Long-term outcomes of kidney donors with fibromuscular dysplasia.

BACKGROUND: Fibromuscular dysplasia (FMD) is a non-atherosclerotic systemic arterial disease that is not infrequently discovered during kidney donor evaluation. Current guidelines do not provide recommendations regarding the use of kidneys from donors with FMD and there is a paucity of data on the outcomes of these donors. METHODS: The Renal and Lung Living Donor Evaluation (RELIVE) study addressed long-term outcomes of 8922 kidney donors who donated between 1963 and 2007. We compared the development of hypertension, cardiovascular disease (CVD), proteinuria and reduced estimated glomerular filtration rate (eGFR) in 113 kidney donors with FMD discovered during donor evaluation versus 452 propensity score matched donors without FMD. Outcomes modeling with logistic and Cox regression analysis and Kaplan-Meier statistics were performed. RESULTS: Donors with FMD were older (51 versus 39 years), were more likely to be women (80% versus 56%) and had a higher systolic blood pressure at donation (124.7 versus 121.3 mmHg) (P < 0.05 for all). After a mean ± standard deviation follow-up of 15.5 ± 8.9 years, a similar proportion of donors with and without FMD were alive, and developed hypertension (22.2% versus 19.8%), proteinuria (20.6% versus 13.7%) and CVD (13.3% versus 13.5%). No donor with FMD developed an eGFR <30 mL/min/1.73 m2 or end-stage kidney disease. The multivariable risk of mortality, CVD and renal outcomes in donors with FMD was not elevated. CONCLUSIONS: Kidney donors with FMD appear to do well, do not appear to incur increased risks of hypertension, proteinuria, CVD or reduced eGFR, and perhaps carefully selected candidates with FMD can safely donate as long as involvement of other vascular beds is ruled out.

Prevalence of smoking and clinical characteristics in fibromuscular dysplasia. The ARCADIA-POL study.

PURPOSE: Smoking was identified as a potential factor contributing to fibromuscular dysplasia (FMD). To evaluate the prevalence of smoking and clinical characteristics in FMD subjects. MATERIAL AND METHODS: We analysed 190 patients with confirmed FMD in at least one vascular bed. The rate of smokers in FMD patients was compared to that in two control groups selected from a nationwide survey. RESULTS: The rate of smokers in FMD patients was 42.6%. There were no differences in frequency of smokers between FMD patients and: a group of 994 matched control subjects from general population and a group of matched hypertensive subjects. There were no differences in the characteristics of FMD (including rates of multisite FMD and significant renal artery stenosis) and its complications (including rates of dissections and aneurysms) between smokers and non-smokers. Smokers as compared with non-smokers were characterized by higher left ventricle mass index. CONCLUSIONS: There is no difference in the rate of smokers between FMD patients and subjects from the general population. Moreover, we did not find any association between smoking and clinical characteristics of FMD patients nor its extent and vascular complications. Our results do not support the hypothesis that smoking is involved in the pathophysiology of FMD.

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.

Possible familial presentation in two siblings with carotid fibromuscular dysplasia.

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease which can affect the cervical, renal and visceral arteries. Here we report on two sisters diagnosed with carotid FMD at the same age, presenting with similar symptoms of pulsating tinnitus. The familial presentation of this rare disorder attracted our attention and was suggestive of a genetic etiology. Conservative treatment with aspirin was initiated. Carotid FMD is a rare disorder of which the exact pathophysiology is not known. A review of the literature on the clinical presentation, diagnosis and management is presented. In addition possible etiological factors and the genetic nature of the disease are discussed.

Renal artery stenosis due to fibromuscular dysplasia in a transplanted kidney from a deceased donor: a difficult diagnosis at color Doppler ultrasonography.

Atherosclerotic renal artery stenosis is a frequent cause of arterial hypertension and/or allograft dysfunction after kidney transplantation and is usually located at the iliac artery anastomosis. Fibromuscular dysplasia is a less frequent, nonatherosclerotic, vascular disease, inducing stenosis at the proximal/mid-distal part of the renal artery. We report the case of a 44-year-old woman, in whom serum creatinine concentration increased and arterial hypertension developed 3 months after renal transplantation. Color Doppler ultrasonography showed a low arterial resistance index and prolonged acceleration time in the interlobar arteries, and a significantly increased peak systolic velocity at the mid third of the renal artery, demonstrating hemodynamically significant stenosis. Percutaneous transluminal angioplasty allowed stenosis correction and was followed by creatinine concentration and arterial blood pressure normalization.

[Surgical management of patients with pathological deformities of carotid arteries].

