Diagnosis Of Fibromuscular Dysplasia Research Articles

Endovascular Treatment of Renal Artery Stenosis Caused by Fibromuscular Dysplasia. A Clinical Case Report

Introduction. Fibromuscular dysplasia (FMD) is an idiopathic, non-atherosclerotic, non-inflammatory disease of arteries. Careful research into this disorder showed that FMD has been found in every arterial bed in the body; the most common arteries affected are renal arteries and extracranial sections of carotid and vertebral arteries. The clinical presentation is determined by the localization of the vasculature affected and the stenosis severity. Today FMD is a very rare disease with the incidence of 4 per 1000 people. The diagnosis today is difficult and may take a long time. According to the latest European Society of Cardiology guidelines renal artery balloon angioplasty is indicated for patients with FMD; if a good angiographic result is achieved (no dissection, TIMI 3 flow) no renal artery stenting required. The treatment success depends on the early diagnosis.Materials and Methods. This paper presents a clinical case of renal artery stenosis caused by fibromuscular dysplasia that was treated successfully with balloon angioplasty without stenting.Results and discussion. Protracted process of diagnosing this disease may result in deteriorating quality of life and poor outcomes such as difficult-to-control hypertension and its sequelae, TIA, stroke, aneurism dissection or rupture. It is worth pointing out that FMD diagnosis may be incidental when imaging is performed for other reasons, or when there is a systolic murmur at arteries in an asymptomatic patient who does not have classic atherosclerosis risk factors. According to the latest guidelines endovascular treatment is indicated for patients with FMD to manage the hypertension; this has proven very effective in improving quality of life.

Prevalence and characteristics of renal artery fibromuscular dysplasia in hypertensive women below 50 years old

Renal fibromuscular dysplasia (FMD) is typically diagnosed in young hypertensive women. The 2014 European FMD Consensus recommended screening in all hypertensive women <30 yo. However, the prevalence of renal FMD in young/middle-aged hypertensive women remains unclear. The aim of this work was to assess the prevalence and characteristics of renal FMD in hypertensive women ≤50 yo. We retrospectively included all consecutive women aged ≤50years referred to our Hypertension Unit from 2014 to 2017 and collected standardized information on patient characteristics and screening modalities. Of 1083 incident hypertensive patients, 157 patients fitted with inclusion criteria. The prevalence of renal FMD varied between 3.2% in the whole sample and 7.5% in patients explored by CTA and/or MRA (n=67). In the subgroup of patients ≤30 yo (n=32), the corresponding figures were 3.1% and 5.6%. The yearly prevalence of FMD tended to increase over time, in parallel with increased use of CTA/MRA as a first-line imaging modality. Out of 5 patients with renal FMD, 2 were revascularized and 1 had extra-renal FMD. The prevalence of renal FMD in young/middle-aged hypertensive women is probably one order of magnitude higher than previously assumed, in the range of 3%-8%, depending on imaging modalities. While the diagnosis of FMD does not influence short-term management in all patients, it may allow close monitoring and prevention of complications of the disease over time. This analysis provides the rationale for a prospective, multicentre study aiming at determining the cost-effectiveness of systematic screening for renal FMD.

COGNITIVE FUNCTION AND DEPRESSION IN PATIENTS WITH FIBROMUSCULAR DYSPLASIA

Although patients with fibromuscular dysplasia (FMD) commonly report symptoms of memory loss, the association of FMD with cognitive function and depression is not known. At a single center, participants with a diagnosis of extracranial or intracranial FMD without a history of stroke were enrolled.

Natural History of Cervical Artery Fibromuscular Dysplasia and Associated Neurovascular Events

