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Alors que diverses méthodes de classification ont été proposées pour déterminer l'épidémiologie du syndrome de Sjögren primitif (pSS), il est nécessaire de discerner si celui-ci est à considérer comme une maladie rare, c'est-à-dire, avec une prévalence inférieure à 50 cas pour 100 000 habitants. Parmi 11 analyses issues de 9 études basées sur des cohortes populationnelles, 8 concluent à une prévalence de moins de 50 cas pour 100 000 habitants et 2 méta-analyses confirment ces résultats, retrouvant de 39 à 43 cas pour 100 000 habitants. Il n'y a pas de différence notable entre les études où le diagnostic était posé par un médecin ou à l'aide des critères de classification AECG 2002. Aucune étude n'a utilisé jusqu'à présent les critères de classification ACR/EULAR 2016. Les femmes sont 6 à 9 fois plus touchées que les hommes, et présentent un pic d'incidence de la maladie entre 60 et 70 ans. Alors que les personnes d'origine Asiatiques sont les plus touchées, les Caucasiens sont également plus atteints par le pSS que les patients d'origine Africaine ou Hispaniques. En définitive, pouvoir considérer le syndrome de Sjögren primitif comme une maladie rare a des implications pour le clinicien, pour le développement de nouvelles molécules thérapeutiques et pour la mise en place des plans nationaux « maladies rares ». While several criteria for classification have been used for epidemiological studies about primary Sjögren syndrome (pSS), it remains unclear if pSS is an orphan disease. Among 11 analyses retrieved from 9 population-based studies, 8 show a prevalence lower than the orphan disease threshold (50/100,000 inhabitants). In two meta-analyses, prevalence is 39 to 43 per 100,000 inhabitants. There is no difference among prevalence in studies using or not classification criteria. ACR/EULAR 2016 criteria have not been used yet. Women are 6 to 9-fold more involved than men in pSS, and incidence for diagnosis is at the highest between 60 and 70 years old. Although Asian people are the most likely to present pSS, Whites are also more likely than Blacks or Hispanics. Collectively, pSS shall be now considered as an orphan disease, with important implications for clinicians, for development of new therapies and for national health programs. [ABSTRACT FROM AUTHOR]

Mycotic/infective native aortic aneurysms (INAA) are managed heterogeneously. In the context of disparate literature, this study aimed to assess the outcomes of INAA surgical management and provide comprehensive data in alignment with recent suggestions for reporting standards. A retrospective review of patients presenting with INAA from September 2002 to March 2020 at two institutions was conducted. In hospital mortality, 90 day mortality, overall mortality, and infection related complications (IRCs) were the study endpoints. Overall survival and IRC free survival were estimated, and predictors of mortality tested using uni- and multivariable analyses. Seventy patients (60 men [86%], median age 68 years [range 59 – 76 years]) were included. Twenty (29%) were ruptured at presentation. INAA location was thoracic in 11 (16%) cases, thoraco-abdominal in seven (10%), and abdominal in 50 (71%). Half of the abdominal INAAs were suprarenal. Two INAAs were concomitantly abdominal and thoracic. Pathogens were identified in 83%. The bacterial spectrum was scattered, with rare Salmonella species (n = 6; 9%). Open surgical repair was performed in 66 (94%) patients, including five conversions of initially attempted endovascular grafts (EVAR), three hybrid procedures, and one palliative EVAR. Vascular substitutes were cryopreserved arterial allografts (n = 67; 96%), prosthesis (n = 2), or femoral veins (n = 1). Kaplan–Meier estimates of overall survival at 30 and 90 days were 87% (95% confidence interval [CI] 76.6 – 93.0) and 71.7% (95% CI 59.2 – 80.9), respectively. The overall in hospital mortality rate was 27.9% (95% CI 1.8 – 66.5). IRCs occurred in seven (10%) patients. The median follow up period was 26.5 months (range 13.0–66.0 months). Chronic kidney disease (CKD) was independently related to in hospital mortality (odds ratio [OR] 20.7, 95% CI 1.8 – 232.7). American Society of Anesthesiologists score of 3 (OR 6.0, 95% CI 1.1 – 33.9), 4 (OR 14.9, 95% CI 1.7 – 129.3), and CKD (OR 32.0, 95% CI 1.2 – 821.5) were related to 90 day mortality. Surgical INAA management has significant mortality and a low re-infection rate. EVAR necessitated secondary open repair, but its limited use in this report did not allow conclusions to be drawn. [ABSTRACT FROM AUTHOR]

