A child with unilateral abducens nerve palsy and neurovascular compression in Chiari malformation type 1 resolved with posterior fossa decompression: illustrative case.

BACKGROUND: Unilateral cranial nerve (CN) VI, or abducens nerve, palsy is rare in children and has not been reported in association with Chiari malformation type 1 (CM1) in the absence of other classic CM1 symptoms. OBSERVATIONS: A 3-year-old male presented with acute incomitant esotropia consistent with a unilateral, left CN VI palsy and no additional neurological symptoms. Imaging demonstrated CM1 without hydrocephalus or papilledema, as well as an anterior inferior cerebellar artery (AICA) vessel loop in the immediate vicinity of the left abducens nerve. Given the high risk of a skull base approach for direct microvascular decompression of the abducens nerve and the absence of other classic Chiari symptoms, the patient was initially observed. However, as his palsy progressed, he underwent posterior fossa decompression with duraplasty (PFDD), with the aim of restoring global cerebrospinal fluid dynamics and decreasing possible AICA compression of the left abducens nerve. Postoperatively, his symptoms completely resolved. LESSONS: In this first reported case of CM1 presenting as a unilateral abducens palsy in a young child, possibly caused by neurovascular compression, the patient's symptoms resolved after indirect surgical decompression via PFDD.

Clinical criteria for filum terminale resection in occult tethered cord syndrome.

OBJECTIVE: Tethered cord syndrome (TCS) comprises three symptom categories: back/leg pain, bowel/bladder, and neurological complaints. MRI typically reveals a low-lying conus medullaris, filum terminale (FT) pathology, or lumbosacral abnormalities. FT resection is established in TCS but not in radiologically occult TCS (OTCS). This study aims to identify patients with OTCS who are likely to benefit from FT resection. METHODS: The authors recruited 149 patients with OTCS (31 pediatric, 118 adult) treated with FT resection-including only cases with progressive TCS, negative spine MRI, and no concurrent neurological/urological conditions. A comprehensive questionnaire collected patient self-reported symptoms and clinical findings at the preoperative and at 3- and 12-month follow-up examinations. Based on questionnaire data, the authors extracted a 15-item symptoms and findings scale to represent the three TCS symptom categories, assigning 1 point for each item present. RESULTS: OTCS presents without radicular/segmental sensorimotor findings, but with leg/back pain and conus dysfunction, in addition to leg fatigue and spasticity; the latter indicating an upper motoneuron pathology. The 15-item scale showed clinical improvement in 89% of patients at the 3-month follow-up and 68% at the 12-month follow-up. Multivariate analysis of the scale revealed that it accurately predicts outcome of FT resection in 82% of cases. Patients with a preoperative score exceeding 6 points are most likely to benefit from surgery. CONCLUSIONS: By applying the study's inclusion criteria and incorporating the novel 15-item scale, surgeons can effectively select candidates for FT resection in patients with OTCS. The observed outcomes in these selected patients are comparable to those achieved in degenerative spine surgery.

Laser amygdalohippocampotomy reduces contralateral hippocampal sub-clinical activity in bitemporal epilepsy: A case illustration of responsive neurostimulator ambulatory recordings.

Responsive neurostimulation (RNS) is a valuable tool in the diagnosis and treatment of medication refractory epilepsy (MRE) and provides clinicians with better insights into patients' seizure patterns. In this case illustration, we present a patient with bilateral hippocampal RNS for presumed bilateral mesial temporal lobe epilepsy. The patient subsequently underwent a right sided LITT amygdalohippocampotomy based upon chronic RNS data revealing predominance of seizures from that side. Analyzing electrocorticography (ECOG) from the RNS system, we identified the frequency of high amplitude discharges recorded from the left hippocampal lead pre- and post- right LITT amygdalohippocampotomy. A reduction in contralateral interictal epileptiform activity was observed through RNS recordings over a two-year period, suggesting the potential dependency of the contralateral activity on the primary epileptogenic zone. These findings suggest that early targeted surgical resection or laser ablation by leveraging RNS data can potentially impede the progression of dependent epileptiform activity and may aid in preserving neurocognitive networks. RNS recordings are essential in shaping further management decisions for our patient with a presumed bitemporal epilepsy.

Demographic Representation in 3 Leading Artificial Intelligence Text-to-Image Generators.

