Pediatric Moyamoya Revascularization Perioperative Care: A Modified Delphi Study.

BACKGROUND: Surgical revascularization decreases the long-term risk of stroke in children with moyamoya arteriopathy but can be associated with an increased risk of stroke during the perioperative period. Evidence-based approaches to optimize perioperative management are limited and practice varies widely. Using a modified Delphi process, we sought to establish expert consensus on key components of the perioperative care of children with moyamoya undergoing indirect revascularization surgery and identify areas of equipoise to define future research priorities. METHODS: Thirty neurologists, neurosurgeons, and intensivists practicing in North America with expertise in the management of pediatric moyamoya were invited to participate in a three-round, modified Delphi process consisting of a 138-item practice patterns survey, anonymous electronic evaluation of 88 consensus statements on a 5-point Likert scale, and a virtual group meeting during which statements were discussed, revised, and reassessed. Consensus was defined as ≥ 80% agreement or disagreement. RESULTS: Thirty-nine statements regarding perioperative pediatric moyamoya care for indirect revascularization surgery reached consensus. Salient areas of consensus included the following: (1) children at a high risk for stroke and those with sickle cell disease should be preadmitted prior to indirect revascularization; (2) intravenous isotonic fluids should be administered in all patients for at least 4 h before and 24 h after surgery; (3) aspirin should not be discontinued in the immediate preoperative and postoperative periods; (4) arterial lines for blood pressure monitoring should be continued for at least 24 h after surgery and until active interventions to achieve blood pressure goals are not needed; (5) postoperative care should include hourly vital signs for at least 24 h, hourly neurologic assessments for at least 12 h, adequate pain control, maintaining normoxia and normothermia, and avoiding hypotension; and (6) intravenous fluid bolus administration should be considered the first-line intervention for new focal neurologic deficits following indirect revascularization surgery. CONCLUSIONS: In the absence of data supporting specific care practices before and after indirect revascularization surgery in children with moyamoya, this Delphi process defined areas of consensus among neurosurgeons, neurologists, and intensivists with moyamoya expertise. Research priorities identified include determining the role of continuous electroencephalography in postoperative moyamoya care, optimal perioperative blood pressure and hemoglobin targets, and the role of supplemental oxygen for treatment of suspected postoperative ischemia.

Clinical factors associated with need for neurosurgical care in young children with imaging for macrocephaly: a case control study.

BACKGROUND: Macrocephaly is present in 2.3% of children with important neurosurgical conditions in the differential diagnosis. The objective of this study was to identify clinical associations with actionable imaging findings among children with head imaging for macrocephaly. METHODS: We conducted a case-control study of head imaging studies ordered for macrocephaly among children 24 months and younger in a multistate children's health system. Four neurosurgeons reviewed the images, determining cases to be a 'concern' if neurosurgical follow-up or intervention was indicated. Electronic health records were reviewed to collect patient-level data and to determine if surgery was performed. Controls were matched 3:1 to cases of 'concern' in a multivariate model using conditional logistic regression. RESULTS: In the study sample (n = 1293), 46 (4%) were concern cases, with 15 (1%) requiring surgery. Significant clinical factors associated with neurosurgical concern were bulging fontanel [aOR 7.47, (95% CI: 2.28-24.44), P < 0.001], prematurity [aOR 21.26, (95% CI: 3.76-120.21), P < 0.001], any delay [aOR 2.67, (95% CI: 1.13-6.27), P = 0.03], and head-weight Z-score difference (W_diff, defined as the difference between the Z-scores of head circumference and weight) [aOR 1.70, (95% CI: 1.22-2.37), P = 0.002]. CONCLUSIONS: Head imaging for macrocephaly identified few patients with findings of concern and fewer requiring surgery. A greater head-weight Z-score difference appears to represent a novel risk factor for neurosurgical follow-up or intervention.

Achondroplasia Natural History Study (CLARITY): 60-year experience with hydrocephalus in achondroplasia from four skeletal dysplasia centers.

