Racial and socioeconomic disparities in the advanced treatment of medically intractable pediatric epilepsy.

OBJECTIVE: Racial and ethnic disparities in healthcare have gained significant importance since the Institute of Medicine published its report on disparities in healthcare. There is a lack of evidence on how race and ethnicity affect access to advanced treatment of pediatric medically intractable epilepsy. In this context, the authors analyzed the latest Kids' Inpatient Database (KID) for racial/ethnic disparities in access to surgical treatment of epilepsy. METHODS: The authors queried the KID for the years 2016 and 2019 for the diagnosis of medically intractable epilepsy. RESULTS: A total of 29,292 patients were included in the sample. Of these patients, 8.9% (n = 2610) underwent surgical treatment/invasive monitoring. The mean ages in the surgical treatment and nonsurgical treatment groups were 11.73 years (SD 5.75 years) and 9.5 years (SD 6.16 years), respectively. The most common insurance in the surgical group was private/commercial (55.9%) and Medicaid in the nonsurgical group (47.7%) (p < 0.001). White patients accounted for the most common population in both groups, followed by Hispanic patients. African American patients made up 7.9% in the surgical treatment group compared with 12.9% in the nonsurgical group. African American (41.1%) and Hispanic (29.9%) patients had higher rates of emergency department (ED) utilization compared with the White population (24.6%). After adjusting for all covariates, the odds of surgical treatment increased with increasing age (OR 1.06, 95% CI 1.053-1.067; p < 0.001). African American race (OR 0.513, 95% CI 0.443-0.605; p < 0.001), Hispanic ethnicity (OR 0.681, 95% CI 0.612-0.758; p < 0.001), and other races (OR 0.789, 95% CI 0.689-0.903; p = 0.006) had lower surgical treatment odds compared with the White population. Medicaid/Medicare was associated with lower surgical treatment odds than private/commercial insurance (OR 0.603, 0.554-0.657; p < 0.001). Interaction analysis revealed that African American (OR 0.708, 95% CI 0.569-0.880; p = 0.001) and Hispanic (OR 0.671, 95% CI 0.556-0.809; p < 0.001) populations with private insurance had lower surgical treatment odds than White populations with private insurance. Similarly, African American patients, Hispanic patients, and patients of other races with nonprivate insurance also had lower surgical treatment odds than their White counterparts after adjusting for all other covariates. CONCLUSIONS: Based on the KID, African American and Hispanic populations had lower surgical treatment rates than their White counterparts, with higher utilization of the ED for pediatric medically intractable epilepsy.

Wound vacuum-assisted closure as a bridge therapy in the treatment of infected cranial gunshot wound in a pediatric patient: illustrative case.

BACKGROUND: The authors reported the first pediatric case of a craniocerebral gunshot injury successfully treated with a wound vacuum-assisted closure (VAC) device after dehiscence and infection of the initial cranial wound. OBSERVATIONS: A 17-year-old boy suffered several gunshots to the left hemisphere, resulting in significant damage to the scalp, calvaria, and brain. Emergency hemicraniectomy was performed, with reconstruction of a complicated scalp wound performed at the initial surgery. The scalp was devitalized and ultimately dehisced, resulting in a cranial infection. It was treated first with a repeated attempt at primary closure, which failed because of persistent devitalized tissue, and was then treated with aggressive debridement followed by placement of a wound VAC device over the exposed brain as a bridge therapy to reconstruction. This procedure was deemed necessary given the active infection. LESSONS: The patient received delayed reconstruction with a free split-thickness skin graft and made a remarkable recovery, with cranioplasty performed 6 months later. The authors reviewed the literature on wound VAC use in cranial wound treatment and proposed it as a legitimate bridge therapy to definitive reconstruction in the setting of dirty wounds, active infection, or even hemodynamically unstable patients.

Novel neoplasms associated with syndromic pediatric medulloblastoma: integrated pathway delineation for personalized therapy.

