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OBJECTIVE The aims of this study were to review the National Spina Bifida Patient Registry (NSBPR) data set to study the rates of tethered spinal cord release (TCR) among patients with myelomeningocele and variability between centers, to compare TCR rates between males and females, and to study the relationships between TCR rates and other condition-specific characteristics. METHODS The NSBPR registry was queried to identify all patients with myelomeningocele. TCR rates were calculated over time using survival analyses; rates between centers and between males and females were compared. Cox proportional hazards models were constructed to identify relationships between TCR rates and sex, functional lesion level, ambulation status, treated hydrocephalus, and prior Chiari decompression. RESULTS Of 6339 patients with information about their operations, 1366 (21.5%) underwent TCR, with significant variability between centers. The majority (75.8%) underwent a single TCR. The annual TCR rate was linear between birth and 13 years (1.8%/year) but declined sharply from 14 to 21 years (0.7%/year). There was no period of time at which the TCR rate accelerated. There were no significant differences in TCR rates between males and females. TCR rate was not related to functional lesion level but was lower among nonambulators compared with community ambulators (p = 0.005) and among those with treated hydrocephalus (HR 0.30, p < 0.001), and higher among those having prior Chiari decompression (HR 1.71, p < 0.001). CONCLUSIONS These results extend the results of prior single-institution studies, demonstrate significant treatment variability between institutions, and challenge the traditional concept that tethering is related to spinal cord stretching due to spinal growth.

OBJECTIVE In this article, the authors describe the impact of the COVID-19 virtual match cycle and discuss approaches to optimize future cycles through applicant and neurosurgical education leadership insights. METHODS Anonymous surveys of neurosurgery program leaders (program directors and program chairs), program administrators (PAs), and 2020–2021 neurosurgery residency match applicants were distributed by the SNS, in conjunction with the Association of Resident Administrators in Neurological Surgery and AANS Young Neurosurgeons Committee. RESULTS Responses were received from 77 (67.0%) of 115 PAs, 119 (51.7%) of 230 program leaders, and 124 (44.3%) of 280 applicants representing geographically diverse regions. During the virtual application cycle relative to the previous year, programs received more Electronic Residency Application Service applications (mean 314.8 vs 285.3, p < 0.0001) and conducted more applicant interviews (mean 45.2 vs 39.9, p = 0.0003). More than 50% of applicants applied to > 80 programs; 60.3% received ≤ 20 interview invitations, and 9% received > 40 invitations. Overall, 65% of applicants completed ≤ 20 interviews, whereas 34.7% completed > 20 interviews. Program leaders described one 4-week home subinternship (93.3%) and two 4-week external subinternships (68.9%) as optimal neurosurgical exposure; 62.8% of program leaders found the standardized letter of recommendation template to be somewhat (47.5%) or significantly (15.3%) helpful. Applicants, PAs, and program leaders all strongly preferred a hybrid model of in-person and virtual interview options for future application cycles over all in-person or all virtual options. Ninety-three percent of applicants reported matching within their top 10–ranked programs, and 52.9% of programs matched residents within the same decile ranking as in previous years. CONCLUSIONS Optimizing a national strategy for the neurosurgery application process that prioritizes equity and reduces costs, while ensuring adequate exposure for applicants to gain educational opportunities and evaluate programs, is critical to maintain a successful training system.

OBJECTIVEThe purpose of this study was to determine the rate of decompression for Chiari malformation type II in individuals with myelomeningocele in the National Spina Bifida Patient Registry (NSBPR). In addition, the authors explored the variation in rates of Chiari II decompression across NSBPR institutions, examined the relationship between Chiari II decompression and functional lesion level of the myelomeningocele, age, and need for tracheostomy, and they evaluated for temporal trends in rates of Chiari II decompression.METHODSThe authors queried the NSBPR to identify all individuals with myelomeningocele between 2009 and 2015. Among these patients, they identified individuals who had undergone at least 1 Chiari II decompression as well as those who had undergone tracheostomy. For each participating NSBPR institution, the authors calculated the proportion of patients enrolled at that site who underwent Chiari II decompression. Logistic regression was performed to analyze the relationship between Chiari II decompression, functional lesion level, age at decompression, and history of tracheostomy.RESULTSOf 4448 individuals with myelomeningocele identified from 26 institutions, 407 (9.15%) had undergone at least 1 Chiari II decompression. Fifty-one patients had undergone tracheostomy. Logistic regression demonstrated a statistically significant relationship between Chiari II decompression and functional lesion level of the myelomeningocele, with a more rostral lesion level associated with a higher likelihood of posterior fossa decompression. Similarly, children born before 2005 and those with history of tracheostomy had a significantly higher likelihood of Chiari II decompression. There was no association between functional lesion level and need for tracheostomy. However, among those children who underwent Chiari II decompression, the likelihood of also undergoing tracheostomy increased significantly with younger age at decompression.CONCLUSIONSThe rate of Chiari II decompression in patients with myelomeningocele in the NSBPR is consistent with that in previously published literature. There is a significant relationship between Chiari II decompression and functional lesion level of the myelomeningocele, which has not previously been reported. Younger children who undergo Chiari II decompression are more likely to have undergone tracheostomy. There appears to be a shift away from Chiari II decompression, as children born before 2005 were more likely to undergo Chiari II decompression than those born in 2005 or later.

OBJECTIVEAlthough the majority of patients with myelomeningocele have hydrocephalus, reported rates of hydrocephalus treatment vary widely. The purpose of this study was to determine the rate of surgical treatment for hydrocephalus in patients with myelomeningocele in the National Spina Bifida Patient Registry (NSBPR). In addition, the authors explored the variation in shunting rates across NSBPR institutions, examined the relationship between hydrocephalus, and the functional lesion level of the myelomeningocele, and evaluated for temporal trends in rates of treated hydrocephalus.METHODSThe authors queried the NSBPR to identify all patients with myelomeningoceles. Individuals were identified as having been treated for hydrocephalus if they had undergone at least 1 hydrocephalus-related operation. For each participating NSBPR institution, the authors calculated the proportion of patients with treated hydrocephalus who were enrolled at that site. Logistic regression was performed to analyze the relationship between hydrocephalus and the functional lesion level of the myelomeningocele and to compare the rate of treated hydrocephalus in children born before 2005 with those born in 2005 or later.RESULTSA total of 4448 patients with myelomeningocele were identified from 26 institutions, of whom 3558 patients (79.99%) had undergone at least 1 hydrocephalus-related operation. The rate of treated hydrocephalus ranged from 72% to 96% among institutions enrolling more than 10 patients. This difference in treatment rates between centers was statistically significant (p < 0.001). Insufficient data were available in the NSBPR to analyze reasons for the different rates of hydrocephalus treatment between sites. Multivariate logistic regression demonstrated that more rostral functional lesion levels were associated with higher rates of treated hydrocephalus (p < 0.001) but demonstrated no significant difference in hydrocephalus treatment rates between children born before versus after 2005.CONCLUSIONSThe rate of hydrocephalus treatment in patients with myelomeningocele in the NSBPR is 79.99%, which is consistent with the rates in previously published literature. The authors’ data demonstrate a clear association between functional lesion level of the myelomeningocele and the need for hydrocephalus treatment.