Tertiary centers recruit a large proportion of locally advanced or recurrent soft tissue sarcomas (STSs) that may have been preoperatively irradiated. The objective of this study was to evaluate the results of oncoplastic surgery (OPS) for patients affected by extremities or parietal trunk STS. This retrospective study includes patients who underwent a flap reconstruction after sarcoma resection between January 2018 and December 2020 at Institut Curie. The primary endpoint was the evaluation of the impact of OPS on the quality of surgical margins. The secondary endpoint was to quantify the morbidity of OPS and identify predictive factors for wound complications. Of 211 patients, 89 (42.2%) had a flap reconstruction. Surgery was realized on an irradiated field in 56 (62.9%) patients. Without OPS, all patients were candidates either for amputation (n = 9,10.1%) due to vessels/nerve infiltration, or R1/R2 resection (n = 80,89.9%). Seventy-two (80.0%) pedicle flaps and 18 (20.0%) free flaps were used. No R2 resections were performed. R0 and R1 margins were achieved in 82 (92.1%) and 7 (7.9%), respectively. The median closest margin was 3 mm (IQR 1–6 mm). Among R1 patients, 5 had positive margins along a preserved critical structure, 2 patients had well-differentiated liposarcomas. The surgical morbidity rate was 33.3% (30/90 flaps). The reoperation rate was 15.7% (14/89 patients). In a referral sarcoma center, the collaboration between the surgical oncologist and the plastic surgery team should be considered upfront in the surgical plan, allowing the most adequate wide oncological resection with acceptable postoperative morbidity. [ABSTRACT FROM AUTHOR]

To investigate the use of cryopreserved arterial allografts (CAA) as a substitute for infected infrarenal aortic prostheses, and its outcomes. A single centre retrospective study of consecutive patients receiving an abdominal aortic CAA after removal of an infected graft was conducted between January 1997 and December 2013. The primary outcome was the rate of allograft related revision surgery. Secondary outcomes were the 30 day mortality rate, survival, primary patency, limb salvage, and infection recurrence. Allograft ruptures secondary to infection and risk factors for allograft failure were also investigated. Two hundred patients (mean age 64.2 ± 9.4 years) were included. In 56 (28%) cases, infection was related to an enteric fistula. The mean follow up duration was 4.1 years. The 30 day mortality rate was 11%. Early revision surgery was needed in 59 patients (29.5%). Among them, 15 (7.5%) were allograft related and led to the death of three patients (1.5%), corresponding to a 7.5% 30 day allograft related revision surgery rate. During the first six months, 17 (8.5%) patients experienced 21 events with complete or partial rupture (pseudo-aneurysm) of the allograft responsible for five (2.5%) deaths, corresponding to a re-infection rate of 8.5%. The multivariable analysis showed that diabetes and pseudo-aneurysm of the native aorta on presentation were predictive factors for short term allograft rupture. After six months, 25 (12.5%) patients experienced long term allograft complications (rupture, n = 2, 1%; pseudo-aneurysm, n = 6, 3%; aneurysm, n = 2, 1%; thrombosis, n = 11, 5.5%; stenosis, n = 4, 2%;) requiring revision surgery resulting in one death. The five year rates of survival, allograft related revision surgery, limb salvage, primary patency, and infection recurrence were 56%, 30%, 89%, 80%, and 12%, respectively. CAAs provide acceptable results to treat aortic graft infection with few early graft related fatal complications. Long term allograft related complications are quite common but are associated with low mortality and amputation rates. [ABSTRACT FROM AUTHOR]

