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OBJECTIVE: When the peritoneal cavity cannot serve as the distal shunt terminus, nonperitoneal shunts, typically terminating in the atrium or pleural space, are used. The comparative effectiveness of these two terminus options has not been evaluated. The authors directly compared shunt survival and complication rates for ventriculoatrial (VA) and ventriculopleural (VPl) shunts in a pediatric cohort. METHODS: The Hydrocephalus Clinical Research Network Core Data Project was used to identify children ≤ 18 years of age who underwent either VA or VPl shunt insertion. The primary outcome was time to shunt failure. Secondary outcomes included distal site complications and frequency of shunt failure at 6, 12, and 24 months. RESULTS: The search criteria yielded 416 children from 14 centers with either a VA (n = 318) or VPl (n = 98) shunt, including those converted from ventriculoperitoneal shunts. Children with VA shunts had a lower median age at insertion (6.1 years vs 12.4 years, p < 0.001). Among those children with VA shunts, a hydrocephalus etiology of intraventricular hemorrhage (IVH) secondary to prematurity comprised a higher proportion (47.0% vs 31.2%) and myelomeningocele comprised a lower proportion (17.8% vs 27.3%) (p = 0.024) compared with those with VPl shunts. At 24 months, there was a higher cumulative number of revisions for VA shunts (48.6% vs 38.9%, p = 0.038). When stratified by patient age at shunt insertion, VA shunts in children < 6 years had the lowest shunt survival rate (p < 0.001, log-rank test). After controlling for age and etiology, multivariable analysis did not find that shunt type (VA vs VPl) was predictive of time to shunt failure. No differences were found in the cumulative frequency of complications (VA 6.0% vs VPl 9.2%, p = 0.257), but there was a higher rate of pneumothorax in the VPl cohort (3.1% vs 0%, p = 0.013). CONCLUSIONS: Shunt survival was similar between VA and VPl shunts, although VA shunts are used more often, particularly in younger patients. Children < 6 years with VA shunts appeared to have the shortest shunt survival, which may be a result of the VA group having more cases of IVH secondary to prematurity; however, when age and etiology were included in a multivariable model, shunt location (atrium vs pleural space) was not associated with time to failure. The baseline differences between children treated with a VA versus a VPl shunt likely explain current practice patterns.
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Epilepsy is a common symptom of pediatric cavernous malformations. In medically refractory patients, surgery can achieve high seizure freedom rates with low morbidity. This video depicts the use of a minipterional craniotomy and transsulcal resection of a frontal opercular cavernous malformation in a 13-year-old female with medically intractable epilepsy. At 1-year follow-up, she was evaluated as Engel class I with a significant improvement in her quality of life. Principles of cavernous malformation resection for the treatment of epilepsy are also reviewed. The video can be found here: https://stream.cadmore.media/r10.3171/2024.4.FOCVID2441.
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PURPOSE: To characterize the frequency of incidental dural tears in pediatric spine surgery, their treatment, complications, and results of long-term follow-up. METHODS: A retrospective review of all pediatric patients who underwent a posterior spinal fusion (PSF) between 2004-2019 at a tertiary children's hospital was conducted. Electronic medical records were reviewed for patient demographics, intra-operative data, presence of an incidental dural tear, repair method, and patient outcomes. RESULTS: 3043 PSFs were reviewed, with 99 dural tears identified in 94 patients (3.3% overall incidence). Mean follow-up was 35.7 months (range 0.1-142.5). When the cause of the dural tear was specified, 69% occurred during exposure, 5% during pedicle screw placement, 4% during osteotomy, 2% during removal of implants, and 2% during intra-thecal injection of morphine. The rate of dural tears during primary PSF was significantly lower than during revision PSF procedures (2.6% vs. 6.2%, p < 0.05). 86.9% of dural tears were repaired and/or sealed intraoperatively, while 13.1% had spontaneous resolution. Postoperative headaches developed in 13.1% of patients and resolved at a mean of 7.6 days. There was no difference in the incidence of headaches in patients that were ordered bedrest vs. no bedrest (p > 0.99). Postoperative infections occurred in 9.5% of patients and 24.1% patients were identified to have undergone a revision surgery. CONCLUSIONS: Incidence of intra-operative dural tears in pediatric spine surgery is 3.3%. Although complications associated with the dural tear occur, most resolve over time and there were no long-term sequelae in patients with 2 years of follow up. LEVEL OF EVIDENCE: Level IV.