Medulloblastoma is the most common pediatric embryonal brain tumor, and may occur in cancer predisposition syndromes. We describe novel associations of medulloblastoma with atypical prolactinoma and dural high-grade sarcoma in Li-Fraumeni syndrome (LFS), and epidural desmoid fibromatosis in familial adenomatous polyposis (FAP)/Turcot syndrome. Genomic analysis showing XRCC3 alterations suggested radiotherapy as contributing factor to the progression of LFS-associated medulloblastoma, and demonstrated different mechanisms of APC inactivation in the FAP-associated tumors. The integrated genomic-transcriptomic analysis uncovered the growth pathways driving tumorigenesis, including the prolactin-prolactin receptor (PRLR) autocrine loop and Shh pathway in the LFS-associated prolactinoma and medulloblastoma, respectively, the Wnt pathway in both FAP-associated neoplasms, and the TGFß and Hippo pathways in the soft tissue tumors, regardless of germline predisposition. In addition, the comparative analysis of paired syndromic neoplasms revealed several growth pathways susceptible to therapeutic intervention by PARP, PRLR, and selective receptor tyrosine kinase (RTK) inhibitors. These could target the defective DNA damage repair in the LFS-associated medulloblastoma, the prolactin autocrine loop in the atypical prolactinoma, the EPHA3/7 and ALK overexpression in the FAP-associated medulloblastoma, and the multi-RTK upregulation in the soft tissue neoplasms. This study presents the spatiotemporal evolution of novel neoplastic associations in syndromic medulloblastoma, and discusses the post-radiotherapy risk for secondary malignancies in syndromic pediatric patients, with important implications for the biology, diagnosis, and therapy of these tumors. Video Abstract.

Focal epilepsy caused by single cerebral cavernous malformation (CCM) is associated with regional and global resting state functional connectivity (FC) disruption.

Epilepsy, including the type with focal onset, is increasingly viewed as a disorder of the brain network. Here we employed the functional connectivity (FC) metrics estimated from the resting state functional MRI (rsfMRI) to investigate the changes of brain network associated with focal epilepsy caused by single cerebral cavernous malformation (CCM). Eight CCM subjects and 21 age and gender matched controls were enrolled in the study. Seven of 8 CCM subjects underwent surgical resection of the CCM and became seizure free and 4 of the surgical subjects underwent a repeat rsfMRI study. We showed that there was both regional and global disruption of the FC values among the CCM subjects including decreased in homotopic FC (HFC) and global FC (GFC) in the regions of interest (ROIs) where the CCMs were located. There was also the disruption of the default mode network (DMN) especially the FC between the middle prefrontal cortex (MPFC) and the right lateral parietal cortex (LPR) among these individuals. We observed the trend of alleviation of these disruptions after the individual has become seizure free from the surgical resection of the CCM. Using a voxel-based approach, we found the disruption of the HFC and GFC in the brain tissue immediately adjacent to the CCM and the severity of the disruption appeared inversely proportional to the distance of the brain tissue to the lesion. Our findings confirm the disruption of normal brain networks from focal epilepsy, a process that may be reversible with successful surgical treatments rendering patients seizure free. Some voxel-based metrics may help identify the epileptogenic zone and guide the surgical resection.

Gender disparities in academic rank achievement in neurosurgery: a critical assessment.

OBJECTIVE: The objective of this study was to evaluate whether there are disparities in academic rank and promotion between men and women neurosurgeons. METHODS: The profiles of faculty members from 50 academic neurosurgery programs were reviewed to identify years in practice, number of PubMed-indexed publications, Doctor of Philosophy (PhD) attainment, and academic rank. The number of publications at each academic rank was compared between men and women after controlling for years in practice by using a negative binomial regression model. The relationship between gender and each academic rank was also determined after controlling for clustering at the institutional level, years in practice, and number of publications. RESULTS: Of 841 faculty members identified, 761 (90%) were men (p = 0.0001). Women represented 12% of the assistant and associate professors but only 4% of the full professors. Men and women did not differ in terms of the percentage holding a PhD, years in practice, or number of publications at any academic rank. After controlling for years in practice and clustering at the facility level, the authors found that men were twice as likely as women to be named full professor (OR 2.2, 95% CI 1.09-4.44, p = 0.03). However, when institution, years in practice, PhD attainment, h-index, and number of publications were considered, men and women were equally likely to attain full professorship (OR 0.9, 95% CI 0.42-1.93). CONCLUSIONS: Data analysis of the top neurosurgery programs suggests that although there are fewer women than men holding positions in academic neurosurgery, faculty rank attainment does not seem to be influenced by gender.

