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Harel Deutsch, George I. Jallo, Alina Faktorovich and Fred Epstein

Object. Improved neuroimaging techniques have led to an increase in the reported cases of intramedullary cavernomas. The purpose of this study was to define the spectrum of presenting signs and symptoms in patients with spinal intramedullary cavernomas and to analyze the role of surgery as a treatment for these lesions.

Methods. The authors reviewed the charts of 16 patients who underwent surgery for spinal intramedullary cavernomas. All patients underwent preoperative magnetic resonance imaging studies. Cavernomas represented 14 (5.0%) of 280 intramedullary lesions found in adults and two (1.1%) of 181 intramedullary lesions found in pediatric cases. A posterior laminectomy and surgical resection of the malformation were performed in all 16 patients.

Conclusions. Magnetic resonance imaging is virtually diagnostic for spinal cavernoma lesions. Patients with spinal intramedullary cavernomas presented with either an acute onset of neurological compromise or a slowly progressive neurological decline. Acute neurological decline occurs secondary to hemorrhage within the spinal cord. Chronic progressive myelopathy occurs due to microhemorrhages and the resulting gliotic reaction to hemorrhagic products. There is no evidence that cavernomas increase in size. The rate of rebleeding is unknown, but spinal cavernomas appear to be clinically more aggressive than cranial cavernomas, probably because the spinal cord is less tolerant of mass lesions. Complete surgical removal of the cavernoma was possible in 15 of 16 of the authors' cases.

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Juan C. Alzate, Karl F. Kothbauer, George I. Jallo and Fred J. Epstein

Object

The authors describe the results of performing a standard posterior craniovertebral decompression and placement, if indicated, of a syringosubarachnoid shunt for the treatment of patients with Chiari I malformation with and without syringomyelia.

Methods

This is a retrospectively analyzed consecutive series of 66 patients (mean patient age 15 years, range 1–53 years). The uniform posterior craniovertebral decompression consisted of a small suboccipital craniectomy, a C-1 laminectomy, microsurgical reduction of the cerebellar tonsils, and dural closure with a synthetic dural graft to increase the cerebrospinal fluid space at the craniocervical junction. The presence of a large syrinx, with significant thinning of the spinal cord tissue and obliteration of the spinal subarachnoid space, particularly when combined with syrinx-related symptoms, was an indication for the placement of a syringosubarachnoid shunt.

In 32 patients Chiari I malformation alone was present, and 34 in patients it was present in combination with syringomyelia. Clinical findings included pain, neurological deficits, and spinal deformity. The presence of syringomyelia was significantly associated with the presence of scoliosis (odds ratio 74.4 [95% confidence interval 8.894–622.4]).

All patients underwent a posterior craniovertebral decompression procedure. In 22 of the 34 patients with syringomyelia a syringosubarachnoid shunt was also placed. The mean follow-up period was 24 months (range 3–95 months). Excellent outcome was achieved in 54 patients (82%) and good outcome in 12 (18%). In no patient were symptoms unchanged or worse at follow-up examination, including four patients who initially required a second operation for persistent syringomyelia. Pain was more likely to resolve than sensory and motor deficits after decompressive surgery.

Radiological examination revealed normalization of tonsillar position in all patients. The syrinx had disappeared in 15 cases, was decreased in size in 17, and remained unchanged in two.

Conclusions

Posterior craniovertebral decompression and selective placement of a syringosubarachnoid shunt in patients with Chiari I malformation and syringomyelia is an effective and safe treatment. Primary placement of a shunt in the presence of a sufficiently large syrinx appears to be beneficial. The question of if and when to place a shunt, however, requires further, preferably prospective, investigation.

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Karl F. Kothbauer, George I. Jallo, Joao Siffert, Elpidio Jimenez, Jeffrey C. Allen and Fred J. Epstein

✓ Chemical agents routinely used in neurosurgery to achieve intraoperative hemostasis can cause a foreign body reaction, which appears on magnetic resonance (MR) images to be indistinguishable from recurrent tumor. Clinical and/or imaging evidence of progression of disease early after surgical resection or during aggressive treatment may actually be distinct features of granuloma in these circumstances.