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Dura-Máter , Complicações Pós-Operatórias , Fusão Vertebral , Humanos , Fusão Vertebral/efeitos adversos , Fusão Vertebral/métodos , Masculino , Estudos Retrospectivos , Feminino , Criança , Dura-Máter/lesões , Adolescente , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Pré-Escolar , Incidência , Complicações Intraoperatórias/epidemiologia , Complicações Intraoperatórias/etiologia , Seguimentos , Achados Incidentais , Reoperação/estatística & dados numéricos , Cefaleia/etiologia , Cefaleia/epidemiologia , Parafusos Pediculares/efeitos adversosRESUMO
STUDY OBJECTIVES: Advances in prenatal repair of myelomeningocele (MMC) have improved outcomes involving different organ systems. There is limited data on respiratory outcomes following prenatal surgical repair. We hypothesize there is no difference in respiratory outcomes between spina bifida (SB) patients who have undergone prenatal versus postnatal repair. METHODS: We performed a retrospective study of 46 infants <1 year with SB seen at Children's Hospital Los Angeles from 2004-2022. Demographic data, timing of closure, neonatal course, Chiari II malformation (CIIM), ventriculoperitoneal shunt (VPS), polysomnography (PSG) results, and need for supplemental oxygen were collected. Unpaired t-test and Chi-square Test were used to analyze results. RESULTS: 31/46 had prenatal repair of MMC; average age at repair was 27 weeks post-conception (PCA). Average age at postnatal repair was 37 PCA. There was no difference in age at PSG. There was no difference in CIIM presence (p=0.61). 60% of patients with postnatal repair and 23% in the prenatal group underwent VPS placement (p=0.01).There was no difference in PSG findings between the two groups: CAI (p=0.11), OAHI (p=0.64), average SpO2 baseline (p=0.91), average SpO2 nadir (p=0.17), average PETCO2 baseline (p=0.87), and average PETCO2 maximum (p=0.54). There were no significant differences in the proportion of patients on supplemental O2 (p=0.25), CSA or OSA between groups. CONCLUSIONS: Patients with SB who've undergone closure of neural tube defect have persistent central apneas, obstructive apneas, and significant hypoxemia. There were no differences in the frequency or severity of sleep-disordered breathing in those with prenatal repair versus postnatal repair.
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OBJECTIVE: Standard MRI protocols lack a quantitative sequence that can be used to evaluate shunt-treated patients with a history of hydrocephalus. The objective of this study was to investigate the use of phase-contrast MRI (PC-MRI), a quantitative MR sequence, to measure CSF flow through the shunt and demonstrate PC-MRI as a useful adjunct in the clinical monitoring of shunt-treated patients. METHODS: The rapid (96 seconds) PC-MRI sequence was calibrated using a flow phantom with known flow rates ranging from 0 to 24 mL/hr. Following phantom calibration, 21 patients were scanned with the PC-MRI sequence. Multiple, successive proximal and distal measurements were gathered in 5 patients to test for measurement error in different portions of the shunt system and to determine intrapatient CSF flow variability. The study also includes the first in vivo validations of PC-MRI for CSF shunt flow by comparing phase-contrast-measured flow rate with CSF accumulation in a collection burette obtained in patients with externalized distal shunts. RESULTS: The PC-MRI sequence successfully measured CSF flow rates ranging from 6 to 54 mL/hr in 21 consecutive pediatric patients. Comparison of PC-MRI flow measurement and CSF volume collected in a bedside burette showed good agreement in a patient with an externalized distal shunt. Notably, the distal portion of the shunt demonstrated lower measurement error when compared with PC-MRI measurements acquired in the proximal catheter. CONCLUSIONS: The PC-MRI sequence provided accurate and reliable clinical measurements of CSF flow in shunt-treated patients. This work provides the necessary framework to include PC-MRI as an immediate addition to the clinical setting in the noninvasive evaluation of shunt function and in future clinical investigations of CSF physiology.
