Segmental arterial mediolysis: a systematic review of 85 cases.

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.