Segmental Arterial Mediolysis: A Systematic Review of 85 Cases

doi:10.1016/j.avsg.2013.03.003

Annals of Vascular Surgery

Volume 28, Issue 1, January 2014, Pages 269-277

https://doi.org/10.1016/j.avsg.2013.03.003 Get rights and content

Background

Segmental arterial mediolysis (SAM) is a nonatherosclerotic, noninflammatory arteriopathy of unknown etiology with life-threatening manifestations. With advances in endovascular techniques, SAM is increasingly being managed without the need for major surgery.

Methods

A systematic review of the literature published on SAM between 1976 and 2012 was performed, focusing on arterial involvement, diagnostic imaging modalities, mortality and morbidity rates, and in particular treatment outcomes with open versus endovascular intervention.

Results

Sixty-two studies reporting on 85 cases of SAM were reviewed. Sixty-nine percent of cases were diagnosed histologically (24% on autopsy). Angiography was the most common form of diagnostic imaging modality (56% of cases). Arterial involvement was largely abdominal or cranial, with splenic arterial involvement being the most prevalent (29% of cases). There was a total SAM-related mortality of 26%. Endovascular intervention, most commonly in the form of coil embolization of aneurysmal vessel(s), was successful in 88% of cases where attempted, with no reported mortality. There was a mortality rate of 9% where open surgery was attempted.

Conclusions

Catheter-based endovascular techniques can be a successful, minimally invasive treatment option in the management of this potentially life-threatening condition, and may also provide a temporary bailout measure in the acute phase before definitive surgical treatment at a later stage.

Introduction

Segmental arterial mediolysis (SAM) is a nonatherosclerotic, noninflammatory arteriopathy of unknown etiology.¹ It is characterized by lysis of the medial layer of the arterial wall, which may result in dissection, stenosis, occlusion, and aneurysm formation.2, 3 Aneurysm rupture commonly presents with life-threatening abdominal, retroperitoneal, or intracranial haemorrhage,⁴ with a previously estimated mortality rate approaching nearly 50%.⁵ Consideration of this pathologic process is therefore important in the differential diagnosis of patients presenting with an acute abdomen, evidence of hemorrhage, or neurologic symptoms.

With recent advances in endoluminal techniques for diagnosis and treatment, minimally invasive endovascular intervention might serve as an attractive therapeutic option to these challenging clinical scenarios. We present a systematic review of the published literature on SAM, with a particular focus on outcomes and mortality with endovascular versus open surgical intervention.

Section snippets

Study Selection

The literature was systematically reviewed to identify all published studies relating to SAM. A public domain database (MEDLINE) was targeted using a Web-based search engine (PubMed) for articles published between 1976 and 2012. Key words included “segmental arterial mediolysis” and previously used descriptions for the disease—“segmental mediolytic arteriopathy” and “segmental mediolytic arteritis.” All abstracts, case reports, patient series and citations scanned were reviewed. References of

Results

The literature search identified 62 studies that met the inclusion criteria and were included in the review. Table I outlines the included studies, the number of cases reported in each study, whether the diagnosis was histologic, any attempted management, and reported outcomes.

The studies reported a total of 85 cases of SAM, of which 51 were men and 34 were women (male:female ratio: 1.5:1). The median age was 57 years (range: 0–91 years). Eighteen patients (21%) were hypertensive, but there

Discussion

SAM was first described by Slavin et al.,⁶⁴ who reported 3 autopsy cases of ruptured aneurysms resulting in massive hemorrhage and death. Since then, additional case reports and advances in diagnostic techniques have increased the understanding of the disease course, sequelae, and prognosis.⁴ SAM is a pathologic diagnosis defined by characteristic histologic and radiographic features. Mediolysis, caused by disruption of the smooth muscle cell membrane,⁶³ results in loss of the supporting

Conclusion

SAM can present with potentially fatal intra-abdominal hemorrhage. Early recognition and appropriate diagnosis and management can lead to significant reduction in subsequent complications and mortality. Endovascular intervention can be a reliable and minimally invasive treatment option, and may also provide a temporary bailout measure in the acute phase before definitive treatment at a later stage. Additional studies are required to identify the exact etiology, predisposing factors, and the

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