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

World J Gastroenterol. 2018 Aug 28;24(32):3637-3649. doi: 10.3748/wjg.v24.i32.3637.

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.

Keywords: Fibromuscular dysplasia; Humans; Mesenteric lesion; Segmental arterial mediolysis; diagnosis.

Publication types

  • Review

MeSH terms

  • Abdominal Pain / diagnostic imaging
  • Abdominal Pain / etiology
  • Abdominal Pain / therapy
  • Aneurysm / diagnostic imaging
  • Aneurysm / etiology
  • Aneurysm / therapy
  • Angiography / methods
  • Arteries / pathology*
  • Cardiovascular Agents / therapeutic use
  • Diagnosis, Differential
  • Endovascular Procedures
  • Fibromuscular Dysplasia / complications
  • Fibromuscular Dysplasia / diagnostic imaging*
  • Fibromuscular Dysplasia / pathology
  • Fibromuscular Dysplasia / therapy
  • Gastroenterology / methods*
  • Gastrointestinal Hemorrhage / diagnostic imaging
  • Gastrointestinal Hemorrhage / etiology
  • Gastrointestinal Hemorrhage / therapy
  • Gastrointestinal Tract / blood supply
  • Gastrointestinal Tract / diagnostic imaging
  • Gastrointestinal Tract / pathology
  • Humans
  • Prognosis
  • Treatment Outcome
  • Tunica Media / diagnostic imaging
  • Tunica Media / pathology*

Substances

  • Cardiovascular Agents