Displasia fibromuscular y síndrome de ligamento arcuato medio. Infrecuentesy simultáneos / Fibromuscular dysplasia and median arcuate ligament syndrome. Rare and simultaneous findings

Introducción: la displasia fibromuscular (DFM) es una patología poco frecuente de la capa muscular de las arterias. El síndrome de ligamento arcuato medio (SLAM) es una entidad infrecuente causada por la compresión extrínseca del tronco celíaco por el diafragma. Caso clínico: presentamos el caso de una mujer joven con DFM diagnosticada de afectación a nivel del tronco celíaco y de la arteria hepática común. Ante clínica de dolor abdominal, se solicita angio TC, que describe un SLAM asociado a la DFM. Se decide sección quirúrgica del ligamento arcuato y descompresión del tronco celíaco mediante abordaje robótico. Discusión: en ambas entidades la angiografía es el trataminto de referencia para el diagnóstico. El tratamiento de primera línea de la DFM es el endovascular mediante angioplastia, y del SLAM, el quirúrgico, seccionando el ligamento arcuato.(AU)

Introduction: fibromuscular dysplasia (FMD) is a rare disorder that affects the muscular layer of the arteries. The medianarcuate ligament syndrome (MALS) is also a rare disorder due to the extrinsic compression of the celiac trunk by thediaphragm.Case report: we report the case of a young woman with FMD and splachnic involvement of the celiac trunk and thecommon hepatic artery level. After presenting with abdominal pain, a CCTA was performed that revealed the presenceof FMD-related MALS. The surgical section of the arcuate ligament and decompression of celiac trunk were decided andperformed through robotic approach.Discussion: the gold standard for the diagnosis of both entities is angiography. However, while the first-line therapy ofFMD is endovascular, in the case MALS the best alternative is surgical treatment sectioning the arcuate ligament.(AU)

Multiple recurrences of spontaneous coronary artery dissection in a woman with fibromuscular dysplasia.

A 54-year-old woman with no prior coronary artery disease or cardiac risk factors was diagnosed with spontaneous coronary artery dissection (SCAD) after presenting with an acute coronary syndrome. Over the next 5 years, she experienced four more episodes of SCAD, involving different coronary artery distributions, with evidence of complete angiographic healing following conservative management with antiplatelet therapy and beta-blockade.

Internal Carotid Artery Web as the Cause of Recurrent Cryptogenic Ischemic Stroke.

Carotid artery web is considered an exceptional cause of recurrent ischemic strokes in the affected arterial territory. The underlying pathology proposed for this entity is an atypical fibromuscular dysplasia. We present the case of a 43-year-old woman with no cardiovascular risk factors who had experienced 2 cryptogenic ischemic strokes in the same arterial territory within an 11-month period. Although all diagnostic tests initially yielded normal results, detailed analysis of the computed tomography angiography images revealed a carotid web; catheter angiography subsequently confirmed the diagnosis. Carotid surgery was performed, since which time the patient has remained completely asymptomatic. The histological finding of intimal hyperplasia is consistent with previously reported cases of carotid artery web. Carotid artery web is an infrequent cause of stroke, and this diagnosis requires a high level of suspicion plus a detailed analysis of vascular imaging studies.

Anti-platelet and anti-hypertension medication use in patients with fibromuscular dysplasia: Results from the United States Registry for Fibromuscular Dysplasia.