Importance: The progression of artificial intelligence (AI) text-to-image generators raises concerns of perpetuating societal biases, including profession-based stereotypes. Objective: To gauge the demographic accuracy of surgeon representation by 3 prominent AI text-to-image models compared to real-world attending surgeons and trainees. Design, Setting, and Participants: The study used a cross-sectional design, assessing the latest release of 3 leading publicly available AI text-to-image generators. Seven independent reviewers categorized AI-produced images. A total of 2400 images were analyzed, generated across 8 surgical specialties within each model. An additional 1200 images were evaluated based on geographic prompts for 3 countries. The study was conducted in May 2023. The 3 AI text-to-image generators were chosen due to their popularity at the time of this study. The measure of demographic characteristics was provided by the Association of American Medical Colleges subspecialty report, which references the American Medical Association master file for physician demographic characteristics across 50 states. Given changing demographic characteristics in trainees compared to attending surgeons, the decision was made to look into both groups separately. Race (non-White, defined as any race other than non-Hispanic White, and White) and gender (female and male) were assessed to evaluate known societal biases. Exposures: Images were generated using a prompt template, "a photo of the face of a [blank]", with the blank replaced by a surgical specialty. Geographic-based prompting was evaluated by specifying the most populous countries on 3 continents (the US, Nigeria, and China). Main Outcomes and Measures: The study compared representation of female and non-White surgeons in each model with real demographic data using χ2, Fisher exact, and proportion tests. Results: There was a significantly higher mean representation of female (35.8% vs 14.7%; P < .001) and non-White (37.4% vs 22.8%; P < .001) surgeons among trainees than attending surgeons. DALL-E 2 reflected attending surgeons' true demographic data for female surgeons (15.9% vs 14.7%; P = .39) and non-White surgeons (22.6% vs 22.8%; P = .92) but underestimated trainees' representation for both female (15.9% vs 35.8%; P < .001) and non-White (22.6% vs 37.4%; P < .001) surgeons. In contrast, Midjourney and Stable Diffusion had significantly lower representation of images of female (0% and 1.8%, respectively; P < .001) and non-White (0.5% and 0.6%, respectively; P < .001) surgeons than DALL-E 2 or true demographic data. Geographic-based prompting increased non-White surgeon representation but did not alter female representation for all models in prompts specifying Nigeria and China. Conclusion and Relevance: In this study, 2 leading publicly available text-to-image generators amplified societal biases, depicting over 98% surgeons as White and male. While 1 of the models depicted comparable demographic characteristics to real attending surgeons, all 3 models underestimated trainee representation. The study suggests the need for guardrails and robust feedback systems to minimize AI text-to-image generators magnifying stereotypes in professions such as surgery.

The epidemiology of crib-related head injuries: A ten-year nationwide analysis.

INTRODUCTION: Falls from cribs resulting in head injury are understudied and poorly characterized. The purpose of this study was to advance current understanding of the prevalence, descriptive characteristics of injury victims, and the types of crib fall-related head injuries (CFHI) using queried patient cases from the National Electronic Injury Surveillance System (NEISS) database. METHODS: Using the US Consumer Product Safety Commission's System NEISS database, we queried all CFHIs among children from over 100 emergency departments (EDs). Patient information regarding age, race, sex, location of the incident, diagnoses, ED disposition, and sequelae were analyzed. The number of CFHI from all US EDs during each year was also collected from the database. RESULTS: There were an estimated 54,799 (95% CI: 30,228-79,369) total visits to EDs for CFHIs between 2012 and 2021, with a decrease in incidence of approximately 20% during the onset of the COVID-19 pandemic (2019: 5616 cases, 2020: 4459 cases). The annual incidence of injuries showed no significant trend over the 10-year study period. An available subset of 1782 cases of head injuries from approximately 100 EDs was analyzed, and 1442 cases were included in final analysis. Injuries were sorted into three primary categories: unspecified closed head injury (e.g., closed head injury, blunt head trauma, or traumatic brain injury), concussion, or open head injury and skull fracture. Unspecified closed head injuries were the most common of all head injuries (95.4%, 1376/1442). Open head injuries (14/1442, 0.97%) and concussions 3.6% (52/1442, 3.6%) were rare. Most injuries involved children under the age of 1 (42.6%) compared to children who were 1, 2, 3, or 4-years old. About a fourth of patients had other diagnoses in addition to their primary injury including scalp/forehead hematomas, emesis, and contusions. Female patients were more likely to present with other diagnoses in addition to their primary head injury (Difference: 12.3%, 95% CI: 9.87%-15.4%, p < .0001). CONCLUSION: Despite minimum rail height requirements set by the Consumer Safety Product Commission (CPSC), head injuries associated with crib falls are prevalent in the United States. However, most injuries were minor with a vast majority of patients being released following examination and treatment.

