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.

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.

Pediatric spinal injury in the US: epidemiology and disparities.

OBJECT: In the US, race and economic status have pervasive associations with mechanisms of injury, severity of injury, management, and outcomes of trauma. The goal of the current study was to examine these relationships on a large scale in the setting of pediatric spinal injury. METHODS: Admissions for spinal fracture without or with spinal cord injury (SCI), spinal dislocation, and SCI without radiographic abnormality were identified in the Kids' Inpatient Database (KID) and the National Trauma Data Bank (NTDB) registry for 2009. Patients ranged in age from birth up to 21 years. Data from the KID were used to estimate nationwide annual incidences. Data from the NTDB were used to describe patterns of injury in relation to age, race, and payor, with corroboration from the KID. Multiple logistic regression was used to model rates of mortality and spinal fusion. RESULTS: In 2009, the estimated incidence of hospital admission for spinal injury in the US was 170 per 1 million in the population under 21 years of age. The incidence of SCI was 24 per 1 million. Incidences varied regionally. Adolescents predominated. Patterns of injury varied by age, race, and payor. Black patients were more severely injured than patients of other races as measured by Injury Severity Scale scores. Among black patients with spinal injury in the NTDB, 23.9% suffered firearm injuries; only 1% of white patients suffered firearm injuries. The overall mortality rate in the NTDB was 3.9%. In a multivariate analysis that included a large panel of clinical and nonclinical factors, black race retained significance as a predictor of mortality (p = 0.006; adjusted OR 1.571 [1.141-2.163]). Rates of spinal fusion were associated with race and payor in the NTDB data and with payor in the KID: patients with better insurance underwent spinal fusion at higher rates. CONCLUSIONS: The epidemiology of pediatric spinal injury in the US cannot be understood apart from considerations of race and economic status.

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.

Fracture of the anterior arch of atlas after minor trauma of the immature spine postlaminectomy.

Torticollis is a common complaint in the pediatric emergency department. Here, we report what we believe to be the first example in a young child of a fracture of the anterior arch of the atlas associated with an acquired, postsurgical defect of the posterior arch. A brief review of pediatric cervical spine injuries and fractures is presented. Atlas laminectomy may predispose patients to isolated atlas fractures even with minor trauma. Those fractures, however, are stable and treated by hard cervical orthosis.

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.

Hospital care of childhood traumatic brain injury in the United States, 1997-2009: a neurosurgical perspective.

OBJECT: The goal in this paper was to study hospital care for childhood traumatic brain injury (TBI) in a nationwide population base. METHODS: Data were acquired from the Kids' Inpatient Database (KID) for the years 1997, 2000, 2003, 2006, and 2009. Admission for TBI was defined by any ICD-9-CM diagnostic code for TBI. Admission for severe TBI was defined by a principal diagnostic code for TBI and a procedural code for mechanical ventilation; admissions ending in discharge home alive in less than 4 days were excluded. RESULTS: Estimated raw and population-based rates of admission for all TBI, for severe TBI, for death from severe TBI, and for major and minor neurosurgical procedures fell steadily during the study period. Median hospital charges for severe TBI rose steadily, even after adjustment for inflation, but estimated nationwide hospital charges were stable. Among 14,932 actual admissions for severe TBI captured in the KID, case mortality was stable through the study period, at 23.9%. In a multivariate analysis, commercial insurance (OR 0.86, CI 0.77-0.95; p = 0.004) and white race (OR 0.78, CI 0.70-0.87; p < 0.0005) were associated with lower mortality rates, but there was no association between these factors and commitment of resources, as measured by hospital charges or rates of major procedures. Increasing median income of home ZIP code was associated with higher hospital charges and higher rates of major and minor procedures. Only 46.8% of admissions for severe TBI were coded for a neurosurgical procedure of any kind. Fewer admissions were coded for minor neurosurgical procedures than anticipated, and the state-by-state variance in rates of minor procedures was twice as great as for major procedures. Possible explanations for the "missing ICP monitors" are discussed. CONCLUSIONS: Childhood brain trauma is a shrinking sector of neurosurgical hospital practice. Racial and economic disparities in mortality rates were confirmed in this study, but they were not explained by available metrics of resource commitment. Vigilance is required to continue to supply neurosurgical expertise to the multidisciplinary care process.

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.

Spinal cord injury without radiographic abnormality and the Chiari malformation: controlled observations.

BACKGROUND: Case reports have suggested an association between the Chiari malformation type 1 (CM1) and spinal cord injury without radiographic abnormality (SCIWORA). No controlled observations have appeared. We report a case-control study. METHODS: The Kids' Inpatient Database (KID) is a product of the Healthcare Utilization Project of the Agency for Healthcare Research and Quality. For the years 1997, 2000, 2003, 2006, and 2009, the KID includes discharge data abstracted from almost 14 million pediatric admissions. We hypothesized that CM1 predisposes children to SCIWORA in the cervicomedullary region, and we reasoned that, if this hypothesis were true, then KID admissions coded for upper cervical SCIWORA would be associated with CM1 more frequently than admissions coded for lower cervical SCIWORA. RESULTS: After exclusion of admissions with a variety of confounding diagnostic codes, we identified 754 admissions coded for upper cervical SCIWORA and 424 coded for lower cervical SCIWORA. Concurrent coding for CM1 was more prevalent among admissions coded for upper cervical SCIWORA than for lower cervical SCIWORA, but the difference did not attain statistical significance (p = 0.093; Fisher's exact test). CONCLUSIONS: These controlled observations fail to support the hypothesis that CM1 is associated with SCIWORA in childhood. If there is a real association, in the context of recent estimates of the prevalence of CM1 in the pediatric population, the magnitude of the incremental risk must be very small.

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 suppuration complicating sinusitis among children: an epidemiological and clinical study.

OBJECT: Intracranial abscess is a very infrequent complication of sinusitis among children. Case series, mostly small and focusing on surgical treatment, have appeared in the literature, but there has been no study on the epidemiology of this condition, nor has there been any large-scale study on the intensity of therapy or outcomes. METHODS: Data were drawn from the Kids' Inpatient Databases for 1997, 2000, 2003, and 2006 and the National Inpatient Sample for 2001. Cases were defined by the conjunction between the diagnostic codes for intracranial abscess and those for acute or chronic sinusitis or mastoiditis. RESULTS: Eight hundred forty-seven hospital admissions were captured. Over the 10 years of the study, admission rates ranged between 2.74 and 4.38 per million children per year. Boys were affected much more commonly than girls. Black children were affected out of proportion to their presence in the population. The overall incidence seemed to peak in early adolescence. Sinogenic cases had a marked seasonal pattern peaking in winter, but no seasonal variation was seen for otogenic cases. Asthma comorbidity was more prevalent among sinogenic cases. The mortality rate was 2%, and death occurred only among sinogenic cases. Moreover, sinogenic cases tended to require more intensive therapy, as measured by the number of procedures, and there was a trend toward less favorable discharge dispositions. Older patients and black patients were less likely to be discharged directly to home. At least a quarter of the cases were managed without neurosurgical intervention. White patients were treated without neurosurgery more often than others. CONCLUSIONS: Analysis of administrative data sets has yielded a descriptive picture of intracranial abscess complicating sinusitis among children, but the very low incidence of this condition impedes prospective clinical research directed at practical management issues.

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.