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OBJECTIVE To report a case series in which basal ganglia calcifications without mass effect proved to be germ cell tumors. DESIGN Case series. SETTING Tertiary care hospital. PATIENTS Four patients. INTERVENTIONS Computed tomography, magnetic resonance imaging, positron emission tomography, biopsy, chemotherapy, and radiation therapy. MAIN OUTCOME MEASURES Recognition of clinical syndrome and radiological features. RESULTS All patients had progressive hemiparesis, and 1 patient also had frontal lobe dementia. Imaging demonstrated progressive asymmetric signal abnormality with basal ganglia calcification and associated brainstem atrophy. Fludeoxyglucose F 18–positron emission tomography showed hypometabolism in contrast to malignant glioma. CONCLUSION Germ cell tumor should be considered in patients with an indolently progressive neurological course, particularly if basal ganglia calcification is present with or without enhancement, asymmetric brain atrophy, or a mass.Arch Neurol. 2009;66(6):789-792--

To determine if an etiological difference exists between isolation of the lateral ventricle and isolation of the fourth ventricle after ventricular shunting.Cases of symptomatic isolation of the lateral and fourth ventricles were reviewed retrospectively. The ages at presentation of ventricular isolation, the time course to development of isolation, the number of shunt surgeries leading up to symptomatic isolation, the types of shunt valves utilized, and the background of infection were analyzed.Twenty-six patients had lateral ventricle isolation and 11 patients had fourth ventricle isolation. Infection, hemorrhage, Chiari malformation/myelomeningocele, and aqueductal stenosis were factors contributing to hydrocephalus requiring treatment in these patients. Compared to 26.9% of patients with lateral ventricle isolation, 90.9% of patients with fourth ventricle isolation had a previous history of infection.Prior meningitis and ventriculitis frequently contributed to fourth ventricle isolation. Lateral ventricle isolation seems to arise from functional obstruction of the foramen of Monro related to prior shunting.

To review the management of epilepsy in patients with tuberous sclerosis complex (TSC) with an emphasis on surgical aspects, neuropathology, and pathogenesis.Review of the literature and presentation of the authors’ experience of surgery for refractory epilepsy in patients with TSC.TSC is a multisystem genetic disorder with variable phenotypic expression. TSC results from a mutation in the TSC1 gene on chromosome 9, which codes for hamartin, or in the TSC 2 gene on chromosome 16 which codes for tuberin. The majority of the patients have TSC as a result of spontaneous genetic mutations while in one-third of the patients, the disorder is inherited in an autosomal dominant manner. Epilepsy is the most common neurological complication, and up to 80–90% of individuals with TSC develop epilepsy at some point in their lifetime. The onset of epilepsy is typically in early childhood. Infantile spasms are a very common early seizure type although partial seizures may occur. Developmental delay, intellectual impairment, autism, behavioral problems, and neuropsychiatric disorders occur commonly in individuals with TSC and may be associated with poorly controlled epilepsy. Antiepileptic drugs are the first-line management for epilepsy but the ketogenic diet, resection of one or more tubers, corpus callosotomy, and vagus nerve stimulation are other therapeutic options for individuals with poorly controlled epilepsy.

The authors report a child in whom residual cerebellar astrocytoma after surgical resection was documented on serial computed tomography scans to undergo gradual complete regression spontaneously over a period of 11 years. In this case, the time course of regression has been well documented.The time course of regression may provide clues as to the underlying cause of this phenomenon.

The treatment of positional plagiocephaly is controversial. A confounding factor is the lack of a proven clinically viable measure to quantify severity and change in plagiocephaly. The use of anthropometric measurements is one proposed method. In this study, the reliability and validity for this method of measurement were investigated. Two clinicians independently recorded caliper measurements of cranial vault asymmetry (CVA) for infants referred for plagiocephaly or torticollis, and an unbiased observer recorded visual analysis scores during the same visit. CVA scores were assigned into three predetermined severity categories (normal CVA < 3 mm, mild/moderate CVA ≤ 12 mm, moderate/severe CVA > 12 mm). CVA measurements and visual analysis scores were recorded for 71 and 54 infants, respectively. Intrarater reliability was established ( = 0.98, = 0.99), but inter-rater reliability was not ( = 0.42). In addition, the inter-rater reliability for the severity categories based upon these measures was poor ( = 0.28) and failed to correlate to the visual analysis ( = 0.31). Development of a stable and meaningful measurement system for the extent of plagiocephaly is needed to allow scientific studies of the natural history of plagiocephaly and effectiveness of interventions.

