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Background Neurosurgical training usually requires long hours for hands-on procedures, making it difficult for inexperienced surgeons to quickly learn in an error-proof environment. The objective of this study was to propose a puzzle-like new model for neurosurgical education that simulates craniosynostosis correction (scaphocephaly type) using Renier's H technique. A model of a 3-dimensional (3D) anatomic simulator for craniosynostosis training is presented and evaluated. Methods The cranial model was created using 1-mm computed tomography scan images from patients with scaphocephaly in the Digital Imaging and Communications in Medicine format. This information was processed using an algorithm to generate a 3D biomodel in resin. The puzzle model and its variable training models were assessed qualitatively by a team of expert neurosurgeons. Next, the model was applied in trainees and was evaluated using specific questionnaires. Results Experts and trainees evaluated the model. The mean number of attempts without errors was 2.3 ± 0.675, for 1 error was 2.2 ± 0.918, and for 2 errors was 1.3 ± 0.707. The mean score of the simulator was 9.2 ± 0.421. Twelve residents (second evaluation) answered the questionnaire with a positive assessment of diagnosis capabilities, appropriateness of the model, time commitment, adequate environment, reliable 3D reconstruction, and teaching method. Three participants had used a 3D simulator previously, and the simulator was evaluated obtaining a 9.9 final average (range, 0–10). Conclusions The puzzle may be a complementary tool for surgical training. It allows several degrees of immersion and realism, offering symbolic, geometric, and dynamic information with 3D visualization. It provides additional data to support the practice of complex surgical procedures without exposing real patients to undue risk.

Recessive CRB2 mutations were recently reported to cause both steroid resistant nephrotic syndrome and prenatal onset ventriculomegaly with kidney disease. We report two Ashkenazi Jewish siblings clinically diagnosed with ciliopathy. Both presented with severe congenital hydrocephalus and mild urinary tract anomalies. One affected sibling also has lung hypoplasia and heart defects. Exome sequencing and further CRB2 analysis revealed that both siblings are compound heterozygotes for CRB2 mutations p.N800K and p.Gly1036Alafs*43, and heterozygous for a deleterious splice variant in the ciliopathy gene TTCB21. CRB2 is a polarity protein which plays a role in ciliogenesis and ciliary function. Biallelic CRB2 mutations in animal models result in phenotypes consistent with ciliopathy. This report expands the phenotype of CRB2 mutations to include lung hypoplasia and uretero-pelvic renal anomalies, and confirms cardiac malformation as a feature. We suggest that CRB2-associated disease is a new ciliopathy syndrome with possible digenic/triallelic inheritance, as observed in other ciliopathies. Clinically, CRB2 should be assessed when ciliopathy is suspected, especially in Ashkenazi Jews, where we found that p.N800K carrier frequency is 1 of 64. Patients harboring CRB2 mutations should be tested for the complete range of ciliopathy manifestations.

Intrathecal baclofen is an expanding accepted treatment for children with cerebral palsy and other causes of spasticity and dystonia. The aims of this review are therefore to (1) delineate the clinical benefits of intrathecal baclofen therapy in pediatric spasticity and dystonia and (2) increase awareness of the potential complications and emergency management measures of intrathecal baclofen therapy. A current literature review demonstrates the benefits and complications of this minimally invasive device. Practical guides for recognizing acute conditions and management recommendations are included. Intrathecal baclofen is increasingly being used to help individuals attain realistic functional goals. Therefore, families and health care professionals should be aware of potential complications, symptoms, and emergency management.

To delineate the impact of early (≤ 25 days of life) versus late (25 days) external ventricular drainage (EVD) on the neurodevelopmental outcome of preterm infants with posthemorrhagic hydrocephalus (PHH) following intraventricular hemorrhage (IVH).We retrospectively categorized 32 premature infants with PHH into two groups according to whether they underwent early (n = 10) or late (n = 22) EVD. We administered the Battelle Developmental Inventory II and a neuromotor examination (median age, 73 months, range: 29-100).In adjusted comparisons, early EVD was associated with better scores than late EVD in adaptive (79 ± 22.6 vs. 58.8 ± 8.1, P = .01), personal social (90.7 ± 26 vs. 67.3 ± 15.9, P = .02), communication (95.4 ± 27.5 vs. 69.6 ± 20.5, P = .04) and cognitive (78.9 ± 24.4 vs. 60.7 ± 11.5, P = .055) functions. Three (30%) early EVD infants had severe (2.5 standard deviation) cognitive disability compared to 18 (82%) late EVD infants (P = .03). The incidences of cerebral palsy and neurosurgical complications were equal for the two groups. Subgroup analyses suggested that early EVD was beneficial in infants with original grade III IVH (n = 15, P0.05), but that it had no beneficial effects in infants with prior parenchymal injury (n = 17, P = NS).In this small retrospective series, early EVD is associated with lower rates of cognitive, communication and social disabilities than later EVD in infants with PHH without prior parenchymal injury. A randomized prospective trial is warranted.

In children, intramedullary spinal cord neoplasms are rare. These are typically low-grade neuroepithelial tumors, most commonly astrocytomas, ependymomas, and gangliogliomas. Malignant transformation, while common in recurrent adult low-grade gliomas, is an unusual event in pediatric low-grade neoplasms, specifically in intramedullary spinal cord tumors. We report two cases of malignant transformation in low-grade neuroepithelial tumors of the pediatric intramedullary spinal cord. Two children with intramedullary tumors, one with a WHO grade I ganglioglioma and one with a low-grade astrocytoma, were treated surgically, diagnosed histologically, and followed through the course of their disease. Both patients’ tumors transformed to higher grades without prior irradiation or chemotherapy, and without a genetic predisposition to tumorigenesis. Malignant transformation can occur in low-grade intramedullary neoplasms in children. This is a novel documented event for pediatric intramedullary spinal cord tumors and a rare event for all pediatric low-grade neuroepithelial tumors without induction by irradiation. A survey of the relevant literature reveals an underwhelming number of studies focusing on malignant transformation in children’s CNS tumors relative to adults. Further investigation into molecular mechanisms of pediatric low-grade neoplasms may reveal more aggressive tumor sub-variants predisposed to malignant degeneration.

Object The rationale for using endoscopy to treat complex cysts and multiloculated hydrocephalus is to combine several CSF compartments into a minimum number, establish a connection to functioning CSF compartments (that is, ventricles), and decrease shunt dependency. The aim is to decrease the number of proximal shunt catheters, the number of shunt revisions, and in selected cases even to avoid a shunt. In cases of distorted anatomy and multiloculated cysts, endoscopy may be problematic because of orientation issues. Standard navigation becomes useless soon after CSF loss due to brain shift. Therefore, the concept of “real-time” navigation and intraoperative imaging in combination with endoscopic surgery has been previously suggested. The goal of the present study was to assess the feasibility and efficacy of combining intraoperative MR (iMR) imaging and navigated neuroendoscopy in infants. Methods The authors report their experience in treating 5 infants (aged 6–14 months), who underwent surgery for multicystic hydrocephalus presenting with shunt malfunction (4 patients) and a quadrigeminal fetal arachnoid cyst (1 patient). In all infants, a low-field portable iMR imaging system (0.12-T PoleStar N-10/0.15-Tesla PoleStar N-20) was used in conjunction with navigated endoscopy. The authors used e-steady, T1-weighted, and T2-weighted sequences (acquisition time 24 seconds to 3.5 minutes). Results The iMR imaging system provided clear images that correlated with the endoscopic appearance of the cystic membranes in all patients, and the images were helpful in determining trajectories and redefining targets. The iMR images documented brain shift and changes in CSF spaces during surgery. There were no intraoperative complications or technical difficulties of visualization. No infection or any other immediate postoperative complication occurred. Patients were followed up for 9 months to 7 years. The infant presenting with the quadrigeminal cyst remains shunt free since surgery, and the patients with multicystic hydrocephalus have 1–2 shunts each. Following endoscopic, iMR imaging–guided surgery, shunt catheter positioning was found to be optimal and as planned according to the postoperative imaging. Conclusions Navigated neuroendoscopy and iMR imaging may complement each other, offering an advantage over other modalities in complicated cases of hydrocephalus. Whenever targets and trajectories need to be redefined, the iMR images provided an updated navigation data set, allowing accurate navigation of the endoscope and minimizing the number of CSF compartments. Direct vision through the endoscope provides microanatomical details for the optimization of fenestration and catheter positioning. The combined usage of the two modalities may transform a conventional procedure into a visually controlled real-time navigated process.

