Search

Objective: The aim of this study was to compare clinical and craniometric outcomes of patients treated for hydrocephalus following fetal myelomeningocele repair (fMMR) via a ventriculoperitoneal shunt (VPS) or endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC)., Methods: This was a retrospective cohort study of children who were treated for hydrocephalus following fMMR via VPS or ETV with or without CPC (ETV ± CPC) at Vanderbilt between 2012 and 2021. The primary outcomes were treatment failure and time to failure (TTF). Secondary outcomes included changes in hydrocephalus metrics (fronto-occipital horn ratio [FOHR] and head circumference measurements) and healthcare resource utilization (number of hospital admissions, clinic visits, and neuroimaging findings)., Results: Among 88 patients who underwent fMMR, 37 (42%) required permanent CSF diversion, of whom 19 received treatment at the authors' institution. Twelve patients underwent ETV ± CPC, and 7 underwent VPS placement at a median corrected age of 23 weeks versus 1 week (p = 0.002). The preoperative median head circumference percentiles and z-scores for patients in the ETV ± CPC cohort were similar to those of the VPS cohort (percentiles: 98.5 vs 94.0, p = 0.064; z-scores: 2.32 vs 1.60, p = 0.111). There was no difference in preoperative median FOHR measurements between the two cohorts (0.57 vs 0.59, p = 0.53). At 6 months postoperatively, the median head circumference percentile and z-score for the ETV ± CPC cohort remained similar between the two cohorts (percentiles: 98.0 vs 67.5, p = 0.315; z-scores: 2.12 vs 0.52, p = 0.307). There was no difference in the change in FOHR (-0.06 vs -0.09, p = 0.37) and change in head circumference percentile (-1.33 vs -28.6, p = 0.058) between the cohorts 6 months after the index CSF diversion procedure. One patient in the ETV ± CPC cohort experienced a seizure and a nonoperative subdural hemorrhage postoperatively; no other complications were observed. Six of the 7 patients in the VPS cohort required shunt revision with a median TTF of 9.8 months while 2 of the 12 ETV ± CPC patients required a repeat ETV at a median of 17.5 months (86% vs 17%, p = 0.013). The median number of hydrocephalus-related hospital readmissions was significantly lower in the ETV ± CPC cohort than in the VPS cohort (0 vs 1, p = 0.006). The ETV ± CPC cohort had fewer CT scans (0 vs 2, p = 0.004) and radiographs (0 vs 2, p < 0.001) than the VPS cohort., Conclusions: In a single-center cohort, hydrocephalic fMMR patients treated via ETV ± CPC remained shunt free, while a majority of patients receiving an upfront shunt required revision. This is the first study comparing ETV ± CPC with VPS in the fMMR hydrocephalus population. While larger, multicenter studies are needed, these results suggest that ETV/CPC may be a preferred means of CSF diversion following fMMR.

Objective: Extent of resection (EOR) is the most important modifiable prognostic variable for pediatric patients with posterior fossa ependymoma. An understanding of primary and recurrent ependymoma complications is essential to inform clinical decision-making for providers, patients, and families. In this study, the authors characterize postsurgical complications following resection of primary and recurrent pediatric posterior fossa ependymoma in a molecularly defined cohort., Methods: The authors conducted a 20-year retrospective single-center review of pediatric patients undergoing resection of posterior fossa ependymoma at the Hospital for Sick Children in Toronto, Canada. Complications were dichotomized into major and minor groups; EOR was compared across complication categories. The association between complication occurrence with length of stay (LOS) and mortality was also assessed using multivariable regressions., Results: There were 60 patients with primary resection included, 41 (68%) of whom were alive at the time of data collection. Gross-total resection was achieved in 33 (58%) of 57 patients at primary resection. There were no 30-day mortality events following primary and recurrent ependymoma resection. Following primary resection, 6 patients (10%) had posterior fossa syndrome (PFS) and 36 (60%) developed cranial neuropathies, 56% of which recovered within 1 year. One patient (1.7%) required a tracheostomy and 9 patients (15%) required gastrostomy tubes. There were 14 ventriculoperitoneal shunts (23%) inserted for postoperative hydrocephalus. Among recurrent cases, there were 48 recurrent resections performed in 24 patients. Complications included new cranial neuropathy in 10 patients (21%), of which 5 neuropathies resolved within 1 year. There were no cases of PFS following resection of recurrent ependymoma. Gastrostomy tube insertion was required in 3 patients (6.3%), and 1 patient (2.0%) required a tracheostomy. Given the differences in the location of tumor recurrence, a direct comparison between primary and recurrent resection complications was not feasible. Following multivariate analysis adjusting for sex, age, molecular status, and EOR, occurrence of major complications was found to be associated with prolonged LOS but not mortality., Conclusions: These results detail the spectrum of postsurgical morbidity following primary and recurrent posterior fossa ependymoma resection. The crude complication rate following resection of infratentorial recurrent ependymoma was lower than that of primary ependymoma, although a statistical comparison revealed no significant differences between the groups. These results should serve to inform providers of the morbidity profile following surgical management of posterior fossa ependymoma and inform perioperative counseling of patients and their families.