Object Tectal plate gliomas are generally low-grade astrocytomas with favorable prognosis, and observation of the lesion and management of hydrocephalus remain the mainstay of treatment. Methods A cohort of patients with tectal plate gliomas at 2 academic institutions was retrospectively reviewed. Results Forty-four patients with a mean age of 10.2 years who harbored tectal plate gliomas were included in the study. The mean clinical and radiological follow-up was 7.6 ± 3.3 years (median 7.9 years, range 1.5–14.7 years) and 6.5 ± 3.1 years (median 6.5 years, range 1.1–14.7 years), respectively. The most frequent intervention was CSF diversion (81.8% of patients) followed by biopsy (11.4%), radiotherapy (4.5%), chemotherapy (4.5%), and resection (2.3%). On MR imaging tectal plate gliomas most commonly showed T1-weighted isointensity (71.4%), T2-weighted hyperintensity (88.1%), and rarely enhanced (19%). The initial mean volume was 1.6 ± 2.2 cm3 and it increased to 2.0 ± 4.4 cm3 (p = 0.628) at the last follow-up. Frontal and occipital horn ratio (FOHR) and third ventricular width statistically decreased over time (p < 0.001 and p < 0.05, respectively). Conclusions The authors' results support existing evidence that tectal plate gliomas frequently follow a benign clinical and radiographic course and rarely require any intervention beyond management of associated hydrocephalus.

An in utero female was found to have a small hemorrhage at the foramen of Monro, hydrocephalus, and what was originally interpreted as a Dandy-Walker variant. At birth she had macrocephaly and numerous cutaneous, multifocal, red-pink blanchable macules. Postnatal MRI demonstrated a hemorrhagic soft-tissue mass involving the upper brainstem, thalamus, and basal ganglia most consistent with in utero complex multifocal intracranial hemorrhage. The skin lesions were thought to be consistent with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). The size and location of the hemorrhage precluded operative intervention, although the hydrocephalus was treated with a ventricular shunt. The child continues to have severe developmental delays. Multifocal lymphangioendotheliomatosis with thrombocytopenia is a multifocal vascular disorder most commonly involving the skin and gastrointestinal tract. Intracranial hemorrhages are rare in this context. This case is the third reported instance of MLT with associated intracranial hemorrhage and the only case described in the neurosurgical literature. The authors review the presenting features and pathophysiology of this condition.

Object Research suggests that there may be a growing disparity between the supply of and demand for both pediatric specialists and neurosurgeons. Whether pediatric neurosurgeons are facing such a disparity is disputable, but interest in pediatric neurosurgery (PNS) has waxed and waned as evidenced by the number of applicants for PNS fellowships. The authors undertook a survey to analyze current neurosurgical residents' perceptions of both benefits and deterrents to a pediatric neurosurgical career. Methods All residents and PNS fellows in the United States and Canada during the academic year 2008–2009 were invited to complete a Web-based survey that assessed 1) demographic and educational information about residents and their residency training, particularly as it related to training in PNS; 2) residents' exposure to mentoring opportunities from pediatric neurosurgical faculty and their plans for the future; and 3) residents' perceptions about how likely 40 various factors were to influence their decision about whether to pursue a PNS career. Results Four hundred ninety-six responses were obtained: 89% of the respondents were male, 63% were married, 75% were in at least their 3rd year of postgraduate training, 61% trained in a children's hospital and 29% in a children's “hospital within a hospital,” and 72% were in programs having one or more dedicated PNS faculty members. The residencies of 56% of respondents offered 6–11 months of PNS training and nearly three-quarters of respondents had completed 2 months of PNS training. During medical school, 92% had been exposed to neurosurgery and 45% to PNS during a clinical rotation, but only 7% identified a PNS mentor. Nearly half (43%) are considering a PNS career, and of these, 61% are definitely or probably considering post-residency fellowship. On the other hand, 68% would prefer an enfolded fellowship during residency. Perceived strengths of PNS included working with children, developing lasting relationships, wider variety of operations, fast healing and lack of comorbidities, and altruism. Perceived significant deterrents included shunts, lower reimbursement, cross-coverage issues, higher malpractice premiums and greater legal exposure, and working with parents and pediatric health professionals. The intrinsic nature of PNS was listed as the most significant deterrent (46%) followed by financial concerns (25%), additional training (12%), longer work hours (12%), and medicolegal issues (4%). The majority felt that fellowship training and PNS certification should be recommended for surgeons treating of all but traumatic brain injuries and Chiari I malformations and performing simple shunt-related procedures, although they felt that these credentials should be required only for treating complex craniosynostosis. Conclusions The nature of PNS is the most significant barrier to attracting residents, although reimbursement, cross-coverage, and legal issues are also important to residents. The authors provide several recommendations that might enhance resident perceptions of PNS and attract trainees to the specialty.

Object The appearance and evolution of neuroimaging abnormalities following abusive head trauma (AHT) is important for establishing the time frame over which these injuries might have occurred. From a legal perspective this frames the timing of the abuse and therefore identifies and excludes potential perpetrators. A previous pilot study involving 33 infants with AHT helped to refine the timing of these injuries but was limited by its small sample size. In the present study, the authors analyzed a larger group of 210 cases involving infants with AHT to chronicle the first appearance and evolution of radiological (CT, MRI) abnormalities. Methods All children younger than 24 months admitted to the Penn State Hershey Medical Center with AHT over a 10-year period were identified from a medical record review; the time of injury was determined through an evaluation of the clinical records. All imaging studies were analyzed, and the appearance and evolution of abnormalities were chronicled on serial neuroimaging studies obtained in the days and weeks after injury. Results One hundred five infants with specific injury dates and available imaging studies were identified; a subset of 43 children additionally had documented times of injury. In infants with homogeneously hyperdense subdural hematomas (SDHs) on initial CT scans, the first hypodense component appeared within the SDH between 0.3 and 16 days after injury, and the last hyperdense subdural component disappeared between 2 and 40 days after injury. In infants with mixed-density SDHs on initial scans, the last hyperdense component disappeared between 1 and 181 days. Parenchymal hypodensities appeared on CT scans performed as early as 1.2 hours, and all were visible within 27 hours after the injury. Rebleeding into SDHs was documented in 17 cases (16%) and was always asymptomatic. Magnetic resonance imaging of the brain was performed in 49 infants. Among those with SDH, 5 patterns were observed. Patterns I and II reflected homogeneous SDH; Pattern I (T1 hyperintensity and T2/FLAIR hypointensity, “early subacute”) more commonly appeared on scans performed earlier after injury compared with Pattern II (T1 hyperintensity and T2/FLAIR hyperintensity, “late subacute”), although there was considerable overlap. Patterns III and IV reflected heterogeneous SDH; Pattern III contained relatively equal mixtures having different intensities, whereas Pattern IV had fluid that was predominantly T1 hypointense and T2/FLAIR hyperintense. Again, Pattern III more commonly appeared on scans performed earlier after injury compared with Pattern IV, although there was significant overlap. Conclusions These data extend the preliminary data reported by Dias and colleagues and provide a framework upon which injuries in AHT can be timed as well as the limitations on such timing estimates.

