Recommendations for the Management of Initial and Refractory Pediatric Status Dystonicus.

Status dystonicus is the most severe form of dystonia with life-threatening complications if not treated promptly. We present consensus recommendations for the initial management of acutely worsening dystonia (including pre-status dystonicus and status dystonicus), as well as refractory status dystonicus in children. This guideline provides a stepwise approach to assessment, triage, interdisciplinary treatment, and monitoring of status dystonicus. The clinical pathways aim to: (1) facilitate timely recognition/triage of worsening dystonia, (2) standardize supportive and dystonia-directed therapies, (3) provide structure for interdisciplinary cooperation, (4) integrate advances in genomics and neuromodulation, (5) enable multicenter quality improvement and research, and (6) improve outcomes. © 2024 International Parkinson and Movement Disorder Society.

Spinal catheter revision in pediatric intrathecal baclofen pumps: risk factors and postoperative outcomes.

OBJECTIVE: Intrathecal baclofen (ITB) pumps are commonly used in pediatric patients with cerebral palsy (CP) and medically refractory spasticity. However, catheter malfunction and associated risk factors are not well understood. The aim of this study was to examine potential risk factors for spinal catheter malfunction and characterize postoperative follow-up to understand the clinical consequences. METHODS: Patients who received ITB pump replacement or revision at Boston Children's Hospital between 2010 and 2023 were retrospectively reviewed. The spinal catheter revision cohort (SCRC) included patients whose spinal catheter was occluded requiring lumbar catheter revision. The second cohort included abdominal pump replacements only (APRC). Between-group comparisons and multivariable regression identified factors associated with catheter revision and postoperative outcomes. RESULTS: Forty-one (33.6%) patients underwent spinal catheter revision and were compared with 81 patients (66.4%) who underwent abdominal pump replacement only. Younger age at surgery and an elevated preoperative lower-extremity modified Ashworth scale grade were associated with spinal catheter revision (p < 0.05). Catheter model type, tip location, and history of spinal fusion were not associated with obstruction. Postoperatively, SCRC patients experienced a higher rate of infection (17.1%) relative to APRC patients (0%) within 30 days from their ITB pump replacement procedure (p < 0.05) and greater likelihood of subsequent ITB system removal compared with the APRC (24.4% vs 7.4%, p < 0.05). Although not differing preoperatively, SCRC patients had lower postoperative ITB doses when compared with the APRC group (median dose 143 vs 350 µg/day, p < 0.05) at hospital discharge and remained statistically different at the 6-month and 1-year follow-ups (p < 0.05). There were no postoperative differences in baclofen overdose, withdrawal, or median number of hospital readmissions within 30 days. Overall, 31.7% of spinal catheter revisions were unanticipated by the clinical team at time of surgery. CONCLUSIONS: Younger age at surgery and increased preoperative lower-extremity tone may be risk factors for catheter obstruction, resulting in a higher rate of postoperative infection and subsequent ITB pump removal compared with pump replacement alone. Spinal catheter occlusion can complicate revision or replacement procedures, especially when unanticipated. Routine clinical assessment may be inadequate for diagnosing insidious catheter malfunction. Catheter occlusion deserves further study, and routine assessment of catheter patency may be warranted to prevent suboptimal tone therapy.

Sport-Related Concussion Preceding Adrenal Insufficiency and Hypopituitarism.

ABSTRACT: A 49-year-old woman with a history of daily inhaled corticosteroid use for asthma presented to a concussion clinic 7 wk after sport-related head injury with headache, visual blurring, dizziness, nausea, fatigue, polydipsia, and polyuria. Examination revealed difficulty with vestibulo-occulomotor testing due to nausea and visual straining. Cranial CT/MR imaging was unremarkable. Laboratory testing revealed critically low serum cortisol, hypernatremia, and urine studies suggesting diabetes insipidus. The patient was referred to the emergency department. Intravenous fluid resuscitation, corticosteroids, and desmopressin led to significant symptomatic relief. She was maintained on oral hydrocortisone after cosyntropin test revealed adrenal insufficiency. Her clinical picture suggested chronic subclinical adrenal suppression from inhaled corticosteroids, which was exacerbated by hypopituitarism possibly from concussion combined with diabetes insipidus. Adrenal insufficiency should be considered in athletes with history of corticosteroid use and endocrine-related symptoms after concussion, because this can create significant morbidity and can mimic traditional symptoms of concussion.

