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Familial intracranial ependymomas

Report of three cases in a family and review of the literature

Vassilios G. Dimopoulos, Kostas N. Fountas and Joe Sam Robinson

Familial cases of intracranial ependymomas have been well documented in the literature. The authors present two cases from a family in which three members harbored intracranial ependymomas. A 54-year-old man with fourth ventricular ependymoma underwent resection of the tumor followed by radiation therapy. His son presented at age 36 years with a fourth ventricular tanycytic ependymoma and underwent total resection of the ependymoma with postoperative radiation therapy. The father's sister had been treated at another institution for a posterior fossa ependymoma. The association of ependymomas with molecular genetic alterations in chromosome 22 has been previously described. Further investigation of the genetic influences may lead to better therapeutic approaches for this relatively rare clinicopathological entity.

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Cervical epidural hematoma in children: a rare clinical entity

Case report and review of the literature

Kostas N. Fountas, Eftychia Z. Kapsalaki and Joe Sam Robinson

✓ Pediatric spinal epidural hematoma is a very rare clinicopathological entity. In the vast majority of cases, spinal epidural hematomas have a nonspecific clinical presentation; this, along with their rapid progression, makes their early diagnosis and prompt surgical evacuation critical. Magnetic resonance imaging is the neuroimaging modality of choice, whereas hemilaminectomy or laminectomy is the indicated surgical intervention. The outcome is good when hematoma evacuation is performed before the onset of complete sensorimotor paralysis.

In this communication, the authors describe a 12-year-old girl with a traumatic acute cervical epidural hematoma. This lesion was successfully evacuated through a hemilaminectomy, and the patient had an excellent outcome. The pertinent literature is reviewed in terms of the incidence, origin, management, and prognosis of this rare and potentially disastrous clinical entity.

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Ioannis Karampelas, Angel N. Boev III, Kostas N. Fountas and Joe Sam Robinson Jr.

The authors offer a brief overview of early theories and treatments of sciatica. Tracing medical traditions through early Greek, Roman, and Eastern epochs, the authors demonstrate the slow sequential steps that were required to delineate this disease as a uniquely human affliction.

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Theofilos G. Machinis, Kostas N. Fountas, Vassilios Dimopoulos and Joe Sam Robinson

The purpose of this article is to provide insight into the development of surgery for acoustic neurinomas throughout the years. The significant contribution of surgical authorities such as Cushing, Dandy, and House are discussed. The advances in surgical techniques from the very first operations for acoustic tumors at the end of the 19th century until today are described, with special emphasis on the technological and diagnostic milestones that preceded each step of this development.

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Theofilos G. Machinis, Kostas N. Fountas, John Hudson, Joe Sam Robinson and E. Christopher Troup

Objective

Ventriculoatrial (VA) shunts remain a valid option for the treatment of hydrocephalus, especially in patients in whom ventriculoperitoneal (VP) shunts fail. Correct positioning of the distal end of the catheter in the right atrium is of paramount importance for maintaining shunt patency and reducing the incidence of VA shunt-associated morbidity. The authors present their experience with real-time transesophageal echocardiography (TEE) monitoring for the accurate placement of the distal catheter of a VA shunt.

Methods

Four patients underwent conversion of a VP shunt to a VA shunt under the guidance of intraoperative fluoroscopy and TEE between May 2003 and December 2004. After induction of general anesthesia, the TEE transducer was advanced into the esophagus. A cervical incision was made and the external jugular vein was visualized. An introducer was passed through an opening in the jugular vein and a guidewire was placed through the introducer. Under continuous TEE guidance, the guidewire was carefully advanced into the superior vena cava. A distal shunt catheter overlying a J-wire was then passed to the superior vena cava, again under TEE guidance. The catheter was advanced to the right atrium after removing the guidewire.

Final visualization with TEE and fluoroscopy revealed a good position of the catheter in the right atrium in all four cases. The mean duration of the operation was 91 minutes (range 65–120 minutes) and the mean operative blood loss was 23 ml (range 10–50 ml). No procedure-related complication was noted.

Conclusions

Real-time TEE is a safe and simple technique for the accurate placement of the distal catheter of a VA shunt.

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Kostas N. Fountas, Robert S. Donner, Leonidas G. Nikolakakos, Carlos H. Feltes, Ioannis Karampelas and Joe Sam Robinson Jr.

✓ The authors report a unique case of diffuse spinal metastatic disease due to a pleomorphic rhabdomyosarcoma (RMS) in an adult. In additon to its overall rarity, peculiar characteristics of the particular tumor included its site of origin, demonstrated radiologically as the lumbar paravertebral musculature (psoas muscle) and the transcanalicular spread into the vertebral canal, resulting in thecal compression at multiple levels. The salient clinicopathological characteristics of RMS, as they related particularly to the spine, are subsequently discussed and a short review of the major therapeutic modalities for these tumors is offered.

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Kostas N. Fountas, Anastasia Tasiou, Eftychia Z. Kapsalaki, Konstantinos N. Paterakis, Arthur A. Grigorian, Gregory P. Lee and Joe Sam Robinson Jr.