Isolated sagittal synostosis can be diagnosed easily on clinical grounds. This study was designed to determine if children could be operated on based solely on a clinical diagnosis or whether such an approach would result in any potentially unnecessary surgeries.Records of 114 consecutive children operated on for isolated nonsyndromic sagittal synostosis over a 14-year period (1987–2000) were reviewed to see whether the clinical findings were in concordance with the intraoperative findings and histopathology of the sagittal suture.The age at surgery ranged from 1.9 to 81.3 months (median 4.3 months). Preoperative skull radiographs were done in 78 children and computer tomography (CT) scans of the head in 12 children. One hundred percent of the skull radiographs and 83.3% of the CT scans were completed prior to referring the children to a pediatric neurosurgeon. CT scans confirmed the diagnosis in all patients in whom it was available. For the rest, pathology reports and/or operative records were available for 108 (94.7%) children and were confirmatory for sagittal craniosynostosis in all.Accurate diagnosis of isolated sagittal synostosis can be made clinically, and operative correction can proceed without a need for radiological investigations, unless the clinical features are not completely typical. This approach could result in significantly reduced radiation exposure for the developing brain and could provide economic benefits to health care providers.

Abstract: Objectives: The purpose of the study was to determine if there are preoperative clinical characteristics that might be predictive of a poor outcome after selective dorsal rhizotomy (SDR) surgery in children with spastic cerebral palsy (CP).Methods: A retrospective analysis was performed on 174 children who had undergone SDR from 1983 to 2001. Patients were divided into two groups according to their outcome at approximately 1 year after surgery: ldquoacceptablerdquo or ldquopoorrdquo outcome. As predictors of outcome, the factors compared were age at operation, types of CP (diplegia, quadriplegia), history of prematurity, prior lower limb orthopedic surgeries, history of seizures, dystonic limbs, opisthotonic posturing, lumbar hyperlordosis, truncal hypotonia, preoperative ambulatory function, preoperative Gross Motor Function Classification System (GMFCS) scores, and presence of intellectual delay and speech delay.Results: Eleven children (6.3%) had a ldquopoorrdquo outcome. The type of CP (P<0.001) and intellectual delay (P=0.015) were significant predictors of outcome in the univariate regression analysis, but only the type of CP retained significant predictive power in the multivariate analysis.Conclusion: These data suggest that preoperative diagnosis is the strongest predictor of outcome after SDR. Intellectual delay demonstrated predictive power only in the univariate model, suggesting that it might have some prognostic value but less than the diagnosis.

Abstract

Objective The current study was performed to determine the rate of allogeneic blood transfusion for coronal and metopic craniosynostosis surgery done by two experienced pediatric neurosurgeons and to identify factors associated with a need for transfusion. Methods First operations for coronal and metopic craniosynostosis correction in children from 1996 to 2002 were reviewed retrospectively. For analysis, the primary endpoint was defined as either the receipt of a blood transfusion or a postoperative Hb level below 70 g/l, even if no blood was transfused. Univariate and multivariate analyses were done to examine the relationship of attaining the primary endpoint to extent of surgery, surgeons, surgery time, preoperative hemoglobin, craniosynostosis type, weight, and age. Results Thirty-two patients were operated on: 17 with unicoronal; 9 with bicoronal, and 6 with metopic craniosynostosis. Median age at operation was 7.5 months. Blood transfusion rates were 0% for unicoronal, 44% for bicoronal, and 33% for metopic synostosis operations. Logistic regression revealed that the best predictor of having a postoperative hemoglobin level of <70 g/l or receiving a blood transfusion was the extent of surgery ( ß=1.59, SE ß=0.57). The odds of reaching the primary endpoint for extensive surgery over basic surgery was 4.9 [95%CI=(1.6,15.0)]. Once extent of surgery was accounted for in the model, no other covariates significantly improved the model. Conclusions Low transfusion rates were achieved in primary operations for coronal and metopic craniosynostosis using simple intraoperative techniques and by accepting a low postoperative hemoglobin level.

