1/51. Clinically aggressive diffuse capillary telangiectasia of the brain stem: a clinical radiologic-pathologic case study.Capillary malformations or telangiectasias of the brain usually exhibit a benign clinical course, although occassionally they may be associated with mild to moderate symptomatology of uncertain origin. We report a case of an exceptionally aggressive capillary telangiectasia in a child, which was associated with progressive neurologic deterioration resulting in death.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
2/51. Spontaneous thrombosis of vein of Galen aneurysmal malformation.Two cases of aneurysmal malformations of the vein of Galen (AVG) with spontaneous thrombosis are reported. Angiogram and MRI before thrombosis demonstrated AVGs with slow arteriovenous shunts and associated stagnation of contrast in the venous sac secondary to severe outflow restriction. Based on these findings, one patient was managed conservatively, and the other underwent placement of a ventriculoperitoneal shunt. Surveillance of the lesions with subsequent MRIs revealed spontaneous thrombosis of the AVGs with excellent clinical outcomes. Proposed mechanisms of spontaneous thrombosis include slow flow shunts, obstruction of the venous outflow or obstruction of the feeding artery. Similar cases in the literature are reviewed with special emphasis on diagnostic tests, symptomatology, mechanisms of thrombosis and therapeutic options.- - - - - - - - - - ranking = 5keywords = malformation (Clic here for more details about this article) |
3/51. Management of vein of galen malformations. A review based on five neurosurgically treated cases and literature reports.The high morbidity and mortality associated with the management of vein of Galen aneurysmal malformations (VGAM) continues to pose a tremendous challenge to the neurosurgeon as well as to the attending interventional radiologist. Since 1985, five patients with VGAM have been referred to the neurosurgical unit of the University of Cologne, two neonates, one infant and two adults. Four patients underwent direct operation and two patients received a shunt. The treatment was performed without mortality. A review of the literature reflects no substantial difference between neurosurgical treatment during the last 15 years (mortality 10%) and endovascular treatment (best series mortality 6%).- - - - - - - - - - ranking = 5keywords = malformation (Clic here for more details about this article) |
4/51. Spinal intradural perimedullary arteriovenous fistula with varix in infant.A rare occurrence of type IV spinal arteriovenous malformation (intradural perimedullary arteriovenous fistula) is described in an 18-month-old boy initially misdiagnosed with guillain-barre syndrome. An intramedullary mixed-intensity mass lesion at Th1 was demonstrated by magnetic resonance imaging together with flow voids over the dorsal aspect of the swollen spinal cord. Angiography demonstrated an intradural perimedullary arteriovenous fistula including an intraparenchymal vascular pocket. After partial embolisation of the posterior spinal arteries through the left intercostal-radicular artery, the arteriovenous fistula was removed completely together with an organised haematoma. The fistula directly opened into a vascular pocket, which was confirmed pathologically to be a varix. The postoperative course was uneventful, and the patient resumed ambulation within 4 months. The case, subclassifiable as a type IVb spinal perimedullary AVF, was unique given its location and the patient's age at presentation.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
5/51. MRI of intraosseous dural arteriovenous malformation: findings in two cases.Intraosseous dural arteriovenous malformation (DAVM) is rare. The intraosseous component of this DAVM has usually been neglected on conventional angiography and found during operation after the bone drilling has been completed. Considerable bleeding usually occurs during operation because of the unexpected hypervascular bone marrow. We report the use of MRI to help in the diagnosis and therapeutic plan for this rare type of DAVM.- - - - - - - - - - ranking = 5keywords = malformation (Clic here for more details about this article) |
6/51. Superficial siderosis associated with multiple cavernous malformations: report of three cases.OBJECTIVE AND IMPORTANCE: Superficial siderosis is a rare but potentially devastating syndrome caused by recurrent subarachnoid hemorrhage. We present three cases of superficial siderosis associated with multiple cavernous malformations, and we review previous reports of superficial siderosis attributable to vascular malformations. CLINICAL PRESENTATION: patients most commonly present with progressive sensorineural hearing loss, cerebellar ataxia, and pyramidal signs. magnetic resonance imaging diagnosis may precede symptom development, however. In two of our cases, superficial siderosis was identified on magnetic resonance imaging scans in the absence of clinical symptoms. INTERVENTION: magnetic resonance imaging studies revealed hemosiderin deposition, characteristic of superficial siderosis, and multiple cavernous malformations in all three cases. Surgical intervention was not pursued. CONCLUSION: We conclude that patients with multiple cavernous malformations and those with perisubarachnoid lesions are at risk for the development of superficial siderosis. Clinicians should recognize the radiographic appearance of superficial siderosis and its clinical presentation in patients with vascular malformations.- - - - - - - - - - ranking = 9keywords = malformation (Clic here for more details about this article) |