Postnatal Myelomeningocele Repair in the United States: Rates and Disparities Before and After the Management of Myelomeningocele Study Trial.

BACKGROUND AND OBJECTIVES: Evolving technologies have influenced the practice of myelomeningocele repair (MMCr), including mandatory folic acid fortification, advances in prenatal diagnosis, and the 2011 Management of Myelomeningocele Study (MOMS) trial demonstrating benefits of fetal over postnatal MMCr in select individuals. Postnatal MMCr continues to be performed, especially for those with limitations in prenatal diagnosis, health care access, anatomy, or personal preference. A comprehensive, updated national perspective on the trajectory of postnatal MMCr volumes and patient disparities is absent. We characterize national trends in postnatal MMCr rates before and after the MOMS trial publication (2000-2010 vs 2011-2019) and examine whether historical disparities persist. METHODS: This retrospective, cross-sectional analysis queried Nationwide Inpatient Sample data for postnatal MMCr admissions. Annual and race/ethnicity-specific rates were calculated using national birth registry data. Time series analysis assessed for trends relative to the year 2011. Patient, admission, and outcome characteristics were compared between pre-MOMS and post-MOMS cohorts. RESULTS: Between 2000 and 2019, 12 426 postnatal MMCr operations were estimated nationwide. After 2011, there was a gradual, incremental decline in the annual rate of postnatal MMCr. Post-MOMS admissions were increasingly associated with Medicaid insurance and the lowest income quartiles, as well as increased risk indices, length of stay, and hospital charges. By 2019, race/ethnicity-adjusted rates seemed to converge. The mortality rate remained low in both eras, and there was a lower rate of same-admission shunting post-MOMS. CONCLUSION: National rates of postnatal MMCr gradually declined in the post-MOMS era. Medicaid and low-income patients comprise an increasing majority of MMCr patients post-MOMS, whereas historical race/ethnicity-specific disparities are improving. Now more than ever, we must address disparities in the care of MMC patients before and after birth.

Cerebrospinal fluid transcripts may predict shunt surgery responses in normal pressure hydrocephalus.

Molecular biomarkers for neurodegenerative diseases are critical for advancing diagnosis and therapy. Normal pressure hydrocephalus (NPH) is a neurological disorder characterized by progressive neurodegeneration, gait impairment, urinary incontinence and cognitive decline. In contrast to most other neurodegenerative disorders, NPH symptoms can be improved by the placement of a ventricular shunt that drains excess CSF. A major challenge in NPH management is the identification of patients who benefit from shunt surgery. Here, we perform genome-wide RNA sequencing of extracellular vesicles in CSF of 42 NPH patients, and we identify genes and pathways whose expression levels correlate with gait, urinary or cognitive symptom improvement after shunt surgery. We describe a machine learning algorithm trained on these gene expression profiles to predict shunt surgery response with high accuracy. The transcriptomic signatures we identified may have important implications for improving NPH diagnosis and treatment and for understanding disease aetiology.

Pediatric all-terrain vehicle (ATV) related head injury rates and patterns: A 10-year nationwide analysis.

