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11/70. 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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12/70. Surgical resection of cerebral arteriovenous malformation combined with pre-operative embolisation.

    To assess the importance of pre-operative embolisation, 27 cases of cerebral artriovenous malformation (AVM) treated in this institute between July 1994 and October 1998 were analysed. The patients' ages ranged from 3 to 70 years (average 36.9) with a follow-up period of 1-41 months (average 19.2). The patient presented with haemorrhage in 21 cases and seizure in five. In 21 of 27 cases, surgical resection of a nidus was performed, gamma knife therapy was applied in three and conservative therapy was chosen in three. Of 21 cases treated surgically, total removal was achieved in 19 cases and a residual nidus was seen in one (a large basal ganglia AVM). In the remaining case, postoperative angiography was not available. Pre-operative embolisation followed by surgical resection of the nidus was performed in seven cases in which there was a large AVM. A volume index was calculated to indicate the size of the nidus using X x Y x Z, where X is the maximum diameter (cm) of the nidus on the lateral angiogram, Y is the diameter (cm) perpendicular to X and Z is the maximum diameter (cm) on the anteroposter or angiogram. The index averaged 45.9 for the cases in which pre-operative embolisation was performed, while it was 5.6 in the cases without embolisation. Pre-operative embolisation was performed to reduce the nidus flow as much as possible, to prevent overload to the surrounding structures. At surgery, the nidus was resected from the surrounding tissue and care was taken not to enter the nidus. Postoperatively, the systolic blood pressure was maintained at 90-100 mmHg for several days in the intensive care unit. The results were excellent in 15 cases, good in three (hemiparesis due to the initial haemorrhage remained in all three), fair in one (a patient with a severe subarachnoid haemorrhage). Two patients died (acute pulmonary oedema and severe meningitis). Minor postoperative bleeding or oozing was seen in three cases. In conclusion, reducing the shunt flow through a nidus in a step-wise fashion with pre-operative embolisation of a large AVM seems to be quite helpful in preventing postoperative haemodynamic overload to the surrounding brain. It is also important not to enter the nidus when it is removed at surgery. This helps to prevent intraoperative and/or postoperative bleeding, and led to successful total removal of the nidus with a good postoperative course.
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13/70. recurrence of a cerebral arteriovenous malformation after surgical excision.

    Complete resection of a cerebral arteriovenous malformation (AVM) should eliminate the future risk of an associated intracranial bleeding. Because total removal of an AVM may be difficult to assess at the time of surgery, postoperative angiography has become the accepted standard for documenting that complete removal has been achieved. However, even angiographically confirmed excision of an AVM does not completely exclude the possibility of rebleeding. Regrowth of an AVM with subsequent haemorrhage has been documented in children and is attributed to forces acting on the immature vasculature. The authors report the case of a 21-year-old man whose AVM recurred 5 years after angiographically proven complete excision. According to the presented case, the authors emphasise that, even in adults, angiographic documentation of total removal does not always eliminate the risk of reformation of an AVM.
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14/70. Cerebellar mutism caused by arteriovenous malformation of the vermis.

    Transient mutism following posterior fossa tumour resection in children is well known in the literature. To our knowledge, this phenomenon has never been reported without surgical intervention. We report a case of cerebellar mutism secondary haemorrhage from a vermian arteriovenous malformation (AVM), which resolved to ataxic dysarthria after 6 weeks. Embolization of the AVM was performed and the patient's clinical status continued to improve gradually till she became normal 6 months from the insult. The mutism was due to rupture of the AVM which might correlate the cerebellar mutism with the transient vasospasm of the blood vessels supplying the cerebellum. It is possible for a spontaneus posterior fossa bleed to result in transient mutism similar to post surgical mutism.
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15/70. language representation in a patient with a dominant right hemisphere: fMRI evidence for an intrahemispheric reorganisation.