Targeted head CT reduction for pediatric patients with hydrocephalus and traumatic brain injury: academic center institutional experience as an example of opportunities for further improvement.

PURPOSE: Recent studies aim to reduce radiation exposure associated with computed tomography (CT) using rapid-sequence magnetic resonance imaging (MRI). We evaluated imaging modalities used for hydrocephalus and traumatic brain injury (TBI) to identify opportunities for further radiation exposure reduction. METHODS: Pediatric (≤ 18 years) patients, with either hydrocephalus or TBI receiving a head CT or head MRI from 2009 to 2017, were quantified using ICD9, ICD10, and CPT codes at a large university hospital. The odds ratios of receiving each imaging modality year-to-year and receiving a MRI or CT in a given year with each diagnosis were calculated. RESULTS: Beginning in 2015, hydrocephalus patients were more likely to receive a MRI vs CT (p < 0.0001), with likelihood increasing in the following 2 years. TBI patients were more likely to receive a CT than an MRI from 2009 to 2017. There was a smaller overall decrease in CT scans in TBI patients (p < 0.05) than hydrocephalus patients (p < 0.0001) and a larger increase in MRI use in hydrocephalus patients (p < 0.05) than TBI patients (p < 0.05) from 2009 to 2017. CONCLUSIONS: Use of CT to evaluate hydrocephalus significantly decreased over the last 3-year time period. However, CT use for TBI patients was inconsistent and did not decrease proportionally, potentially representing a belief that CT is more effective than MRI for detecting hemorrhage. There was greater use of MRI as an alternate imaging method in hydrocephalus patients than TBI patients. Head injury remains an area of improvement to decrease pediatric radiation exposure at our institution and may be an area in need of attention more broadly.

Sport-Related Concussion Preceding Adrenal Insufficiency and Hypopituitarism.

A 49-year-old female with history of daily inhaled corticosteroid use for asthma presented to a concussion clinic 7 wk after sport-related head injury with headache, visual blurring, dizziness, nausea, fatigue, polydipsia, and polyuria. Examination revealed difficulty with vestibuloocculomotor testing due to nausea and visual straining. Cranial computed tomography/magnetic resonance imaging was unremarkable. Laboratory testing revealed critically low serum cortisol, hypernatremia, and urine studies suggesting diabetes insipidus. The patient was referred to the emergency department. Intravenous fluid resuscitation, corticosteroids, and desmopressin led to significant symptomatic relief. She was maintained on oral hydrocortisone after cosyntropin test revealed adrenal insufficiency. Her clinical picture suggested chronic subclinical adrenal suppression from inhaled corticosteroids which was exacerbated by hypopituitarism from concussion combined with diabetes insipidus. Adrenal insufficiency should be considered in athletes with history of corticosteroid use and endocrine-related symptoms after concussion, because this can create significant morbidity and can mimic traditional symptoms of concussion.

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.

Thirty-day medical and surgical readmission following prenatal versus postnatal myelomeningocele repair.

