Acute dissection into a large abdominal aortic aneurysm in a patient with Marfan syndrome

Synchronous aortic dissection (AD) with involvement of pre-existing infrarenal abdominal aortic aneurysms (AAAs) are fortunately rare. Extension of the dissection flap to involve the aneurysm is particularly dangerous with pressurization of the false lumen within a diseased aortic wall. The aim of this article is to present a unique case of an acute AD with extension to involve a large AAA in a patient with an underlying connective tissue disorder.

A 24-year-old male Marfan syndrome patient with prior type A dissection repair presented with a descending thoracic AD and a separate 7.0-cm infrarenal AAA. He was initially medically managed but developed acute extension of the dissection flap to involve the large aneurysm.

The patient was initially treated with open infrarenal aortic replacement under left atrialfemoral artery bypass. Three years later, he developed degeneration of the residual aorta and underwent a two-stage hybrid repair. He underwent an additional stent graft for a distal anastomotic leak. At 5 years of follow-up, there was positive aneurysm remodeling and sac regression.

This case illustrates the challenges in treating a patient with synchronous aortic processes. False lumen pressurization of a large aneurysm wall is fortunately rare but can be lethal. Prompt intervention is required but the presence of an underlying connective tissue disorder may limit endovascular treatment options. Careful case planning is mandatory and multiple interventions should be anticipated.