Aims Since December 2015, the European/International Fibromuscular Dysplasia (FMD) Registry enrolled 1022 patients from 22 countries. We present their characteristics according to disease subtype, age and gender, as well as predictors of widespread disease, aneurysms and dissections. Methods and results All patients diagnosed with FMD (string-of-beads or focal stenosis in at least one vascular bed) based on computed tomography angiography, magnetic resonance angiography, and/or catheter-based angiography were eligible. Patients were predominantly women (82%) and Caucasians (88%). Age at diagnosis was 46 ± 16 years (12% ≥65 years old), 86% were hypertensive, 72% had multifocal, and 57% multivessel FMD. Compared to patients with multifocal FMD, patients with focal FMD were younger, more often men, had less often multivessel FMD but more revascularizations. Compared to women with FMD, men were younger, had more often focal FMD and arterial dissections. Compared to younger patients with FMD, patients ≥65 years old had more often multifocal FMD, lower estimated glomerular filtration rate and more atherosclerotic lesions. Independent predictors of multivessel FMD were age at FMD diagnosis, stroke, multifocal subtype, presence of aneurysm or dissection, and family history of FMD. Predictors of aneurysms were multivessel and multifocal FMD. Predictors of dissections were age at FMD diagnosis, male gender, stroke, and multivessel FMD. Conclusions The European/International FMD Registry allowed large-scale characterization of distinct profiles of patients with FMD and, more importantly, identification of a unique set of independent predictors of widespread disease, aneurysms and dissections, paving the way for targeted screening, management, and follow-up of FMD.

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Severe intra-operative and post-operative bleeding is a potentially life-threatening complication of gynaecologic surgery. A sound clinical judgement and the adequate assessment and preparation of the patient are the best pre-operative means to avoid its occurrence. Intraoperative prevention requires knowledge of surgical anatomy and haemostatic techniques. The management of haemorrhagic complications can be extremely challenging. Its success depends on the perfect integration of surgical expertise, the supporting role of the anaesthesiologist and on the availability of a fully equipped interventional radiology team with much experience. A methodical and stepwise surgical approach is needed to selectively dissect and identify the bleeding site without damaging adjacent structures. In case of more diffuse or massive bleeding, the performance of a bilateral ligation of the anterior branch of the internal iliac arteries at an early stage is an appropriate measure. The anaesthesiologist is responsible for the maintenance of the patient's haemodynamic function and the prevention of coagulopathy. Compression or atraumatic clamping of the aorta or placement of a pelvic packing can be temporarily applied to stabilize the patient's condition. Only once this is achieved, can the subsequent option of angiographic arterial embolisation be considered.

Arterial aneurysms and pseudo-aneurysms are a rare but recognized cause of obstetric hemorrhage. Diagnosis during pregnancy, prior to rupture, is exceptional. We report the first case of diagnosis and treatment of an uterine artery pseudo-aneurysm during pregnancy.

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.

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