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Objective To develop and validate a model to predict seizure freedom in children undergoing cerebral hemispheric surgery for the treatment of drug-resistant epilepsy. Methods We analyzed 1267 hemispheric surgeries performed in pediatric participants across 32 centers and 12 countries to identify predictors of seizure freedom at 3 months after surgery. A multivariate logistic regression model was developed based on 70% of the dataset (training set) and validated on 30% of the dataset (validation set). Missing data were handled using multiple imputation techniques. Results Overall, 817 of 1237 (66%) hemispheric surgeries led to seizure freedom (median follow-up = 24 months), and 1050 of 1237 (85%) were seizure-free at 12 months after surgery. A simple regression model containing age at seizure onset, presence of generalized seizure semiology, presence of contralateral 18-fluoro-2-deoxyglucose-positron emission tomography hypometabolism, etiologic substrate, and previous nonhemispheric resective surgery is predictive of seizure freedom (area under the curve = .72). A Hemispheric Surgery Outcome Prediction Scale (HOPS) score was devised that can be used to predict seizure freedom. Significance Children most likely to benefit from hemispheric surgery can be selected and counseled through the implementation of a scale derived from a multiple regression model. Importantly, children who are unlikely to experience seizure control can be spared from the complications and deficits associated with this surgery. The HOPS score is likely to help physicians in clinical decision-making.

Selective dorsal rhizotomy (SDR) for the management of lower extremity spasticity is a surgical technique that has existed since the 1900s. While much evidence supports its efficaciousness in reducing tone in the short term, limited information exists detailing the long-term outcome and evolution over time of patients undergoing SDR. All publications with 10 years or more of outcome data on patients undergoing SDR were identified from Medline and Embase databases using the search term “Rhizotmy.” Only publications that were in English included patients with cerebral palsy under the age of 21 and discussed SDR for lower extremity spasticity were included. Case reports, reviews without primary data, or publications not accessible online were excluded from the review. A total of 2128 publications were initially identified, of which 19 papers describing 1054 patients fit inclusion and exclusion criteria. GMFCS in most patients improved or remained stable over time after surgery, although durability of improvement and final outcome was dependent on initial functional status. Tone was noted to also substantially improve although a small proportion required additional oral or injectable pharmacologic agents and an even smaller proportion required baclofen pumps. Upper extremity function was also noted to improve substantially after SDR. A significant number of patients were independent for activities of daily living and were engaged in secondary education or gainful employment. A significant proportion of patients still required orthopedic surgery to the lower extremities after SDR, although the incidence of spinal deformity was not observed to be substantially elevated over that of natural history. SDR offers substantial improvements to a number of domains beyond pure tone reduction. These changes are durable over time, although patient selection is crucial in identifying those patients that will have the most benefit. Long-term follow-up is in important in this population given the potential need for further interventions that still exist in many patients.

The aim of this study was to evaluate the outcomes and complications for individual surgeons at British Columbia Children’s Hospital for the treatment of Chiari I Malformation (CMI) in children. This was a retrospective review of patients with CMI who had surgery from 1986 to 2015. We assessed the Chicago Chiari Outcome Scores (CCOS) and complication rates by surgeon. Seventy patients, 38 males and 32 females, underwent posterior fossa decompression including 14 extradural and 56 intradural approaches. Syringomyelia was present in 74.3%. Most syringomyelia improved with no difference between intradural and extradural surgeries. After initial surgery, 13 patients (18.6%) had complications including 2/14 (14.3%) of extradural and 11/56 (19.6%) of intradural surgeries. Two patients required surgical intervention for complications whereas 11 had transient complications. The complication rate by surgeon ranged from 11 to 20% for extradural (2 surgeons only) and 10.5 to 40% for intradural surgeries (4 surgeons). The CCOS ranged from 12 to 15 for extradural and 6 to 16 for intradural. The CCOS ranges for surgeons 1 and 2 were 12–15 and 13–15 respectively for extradural. The CCOS ranges for surgeons 1, 2, 3, and 4 were 12–16, 6–15, 12–16, and 12–16 respectively for intradural. Thirteen patients had a second surgery for CMI. The final CCOS was good in 86% and moderate in 14%. There was variability in surgeries performed at BCCH by different surgeons, with variations in CCOS and complication rates. This information is important during decision making, consent process, and for quality improvement.

In 2003, pediatric neurosurgeons were surveyed under the auspices of the education committee of the International Society for Pediatric Neurosurgery (ISPN) to determine prevailing opinions regarding the management of Chiari I malformation (C1M) with and without associated syringomyelia. In the ensuing years, there has been further information from multiple C1M studies, with regards to indications, success rates of different surgical interventions, and complications. The purpose of this study was to re-evaluate current opinions and practices in pediatric C1M. Pediatric neurosurgeons worldwide were surveyed, using an e-mail list provided by the ISPN communication committee chairperson. Respondents were given scenarios similar to the 2003 C1M survey in order to determine opinions regarding whether to surgically intervene, and if so, with which operations. Of 300 surveys electronically distributed, 122 responses were received (40.6% response rate)—an improvement over the 30.8% response rate in 2003. Pediatric neurosurgeons from 34 different countries responded. There was broad consensus that non-operative management is appropriate in asymptomatic C1M (> 90%) as well as asymptomatic C1M with a small syrinx (> 65%). With a large syrinx, a majority (almost 80%) recommended surgical intervention. Scoliotic patients with CIM were generally offered surgery only when there was a large syrinx. There has been a shift in the surgical management over the past decade, with a bone-only decompression now being offered more commonly. There remains, however, great variability in the operation offered. This survey, with a relatively strong response rate, and with broad geographic representation, summarizes current worldwide expert opinion regarding management of pediatric C1M. Asymptomatic C1M and C1M with a small syrinx are generally managed non-operatively. When an operation is indicated, there has been a shift towards less invasive surgical approaches.

