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.

Determinants of symptom presentation and resolution following concussions in high school sports.

Sport-related concussions (SRCs) are prevalent in high school (HS) sports, though the determinants of, and relationships between concussion symptoms in this population remain relatively unknown. We analysed SRC data captured within the HS RIO injury surveillance system during 2014/15-2018/19. We used Generalized Estimating Equations to simultaneously assess covariate predictors of symptom presentations and identify pairwise symptom associations and employed multivariable ordinal logistic regressions to determine symptom resolution time (SRT) predictors. Among the 8,969 concussions assessed, headaches (94.3%) and dizziness (73.3%) were the most prevalently reported symptoms. In 5,953 boys' concussion assessed, class year, event type and injury mechanism emerged as determinants of various concussion symptoms, while in 3,016 girls' concussions assessed, class year, event type, injury mechanism, and sport type emerged as significant symptom determinants. In both groups, difficulty concentrating, sensitivity to light, sensitivity to noise, and disorientation were symptoms with the strongest associations with other symptoms. Odds of longer SRT were higher with greater endorsement (i.e. counts) of the subset of symptoms with strongest cross-domain associations (ORadj. = 1.35; 95% CI = [1.30, 1.40]). Our findings provide valuable information for informing sideline and follow-up clinical concussion assessment algorithms in high school athletes.

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.

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.

Prenatal Surgery for Open Fetal Spina Bifida in Patients with Obesity: A Review of Current Evidence and Future Directions.

Background: Obesity rates have significantly increased globally, affecting up to 40% of women of childbearing age in the United States. While prenatal repair of open fetal spina bifida has shown improved outcomes, most fetal surgery centers exclude patients with a body mass index (BMI) ≥ 35 kg/m2 based on criteria from the Management of Myelomeningocele Study (MOMS) trial. This exclusion raises concerns about healthcare equity and highlights a significant knowledge gap regarding the safety and efficacy of fetal spina bifida repair in patients with obesity. Objective: To review the current state of knowledge regarding open fetal surgery for fetal spina bifida in patients with obesity, focusing on safety, efficacy, and clinical considerations. Methods: A comprehensive literature search was conducted using the PubMed and EMBASE databases, covering articles from the inception of the databases to April 2024. Studies discussing fetal surgery for neural tube defects and documenting BMI measurements and their impact on surgical outcomes, published in peer-reviewed journals, and available in English were included. Quantitative data were extracted into an Excel sheet, and data synthesis was conducted using the R programming language (version 4.3.3). Results: Three retrospective studies examining outcomes of prenatal open spina bifida repair in a total of 43 patients with a BMI ≥ 35 kg/m2 were identified. These studies did not report significant adverse maternal or fetal outcomes compared to patients with lower BMIs. Our pooled analysis revealed a perinatal mortality rate of 6.1% (95% CI: 1.76-18.92%), with 28.0% (95% CI: 14.0-48.2%) experiencing the premature rupture of membranes and 82.0% (95% CI: 29.2-98.0%) delivering preterm (<37 weeks). Membrane separation was reported in 10.3% of cases (95% CI: 3.3-27.7%), the mean gestational age at birth was 34.3 weeks (95% CI: 32.3-36.3), and the average birth weight was 2651.5 g (95% CI: 2473.7-2829.4). Additionally, 40.1% (95% CI: 23.1-60.0%) required a ventriculoperitoneal shunt. Conclusion: While current evidence suggests that fetal spina bifida repair may be feasible in patients with obesity, significant limitations in the existing body of research were identified. These include small sample sizes, retrospective designs, and a lack of long-term follow-up data. There is an urgent need for large-scale, prospective, multicenter studies to definitively establish the safety and efficacy of fetal spina bifida repair in patients with obesity. Such research is crucial for developing evidence-based guidelines, improving clinical outcomes, and addressing healthcare disparities in this growing patient population with obesity.

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.

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.

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.