7/51. Spinal arteriovenous malformations: a review with case illustrations.Spinal arteriovenous malformations are united by the existence of arteriovenous shunting but are quite heterogeneous in terms of pathology. Until recently, the pathological confusion has been such that management has been poorly understood and this is magnified by the rarity of the lesions. Type 1 AVMs, where the fistula is located in the dura, usually present with a venous hypertensive myelopathy and are relatively easily dealt with surgically. Type 2 AVMs, most closely mimicking the parenchymal AVMs of the brain, usually present with haemorrhage and may be surgically remediable but with much greater risk than the type 1 lesions. Type 3 AVMs, with a diffuse location through both the cord and extra-CNS tissue, usually present early in life with a myelopathy and are often untreatable. Type 4 AVMs, with a fistula located on the pial surface of the cord, usually present with a venous hypertensive myelopathy or subarachnoid haemorrhage, can be treated relatively easily by surgery when small but may be better treated endovascularly when the fistula is large.The purpose of this review is to summarise the current pathological, clinical and management literature with illustrative cases underscoring the important features of this heterogeneous disorder.- - - - - - - - - - ranking = 5keywords = malformation (Clic here for more details about this article) |
8/51. Symptomatic unruptured capillary telangiectasia of the brain stem: report of three cases and review of the literature.Three young patients with transient or intermittent focal neurological signs suggesting brain stem involvement are described, in whom high field MRI showed focal areas of hyperintensity in T2 weighted spin echo images, hypointensity in T2* weighted gradient echo images, and enhancement in postcontrast T1 weighted images consistent with unruptured capillary telangiectasia of the brain stem. The first patient was a 28 year old woman who complained of recurrent left ear tinnitus, exacerbated during the menstrual period; MRI demonstrated that the vascular anomaly involved the left acoustic pathway. The second patient was a 30 year old woman who had three episodes of paroxysmal left lip movement 4 weeks after child delivery; MRI showed capillary telangiectasia in the right corticonuclear pathway. The third patient, a 36 year old man, had a transient right Bell's palsy; MRI disclosed two circumscribed areas consistent with capillary telangiectasia in the left corticospinal tract and medial longitudinal fasciculus. Steroid receptors in the telangiectatic vessels walls might account for the recurrent and transient course seen in our two female patients. awareness of the MRI features of capillary telangiectasia may help in defining the real incidence, clinical correlation, and the risk of haemorrhagic complications of these vascular malformations.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
9/51. Venous malformation serving as the draining vein of an adjoining arteriovenous malformation. Case report and review of the literature.BACKGROUND: Widely accepted pathologic classification of venous malformations includes discrete venous, arteriovenous, capillary, and cavernous malformations, each with distinct pathological criteria for definition. Several authors have described mixed or transitional vascular malformations with pathological features of more than one type of malformation within the same lesion. We present a rare case of a venous malformation associated with an arteriovenous malformation (AVM). CASE DESCRIPTION: A 37-year-old woman presented with a loss of consciousness. Computed tomography showed an enlarged vein running along the lateral wall of the right lateral ventricle. A cerebral angiogram demonstrated an AVM and a venous malformation in the right hemisphere; the AVM and the venous malformation were located in proximity to each other with the AVM using the venous malformation as its draining vein. In this case, where an AVM used the venous malformation as the draining vein, only the AVM was treated by proton-beam radiosurgery. Follow-up magnetic resonance angiography demonstrated complete obliteration of the AVM with the venous malformation remaining unchanged. CONCLUSION: Arteriovenous shunting would have disturbed venous drainage resulting in the development of the venous malformation. Thus, in addition to demonstrating a rare coexistence of AVM and venous malformation, this case also offers a new insight into the pathogenesis of these vascular malformations.- - - - - - - - - - ranking = 22keywords = malformation (Clic here for more details about this article) |
10/51. Developmental deep venous system anomaly associated with congenital malformation of the brain.We report a rare case of developmental deep venous system anomaly. The great vein of Galen and the straight sinus were absent. Both internal cerebral veins and the basal veins of Rosenthal drained into a large frontal interhemispheric falcine vein, which eventually drained into the superior sagittal sinus. The patient also had an associated neuronal migration anomaly.- - - - - - - - - - ranking = 4keywords = malformation (Clic here for more details about this article) |
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