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1/52. Cerebral arteriovenous malformation in pregnancy: presentation and neurologic, obstetric, and ethical significance.

    Cerebral arteriovenous malformations infrequently complicate pregnancy. We sought to determine the neurologic, obstetric, and ethical significance of such malformations. We present the clinical course of 2 pregnant women with arteriovenous malformations who experienced cerebral hemorrhage and a loss of capacity for decision making. We also review the neurologic and obstetric significance of arteriovenous malformations in pregnancy. Various treatment options with concern for pregnancy and the prognosis for arteriovenous malformations are outlined. The ethical issues involved for pregnant patients whose decisional capacity is compromised as a result of cerebral injury are explored. A review of persistent vegetative state and brain death (death by neurologic criteria) occurring in pregnancy allows us to explore many issues that are applicable to decisionally incapacitated but physiologically functioning pregnant women. We outline a document, the purpose of which is to obtain advance directives from pregnant women regarding end-of-life decisions and to appoint a surrogate decision maker. We believe that evaluation and treatment of the arteriovenous malformation may be undertaken without regard for the pregnancy and that the pregnancy should progress without concern for the arteriovenous malformation.
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2/52. De novo cavernoma case report and review of literature.

    BACKGROUND: De novo cavernoma, reported with the familial form of disease, is rare in cases with a negative family history. Cranial radiation, coexistent vascular malformation, genetic and hormonal factors, previous surgery for intracranial lesions, or other apparently unrelated intracranial lesions have been reported as risk factors. methods: We report a case of de novo cavernoma without a family history and without previous irradiation or any other known risk factors. The genesis of this lesion is discussed. RESULTS: To our knowledge, this is the first case, based on two separate magnetic resonance imaging (MRI) studies, demonstrating evidence of de novo cavernous malformations in the absence of familial history, brain radiation therapy, or other apparently unrelated intracranial tissue lesions. Based on previous negative computed tomography scans, other cases have been presented as de novo cavernous angiomas; thus it is possible that the newly discovered cavernoma existed previously but had been missed on previous poorer quality or lower resolution imaging studies. CONCLUSIONS: Cavernoma can arise even without an associat family history; in our case, a previous head injury could have set off either a genetic cascade with attendant endothelial proliferation or a latent virus.
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3/52. Haemorrhagic complication after total extirpation of huge arteriovenous malformations.

    Two cases with huge arteriovenous malformations (AVMs) who developed haemorrhagic complications after surgery are described. The cause of the postoperative haemorrhage was considered to be the normal perfusion pressure breakthrough phenomenon and/or occlusive hyperaemia. These two haemodynamic insults possibly occur simultaneously and induce life-threatening haemorrhage. It was concluded that a huge high-flow AVM with a large venous ampulla in its deep drainers has a high risk of a postoperative intravenous thrombosis, resulting in haemorrhage together with normal perfusion pressure breakthrough phenomenon.
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4/52. Endovascular particulate and alcohol embolization for near-fatal epistaxis from a skull base vascular malformation.

    A three-year old girl who initially presented at 16 months with epistaxis from an anterior skull base vascular anomaly returned with near-fatal epistaxis despite embolization of the internal maxillary artery at the time of initial presentation. Repeat angiography demonstrated blood supply to the vascular lesion coming from branches of both the external and internal carotid arteries. The patient underwent four sessions of endovascular embolization with polyvinyl alcohol, alcohol, and gelatin microspheres. At the conclusion of treatment, her epistaxis had resolved and the only residual blood supply to the lesions came from bilateral ophthalmic arteries intentionally not sacrificed to prevent loss of vision. Endovascular embolization of both the internal and external carotid arteries is a challenging but important treatment option for life-threatening epistaxis resulting from vascular lesions of the skull base.
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5/52. Cerebellar arteriovenous malformation with facial paralysis, hearing loss, and tinnitus: a case report.

    OBJECTIVE: To describe cerebellar arteriovenous malformation in a 21-year-old man with symptoms resembling those of ear disease and to discuss the relationship between the findings of neurotologic examination and magnetic resonance imaging. STUDY DESIGN: Case report. SETTING: Department of otolaryngology, Head-and-neck Surgery of the Kyoto University Hospital, which is a tertiary care center, in Kyoto, japan. PATIENT: A 21-year-old man had cerebellar arteriovenous malformation and symptoms resembling those of ear disease: recurrent left facial paralysis, left retrocochlear hearing loss, and tinnitus. Auditory brainstem responses showed only waves I and II on his left side. Downbeat nystagmus was seen by anteflexion and retroflexion of his neck. He also experienced a slight sensory disturbance on the left side of his face and right lower extremity. magnetic resonance imaging and vertebral angiography revealed a cerebellar arteriovenous malformation and a varix functioning as a drainer of the arteriovenous malformation surrounded by an edematous region probably induced by a small hemorrhage in the brainstem. INTERVENTION: Conservative treatment, including intravenous prednisolone, vitamin B12, and oral adenosine triphosphate was performed followed by total extirpation of the arteriovenous malformation. CONCLUSION: In examining patients with peripheral facial paralysis (sometimes recurrent with a short interval) and other symptoms resembling those of ear disease, especially those suggesting certain central disorders, it is important to take intracranial arteriovenous malformation into consideration because the condition may be sometimes life threatening if overlooked.
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6/52. Magnetic resonance demonstration in the newborn of generalized cerebral venous dilation with spontaneous resolution.

