• Vol. 48 No. 5
  • 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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