Primary intramedullary spinal cord germinoma (PISCG) is an exceedingly rare diagnosis, with fewer than 30 cases reported in the literature. It is even less common in the pediatric population. Usually, initial imaging at patient presentation reveals a mass. The authors describe the unique case of a child whose initial imaging showed only focal spinal cord atrophy, which was the earliest sign of a slowly growing intramedullary lesion that was eventually proven via biopsy to represent a PISCG. The authors outline this child's diagnostically challenging presentation, review the events leading up to a diagnosis, briefly discuss PISCG, and summarize their recommendations for other physicians who may encounter a similar case. They assert that PISCG should be considered as a rare entity in the differential diagnosis of progressive spinal cord dysfunction even in the absence of an MRI abnormality of an intrinsic spinal cord mass, especially if there is unexplained focal atrophy of the cord.

Purpose The American College of Surgeons (ACS) National Surgical Quality Improvement Program Pediatric (NSQIP-P) expanded to beta phase testing with the enrollment of 29 institutions. Data collection and analysis were aimed at program refinement and development of risk-adjusted models for inter-institutional comparisons. Methods Data from the first full year of beta-phase NSQIP-P were analyzed. Patient accrual used ACS-NSQIP methodology tailored to pediatric specialties. Preliminary risk adjusted modeling for all pediatric and neonatal operations and pediatric (excluding neonatal) abdominal operations was performed for all cause morbidity (other than death) and surgical site infections (SSI) using hierarchical logistic regression methodology and eight predictor variables. Results were expressed as odds ratios with 95% confidence intervals. Results During calendar year 2010, 29 institutions enrolled 37,141 patients. 1644 total CPT codes were entered, of which 456 accounted for 90% of the cases. 450 codes were entered only once (1.2% of cases). For all cases, overall mortality was 0.25%, overall morbidity 7.9%, and the SSI rate 1.8%. For neonatal cases, mortality was 2.39%, morbidity 18.7%, and the SSI rate 3%. For the all operations model, risk-adjusted morbidity institutional odds ratios ranged 0.48–2.63, with 9/29 hospitals categorized as low outliers and 9/29 high outliers, while risk-adjusted SSI institutional odds ratios ranged 0.36–2.04, with 2/29 hospitals low outliers and 7/29 high outliers. Conclusion This report represents the first risk-adjusted hospital-level comparison of surgical outcomes in infants and children using NSQIP-P data. Programmatic and analytic modifications will improve the impact of this program as it moves into full implementation. These results indicate that NSQIP-P has the potential to serve as a model for determining risk-adjusted outcomes in the neonatal and pediatric population with the goal of developing quality improvement initiatives for the surgical care of children.

Depressed skull fractures in neonates involve the inward buckling of the skull bones to resemble a cup shape or “ping-pong ball” shape. In the past, several methods have been used to treat these fractures for cosmetic effect, including the use of vacuum extraction. The aim of this study was to demonstrate the use of a novel treatment method for these skull fractures. All patients presented to Hershey Medical Center between May 2007 and March 2010. All underwent treatment of their ping-pong skull fracture using a novel technique of percutaneous screw elevation with self-tapping microscrews (4 or 5 mm) typically used in neurosurgery for cranial plating. Four patients were treated, all between the ages of 2 days and 4 months. Two of the patients were treated under general anesthesia and 2 with local anesthesia only. All patients had good cosmetic results and experienced no adverse events. Percutaneous screw elevation of the ping-pong type, simple depressed skull fracture in neonates is a feasible method of treatment with a low complication rate associated with the procedure and no risk from anesthesia.

Object The thresholds for shunting CSF in children with asymptomatic hydrocephalus are unclear; there are neither guidelines nor sufficient research to determine what degree of hydrocephalus should be treated. The authors hypothesize that 1) pediatric neurosurgeons currently have high thresholds for recommending treatment for these children, but 2) there is significant variability among these treatment thresholds. Methods The authors surveyed attendees of the Joint Pediatric Neurosurgery Section meeting in Spokane, Washington, in December 2008, regarding their treatment thresholds for 22 clinical scenarios. Each participant was provided an illustration of 5 imaging studies (3 slices each) showing progressively larger ventricles. For each scenario, respondents were asked to indicate the minimum ventricular size they would treat, if any. Responses were quantified from 1 to 6 from smaller to larger, with 6 being no treatment, and a mean theoretical treated ventricular size (MTTVS) was calculated for each scenario. Results Respondents were relatively conservative in recommending treatment, with MTTVSs of 3.7–6.0; in 13 scenarios, the MTTVS was greater than 5.0 (larger than the largest presented ventricular size). For scenarios in which a mean frontooccipital ratio could be calculated, the value ranged from 0.55 to 0.67 (moderate to severe hydrocephalus). Although there were clear majority responses for each scenario, there was also significant variability. There were no patterns of association with the respondent's age, training, board certification, or type or location of practice. Conclusions This study demonstrates that pediatric neurosurgeons' thresholds for treating asymptomatic children with hydrocephalus are generally high, but there is also significant variability.

Background Neural tube defects (NTDs), including spina bifida and anencephaly, are the second most common birth defect with an incidence of 1/1000. Genetic factors are believed to contribute to NTD risk and family-based studies can be useful for identifying such risk factors. Methods We ascertained 1066 NTD families (1467 affected patients), including 307 multiplex NTD families. We performed pedigree analysis to describe the inheritance patterns, pregnancy outcomes, and recurrence risks to relatives of various types. Results Myelomeningocele or spina bifida (66.9%) and cranial defects (17.7%) were the most common NTD subtypes observed. The overall male:female ratio for affected individuals was 0.82, and there were even fewer males among individuals with an upper level NTD (0.62). Among twins, 2 of the 5 monozygotic twins and only 3 of 35 dizygotic twins were concordant, while 27% of the same sex twins were concordant, but none of the different sex twins. The estimated 6.3% recurrence risk to siblings (CI 0.04-0.08) is consistent with previous reports. Families with two or more affected individuals show a higher proportion of female transmitters (p = 0.0002). Additionally, the number of affected relatives in maternal compared to paternal lineages was more than double (p = 0.006). There were significantly more miscarriages, infant deaths, and stillborn pregnancies of the maternal aunts and uncles (p Conclusions Our data provide several lines of evidence consistent with a maternal effect, as well as a sex-influenced effect, in the etiology of NTDs.