The aim of this study was to determine the outcomes for children who underwent selective dorsal rhizotomy (SDR) for the treatment of spasticity related to spinal pathology. We performed a retrospective review of all cases of SDR at our institution over the last 30 years and identified patients in whom spasticity was attributed to spinal rather than cerebral pathology. We gathered demographic information and recorded functional status and spasticity scores pre-operatively and over long-term follow-up. We identified four patients who underwent SDR for spinal-related spasticity. All four had hereditary spastic paraparesis (HSP). All patients had reduced spasticity in the lower limbs after SDR, which was maintained over long-term follow-up. Two patients had a more severe and progressive subtype of HSP, and both these patients exhibited functional decline despite improvement in tone. Our findings suggest SDR is a reasonable option to consider for relief of spinal-related spasticity in uncomplicated hereditary spastic paraparesis. However, SDR for the treatment of complicated HSP seems to carry more risks and have a less predictable outcome. Overall, SDR is probably best reserved for pathologies that are relatively stable in their disease course.

To compare histologically transected fila from pediatric patients with tethered cord syndrome (TCS), with and without a low conus, with controls, focusing on collagenous and elastic tissue.Thirty fila from patients with TCS, including 5 where minimal cautery was used prior to filum section, were compared with fila from 27 pediatric cadavers without TCS (controls). Sections of fila were stained with HE, Masson trichrome and Verhoeff von Gieson elastic stains, and 7 with Gordon and Sweet's reticulin stain.Fila from controls showed loose fibrous connective tissue (FCT) with thin and evenly dispersed elastic fibers (EFs). Reticulin fibers (RFs) were seen in blood vessel walls and nerve twigs. Fat was identified microscopically in 2 fila. All fila from patients with TCS had dense FCT. The EFs were in normal numbers in 17, and focally or diffusely decreased in 13. All 25 patients where the fila were cauterized during resection had thick and coiled EFs. Coiling was not seen when minimal cautery was applied. RFs were seen in blood vessel walls and nerve twigs. Fat was identified in 19 patients. Findings were similar, whether the conus termination was normal or low.The fila of all patients with TCS, whether or not the conus was low, showed abnormal FCT. EFs were decreased in 48 % of patients; however, there were thick and coiled EFs in all patients. Coiling of EFs, initially thought to be an abnormality in patients, is considered most likely to be a result of cautery (i.e., artifactual/iatrogenic coiling).

Occult tethered cord syndrome, in which there is normal neuroanatomic imaging despite clinical and urodynamic evidence of neuropathic bladder behavior, is controversial. Several uncontrolled series describe improvement in bladder function following section of the filum terminale. We performed a pilot randomized, controlled study comparing medical treatment to surgical section of the filum plus medical treatment in children with occult tethered cord syndrome.Children refractory to standard medical management for 1 year or more with normal conus position on magnetic resonance imaging and abnormal urodynamics were randomized. Exclusion criteria included any neurological conditions, spinal dysraphism, bladder outlet obstruction and an atonic bladder. Patients were assessed at randomization and 1 year later with a standardized urodynamic score, the validated PEMQOL (Pediatric Enuresis Module on Quality of Life™) scale, and a validated bowel and bladder dysfunction score.After 8 years we accrued 21 patients. The bowel and bladder dysfunction score improved in the surgical and medical arms (20% and 24%) and the urodynamic score improved slightly (6% and 4%, respectively). The PEMQOL Child and Family Impact Scales improved modestly in both groups. All differences were nonsignificant. Interim analysis indicated that more than 700 patients in each arm would be required to demonstrate a statistical difference with respect to urodynamic score based on our preliminary data.There appears to be no objective difference in urological outcome between medical management plus or minus filum section for patients with occult tethered cord syndrome. These data challenge the existence of the concept of occult tethered cord syndrome, in which bowel and bladder dysfunction score is attributed to tethering by the filum despite a normally located conus.

Intraoperative ultrasound (iUS) is a valuable tool—inexpensive, adds minimal surgical time, and involves minimal risk. The diagnostic predictive value of iUS is not fully characterized in Pediatric Neurosurgery. Our objective is to determine if surgeon-completed iUS has good concordance with post-operative MRI in estimating extent of surgical resection (EOR) of pediatric brain tumors. We reviewed charts of all pediatric brain tumor resections (single institution 2006–2013). Those with iUS and postoperative imaging (

The purpose of this study is to evaluate long-term outcomes after selective dorsal rhizotomy (SDR) for children with spastic cerebral palsy. This is a retrospective review of a prospective database of patients who underwent SDR at British Columbia Children’s Hospital. Hip adductor spasticity, hip range of motion (ROM), quadriceps strength, and motor function were assessed pre-operatively, at 6 months to 5 years and more than 10 years postoperatively. Patients were stratified by Gross Motor Function Classification System (GMFCS) level into group 1 (GMFCS II and III) and group 2 (GMFCS IV and V). Forty-four patients, with mean age at SDR of 4.5 years (range 2.9–7.7), were followed for a mean 14.4 years. Spasticity (Modified Ashworth Scale) decreased 1.5 (p

Pseudomeningoceles are common complications after posterior fossa and intradural spinal surgery and are often asymptomatic. Management guidelines are lacking, and anecdotally, we have encountered radically different suggested treatments varying from observation to immediate surgical intervention. The goal of this study was to determine the prevailing opinions among neurosurgeons on the management of this condition. Neurosurgeons from around the world were invited via an International Society for Pediatric Neurosurgery (ISPN) and Neurosurgery ListServ e-blast to participate in a 33-question survey on the management of pseudomeningoceles, presented as simulated scenarios after posterior fossa and spinal intradural surgery. Two hundred forty-one responses were obtained. Pseudomeningoceles after posterior fossa tumor resection, in the absence of hydrocephalus, were typically managed nonoperatively for 7 to 14 days before re-exploration. Only 0.5 % of the surgeons would offer upfront repair of the pseudomeningocele. In the presence of hydrocephalus, 48 % of the neurosurgeons intervene initially with cerebrospinal fluid (CSF) diversion and would change therapy if the lesion did not resolve in 2 to 4 days. Ninety percent of the surgeons manage spinal pseudomeningoceles nonoperatively for 7–14 days before re-exploration is considered. The most common steps taken to prevent pseudomeningoceles are watertight closure, tissue glues, and duroplasty. The present zeitgeist suggests that, in the absence of hydrocephalus, initial observation is appropriate for cranial and spinal pseudomeningoceles. Operative revision should be reserved for failure of conservative treatment. If hydrocephalus is present, consideration should be made for CSF diversion. This study may serve as a guideline regarding acceptable management.

