Stent-assisted transcatheter coil embolization of wide-necked renal artery aneurysms

Learning Objectives: 1. Describe the role of endovascular procedures in vascular connective tissue disease (CTD) related emergencies. 2. Demonstrate the effective use as well as potential complications following endovascular repair for vascular CTD related emergencies. 3. Review the most common vascular CTD’s and the most frequently associated vascular emergencies. Background: While rare, connective tissue diseases are responsible for an important subset of vascular emergencies. The predominant pathologies include Marfan Syndrome (Prevalence 1/5,000), Ehlers Danlos Syndrome (EDS) Type IV (Prevalence 1/90,000), and Loeys-Dietz Syndrome (LDS, Prevalence 1/1,000,000). These patients have shortened lifespans and can present with acute arterial dissections, aneurysms, and ruptures. Marfan syndrome often presents radiographically with anuloaortic ectasia, valvular disease, and/or aortic dissection. EDS presents at a young age with multi-territory aneurysm formation, dissection, occlusion, and/or vascular rupture. LDS is a recently recognized and more aggressive pathology in which aortic dissection is the most common cause of death. Clinical Findings/Procedure Details: Endovascular management of acute dissections, aneurysms, and even ruptures is now common practice in post-traumatic, atherosclerotic, and iatrogenic scenarios. However, there is a paucity of literature regarding its use in the subset of patients with a vascular CTD. The existing literature for the most part recommends avoidance of an endovascular approach and defers to open surgical intervention. We describe here the successful use of endovascular techniques as a rescue therapy in this patient population. With appropriate and close imaging follow-up this management strategy can serve as either definitive treatment or as a bridge/adjunct therapy to surgical management. Conclusions: CTD’s are an important group of pathology that need to be included in the differential diagnosis of young patients with acute vascular emergencies. This patient population can undergo successful endovascular management despite the increased vessel fragility and propensity for postprocedural complications. Following treatment, close interval follow-up is essential for continued clinical success.