Mutations of the MAPK/TSC/mTOR pathway characterize periventricular glioblastoma with epithelioid SEGA-like morphology-morphological and therapeutic implications.

Epithelioid glioblastoma is a recognized glioblastoma variant, recently added to the World Health Organization brain tumor classification, with similar prognosis as the classic variant and B-Raf V600E mutations in 50% of the cases. We identified a new subset of epithelioid glioblastoma with periventricular location and subependymal giant cell astrocytoma (SEGA)-like morphology. Genomic profiling of these tumors revealed driver mutations in NF1, subclonal mutations in TSC1, and a novel driver mutation in MTOR, suggesting upregulation of the MAPK/TSC1/mTOR pathway. Strong mTOR activation was confirmed by immunohistochemistry for the mTOR kinase target 4E-BP1. TSC1 and MTOR mutations have been previously described in low-grade glioma, such as SEGA, and focal cortical dysplasia, respectively, that display large cells with abundant cytoplasm, most likely resulting from the biogenetic signaling of mTOR. Unlike these, the mutations in SEGA-like glioblastoma occurred in the context of other genetic aberrations present in high-grade neoplasms, including in the CDKN2A/B, PIK3R1, PIK3CA and EGFR genes. For one patient with two temporally distinct specimens, the subclonal TSC1 pathogenic mutation was detected only in the specimen showing SEGA-like morphology, indicating requirement for mTOR activation as trigger for specific epithelioid/SEGA-like morphology. As FDA-approved kinase inhibitors are available and target many steps of the MAPK/mTOR pathway, recognition of this new subset of periventricular high-grade gliomas with clear phenotypic-genotypic correlates is essential for prompt biomarker testing and appropriate targeted therapeutic management of these patients.

Noninvasive Intracranial Pressure Monitoring for Severe Traumatic Brain Injury in Children: A Concise Update on Current Methods.

BACKGROUND: Traumatic brain injury (TBI) is a leading cause of pediatric morbidity and mortality worldwide and intracranial pressure (ICP) monitoring plays a crucial role in its management. Based on existing literature, we review the current practicing noninvasive ICP monitoring devices and their accuracy in predicting increased ICP in pediatric TBI. METHODS: A thorough literature search was conducted on PubMed, Medline, and the Cochrane database, articles were selected systematically and reviewed completely, and relevant data were summarized and discussed. RESULTS: A total of 27 articles pertaining to pediatric TBI were included and reviewed. We found various modalities of noninvasive ICP monitoring devices used over the last few years. The noninvasive modalities so far attempted in pediatric TBI and so reviewed here are transcranial Doppler, optic nerve sheath diameter, otoacoustic emission, near-infrared spectroscopy, contrast-enhanced ultrasonography, and quantitative pupillometry. CONCLUSIONS: Invasive monitoring methods are the current gold standard for monitoring ICP; however, complications caused by their invasive nature are of concern. Of all the noninvasive methods based on the literature, we found transcranial Doppler and optic nerve sheath diameter assessment to be the best tools to monitor ICP in pediatric TBI. The promising results and developments of noninvasive ICP monitoring modalities with its ideal features of high sensitivity, diagnostic accuracy, and simple acquisition technique may make it the future of neurointensive monitoring in pediatric TBI.

Impact of Psychiatric Comorbidities on Short-Term Outcomes Following Intervention for Lumbar Degenerative Disc Disease.