Surgical management of pathological deformities of the internal carotid arteries, a cause of chronic brain ischemia, is discussed. This pathology is very common and is found in 25% of all individuals who underwent preventive medical examination according to the ultrasonography data. Most deformities do not pose any threat to patients, while some of them may cause ischemic stroke and chronic brain ischemia. The study included 165 patients with the known follow-up history who had been operated on at the N.N. Burdenko Neurosurgical Institute since 2001. A total of 196 reconstructive interventions of carotid arteries were analyzed. The indications for surgical management of pathological deformities based on clinical symptoms and identification of the signs of vascular wall dysplasia are thoroughly discussed. The local and cerebral hemodynamics during pre- and postoperative period are analyzed. The results of pathomorphological examination of the resected fragments of the deformed arteries are presented; they show that the changes are identical to those in patients with fibromuscular dysplasia. The follow-up history of the patients was recorded; it showed a sustained regression of transitory ischemic strokes and cerebral symptoms in most cases (69%). For proper indications for surgical management, reconstructive surgical interventions are a reliable and effective method for treating chronic brain ischemia and preventing recurrent ischemic strokes in patients with deformities of carotid arteries.

Evaluation of characteristics, associations and clinical course of isolated spontaneous renal artery dissection.

BACKGROUND: Spontaneous renal artery dissection (SRAD) is a rare entity of unknown etiology. We aimed to study the clinical course and outcomes and compare the characteristics of patients with SRAD with those of the general population. METHODS: All cases of isolated renal artery dissection diagnosed at the University of Michigan Hospitals between January 2000 and July 2012 were identified by the ICD-9 code. Cases were matched by age, gender and race with individuals from the 2009-2010 National Health and Nutrition Examination Survey (NHANES). Characteristics and awareness of comorbid conditions were compared. Information about the clinical course after diagnosis was retrieved from the case group to ascertain their outcomes. RESULTS: Overall, 17 patients with SRAD with a mean age of 38.6 years (SD = 8.3) were identified. Eleven patients were male and 14 were white. The most common presenting symptom was excruciating sudden-onset flank pain ipsilateral to the site of dissection. Fibromuscular dysplasia, Ehlers-Danlos and polyarteritis nodosa were present in 4, 4 and 1 patients, respectively. After adjusting in a multivariable model, the case group was more likely to report history of hypertension, cancer and connective tissue disorders (P < 0.001), and less likely to have obesity (BMI ≥30 kg/m(2)) compared with the general population. Supportive medical treatment, endovascular intervention and surgery were required in 8, 5 and 4 cases, respectively. After discharge from the hospital, hypertension was adequately controlled in all the patients but one. CONCLUSION: SRAD may be part of a syndrome having multi-organ involvement. With appropriate medical or surgical management, long-term clinical outcome appears favorable.

Large volume haemorrhage following transoral robotic surgery (TORS) as a result of fibromuscular dysplasia: first reported case.

We present a case of a 61-year-old woman who suffered a haemorrhagic complication of an aneurysmal left lingual artery, secondary to fibromuscular dysplasia, following transoral robotic surgery (TORS). She was admitted through the emergency department 3 days after resection of a central base of tongue tumour. She suffered a massive haemorrhage requiring intensive care admission, blood transfusion, intubation, operative and endovascular intervention. The diagnosis of fibromuscular dysplasia was made at angiography. During attempts to embolise the culprit left lingual artery, the vessel dissected at its origin leading to arrest of bleeding. Repeat angiograms during her admission demonstrated unchanged appearances and ruled out recanalisation of the vessel. She was extubated and remained stable on the ward, discharged home some days later in good health. To our knowledge, this is the first ever reported case of fibromuscular dysplasia affecting the lingual artery leading to aneurysmal dilation and severe haemorrhage following TORS.

First international consensus on the diagnosis and management of fibromuscular dysplasia.

This article is a comprehensive document on the diagnosis and management of fibromuscular dysplasia (FMD) which was commissioned by the Working Group 'Hypertension and the Kidney' of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM). This document updates previous consensus documents/scientific statements on FMD published in 2014 with full harmonization of the position of European and US experts. In addition to practical consensus-based clinical recommendations, including a consensus protocol for catheter-based angiography and percutaneous angioplasty for renal FMD, the document also includes the first analysis of the European/International FMD Registry and provides updated data from the US Registry for FMD. Finally, it provides insights on ongoing research programs and proposes future research directions for understanding this multifaceted arterial disease.

Colonic Adventitial Fibromuscular Dysplasia: A Nonspecific Arteriopathy Associated With Hirschsprung Disease and Other Obstructive Disorders.

