Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021.

Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that may involve medium-sized muscular arteries throughout the body. The pathogenesis of FMD remains poorly understood, but a combination of genetic and environmental factors may be involved. The majority of FMD patients are women, but men may have a more progressive disease, especially when smoking. Besides the classical phenotype of string of beads or focal stenosis, arterial aneurysms, dissections, and tortuosity are frequent manifestations of the disease. However, the differential diagnosis of FMD is extensive and includes imaging artefacts as well as other arterial diseases. Diagnosis is based on CT-, MR-, or conventional catheter-based angiography during work-up of clinical manifestations, but clinically silent lesions may be found incidentally. Arterial hypertension and neurological symptoms are the most frequent clinical presentations, as renal and cerebrovascular arteries are the most commonly involved. However, involvement of most arteries throughout the body has been reported, resulting in a variety of clinical symptoms. The management of FMD depends on the vascular phenotype as well on the clinical picture. Ongoing FMD-related research will elaborate in depth the current progress in improved understandings of the disease's clinical manifestations, epidemiology, natural history and pathogenesis. This review is focused on the clinical management of adult FMD in daily practice.

Visceral Fibromuscular Dysplasia: From asymptomatic disorder to emergency.

Fibromuscular dysplasia (FMD) is an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease of the musculature of arterial walls, leading to stenosis of small and medium-sized arteries, mostly involving renal and cervical arteries. As a result of better and more systematic screening, it appears that involvement of the splanchnic vascular bed is more frequent than originally assumed. We review epidemiology, pathogenesis, clinical picture as well as diagnosis and treatment of visceral artery (VA) FMD. The clinical picture is very diverse, and diagnosis is based on CT-, MR- or conventional catheter-based angiography. Involvement of VAs generally occurs among patients with multi-vessel FMD. Therefore, screening for VA FMD is advised especially in renal artery (RA) FMD and in case of aneurysms and/or dissections. Treatment depends on the clinical picture. However, the level of evidence is low, and much of the common practice is extrapolated from visceral atherosclerotic disease.

Endovascular techniques in the management of acute arterioenteric fistulas.

PURPOSE: To report the use of endovascular techniques to emergently treat hemorrhagic complications of rare arterioenteric fistulas. CASE REPORTS: Two patients, a 71-year-old man and a 61-year-old woman, presented with acute bleeding arising from primary arterioenteric fistulas. In the first patient, a fistula between the iliac artery and the small intestine complicated laparoscopic treatment of acute appendicitis. In the second patient, irradiation of a metastatic cervical carcinoma led to a fistula between the right iliac artery and the terminal ileum. In both patients, the hemorrhage was controlled with implantation of a Jostent Peripheral Stent-Graft. The man is alive at 3 years with a patent endograft, but the woman died 1 month after treatment from complications of tumor progression. CONCLUSIONS: Endovascular application of covered stents provides an alternative treatment, avoiding extensive surgery. In cases of neoplastic erosion of a large vessel, endovascular stenting can offer a palliative solution.