Background and Purpose: Fibromuscular dysplasia (FMD) is a non-atherosclerotic arteriopathy most often affecting the carotid and renal arteries. In the United States Registry for FMD, 41.7% of patients experienced an aneurysm and/or dissection by the time of entry into the Registry. We sought to determine the occurrence of neurovascular events after FMD diagnosis and any changes on cervical artery imaging that may be attributable to FMD. Methods: Patients followed at the Mount Sinai Medical Center (US Registry for FMD enrollment center) with confirmed FMD and > 1 cervical artery imaging study (at least ± 6 months from the baseline carotid duplex ultrasound [CDU]) between the years 2003 and 2015 were included. Medical records and cervical artery imaging ([CDU], magnetic resonance angiogram [MRA], and computed tomography angiogram [CTA]) were reviewed. New arterial dissection, aneurysm, transient ischemic attack, stroke, or new FMD findings were recorded. Results: Among 146 FMD patients with complete information, 52 (35.6%) had an aneurysm and 52 (35.6%) had a dissection. Mean clinical follow-up was 35.3 ± 25.3 months (range 5–153 months); patients underwent 4 ± 2.7 CDU (range 1–17); 86.3% had ≥1 neck MRA or CTA. After FMD diagnosis, 3 patients (2%) experienced a new carotid artery dissection; 1 patient experienced a stroke due to concomitant atherosclerosis. No new aneurysms occurred. In patients with cervical artery FMD, imaging findings remained stable throughout follow-up. No patient developed new cervical artery FMD findings on follow-up imaging. Conclusions: No new cervical artery FMD or aneurysm was observed on subsequent imaging. New carotid dissection was uncommon over a mean follow-up period of 35.3 ± 25.3 months and was the only non-atherosclerotic vascular event observed after FMD diagnosis.

Abstract 121: Anxiety and Depression in Patients With Fibromuscular Dysplasia: A Report From the US Registry for Fibromuscular Dysplasia

Introduction/Background: Fibromuscular dysplasia (FMD) is a non-inflammatory, non-atherosclerotic arterial disease that may result in arterial stenosis, dissection, aneurysm, and tortuosity. Anxiety (A) and depression (D) are prevalent in patients with rare and uncommon diseases compared to the general population; however, there are limited data on A and D in the FMD population. This study describes characteristics of FMD patients with and without A and/or D (A/D). Methods: Patients enrolled in the US Registry for FMD between 2009-2017 and had A/D information were analyzed (n=985). Diagnosis of A/D was ascertained from patient charts or self-reported via medical history questionnaires at time of registry enrollment. Demographics, family history, past medical history, presenting symptoms, and affected vascular beds were compared between patients with and without A/D. Results: A total of 351 (35.6%) had A/D. Of patients with A/D, 126 (35.9%) had both. Patients with A/D were more likely to have a family history of aneurysm (33.6% v. 23.7%, p=0.001) and stroke (53.6% v. 46.1%, p=0.03), and have a medical history of hemispheric TIA (15.5% v. 10.9%, p=0.04), subarachnoid hemorrhage (4.4% v. 1.8%, p=0.015) and smoking (43% v. 28%, p&lt;0.001). Patients with A/D were more likely to present at the time of diagnosis with headache (68.6% v. 53.8%, p&lt;0.001), pulsatile tinnitus (42.2% v. 34%, p=0.014), dizziness (45.3% v. 29.2%, p&lt;0.001), neck pain (40% v. 24.9%, p&lt;0.001) and carotid/vertebral arterial dissection (21.6% v. 14.7%, p=0.007). Patients with A/D were less likely to have renal artery FMD (58.4% v. 68.9%, p=0.002). No differences in the time between symptom onset and FMD diagnosis (2.7±6.2 v. 3.1±6.9 years, p=0.89) or number of vascular beds involved (1.9±1.1 v. 1.9±1.1, p=0.77) were seen between groups. Conclusions: More than 1/3 of patients in the FMD registry had A, D, or both, and these patients showed multiple differences compared to patients without A/D. More study is required to understand this relationship. Given the high prevalence of A/D in this population, FMD patients should be screened for the presence of A/D and offered appropriate treatment.

A Challenging Case of Fibromuscular Dysplasia in a Transgender Patient: Is There a Hormonal Link?

IntroductionFibromuscular dysplasia (FMD) and superior mesenteric artery (SMA) aneurysms are rare vascular conditions. An unusual combination of both diseases is reported.Case reportA 54 year old woman presented with symptomatic SMA aneurysm. A diagnosis of FMD was made on the basis of computed tomography angiography (CTA). The patient had undergone gender reassignment surgery 10 years previously and continued to use both topical and oral hormonal therapy. The patient received open anatomical bypass through a retroperitoneal approach using great saphenous vein.ConclusionSuperior mesenteric artery aneurysms are rare and a diagnosis of FMD should be considered as part of the diagnosis process. Anatomical bypass should be considered carefully in relation to a patient's fitness as well as anatomical suitability.

