Nonatherosclerotic Obstructive Vascular Diseases of the Mesenteric and Renal Arteries

Abstract

Nonatherosclerotic vascular diseases of the mesenteric and renal arteries are considered to occur less frequently than those caused by occlusive atherosclerotic disease. However, when present, they pose a significant diagnostic and therapeutic challenge. Such disorders include fibromuscular dysplasia, median arcuate ligament syndrome, the renal nutcracker syndrome, and some forms of acute and chronic mesenteric ischemia (embolic and thrombotic). This is a heterogeneous group of disorders with substantial differences in the pathogenesis and diagnostic approaches to these diseases. We provide an overview of the pathogenesis, clinical presentation, diagnosis, and current management of fibromuscular dysplasia, median arcuate ligament syndrome, and the renal nutcracker syndrome.

An unusual case of left renal artery compression: a rare type of median arcuate ligament syndrome

“…Usually, the MAL crosses the aorta at the level of L1; therefore, it is located above the origin of the CT. However, in 10 to 24 % of the overall population, inadequate caudal migration of the artery during embryogenesis or low insertion of the ligament produces compression of the proximal part of the CT [ 4 , 7 ].…”

“…A review of literature reveals that the prevalence of the syndrome is two per 100,000 patients and a female to male ratio is 2–3:1 [ 12 ]. It rarely causes ischemia or decreased blood flow to abdominal organs, leading to pain [ 4 , 6 ]. Arterial constriction by the diaphragmatic crura has also been reported to involve multiple vessels [ 2 , 9 ].…”

Awareness can prompt the search for clinical zebras

“…The pattern of fi ndings (stenoses, aneurysms, or dissections), in the context of the clinical history and examination, helps to distinguish atherosclerosis and vasculitis from their mimics. 4,5 As Rodriguez points out, diagnosing the uncommon requires "meticulous evaluation to rule out more common pathology." 3 Circling back to my original effort to understand what prompts me, or any clinician, to look hard for the uncommon causes of common symptoms, it seems to be the gestalt that speaks to some part of the total patient presentation that doesn't quite fi t the expected.…”

Type A Aortic Dissection in Fibromuscular Dysplasia

“…To the Editor: Fibromuscular dysplasia (FMD) is a rare, noninflammatory vascular disease characterized by arterial stenosis and aneurysm or dissection most commonly affecting renal, carotid, and vertebral arteries. 1 Although multiple arterial involvement has been described in the literature, [2][3][4][5] no surveillance strategies are in place, which often results in catastrophic consequences.…”