The role of traditional social determinants of health in referral patterns, timing of surgery, and Chiari Health Index for Pediatrics-determined quality of life in children with symptomatic Chiari type I malformation.

OBJECTIVE: Understanding the impact of the social determinants of health on the utilization of healthcare resources is an important step in eliminating inequalities. The goal of this study was to determine the role of social determinants of health in referral patterns, timing of consultation/intervention, and quality of life in children with Chiari malformation type I (CM-I). METHODS: A retrospective study was conducted of children aged 0 to 18 years who underwent surgical treatment for CM-I at a single pediatric facility from 2015 to 2019. The variables included demographic and socioeconomic characteristics, referral patterns, timing, and quality of life data based on the Chiari Health Index for Pediatrics (CHIP). RESULTS: The cohort consisted of 103 surgically treated CM-I patients. No differences were seen in race, sex, insurance, or household income when evaluating referral source (community, specialist, or emergency department) or when comparing patients with incidental versus symptomatic findings. In the evaluation of timing from initial evaluation to surgery, no statistical differences were seen between racial, sex, insurance status, or income groups. Children from households of lower median family income were significantly more likely to report pain at the time of consultation (pain group median [interquartile range] $46,660 [$41,004-$50,367] vs nonpain group $53,604 [$41,427-$59,828], p = 0.004). Those in the lower-income group also reported lower CHIP scores corresponding to increased symptomatology in the nonpain physical symptoms (p = 0.004) and psychosocial domains (p = 0.018). CONCLUSIONS: There was no evidence of a difference in referral patterns or a delay in time from clinic presentation to surgery based on the traditional social determinants of health categories. Children from households in the lower-income group were associated with increased severity of pain and nonpain symptoms.

Treatment of hydrocephalus following posterior fossa tumor resection: a multicenter collaboration from the Hydrocephalus Clinical Research Network.

OBJECTIVE: Persistent hydrocephalus following posterior fossa brain tumor (PFBT) resection is a common cause of morbidity in pediatric brain tumor patients, for which the optimal treatment is debated. The purpose of this study was to compare treatment outcomes between VPS and ETV in patients with persistent hydrocephalus following surgical resection of a PFBT. METHODS: A post-hoc analysis was performed of the Hydrocephalus Clinical Research Network (HCRN) prospective observational study evaluating VPS and ETV for pediatric patients. Children who experienced hydrocephalus secondary to PFBT from 2008 to 2021 were included. Primary outcomes were VPS/ETV treatment failure and time-to-failure (TTF). RESULTS: Among 241 patients, the VPS (183) and ETV (58) groups were similar in age, extent of tumor resection, and preoperative ETV Success Score. There was no difference in overall treatment failure between VPS and ETV (33.9% vs 31.0%, p = 0.751). However, mean TTF was shorter for ETV than VPS (0.45 years vs 1.30 years, p = 0.001). While major complication profiles were similar, compared to VPS, ETV patients had relatively higher incidence of minor CSF leak (10.3% vs. 1.1%, p = 0.003) and pseudomeningocele (12.1% vs 3.3%, p = 0.02). No ETV failures were identified beyond 3 years, while shunt failures occurred beyond 5 years. Shunt infections occurred in 5.5% of the VPS cohort. CONCLUSIONS: ETV and VPS offer similar overall success rates for PFBT-related postoperative hydrocephalus. ETV failure occurs earlier, while susceptibility to VPS failure persists beyond 5 years. Tumor histology and grade may be considered when selecting the optimal means of CSF diversion.

The Nomenclature of Chiari Malformations.

The current nomenclature of Chiari malformations includes the standard designations, Chiari 1-4, which were described by Hans Chiari in the late nineteenth century, and more recent additions, Chiari 0, 0.5, and 1.5, which emerged when the standard nomenclature failed to include important anatomical variations. The authors describe these entities and propose that to best optimize clinical care and research, it would be wise to place less focus on the eponyms and more effort on developing a descriptive or pathophysiological nomenclature.

A comparison of MRI appearance and surgical detethering rates between intrauterine and postnatal myelomeningocele closures: a single-center pilot matched cohort study.