Educational efforts have been shown to decrease the incidence of abusive injuries. Information related to factors that associate with child abuse would further these educational efforts. We undertook this study to determine the influence of temporal factors and a national preventative program on the incidence of abusive injuries in young children.The Pennsylvania Trauma Outcome Study database was queried for all children 36 months old or younger admitted with abusive injuries. Data collected included day, month, and season. Data were analyzed by Poisson regression.Six hundred seventy-two cases of child abuse were reported, with a linear increase in the number of cases per year (P.001). Sunday had fewer abuse cases admitted than any other day of the week (risk ratio, 0.70; P = .008). Younger children were less likely to be injured during April, which is National Child Abuse Prevention Month, and more likely to be injured during August and October (all P.05).We observed fewer inflicted injuries in young children on Sundays, and during April. The causes for these significant variations deserve further study.

OBJECTIVE: Children who live, work, and play on farms with barn design that includes hay-holes are at risk for a particular type of fall. This study retrospectively reviews all children admitted to a pediatric trauma center with injuries due to fall through a hay-hole over a 19-year period. This study is the first to specifically describe hay-hole fall injuries. METHODS: A retrospective review from a 19-year period at a rural pediatric trauma center identified 66 patients who sustained injuries from a hay-hole fall. Charts were reviewed for patient demographics, injuries, interventions, and hospital course. RESULTS: Sixty-six patients sustained injuries from hay-hole falls. Median patient age was 4 years, and median Injury Severity Score was 14. Forty-one percent of patients were admitted to the intensive care unit, and 26% of patients were intubated. Injuries included skull fracture (73%), facial fracture (27%), intracranial hemorrhage (53%), and noncraniofacial injuries (12%). Eighteen percent required a therapeutic intervention. There was 1 fatality (2%). CONCLUSIONS: Hay-hole fall appears to be a distinct injury mechanism, and patients present with different injury patterns than other types of falls. In this study, a high proportion of patients were young, and craniofacial injuries accounted for the majority of injuries. Only a small proportion of patients sustained noncraniofacial injuries. Injury prevention strategies should be targeted to this unique agrarian injury. Language: en

Cerebellar mutism syndrome (CMS) is a unique postoperative syndrome typically arising 1 to 2 days after resection of a midline posterior fossa tumor; it consists of diminished speech progressing to mutism, emotional lability, hypotonia, and ataxia. Most descriptions have been limited to small institutional series using a retrospective chart review methodology.The authors incorporated a CMS questionnaire in two large clinical trials (Children's Cancer Group [CCG] 9931, treatment for high-risk medulloblastoma/primitive neuroectodermal tumor; and CCG/Pediatric Oncology Group [POG] A9961, treatment for average-risk medulloblastoma) to prospectively survey for incidence, severity, and possible causes of CMS in children with newly diagnosed medulloblastoma. Information pertaining to 450 of the 463 patients enrolled in the studies was available for review (82 patients in CCG 9931, and 368 patients in CCG/POG A9961). Cerebellar mutism syndrome occurred in 107 (24%) of 450 children. Symptom intensity was judged to have been severe in 43%, moderate in 49%, and mild in 8% of these 107 patients. Mutism and ataxia were the features most frequently judged as severe. In both cohorts, preoperative brainstem invasion was the only feature that correlated with risk of CMS. One year after diagnosis, nonmotor speech/language deficits, neurocognitive deficits, and/or ataxia persisted in a significant fraction of patients.Nearly one quarter of patients who underwent resection of a medulloblastoma developed symptoms of CMS, of which 92% were judged to be of moderate or severe intensity. Brainstem invasion by tumor was the only risk factor that correlated positively with CMS occurrence; there was a negative correlation with cerebellar hemisphere tumor location. As more radical resections are attempted for medulloblastoma, the potential for increased morbidity must be carefully weighed against prognostic factors, especially in patients with brainstem invasion.

BACKGROUND Vitamin A (retinol), in the form of retinoic acid (RA), is essential for normal development of the human embryo. Studies in the mouse and zebrafish have shown that retinol is metabolized in the developing spinal cord and must be maintained in a precise balance along the anteroposterior axis. Both excess and deficiency of RA can affect morphogenesis, including failures of neural tube closure. METHODS We chose to investigate 5 genes involved in the metabolism or synthesis of RA, ALDH1A2, CYP26A1, CYP26B1, CRABP1, and CRABP2, for their role in the development of human neural tube defects, such as spina bifida. RESULTS An association analysis using both allelic and genotypic single-locus tests revealed a significant association between the risk for spina bifida and 3 polymorphisms in the gene ALDH1A2; however, we found no evidence of a significant multilocus association. CONCLUSIONS These results may suggest that polymorphisms in ALDH1A2 may influence the risk for lumbosacral myelomeningocele in humans. Birth Defects Research (Part A), 2005. © 2005 Wiley-Liss, Inc.

OBJECT: The authors conducted a study to determine clinical, patient/family satisfaction, and financial outcomes following application of a management scheme that involves evaluation of computerized tomography (CT) scans and emergency department observation, rather than overnight admission, for children who have sustained accidental minor closed head injuries (Glasgow Coma Scale Scores 13-15) and who have met predefined clinical and radiographic criteria. METHODS: During 18 consecutive months, all children age 24 months and older who sustained accidental minor head injuries were managed prospectively according to a standard protocol. All children meeting prospectively established clinical criteria underwent immediate CT scanning and were observed in the emergency department. Those in whom there were no intracranial radiographically demonstrated abnormalities and who met established clinical criteria were discharged to home observation. Two hundred fifteen children met the criteria for the study. Falls (53%) and motor vehicle accidents (13%) constituted the most common mechanisms of injury. Of the patients for whom information was recorded, 40% experienced a loss of consciousness and 49% had amnesia. Repeated vomiting occurred in 45%. Skull fractures were rare. No child suffered a clinical complication or neurological deterioration. Two patients (0.9%) underwent reevaluation within 48 hours for persistent symptoms; no intracranial abnormality was demonstrated in either on repeated CT scanning and both recovered uneventfully. Follow-up phone surveys in a subgroup of patients indicated universal parent satisfaction. Compared with a control group that underwent both CT scanning and were admitted to the hospital, statistically significant cost savings were realized in the cohort. CONCLUSIONS: A management scheme that involves routine initial CT studies and a brief period of observation in the emergency department is safe and readily accepted by patients and families and can achieve significant cost savings.