OBJECTIVE: The objective of this study was to describe the incidence and management of hydrocephalus in patients with achondroplasia over a 60-year period at four skeletal dysplasia centers. METHODS: The Achondroplasia Natural History Study (CLARITY) is a registry for clinical data from achondroplasia patients receiving treatment at four skeletal dysplasia centers in the US from 1957 to 2017. Data were entered and stored in a REDCap database and included surgeries with indications and complications, medical diagnoses, and radiographic information. RESULTS: A total of 1374 patients with achondroplasia were included in this study. Of these, 123 (9%) patients underwent treatment of hydrocephalus at a median age of 14.4 months. There was considerable variation in the percentage of patients treated for hydrocephalus by center and decade of birth, ranging from 0% to 28%, although in the most recent decade, all centers treated less than 6% of their patients, with an average of 2.9% across all centers. Undergoing a cervicomedullary decompression (CMD) was a strong predictor for treatment of hydrocephalus (OR 5.8, 95% CI 3.9-8.4), although that association has disappeared in those born since 2010 (OR 1.1, 95% CI 0.2-5.7). In patients born since 1990, treatment of hydrocephalus with endoscopic third ventriculostomy (ETV) has become more common; it was used as the first line of treatment in 38% of patients in the most recent decade. Kaplan-Meier analysis suggests that a single ETV will treat hydrocephalus in roughly half of these patients. CONCLUSIONS: While many children with achondroplasia have features of hydrocephalus with enlarged intracranial CSF spaces and relative macrocephaly, treatment of hydrocephalus in achondroplasia patients has become relatively uncommon in the last 20 years. Historically, there was a significant association between symptomatic foramen magnum stenosis and treatment of hydrocephalus, although concurrent treatment of both has fallen out of favor with the recognition that CMD alone will treat hydrocephalus in some patients. Despite good experimental data demonstrating that hydrocephalus in achondroplasia is best understood as communicating in nature, ETV appears to be reasonably successful in certain patients and should be considered an option in selected patients.

Cerebral revascularization surgery reduces cerebrovascular events in children with sickle cell disease and moyamoya syndrome: Results of the stroke in sickle cell revascularization surgery retrospective study.

BACKGROUND: Recent studies suggest that cerebral revascularization surgery may be a safe and effective therapy to reduce stroke risk in patients with sickle cell disease and moyamoya syndrome (SCD-MMS). METHODS: We performed a multicenter, retrospective study of children with SCD-MMS treated with conservative management alone (conservative group)-chronic blood transfusion and/or hydroxyurea-versus conservative management plus surgical revascularization (surgery group). We monitored cerebrovascular event (CVE) rates-a composite of strokes and transient ischemic attacks. Multivariable logistic regression was used to compare CVE occurrence and multivariable Poisson regression was used to compare incidence rates between groups. Covariates in multivariable models included age at treatment start, age at moyamoya diagnosis, antiplatelet use, CVE history, and the risk period length. RESULTS: We identified 141 patients with SCD-MMS, 78 (55.3%) in the surgery group and 63 (44.7%) in the conservative group. Compared with the conservative group, preoperatively the surgery group had a younger age at moyamoya diagnosis, worse baseline modified Rankin scale scores, and increased prevalence of CVEs. Despite more severe pretreatment disease, the surgery group had reduced odds of new CVEs after surgery (odds ratio = 0.27, 95% confidence interval [CI] = 0.08-0.94, p = .040). Furthermore, comparing surgery group patients during presurgical versus postsurgical periods, CVEs odds were significantly reduced after surgery (odds ratio = 0.22, 95% CI = 0.08-0.58, p = .002). CONCLUSIONS: When added to conservative management, cerebral revascularization surgery appears to reduce the risk of CVEs in patients with SCD-MMS. A prospective study will be needed to validate these findings.

Current state and future direction of quality improvement in pediatric neurosurgery: a survey of pediatric neurosurgeons.