Background Smooth muscle differentiation ("adventitial fibromuscular dysplasia," AFD) was purported as specific to arteries in the transition zone of Hirschsprung disease (HSCR) patients. We investigated AFD in an HSCR population and controls and consider the pathogenesis and significance of the vascular pathology. Design Vascular histology in sections from colonic HSCR resections (n = 55) was compared with age- and site-matched controls with (n = 19) and without (n = 28) non-HSCR obstructive conditions. Vascular pathology was mapped, and correlations were sought between the vascular findings and bowel distension, inflammation, neuromuscular anatomy, preoperative clinical variables, and postsurgical complications. Results One of 2 forms of AFD was identified in 42% (23/55) of the HSCR resections: the previously described "mature" form with adventitial bundles of differentiated smooth muscle cells (7/23, all submucosal) and a newly described "immature" AFD characterized by densely packed myofibroblasts in the arterial adventitia (16/23, 3 submucosal, 3 serosal, and 10 both). Adventitial inflammation and/or medial necrosis was present in the immature form (6/16). Mature submucosal AFD was present in 2/28 (7.1%) nonobstructive and 5/19 (26%) obstructive non-HSCR controls ( P = .10). Immature AFD was only found in less than 1-month-olds, and mature AFD only in older patients, including the 7 affected controls. AFD did not correlate with sex, syndromic status, length of the aganglionic segment, or postoperative complications. AFD was present in grossly dilated (17/23) and narrowed (10/23) regions and in the aganglionic (2/23), ganglionic (14/23), or both (7/23) segments. In several cases, AFD existed proximal to the histological transition zone. Conclusion AFD occurs in HSCR and other obstructive conditions but is significantly less common in the colons of patients with no history of dysmotility. The pathology likely progresses from a reversible accumulation of myofibroblasts in neonates to a stable population of mature smooth muscle cells. The distribution of vascular lesions does not correlate with neuropathological findings and suggests a nonspecific form of vascular injury, possibly related to bowel distension. AFD in HSCR resections has not been shown to be clinically significant and should not influence management.

Fibromuscular dysplasia in association with intrauterine cocaine exposure.

BACKGROUND: Fibromuscular dysplasia (FMD) is an idiopathic disease of small- and medium-sized arteries, involving one or more vascular beds. Patients may present with a range of symptoms, which may not readily lead to a diagnosis of FMD. While maternal cocaine abuse during pregnancy has previously been associated with vascular alterations in the fetus, an association specifically with FMD has not previously been described. METHODS/RESULTS: In this case report, a 21-month-old male presented with a 3-week history of daily vomiting, with temporary improvement of symptoms, then relapse followed by loss of consciousness. His medical history was significant only for maternal cocaine use. Clinical evaluation revealed dilated cardiomyopathy, and a presumptive diagnosis of myocarditis was rendered. Respiratory arrest and death occurred 2 days after admission. Postmortem examination demonstrated intimal-type multivessel FMD, which was determined to be the cause of the clinical presentation. CONCLUSION: Without a postmortem examination, it is unlikely that a diagnosis of intimal fibroplasia, a rare variant of FMD (5% of cases), would have been made. This case thus illustrates the continuing utility of the classic postmortem examination. More intriguingly, the case suggests a possible relationship between in utero cocaine exposure and the development of fibromuscular dysplasia in the child.

Coronary Tortuosity: A Clue to the Diagnosis of Fibromuscular Dysplasia?

BACKGROUND: Fibromuscular dysplasia (FMD) is a systemic, nonatherosclerotic, noninflammatory vasculopathy that is often overlooked by clinicians. Clinical clues could help in selecting patients for further evaluation for the presence of FMD. Recently, it was observed that tortuosity of the coronary arteries is often present in patients with FMD-related abnormalities of the coronary artery. Therefore, we wondered if the presence of coronary tortuosity might provide a clinical clue to the diagnosis of extracoronary FMD. CASES: We describe 5 cases of FMD in whom diagnostic studies for FMD were initiated because of the presence of coronary tortuosity. FMD was found in all 5 patients in the renal and/or cervical arteries. CONCLUSIONS: Our 5 cases suggest that exertional chest pain in the presence of coronary tortuosity may be helpful in selecting patients for further evaluation for the presence of FMD. Further research should focus on the prevalence of FMD among patients with coronary tortuosity and whether the presence of additional clinical clues (such as the presence of hypertension at young age or pulsatile tinnitus) next to coronary tortuosity can predict the risk for FMD in individual patients.

Fibromuscular dysplasia recurrence after kidney transplantation: case report.

We report the extremely rare recurrence of intimal fibroplasia, a rare form of fibromuscular dysplasia, in a kidney recipient at 6 months after transplantation from a living-related donor. The patient was successfully treated and maintains good kidney function, however, the case raises the question of whether kidneys with fibromuscular dysplasia should be included in the expanded criteria for kidney transplantation.

The correlation between the tumor necrosis and renal artery changes in renal cell carcinoma.