Tethered cord syndrome is a well-defined condition, the management of which is fairly uniform. In contrast, occult tethered cord syndrome is a recently defined entity, where the management is still controversial. The pathophysiology is unclear and may be conceptually incongruent with current understanding of typical tethered cord syndrome. Presentation, investigation, and management of this condition are reviewed, and current understanding is presented. The aim of this study is to review the presentation, pathophysiology, investigation, and management of occult tethered cord syndrome. Literature review. Patients with occult tethered cord syndrome presents predominantly with urologic symptoms. Adult and pediatric patients vary slightly in their presentation with a higher incidence of pain in the former and incontinence in the latter. Operative management for these patients is associated with consistent improvement in urologic function in particular, although surgery is also associated with risk of worsening symptoms. The natural history of untreated patients is unknown. As occult tethered cord syndrome becomes increasingly recognized, it is important to be aware of the potential benefits of operative intervention for appropriately selected patients. Given that the natural history of this entity remains unknown, a clinical trial is currently underway that may assist in defining the role for operative management in treating this condition.

Desmoplastic infantile astrocytoma (DIA) and ganglioglioma (DIG) are uncommon and generally benign intracranial tumors that typically affect infants. Unusual cases bearing aggressive clinical and malignant histological features have been described in the literature. We report a patient who was diagnosed at the age of 6 weeks with a DIA that relapsed 3 months postoperatively despite gross total resection (GTR). Pathologic examination revealed several mitoses, not only in the primitive neuroectodermal tumor (PNET)-like areas, but also unexpectedly in the predominating spindle cell component. The Ki67 proliferative index within the spindled component was 25%. The tumor continued to grow after initial relapse despite 2 courses of intensive chemotherapy (including vincristine, carboplatin, and temozolomide), but the recurrence was eventually controlled with oral etoposide. Currently, the patient is stable at 9 months after her initial diagnosis. This case illustrates that rare cases of DIA may display malignant histologic features in the typically benign and predominating spindle cell component and behave in an aggressive clinical manner despite GTR. As such, we recommend early and extended clinical follow-up of all DIA and DIG cases.

Background/Aims: Lumbar disc herniation (LDH) can be associated with ring apophysis fracture (RAF), which is found in 6% of adult cases. However, the incidence and management of RAF in pediatric LDH is not well documented. The purpose of this study is to determine the prevalence of RAF in pediatric LDH, identify risk factors and explore the influence of RAF on the surgical management and outcome of LDH patients. Methods: Hospital records and images were retrospectively reviewed for all pediatric patients (Results: Forty-two patients met the study inclusion criteria. RAF was present in 38% of the pediatric patients with LDH. There was a significant correlation with gender (p = 0.021; 55% of the males had RAF, and 20% of the females) and association with central herniations (p = 0.003). At the last follow-up, 58% of the patients with RAF were symptom free, compared with 68% of the patients with no RAF. Conclusion: RAF is more frequently associated with LDH in children than in adults. Gender and central disc herniation are associated with RAF. Given the frequency of RAF, to properly identify these fractures and anticipate their treatment, preoperative CT would be necessary.

The author relates how he ended up somewhat unexpectedly as a pediatric neurosurgeon and how fortunate he feels to have become a pediatric neurosurgeon. He reflects on his life and on the importance of trying to do one’s best. He comments about the importance of listening, empathizing, and being compassionate as characteristics of the pediatric neurosurgeon and notes that by trying one’s best to be a good pediatric neurosurgeon one adopts more of these characteristics by learning from patients and their parents. He discusses the many ways in which the practice of pediatric neurosurgery and his involvement in the International Society for Pediatric Neurosurgery have created meaning in his life, which has led to a feeling of happiness and fulfillment.

Ten year survival rates for patients with craniopharyngioma vary from 24 to 100%. A review of the database of all children diagnosed with craniopharyngioma in British Columbia (BC) revealed that several patients died >10 years after diagnosis. This retrospective study investigates the causes and timing of deaths and reports the overall survival in this population based group of patients. A chart review was conducted on all patients aged

Summary Objective: To determine the clinical characteristics, surgical challenges, and outcome in children younger than 3 years of age undergoing epilepsy surgery in Canada. Methods: Retrospective data on patients younger than age 3 years who underwent epilepsy surgery at multiple centers across Canada from January 1987 to September 2005 were collected and analyzed. Results: There were 116 patients from eight centers. Seizure onset was in the first year of life in 82%, and mean age at first surgery was 15.8 months (1–35 months). Second surgeries were done in 27 patients, and a third surgery in 6. Etiologies were malformations of cortical development (57), tumor (22), Sturge-Weber syndrome (19), infarct (8), and other (10). Surgeries comprised 40 hemispheric operations, 33 cortical resections, 35 lesionectomies, 7 temporal lobectomies, and one callosotomy. There was one surgical mortality. The most common surgical complications (151 operations in 116 patients) were infection (17) and aseptic meningitis in 13. Of 107 patients with seizure outcome assessed more than one year postoperatively, 72 (67.3%) were seizure free (Engel I), 15(14%) had >90% improvement (Engel II), 12 had >50% improvement (Engel III), and 8 did not benefit from surgery (Engel IV). Development improved in 55.3% after surgery. Conclusion: Epilepsy surgery in children younger than 3 years of age is relatively safe and is effective in controlling seizures. Very young age is not a contraindication to surgery in children with refractory epilepsy, and early surgery may impact development positively.

The perceived need for electrophysiological guidance (EPG) during selective dorsal rhizotomy (SDR) has limited the frequency with which SDR is performed. The need for EPG during SDR has been questioned. At our institution, of200 children with SDR for spastic cerebral palsy, 22 children underwent SDR without EPG using clinical guidance (no EPG group). Electrophysiological stimulation was used to distinguish dorsal from ventral roots. The remainder had SDR with EPG. The purpose of this study was to compare outcomes between the groups having SDR with and without EPG.The 22 patients in the no EPG group were matched with 22 controls in whom EPG was used, with respect to Gross Motor Function Classification System score (GMFCS) and age. The 12-month outcomes with respect to motor function score, hip adductor spasticity (Ashworth), hip abduction range of motion (ROM), quadriceps power [Medical Research Council (MRC)], WeeFIM, Quality of Upper Extremities Skills Test (QUEST), and incidence of complications were compared.There were no statistically significant differences preoperatively with respect to GMFCS, age, gross motor function, Ashworth or MRC scores, joint ROM, WeeFIM, or QUEST. At 1 year after SDR, there were no differences between the groups in the incidence of complications or outcome measures. Percentage of dorsal roots cut was similar, but the duration of surgery was significantly shorter in the no EPG group.There was no advantage of doing SDR with EPG compared to no EPG. SDR can reasonably be done in centers where EPG is not available, but electrophysiological stimulation to distinguish dorsal from ventral roots may be useful in avoiding complications.