Case reportA case of spontaneous hemorrhage into an intradural extramedullary neurenteric cyst at the C4–C6 level in a 6-year-old boy is reported. The child presented with sudden neck pain, torticollis, and myelopathy. The cyst was of high signal intensity on both T1- and T2-weighted magnetic resonance images, suggesting hemorrhage into the cyst. The combination of CT and MR provided useful preoperative information regarding the nature and the location of the cyst. A posterior approach via osteoplastic laminotomy was used for this anteriorly localized cyst. The use of intraoperative ultrasound was helpful in identifying and puncturing the cyst to minimize the spinal cord manipulation during the excision of the cyst wall.DiscussionWe report the first documented case of hemorrhage into a spinal neurenteric cyst that has been confirmed surgically and histopathologically.

IntroductionThe availability of magnetic resonance imaging (MRI) has resulted in an increasing number of asymptomatic, minimally symptomatic, and doubtfully symptomatic patients being diagnosed with a Chiari I malformation with or without syringomyelia. In an attempt to clarify how neurosurgeons manage these clinical problems, an international survey on the Chiari I malformation and related syringomyelia was undertaken.MethodA questionnaire on the expected natural course of the disease and on aspects of the surgical technique for a number of hypothetical cases relating to Chiari I malformation with and without syringomyelia was used to survey Pediatric Neurosurgeons worldwide.ResultsOf 246 questionnaires distributed, 76 (30.8%) were completed and returned. There was a consensus that no operation should be carried out in asymptomatic patients with a Chiari I malformation, unless there is associated syringomyelia. There was a consensus that decompression of the Chiari malformation should be performed in patients with scoliosis when syringomyelia is present, and the majority decompressed the Chiari malformation in scoliotic patients even in the absence of syringomyelia. Suboccipital decompression was the standard surgical procedure for Chiari I malformations. The majority of respondents favored routine dural opening at surgery and closure with a pericranial or synthetic patch graft. In the case of a persistent or progressive syrinx after suboccipital decompression, the majority recommended shunting of the syrinx to the subarachnoid space or to the pleural cavity.ConclusionThere continues to be much variation in the management of the Chiari I malformation.

Prior studies have found no adverse effects of pediatric epilepsy surgery on IQ. However, empirical techniques such as regression models, designed to account for confounding factors such as practice effects and test–retest reliability and able to provide a standardized method for evaluating outcome, have not been used in studying change after pediatric epilepsy. The goal of this study was to demonstrate the regression technique while empirically measuring the effect of epilepsy surgery on IQ in a group of pediatric patients. Predictors of retest IQ (e.g., baseline IQ, retest interval, demographics, epilepsy severity) were evaluated in a control group with intractable seizures (N = 23) assessed twice with the WISC–III. The resulting equation was used to evaluate IQ changes in a second group of children who underwent epilepsy surgery (N = 22). In controls, baseline IQ was a strong predictor of retest IQ. Number of AEDs was inversely related to retest IQ. Based on the control regression, four children (18%) in the surgical sample obtained significantly higher than expected postsurgical IQ scores and one child (5%) obtained a lower than expected IQ score. This study demonstrates that regression-based techniques yield informative estimates on outcome and may be an improvement over prior methods of measuring change after pediatric epilepsy surgery. (JINS, 2003, 9, 879–886.)