Arterial involvement in Behçet disease (BD) is rare, and its surgical management is a major concern because of its high recurrence rate. This study evaluated the influence of the surgical technique, device, and immunosuppressive treatment used on the postoperative recurrence in patients with non-pulmonary arterial BD. A single-center, retrospective study was conducted of 23 patients meeting the international criteria for BD who underwent surgery for arterial involvement between May 1996 and September 2015. Recurrence was defined as the occurrence of arterial aneurysm or thrombosis during follow-up. Perioperative medical treatment and surgical technique used were reported. There were 47 surgical procedures performed in 23 patients. Mean follow-up was 8.4 ± 7.5 years. Initial arterial lesions were aneurysms and thrombosis in 85% and 15% of cases, respectively. Arterial lesions were aortic and peripheral in 48% and 52% of cases. Recurrence rate was 51%. Recurrences developed within <1 year in 24% of cases and at the same anatomic site in 92% of cases. Among the 24 recurrences, 17 were false aneurysms, 6 were thrombosis, and 1 was a true aneurysm in a different arterial site. To treat the arterial lesion, direct anastomosis was performed in 6 cases; bypass using the saphenous vein, graft, or allograft was performed in 6, 27, and 5 cases, respectively; and stent graft was used in 3 cases. Vascular lesions involved the aorta in 19 cases and a peripheral artery in 28 cases. Preoperative medical treatments, including colchicine, steroids, and immunosuppressants, significantly decreased recurrence rate: 28% (7/25) vs 75% (15/20) in untreated patients (P =.002). The recurrence rate was 42.5% (17/40) in patients treated postoperatively vs 80% (4/5) in untreated patients. The nature of the device used (vein, prosthetic graft, allograft, stent graft, or direct anastomosis) did not change the risk of recurrence. When anastomoses were protected using the prosthetic sleeving technique, the recurrence rate was three times lower (P =.08). Relapse is a main concern after surgical repair of arterial BD. This study suggests the need for targeted perioperative medical management to reduce the risk of arterial recurrence in BD patients. To this end, a multidisciplinary approach is mandatory. The use of sleeve anastomosis is associated with a numerically lower risk of recurrence. However, further studies are needed to confirm this efficacy. [ABSTRACT FROM AUTHOR]

Carotid endarterectomy and carotid artery stenting are both valid therapeutic options for the treatment of radiation-induced carotid stenosis (RICS). The second has the advantage of being less invasive, although it seems to result in more restenosis than the first. Meanwhile, progress in radiation therapy and head and neck surgery has significantly increased the survival of these patients. As a result, treatment of RICS should be considered from a long-term perspective. This works presents perioperative and follow-up outcomes of surgical treatment of RICS. This single-center retrospective study included all patients who underwent carotid endarterectomy for RICS from January 1998 to June 2017. Clinical and duplex ultrasound examination-based follow-up was performed postoperatively, at 1 month, 6 months, 12 months, and yearly thereafter. Kaplan-Meier curves were used for survival plots based on a log-rank test. Any abnormal finding led to angio-computed tomography scan and specialized neurovascular examination. Between 1998 and 2017, 128 patients (162 lesions) were treated. The median interval between radiation therapy and surgery was 16 years. Forty-five patients (35%) were symptomatic. The eversion technique was performed in 79 cases (49%), and the patch was favored in 24 cases (15%), prosthetic bypass graft in 51 cases (31%), and a venous bypass graft in 8 cases (5%). Two postoperative deaths (1.5%) (one secondary to massive stroke) were noted. The primary end point of early postoperative cerebrovascular event was 2.5%. Two cervical hematomas (1.2%) required surgical revision and seven cases of permanent cranial nerve injury were recorded. The median follow-up was 29 months (range, 2-199 months). There were no additional strokes. The 3-year primary patency rate was 96% and the 3-year freedom from neurologic event was 98%. Open surgical treatment of RICS lesions is a safe and durable option. Our results suggest that the outcomes of such treatment are good and in particular that rates of cerebrovascular event and restenosis are low and that cranial nerve injury should not be a concern. As a result, we consider that open surgery for RICS lesions should be offered as a first-line treatment. However, comparative data are mandated to address this issue. [ABSTRACT FROM AUTHOR]