INTRODUCTION: Intrauterine myelomeningocele repair (IUMR) and postnatal myelomeningocele repair (PNMR) differ in terms of both setting and surgical technique. A simplified technique in IUMR, in which a dural onlay is used followed by skin closure, has been adopted at our institution. The goal of this study was to compare the rates of clinical tethering in IUMR and PNMR patients, as well as to evaluate the appearance on MRI. METHODS: We conducted a retrospective review of 36 patients with MMC repaired at our institution, with 2:1 PNMR to IUMR matching based on lesion level. A pediatric neuroradiologist blinded to the clinical details reviewed the patients' lumbar spine MRIs for the distance from neural tissue to skin and the presence or absence of a syrinx. An EMR review was then done to evaluate for detethering procedures and need for CSF diversion. RESULTS: Mean age at MRI was 4.0 years and mean age at last follow-up was 6.1 years, with no significant difference between the PNMR and IUMR groups. There was no significant difference between groups in the distance from neural tissue to skin (PNMR 13.5 mm vs IUMR 17.6 mm; p = 0.5). There was no difference in need for detethering operations between groups (PNMR 12.5% vs IUMR 16.7%; RR 0.75; CI 0.1-5.1). CONCLUSIONS: There was no significant difference between postnatal- and intrauterine-repaired myelomeningocele on MRI or in need for detethering operations. These results imply that a more straightforward and time-efficient IUMR closure technique does not lead to an increased rate of tethering when compared to the multilayered PNMR.

Pediatric spinal injury patterns and management in all-terrain vehicle and dirt bike crashes, 2010-2019.

OBJECTIVE: Pediatric spinal injuries in all-terrain vehicle (ATV) and dirt bike crashes are relatively uncommon but may be associated with significant morbidity. There are no recent studies examining these injuries, their management, and outcomes. Therefore, a retrospective study was performed to characterize pediatric spinal injuries related to ATV and dirt bike crashes over the last decade. METHODS: Data on all patients involved in ATV or dirt bike crashes evaluated at a regional level 1 pediatric trauma center over a 10-year period (2010-2019) were analyzed. Descriptive statistics were analyzed and chi-square, Fisher exact, and Mann-Whitney U-tests were performed comparing the demographics, injury characteristics, and clinical outcomes in patients with versus those without spinal injuries. RESULTS: Of 680 patients evaluated, 35 (5.1%) were diagnosed with spinal injuries. Over the study period, both spinal injuries and emergency department visits related to ATV or dirt bike crashes increased in frequency. All spinal injuries were initially diagnosed on CT scans, and 57.9% underwent spinal MRI. Injuries were most commonly thoracic (50%), followed by cervical (36.8%). The injuries of most patients were classified as American Spinal Injury Association (ASIA) grade E on presentation (86.8%), while 2 (5.3%) had complete spinal cord injuries (ASIA grade A) and 3 patients (8.6%) were ASIA grade B-D. Operative management was required for 13 patients (28.9%). Nonoperative management was used in 71.1% of injuries, including bracing in 33% of all injuries. Patients with spinal injuries were older than those without (13.4 ± 3.35 vs 11.5 ± 3.79 years, p = 0.003). Spinal injuries occurred via similar crash mechanisms (p = 0.48) and in similar locations (p = 0.29) to nonspinal injuries. Patients with spinal injuries more frequently required admission to the intensive care unit (ICU; 34.2% vs 14.6%, p = 0.011) and had longer hospital stays (mean 4.7 ± 5.5 vs 2.7 ± 4.0 days, p = 0.0025). CONCLUSIONS: Although infrequent among young ATV and dirt bike riders, spinal injuries are associated with longer hospital stays, increased ICU use, and required operative intervention in 29%. Increasing awareness among ATV and dirt bike riders about the severity of riding-related injuries may encourage safer riding behaviors.

Relationship between Anterior Pituitary Volume and IGF-1 Serum Levels in Soldiers with Mild Traumatic Brain Injury History.

