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Kee D. Kim, Jesse D. Babbitz and Jeffrey Mimbs

The surgical management of thoracic disc disease remains challenging. Outcomes after laminectomy had been poor, and modern posterolateral, lateral, and anterior approaches have evolved to replace this older procedure. Each has its own set of complications, and all are hampered, to varying degrees, by the limited visualization of the ventral disc space and spinal cord during decompression. The authors present their early experience with computer-assisted image guidance as an adjunctive tool for preoperative planning and navigation in the treatment of thoracic disc disease. Five consecutive patients underwent image-guided costotransversectomies between January 1999 and April 2000. The levels of herniation were T8–9 in three and T7–8 and T5–6, respectively, in the other two. There were four centrolateral herniations and one midline herniation. Three discs were soft and two hard. Two patients had previously undergone failed disc excisions. All patients had axial pain and myeloradiculopathies preoperatively. Three were unable to walk.

Four patients enjoyed good or excellent outcomes, with return of ambulation. One patient experienced only mild improvement in her severe paraparesis. Image-guidance was invaluable in planning the corpectomy and aiding visualization in situations in which the dura or disc were obscured; its use allowed successful surgical excisions in the most challenging circumstances.

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Jeffrey C. Allen, Jae Ho Kim and Roger J. Packer

✓ A neoadjuvant (preradiotherapy) chemotherapy regimen consisting of either cyclophosphamide alone (60 to 80 mg/kg) or a modified multidrug regimen (vinblastine, bleomycin, cyclophosphamide, and cisplatin) was administered to 15 newly diagnosed patients with histologically confirmed, fully staged, primary germ-cell tumors (GCT's) of the central nervous system (CNS). There were 11 patients with germinomas and four with non-germinoma malignant GCT's. There were six females and nine males, whose median age was 13 years (range 4 months to 24 years). Seven germinoma patients (64%) had disseminated disease. For the germinoma patients, the subsequent radiotherapy dose was modified based on the response to the neoadjuvant chemotherapy, and craniospinal radiotherapy was given only to those with disseminated CNS disease at diagnosis. Ten of the 11 germinoma patients had complete disappearance of all evaluable disease after two courses of chemotherapy (cyclophosphamide in eight and multidrug in three) and one had a partial response. The planned dose of radiotherapy to the primary tumor was reduced from 5500 to 3000 rads, and the craniospinal dose was lowered from 3600 to 2000 rads. Ten patients remain in continuous disease-free remission 20+ to 89+ months after diagnosis (median follow-up period 47 months). All four patients with non-germinoma GCT's received the multidrug regimen, and two of three patients with evaluable disease had a partial response. High-dose regional and craniospinal radiotherapy was administered thereafter, but only two patients remain in their first remission.

Previously untreated germinoma is a highly chemosensitive disease and the neoadjuvant treatment strategy permits the identification of active chemotherapy regimens in newly diagnosed patients. Patients who have complete responses to neoadjuvant chemotherapy tolerate a significant radiotherapy dose reduction without compromising long-term survival, thereby allowing a reduction of some of the late effects of therapeutic radiation. Germinomas tend to disseminate early in the course of the disease and a pre-therapy staging evaluation permits individualized radiotherapy treatment planning.

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Nicholas D. Coppa, H. Jeffrey Kim and Kevin M. McGrail

✓ The spontaneous resolution of syringomyelia in the setting of a Chiari malformation Type I (CM-I) has been reported infrequently. Several theories about the pathogenesis and spontaneous resolution of syringomyelia associated with CM-I have been proposed. The authors present the case of a patient with spontaneous resolution of a CM-I and syringomyelia coinciding with the development of cerebrospinal fluid (CSF) otorrhea. Although cases of spontaneous resolution of syringomyelia have been reported, this is the first reported case of spontaneous resolution of syringomyelia and a CM-I associated with the simultaneous development of CSF otorrhea.