OBJECTIVE: Quality improvement (QI) is a methodology used to implement sustainable, meaningful change to improve patient outcomes. Given the complex pathologies observed in pediatric neurosurgery, QI projects could potentially improve patient care. Overall, there is a need to characterize the degree of QI opportunities, training, and initiatives within the field of pediatric neurosurgery. Herein the authors aimed to define the current QI landscape in pediatric neurosurgery. METHODS: A cross-sectional survey was sent to all members of the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Section on Pediatric Neurological Surgery via email. The responses were anonymized. Questions addressed several relatable QI topics including 1) training and participation in QI; 2) QI infrastructure; 3) QI program incentives; and 4) general opinions on the National Surgical Quality Improvement Program (NSQIP) database, various QI topics, and QI productivity. RESULTS: Responses were received from 129 participants (20% response rate). Most respondents practiced in an academic setting (59.8%) and at a free-standing pediatric hospital (65.4%). Participation in QI projects was high (81.7%), but only 23.8% of respondents had formal QI training. Only 36.5% of respondents had institutional requirements for QI work; the majority of those were only required to participate as a project team member. Nearly half of the respondents did not receive incentives or institutional support for QI. The majority agreed ("strongly" and "somewhat") that a QI course would be beneficial (75.5%), that QI projects should be considered for publication in neurosurgery journals (88.1%), and that there is a need for national quality metrics (81.4%). Over 88% have an interest in seeing QI project presentations at the annual Pediatric Joint Section meeting. Only 26.3% believed that the NSQIP was a useful QI guide. Respondents suggested further study of the following QI topics: overall rates of infection and their prevention, hydrocephalus, standardized treatment algorithms for common disorders, team communication, pediatric neurosurgery-specific database, access to care, and interprofessional education. CONCLUSIONS: Areas of opportunity include specialty-specific QI education, tactics for obtaining support to build the QI infrastructure, increased visibility of QI work within pediatric neurosurgery, and a review of available registries to provide readily available data relevant to this specialty.

Treatment of Sagittal Craniosynostosis Using Osteoplastic Parietal Craniotomy Flap With Distraction Osteogenesis: A Variation on Technique.

ABSTRACT: Late presentation sagittal craniosynostosis presents a unique challenge due to the decreased ability of the skull to repair the bony defects created by standard of care techniques. Distraction osteogenesis is a viable strategy to correct this defect in late presenting cases. The authors describe a variation in technique in which the temporalis muscle origin is retained, creating an osteoplastic bone flap with retained vascularity through the tem-poralis muscle. This may improve postoperative bony healing of bony defects in this compromised population. The authors present two patients who presented to them late with sagittal synostosis who were treated with distraction osteogenesis in which vascular continuity to the parietal bones is preserved through the temporalis muscle.

Diagnostic Accuracy of Non-Invasive Thermal Evaluation of Ventriculoperitoneal Shunt Flow in Shunt Malfunction: A Prospective, Multi-Site, Operator-Blinded Study.

BACKGROUND: Thermal flow evaluation (TFE) is a non-invasive method to assess ventriculoperitoneal shunt function. Flow detected by TFE is a negative predictor of the need for revision surgery. Further optimization of testing protocols, evaluation in multiple centers, and integration with clinical and imaging impressions prompted the current study. OBJECTIVE: To compare the diagnostic accuracy of 2 TFE protocols, with micropumper (TFE+MP) or without (TFE-only), to neuro-imaging in patients emergently presenting with symptoms concerning for shunt malfunction. METHODS: We performed a prospective multicenter operator-blinded trial of a consecutive series of patients who underwent evaluation for shunt malfunction. TFE was performed, and preimaging clinician impressions and imaging results were recorded. The primary outcome was shunt obstruction requiring neurosurgical revision within 7 d. Non-inferiority of the sensitivity of TFE vs neuro-imaging for detecting shunt obstruction was tested using a prospectively determined a priori margin of -2.5%. RESULTS: We enrolled 406 patients at 10 centers. Of these, 68/348 (20%) evaluated with TFE+MP and 30/215 (14%) with TFE-only had shunt obstruction. The sensitivity for detecting obstruction was 100% (95% CI: 88%-100%) for TFE-only, 90% (95% CI: 80%-96%) for TFE+MP, 76% (95% CI: 65%-86%) for imaging in TFE+MP cohort, and 77% (95% CI: 58%-90%) for imaging in the TFE-only cohort. Difference in sensitivities between TFE methods and imaging did not exceed the non-inferiority margin. CONCLUSION: TFE is non-inferior to imaging in ruling out shunt malfunction and may help avoid imaging and other steps. For this purpose, TFE only is favored over TFE+MP.

