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OBJECTIVE: Intracranial complications of acute bacterial sinusitis are rare pathologies that occur in children, and are associated with significant neurological morbidity and mortality. There is a subjective concern among neurosurgeons that the incidence of this rare disease has increased since the onset of the novel COVID-19 pandemic. The primary objective of this study was to review the presentation and management of patients admitted at the authors' institution with intracranial extension of sinusitis, to better understand the local disease burden relative to the COVID-19 pandemic. METHODS: This is a single-center retrospective observational cohort study. The patients underwent neurosurgical intervention for intracranial extension of sinusitis between January 1, 2007, and March 1, 2023. The historical cohort was defined as those patients who presented prior to March 2020. Clinical covariates such as surgical and microbiological data were collected and analyzed. RESULTS: A total of 78 patients (55 historical, 23 new) were included; they had a median age of 11.7 years and a male predominance of 69.2%. There was a significant increase in the annual rate of neurosurgical intervention for suppurative intracranial extension of acute bacterial sinusitis after the onset of the COVID-19 pandemic, with an average of 4.2 cases per year prior to March 2020 compared to 7.7 cases per year after that date (p = 0.013). This increase was largely driven by the unprecedented case volume of 13 cases in 2022. Patients in the new cohort were older (p = 0.009) and more likely to have Pott's puffy tumor/frontal bone osteomyelitis (p = 0.003) at the time of presentation than patients in the historical cohort. Patients in the new cohort had lower rates of readmission within 30 days of discharge than those in the historical cohort (p = 0.047). In both cohorts, patients with seizure on presentation were more likely to have neurological sequelae at last follow-up (p = 0.004), which occurred at a median of 2.9 months after discharge. CONCLUSIONS: Clinicians encountering pediatric patients presenting with persistent symptoms of acute bacterial sinusitis must have a high index of suspicion for suppurative intracranial extension. Prompt neuroimaging and subsequent neurosurgical intervention are critical to ensure timely diagnosis and treatment. The results in this study show a significant increase in the number of neurosurgical interventions for suppurative intracranial extension of sinusitis per year after the onset of the COVID-19 pandemic. Further research is needed to understand the underlying pathophysiology of this clinical phenomenon.
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BACKGROUND: Tonic and atonic "drop attack" seizures are a classic and morbid semiology in Lennox-Gastaut syndrome, resulting in frequent injuries and emergency room visits, in addition to neurocognitive sequelae. Recent years have seen a growing interest in less invasive techniques for performing the classic surgical treatment for drop attacks in Lennox-Gastaut syndrome, that is, corpus callosotomy. OBSERVATIONS: A 5-year-old boy with Lennox-Gastaut syndrome presented for surgical evaluation. He experienced up to 20 daily tonic seizures despite multiple antiseizure medications. Preoperative imaging revealed highly abnormal anatomy with severe ventriculomegaly and thinning of the cortex and corpus callosum. Open microsurgery or an interhemispheric bimanual endoscopic approach to corpus callosotomy posed a risk for ventricular collapse and subdural hematoma, and the corpus callosum was too thin for laser ablation. A fully endoscopic transventricular "inside-out" complete corpus callosotomy was performed through a 7-mm burr hole via a single working channel without intraoperative complications. The patient continues to experience daily seizures but with a reduced frequency and intensity and a family-reported increased quality of life. LESSONS: In cases of drug-resistant tonic and atonic seizures associated with ventriculomegaly, a fully endoscopic transventricular complete corpus callosotomy can be performed safely, potentially limiting the risk of ventricular collapse and subdural bleeding. https://thejns.org/doi/10.3171/CASE24160.
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The transpalpebral approach provides a minimally invasive corridor to the anterior skull base and temporal lobe. It has been described for anterior circulation aneurysms and skull base tumors as well as more recently for resection of epileptogenic pathology in the adult population. We describe our experience using this approach in a 13-year-old adolescent boy suffering from epilepsy secondary to concomitant left temporal focal cortical dysplasia and pleomorphic xanthoastrocytoma extending throughout the amygdala with excellent results.1-5 To the best of our knowledge, this is the first published case using the transpalpebral approach for this pathology, as well for epilepsy in the pediatric population. The patient consented to the procedure and to the publication of his image.