Brain tumors are one of the most common types of solid neoplasm in children. As life expectancy of these patients has increased with new and improved therapies, the morbidities associated with the treatments and the tumor itself have become more important.One of the most common morbidities is growth hormone deficiency, and since recombinant growth hormone (GH) became available, its use has increased exponentially. There is concern that in the population of children with brain tumors, GH treatment might increase the risk of tumor recurrence or progression or the appearance of a second neoplasm. In the light of this ongoing concern, the current literature has been reviewed to provide an update on the risk of tumor recurrence, tumor progression, or new intracranial tumor formation when GH is used to treat GH deficiency in children, who have had or have intracranial tumors.On the basis of this review, the authors conclude that the use of GH in patients with brain tumor is safe. GH therapy is not associated with an increased risk of central nervous system tumor progression or recurrence, leukemia (de novo or relapse), or extracranial non-leukemic neoplasms.

A head fixation device with pins is commonly used for immobilization of the patient’s head during craniotomy. The safety of head fixation devices in children has been discussed rarely in the literature. The purpose of this report is to review our experience with complications of head fixation with pins in children undergoing craniotomies and to review the literature on this subject. The database of the Division of Neurosurgery was reviewed to identify children who had cranial complications related to the use of a pin head fixation device. The charts of these patients were reviewed retrospectively. Five of 766 children (0.65%) undergoing craniotomies with pin fixation of the head had depressed skull fractures and/or epidural hematomas from the pin fixation. Age ranged from 2.6 to 7.5 years; all fractures were temporal and occurred during posterior fossa craniotomies. Depressed skull fractures and associated epidural hematomas need to be considered as possible complications of pin fixation of the head for craniotomy in young children.

The significance of beaten copper appearance (BCA) on skull radiographs in children following surgery for isolated sagittal craniosynostosis has not been studied. This study was designed to look for any correlation between BCA and symptoms suggestive of intracranial hypertension in this group of patients.Forty-eight consecutive children, who were operated for isolated sagittal synostosis from 1987 to 2000 and had postoperative skull radiographs, were included. Patients were divided into: (a) BCA group (n = 20), consisting of children who had beaten copper appearance on skull radiographs at last follow up, and (b) Non-BCA group (n = 28), consisting of children who did not have this finding. Records were reviewed to look for symptoms suggestive of intracranial hypertension, such as headache, head banging, and irritability.Median age at surgery was 4.8 months for BCA group and 4 months for the non-BCA group. Follow up ranged from 4 to 156 months with a mean of 36.2 months. Total of 28.6% (n = 6) of the children with follow up radiographs done ator =18 months of age had BCA. The incidence of BCA increased to 83.3% in children with skull radiographs performed after 48 months of age. In 18 (90%) children, the BCA was 'diffuse' with 5 (25%) children having the maximum possible score of 8. In the BCA group, 45% (n = 9) had symptoms compared to 10.7% (n = 3) in the control group (p = 0.0068).This study suggests a significant number of children with BCA on radiographs develop symptoms suggestive of raised ICP following surgical treatment in infancy and prolonged follow up may be warranted in this group of patients.

The purpose of this report is to review the historical development, current operative techniques, selection criteria, outcomes, and complications of selective dorsal rhizotomy (SDR) for treatment of spastic cerebral palsy (CP).This review is based on a review of literature and personal observations.SDR has evolved from the 1960s onwards into a standard neurosurgical procedure for spastic CP. There is much variation in the operative technique among surgeons with respect to the level of exposure, electrophysiological guidance, and extent of rhizotomies. Appropriate selection of patients for SDR requires determination that spasticity, not dystonia, is the major disabling hypertonia and that the lower limbs are maximally involved. Positive outcomes have been well demonstrated in the impairment, functional limitations, and disability dimensions, as per the National Center for Medical Rehabilitation Research Model of Dimensions of the Disabling Process. Complications have been relatively few.SDR is the procedure of choice for treatment of spasticity in spastic diplegic CP and in selected children with spastic quadriplegic CP. Optimal selection and outcomes are achieved using a multidisciplinary approach.

Ascites and abdominal pseudocysts are two complications that can occur following placement of a ventriculoperitoneal (VP) shunt. Although various factors have been implicated, the exact pathogenesis of the two conditions remains elusive. To the authors' knowledge, there are no studies in which these two obviously related conditions have been compared.The authors retrospectively reviewed the cases of children with abdominal complications caused by a VP shunt. There were 15 patients who developed a pseudocyst and five patients who developed ascites. The cases were analyzed to identify common and distinguishing factors that may help in identifying the mechanism involved. Abdominal symptoms were the mode of presentation for patients with ascites, whereas shunt malfunction was the mode of presentation in 60% of those with pseudocysts. Culture-proven infection, abdominal surgery, and the number of revisions seemed to be more common in cases with pseudocysts than in ascites. The fluid in ascites was found to be a transudate irrespective of the origin of hydrocephalus. Alternative drainage sites were required in the treatment of patients with ascites, and reimplantation in the peritoneum was possible in 66.7% of those with pseudocysts. In the long-term, however, peritoneal reimplantation was possible in three of the five patients with ascites.Abdominal pseudocysts and ascites, after VP shunt treatment, are distinct conditions with different modes of presentation and findings during examination of fluid, and therefore they require different management strategies.

