An incidental finding of localized aortic arch dissection in a polytraumatized patient. A case report and state of the art of non-A non-B aortic dissection.

Non-A non-B aortic dissection is considered a rare nosological entity, included in the Stanford classification, representing a small percentage of the total aortic dissections that occur annually. Regarding this form, the literature reports a more complicated disease course compared to other types of dissection. We describe the case of a 76-year-old patient who accessed the triage section of an emergency department for a polytrauma picture and, after surgical treatment of a leg fracture, received a diagnosis of non-A non-B aortic dissection, "localized" to the arch and incidentally detected. The angio-computed tomography (CT) showed that the only intimal tear was located in the central portion of the aortic arch, with no exit tear. No signs of malperfusion or clinical symptoms related to the aortic finding were evident. The Aortic Team decided on a conservative approach, whereby the patient was started on medical therapy to control blood pressure in a monitored bed of a semi-intensive care unit. The persistent asymptomatic state, a condition of hemodynamic stability, and an unchanged angio-CT picture enabled discharge on day 7 and the assignment to a close follow-up.

Renal involvement in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL): report of a case with a six-year follow-up.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a disorder of the cerebral small blood vessels caused by a mutation in the NOTCH3 gene, which encodes a large transmembrane receptor NOTCH3. It is associated with systemic arteriopathy involving small arteries, besides the brain, in skin, spleen, liver, muscle, aorta and in the kidney. The key pathological finding is the accumulation of granular osmiophilic material (GOM) on degenerating vascular smooth muscle cells. In the kidney GOMs have been described only in a very limited number of CADASIL patients. We describe a genetically confirmed CADASIL patient with mild renal dysfunction and GOMs in the interlobular and juxtaglomerular arteries and, for the first time, also within the glomerulus, whose nephrology conditions remained stable, whereas the neurological manifestations markedly worsened over a six-year follow-up period. The reasons for this discrepancy are probably related to differences in the structure and function of brain and kidney blood vessels.