1/28. Ocular malformations, moyamoya disease, and midline cranial defects: a distinct syndrome.PURPOSE: To report a 10-year-old girl with developmental anomalies of both optic disks, a chorioretinal coloboma, sphenopharyngeal meningoencephalocele, and moyamoya disease. methods: A full ophthalmologic examination, cranial magnetic resonance imaging and magnetic resonance angiography, and cerebral angiography were performed. RESULTS: The patient had a morning glory disk anomaly and microphthalmos of the right eye and optic nerve hypoplasia and retinochoroidal coloboma in the left eye. She had a midfacial cleft and an episode of seizures and a stroke. magnetic resonance imaging showed a sphenopharyngeal meningoencephalocele. magnetic resonance angiography and cerebral angiography demonstrated a pattern consistent with moyamoya disease. CONCLUSIONS: This patient had a distinct syndrome of optic disk, retinochoroidal, and carotid circulation anomalies with midline cranial defects. The recognition and treatment of the vascular abnormalities and cranial defects may prevent complications such as strokes that may occur during or after general anesthesia.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
2/28. association of cerebral arteriovenous malformations and spontaneous occlusion of major feeding arteries: clinical and therapeutic implications.OBJECTIVE: The spontaneous occlusion of a cerebral arteriovenous malformation (AVM) occurs rarely. Occlusion of a parent artery feeding the AVM is even more rare, and its incidence is unknown. We undertook this study to determine the incidence of occlusion of a major artery feeding an AVM and to recommend a management strategy for such an AVM. methods: We identified AVMs associated with an occluded artery by performing a retrospective angiographic analysis of 500 patients with AVMs who presented to Henry Ford Hospital from 1976 to 1998. RESULTS: A review of the angiograms revealed that 7 (1.4%) of 500 patients with an AVM had occlusion of one or more major arteries feeding the nidus. In four patients, an internal carotid artery and its bifurcation were occluded; in two patients, the M1 segment of the middle cerebral artery was occluded, and in one patient, a vertebral artery was occluded. Pial collaterals and/or a moyamoya pattern of anastomoses developed in all patients, with the exception of one who had vertebral artery occlusion. Five patients underwent definitive treatment: one received radiosurgery, and four underwent surgical excision. One of the surgically treated patients died of complications from excessive blood loss and coagulopathy, but the other three had no postoperative complications. CONCLUSION: The occlusion of a major artery feeding an AVM occurs rarely (1.4%). These AVMs are moderate to large in size (>3 cm). To prevent collateral flow-related complications of cortical "steal" and hemorrhage, as well as the usual risk of hemorrhage from the AVM itself, surgical management should be considered for these AVMs.- - - - - - - - - - ranking = 43.096475839129keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
3/28. Morning glory disk anomaly, choroidal coloboma, and congenital constrictive malformations of the internal carotid arteries (moyamoya disease).PURPOSE: To report a patient with congenital constrictive malformations of the internal carotid arteries and a morning glory disc anomaly (MGDA). methods: Descriptive case report. RESULTS: A 14-year-old girl underwent serial ophthalmologic examinations since the age of seven years because of blurred vision and floaters. A right optic disk anomaly was present that was most compatible with an MGDA accompanied by an inferior choroidal coloboma. Because of the association of such disc malformations with moyamoya disease, the patient underwent cerebral MRI and MRA that showed narrowing of both internal carotid arteries, a finding consistent with early-stage moyamoya disease. CONCLUSION: This case illustrates the importance of recognizing the association of MGDA with moyamoya disease, a treatable condition that may have devastating neurological consequences.- - - - - - - - - - ranking = 1.5keywords = malformation (Clic here for more details about this article) |
4/28. Management of residual dysplastic vessels after cerebral arteriovenous malformation resection: implications for postoperative angiography.OBJECTIVE: The verification of surgical resection of cerebral arteriovenous malformations (AVMs) relies on angiography. Abnormal vasculature often is identified after removal of the AVM. Differentiation of dysplastic feeding vessels that resemble the neovascularity of moyamoya disease, as distinct from residual AVM, is crucial for preserving critical brain areas. We review a large experience with immediate postoperative angiography after AVM resection and discuss the implications for management of abnormal dysplastic vessels discovered after AVM resection. methods: Beginning in 1992, 86 consecutive patients with AVMs underwent operations by standard protocol for immediate postoperative angiography under the same general anesthetic. Angiographic interpretation dictated admission to the intensive care unit or return to the operating room for further resection. RESULTS: In 78 patients, the angiogram revealed complete resection. Two patients were returned to the operating room, one for residual malformation with an early draining vein, and one for resection of residual dysplastic vessels. There was one postoperative hemorrhage in a patient whose postoperative angiogram was falsely negative for AVM. Six patients with residual dysplastic vessels mimicking residual AVM, but without an early draining vein, were managed conservatively. Delayed follow-up angiography demonstrated spontaneous involution of these abnormal vessels in all of these patients. CONCLUSION: Residual dysplastic feeding vessels resembling the neovascularity of moyamoya disease but not associated with an early draining vein do not necessarily represent residual malformation after AVM resection. The abnormal vessels will proceed to complete spontaneous resolution. Given the difficulty of interpreting intraoperative angiography, immediate postoperative angiography may be a viable alternative after AVM resection.- - - - - - - - - - ranking = 43.596475839129keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