Objective: Early suturectomy with a rigid endoscope followed by orthotic cranial helmet therapy is an accepted treatment option for single-suture craniosynostosis. To the authors' knowledge, flexible endoscope-assisted suture release (FEASR) has not been previously described. Presented herein is their experience with FEASR for the treatment of isolated sagittal craniosynostosis., Methods: A retrospective analysis of the health records of patients who had undergone FEASR between March 2018 and December 2020 was performed. Patients under the age of 6 months who had been diagnosed with isolated sagittal synostosis were considered eligible for FEASR. Exclusion criteria included syndromic synostosis or multiple-suture synostosis. The cephalic index, the primary measure of the cosmetic endpoint, was calculated at prespecified intervals: immediately preoperatively and 6 weeks and 12 months postoperatively. Parental satisfaction with the cosmetic outcome was determined throughout the clinical follow-up and documented according to a structured questionnaire for the first 12 months., Results: A total of 18 consecutive patients met the criteria for study inclusion. The mean patient age at the time of surgery was 3.4 months (range 2-6 months). All patients underwent a wide craniectomy with no need to convert to an open procedure. The mean craniectomy width was 3.61 cm. Estimated blood loss ranged from 5 to 30 ml. The mean operative time was 75 minutes. No intraoperative complications were observed. The average length of stay was 2.6 days. The mean cephalic index was 67.7 preoperatively, 77.1 at 6 weeks postoperatively, and 76.3 at 1 year postoperatively. The mean percentage change in the cephalic index from preoperatively to the 12-month follow-up was 10.44 (p < 0.001). The mean follow-up was 17 months (range 12-28 months). All parents were satisfied with the cosmetic outcome of the procedure. No patients developed symptoms of raised intracranial pressure (ICP) or needed invasive ICP monitoring during the follow-up period. No patients required reoperation., Conclusions: In this modest single-hospital series, the authors demonstrated the feasibility of FEASR in treating sagittal synostosis with favorable cosmetic outcomes. The morbidity profile and resource utilization of the procedure appear similar to those of procedures conducted via traditional rigid endoscopy.

Objective: Epilepsy disproportionately affects low- and/or middle-income countries (LMICs). Surgical treatments for epilepsy are potentially curative and cost-effective and may improve quality of life and reduce social stigmas. In the current study, the authors estimate the potential need for a surgical epilepsy program in Haiti by applying contemporary epilepsy surgery referral guidelines to a population of children assessed at the Clinique d'Épilepsie de Port-au-Prince (CLIDEP)., Methods: The authors reviewed 812 pediatric patient records from the CLIDEP, the only pediatric epilepsy referral center in Haiti. Clinical covariates and seizure outcomes were extracted from digitized charts. Electroencephalography (EEG) and neuroimaging reports were further analyzed to determine the prevalence of focal epilepsy or surgically amenable syndromes and to assess the lesional causes of epilepsy in Haiti. Lastly, the toolsforepilepsy instrument was applied to determine the proportion of patients who met the criteria for epilepsy surgery referral., Results: Two-thirds of the patients at CLIDEP (543/812) were determined to have epilepsy based on clinical and diagnostic evaluations. Most of them (82%, 444/543) had been evaluated with interictal EEG, 88% of whom (391/444) had abnormal findings. The most common finding was a unilateral focal abnormality (32%, 125/391). Neuroimaging, a prerequisite for applying the epilepsy surgery referral criteria, had been performed in only 58 patients in the entire CLIDEP cohort, 39 of whom were eventually diagnosed with epilepsy. Two-thirds (26/39) of those patients had abnormal findings on neuroimaging. Most patients (55%, 18/33) assessed with the toolsforepilepsy application met the criteria for epilepsy surgery referral., Conclusions: The authors' findings suggest that many children with epilepsy in Haiti could benefit from being evaluated at a center with the capacity to perform basic brain imaging and neurosurgical treatments.