OBJECTIVE: Hypodensities on computed tomographic (CT) brain scans are thought to take at least 6 hours to become apparent after blunt head trauma. This finding, in conjunction with the later evolution of the hypodensities, is used in timing the injury in children with suspected non-accidental brain injury, in whom the history may be inaccurate. The purpose of this study is to report the occurrence of diffuse cerebral parenchymal hypodensities on CT scans performed within 5 hours of a well-defined accidental head injury. METHODS: A retrospective review was performed of five patients admitted to British Columbia Children's Hospital who had accidental head injury and who were identified as having diffuse cerebral hemispheric hypodensities on early CT scans. RESULTS: We present five patients (age range, 4 mo-14 yr) with well-documented accidental head injuries who demonstrated obvious and extensive CT brain scan cerebral hemispheric hypodensity from 60 minutes to 4.5 hours after trauma. All five patients presented with severe head injuries and immediate, unremitting coma, and all five progressed rapidly to brain death within 48 hours. CONCLUSION: It is unusual, but possible, to develop CT hypodensities as early as 1 hour after accidental head injury. In our small series of cerebral hemispheric hypodensity occurring less than 5 hours after trauma, all five patients had a uniformly fatal outcome. These observations may be important medicolegally in the assessment of the timing of head injury when the history of the trauma is not clear, as in children with suspected non-accidentally inflicted injury. It is inappropriate to generalize these findings to patients who are not unconscious immediately after a head injury, who regain consciousness after an injury before deteriorating, or who do not progress rapidly to brain death. Language: en

Summary: Health-related quality of life (HRQOL) is an important outcome measure in clinical research. Given the psychosocial and behavioral difficulties associated with pediatric epilepsy, evaluating HRQOL in this patient population is of particular importance. Though HRQOL has been examined in pediatric patients receiving focal resection or pharmacological (antiepileptic drug; AED) treatment, it has not been assessed in patients receiving hemispherectomy (HE) for intractable epilepsy. The current study evaluated HRQOL in a sample of pediatric HE cases (N = 26) using previously validated questionnaires relative to surgical (N = 30) and nonsurgical (N = 84) comparison groups. Compared with focal resection and nonsurgical patients, parents of children who received HE reported similar levels of HRQOL. In surgical cases, worse HRQOL was correlated with residual seizure frequency. In both surgical and nonsurgical cases, female gender, higher AED load, and lower functional independence predicted worse HRQOL. Interestingly, HE status (i.e., having undergone HE) predicted fewer epilepsy-related limitations. Consistent with previous findings, AED load, in addition to lower functional abilities, appear particularly detrimental to life quality in pediatric epilepsy. HE, however, is not associated with increased risk for poor HRQOL. When considered in light of the multiple, significant risk factors for poor outcome associated with HE, children who undergo the procedure fare surprisingly well.

Cerebellar astrocytomas (CA) are one of the most common posterior fossa tumors in children. The vast majority is low grade, and prognosis for long-term survival is excellent.Recent literature about CA was reviewed to provide an up to date overview of the epidemiology, pathology, molecular and cell biology, diagnosis, presentation, management, and long-term outcomes.Surgical resection remains the first-line treatment with complete removal of the tumor the goal. However, even when only subtotal resection has been achieved, there is a significant chance that the tumor will remain stable or will regress spontaneously. Adjuvant chemotherapy is reserved for those tumors that progress despite surgery, and more personalized chemotherapy is being pursued with better understanding of the molecular genetics of this tumor. Radiotherapy has generally not been recommended, but stereotactic radiotherapy and conformal proton beam radiotherapy may be reasonable options in the setting of relapse or progression. In the long term, permanent neurologic deficits, mainly cerebellar dysfunction, are common, but quality of life and cognitive function are generally good.Low-grade CA remains primarily a surgical disease, with excellent survival rates. Care must be taken with surgery and adjuvant treatments to preserve neurologic function to allow for optimal outcomes in the long term.

The optimal antibiotic prophylaxis for pediatric shunt-related procedures is not clear. There is much inconsistency among different medical centers. This paper summarizes and analyzes the various prophylactic antibiotic regiments used for shunt-related surgeries at different pediatric neurosurgery centers in the world.A survey questionnaire was distributed through the Pediatric Neurosurgery list-server (an e-mail-based special interest group in pediatric neurosurgery). Forty-five completed questionnaires were received, one per medical center, primarily from pediatric neurosurgeons with the following geographic breakdown: 25 from North America, 13 from Europe, and 7 from Asia and other countries. All centers routinely administered prophylactic antibiotics for shunt-related procedures. The drugs of choice were first-generation cephalosporins (23), second-generation cephalosporins (10), naficillin/oxacillin (4), vancomycin (3), clindamycin (1), amoxicillin (1), and mixed protocols in three centers. The initial drug administration ("first dose") was: in the department before transfer to operating room (5), upon arrival to operating room (11), at induction of anesthesia (13), and at initial skin incision (16). The duration of antibiotic dosage also varied: single dose (13), 24-h administration (26), 48-h administration (2), and longer than 48 h in four centers.Two general tendencies were noted, common to the majority of participating centers. There was a general trend to modify antibiotic treatment protocol in "high-risk" populations. The second common theme noted in more than half of responding centers was the use of long-term antibiotic treatment for externalized devices (such as externalized shunts, external ventricular drains or lumbar drains), usually till the device was in place.

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.

BACKGROUND High-grade astrocytomas (HGA) carry a dismal prognosis and compose nearly 20% of all childhood brain tumors. The role of high-dose chemotherapy (HDCT) in the treatment of HGA remains unclear. METHODS In a nationwide study, The Children's Cancer Group (CCG) prospectively evaluated 102 children with HGA and postoperative residual disease for efficacy and toxicity of four courses of HDCT before radiotherapy (RT). Patients were randomly assigned to one of three couplets of drugs: carboplatin/etoposide (Regimen A), ifosfamide/etoposide (Regimen B), or cyclophosphamide/etoposide (Regimen C). After HDCT, all patients were to receive local RT followed by lomustine and vincristine. Twenty-six patients were excluded after central neuroradiographic review (n = 8) or pathology review (n = 18). RESULTS Of 76 evaluable patients (median age, 11.95 yrs; range, 3–20 yrs), 30 patients relapsed during HDCT, and 11 others did not complete HDCT because of toxicity. Nonhematologic serious toxicities were common (29%), and 21% of patients did not receive RT. Objective response rates were not associated with amount of residual disease and did not statistically differ between regimens: 27% (Regimen A), 8% (Regimen B), and 29% (Regimen C). Overall survival (OS) was 24% ± 5% at 5 years and did not differ between groups. Median time to an event was longest for Regimen A (283 days compared with 83 and 91 days for Regimens B and C, respectively). The five-year, event-free survival (EFS) rate for all patients was 8% ± 3% and 14% ± 7% for Regimen A (P = 0.07). CONCLUSIONS OS and EFS were not affected by histologic grade. Patients who responded to HDCT had a nominally higher survival rate (P = 0.03 for trend). The authors conclude that these commonly used HDCT regimens provide no additional clinical benefit to conventional treatment in HGA, regardless of the amount of measurable residual tumor. Cancer 2005. © 2005 American Cancer Society.