Readmission and reoperation for hydrocephalus: a population-based analysis across the spectrum of age.

OBJECTIVE: Hydrocephalus is a common, chronic illness that generally requires lifelong, longitudinal, neurosurgical care. Except at select research centers, surgical outcomes in the United States have not been well documented. Comparative outcomes across the spectrum of age have not been studied. METHODS: Data were derived for the year 2015 from the Nationwide Readmissions Database, a product of the Healthcare Cost and Utilization Project of the Agency for Healthcare Research and Quality. In this data set patients are assigned state-specific codes that link repeated discharges through the calendar year. Discharges with diagnostic codes for hydrocephalus were extracted, and for each patient the first discharge defined the index admission. The study event was readmission. Observations were censored at the end of the year. In a similar fashion the first definitive surgical procedure for hydrocephalus was defined as the index operation, and the study event was reoperation for hydrocephalus or complications. Survival without readmission and survival without reoperation were analyzed using life tables and Kaplan-Meier plots. RESULTS: Readmission rates at 30 days ranged between 15.6% and 16.8% by age group without significant differences. After the index admission the first readmission alone generated estimated hospital charges of $2.25 billion nationwide. Reoperation rates at 30 days were 34.9% for infants, 39.2% for children, 47.4% for adults, and 32.4% for elders. These differences were highly significant. More than 3 times as many index operations were captured for adults and elders as for infants and children. Estimated 1-year reoperation rates were 74.2% for shunt insertion, 63.9% for shunt revision, but only 34.5% for endoscopic third ventriculostomy. Univariate associations with survival without readmission and survival without reoperation are presented. CONCLUSIONS: In the United States hydrocephalus is predominantly a disease of adults. Surgical outcomes in this population-based study were substantially worse than outcomes reported from research centers. High reoperation rates after CSF shunt surgery accounted for this discrepancy.

Adults with myelomeningocele and other forms of spinal dysraphism: hospital care in the United States since the turn of the millennium.

OBJECTIVE The natural history and management of myelomeningocele (MM) in children is fairly well understood. There is a deficiency of knowledge regarding the care of adults, however, even though there are now more adults than children living with MM. The purpose of this study was to characterize the hospital care of adults with MM and hydrocephalus on a nationwide population base. Adults with other forms of spina bifida (SB) were studied for contrast. METHODS The Nationwide Inpatient Sample for the years 2001, 2004, 2007, and 2010 was queried for admissions with diagnostic ICD-9-CM codes for MM with hydrocephalus and for other forms of SB. RESULTS There were 4657 admissions of patients with MM and 12,369 admissions of patients with SB in the sample. Nationwide rates of admission increased steadily for both MM and SB patients throughout the study period. Hospital charges increased faster than the health care component of the Consumer Price Index. Patients with MM were younger than patients with SB, but annual admissions of MM patients older than 40 years increased significantly during the study period. With respect to hospital death and discharge home, outcomes of surgery for hydrocephalus were superior at high-volume hospitals. Patients with MM and SB were admitted to the hospital more frequently than the general population for surgery to treat degenerative spine disease. CONCLUSIONS Patients with MM and SB continue to require neurosurgical attention in adulthood, and the demand for services for older patients with MM is increasing. Management of hydrocephalus at high-volume centers is advantageous for this population. Patients with MM or SB may experience high rates of degenerative spine disease.

Beta Hairpin Peptide Hydrogels as an Injectable Solid Vehicle for Neurotrophic Growth Factor Delivery.