PURPOSE: A high mild traumatic brain injury (mTBI) incidence rate exists in military and sport. Hypopituitarism is an mTBI sequela; however, few studies have examined this phenomenon in those with an mTBI history. This cross-sectional study of Special Operations Forces combat soldiers aimed 1) to relate anterior pituitary gland volumes (actual and normalized) to insulin-like growth factor 1 (IGF-1) concentrations, 2) to examine the effect of mTBI history on anterior pituitary gland volumes (actual and normalized) and IGF-1 concentrations, and 3) to measure the odds of demonstrating lower anterior pituitary gland volumes (actual and normalized) or IGF-1 concentrations if self-reporting mTBI history. METHODS: Anterior pituitary gland volumes were manually segmented from T1-weighted 3D brain MRI sequences; IGF-1 serum concentrations were quantified using commercial enzyme-linked immunosorbent assays. Correlations and linear regression were used to determine the association between IGF-1 serum concentration and anterior pituitary gland volume (n = 74). Independent samples t-tests were used to compare outcomes between mTBI groups and logistic regression models were fit to test the odds of demonstrating IGF-1 concentration or anterior pituitary volume less than sample median based on mTBI group (n = 54). RESULTS: A significant linear relationship between the subjects' anterior pituitary gland volumes and IGF-1 concentrations (r72 = 0.35, P = 0.002) was observed. Soldiers with mTBI history had lower IGF-1 concentrations (P < 0.001) and lower anterior pituitary gland volumes (P = 0.037) and were at greater odds for IGF-1 serum concentrations less than the sample median (odds ratio = 5.73; 95% confidence interval = 1.77-18.55). CONCLUSIONS: Anterior pituitary gland volume was associated with IGF-1 serum concentrations. Mild TBI history may be adversely associated with anterior pituitary gland volumes and IGF-1 concentrations. Longitudinal IGF-1 and anterior pituitary gland monitoring may be indicated in those who report one or more mTBI.

Ventriculoperitoneal shunt management in children undergoing liver transplantation.

There is a lack of data to guide neurosurgeons on the management of ventriculoperitoneal shunts (VPS) in patients undergoing abdominal transplant operations. We present the cases of two pediatric patients with VPS undergoing liver transplantation who were successfully managed with externalization of the VPS at time of transplantation, with subsequent re-internalization once cleared by the transplant surgery team. We present this as an effective management strategy in patients undergoing liver transplantation.

Sports Medicine Fellowship Training Improves Sport-related Concussion Evaluation.

Evaluating acute sport-related concussion (SRC) differs among providers, who apply international consensus and national guidelines variably. Retrospective medical record review was completed for 889 outpatients aged 7 to 18 years, diagnosed with SRC from 2014 to 2017. Associations between board-certified sports medicine fellowship (SMF) training status and assessment of concussion-specific history and physical examination items were tested using nonparametric χ tests. Differential odds (by fellowship training status) of using history and physical examination items were assessed using multivariable logistic regression modeling. Each SRC history and physical examination items were assessed in higher proportions by SMFs, as compared with non-SMFs, statistically significant for all tested items with the exception of headache history. SMFs demonstrated higher odds of detailed neurologic examinations and clinical cognitive testing compared with non-SMFs, after adjusting for patient and physician characteristics. Physicians caring for concussed patients should seek continuing education opportunities aligning them with training provided to sports medicine fellows.

Neonatal Hydrocephalus Treatment with Ultrasmall Valve Implantation.