Prevalence of Intracranial Aneurysm in Women With Fibromuscular Dysplasia

The prevalence of intracranial aneurysm in patients with fibromuscular dysplasia (FMD) is uncertain. To examine the prevalence of intracranial aneurysm in women diagnosed with FMD. This cross-sectional study included 669 women with intracranial imaging registered in the US Registry for Fibromuscular Dysplasia, an observational disease-based registry of patients with FMD confirmed by vascular imaging and currently enrolling at 14 participating US academic centers. Registry enrollment began in 2008, and data were abstracted in September 2015. Patients younger than 18 years at the time of FMD diagnosis were excluded. Imaging reports of all patients with reported internal carotid, vertebral, or suspected intracranial artery aneurysms were reviewed. Only saccular or broad-based aneurysms 2 mm or larger in greatest dimension were included. Extradural aneurysms in the internal carotid artery were included; fusiform aneurysms, infundibulae, and vascular segments with uncertainty were excluded. Percentage of women with FMD with intracranial imaging who had an intracranial aneurysm. Of 1112 female patients in the registry, 669 (60.2%) had undergone intracranial imaging at the time of enrollment (mean [SD] age at enrollment, 55.6 [10.9] years). Of the 669 patients included in the analysis, 86 (12.9%; 95% CI, 10.3%-15.9%) had at least 1 intracranial aneurysm. Of these 86 patients, 25 (53.8%) had more than 1 intracranial aneurysm. Intracranial aneurysms 5 mm or larger occurred in 32 of 74 patients (43.2%), and 24 of 128 intracranial aneurysms (18.8%) were in the posterior communicating or posterior arteries. The presence of intracranial aneurysm did not vary with location of extracranial FMD involvement. A history of smoking was significantly associated with intracranial aneurysm: 42 of 78 patients with intracranial aneurysm (53.8%) had a smoking history vs 163 of 564 patients without intracranial aneurysm (28.9%; P < .001). The prevalence of intracranial aneurysm in women diagnosed with FMD is significantly higher than reported in the general population. Although the clinical benefit of screening for intracranial aneurysm in patients with FMD has yet to be proven, these data lend support to the recommendation that all patients with FMD undergo intracranial imaging if not already performed.

Clinical analysis of renal artery branch lesions caused by fibromuscular dysplasia in children

Objective To explore the lesion types of renal branches and evaluate the clinical results of endovascular treatment for renal artery branch lesions associated with fibromuscular dysplasia (FMD). Methods Eight cases with renal artery branch lesions due to FMD from June 2014 to December 2015 in Department of Vascular and Endovascular Surgery, Henan Provincial People′s Hospital were retrospectively reviewed.The computed tomography angiography was routinely performed to ascertain the property of renal artery lesion, and digital-subtraction angiography was further performed to confirm FMD diagnosis and analyze lesion characteristics.The renal artery branch lesion types, blood pressure (BP), renin activity, glomerular filtration rate (GFR) and restenosis after operation were observed, and the outcomes were recorded. Results In 8 patients, 6 patients were found with the second grade renal branch lesions and 2 patients were found with tertiary branch lesions, involving 2 and more branch arteries for 4 cases and 1 branch for 4 cases.Five cases with branch-type cystic dilatation or aneurysms with coexisting stenosis including 1 case with dissecting aneurysm and renal local infarction, and 1 case with aneurysm located in the bifurcations of branches, and 3 cases with only branch stenosis were found.The angiography showed the small branch lesions of renal artery indicating FMD-mediated focal (5 cases) and multiple stenosis (3 cases). The branch stenosis was not operated in 1 case with dissecting aneurysm and renal local infarction, as BP could be controlled by antihypertensive treatment.Other 7 cases underwent endovascular angioplasties and the technical successful rate was 100% without complications.After 1 week of operation, BP was improved, renal function remained normal, plasma renin activity was reduced and GFR was recovered for all patients.During the follow-up period (3 to 12 months), BP improvement was observed in 3 cases, and cure were found in 4 cases, and patient′s GFR and plasma renin activity were improved in all patients.There were 2 cases with re-intervention during the follow-up period due to restenosis, and the involved branches were unobstructed without obvious stenosis after 1-year follow-up. Conclusions FMD can affect the renal branch artery with different lesions types and endovascular treatment is the first choice for the renal branch lesion of FMD. Key words: Fibromuscular dysplasia; Renal artery stenosis; Renal artery aneurysm; Endovascular treatment