The authors review current views on of the embryogenesis of the neural tube defects (NTDs) myelomeningocele and anencephaly. In this context, the following four approaches to the study of NTDs are discussed: normal morphogenesis and timing of early human neural development from conception to the ascent of the conus medullaris; mechanical and molecular biology of neural tube closure derived from experimental and animal models; morphological and biomechanical features of the NTDs myelomeningocele and anencephaly; and the experimental evidence for the importance of both genetic and environmental influences on human NTDs. Although considerable insight into both normal neural tube closure and the factor(s) by which this process may be disrupted has been reported in recent years, the exact mechanism(s) by which human myelomeningoceles and anencephaly arise remain elusive.

It is the Chiari II malformation and its effects that determine the quality of life of the individual born with spina bifida. The cause of this malformation has been a source of debate for many years. Understanding the cause enables strategies for the management of problems created by this malformation to be developed. An open neural tube defect allows fluid to escape from the cranial vesicles, altering the intracranial environment and leads to all of the brain changes seen in the Chiari II malformation. Decompression of the intracranial vesicles causes overcrowding, decrease in the size of the third ventricle, and changes in the fetal skull. It also permanently links the intracranial ventricular system to the spinal cord central canal.

Objective: A recent Internet survey of pediatric neurosurgeons showed that 86% routinely admitted children with immediate posttraumatic seizures (PTS) for a brief period of observation. We wished to determine whether certain children meeting predefined criteria could instead be safely discharged from the emergency room. Methods: We reviewed the records of children admitted during the past 5 years with a diagnosis of seizure and head injury. Children with a minor head injury, a PTS occurring within 24 h of injury and no intracranial abnormalities on admission CT scan were included. Children with previous neurological conditions, a history of prior seizures (other than PTS or febrile seizures), a prior history of anticonvulsant use, or intracranial abnormalities on the admission CT scan were excluded. Records were abstracted for child’s age, gender, length of admission, previous history of PTS or febrile seizures, mechanism of injury, location of impact, time between impact and PTS, the number, length and type of PTS, Glasgow Coma Score (GCS) on admission, subsequent complications and hospital costs. Results: Seventy-one children met the inclusion criteria. Eleven children presented to the emergency room with prolonged seizures, transient apnea or persistently low GCS and required admission to the intensive care unit (ICU). Among the 60 remaining children with simple PTS, none had further seizures during the follow-up period, and none had significant complications. The average cost of hospitalization was known for 58 children; after excluding the costs for 5 patients who were admitted to the ICU, the average hospital cost amounted to USD 1,615 per patient. Conclusions: Our data suggest that children with isolated minor head injuries and simple PTS who recover fully in the emergency room, whose CT scans show no intracranial abnormalities and who have no prior history of neurological disease, epilepsy or anticonvulsant use are at low risk for recurrent seizures or neurological complications, and could potentially be sent home to a reliable caretaker and a stable home situation. However, because of the limited sample size in this study, the statistical risk of a bad outcome may be as high as 9%; we therefore suggest that much larger studies are potentially needed before this becomes a standard policy.

Complications of paranasal sinusitis constitute true surgical and medical emergencies. These complications appear to be more prevalent and seem to present in a more fulminant manner in the pediatric age group. The most common complication of paranasal sinusitis is orbital cellulitis followed collectively by all the intracranial complications. These include meningitis, subdural empyema, intracerebral abscess, epidural abscess and rarely cavernous or superior sagittal sinus thrombosis. We report the case of a 7-year old boy who presented with posterior scalp cellulitis and abscess as a complication of minimally symptomatic paranasal sinusitis. A combined neurosurgical and otolaryngologic approach was required to treat a unilateral ethmoid and frontal sinusitis associated with an epidural abscess abutting the length of the superior sagittal sinus and a posterior subgaleal abscess. The pertinent anatomy allowing for the development of this disease process is discussed. The danger of neurologic sequellae resulting from thrombosis of the superior sagittal sinus is emphasized. Aggressive treatment utilizing a multi-disciplinary surgical approach as well as broad spectrum antibiotics is paramount to obtain the best chance for a full recovery.

The American Society of Pediatric Neurosurgeons (ASPN) has expressed a commitment to have all of its members’ qualifying research efforts published in peer-reviewed pediatric neurosurgical journals such as Pediatric Neurosurgery. To test this commitment, citations from January 1985 through December 1994 were analyzed for all 76 current members of the ASPN. The citations were divided into those of general or adult neurosurgical interest, and those of pediatric neurosurgical interest based upon title, key words, and/or abstract. Each pediatric neurosurgical citation was further classified by topic, and by the type of journal in which it appeared (pediatric neurosurgical, general or adult neurosurgical, and other pediatric or adult journal, subspecialty journal, or basic science journal). A total of 1,887 individual author citations were identified during the study period; of these, 1,586 citations (84%) were classified as pediatric neurosurgical citations. These included 1,391 citations from peer-reviewed publications and 195 citations from Concepts in Pediatric Neurosurgery. As a society, the ASPN published only one third of its citations in peer-reviewed pediatric neurosurgery journals; the remainder were cited in general neurosurgical or other journals. Even when only citations from neurosurgical journals (in which pediatric neurosurgeons were more likely to be primary authors and therefore to have more control over the journal of publication) were analyzed, less than 50% of citations appeared in peer-reviewed pediatric neurosurgery journals. Nearly three quarters of ASPN members failed to provide even a modest commitment – publishing 51% or more of their pediatric citations in peer-reviewed pediatric neurosurgical journals. When the analysis was again limited to only those citations published in neurosurgery journals, over half of the members failed this ‘51% rule’. These results suggest the need for a firmer commitment from ASPN members to publish in peer-reviewed pediatric neurosurgery journals.

If meningomyelocele is indeed a progressive intrauterine process, then early delivery or possibly intrauterine repair of meningomyelocele becomes an issue. Utilizing the delayed splotch (Spd) mouse, a genetically transmitted neural tube defect model, we looked for evidence of abnormalities of neural tissue exposed to amniotic fluid. Affected embryonic and fetal mice were examined with the light microscope, and also with the transmission and scanning electron microscope. Neuronal development and programmed cell death paralleled normal fetal development. No evidence of inflammation on or within the exposed neural tissue was observed. Because the vascular supply to the alar and basilar plate are different, vascular development was also examined and no difference could be found. In conclusion, we found no evidence of deterioration of the exposed neural tube during the gestational period of a mouse, which suggests that exposure of unneurulated spinal cord to amniotic fluid is not a risk factor to the fetus with a neural tube defect.