STUDY DESIGN: Retrospective, observational cohort study. OBJECTIVE: To investigate the impact of psychiatric diseases on short-term outcomes in patients undergoing fusion surgery for lumbar degenerative disc disease (LDDD). SUMMARY OF BACKGROUND DATA: Limited literature exists on the prevalence and impact of psychiatric comorbidities on outcomes in patients undergoing surgery for LDDD. METHODS: Adult patients (>18 yr) registered in the Nationwide Inpatient Sample database (2002-2011) and undergoing an elective spine fusion for LDDD that met inclusion criteria formed the study population. Defined primary outcome measures were discharge disposition, length of stay, hospitalization cost, and short-term postsurgical complications (neurological, respiratory, cardiac, gastrointestinal, wound complication and infections, venous thromboembolism, and acute renal failure). Multivariable regression techniques were used to explore the association of psychiatric comorbidities on short-term outcomes by adjusting for patient demographics, clinical, and hospital characteristics. RESULTS: Of the 126,044 adult patients undergoing fusion surgery for LDDD (mean age: 54.91 yr, 58% female) approximately 18% had a psychiatric disease. Multivariable regression analysis revealed patients with psychiatric disease undergoing fusion surgery have higher likelihood for unfavorable discharge (odds ratio [OR] 1.41; 95% confidence interval [CI] 1.35-1.47; P < 0.001), length of stay (OR 1.03; 95% CI 1.02-1.04; P < 0001), postsurgery neurologic complications (OR 1.25; 95% CI 1.13-1.37; P < 0.001), venous thromboembolic events (OR 1.38 95% CI 1.26-1.52; P < 0.001), and acute renal failure (OR 1.17; 95% CI 1.01-1.37; P = 0.040). Patients with psychiatric disease were also associated to have higher hospitalization cost (6.3% higher; 95% CI: 5.6%-7.1%; P < 0.001) compared to those without it. CONCLUSION: Our study quantifies the estimates for presence of concomitant psychiatric comorbid conditions on short outcomes in patients undergoing fusions for LDDD. The data provide supporting evidence for adequate preoperative planning and postsurgical care including consultation for mental health for favorable outcomes. LEVEL OF EVIDENCE: 4.

Tentorial Meningiomas: Reappraisal of Surgical Approaches and Their Outcomes.

BACKGROUND: Tentorial meningiomas are notorious for their critical location. Selection of a suitable approach that exposes the multicompartmental growth of tumor is important for a complete and safe resection. This paper discusses about various operative approaches and their overall surgical outcome. METHODS: We retrospectively reviewed our 41 patients with tentorial meningiomas. They were classified according to the modified Yasargil classification. The symptomatic improvement and progression-free survival (PFS) were analyzed at follow-up. RESULTS: Tumors were almost equally distributed in all location groups. Tumors along the lateral tentorial hiatus were operated on via a subtemporal or transsylvian approach. Tumors along the posterior tentorial hiatus, tentorial membrane, or torcula were operated on by an occipital interhemispheric transtentorial, infratentorial supracerebellar, or a combined approach. Tumors along the petrous attachment were operated on by a retromastoid suboccipital or a combined presigmoid-retrosigmoid approach. Seventy-six percent had total excision (Simpson grade 1 and 2). Group II tumors had the highest total resection rate (100%). Headache and diplopia were the symptoms that significantly improved postoperatively. Over a median follow-up of 65 months, 13 patients (31.7%) had recurrence. There was no significant difference in recurrence rates and PFS in tumors at different locations. Extent of excision and tumor grade were the significant factors affecting PFS in both univariate and multivariate analysis (P = 0.01 and 0.03, respectively). CONCLUSIONS: Similar to intracranial meningiomas at other locations, extent of resection and tumor grade significantly affect the PFS for tentorial meningiomas. Careful preoperative planning based on the location and extension of the tumor guides the optimal surgical approach that translates into maximal safe resection.

Proposing a validated clinical app predicting hospitalization cost for extracranial-intracranial bypass surgery.

