Search

WE HAVE IMPROVED upon a brain stem mapping technique that can be used to locate cranial motor nuclei on the floor of the fourth ventricle. This technique helped to intraoperatively locate the facial colliculus and the motor nuclei of cranial nerves IX/X and XII in 14 patients undergoing removal of brain stem tumors. The motor nuclei of these cranial nerves are usually located relative to specific anatomic landmarks on the ventricular floor. These landmarks were not evident in most patients studied because of the distorting effects of the tumor. Different points of the floor of the fourth ventricle were electrically stimulated while electromyographic responses were recorded with electrodes inserted in the orbicularis oculi and orbicularis oris muscles, the lateral posterior pharyngeal wall, and the intrinsic muscles of the tongue. Mapping was performed before and after tumor resection. The technique was found to be useful for locating cranial motor nuclei before tumor resection. It enabled surgeons to avoid damaging the nuclei when entering the brain stem. This technique, however, has certain limitations. Because this is a mapping technique, not a monitoring technique, continuous monitoring during tumor resection was unavailable. Also, the presence of a muscle response after tumor resection did not always indicate preservation of immediate postoperative function (7 days after surgery) because damage to the corticobulbar tract and/or the underlying neural circuitry was not detectable by mapping. Further research is needed to determine the point of stimulation in the functional motor circuitry and the relationship between intraoperative recordings and postoperative function in the long term.

✓ Spinal cord lipomas are rare lesions, accounting for approximately 1% of all spinal cord tumors. True intramedullary spinal cord lipomas are extremely rare and are represented in the literature as scattered, single case reports. The authors present a series of six patients with intramedullary spinal cord lipomas managed at our institution from July, 1985 to July, 1993. The patients' ages ranged from 8 to 45 years. Four patients presented with newly diagnosed tumors and two had undergone previous surgery. Patients usually presented with long histories of disability followed by rapid progression of their symptoms. Most patients were in poor neurological condition on presentation. Presenting symptoms included spinal pain, dysesthetic sensory changes, gait difficulties, weakness, and incontinence. Three patients had cervical tumors, two had cervicothoracic tumors, and one patient had a thoracic tumor. Diagnostic studies, including magnetic resonance imaging, were obtained in all patients. No patient exhibited any form of spinal dysraphism or had a dural defect. All patients underwent decompressive, subtotal resections of 40% to 70% of their lesions. Follow-up times ranged from 12 to 96 months. All patients had resolution of their pain, but they generally showed no neurological improvement. As of their most recent follow-up visit, none of the patients was neurologically normal; three can function independently, although with neurological deficits. The other three patients cannot function independently and have severe neurological deficits. The authors conclude that patients with intramedullary spinal cord lipoma who present with significant neurological compromise have a very poor prognosis with regard to neurological function and generally show no improvement with surgical resection.

Fourth-ventricular shunting is commonly used to treat symptomatic posterior fossa cysts of the Dandy-Walker malformation and trapped fourth ventricle. Although the benefits of this procedure have been widely reported, there is a paucity of data on the pitfalls of posterior fossa shunting in the neurosurgical literature. During the 4-year period from July 1989 to June 1993, we placed fourth-ventricular shunts in 12 patients. Remarkably, 5 patients suffered complications related to posterior fossa catheter placement (42% rate). Three of these patients developed new cranial nerve dysfunction caused by direct injury to the floor of the fourth ventricle, 1 patient suffered an intracystic hemorrhage and acute shunt malfunction, and 1 patient had the catheter tip in the brainstem on postoperative studies without new neurological deficit. We conclude that placement of fourth-ventricular shunts can be fraught with complications which we believe is related to technique. We propose that altering the trajectory of the ventricular catheter from our usual midline technique to a more lateral position will lessen the chances for injury to the floor of the fourth ventricle. In this manner we hope to decrease our incidence of complications for this procedure.