Occipital plagiocephaly is characterized by both unilateral occipital flattening and ipsilateral frontal prominence with anterior deviation of the ipsilateral ear, yielding a characteristic parallelogram shape to the cranium. Radiographic changes in the lambdoid suture are often evident, but the lambdoid suture is usually patent over most or all of its length on skull X-rays and/or CT scans. Both lambdoid synostosis and deformational forces have been implicated as potentially causal in the pathogenesis of this deformity. We proposed a unifying theory which incorporates a common pathogenesis for both deformational plagiocephaly and most cases of lambdoid 'synostosis'. According to this hypothesis, intrauterine and/or postnatal deformation forces are responsible for the primary calvarial deformation. These forces initially act in reversible manner to produce the typical parallelogram-shaped skull deformity. However, with continued deformation, more enduring secondary pathological changes may eventually occur in the lambdoid suture and basicranium which are more difficult to correct even if the offending deformational forces are subsequently removed or reversed.

BACKGROUND Neural tube defects (NTDs) are the second most common birth defects, after congenital heart defects. Telomerase, the reverse transcriptase that maintains telomere DNA, has been shown to be important for neural tube development and bilateral symmetry in the brain. In knockout mice null for the telomerase RNA component (TERC), telomere loss results in the failure of neural tube closure, primarily at the forebrain and midbrain. METHODS We investigated TERC for variants that may predispose to human NTDs in 477 NTD cases with a variety of phenotypic presentations. RESULTS Two novel single nucleotide polymorphisms were identified in the human TERC sequence but showed no association with the NTD phenotype. CONCLUSIONS Variants in TERC are unlikely to be a major risk factor for the most common form of human NTDs, lumbosacral myelomeningocele. Birth Defects Research (Part A), 2004. © 2004 Wiley-Liss, Inc.

Previous classifications of split cord malformations (SCMr’s), including such terms as diastematomyelia and diplomyelia, have done little to foster a true understanding of these disorders. The authors instead propose a unifying classification in which all SCMr’s share a common embryogenesis. SCMr’s are divided into two types, based upon the composition of the dural coverings and intervening mesenchymal tissue. Type I malformations are composed of two dural sacs and a bony or fibrocartilaginous spur; Type II malformations are composed of a single dural sac and intradural fibrous bands. In either case, the intervening mesenchymal elements contribute to progressive neurologic, urologic, and orthopedic deterioration from spinal cord tethering. The natural history of these lesions supports an early and aggressive operative approach to untether the spinal cord before clinical deterioration begins.

Hydrocephalus develops in approximately 85% of patients with myelomeningoceles and appears most frequently to result from obstruction to cerebrospinal fluid (CSF) flow within the posterior fossa subarachnoid space and at the tentorial hiatus, due to posterior fossa crowding from the Chiari malformation. The presenting features of hydrocephalus are legion; although signs and symptoms of intracranial hypertension are most common, many patients exhibit more subtle and confusing signs, such as intellectual deterioration or behavioral changes, or those that mimic brainstem compression from the Chiari malformation or spinal cord dysfunction due to tethering or syringomyelia. Prompt recognition of these signs and symptoms will direct appropriate therapy toward shunt revision and will help the clinician avoid potentially unnecessary and more dangerous procedures.

Unusual complications of peritoneal shunts are a well-known occurrence. The authors present 2 cases of intracardiac migration of a distal shunt catheter, summarizing the diagnosis and management of each case. This complication seems to be a rare occurrence; the transgression of the jugular vein leading to intracardiac migration of a shunt catheter has been reported only 6 times previously. The authors highlight the importance of careful and proper placement of the distal peritoneal catheter during the tunneling process, in particular avoiding too deep a penetration of the shunt passer into the neck tissues and too medial a shunt passage near the sternal notch to avoid vascular structures.

Object The aim of this study was to characterize the prevalence and demographic features of abusive head trauma (AHT) among infants and children < 36 months of age in Pennsylvania. Methods The authors included all cases of substantiated AHT involving children < 36 months of age in Pennsylvania between 1996 and 2002 that had been reported to a statewide registry. Demographic information was derived from child abuse reports and birth certificates; the study cohort was contrasted with all infants born in Pennsylvania during the same period. Results The study identified 327 cases. The incidence was 14.7 cases (95% confidence interval 13.1–16.5) per 100,000 person-years for the first 2 years of life with a higher incidence during the 1st year (26.0 cases per 100,000 person-years) than the 2nd year (3.4 cases per 100,000 person-years). The incidence was similar among metropolitan, non-metropolitan, and rural counties. Significantly more cases occurred during the holiday months (October–December). The median age of victims was 4.1 months. Both victims and perpetrators were more commonly male (58.4% of victims, and 70% of identified perpetrators). Compared with the entire population of Pennsylvania parents, the parents of the study cohort were more likely to be younger, less educated, and unmarried. Both mothers and fathers were more often African-American and fathers more often Hispanic. Finally, mothers more often smoked during pregnancy, sought prenatal care later in the pregnancy, and delivered low birth weight infants. Conclusions This population-based study of abusive head injuries throughout an entire state adds significantly to the growing knowledge about this condition. The results suggests that families of infants with abusive head injuries have significantly different demographic features compared with the general population, although which of these variables is independently significant cannot be ascertained from this study and require further investigation.

Object The mean level of the conus medullaris (CM) has been estimated to lie opposite the L1/2 disc space in several previous studies using ultrasound, CT myelography, and magnetic resonance (MR) imaging, but these studies have been limited in examining only the lumbar spine and including patients being evaluated for back pain and sciatica (creating a selection bias). Moreover, significant variability was found in the termination of the CM, with a small subset of subjects having a CM as low as the mid-body of L4. The authors sought to determine the normal level of the CM and its variability. Methods Children with brain or spinal cord tumors who underwent whole-spine surveillance MR imaging were identified retrospectively. The level of the CM was identified in each subject by counting down from C1. Vertebral anomalies, such as lumbarized S1, sacralized L5, or fewer rib-bearing segments, and the presence of fatty filum were noted. Results Findings regarding the level of termination of the CM were tightly grouped; the average was at the lower third of L1 and the mode of the distribution was at the L1/2 disc space, with very little variation. No CM ended below the mid-body of L2. The level of the CM was not significantly different among individuals with lumbarized or sacralized vertebrae or 11 rib-bearing segments. Conclusions The CM terminates most commonly at the L1–2 disc space and in the absence of tethering, the CM virtually never ends below the mid-body of L2. A CM that appears more caudal on neuroimages should be considered tethered.

Congenital malformations of the vertebrae are best understood in the context of our understanding of normal vertebral development. The author provides an in-depth discussion of normal vertebral development from gastrulation through vertebral ossification and provides multiple examples of how various disorders of vertebral development could lead to a variety of observed vertebral malformations.