OBJECT: United States healthcare reforms are focused on curtailing rising expenditures. In neurosurgical domain, limited or no data exists identifying potential modifiable targets associated with high-hospitalization cost for cerebrovascular procedures such as extracranial-intracranial (ECIC) bypass. Our study objective was to develop a predictive model of initial cost for patients undergoing bypass surgery. METHODS: In an observational cohort study, we analyzed patients registered in the Nationwide Inpatient Sample (2002-2011) that underwent ECIC bypass. Split-sample 1:1 randomization of the study cohort was performed. Hospital cost data was modelled using ordinary least square to identity potential drivers impacting initial hospitalization cost. Subsequently, a validated clinical app for estimated hospitalization cost is proposed (https://www.neurosurgerycost.com/calc/ec-ic-by-pass). RESULTS: Overall, 1533 patients [mean age: 45.18 ± 19.51 years; 58% female] underwent ECIC bypass for moyamoya disease [45.1%], cerebro-occlusive disease (COD) [23% without infarction; 12% with infarction], unruptured [12%] and ruptured [4%] aneurysms. Median hospitalization cost was $37,525 (IQR: $16,225-$58,825). Common drivers impacting cost include Asian race, private payer, elective admission, hyponatremia, neurological and respiratory complications, acute renal failure, bypass for moyamoya disease, COD without infarction, medium and high volume centers, hospitals located in Midwest, Northeast, and West region, total number of diagnosis and procedures, days to bypass and post-procedural LOS. Our model was validated in an independent cohort and using 1000-bootstrapped replacement samples. CONCLUSIONS: Identified drivers of hospital cost after ECIC bypass could potentially be used as an adjunct for creation of data driven policies, impact reimbursement criteria, aid in-hospital auditing, and in the cost containment debate.

Impact of Hospital Caseload and Elective Admission on Outcomes After Extracranial-Intracranial Bypass Surgery.

BACKGROUND: Limited information exists evaluating the impact of hospital caseload and elective admission on outcomes after patients have undergone extracranial-intracranial (ECIC) bypass surgery. Using the Nationwide Inpatient Sample (NIS) for 2001-2014, we evaluated the impact of hospital caseload and elective admission on outcomes after bypass. METHODS: In an observational cohort study, weighted estimates were used to investigate the association of hospital caseload and elective admission on short-term outcomes after bypass surgery using multivariable regression techniques. RESULTS: Overall, 10,679 patients (mean age, 43.39 ± 19.63 years; 59% female) underwent bypass across 495 nonfederal U.S. hospitals. In multivariable models, patients undergoing bypass at high-volume centers were associated with decreased probability of mortality (odds ratio [OR], 0.39; 95% confidence interval [CI], 0.22-0.70; P < 0.001), length of stay (OR, 0.86; 95% CI, 0.82-0.90; P < 0.001), postbypass neurologic complications (OR, 0.66; 95% CI, 0.49-0.89; P = 0.007), venous thromboembolism (OR, 0.69; 95% CI, 0.49-0.97; P = 0.033), and acute renal failure (OR, 0.45; 95% CI, 0.26-0.80; P = 0.007), and higher hospitalization cost (26.3% higher) compared with low-volume centers. Likewise, patients undergoing elective bypass were associated with decreased likelihood of mortality (OR, 0.38; 95% CI, 0.25-0.59; P < 0.001), unfavorable discharge (OR, 0.57; 95% CI, 0.43-0.76; P < 0.001), length of stay (OR, 0.62; 95% CI, 0.59-0.64; P < 0.001), venous thromboembolism (OR, 0.61; 95% CI, 0.49-0.77; P < 0.001), acute renal failure (OR, 0.64; 95% CI, 0.43-0.94; P = 0.022), wound complications (OR, 0.71; 95% CI, 0.53-0.96; P = 0.028), and lower hospitalization cost (34.5% lower) compared with nonelective admissions. CONCLUSIONS: Our findings serve as a framework for strengthening referral networks for complex cases to centers performing high volumes of cerebral bypass. Also, our study supports improved outcomes in select patients undergoing elective bypass procedures.