Spontaneous Coronary Artery Dissection Associated With Pregnancy

BackgroundSpontaneous coronary artery dissection (SCAD) is the most common cause of pregnancy-associated myocardial infarction and remains poorly characterized. ObjectivesThis study sought to assess presentation, clinical factors, and outcomes of pregnancy-associated spontaneous coronary artery dissection (P-SCAD) compared with spontaneous coronary artery dissection not associated with pregnancy (NP-SCAD). MethodsA Mayo Clinic registry was established in 2010 to include comprehensive retrospective and prospective SCAD data. Records were reviewed to identify women who were pregnant or ≤12 weeks postpartum at time of SCAD. Complete records were available for 323 women; 54 women met criteria for P-SCAD (4 during pregnancy) and they were compared with 269 women with NP-SCAD. ResultsMost events occurred within the first month postpartum (35 of 50). Compared with NP-SCAD, P-SCAD patients more frequently presented with ST-segment elevation myocardial infarction (57% vs. 36%; p = 0.009), left main or multivessel SCAD (24% vs. 5%; p < 0.0001; and 33% vs. 14%; p = 0.0027, respectively), and left ventricular function ≤35% (26% vs. 10%; p = 0.0071). Among women with imaging of other vascular territories, P-SCAD was less likely with a diagnosis of fibromuscular dysplasia and extracoronary vascular abnormalities (42% vs. 64%; p = 0.047; and 46% vs. 77%; p = 0.0032, respectively). Compared with U.S. birth data, women with P-SCAD were more often multiparous (p = 0.0167), had a history of infertility therapies (p = 0.0004), and had pre-eclampsia (p = 0.001). On long-term follow-up (median 2.3 years) recurrent SCAD occurred in 51 patients, with no difference in the Kaplan Meier 5-year recurrence rates (10% vs. 23%; p = 0.18). ConclusionsP-SCAD patients had more acute presentations and high-risk features than women with NP-SCAD did. The highest frequency of P-SCAD occurred during the first postpartum month and P-SCAD patients less often had extracoronary vascular abnormalities. Hormonal, hemodynamic variations, and yet-undefined mechanisms might be significant contributors to P-SCAD. (The “Virtual” Multicenter Spontaneous Coronary Artery Dissection [SCAD] Registry [SCAD]; NCT01429727; Genetic Investigations in Spontaneous Coronary Artery Dissection [SCAD]; NCT01427179)

Abstract 099: Prevalence of Headaches in Patients with Fibromuscular Dysplasia: A Report from the US Registry for FMD

Introduction: Fibromuscular dysplasia (FMD) is a polyvasculopathy that may result in aneurysm or dissection in affected arterial beds. A common symptom of FMD is headache (HA). The purpose of this study is to describe and compare characteristics of FMD patients with (w/HA) and without headaches (wo/HA) in the current US Registry for FMD. Method: Data regarding demographics, family history, medical history, and vascular beds affected in patients with FMD were collected from the US Registry for FMD between 2009 and 2016. Patients with complete HA data were included, and data were compared between patients w/HA and wo/HA as reported at time of registry enrollment. Results: Of the 1,433 patients in the registry, 1,251 (87.3%) had complete HA data. Most were female (94.1%), with a mean age of 56.0±12.4. A history of HA was reported in 844 (67.5%) patients and 54.2% reported a HA at least weekly. No difference in gender distribution was seen between HA groups. Patients w/HA were younger at the time of FMD diagnosis (50.6±11.8 years v 58.0±13.8 years, p&lt;0.001) and were less likely to have a history of HTN (66.8% v 74.8%, p=0.004), hyperlipidemia (36.9% v 47.2%, p=0.004), or renal failure (2.6% v 5.7%, p=0.009). Patients w/HA were more likely to have a history of aneurysm or dissection in the extracranial carotid, intracranial, or vertebral arteries (aneurysm: 13.0% v 7.2% p=0.003; dissection: 25.1% v 8.3%, p&lt;0.001). No differences in extracranial carotid, intracranial, or vertebral FMD involvement between patients w/ and w/o HA were seen when those with history of aneurysm or dissection of those arteries were excluded (71.2% v 66.8%, p=0.20). Headache patients more often reported a history of depression (25.1% v 16.5%, p=0.01) and anxiety (28.3% v 18.0%, p=0.003). Conclusion: More than half of FMD patients experience HAs, most on an at least weekly basis. FMD patients with HA tended to be younger, and more likely to have extracranial carotid, intracranial, or vertebral disease as well as aneurysm and dissection. Depression and anxiety was also more common in FMD patients with HA. Further research is needed to classify the types of headaches being experienced as well as effective management strategies.