This study is a comparative analysis and meta‐analysis of three randomized clinical trials. Children with spastic diplegia received either 'selective’dorsal rhizotomy (SDR) plus physiotherapy (SDR+PT) or PT without SDR (PT‐only). Common outcome measures were used for spasticity (Ashworth scale) and function (Gross Motor Function Measure [GMFM]). Baseline and 9‐ to 12‐month outcome data were pooled (n=90). At baseline, 82 children were under 8 years old and 65 had Gross Motor Function Classification System level II or III disability. Pooled Ashworth data analysis confirmed a reduction of spasticity with SDR+PT (mean change score difference ‐1.2; Wilcoxon p<0.001). Pooled GMFM data revealed greater functional improvement with SDR+PT (difference in change score +4.0, p=0.008). Multivariate analysis in the SDR+PT group revealed a direct relationship between percentage of dorsal root tissue transected and functional improvement. SDR+PT is efficacious in reducing spasticity in children with spastic diplegia and has a small positive effect on gross motor function.

Management of spinal cord compression from a primary paraspinal endodermal sinus tumor (EST) is described. A 17-month-old child presented for treatment with near-complete paraplegia secondary to spinal cord compression from a primary paraspinal EST. The child was treated with cisplatin-based chemotherapy without laminectomy or radiation therapy. Rapid resolution of symptoms was observed. The child had an excellent tumor response and complete neurologic recovery with no sequelae. Chemotherapy alone is an alternative to laminectomy or radiation therapy in the management of epidural cord compression from EST, even when the cord compression is severe.

Abstract: Object: The purpose of this article was to review the published outcomes after selective dorsal rhizotomy (SDR) for treatment of spastic cerebral palsy. Methods: A literature search identified all articles related to outcomes after SDR. The outcomes were reviewed according to a paradigm developed by the National Center for Medical Rehabilitation Research (NCMRR). The quality of the evidence for each outcome was assessed using Sackett’s criteria and the classification system developed by the Brain Trauma Foundation and the American Association of Neurological Surgeons. Results: There is very strong evidence for benefits of SDR in the impairment domain of the NCMRR classification. SDR has been shown conclusively to decrease lower limb spasticity and increase lower limb range of motion. There is strong, but not as conclusive evidence that SDR has a positive impact in the functional limitation dimension, with improvements in motor function, and in particular the Gross Motor Function Assessment (GMFM). There is a moderate degree of certainty that SDR results in improvements in the disability dimension, as evidenced particularly by improvements in the Functional Independence Measure for Children (WeeFIM) and Pediatric Evaluation of Disability Inventory (PEDI). There is a moderate degree of certainty that SDR results in positive suprasegmental effects, especially related to upper limb function and cognition. There is weak evidence that SDR may reduce the need for orthopedic procedures in patients with spastic cerebral palsy, and the impact on hip subluxation relative to the natural history of this problem is unclear. Conclusions: This information could help to define the role of SDR in the management of the child with spastic cerebral palsy, in the light of alternative therapies, such as intrathecal baclofen and botulinum toxin, which have been introduced more recently. It also reveals the need for further studies, particularly dealing with quality of life and economic impact.

Unethical practices are not unusual in medical scientific articles, but they are not always recognized as being unethical. The author discusses both obvious unethical behavior, such as fraud, plagiarism, and conflict of interest, and a number of other practices, such as errors in citations, misuse of statistics, and wasteful publication, which are more widespread and generally accepted, but which may be considered unethical. Increased awareness of these issues could reduce the extent of these practices.

Spinal deformity is not usually regarded as a potential complication of posterior fossa tumor resection. After cervical subluxation had been recognized in 2 children following removal of posterior fossa tumors, a review was carried out to determine the incidence of this complication. Of the 72 children who had undergone resection of a posterior fossa tumor, 4 patients, including the original 2, developed a cervical spinal deformity. Factors that predisposed to this complication included laminectomy of C2 or lower, local wound complications and possibly post-operative neck weakness. It is important to be aware that cervical subluxation can occur after resection of posterior fossa tumors, when laminectomy extending below C1 has been performed. Patients at risk should be followed closely.

Cervical spina bifida, either occult or overt, is relatively uncommon and tends to be associated with other abnormalities of the central nervous system and spine. Cervical spina bifida cystica is characterized by two types of abnormalities: the myelocystocele herniating posteriorly into a meningocele, and a meningocele with or without an underlying split cord malformation. In both the occult dysraphic states and spina bifida cystica, there is underlying tethering of the cervical spinal cord and surgical treatment is indicated for untethering of the spinal cord. Neurologic dysfunction relating to the cervical spina bifida may be mild and may not be identifiable in infancy; however, it may become apparent as the child grows older.