Treatment of renal artery aneurysms (RAA) remains controversial. Endovascular treatment has increased for main trunk and for very distal aneurysms, whereas for lesions located at the bifurcation surgical treatment seems to be a valid option. The goal of this study was to describe the technique of direct reconstruction of RAA and to report on outcomes. This study comprised single centre prospective collection of data with retrospective analysis (January 2015 to August 2018) of patients operated on for distal RAA using direct reconstruction. A total of 24 RAA in 21 patients (seven men and 14 women, mean age 59 ± 13 years) was included. History of hypertension was found in 15 patients and renal insufficiency was present in one. Mean pre-operative systolic and diastolic blood pressures were 134 ± 21 mmHg and 74 ± 10 mmHg, and mean pre-operative rates of creatinine and glomerular filtration rate were 67 ± 13 μmol/L and 93 ± 49 mL/min/1.73 m2, respectively. Indications for repair were a diameter >20 mm in seven cases (mean diameter = 25 ± 2 mm) or rapid growth in one case, symptomatic aneurysm in 12 cases (hypertension, haematuria, pain), and a concomitant lesion in four cases (splenic aneurysm, abdominal aortic aneurysm, occlusive visceral artery lesions). All lesions were distal, main artery bifurcation in 22 cases and hilar in two cases. The main aetiology was fibromuscular dysplasia (16 cases) followed by atherosclerosis (seven cases) and one case of Ehlers Danlos Syndrome. In situ reconstruction was possible for 22 RAA, while two cases required kidney autotransplantation. The mean renal ischaemia time was 18 ± 5 min. At two years, the patency rate was 100%, and mean systolic blood pressure decreased (134 mmHg–122 mmHg, p =.047). Renal function was stable from 93 ± 49 pre-operatively to 95 ± 35 mL/min/1.73 m2 in the post-operative course (p =.56). Direct reconstruction appears to be efficient for most RAA. This technique is complementary to ex vivo autotransplantation and endovascular treatment. [ABSTRACT FROM AUTHOR]

Background: Vitamin B12 (B12) has a fundamental role in both central and peripheral nervous system function at all ages. Neurologic manifestations may be the earliest and often the only manifestation of B12 deficiency. Mostly because of the poor sensitivity of methods of determination for B12 levels, peripheral neuropathy remains a classical but underdiagnosed complication of B12 deficiency. So the clinical and electrophysiological characteristics of B12-responsive neuropathy are not well known. Methods: A retrospective study of patients with B12-responsive neuropathy was conducted at our hospital on a 3-year period. The criteria for inclusion were: (a) neuropathy confirmed by the electrophysiological study (nerve conduction study); and (b) improvement of at least 1 point of the total Overall Neuropathy Limitations Scale score after vitamin B12 treatment. Results: Nine patients were identified. Serum B12 level was low in only four. Four patients had sensorimotor (predominantly sensory) axonal polyneuropathy while five had only sensory neuronopathy. Six improved in less than 1 month after B12 supplementation. Conclusion: B12-responsive neuropathy is a more heterogeneous group of neuropathy than previously described. B12 deficiency is a cause of peripheral neuropathy and should systematically be ruled out in the clinical setting of idiopathic neuropathy or sensory neuronopathy because of potential reversibility. Abbreviations: B12: vitamin B12; CMAP: compound muscle action potentials; DRG: dorsal root ganglia; ENMG: electroneuromyography; MCCT: motor central conduction time; MEP: motor evoked potentials; MMA: methylmalonic acid; MMCoAM: L-methylmalonyl–CoenzymeA mutase; ONLS: overall neuropathy limitations scale; SCV: sensory conduction velocities; SNAP: sensory nerve action potentials; SNN: sensory neuronopathy; SSS: SNAP sum score [ABSTRACT FROM AUTHOR]

Abstract Objective The objective of this study was to assess outcomes of a hybrid technique for treatment of abdominal aortic aneurysm (AAA) associated with iliac aneurysm without distal neck by combining an AAA endovascular repair approach with open surgery for preservation of the internal iliac artery (IIA). Methods The files of 51 patients operated on between 1998 and 2017 in a single vascular surgery department were retrospectively analyzed. Inclusion criteria were patients with AAA associated with uni-iliac or bi-iliac aneurysm without suitable distal sealing zone. Surgery consisted of deployment of an aortouni-iliac stent graft combined with an extra-anatomic crossover prosthetic bypass. With use of a limited retroperitoneal approach, the contralateral proximal common iliac aneurysm was surgically excluded and the IIA revascularized by direct ilioiliac anastomosis or terminal common iliac suture, preserving the iliac bifurcation. Results The patients' mean age was 74 years (58-88 years), and 92% were men. The mean follow-up was 5.8 years (0.1-18 years). Twenty-nine patients (57%) had one or more high-risk criteria for open surgery. Nineteen patients (37.3%) had aortouni-iliac aneurysms, 19 (37.3%) aortobi-iliac aneurysms, 5 (10%) isolated iliac aneurysms, and 8 (15.7%) bi-iliac aneurysms without aortic location. Four patients (7.8%) also had IIA aneurysms. Surgery was successful in all cases. Two patients (4%) died during the 30 days after surgery. One surgically preserved IIA occluded within the first month, resulting in buttock claudication. The 5-year IIA primary patency rate was 96%. Type I proximal endoleaks occurred in two patients, requiring additional surgery 3 years and 13 years after the initial surgery, respectively. Conclusions This hybrid technique, consisting of AAA endovascular exclusion combined with open IIA revascularization, is safe and effective for preservation of pelvic vascularization. It is associated with long-term patency and low morbidity rates. We have been using this technique since before the advent of branched dedicated devices, allowing preservation of the IIA with good results. This technique should continue to be proposed, especially in patients not eligible for endovascular iliac branch repair because of anatomic contraindications, to avoid pelvic ischemia if the IIA has to be sacrificed. [ABSTRACT FROM AUTHOR]