OBJECTIVE: To determine whether estimates of regional cerebral blood flow (rCBF) using xenon computed tomography (XeCT) in children with moyamoya disease can predict stroke risk before and after treatment. METHOD: Seven patients with moyamoya disease underwent 22 serial Xe computed tomographic scans. Estimates of rCBF were obtained at three computed tomographic levels by use of a 5-minute inhalation of 28% Xe. Acetazolamide challenge was performed in eight scans. For comparison of abnormal vessel distribution and areas of infarction, 17 intra-arterial digital subtraction angiograms, 47 computed tomographic scans, and 15 magnetic resonance imaging scans were available. Follow-up exceeded 36 months in all patients. Mean follow-up for the interventional group was 65.2 months (n =5; range, 37–109 mo) and 38 months for the nonoperative patients (n =2; 36 and 40 mo). RESULTS: Of six Xe computed tomographic scans obtained at diagnosis, four revealed regions of oligemia, augmented vertebrobasilar flow, and regions of carotid steal after acetazolamide. In the delay between diagnosis and treatment, three patients had strokes in ischemic areas identified by XeCT. Of the 10 posttreatment scans obtained from 4 patients, 2 revealed improved tissue perfusion with angiography confirming successful encephaloduroangiomyosynangiosis. In 2 others, XeCT performed 6 months posttreatment revealed improved perfusion without angiographic change, and angiography at 1 year revealed failed encephaloduroangiomyosynangiosis and new native collaterals. None of the patients with improved rCBF had new strokes. Eleven of 14 Xe computed tomographic scans were obtained within 30 days of angiography. Comparison of these studies demonstrates that regions of oligemia were confined to areas associated with vessel stenosis and little neovascularity or collateral pathways. CONCLUSION: XeCT, particularly with acetazolamide challenge, objectively quantifies rCBF. Our preliminary data suggest that it may permit assessment of stroke risk in children with moyamoya disease and may predict surgical outcome earlier than angiography.

A 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.We report the first documented case of hemorrhage into a spinal neurenteric cyst that has been confirmed surgically and histopathologically.

Summary: Purpose: Validity studies on health-related quality of life (HRQOL) scales for pediatric epilepsy are few, and cross-validation with other samples has not been reported. This study was designed to assess the validity of three parent-rated measures of HRQOL in pediatric epilepsy: (a) the Impact of Childhood Illness Scale (ICI), (b) the Impact of Child Neurologic Handicap Scale (ICNH), and (c) the Hague Restrictions in Epilepsy Scale (HARCES). Methods: Retrospective data were examined for 44 children with intractable epilepsy. Validity was assessed by evaluating differences across epilepsy severity groups as well as correlations between HRQOL scales and neurologic variables (seizure severity, epilepsy duration, current/prior antiepileptic medications) and psychosocial measures (emotional functioning, IQ, social skills, adaptive behavior). Scale overlap with a global QOL rating also was assessed. Results: The HRQOL measures were moderately to highly intercorrelated. The scales differed in terms of their associations with criterion measures. The HARCES was related to the highest number of neurologic variables and the ICNH to the fewest. All three scales were related to psychosocial functioning and to global quality of life. Conclusions: The results of this study suggest that the three measures are likely adequate measures of HRQOL for use in intractable childhood epilepsy. The measures were highly intercorrelated, and they were all broadly related to criterion measures reflecting specific domains of HRQOL as well as global QOL. Some differences between scales emerged, however, that suggest care in choosing HRQOL instruments for children with epilepsy. Key Words: Quality of life— Epilepsy—Childhood—Validity—Parent ratings.

Summary:Disabling hypertonia—spasticity, dystonia, or a combination—is a major clinical problem in children and is most commonly seen in cerebral palsy. This article reviews current definitions of various types of hypertonia and the management of hypertonia in these children. The multidisciplinary n

A previous randomized clinical trial compared selective dorsal rhizotomy (SDR) plus postoperative intensified physiotherapy (group 1) with intensified physiotherapy alone (group 2) for children with spastic diplegic cerebral palsy. At the end of this trial, all patients in group 2 had an SDR, followed by further intensified physiotherapy. This study was performed to determine if the additional intensified physiotherapy before SDR, as occurred in group 2, improved long-term motor outcome. Outcomes were compared in the two groups, i.e. group 1 without intensified physiotherapy before SDR and group 2 with intensified physiotherapy before SDR. The primary outcome measure was the Gross Motor Function Measure (GMFM). Lower-limb spasticity, range and strength were secondary outcome measures. Baseline assessments had been done for the prior clinical trial. For this study, patients were reassessed by physiotherapists blinded to the treatment group. Thirteen children in each group were studied at a mean follow-up of 53 months. The mean improvement in GMFM was 10.0 in group 1 and 10.4 in group 2 (p = 0.9). Improvements in spasticity and range were similar in the two groups. There was no significant change in muscle strength in either group. It was concluded that in this relatively small series of patients, additional intensified physiotherapy before SDR did not improve motor outcomes.

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.