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STUDY DESIGN: Modified Delphi consensus study. OBJECTIVE: To develop consensus-based best practices for the care of pediatric patients who have implanted programmable devices (IPDs) and require spinal deformity surgery. SUMMARY OF BACKGROUND DATA: Implanted programmable devices (IPDs) are often present in patients with neuromuscular or syndromic scoliosis who require spine surgery. Guidelines for monitoring and interrogating these devices during the peri-operative period are not available. METHODS: A panel was assembled consisting of 25 experts (i.e., spinal deformity surgeons, neurosurgeons, neuro-electrophysiologists, cardiologists, and otolaryngologists). Initial postulates were based on literature review and results from a prior survey. Postulates addressed the following IPDs: vagal nerve stimulators (VNS), programmable ventriculo-peritoneal shunts (VPS), intrathecal baclofen pumps (ITBP), cardiac pacemakers and implantable cardioverter-defibrillators (ICD), deep brain stimulators (DBS), and cochlear implants. Cardiologist and otolaryngologists participants responded only to postulates on cardiac pacemakers or cochlear implants, respectively. Consensus was defined as ≥80% agreement, items that did not reach consensus were revised and included in subsequent rounds. A total of three survey rounds and one virtual meeting were conducted. RESULTS: Consensus was reached on 39 total postulates across six IPD types. Postulates addressed general spine surgery considerations, use of intraoperative monitoring and cautery, use of magnetically-controlled growing rods (MCGRs), and use of an external remote controller to lengthen MCGRs. Across IPD types, consensus for the final postulates ranged from 94.4-100%. Overall, experts agreed that MCGRs can be surgically inserted and lengthened in patients with a variety of IPDs and provided guidance for the use of intraoperative monitoring and cautery, which varied between IPD types. CONCLUSION: Spinal deformity correction surgery often benefits from the use of intraoperative monitoring, monopolar and bipolar cautery, and MCGRs. Final postulates from this study can inform the peri- and post-operative practices of spinal deformity surgeons who treat patients with both scoliosis and IPDs. LEVEL OF EVIDENCE: V- Expert opinion.
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BACKGROUND CONTEXT: Intraoperative neurophysiological monitoring (IONM) is used to reduce the risk of spinal cord injury during pediatric spinal deformity surgery. Significant reduction and/or loss of IONM signals without immediate recovery may lead the surgeon to acutely abort the case. The timing of when monitorable signals return remains largely unknown. PURPOSE: The goal of this study was to investigate the correlation between IONM signal loss, clinical examination, and subsequent normalization of IONM signals after aborted pediatric spinal deformity surgery to help determine when it is safe to return to the operating room. STUDY DESIGN/SETTING: This is a multicenter, multidisciplinary, retrospective study of pediatric patients (<18 years old) undergoing spinal deformity surgery whose surgery was aborted due to a significant reduction or loss of IONM potentials. PATIENT SAMPLE: Sixty-six patients less than 18 years old who underwent spinal deformity surgery that was aborted due to IONM signal loss were enrolled into the study. OUTCOME MEASURES: IONM data, operative reports, and clinical examinations were investigated to determine the relationship between IONM loss, clinical examination, recovery of IONM signals, and clinical outcome. METHODS: Information regarding patient demographics, deformity type, clinical history, neurologic and ambulation status, operative details, IONM information (eg, quality of loss [SSEPs, MEPs], laterality, any recovery of signals, etc.), intraoperative wake-up test, postoperative neurologic exam, postoperative imaging, and time to return to the operating were all collected. All factors were analyzed and compared with univariate and multivariate analysis using appropriate statistical analysis. RESULTS: Sixty-six patients were enrolled with a median age of 13 years [IQR 11-14], and the most common sex was female (42/66, 63.6%). Most patients had