Positioned anatomically between the spinal epidural space and the intramedullary compartment, the spinal subdural space remains the least common area of localized infection in the central nervous system. Infectious processes of the subdural spinal space include subdural spinal empyema, subdural spinal abscess, infected spinal subdural cyst, and infectious spinal subdural cyst. To date, there has been no systematic review of these entities in children, with the cumulative knowledge of the pathophysiologic, microbiologic, and demographic characteristics of these infections relegated solely to few small series and case reports. A series of 11 recent cases culled from the collaboration of international authors are presented. In addition, an exhaustive MEDLINE search and manual review of the international literature was performed, identifying a total of 73 cases of spinal subdural infections in patients under the age of 21. Data of interest include the age, sex, signs, and symptoms at presentation, spinal location of infection, presence of spinal dysraphism, and other comorbidities, offending organism, treatment, outcome, and follow-up. Patients ages ranged from 4 weeks to 20 years (mean, 6.5 years). Males outnumbered females by a ratio of 2:1. Over half (53 %) of spinal subdural infections in children were associated with spinal dysraphism or other congenital abnormalities of the spine. The commonest organism to infect the spinal subdural space in children is mycobacterium tuberculosis and the thoracic spinal region was most commonly infected. The disease is usually treated surgically, although a more expectant approach consisting of antibiotics and observation has also been proposed.

On December 5, 1960, 4-month-old Theo Dahl, the only son of best-selling author Roald Dahl (1916-1990), had his skull shattered in a horrific traffic accident. What began as a personal tragedy for the Dahl family would soon evolve into a dogged crusade by Dahl to expand upon preexisting valve technology with the goal of developing a shunt that would not become obstructed. Based upon exclusive access to private archives of the Dahl estate, as well as interviews with those involved, this article tells the intricate tale of one famous father's drive to significantly alter the natural history of pediatric hydrocephalus. Dahl's collaboration with British toymaker Stanley Wade and pioneering pediatric neurosurgeons Joseph Ransohoff, Kenneth Shulman, and Kenneth Till to create the Wade-Dahl-Till (WDT) valve is examined in detail. The ensuing rift between the American and British contingents, the valve's multiple design revisions, and the goal of creating an affordable shunt for children in developing countries are among the issues addressed. The development of the WDT valve marked a significant turning point in the surgical management of pediatric hydrocephalus in general and in shunt valve technology in particular.

The authors treated a 14-year-old girl with posthemorrhagic hydrocephalus and an infected ventriculopleural shunt. She was taken for endoscopic third ventriculostomy with the hope of removing the hardware entirely, but she developed an arrhythmia and hypertension, followed rapidly by complete cardiac arrest. She was resuscitated successfully, made a complete recovery, and subsequently underwent a conventional shunt replacement. This is a newly reported life-threatening complication of neuroendoscopy that must be anticipated by any surgeon undertaking such a procedure

Patients with medulloblastoma frequently present with hydrocephalus. While not all patients with medulloblastoma will continue to suffer from hydrocephalus after tumor resection, there is a significant proportion who will require a permanent shunt. We have retrospectively reviewed a group of children with medulloblastoma not shunted preoperatively, and have identified several characteristics which are associated with a requirement for permanent shunt. We studied 42 patients and found that 17 patients (40%) required permanent shunts within 4 weeks of craniotomy. The shunted patients were younger (5.4 +/- 2.9 vs. 10.0 +/- 5.7 years; p0.01), had larger ventricles (p0.05), and had more extensive tumors (Chang's stage T3 and T4; p0.01). These variables were all independently significant. In addition, we found that the patients requiring postcraniotomy shunts had a much higher rate of morbidity including a postoperative pseudobulbar syndrome. Of interest, we found that none of our patients without the above characteristics required a shunt. In particular, we found that no patient older than 10 years required a shunt. Thus, we conclude that younger patients with moderate to severe preoperative hydrocephalus and extensive tumors may benefit from perioperative CSF diversion. Other patients not meeting the above criteria can probably be safely managed with perioperative corticosteroids alone.