INTRODUCTION Tethering is assumed to be the primary cause of deterioration seen in children with transitional lipomyelomeningocele as they age. The inevitability of deterioration has led to recommendations for prophylactic interventions to stabilize or prevent further clinical deterioration. OBJECTIVE Determine the frequency and patterns of functional deterioration observed after successful untethering in patients with transitional lipomyelomeningocele and compare functional outcomes with what is known regarding untreated patients. METHODS Fifty patients having transitional LMMC, treated at a single institution and followed in a multidisciplinary clinic were retrospectively reviewed to determine their clinical status prior to untethering, and the time to development of new symptoms or signs following untethering. RESULTS 82% of patients were diagnosed and 78% underwent untethering prior to one year of age. All patients had a cutaneous lumbosacral lipoma, 22 patients were considered normal at presentation and 28 showed abnormalities on clinical examination. Forty-nine patients were untethered successfully and all were available for follow-up ranging from 2 to 138 months (mean 39 months). Acute morbidity was limited to transient neurogenic bladder dysfunction and minor wound complications. Late clinical deterioration occurred in the majority of patients. Orthopedic and neurological deterioration occurred over the first 60 months following untethering and urological deterioration occurred thereafter. CONCLUSIONS Functional loss after untethering is common and the pattern of loss is likely a reflection of the ability to detect abnormalities in this infant population. Untethering does not usually result in permanent acute morbidity, and does not prevent longer-term functional deterioration. The ratio of asymptomatic to symptomatic patients at follow-up in this operative series is similar to age-matched historical series of untreated patients.

Surgical removal of cystic craniopharyngiomas in children is associated with significant operative morbidity and recurrence rates. The purpose of this study was to review our experience with a less invasive therapy, namely, intratumoral bleomycin, in the treatment of predominantly cystic craniopharyngiomas. All children with craniopharyngiomas treated at a tertiary care pediatric neurosurgical center since 1994, when bleomycin was first used, were reviewed retrospectively. Seven patients received intratumoral bleomycin therapy. Patients received 2–5 mg bleomycin per dose, 3 times per week, for 3–5 weeks as an initial course. Mean follow-up of these patients was 3 years. In 4 patients, treatment resulted in a significant decrease (>50%) in tumor size, which has remained stable. Two patients’ tumors progressed and underwent resection, and 1 patient had surgical removal because of persistent headaches, although no growth of residual tumor had been noted. One patient developed peritumoral edema as a result of bleomycin therapy. Intratumoral bleomycin is a useful alternative therapy for cystic craniopharyngiomas, and may control tumor growth and delay potentially harmful resection and/or radiotherapy in young children.

Background: A previously reported multicenter randomized trial assessed whether 2 new shunt valve designs would reduce shunt failure rates compared to differential pressure valves. The study did not show a significant difference in the time to first shunt failure. Patients entered the trial between October 1, 1993, and October 31, 1995. The primary results were based on the patients’ status as of October 31, 1996 (a minimum follow-up of 1 year). This report describes the late complications based on the patients’ most recent follow-up. Methods: Three hundred and forty-four hydrocephalic children at 12 North American and European centers were randomized to 1 of 3 valves: a standard differential pressure valve; a Delta valve (PS Medical-Medtronic) or a Sigma valve (NMT Cordis). Patients were followed until their first shunt failure. Shunt failure was defined as shunt surgery for obstruction, overdrainage, loculation or infection. If the shunt did not fail, follow-up was continued until August 31, 1999. Results: One hundred and seventy-seven patients had shunt failure. Shunt obstruction occurred in 131, overdrainage in 13, loculated ventricles in 2 and infection in 29. The overall shunt survival was 62% at 1 year, 52% at 2 years, 46% at 3 years, 41% at 4 years. The survival curves for the 3 valves were similar to those from the original trial and did not show a survival advantage for any particular valve. Conclusions: Prolonged follow-up to date does not alter the primary conclusions of the trial: there does not appear to be one valve that is clearly the best for the initial treatment of pediatric hydrocephalus.

The authors present a patient with diastematomyelia and a spinal intramedullary teratoma, remote from the split cord malformation. A split cord malformation at the L2–L3 level was initially discovered during investigations for thoracic congenital scoliosis, and this was treated surgically. The teratoma, which was at the level of the scoliosis, went undiagnosed until neurological deterioration occurred many years later. Surgical removal of the teratoma resulted in return to normal function. The potential for coexisting congenital anomalies at separate levels of the spinal cord must be considered in radiological investigations of a developmental spinal lesion.

We report a male term newborn presenting with a congenital macrocephaly 3.5 standard deviations above the median, with a wide and tense anterior fontanel, splayed calvarial sutures, and muscular hypotonia. Antenatal head circumferences were repeatedly below the median. A postnatal head ultrasound showed a large right intracerebral mass with right lateral ventricle compression, right temporal horn dilation, and right frontal horn enlargement with lateral displacement. Additional imaging by computed tomography scan and magnetic resonance imaging was performed. A decompression was performed and histology, immunohistochemistry, and molecular biology supported the diagnosis of a primitive neuroectodermal tumor. A MYCN gene amplification assay remained negative. The incidence of neonatal brain tumors is between 1.4 and 4.1/100,000 live births. Their most common presentation is macrocephaly, hydrocephalus, stillbirth, or diagnosis by pre- or postnatal imaging. Although hydrocephaly and intra- or extracranial hemorrhage are the most frequent causes of congenital macrocephaly, this should be initially investigated by head ultrasound. A suspected malignancy will be confirmed by histopathology, immunohistochemistry, and molecular biology.

A major concern during the early postoperative period after surgical resection of optic chiasmatic gliomas is the derangement of sodium and water metabolism which may add to the morbidity of the procedure. The purpose of this study was to characterize the abnormalities of water and sodium metabolism in children with optic chiasmatic gliomas treated surgically at British Columbia’s Children’s Hospital and to identify therapeutic modalities which might prevent or ameliorate the development of these complications. A retrospective chart review of children with optic/chiasmatic gliomas undergoing operations on the tumor was performed and the pre- and postoperative radiographs reviewed by an independent neuroradiologist. There were 11 patients who underwent 13 operations for either resection (n = 9) or biopsy (n = 4) of their optic chiasm tumor. The extent of resection in patients undergoing more than simple biopsy ranged from 83 to 99%, and all patients with resection had exophytic tumor extending into the hypothalamus. Postoperative syndrome of inappropriate antidiuretic hormone secretion (SIADH) and/or diabetes insipidus occurred after 8 of the 9 tumor resections and was associated with significant morbidity. No disturbance of water metabolism occurred after biopsy only. In patients with SIADH, the urinary sodium level rose markedly 6–12 h prior to the development of hyponatremia, and it was concluded that this was a valuable predictor of impending hyponatremia. Replacement of urine output was noted to be the most reliable way to avoid rapid fluctuations in serum sodium and to avoid the morbidity of diabetes insipidus or SIADH and is recommended in the postsurgical patients who cannot regulate fluid intake.