    The authors present serial magnetic resonance (MR) images of an infant with cardiac failure and generalized cerebral venous dilation, which was initially misdiagnosed in the first week of life on cranial ultrasound as a vein of Galen malformation. At 3 months of age, repeat MR imaging demonstrated complete resolution of this marked cerebral venous distension with no evidence for cerebral injury. This case illustrates the value of MR in the identification of this disorder and its distinction from more serious conditions, such as vein of Galen malformation and venous sinus thrombosis. Complete resolution of the venous dilation and the lack of definable parenchymal injury suggest a good prognosis for this disorder.
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7/52. MR perfusion imaging in a case of cerebral proliferative angiopathy.

    Proliferative angiopathy is an uncommon type of cerebral arteriovenous shunt characterized by an extensive capillary network with normal brain intermingled and few clinical symptoms (mostly seizures and headaches). This case report depicts an extensive proliferative angiopathy located in the right hemisphere. Its hemodynamic disturbances were studied with MR perfusion imaging (local or remote areas of increased time to peak, decrease ratio of signal, and relative regional cerebral blood volume values).
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8/52. Spontaneous disappearance of vein of galen malformation and posterior fossa venous pouch.

    Cerebrovascular anomalies remain an issue of controversy regarding diagnosis, classification, and treatment. We report the first case of total and asymptomatic regression and disappearance of a vein of Galen malformation associated with a posterior fossa venous pouch. Different aspects of the vein of Galen are discussed together with emphasis on the underlying mechanisms of spontaneous thrombosis and regression.A 4-month-old boy presented with macrocrania and signs of intracranial hypertension. Computerized tomography disclosed two masses, the first was a giant aneurysmal dilatation in the posterior fossa, and the second was a gigantic pouch at the level of the vein of Galen. hydrocephalus was treated by ventriculo-peritoneal shunting. Two months later, the shunt was revised, and posterior fossa was explored without active treatment. Both abnormalities regressed spontaneously. No recurrence occurred, and the child remained neurologically intact. Total disappearance of the masses as well as normal brain and cerebrovascular anatomy were confirmed by angiography, MRI, and MRA. Over a follow-up period of 17 years, the patient did not develop complications. He had perfect clinical tolerance and resumed a normal life.
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9/52. Recombinant tissue plasminogen activator in the treatment of intraventricular hemorrhage secondary to periventricular arteriovenous malformation before surgery: case report.

    OBJECTIVE AND IMPORTANCE: Intraventricular hemorrhage (IVH) is known to cause acute obstructive hydrocephalus, refractory elevated intracranial pressures (ICPs), and lowered cerebral perfusion pressures, leading to cortical ischemia. Frequent obstruction of external ventricular drains as a result of thrombus is a recurring theme. We present a case of IVH secondary to periventricular arteriovenous malformation (AVM) that was not visible at admission angiography and was treated by intraventricular infusion of recombinant tissue plasminogen activator before surgical intervention. CLINICAL PRESENTATION: An 11-year-old boy presented with acute onset of headache followed by two seizures, loss of consciousness, decerebration, right temporal hematoma, IVH, and acute obstructive hydrocephalus. INTERVENTION: A right external ventricular drain was placed but functioned poorly. ICP could not be controlled by conventional methods. Five milligrams of recombinant tissue plasminogen activator was injected into the ventricular system via the external ventricular drain. This was repeated daily for 4 days. This treatment resulted in progressive improvement in ICP and clinical status. Once the clot partially cleared, magnetic resonance imaging and magnetic resonance angiography suggested the presence of a right periventricular arteriovenous malformation, which was confirmed by angiography and subsequently resected. CONCLUSION: Recombinant tissue plasminogen activator is effective in resolving IVH causing obstructive hydrocephalus and uncontrollable ICP posing a life-threatening situation, secondary to ruptured arteriovenous malformation, before surgical intervention.
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10/52. Immunohistochemical analysis of a cerebral arteriovenous malformation obliterated by radiosurgery and presenting with re-bleeding. Case report.

    The purpose of this study was to analyze immunohistochemical characteristics of the cell population in a radiosurgically obliterated cerebral arteriovenous malformation (AVM) after recurrent hemorrhage. Immunohistochemical reactions were carried out on paraffin-embedded histological sections for von willebrand factor (FVIII), CD34 endothelial antigen (CD34), vimentin and alpha-smooth-muscle actin (SMA) cytoskeletal proteins. Histopathological analysis revealed that the majority of AVM channels were replaced by hypocellular scar tissue. However some of them still disclosed thrombus organization by granulation tissue seven years after radiosurgery. FVIII and CD34 reactions revealed vessel neoformation in thromboses. Proliferating spindle-shaped cells with SMA and vimentin expression were identified in the granulation tissue. These histopathological findings suggest the role of re-canalization in recurrence of hemorrhage following radiosurgical obliteration of the AVM. Contribution of myofibroblastic elements in the vessels' occlusion after radiosurgery is also demonstrated.
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