OBJECTIVE: Hospital readmission is an important quality metric that has not been evaluated in prenatal versus postnatal myelomeningocele (MMC) repair. This study compares hospital readmission outcomes between these two groups as well as their etiologies. METHODS: The medical records of patients who had undergone MMC repair in the period from 2011 to 2017 at a single academic medical center were retrospectively reviewed. Collected clinical data included surgery and defect details, neonatal intensive care unit (NICU) stay, and any readmissions or surgical procedures up to 1 year after surgery. Patient and defect characteristics, readmission outcomes at 30 and 60 days and 1 year after discharge from the NICU, and cerebrospinal fluid (CSF) diversion surgery rates were analyzed with the two-tailed t-test and/or k-sample test on the equality of medians. RESULTS: A total of 24 prenatal and 34 postnatal MMC repairs were completed during the study period. Prenatally repaired patients were born more prematurely (p < 0.001) and with lower birth weights (p < 0.001), although the NICU stay was similar between the two groups (p = 0.59). Fewer prenatally repaired patients were readmitted at 30 days (p = 0.005), 90 days (p = 0.004), and 1 year (p = 0.007) than the postnatal repair group. Hydrocephalus was the most common readmission etiology, and 29% of prenatal repair patients required CSF diversion at 1 year versus 81% of the postnatal repair group (p < 0.01). Prenatal patients who required CSF diversion had a higher body weight (p = 0.02) and an older age (p = 0.01) at the time of CSF diversion surgery than the postnatal group. CONCLUSIONS: Patients with prenatal MMC repair had fewer hospital readmissions at 30 days, 60 days, and 1 year than the postnatal repair group, despite similar NICU lengths of stay. The prenatal repair group had lower requirements for CSF diversion at 1 year and was older with greater body weights at the time of CSF diversion surgery, compared to those of the postnatal repair group. Future study of hospital quality metrics such as readmissions should be performed to better understand outcomes of these two procedures.

Challenges and opportunities for effective data collection in global neurosurgery: traumatic brain injury surveillance experience in Malawi.

Global health research can transform clinical and surgical practice worldwide. Partnerships between US academic centers and hospitals in low- and middle-income counties can improve clinical care at the host institution hospital and give the visiting institution access to a large volume of valuable research data. Recognizing the value of these partnerships, the University of North Carolina (UNC) formed a partnership with Kamuzu Central Hospital (KCH) in Lilongwe, Malawi. The Department of Neurosurgery joined the partnership with KCH and designed a Head Trauma Surveillance Registry. The success of this registry depended on the development of methods to accurately collect head injury data at KCH. Since medical record documentation is often unreliable in this setting, data collection teams were implemented to capture data from head trauma patients on a 24-hours-a-day, 7-days-a-week basis. As data collection improved, pilot groups tested methods to collect new variables and the registry expanded. UNC provided onsite and remote oversight to strengthen the accuracy of the data. Data accuracy still remains a hurdle in global research. Data collection teams, oversight from UNC, pilot group testing, and meaningful collaboration with local physicians improved the accuracy of the head trauma registry. Overall, these methods helped create a more accurate epidemiological and outcomes-centered analysis of brain injury patients at KCH to date.

Magnetic Resonance Imaging/Angiography Versus Catheter Angiography for Annual Follow-up of Pediatric Moyamoya Patients: A Cost Outcomes Analysis.

BACKGROUND: Digital subtraction angiography (DSA) assesses revascularization in pediatric moyamoya patients after surgery, but MRI and angiography (MRI/A) may provide comparable data. OBJECTIVE: To evaluate DSA and MRI/A with respect to clinical utility in postoperative follow-up, complication profile, and relative cost at 1 year. METHODS: All pediatric moyamoya patients who received bilateral indirect revascularization between 2011 and 2020 were retrospectively reviewed at 1 institution. Patients who underwent MRI/A-only, DSA-only, or both after 1 year were compared. RESULTS: Eighty-two patients were included. At 1 year, patients who underwent either MRI/A (n = 29) or DSA (n = 40) had no significant differences in detection rate of new at-risk hypovascular territories (6.9% vs 2.5%, P = .568) or need for subsequent revascularization beyond the mean 40 ± 24-month follow-up period (3.4% vs 5.0%, P > .9). Among patients who underwent both MRI/A and DSA (n = 13), both studies identified the same at-risk territories. No patients experienced MRI/A-related complications, compared with 3 minor DSA-related complications. The use of MRI/A yielded a 6.5-fold reduction in cost per study vs DSA at 1 year. CONCLUSION: Using DSA to follow moyamoya patients after indirect revascularization is generally safe but associated with a low rate of minor complications and a 6.5-fold greater financial cost relative to MRI/A. These data support changing practice to eliminate the use of DSA when following routine bilateral moyamoya cases in the absence of clinical symptoms or specific concerns. Using MRI/A as the primary postoperative follow-up modality in this select population provides noninferior care and greater patient access, while reducing cost and potentially decreasing risk.