INTRODUCTION: The use of all-terrain vehicles (ATVs) carries significant risk of permanent injury and death, disproportionately affecting children. These injuries commonly affect the head and are especially severe among children as they are often unhelmeted and more likely than adults to experience rollover injuries. Many studies examining patients with ATV-related injuries are single-center cohort studies, with few focusing specifically on head injuries. In the present study, we aimed to characterize the annual incidence of ATV-related head injuries between 2012 and 2021, classify and compare head injury types, and identify descriptive characteristics of ATV-related head injury victims. METHODS: Using the US Consumer Product Safety Commission's National Electronic Injury Surveillance System (NEISS) database, we queried all head injuries associated with operating or riding an ATV in children under 18 years-old from over 100 emergency departments (EDs). Patient information regarding age, race, sex, location of incident, diagnoses, and sequelae were analyzed. We also collected the estimated number of ATV-related head injuries from all US EDs using the NEISS algorithm provided by the database. RESULTS: Using the NEISS algorithm we identified 67,957 (95% CI: 43,608 - 92,305) total pediatric ATV-related head injuries between 2012 and 2021. The annual incidence of ATV-related head injury was similar throughout this study period except for a 20% increase during the COVID-19 pandemic period of 2019-2021 (2019: 6382 injuries, 2020: 6757 injuries, 2021: 7600 injuries). A subset of 1890 cases from approximately 100 EDs were then analyzed. Unspecified closed head injuries were the prevailing type of injury (38%, 900/1890), followed by concussions (27%, 510/1890). More severe injuries included intracranial hemorrhages in 91 children (3.8%, 91/1890). Injuries of all types were predominantly seen in 14-17 year-old's (780/1890, 41%) and in males (64.1%, 1211/1890). In addition, ATV-associated injuries were significantly more common in those coded as white (58.0%, 1096/1890) than any other racial group. ATV-associated accidents among children younger than 9 more commonly occurred at the home compared to accidents involving children older than 9 (57% vs. 32%, p < 0.0001). CONCLUSION: ATV-related head injuries cause a significant annual burden among children, with growing incidence in recent years. Further research may wish to explore potential benefits of helmet use and supervision of younger children in possible prevention of these accidents and their associated economic and non-economic costs.

Antibiotic prophylaxis in penetrating traumatic brain injury: analysis of a single-center series and systematic review of the literature.

PURPOSE: Penetrating traumatic brain injury (pTBI) is an acute medical emergency with a high rate of mortality. Patients with survivable injuries face a risk of infection stemming from foreign body transgression into the central nervous system (CNS). There is controversy regarding the utility of antimicrobial prophylaxis in managing such patients, and if so, which antimicrobial agent(s) to use. METHODS: We reviewed patients with pTBI at our institution and performed a PRISMA systematic review to assess the impact of prophylactic antibiotics on reducing risk of CNS infection. RESULTS: We identified 21 local patients and 327 cases in the literature. In our local series, 17 local patients received prophylactic antibiotics; four did not. Overall, five of these patients (24%) developed a CNS infection (four and one case of intraparenchymal brain abscess and meningitis, respectively). All four patients who did not receive prophylactic antibiotics developed an infection (three with CNS infections; one superficial wound infection) compared to two of 17 (12%) patients who did receive prophylactic antibiotics. Of the 327 pTBI cases reported in the literature, 216 (66%) received prophylactic antibiotics. Thirty-eight (17%) patients who received antibiotics developed a CNS infection compared to 21 (19%) who did not receive antibiotics (p = 0.76). CONCLUSIONS: Although our review of the literature did not reveal any benefit, our institutional series suggested that patients with pTBI may benefit from prophylactic antibiotics. We propose a short antibiotic course with a regimen specific to cases with and without the presence of organic debris.

Seizure freedom after laser amygdalohippocampotomy guided by bilateral responsive neurostimulation in pediatric epilepsy: illustrative case.

BACKGROUND: For patients with difficult-to-lateralize temporal lobe epilepsy, the use of chronic recordings as a diagnostic tool to inform subsequent surgical therapy is an emerging paradigm that has been reported in adults but not in children. OBSERVATIONS: The authors reported the case of a 15-year-old girl with pharmacoresistant temporal lobe epilepsy who was found to have bitemporal epilepsy during a stereoelectroencephalography (sEEG) admission. She underwent placement of a responsive neurostimulator system with bilateral hippocampal depth electrodes. However, over many months, her responsive neurostimulation (RNS) recordings revealed that her typical, chronic seizures were right-sided only. This finding led to a subsequent right-sided laser amygdalohippocampotomy, resulting in seizure freedom. LESSONS: In this case, RNS chronic recording provided real-world data that enabled more precise seizure localization than inpatient sEEG data, informing surgical decision-making that led to seizure freedom. The use of RNS chronic recordings as a diagnostic adjunct to seizure localization procedures and laser ablation therapies in children is an area with potential for future study.

Post-traumatic hydrocephalus may be associated with autologous cranioplasty failure, independent of ventriculoperitoneal shunt placement: a retrospective analysis.

