• Vol. 48 No. 5, 165–165
  • 15 May 2019

Small Head with Big Vessels



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A 46-year-old gentleman with normal intellectual and cognitive function presented to the emergency department with acute left-sided chest pain and palpitations. He was of normal stature, although joint hyperextensibility and microcephaly were noted on examination. Upon further history acquisition, a positive maternal family history of vascular disorder was obtained. Computed tomography (CT) aortogram was performed and showed intramural haematoma of the ascending aorta, along with the presence of dural ectasia (Fig. 1). He subsequently underwent emergency aortic repair. During the postoperative period, he developed facial twitching and neuroimaging studies of the brain were performed (Fig. 2). What is the most likely diagnosis?

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