Object

Cerebral vasospasm is a common and potentially devastating complication of aneurysmal subarachnoid hemorrhage (aSAH). Inflammatory processes seem to play a major role in the pathogenesis of vasospasm. The Creactive protein (CRP) constitutes a highly sensitive inflammatory marker. The association of elevated systemic CRP and coronary vasospasm has been well established. Additionally, elevation of the serum CRP levels has been demonstrated in patients with aSAH. The purpose of the current study was to evaluate the possible relationship between elevated CRP levels in the serum and CSF and the development of vasospasm in patients with aSAH.

Methods

. A total of 41 adult patients in whom aSAH was diagnosed were included in the study. Their demographics, the admitting Glasgow Coma Scale (GCS) score, Hunt and Hess grade, Fisher grade, CT scans, digital subtraction angiography studies, and daily neurological examinations were recorded. Serial serum and CSF CRP measurements were obtained on Days 0, 1, 2, 3, 5, 7, and 9. All patients underwent either surgical or endovascular treatment within 48 hours of their admission. The outcome was evaluated using the Glasgow Outcome Scale and the modified Rankin Scale.

Results

The CRP levels in serum and CSF peaked on the 3rd postadmission day, and the CRP levels in CSF were always higher than the serum levels. Patients with lower admission GCS scores and higher Hunt and Hess and Fisher grades had statistically significantly higher levels of CRP in serum and CSF. Patients with angiographic vasospasm had higher CRP measurements in serum and CSF, in a statistically significant fashion (p < 0.0001). Additionally, patients with higher CRP levels in serum and CSF had less favorable outcome in this cohort.

Conclusions

Patients with aSAH who had high Hunt and Hess and Fisher grades and low GCS scores showed elevated CRP levels in their CSF and serum. Furthermore, patients developing angiographically proven vasospasm demonstrated significantly elevated CRP levels in serum and CSF, and increased CRP measurements were strongly associated with poor clinical outcome in this cohort.

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Vassilios Dimopoulos, Kostas N. Fountas, Theofilos G. Machinis, Carlos Feltes, Induk Chung, Kim Johnston, Joe Sam Robinson and Arthur Grigorian

Cauda equina syndrome is a well-documented complication of uneventful lumbar microdiscectomy. In the vast majority of cases, no radiological explanation can be obtained. In this paper, the authors report two cases of postoperative cauda equina syndrome in patients undergoing single-level de novo lumbar microdiscectomy in which intraoperative electrophysiological monitoring was used. In both patients, the amplitudes of cortical and subcortical intraoperative somatosensory evoked potentials (SSEPs) abruptly decreased during discectomy and foraminotomy. In the first patient, a slow, partial improvement of SSEPs was observed before the end of the operation, whereas no improvement was observed in the second patient. In the first case, clinical findings consistent with cauda equina syndrome were seen immediately postoperatively, whereas in the second one the symptoms developed within 1.5 hours after the procedure. Postoperative magnetic resonance images obtained in both patients, and a lumbar myelogram obtained in the second one revealed no signs of conus medullaris or nerve root compression. Both patients showed marked improvement after an intense course of rehabilitation. The authors' findings support the proposition that intraoperative SSEP monitoring may be useful in predicting the development of cauda equina syndrome in patients undergoing lumbar microdiscectomy. Nevertheless, further prospective clinical studies are necessary for validation of these findings.

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Carlos Feltes, Kostas N. Fountas, Theofilos Machinis, Leonidas G. Nikolakakos, Vassilios Dimopoulos, Rostislav Davydov, Mozaffar Kassam, Kim W. Johnston and Joe Sam Robinson

Object

Painful osteoporotic vertebral compression fractures (VCFs) are a significant cause of disability in the elderly population. Kyphoplasty, a recently developed minimally invasive procedure, has been advocated for the successful management of these fractures in terms of immediate pain relief, and also for restoration of the premorbid level of daily activities. In this retrospective study the authors report on their experience with the early management of VCFs with kyphoplasty.

Methods

A retrospective analysis was conducted in 13 patients (seven women and six men) whose ages ranged from 48 to 87 years (mean age 71.5 ± 11 years [mean ± standard deviation]). The interval between onset of symptoms and surgical intervention ranged from 4 to 9 weeks. Twenty levels (12 thoracic, eight lumbar) were treated in this cohort. Immediate and early postoperative (1-month follow-up visit) visual analog scale (VAS) pain scores, activity levels, and restoration of vertebral body (VB) height were assessed.

The mean preoperative VAS score was 8 ± 1, whereas the immediate and early postoperative scores were 1 ± 1. These findings reflected a resolution of 90 to 100% of preoperative pain. All patients resumed routine activities within hours of the procedure, although improvement in VB height was not accomplished in this cohort. No major complications were encountered in this clinical series.

Conclusions

Kyphoplasty is a safe and effective method for the treatment of osteoporotic VCFs. Failure to restore VB height does not seem to interfere with the excellent pain management and good functional outcome provided by this procedure.