1/40. 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 = intima (Clic here for more details about this article) |
2/40. Successful treatment of intimal hyperplasia in renal arteries by endovascular brachytherapy.PURPOSE: The present study shows the possibility of preventing restenosis of renal arteries by endovascular brachytherapy. methods AND MATERIALS: We present a patient suffering from rapid restenosis of both renal arteries with decreasing renal function. Percutaneous transluminal angioplasty (PTA) and stent implantation were unable to stop hypertension and to stabilize renal function. Both renal arteries and the right pole artery were treated by endovascular brachytherapy in one session. RESULTS: Six months after intervention, intraarterial digital subtraction angiography (DSA) showed no evidence of recurrence, and the blood pressure remained normal without medical treatment. CONCLUSION: Endovascular brachytherapy can help to prevent restenosis in renal arteries. It is possible to treat both renal arteries and one pole artery in one session without any disadvantage.- - - - - - - - - - ranking = 4keywords = intima (Clic here for more details about this article) |
3/40. Arrhythmic sudden cardiac death in a 3-year-old child with intimal fibroplasia of coronary arteries, aorta, and its branches.We report an unusual case of "arrhythmic" sudden cardiac death in a 3-year-old child who died of ischemic myocardial lesions as a result of intimal fibroplasia of the coronary arteries. Also affected were the aorta and its major branches, whereas renal and mesenteric arteries, celiac trunk, and systemic veins were normal. Histopathologic examination showed severe concentric thickening of intima because of a proliferation of spindle-shaped cells (mesenchymal cells) set in an abundant extracellular matrix. In some vascular segments the intima was densely fibrotic and hyalinized. No significant inflammation, foam cells, cholesterol clefts, or other evidence of atheroma were present. The intimal lesions did not involve the media and/or the adventitia. Immunohistochemical staining of intima showed the proliferating mesenchimal cells to be myofibroblastic. Reactions for vimentin and smooth muscle actin were positive, while those for desmin, myosin, CD34, and factor viii were negative.- - - - - - - - - - ranking = 9keywords = intima (Clic here for more details about this article) |
4/40. Childhood subarachnoid hemorrhage associated with fibromuscular dysplasia.A rare case of childhood subarachnoid hemorrhage (SAH) is reported. A 5-year-old boy presented with SAH. A cerebral angiogram showed obstruction of the first segment of the left anterior cerebral artery (A1). The A1 was surgically trapped to prevent rebleeding. Proliferation of myofibroblasts was revealed in the intima of the A1. cerebral infarction appeared in the territory of the left middle cerebral artery (MCA) on day 2 after onset of SAH, and the patient suffered a second SAH on day 26. The second angiogram showed stenosis and a saccular aneurysm in the left MCA. A second surgery, neck clipping of the aneurysm, was needed. The multiple arterial lesions and histological findings indicated that the SAHs and the infarction were caused by the dissecting aneurysm affected with fibromuscular dysplasia (FMD). Intracranial FMD is mainly detected in children and causes cerebral ischemia and bleeding. Surgical treatment of childhood SAH should be discretely chosen with due consideration for FMD.- - - - - - - - - - ranking = 1keywords = intima (Clic here for more details about this article) |
5/40. fibromuscular dysplasia of coronary arteries resulting in myocardial infarction associated with hypertrophic cardiomyopathy in Noonan's syndrome.The first autopsy case of fibromuscular dysplasia in the coronary arteries associated with hypertrophic cardiomyopathy in Noonan's syndrome is reported. A 16-month-old female infant with no significant family history was diagnosed with Noonan's syndrome and subsequently died of cardiac and respiratory failure. autopsy revealed cardiac hypertrophy, atrial septal defect, and scar lesions in the left ventricle and ventricular septum. Histologically, the myocardium exhibited myocardial fiber disarray, which was indicative of hypertrophic cardiomyopathy. The main trunks of the coronary arteries showed protuberant intimal thickening with interruption of the internal elastic lamina. Intramyocardial coronary arteries also exhibited various degrees of irregular intimal proliferation and diffuse fibrous thickening of the adventitia. These arterial lesions were consistent with fibromuscular dysplasia. Small arteries around the scar showed remarkable stenoses, which probably led to myocardial ischemia. The fibromuscular dysplasia in this case was considered to arise as a cardiovascular disorder in conjunction with Noonan's syndrome.- - - - - - - - - - ranking = 2keywords = intima (Clic here for more details about this article) |