Fibromuscular dysplasia - results of a multicentre study in Flanders.

Fibromuscular dysplasia (FMD) is an idiopathic, non-inflammatory, non-atherosclerotic vascular disease, resulting in focal narrowing of small and medium-sized arteries. Systematic recording of clinical data in central databases as in the US and France provided new insights into FMD. The main objectives of this multicentre study were to explore the epidemiology, pattern of vascular involvement, clinical manifestations, and management of FMD patients in Flanders. Multicentre, retrospective registry of patients diagnosed with FMD based on medical imaging. Hundred-twenty-three FMD patients (83.7 % female) were included. Mean age at FMD diagnosis was 57.3 years (SD 15.8). More than half of patients (59.5 %) were hypertensive at the time of diagnosis. Neurological complaints such as headache (26.4 %) and dizziness (23.1 %) were also frequently reported. FMD was discovered incidentally in 10 patients (8.3 %). Nearly one quarter (22.8 %) of patients experienced a cerebrovascular event. Aneurysms were found in one-fifth (20.3 %) of patients and 11.4 % had an arterial dissection. FMD affected most frequently the renal (85.3 %), carotid (74.7 %), and vertebral (39.8 %) arteries. Renovascular FMD was more prevalent in men, whereas cerebrovascular FMD was more frequent in women. Multiple affected sites were documented in 25 of 61 (41.0 %) patients, having two or more vascular beds imaged. Digital subtraction angiography was most frequently used for detecting FMD. One third (32.9 %) of patients received an interventional treatment, mainly patients with renovascular FMD (32.8 % underwent percutaneous transluminal angioplasty) and patients with an intracranial carotid aneurysm (36.4 % were treated by means of coiling). Although differences existed, results of the Flemish registry were broadly in line with the US and French registries. Patient databases help to learn more about the natural history, progression, and management of FMD, based on real life clinical evidence.

Clinical Commentary on "My Life With Fibromuscular Dysplasia: A Sword of Damocles".

Reading the perspective written by Dr Young about her experiences with fibromuscular dysplasia (FMD) filled my mind with the faces of literally dozens of patients like her whom I have had the privilege to meet as they came to grips with their diagnosis of FMD and with what it would mean for them and their families. Dr Young’s experiences and observations, although clearly informed by her being an experienced physician, are nonetheless quite like most people who find themselves not only threatened by the seriousness of this diagnosis but isolated in a real way by the rarity of FMD, and the confusion the diagnosis can cause. There are 4 special challenges for us as we help our patients face rare, serious diseases. Dr Young describes them eloquently. One is the time that passed from the initial onset of her symptoms to a diagnosis. Second, she also shared the fright that comes from realizing that many of her physicians were …

My Life With Fibromuscular Dysplasia: A Sword of Damocles.

My story begins in December 2007 when I visited my general practitioner (GP) for one of the first times since the birth of my third son in 1992. At the time, I worked as a GP myself and had been at the same busy Scottish practice since qualifying in 1986. I also led an active life outside of work, ran a youth group at church and enjoyed regular gym workouts and several sports. Being a mother of 3 boys kept me constantly on the go! I had never suffered any major illness, was on no medication, and had no obvious risk factors for heart or vascular disease. Having suffered 3 months of morning headaches and some elevated blood pressure readings (around 170/100) taken at my office, I felt I should seek medical advice. I was also experiencing extra heart beats (ventricular ectopics) fairly frequently and some mild left-sided upper back discomfort. Some basic blood tests were done, along with another blood pressure reading, and I was referred to a cardiologist. The cardiologist performed an EKG, which showed normal patterns other than frequent ventricular ectopics. My blood pressure readings were mildly elevated at 158/100 and later 142/95. No heart murmurs were found, and an echocardiogram was normal. An appointment was arranged for me to have ambulatory blood pressure recordings to see how my blood pressure behaved over a 24-hour period, and an initial diagnosis of white coat hypertension was made. This is defined as blood pressure that is raised when measured by medical professionals but normal outside the doctor’s office. My ambulatory blood pressure recordings were within normal limits, and no further action was taken. My morning headaches and occasional palpitations continued, but I was able to manage the headaches with simple analgesics. In September 2009, at age 49, I suffered …

Revisiting Fibromuscular Dysplasia: Rationale of the European Fibromuscular Dysplasia Initiative.