We present a case illustrating the usefulness of motor evoked potentials (MEPs) in differentiating psychogenic from organic postoperative paralysis. Discussed is a 12-year-old girl who underwent surge

Selective dorsal rhizotomy has shown great promise as a treatment for the functional disabilities and deforming hypertonia of spastic cerebral palsy. At New York University Medical Center, 200 children underwent this procedure between 1986 and 1990. All groups, whether walkers, crawlers, or nonlocomotors, showed improvement in the tone and range of most muscles tested. Half of these patients experienced complications. Thirty-five of these were serious and included bronchospasm (5.5%), aspiration pneumonia (3.5%), urinary retention (7%), and sensory loss (2%). There are, however, clear indications that warn of these complications; monitoring and prophylactic treatment can minimize their effects, and the possibility of such problems is more than offset by the proven benefits of this operative procedure.

Intrinsic tumors of the medulla are amenable to gross total or radical subtotal resection in 30% to 50% of cases. Many of these lesions are histologically benign, and, with careful selection, aggressive surgical treatment has a role.

In 2005, bevacizumab was approved by Health Canada for patients with metastatic colorectal cancer (mcrc). Newfoundland and Labrador was one of the first Canadian provinces to fund this agent in combination with folfiri (irinotecan, 5-fluorouracil, leucovorin) chemotherapy. In this analysis, the entire provincial bevacizumab sample for the first 2 years was assessed for overall safety and efficacy. The medical records of 43 patients with mcrc who had received folfiri with bevacizumab were identified and reviewed. The longitudinal data collection format that was adopted assessed occurrences of adverse events after each cycle of treatment. Toxicity outcomes such as gastrointestinal (gi) perforations, bleeding, diarrhea, myelosuppression, proteinuria, and venous thromboembolic events (vtes) were collected and graded using the U.S. National Cancer Institute&rsquo, s Common Terminology Criteria for Adverse Events, version 3.0. Time to treatment failure (ttf) and overall survival (os) were determined using the Kaplan&ndash, Meier method. Overall, the 43 study patients received 398 cycles of anticancer therapy (median: 6 cycles, range: 1&ndash, 24 cycles). No gi perforations were identified. However, 4 bleeding events occurred (9.3%), 3 requiring permanent discontinuation of bevacizumab. Also, 6 grade 3 or 4 vtes occurred (14.0%), 3 of which required a hospital admission. In addition, grades 3 and 4 diarrhea, febrile neutropenia, and proteinuria showed cumulative incidences of 11.6%, 2.3%, and 2.3% respectively. Median ttf was 6.3 months, median os was 24.4 months. Bevacizumab in combination with folfiri appears to be well tolerated, and efficacy is consistent with trial reports. However, patients should be closely monitored to avoid potentially serious events such as bleeding and vtes.

In 31 children (age, 2-17 years) and 1 adult, individual dorsal root action potentials (DRAPs) from the S1-S3 roots were recorded intraoperatively after electrical stimulation of the dorsal penile or clitoral nerves, in preparation for surgery within the cauda equina. In most patients, pudendal afferent activity was present in S2 and S3 bilaterally; in some, the afferent activity was confined to a single root bilaterally, and in one, to a single root on one side. Dorsal root action potentials of small amplitude were recorded from S1 in 15 patients, although in no patient was S1 the primary carrier of these afferents. No lesion of the roots or rootlets carrying significant afferent activity was created during the rhizotomy, and no dysfunction in micturition resulted. We propose that the neurophysiological identification of roots and rootlets carrying afferent activity from the penile or clitoral nerves allows for rhizotomy of the S2 roots with the least possible risk of postoperative micturition and sexual dysfunction.