Recurrent abdominal dislodgement of intrathecal pump and utility of infraclavicular site for patients with elevated body mass index: illustrative case.

BACKGROUND: Intrathecal baclofen, delivered via implanted pump, has been used to manage spasticity for approximately 40 years. The device is typically subcutaneously or subfascially implanted in the abdominal wall. There are very few cases reported of the pump being implanted in other locations. OBSERVATIONS: This case describes the complicated course of a patient presenting with multiple episodes of catheter malfunction related to pump flipping in the abdominal pocket. The patient was successfully treated with repositioning of the pump into the infraclavicular fossa. LESSONS: Infraclavicular placement of the implanted pump allowed for a more secure pocket base for this patient and less strain applied to the pump, minimizing the risk of disruption of pump positioning and interruption of drug delivery.

Pediatric Moyamoya Syndrome Secondary to Tuberculous Meningitis: A Case Report.

Objectives: Tuberculosis is uncommon in the United States and a rare cause of meningitis in children with severe neurologic consequences. Tuberculous meningitis (TBM) is an even rarer cause of moyamoya syndrome with only a handful of cases previously reported. Methods: We report the case of a female patient who initially presented at 6 years of age with TBM and developed moyamoya syndrome requiring revascularization surgery. Results: She was found to have basilar meningeal enhancement and right basal ganglia infarcts. She was treated with 12 months of antituberculosis therapy and 12 months of enoxaparin and maintained on daily aspirin indefinitely. However, she developed recurrent headaches and transient ischemic attacks and was found to have progressive bilateral moyamoya arteriopathy. At age 11 years, she underwent bilateral pial synangiosis for the treatment of her moyamoya syndrome. Discussion: Moyamoya syndrome is a rare but serious sequalae of TBM and may be more common in pediatric patients. The risk of stroke may be mitigated by pial synangiosis or other revascularization surgeries in carefully selected patients.

Intracranial complications of hypercoagulability and superinfection in the setting of COVID-19: illustrative cases.

BACKGROUND: Hypercoagulability with thrombosis and associated inflammation has been well-documented in COVID-19, and catastrophic cerebral venous sinus thromboses (CVSTs) have been described. Another COVID-19-related complication is bacterial superinfection, including sinusitis. Here, the authors reported three cases of COVID-19-associated sinusitis, meningitis, and CVST and summarized the literature about septic intracranial thrombotic events as a cause of headache and fever in COVID-19. OBSERVATIONS: The authors described three adolescent patients with no pertinent past medical history and no prior COVID-19 vaccinations who presented with subacute headaches, photosensitivity, nausea, and vomiting after testing positive for COVID-19. Imaging showed subdural collections, CVST, cerebral edema, and severe sinus disease. Two patients had decline in mental status and progression of neurological symptoms. In all three, emergency cranial and sinonasal washouts uncovered pus that grew polymicrobial cultures. After receiving broad-spectrum antimicrobials and various additional treatments, including two of three patients receiving anticoagulation, all patients eventually became neurologically intact with varying ongoing sequelae. LESSONS: These cases demonstrated similar original presentations among previously healthy adolescents with COVID-19 infections, concurrent sinusitis precipitating CVST, and subdural empyemas. Better recognition and understanding of the multisystem results of severe acute respiratory syndrome coronavirus 2 and the complicated sequelae allows for proper treatment.

Impact of Surgical and Medical Treatment on Survival of Patients with Cerebral Aspergillosis: Systematic Review of the Literature.

