Gastric pseudoaneurysm in the setting of Loey’s Dietz Syndrome

Abstract

Loey’s Dietz syndrome is a disorder of connective tissue caused by a mutation in the genes that encode transforming growth factor (TGF) beta receptor 1 and 2. It is an autosomal dominant disorder similar to Marfan’s syndrome but with a more aggressive clinical course. Patients with Loey’s-Dietz syndrome have progressive dilatation of the aortic root that can lead to aortic dissection and rupture. The location of non-aortic arterial aneurysms may be wide spread but often occur in the head and neck vessels.