Since 1986 250 patients have undergone selective posterior rhizotomy for spastic cerebral palsy at New York University Medical Center. Severe, perioperative complications were experienced by 15% of these patients and consisted of bronchospasm, aspiration pneumonia, urinary retention, ileus, sensory loss. Additionally, most of our patients had severe pain immediately after the surgery and nearly half had dysesthesias for up to 3 weeks. Long-term complications such as decreasing range of muscle movement and hip dislocation are now becoming apparent. This patient population must be viewed as having special risks attendant with surgery and particularly with selective posterior rhizotomy.

From its introduction in the early 1970s, intraoperative neurophysiological monitoring has evolved into an extremely useful and reliable adjunct for operating on the central and peripheral nervous system.This manuscript reviews the author's experience with its evolution in his practice and how it impacts it today.

The past 2 years have been two of the most memorable of my life. It was a humbling experience to be elected President-Elect at our Taipei meeting in 2006 and to then receive the poncho in 2007. And, it was a joyous experience to work with what has to have been one of the most talented and industrious executives in the history of the International Society for Pediatric Neurosurgery (ISPN). These are memories I will always cherish. I thank you for the honor of letting me work for an organization that is so good and that I care so much about. In 1972, 11 neurosurgeons assembled in Chicago and agreed to form the ISPN. The energy for this stemmed from a belief that in order to insure a rapid evolution of our specialty, a sharing of the collective knowledge was mandatory. This type of communication has always been at the core of the ISPN′s activities, and in 2000, our then president Hal Rekate led us in putting this mission on paper (Fig. 1). Our mission is the promotion of health in our young patients. We are no different than other specialties caring for children. We are passionate to further our specialty, and the energy for this comes from our love of children and our desire to serve them well. We take time away from our families to study, we spend our money to travel to learn new techniques, and we sacrifice our time to educate others. To accomplish these tasks, we have traditionally used our printed journals, annual scientific meeting, and educational courses held around the world. These tools were developed in response to the realities of the mid-twentieth century and have been very successful in driving the expansion of our specialty. The ISPN welcomes new members every year, and the attendance at our annual meeting continues to grow. The number and sophistication of the presentations at our annual meeting continues to expand. We are challenged by people such as Archbishop Tutu and the needs of emerging societies in Africa, Asia, and the Americas to support the development of pediatric neurosurgery in their communities. We need to congratulate ourselves on the great strides we have taken toward meeting our mission. But, before we do, before we sit back and reflect on our accomplishments, we had better take a good hard look at what is happening in our world. I do not need to tell you that it is rapidly changing. Therefore, I feel that it is timely for the ISPN to reflect on our mission and how we can support the needs of societies where our specialty is not fully developed. When we look at our world, we are seeing a rapid equilibration in access to information. This has been due to the emerging world′s rising capacity for wealth generation (Fig. 2) [1]. While for the past 400 to 500 years, a small handful of countries with relatively small populations dominated the world economy and controlled access to much information, this is rapidly changing as nations with massive populations experience staggering rates in the growth in their economies. India and China, the prime examples of these phenomena, have liberated much of their economies, and they now function in a largely free-market environment. The result is a freer access to information and the generation of wealth. The key here is the freeing of their economies from state control, and this lesson is being learned in other emerging countries such as Brazil and Childs Nerv Syst (2009) 25:1157–1161 DOI 10.1007/s00381-009-0929-0

Intrinsic focal tumor of the medulla oblongata is an uncommon entity. Of the 51 cases presenting for treatment of a brainstem glioma between 1984 and 1990, only 7 were confined to the medulla. Radical excision was performed on each patient. Four of the first 5 patients who were extubated in the recovery room had CO2 retention, with associated respiratory arrest and hypoxia. Three suffered permanent cranial nerve deficits as a result. It is the purpose of this report to describe the serious complications of medullary surgery and to make recommendations as to how they may be avoided.

Chronic headaches in a shunt-dependent patient with small ventricles has long been treated with little or no regard to intracranial pressure. In this study, pressure monitoring on 12 such patients demonstrated that they fell into three distinct categories: 3 had headaches caused by intracranial hypertension, 2 had headaches from hypotension, and 7 showed no relation of symptoms to pressure. As therapeutic procedures for treating these three categories are entirely different and sometimes opposing, it is clear that intracranial pressure monitoring is essential to successful management of this complaint.