OBJECTIVE: Neonatal hydrocephalus remains a difficult condition to manage, due to high failure rates among all management strategies. Neurosurgeons commonly manage hydrocephalus with ventriculoperitoneal shunt (VPS) implantation, and valves of variable sizes and profiles are available for implantation. This study examines primary ventricular shunt valve implantation complication rates based on valve profiles in pediatric patients with hydrocephalus. METHODS: This study retrospectively reviews pediatric patients younger than 1 year of age who underwent ventricular shunt placement at a single institution from January 2001 to January 2017. Patients were classified by valve profile and categorized as either ultrasmall valves or regular-sized valves. Time until complication and type of complication were studied. RESULTS: A total of 156 patients met the inclusion criteria. Forty-eight (31%) patients received an ultrasmall shunt valve, while 108 patients received a regular valve. On average, patients undergoing ultrasmall valve placement were younger (2.1 months) than patients undergoing placement of regular valves (3.1 months) (P = 0.03). The overall complication rate within 2 years of VPS placement was 37.5% in patients with the ultrasmall valve and 41.7% in the regular valve population. There was no difference in 1-year shunt survival rate between the 2 cohorts. CONCLUSION: Our review did not find a significant difference in complication rates between ultrasmall and regular valves in patients under 1 year of age. However, the etiology of shunt malfunction did differ between the groups. This work further supports evidence suggesting a surgeon's preference for shunt hardware alone does not significantly impact outcome.

Facial nerve palsy following mild mastoid trauma on trampoline.

We present a case report of a 14-year-old boy who was jumping on a trampoline when he struck his right mastoid on a support pole. The following day, he developed a right-sided facial droop and inability to close his right eye. He presented to the emergency department, where CT of his temporal bone was negative and he was started on prednisone. Over the next month, he had spontaneous recovery of his facial nerve (FN) function. In cases of traumatic FN palsy, urgent referral to otolaryngology is needed, even without a fracture of the temporal bone, as edema within the facial nerve could require decompressive surgery. Steroids, while used in this patient, are of questionable benefit in the limited data available. Patient's with traumatic FN palsies should be instructed to use eye lubricant frequently and tape his eye closed at night, as corneal drying could lead to permanent damage. Proper evaluation, management, and referral are needed in cases of traumatic FN palsy to prevent long-term morbidity.

Chiari decompression outcomes using ligamentum nuchae harvest and duraplasty in pediatric patients with Chiari malformation type I.

OBJECTIVE The choice of graft material for duraplasty in decompressions of Chiari malformations remains a matter of debate. The authors present a detailed technique for harvesting ligamenta nuchae, as well as the clinical and radiographic outcomes of this technique, in a case series. METHODS The authors conducted a retrospective study evaluating the outcomes of Chiari malformation type I decompression and duraplasty in children aged 0-18 years at a single institution from 2013 to 2016. They collected both intraoperative and postoperative variables and compared them qualitatively to published data. RESULTS During the study period, the authors performed 25 Chiari malformation decompressions with ligamentum nuchae graft duraplasties. Of the 25 patients, 10 were females, and the mean age at surgery was 8.6 years (range 13 months to 18 years). The median operative time was 163 minutes (IQR 152-187 minutes), with approximately 10 minutes needed by a resident surgeon to harvest the graft. The mean length of stay was 3 nights (range 2-6 nights), and the mean follow-up was 12.6 months (range 0.5-43.5 months). One patient (4%) developed a CSF leak that was repaired using an oversewing patch. There were no postoperative pseudomeningoceles or infections. Of the 19 patients presenting with a syrinx, imaging showed improvement in 10 (53%) and 8 (42%) had stable syrinx size on imaging. Of 16 patients presenting with a symptomatic Chiari malformation, 14 (87.5%) experienced resolution of symptoms and in 1 (4%) symptoms remained the same. One patient (4%) presented with worsening syrinx and symptoms 1.5 months after initial surgery and underwent repeat decompression. CONCLUSIONS The authors describe a series of clinical and imaging outcomes of patients who underwent Chiari malformation decompression and duraplasty with a harvested ligamentum nuchae. The rates of postoperative CSF leak are similar to established techniques of autologous and artificial grafts, with similarly successful outcomes. Further study will be needed with larger patient cohorts to more directly compare duraplasty graft outcomes.

Filum terminale lipomas: imaging prevalence, natural history, and conus position.