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Derivações do Líquido Cefalorraquidiano , Hidrocefalia , Humanos , Criança , Hidrocefalia/diagnóstico por imagem , Hidrocefalia/cirurgia , Imageamento por Ressonância Magnética/métodos , Procedimentos Neurocirúrgicos , Próteses e Implantes , Líquido Cefalorraquidiano/fisiologiaRESUMO
INTRODUCTION: Clinical clearance of a child's cervical spine after trauma is often challenging because of impaired mental status or an unreliable neurologic examination. Magnetic resonance imaging (MRI) is the criterion standard for excluding ligamentous injury in children but is constrained by long image acquisition times and frequent need for anesthesia. Limited-sequence magnetic resonance imaging (LSMRI) is used in evaluating the evolution of traumatic brain injury and may also be useful for cervical spine clearance while potentially avoiding the need for anesthesia. The purpose of this study was to assess the sensitivity and negative predictive value of LSMRI as compared with criterion standard full-sequence MRI as a screening tool to rule out clinically significant ligamentous cervical spine injury. METHODS: We conducted a 10-center, 5-year retrospective cohort study (2017-2021) of all children (0-18 years) with a cervical spine MRI after blunt trauma. Magnetic resonance imaging images were rereviewed by a study pediatric radiologist at each site to determine if the presence of an injury could be identified on limited sequences alone. Unstable cervical spine injury was determined by study neurosurgeon review at each site. RESULTS: We identified 2,663 children younger than 18 years who underwent an MRI of the cervical spine with 1,008 injuries detected on full-sequence studies. The sensitivity and negative predictive value of LSMRI were both >99% for detecting any injury and 100% for detecting any unstable injury. Young children (younger than 5 years) were more likely to be electively intubated or sedated for cervical spine MRI. CONCLUSION: Limited-sequence magnetic resonance imaging is reliably detects clinically significant ligamentous injury in children after blunt trauma. To decrease anesthesia use and minimize MRI time, trauma centers should develop LSMRI screening protocols for children without a reliable neurologic examination. LEVEL OF EVIDENCE: Diagnostic Test/Criteria; Level III.
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Vértebras Cervicais , Imageamento por Ressonância Magnética , Sensibilidade e Especificidade , Traumatismos da Coluna Vertebral , Humanos , Imageamento por Ressonância Magnética/métodos , Estudos Retrospectivos , Criança , Pré-Escolar , Vértebras Cervicais/lesões , Vértebras Cervicais/diagnóstico por imagem , Adolescente , Feminino , Lactente , Masculino , Traumatismos da Coluna Vertebral/diagnóstico por imagem , Traumatismos da Coluna Vertebral/cirurgia , Ferimentos não Penetrantes/diagnóstico por imagem , Ferimentos não Penetrantes/cirurgia , Valor Preditivo dos Testes , Recém-NascidoRESUMO
OBJECTIVE: The optimal timing of ventricular shunt placement in low-weight and preterm infants remains an unresolved topic in modern pediatric neurosurgery. Shunt placement for hydrocephalus is performed over a wide range of infant weights, and the standard weight threshold for shunt placement can vary substantially across institutions. The aim of this study was to investigate shunt outcome in infants of low body weight. METHODS: An IRB-approved retrospective analysis of 76 infants (29 females, 47 males) who received primary shunt placement between 2003 and 2018 was performed. Uniform criteria were used over the entire dataset to determine the safety for ventriculoperitoneal (VP) shunt placement: 1) weight near or above 1500 g, 2) feeding tolerance, and 3) lack of necrotizing enterocolitis or active systemic infection. Infants were classified into a low-weight (LW) (< 2000 g) or standard weight (SW) (2000-3000 g) group based on their body weight at the time of initial shunt placement. Shunt survival was compared between the groups. The threshold weight separating the LW and SW groups and outcomes was additionally varied and systematically reanalyzed. RESULTS: Shunts were placed in 24 LW infants and 52 SW infants over the inclusion period. Etiologies for hydrocephalus were similar across groups: predominantly intraventricular hemorrhage (54%) (p = 0.13) and open neural tube defect (29%) (p = 0.61). Both LW and SW groups had 58% 1-year shunt survival rates. Overall, 46% of shunts failed in the LW group compared with 54% in the SW group over a median follow-up of 47 months (range 0-170 months). A log-rank test comparing shunt survival rates did not show significance (p = 0.43). Groups were repartitioned using a range of threshold weights (1600-2400 g) to divide LW from SW infants. The lack of association between VP shunt placement in LW infants and time frame of revision was consistently observed over the full range of varied threshold weights. CONCLUSIONS: There was no significant difference in overall time to shunt revision between infants weighing < 2000 g and infants weighing 2000-3000 g. No correlation between weight and shunt survival was detected. Combined with other clinical features pertinent to the management of hydrocephalus in the neonatal population, this investigation provides insight toward clinical decision-making regarding infants of low birth weight and suggests that further multi-institutional study on this topic is warranted.