The traditional treatment for all forms of hydrocephalus has been the implantation of ventricular shunt systems; however, these systems have inherent tendencies toward complications such as malfunction and infection. A significant advance in the treatment of hydrocephalus has been the evolution of endoscopy. The recent technological advances in this field have led to a renewed interest in endoscopic third ventriculostomy as the treatment of choice for obstructive hydrocephalus. Although several different endoscopes are available, the authors favor a rigid one to perform a blunt fenestration of the third ventricle floor. This description of the technique stresses the nuances for successful completion of this procedure.

Meningioangiomatosis is an uncommon clinical entity. This lesion has been reported at time of autopsy in patients with neurofibromatosis (NF) and in case reports of patients without NF Type 2 (NF2). The authors report a series of six patients with meningioangiomatosis who do not have NF2 and describe the clinical presentation, diagnosis of disease, and treatment. They also review the literature concerning this entity.Six patients with meningioangiomatosis were treated at the authors' institutions from 1994 to 2001. The mean age of the patients was 10.7 years (range 5-14 years). All of the children presented with a seizure disorder. Surgery was performed in all children, and a gross-total resection was accomplished. All patients exhibited clinical improvement. At last follow up (mean 6.3 years) all patients are free of seizures and are not taking anticonvulsant medications. No signs of recurrence have been noted on imaging studies.The authors advocate a gross-total resection of meningioangiomatosis for the treatment of seizure disorder in this population.

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.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.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.

OBJECTIVE: Although optimal treatment for intrinsic focal tumors of the medulla remains controversial, many surgeons advocate radical surgery for patients with these tumors. Postoperative surgical morbidity may include loss of lower cranial nerve function and significant motor deficits. Recovery of lower cranial nerve dysfunction after radical surgery has not been reported previously. METHODS: Forty-one children and adolescents with tumors involving the medulla underwent operations between 1986 and 1997. Nineteen (46%) of these patients experienced loss of lower cranial nerve function requiring tracheostomy, ventilator support, and feeding gastrostomy. A retrospective analysis of this patient population and the time to cranial nerve recovery was undertaken. RESULTS: Thirteen (68%) of the 19 patients with loss of lower cranial nerve function had full recovery of lower cranial nerve function. Two patients (11%) have had significant improvement in their lower cranial nerve function, and four patients (21%) have remained without lower cranial nerve function. CONCLUSION: Lower cranial dysfunction is common after surgery for intrinsic medullary tumors. However, the majority of patients who require tracheostomy or gastrostomy tubes will recover cranial nerve function.

Objective and importance Intrathecal baclofen delivery for the treatment of spasticity has been used for almost 20 years with a great deal of success. A wide variety of complications and pitfalls have been described. This report details a novel complication involving inadvertent and initially unrecognized canalization of the subdural space with the spinal catheter, which ultimately resulted in an overdose. Clinical presentation An intrathecal pump system was implanted in a 15-year-old girl with spasticity. This initially resulted in a lack of therapeutic effect. The diagnostic workup ultimately led to contrast administration through the pump system, which precipitated a baclofen overdose when sequestered medication in the subdural compartment was released into the intrathecal space. Intervention The spinal catheter was subsequently revised, and the patient made a full recovery. Conclusion The possibility of a subdural catheter should be included in the differential diagnosis in patients who experience a lack of drug effect after pump implantation, despite increases in dosage. Close monitoring is required because of the risk of spontaneous or induced overdose, which may occur when a communication develops between the subdural and intrathecal compartments.

I have little doubt that most arachnoidal cysts will be managed endoscopically in the future given the advances we have seen over the last decade in our instrumentation. Our excitement to employ this new technology should be governed by the reality that we are still learning and that our current success rate is not quite as good as what can be expected when using microneurosurgery.

