Marfan syndrome - vascular manifestations

Case contributed by RMH Report Writing

Presentation

Known Marfan syndrome. Now presenting with acute onset chest pain. Suspected dissection, has a history of aortic stent, investigate possible endoleak.

Patient Data

Age: 30 years
Gender: Male
CT

CT Angiogram Chest and Abdomen

No previous imaging available for comparison.

An intimal flap is identified in the aortic arch arising at the level of the origin of the left subclavian artery and extending into the mid-descending thoracic aorta at the level of T6. The intimal flap extends into the left subclavian artery for a short distance.

Left subclavian artery branches opacify normally and arise from the true lumen. The left vertebral artery appears to arise from the the edge of the dissection flap posteriorly from the false lumen.

it appears dominant and  opacifies normally.

Endovascular repair of thoracoabdominal aortic aneurysm. No extravasation of contrast to suggest leak.

Imaged abdominal viscera are unremarkable. The lungs are clear. No mediastinal, hilar or axillary lymphadenopathy. Sternotomy wires. No suspicious bony lesion.

Conclusion:

Type B aortic dissection extending from the origin of the left subclavian artery to the mid-descending thoracic aorta at the level of T6. The intimal flap extends into the left subclavian artery.

Case Discussion

The CT reveals a type 2 endoleak and a dissection of the descending thoracic aorta

Marfan syndrome is a multisystem connective tissue disease with autosomal dominant inheritance which primarily involving skeleton, eyes, and cardiovascular systems. It results from a defect in fibrillin 1 (FBN1) gene located on chromosome 15q21.1 which is responsible for cross-linking collagen.

 

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Case information

rID: 40612
Case created: 28th Oct 2015
Last edited: 8th Feb 2017
Tag: rmh
Inclusion in quiz mode: Included

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