Objective: There are limited data on the pediatric neurosurgical workforce in Asia and Australasia. The training and clinical practice of pediatric neurosurgeons need to be characterized in order to identify gaps in knowledge and skills, thereby establishing a framework from which to elevate pediatric neurosurgical care in the region., Methods: An online survey for pediatric neurosurgeons was created in REDCap (Research Electronic Database Capture), collecting demographic information and data on pediatric neurosurgical training and clinical practice. The link to answer the survey was sent to the mailing lists of the Asian Australasian Society for Pediatric Neurosurgery and the Japanese Society for Pediatric Neurosurgery, disseminated during the 2019 Asian Australasian Pediatric Neurosurgery Congress, and spread through social media. The survey was open to neurosurgeons who operated on patients ≤ 18 years old in Asian Australasian countries, whether or not they had completed fellowship training in pediatric neurosurgery. Descriptive statistics were computed and tabulated. Data were stratified and compared based on surgeon training and World Bank income group., Results: A total of 155 valid survey responses were analyzed, representing neurosurgeons from 21 countries. A total of 107 (69%) considered themselves pediatric neurosurgeons, of whom 66 (43%) had completed pediatric neurosurgery training. Neurosurgeons in East Asia commonly undergo a fellowship in their home countries, whereas the rest train mostly in North America, Europe, and Australia. A majority (89%) had operating privileges, and subspecialty pediatric training usually lasted from 6 months to 2 years. On average, trained pediatric neurosurgeons perform a higher number of pediatric neurosurgical operations per year compared with nonpediatric-trained respondents (131 ± 129 vs 56 ± 64 [mean ± SD], p = 0.0001). The mean number of total neurosurgical operations per year is similar for both groups (184 ± 129 vs 178 ± 142 [mean ± SD], p = 0.80). Respondents expressed the desire to train further in pediatric epilepsy, spasticity, vascular malformations, craniofacial disorders, and brain tumors., Conclusions: Both pediatric and general neurosurgeons provide neurosurgical care to children in Asia and Australasia. There is a need to increase pediatric neurosurgery fellowship programs in the region. Skill sets and training needs in pediatric neurosurgery vary depending on the country's economic status and between pediatric-trained and nonpediatric-trained surgeons.

Objective: Selective dorsal rhizotomy (SDR) is a procedure primarily performed to improve function in a subset of children with limitations related to spasticity. There is substantial variability in operative techniques among centers and surgeons. Here, the authors provide a technical review of operative approaches for SDR., Methods: Ovid MEDLINE, Embase, and PubMed databases were queried in accordance with PRISMA guidelines. All studies included described a novel surgical technique. The technical nuances of each approach were extracted, including extent of exposure, bone removal, and selection of appropriate nerve roots. The operative approach preferred at the authors' institution (the "2 × 3 exposure") is also detailed., Results: Five full-text papers were identified from a total of 380 articles. Operative approaches to SDR varied significantly with regard to level of exposure, extent of laminectomy, and identification of nerve roots. The largest exposure involved a multilevel laminectomy, while the smallest exposure involved a keyhole interlaminar approach. At the Hospital for Sick Children, the authors utilize a two-level laminoplasty at the level of the conus medullaris. The benefits and disadvantages of the spectrum of techniques are discussed, and illustrative figures are provided., Conclusions: Surgical approaches to SDR vary considerably and are detailed and illustrated in this review as a guide for neurosurgeons. Future studies should address the long-term impact of these techniques on functional outcomes and complications such as spinal deformity.