Since the beginnings of medicine, physicians have sought minimally invasive ways to peer into body cavities. It is only in the last several decades that the promises of endoscopy have begun to be answered. What follows is a brief outline of the development of endoscopic technology and its application to the nervous system both for diagnostic and therapeutic procedures.

Chronic headaches are a well-documented complaint of shunted hydrocephalic patients. However, it is also one of the signs of shunt malfunction. Cranial compliance deficiency may be a cause of chronic headaches in some chronically shunted patients with functioning shunts (often with slit or smaller than normal ventricles). This study aims to use a novel, non-invasive imaging technique, Magnetic Resonance Elastography (MRE) to investigate the role of brain viscoelasticity in pediatric hydrocephalic patients.

Background Endoscopic third ventriculostomy has become a popular alternative to ventricular shunts for noncommunicating hydrocephalus. Although endoscopic third ventriculostomy is a safe procedure, several complications related to this procedure have been reported in the literature. The authors present a rare case of symptomatic bilateral subdural hematomas after an uneventful endoscopic third ventriculostomy. Case description A 51-year-old male patient presented with symptoms of obstructive hydrocephalus, headaches and memory disturbance. Magnetic resonance imaging demonstrated hydrocephalus secondary to aqueductal stenosis. An endoscopic third ventriculostomy was performed. The patient was discharged home in several days without complication. He then presented with headaches 3 weeks following surgery. A computed tomography study demonstrated bilateral subdural hematomas. These were treated with burr hole evacuation and drainage. Postoperatively, his headaches improved. At last follow-up he remains symptom-free and has radiographic evidence of a patent ventriculostomy. Conclusion This case confirms chronic subdural hematoma formation is a possible complication following third ventriculostomy. Patients should be followed closely for possible subdural hematoma formation.

Object. In this study the authors evaluate the safety, efficacy, and indications for endoscopic third ventriculostomy (ETV) in patients with a history of subarachnoid hemorrhage or intraventricular hemorrhage (IVH) and/or cerebrospinal fluid (CSF) infection. Methods. The charts of 101 patients from seven international medical centers were retrospectively reviewed; 46 patients had a history of hemorrhage, 42 had a history of CSF infection, and 13 had a history of both disorders. All patients experienced third ventricular hydrocephalus before endoscopy. The success rate for treatment in these three groups was 60.9, 64.3, and 23.1%, respectively. The follow-up period in successfully treated patients ranged from 0.6 to 10 years. Relatively minor complications were observed in 15 patients (14.9%), and there were no deaths. A higher rate of treatment failure was associated with three factors: classification in the combined infection/hemorrhage group, premature birth in the posthemorrhage group, and younger age in the postinfection group. A higher success rate was associated with a history of ventriculoperitoneal (VP) shunt placement before ETV in the posthemorrhage group, even among those who had been born prematurely, who were otherwise more prone to treatment failure. The 13 premature infants who had suffered an IVH and who had undergone VP shunt placement before ETV had a 100% success rate. The procedure was also successful in nine of 10 patients with primary aqueductal stenosis. Conclusions. Patients with obstructive hydrocephalus and a history of either hemorrhage or infection may be good candidates for ETV, with safety and success rates comparable with those in more general series of patients. Patients who have sustained both hemorrhage and infection are poor candidates for ETV, except in selected cases and as a treatment of last resort. In patients who have previously undergone shunt placement posthemorrhage, ETV is highly successful. It is also highly successful in patients with primary aqueductal stenosis, even in those with a history of hemorrhage or CSF infection.

A case of hypotension caused by hemorrhage in a patient receiving systemic methotrexate to treat a known ectopic pregnancy is presented. A primary goal of gynecologic practice in the 1990s is to conserve tubal patency and fertility. Consequently, ectopic pregnancy is more frequently being managed by conservative strategies. The emergency physician must be familiar with conservative methods for the management of ectopic pregnancy and the complications that may cause emergency presentations in these patients.

