Diagnosis and management of fibromuscular dysplasia and segmental arterial mediolysis in gastroenterology field: A mini-review

Abstract

The vascular diseases including aneurysm, occlusion, and thromboses in the mesenteric lesions could cause severe symptoms and appropriate diagnosis and treatment are essential for managing patients. With the development and improvement of imaging modalities, diagnostic frequency of these vascular diseases in abdominal lesions is increasing even with the small changes in the vasculatures. Among various vascular diseases, fibromuscular dysplasia (FMD) and segmental arterial mediolysis (SAM) are noninflammatory, nonatherosclerotic arterial diseases which need to be diagnosed urgently because these diseases could affect various organs and be lethal if the appropriate management is not provided. However, because FMD and SAM are rare, the cause, prevalence, clinical characteristics including the symptoms, findings in the imaging studies, pathological findings, management, and prognoses have not been systematically summarized. Therefore, there have been neither standard diagnostic criteria nor therapeutic methodologies established, to date. To systematically summarize the information and to compare these disease entities, we have summarized the characteristics of FMD and SAM in the gastroenterological regions by reviewing the cases reported thus far. The information summarized will be helpful for physicians treating these patients in an emergency care unit and for the differential diagnosis of other diseases showing severe abdominal pain.

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

“…FMD and SAM are noninflammatory, nonatherosclerotic arterial diseases originally diagnosed based on histological findings; however, with marked advances in imaging modalities, including CT and magnetic resonance imaging, opportunities to diagnose these diseases based on imaging findings and clinical information without a pathological examination are increasing (3).…”

“…It is therefore possible to diagnose these diseases clinically and suggest appropriate therapeutic options (Table 1, 2). For example, FMD affects middle-aged women, whereas there is no predilection for age or gender for SAM (3,5,13,14). In addition, while FMD often shows stenosis and aneurysms in medium-sized arteries, including the renal, extracranial, carotid, and vertebral arteries (12), SAM shows changes in larger arteries, such as the celiac and mesenteric arteries (13), leading to a higher risk of arterial rupture and hemorrhaging from the weakened arterial wall in these larger arteries (15).…”

“…As these diseases can affect various organs and be lethal if not properly managed, an appropriate diagnosis followed by treatment in the early phase is essential (1,2). However, as the disease entities are rare, we have summarized the characteristics of FMD and SAM in the gastroenterologic regions and reviewed reported cases with a comparison of the imaging findings and histological analyses (3).…”

Segmental Arterial Mediolysis with Preceding Symptoms Resembling Viral Infection Hampers the Differentiation from Polyarteritis Nodosa

“…Treatment includes embolization, bypass, and resection of injured arteries. In addition, anti-hypertensive therapy can prevent further worsening of arterial lesions (7).…”

“…Aside from angiographic findings, the presence of orchitis, which is a characteristic feature of PN, made disease interpretation even more difficult. The final diagnosis was based on the pathological findings of the arterial lesions of the surgical specimens (7).…”