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Hidrocefalia , Recém-Nascido de Baixo Peso , Derivação Ventriculoperitoneal , Humanos , Hidrocefalia/cirurgia , Masculino , Feminino , Estudos Retrospectivos , Recém-Nascido , Derivação Ventriculoperitoneal/métodos , Lactente , Resultado do Tratamento , Recém-Nascido Prematuro , Fatores de TempoRESUMO
OBJECTIVE: The Hydrocephalus Clinical Research Network (HCRN) conducted a prospective study 1) to determine if a new, better-performing version of the Endoscopic Third Ventriculostomy Success Score (ETVSS) could be developed, 2) to explore the performance characteristics of the original ETVSS in a modern endoscopic third ventriculostomy (ETV) cohort, and 3) to determine if the addition of radiological variables to the ETVSS improved its predictive abilities. METHODS: From April 2008 to August 2019, children (corrected age ≤ 17.5 years) who underwent a first-time ETV for hydrocephalus were included in a prospective multicenter HCRN study. All children had at least 6 months of clinical follow-up and were followed since the index ETV in the HCRN Core Data Registry. Children who underwent choroid plexus cauterization were excluded. Outcome (ETV success) was defined as the lack of ETV failure within 6 months of the index procedure. Kaplan-Meier curves were constructed to evaluate time-dependent variables. Multivariable binary logistic models were built to evaluate predictors of ETV success. Model performance was evaluated with Hosmer-Lemeshow and Harrell's C statistics. RESULTS: Seven hundred sixty-one children underwent a first-time ETV. The rate of 6-month ETV success was 76%. The Hosmer-Lemeshow and Harrell's C statistics of the logistic model containing more granular age and etiology categorizations did not differ significantly from a model containing the ETVSS categories. In children ≥ 12 months of age with ETVSSs of 50 or 60, the original ETVSS underestimated success, but this analysis was limited by a small sample size. Fronto-occipital horn ratio (p = 0.37), maximum width of the third ventricle (p = 0.39), and downward concavity of the floor of the third ventricle (p = 0.63) did not predict ETV success. A possible association between the degree of prepontine adhesions on preoperative MRI and ETV success was detected, but this did not reach statistical significance. CONCLUSIONS: This modern, multicenter study of ETV success shows that the original ETVSS continues to demonstrate good predictive ability, which was not substantially improved with a new success score. There might be an association between preoperative prepontine adhesions and ETV success, and this needs to be evaluated in a future large prospective study.
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Hidrocefalia , Terceiro Ventrículo , Ventriculostomia , Humanos , Ventriculostomia/métodos , Hidrocefalia/cirurgia , Hidrocefalia/diagnóstico por imagem , Feminino , Masculino , Terceiro Ventrículo/cirurgia , Terceiro Ventrículo/diagnóstico por imagem , Criança , Pré-Escolar , Estudos Prospectivos , Lactente , Resultado do Tratamento , Adolescente , Neuroendoscopia/métodos , SeguimentosRESUMO
BACKGROUND: Aneurysmal bone cysts (ABCs) are rare, highly vascular osteolytic bone lesions that predominantly affect pediatric populations. This report evaluates the clinicopathological data of pediatric patients with spinal ABCs. The medical records for all patients at Children's Hospital Los Angeles with biopsy-proven ABCs of the spine between 1998 and 2018 were evaluated. OBSERVATIONS: Seventeen patients, 6 males and 11 females, were identified. The mean age at surgery was 10.4 years (range, 3.5-20 years). The most common presenting complaint was pain at the lesion site 16/17 (94%), followed by lower-extremity weakness 8/17 (47%). Resection and intralesional curettage were performed in all patients. Three (18%) of 17 patients underwent selective arterial embolization prior to resection. Spinal stability was compromised in 15 of 17 patients (88%), requiring instrumented fusion. Five (29%) of the 17 patients received additional therapy including radiation, calcitonin-methylprednisolone, or phenol. Four (23.5%) of 17 patients experienced a recurrence, and the mean time to recurrence was 15 months. The postoperative follow-up ranged from 6 to 108 months (median, 28 months). Reoperation occurred after an average of 35 months. At the recent follow-up, patients were free of disease. LESSONS: Gross-total resection by intralesional curettage with case-dependent instrumented spinal fusion for instability remains an effective strategy for managing pediatric spinal ABCs. Long-term follow-up is necessary to detect tumor recurrence.
