Segmental arterial mediolysis.
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| Abstract |
Segmental arterial mediolysis (SAM) is an uncommon, nonatherosclerotic, noninflammatory, large- to medium-sized arteriopathy first described in 1976. It is characterized histologically by vacuolization and lysis of the outer arterial media leading to dissecting aneurysms and vessel rupture presenting clinically with self-limiting abdominal pain or catastrophic hemorrhages in the abdomen. Patients of all ages are affected with a greater incidence at the fifth and sixth decades. There is a slight male predominance. Imaging findings overlap with inflammatory vasculitis, collagen vascular disease, and fibromuscular dysplasia. The presence of segmental dissections involving the celiac, mesenteric, and/or renal arteries is the key distinguishing features of SAM. Inflammatory markers, genetic tests for collagen vascular disorders, and hypercoagulable studies are negative. Anti-inflammatory agents and immunosuppressants are not effective. A mortality rate of 50 % has been attributed to the acute presentation with aneurysmal rupture necessitating urgent surgical or endovascular treatments; in the absence of the acute presentation, SAM is a self-limiting disease and is treated conservatively. There are no established guidelines on medical therapy, although optimal control of blood pressure is considered the main cornerstone of medical therapy. The long-term prognosis is not known.
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| Authors | Anil Kumar Pillai, Shams I Iqbal, Raymond W Liu, Niranjan Rachamreddy, Sanjeeva P Kalva |
| Journal | Cardiovascular and interventional radiology (Cardiovasc Intervent Radiol) Vol. 37 Issue 3 Pg. 604-12 (Jun 2014) ISSN: 1432-086X [Electronic] United States |
| PMID | 24554198 (Publication Type: Journal Article, Review) |
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