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Jeffrey G. Ojemann, Robert L. Grubb, Michael Kyriakos and Kim B. Baker

✓ This 52-year-old woman developed crystal deposition disease involving the cervical vertebrae. She presented with symptomatic spinal cord compression secondary to extensive calcified lesions in the posterior elements of the cervical spine. Surgical decompression with posterior fusion was performed. Histological examination showed hardened deposits of calcium carbonate involving the soft tissue, and dissolution of the vertebral bone trabeculae. There was no inflammatory response to these deposits. One year postoperatively the patient developed severe pulmonary disease associated with the collagen-vascular disorder, scleroderma (calcinosis, Raynaud's phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia [CREST] syndrome). Calcium carbonate deposition disease represents an unusual clinical entity that is possibly associated with scleroderma or other collagen-vascular diseases, and it is distinct from ligamentum flavum calcification, calcium pyrophosphate deposition disease, and hydroxyapatite deposition disease.

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Louis J. Kim, Volker K. H. Sonntag, Jonathan T. Hott, Jeffrey A. Nemeth, Jeffrey D. Klopfenstein and Lisa Tweardy

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Louis J. Kim, Harold L. Rekate, Jeffrey D. Klopfenstein and Volker K. H. Sonntag

Object. The reduction of basilar invagination associated with Chiari I malformations in pediatric patients is often possible. Eleven children ranging in age from 1.5 to 17 years underwent a novel treatment method involving decompression, manual reduction, and posterior instrumentation-augmented fusion. Outcomes were evaluated retrospectively.

Methods. After decompression of the lesion, manual craniocervical distraction and extension were performed, followed by posterior occipitocervical fusion involving rigid internal fixation. All patients were symptomatic at presentation. Intraoperative monitoring included somatosensory evoked potentials, fluoroscopy, and direct intradural demonstration of the craniocervical junction. No new neurological deficits occurred immediately after surgery. Perioperatively, symptoms in seven patients improved significantly and in four they remained unchanged. Three patients required subsequent transoral resections. During long-term follow up (mean 39.4 months, range 3–92 months) symptoms improved markedly in nine, remained unchanged in one, and progressively worsened in one patient.

Conclusions. In selected cases, manual cervical distraction and extension, posterior fixation, and fusion appear to provide a safe, effective standalone treatment for basilar invagination associated with Chiari malformation in children.

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Hyunmi Kim, Catherine Lee, Robert Knowlton, Curtis Rozzelle and Jeffrey P. Blount

Object

Depth electrodes provide a better sampling of sulci and regions of cortex that lie tangential to the plane of subdural electrodes. The aim of this study was to evaluate the utility of supplemental depth electrodes in the surgical treatment of pediatric patients with neocortical epilepsy.

Methods

Cases involving 12 consecutive pediatric patients (mean age [SD] 10.9 ± 4.4 years) were reviewed. Focal resective surgery (in 9 cases) or functional hemispherectomy (in 3 cases) was performed after intracranial monitoring. The mean total number of electrodes was 118 ± 29; the mean numbers of grid, strip, and depth electrodes were 95 ± 27, 10 ± 6, and 13 ± 5, respectively The most common pathological condition was focal cortical dysplasia.

Results

In 4 cases, depth electrodes demonstrated the ictal onset zone in an area not easily accessible by grids or strips (in the basal temporal region in 3 cases and the upper opercular region in 1 case). In 3 of these 4 cases, the ictal onset zone was defined exclusively by the depth electrodes. In each of these 3 cases, the surface electrodes (on grids or strips) demonstrated early propagation but not ictal onset. In 9 cases, the depth electrodes also demonstrated the early propagation zone. The information about the ictal onset zone and the early propagation zone helped to provide additional information that affected the extent (in 7 cases) or depth (in 3 cases) of the resection. The proportion of the electrodes involved in resection relative to the total number of implanted electrodes was low (mean ± SD, 0.26 ± 0.09). Nine patients (75.0%) became seizure free (Engel class IA outcome) after surgery (mean duration of follow-up 25.7 ± 4.29 months). There were no surgical complications related to intracranial electroencephalography monitoring.

Conclusions

Most patients (75.0%) became seizure free after extensive monitoring and more limited resection of seizure-onset regions. Supplemental depth electrodes contribute to improved outcome by providing information about the ictal onset zone that is not accessible by grid or strip electrodes in some cases. The supplemental depth electrodes conferred an extra dimension of depth to the analysis, which allowed for successful outcome with more limited resection.

