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 po...

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Published inCardiovascular pathology Vol. 25; no. 2; pp. 113 - 115
Main Authors Hall, E. Tyler, Gibson, Blake A, Hennemeyer, Charles T, Devis, Paola, Black, Stacey, Larsen, Brandon T
Format Journal Article
LanguageEnglish
Published United States Elsevier Inc 01.03.2016
Subjects
Fatal Outcome
Female
Fibromuscular dysplasia
Fibromuscular Dysplasia - pathology
Humans
Middle Aged
Pathology
Segmental arterial mediolysis
Tunica Media - pathology
Vascular Diseases - pathology
Segmental arterial mediolysis
Fibromuscular dysplasia
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Summary: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.
Bibliography:ObjectType-Case Study-2
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ISSN:1054-8807
1879-1336
DOI:10.1016/j.carpath.2015.11.004