Fibromuscular dysplasia (FMD) has been defined as an idiopathic, segmental, nonatherosclerotic, and noninflammatory disease of the musculature of arterial walls, leading to stenosis of small- and medium-sized arteries.1,2 Furthermore, to confirm the diagnosis of FMD, arterial diseases of monogenic origin, inflammatory arterial diseases, and the use of arterial vasoconstrictors have to be excluded. Renal FMD may lead to renovascular hypertension, less frequently renal artery dissection, renal infarction, and aneurysm rupture.1 Cervico-cephalic FMD can result in ischemic or hemorrhagic stroke, cervical artery dissection, and may be also associated with intracerebral aneurysms and risk of subarachnoid hemorrhage.3 In some patients, the diagnosis of FMD can lead to invasive procedures, such as percutaneous angioplasty, reconstructive surgery, or intracranial aneurysm clipping. Thus, both the disease and its treatment can lead to significant morbidity and mortality.1 The French4–6 and the US7–10 registries have led to a reappraisal of the frequency, demographic characteristics, classification, pathophysiology, and management of FMD. The face of the disease is evolving from a rare disease of renal arteries accounting for a minority of cases of secondary hypertension in young women to a systemic vascular disease affecting renal and also cervico-cephalic, coronary, and iliac arteries1,11 (Table 1). In various cohorts, despite incomplete exploration, mostly driven by symptoms and clinical complaints, FMD of ≥2 vascular beds was consistently found in >30% of cases.1,7,12,13 The French ARCADIA (Assessment of Renal and Cervical Artery Dysplasia) registry, which required a standardized vascular work-up in all patients, will soon provide a more precise estimate of the frequency of diffuse FMD. Besides female sex and repeated mechanical trauma because of increased kidney mobility,14 recent works suggest a contribution of smoking6 in progression of the …

NATURAL HISTORY OF CERVICAL ARTERY FIBROMUSCULAR DYSPLASIA AND ASSOCIATED NEUROVASCULAR EVENTS

Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory arteriopathy that most often affects the carotid and renal arteries. In the US Registry for FMD an aneurysm was identified in 21.7% and dissection in 25.7% of patients before or at FMD diagnosis. Occurrence of vascular events

Fibromuscular dysplasia: what the radiologist should know: a pictorial review.

Fibromuscular dysplasia (FMD) is an idiopathic, segmentary, non-inflammatory and non-atherosclerotic disease that can affect all layers of both small- and medium-calibre arteries. The prevalence of FMD is estimated between 4 and 6 % in the renal arteries and between 0.3 and 3 % in the cervico-encephalic arteries. FMD most frequently affects the renal, carotid and vertebral arteries, but it can theoretically affect any artery. Radiologists play an important role in the diagnosis of FMD, and good knowledge of FMD’s signs will certainly help reduce the delay between the first symptoms and diagnosis. The common string-of-beads aspect is well known, but less common presentations also have to be considered. These less common imaging findings include vascular loops, fusiform vascular ectasia, arterial dissection, aneurysm and subarachnoid haemorrhage. These radiologic presentations should be known by radiologists in order to diagnose possible FMD, particularly when present in young females or when associated with personal or familial hypertension, to reduce the delay between the onset of the first symptom and the final diagnosis. The patients have to be referred to specialised FMD centres for dedicated management.Teaching Points• Fibromuscular dysplasia is not a rare disease. • Radiologists should recognise less common presentations to orient specific management. • Vascular loops, fusiform vascular ectasia and a “string-of-beads” aspect are typical presentations. • Arterial dissection, aneurysm and subarachnoid haemorrhage are less typical radiologic presentations.