Objective A suitable ipsilateral great saphenous vein (GSV) autograft is widely considered the best material for arterial reconstruction of a popliteal artery aneurysm (PAA). There are, however, cases in which such a GSV is absent, diseased, or of too small diameter for this use. Alternatives to GSV are synthetic conduits, but with a reduced long-term patency, in particular for infragenicular bypass; other venous autografts of marginal use; and stent grafts still in the first stages of their evaluation. However, a sufficiently long segment of the ipsilateral superficial femoral artery (SFA) is often preserved in patients with a PAA. Such a segment may be used as an autograft for popliteal reconstruction. Moreover, the morphometric characteristics of the SFA often optimally match those of the distal native popliteal bifurcation. SFA autografts (SFAAs) have therefore become our choice when the ipsilateral GSV is not suitable. We herein present the long-term results of SFAA for the treatment of PAA in the absence of a suitable GSV. Methods Within this single-center study, all cases during the last 26 years were retrospectively reviewed. Demographics, risk factors, comorbidities, morphometrics of the PAA, and preoperative and follow-up data were intentionally sought. Results From 1997 to 2017, there were 67 PAAs treated with an SFAA. The mean age of the patients was 67.67 ± 12 years, and 98% were male. Symptoms included intermittent claudication in 25% (17), critical limb ischemia in 7% (5), and acute ischemia in 10% (7) of the patients; 51% (34) of the patients were asymptomatic. The mean aneurysm diameter of the treated PAA was 29 ± 11 mm (12-61 mm). The mean operative time was 254.8 ± 65.6 minutes (140-480 minutes), with a mean cross-clamp time of 64.5 ± 39 minutes (19-240 minutes). The median length of stay was 9 ± 6.4 days (5-42 days). There were no early amputations or deaths in the series. During a mean follow-up of 47.91 ± 48.23 months, there were 2 anastomotic stenoses, 11 thromboses, 1 infection, and 1 aneurysmal degeneration of the graft; 6 patients died of unrelated causes. The 1-, 3-, 5-, and 10-year primary and secondary patency rates were 93% and 96%, 85% and 90%, 78% and 87%, and 56% and 87%, respectively. Conclusions These data suggest that SFAA use to treat PAA is a safe and durable option. A prospective and comparative work is necessary to confirm these results and to determine the interest of this technique as a first-line strategy. [ABSTRACT FROM AUTHOR]

Objective The objective of this study was to describe large-vessel vasculitis (LVV) in patients with human immunodeficiency virus (HIV) infection. It is a retrospective single-center study conducted between 2000 and 2015 through a university hospital of 11 HIV-infected patients with LVV. Methods The characteristics and outcome of 11 HIV-infected patients with LVV (7 patients fulfilled international criteria for Takayasu arteritis, 5 patients had histologic findings of vasculitis, and 5 patients had imaging features of aortitis) were analyzed and compared with those of 82 patients with LVV but without HIV infection. Results Concerning the HIV-infected patients with LVV (n = 11), the mean age was 40 years (range, 36-56 years), and 55% of patients were female. At diagnosis of LLV, the mean initial CD4 cell count was 455 cells/mm 3 (range, 166-837 cells/mm 3 ), and the median HIV viral load was 9241 copies. Vascular lesions were located in the aorta (n = 7), in supra-aortic trunks (n = 7), and in digestive arteries (n = 3). Inflammatory aorta infiltrates showed a strong expression of interferon-γ and interleukin 6. In HIV-negative LVV patients (n = 82), the median age was 42 years, and 88% of the patients were women. Thirty patients had an inflammatory syndrome. Seventy patients had been treated with glucocorticosteroids and 57 with immunosuppressive treatments. Compared with their negative counterparts, HIV-positive patients with LVV were more frequently male ( P = .014), had more vascular complications (ie, Ishikawa score; P = .017), and had more frequent revascularization ( P = .047). After a mean follow-up of 96 months, four relapses of vasculitis were reported, and one patient died. Regardless of the HIV virologic response, antiretroviral therapy improved LVV in only one case. Conclusions LVV in HIV-infected patients is a rare and severe entity. [ABSTRACT FROM AUTHOR]

