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1/268. Posterior inferior cerebellar artery aneurysms associated with posterior fossa arteriovenous malformation: report of five cases and literature review.

    BACKGROUND: The association of posterior inferior cerebellar artery (pica) aneurysms with posterior fossa arteriovenous malformation (AVM) is uncommon. Over the past 3 years, five patients with this condition were treated at this institution. A review of the clinical history of these and other reported cases has illuminated common threads in the presentation, treatment, and outcome of these lesions. methods: The findings of 27 patients (5 from our institution and 22 from the medical literature) with pica aneurysms associated with AVMs were reviewed. RESULTS: Eighty-four percent of individuals presented with sub-arachnoid hemorrhage (SAH); 89% of these episodes resulted from aneurysm rupture documented by either intraoperative inspection or autopsy. All aneurysms were located on a feeding artery to the AVM, and 81% originated from distal portions of pica. The majority of patients presented with Hunt & Hess grade I SAH; all patients who presented with hemorrhage were treated surgically. Surgical strategy was directed both to secure the aneurysm and to resect the AVM during the course of a single procedure. Although four individuals either died on admission or in the perioperative period, overall outcome was excellent or good in 82% of patients. CONCLUSIONS: pica aneurysms associated with AVMs most often involve the distal segments of the artery. patients usually present with SAH secondary to aneurysmal rupture. Surgical clipping of the aneurysm and excision of the AVM is possible in a single procedure with minimal morbidity. overall prognosis is favorable in 80% of the cases.
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2/268. Ruptured aneurysm of the orbitofrontal artery associated with dural arteriovenous malformation in the anterior cranial fossa--case report.

    A 27-year-old male presented with a rare association of a ruptured orbitofrontal artery aneurysm and a dural arteriovenous malformation (DAVM) fed by both ethmoidal arteries, manifestation as severe headache, nausea, and vomiting. Computed tomography revealed a hematoma within the right frontal lobe and diffuse subarachnoid hemorrhage. The aneurysm was clipped successfully and the hematoma was evacuated. After an uneventful postoperative course, the patient was referred for gamma knife radiosurgery to treat the DAVM. In this case, the DAVM was asymptomatic and pathogenetically unrelated to the aneurysm, which demanded urgent treatment.
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3/268. Early rebleeding from intracranial dural arteriovenous fistulas: report of 20 cases and review of the literature.

    OBJECT: In this study the authors sought to estimate the frequency, seriousness, and delay of rebleeding in a homogeneous series of 20 patients whom they treated between May 1987 and May 1997 for arteriovenous fistulas (AVFs) that were revealed by intracranial hemorrhage (ICH). The natural history of intracranial dural AVFs remains obscure. In many studies attempts have been made to evaluate the risk of spontaneous hemorrhage, especially as a function of the pattern of venous drainage: a higher occurrence of bleeding was reported in AVFs with retrograde cortical venous drainage, with an overall estimated rate of 1.8% per year in the largest series in the literature. However, very few studies have been designed to establish the risk of rebleeding, an omission that the authors seek to remedy. methods: Presenting symptoms in the 20 patients (17 men and three women, mean age 54 years) were acute headache in 12 patients (60%), acute neurological deficit in eight (40%), loss of consciousness in five (25%), and generalized seizures in one (5%). Results of the clinical examination were normal in five patients and demonstrated a neurological deficit in 12 and coma in three. Computerized tomography scanning revealed intracranial bleeding in all cases (15 intraparenchymal hematomas, three subarachnoid hemorrhages, and two subdural hematomas). A diagnosis of AVF was made with the aid of angiographic studies in 19 patients, whereas it was a perioperative discovery in the remaining patient. There were 12 Type III and eight Type IV AVFs according to the revised classification of Djindjian and Merland, which meant that all AVFs in this study had retrograde cortical venous drainage. The mean duration between the first hemorrhage and treatment was 20 days. Seven patients (35%) presented with acute worsening during this delay due to radiologically proven early rebleeding. Treatment consisted of surgery alone in 10 patients, combined embolization and surgery in eight, embolization only in one, and stereotactic radiosurgery in one. Three patients died, one worsened, and in 16 (80%) neurological status improved, with 15 of 16 AVFs totally occluded on repeated angiographic studies (median follow up 10 months). CONCLUSIONS: The authors found that AVFs with retrograde cortical venous drainage present a high risk of early rebleeding (35% within 2 weeks after the first hemorrhage), with graver consequences than the first hemorrhage. They therefore advocate complete and early treatment in all cases of AVF with cortical venous drainage revealed by an ICH.
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4/268. An adult case of recurrent arteriovenous malformation after "complete" surgical excision: a case report.

    BACKGROUND: Complete surgical excision of arteriovenous malformations (AVM) documented by postoperative angiography is considered a cure. However, recent reports have shown that AVMs in children may recur after negative postoperative angiograms, and some suggest that it may reflect the immaturity of their cerebrovasculature. This case report demonstrates that AVM in adults may also recur, despite postoperative angiograms confirming complete removal. CASE DESCRIPTION: This 28-year-old man presented in 1994 with a focal motor seizure and was found to have an AVM in the right frontal lobe. He underwent surgical excision of the AVM; postoperative angiograms taken immediately after surgery and 15 days later showed no residual AVM. He remained free of symptoms after surgery and it was considered that a complete removal had been achieved. In 1998 he developed a subarachnoid hemorrhage, and subsequent angiograms revealed a small AVM in an adjacent location. CONCLUSION: This is the oldest patient reported in the literature with a recurrence of AVM, despite postoperative angiograms confirming complete removal. Angiographically invisible immature vessels, which might have been left in the surgical field, might have formed a new malformation later. We still believe that such recurrence must be very rare after AVM surgery, but we now recommend follow-up angiography at yearly intervals to our patients.
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5/268. Cerebral arteriovenous malformations and movement disorders.