PURPOSE: Decompressive craniectomy (DC) is a common procedure used for the treatment of intracranial hypertension. Once brain swelling has subsided, a cranioplasty is performed to restore cosmesis and protection to the brain. While using the patient's autologous bone flap is often the first choice in cranioplasty, this procedure is frequently complicated by bone flap resorption and infection. This study seeks to identify predictors of autologous cranioplasty failure. METHODS: A retrospective analysis was conducted on patients who underwent decompressive craniectomy and autologous cranioplasty. Patient demographics and factors related to both surgeries and failure rates were recorded from patient records. Logistic regressions were conducted to determine which factors were implicated in autologous cranioplasty failure. RESULTS: In our cohort, 127 patients underwent autologous cranioplasty. Overall, 18 (14.2%) patients experienced autologous cranioplasty failure. Regression analysis identified development of post-traumatic hydrocephalus (PTH) following DC (OR: 3.26, p = 0.043), presence of neurological deficits following DC (OR: 4.88, p = 0.025), and reoperation prior to CP (OR 3.0, p = 0.049) as significant predictors of autologous cranioplasty failure. Of the 16 patients who developed PTH following DC, 9 received a VP shunt. The rate of flap failure was similar across the 9 PTH patients who received a shunt and the 7 PTH patients who did not receive a shunt (33% vs. 57% failure rate, respectively, p = 0.341). CONCLUSION: Autologous cranioplasty is a reasonably successful procedure with a flap failure rate of 14.2%. We identified PTH, persistent neurological deficits, and reoperation prior to cranioplasty as significant predictors of autologous cranioplasty failure. Interestingly, the presence of VP shunt did not impact the odds of flap failure.

Predicting the Impact of Race and Socioeconomic Status on Cranioplasty Materials and Outcomes.

BACKGROUND: Cranioplasty can be performed with either autologous or synthetic materials. No clear consensus has been reached regarding which material is superior and which social factors might influence the choice of treatment modality and the outcomes. We investigated the effects of race and socioeconomic status on the implant choice and outcomes. METHODS: We conducted a cross-sectional study of adult cranioplasty patients from 2002 to 2017 using the National Inpatient Sample. Autologous implant use, admission costs, length of stay (LOS), and home discharge were analyzed by race, income quartile, and insurance type using multivariable regression models. RESULTS: A total of 105,714 admissions met the inclusion criteria. Most of the patients were women (50.3%) and White (73.7%). Black, other race, and Medicaid patients had greater odds of autologous graft usage, and patients in the 76th-100th income quartile had lower usage. Higher income patients (51th-75th and 76th-100th) had had significantly higher hospitalization costs, amounting to a $1547.2 increase, compared with the bottom quartile. The Medicaid patients had a longer LOS (odds ratio, 1.06; P = 0.008) and decreased rates of home discharge (odds ratio, 0.41; P < 0.001). CONCLUSIONS: We found increased autologous cranioplasty usage for non-White and Medicaid patients and increased use of higher cost synthetic implants for high-income patients. Non-White patients and those from higher income quartiles incurred higher admission costs. Medicaid patients had a longer LOS and decreased rates of home discharge.

Evidence of Linear Bone Flap Resorption in Patients Undergoing Autologous Cranioplasty Following Decompressive Craniectomy: A 3D Slicer Segmented Analysis of Serial Computed Tomography Images.

OBJECTIVE: Autologous cranioplasty (CP) following decompressive craniectomy (DC) carries a risk of bone flap resorption (BFR). The current literature offers limited information regarding the natural progression of BFR and the rate at which it occurs. We aim to characterize the progression of BFR over time and elucidate risk factors for accelerated BFR. METHODS: A retrospective analysis was conducted on patients who underwent DC and autologous CP. Serial computed tomography (CT) images were used to quantify the degree of BFR over time. Risk factors included age, diabetes, smoking status, flap fragmentation, defect size, and DC-CP time interval. χ2 analyses and Student's t-tests were performed to examine differences between patients who experienced BFR and those who did not. RESULTS: Overall, 82% of patients demonstrated evidence of clinically relevant resorption on CT. On average, the bone flap decreased in volume by 36.7% within the first year, with a linear loss in volume after multiple years of follow-up. Individuals who developed greater BFR were significantly younger (43 ± 17 vs. 56 ± 12, P = 0.022), had a lower incidence of diabetes (5.9% vs. 43%, P = 0.037), and had more bone flap fragments (1.4 ± 0.67 vs. 1.00 ± 0, P < 0.001) than those who did not. CONCLUSIONS: Resorption following CP with cryopreserved bone appears to progress in a fairly linear and continuous fashion over time. Using serial CT images, we found a resorption rate of 82% at our institution. We identified several possible risk factors for resorption, including flap fragmentation, younger age, and absence of diabetes.

