Renal arterial fibromuscular dysplasia: acute renal infarction in three patients with angiographic evidence of medial fibroplasia

Abstract

Fibromuscular dysplasia of the renal arteries usually presents with hypertension but, rarely, it can present with acute renal infarction. Of the four types of fibromuscular dysplasia, medial fibroplasia has been said not to progress to renal arterial occlusion (Schreiber et al, 1984) and not to predispose to arterial thrombosis or dissection (Stewart et al, 1970). We report on three patients who presented with acute renal infarction, whose renal angiographic features were more suggestive of the medial fibroplasia type of fibromuscular dysplasia. A 33-year-old man was admitted with severe right loin pain. The right kidney was non-functioning on intravenous urography, but an ultrasound examination of both kidneys was normal. A right retrograde pyelogram was normal, but dynamic renography (99Tcm-DTPA) revealed that the right kidney was not perfused. At angiography the right renal artery was completely occluded by thrombus 3 cm from its origin (Fig. 1). The left main renal artery was irregular and two stenoses were seen in one of the segmental vessels (Fig. 2). The thrombus in the right main renal artery was treated with intra-arterial streptokinase to reveal a 75% stenosis. This was treated with balloon angioplasty but peripheral perfusion could not be restored and the kidney remained non-functioning. The angiographic appearances were thought to represent the medial fibroplasia form of fibromuscular dysplasia. Subsequent investigations failed to reveal any other cause of arterial thrombosis or embolism.

Bilateral renal infarctions complicating fibromuscular dysplasia of renal arteries in a young male

“…To the authors' knowledge, bilateral renal infarction in the context of FMD has been reported only twice in the literature so far [11,15]. In patients with FMD, concomitant renal infarction is quite rare in the medial subtype, with most of those cases having either perimedial hyperplasia [16] or dissection [17]. The underlying mechanism of acute renal infarction with FMD is not clear, but it is speculated that shearinduced platelet activation may induce thrombus formation and subsequent embolization at the sites of aneurysms; in other cases, dissection is responsible [17].…”

A case of renal artery thrombosis with renal infarction

“…Bilateral infarction has also been reported,[3] including bilateral (global) renal infarction subsequent to dissecting aneurysms of the aorta,[13] with septic emboli from endocarditis, lupus vasculitis or with sickle cell disease[14] or fibromuscular dysplasia of the renal arteries. [1516] Reports of other causes associated with renal infarction include trauma, vasculitis, instrumentation, transplant, sepsis,[14] sickle cell disease[1417] and antiphospholipid antibody syndrome,[1819] cocaine use,[20] and subsequent to carotid artery dissection. [21]…”

Fibromuscular dysplasia presenting as a renal infarction: a case report

“…Progression to renal infarction in FMD is rare. Most patients who develop renal infarction have perimedial dysplasia [9]. In perimedial dysplasia, large parts of the media are replaced by collagen, with irregular thickening of the media which may lead to total occlusion.…”