idiopathic scoliosis (33/66, 50%). The most common causes of IONM loss were screw placement (27/66, 40.9%) followed by rod correction (19/66, 28.8%). All patients had either complete bilateral (39/66, 59.0%), partial bilateral (10/66, 15.2%) or unilateral (17/66, 25.8%) MEP loss leading to termination of the case. Overall, when patients were returned to the operating room 2 weeks postoperatively, nearly 75% (40/55) had monitorable IONM signals. Univariate analysis demonstrated that bilateral SSEP loss (p=.019), bilateral SSEP and MEP loss (p=.022) and delayed clinical neurologic recovery (p=.008) were significantly associated with having unmonitorable IONM signals at repeat surgery. Multivariate regression analysis demonstrated that delayed clinical neurologic recovery (> 72 hours) was significantly associated with unmonitorable IONM signals when returned to the operating room (p=.006). All patients ultimately made a full neurologic recovery. CONCLUSIONS: In children whose spinal deformity surgery was aborted due to intraoperative IONM loss, there was a strong correlation between combined intraoperative SSEP/MEP loss, the magnitude of IONM loss, the timing of clinical recovery, and the time of electrophysiological IONM recovery. The highest likelihood of having a prolonged postoperative neurological deficit and undetectable IONM signals upon return to the OR occurs with bilateral complete loss of SSEPs and MEPs.
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Monitorização Neurofisiológica Intraoperatória , Humanos , Monitorização Neurofisiológica Intraoperatória/métodos , Criança , Feminino , Masculino , Adolescente , Estudos Retrospectivos , Traumatismos da Medula Espinal/cirurgia , Pré-Escolar , Recuperação de Função Fisiológica , Escoliose/cirurgiaRESUMO
While recent technological advancements are reshaping the landscape of surgical epilepsy management, the established techniques of resective and disconnective surgeries guided by electrographic monitoring remain the workhorse interventions for the management of refractory seizures and have the highest likelihood of achieving complete seizure resolution. Here we discuss examples of recent developments in surgical approaches and techniques for resective and disconnective surgeries with discussion of their indications and potential advantages.
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Procedimentos Neurocirúrgicos , Criança , Humanos , Epilepsia Resistente a Medicamentos/cirurgia , Eletroencefalografia , Epilepsia/cirurgia , Procedimentos Neurocirúrgicos/métodosRESUMO
Trans-sylvian peri-insular hemispherotomy represents a functional hemispherectomy with minimal brain removal used for treatment of refractory hemispheric epilepsy.1 Exposure for this procedure is achieved by craniotomy. Refinement in the hemispherotomy technique, including trends toward minimizing cortical resection, has contributed to a substantial drop in complication rates.2 We present a refinement of this technique, allowing for complete hemispheric disconnection through a single burr hole. In this instance, this technique was applied in the case of a 4-year-old girl who presented with medically refractory epilepsy, which had developed on the first day of life due to a perinatal incomplete left middle cerebral artery stroke. Postoperatively, the patient experienced no worsening of her preexisting right-sided hemiparesis and remains seizure-free 18 months postoperatively, now off medication. While the trans-sylvian peri-insular hemispherotomy represents an established surgical technique, this is the first report of this procedure performed in a minimally invasive fashion through a single burr hole. Beyond the minimal incision and small aperture in the skull, seldom appreciated nuances of hemispheric disconnection are described and demonstrated, including amygdala disconnection, hippocampal tail disconnection directly into splenium disconnection, concomitant intermediate disconnection and callosotomy, and frontobasal disconnection landmarks. Consent was obtained from the patient's parents for the surgical procedure, use of outcome videos, and for publication of this video and associated materials. The participants and patient's parents consented to publication of their images and that of the patient.