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BACKGROUND AND OBJECTIVE: Cerebral revascularization of multiple territories traditionally requires multiple constructs, serial anastomoses, or a combination of direct and indirect approaches. A novel 3-vessel anastomosis technique allows for direct, simultaneous multiterritory cerebral revascularization using a single interposition graft. We herein present our experience with this approach. METHODS: Retrospective review of perioperative data and outcomes for patients undergoing multiterritory cerebral revascularization using a 3-vessel anastomosis from 2019 to 2023. RESULTS: Five patients met inclusion criteria (median age 53 years [range 12-73]). Three patients with complex middle cerebral artery aneurysms (1 ruptured) were treated with proximal ligation or partial/complete clip trapping and multiterritory external carotid artery-M2-M2 revascularization using a saphenous vein interposition graft. Two patients with moyamoya disease, prior strokes, and predominately bilateral anterior cerebral artery hypoperfusion were treated with proximal superficial temporal artery-A3-A3 revascularization using a radial artery or radial artery fascial flow-through free flap graft. No patients experienced significant surgery-related ischemia. Bypass patency was 100%. One patient had new strokes from vasospasm after subarachnoid hemorrhage. One patient required a revision surgery for subdural hematoma evacuation and radial artery fascial flow-through free flap debridement, without affecting bypass patency or neurologic outcome. On hospital discharge, median Glasgow Outcome Scale and modified Rankin Scale scores were 4 (range 3-5) and 2 (range 0-5), respectively. On follow-up, 1 patient died from medical complications of their presenting stroke; Glasgow Outcome Scale and modified Rankin Scale scores were otherwise stable or improved. CONCLUSION: The 3-vessel anastomosis technique can be considered for simultaneous revascularization of multiple intracranial territories.
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Revascularização Cerebral , Aneurisma Intracraniano , Acidente Vascular Cerebral , Humanos , Criança , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Revascularização Cerebral/métodos , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/métodos , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/cirurgia , Anastomose Cirúrgica/métodosRESUMO
BACKGROUND: Venous sinus stenosis has been implicated in intracranial hypertension and can lead to papilledema and blindness. The authors report the unique case of a cerebellar transtentorial lesion resulting in venous sinus stenosis in the torcula and bilateral transverse sinuses that underwent resection. OBSERVATIONS: A 5-year-old male presented with subacute vision loss and bilateral papilledema. Imaging demonstrated a lesion causing mass effect on the torcula/transverse sinuses and findings of increased intracranial pressure (ICP). A lumbar puncture confirmed elevated pressure, and the patient underwent bilateral optic nerve sheath fenestration. Cerebral angiography and venous manometry showed elevated venous sinus pressures suggestive of venous hypertension. The patient underwent a craniotomy and supracerebellar/infratentorial approach. A stalk emanating from the cerebellum through the tentorium was identified and divided. Postoperative magnetic resonance imaging showed decreased lesion size and improved sinus patency. Papilledema resolved and other findings of elevated ICP improved. Pathology was consistent with atrophic cerebellar cortex. Serial imaging over 6 months demonstrated progressive decrease in the lesion with concurrent improvements in sinus patency. LESSONS: Although uncommon, symptoms of intracranial hypertension in patients with venous sinus lesions should prompt additional workup ranging from dedicated venous imaging to assessments of ICP and venous manometry.
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Background: Infancy is characterized by rapid neurological transformations leading to consolidation of lifelong function capabilities. Studying the infant brain is crucial for understanding how these mechanisms develop during this sensitive period. We review the neuroimaging modalities used with infants in stimulus-induced activity paradigms specifically, for the unique opportunity the latter provide for assessment of brain function. Methods: Conducted a systematic review of literature published between 1977-2021, via a comprehensive search of four major databases. Standardized appraisal tools and inclusion/exclusion criteria were set according to the PRISMA guidelines. Results: Two-hundred and thirteen papers met the criteria of the review process. The results show clear evidence of overall cumulative growth in the number of infant functional neuroimaging studies, with electroencephalography (EEG) and functional near-infrared spectroscopy (fNIRS) to be the most utilized and fastest growing modalities with behaving infants. However, there is a high level of exclusion rates associated with technical limitations, leading to limited motor control studies (about 6%) in this population. Conclusion: Although the use of functional neuroimaging modalities with infants increases, there are impediments to effective adoption of existing technologies with this population. Developing new imaging modalities and experimental designs to monitor brain activity in awake and behaving infants is vital.
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INTRODUCTION: Hydrocephalus is a common pediatric neurosurgical pathology, typically treated with a ventricular shunt, yet approximately 30% of patients experience shunt failure within the first year after surgery. As a result, the objective of the present study was to validate a predictive model of pediatric shunt complications with data retrieved from the Healthcare Cost and Utilization Project (HCUP) National Readmissions Database (NRD). METHODS: The HCUP NRD was queried from 2016 to 2017 for pediatric patients undergoing shunt placement using ICD-10 codes. Comorbidities present upon initial admission resulting in shunt placement, Johns Hopkins Adjusted Clinical Groups (JHACG) frailty-defining criteria, and Major Diagnostic Category (MDC) at admission classifications were obtained. The database was divided into training (n = 19,948), validation (n = 6,650), and testing (n = 6,650) datasets. Multivariable analysis was performed to identify significant predictors of shunt complications which were used to develop logistic regression models. Post hoc receiver operating characteristic (ROC) curves were created. RESULTS: A total of 33,248 pediatric patients aged 6.9 ± 5.7 years were included. Number of diagnoses during primary admission (OR: 1.05, 95% CI: 1.04-1.07) and initial neurological admission diagnoses (OR: 3.83, 95% CI: 3.33-4.42) positively correlated with shunt complications. Female sex (OR: 0.87, 95% CI: 0.76-0.99) and elective admissions (OR: 0.62, 95% CI: 0.53-0.72) negatively correlated with shunt complications. ROC curve for the regression model utilizing all significant predictors of readmission demonstrated area under the curve of 0.733, suggesting these factors are possible predictors of shunt complications in pediatric hydrocephalus. CONCLUSION: Efficacious and safe treatment of pediatric hydrocephalus is of paramount importance. Our machine learning algorithm delineated possible variables predictive of shunt complications with good predictive value.
