Segmental arterial mediolysis: a precursor to fibromuscular dysplasia?

We describe five cases of segmental arterial mediolysis (SAM), formerly known as segmental mediolytic arteritis. SAM occurs in epicardial coronary arteries and in the abdominal splanchnic arteries. Patients with abdominal SAM are generally elderly, whereas coronary SAM presents in neonates, children, and young adults. SAM is initiated by mediolysis of the outer media, which can expand to involve the mid- and inner media. Accompanying alterations include fibrinous linear deposits at the medial adventitial junction and replacement of the lysed muscle fibers by fibrin, erythrocytes, and granulation tissue. Transmural mediolysis results in arterial wall gaps frequently complicated by dissecting hematomas and aneurysms. Abdominal hemorrhages stem from these complications. SAM involving abdominal splanchnic arteries clinically presents with abdominal pain and distension, falling hematocrit, and shock. Simultaneous involvement of more than one abdominal artery is frequent, and branches of the celiac axis are most commonly affected. Various disease states that provoke pathologic stimuli for endothelial mediated vasoconstriction occur in the immediate clinical background of patients with SAM. Certain morphologic features of SAM suggest that this arterial lesion is due to vasospasm, which we putatively ascribe to focal endothelial paracrine dysfunction. Organization of uncomplicated SAM lesions could resemble certain types of fibromuscular dysplasia, suggesting that the genesis of such arterial lesions is related to vasospasm.