Selective dorsal rhizotomy (SDR) has been shown to be an effective treatment for the spasticity of cerebral palsy, but few studies have addressed specifically the side effects of the procedure. A retrospective study was performed to determine the frequency and nature of complications in 158 children who had undergone SDR at British Columbia’s Children’s Hospital from 1987 to 1996. Intraoperative, preoperative (immediate postoperative until discharge at approximately 7 days) and postdischarge complications occurred in 3.8, 43.6 and 30% of patients, respectively. The most common intraoperative complication was aspiration pneumonia, which was experienced by 2 patients (1.3%). Perioperatively, sensory changes were found in 8.9% of the children, and transient urinary retention in 4.4%. Complications after discharge included back pain starting more than 6 months after surgery in 10.8%, sensory changes in 13.9%, and neurogenic bladder or bowel problems in 12.7%. Persistent sensory changes occurred in 3.8%, were not important functionally, and tended to occur in patients with the largest amount of dorsal root tissue cut. In 8 patients (5.1%), bladder and/or bowel dysfunction attributed to the SDR was present at the latest follow-up, although in only 2 patients (1.3%) this dysfunction was a definite complication of the rhizotomy. The use of pudendal monitoring and/or cutting less than 50% of the S2 roots may have been associated with a lower incidence of long-term sphincter dysfunction. Data about the nature and frequency of complications may result in further modifications to the SDR procedure, and is critical for counseling about SDR and alternative options available for treatment of the child with spastic cerebral palsy.

Acute subdural hematomas in the full-term neonate are usually thought to be a manifestation of birth trauma. Most commonly, these hematomas are associated with a tentorial tear, and less frequently are secondary to damage to the occipital sinus accompanying occipital osteodiastasis, or to rupture of bridging superficial cerebral convexity veins. We report 6 cases of acute subdural hematoma associated with cerebral infarction (SDH/CI) in term neonates, an entity which has not been well-documented previously. This combination accounted for half the cases of neonatal acute subdural hematomas referred to a pediatric neurosurgical service. All children presented within 72 h of birth, usually after an uneventful delivery, and typically with seizures (5/6), apneic spells (3/6) and a full or bulging fontanel (5/6). Although the patients were referred to the neurosurgical servive for management of a convexity subdural hematoma, the CT scans showed a large associated cerebral infarct, which was hemorrhagic in 5 of the 6 cases, most commonly in the distribution of the middle cerebral artery. Craniotomy was performed in 4 patients, needle aspiration of the subdural hematoma in 1, and no surgical intervention in 1. The outcome in 3 patients, who have been followed for more than 1 year, has been good, with some mild focal neurologic deficits, but no seizure activity. The pathogenesis of this entity is unclear, but the acute subdural hematoma in these cases is probably secondary to the cerebral infarction. Recognition of SDH/CI as a specific entity in term neonates has significant management and possibly medicolegal implications.

The classification and embryogenesis of cystic cervical dysraphic lesions are discussed in the light of the authors' experience and review of the literature. It is felt that these lesions are best described as meningoceles or myelocystoceles, and the use of the term 'myelomeningocele' may be more confusing than clarifying. The authors hypothesize that the cervical meningocele and the myelocystocele are part of a spectrum of the same underlying developmental abnormality, namely limited dorsal myeloschisis, with the eventual abnormality depending on the presence or absence of associated hydromyelia.

As the incidence of deformational plagiocephaly (DP) has risen, so has the demand on clinicians to make appropriate treatment recommendations. While knowledge of risk factors and natural history continue to evolve, there is uncertainty and controversy regarding intervention approaches. The purpose of this report is to review current treatment approaches for DP, in particular the use of orthotic helmets. A narrative literature review was conducted to summarize current evidence supporting preventative measures and re-positional and orthotic interventions. When started early at under 2 months of age, positional efforts and ‘tummy time’ can be effective in preventing and improving DP, but these strategies need to be better promoted to caregivers. The timing, severity and parental concerns as indicators for orthotic treatment are reviewed. Limitations discussed include high cost, lower level of evidence and poor understanding of long-term outcomes and caregiving implications. For optimal outcome, current evidence supports use of repositioning in young infants and start of orthotic helmet treatment for infants with significant DP at 4–6 months. Further research is needed to better understand the parameters for use (stratified by age and severity), long-term outcomes and natural history and the impact on caregiving, as well as the cultural significance of head shape.

Ventricular asymmetry is common after ventricular shunting, and occasionally isolation of the contralateral ventricle necessitates a second shunt. The following study was performed to determine whethe

Background Section of a tight filum terminale is a minimally invasive procedure compared with cord untethering procedures used for more complex spinal abnormalities. Anecdotal evidence suggests, however, that the risk of symptomatic retethering resulting from scarring might be higher than previously thought. Objective To determine the frequency of symptomatic retethering after section of a tight filum terminale and to explore possible risk factors. Methods We reviewed databases at 2 pediatric neurosurgery centers for all patients who had surgery for a suspected tight filum terminale between January 1982 and June 2009. Results We identified 152 patients. The median length of follow-up was 78 months. Thirteen patients (8.6%) went on to retether symptomatically at a median time of 23.4 months after the initial procedure. Eight had early retethering (within 2 years) and 5 had late retethering (after 7 years). Compared with late retetherers, early retetherers were older at initial surgery (median, 9.4 vs 0.9 years of age), had a higher level of the conus (median, L1/L2 vs L3/L4), had more arachnoiditis after initial surgery, and required more repeat untethering procedures. Late retetherers were younger at initial surgery than those who did not retether (median, 0.9 vs 4.5 years of age). Conclusion Symptomatic retethering is not uncommon after a simple filum snip, and long-term follow-up is warranted. Two distinct patterns of retethering were observed. Arachnoiditis caused by infection or a cerebrospinal fluid fistula may predispose to early retethering, whereas early surgery for prevention of tethered cord symptoms may predispose to late retethering.