Pediatric brain tumors (PBTs) are the most common solid tumors and the leading cause of cancer-related morbidity and mortality in childhood. Previous studies have shown a significant delay between the onset of symptoms and the diagnosis of these tumors. Delayed diagnosis of PBTs may lead to acute situations and irreversible neurological damage. In this study, we looked for the incidence of delayed diagnosis of PBTs in Israel. We tried to find the reasons for these delays and the associated risk factors in order to provide a feedback to the system for improved education and earlier diagnosis.We analyzed the charts of 330 consecutive children aged 0-18 years diagnosed with brain tumors, between the years 1996 and 2004. In the cases where delay in diagnosis was suspected, further information was collected from a family interview.The average "time to diagnosis" was 7.7 months (SD ± 16.7). Symptomatic deterioration from the first symptom until diagnosis was found in about 50% of the cases. Unacceptable delay in diagnosis was found in 27% of the children. The major reason for delay was "delay in indicated imaging." Symptoms that were found to be associated with delayed diagnosis were torticollis, ataxia, and motor dysfunction. Interestingly, the examination by specialists such as ophthalmologists or neurologists was also associated with delayed diagnosis.There is an unacceptable rate of delay in the diagnostic process of PBTs in Israel. Greater awareness and familiarity with signs and symptoms associated with these tumors and lowering imaging threshold might minimize this phenomenon.

This study evaluates the safety, efficacy, and indications for continuous lumbar drainage (CLD) in patients following endoscopic third ventriculostomy (ETV).We retrospectively reviewed the clinical data of 22 consecutive patients treated between 1996 and 2010 with CLD after ETV. The decision to insert a CLD was made in selected patients only. CLD was inserted in cases of high measured intracranial pressure (12 patients), clinical symptoms indicative of continuing hydrocephalus (2 patients), and "prophylactically" in 8 patients, based either on the clinical condition of patients before ETV or on technical difficulties during the ETV procedure, which seemed to increase the risk of ETV failure. CLD insertion took place either in the operating room immediately following the ETV procedure or under very specific conditions and with close patient monitoring in an ICU setting. Only four patients eventually required shunting, all within 1 month after ETV. Therefore, the overall ETV success rate was 81.8% (18/22 patients). Of the 14 patients suffering from measured or clinically observed continuing hydrocephalus, 12 (85%) ultimately recovered without the need for a permanent shunt. Without the CLD, some of these patients would probably have been declared "failures" and referred for a standard shunt. CLD provided a time window following ETV for the absorption system to recover and return to full functionality.Selective usage of CLD is a reasonable and safe method to gain time and possibly facilitates the recovery of absorption capacity following ETV. CLD should be considered before conceding a post-ETV patient as a failure.

In most of the children with posthemorrhagic hydrocephalus (PHH), multidisciplinary follow-up is performed, with the focus on consequences of prematurity, cerebral palsy (CP) and hydrocephalus. A large fourth ventricle is common in these children but imaging performed in order to document ventricles and tissue damage is not oriented to exclude coexisting rare pathologies. We report a 3-year-old child with spastic CP, secondary to prematurity and PHH. A ventriculoperitoneal shunt was inserted at the age of 2 months. On follow-up imaging the child demonstrated well-drained supratentorial ventricles with a persistent large fourth ventricle. Because of a neurological change in spasticity and new-onset torticollis, a repeat MRI was performed, suggesting a cystic, nonenhancing lesion of the fourth ventricle. The surgical exploration revealed a large dermoid of the fourth ventricle. We analyze the differential diagnosis of a clinically significant large fourth ventricle in a shunted child with PHH and CP. This includes conditions without pressure in the posterior fossa such as tissue loss due to cerebellar atrophy, or pathologies causing a true increase in pressure of the fourth ventricle (isolated fourth ventricle, cystic lesions and neoplasms of the fourth ventricle). Neurologically compromised children pose additional challenges in reaching a definitive diagnosis and hence require a careful regular assessment of their clinical status with additional well-timed imaging with appropriate protocols to allow appropriate treatment when indicated and to avoid morbidity due to delayed diagnosis. We present a rare coexistence of a dermoid tumor within the fourth ventricle in a CP child with PHH and express the dilemmas associated with its management.

Object Trapped fourth ventricle (TFV) is a rare late complication of postinfectious or posthemorrhagic hydrocephalus. This entity is distinct from a large fourth ventricle because TFV entails pressure in the fourth ventricle and posterior fossa due to abnormal inflow and outflow of CSF, causing significant symptoms and signs. As TFV is mostly found in children who were born prematurely and have cerebral palsy, diagnosis and treatment options are a true challenge. Methods Between February 1998 and February 2007, 12 children were treated for TFV in Dana Children's Hospital by posterior fossa craniotomy/craniectomy and opening of the TFV into the spinal subarachnoid space. The authors performed a retrospective analysis of relevant data, including pre- and postoperative clinical characteristics, surgical management, and outcome. Results Thirteen fenestrations of trapped fourth ventricles (FTFVs) were performed in 12 patients. In 6 patients with prominent arachnoid thickening, a stent was left from the opened fourth ventricle into the spinal subarachnoid space. One patient underwent a second FTFV 21 months after the initial procedure. No perioperative complications were encountered. All 12 patients (100%) showed clinical improvement after FTFV. Radiological improvement was seen in only 9 (75%) of the 12 cases. The follow-up period ranged from 2 to 9.5 years (mean 6.11 ± 2.3 years) after FTFV. Conclusions Fenestration of a TFV via craniotomy is a safe and effective option with a very good long-term outcome and low rate of morbidity.

Slit ventricle syndrome (SVS) remains a major problem for early shunted children. Several conservative and surgical treatment paradigms have been suggested; however, there is no consensus on the optimal surgical treatment. We present our experience using bilateral subtemporal decompressions with dura and arachnoid opening for the treatment of a subgroup of children with severe and resistant SVS. Fifteen children with severe and resistant SVS underwent a modified bilateral subtemporal craniectomy, with dura and arachnoid opening. Their clinical and radiological data were retrospectively reviewed. Seven (46.6%) patients had a complete recovery from their symptoms with a follow-up of 5.9 ± 2.6 years.The remaining eight (53.3%), underwent additional surgeries. Four (26.6%), had a single proximal shunt revision after dilatation of their ventricles. Following these procedures these four children are well and stable with a follow-up of 1.8 ± 2 years. The other four had further cranial vault expansion, one of which was followed by a proximal shunt revision. Thus, 11 of these 15 patients (73.4%) had a very good outcome, attributable to this technique, with a mean follow-up of 4.5 ± 3 years. Modified bilateral subtemporal decompression with dura and arachnoid opening yields a high cure rate for severe and resistant slit ventricle syndrome. Proximal shunt revision may be safely performed in a subset of patients that dilate their ventricles following the procedure. Further cranial expansion may be reserved for children with recurrent SVS symptoms who do not respond to STD and remain with very small ventricles.