Objective To report a case of idiopathic thrombocytopenic purpura (ITP) complicated by an intracranial hemorrhage (ICH) in a child with a previously undiagnosed arteriovenous malformation. Case We describe a child with known ITP who developed a severe headache, was evaluated in an emergency department of a community hospital, and was found by computer tomography (CT) scan to have an ICH. Despite treatment with platelets, corticosteroids, and intravenous immunoglobulin, she subsequently developed an acute change in mental status. A second CT scan showed that the hemorrhage had significantly increased in size despite treatment. The patient underwent an emergent splenectomy prior to a craniotomy to remove the hemorrhage. At the time of surgery, it was discovered that she had an arteriovenous malformation at the sight of the hemorrhage. Her recovery was unremarkable and she was discharged to home with no neurologic sequelae. Conclusions ICH is a rare but life-threatening complication of ITP. Neurologic symptoms in a child with ITP should be quickly evaluated by CT scan. Most experts suggest careful observation for most cases of ITP. However, when neurologic symptoms occur, more aggressive treatment options must be used. Care of this child included an emergency splenectomy prior to her craniotomy. Pediatric emergency medicine practitioners must be aware of these neurologic symptoms and must not hesitate to involve pediatric surgeons and neurosurgeons in the care of the child. Prompt recognition and early intervention are the keys to improving outcomes when ICH complicates ITP.

Although described only a quarter century ago, progress in this area is rapid and increasing at an accelerated rate. As a group, neurosurgeons, and particularly pediatric neurosurgeons, potentially have a lot to offer, both in the hospital, the courtroom, and the laboratory. As practitioners who see large numbers of brain injuries, both accidental and inflicted, they can provide a unique perspective to child abuse colleagues; unfortunately, neurosurgeons shy away from doing so for a variety of reasons: (1) they are too busy with clinical practice and cannot afford to devote the (largely uncompensated) time involved in keeping up with the literature on the subject, reviewing the case files in detail, and testifying in court; (2) they are reluctant to take the stand and potentially be subjected to the ridicule (and even abuse) hurled by attorneys in the defense of their clients; (3) they are uncertain in their minds about the validity of the evidence in some cases, especially when the evidence is not iron-clad; and (4) they perhaps fear later retribution from people they have helped convict for these crimes. Whatever the reasons, neurosurgeons need to be more involved in these cases and to be both knowledgeable and reasonable in assessments of the cause of injury. In no other area of neurosurgery is the truth so critical, because the lives of the infants for whom they care, and those who might be accused unjustly of perpetrating a crime that they did not commit, hang in the balance. Neurosurgeons must be unerringly accurate in obtaining and recording clinical information and physical findings. When asked, they must not shy away from providing an answer, but only if well enough versed in the literature to be capable of so doing, because to provide false or inaccurate information is a disservice; to do so may condemn an innocent person to prison, or an innocent infant to death. If there is uncertainty, it is probably in the best interests of the child to have the case analyzed and represented by an expert who knows the literature well; there are many excellent and objective child abuse experts who can do this. Finally, the author believes that this should be a much more active area of research for neurosurgeons. Although there has been much progress, there are also a number of uncertainties that remain to be answered, only a few of which have been touched on here. There is much to be done in this important field; neurosurgeons must concentrate on finding adequate answers for those questions that remain.

Object. The authors conducted a study to determine correlations between clinical syndromes and early magnetic resonance (MR) imaging—documented findings in children with spinal cord injury without radiographic abnormality (SCIWORA). Methods. The authors retrospectively reviewed the records obtained in 20 patients who presented with SCIWORA to the Children's Hospital of Buffalo between 1992 and 1999. Initial neurological syndromes, subsequent hospital course and outcome, and early MR imaging findings obtained using conventional sequences on a 1.5-tesla unit were recorded. Neurological syndromes on presentation were complete (Frankel Grade A) in two patients (10%), severe partial (Frankel Grade C) in one patient (5%), and mild partial (Frankel Grade D) in 17 patients (85%). Partial neurological deficits resolved in 14 (78%) of 18 patients within 72 hours and lasted more than 72 hours in four patients (22%). Magnetic resonance imaging was performed in both patients presenting with complete injuries and in 17 of 18 patients presenting with partial neurological deficits. The studies were obtained within 24 hours in 17 patients (85%). Neuroimaging revealed spinal cord swelling at the cervical level in one of the children with complete injury and cord edema with associated hemorrhage at cervical and thoracic levels in the other. Neural and extraneural elements were shown to be normal in all 17 patients with partial injuries who underwent MR imaging, including in the four patients with partial motor deficits lasting more than 72 hours. Conclusions. In this series, the predominant neurological presentation of SCIWORA was a mild, partial syndrome that resolved within 72 hours. Magnetic resonance imaging revealed abnormal features only in those patients with complete neurological deficits. These findings suggest that in the acute setting conventional MR imaging sequences may lack the sensitivity to demonstrate neural and extraneural abnormalities associated with partial or temporary neurological deficits of SCIWORA, even when those deficits persist beyond 72 hours.

While cranial birth injuries in term infants are well recognized, to date, only small case series have been described. In an attempt to further define the spectrum of cranial birth injuries, we analyzed 41 consecutive cranial birth injuries at our institution over the period 1991–1998. The most common clinical presentations were apnea (39%) and seizures (37%). Average Apgar scores were 5.7 at 1 min and 7.3 at 5 min; 54% of infants had abnormally low Apgar scores at 1 min and 31% had abnormally low scores at 5 min. The most common intracranial lesion was subdural hemorrhage, present in 73% of infants; most had either a tentorial (57%) and/or interhemispheric (50%) location. Operative treatment was required in 5 infants (12%). Two of the 41 infants (4.8%) died. The study group was compared with a control group of 63 randomly selected births without cranial injury. Using a stepwise logistic regression model, independently significant variables included neonatal birth weight, Apgar scores at 1 and 5 min and mode of delivery. Compared with the controls, the study group had a significantly higher incidence of forceps and/or vacuum deliveries. Combining vacuum, forceps and urgent cesarean section deliveries together as ‘urgent’ and elective cesarean and spontaneous vaginal deliveries as ‘nonurgent’, we could find no significant differences between these two groups. Our data conflict with those of Towner et al. [N Engl J Med 1999;341:1709–1714], and suggest that the method of assisted delivery, rather than the urgency of the delivery or dysfunctional labor per se, is a more important variable in cranial birth injuries.

Objective: Remifentanil hydrochloride is a new titratable opioid agonist with rapid onset and offset of action. We present 2 cases of intracranial mass lesions in whom remifentanil was used to obtain serial neurological examinations. Methods: Patients received bolus doses of remifentanil 1 µg/kg followed by continuous infusions of 0.1–0.5 µg/kg/min, titrated to effect. After the clinical examination, the remifentanil was restarted to the desired effect. Conclusion: The ultra-short duration of action of remifentanil makes it ideally suited for selected pediatric neurosurgical patients in whom frequent, serial neurological examinations are necessary. There were no side effects associated with the use of remifentanil.