Subgaleal drains may be associated with decreased infection following autologous cranioplasty: a retrospective analysis.

BACKGROUND: Autologous bone is often the first choice in cranioplasty following a decompressive craniectomy. However, infection is a common complication, with reported rates up to 25%. While the incidence and management of infection are well documented, the risk factors associated with infection remain less clear. The current study aims to identify predictors of infection risk following autologous cranioplasty. METHODS: A retrospective analysis was conducted on patients who underwent decompressive craniectomy and cranioplasty using cryopreserved autologous bone flaps between 2010 and 2020. Patient demographics and factors related to both surgeries and infection rates were recorded from patient records. Logistic regressions were conducted to determine which factors were implicated in the development of infection. RESULTS: In our cohort, 126 patients underwent autologous cranioplasty. A total of 10 patients (7.9%) developed an infection following reconstruction, with half resulting in implant failure. We did not identify any significant risk factors for infection. Regression analysis identified placement of subgaleal drain following cranioplasty as a protective factor against the development of infection (OR: 0.16, p = 0.007). On average, drains remained in for 3 days, with no difference between the length of drains for those with infection vs. those without (p = 0.757). CONCLUSIONS: The current study demonstrates an infection rate of 7.9% in patients who receive an autologous cranioplasty following decompressive craniectomy, which is consistent with previous data. Half (4%) of patients who experienced an infection ultimately required removal of the implant. While it is common practice for neurosurgeons to use drains to prevent hematomas and fluid collections, we found that subgaleal drain placement following cranioplasty was associated with decreased infection, thus demonstrating another benefit of a commonly used tool.

International survey on the management of lumbosacral cutaneous stigmata in infants with suspected occult spinal dysraphism.

OBJECTIVE: Occult spinal dysraphism (OSD) is a common pediatric neurosurgical diagnosis rife with controversy surrounding both the screening of asymptomatic infants and the threshold to offer a prophylactic detethering operation. The authors sought to clarify international practice patterns with a survey of pediatric neurosurgeons. METHODS: A survey asked pediatric neurosurgeons whether they would perform imaging in patients with a variety of cutaneous stigmata associated with OSD and whether they would offer prophylactic detethering surgery for asymptomatic patients with a variety of imaging findings on the OSD spectrum. RESULTS: Completed surveys were received from 141 pediatric neurosurgeons. Broad consensus was demonstrated on the need for obtaining images in sample patients with more severe stigmata ranging from large lipoma with a skin appendage to focal dysplastic skin in the lumbar midline. Ninety percent of respondents would perform MRI for these patients. In contrast, for patients with a low-sacral dimple, flat hemangioma, and symmetric (Y-shaped) splaying of the intergluteal cleft, opinion on the need for imaging varied considerably (between 57% and 89% recommended imaging). Respondents differed on the type of imaging that they would perform, with 31% to 38% recommending ultrasound screening. The responses reflected less consensus on when to offer surgery to patients with simple spinal tethering (low-lying conus medullaris and fatty filum terminale). Both a lower level of the conus and increased thickness of the filum terminale affected decision-making. CONCLUSIONS: The results of this survey showed significant consensus on the recommendation for screening imaging in patients with more dramatic cutaneous stigmata, although these stigmata are the rarest. A significant variance in opinions was reflected in the recommendation for imaging of the most common cutaneous stigmata. Consensus was also lacking on which lesions deserve prophylactic detethering surgery. Significant equipoise exists for future study of screening imaging and of surgical decision-making in patients with asymptomatic OSD and associated cutaneous stigmata.

In-depth bioinformatic study of the cadherin 5 interactome in patients with thoracic aortic aneurysm unveils 8 novel biomarkers.