Objective: The authors aimed to evaluate clinical, radiological, and surgical factors associated with posterior fossa tumor resection (PFTR)-related outcomes, including postoperative complications related to dural augmentation (CSF leak and wound infection), persistent hydrocephalus ultimately requiring permanent CSF diversion after PFTR, and 90-day readmission rate., Methods: Pediatric patients (0-17 years old) undergoing PFTR between 2000 and 2016 at Monroe Carell Jr. Children's Hospital of Vanderbilt University were retrospectively reviewed. Descriptive statistics included the Wilcoxon signed-rank test to compare means that were nonnormally distributed and the chi-square test for categorical variables. Variables that were nominally associated (p < 0.05) with each outcome by univariate analysis were included as covariates in multivariate linear regression models. Statistical significance was set a priori at p < 0.05., Results: The cohort consisted of 186 patients with a median age at surgery of 6.62 years (range 3.37-11.78 years), 55% male, 83% Caucasian, and average length of follow-up of 3.87 ± 0.25 years. By multivariate logistic regression, the variables primary dural closure (PDC; odds ratio [OR] 8.33, 95% confidence interval [CI] 1.07-100, p = 0.04), pseudomeningocele (OR 7.43, 95% CI 2.23-23.76, p = 0.0007), and hydrocephalus ultimately requiring permanent CSF diversion within 90 days of PFTR (OR 9.25, 95% CI 2.74-31.2, p = 0.0003) were independently associated with CSF leak. PDC versus graft dural closure (GDC; 35% vs 7%, OR 5.88, 95% CI 2.94-50.0, p = 0.03) and hydrocephalus ultimately requiring permanent CSF diversion (OR 3.30, 95% CI 1.07-10.19, p = 0.0007) were associated with wound infection requiring surgical debridement. By multivariate logistic regression, GDC versus PDC (23% vs 37%, OR 0.13, 95% CI 0.02-0.87, p = 0.04) was associated with persistent hydrocephalus ultimately requiring permanent CSF diversion, whereas pre- or post-PFTR ventricular size, placement of peri- or intraoperative extraventricular drain (EVD), and radiation therapy were not. Furthermore, the addition of perioperative EVD placement and dural closure method to a previously validated predictive model of post-PFTR hydrocephalus improved its performance from area under the receiver operating characteristic curve of 0.69 to 0.74. Lastly, the authors found that autologous (vs synthetic) grafts may be protective against persistent hydrocephalus (p = 0.02), but not CSF leak, pseudomeningocele, or wound infection., Conclusions: These results suggest that GDC, independent of potential confounding factors, may be protective against CSF leak, wound infection, and hydrocephalus in patients undergoing PFTR. Additional studies are warranted to further evaluate clinical and surgical factors impacting PFTR-associated complications.

Objective: The optimal revascularization for pediatric moyamoya for reducing the incidence of future stroke events remains to be determined., Methods: The authors conducted a search of PubMed, MEDLINE, Embase, and Web of Science electronic databases from inception until January 2019. The primary endpoint was the presence of a future ischemic stroke event. Secondary endpoints were angiographic outcomes as measured by postoperative Matsushima grade and clinical symptom persistence. Patients who underwent either direct or combined direct/indirect revascularization were classified into the direct cohort. Data from each study on presence of postoperative stroke events were used to generate standardized mean differences and 95% confidence intervals, which were combined using inverse variance-weighted averages of standardized mean differences in a random effects model., Results: Twenty-nine studies met the inclusion criteria for analysis, comprising 2258 patients (1011 males, mean age 8.3 ± 1.8 years) who were followed up clinically for an average of 71.4 ± 51.3 months. One hundred fifty-four patients underwent direct bypass alone, 680 patients underwent either direct or combined direct/indirect revascularization procedures, while 1424 patients underwent indirect bypass alone. The frequencies of future stroke events in patients undergoing direct bypass alone, combined bypass, or indirect bypass alone were 1 per 190.3 patient-years, 1 per 108.9 patient-years, and 1 per 61.1 patient-years, respectively, in each cohort. The estimated stroke rates were 9.0% with indirect revascularization, 4.5% with direct revascularization alone, and 6.0% with combined revascularization. A forest plot did not reveal any significant differences in the incidence of future stroke events or angiographic outcomes following direct-only, combined, or indirect-only revascularization., Conclusions: Direct, indirect, and combined indirect/direct bypass techniques are all effective revascularization options for pediatric moyamoya disease. A relative paucity of granular studies-and inherent surgical selection bias-limits direct comparison between interventions. Suitably designed prospective cohort studies may be useful in identifying patients likely to receive benefit from specific procedures.

The two-hit hypothesis of neural injury in the wake of open neural tube defects suggests an opportunity for preservation of function and potential reversibility of early morphological changes in the fetus diagnosed with myelomeningocele. The Management of Myelomeningocele Study (MOMS) demonstrated reduced need for shunting and improved neurological function in patients treated in utero relative to postnatally, thereby offering level 1 evidence supporting fetal repair. Subsequent studies have offered additional information about urological, orthopedic, radiological, and maternal factors surrounding fetal repair. The quest for robust long-term neurocognitive and motor function data is underway and poised to shape the future of fetal repair. In addition, technical innovations such as fetoscopic surgery aim to minimize maternal morbidity while conferring the beneficial effects observed with open intrauterine intervention.