6/40. Moyamoya and extracranial vascular involvement: fibromuscular dysplasia? A report of two children.We present two patients with moyamoya syndrome and the unusual involvement of extracranial vessels. The first case illustrates the rare association between moyamoya and primary pulmonary hypertension. In the second patient, moyamoya was complicated by stenoses of vertebral, renal, and mesenteric arteries. In both cases, a systemic intima-proliferative disease, such as fibromuscular dysplasia (FMD), was suspected to be the cause of both intracranial and extracranial arterial disease.- - - - - - - - - - ranking = 1keywords = intima (Clic here for more details about this article) |
7/40. Pulmonary vascular changes in scleroderma.To determine the incidence and significance of pulmonary vascular changes in scleroderma, all necropsy reports of patients with scleroderma who died at the massachusetts General Hospital were analyzed and correlated with clinical data. The records of 30 such patients were available. Fourteen had moderate or marked abnormalities in the pulmonary arterial tree. Nine of these 14 patients had predominantly respiratory symptoms. The arterial changes consisted pathologically of intimal and medial hyperplasia affecting pulmonary arteries of all sizes. Of the eight patients with the most severe pathologic changes in the pulmonary arteries, five had slight or no interstitial fibrosis. Three of these five patients had rapidly progressive respiratory failure and severe pulmonary hypertension leading to death. Such cases form a distinct clinicopathologic entity of malignant pulmonary hypertension in scleroderma, comparable to the better recognized entity of malignant renal hypertension in scleroderma.- - - - - - - - - - ranking = 1keywords = intima (Clic here for more details about this article) |
8/40. Primary intimal fibroplasia in a child with Down's syndrome.A 14-year-old girl known to have trisomy 21 and who came to us with weight loss followed by hypertension proved to have diffuse arterial dysplasia involving the intimal layer (primary intimal fibroplasia). This is a rare cause of elevated blood pressure in children and has not been previously reported in a child with Down's syndrome. Unusual features of this case also include the widespread distribution of the lesions limited to the large muscular distributing arteries, the rapidly progressive course, and the predominance of abdominal signs and symptoms.- - - - - - - - - - ranking = 6keywords = intima (Clic here for more details about this article) |
9/40. Multifocal infantile myofibromatosis and generalized fibromuscular dysplasia in a child: evidence for a common pathologic process?Infantile myofibromatosis (IM) is a condition characterized by the formation of spindle cell tumors of skin, soft tissue, and viscera. Although small vessel involvement by the process is a frequently identified and indeed diagnostically useful histological finding, involvement of large vessels is not widely reported. fibromuscular dysplasia (FMD) is a noninflammatory arteriopathy characterized by intimal, medial, and/or adventitial fibroplasias leading to luminal compromise and aneurysm formation. Although venous disease has been reported, involvement of arterioles and viscera has not been identified. We report a patient in whom IM was diagnosed, on the basis of multiple soft tissue tumors present from birth, who subsequently developed generalized and ultimately fatal FMD. These two conditions exhibit overlapping pathologic features, including pronounced intimal fibroplasia. Their occurrence in a single individual may provide insights into the pathogenesis of both conditions, suggesting that they represent part of the same spectrum of vascular myofibroblastic proliferations.- - - - - - - - - - ranking = 2keywords = intima (Clic here for more details about this article) |
10/40. fibromuscular dysplasia of bilateral brachial arteries--a case report and literature review.fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic disease of the vascular system, that can affect intima, media, or adventitia. The dysplasia in these layers leads to decrease in the lumen and increased turbulence leading to diminished flow through the affected portion. The treatment of FMD thus far has been either surgical and/or interventional. The authors describe a patient who presented with painful digits and who was found to have bilateral brachial artery FMD and was treated with balloon angioplasty with complete resolution of symptoms.- - - - - - - - - - ranking = 1keywords = intima (Clic here for more details about this article) |
| | Next -> |