There is intense interest in developing novel methods for the sustained delivery of low levels of clinical therapeutics. MAX8 is a peptide-based beta-hairpin hydrogel that has unique shear thinning properties that allow for immediate rehealing after the removal of shear forces, making MAX8 an excellent candidate for injectable drug delivery at a localized injury site. The current studies examined the feasibility of using MAX8 as a delivery system for nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF), two neurotrophic growth factors currently used in experimental treatments of spinal cord injuries. Experiments determined that encapsulation of NGF and BDNF within MAX8 did not negatively impact gel formation or rehealing and that shear thinning did not result in immediate growth factor release. ELISA, microscopy, rheology, and Western blotting experiments collectively demonstrate the functional capabilities of the therapeutic-loaded hydrogels to (i) maintain a protective environment against in vitro degradation of encapsulated therapeutics for at least 28 days; and (ii) allow for sustained release of NGF and BDGF capable of initiating neurite-like extensions of PC12 cells, most likely due to NGF/BDGF signaling pathways. Importantly, while the 21 day release profiles could be tuned by adjusting the MAX8 hydrogel concentration, the initial shear thinning of the hydrogel (e.g., during injection) does not induce significant premature loss of the encapsulated therapeutic, most likely due to effective trapping of growth factors within structurally robust domains that are maintained during the application of shear forces. Together, our data suggests that MAX8 allows for greater dosage control and sustained therapeutic growth factor delivery, potentially alleviating side effects and improving the efficacy of current therapies.

Incidental findings on brain and spine imaging in children.

In recent years, the utilization of diagnostic imaging of the brain and spine in children has increased dramatically, leading to a corresponding increase in the detection of incidental findings of the central nervous system. Patients with unexpected findings on imaging are often referred for subspecialty evaluation. Even with rational use of diagnostic imaging and subspecialty consultation, the diagnostic process will always generate unexpected findings that must be explained and managed. Familiarity with the most common findings that are discovered incidentally on diagnostic imaging of the brain and spine will assist the pediatrician in providing counseling to families and in making recommendations in conjunction with a neurosurgeon, when needed, regarding additional treatments and prognosis.

Thirty-day outcomes of cerebrospinal fluid shunt surgery: data from the National Surgical Quality Improvement Program-Pediatrics.

OBJECT: Cerebrospinal fluid shunts are the mainstay of the treatment of hydrocephalus. In past studies, outcomes of shunt surgery have been analyzed based on follow-up of 1 year or longer. The goal of the current study is to characterize 30-day shunt outcomes, to identify clinical risk factors for shunt infection and failure, and to develop statistical models that might be used for risk stratification. METHODS: Data for 2012 were obtained from the National Surgical Quality Improvement Program-Pediatrics (NSQIP-P) of the American College of Surgeons. Files with index surgical procedures for insertion or revision of a CSF shunt composed the study set. Returns to the operating room within 30 days for shunt infection and for shunt failure without infection were the study end points. Associations with a large number of potential clinical risk factors were analyzed on a univariate basis. Logistic regression was used for multivariate analysis. RESULTS: There were 1790 index surgical procedures analyzed. The overall rates of shunt infection and shunt failure without infection were 2.0% and 11.5%, respectively. Male sex, steroid use in the preceding 30 days, and nutritional support at the time of surgery were risk factors for shunt infection. Cardiac disease was a risk factor for shunt failure without infection, and initial shunt insertion, admission during the second quarter, and neuromuscular disease appeared to be protective. There was a weak association of increasing age with shunt failure without infection. Models based on these factors accounted for no more than 6% of observed variance. Construction of stable statistical models with internal validity for risk adjustment proved impossible. CONCLUSIONS: The precision of the NSQIP-P dataset has allowed identification of risk factors for shunt infection and for shunt failure without infection that have not been documented previously. Thirty-day shunt outcomes may be useful quality metrics, possibly even without risk adjustment. Whether important variation in 30-day outcomes exists among institutions or among neurosurgeons is yet unknown.

Quality measurement in the shunt treatment of hydrocephalus: analysis and risk adjustment of the Revision Quotient.