    Studies have suggested that congenital left hemispheric (LH) frontal arteriovenous malformations (AVMs) are associated with an early transfer of language to right hemisphere (RH) frontal regions. The question remains whether such anatomofunctional reorganisation is due to RH compensatory abilities or to a general principle of lateral shift. In this study, we used fMRI language paradigms to investigate the case of a patient presenting aphasic symptoms following an haemorrhage due to a right frontal AVM. Prior to surgery, fMRI showed that language processing was confined to the RH, suggesting that language had not shifted during childhood from this congenitally dominant RH to the LH. After surgery, the patient presented severe aphasia that recovered to presurgical level within 70 days. At this time, fMRI showed that language tasks were still not associated with activations in the LH. These results suggest that the principles of early cerebral reorganisation after congenital lesions may differ in the RH and the LH. In addition, they support the idea that efficient restoration of language is achieved if a sufficiently large neuronal network is preserved around the lesion.
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keywords = haemorrhage
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16/70. An angiographic lesion mimicking pseudo-aneurysm in cerebral arteriovenous malformation.

    In cerebral arteriovenous malformations (AVMs), a pseudo-aneurysm represents rupture site, and its presence is known as a factor for rebleeding. We report a case of cerebral AVM presenting with intracerebral haemorrhage in which cerebral angiography showed a lesion mimicking pseudo-aneurysm. Although the patient needed urgent surgical decompression, it was delayed because early haematoma evacuation would induce rebleeding from the rupture site. The authors attempted to occlude the pseudoaneurysm interventionally before surgery. After surgical excision, the lesion that was believed to be a pseudo-aneurysm was revealed as a partially thrombosed venous sac having a thick fibrous wall. In this report, the authors discuss the pitfalls in the interpretation of pseudo-aneurysms in angiographic AVM architecture.
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keywords = haemorrhage
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17/70. Dopa-responsive parkinsonism secondary to right temporal lobe haemorrahage.

    A 46-year-old man developed a symmetrical parkinsonian syndrome 7 weeks after large right temporal intracerebral haemorrhage resulting from a ruptured arteriovenous malformation. His signs included bradykinesia, rigidity, start hesitation, and poor postural reflexes, without a resting tremor. He also had signs of a Parinaud's syndrome. Computed tomography and magnetic resonance imaging of the brain demonstrated changes in the right temporal lobe associated with the haemorrhage but no abnormality of the basal ganglia or midbrain. levodopa therapy produced a dramatic improvement within a few days of commencement. We postulate that the parkinsonism resulted from midbrain compression secondary to transtentorial herniation. Although parkinsonism is a rare complication of lobar intracerebral haemorrhage, it is important to recognise as it may be potentially treatable.
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18/70. Primary intraventricular haemorrhage: a rare presenting feature of arteriovenous malformation in children.

    Two cases of arteriovenous malformation (AVM) of the brain presented with primary intraventricular haemorrhage in eleven and thirteen years old children, respectively. The cases responded favourably to therapy with steroids, anti-epileptic drugs and therapeutic embolisation in the former. Though such presentation accounts for 3% of the adult intracranial bleeds secondary to AVM rupture, the same has not been reported in children.
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19/70. Early childhood presentation of neurovascular disease in hereditary haemorrhagic telangiectasia.

    This paper describes an unusual case of hereditary haemorrhagic telangiectasia related cerebrovascular disease with an arteriovenous malformation and aneurysm presenting with intracranial haemorrhage in early childhood.
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keywords = haemorrhage
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20/70. Bilateral segmental regression of the carotid and vertebral arteries with rete compensation in a Western patient.

    We report a case of symptomatic symmetrical, bilateral absence of the cavernous internal carotid (ICA) and transdural vertebral artery (VA) segments with formation of a rete mirabile. There have been similar reports in Asian patients; ours is the first in the Western population. A 29- year-old woman presented with recurrent temporary blindness and an episode of minor subarachnoid haemorrhage. angiography demonstrated absence of the C5 and C6 segments of the ICA and the C1/2 segments of the VA bilaterally, with typical carotid rete mirabile formation to reconstitute the distal flow. Comparative anatomy and embryology show retia in several species and point to their secondary origin. The anomalies of the ICA cannot be called agenesis but rather absence since they are likely to result from perinatal disappearance of the artery rather its failure to develop.
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