Objective To reassess the utility and validity of ultrasound (US) screening in infants with lumbar midline skin stigmata (MSS) that may be associated with tethering of the spinal cord. Study design We conducted a prospective observational study of 254 infants under age 6 months with suspicious dorsal MSS between 2005 and 2007. All infants were examined by US and neurosurgical clinical evaluation, and 50 infants also underwent magnetic resonance imaging (MRI). The US and MRI findings were analyzed for correlation. Associations between the imaging findings and the presence of the low-risk skin lesions simple dimple (113 cases) and deviated gluteal fold (DGF; 44 cases) also were evaluated. Results Analysis of US and MRI results for the cohort of 50 neonates in whom both examinations were performed showed high concordance. The low-risk group of infants with simple dimple and DGF constituted 157 US procedures, 96% of which were of high quality, providing clear visualization of spinal components. None demonstrated any clinically significant pathological findings. Conclusions Our data reaffirm the reliability of US as a screening tool for tethered cord syndrome. Infants with low-risk lesions, such as simple dimple and DGF, may be absolved from US screening, because these findings alone do not indicate underlying pathological lesions. We propose a simplified diagnostic classification system for MSS.

Intraoperative ultrasound (IOUS) serves as a basic imaging tool in neurosurgery. However, its low quality and difficulty in interpreting the images make its use as a resection control tool and navigation system cumbersome. We present our experience using a high-resolution 3D IOUS system combined with a navigation system in pediatric cranial surgery. We retrospectively reviewed 16 pediatric neurosurgical procedures in which a high-resolution 3D IOUS combined with a navigation system was used. The system enables basic navigation based on preoperative computed tomography or magnetic resonance imaging scans. In addition, IOUS images serve as a data set for updated intraoperative navigation. The indications for IOUS were preoperative navigation to define the skin incision and exact craniotomy site, and for real-time neuronavigation and resection control during tumor removal. The added time per case was short and no technical difficulties were encountered. High-resolution 3D IOUS combined with navigation systems has advantages for the pediatric neurosurgical population, including both real-time basic navigation and improved resection control.

Neuronavigation has become a cornerstone of neurosurgery. Navigation systems are categorized into two main groups: those based on preoperative imaging and those based on real-time intraoperative acquired images.The preoperative imaging systems, either computed tomography (CT)- or magnetic resonance imaging (MRI)-based, are straight-forward systems that are routinely used in most institutions. Image accuracy, however, decreases secondary to brain and lesion shifts that occur during surgery. Intraoperative, real-time navigation systems overcome anatomical shifts by updating the image base of the navigation during surgery, thus, maintaining precise navigation capabilities throughout the surgical procedure.In this article, we review the main neuronavigation systems and their applications, emphasizing their unique advantages and usage within the pediatric population.

Objectives: Intraoperative MRI (iMRI) is used mainly in the adult neurosurgical population. The main indications for iMRI usage are resection control and updated intraoperative navigation capabilities. In this paper we present our experience using this technique in children. Specific advantages of iMRI for this age group are discussed. Methods and Results: We retrospectively reviewed 31 pediatric neurosurgical procedures in which a portable iMRI system was used. The indications for iMRI usage were preoperative navigation, resection control during tumor removal, shunt placements, and needle biopsy. In 7 children the use of the iMRI changed the course of the surgical procedure. Operative morbidity and mortality were not increased with use of the iMRI. Conclusions: iMRI systems have advantages for the pediatric neurosurgical population, including both real-time basic navigation and improved resection control.

Objective To determine if the severity of antenatally diagnosed hemorrhagic fetal brain insults and fetal stroke detected by ultrasound and magnetic resonance imaging (MRI) predicts postnatal neurodevelopmental prognosis. Methods The in-utero presentation and postnatal neurodevelopmental outcome of sonographically detected subdural hematoma or fetal stroke presenting as intraventricular hemorrhage (IVH) or intraparenchymal brain hemorrhage were investigated. Results Of 33 fetuses diagnosed with hemorrhagic brain lesions, 17 were electively terminated and two suffered intrauterine fetal demise. Thirteen were liveborn, seven by Cesarean delivery and six by spontaneous vaginal delivery. One case was lost to follow-up. Eight neonates had moderate to severe neurological deficit by a mean age of 35 (range, 6–96) months. One died at 2 months of age. These nine were diagnosed with Grade III–IV IVH in utero. Four neonates had normal neurological outcome by a mean age of 41 (range, 30–48) months; these four were diagnosed with subdural hematoma (n = 1) or Grade I–II IVH (n = 3) in utero. Fourteen cases were followed up with MRI, which confirmed ultrasound findings in 10 (71%) cases. In three (21%) cases MRI diagnosis was more accurate and the severity of grading was greater than that obtained on ultrasound imaging. Unilateral left hemispheric lesions were much more common than right-sided lesions (13 vs. 1, respectively). Conclusions An antenatal sonographic diagnosis of fetal stroke with IVH Grade III–IV or with brain parenchymal involvement appears to be associated with poor neurological outcome. MRI may contribute to the accuracy of diagnosis, particularly in Grade II and III lesions. Left-sided unilateral lesions are more common than right-sided ones. Copyright © 2005 ISUOG. Published by John Wiley & Sons, Ltd.

The objective was to report our positive experience of using dexamethasone to treat 13 patients with symptomatic slit ventricle syndrome (SVS).Thirteen SVS patients who received dexamethasone during acute episodes were studied. The etiology for hydrocephalus was prematurity and intraventricular hemorrhage in 9 patients and neonatal meningitis, chorioamnionitis, Dandy-Walker variant, and congenital in 1 case each. The shunt was inserted at 1.8+/-1.0 months of age and SVS was diagnosed at 4.9+/-3.2 years of age.All patients reported relief and shorter duration of symptoms with dexamethasone. Surgical intervention was decided upon and carried out within 11+/-8 months of SVS diagnosis in 9 out of 13 patients. The other 4 are being monitored and continue to receive dexamethasone when needed.Dexamethasone appears to be a useful treatment in acutely increased intracranial pressure caused by SVS. It can provide temporary relief during the decision-making process of whether and when to perform surgery.

The femtomolar-acting eight-amino-acid peptide (NAP), derived from activity-dependent neuroprotective protein (ADNP), provides long-term protection against the deleterious effects of closed head injury (CHI) in mice. Fifteen minutes after injury, mice were divided into two groups, control and NAP-treated and a single subcutaneous injection of NAP or vehicle was administered. A third group served as sham-treated (not subjected to head trauma). Each mouse was assessed for its clinical function, using neurological severity score, at various time intervals following CHI, up to 30-45 d. Total cerebral cortex RNA was prepared from the site of injury of CHI mice, and from parallel regions in peptide-treated and sham brains. RNA was then reversed transcribed to yield radioactive cDNA preparations that were hybridized to Atlas array membranes containing 1200 cDNAs spots. Comparison of sham-treated individual mice showed differential expression levels of at least 15 mRNA species. Furthermore, results indicated that one of the genes that did not change among individuals but specifically increased after CHI and decreased after NAP treatment was the cell surface glycoprotein Mac-1 (CD11B antigen). Thus, Mac-1 is suggested as a marker for the long-term outcome of head injury and as a potential target for NAP protective actions.