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Hidrocefalia , Derivação Ventriculoperitoneal , Criança , Humanos , Feminino , Derivação Ventriculoperitoneal/efeitos adversos , Derivação Ventriculoperitoneal/métodos , Estudos Retrospectivos , Hidrocefalia/etiologia , Procedimentos Neurocirúrgicos/métodos , ComorbidadeRESUMO
Background: Central nervous system tumors are the most common pediatric solid tumors and the most frequent cause of cancer-related morbidity in childhood. Significant advances in understanding the molecular features of these tumors have facilitated the development of liquid biopsy assays that may aid in diagnosis and monitoring response to therapy. In this report, we describe our comprehensive liquid biopsy platform for detection of genome-wide copy number aberrations, sequence variants, and gene fusions using cerebrospinal fluid (CSF) from pediatric patients with brain, spinal cord, and peripheral nervous system tumors. Methods: Cell-free DNA was isolated from the CSF from 55 patients, including 47 patients with tumors and 8 controls. Results: Abnormalities in cell-free DNA were detected in 24 (51%) patients including 11 with copy number alterations, 9 with sequence variants, and 7 with KIAA1549::BRAF fusions. Positive findings were obtained in patients spanning histologic subtypes, tumor grades, and anatomic locations. Conclusions: This study demonstrates the feasibility of employing this platform in routine clinical care in upfront diagnostic and monitoring settings. Future studies are required to determine the utility of this approach for assessing response to therapy and long-term surveillance.
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The rate model of basal ganglia function predicts that muscle activity in dystonia is due to disinhibition of thalamus resulting from decreased inhibitory input from pallidum. We seek to test this hypothesis in children with dyskinetic cerebral palsy undergoing evaluation for deep brain stimulation (DBS) to analyze movement-related activity in different brain regions. The results revealed prominent beta-band frequency peaks in the globus pallidus interna (GPi), ventral oralis anterior/posterior (VoaVop) subnuclei of the thalamus, and subthalamic nucleus (STN) during movement but not at rest. Connectivity analysis indicated stronger coupling between STN-VoaVop and STN-GPi compared to GPi-STN. These findings contradict the hypothesis of decreased thalamic inhibition in dystonia, suggesting that abnormal patterns of inhibition and disinhibition, rather than reduced GPi activity, contribute to the disorder. Additionally, the study implies that correcting abnormalities in GPi function may explain the effectiveness of DBS targeting the STN and GPi in treating dystonia.
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OBJECTIVE: Abdominal pseudocyst (APC) can cause distal site failure in children with ventriculoperitoneal shunts and is specifically designated as an infection in Hydrocephalus Clinical Research Network (HCRN) protocols. Specific management and outcomes of children with APCs have not been reported in a multicenter study. In this study, the authors investigated the management and outcomes of APC in children with shunted hydrocephalus who were treated at centers in the HCRN. METHODS: The HCRN Registry was queried to identify children < 18 years old with shunts who were diagnosed with an APC (i.e., a loculated abdominal fluid collection containing the peritoneal catheter with abdominal distention and/or displacement of peritoneal contents). The primary outcome was shunt failure after APC treatment. The primary variable was reimplantation of the distal catheter after pseudocyst treatment back into the peritoneum versus implantation in a nonperitoneal site. Other risk factors for shunt failure after APC treatment and variability in APC management were investigated. RESULTS: Among 141 children from 14 centers who underwent first-time management of an APC over a 14-year period, the median time from previous shunt surgery to APC diagnosis was 3.8 months. Overall, 17.7% of children had a positive culture: APC cultures were positive in 14.2% and CSF cultures in 15.6%. Six other children underwent shunt revision without removal; all underwent reoperation within 1 month. There was no difference in shunt survival (log-rank test, p = 0.42) or number of subsequent revisions within 6, 12, or 24 months for shunts reimplanted in the abdomen versus those implanted in a nonperitoneal location. Nonperitoneal implantation was associated with more noninfectious revisions (42.3% vs 22.9%, p = 0.019), whereas infection was more common after reimplantation in the abdomen (25.7% vs 7.0%, p = 0.003). Univariable analysis demonstrated that younger age at APC diagnosis (8.3 vs 12.2 years, p = 0.006) and prior shunt procedure within 12 weeks of APC diagnosis (59.5% vs 40.5%, p = 0.012) were associated with shunt failure after APC treatment. Multivariable modeling confirmed that prior shunt surgery within 12 weeks of APC diagnosis was independently associated with failure (HR 1.79 [95% CI 1.04-3.07], p = 0.035). CONCLUSIONS: In the HCRN, APCs in the setting of CSF shunts are usually managed with externalization. Shunt surgery within 12 weeks of APC diagnosis was associated with risk of failure after APC treatment. Although no differences were found in overall shunt failure rate, noninfectious shunt revisions were more common in the nonperitoneal distal catheter sites, and infection was a more common reason for failure after reimplantation of the shunt in the abdomen.