OBJECTIVE: Cerebral aspergillosis carries a high mortality. Rapid diagnosis and treatment can increase survival, but symptoms and imaging findings are nonspecific. The literature on cerebral aspergillosis consists mostly of case reports and case series and lacks large-scale review of data. METHODS: We performed a review of the literature using PubMed in March 2019. We recorded the year of publication, age and sex of patients, neurosurgical involvement, the antifungals administered, use of intrathecal antifungals, and the outcome of patients. The relationships among variables were tested using bivariant statics and linear regression. RESULTS: A total of 324 studies met the eligibility criteria, and 198 studies including 248 patients were included. Surgical resection (odds ratio [OR], 0.45; 95% confidence interval [CI], 0.25-0.80; P < 0.01) and administration of voriconazole (OR, 0.32; 95% CI, 0.18-0.55; P < 0.001) or itraconazole (OR, 0.36; 95% CI, 0.16-0.72; P < 0.001) were shown to be significantly associated with survival. CONCLUSIONS: Given the significant survival benefits for patients who received voriconazole and surgical intervention, we suggest early antifungal medical treatment and resection.

Rapid-sequence MRI for evaluation of pediatric traumatic brain injury: a systematic review.

OBJECTIVE: Rapid-sequence MRI (RSMRI) of the brain is a limited-sequence MRI protocol that eliminates ionizing radiation exposure and reduces imaging time. This systematic review sought to examine studies of clinical RSMRI use for pediatric traumatic brain injury (TBI) and to evaluate various RSMRI protocols used, including their reported accuracy as well as clinical and systems-based limitations to implementation. METHODS: PubMed, EMBASE, and Web of Science databases were searched, and clinical articles reporting the use of a limited brain MRI protocol in the setting of pediatric head trauma were identified. RESULTS: Of the 1639 articles initially identified and reviewed, 13 studies were included. An additional article that was in press at the time was provided by its authors. The average RSMRI study completion time was variable, spanning from 1 minute to 16 minutes. RSMRI with "blood-sensitive" sequences was more sensitive for detection of hemorrhage compared with head CT (HCT), but less sensitive for detection of skull fractures. Compared with standard MRI, RSMRI had decreased sensitivity for all evidence of trauma. CONCLUSIONS: Protocols and uses of RSMRI for pediatric TBI were variable among the included studies. While traumatic pathology missed by RSMRI, such as small hemorrhages and linear, nondisplaced skull fractures, was frequently described as clinically insignificant, in some cases these findings may be prognostically and/or forensically significant. Institutions should integrate RSMRI into pediatric TBI management judiciously, relying on clinical context and institutional capabilities.

National Imaging Trends in Pediatric Traumatic Brain Injury and Hydrocephalus.

OBJECTIVE: Reduction in use of computed tomography (CT) in favor of rapid-sequence magnetic resonance imaging (MRI) to decrease pediatric radiation exposure has varied across institutions in the United States. The aims of this study were to understand national trends in CT and rapid-sequence MRI usage and identify variables affecting imaging practices and obstacles to CT reduction. METHODS: This was a retrospective review of deidentified discharge data for children with hydrocephalus and traumatic brain injury (TBI) in the Healthcare Cost and Utilization Project Kids' Inpatient Database in 2000, 2003, 2006, 2009, 2012, and 2016. Utilization of MRI without contrast and CT was extracted using International Classification of Diseases, Ninth Revision, and International Classification of Diseases, Tenth Revision, codes. Hospital region and age cohorts were extracted and used to categorize data. χ2 tests and logistic regression were used for analysis. RESULTS: Hospitalizations utilizing CT decreased (P < 0.05) and hospitalizations utilizing MRI increased (P < 0.05) overall in both diagnosis groups throughout the years analyzed. However, there was significant regional variation in imaging. The Northeast had higher CT rates (P < 0.05) and the South had lower CT rates in patients with hydrocephalus and TBI (P < 0.05). No regional variation was found for rates of MRI use in patients with TBI. CONCLUSIONS: Nationwide, the average number of discharges after hospitalizations utilizing CT in patients with hydrocephalus and TBI has decreased, while discharges after hospitalizations utilizing MRI as an alternative imaging modality have increased. Despite successful overall CT reduction, significant regional variation exists within this trend showing inconsistent reduction of CT use.