Background. Even though vestibular schwannomas rarely present during pregnancy, symptoms may appear or worsen particularly in this period. The clinical picture may include tinnitus, hearing abnormalities, and in large tumors, brain-stem and cerebellar compression with involvement of additional cranial nerves. Large vestibular schwannomas (also known as Acoustic Neurinomas) present a great challenge in peripartum management of both the mother and the fetus. Material and method. We present a case of a 24-year old woman, with headache, papilledema, ataxia, and multiple cranial nerve weakness, diagnosed in the 35th week of pregnancy. MRI demonstrated a huge vestibular schwannoma compressing the brainstem and causing obstructive hydrocephalus. Result. In the presence of high intra-cranial pressure a ventriculo-peritoneal shunt was first inserted, enabling delay of tumor surgery until after delivery. A successful elective cesarean section followed at 37 weeks, and radical tumor surgery was performed a week later. Maternal and fetal outcome were excellent. Discussion. The options, sequence and timing of the neurosurgical and obstetrical interventions are discussed. Other reports of large vestibular schwannomas that presented during pregnancy are reviewed. Advances in neurosurgery, neuroradiology, neuroanesthesiology and obstetrics are highlighted, and their impact on outcome is discussed in comparison to the poor results reported in the past. Emphasis is made on the importance of early diagnosis, that necessitates high-index of suspicion by the obstetrician, in any pregnant woman presenting abnormal neurological signs. Conclusion. We conclude that with a cooperative team approach, maternal and fetal prognosis can today be excellent, even in cases of large vestibular schwannomas diagnosed in the late stage of pregnancy.

We report a bizarre presentation of a distal shunt malfunction in a 5-year-old child with a ventriculoperitoneal shunt. The plain radiographs done as a workup for possible shunt malfunction demonstrated the distal shunt tip reentering the abdominal wall from inside the peritoneal cavity. We discuss the possible explanation for the phenomenon. We conclude that the case is just another reminder of the fact that shunt malfunctions can have multiple presentations; hence, a regular follow-up with appropriate imaging and high index of suspicion is mandatory in preventing morbidities.

Cystic pilocytic astrocytomas (CPA) consist of a mural nodule and an accompanying cyst, which may prominently enhance on MRI after contrast administration. This raises the question whether an enhanced wall represents a tumor and thus should be resected together with the solid nodule, as radical tumor surgery is associated with better prognosis. Until now, no systematic histopathological examinations of cyst walls have been reported in correlation with MRI, intraoperative appearance and postoperative clinical and MRI follow-up. We present 3 patients with CPAs and brightly enhanced cyst walls on MRI. Because of the benign, transparent appearance of the cyst wall intraoperatively, it was biopsied but not resected, and only radical removal of the nodule and its immediate surroundings was performed. Separate specimens taken from the cyst wall showed no tumor. MRI performed annually, up to 48–56 months after surgery showed no recurrence of the cyst or the tumor. In such cases of CPA, we suggest that enhancement of cyst walls may represent reactive rather than tumoral tissue, and may be left intact without risking worse prognosis. Mechanisms leading to cyst wall enhancement and the optimal surgical treatment are discussed.

Background: Epidural hematoma (EDH) in infants may be difficult to diagnose. The mechanism of injury and the clinical presentation are different from those in older children. We characterized the clinical and radiologic parameters of EDH in infants and correlated them with outcome. Because there are no optimal prognostic tools or clear guidelines to perform computed tomography in this unique population, a new approach of neurotrauma scoring is suggested. Methods: Eleven infants (

✓ A multilocular extradural cervical spinal hydatid cyst that causes severe spinal cord compression and quadriplegia is relatively rare and difficult to treat. In a patient with this disorder, computerized tomography—guided needle aspiration of the cyst loculations and irrigation using hypertonic saline eliminated the need for emergency surgery and provided complete resolution of the patient's quadriplegia. The subsequent course of the disease was controlled by treatment with albendazole. Magnetic resonance imaging performed 4 months after the procedure demonstrated collapsed cysts and absence of spinal cord compression.

Cerebrospinal fluid (CSF) cranial leaks in neonates are rare, and only four case reports have been published previously. Two of the previous cases were also in preterm newborns delivered at 30 weeks gestation. We present a case of CSF leak in a premature neonate delivered at 30 weeks gestation. Our approach to treatment was different from that previously described. After the initial treatment with local packing bandage failed to stop CSF leakage, local exploration with wound suturing was done, with resultant ceasing of leakage within 12 hours of birth. This case presents a rare complication of CSF leakage caused by internal scalp monitoring. This complication seems to be more frequent in premature babies, thus special consideration should be taken in premature infants before applying such monitoring.

In this article, Garel et al discuss the main steps of cerebellar development, describe the normal imaging pattern of the fetal cerebellum (by ultrasonography [US] and magnetic resonance imaging [MRI]), and present the main pathologies of the fetal cerebellum as may be diagnosed by fetal imaging—all in an attempt to create a unified terminology for practical aspects of neurofetal counseling. Neurofetal counseling is becoming an important part of the practice of the pediatric neurologist, neurosurgeon, geneticist, and neonatologist. It is truly an issue that has to be addressed by a multidisciplinary task force to combine understanding of neurofetal imaging, laboratory results, postnatal neurodevelopment, and treatment options of certain pathologies—all to facilitate a practical tool for appropriate decision making when a fetal brain abnormality is suspected by fetal ultrasound. Termination of pregnancy is only one aspect of neurofetal counseling; even if abortion is not a valid option in some countries or traditions, counseling is still important, as understanding the precise pathology in a fetus may shed light on a misdiagnosed familial/genetic pathology and may have a practical impact on future pregnancies in the same family. Moreover, neurofetal counseling deals with timing and mode of delivery, to be decided together with the perinatologist, and includes recommendations about further fetal follow-up until expected delivery. For example, progressive ventriculomegaly with increased head circumference and biparietal diameter may dictate elective cesarean section as soon as maturity of the lungs is expected. One of the most important issues for the family is the postnatal prognosis; and in some countries where abortion is allowed, termination of pregnancy may be relevant even in very late stages of gestation if the anticipated prognosis is bad. Thus, a multidisciplinary approach with a common language between the gynecologist/perinatologist, radiologist, and practitioners in all other disciplines is a critical tool for appropriate management. Several factors have changed modern neurofetal counseling: improved ultrasonography including three-dimensional images, the introduction of fetal MRI during the late 1990s, genetic information that is accumulated constantly, and our ability to use late-outcome data regarding neurodevelopment of fetuses suspected to have brain abnormalities. Our treatment options have also improved: preimplantation genetic diagnosis, fetal treatment mainly by fetal surgeries (eg, in fetal meningomyelocele), and major progress in postnatal-surgical options in some fetal pathologies (eg, obstructive hydrocephalus and intracranial cysts, introducing neuroendoscopy into the armamentarium of the pediatric neurosurgeon). Still, the main issue in fetal neurocounseling is to provide a reliable prognosis to an abnormal appearance of a fetal brain. For this to occur, two preconditions are mandatory: (1) better understanding of the ultrasonographic images and (2) accurate interpretation of such images by using a common language among different consultants from several disciplines. One of the main fields with pitfalls in diagnosis and prognostication is the fetal posterior fossa, including the abnormalities of the fetal cerebellum. Magnetic resonance imaging has dramatically changed our understanding of the developing fetal cerebellum and our ability to demonstrate not only anatomy but also processes. A fascinating example is the demonstration of secondary chiari 2 malformation in fetuses with a meningomyelocele that is resolving after fetal surgery. However, issues such as the timing of MRI to demonstrate the posterior fossa and indications for fetal brain MRI are not fully resolved. Thus, even with better quality magnetic resonance images that depict anatomy more accurately, final diagnosis is not always possible. The importance of the cerebellum in normal neurodevelopment is well acknowledged, thanks to several recent reports regarding the association between abnormal child development and anatomical damage to a potentially normal cerebellum in a developing brain (eg, in premature babies with cerebellar hemorrhage). The strong association between abnormal findings