The care of patients with shunted hydrocephalus is one of the most common clinical problems facing a pediatric neurosurgeon. Despite the prevalence of this clinical entity, it is our impression that the management strategies for these patients vary significantly amongst pediatric neurosurgeons. In an effort to better understand how varied these practices are, we surveyed 261 neurosurgeons with an expressed interest in pediatric neurosurgery, of whom 149 (57%) responded. The survey contained questions pertaining to preoperative and intraoperative management, choice of shunt valve, treatment philosophies with respect to shunt revision, evaluation process for potential shunt malfunction, treatment strategies for shunt infections and routine follow-up care. The answers to the questions confirmed our expectations that there are a variety of ways to manage these patients.

Most shunt malfunctions present with signs and symptoms of high intracranial pressure, and computed tomography scans demonstrate ventricular enlargement. However, several authors have described a rare ‘low-pressure’ hydrocephalic state in which ventricular enlargement can occur in the face of low, or even negative, intracranial pressures. We report 2 children with obstructive hydrocephalus in whom this ‘low-pressure state’ followed a lumbar puncture; in both children, the shunts were functioning properly despite increased ventricular size on computed tomography scans, and all symptoms resolved (and the ventricles returned to baseline) following a period of enforced recumbency without shunt revision. We hypothesize that subarachnoid cerebrospinal fluid leakage through the puncture site in the lumbar theca decreases the intracranial pressures globally to a point below the opening pressures of the shunt valves. The ventricular cerebrospinal fluid, unable to be drained through either the subarachnoid space or the shunt, accumulates within the ventricular system under low pressure. One consistent feature in our 2 patients has been the postural nature of the headaches. We recommend enforced recumbency and, if necessary, a blood patch to seal the lumbar leakage. Shunt revision or prolonged external ventricular drainage appears to be unnecessary in these patients. Finally, neurosurgeons should be aware of this potential complication.

Congenital vertebral dislocation (CVD) is a rare congenital spinal malformation characterized by a translatory or rotatory vertebral displacement, or both, at a single level, that results in an abrupt angulation of the neural canal. The more caudal vertebra is dysplastic and appears at first glance to be posteriorly dislocated into the vertebral canal as a posterior hemivertebra, but is actually well aligned with the more caudal vertebral column. Unfortunately, the present classification of complex congenital vertebral anomalies is confusing, and CVD has been grouped together with other congenital vertebral malformations under the terms ‘segmental spinal dysgenesis’, ‘medial spinal aplasia’, and others. Moreover, a putative embryonic mechanism has never been proposed for CVD. Based upon our experience with 6 children and a critical review of the literature, we identify CVD as a distinct entity having characteristic anatomical features. We propose a novel putative embryonic mechanism – early embryonic ‘buckling’ – which likely occurs by the 6th embryonic week, prior to the period of axonal outgrowth and the beginning of vertebral chondrification.

Certain CT and/or MRI abnormalities have been used medicolegally to time intracranial injuries from the infant shaken impact syndrome (ISIS). For example, parenchymal hypodensities on CT scans are said to arise only after 6–48 h have elapsed postinjury, and the presence of chronic or mixed subdural hematomas suggests injury that occured 1–4 weeks prior. However, these statements are based largely upon inference from data obtained in other conditions such as ischemic anoxic injury and chronic subdural hemorrhage in adults. Direct evidence about the evolution of intracranial injuries in infants with ISIS is sparse, and the radiographic changes following ISIS have never been systematically studied on serial imaging studies. One hundred-seventeen serial CT and MRI scans obtained from 33 infants with ISIS were reviewed retrospectively. The exact scan dates and times were obtained directly from the scans. Acute subdural hemorrhage was the most common intracranial abnormality and was present in 27 (81%) of the 33 infants. Other intracranial abnormalities included chronic subdural collections, subarachnoid hemorrhage, epidural hematomas, parenchymal hypodensities, edema and contusions, and atrophy and encephalomalacia. In 15 of the 33 infants, the injury could be timed with reasonable certainty, and the evolution of the radiographic changes followed over time. Six of the 15 infants had evidence of prior cranial trauma such as chronic subdural collections (5 infants) or mild atrophy (1 infant). Of the remaining 9 infants, parenchymal abnormalities such as hypodensities, edema and contusion appeared in virtually all of the initial scans performed approximately 3 h following the report of injury. One ‘chronic’ subdural collection was absent on the first scan performed 2.75 h postinjury, but appeared on a second scan performed 17 h later, suggesting that some ‘chronic’ subdural fluid collections may arise much sooner than previously thought. These findings challenge some of the current dogma about the timing of radiographic changes following abuse and are important in timing the alleged abuse for legal purposes.

Currarino’s triad is a congenital malformation involving the combination of anorectal stenosis, a presacral mass (most often a teratoma or ventral menigocele) and an anterior sacral bony defect (scimitar sacrum). Current theories regarding its embryogenesis are difficult to reconcile with our current understanding of caudal neuraxial and hindgut development. Caudal neuraxial structures develop from the caudal eminence (or tail bud), which normally separates from the hindgut endoderm concurrent with ingrowth of the posterior notochord during late gastrulation. We describe the first reported association of Currarino’s triad with a caudal split cord malformation. It has previously been proposed that split cord malformations and related ‘complex dysraphic malformations’ involving abnormalities of one or more of the three primary germ layers arise through disordered midline axial integration during gastrulation. The presence of a split cord malformation in a patient with Currarino’s triad suggests that the two disorders share a common embryogenetic pathway. We propose that the malformations of Currarino’s triad arise through a failure of dorsoventral separation of the caudal eminence from the hindgut endoderm during late gastrulation.

Although a number of operative positions have been described for approaching midline posterior fossa tumors, all have potential disadvantages and complications. We describe a modified lateral decubitus position for the removal of posterior fossa tumors. The position allows blood and cerebrospinal fluid to drain from the operative wound and provides a straight-on view of the posterior fossa contents. It also allows the surgeon to sit comfortably during tumor removal, thereby reducing operator fatigue. We have found this to be a reliable and valuable approach for posterior fossa pathology.

Closure of a myelomeningocele is a deceptively simple operation; however, attention to several subtle details can significantly reduce operative complications. Important preoperative concerns include social issues of dealing with a distraught and often overwhelmed family, the timing of surgery, and assessment of associated severe or life-threatening malformations. Operative intervention should be directed toward preserving neurological function and optimizing the subsequent repair of a tethered spine should this become necessary. Careful attention to the vascular supply to the placode, precise separation of neural from cutaneous tissues, a diligent search for associated tethering anomalies such as diastematomyelia and a thickened filum terminale, careful pia to pia reconstruction of the placode, and simple but meticulous wound closure all help in achieving these aims. The timely management of associated hydrocephalus will help to avoid cerebrospinal fluid leakage and wound breakdown. Close attention to these details will ameliorate many of the immediate and delayed complications of myelomeningocele closure.