Objective: The management of vascular anomalies is complex and requires a multidisciplinary team with a combination of medical, surgical, and intervention treatments. Medical treatment is limited and has conflicting results. Off-label use of mammalian target of rapamycin inhibitors shows promising results. The objective of this study was to systematically evaluate the literature published about the efficacy and safety of sirolimus in the treatment of vascular anomalies. Methods: A systematic review of the published literature was conducted using the PubMed database and Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Results: There were 73 articles included: 2 randomized controlled studies, 2 nonrandomized prospective studies, and 69 retrospective case reports and case series. In total, 373 patients were included. Sirolimus was administered topically to 56 patients and orally to 317 patients. Sirolimus was highly effective in the treatment of vascular tumors associated with Kasabach-Merritt phenomenon (95.5% of the patients clinically improved and 93% had normalization of coagulopathy), venous malformations (size reduction was observed in 88.9% of patients), and lymphatic malformations (clinical improvement in 94.9% of patients). Topical sirolimus results were conflicting. Arteriovenous malformations were not improved by sirolimus. Conclusions: Low-level evidence suggests that sirolimus can improve the prognosis of vascular anomalies, most notably vascular tumors associated with life-threatening coagulopathy and venous and lymphatic malformations. Further research is needed to establish the benefits of sirolimus in the management of vascular anomalies.

Raynaud's phenomenon and digital ulcers (DUs) are frequent among systemic sclerosis (SSc) patients. Our aim was to investigate the diagnostic and predictive value for DU of endothelial dysfunction biomarkers (flow-mediated dilatation (FMD), serum levels of endothelin-1 (ET-1), and ADMA), angiogenic/angiostatic biomarkers (vascular endothelial growth factor (VEGF), endoglin, and endostatin), and nailfold videocapillaroscopy (NVC). We compared our results with a literature review. In a cohort study of 77 SSc patients, we followed two groups of patients: (i) naïve DU patients (39) and (ii) active DU at baseline (38 patients) for 3 years. Telangiectasia (p < 0.001) and diffuse disease subset (p = 0.001) were significantly more frequent in patients with active DU at enrolment. Additionally, NVC late scleroderma pattern (AUC 0.846, 95%CI 0.760-0.932), lower values of FMD (AUC 0.754, 95%CI 0.643-0.864), increased serum levels of ET-1 (AUC 0.758, 95%CI 0.649-0.866), ADMA (AUC 0.634, 95%CI 0.511-0.757), and endoglin as well as low VEGF serum levels (AUC 0.705, 95%CI 0.579-0.830) were significantly associated to new DU events in the 3-year follow-up. Cox regression analysis showed that FMD > 9.41 % (HR 0.37, 95%CI 0.14-0.99); ET-1 >11.85 pmol/L (HR 3.81, 95%CI 1.41-10.26) and late NVC pattern (HR 2.29, 95%CI 0.97-5.38) were independent predictors of DU recurrence. When estimating the probability of occurrence of first DU in naïve DU patients, only late NVC pattern (HR 12.66, 95%CI 2.06-77.89) was an independent predictor factor. In conclusion, late scleroderma patterns in NVC are the best independent predictors of SSc patients who are at risk of developing DU. Endothelial dysfunction assessed by FMD and ET-1 was also found to be an independent predictor of DU recurrence in a 3-year follow-up.

ObjectiveInternal iliac artery aneurysms (IIAA) are rare, representing only 0.3% of aortoiliac aneurysms. Its treatment with open surgery is complex and associated with high morbidity and mortality, which led to increasing application of endovascular solutions. In this study, we aimed to evaluate outcomes of endovascular aneurysm repair (EVAR) of IIAA in one institution.MethodsWe retrospectively reviewed all cases of IIAA treated with endovascular techniques between 2003 and 2014. Endpoints were morbidity, mortality, freedom from pelvic ischemic symptoms (buttock claudication, ischemic colitis, and spinal cord injury), and need for reintervention.ResultsThere were 16 patients, 13 males and 3 females, with mean age of 75.1±7 years. A total of 20 IIAA (4 cases were bilateral), with mean diameter of 37.9 mm, were treated. EVAR was performed in 13 (81.3%) patients, with associated internal iliac artery's outflow occlusion in 2. Iliac branch device was used in one patient. Two patients underwent endovascular IIAA embolization alone. One patient underwent percutaneous, transgluteal, IIAA embolization. IIAA flow preservation in at least one internal iliac artery was possible in 9 (56.3%) patients. Early mortality was 7% (1 case). Early morbidity was 18.8%. Pelvic ischemic complications occurred in 1 (7%) patient with buttock claudication. Late reintervention was needed in 3 patients, none of them for IIAA related complications.ConclusionEndovascular treatment of IIAA is technically feasible and durable. Although overall morbidity is relatively high, major complications are infrequent and perioperative mortality is low. internal iliac artery flow preservation is technically challenging and, in a significant number of cases, not possible at all.

Patients ≥75 years old undergoing AVR presented good medium-term survival. Predictors of an adverse outcome were significant pulmonary hypertension and diabetes. At follow-up, most achieved improvement of QoL and remained autonomous. These results stress that excellent long-term outcomes with AVR can be achieved in appropriately selected elderly patients.