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Cistos , Hidrocefalia , Humanos , Criança , Lactente , Adolescente , Abdome/cirurgia , Derivação Ventriculoperitoneal/efeitos adversos , Procedimentos Neurocirúrgicos/efeitos adversos , Hidrocefalia/cirurgia , Hidrocefalia/complicações , Cistos/etiologia , Reoperação , Derivações do Líquido Cefalorraquidiano/efeitos adversosRESUMO
INTRODUCTION: In carefully selected patients with medically refractory epilepsy, disconnective hemispherotomy can result in significant seizure freedom; however, incomplete disconnection can result in ongoing seizures and poses a significant challenge. Completion hemispherotomy provides an opportunity to finish the disconnection. We describe the use of magnetic resonance-guided laser interstitial thermal ablation (MRgLITT) for completion hemispherotomy. METHODS: Patients treated with completion hemispherotomy using MRgLITT at our institution were identified. Procedural and seizure outcomes were evaluated retrospectively. RESULTS: Five patients (3 males) underwent six MRgLITT procedures (one child treated twice) for completion hemispherotomy at a median age of 6 years (range 1.8-12.9). Two children had hemimegalencephaly, two had Rasmussen encephalitis, and one had polymicrogyria. All five children had persistent seizures likely secondary to incomplete disconnection after their functional hemispherotomy. The mean time from open hemispherotomy to MRgLITT was 569.5 ± 272.4 days (median 424, range 342-1,095). One patient underwent stereoelectroencephalography before MRgLITT. The mean number of ablation targets was 2.3 ± 0.47 (median 2, range 2-3). The mean length of the procedure was 373 min ± 68.9 (median 374, range 246-475). Four of the five patients were afforded improvement in their neurocognitive functioning and speech performance after ablation, with mean daily seizure frequency at 1 year of 1.03 ± 1.98 (median 0, range 0-5). Two patients achieved Engel Class I outcomes at 1 year after ablation, one was Engel Class III, and two were Engel Class IV. The mean follow-up time was 646.8 ± 179.5 days (median 634, range 384-918). No MRgLITT-related complications occurred. Delayed retreatment (>1 year) occurred in three patients: one child underwent redo ablation and two underwent anatomic hemispherectomy. CONCLUSION: We have demonstrated the feasibility of a minimally invasive approach for completion hemispherotomy using MRgLITT. Delayed retreatment was needed in three patients; thus, further study of this technique with comparison to other surgical techniques is warranted.
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Epilepsia Resistente a Medicamentos , Hemisferectomia , Terapia a Laser , Criança , Masculino , Humanos , Lactente , Pré-Escolar , Estudos Retrospectivos , Resultado do Tratamento , Imageamento por Ressonância Magnética/métodos , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia Resistente a Medicamentos/cirurgia , Convulsões/cirurgia , Terapia a Laser/efeitos adversos , Hemisferectomia/efeitos adversos , Hemisferectomia/métodos , Espectroscopia de Ressonância Magnética/efeitos adversosRESUMO
OBJECTIVE: The focus of this modified Delphi study was to investigate and build consensus regarding the medical management of children with moderate and severe acute spinal cord injury (SCI) during their initial inpatient hospitalization. This impetus for the study was based on the AANS/CNS guidelines for pediatric SCI published in 2013, which indicated that there was no consensus provided in the literature describing the medical management of pediatric patients with SCIs. METHODS: An international, multidisciplinary group of 19 physicians, including pediatric neurosurgeons, orthopedic surgeons, and intensivists, were asked to participate. The authors chose to include both complete and incomplete injuries with traumatic as well as iatrogenic etiologies (e.g., spinal deformity surgery, spinal traction, intradural spinal surgery, etc.) due to the overall low incidence of pediatric SCI, potentially similar pathophysiology, and scarce literature exploring whether different etiologies of SCI should be managed differently. An initial survey of current practices was administered, and based on the responses, a follow-up survey of potential consensus statements was distributed. Consensus was defined as ≥ 80% of participants reaching agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). A final meeting was held virtually to generate final consensus statements. RESULTS: Following the final Delphi round, 35 statements reached consensus after modification and consolidation of previous statements. Statements were categorized into the following eight sections: inpatient care unit, spinal immobilization, pharmacological management, cardiopulmonary management, venous thromboembolism prophylaxis, genitourinary management, gastrointestinal/nutritional management, and pressure ulcer prophylaxis. All participants stated that they would be willing or somewhat willing to change their practices based on consensus guidelines. CONCLUSIONS: General management strategies were similar for both iatrogenic (e.g., spinal deformity, traction, etc.) and traumatic SCIs. Steroids were recommended only for injury after intradural surgery, not after acute traumatic or iatrogenic extradural surgery. Consensus was reached that mean arterial pressure ranges are preferred for blood pressure targets following SCI, with goals between 80 and 90 mm Hg for children at least 6 years of age. Further multicenter study of steroid use following acute neuromonitoring changes was recommended.
RESUMO
OBJECTIVE: Advancements in MRI technology have provided improved ways to acquire imaging data and to more seamlessly incorporate MRI into modern pediatric surgical practice. One such situation is image-guided navigation for pediatric neurosurgical procedures, including intracranial catheter placement. Image-guided surgery (IGS) requires acquisition of CT or MR images, but the former carries the risk of ionizing radiation and the latter is associated with long scan times and often requires pediatric patients to be sedated. The objective of this project was to circumvent the use of CT and standard-sequence MRI in ventricular neuronavigation by investigating the use of fast MR sequences on the basis of 3 criteria: scan duration comparable to that of CT acquisition, visualization of ventricular morphology, and image registration with surface renderings comparable to standard of care. The aim of this work was to report image development, implementation, and results of registration accuracy testing in healthy subjects. METHODS: The authors formulated 11 candidate MR sequences on the basis of the standard IGS protocol, and various scan parameters were modified, such as k-space readout direction, partial k-space acquisition, sparse sampling of k-space (i.e., compressed sensing), in-plane spatial resolution, and slice thickness. To evaluate registration accuracy, the authors calculated target registration error (TRE). A candidate sequence was selected for further evaluation in 10 healthy subjects. RESULTS: The authors identified a candidate imaging protocol, termed presurgical imaging with compressed sensing for time optimization (PICO). Acquisition of the PICO protocol takes 25 seconds. The authors demonstrated noninferior TRE for PICO (3.00 ± 0.19 mm) in comparison with the default MRI neuronavigation protocol (3.35 ± 0.20 mm, p = 0.20). CONCLUSIONS: The developed and tested sequence of this work allowed accurate intraoperative image registration and provided sufficient parenchymal contrast for visualization of ventricular anatomy. Further investigations will evaluate use of the PICO protocol as a substitute for CT and conventional MRI protocols in ventricular neuronavigation.
Assuntos
Neuronavegação , Cirurgia Assistida por Computador , Humanos , Criança , Neuronavegação/métodos , Encéfalo , Imageamento por Ressonância Magnética/métodos , Procedimentos Neurocirúrgicos/métodosRESUMO
Background: Myelomeningocele (MMC) causes significant morbidity and mortality. Efforts have been directed to correct this defect in utero. The neuropathology literature on antenatally repaired MMC and associated complications in humans is limited. Case report: A 12-day-old female, who underwent prenatal MMC repair via a two-layer closure (dural replacement patch, primary skin closure), was born at 34 weeks' gestation. Her group B streptococcus positive mother received appropriate antepartum prophylactic antibiotics. She remained stable until day 11 of life when she underwent rapid clinical deterioration. Despite aggressive intervention, she expired on day 12. Review of placental pathology showed maternal and fetal inflammatory response. Autopsy revealed Gram-positive cocci and inflammation within the basilar leptomeninges and lumbosacral region. Neural and dermal elements were present within the MMC repair. Conclusion: This case documents integration of the dermal matrix patch to neural elements, adhering the spinal cord to scar tissue, the clinical implications of which remain unclear.