The rate of brain abnormalities in asymptomatic term neonates varies substantially in previous studies. Some of these rates may justify general screening of healthy newborns by head ultrasound (HUS).To assess the incidence of intracranial abnormalities among asymptomatic term newborns with HUS and to detect high-risk populations that might need such screening.This was a prospective study in 493 term newborns who underwent HUS and a neurological evaluation during the first 3 days of life. The neurological examination results were unknown to the sonographist and the examiner was blinded to the HUS findings. The abnormal HUS findings were classified as significant or non-significant according to the current literature.Abnormal HUS was found in 11.2% of the neonates. Significant findings were noted in 3.8% of the infants. There was no association between non-structural HUS findings (hemorrhage or echogenicity) and mode of delivery. There was no relationship between any HUS abnormality and birth weight, head circumference and maternal age, ethnicity, education or morbidity. The rate of abnormal neurological, hearing or vision evaluation in infants with a significant abnormal HUS (5.2%) was comparable to the rate in infants with normal or non-significant findings on HUS (3.1%).There is no indication for routine HUS screening in apparently healthy term neonates due to the relatively low incidence of significant brain abnormalities in these infants in our population.

Endoscopic third ventriculostomy (ETV) has become standard for obstructive hydrocephalus. Even a successful ETV can obstruct, leading to recurrence of symptoms and even death. A possible solution to this problem is leaving an Ommaya reservoir (OR) following the ETV. OR can be tapped in an emergency and for diagnostic purposes. No specific complications have been attributed to OR in this setting. We present our experience with OR in children undergoing ETV for hydrocephalus.A retrospective study was conducted in hydrocephalic children that underwent ETV with OR insertion over 13 years (1997-2010) from a single institution. Data were collected from charts and follow-ups.Twelve patients (from 200 patients who had an ETV) underwent placement of OR with ETV. OR was reserved for a subgroup of patients in whom we anticipated complications-in children that presented with acute hydrocephalus and were in deteriorating condition, for pathologies believed to have a low predicted ETV success rate, or when the surgeon felt that the ETV procedure was suboptimal. OR was tapped in eight patients. Complications occurred in four patients: two cases of subdural effusion, one case of chronic subdural hematoma, and one CSF leak. Four ORs were removed due to complications, and four were converted to shunts.OR should be considered in selected patients undergoing ETV. Despite its obvious advantages, OR may be associated with a relatively high risk of extraaxial fluid collections. This association requires further investigation.

Arachnoid cysts occupying the suprasellar region comprise 10-15% of intracranial distribution. Unlike large suprasellar cysts, pure interpeduncular cysts (IPCs) are rare, and their natural history is unknown. We describe a small series of children diagnosed with IPC and their long-term natural history.A retrospective review was conducted of interpeduncular arachnoid cysts diagnosed over the years 2000-2010 at our center. Patients with clearly suprasellar cysts were excluded. Serial magnetic resonance imaging and long-term follow-up examinations were analyzed. Additionally, we conducted an extensive literature review focusing on the differences between suprasellar cysts and IPCs.We identified three pediatric patients with "pure" IPC; all of these had a follow-up of more than 5 years, and none was operated. Only six additional cases were identified in the literature. In both our experience and in the literature review, IPCs proved stable over the course of time, both radiologically as well as clinically.The clinical and radiological features of IPCs are not well defined. Variations in the relationship of arachnoid cysts in this area to Liliequist's membrane may explain the different subgroups that have been identified as well as the confusing nomenclature. IPCs are usually diagnosed as incidental findings or present with mild endocrine disorders. Associated findings of hydrocephalus, mass effect, and compression of neighboring structures, such as the chiasm, are not as frequent as with suprasellar cysts. Given the high likelihood of continuing stability, a conservative strategy of follow-up is recommended for pure IPCs that demonstrate preservation of the third ventricle.

We designed a survey to investigate current international management trends of neonates with lumbar midline skin stigmata suspicious of tethered cord, among pediatric neurosurgeons, focusing on the lower risk stigmata, simple dimples, deviated gluteal folds, and discolorations. Our findings will enable physicians to assess their current diagnosis routine and aid in clarifying management controversies.A questionnaire on the proposed diagnostic evaluation of seven case reports, each accompanied by relevant imaging, was distributed by e-mail to members of the International Society for Pediatric Neurosurgery, the European Society for Pediatric Neurosurgery, and via the PEDS server list between March and August 2008.Sixty-two questionnaires, completed by experienced professionals with a rather uniform distribution of experience levels, were analyzed. Forty-eight percent do not recommend any imaging of simple dimples, 30% recommend US screening and 22% recommend MR. Seventy-nine percent recommend imaging of deviated gluteal fold with 30% recommending MR. Ninety-two percent recommend imaging infants with hemangiomas with 74% recommending MR. MR for sinus tracts is recommended by 90% if sacral and by 98% if lumber. Eighty-four percent recommend MR for filar cyst.Our survey demonstrates that management of low-risk skin stigmata, simple dimple, deviated gluteal fold, and discolorations lacks consensus. In addition, a significant sector of the professional community proposes a work-up of simple dimples, sacral tracts, and filar cysts that contradicts established recommendations. A simple classification system is needed to attain a better approach, enabling correct diagnosis of tethered cord without exposing neonates to unnecessary examinations.

Post-traumatic cerebrospinal fluid leak from the anterior cranial fossa in children may be isolated or combined with severe facial and calvarial injury. Untreated leak may result with meningitis, hydrocephalus, and abnormal neurocognitive development. We present nine children, ages 4–16 years, with complicated craniofacial injury treated by a combined subcranial and intracranial approach. A continuous lumbar drainage was kept for several days, and prophylactic antibiotics and anti-convulsive medications were routinely given. A multidisciplinary approach including discussion before surgery about other surgical options (endoscopic extracranial and intracranial alone) were performed. None of the operated children had episodes of meningitis/leak after the combined approach, suggesting that appropriate sealing of the base of the skull has been achieved. There was no mortality, and the long-term follow-up showed good developmental and cosmetic results. Most of the children had significant brain contusions prior to surgery; however, these did not progress as minimal retraction was enabled by the extensive subcranial and intracranial approach. Child’s age, anatomy of the bone, extent of cranial injury, and clinical parameters should be seriously considered when choosing the technical methods as for sealing base of skull and reconstruction of facial/cranial bones. Young age does not seem to be a contraindication to the combined approach, thus, we recommend considering it in extensive base of skull fractures when concomitant cranial, maxillofacial, and orbital fractures coexist, as alternative options may not suffice in these cases.