Impact of Prehospital Transportation on Survival in Skiers and Snowboarders with Traumatic Brain Injury.

INTRODUCTION: Prehospital helicopter use and its impact on outcomes in snowboarders and skiers incurring traumatic brain injury (TBI) is unknown. The present study investigates the association of helicopter transport with survival of snowboarders and skiers with TBI, in comparison with ground emergency medical services (EMS), by using data derived from the National Trauma Data Bank (2007-2014). METHODS: Primary and secondary endpoints were defined as in-hospital survival and absolute risk reduction based upon number needed to transport (treat) respectively. Multivariable regression models including traditional logit model, model fitted with generalized estimating equations, and those incorporating results from propensity score matching methods were used to investigate the association of helicopter transport with survival compared with ground EMS. RESULTS: Of the 1018 snowboarders and skiers who met the criteria, 360 (35.4%) were transported via helicopters whereas 658 (64.6%) via ground EMS with a mortality rate of 1.7% and 1.5%, respectively. Multivariable log-binomial models demonstrated association of prehospital helicopter transport with increased survival (odds ratio 8.58; 95% confidence interval 1.09-67.64; P = 0.041; absolute risk reduction: 10.06%). This finding persisted after propensity score matching (odds ratio 24.73; 95% confidence interval 5.74-152.55; P < 0.001). The corresponding absolute risk reduction implies that approximately 10 patients need to be transported via helicopter to save 1 life. CONCLUSIONS: Based on our robust statistical analysis of retrospective data, our findings suggest prehospital helicopter transport improved survival in patients incurring TBI after snowboard- or ski-related falls compared with those transported via ground EMS. Policies directed at using helicopter services at remote winter resorts or ski or snowboarding locations should be implemented.

Factors Associated with Abducens Nerve Recovery in Patients Undergoing Surgical Resection of Sixth Nerve Schwannoma: A Systematic Review and Case Illustration.

BACKGROUND: Limited or no literature exists identifying factors associated with functional nerve recovery in patients undergoing resection of sixth cranial nerve (CN VI) schwannomas. METHODS: A systematic review of literature was performed on CN VI schwannomas that were treated surgically. Synthesizing the findings pooled from the literature, we investigated associations of patient demographics and clinical characteristics with postsurgical CN VI functional recovery in multivariable regression models. In addition, we present the findings of an adolescent woman surgically managed for intracavernous CN VI schwannoma. Complete encasement of the cavernous segment of the internal carotid artery is unique to our case. RESULTS: We synthesized data of 32 patients from 29 studies, and our index case. Overall, the mean age of the patients was 44.0 ± 16.5 years, and approximately 52% (n = 17) were female. Most tumors were left-sided (n = 18; 54.5%), with an average size of 3.46 ± 1.71 cm. The most common location was cisternal (n = 11; 33%), followed by cavernous sinus (CS) proper (n = 9; 27%), cisterocavernous (n = 8; 24%), orbital (n = 4; 12%) and caverno-orbital (n = 1; 3%). CN VI recovery was reported in less than half the cohort (n = 14; 45%). Tumor extension in the CS was significantly associated with lesser likelihood (odds ratio [OR], 0.07; 95% confidence interval [CI], 0.01-0.98; P = 0.048) of postsurgical CN VI recovery. Although female gender (OR, 0.86; 95% CI, 0.07-10.09; P = 0.906), large tumor size (>2.5 cm) (OR, 0.45; 95% CI, 0.07-2.89; P = 0.397), and solid consistency (OR, 0.37; 95% CI, 0.03-4.19; P = 0.421) were associated with lesser odds for recovery, these were not statistically significant. Likewise, although gross total resection (OR, 6.28; 95% CI, 0.33-118.25; P = 0.220) was associated with higher odds of nerve recovery, the estimates were statistically insignificant. CONCLUSIONS: CS involvement is associated with lesser odds for functional nerve recovery in patients undergoing surgical resection for CN VI schwannoma.