Treatment of renal fibromuscular dysplasia in an adolescent male: A case report

Introduction: Fibromuscular dysplasia (FMD) is a rare vascular disease that causes stenosis in the major arteries such as the renal and carotid arteries. The exact prevalence of FMD in the general population is not known. It is often presented with symptoms of hypertension. Mainly reported in middle-aged females, the diagnosis of FMD is often delayed. There are very few case reports of FMD in males and children in literature. Case Report: We report the case of a 13-year-old asymptomatic boy found to have FMD in the right renal artery (RRA). An angiography revealed high grade stenosis that was corrected by surgery. A hepatorenal bypass using anastomosis of the gastroduodenal artery (GDA) to RRA was deemed appropriate. An angioplasty at the anastomosis six weeks after this procedure corrected an upward trend in postsurgical blood pressure readings and RRA blood velocities. Two years after the surgery, the patient has well-controlled blood pressure, managed with low dose of ACE-inhibitors. Conclusion: Hepatorenal bypass of RRA through anastomosis of GDA was a viable surgical option in this case. The monitoring of blood pressure readings allowed for early detection and correction of any post-surgical stenosis.

Fibromuscular Dysplasia Leading to Spontaneous Coronary Artery Dissection with Sudden Cardiac Arrest.

A 30-year-old previously healthy female, who was six-week postpartum, experienced sudden collapse and tonic-clonic seizure. Emergency medicine services arrived at the scene and the patient was found to be in ventricular fibrillation. Advanced cardiovascular life support (ACLS) was initiated with return of spontaneous circulation. Afterwards, her initial EKG showed atrial fibrillation with rapid ventricular rate, ST elevation in leads II, III, and aVF, and ST depression in V2–V4. She was transferred to a tertiary care hospital where emergent angiogram was performed revealing obstruction of blood flow in the proximal and mid right coronary artery (RCA). A hazy and irregularly contoured appearance of the RCA was consistent with diagnosis of fibromuscular dysplasia. Subsequently, intravascular ultrasonogram (IVUS) was performed which confirmed the diagnosis of RCA dissection. Successful revascularization of the RCA was performed using two bare mental stents. After a complicated course in hospital, she was discharged in stable condition and did very well overall.

Clinical management of renal artery fibromuscular dysplasia: temporal trends and outcomes.

Renal artery fibromuscular dysplasia (FMD) is a heterogeneous condition with a broad and evolving phenotypic and angiographic spectrum. We documented therapeutic management in patients with renal artery FMD, by analyzing the medical records of hypertensive patients diagnosed with FMD at a single referral center from 1986 to 2012. Characteristics at presentation, treatments and outcomes were compared between patients diagnosed with FMD before and after the year 2000. Characteristics at presentation and outcomes were compared between patients managed conservatively, by angioplasty or surgery. Patients seen since 2000 (n = 278) were older, had lower blood pressure (BP) levels and were more often managed conservatively than those diagnosed before 2000 (n = 134). Revascularized patients had more often focal FMD and were more often men, diagnosed with hypertension and FMD at a younger age, had higher BP levels and a higher prevalence of renal asymmetry or infarction than patients treated conservatively. At the most recent visit, BP was below 140/90 mmHg in two-thirds of the patients, in all treatment groups. Multifocal FMD was managed conservatively in 60% of the cases (older patients, less severe hypertension) with similar BP outcome compared to revascularization, even after adjustment for potential confounding factors. The trends towards the diagnosis of FMD in older patients with less severe hypertension underscore the need for a careful selection of patients who might benefit from revascularization. This is especially true for multifocal FMD, which might be adequately managed by medication in a substantial number of cases.

Clinical Features and Endovascular Management of Iliac Artery Fibromuscular Dysplasia

PurposeTo identify the spectrum of clinical presentation of iliac artery fibromuscular dysplasia (FMD) and to evaluate the outcomes of endovascular management of iliac artery FMD for claudication. Materials and MethodsAll patients in a single institution with a diagnosis of FMD between January 1980 and December 2010 were identified. Iliac artery FMD was present in 14 patients. Associated risk factors included hypertension (79%), hyperlipidemia (64%), smoking history (36%), coronary artery disease (21%), diabetes (0%), and obesity (36%). ResultsIn eight (57%) patients, iliac artery FMD was incidentally found on imaging. Lifestyle-limiting claudication involving one or both extremities was present in six (43%) patients. All six patients were reported to have mild peripheral arterial disease based on ankle-brachial index measurements (0.7–0.9). These six patients underwent 10 endovascular procedures for claudication, including angioplasty (n = 8) and self-expanding stent placement (n = 2). Mean symptom-free survival was 56.3 months. ConclusionsIliac artery FMD that is amenable to endovascular treatment may be found incidentally or may manifest with disabling claudication.