A series of three cases was retrospectively analyzed for clinical, imaging, surgical, and pathological findings, and the consequences they held for further disease management.

All patients were boys (3, 3, and 6 years of age, respectively) and all harbored primitive neuroectodermal tumors. Two tumors were located in the posterior fossa and one was located in the right parietal lobe. Two boys exhibited clinical symptoms, which were unexpected under the circumstances and prompted new imaging studies. One patient was asymptomatic and imaging was performed at planned routine time intervals. The MR images revealed circumscribed, streaky enhancement in the resection cavity that was suggestive of recurrent disease. This occurred 2 to 7 months after the first surgery. At repeated surgery, the resected material had the macroscopic appearance of gelatin sponge in one case and firm scar tissue in the other cases. Histological analysis revealed foreign body granulomas in the resected material, with Gelfoam or Surgicel as the underlying cause. No recurrent tumor was found and the second surgery resulted in imaging-confirmed complete resection in all three patients. Because recurrent disease was absent, the patients continued to participate in their original treatment protocols. All patients remain free from disease 34, 32, and 19 months after the first operation, respectively.

During or after treatment for a central nervous system neoplasm, if unexpected clinical or imaging evidence of recurrence is found, a second-look operation may be necessary to determine the true nature of the findings. If the resection yields recurrent tumor, additional appropriate oncological treatment is warranted, but if a foreign body reaction is found, potentially harmful therapy can be withheld or postponed.

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Andleeb Khan, George I. Jallo, Ya J. Liu, Benjamin S. Carson Sr. and Michael Guarnieri

Object. The aim of this study was to investigate the optimal delivery rates of chemotherapy for the treatment of central nervous system tumors and to determine whether local delivery can lower toxicity profiles and increase target concentrations of chemotherapy.

Methods. The authors used two brain tumor models in rats. Slow (1 µl/hour) and fast (10 µl/hour) pumps were used to deliver chemotherapy—carboplatin, doxorubicin, and a high-molecular-weight transferrin-doxorubicin conjugate to the brains of normal rats and rats previously injected with F98 or 9L rat brain tumor cells. Brains were cut in 1-mm sections rostral and caudal from the infusion point. Slices were analyzed for doxorubicin and platinum by fluorescence and atomic absorption, respectively.

In the normal tissues, the volume of drug distribution is generally greater at the faster flow rate. In abnormal tissues, distribution is similar at slow and fast infusion rates for low-molecular-weight drugs and greater at slow rates for a high-molecular-weight targeted toxin.

Conclusions. After local administration the distribution of chemotherapy appears to be significantly influenced by tumor metabolism. Additional studies are needed to determine the optimal delivery rates for the interaction of the drug with the targeted tumor.

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Raj K. Shrivastava, Fred J. Epstein, Noel I. Perin, Kalmon D. Post and George I. Jallo

Object. Intramedullary spinal cord tumors (IMSCTs) in the older-age adult population pose complex management issues regarding the extent of resection and functional outcome, especially in terms of quality of life. Historically, IMSCTs in the older adult population were treated with irradiation alone because it was assumed that functional recovery would be poor. The authors examined their IMSCT database and report the first large series of IMSCTs in patients older than 50 years of age.

Methods. In this retrospective clinical and chart review there were 30 cases meeting inclusion criteria drawn from databases at three different institutions. A modified McCormick Scale was used to assess functional levels in all 30 patients pre- and postoperatively. The mean age of patients in this cohort was 59.8 years (range 50–78 years), and the mean follow- up period was 10.6 years (range 2–16 years).

Ependymoma was the most common tumor (83%), and 55% were located in the thoracic spine. The most common presenting symptom was sensory dysesthesia, with rare motor loss. The prodromal period to treatment was 19.4 months. Based on the McCormick Scale score at last follow-up examination 67% of patients were clinically functionally the same, 9% were worse, and 24% were improved after surgery. There were two deaths due tumor progression (both malignant tumors) and one recurrence (anaplastic astrocytoma). All three patients in whom malignant astrocytomas were diagnosed underwent postoperative radiation therapy.