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PURPOSE: Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) represent a rare pediatric-type tumor that most commonly presents as medically refractory epilepsy. PLNTY has only recently been recognized as a distinct clinical entity, having been first described in 2016 and added to the World Health Organization classification of CNS tumors in 2021. Molecular studies have determined that PLNTY is uniformly driven by aberrant MAPK pathway activation, with most tumors carrying either a BRAF V600E mutation or activating FGFR2 or FGFR3 fusion protein. Although it is known that these driver mutations are mutually exclusive, little is known about differences in clinical presentation or treatment outcomes between PLNTY cases driven by these distinct mutations. METHODS: We performed a systematic review and cumulative analysis of PLNTY cases to assess whether or not PLNTY tumors carrying the BRAF V600E mutation exhibit different clinical behaviors. By searching the literature for all cases of PLNTY wherein BRAF V600E status was characterized, we compiled a dataset of 62 unique patient instances. Using a logistic regression-based approach, we assessed a primary outcome of what factors of a clinical presentation were associated with BRAF V600E mutations and a secondary outcome of what factors predicted total seizure freedom post-surgical resection. RESULTS: PLNTY cases carrying BRAF V600E mutations in the literature were strongly positively associated with adult patients (p = 0.0055, OR = 6.556; 95% Conf. Int. = 1.737-24.742). BRAF V600E status was also positively associated with tumor involvement of the temporal lobe (p = 0.0046, OR = 11.036; 95% Conf. Int. = 2.100-58.006). Male sex was also positively associated with BRAF V600E status, but the result did not quite achieve statistical significance (p = 0.0731). BRAF V600E status was not found to be associated with post-operative seizure freedom. CONCLUSIONS: These findings indicate that BRAF V600E-positive PLNTY exhibit characteristic clinical presentations but are not necessarily different in treatment responsiveness. Non-BRAF V600E tumors are more commonly associated with young patients.
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Neoplasias Encefálicas , Neoplasias Neuroepiteliomatosas , Proteínas Proto-Oncogênicas B-raf , Criança , Humanos , Masculino , Neoplasias Encefálicas/patologia , Mutação , Neoplasias Neuroepiteliomatosas/genética , Proteínas Proto-Oncogênicas B-raf/genética , Convulsões/complicaçõesRESUMO
Middle meningeal artery (MMA) embolization has gained acceptance as a treatment for chronic subdural hematoma (cSDH) in adult patients but has not been well described in pediatric patients. Standard cSDH treatment has historically consisted of burr hole drainage with or without subdural drain placement. However, due to the high rate of recurrence and frequency of comorbidities within this population, as both pediatric and adult patients with cSDH frequently have concurrent cardiac disease and a need for anticoagulant therapies, MMA embolization has increasingly demonstrated its value as both an adjunctive and primary treatment. In this report, the authors present 3 cases of successful MMA embolization in medically complex children at a single institution. MMA embolization was used as a primary treatment modality and as an adjunctive therapy in the acute setting following surgical hematoma evacuation. Two patients were receiving anticoagulation treatment requiring reversal. Technical considerations specific to the pediatric population as well as those common to both the pediatric and adult populations are addressed. Further work is needed to define the optimal indications and outcomes for MMA embolization in children with cSDH.
