1/19. Case report of fibromuscular dysplasia presenting as stroke in a 16-year-old boy.fibromuscular dysplasia of the carotid artery is uncommon, but not rare. Although the true incidence and prevalence of the disease are not known, reported figures in adults range between 0.6% by angiography and 1.1% at autopsy. Most case reports of stroke caused by carotid fibromuscular dysplasia describe findings in adult subjects, although there are a few reports of the disease in children. In the present case, we describe a 16-year-old boy with fibromuscular dysplasia confined to one internal carotid artery and its branches, and in whom the disease declared itself by stroke. This case serves as a basis for considering diagnostic methods, treatment options, and future research in pediatric patients with cerebrovascular disease caused by fibromuscular dysplasia.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/19. Pulmonary arterial fibromuscular dysplasia: a rare cause of fulminant lung hemorrhage.Arterial fibromuscular dysplasia (FMD) represents a collection of noninflammatory and nonatherosclerotic vascular diseases with a poorly understood etiology. Classically occurring in renal and cerebral arteries, this entity has also been reported in coronary, carotid, and other medium and small arteries. One case occurring in the pulmonary vasculature has been reported. Fatal hemothorax and lung hemorrhage have multiple causes, including other vascular malformations and connective tissue disorders; however, cases of pulmonary FMD are exceedingly rare. We report what appears to be the second such association, occurring in a 69-year-old man. The patient presented with a 3-week history of increasing dyspnea, fatigue, and productive cough; 3 days of increasing back and chest pain; and syncope. Chest radiograph showed a "white-out" of the left lung. The patient died shortly after admission from a fulminant respiratory disease of undetermined etiology. At autopsy he was found to have a massive left hemothorax resulting from an unsuspected pulmonary arterial fibromuscular dysplasia.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
3/19. De novo formation of an aneurysm in a case of unusual intracranial fibromuscular dysplasia.Intracranial fibromuscular dysplasia (FMD) is a vascular disease of unknown origin occurring predominantly in young women. The internal carotid artery is most often involved, but other cerebral arteries may also be affected. We report the case of a young woman presenting with an unusual angiographic appearance of intracranial FMD of the internal carotid artery (ICA) that could not be categorized into any type of the Osborn-Anderson classification. During follow up the patient presented with an intracerebral and subarachnoid hemorrhage. Repeated angiography revealed multiple aneurysms in the pathologic segment of the vessel. The patient underwent surgical treatment with clipping of the aneurysms, wrapping of the pathologic segment of the ICA and biopsy of the superficial temporal artery. Histopathological sections revealed FMD of the intimal type. alpha(1)-antitrypsin blood levels were normal. Cases of intracranial FMD previously reported in the literature are reviewed and various aspects of this rare disease are discussed.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
4/19. Severe hypertension in children with renovascular disease.Renovascular disease is an important cause of hypertension in children and is associated with considerable morbidity and mortality risks. Secondary hypertension is more common in children than in adults, with children accounting for 75% to 80% of cases. In 70% of secondary hypertension in children, the cause is fibromuscular hyperplasia. Other associated conditions are aorto-aortitis, the midaortic syndrome, and Williams-Bueren syndrome. Imaging techniques have an important role in the early discovery of renal artery stenosis. Although renal arteriography remains the definitive method, noninvasive and less invasive radiographic procedures such as ultrasonography with duplex Doppler scanning and radionuclide scintigraphy have been used as adjunct diagnostic tools in children. The authors describe three young children with renovascular hypertension in whom dynamic radionuclide scintigraphy with Tc-99m MAG3 played an essential role in the diagnosis of renovascular hypertension.- - - - - - - - - - ranking = 5keywords = vascular disease (Clic here for more details about this article) |
5/19. Percutaneous angioplasty for renovascular hypertension due to fibromuscular dysplasia.Because the cause of hypertension is reversible in only 5 percent of patients, extensive initial work-up should only be considered in selected cases. Secondary causes should be suspected in patients whose hypertension begins before age 30 or after age 50 and in patients whose hypertension suddenly worsens after a long period of good control, becomes severe or malignant, or remains refractory to maximal medical therapy. A sudden reduction in renal function in a hypertensive patient and the discovery of a unilateral small kidney may also raise suspicion of a secondary cause. Renovascular disease, one of the most common secondary causes of hypertension, is usually the result of atherosclerosis in older patients and the result of fibromuscular dysplasia in younger patients. Physical examination seldom contributes to the diagnosis. The classic upper abdominal or flank bruit occurs in only 30 to 50 percent of patients with renovascular disease, and is not uncommon in patients with essential hypertension. The gold standard for diagnosis of renovascular disease remains the arteriogram. Transluminal renal angioplasty may be performed during arteriography if a high-grade stenosis is identified. Other management options include medical therapy and surgical revascularization with grafts.- - - - - - - - - - ranking = 3keywords = vascular disease (Clic here for more details about this article) |