Object: The goal of this study was to evaluate the safety, efficacy, and indications for repeat endoscopic third ventriculostomies (ETV). Methods and results: We reviewed the records of 20 patients who had undergone repeat ETV from 1987 to 1999. Their ages ranged from 8 months to 53 years (mean 17 years). The primary etiologies of hydrocephalus were: primary aqueductal stenosis (9 cases), tumor (5), Chiari malformation (2), prior infection (2), prior intraventricular hemorrhage (1), and blocked foramen of Monro (1 patient). The interval between the first and second ETVs ranged from 8 days to almost 6 years (mean 12.8 months). The intraoperative findings at repeat surgery were: occlusion of the primary orifice by scar (10 cases), virginal floor of the third ventricle (5 cases), pinhole ventriculostomy (3 cases), incompletely penetrated membrane (1 case), and blood clot occluding the orifice (1 case). The follow-up period ranged from 3 to 47 months (median 20 months). Repeat ETV was successful in 13 patients (65%). These patients did not require further shunting or other procedures during follow-up. Seven patients (35%) required placement of a shunt after repeat ETV. Several complications were observed in 1 patient (5%), including seizures, elevated ICP, bilateral pulmonary edema, and cardiac arrhythmia. This patient ultimately recovered fully; the ETV was successful, and the patient did not require a shunt. Conclusions: Based on the experience of this group of patients, repeat ETV is as effective and as safe as a primary ETV procedure, and should be attempted in selected patients.

Intrathecal baclofen infusion has demonstrated effectiveness in decreasing spasticity of spinal origin. Oral antispasticity medication is minimally effective or not well tolerated in cerebral palsy. This study assessed the effectiveness of intrathecal baclofen in reducing spasticity in cerebral palsy. Candidates were screened by randomized, double-blind, intrathecal injections of baclofen and placebo. Responders were defined as those who experienced an average reduction of 1.0 in the lower extremities on the Ashworth Scale for spasticity. Responders received intrathecal baclofen via the SynchroMed System and were followed for up to 43 months. Fifty-one patients completed screening and 44 entered open-label trials. Lower-extremity spasticity decreased from an average baseline score of 3.64 to 1.90 at 39 months. A decrease in upper-extremity spasticity was evidenced over the same study period. Forty-two patients reported adverse events. Most common reports were hypotonia, seizures (no new onset), somnolence, and nausea or vomiting. Fifty-nine percent of the patients experienced procedural or system-related events. Spasticity in patients with cerebral palsy can be treated effectively by continuous intrathecal baclofen. Adverse events, although common, were manageable. (J Child Neurol 2000;15:71-77).

Six patients presented with either entrapped fourth ventricles or noncommunicating cerebrospinal fluid collections of the posterior fossa requiring drainage. These collections were treated with shunt systems whose proximal catheter was placed into the fourth ventricle via a coronal burr hole using an endoscope guided by Eleckta's ISG Viewing Wand. The technique and its advantages are described as are the complications and early outcomes.