Necrosis, cysts, hemorrhage, and calcification represent common findings in renal cell carcinoma. Different lesions, including arteriosclerosis or fibromuscular dysplasia, or both, may involve the main renal artery. This study analyzed the relationship between the presence and extent of necrosis in renal cell carcinoma with renal artery changes in a consecutive series of 112 patients (71 men, 41 women) with mean renal cell carcinoma of 7.7 cm (range, 2 to 20 cm). Necrosis was seen macroscopically and confirmed microscopically in 88 cases (78.6%), with 64 tumors having less than 50% and 24 more than 50% necrosis. Fibromuscular dysplasia was found in 41 patients (36.6%; 17 men, 24 women) and atherosclerotic changes in 21 patients (18.8%; 18 men, 3 women). The results suggest that necrosis of renal cell carcinoma was significantly more common in women with associated fibromuscular dysplasia (especially type I) and men with atherosclerotic changes of renal artery.

[Arterial fibromuscular dysplasia].

The paper reviews the data available in the literature on the etiology, pathogenesis, pathomorphology and examinations of patients with fibromuscular dysplasia. The types of fibromuscular dysplasia are discussed in detail in relation to the damage to vascular wall layers. These include intimal fibroplasia, medial hyperplasia, medial fibroplasias, and perimedial fibroplasia with a well-defined definition pathomorphological and angiographic criteria. The role of various etiological factors in the development of fibromuscular dysplasia is given, by presenting the genetical, mechanical, ishemic, humoral, and hormonal hypothesis of pathogenesis; differential diagnostic features are determined. Emphasis is laid on the necessity and topicality of studying the etiology, pathogenesis, and pathomorphology of dysplasia to gain a better insight into this disease, to choose an effective treatment.

Fibromuscular dysplasia: Advances in understanding and management.

Several key findings in recent years have reshaped our understanding of fibromuscular dysplasia (FMD), an uncommon nonatherosclerotic disease of medium-sized arteries that affects mainly women. While the true prevalence of this disease remains unknown, studies suggest that more people may be affected than previously reported. Better understanding of the clinical manifestations and natural history of FMD and advances in diagnostic imaging have altered the clinical approach to managing patients with this uncommon vascular disease. Although there are a multitude of unanswered questions regarding FMD, this review highlights recent insights and how this information has modified clinical care for those affected.

Complementary roles of color-flow duplex imaging and intravascular ultrasound in the diagnosis of renal artery fibromuscular dysplasia: should renal arteriography serve as the "gold standard"?

OBJECTIVES: The purpose of this study was to compare color-flow duplex imaging (CFDI), intravascular ultrasound (IVUS), and renal arteriography in diagnosing renal artery (RA) fibromuscular dysplasia (FMD) and correlating with the hemodynamic response to balloon angioplasty (BA) in patients with drug-resistant hypertension. BACKGROUND: Renal arteriography is generally regarded as the gold standard for diagnosing RA FMD. The observation that CFDI and IVUS depicted endoluminal abnormalities suggestive of RA FMD in some patients with normal renal arteriograms prompted comparison of these modalities in a consecutive series of patients. METHODS: Twenty hypertensive patients with CFDI suggestive of RA FMD (mid-to-distal flow derangement and velocity augmentation) underwent renal arteriography, IVUS, and BA, with both immediate and long-term blood pressure (BP) response assessment. RESULTS: All patients were women, aged 31 to 86 years (mean 62 years). On IVUS, various endoluminal defects (eccentric ridges; fluttering membranes; spiraling folds) were depicted at locations predicted by CFDI and were uniformly identified at sites where arteriography depicted classic evidence of FMD (8 patients). However, similar defects were detected by IVUS when angiography was borderline (7 patients) or normal (5 patients). Balloon angioplasty eliminated (16 patients) or reduced (4 patients) the IVUS findings and lowered systolic BP in all (mean reduction 53 mm Hg, p < 0.0001). This reduction was maintained during follow-up of 4 to 22 (mean 13) months (mean reduction 44 mm Hg, p < 0.0001), independent of baseline angiographic appearance. CONCLUSIONS: Both CFDI and IVUS depict the blood flow and endoluminal abnormalities of RA FMD. Balloon angioplasty eliminates or improves IVUS findings and produces substantial, sustained BP reduction, an effect that is independent of baseline arteriographic appearance, calling into question the legitimacy of arteriography as the diagnostic gold standard.

Carotid artery stenting for the high surgical risk patients.

Obstructive atherosclerotic carotid bifurcation disease is responsible for approximately 30% of stroke cases in the United States. Any successful treatment of this disease should demonstrate significant and durable reduction in the related risk for stroke without compromising the patient safety. The indications for CAS are evolving as the techniques, the stents and catheter technology advance. There is an ongoing need to improve the equipment suitable for carotid stenting and a need to enhance the safety of the procedure. Often, when a technical problem is encountered, it is attributed to the inadequacy of the devices currently available. As the technology improves, particularly with the application of the anti-embolization devices, the indications and contraindication may need to be revised.