UNLABELLED: OBJECT.: The Revision Quotient (RQ) has been defined as the ratio of the number of CSF shunt revisions to the number of new shunt insertions for a particular neurosurgical practice in a unit of time. The RQ has been proposed as a quality measure in the treatment of childhood hydrocephalus. The authors examined the construct validity of the RQ and explored the feasibility of risk stratification under this metric. METHODS: The Kids' Inpatient Database for 1997, 2000, 2003, 2006, and 2009 was queried for admissions with diagnostic codes for hydrocephalus and procedural codes for CSF shunt insertion or revision. Revision quotients were calculated for hospitals that performed 12 or more shunt insertions annually. The univariate associations of hospital RQs with a variety of institutional descriptors were analyzed, and a generalized linear model of the RQ was constructed. RESULTS: There were 12,244 admissions (34%) during which new shunts were inserted, and there were 23,349 admissions (66%) for shunt revision. Three hundred thirty-four annual RQs were calculated for 152 different hospitals. Analysis of variance in hospital RQs over the 5 years of study data supports the construct validity of the metric. The following factors were incorporated into a generalized linear model that accounted for 41% of the variance of the measured RQs: degree of pediatric specialization, proportion of initial case mix in the infant age group, and proportion with neoplastic hydrocephalus. CONCLUSIONS: The RQ has construct validity. Risk adjustment is feasible, but the risk factors that were identified relate predominantly to patterns of patient flow through the health care system. Possible advantages of an alternative metric, the Surgical Activity Ratio, are discussed.

Quality measures for the management of hydrocephalus: concepts, simulations, and preliminary field-testing.

OBJECT: The authors define and examine the properties of 2 new, practice-based quality measures for the management of hydrocephalus. METHODS: The Surgical Activity Rate (SAR) is defined as the number of definitive operations for the treatment of hydrocephalus performed in a neurosurgical practice over the course of a year, divided by the number of patients with hydrocephalus seen in follow-up during that year. The Revision Quotient (RQ) is defined as the number of definitive revision operations performed in a neurosurgical practice in the course of a year, divided by the number of definitive initial operations during that year for patients with newly diagnosed hydrocephalus. Using published actuarial shunt survival data, the authors conducted Monte Carlo simulations of a pediatric neurosurgical practice to illustrate the properties and interpretations of the SAR and RQ. They used data from the Kids' Inpatient Database (KID) for 2009 to calculate RQs for hospitals accounting for more than 10 admissions coded for initial CSF shunt insertions. RESULTS: During the initial growth phase of a simulated neurosurgical practice, the SAR approached its steady-state value much earlier than the RQ. Both measures were sensitive to doubling or halving of monthly failure rates. In the 2009 KID, 117 hospitals reported more than 10 initial shunt insertions. The weighted mean (± standard deviation) RQ for these hospitals was 1.79 ± 0.69. Among hospitals performing 50 or more initial shunt insertions, the RQ ranged between 0.71 and 3.65. CONCLUSIONS: The SAR and RQ have attractive qualitative features as practice-based quality measures. The RQ, at least, exhibits clinically meaningful interhospital variation as applied to CSF shunt surgery. The SAR and RQ merit prospective field-testing as measures of quality in the management of childhood hydrocephalus.

Cerebellar gliomatosis in a toddler: case report of a challenging condition and review of the literature.

Gliomatosis confined to the cerebellum is most unusual. We report such a case in a 20-month-old male who presented with unsteadiness. Magnetic resonance imaging revealed a diffuse area of abnormal signal intensity within both cerebellar hemispheres, which did not enhance after contrast administration. The patient underwent a biopsy, which revealed a diffuse glioma infiltrating the cerebellum. Overall, the tumor cells had oligodendroglioma-like features and exhibited only focal vimentin immunoreactivity. They were negative for glial fibrillary acidic protein, synaptophysin, ßIII-tubulin, and neurofilament protein. Immunofluorescence, performed on primary biopsy explants maintained in cell culture without exposure to growth factors or differentiation-promoting agents, revealed widespread nestin immunoreactivity and immunolabeling of occasional cells with antibodies to platelet-derived growth factor-α and O1/O4, markers of oligodendrocyte precursor-cells and immature oligodendrocytes, respectively. Fluorescent in situ hybridization performed on explants, touch preparations, and paraffin sections failed to reveal loss of heterozygosity for either 1p36 or 19q13. The patient was treated with temozolomide and remains stable, albeit with residual quiescent tumor, more than 3 years after surgery. This report calls attention to an unusual presentation of gliomatosis confined to the cerebellum of a toddler and addresses salient aspects of clinical and radiological differential diagnosis, as well as therapeutic challenges encountered.

Developmental anatomy of the atlas and axis in childhood by computed tomography.