Revisiting racial disparities in access to surgical management of drug-resistant temporal lobe epilepsy post implementation of Affordable Care Act.

OBJECTIVES: Prior to enactment of the Affordable Care Act(ACA), several reports demonstrated remarkable racial disparities in access to surgical care for epileptic patients. Implementation of ACA provided healthcare access to 7-16 million uninsured Americans. The current study investigates racial disparity post ACA era in (1) access to surgical management of drug-resistant temporal lobe epilepsy (DRTLE); (2) short-term outcomes in the surgical cohort. PATIENT AND METHODS: Adult patients with DRTLE registered in the National Inpatient Sample (2012-2013) were identified. Association of race (African Americans and other minorities with respect to Caucasians) with access to surgical management of TLE, and short-term outcomes [discharge disposition, length of stay (LOS) and hospital charges] in the surgical cohort were investigated using multivariable regression techniques. RESULTS: Of the 4062 patients with DRTLE, 3.6%(n=148) underwent lobectomy. Overall, the mean age of the cohort was 42.35±16.33years, and 54% were female. Regression models adjusted for patient demographics, clinical and hospital characteristics demonstrated no racial disparities in access to surgical care for DRTLE. Likewise, no racial disparity was noted in outcomes in the surgical cohort. CONCLUSION: Our study reflects no racial disparity in access to surgical care in patients with DRTLE post 2010 amendment of the ACA. The seismic changes to the US healthcare system may plausibly have accounted for addressing the gap in racial disparity for epilepsy surgery.

Cranium Bifidum Occultum Associated with Hypertelorism Treated with Posterior Vault Reconstruction and Orbital Box Osteotomies: Case Report and Technical Note.

Cranium bifidum occultum is a disorder of skull ossification presenting as an enlarged posterior fontanelle in the upper posterior angle of the parietal bone near the intersection of the sagittal and lambdoid sutures. The standard treatment for cranium bifidum occultum is observation. We present a case of a 5-year-old boy who presented with a 15 × 4.5 cm midline posterior cranial vault defect consistent with diagnosis of cranium bifidum occultum associated with orbital hypertelorism and a widened nose. The patient underwent posterior vault reconstruction for correction of cranium bifidum occultum defect followed by bifrontal craniotomy and orbital box osteotomies for correction of orbital hypertelorism and nasal deformity. To our knowledge, this is the first reported case describing surgical treatment for cranium bifidum occultum associated with orbital hypertelorism.

Role of radiological parameters in predicting overall shunt outcome after ventriculoperitoneal shunt insertion in pediatric patients with obstructive hydrocephalus.

OBJECTIVE Despite significant advances in the medical field and shunt technology, shunt malfunction remains a nightmare of pediatric neurosurgeons. In this setting, the ability to preoperatively predict the probability of shunt malfunction is quite compelling. The authors have compared the preoperative radiological findings in obstructive hydrocephalus and the subsequent clinical course of the patient to determine any association with overall shunt outcome. METHODS This retrospective study included all pediatric patients (age < 18 years) who had undergone ventriculoperitoneal shunt insertion for obstructive hydrocephalus. Linear measurements were taken from pre- and postoperative CT or MRI studies to calculate different indices and ratios including Evans' index, frontal horn index (FHI), occipital horn index (OHI), frontooccipital horn ratio (FOHR), and frontooccipital horn index ratio (FOIR). Other morphological features such as bi- or triventriculomegaly, right-left ventricular symmetry, and periventricular lucency (PVL) were also noted. The primary clinical outcomes that were reviewed included the need for shunt revision, time interval to first shunt revision, frequency of shunt revisions, and revision-free survival. RESULTS A total of 121 patients were eligible for the analysis. Nearly half of the patients (47.9%) required shunt revision. The presence of PVL was associated with lower revision rates than those in others (39.4% vs 58.2%, p = 0.03). None of the preoperative radiological indices or ratios showed any correlation with shunt revision. Nearly half of the patients with shunt revision required early revision (< 90 days of primary surgery). The reduction in the FOHR was high in patients who required early shunt revision (20.16% in patients with early shunt revision vs 6.4% in patients with late shunt revision, p = 0.009). Nearly half of the patients (48.3%) requiring shunt revision ultimately needed more than one revision procedure. Greater occipital horn dilation on preoperative images was associated with a lower frequency of shunt revision, as dictated by a high OHI and a low FOIR in patients with a single shunt revision as compared with those in patients who required multiple shunt revisions (p = 0.029 and 0.009, respectively). The mean follow-up was 49.9 months. Age was a significant factor affecting shunt revision-free survival. Patients younger than 6 months of age had significantly less revision-free survival than the patients older than 6 months (median survival of 10.1 vs 94.1 months, p = 0.004). CONCLUSIONS Preoperative radiological linear indices and ratios do not predict the likelihood of subsequent shunt malfunction. However, patients who required early shunt revision tended to have greater reductions in ventricular volumes on postoperative images. Therefore a greater reduction in ventricular volume is not actually desirable, and a ventricular volume high enough to reduce intracranial pressure is instead to be aimed at for long-term shunt compliance.