Résumé: Le traitement des néphropathies lupiques (NL) prolifératives a fait récemment l’objet de recommandations européennes et américaines. L’objectif de ce travail était de décrire les pratiques actuelles en France. Entre mars 2012 et avril 2013, un questionnaire informatisé a été envoyé aux néphrologues et internistes français via leur société savante. Cent neuf spécialistes (60 internistes, 48 néphrologues et 1 rhumatologue), en majorité hospitaliers, ont répondu au questionnaire. Le traitement immunosuppresseur d’induction utilisé en première intention était le cyclophosphamide (CYC) intraveineux (IV) à faible dose (Euro-Lupus, 67 %), suivi du mycophénolate mofétil (MMF) (20 %) et du CYC à forte dose (NIH, 9 %). Le traitement d’entretien après CYC reposait souvent sur le MMF (58 %) versus 14 % pour l’azathioprine (AZA) et 25 % utilisant de façon équivalente AZA ou MMF. Après une induction par MMF, l’entretien reposait majoritairement sur le MMF (77 %). Un antipaludéen de synthèse était instauré systématiquement par 88 % des médecins. Chez les patients stables, en rémission, le traitement d’entretien était poursuivi 2 ans (40 %), 3 ans (25 %) ou au-delà de 3 ans (34 %). Une corticothérapie à faible dose était poursuivie au long cours par 54 % des médecins. Il n’y avait pas de différence de pratiques entre néphrologues et internistes, y compris sur la prescription systématique d’antipaludéens de synthèse. En France, la prise en charge des NL prolifératives est assez homogène et conforme aux recommandations internationales les plus récentes. [Copyright &y& Elsevier]

Objective: The choice between open surgery (OS) and transluminal carotid angioplasty with stenting (CAS) for the treatment of primary carotid stenosis remains controversial. However, CAS is considered a valid option for selected cases, such as recurrent carotid stenosis (RCS). Tertiary RCS seems to be a concerning issue after CAS but few large reports focused on the durability of CAS and OS. We report our early and long-term results with OS for RCS. Methods: From 1989 to 2006, perioperative data regarding 4245 consecutive surgical carotid reconstructions was prospectively collected. Patients whose indication was RCS were subjected to further analysis. Indications for surgery were symptomatic RCS >50% or asymptomatic RCS >80%. Freedom from neurologic event was defined as the absence of any ipsilateral symptom at any time after the procedure. Kaplan-Meier analysis was used to estimate freedom from reintervention, freedom from restenosis >50% and occlusion, freedom from neurologic event and survival. Results: A total of 119 patients (2.8%) with RCS underwent OS. The average time from the primary OS was 59.4 ± 54.5 months (range, 2-204). Forty-nine patients (41%) were symptomatic. In 103 patients (87%), the technique did not differ from a primary approach. Postoperative (<30 days) combined stroke and death rate was 3.3%. Cranial nerve injury occurred in 5 cases (4.2%). With a mean follow-up of 53 ± 48 months (range, 1-204), 3 patients had an ipsilateral stroke (including one hemorrhagic stroke) and 7 were diagnosed with a tertiary RCS >50%. At 5 years, Kaplan-Meier estimates of freedom from reintervention, freedom from restenosis and occlusion, freedom from neurologic event, and survival were 99%, 91%, 89%, and 91%, respectively. Conclusion: OS for RCS is not a high-risk procedure and provides excellent long-term results, with low rates of tertiary RCS and reinterventions. The comparison between OS and CAS in this indication suffers from the absence of standardized follow-up paradigms after primary OS and the lack of prospective randomized trial comparing the two techniques. Despite these limitations in the available data, we conclude that OS should remain the first line therapy when treatment of RCS is indicated. [Copyright &y& Elsevier]