5/28. Radiosurgical treatment of a cerebral arteriovenous malformation in a patient with moyamoya disease: case report.OBJECTIVE AND IMPORTANCE: A case of a cerebral arteriovenous malformation (AVM), which was treated with gamma knife surgery, in a patient with moyamoya disease is described. This is the first report of radiosurgical treatment of a cerebral AVM in a patient with moyamoya disease; the outcome was satisfactory. CLINICAL PRESENTATION: The patient presented with frequent episodes of transient ischemic attacks and seizures. INTERVENTION: Staged encephaloduroarteriosynangiosis was performed on both sides, with an interval of 10 months. Postoperative cerebral angiography demonstrated augmented cerebral perfusion, which increased the size of the AVM nidus. The patient subsequently underwent gamma knife surgery, and the AVM completely disappeared 2 years later. CONCLUSION: radiosurgery may be a good treatment option for AVMs accompanying moyamoya disease, allowing the preservation of collateral vessels and the prevention of possible misery perfusion.- - - - - - - - - - ranking = 43.096475839129keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
6/28. livedo reticularis in a child with moyamoya disease.moyamoya disease is a rare, chronic cerebrovascular occlusive disease of unknown etiology. It is characterized by progressive stenosis of the arteries of the circle of willis leading to an abnormal capillary network and resultant ischemic strokes or cerebral hemorrhages. The association of moyamoya disease with livedo reticularis has been described in a previously reported patient with a factor v Leiden mutation, leading to hypercoagulation. We describe a girl with livedo reticularis and moyamoya disease with extensive cardiovascular malformations, but without a primary coagulopathy.- - - - - - - - - - ranking = 0.25keywords = malformation (Clic here for more details about this article) |
7/28. Moyamoya angiopathy with dolichoectatic internal carotid arteries, patent ductus arteriosus and pupillary dysfunction: a new genetic syndrome?We report on 2 children with moyamoya angiopathy and bilateral dolichoectatic internal carotid arteries in combination with iris hypoplasia with bilateral fixed dilated pupils and a history of patent ductus arteriosus. Both were symptomatic with moyamoya angiopathy and underwent bilateral extracranial-intracranial (EC-IC) bypass operations for cerebral revascularization. This is the first report on moyamoya angiopathy and bilateral dolichoectatic internal carotid arteries with simultaneous occurrence of ocular and cardiovascular malformations. There have been descriptions of cerebral vascular abnormalities in combination with either congenital heart disease or ocular abnormalities but not with both presenting together. The combination of these separate congenital developmental defects may not be purely coincidental: we propose that the 2 probands are affected with a not yet recognized clinical syndrome of probably genetic etiology.- - - - - - - - - - ranking = 0.25keywords = malformation (Clic here for more details about this article) |
8/28. Caudate hemorrhage with moyamoya-like vasculopathy from atherosclerotic disease.BACKGROUND AND PURPOSE: Caudate hemorrhage usually results from hypertension, rupture of arteriovenous malformation or aneurysm, or rarely, moyamoya disease. Moyamoya-like changes related to severe atherosclerotic occlusive disease, usually causing ischemic stroke, have been reported. CASE DESCRIPTION: A 51-year-old normotensive patient was admitted with headache due to a left caudate hematoma with ventricular extension. There was a history of smoking, leg claudication, elevated cholesterol, and coronary artery disease. Angiography demonstrated complete extracranial carotid occlusion on the left and atherosclerotic stenosis at the bifurcation on the right, with supraophthalmic occlusion distally. At the base of the brain, bilateral moyamoya-like vessels, presumed to be secondary to atherosclerotic occlusion, were evident, but neither aneurysm nor arteriovenous malformation was present. Cerebral blood flow and transcranial Doppler studies indicated severely impaired cerebral perfusion that improved after bilateral extracranial-to-intracranial bypass surgery. CONCLUSIONS: Atherosclerotic occlusive carotid disease with moyamoya-like changes may be a rare cause of caudate hemorrhage. A decrease in moyamoya vessels with bypass surgery may reduce the risk of recurrent hemorrhage.- - - - - - - - - - ranking = 17.238590335651keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
9/28. Hemorrhage from moyamoya-like vessels associated with a cerebral arteriovenous malformation. Case report.The authors describe a case of subarachnoid hemorrhage from moyamoya-like vessels associated with an arteriovenous malformation (AVM) in a 44-year-old Hispanic man who presented with severe headache. The AVM was located in the left parietal lobe and the ipsilateral middle cerebral artery was occluded. Although the patient was initially neurologically intact, he began to experience neurological deficits from mild vasospasm, illustrating the sensitivity of the underperfused portion of brain surrounding an AVM. His neurological deficits improved with aggressive hydration and elevated blood pressure. After a 3-week period, the AVM was resected without complication and all of the patient's neurological deficits resolved. The authors review radiographic findings of this unique case.- - - - - - - - - - ranking = 43.096475839129keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
10/28. Development of a de novo cerebral arteriovenous malformation in a child with sickle cell disease and moyamoya arteriopathy. Case report.The authors report the unique case of a 6-year-old African-American girl with sickle cell disease (SCD) and an associated moyamoya arteriopathy who developed a de novo arteriovenous malformation (AVM) of the cerebral circulation. Based on preoperative cerebral angiography, computerized tomography angiography, and magnetic resonance imaging, the incidentally discovered lesion was originally thought to be a direct arteriovenous fistula with an associated varix. At surgery, however, a 1.5-cm AVM was identified adjacent to the deep surface of the varix, and it was successfully resected. The diagnosis of cerebral AVM was then confirmed histopathologically. Based on a review of the literature, no published correlation between cerebral AVMs and SCD exists. In addition to reporting this case, the authors provide a description of AVM pathogenesis, with particular emphasis on acquired AVMs of the cerebral circulation.- - - - - - - - - - ranking = 43.096475839129keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
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