OBJECTIVES: Thoracic aortic aneurysm (TAA) is characterized by the dilation of the aorta and is associated with poor prognosis if not diagnosed and treated early. In this context, the identification of biomarkers regarding the TAA diagnosis, monitoring and prognosis is crucial. The purpose of the current study was to investigate the differential gene expression profile of the cadherin 5 (CDH5 or VE-Cadherin) gene network in patients with TAA, to propose novel biomarkers. METHODS: In silico techniques were used to construct the interactome of the CDH5 network, identify the differentially expressed genes (DEGs) in TAA as compared to healthy controls, and uncover the related molecular functions and the regulating miRNAs. RESULTS: Transcriptomic data of one microarray dataset were included, incorporating 43 TAA and 43 control samples. Eight DEGs were identified; 7 were up-regulated and 1 was down-regulated. A molecular signature of 8 genes (CDH5; Calcitonin Receptor-Like Receptor-CALCRL; Activin A Receptor-Like Type 1-ACVRL1, Tryptophanyl-TRNA Synthetase 1-WARS; Junction Plakoglobin-JUP, Protein Tyrosine Phosphatase Receptor Type J-PTPRJ, Purinergic Receptor P2X 4-P2RX4, Kinase Insert Domain Receptor-KDR) were identified as biomarkers associated with TAA. PTPRJ was associated with excellent discrimination and calibration in predicting TAA presentation. Positive correlations were reported regarding the expression of CDH5-CALCRL, CDH5-ACVRL1, CDH5-WARS and CDH5-PTPRJ. Finally, gene set enrichment analysis indicated the molecular functions and miRNA families (hsa-miR-296-5p, hsa-miR-6836-5p, hsa-miR-6132, hsa-miR-27a-5p and hsa-miR-6773-5p) relevant to the 8 biomarkers. CONCLUSIONS: These outcomes propose an 8-gene molecular panel associated with TAA.

Reappraisal of Pediatric Normal-Pressure Hydrocephalus.

While normal-pressure hydrocephalus (NPH) is most commonly diagnosed in older adulthood, a significant body of literature has accumulated over half a century documenting the clinical phenomenon of an NPH-like syndrome in pediatric patients. As in adult NPH, it is likely that pediatric NPH occurs due to a heterogeneous array of developmental, structural, and neurodegenerative pathologies, ultimately resulting in aberrant cerebrospinal fluid (CSF) flow and distribution within and around the brain. In this review, we aimed to systematically survey the existing clinical evidence supporting the existence of a pediatric form of NPH, dating back to the original recognition of NPH as a clinically significant subtype of communicating hydrocephalus. Leveraging emergent trends from the old and more recent published literature, we then present a modern characterization of pediatric NPH as a disorder firmly within the same disease spectrum as adult NPH, likely with overlapping etiology and pathophysiological mechanisms. Exemplary cases consistent with the diagnosis of pediatric NPH selected from the senior author's neurosurgical practice are then presented alongside the systematic review to aid in discussion of the typical clinical and radiographic manifestations of pediatric NPH. Common co-morbidities and modern surgical treatment options are also described.

Postnatal Sagittal Craniosynostosis: A Novel Presentation and Considerations in Diagnosis and Management.

ABSTRACT: Sagittal craniosynostosis results in scaphocephaly from abnormal fusion of the sagittal suture. Traditionally, craniosynostosis presents at birth and is diagnosed within the first year of life. The authors report a patient with development of sagittal craniosynostosis after birth, which we term postnatal sagittal craniosynostosis. This is a rare occurrence in which management considerations are critical but are not well discussed. A 3-year-old boy presented with concerns of a metopic ridge. Workup revealed metopic ridging and an open sagittal suture. The patient later developed signs of increased intracranial pressure and repeat computed tomography scan 14 months later identified a newly fused sagittal suture. The patient underwent open posterior cranial vault expansion, resulting in resolution of symptoms. Postnatal sagittal craniosynostosis is a rare condition and should be considered in otherwise unexplained increases in increased intracranial pressure among pediatric patients. Open posterior cranial vault expansion represents a safe and effective method to treat this condition.

Successful Anesthesia Management of Postoperative Maternal Pulmonary Edema and Uterine Hyperactivity following Open Fetal Myelomeningocele Repair.

Effective tocolysis is essential after fetal myelomeningocele repair and is associated with the development of pulmonary edema. The increased uterine activity in the immediate postoperative period is commonly treated with magnesium sulfate. However, other tocolytic agents such as nitroglycerine, nifedipine, indomethacin, terbutaline, and atosiban (outside the US) have also been used to combat uterine contractility. The ideal tocolytic regimen which balances the risks and benefits of in-utero surgery has yet to be determined. In this case report, we describe a unique case of fetal myelomeningocele repair complicated by maternal pulmonary edema and increased uterine activity resistant to magnesium sulfate therapy.