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Embolização Terapêutica , Hematoma Subdural Crônico , Adulto , Humanos , Criança , Hematoma Subdural Crônico/diagnóstico por imagem , Hematoma Subdural Crônico/cirurgia , Artérias Meníngeas/diagnóstico por imagem , Artérias Meníngeas/cirurgia , Trepanação , DrenagemRESUMO
OBJECTIVE: The authors of this study evaluated the safety and efficacy of stereotactic laser ablation (SLA) for the treatment of drug-resistant epilepsy (DRE) in children. METHODS: Seventeen North American centers were enrolled in the study. Data for pediatric patients with DRE who had been treated with SLA between 2008 and 2018 were retrospectively reviewed. RESULTS: A total of 225 patients, mean age 12.8 ± 5.8 years, were identified. Target-of-interest (TOI) locations included extratemporal (44.4%), temporal neocortical (8.4%), mesiotemporal (23.1%), hypothalamic (14.2%), and callosal (9.8%). Visualase and NeuroBlate SLA systems were used in 199 and 26 cases, respectively. Procedure goals included ablation (149 cases), disconnection (63), or both (13). The mean follow-up was 27 ± 20.4 months. Improvement in targeted seizure type (TST) was seen in 179 (84.0%) patients. Engel classification was reported for 167 (74.2%) patients; excluding the palliative cases, 74 (49.7%), 35 (23.5%), 10 (6.7%), and 30 (20.1%) patients had Engel class I, II, III, and IV outcomes, respectively. For patients with a follow-up ≥ 12 months, 25 (51.0%), 18 (36.7%), 3 (6.1%), and 3 (6.1%) had Engel class I, II, III, and IV outcomes, respectively. Patients with a history of pre-SLA surgery related to the TOI, a pathology of malformation of cortical development, and 2+ trajectories per TOI were more likely to experience no improvement in seizure frequency and/or to have an unfavorable outcome. A greater number of smaller thermal lesions was associated with greater improvement in TST. Thirty (13.3%) patients experienced 51 short-term complications including malpositioned catheter (3 cases), intracranial hemorrhage (2), transient neurological deficit (19), permanent neurological deficit (3), symptomatic perilesional edema (6), hydrocephalus (1), CSF leakage (1), wound infection (2), unplanned ICU stay (5), and unplanned 30-day readmission (9). The relative incidence of complications was higher in the hypothalamic target location. Target volume, number of laser trajectories, number or size of thermal lesions, or use of perioperative steroids did not have a significant effect on short-term complications. CONCLUSIONS: SLA appears to be an effective and well-tolerated treatment option for children with DRE. Large-volume prospective studies are needed to better understand the indications for treatment and demonstrate the long-term efficacy of SLA in this population.
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The molecular diversity of glia in the human hippocampus and their temporal dynamics over the lifespan remain largely unknown. Here, we performed single-nucleus RNA sequencing to generate a transcriptome atlas of the human hippocampus across the postnatal lifespan. Detailed analyses of astrocytes, oligodendrocyte lineages, and microglia identified subpopulations with distinct molecular signatures and revealed their association with specific physiological functions, age-dependent changes in abundance, and disease relevance. We further characterized spatiotemporal heterogeneity of GFAP-enriched astrocyte subpopulations in the hippocampal formation using immunohistology. Leveraging glial subpopulation classifications as a reference map, we revealed the diversity of glia differentiated from human pluripotent stem cells and identified dysregulated genes and pathological processes in specific glial subpopulations in Alzheimer's disease (AD). Together, our study significantly extends our understanding of human glial diversity, population dynamics across the postnatal lifespan, and dysregulation in AD and provides a reference atlas for stem-cell-based glial differentiation.
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Doença de Alzheimer , Transcriptoma , Humanos , Transcriptoma/genética , Longevidade/genética , Neuroglia/patologia , Hipocampo , Astrócitos/patologia , Doença de Alzheimer/patologiaRESUMO
Heterozygous pathogenic variants in DNM1 cause developmental and epileptic encephalopathy (DEE) as a result of a dominant-negative mechanism impeding vesicular fission. Thus far, pathogenic variants in DNM1 have been studied with a canonical transcript that includes the alternatively spliced exon 10b. However, after performing RNA sequencing in 39 pediatric brain samples, we find the primary transcript expressed in the brain includes the downstream exon 10a instead. Using this information, we evaluated genotype-phenotype correlations of variants affecting exon 10a and identified a cohort of eleven previously unreported individuals. Eight individuals harbor a recurrent de novo splice site variant, c.1197-8G>A (GenBank: NM_001288739.1), which affects exon 10a and leads to DEE consistent with the classical DNM1 phenotype. We find this splice site variant leads to disease through an unexpected dominant-negative mechanism. Functional testing reveals an in-frame upstream splice acceptor causing insertion of two amino acids predicted to impair oligomerization-dependent activity. This is supported by neuropathological samples showing accumulation of enlarged synaptic vesicles adherent to the plasma membrane consistent with impaired vesicular fission. Two additional individuals with missense variants affecting exon 10a, p.Arg399Trp and p.Gly401Asp, had a similar DEE phenotype. In contrast, one individual with a missense variant affecting exon 10b, p.Pro405Leu, which is less expressed in the brain, had a correspondingly less severe presentation. Thus, we implicate variants affecting exon 10a as causing the severe DEE typically associated with DNM1-related disorders. We highlight the importance of considering relevant isoforms for disease-causing variants as well as the possibility of splice site variants acting through a dominant-negative mechanism.
