Marfan syndrome

Case contributed by Dr Mostafa Mahmoud El Feky

Presentation

Low back pain, urinary incontinence.

Patient Data

Age: 40
Gender: Female
CT

 Thoracic aorta:

  • Extensive aortic root dilatation measuring up to 9.6 cm regarding its maximum dimensions as well as the sino-tubular junction, this is associated with lumbosacral dural ectasia manifested as scalloping of the dorsal surface of the vertebrae with pre-sacral and lateral sacral meningioceles.
  • Gross cardiomegaly.
  • Normal caliber of the ascending, arch and descending thoracic aorta, with no stenotic or dilated segments. The visceral branches of the arch of aorta (left subclavian, left common carotid and brachiocephalic arteries) are patent showing normal course and caliber. No evidence of aneurysmal dilatation. No dissection flap seen.

 Abdominal aorta:

  • Normal caliber of the supra and infra-renal abdominal aorta, with no stenotic or dilated segments. The visceral branches of the abdominal aorta (celiac trunk and inferior mesenteric arteries) are patent showing normal course and caliber.

Case Discussion

The findings on this CT that suggests Marfan syndrome as the unifying diagnosis include:

  • diffuse dural ectasia
  • enlarged sacral foramina, presacral and lateral sacral meningocoele with scalloping of the dorsal surface of vertebral bodies
  • dilated aortic root with multichamber gross cardiac enlargement and passive congestive hepatomegaly
PlayAdd to Share

Case information

rID: 44561
Case created: 27th Apr 2016
Last edited: 21st Apr 2017
Inclusion in quiz mode: Included

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.