Conclusions. In the population of patients older than age 50 years, thoracic ependymomas are the most common IMSCTs that present characteristically with sensory symptoms. The longer prodromal period in the older adult population may reflect the fact that their diagnosis and workup is inadequate. There was no significant increase in the length of stay in the neurosurgical ward. The authors recommend motor evoked potential-guided aggressive microsurgical resection, because the long-term outcome of benign lesions is excellent (good functional recovery and no tumor recurrence).

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Gary L. Gallia, Carolyn Moore, Lori Jordan, Philippe Gailloud and George I. Jallo

✓Neonatal intracranial aneurysms are rare. The authors report the case of a 4-week-old girl who presented with left-eye ptosis and proptosis. Computerized tomography scanning and magnetic resonance imaging demonstrated a mass involving the left cavernous sinus and middle cranial fossa. Cerebral angiography revealed a large complex left cavernous carotid artery (CA) aneurysm. The patient underwent endovascular treatment in which detachable coils and n-butyl cyanoacrylate glue were used to achieve complete obliteration of the aneurysm. To the authors' knowledge, this is the first reported neonatal intracranial aneurysm originating from the cavernous CA and treated endovascularly. The authors review the literature on neonatal intracranial aneurysms.

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Khan W. Li, Chanland Roonprapunt, Herman C. Lawson, I. Rick Abbott, Jeffrey Wisoff, Fred Epstein and George I. Jallo

Object

Tectal gliomas are a distinct form of pediatric brainstem tumor that present in patients with symptoms related to increased intracranial pressure due to obstructive hydrocephalus. The natural history of these lesions is often uniquely indolent. Thus, initial surgical therapies are directed at treatment of hydrocephalus, usually with ventricular shunt placement. Recently, third ventriculostomy has been used in patients with tectal gliomas, both as an initial procedure and after shunt failures. In this report the authors review their experience with the treatment of hydrocephalus in patients with tectal gliomas.

Methods

The authors reviewed 31 consecutive cases of tectal gliomas and compared the success rates of ventricular shunt placement with the success rates of endoscopic third ventriculostomy (ETV). Shunt placement procedures were associated with a significant number of malfunctions, and most patients required shunt revisions. The ETV procedure was attempted both as an initial treatment and after shunt malfunction. Overall, ETV was attempted in 18 patients and was performed successfully in all cases. At the time of follow-up evaluation, 16 patients (89%) were shunt free.

Conclusions

The authors found that ETV could be performed with good long-term success both as an initial treatment and after shunt failure. Overall, ETV was found to be superior to ventricular shunt placement in the management of hydrocephalus associated with tectal gliomas.

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James Lee, George I. Jallo, Michael Guarnieri, Benjamin S. Carson Sr. and Margret B. Penno

Object

Survival rates for high-grade brainstem tumors are approximately 10% and optimal therapy has yet to be determined. Development of a satisfactory brainstem tumor model is necessary for testing new therapeutic paradigms that may prolong survival. The authors report the technique, functional progression, radiological appearance, and histopathological features of a novel brainstem tumor model in rats.

Methods

Thirty female Fischer 344 rats were randomized (10 animals/group) to receive an injection of either 3 μl of 9L gliosarcoma cells (100,000 cells), 3 μl of F98 glioma cells (100,000 cells), or 3 μl of medium (Dulbecco modified Eagle medium) into the pontine tegmentum of the brainstem. Using a cannulated guide screw system implanted in the skull of the animal, rats in each group were injected at coordinates 1.4 mm to the right of the sagittal and 1 mm anterior to the lambdoid sutures, at a depth of 7 mm from the dura mater. The angle of the syringe during injection was anteflexed 5° from the vertical. Postoperatively, the rats were evaluated for neurological deficits by using an automated rotarod test. High-resolution [18F]fluorodeoxyglucose–positron emission tomography (FDG-PET) fused with computerized tomography (CT) scans were acquired pre- and postoperatively through the onset of hemiparesis and correlated accordingly. Kaplan–Meier curves were generated for survival and disease progression, and brains were processed postmortem for histopathological investigation.

