1/47. Neurofibromatosis, stroke and basilar impression. Case report.Neurofibromatosis type 1 (NF1) can virtually affect any organ, presenting most frequently with "cafe au lait" spots and neurofibromas. Vasculopathy is a known complication of NF1, but cerebrovascular disease is rare. We report the case of a 51-year-old man admitted to the hospital with a history of stroke four months before admission. On physical examination, he presented various "cafe au lait" spots and cutaneous neurofibromas. neurologic examination demonstrated right-sided facial paralysis, right-sided hemiplegia, and aphasia. Computed tomography scan of head showed hypodense areas in the basal ganglia and centrum semiovale. Radiographs of cranium and cervical spine showed basilar impression. angiography revealed complete occlusion of both vertebral and left internal carotid arteries, and partial stenosis of the right internal carotid artery. A large network of collateral vessels was present (moyamoya syndrome). It is an uncommon case of occlusive cerebrovascular disease associated with NF1, since most cases described in the literature are in young people, and tend to spare the posterior cerebral circulation. Basilar impression associated with this case may be considered a pure coincidence, but rare cases of basilar impression and NF1 have been described.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/47. Multiple coronary artery aneurysms in a child with neurofibromatosis type 1.A number of frequently unrecognised vascular manifestations have been described in patients with neurofibromatosis type 1 (NF1), including involvement of the great vessels, cerebral, visceral and renal arteries. Rarely, changes in the coronary arteries have been reported in adults with NF1. We report on a 16-year-old boy affected by NF1 with dysmorphic features and three aneurysms in the mid-portion of the left descending coronary artery disclosed by chance during investigation for a malignant peripheral nerve sheath tumour. Molecular analysis detected a gross de novo deletion in the NF1 gene. The boy had had no previous cardiac symptoms but died suddenly after developing signs and symptoms suggestive of myocardial infarction. CONCLUSION: To the best of our knowledge, this represents the first report of multiple lesions in the coronary arteries in a child affected by neurofibromatosis type 1 with a known deletion of the neurofibromatosis type 1 gene.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/47. An anterior tibial artery aneurysm in a patient with neurofibromatosis.Von Recklinghausen's Type 1 neurofibromatosis (NF) is a genetic disorder of neuroectodermal tissue. Arterial involvement has also been recognized. Stenotic lesions predominate, but aneurysms have been documented as well. Aortoiliac, visceral, and cervical aneurysms are well represented in the literature. Rarely noted, however, have been peripheral aneurysms. Typically in more proximal vessels, subclavian, femoral, and popliteal aneurysms have been mentioned. We present the case of a patient with type 1 NF and an anterior tibial artery aneurysm that was subsequently resected. neurofibromatous invasion of the vessel was confirmed by means of pathological tests. We think this is the first reported case of a tibial artery aneurysm associated with NF.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
4/47. Embolization of a ruptured intercostal artery aneurysm in type I neurofibromatosis.We present the case of a female with history of a ruptured lumbar aneurysm years ago. She was known to have neurofibromatosis type I with the typical clinical signs. The patient was transferred to us with a hematothorax and an aortic lesion was suspected on the outside CT scan. Reevaluation of the investigation raised suspicion of a ruptured intercostal artery aneurysm, which was consequently demonstrated on angiography. The aneurysm was embolized and the patient recovered uneventful. We will discuss the optimal therapy for vessel lesions in neurofibromatosis type I.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/47. Diffuse, retroperitoneal mesenteric and intrahepatic periportal plexiform neurofibroma in a 5-year-old boy.We present a case of plexiform neurofibroma involving the retroperitoneum, mesentery, and liver in a 5-year-old boy who underwent evaluation for extent of a palpable left neck mass. The mass had intrathoracic extension with great vessel encasement and extension into the abdomen. Abdominal CT revealed a diffuse low-attenuation non-enhancing mass encasing the retroperitoneal vessels with serpiginous extension into the liver along the portal vein. This spread pattern of plexiform neurofibroma is an unusual manifestation of neurofibromatosis in a young child.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
6/47. A case of intracranial arteriovenous fistula in an infant with neurofibromatosis type 1.INTRODUCTION: Reported cases of arteriovenous fistula (AVF) with neurofibromatosis type1 (NF1) are rare. CASE REPORT: In this paper we report the first case of intracranial AVF in an NF1 infant who developed heart failure. Endovascular treatment using coils successfully obliterated the AVF. The mechanism underlying the AVF in this case was believed to be a congenital mesenchymal abnormality of the intracranial vessels. DISCUSSION: The mechanism underlying the development of heart failure in this case is also discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/47. perfusion MRI abnormalities in the absence of diffusion changes in a case of moyamoya-like syndrome in neurofibromatosis type 1.We report on a 12-year-old boy with neurofibromatosis type 1 who suffered a transient ischemic attack. angiography revealed occlusion of intracranial arteries, moyamoya vessels and leptomeningeal collaterals. The conventional T2-weighted and the diffusion-weighted MRI images demonstrated no pathology. Dynamic first-pass postgadolinium T2* perfusion-weighted MRI depicted altered hemodynamics in the vascular territory of the left middle cerebral artery, which defined this region as ischemic tissue at risk. The patient suffered a repeat transient ischemic attack 5 days later.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/47. Spontaneous hemothorax associated with von Recklinghausen's disease.Spontaneous hemothorax is a rare and life threatening complication of neurofibromatosis. Two types of vascular involvement have been described: (a) stenotic or aneurysmal alterations in large vessels such as the aorta and its branches; and (b) dysplastic features in smaller vessels. thoracotomy and surgical ligation of the bleeding vessels is primarily indicated in the presence of active bleeding with associated hemodynamic compromise. A more conservative approach with endovascular embolization or non-operative management have also been reported in case of hemodynamic stability. We present a rare case of spontaneous hemothorax treated conservatively in a patient with von Recklinghausen's disease.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
9/47. Retinal vascular abnormalities in neurofibromatosis type 1.Microvascular retinal abnormalities, presenting in a corkscrew configuration, have been very recently described in patients with neurofibromatosis type 1 (NF-1). We report one more patient with NF-1 who had distinctive corkscrew retinal vessels superior and inferior to the fovea. This patient further supports the existence of a true association between this recently described retinal finding and NF-1.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/47. A case of neurofibromatosis associated with a coronary artery aneurysm and myocardial infarction.Aneurysms of the aorta, vertebral arteries, carotid artery, thyrocervical trunk, and mesenteric and celiac arteries have been described in patients with neurofibromatosis. These aneurysms may have part of the vessel wall replaced by neurofibromatosis tissue. One previous case reports a coronary artery aneurysm in a patient with neurofibromatosis who suffered a myocardial infarction due to thrombosis formation within the aneurysm. We document a second case of aneurysmal dilatation of a coronary artery in a patient with neurofibromatosis. This patient also had a myocardial infarction in a vascular distribution not involved by the aneurysm. The vascular changes associated with neurofibromatosis are reviewed. Aneurysmal dilatation of the coronary arteries may be a further vascular manifestation of this condition.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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