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Kim Kramer, Heather J. McCrea, Cheryl Fischer and Jeffrey P. Greenfield

Successful delivery of intraventricular radioimmunotherapy is contingent on adequate CSF flow. The authors present a patient with medulloblastoma in whom obstructed CSF flow was causing hydrocephalus, which was initially corrected by implantation of a programmable shunting device. While managing the hydrocephalus, an endoscopic third ventriculostomy (ETV) needed to be performed in a collapsed ventricular system to ensure adequate radioimmunotherapy distribution.

This 18-month-old patient with medulloblastoma involving leptomeningeal dissemination presented for intraventricular radioimmunotherapy. A CSF 111In-DTPA scintigraphy study obtained through the existing programmable ventriculoperitoneal shunt demonstrated activity in the lateral and third ventricles, but no activity over the cerebral convexities or spinal canal, consistent with obstruction at the level of the cerebral aqueduct. By maximization of ventricular size in a controlled setting, the patient was able to undergo a trial of ETV through very small ventricles. A postoperative CINE MR imaging study confirmed patent ETV. The pressure settings on the shunt were kept at the highest opening pressure (200 mm H2O) to maximize flow through the stoma and improve the distribution of CSF throughout the subarachnoid space. The CSF flow scintigraphy study was again performed, this time with tracer activity demonstrated down the thecal sac at 3 hours, and symmetrically over the cerebral convexities at 24 hours. The patient began weekly intraventricular administration of 131I-3F8 therapy.

Successful rerouting of CSF flow for the purpose of therapeutic radioisotope administration is possible. Endoscopic third ventriculostomy can be considered in patients with programmable shunting devices; normal or slit ventricles do not preclude successful ETV.

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Young-Baeg Kim, Jeffrey M. Gidday, Ernesto R. Gonzales, Aarti R. Shah and T. S. Park

✓ Hypoglycemia increases the vulnerability of the perinatal brain to asphyxia, but it is not known if hypoglycemia-induced changes in cerebral hemodynamics and vascular reactivity underlie this vulnerability. This study tested the hypothesis that hypoglycemia exacerbates postischemic hypoperfusion, and impairs postischemic CO2 reactivity. The authors also examined the hypothesis that postischemic hypoperfusion is associated with a reduction in the interstitial concentration of the vasodilator metabolite adenosine. Global cerebral ischemia of 10 minutes duration was induced in newborn pigs anesthetized with isoflurane by occlusion of subclavian and brachiocephalic arteries; cortical cerebral blood flow (CBF) and interstitial adenosine concentration were evaluated simultaneously using the combined hydrogen clearance/microdialysis technique. Hypoglycemia (blood glucose < 25 mg/dl) was induced by regular insulin (25 IU/kg) administered intravenously 2 hours prior to induction of ischemia. In the eight normoglycemic animals, baseline CBF was 38 ± 4 ml/min/100 gm and baseline adenosine concentration was 1.2 ± 0.1 µM; in the eight hypoglycemic animals, these values were 39% (p < 0.05) and 62% (p < 0.05) greater, respectively, under baseline conditions. At 1 hour of postischemic reperfusion in normoglycemic animals, CBF was reduced 39% relative to the preischemic baseline (p < 0.01), concomitant with a 27% reduction (p < 0.05) in adenosine concentration, suggesting that this lowered concentration may underlie delayed hypoperfusion.

These postischemic reductions in CBF and interstitial adenosine concentration were significantly greater in hypoglycemic animals, with CBF and adenosine concentration reduced 70% (p < 0.001) and 71% (p < 0.01), respectively, relative to baseline. In nine animals preischemic reactivity to hypercapnia was unaffected by hypoglycemia. Postischemic hypercapnic reactivity was retained in the eight normoglycemic animals, but was attenuated 73% (p < 0.05) in hypoglycemic animals. Thus, in the newborn pig, hypoglycemia exacerbates postischemic cortical hypoperfusion and impairs postischemic cerebrovascular reactivity to hypercapnia.