Objective: The Management of Myelomeningocele Study (MOMS) established several important benefits of myelomeningocele fetal closure, including decreased need for shunting at 30 months. However, the effects of fetal closure on long-term quality of life (QOL) have not been studied. In this study, the authors aimed to analyze the differences in long-term QOL between children treated with intrauterine repair and those treated with postnatal repair., Methods: Caregivers of children treated with either intrauterine or postnatal closure at a tertiary acute care hospital between 1997 and 2003 were contacted to participate. The Pediatric Quality of Life Inventory (PedsQL 4.0) and a surgical history questionnaire were administered over the phone. Responses to the QOL survey were reverse scored and linearly transformed to a 0-100 scale, with a higher score indicating better QOL. The Mann-Whitney U-test was used to analyze differences in means., Results: Seventy-four children had MMC repair between 1997 and 2003. Twenty-three (31%) of the patients' families responded to the PedsQL 4.0 questionnaire. Of these 23 children, 11 had intrauterine closure and 12 had postnatal closure. The intrauterine group did not differ in age (median [IQR] 17 years [14-17 years] vs 15 years [14-19 years], p = 0.926), sex (27.3% vs 41.7% male, p = 0.469), or lesion level (p = 0.199) from the postnatal group. Fewer patients in the intrauterine group underwent neurosurgical procedures than those in the postnatal group (55.6% vs 100%, p = 0.018). However, of the children receiving neurosurgical procedures, there was no difference in the number of procedures between the intrauterine and postnatal groups (median [IQR] 4 [2-10.5] vs 2.5 [1.75-6.25], p = 0.458). There was no difference in the percentage of children receiving nonneurosurgical procedures between the two groups (100% vs 100%, p > 0.99). Children who underwent intrauterine closure had significantly higher psychosocial health (median [IQR] 70.0 [56.7-83.3] vs 55.0 [42.1-60.0], p = 0.015) as well as total QOL (median [IQR] 56.5 [55.4-81.5] vs 49.5 [32.9-59.0], p = 0.019) than children with postnatal closure. Physical health was not significantly different between the two groups (median [IQR] 62.5 [37.5-78.1] vs 39.1 [18.8-59.4], p = 0.108)., Conclusions: Relative to postnatal closure, children who underwent intrauterine closure of MMC demonstrated better long-term QOL as measured by psychosocial and overall QOL metrics. Given that not all medical and socioeconomic confounders were adjusted for due to the low sample size, validation of these results in a larger population and across multiple centers is needed.

OBJECTIVEThere remains uncertainty regarding the appropriate level of care and need for repeating neuroimaging among children with mild traumatic brain injury (mTBI) complicated by intracranial injury (ICI). This study's objective was to investigate physician practice patterns and decision-making processes for these patients in order to identify knowledge gaps and highlight avenues for future investigation.METHODSThe authors surveyed residents, fellows, and attending physicians from the following pediatric specialties: emergency medicine; general surgery; neurosurgery; and critical care. Participants came from 10 institutions in the United States and an email list maintained by the Canadian Neurosurgical Society. The survey asked respondents to indicate management preferences for and experiences with children with mTBI complicated by ICI, focusing on an exemplar clinical vignette of a 7-year-old girl with a Glasgow Coma Scale score of 15 and a 5-mm subdural hematoma without midline shift after a fall down stairs.RESULTSThe response rate was 52% (n = 536). Overall, 326 (61%) respondents indicated they would recommend ICU admission for the child in the vignette. However, only 62 (12%) agreed/strongly agreed that this child was at high risk of neurological decline. Half of respondents (45%; n = 243) indicated they would order a planned follow-up CT (29%; n = 155) or MRI scan (19%; n = 102), though only 64 (12%) agreed/strongly agreed that repeat neuroimaging would influence their management. Common factors that increased the likelihood of ICU admission included presence of a focal neurological deficit (95%; n = 508 endorsed), midline shift (90%; n = 480) or an epidural hematoma (88%; n = 471). However, 42% (n = 225) indicated they would admit all children with mTBI and ICI to the ICU. Notably, 27% (n = 143) of respondents indicated they had seen one or more children with mTBI and intracranial hemorrhage demonstrate a rapid neurological decline when admitted to a general ward in the last year, and 13% (n = 71) had witnessed this outcome at least twice in the past year.CONCLUSIONSMany physicians endorse ICU admission and repeat neuroimaging for pediatric mTBI with ICI, despite uncertainty regarding the clinical utility of those decisions. These results, combined with evidence that existing practice may provide insufficient monitoring to some high-risk children, emphasize the need for validated decision tools to aid the management of these patients.