BACKGROUND Intraparenchymal nerve sheath tumors of the central nervous system are rare, usually benign tumors. Three cases with tumor recurrences have previously been reported in the literature. The authors report a case of a malignant intracerebral nerve sheath tumor in a girl age 8 years and analyze the biologic characteristics of this tumor. METHODS The tumor was analyzed by histologic, immunohistochemical, and ultrastructural techniques. RESULTS Magnetic resonance imaging revealed a mixed, attenuating, contrast-enhancing mass in the right posterior temporal lobe. Histopathology of the resected specimen revealed a uniformly S-100 positive and reticulin rich biphasic tumor with a characteristic distribution of spindle and epithelioid cells, with the latter almost entirely confined to the invading edge of the tumor. There was extensive brain infiltration in the form of lobules and fingerlike processes with a targetoid appearance. The spindle cells showed palisading and had elongated, wavy nuclei. Electron microscopy demonstrated basal lamina around both cell types with scattered Luse bodies in between. The patient is perfectly healthy and recurrence free 17 months after surgery. CONCLUSIONS This represents the fourth and the youngest case in the literature of a malignant intracerebral nerve sheath tumor and highlights the distinctive clinicopathologic features of these tumors. First, these tumors have very great infiltrative potential, as observed histopathologically and reflected in their frequent recurrences. Second, epithelioid cells confined to the infiltrative edge suggest their aggressive role. Epithelioid cells have been shown to represent a malignant component of systemic schwannomas with malignant transformation; however, this case does not have any evidence of origin from a preexisting benign schwannoma. Third, despite histologic similarities to neuraxial desmoplastic neuroepithelial tumors, none of the malignant schwannomas had any evidence of divergent differentiation towards astrocytic or neuronal lineage, and had a much worse prognosis. Fourth, the event free survival after initial resection appears to be an important predictor of overall survival in the reviewed cases. Chemotherapy and radiation therapy have not been successful in the treatment of this rare entity. Hence, an accurate diagnosis and planned extensive resection appear to be the key elements in its management. Cancer 1998;82:545-52. © 1998 American Cancer Society.

479 Background: Sunitinib is a standard of care for first-line treatment of metastatic clear cell renal-cell carcinoma (mRCC). Despite having a relatively good safety profile, Sunitinib does have several clinically important toxicities. With the rapid rise in the use of Sunitinib and other oral cancer agents, we instituted a pharmacist-led monitoring program in the ambulatory care setting to prospectively document, monitor, and manage toxicities in our Canadian province. Methods: The pharmacist-led monitoring program consisted of patient assessments in clinic with the oncology team combined with a call back program. The program consisted of a patient assessment in the oncology clinic on day 1 of a Sunitinib cycle followed with a call back on day 14. A chart review of consecutive patients who were prospectively monitored by this progam after receiving Sunitinib for mRCC was conducted. Treatment specific data for the first six cycles of therapy included dose reductions, therapy delays/interruptions, therapy discontinuation, and reason for each was recorded. Toxicity data including the occurrence and severity grade was collected. The time to treatment failure (TTF) defined as the time from therapy initiation to treatment discontinuation for any reason was measured. Results: Fifty six patients are included in the study cohort. Of these, 52 (92.86%) started at the standard 50 mg once daily and the remainder at a reduced dose. Additionally, 15 patients experienced hypertension requiring drug therapy adjustment or additional antihypertensive therapy. Two patients required drug therapy for hypothyroidism. There were a total of 39 dose reductions in this patient population over a six cycle period. The majority of dose reductions (46.2%) and therapy interruptions (34.5%) occurred during cycle one. The time to treatment failure for the 45 patients that discontinued therapy was 9.72 months. Conclusions: Pharmacist-Led monitoring of oral cancer therapies is a practical and feasible method of monitoring patients on Sunitinib for mRCC. The success has led to its implementation with other agents and disease sites.

Publisher Summary Selective functional, dorsal (or posterior) rhizotomy (SDR) has become an increasingly popular means of treating children with congenital spastic diplegic and quadriplegic cerebral palsy. SDR is a deafferentation procedure that supposedly alters the modulating milieu of the interneuronal pool responsible for controlling the excitability of α-motoneurons. One of this pool's primary functions is to modulate the pattern and reactivity of the spinal cord's reflex circuitry. The pool receives inhibitory innervation from Ia afferents, flexor reflex afferents, and high-threshold afferents. Selective dorsal rhizotomies reliably reduce spasticity and that this reduction is permanent. There is increasing evidence to suggest that this reduction also translates into improvement in function. A common way to measure the impact of treatment on children with cerebral palsy is to record the available range of passive motion at a given joint (goniometric measurement). Another way to measure the functional impact of SDR is to analyze the children's gait pattern before and after surgery using computerized gait analysis systems.