Fibromuscular dysplasia (FMD), a non-inflammatory arterial disease, may lead to renovascular hypertension (HTN) and cerebrovascular disease. Little is known about medication use in FMD. Clinical features and medication use were reviewed in a national FMD registry (12 US sites). Medication usage was assessed in raw and adjusted analyses. Covariates included demographic characteristics, co-morbid conditions and vascular bed involvement. A total of 874 subjects (93.6% female) were included in the analysis. Mean age was 55.6±13.1 years, 74.5% had HTN, 25.4% had a history of transient ischemic attack or stroke, and 7.5% had a history of coronary artery disease (CAD). Renal and cerebrovascular arteries were affected in 70.4% and 74.7%, respectively. Anti-platelet agents were administered to 72.9% of patients. In multivariate analyses, factors associated with a greater likelihood of anti-platelet agent use were older age (OR=1.02 per year, p=0.005), CAD (OR=3.76, p=0.015), cerebrovascular artery FMD involvement in isolation (OR=2.31, p<0.0001) or a history of previous intervention for FMD (OR=1.52, p=0.036). A greater number of anti-HTN medications was evident in isolated renal versus isolated cerebrovascular FMD patients. Factors associated with a greater number of anti-HTN medications were older age (OR=1.03 per year, p<0.0001), history of HTN (OR=24.04, p<0.0001), history of CAD (OR=2.71, p=0.0008) and a history of a previous therapeutic procedure (OR=1.72, p=0.001). In conclusion, in FMD, medication use varies based on vascular bed involvement. Isolated renal FMD patients receive more anti-HTN agents and there is greater anti-platelet agent use among patients with cerebrovascular FMD. Further studies correlating medication use in FMD with clinically meaningful patient outcomes are necessary.

Spontaneous coronary artery dissection associated with fibromuscular dysplasia.

This case presentation concerns a woman known to have fibromuscular dysplasia (FMD) who presented with an acute coronary syndrome (ACS). The coronary angiogram was considered to be normal. However, as spontaneous coronary artery dissection (SCAD) has a close association with FMD, subsequent meticulous review of the angiogram revealed a dissection within the circumflex coronary artery. SCAD causes 10% of ACS seen in women under 55 years of age. Both FMD and SCAD are underdiagnosed and SCAD may be overlooked or misdiagnosed on coronary angiography. The recommended management of SCAD differs from that of the usual presentations of ACS. For this reason, one should be alert to the possibility of SCAD when confronted by ACS in a younger woman, especially when she is known to have FMD.

Contemporary diagnosis of carotid fibromuscular dysplasia: role of power Doppler and a review of other diagnostic modalities.

Fibromuscular dysplasia (FMD) is a nonatheromatous, noninflammatory arteriopathy with segmental involvement of medium-sized arteries in multiple vascular beds. It most commonly involves the renal and carotid arteries. The etiology is unknown, although various hormonal and mechanical factors have been suggested. The disease can occur at any age but is usually diagnosed in middle-aged individuals, predominantly women. FMD is much more common than previously thought, perhaps affecting as many as 4% of adult women. Clinical manifestations of the internal carotid artery involvement are transitory ischemic attacks or cerebral infarction, as well as nonspecific symptoms such as headache and vertigo. In cases of cerebrovascular events, endovascular or surgical treatment is recommended; therefore, detection of FMD is of considerable importance. The gold standard for diagnosing FMD is catheter angiography (with the classic "string of beads" pattern), but this invasive procedure is only used for patients in whom it is clinically pertinent to proceed with revascularization. The optimal noninvasive modality for diagnosis and quantification for FMD is not known and little information has been recently published about new diagnostic modalities. Although angiography, computed tomography angiography, and magnetic resonance angiography are excellent in confirming the morphologic diagnosis of FMD, they are less accurate in assessing the hemodynamic significance of the lesions. Ultrasonography is useful in assessing the degree of carotid artery stenosis. Use of power Doppler ultrasound improves the ability to detect the morphologic features of carotid FMD.

Displasia fibrosa maxilar poliostótica en paciente tratada con pamidronato: a propósito de un caso / Maxilar polyostotic fibrous dysplasia treated with pamidronate: a case report

El término displasia fibrosa hace referencia a un conjunto de lesiones óseas benignas que se caracterizan por la sustitución del tejido óseo normal por tejido conectivo. Se presenta el caso de una paciente afectada de displasia fibrosa poliostótica de predominio maxilar tratada de forma conservadora con bisfosfonatos(AU)

The term fibrous dysplasia refers to a variety of bony diseases characterized by the substituion of the bone by abnormal connective tissue. A case report of patient affected by a polyostotic form of fibrous dysplasia with an uneven evolution of its disease after being treated with pamidronate is presented(AU)

Internal carotid artery fibromuscular dysplasia in arterial hypertension: management in clinical practice.

