Rare Mesenteric Arterial Diseases: Fibromuscular Dysplasia and Segmental Arterial Mediolysis and Literature Review

Abstract

Fibromuscular dysplasia (FMD) and segmental arterial mediolysis (SAM) are noninflammatory, nonatherosclerotic arterial diseases that cause aneurysm, occlusion, and thromboses. These diseases are rarely seen in mesenteric arterial lesions; however, as they can be lethal if appropriate management is not provided, the accumulation of clinical information from cases is essential. We herein report the cases of a 57-year-old man diagnosed with FMD and a 63-year-old man diagnosed with SAM. We conclude that an early diagnosis with imaging modalities and clinical information followed by the appropriate treatment improves the prognosis of these arterial diseases.

Segmental arterial mediolysis: differentiation of rare arteriopathy from vascular mimics

“…3 Therefore, SAM presents with a different clinical profile compared with FMD and can diagnosed clinically. 5 Our patient's demographics, presentation, laboratory and imaging were most consistent with SAM. We conclude by urging providers to consider SAM as a rare but dangerous diagnosis in patients with multiple aneurysmal findings.…”

A case of subarachnoid hemorrhage caused by multiple cerebral aneurysms due to segmental arterial mediolysis

“…[10] However, since pathological diagnosis cannot be made in all cases, SAM may be diagnosed only by diagnostic imaging or clinical findings. [11] FMD is histopathologically characterized by thickening or thinning of the blood vessel wall due to hyperplasia or the disappearance of smooth muscle and fibers. [23] e pathological findings of SAM are characterized by vacuolar degeneration of smooth muscle cells in the tunica media, which progresses to dissection of the tunica media from the adventitia, and formation of an aneurysm, resulting in the appearance of medial islands of residual tunica media in the arterial wall.…”

Clinical Presentation, Imaging, and Management of Segmental Arterial Mediolysis: A Rare Vascular Disorder

“…FMD predominantly affects middle-aged women with a predilection for renal, extracranial carotid, and vertebral arteries, whereas SAM has no age or gender predilection and most commonly involves visceral arteries like the mesenteric and celiac arteries. 11 SAM usually has an acute presentation, with abdominal pain or intra-abdominal hemorrhage and derangement of renal function in cases of renal artery involvement, whereas FMD is asymptomatic except in some cases who may present with hypertension. 8 Though most of the literature suggests that FMD and SAM are two separate entities, some researchers believe that both of them are at different ends of the spectrum of the same disease.…”

“…FMD predominantly affects middle-aged women with a predilection for renal, extracranial carotid, and vertebral arteries, whereas SAM has no age or gender predilection and most commonly involves visceral arteries like the mesenteric and celiac arteries. 11 …”