OBJECTIVE It has been suggested that the treatment of infant hydrocephalus results in different craniometric changes depending upon whether ventriculoperitoneal shunt (VPS) placement or endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) is performed. Without an objective and quantitative description of expected changes to the infant cranium and ventricles following ETV/CPC, asserting successful treatment of hydrocephalus is difficult. By comparing infants successfully treated via ETV/CPC or VPS surgery, the authors of this study aimed to define the expected postoperative cranial and ventricular alterations at the time of clinical follow-up. METHODS Patients who underwent successful treatment of hydrocephalus at 4 institutions with either VPS placement or ETV/CPC were matched in a 3:1 ratio on the basis of age and etiology. Commonly used cranial parameters (including head circumference [HC], HC z-score, fontanelle status, and frontooccipital horn ratio [FOHR]) were compared pre- and postoperatively between treatment cohorts. First, baseline preoperative values were compared to ensure cohort equivalence. Next, postoperative metrics, including the relative change in metrics, were compared between treatment groups using multivariate linear regression. RESULTS Across 4 institutions, 18 ETV/CPC-treated and 54 VPS-treated infants with hydrocephalus were matched and compared at 6 months postoperatively. The most common etiologies of hydrocephalus were myelomeningocele (61%), followed by congenital communicating hydrocephalus (17%), aqueductal stenosis (11%), and intraventricular hemorrhage (6%). The mean age at the time of CSF diversion was similar between ETV/CPC- and VPS-treated patients (3.4 vs 2.9 months; p = 0.69), as were all preoperative cranial hydrocephalus metrics (p > 0.05). Postoperatively, the ventricle size FOHR decreased significantly more following VPS surgery (-0.15) than following ETV/CPC (-0.02) (p < 0.001), yielding a lower postoperative FOHR in the VPS arm (0.42 vs 0.51; p = 0.01). The HC percentile was greater in the ETV/CPC cohort than in the VPS-treated patients (76th vs 54th percentile; p = 0.046). A significant difference in the postoperative z-score was not observed. With both treatment modalities, a bulging fontanelle reliably normalized at last follow-up. CONCLUSIONS Clinical and radiographic parameters following successful treatment of hydrocephalus in infants differed between ETV/CPC and VPS treatment. At 6 months post-ETV/CPC, ventricle size remained unchanged, whereas VPS-treated ventricles decreased to a near-normal FOHR. The HC growth control between the procedures was similar, although the final HC percentile may be lower after VPS. The fontanelle remained a reliable indicator of success for both treatments. This study establishes expected cranial and ventricular parameters following ETV/CPC, which may be used to guide preoperative counseling and postoperative decision making.

The authors present a case of monozygotic twins with hereditary hemorrhagic telangiectasia (HHT) who experienced cerebral arteriovenous malformation (AVM) hemorrhage at a very young age. The clinical variables influencing HHT-related AVM rupture are discussed, and questions surrounding the timing of screening and intervention are explored. This is only the second known case of monozygotic HHT twins published in the medical literature, and the youngest pair of first-degree relatives to experience AVM-related cerebral hemorrhage. Evidence guiding the screening and management of familial HHT is lacking, and cases such as this underscore the need for objective and validated protocols.

OBJECTIVE The authors aimed to provide an updated and consolidated report on the epidemiology, management, and functional outcome of cases of myelomeningocele (MMC) in patients with scoliosis. METHODS A comprehensive literature search was performed using MEDLINE, Embase, Google Scholar, and the Cochrane Database of Systematic Reviews on cases of MMC in patients with scoliosis between 1980 and 2016. The initial search yielded 670 reports. After removing duplicates and applying inclusion criteria, we included 32 full-text original articles in this study. RESULTS Pooled statistical analysis of the included articles revealed the prevalence of scoliosis in MMC patients to be 53% (95% CI 0.42-0.64). Slightly more females (56%) are affected with both MMC and scoliosis than males. Motor level appears to be a significant predictor of prevalence, but not severity, of scoliosis in MMC patients. Treatment options for these patients include tethered cord release (TCR) and fusion surgeries. Curvature improvement and stabilization after TCR may be limited to patients with milder (< 50°) curves. Meanwhile, more aggressive fusion procedures such as a combined anterior-posterior approach may result in more favorable long-term scoliosis correction, albeit with greater complication rates. Quality of life metrics including ambulatory status and sitting stability are influenced by motor level of the lesion as well as the degree of the scoliosis curvature. CONCLUSIONS Scoliosis is among the most common and challenging comorbidities from which patients with MMC suffer. Although important epidemiological and management trends are evident, larger, prospective studies are needed to discover ways to more accurately counsel and more optimally treat these patients.