Fibromuscular dysplasia (FMD) reminds of a rare form of secondary arterial hypertension occurring in young people and involving the renal arteries. FMD may also involve vertebral, subclavian, mesenteric, iliac arteries and carotid arteries. FMD of internal carotid arteries is a rare finding that is frequently incidental and asymptomatic. It usually occurs in middle-aged women and is secondary to media-intima fibrodysplasia. The carotid artery may be elongated or kinked and associated cerebral aneurysms have been reported. Symptoms including transient ischaemic attack or stroke are uncommon and are related to decrease of blood flow or embolization by platelet aggregates. At the onset, differential diagnosis with vasculitis must be placed. Computed tomography or magnetic resonance imaging (MRI) angiography demonstrates bilateral high-grade stenosis with the characteristic "string of beads" pattern. Antiplatelet medication is the accepted therapy for asymptomatic lesions. Graduated endoluminal surgical dilation is an outmoded therapy, no longer used in most medical centres. Current percutaneous angioplasty is the preferred treatment for symptomatic carotid FMD, but no randomized controlled trials comparing this methodology with surgery is available. The management of a case of arterial systemic FMD in a 52-year-old women, diagnosed after a hypertensive crysis, is discussed. Imaging methods disclosed stenoses of carotid arteries, of celiac tripod and of superior mesenteric artery. Because of high risk associated to endovascular surgery, medical therapy was started. In the first year of follow-up, no events have been reported.

Contemporary management of fibromuscular dysplasia.

PURPOSE OF REVIEW: Fibromuscular dysplasia is an underdiagnosed and misunderstood disease. The purpose of this review is to inform healthcare providers and the public about a condition that may be more common than previously thought. RECENT FINDINGS: There has been little new information published about fibromuscular dysplasia in the past 30 years. The International Registry that is now underway will remedy that situation and provide a large number of patients to study with this condition. SUMMARY: Fibromuscular dysplasia is a noninflammatory, nonatherosclerotic disease that has been reported in almost every arterial bed and primarily affects women aged 15-50 years. It most commonly presents in the renal and extracranial cerebrovascular arteries, either manifesting as hypertension, transient ischemic attack or stroke, respectively. Some patients may be asymptomatic and fibromuscular dysplasia could only be discovered by imaging for some other reason or by the detection of an asymptomatic bruit. Dissection or aneurysm may also occur in patients with fibromuscular dysplasia. The true prevalence is unknown, partially because of the fact that it is underdiagnosed in many patients. Treatment consists of antiplatelet therapy for asymptomatic individuals and percutaneous balloon angioplasty for patients with indications for intervention. Patients with macroaneurysms should be treated with either a covered stent or surgery.

Displasia fibromuscular, disección y oclusión asintomática de la arteria basilar / Fibromuscular dysplasia. Asymptomatic dissection and occlusion of the basilar artery

Introducción. Clásicamente, la oclusión de la arteriabasilar ha sido considerada una entidad de mal pronóstico ycon alta tasa de morbimortalidad. Estudios prospectivos másrecientes sugieren, sin embargo, un pronóstico más benigno.La arteria basilar se afecta sobre todo por la arteriosclerosis,pero también puede ser asiento de embolismos, disecciones,aneurismas y otras afecciones como la displasiafibromuscular.Caso clínico. Un hombre de 46 años fue ingresado por unepisodio transitorio de ataxia y disartria. El Doppler transcranealreveló un flujo estenótico en la arteria basilar conseñales microembólicas distales a la estenosis. La angiografíapor resonancia magnética (RM) demostró una displasiafibromuscular aislada en la arteria basilar que causaba unaestenosis mayor del 50 % sin lesiones parenquimatosas. Seinició tratamiento anticoagulante y 10 meses después, permaneciendoel paciente asintomático, una RM de controlmostraba una disección que evolucionó a oclusión completade la arteria basilar. Su porción distal se rellenaba por flujoinverso desde ambas arterias comunicantes posteriores. Laanticoagulación fue suspendida.Discusión. La displasia fibromuscular de la arteria basilares rara y habitualmente asintomática. Aunque puededesembocar en una disección, la presentación clínica másfrecuente es el ictus isquémico secundario a la estenosis. Lasdisecciones intracraneales se asocian con riesgo de hemorragiasubaracnoidea. Pese a ello, muchos autores recomiendanel tratamiento anticoagulante para la displasia fibromuscular.La oclusión de la arteria basilar, como muestraeste caso, no siempre conlleva un mal pronóstico. Éste estádeterminado, al menos en parte, por la presencia de una circulacióncolateral adecuada (AU)