Epidemiology of adult-onset hydrocephalus: institutional experience with 2001 patients.

OBJECTIVE Adult-onset hydrocephalus is not commonly discussed in the literature, especially regarding its demographic distribution. In contrast to pediatric hydrocephalus, which is related to a primary CSF pathway defect, its development in adults is often secondary to other pathologies. In this study, the authors investigated the epidemiology of adult-onset hydrocephalus as it pertains to different etiologies and in reference to age, sex, and race distributions. METHODS The authors retrospectively reviewed the clinical notes of 2001 patients with adult-onset hydrocephalus who presented to Louisiana State University Health Sciences Center within a 25-year span. Significant differences between the groups were analyzed by a chi-square test; p < 0.05 was considered significant. RESULTS The overall mean (± SEM) incidence of adult hydrocephalus in this population was 77 ± 30 per year, with a significant increase in incidence in the past decade (55 ± 3 [1990-2003] vs 102 ± 6 [2004-2015]; p < 0.0001). Hydrocephalus in a majority of the patients had a vascular etiology (45.5%) or was a result of a tumor (30.2%). The incidence of hydrocephalus in different age groups varied according to various pathologies. The incidence was significantly higher in males with normal-pressure hydrocephalus (p = 0.03) or head injury (p = 0.01) and higher in females with pseudotumor cerebri (p < 0.0001). In addition, the overall incidence of hydrocephalus was significantly higher in Caucasian patients (p = 0.0002) than in those of any other race. CONCLUSIONS Knowledge of the demographic variations in adult-onset hydrocephalus is helpful in achieving better risk stratification and better managing the disease in patients. For general applicability, these results should be validated in a large-scale meta-analysis based on a national population database.

Psammomatous Cavernous Malformation Presenting as Drug-Resistant Epilepsy: Case Illustration and Review of Literature.

BACKGROUND: Psammoma bodies (PBs) are whorled, laminated hyaline spherules containing calcium deposits. Intracranially, the presence of PBs is associated with variants of meningioma and pituitary lesions, as well as aging choroid plexus. Limited information exists on their presence in vascular malformation. RESULTS: In this report, we describe a case of an adolescent male with drug-resistant epilepsy that was surgically managed at our regional epilepsy center. The epileptogenic focus was determined to be emanating from an indolent right insular lesion. Histopathologic evaluation showed the abundance of intravascular and perivascular PBs. Immunohistochemical evaluation confirmed the vascular origin using vascular markers. The unusual presence of PBs in a vascular lesion was unanticipated. CONCLUSIONS: Based on our case, we present the clinicoradiologic characteristics, supplemented with intraoperative findings, for this unusual lesion. In addition, because of the unusual presence of PBs in vascular lesions, we provide the findings of a systematic literature review to show the association of PBs with intracranial vascular lesions.