OBJECTIVE Up to one-third of patients with a posterior fossa brain tumor (PFBT) will experience persistent hydrocephalus mandating permanent CSF diversion. The optimal hydrocephalus treatment modality is unknown; the authors sought to compare the durability between endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) therapy in the pediatric population. METHODS The authors conducted a systematic review of articles indexed in PubMed between 1986 and 2016 describing ETV and/or VPS treatment success/failure and time-to-failure rate in patients < 19 years of age with hydrocephalus related to a PFBT. Additionally, the authors conducted a retrospective review of their institutional series of PFBT patients requiring CSF diversion. Patient data from the systematic review and from the institutional series were aggregated and a time-to-failure analysis was performed comparing ETV and VPS using the Kaplan-Meier method. RESULTS A total of 408 patients were included from 12 studies and the authors' institutional series: 284 who underwent ETV and 124 who underwent VPS placement. The analysis included uncontrolled studies with variable method and timing of CSF diversion and were subject to surgeon bias. No significant differences between cohorts were observed with regard to age, sex, tumor grade or histology, metastatic status, or extent of resection. The cumulative failure rate of ETV was 21%, whereas that of VPS surgery was 29% (p = 0.105). The median time to failure was earlier for ETV than for VPS surgery (0.82 [IQR 0.2-1.8] vs 4.7 months [IQR 0.3-5.7], p = 0.03). Initially the ETV survival curve dropped sharply and then stabilized around 2 months. The VPS curve fell gradually but eventually crossed below the ETV curve at 5.7 months. Overall, a significant survival advantage was not demonstrated for one procedure over the other (p = 0.21, log-rank). However, postoperative complications were higher following VPS (31%) than ETV (17%) (p = 0.012). CONCLUSIONS ETV failure occurred sooner than VPS failure, but long-term treatment durability may be higher for ETV. Complications occurred more commonly with VPS than with ETV. Limited clinical conclusions are drawn using this methodology; the optimal treatment for PFBT-related hydrocephalus warrants investigation through prospective studies.

OBJECTIVE Endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) offers an alternative to shunt treatment for infantile hydrocephalus. Diagnosing treatment failure is dependent on infantile hydrocephalus metrics, including head circumference, fontanel quality, and ventricle size. However, it is not clear to what degree these metrics should be expected to change after ETV/CPC. Using these clinical metrics, the authors present and analyze the decision making in cases of ETV/CPC failure. METHODS Infantile hydrocephalus metrics, including bulging fontanel, head circumference z-score, and frontal and occipital horn ratio (FOHR), were compared between ETV/CPC failures and successes. Treatment outcome predictive values of metrics individually and in combination were calculated. RESULTS Forty-four patients (57% males, median age 1.2 months) underwent ETV/CPC for hydrocephalus; of these patients, 25 (57%) experienced failure at a median time of 51 days postoperatively. Patients experiencing failure were younger than those experiencing successful treatment (0.8 vs 3.9 months, p = 0.01). During outpatient follow-up, bulging anterior fontanel, progressive macrocephaly, and enlarging ventricles each demonstrated a positive predictive value (PPV) of no less than 71%, but a bulging anterior fontanel remained the most predictive indicator of ETV/CPC failure, with a PPV of 100%, negative predictive value of 73%, and sensitivity of 72%. The highest PPVs and specificities existed when the clinical metrics were present in combination, although sensitivities decreased expectedly. Only 48% of failures were diagnosed on the basis all 3 hydrocephalus metrics, while only 37% of successes were negative for all 3 metrics. In the remaining 57% of patients, a diagnosis of success or failure was made in the presence of discordant data. CONCLUSIONS Successful ETV/CPC for infantile hydrocephalus was evaluated in relation to fontanel status, head growth, and change in ventricular size. In most patients, a designation of failure or success was made in the setting of discordant data.

