Intracranial Atypical Fibromuscular Dysplasia With Ruptured Aneurysm-Case Report-

Abstract

A 53-year-old woman was admitted with severe subarachnoid hemorrhage due to rupture of an aneurysm associated with atypical intracranial fibromuscular dysplasia (FMD). Angiography demonstrated the aneurysm and very irregular form of the left internal carotid artery (ICA), the right ICA, and right proximal middle cerebral artery (MCA). Other arteries showed signs of atherosclerosis. The aneurysm was treated by embolization, but she subsequently died of shock of unknown cause. Detailed examination of serial angiograms detected enlargement of the aneurysm and progression of the irregular appearance of the ICA. FMD is a non-inflammatory and non-atheromatous arteriopathy that commonly affects the cervical ICA and sometimes the intracranial ICA. The association with saccular aneurysm is widely known and the prevalence of incidental aneurysms is higher than that in the general population. The common``string of beads'' finding is easily distinguished from other vascular diseases, but non-specific findings such as``tubular stenosis'' and``diverticular-like outpouching'' are harder to differentiate. FMD is associated with various complications and appropriate periodic follow-up examination is required. Detailed analysis of serial angiograms may facilitate diagnosis of this condition.

A case of fibromuscular dysplasia related intracerebral hemorrhage without angiographically cerebral abnormal vessels

“…Most patients with FMD have a good clinical course; however, some of them with cerebrovascular disorders, called cerebrovascular FMD, including cerebral aneurysm or dissection, have a high risk of stroke and death; [10,11] thus, their outcome is worse. [8] Here, we experienced a case with intracerebral hemorrhage (ICH) due to FMD without findings of culprit aneurysm or dissection in cerebral arteries and presented with characteristic pathological findings.…”

Rete mirabile bei segmentaler Agenesie der A. carotis interna

“…Bei unserem Patienten 1 ist die histologisch nachgewiesene fibromuskuläre Dysplasie eine bekannte Ursache für die Entstehung extraund intrakranieller Stenosen und Aneurysmen [32]. Ein gemeinsames Auftreten von CRM und Pseudoxanthoma elasticum wurde ebenfalls berichtet [1,20].…”