Summary: Six children (ages 2 to 14 years) with recurrent or poor risk primary brain tumors were treated with the `8 drugs in 1 day' chemotherapy regimen. As renal function deteriorated (median after five cycles), carboplatin was substituted in place of cisplatin at a median dose of 300 mg/m2 as a one hour infusion at hour three. Four patients also had their lomustine deleted and replaced by etoposide 100 mg/m2 at hour four. Compared to earlier cycles of chemotherapy, there was a moderate increase in transfusion requirement (especially platelets) but the incidences of fever and neutropenia were unchanged. Glomerular function as measured by nuclear glomerular filtration rate (GFR) remained unchanged or improved at the end of therapy. The severity of nausea and vomiting after substitution was markedly improved. Four of the six patients are still in complete remission 19 to 41 months after diagnosis or recurrence. It is feasible to substitute carboplatin and etoposide into the ‘8 drugs in 1 day’ regimen when extramedullary toxicity is found.

Four patients with malignant gliomas recurring after surgical resection, radiotherapy and chemotherapy were entered into a trial of immunotherapy. Partial removal of the recurrent tumors was performed and autologous mononuclear cell infusions were given into the resection cavity via a subgaleal reservoir that had been inserted at the time of tumor resection. Mononuclear cells were obtained using a cell separator and Ficoll-hypaque separation techniques. Patients were followed carefully with clinical examinations, CT brain scans and immunological testing. All patients have died and complete autopsies were done. We looked specifically for evidence of mononuclear cell penetration into the tumor and possible adverse effects on the normal brain or in the rest of the body. The results indicate that this type of therapy can be performed safely and may be useful in the treatment of patients with malignant gliomas.

Intramedullary spinal cord tumors are uncommon in children, and delayed diagnosis is common. Recent technologic advances, including magnetic resonance imaging, intraoperative ultrasonography, the ultrasonic surgical aspirator, and the operative laser have made radical resection of intramedullary tumors feasible. The long-term results of modern-day surgery using these techniques are only now becoming available, and the role of adjuvant radiotherapy or chemotherapy for the usual low-grade tumors remains unclear.

Enhanced and non-enhanced computed tomography (CT) brain scans were performed within 72 h of surgery on 21 children in whom brain tumors had been resected totally or subtotally, and scans were repeated at varying intervals thereafter. Biopsies of the resection margins were performed in 12 patients at the end of the surgical procedure. The immediate CT scan showed enhancement in the resection margin in 13 of the 21 patients and in 9 of the 13, the enhancement disappeared on follow-up scans. There was discordance between the results of immediate CT scan examination and the biopsies of the resection margins in 7 of the 12 cases. The advantages and disadvantages of an immediate postoperative scan versus a more delayed CT scan are discussed.

Selective functional posterior rhizotomy (SFPR) is a popular operation for the treatment of spasticity in children with cerebral palsy, but the physiologic basis of the procedure is poorly understood. As part of SFPR operations in 60 consecutive children, the responses to electrical stimulation of posterior lumbosacral roots and rootlets, and the corresponding anterior roots were studied. In addition, similar electrical stimulation of posterior roots was performed in four nonspastic “control” children. Sustained responses to 50 Hz stimulation, one of the criteria used to signify abnormality in the spastic children, was found frequently in the “control” children. Contralateral spreal to the lower limb muscles and suprasegmental spread to the upper limbs, face, and neck were determined to be the most valid criteria which differentiated abnormal from normal responses. Stimulation of anterior nerve roots at 50 Hz caused sustained responses and ipsilateral lower limb spread, at a low threshold compared to that of corresponding posterior roots. The results of this study bring into question the validity of some of the criteria that are used to select abnormal posterior rootlets in the SFPR procedure, and suggest criteria that may be more valid based on findings in nonspastic children.