Takayasu arteritis (TAK) is a large vessel vasculitis resulting in artery wall remodeling with segmental stenosis and/or aneurysm formation. Mast cells (MCs) are instrumental in bridging cell injury and inflammatory response. This study sought to investigate the contribution of MCs on vessel permeability, angiogenesis, and fibrosis in patients with TAK. MC activation and their tissue expression were assessed in sera and in aorta from patients with TAK and from healthy donors (HDs). In vivo permeability was assessed using a modified Miles assay. Subconfluent cultured human umbilic vein endothelial cells and fibroblasts were used in vitro to investigate the effects of MC mediators on angiogenesis and fibrogenesis. This study found increased levels of MC activation markers (histamine and indoleamine 2,3-dioxygenase) in sera of patients with TAK compared with in sera of HDs. Marked expression of MCs was shown in aortic lesions of patients with TAK compared with in those of noninflammatory aorta controls. Using Miles assay, this study showed that sera of patients with TAK significantly increased vascular permeability in vivo as compared with that of HDs. Vessel permeability was abrogated in MC-deficient mice. MCs stimulated by sera of patients with TAK supported neoangiogenesis (increased human umbilic vein endothelial cell proliferation and branches) and fibrosis by inducing increased production of fibronectin, type 1 collagen, and α-smooth muscle actin by fibroblasts as compared to MCs stimulated by sera of HD. MCs are a key regulator of vascular lesions in patients with TAK and may represent a new therapeutic target in large vessel vasculitis. [ABSTRACT FROM AUTHOR]

Background: Involvement of the inferior vena cava (IVC) is a controversial risk factor for surgical treatment of adrenocortical carcinoma (ACC). This study aims to assess the outcome of an aggressive surgical policy for ACC extending into the IVC and discuss treatment strategies based on a review of the literature. Methods: Over a 25-year period, 15 patients were treated for ACC extending into the IVC. The upper limit of the extension was the infrahepatic IVC in 2 patients, retrohepatic IVC in 6, and suprahepatic IVC in 7, including 4 with extension into the right atrium. Seven patients presented with concurrent metastases. The operative technique was thrombectomy (n = 13), partial resection with direct closure (n = 1), and total resection with replacement of the IVC (n = 1). Venous control was achieved by caval clamping alone (n = 4), hepatic vascular exclusion (n = 5), and the use of normothermic cardiopulmonary bypass or hypothermic circulatory arrest (n = 6). Results: Two patients died postoperatively. Ten patients died of metastatic complications at 4 to 31 months. Median survival time was 8 months. Three patients were still alive after 24, 25, and 45 months of follow-up, one of whom was reoperated at 17 months for a local recurrence. No evidence of recurrent intravenous involvement was found during follow-up in any patient in whom complete resection was achieved. Conclusions: Our findings suggest that surgical treatment can be effective for management of ACC with extension into the IVC. Long-term prognosis is poor owing to delay in diagnosis, frequent associated metastatic disease and lack of effective adjuvant treatment. [Copyright &y& Elsevier]

Purpose: This report presents our experience with open repair of post-traumatic aneurysms of the aortic isthmus using recent surgical techniques, including distal aortic perfusion and the preferential use of direct aortoaortic anastomosis without interposition of prosthetic material. Methods: From 1990 to 2004, the senior author (EK) patients (21 men; mean age, 40.3 years) who presented with post-traumatic aneurysms of the aortic isthmus were treated operatively, either with (20 patients) or without (3 patients) distal aortic perfusion, or endovascularly with a stent graft (3 patients). In 15 (75 %) of the 20 patients treated with distal aortic perfusion, the technique consisted of resection followed by direct aortoaortic anastomosis. Eight patients, including the three patients treated with simple clamping, had prosthetic replacement. Results: No postoperative deaths or permanent spinal cord complications occurred. One patient required reoperation to control hemorrhage. Aortography or computed tomography angiography was performed on 12 of the 15 patients treated by direct aortoaortic anastomosis, with a mean follow-up of 58.7 ± 8.9 months. No morphologic abnormality was found. Conclusion: This study shows that low-risk patients with a chronic post-traumatic aneurysm of the aortic isthmus can be successfully treated with excellent long-term results by resection and direct aortoaortic anastomosis without prosthetic interposition. In our opinion, endovascular repair should only be used in patients who present with absolute contraindications for open surgical repair. [Copyright &y& Elsevier]