    A series of six patients with movement disorders associated with cerebral arteriovenous malformations (AVM) is reported. The AVMs were classified according to the Spetzler-Martin classification as grade V (one patient), grade IV (four patients), and as grade III (one patient). One patient had action-induced hemidystonia caused by a contralateral frontoparietal AVM which compressed the putamen and was supplied partially by enlarged lenticulostriate arteries. Two patients presented with unilateral cortical tremor associated with contralateral high-frontal cortical/subcortical AVMs sparing the basal ganglia. Another patient developed hemidystonia and hemichorea-hemiballism after bleeding of a contralateral temporooccipital AVM and subsequent ischemia. Two patients had focal dystonia after thalamic and basal ganglia hemorrhage from AVMs. Five patients were operated on. The movement disorder was abolished in one patient postoperatively. Different mechanisms were identified that are relevant for the development of AVM-related movement disorders: mass effect, diaschisis, local parenchymal altered cerebral blood flow, and hemorrhagic or ischemic structural lesions.
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6/268. 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.
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7/268. Cerebrovascular biomodelling: a technical note.

    BACKGROUND: Recently computed tomographic angiography (CTA) and MR angiography (MRA) have been used to image cerebrovascular structures. Although CTA and MRA are accurate and sensitive imaging modalities, limitations have been identified in relation to image interpretation. Stereolithographic (SL) biomodelling is a new technology that allows three-dimensional (3D) CT and MR data to be used to accurately manufacture solid plastic replicas of anatomical structures. A prospective trial of SL biomodelling in cerebrovascular surgery has been performed to investigate the feasibility and clinical utility of this new display medium. methods: Fifteen patients with cerebral aneurysms and 1 patient with a cerebral arteriovenous malformation (AVM) were selected. 3D CT and/or MR angiograms were acquired and 19 solid anatomical biomodels manufactured using the rapid prototyping technology of stereolithography. The biomodels were used for patient education, diagnosis, operative planning and surgical navigation. RESULTS: The biomodels replicated the CTA and MRA source data. The accuracy of one biomodel was verified by comparison with a post mortem specimen, which corresponded exactly in the x and y planes but differed by 2 mm in the z plane. The ability to closely study an overview of complex cerebrovascular anatomy from any perspective on a solid biomodel was reported to enhance the surgeon's understanding, particularly when conventional images were equivocal. Cerebrovascular biomodels were found to be useful when positioning the patient's head for surgery, for selecting the best aneurysm clip and for the simulation of clipping. Patient informed consent was anecdotally improved. Disadvantages of the technology were the cost and manufacturing time. CONCLUSIONS: Cerebrovascular biomodelling may have utility in complex cases or when the standard imaging is felt to be equivocal.
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8/268. Intraoperative cardiac arrest in a neurosurgical patient: what are the options?

    Intraoperative cardiac arrest is uncommon. We describe a case of intraoperative cardiac arrest in a patient undergoing anesthesia for surgical repair of an intracranial arteriovenous malformation (AVM).
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9/268. Radiographic evidence and surgical confirmation of a saccular aneurysm on a hypoplastic duplicated A1 segment of the anterior cerebral artery: case report.

    OBJECTIVE AND IMPORTANCE: True duplication of the A1 segment of the anterior cerebral artery is extremely rare, as is finding a true A1 segment saccular aneurysm. We report the angiographic and surgical findings of such a case with the additional association of a hypoplastic ipsilateral M1 segment of the middle cerebral artery. CLINICAL PRESENTATION: A 68-year-old man presented with a Hunt and Hess Grade II subarachnoid hemorrhage and symptoms of headache, nuchal rigidity, and facial paresis. INTERVENTION: Angiographic evaluation with superselective exploration revealed a small ruptured aneurysm located on a duplicated hypoplastic A1 segment of the left anterior cerebral artery with associated middle cerebral artery stenosis and secondary early moyamoya changes. Surgical clipping of the aneurysm was performed successfully while sparing the hypoplastic A1 segment. CONCLUSION: A1 aneurysms occurring on a duplicated anterior cerebral artery segment probably develop from a congenital weakness of the parent vessel and increased local shear stress. Superselective angiography was helpful in the preoperative planning and facilitated the decision to treat with surgical clipping instead of embolization.
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10/268. Familial arteriovenous malformations in siblings.

    BACKGROUND: Familial arteriovenous malformations (AVMs) of the brain are rare. We present two sisters with the same parents who harbored AVMs that were successfully treated. methods: The elder sister presented with a unilateral migrainous type of headache overlying the right parietal area. The younger one suffered from exercise-induced headaches. Both were neurologically intact. magnetic resonance imaging scans of the brain and cerebral angiography delineated the lesions. Both sisters underwent endovascular embolization followed by surgical resection. RESULTS: Postoperatively, aside from a left inferior quadrantanopsia in the elder sister, both were neurologically intact. CONCLUSIONS: We report the rare occurrence of familial AVMs in two siblings and review the literature of 14 reports. No genetic predisposition was found.
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