OBJECT Osseous anomalies of the craniocervical junction are hypothesized to precipitate the hindbrain herniation observed in Chiari I malformation (CM-I). Previous work by Tubbs et al. showed that posterior angulation of the odontoid process is more prevalent in children with CM-I than in healthy controls. The present study is an external validation of that report. The goals of our study were 3-fold: 1) to externally validate the results of Tubbs et al. in a different patient population; 2) to compare how morphometric parameters vary with age, sex, and symptomatology; and 3) to develop a correlative model for tonsillar ectopia in CM-I based on these measurements. METHODS The authors performed a retrospective review of 119 patients who underwent posterior fossa decompression with duraplasty at the Monroe Carell Jr. Children's Hospital at Vanderbilt University; 78 of these patients had imaging available for review. Demographic and clinical variables were collected. A neuroradiologist retrospectively evaluated preoperative MRI examinations in these 78 patients and recorded the following measurements: McRae line length; obex displacement length; odontoid process parameters (height, angle of retroflexion, and angle of retroversion); perpendicular distance to the basion-C2 line (pB-C2 line); length of cerebellar tonsillar ectopia; caudal extent of the cerebellar tonsils; and presence, location, and size of syringomyelia. Odontoid retroflexion grade was classified as Grade 0, > 90°; Grade I,85°-89°; Grade II, 80°-84°; and Grade III, < 80°. Age groups were defined as 0-6 years, 7-12 years, and 13-17 years at the time of surgery. Univariate and multivariate linear regression analyses, Kruskal-Wallis 1-way ANOVA, and Fisher's exact test were performed to assess the relationship between age, sex, and symptomatology with these craniometric variables. RESULTS The prevalence of posterior odontoid angulation was 81%, which is almost identical to that in the previous report (84%). With increasing age, the odontoid height (p < 0.001) and pB-C2 length (p < 0.001) increased, while the odontoid process became more posteriorly inclined (p = 0.010). The pB-C2 line was significantly longer in girls (p = 0.006). These measurements did not significantly correlate with symptomatology. Length of tonsillar ectopia in pediatric CM-I correlated with an enlarged foramen magnum (p = 0.023), increasing obex displacement (p = 0.020), and increasing odontoid retroflexion (p < 0.001). CONCLUSIONS Anomalous bony development of the craniocervical junction is a consistent feature of CM-I in children. The authors found that the population at their center was characterized by posterior angulation of the odontoid process in 81% of cases, similar to findings by Tubbs et al. (84%). The odontoid process appeared to lengthen and become more posteriorly inclined with age. Increased tonsillar ectopia was associated with more posterior odontoid angulation, a widened foramen magnum, and an inferiorly displaced obex.

Object: The clinical significance of radiological measurements of the craniocervical junction in pediatric Chiari I malformation (CM-I) is yet to be fully established across the field. The authors examined their institutional experience with the pB-C2 line (drawn perpendicular to a line drawn between the basion and the posterior aspect of the C-2 vertebral body, at the most posterior extent of the odontoid process at the dural interface). The pB-C2 line is a measure of ventral canal encroachment, and its relationship with symptomatology and syringomyelia in pediatric CM-I was assessed., Methods: The authors performed a retrospective review of 119 patients at the Monroe Carell Jr. Children's Hospital at Vanderbilt University who underwent posterior fossa decompression with duraplasty, 78 of whom had imaging for review. A neuroradiologist retrospectively evaluated preoperative and postoperative MRI examinations performed in these 78 patients, measuring the pB-C2 line length and documenting syringomyelia. The pB-C2 line length was divided into Grade 0 (<3 mm) and Grade I (≥3 mm). Statistical analysis was performed using the t-test for continuous variables and Fisher's exact test analysis for categorical variables. Multivariate logistic and linear regression analyses were performed to assess the relationship between pB-C2 line grade and clinical variables found significant on univariate analysis, controlling for age and sex., Results: The mean patient age was 8.5 years, and the mean follow-up duration was 2.4 years. The mean pB-C2 line length was 3.5 mm (SD 2 mm), ranging from 0 to 10 mm. Overall, 65.4% of patients had a Grade I pB-C2 line. Patients with Grade I pB-C2 lines were 51% more likely to have a syrinx than those with Grade 0 pB-C2 lines (RR 1.513 [95% CI 1.024-2.90], p=0.021) and, when present, had greater syrinx reduction (3.6 mm vs 0.2 mm, p=0.002). Although there was no preoperative difference in headache incidence, postoperatively patients with Grade I pB-C2 lines were 69% more likely to have headache reduction than those with Grade 0 pB-C2 lines (RR 1.686 [95% CI 1.035-2.747], p=0.009). After controlling for age and sex, pB-C2 line grade remained an independent correlate of headache improvement and syrinx reduction., Conclusions: Ventral canal encroachment may explain the symptomatology of select patients with CM-I. The clinical findings presented suggest that patients with Grade I pB-C lines2, with increased ventral canal obstruction, may experience a higher likelihood of syrinx reduction and headache resolution from decompressive surgery with duraplasty than those with Grade 0 pB-C2 lines.