OBJECT: Filum terminale lipomas (FTLs) are being identified with increasing frequency due to the increasing utilization of MRI. Although an FTL may be associated with tethered cord syndrome (TCS), in many cases FTLs are diagnosed incidentally in patients without any symptoms of TCS. The natural history of FTLs is not well defined. METHODS: The authors searched the clinical and imaging records at a single institution over a 14-year interval to identify patients with FTLs. For patients with an FTL, the clinical records were reviewed for indication for imaging, presenting symptoms, perceived need for surgery, and clinical outcome. A natural history analysis was performed using all patients with more than 6 months of clinical follow-up. RESULTS: A total of 436 patients with FTL were identified. There were 217 males and 219 females. Of these patients, 282 (65%) were adults and 154 (35%) were children. Symptoms of TCS were present in 22 patients (5%). Fifty-two patients underwent surgery for FTL (12%). Sixty-four patients (15%) had a low-lying conus and 21 (5%) had a syrinx. The natural history analysis included 249 patients with a mean follow-up time of 3.5 years. In the follow-up period, only 1 patient developed new symptoms. CONCLUSIONS: Filum terminale lipomas are a common incidental finding on spinal MRI, and most patients present without associated symptoms. The untreated natural history is generally benign for asymptomatic patients.

Anterior sacral pseudomeningocele following minimal trauma: case report.

Sacral fractures are rare and seldom result in formation of a sacral pseudomeningocele. Treatment of these pseudomeningoceles usually consists of conservative management with flat bedrest or open operative management. The authors describe the case of a 55-year-old woman with an anterior sacral pseudomeningocele that was successfully treated using a lumbar drain for temporary continuous CSF drainage. The patient first presented to an outside institution several days after sacral trauma from an ice skating fall. Initial symptoms included throbbing headaches relieved by lying flat. Head and cervical spine CT demonstrated no abnormality. As symptoms worsened, she presented to another institution where MRI of the lumbar spine indicated sacral fracture with pseudomeningocele. The patient subsequently transferred to the authors' facility, where symptoms included headaches and occasional mild sacral pain. Given her headaches and the authors' concern for CSF leak, another head CT scan was performed. This revealed no subdural hematoma or other abnormality. A subsequent CT myelogram revealed an anterior sacral pseudomeningocele at S3-4 with an anterior irregular linear filling defect, likely representing torn dura. Treatment included placement of a lumbar drain (10 ml/hr) and flat bedrest. Resolution of the CSF leak occurred on postprocedure Day 9. At the 4-week follow-up visit, the patient had no clinical symptoms of CSF leak and no neurological complaints. To our knowledge, this is the first description of temporary continuous CSF drainage used to treat a posttraumatic sacral pseudomeningocele. This technique may reduce the need for potentially complicated surgical repair of sacral fractures associated with CSF leak in select patients.

Role of GSK3 beta in behavioral abnormalities induced by serotonin deficiency.

Dysregulation of brain serotonin (5-HT) neurotransmission is thought to underlie mental conditions as diverse as depression, anxiety disorders, bipolar disorder, autism, and schizophrenia. Despite treatment of these conditions with serotonergic drugs, the molecular mechanisms by which 5-HT is involved in the regulation of aberrant emotional behaviors are poorly understood. Here, we generated knockin mice expressing a mutant form of the brain 5-HT synthesis enzyme, tryptophan hydroxylase 2 (Tph2). This mutant is equivalent to a rare human variant (R441H) identified in few individuals with unipolar major depression. Expression of mutant Tph2 in mice results in markedly reduced ( approximately 80%) brain 5-HT production and leads to behavioral abnormalities in tests assessing 5-HT-mediated emotional states. This reduction in brain 5-HT levels is accompanied by activation of glycogen synthase kinase 3beta (GSK3beta), a signaling molecule modulated by many psychiatric therapeutic agents. Importantly, inactivation of GSK3beta in Tph2 knockin mice, using pharmacological or genetic approaches, alleviates the aberrant behaviors produced by 5-HT deficiency. These findings establish a critical role of Tph2 in the maintenance of brain serotonin homeostasis and identify GSK3beta signaling as an important pathway through which brain 5-HT deficiency induces abnormal behaviors. Targeting GSK3beta and related signaling events may afford therapeutic advantages for the management of certain 5-HT-related psychiatric conditions.