The long-term functional outcome of 101 children born with meningomyelocele between 1971 and 1981 was assessed, by a combination of retrospective chart review and follow-up assessments. The children had been managed at birth using a process ofnonstandardized selection. Eighty-three of the 101 patients survived after a minimum follow-up of 8.6 years, for a mortality rate of 18%. Forty-four of 83 children (53%) were community ambulators, and this correlated well with the presence of intact quadriceps function. Forty-eight children (58%) attended normal school and were grade-appropriate. Sixty-two of 83 patients (75%) were socially continent of urine, and 71/83 (86%) were socially continent of stool. Hydrocephalus was present in 93 of the 101 children in the study, and 85 children were shunted. Half of the shunted children required a shunt revision in the first year of life, and thereafter the rate of revision decreased, so that after 2 years the risk of revision was approximately 10% per year.

Localized periaqueductal tumors usually present as hydrocephalus, which is then thought to be late-onset congenital aqueductal narrowing. In the past, radiological investigations, including positive contrast ventriculography, pneumoencephalography and even contrast CT scanning, have frequently failed to show tumors in this region in the early stages. However, recent experience using MRI on patients with unexplained late-onset aqueductal stenosis has in some instances shown the presence of a localized periaqueductal tumor as the cause of obstruction of the aqueduct. Four patients are described with hydrocephalus secondary to presumed late-onset congenital aqueductal stenosis, all of whom were shown to have a periaqueductal tumor. One patient had been investigated with a pneumoencephalogram and positive contrast ventriculogram and CT with an early-generation scanner, but the tumor was diagnosed only at the time of autopsy. In one patient, the tumor was diagnosed by CT and also confirmed with MRI; a histologic diagnosis has been obtained. In two other patients, CT with and without contrast enhancement was negative and in one of these, a positive contrast ventriculogram was also negative, but the tumor was easily identified on MRI scans. In both of these patients, a histologic diagnosis was obtained by stereotactic biopsy. Periaqueductal tumors must be considered in the differential diagnosis of patients who present with late onset aqueductal occlusion and in such patients, MRI would appear to be the investigation of choice.

BackgroundWith the advent of MRI, the diagnosis of Chiari I malformation is being made in younger patients, often with minimal or no neurologic symptoms.Clinical featuresThe symptoms and signs of a Chiari I malformation may be related to the compression of neural and/or dural structures at the cranio-cervical junction or may be related to the associated syringomyelia. Scoliosis is a particularly important and common finding in those children with syringomyelia.

The use of a ventriculosubgaleal shunt for temporary treatment of hydrocephalus in a child with multiple recurrent shunt infections related to eczema in the neck is described. Further shunt infection was avoided, the eczema cleared, and a permanent ventriculoperitoneal shunt was inserted successfully after 2 months.

A case is reported of methotrexate leukoencephalopathy presenting as an intracerebral rim-enhancing mass lesion of CT scan. The implications of this rare presentation of methotrexate leukoencephalopathy for therapy are discussed.

In order to restore spinal integrity following posterior exposures of the spinal canal in children, we describe modifications of Raimondi's laminotomy technique. The use of a pneumatic dissecting tool with footplate to create a hinged osteoplastic laminotomy is described, as are techniques for secruing the laminotomy flap in place at the end of the procedure.

Abstract: Introduction: The authors describe an extremely simple technique of head fixation for image-guided neurosurgery in young children in whom standard pin fixation cannot be used.Materials and methods: This involves positioning the head on a horseshoe headrest and using a ‘U-drape’ to fix the head to the headrest.Result: Over the last 5 years, this technique of head fixation (in conjunction with Stealthstation®) has been used for various neurosurgical procedures in more than ten infants successfully.

A modification of the technique combining sagittal strip, circular occipital and biparietal wedge craniectomies (Albright's procedure) for the treatment of sagittal synostosis, with prominent occipital bossing, is described. “Keyhole,” as opposed to wedge, parietal craniectomies allow improvement in the shape of the skull, beginning intraoperatively, while eliminating the outbending of the parietal bone that occurs at the apex of a wedge craniectomy.

Without Abstract: