Central nervous system embryonal tumors with EWSR1-PLAGL1 rearrangements reclassified as INI-1 deficient tumors at relapse.

PURPOSE: Central nervous system (CNS) embryonal tumors are a diverse group of malignant tumors typically affecting pediatric patients that recently have been better defined, and this paper describes evolution of a unique type of embryonal tumor at relapse. METHODS: Two pediatric patients with CNS embryonal tumors with EWSR1-PLAGL1 rearrangements treated at Arkansas Children's Hospital with histopathologic and molecular data are described. RESULTS: These two patients at diagnosis were classified as CNS embryonal tumors with EWSR1-PLAGL1 rearrangements based on histologic appearance and molecular data. At relapse both patient's disease was reclassified as atypical teratoid rhabdoid tumor (ATRT) based on loss of INI-1, presence of SMARCB1 alterations, and methylation profiling results. CONCLUSION: CNS embryonal tumors with EWSR1-PLAGL1 rearrangements acquire or include a population of cells with SMARCB1 alterations that are the component that predominate at relapse, suggesting treatment aimed at this disease component at diagnosis should be considered.

Arachnoid cyst in the pediatric patient: What the radiologist needs to know.

Arachnoid cysts are the most common incidentally discovered intracranial lesions on imaging and the most common cystic intracranial lesions. They may be developmental or secondary. A relative lack of recent literature and any comprehensive radiological review on arachnoid cysts has led to a general lack of awareness among radiologists of symptomatic or complicated arachnoid cysts. This is particularly concerning in pediatric patients. While arachnoid cysts are asymptomatic in most cases, they can cause clinical symptoms in a minority of cases, especially when they occur in unusual sites. These include intraventricular locations where they may cause hydrocephalus, the basal cisterns where they may compress cranial nerves, the cerebellopontine angle where they have to be differentiated from a number of cystic lesions, the cavum septum pellucidum or cavum velum interpositum, the choroid fissure where they can entrap the temporal horn and compress the hippocampus, the posterior fossa where they need to be differentiated from other posterior fossa cystic lesions, and within the spinal canal where there is a concern for cord or nerve root compression. Larger cysts are more prone to complications such as mass effect, hemorrhage, and rupture. Hemorrhage and rupture often present with acute symptoms. Ruptured cysts lose their characteristic imaging appearance and can mimic several ominous pathologies. It therefore becomes vital to accurately diagnose these cases as complications of pre-existing arachnoid cysts for appropriate management. A detailed review of all diagnostic imaging aspects of arachnoid cysts will help fill in the existing information void on this important entity.

The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

Can you see with CT? Is cervical spine computed tomographic imaging sufficient in pediatric trauma?

BACKGROUND: Traumatic cervical spine injury (CSI) is fundamentally different in children, and imaging recommendations vary; however, prompt diagnosis is necessary. METHODS: We conducted a retrospective cohort study, evaluating children who presented after traumatic injury from 7/1/2012 to 12/31/2019 receiving a cervical spine CT. Evaluation of the incidence and clinical significance of CSI undetected on CT subsequently diagnosed on MRI was conducted. Additionally, all with CSI underwent image review to evaluate for potential overlooked, but visible pathology. RESULTS: 1487 children underwent a cervical spine CT, revealing 52 with CSI. 237 underwent MRI due to an abnormal CT or continued clinical concern. Ultimately, three were discovered to have clinically significant CSI missed on CT. In all cases, retrospective review demonstrated a retroclival hematoma when soft tissue windows were formatted in sagittal and coronal views. CONCLUSIONS: A normal CT may be sufficient to rule-out clinically significant CSI. However, the presence of a retroclival hematoma must be evaluated.

Connectomic profiling and Vagus nerve stimulation Outcomes Study (CONNECTiVOS): a prospective observational protocol to identify biomarkers of seizure response in children and youth.

INTRODUCTION: Vagus nerve stimulation (VNS) is a neuromodulation therapy that can reduce the seizure burden of children with medically intractable epilepsy. Despite the widespread use of VNS to treat epilepsy, there are currently no means to preoperatively identify patients who will benefit from treatment. The objective of the present study is to determine clinical and neural network-based correlates of treatment outcome to better identify candidates for VNS therapy. METHODS AND ANALYSIS: In this multi-institutional North American study, children undergoing VNS and their caregivers will be prospectively recruited. All patients will have documentation of clinical history, physical and neurological examination and video electroencephalography as part of the standard clinical workup for VNS. Neuroimaging data including resting-state functional MRI, diffusion-tensor imaging and magnetoencephalography will be collected before surgery. MR-based measures will also be repeated 12 months after implantation. Outcomes of VNS, including seizure control and health-related quality of life of both patient and primary caregiver, will be prospectively measured up to 2 years postoperatively. All data will be collected electronically using Research Electronic Data Capture. ETHICS AND DISSEMINATION: This study was approved by the Hospital for Sick Children Research Ethics Board (REB number 1000061744). All participants, or substitute decision-makers, will provide informed consent prior to be enrolled in the study. Institutional Research Ethics Board approval will be obtained from each additional participating site prior to inclusion. This study is funded through a Canadian Institutes of Health Research grant (PJT-159561) and an investigator-initiated funding grant from LivaNova USA (Houston, TX; FF01803B IIR).

Complications and outcomes of posterior fossa decompression with duraplasty versus without duraplasty for pediatric patients with Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM). METHODS: The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. RESULTS: A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD. CONCLUSIONS: PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

Management of Sleep Apnea in Children with Chiari I Malformation: A Retrospective Study.

INTRODUCTION: The literature indicates that decompression of Chiari I malformations (CM-1) may resolve symptoms of sleep apnea. This study aims to identify the incidence of obstructive sleep apnea (OSA), central sleep apnea (CSA), and mixed sleep apnea in a cohort of pediatric CM-1 patients treated at our institution. We also assessed apnea-hypopnea index and symptomatology before and after surgery to investigate if Chiari decompression is a viable treatment for sleep apnea in CM-1 patients. Improvement relative to ENT surgical intervention was also considered. METHODS: We identified 75 patients who underwent polysomnography (PSG) from our database of 465 CM-1 patients. Sleep apnea diagnosis was based on the sleep physician's overall interpretation of the PSG. Symptomatology pre- and post-surgery was analyzed. RESULTS: Of the 75 CM-1 patients that underwent PSG, 23 were diagnosed with sleep apnea. Sixteen had OSA, 6 had CSA, and 1 had mixed apnea. Twelve OSA patients received ENT intervention. Eight improved and 2 further improved after Chiari decompression. Of the 4 patients that did not improve, one of those later improved following Chiari decompression. Of the 6 CSA patients, 2 underwent Chiari decompression, but only one improved. The mixed apnea patient underwent several ENT interventions that did not relieve symptoms but improved following Chiari decompression. DISCUSSION/CONCLUSIONS: Based on our results, sleep apnea in CM-1 patients may be obstructive, central, or mixed and is likely multifactorial. A multidisciplinary approach to the management of these patients is important, including neurosurgery, otolaryngology, and sleep medicine. Future prospective studies will lend further insight into this condition and its management.

Chiari Malformation in Children.

Chiari malformation type 1 (CM1) is often found incidentally. However, patients with symptoms or signs referable to CM1 or an associated syrinx will likely benefit from surgical intervention. Patients who are not symptomatic from CM1 at presentation are unlikely to become symptomatic at follow-up.

Extradural decompression versus duraplasty in Chiari malformation type I with syrinx: outcomes on scoliosis from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Scoliosis is common in patients with Chiari malformation type I (CM-I)-associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression. METHODS: A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty. RESULTS: In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion. CONCLUSIONS: In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

Dural augmentation approaches and complication rates after posterior fossa decompression for Chiari I malformation and syringomyelia: a Park-Reeves Syringomyelia Research Consortium study.

OBJECTIVE: Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM. METHODS: The Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft. RESULTS: A total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain. CONCLUSIONS: In the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.

Fatal Cerebral Vasospasm following a Haemophilus influenzae Meningitis in a Young Child with Ventriculoperitoneal Shunt.

INTRODUCTION: Despite the successful implementation of Haemophilus influenzae vaccination, invasive serotypes still lead to a fatal infection. We recently cared for a patient with a ventriculoperitoneal shunt (VPS) and H. influenzae meningitis and septicemia complicated by vasospasm. Vasospasm caused by Haemophilus central nervous system infection has not been previously reported. CASE PRESENTATION: A 34-month-old patient with a recent VPS presented with H. influenzae meningitis and sepsis. Despite the explant of hardware, followed by maximum medical management, the patient developed stroke due to severe vasospasm, which led to diffused anoxic brain injury. CONCLUSIONS: We aim to alert for the possible critical condition caused by H. influenzae. It is essential to treat the underlying illness, despite the presence of a VPS. Surgical implant tends to be overlooked by other subspecialists. Being vaccinated to H. influenzae does not protect from different subtypes like non-typeable H. influenzae. The cause of vasospasm remains unclear.

Occipital-Cervical Fusion and Ventral Decompression in the Surgical Management of Chiari-1 Malformation and Syringomyelia: Analysis of Data From the Park-Reeves Syringomyelia Research Consortium.

BACKGROUND: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P < .001) were increased within the OCF group, whereas only basilar invagination (1/4, P < .001) was increased in the OCF/VD group. Clivo-axial angle (CXA) was significantly lower for both OCF (128.8 ± 15.3°, P = .008) and OCF/VD (115.0 ± 11.6°, P = .025) groups when compared to PFD-only group (145.3 ± 12.7°). pB-C2 did not differ among groups. CONCLUSION: Although PFD alone is adequate for treating the vast majority of CM-1/SM patients, OCF or OCF/VD may be occasionally utilized. Cranial base and spine pathologies and CXA may provide insight into the need for OCF and/or OCF/VD.

Hypothermia as an Outcome Predictor Tool in Pediatric Trauma: A Propensity-Matched Analysis.

OBJECTIVE: Hypothermia is an independent risk factor for mortality in adult trauma patients. Two small studies have shown similar results in pediatric trauma patients. Temperature is not included in any pediatric trauma assessment scores. This study sought to compare mortality and various descriptive outcomes between pediatric hypothermic and normothermic trauma patients. METHODS: Data were obtained from the National Trauma Database from 2009 to 2012. Patients meeting inclusion criteria were stratified by presence of isolated head injury, head injury with multiple trauma, and absence of head injury. These groups were then subdivided into hypothermic (temperature ≤36°C) and normothermic groups. We used propensity score matching to 1:1 match hypothermic and normothermic patients. Mortality, neurosurgical interventions, endotracheal intubation, blood transfusion, length of stay, laparotomy, thoracotomy, conversion of cardiac rhythm, and time receiving mechanical ventilation were evaluated. RESULTS: Data from 3,011,482 patients were obtained. There were 414,562 patients who met the inclusion criteria. In all patients meeting inclusion criteria, hypothermia was a significant risk factor in all outcomes measured. Following stratification and 1:1 matching, in all groups, hypothermia was associated with increased mortality (P < 0.0001), increased rate of endotracheal intubation (P < 0.0002), increased need for blood transfusion (P < 0.0025), and conversion of cardiac rhythm (P < 0.0027). CONCLUSION: Hypothermia has been shown to be a significant prognostic indicator in the pediatric trauma patient with further potential application. Future studies are indicated to evaluate the incorporation of hypothermia into the Pediatric Trauma Score not only to help predict injury severity and mortality but also to improve appropriate and expeditious patient transfer to pediatric trauma centers and potentially facilitate earlier intervention.

Natural history of Chiari I malformation in children: a retrospective analysis.

PURPOSE: While there are increasing numbers of studies published regarding Chiari I malformation (CM1) in children, most of these focus on surgical indications, technique, and outcomes. Few studies examine the natural history of CM1 once the decision is made to treat a patient conservatively. In this study, we seek to determine the percentage of pediatric patients who undergo surgery for CM1, both after initial consultation and in a delayed fashion, the natural history of CM1 after a decision to pursue non-operative management, and attempt to identify patient factors that may predict development of new or worsening CM1 symptoms. METHODS: From our database of 465 pediatric patients with CM1, we identified those who were seen for initial consultation from July 1, 2011, to June 30, 2016. We examined rates of surgical intervention, types of surgical intervention, age, gender, and presence or absence of headache and syrinx, and looked carefully at the patients who had new or worsening symptoms prompting delayed surgical intervention. RESULTS: We identified 226 patients meeting inclusion criteria. Overall, 15% of patients had surgery, the majority being Chiari decompression. Just over half of these patients had surgery within 6 months of initial consultation. Of those with delayed surgery, only 4 patients had new symptoms/syrinx and 1 patient had symptom progression. The other patients had various reasons for surgical delay not related to symptom development or progression. There were no obvious commonalities among these 5 patients that could predict progression prospectively. All patients who had surgery did so within 2 years of initial consultation. CONCLUSION: Overall, the natural history of asymptomatic CM1 is benign. Patients treated non-operatively are unlikely to progress. If they do progress, this is likely to occur within 2 years of initial consultation. There were no factors identified in this study that predicted new or worsening symptoms over time.

Soft tissue density within the foramen magnum, a predictor for surgical intervention in pediatric patients with Chiari malformation type I.

OBJECTIVE: Chiari malformation type I (CMI) is diagnosed as herniation of the cerebellar tonsils by at least 5 mm below the foramen magnum. However, the degree of tonsillar herniation is a poor predictor of the need for decompression surgery. Exploration for an alternative morphological predictor for surgical intervention could provide greater insight into the development of an appropriate treatment plan for these patients. To investigate this issue, the authors calculated the soft tissue density within the foramen magnum as a measure of impaction of the cerebellar tonsils. Soft tissue density within the foramen magnum and degree of tonsillar herniation were then assessed for their correlation with the need for surgical intervention. METHODS: The authors conducted a retrospective, longitudinal chart review of pediatric patients with CMI. Those who had undergone surgical intervention were considered symptomatic and those who had been treated conservatively, as asymptomatic. Soft tissue density was found by dividing the soft tissue occupancy of the foramen magnum (brainstem and cerebellar tonsils) by the total area of the bony foramen magnum. The predictive value of these two measurements for the need of surgery was determined. RESULTS: Of the 465 patients seen for CMI at the authors' institution between July 1, 2011, and May 31, 2017, 80 underwent surgical intervention and 385 were asymptomatic. The average tissue density was significantly greater in the surgical group than in the asymptomatic group (83.3% and 78.6%, respectively, p < 0.0001). The average tonsillar descent for surgical patients was 10.8 mm compared to 9.8 mm for asymptomatic patients (p = 0.140). The point-biserial correlation coefficient was assessed, and soft tissue density was found to positively correlate with the need for surgical intervention (rpb = 0.199, p = 0.0001), whereas tonsillar herniation did not correlate with the need for surgery (rpb = 0.083, p = 0.115). Additionally, the degree of tonsillar herniation did not correlate with soft tissue density (r = 0.09), indicating that soft tissue density is an independent morphological parameter. CONCLUSIONS: The study findings suggest that the need for surgical intervention in CMI patients is positively correlated with increasing soft tissue density within the foramen magnum, whereas the degree of tonsillar herniation did not show a correlation with the need for surgical intervention. Additionally, soft tissue density is a factor independent of the degree of tonsillar herniation. Further investigation of tissue density within the foramen magnum is needed in the hope of discovering a clinically applicable parameter that would indicate a need for surgical intervention in patients with CMI.

Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established. METHODS: Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length. RESULTS: The authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16-0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34-1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12-0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02-2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31-3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67-5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate. CONCLUSIONS: These data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.

Hemispheric Cerebral Oximetry Monitoring During Pediatric Seizure Activity in a Pediatric Emergency Department.

BACKGROUND: Sustained neuronal activity during seizures causes cellular perturbations, alterations in cerebral physiology, and potentially neurological injury, a neurological emergency. With variable clinical manifestations of seizures, frequent failure of seizure recognition by providers in pediatric and developmentally challenged patients can increase seizure complications. Neuroresuscitation should include rapid cerebral physiology assessment for increased seizure recognition and optimal neurological outcomes. In neurological emergencies, cerebral oximetry has demonstrated its utility in altered cerebral physiology and a standard combat neurological assessment tool. During adult seizures, cerebral oximetry (regional cerebral oxygen saturation [rcSO2]) has been shown as a useful neurological assessment tool, but research is lacking in pediatric emergency department (PED) seizure patients. OBJECTIVE: The aim of this study was to identify trends in rcSO2 readings for patients presenting to the PED with seizure activity and in the postseizure state in order to evaluate usefulness of rcSO2 as a neurological assessment tool in pediatric seizure patients. METHODS: This was a PED observational case series comparing hemispheric rcSO2 readings in first-time clinically evident generalized and focal seizure patients to first-time postseizure patients with no PED seizures. RESULTS: Generalized or focal seizure (n = 185) hemispheric rcSO2 revealed significant differences compared with nonseizure and controls' rcSO2 readings (n = 115) (P < 0.0001). Generalized and focal seizure rcSO2's were either less than 60% or greater than 80% compared with nonseizure rcSO2 (P < 0.0001). Ipsilateral focal seizure rcSO2 correlated to seizure side (P < 0.0001) and was less than the contralateral rcSO2 (P < 0.0001), with interhemispheric rcSO2 discordance greater than 16 (P < 0.0001). Seizure to preseizure rcSO2 discordance was as follows: generalized 15.2, focal: left 19.8, right 20.3 (P < 0.0001). CONCLUSIONS: Hemispheric during-seizure rcSO2 readings significantly correlated with generalized and focal seizures and reflected altered cerebral physiology. Ipsilateral focal seizure rcSO2 readings correlated to the focal side with wide interhemispheric rcSO2 discordance. All postseizure rcSO2 readings returned to preseizure readings, showing altered cerebral physiology resolution. Overall, in generalized or focal seizure, rcSO2 readings were less than 60% or greater than 80%, and in focal seizure, interhemispheric rcSO2 discordance was greater than 10. During seizures, hemispheric rcSO2 readings demonstrated its potential pediatric seizure utility. Utilizing rcSO2 readings related to seizure activity could expedite pediatric and developmentally challenged patients' seizure recognition, cerebral assessment, and interventions especially in pharmacoresistant seizures.

Effects of growth hormone therapy in pediatric patients with growth hormone deficiency and Chiari I malformation: a retrospective study.

PURPOSE: The safety and efficacy of growth hormone replacement therapy (GHRT) on pediatric patients with growth hormone deficiency (GHD) and Chiari I malformation (CIM) are not well investigated within the current body of literature. With no clear indication of the effects of GHRT on CIM disease progression, we sought to determine the effect of GHRT on tonsillar herniation and progression of CIM symptomatology. METHODS: From a previously established database of 465 patients with radiologically confirmed CIM defined as > 5 mm of tonsillar descent on head magnetic resonance imaging (MRI), we identified 20 patients who also had GHD. Using the imaging analysis software package, ANALYZE, the degree of change in tonsillar herniation was documented between initial and final MRI measurements. The radiologic and clinical changes over time were examined via a proportional odds model, Student's t test, Mann-Whitney test, or a mixed model corresponding to the outcomes measured either on an ordinal scale or on a quantitative scale. RESULTS: Incidence of GHD in our CIM population was 4.3%. There was no significant effect of GHRT on the degree of tonsillar herniation in patients with GHD and CIM. No patient became symptomatic, developed syringomyelia, or required surgical intervention for CIM. CONCLUSION: Based on our findings with a larger sample size, along with recent reports, the incidence of patients with CIM and GHD we reported (0.86-5%) is likely more indicative of the actual incidence of GHD and CIM than the prior findings within the literature (9.1-20%). We also suggest that GHRT does not significantly affect CIM morphology or symptomatology. Therefore, neurosurgeons should have no hesitation clearing these patients for GHRT.