Dermal sinuses are congenital malformations, representing a subtype of occult spinal dysraphism (OSD). A short historical review presented by [1] cited the first description of dermal sinuses by Ogle in 1865. The first description in English literature was by Verebely in 1913. The incidence of dermal sinuses is frequently noted as 1:2,500 live births [2, 3]. However, this number is based on two studies from 1954 and 1975, both of which were done before the MRI era, and before it was recognized that coccygeal pits and dermal sinus are two distinct entities. Thus, the true incidence is not known [1]. Dermal sinuses are thought to develop in response to an abnormal separation of the cutaneous and the neural ectoderm between the third and fifth weeks of gestation [4, 5, 6]. When this separation fails, a persistent connection, or tract, occurs between the skin and deeper structures. Depending on the degree of incomplete separation, the deeper end of the tract may reach subcutaneous tissue, fascia, dura, or even intradural neural tissue. Another possible underlying mechanism is a defect in notochordal formation with sagittal splitting of the spinal cord and persistence of a cutaneo-endo-mesenchymal fistula.

Memory and neurobehavioral dysfunctions are among the sequelae of traumatic brain injury (TBI). The Neurological Severity Score (NSS) includes 10 tasks and was previously designed to assess the functional status of mice after TBI. The object recognition task (ORT) measures specific episodic memory and is expressed by the percent time spent by an animal at a novel, unfamiliar object (Discrimination Index [DI]). It is an ideal tool for evaluating cognitive function after TBI. The present study sought to validate the use of the NSS and ORT in severe and mild focal TBI in mice, and to confirm that the spontaneous recovery and the radiological abnormalities, shown by T2-weighted magnetic resonance imaging (MRI), are dependent upon injury severity. Mice were subjected to severe and mild closed head injury (NSS at 1 h 7.52 +/- 0.34 and 4.62 +/- 0.14, respectively). NSS was evaluated for 25 days and showed a decrease by 3.86 +/- 0.26 and 2.54 +/- 0.35 units in the severely and mildly injured mice, respectively. ORT revealed DI in severely injured group of 51.7 +/- 6.15%, (vs approximately 75-80% in uninjured animal) on day 3 and 66.2 +/- 6.81% on day 21. In contrast, the mildly injured mice did not show cognitive impairment throughout the same period. The damage seen by MRI at 24 h after injury, strongly correlated with NSS(1h) (R = 0.87, p0.001). We conclude that NSS is a reliable tool for evaluation of neurological damage in head-injured mice, NSS(1h) predicts the motor dysfunction, cognitive damage, and brain-damage characteristics as depicted by T2-weighted MRI. The combined assessment of neurobehavioral and cognitive function along with MRI is most useful in evaluating recovery from injury, especially when testing effectiveness of novel treatments or genetic manipulations.

The standard treatment for hydrocephalus is a ventriculoperitoneal shunt which is associated with high rate long-term morbidity. Seeking alternative treatments, neuroendoscopic third ventriculostomies (ETV) have been performed in patients with obstructive hydrocephalus. During this procedure, the third ventricular floor is perforated thus bypassing the pathological obstruction. In this procedure, no hardware is left and fluid pressures are more physiologically balanced compared to shunted cases.To present our experience with endoscopic third ventriculostomy (ETV) in patients below the age of 18 years.Retrospective analysis of 112 ETV's: in 89 cases (group A) the hydrocephalus was secondary to aqueductal stenosis (primary or secondary). In 23 cases (group B) the hydrocephalus was secondary to other reasons.A total of 70% of patients in group A did not need a shunt during their follow-up in comparison with 56% in group B. In a multivariable analysis--the only significant predictor for failure of the ETV was age below two years at the time of the procedure. Two patients died perioperatively. Both suffered from malignant disseminated tumors. One death was related to oncological reasons; the other death was related to intraventricular bleeding secondary to the procedure. Perioperative morbidity was 23% (decreased to 8% during recent years). No permanent morbidity occurred.Endoscopic third ventriculostomy is the treatment of choice for obstructive hydrocephalus secondary to aqueductal stenosis among patients older than two years. In younger patients, future studies are needed to prove whether a shunt or a ventriculostomy are the preferred treatment.

The authors' aim in this study was to review their experience in the use of indirect revascularization alone in a series of 14 children with moyamoya disease, in which numerous bur holes and arachnoid openings were made over each affected hemisphere.Revascularization through multiple bur holes and arachnoid openings was performed in 14 children (mean age at diagnosis 6.5 years [range 3-15 years]) who suffered from progressive moyamoya disease. The authors performed surgery in a total of 24 hemispheres during 18 procedures. Ten children underwent bilateral multiple bur hole procedures, three underwent a unilateral procedure in the more severely affected hemisphere, and one child had previously undergone an encephaloduroarteriomyosynangiosis on the contralateral side. Ten to 24 bur holes were made in the frontotemporoparietooccipital area of each hemisphere, depending on the site and extent of the disease. Early postoperative perfusion magnetic resonance imaging studies, performed in the five most recent cases, showed restoration of cortical perfusion as early as 3 months, which was confirmed on subsequent angiography studies (performed between 8 and 12 months postoperatively) that showed excellent revascularization of the ischemic brain by external carotid artery collateral vessels. None of the children sustained further ischemic attacks postoperatively. Motor improvement was noted in those who had presented with paresis. A single seizure episode occurred in two patients at 2 weeks and 5 months after surgery; both children had presented with epilepsy. There were no postoperative deaths, and only one complication (an infected lumbar shunt in the patient who required cerebrospinal fluid [CSF] drainage). Five of the 18 procedures were complicated by subcutaneous CSF collections, which resolved with tapping and compressive head dressings; a transient lumbar drain was necessary in one case.The results obtained in this series suggest that in children with moyamoya disease this simple technique is both effective and safe. Furthermore, it is effective as a sole treatment without supplementary revascularization procedures.

Object. The goal of this study was to determine the importance magnetic resonance (MR) imaging holds as a complementary fetal imaging modality to ultrasonography in deciding postnatal neurosurgical management. Methods. Between 1999 and 2003, 320 fetal MR imaging studies were performed at a single institution. Twentyfour fetuses were found to have central nervous system abnormalities that could potentially require a neurosurgical intervention. The diagnoses included spinal anomalies (scoliosis, myelomeningocele, and closed spinal dysraphism) and brain anomalies (ventriculomegaly with or without hemorrhage, intracranial cyst, craniosynostosis, and encephalocele). Fourteen of the 24 fetuses underwent surgery based on findings of prenatal MR imaging. In seven cases the pregnancy was terminated, and in three cases conservative follow up continues. Conclusions. In a variety of brain and spine disorders, prenatal MR imaging can delineate and characterize the abnormality, and thus assist in the diagnosis and in the planning of postnatal surgery and management. This modality provides important multiplanar images and may obviate the need for early postnatal computed tomography or MR imaging. Postnatal management can often be guided by prenatal MR imaging findings.

The authors discuss the indications for and timing of a diagnostic neurosurgical procedure in children with diabetes insipidus (DI) and a thickened pituitary stalk (TPS) on magnetic resonance (MR) imaging.Seven children with a TPS who presented with DI eventually underwent surgery for diagnostic purposes. The ages at onset of DI were 6 to 16 years, and the follow-up period until surgery was 26.9 +/- 11.9 months. In four of seven children, the stalk appeared normal on the first MR image, but it was thickened and variably enhancing on later images in all instances. The reason for eventual surgery was endocrinological deterioration in two of seven children, radiological progression in two children, and a combination of the two in three children. Three children experienced visual disturbances and four children had optic nerve, chiasma, or hypothalamus involvement. All children suffered additional endocrinological abnormalities pursuant to the initial DI. A definitive diagnosis was achieved in six of seven children: germinomas in five and Langerhans cell histiocytosis in one. One child had lymphocytic infiltrate. None of the children deteriorated neurologically or endocrinologically after the operation. On follow up, vision deficit was irreversible in all children who demonstrated visual abnormalities before treatment.Surgery should be performed in children with a TPS and DI for early diagnosis and disease-oriented therapy when there is further endocrinological, radiological, or clinical deterioration. The complication rate is low in open biopsies, and histological diagnosis is achieved in most of the cases. All children who present with central DI must undergo head MR imaging, and even if results are normal, close radiological and clinical follow up is mandatory.