The 9L and F98 tumor cells grew in 95% of the animals in which they were injected and resulted in a statistically significant mean onset of hemiparesis of 16.5 ± 0.56 days (p = 0.001, log-rank test), compared with animals in the control group, which had no neurological deficits by Day 45. The FDG-PET studies coregistered with CT scans demonstrated space-occupying brainstem lesions, and this finding was confirmed by histological studies. Animals in the control group showed no functional, radiological, or pathological signs of tumor.

Conclusions

Progression to hemiparesis was consistent in all tumor-injected animals, with predictable onset of symptoms occurring approximately 17 days postsurgery. The histopathological and radiological characteristics of the 9L and F98 brainstem tumors were comparable to those of aggressive primary human brainstem tumors. Establishment of this animal tumor model will facilitate the testing of new therapeutic paradigms for the treatment of these lesions.

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George I. Jallo, Ian Suk and László Bognár

✓Many subfrontal and orbitofrontal craniotomy techniques have been proposed and developed for anterior cranial fossa lesions. The purpose of this study was to evaluate the surgical experience with the frontolateral keyhole craniotomy through a superciliary skin incision in children.

The keyhole craniotomy is a modification of the traditional pterional approach. This modified approach, a craniotomy with a 2.5 × 3—cm bone opening just above the eyebrow through a superciliary incision, has been previously described in adults for many lesions situated in the anterior cranial fossa, including tumors and aneurysms. The authors review their experience in using this approach in 27 children for a variety of intracranial lesions.

This approach was used for 28 procedures in children ranging in age from 1 to 16 years (mean age 10 years). The lesions included arachnoid cysts, cerebrospinal fluid fistulas, and tumors; no vascular lesions were treated.

The authors have found this craniotomy to be a safe and simple approach for treating anterior cranial fossa and suprasellar lesions in children.

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Daniel M. Sciubba, R. Morgan Stuart, Matthew J. McGirt, Graeme F. Woodworth, Amer Samdani, Benjamin Carson and George I. Jallo

Object

The majority of shunt infections occur within 6 months of shunt placement and chiefly result from perioperative colonization of shunt components by skin flora. Antibiotic-impregnated shunt (AIS) systems have been designed to prevent such colonization. In this study, the authors evaluate the incidence of shunt infection after introduction of an AIS system in a population of children with hydrocephalus.

Methods

The authors retrospectively reviewed all pediatric patients who had undergone cerebrospinal fluid (CSF) shunt insertion at their institution over a 3-year period between April 2001 and March 2004. During the 18 months prior to October 2002, all CSF shunts included standard, nonimpregnated catheters. During the 18 months after October 2002, all CSF shunts included antibiotic-impregnated catheters. All patients were followed up for 6 months after shunt surgery, and all shunt-related complications, including shunt infection, were evaluated. The independent association of AIS catheter use with subsequent shunt infection was assessed via multivariate proportional hazards regression analysis.

A total of 211 pediatric patients underwent 353 shunt placement procedures. In the 18 months prior to October 2002, 208 (59%) shunts were placed with nonimpregnated catheters; 145 (41%) shunts were placed with AIS catheters in the 18 months after October 2002. Of patients with nonimpregnated catheters, 25 (12%) experienced shunt infection, whereas only two patients (1.4%) with antibiotic-impregnated catheters experienced shunt infection within the 6-month follow-up period (p < 0.01). Adjusting for intercohort differences via multivariate analysis, AIS catheters were independently associated with a 2.4-fold decreased likelihood of shunt infection.

Conclusions

The AIS catheter significantly reduced incidence of CSF shunt infection in children with hydrocephalus during the early postoperative period (< 6 months). The AIS system used is an effective instrument to prevent perioperative colonization of CSF shunt components.