Pediatric nonoperative skull fractures: delayed complications and factors associated with clinic and imaging utilization.

OBJECTIVE: Skull fractures represent a common source of morbidity in the pediatric trauma population. This study characterizes the type of follow-up that these patients receive and discusses predictive factors for follow-up. METHODS: The authors reviewed cases of nonoperative pediatric skull fractures at a single academic hospital between 2007 and 2017. Clinical patient and radiological fractures were recorded. Recommended neurosurgical follow-up, follow-up appointments, imaging studies, and fracture-related complications were recorded. Statistical analyses were performed to identify predictors for outpatient follow-up and imaging. RESULTS: The study included 414 patients, whose mean age was 5.2 years; 37.2% were female, and the median length of stay was 1 day (IQR 0.9-4 days). During 438 clinic visits and a median follow-up period of 8 weeks (IQR 4-12, range 1-144 weeks), 231 imaging studies were obtained, mostly head CT scans (55%). A total of 283 patients were given recommendations to attend follow-up in the clinic, and 86% were seen. Only 12 complications were detected, including 7 growing skull fractures, 2 traumatic encephaloceles, and 3 cases of hearing loss. Primary care physician (PCP) status and insurance status were associated with a recommendation of follow-up, actual follow-up compliance, and the decision to order outpatient imaging in patients both with and without intracranial hemorrhage. PCP status remained an independent predictor in each of these analyses. Follow-up compliance was not associated with a patient's distance from home. Among patients without intracranial hemorrhage, a follow-up recommendation and actual follow-up compliance were associated with pneumocephalus and other polytraumatic injuries, and outpatient imaging was associated with a bilateral fracture. No complications were found in patients with linear fractures above the skull base in those without an intracranial hemorrhage. CONCLUSIONS: Pediatric nonoperative skull fractures drive a large expenditure of clinic and imaging resources to detect a relatively small profile of complications. Understanding the factors underlying the decision for clinic follow-up and additional imaging can decrease future costs, resource utilization, and radiation exposure. Factors related to injury severity and socioeconomic indicators were associated with outpatient imaging, the decision to follow up patients in the clinic, and patients' subsequent attendance. Socioeconomic status (PCP and insurance) may affect access to appropriate neurosurgical follow-up and deserves future research attention. Patients with no intracranial hemorrhage and with a linear fracture above the skull base do not appear to be at risk for delayed complications and could be candidates for reduced follow-up and imaging.

Using group-specific PCR to detect predation of mayflies (Ephemeroptera) by wolf spiders (Lycosidae) at a mercury-contaminated site.

Bioaccumulation of contaminants can occur across ecosystem boundaries via transport by emergent aquatic insects. In the South River, Virginia, USA, aquatic mercury has contaminated songbirds nesting in adjacent riparian forests. Spiders contribute the majority of mercury to these songbirds' diets. We tested the hypothesis that massive annual mayfly emergences provide a vector for mercury from river sediments to the Lycosid spiders most frequently eaten by contaminated songbirds. We designed mayfly-specific PCR primers that amplified mtDNA from 76% of adult mayflies collected at this site. By combining this approach with an Agilent 2100 electrophoresis system, we created a highly sensitive test for mayfly predation by Lycosids, commonly known as wolf spiders. In laboratory spider feeding trials, mayfly DNA could be detected up to 192h post-ingestion; however, we detected no mayfly predation in a sample of 110 wolf spiders collected at the site during mayfly emergence. We suggest that mayfly predation is not an important mechanism for dietary transfer of mercury to wolf spiders and their avian predators at the South River. Instead, floodplain soil should be considered as a potential proximate source for mercury in the terrestrial food web.