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Wouter I. Schievink, David G. Piepgras, Franklin Earnest IV and Hymie Gordon

✓ Spontaneous bilateral carotid-cavernous fistulae and cervical artery dissection is reported in a 20-year-old woman with Ehlers-Danlos syndrome Type IV. The clinical features of 16 previously published cases of spontaneous carotid-cavernous fistulae associated with Ehlers-Danlos syndrome Type IV are reviewed, for a total of 17 cases. The mean age of the 14 women and three men was 31.6 years. Only direct fistulae were encountered. Diagnostic neuroangiography carried morbidity and mortality rates of 36% and 12%, respectively; neuroradiological treatment resulted in death in one of six patients. The possible value of desmopressin in the management of these patients is discussed. In view of the risks of arterial puncture and surgery, the authors emphasize the importance of early recognition of Ehlers-Danlos syndrome.

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Patrick J. Kelly, Bruce A. Kall, Stephan Goerss and Franklin Earnest IV

✓ Computer interpolation of stereotaxic computerized tomography (CT) scanning data allows the transposition of a tumor volume in stereotaxic space. A stereotaxically directed and computer-monitored CO2 laser is then utilized to vaporize that volume as the surgeon monitors the position of a cursor representing the laser beam against planar contours of the tumor displayed on an operating room computer monitor. Computer-assisted stereotaxic laser microsurgery provides precise three-dimensional control for aggressive resection of deep-seated tumors from neurologically important areas with acceptable postoperative results. Thus, a significant cytoreduction can be achieved in addition to providing a tissue diagnosis and internal decompression.

The authors report 83 computer-assisted stereotaxic laser procedures for tumor excision in 78 patients. The tumors were located in the thalamus/basal ganglia in 15 patients, ventricular system in five, corpus callosum in four, brain stem in three, and deep and centrally in the hemispheres in 51. Histologically, there were 26 glioblastomas, seven grade III astrocytomas, 14 grade II astrocytomas, 14 metastatic tumors, nine vascular lesions, and eight miscellaneous lesions. Resection of these subcortical lesions was confirmed by postoperative contrast-enhanced CT scanning. Neurological examinations performed 1 week after the 83 procedures revealed that 48 patients had improved from their preoperative level and 23 were unchanged (12 were neurologically normal preoperatively). Twelve patients had an increase in a preoperative neurological deficit, three of whom died in the postoperative period: one from infection, one from pulmonary emboli, and one from brain-stem edema.

The average survival period (37.6 weeks) of patients having glioblastomas treated by this technique and irradiation was no different from that of patients having glioblastomas in more favorable locations treated by conventional surgery and irradiation. Patients with circumscribed lower-grade astrocytomas did better in terms of morbidity and completeness of resection than those with infiltrative neoplasms. Other circumscribed lesions, such as metastatic tumors, vascular lesions, and intraventricular tumors, were easily resected by the technique described.

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S. V. Ramana Reddy, William E. Karnes, Franklin Earnest IV and Thoralf M. Sundt Jr.

✓ A case of spontaneous vertebral arteriovenous fistula in association with fibromuscular dysplasia is reported. The patient presented with progressive cervical myelopathy and cervical bruit. The pathogenesis of the fistula development and the spinal cord symptoms is discussed. Symptoms subsided after obliteration of the fistula.