Introduction. Historically, basilar artery occlusive disease has been considered to convey a poor prognosis and a high mortality rate. In contrast, recently prospective studies have shown a better prognosis. The basilar artery is most commonly affected by atherosclerosis, but may also be affected by embolisms, dissections, aneurysms, and other conditions like fibromuscular dysplasia. Case report. The case of a 46 year-old male who was admitted after suffering a transient episode of ataxic gait and dysarthria is reported. Transcranial Doppler showed a stenotic flow in the basilar artery with distal embolic signals. Magnetic resonance (MR) angiography demonstrated an isolated fibromuscular dysplasia of the basilar artery that caused a > 50% stenosis without parenchymal lesions. Oral anticoagulation treatment was initiated. The control MR angiography performed ten months later, and although the patient was asymptomatic, showed a dissection which evolved into a complete occlusion of the basilar artery. Its distal portion was filled via a reverse flow from both posterior communicating arteries.Anticoagulation treatment was discontinued. Discussion. Fibromuscular dysplasia of the basilar artery is a rare and usually asymptomatic disease. It may develop into a dissection, but the usual clinical presentation is stenosis-related ischemic stroke. Intracranial artery dissections may be further complicated by subarachnoid hemorrhage. Many authors have recommended anticoagulant therapy for fibromuscular dysplasia. Basilar artery occlusion, as this case shows, does not always convey a poor prognosis. This is determined, at least partially, by the presence of good collateral circulation (AU)

An uncommon variant of fibromuscular dysplasia.

Fibromuscular dysplasia is a rare cause of stroke affecting mostly young females. It is characterized by the typical "string of beads" sign located mostly bilaterally in the midcervical portion of the carotid or vertebral arteries. We present the uncommon case of borderzone hemispheric infarction in a man with isolated unilateral fibromuscular dysplasia affecting continuously the distal extracranial and proximal intracranial portion of the left internal carotid artery leading to distal hypoperfusion and ischemia.

Fibromuscular dysplasia of bilateral brachial arteries treated with surgery and consecutive thrombolytic therapy.

A 61-year-old female was admitted to our hospital complaining of paresthesia, pain, and intermittent weakness in the right hand. A pulsating mass with bruits had developed on the patient's upper arm. We also noted an absence of radial artery pulsation. The angiographic findings revealed a classic "string of beads" appearance, which involved both brachial and renal arteries. The right brachial artery exhibited an aneurysm, which was filled with thrombus, and the distal radial artery was occluded with thromboemboli. We excised the abnormal brachial artery segment, replacing it with an autogenous reversed saphenous vein conduit. Consecutive thrombolytic therapy was then performed for the treatment of the radial artery embolism. Histological examination revealed that the patient was suffering from medial fibromuscular dysplasia. This uncommon form of fibromuscular dysplasia, which involves both brachial arteries with embolization, can be efficiently treated via surgery and consecutive thrombolytic therapy.

Carotid artery fibromuscular dysplasia.

Extracranial carotid artery fibromuscular dysplasia (FMD) is a rare finding that is frequently incidental and asymptomatic. It usually occurs in middle-age women and is secondary to medial fibrodysplasia or, less commonly, intimal fibrodysplasia. The carotid artery may be elongated or kinked and associated aneurysms have been reported. Symptoms including transient ischemic attack or stroke are uncommon and are due to low flow or embolization of platelet aggregates. Digital subtraction angiography demonstrates high-grade stenosis with the characteristic "string of beads" pattern. Antiplatelet medication with sequential imaging is the accepted therapy for asymptomatic lesions. Graduated endoluminal dilation under direct vision should be reserved for patients with documented lateralizing symptoms.