Surgical challenges in the management of cervical kyphotic deformity in patients with severe osteoporosis: an illustrative case of a patient with Hajdu-Cheney syndrome.

PURPOSE: No standard strategy exists for the management of cervical kyphotic deformity in patients with severe osteoporosis. In fact, in such subpopulation, standard algorithms commonly used in patients with normal bone mineral density may not be applicable. In this Grand Rounds, the authors present a challenging case of a patient with Hajdu-Cheney syndrome, a rare disorder of bone metabolism induced by a Notch-2 mutation, who presented with cervical kyphotic deformity and severe osteoporosis. METHODS: A 65-year-old female patient with a previous diagnosis of Hajdu-Cheney syndrome presented with cervical myelopathy and cervical kyphotic deformity. The initial MRi demonstrated multilevel cervical canal stenosis. The CT-scan also revealed marked spondylolisthesis of C6 over C7 as well as numerous laminar and pedicle fractures, resulting in a cervical kyphosis of approximately 50 degrees. RESULTS: The patient was submitted to 360-degree decompression and fusion of the cervical spine consisting of a staged C6 anterior corpectomy and multilevel microdiscectomies with wide opening of the posterior longitudinal ligament in order to provide a satisfactory release of anterior spinal structures, followed by 24 h of cervical halo-traction, a second anterior approach for bone graft implantation in the site of the corpectomy as well as insertion of allografts and completion of the ACDF C2-T1 and plating, and, finally, a posterior C2-T3 pedicle screw instrumentation using intra-operative CT-scan (O-arm) navigation guidance. CONCLUSIONS: This case illustrates some intra-operative nuances as well as specific surgical recommendations for cervical deformity surgery in patients with severe osteoporosis, such as avoidance of Caspar pins for interbody distraction, use of intra-operative fluoroscopy for achievement of bicortical purchase of anterior cervical screws and placement of pedicle screws during posterior instrumentation. Moreover, such illustrative case demonstrates that, in the subpopulation of patients with severe osteoporosis, it may be possible to successfully apply cervical distraction after an isolated anterior approach with a satisfactory improvement in the cervical alignment, possibly avoiding more laborious 540-degree approaches such as the previously described back-front-back or front-back-front surgical algorithms.

Can a closed carotid artery be reopened?

Carotid occlusion is a disease that presents a difficult decision for the treating provider. Traditionally, many providers would opt for expectant management with risk factor reduction and supportive therapy. There is a growing body of literature however pointing to possible improved outcomes of more aggressive treatments, including reopening of the occluded carotid. In this review, we discuss the difficulties involved in diagnosing a patient presenting with symptomatic carotid occlusion, the natural history of the disease, and the emerging treatment options and paradigms of different institutions based on recent literature.

Cortical vision loss as a prominent feature of H1N1 encephalopathy.

A 20-year-old woman infected with the 2009 H1N1 strain of influenza A developed bilateral visual loss. Brain MRI showed restricted diffusion of the parietal and occipital lobes, and her spinal fluid did not contain inflammatory cells. This report describes an unusual case of H1N1 influenza A virus infection primarily affecting the posterior visual pathways.

Computer-aided volumetric analysis as a sensitive tool for the management of incidental meningiomas.

INTRODUCTION: Meningiomas are typically slow-growing lesions that, depending on the location, can be relatively benign. Knowing their exact rate of growth can be helpful in determining whether surgery is necessary. METHODS: In this study we retrospectively reviewed the meningioma practices of the two senior authors (JR, MR). Our goal was to measure meningioma growth using a variety of methods (linear using diameters, and volumetric using the computer-aided perimeter and cross-sectional diameter methods) to compare rates of growth among the methods. Of 295 meningioma patients seen over an 8-year period, we identified a cohort of 31 patients with at least 30 months of follow-up. Volumes were calculated using medical imaging software with T1 post-contrast magnetic resonance imaging. Doubling times and growth rates were calculated. RESULTS: Of the 31 patients, 26 (84%) were shown to have growing meningiomas. The perimeter methodology measured higher growth rates than the diameter method for both doubling times as well as percentage annual growth (p<0.01). The mean doubling time was 13.4 years (range, 2.1­72.8 years) and 17.9 years (range, 4­92.3 years) comparing perimeter and diameter methods, respectively. The mean percentage of annual growth was 15.2% (range, 1.8­61.7%) and 5.6% (range, 0.7­12.2%), comparing perimeter and diameter methods, respectively. Linear growth was calculated at 0.7 mm/year. CONCLUSION: Overall, we found that computer-aided perimeter methods showed a more accurate picture of tumor progression than traditional methods, which generally underestimated growth.

Glioblastoma formation in a recurrent intracranial epidermoid cyst: a case report.

BACKGROUND: Transformation to glioblastoma following recurrent epidermoid cyst resection has not been reported. Chronic inflammation can underlie malignant transformation of epidermoid cysts. Astrogliosis following repeated resections may have induced the rare transformation to glioblastoma. CLINICAL PRESENTATION: A patient presenting with left lower extremity weakness was found to harbor a parietal mass lesion. Histopathology demonstrated an epidermoid cyst. Following multiple re-resections, an intra-axial mass was discovered within the operative bed, confirmed as glioblastoma. CONCLUSION: This is the first report of glioblastoma associated with a resected epidermoid cyst. Subsequent to resection, the chronic inflammatory milieu propagated by astrogliosis is thought to have induced malignancy. The progression to glioblastoma draws attention to neoplastic transformation in the context of recurrent epidermoids.