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Fabry's disease is a rare, X linked recessive disease affecting 1 in 40 000 persons. The symptoms result from a lack of or a non-functioning enzyme α galactosidase, which leads to globotriaosylceramide accumulation in the walls of blood vessels. Mortality is generally from cardiac or renal complications and death from subarachnoid haemorrhage is distinctly rare. The authors report a man with Fabry's disease who died after subarachnoid haemorrhage from a progressively enlarging fusiform basilar aneurysm.

OBJECTIVEOccipitocervical fusions in the pediatric population are rare but can be challenging because of the smaller anatomy. The procedure is even more exacting in patients with prior suboccipital craniectomy. A proposed method for occipitocervical fusion in such patients is the use of occipital condyle screws. There is very limited literature evaluating the pediatric occipital condyle for screw placement. The authors examined the occipital condyle in pediatric patients to determine if there was an age cutoff at which condylar screw placement is contraindicated.METHODSThe authors performed a retrospective morphometric analysis of the occipital condyle in 518 pediatric patients aged 1 week–9 years old. Patients in their first decade of life whose occipital condyle was demonstrated on CT imaging in the period from 2009 to 2013 at the Augusta University Medical Center and Children’s Hospital of Georgia were eligible for inclusion in this study. Exclusion criteria were an age older than 10 years; traumatic, inflammatory, congenital, or neoplastic lesions of the occipital condyles; and any previous surgery of the occipitocervical junction. Descriptive statistical analysis was performed including calculation of the mean, standard deviation, and confidence intervals for all measurements. Probability values were calculated using the Student t-test with statistical significance determined by p < 0.05.RESULTSOverall, male patients had statistically significantly larger occipital condyles than the female patients, but this difference was not clinically significant. There was no significant difference in left versus right occipital condyles. There were statistically significant differences between age groups with a general trend toward older children having larger occipital condyles. Overall, 20.65% of all patients evaluated had at least one measurement that would prevent occipital condyle screw placement including at least one patient in every age group.CONCLUSIONSOccipital condyle screw fixation is feasible in pediatric patients younger than 10 years. More importantly, all pediatric patients should undergo critical evaluation of the occipital condyle in the axial, sagittal, and coronal planes preoperatively to determine individual suitability for occipital condyle screw placement.

: The neurosurgery service at the Medical College of Georgia, Georgia Regents University at Augusta has a rich history spanning almost 6 decades. Here, we review the development of neurological surgery as a specialty in Augusta and the history of the Department of Neurosurgery at Georgia Regents University. This article describes some of the early neurosurgeons in the city and those who have contributed to the field and helped to shape the department. Our functional and stereotactic program is emphasized. Our surgical epilepsy program dates back more than a half-century and remains a highly experienced program. We also describe our affiliation with the medical illustration graduate program, which was the first to be accredited and remains 1 of 4 such programs in the world. Finally, we list our alumni, former faculty, and current faculty, as well as the major accomplishments in our first decade as a full department.

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