Spontaneous dissection of the vertebral artery (VA) is usually managed medically. The objective of this report was to describe 10 patients treated surgically for spontaneous dissection of the VA. Seven men and three women with a mean age of 52.5?±?11.3 years were treated between December 1978 and January 2001. In eight cases the presenting symptom was neck pain. Transient ischemic attack or completed stroke in the vertebrobasilar distribution followed in nine cases. In the remaining case, symptoms resulted from irritation of the superior roots of the brachial plexus. Dissection was located in one segment of the VA in seven cases and two contiguous segments in three cases. Lesions involved aneurysm in seven cases, tight stenosis in two, and occlusion in one. The decision to perform surgery was made because of either continued symptoms despite maximal anticoagulation therapy or the presence of an aneurysm causing recurrent thromboembolism or threatening rupture. In eight cases the revascularization procedure consisted of bypass from the carotid artery to the distal VA. In the remaining two cases revascularization was achieved by transposition of the VA directly onto the common or internal carotid artery. Postoperative recovery was uneventful in all cases. No further neurological events were observed in any patient at a mean follow-up of 96.9?±?66 months (range, 12-216 months). Long-term resolution of vertebrobasilar symptoms was achieved in all cases, including one patient whose bypass occluded at 6 months. From these results we conclude that surgical treatment is the method of choice for spontaneous dissection of the extracranial VA associated with continued vertebrobasilar symptoms despite maximal medical therapy or with an expanding aneurysm. The most useful technique is bypass to the distal VA. Morbidity is low and long-term outcome is excellent. [ABSTRACT FROM AUTHOR]

From June 1974 to December 2001 we performed operative treatment on 33 patients with descending thoracic or thoracoabdominal aortic aneurysm in association with Takayasu disease. There were 25 men and 8 women with a mean age of 40.2 years (range 16-64 years). Nineteen patients came from North Africa, 6 were from France, and 8 were from various locations in the world. The revealing symptom was hypertension in 12 cases, thoracic or abdominal pain in 7, isolated inflammatory syndrome in 5, neurologic or ocular manifestations in 3, rupture in 3, and embolization to the lower extremity in 1. In the remaining two cases discovery was coincidental. The aneurysm was confined to the thoracic aorta in 10 cases and involved both the thoracic and abdominal aorta in 23 cases. There were 8 type I, 6 type II, 4 type ill, and 5 type IV aneurysms according to Crawford's classification. Two patients had undergone previous repair of the thoracoabdominal aorta. Four patients required first-stage treatment of a renal artery lesion to control hypertension. Six patients had associated aneurysms of the proximal aorta, including five treated via the distal elephant trunk technique in first-stage procedures. Aneurysm repair consisted of prosthetic replacement of the thoracoabdominal aorta in 31 cases, exclusion bypass in 1 case, and stent graft placement in 1 case. The procedure was performed with cross-clamping alone in 13 cases, distal perfusion in 17 cases, and deep hypothermic circulatory arrest in 3 cases. Twenty patients (61 %) had associated renal and/or intestinal artery lesions that were treated during the same procedure as that for the thoracoabdominal aorta in 19 patients (58%). A total of 24 procedures were performed on renal arteries (17 revascularizations, 7 nephrectomies). Associated supraaortic trunks lesions were present in 15 patients (45%) and were treated in 12 patients, including 8 in first-stage procedures prior to thoracoabdominal aortic aneurysm repair. Three patients died of multiple organ failure, after reoperation in two cases and infection in one case involving prior long-term corticosteroid therapy. Three patients developed paraplegia, including one who had undergone emergency treatment following rupture. Two patients required reoperation, for hematoma in one case and bowel necrosis in one. Four patients developed respiratory complications requiring artificial ventilation for more than 48 hr. During follow-up, two patients died from complications after repair of the proximal aorta and one patient required nephrectomy. Despite the extent of aneurysmal lesions and high frequency of association with visceral and supraaortic vessel lesions, the outcome of surgery in patients presenting with descending thoracic or thoracoabdominal aortic aneurysm in association with Takayasu disease was satisfactory. [ABSTRACT FROM AUTHOR]