The classification of hydrocephalus in newborns and in infants is different from the classification in adulthood. This difference exists due to disparity in the source pathologies that produce the hydrocephalus, and the practical distinctions in prognosis and treatment choices. The objective of this paper is to present the spectrum of obstructive-communicating hydrocephalus, which is more complex in the pediatric group, and to propose the relevance of this particular classification to treatment options. The authors categorized infants with active hydrocephalus at time of presentation into the following four groups along the spectrum of communicating vs obstructive HCP. Group 1: patients with a purely absorptive (communicating) HCP. In these patients, tetraventricular dilatation is usually observed with occasional extraaxial fluid accumulation. An extracranial CSF diversion (shunt) is the treatment of choice. Group 2: patients with an obstructive component together with a persistent absorptive component. In these patients, a technically successful endoscopic procedure will not prevent progression of clinical symptoms of HCP. An extracranial CSF diversion (shunt) should be the treatment of choice even though some of these patients are currently treated by endoscopy. Group 3: patients with an obstructive component together with a temporary absorptive component. In these patients, a technically successful ETV should be followed by temporary CSF drainage [via LP, continuous spinal drainage (CLD), or ventriculostomy] with or without supplemental medical treatment (i.e., Diamox) for several days. Such temporary drainage may decrease failure rate in this subgroup. Group 4: patients with a purely obstructive HCP. In these patients, an endoscopic procedure (ETV) is the treatment of choice. According to this spectrum classification, the authors classify different entities with representative cases and discuss relevancy to treatment options and prognosis. The data suggest that obstructive hydrocephalus in the very young population may be rather a combination of obstructive and absorptive problem. The outcome of the patient depends mainly not only on the basic pathology causing the hydrocephalus but also on the treatment that is chosen and its complications. While bleeding and infection represent the major causes for communicating hydrocephalus, patients with complex pathologies of congenital type and intra- or interventricular obstructions may reflect obstructive hydrocephalus. Treatment of these patients may be successful by shuntless procedures if the absorptive problem is not the major component. In transient absorptive hydrocephalus, temporary measures were effective in many cases leading to successful procedures of ETV and/or posterior-fossa decompression in selected cases. Shuntless procedures are the dream of a pediatric neurosurgeon provided it solves the problem and does not imply unacceptable risk. However, the benefit has to be evaluated years after the procedure is performed, as only prospective multicenter studies will truly show which procedure may have the best overall results in the developing child. Until such studies are available, understanding the basic pathology or the combination of pathologies leading to hydrocephalus in a given child may open the window of opportunities for other than shunt surgery in many hydrocephalic children with major obstructive component.

Background. This study aimed to examine the sur- gical, oncologic, and developmental results of infants and chil- dren undergoing extirpation of skull base tumors. Methods. Sixty-seven children aged 0.5 to 18 years (mean, 11 years) who were operated on during a 6-year period made up the study cohort. Eighteen cases (27%) involved malig- nant tumors, and 49 (73%) involved benign tumors. The most common benign tumors were craniopharyngioma (n = 10) and juvenile nasopharyngeal angiofibroma (n = 8). The most common malignant tumor was sarcoma (n = 5). Thirty-six tumors (55%) involved the anterior skull base, and the rest involved the lateral (n = 24) and posterior (n = 7) skull base. Subcranial, transfacial, and subfrontal approaches were used for extirpation of anterior skull base tumors. Voluminous or malignant tumors were excised by use of combined approaches (subcranial-transfacial, subcranial-degloving, or pterional-degloving). Twenty-two children underwent adjuvant therapy (chemotherapy, radiation, or both). Postoperative follow- up was 3 to 60 months. Results. No severe postoperative complications (ie, menin- gitis, cerebrospinal fluid leak, tension pneumocephalus) and no perioperative mortality occurred. Two and a half years later, 54 of the children (80%) are alive and well. Five children, two with optic glioma and one each with squamous cell carcinoma, ependymoma, and germinoma, have died of their disease. The subcranial approach had no cosmetic impact on the craniofacial development of the patients. Conclusion. The extirpation of skull base tumors by use of conventional surgical techniques is feasible and safe among infants and children. The complication and mortality rates are lower than those in adults. The long-term cosmetic effect of the subcranial approach is negligible. A 2005 Wiley Periodicals, Inc. Head Neck 27: 682 - 689, 2005

Background Venous thromboembolism (VTE) is a common event in adults with malignant brain tumors approaching 24% throughout the course of the disease. The high morbidity and mortality of this complication yielded several protocols for prevention of the disease in adults undergoing neurosurgery for brain tumors and possible primary prevention afterwards. We investigated the incidence and complications of VTE in pediatric neuro-oncology patients. Procedure We analyzed, retrospectively, the files of all consecutive patients under the age of 18 years who were hospitalized for the treatment of brain tumors between the years 1990 and 2003 in two leading, closely related, Israeli neuro-oncology centers. Results A total of 462 children were analyzed. Three hundred eighty-four patients underwent surgery and 78 were treated medically. Only three (0.64%) of the patients developed clinical episodes of VTE that were treated conservatively. Two of these patients developed intracranial bleeding while on secondary prevention for the disease. Conclusions Although this study has considerable limitations in terms of retrospective design, heterogeneous group of patients and diagnoses, the changing awareness for thrombosis over the last 14 years and the inclusion of symptomatic VTE events only, our surprising data suggest that, as opposed to adults, the risk of clinically significant VTE in children with brain tumors may be exceedingly low. These findings set the stage for future forthcoming evaluations in view of the prospective studies that were done in adults and the possible significant implications for the prevention and possible etiologies of the disease. © 2004 Wiley-Liss, Inc.

Object: The association of spinal dysraphism and terminal sy- ringomyelia is a well-known entity, and untethering with or without syr- inx drainage is usually the surgery of choice. However, progression of the previously existing syrinx after an untethering procedure is an uncom- mon course. The objective of this study was to discuss the diagnostic, surgical, and follow-up consequenc- es of such an occurrence. Methods: Four children with occult spina bif- ida associated with terminal syrinx at the time of initial presentation are in- cluded in this study. After the prima- ry releasing procedure, all developed enlargement of the syrinx. Two of them were operated on for placement of a syringo-subarachnoid shunt, while the other two were followed up conservatively. One of the shunt- ed patients also had a second releas- ing procedure. Conclusion: Progres- sion of the pre-existing terminal syr- inx after the primary untethering procedure should be kept in mind even in the absence of overt neuro- logical progression.

The femtomolar-acting eight-amino-acid peptide (NAP), derived from activity-dependent neuroprotective protein (ADNP), provides long-term protection against the deleterious effects of closed head injury (CHI) in mice. Fifteen minutes after injury, mice were divided into two groups, control and NAP-treated and a single subcutaneous injection of NAP or vehicle was administered. A third group served as sham-treated (not subjected to head trauma). Each mouse was assessed for its clinical function, using neurological severity score, at various time intervals following CHI, up to 30-45 d. Total cerebral cortex RNA was prepared from the site of injury of CHI mice, and from parallel regions in peptide-treated and sham brains. RNA was then reversed transcribed to yield radioactive cDNA preparations that were hybridized to Atlas array membranes containing 1200 cDNAs spots. Comparison of sham-treated individual mice showed differential expression levels of at least 15 mRNA species. Furthermore, results indicated that one of the genes that did not change among individuals but specifically increased after CHI and decreased after NAP treatment was the cell surface glycoprotein Mac-1 (CD11B antigen). Thus, Mac-1 is suggested as a marker for the long-term outcome of head injury and as a potential target for NAP protective actions.

