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Author Spotlight: Advancing Spectral Characterization of Physiological and Malperfused Tissues
Published on: July 5, 2024
Segmental arterial mediolysis
Anil Kumar Pillai1, Shams I Iqbal, Raymond W Liu
1Department of Radiology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA, anil.pillai@utsouthwestern.edu.
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.
Insights
Segmental arterial mediolysis (SAM) is a rare arteriopathy causing dissecting aneurysms and rupture. While often self-limiting, acute presentations carry a 50% mortality risk, requiring prompt intervention.
Area of Science:
- Vascular Medicine
- Pathology
- Radiology
Background:
- Segmental arterial mediolysis (SAM) is a rare, nonatherosclerotic arteriopathy affecting large to medium-sized arteries.
- Histologically, it involves vacuolization and lysis of the outer media, leading to dissecting aneurysms and potential vessel rupture.
- Clinical presentation ranges from self-limiting abdominal pain to life-threatening hemorrhage.
Purpose of the Study:
- To review the characteristics, diagnosis, and management of Segmental arterial mediolysis.
- To highlight key distinguishing features of SAM from other vasculopathies.
- To discuss the acute and long-term prognosis of SAM.
Main Methods:
- Literature review of Segmental arterial mediolysis cases.
- Analysis of histological findings and imaging characteristics.
- Review of clinical presentations, treatment strategies, and outcomes.
Main Results:
- SAM affects all ages, with a predilection for the 5th and 6th decades and a slight male predominance.
- Key imaging findings include segmental dissections of the celiac, mesenteric, and/or renal arteries.
- Inflammatory markers and specific genetic/coagulable studies are typically negative, differentiating SAM from other vasculitides.
Conclusions:
- Segmental arterial mediolysis is a distinct arteriopathy with specific diagnostic criteria.
- Acute SAM with aneurysmal rupture has a high mortality rate (50%), necessitating urgent surgical or endovascular treatment.
- Conservative management is appropriate for non-acute cases, with blood pressure control being the cornerstone of therapy.
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