total of forty renal arteries affected by the disease. Surgical procedures consisted in renal artery revascularization in thirty-one kidneys, in aneurysmectomy alone or associated with aorto-renal bypass in six cases, and in a nephrectomy as a single procedure in one case. Three kidneys were considered lost and were left untouched. There was no operative mortality. Evaluated between 1 and 173 months following the operation (mean 79 months/6.6 years) twenty-six patients (86.6%) were classified as either cured or improved. Only two patients (6.6%) did not get any improvement with the surgical treatment. Two patients whose condition after the operation was considered satisfactory were lost to follow-up. The results of this experience are compared with other series published in the international literature, as well as with the percutaneous transluminal angioplasty which has been regarded as an alternative to arterial reconstructive surgery.

[Unusual procedures used in reconstructive surgery and/or revascularization of the aorta]. / Procedimentos invulgares utilizados na cirurgia de reconstrução e/ou revascularização da aorta.

Since the advent of arterial surgery, four decades of development have evolved, characterized not only by a remarkable evolution of concepts, but also by the improvement of technics, means and methods utilized for arterial reconstructive surgery. This progress led to the promotion and improvement of the conventional surgical methods dedicated to the treatment of the most common aortic disorders, obstructive or aneurysmal. Endarterectomy, revascularization using synthetic bypasses or interposition of arterial prosthesis are the most common and standardized procedures which are used worldwide, giving gratifying and long lasting results, involving low or negligible risks. In clinical practice however one sometimes, faces rare conditions, poorly understood due to their rarity, in which the conventional methods may be regarded as inappropriate, unfit or even formally contraindicated. Some unusual or uncommon methods were devised to deal with such conditions, which are less popular due to their rarity, but may also be considered just as valid and reliable as conventional methods, bearing satisfactory and rewarding results. Among them we stress the bypass originated in the ascending aorta, the bypass from the descending thoracic aorta and the bypass arising from the supraceliac aorta. The main features of these procedures as well as the personal experience of their utilization obtained by the senior author constitute the essence of this presentation.

The fate of the donor artery in extraanatomic revascularization.

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.