To survey the current monitoring and treatment policies for patients with severe traumatic brain injury (STBI) in Israel, and to compare the management of pediatric and adult intensive care units (ICUs).Questionnaires were sent to the medical directors of all ICUs managing STBI patients.All 21 ICUs responded to the questionnaire. All of the units were within tertiary hospitals. An intracranial pressure (ICP) monitoring device was used in over 75% of the patients in 6 out of7 (86%) of the pediatric intensive care units (PICUs), compared with 11 out of 14 (79%) of the adults ICUs. Mannitol was used in all of the units for documented elevated ICP. Mild hyperventilation (4-4.6 kPa) was applied in 52% of the units. Mild hypothermia was routinely used in 4 out of 7 (57%) and hypertonic solutions (NaCI 3%) in 3 out of 7 (43%) of the PICUs, compared with only 2 out of 14 (14%) and none (0%) of the adults ICUs respectively. PICUs aimed for a lower ICP (or =15 mm Hg) and cerebral perfusion pressure (or =50 mm Hg) than adult ICUs (or =20 mm Hg andor =60 mmHg respectively). Barbiturates were used only in patients with refractory intracranial hypertension.This survey reveals a relatively high degree of homogeneity in the treatment of STBI patients in Israel. Most patients are treated in accordance with recently published literature. We attribute this uniformity to the fact that all patients are being treated within tertiary care, university-affiliated centers. PICUs are faster at implementing new modalities of treatment and tend to adopt more aggressive treatment strategies.

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.

Introduction Selective dorsal rhizotomy (SDR) is commonly guided by an intraoperative pathoelectrophysiologic response (IPR) to electrical stimulation of posterior rootlets. The rootlets guided partial transection leads to reduced spasticity postoperatively. Prior studies have shown a statistically significant correlation between the extent of IPR and the preoperative degree of lower extremity GMFM (gross motor function measure) as well as postoperative improvement. Studies have also shown cognitive function (CF) improvement in patients who have undergone SDR, yet it is unknown whether this is a direct or indirect effect of the spasticity reduction. No study has investigated the correlation between IPR and cognitive function.The goal of this study was to answer the question: is there a direct correlation between the extent of IPR and postoperative CF improvement?. Material and methods 11 children with baseline GMFCS 3–5 underwent SDR directed by IPR (5 female, 6 male, age range 3–10 years).IPR severity was classified according to a Modified Phillips & Parks scale (1–6). The CF included communication skills, speech, eye contact, and caregiver’s evaluation. CF Data was stratified by a 3 degree scale (normal, abnormal, absent). The change in each function was scored on a 5 degree score, (−2)–(+2). Intraoperatively, rootlets displaying any degree of IPR were transected unless motor sphincter innervation was evident. The degree and extent of IPR and transaction were correlated with cognitive parameters. Results 177 IPR rootlets were transected. 91% of patients showed CF improvement after SDR. At this time, no specific direct correlation was found between IPR and CF improvement, including specific CF components: eye contact, speech, communication and caregiver’s evaluation. This could be due to small sample size and nearly global CF improvement. Conclusion Cognitive improvement after SDR is a common and consistent finding that needs to be further investigated. Presently, IPR cannot be shown to be a marker for CF changes post-SDR.

Object: Accurate placement of ventricular catheters in children with small ventricles can be difficult. Too often, shunt catheters are misplaced with regard to optimal postion and trajectory. The objective of this study was to determine whether neuronavigation-guided free-hand placement of ventricular catheters is an effective adjunct for children with hydrocephalus and small ventricles. Methods: Nine children with hydrocephalus (ages 1–12 years) participated in this study. Four children were diagnosed as suffering from slit ventricle syndrome and 5 children had small to mildly dilated ventricles. Of the 9 shunted children, 6 underwent previous shunt placements, and 1 child previously underwent an endoscopic third ventriculostomy. In 8 children the primary procedure was insertion of ventricular catheters using a frameless neuronavigation system. In 1 child, the neuronavigation system was used after failure to insert the ventricular catheter using a standard technique. All children showed significant improvement of their symptoms and signs following the procedure and none of them required shunt revision during the follow up period (mean 8±5 months). Conclusion: The usage of a neuronavigation system is safe and may be beneficial for optimal postioning and trajectory of ventricular catheters in children with small ventricles or an abnormal ventricular anatomy.

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.

Object: Optic pathway gliomas in children can involve the optic nerve, chiasm, and hypothalamus. This uncommon, slowly growing tumor can cause hydrocephalus, which usually requires placement of a ventriculoperitoneal (VP) shunt. Symptomatic ascites may occasionally develop as a complication of the VP shunt procedure. The purpose of this study was to assess the risk factors associated with CSF ascites in children with optic pathway gliomas. Methods: Twenty-two children (ages 4 months to 20 years) with chiasmatic-hypothalamic optic gliomas participated in this study. Four children were diagnosed with a chiasmatic glioma, 7 with a hypothalamic glioma, and 11 with a glioma involving both the optic chiasm and hypothalamus. Twelve children (55%) developed hydrocephalus and required VP shunt placement. Of the 12 shunted children, 4 (33%) developed CSF ascites. The incidence of ascites was not associated with infection, tumor metastasis, or multiple shunt revisions. There was no correlation with the size of the tumor. All 4 children with ascites had tumor involving the optic chiasm or optic nerve. None of the 5 children with pure hypothalamic glioma who underwent VP shunt placement have developed ascites. Among the 7 children suffering from chiasmatic or optic nerve gliomas who developed hydrocephalus, the risk of developing ascites as a complication of VP shunt placement was 57% (4/7). Ventriculoatrial (VA) shunt was the treatment of choice for children with VP shunt-induced ascites. After placement of a VA shunt the ascites subsided. The children did not develop further complications. Conclusion: We conclude that glioma involving the optic chiasm or nerve is associated with a high risk of developing ascites following VP shunt placement. VA shunt may be the treatment of choice for children with chiasmatic or optic nerve gliomas who require a CSF diversion procedure.

Objective: This study evaluates the benefits of and indications for the orbito-cranial approach (OCA) in pediatric patients. Methods and results: The authors report their recent experience of using the OCA in 9 pediatric patients, 6 boys and 3 girls. The patients' ages ranged from 3 to 17 years (mean 9.6±5.16 years). Follow-up periods varied between 6 and 21 months (mean 12.6±5.9 months). Five patients were operated on for craniopharyngiomas, 2 for chiasmatic-hypothalamic astrocytomas, 1 for a recurrent hypothalamic gangliocytoma, and 1 for a hypothalamic hamartoma. In 7 cases a neuronavigation system (BrainLab) was utilized. The lesions were removed totally in 5 patients, near-totally in 1, subtotally in 2, and partially in 1 patient. An average increase of 30% in the area of vertical exposure significantly decreased the need for brain retraction. There was no mortality in this series. The only complications connected with the surgical approach were transient subgaleal cerebro-spinal fluid collections in 7 of 9 children and a subgaleal-peritoneal shunt placement in another patient. Conclusions: Our experience with this series of patients suggests that the OCA is as safe and beneficial in pediatric patients as it is in adults. It facilitates tumor removal by providing shorter access to and better exposure of the suprasellar area, thereby minimizing brain retraction.