Patients with NF-I have a wide spectrum of vascular abnormalities, most notably aneurysms or stenoses of the aortic, renal, and mesenteric circulation. Operative treatment of symptomatic patients with vascular lesions or large aneurysms is safe, effective, and durable.

Saccular aneurysms (especially those with lobulated contour) with rapid expansion or development and adjacent mass, stranding, and/or fluid in an unusual location are highly suspicious for an infected aneurysm.

Endovascular repair of pararenal aortic aneurysms and TAAAs, using manufactured F-BEVAR with supraceliac sealing zones, is safe and efficacious. Long-term follow-up is needed to assess the impact of four-vessel designs on device-related complications and progression of aortic disease.

CT and MRI findings of TA include vascular wall thickening and enhancement early in the disease, and arterial stenoses, occlusions, and aneurysms later in the disease. Cross-sectional imaging is useful for establishing the diagnosis of TA and for showing response to nonsurgical therapy or for planning a surgical intervention.

Background: Aortic syndromes (AS), including aortic dissection (AD), intramural hematoma (IMH) and penetrating aortic ulcer (PAU), carry significant acute and long-term morbidity and mortality. However, the contemporary incidence and outcomes of AS are unknown. Methods and Results: We utilized the Rochester Epidemiology Project record linkage system to identify all Olmsted County, Minnesota, residents with AS (1995–2015). Diagnostic imaging, medical records, and death certificates were reviewed to confirm the diagnosis and AS subtype. Age- and sex-adjusted incidence rates were estimated using annual county-level census data. Survival for patients with AS was compared to age- and sex-matched controls using Cox regression to adjust for comorbid conditions. We identified 133 patients with AS (77-AD, 21-IMH, and 35-PAU). Average age was 71.8 years (SD 14.1) and 57% were male. The age- and sex-adjusted incidence was 7.7 per 100,000 person-years, was higher for males than females (10.2 vs. 5.7 per 100,000 person-years), and increased with age. Among subtypes, the incidence of AD was highest (4.4 per 100,000 person-years), while the incidence of PAU and IMH were lower (2.1 and 1.2 per 100,000 person-years). Overall, the incidence of AS was stable over time (p trend=.33), although the incidence of PAU appeared to increase from 0.6 to 2.6 per 100,000 person-years (p=.008) with variability over the study interval. Patients with AS had more than twice the mortality rate at 5, 10, and 20 years when compared to population-based controls (5, 10 and 20-year mortality 39%, 57%, 91% versus 18%, 41%, and 66%; overall adjusted mortality HR=2.1, p<0.001). Survival was lower than expected up to 90 days after AS diagnosis and did not differ significantly by subtype or by 5-year strata of diagnosis. Conclusions: Overall, the incidence of AD and IMH has remained stable since 1995, despite the decline noted for other cardiovascular disease. AS confers increased early and long-term mortality that has not changed. These data highlight the need to improve long term care to impact the prognosis of this patient group.