title: Segmental arterial mediolysis and fibromuscular dysplasia: what comes first, the chicken or the egg?
abstract: Segmental arterial mediolysis (SAM) is a rare vasculopathy characterized by lysis of the outer media in splanchnic arteries and formation of dissecting pseudoaneurysms that may spontaneously rupture, leading to massive and often fatal intraabdominal hemorrhage. The pathogenesis of SAM is poorly understood. Healed SAM lesions closely resemble fibromuscular dysplasia (FMD), leading some authors to postulate that SAM represents a precursor to FMD despite distinct clinical differences between these two disorders. Herein, we present a 61-year-old woman with fatal SAM who showed histologic features in her aorta suggesting the opposite pathogenetic relationship, with an unclassified "FMD-like" arteriopathy preceding development of SAM.
authors.affiliation: Department of Medical Imaging, University of Arizona and Banner-University Medical Center, Tucson, AZ, USA.
authors.forename: E Tyler
authors.initials: ET
authors.lastname: Hall
authors.affiliation: College of Medicine, University of Arizona and Banner-University Medical Center, Tucson, AZ, USA; Department of Pathology, University of Arizona and Banner-University Medical Center, Tucson, AZ, USA.
authors.forename: Blake A
authors.initials: BA
authors.lastname: Gibson
authors.affiliation: Department of Medical Imaging, University of Arizona and Banner-University Medical Center, Tucson, AZ, USA.
authors.forename: Charles T
authors.initials: CT
authors.lastname: Hennemeyer
authors.affiliation: Department of Medical Imaging, University of Arizona and Banner-University Medical Center, Tucson, AZ, USA.
authors.forename: Paola
authors.initials: P
authors.lastname: Devis
authors.affiliation: Department of Medical Imaging, University of Arizona and Banner-University Medical Center, Tucson, AZ, USA.
authors.forename: Stacey
authors.initials: S
authors.lastname: Black
authors.affiliation: Department of Pathology, University of Arizona and Banner-University Medical Center, Tucson, AZ, USA. Electronic address: blarsen@email.arizona.edu.
authors.forename: Brandon T
authors.initials: BT
authors.lastname: Larsen
mesh_terms: D017809:Fatal Outcome; D005260:Female; D005352:Fibromuscular Dysplasia / Q000473:pathology*; D006801:Humans; D008875:Middle Aged; D017540:Tunica Media / Q000473:pathology*; D014652:Vascular Diseases / Q000473:pathology*
keywords: Fibromuscular dysplasia; Segmental arterial mediolysis