6/19. aneurysm in the skin: arterial fibromuscular dysplasia.We describe a pulsatile aneurysm in the skin of 16-year-old boy that was found to be a sign of a systemic vascular disease, that is, arterial fibromuscular dysplasia. The patient had aneurysms in the renal, cerebral, coronary, and other arteries; he developed renovascular hypertension and had a cerebrovascular accident and acute myocardial infarction at 17 years of age. This disease has not been previously reported in the dermatologic literature.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/19. Intracranial atypical fibromuscular dysplasia with ruptured aneurysm--case report.A 53-year-old woman was admitted with severe subarachnoid hemorrhage due to rupture of an aneurysm associated with atypical intracranial fibromuscular dysplasia (FMD). angiography demonstrated the aneurysm and very irregular form of the left internal carotid artery (ICA), the right ICA, and right proximal middle cerebral artery (MCA). Other arteries showed signs of atherosclerosis. The aneurysm was treated by embolization, but she subsequently died of shock of unknown cause. Detailed examination of serial angiograms detected enlargement of the aneurysm and progression of the irregular appearance of the ICA. FMD is a non-inflammatory and non-atheromatous arteriopathy that commonly affects the cervical ICA and sometimes the intracranial ICA. The association with saccular aneurysm is widely known and the prevalence of incidental aneurysms is higher than that in the general population. The common "string of beads" finding is easily distinguished from other vascular diseases, but non-specific findings such as "tubular stenosis" and "diverticular-like outpouching" are harder to differentiate. FMD is associated with various complications and appropriate periodic follow-up examination is required. Detailed analysis of serial angiograms may facilitate diagnosis of this condition.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/19. Renal fibromuscular dysplasia in elderly persons.renal artery stenosis (RAS) is a significant cause of secondary hypertension, the progression of which can lead to renal insufficiency, uncontrolled hypertension, and even end-stage renal disease. The 2 most common forms of RAS are atherosclerotic renovascular disease and fibromuscular dysplasia (FMD). atherosclerosis accounts for 90% of all cases of RAS and generally affects an elderly population. Conversely, FMD accounts for approximately 10% of all RAS cases and is described as affecting a younger population. Four cases of FMD in individuals older than 70 years are presented, in a period of 1 year at 1 facility. This case series calls into question the previously reported low prevalence of FMD in elderly persons. It is conceivable that renal artery investigation might be denied an elderly patient thought to have atherosclerotic disease. Because conventional angioplasty is considered the treatment of choice for patients with FMD because of the high response rate for uncontrolled hypertension, the prevalence of FMD in the elderly population should be reevaluated to detect and treat this population accordingly.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
9/19. fibromuscular dysplasia of the brachial artery: a case report and review of the literature.fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease that involves primarily medium-sized and small arteries. fibromuscular dysplasia is characterized by medical fibrosis with or without smooth muscle cell hyperplasia and may produce luminal impingement with severe turbulence. Secondary aneurysmal deformity with or without thrombosis may also contribute to the obstruction. fibromuscular dysplasia most commonly involves the renal and carotid arteries, with upper-extremity disease rarely reported. This case report describes a patient with digital embolization from brachial artery fibromuscular dysplasia. angiography demonstrated significant narrowing and irregularity with a characteristic "string-of-beads" appearance of the right midbrachial artery. The abnormal segment was resected and reconstructed with a reversed saphenous vein graft. Histologic studies revealed disruption of the internal elastic lamina and disorientation of the hyperplastic medial smooth muscle cells characteristic of fibromuscular dysplasia.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
10/19. Recurrent transient ischemic attacks and stroke in association with an internal carotid artery web.fibromuscular dysplasia is a nonatherosclerotic vascular disease that most commonly affects cervical carotid arteries at the C1-C2 level when cephalic arteries are involved. Several histopathologic and angiographic subtypes of fibromuscular dysplasia exist; most have a benign natural history. We describe the third reported case of a pathologically proven, symptomatic proximal internal carotid artery web and suggest that patients with this lesion are at a higher risk for stroke.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
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