OBJECT: The CT modality plays a central role in the diagnosis of cervical spine fractures. In childhood, radiolucent synchondroses between ossification centers can resemble fractures, and they can be the sites of fractures as well. Recognition of cervical spine fractures in children requires familiarity with normal developmental anatomy and common variants as they appear on CT scans. METHODS: A convenience sample of 932 CT scans of the cervical spine accessible on the picture archiving and communications system (known as PACS) at a single children's hospital was examined. Scans were excluded from further analysis if they did not include the atlantoaxial region or were otherwise technically unsatisfactory; if the patient carried the diagnosis of a skeletal dysplasia; or if there were developmental lesions noted at other levels of the spine. No more than 1 scan per patient was analyzed. Synchondroses were graded as radiolucent, not totally radiolucent but still visible, or no longer visible. Their locations and symmetries were noted. The presence or absence of the tubercles of the transverse ligament was noted as well. RESULTS: After exclusions, 841 studies of the atlas and 835 studies of the axis were analyzed. The 3 common ossification centers of the atlas arose in the paired neural arches and the anterior arch, but in as many as 20% of cases the anterior arch developed from paired symmetrical ossification centers. The 5 common ossification centers of the axis arose in the paired neural arches, in the basal center, in the dentate center (from which most of the dentate process develops), and in the very apex of the dentate process. The appearance of each synchondrosis was noted at sequential ages. The tubercles for the transverse ligament generally did not appear until the ossification of the synchondroses of the atlas was far advanced. Anomalies of the atlas included anterior and posterior spina bifida, absence of sectors of the posterior arch, and anomalous ossification centers and synchondroses. Anomalies of the axis were much less common. What appeared possibly to be chronic, incompletely healed fractures of the atlas were discovered on review for this analysis in 6 cases. No fractures of the axis were discovered. CONCLUSIONS: There is substantial variation in the time course and pattern of development of the atlas, and anomalies are common. Some fractures of the atlas may escape recognition without manifest sequelae. Variation in the time course of the development of the axis is notable as well, but anomalies seem much less common.

Intracranial localisation of ictal urinary urge epileptogenic zone to the non-dominant temporal lobe.

Ictal urinary urge is a rare symptom of focal epilepsy usually localising to the non-dominant hemisphere, specifically, the temporal lobe. Lateralisation in previously described cases has been established using scalp video-EEG monitoring or functional imaging. We report the case of a 19-year-old girl with refractory epilepsy and ictal urinary urge arising from the non-dominant temporal lobe, confirmed by invasive, subdural EEG monitoring. Since undergoing a temporal lobectomy two and a half years ago, the patient has not experienced ictal urinary urge. To our knowledge, this is the first report demonstrating localisation of ictal urinary urge epileptogenic zone to the non-dominant temporal lobe by invasive intracranial monitoring.

Treatment of myelomeningocele: a review of outcomes and continuing neurosurgical considerations among adults.

OBJECT: Myelomeningocele is the most severe congenital malformation of the CNS that is compatible with survival. From the time of the development of practical treatment for hydrocephalus in the late 1950s, affected individuals began to survive into adulthood in substantial numbers. Data on the neurological status of these individuals are sparse, as are descriptions of their continuing requirements for neurosurgical care. METHODS: A review of the literature was undertaken using the PubMed database maintained by the National Library of Medicine. Formal grading of the quality of evidence was not attempted, but methodological issues affecting validity or generalizability were noted. RESULTS: Observations from 2 major longitudinal studies of cohorts of patients treated without selection using contemporary neurosurgical techniques have been published at intervals beginning in the mid-1970s. Numerous cross-sectional, institutional reviews have focused on neurosurgical issues in adulthood: hydrocephalus, Chiari malformation Type II and syringomyelia, and secondary spinal cord tethering. The organization of medical services for adults with myelomeningocele has received limited study. CONCLUSIONS: Surviving adults with myelomeningocele achieve a wide range of neurological and functional outcomes, the most critical and adverse determinant of which is symptomatic CSF shunt failure. From a neurosurgical standpoint, adults with myelomeningocele remain clinically active indefinitely, and they deserve periodic neurosurgical surveillance.