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Encefalopatias , Dinaminas , Síndromes Epilépticas , Humanos , Encefalopatias/genética , Causalidade , Dinaminas/genética , Éxons/genética , Heterozigoto , Mutação/genética , Síndromes Epilépticas/genéticaRESUMO
OBJECTIVE: Stereoelectroencephalography (SEEG) is a widely used technique for localizing seizure onset zones prior to resection. However, its use has traditionally been avoided in children under 2 years of age because of concerns regarding pin fixation in the immature skull, intraoperative and postoperative electrode bolt security, and stereotactic registration accuracy. In this retrospective study, the authors describe their experience using SEEG in patients younger than 2 years of age, with a focus on the procedure's safety, feasibility, and accuracy as well as surgical outcomes. METHODS: A retrospective review of children under 2 years of age who had undergone SEEG while at Children's Hospital of Philadelphia between November 2017 and July 2021 was performed. Data on clinical characteristics, surgical procedure, imaging results, electrode accuracy measurements, and postoperative outcomes were examined. RESULTS: Five patients younger than 2 years of age underwent SEEG during the study period (median age 20 months, range 17-23 months). The mean age at seizure onset was 9 months. Developmental delay was present in all patients, and epilepsy-associated genetic diagnoses included tuberous sclerosis (n = 1), KAT6B (n = 1), and NPRL3 (n = 1). Cortical lesions included tubers from tuberous sclerosis (n = 1), mesial temporal sclerosis (n = 1), and cortical dysplasia (n = 3). The mean number of placed electrodes was 11 (range 6-20 electrodes). Bilateral electrodes were placed in 1 patient. Seizure onset zones were identified in all cases. There were no SEEG-related complications, including skull fracture, electrode misplacement, hemorrhage, infection, cerebrospinal fluid leakage, electrode pullout, neurological deficit, or death. The mean target point error for all electrodes was 1.0 mm. All patients proceeded to resective surgery, with a mean follow-up of 21 months (range 8-53 months). All patients attained a favorable epilepsy outcome, including Engel class IA (n = 2), IC (n = 1), ID (n = 1), and IIA (n = 1). CONCLUSIONS: SEEG can be safely, accurately, and effectively utilized in children under age 2 with good postoperative outcomes using standard SEEG equipment. With minimal modification, this procedure is feasible in those with immature skulls and guides the epilepsy team's decision-making for early and optimal treatment of refractory epilepsy through effective localization of seizure onset zones.
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Epilepsia Resistente a Medicamentos , Epilepsia , Esclerose Tuberosa , Criança , Pré-Escolar , Epilepsia Resistente a Medicamentos/cirurgia , Eletrodos Implantados , Eletroencefalografia/métodos , Epilepsia/diagnóstico por imagem , Epilepsia/cirurgia , Proteínas Ativadoras de GTPase , Histona Acetiltransferases , Humanos , Lactente , Estudos Retrospectivos , Convulsões/cirurgia , Técnicas Estereotáxicas , Esclerose Tuberosa/cirurgiaRESUMO
Immature dentate granule cells (imGCs) arising from adult hippocampal neurogenesis contribute to plasticity and unique brain functions in rodents1,2 and are dysregulated in multiple human neurological disorders3-5. Little is known about the molecular characteristics of adult human hippocampal imGCs, and even their existence is under debate1,6-8. Here we performed single-nucleus RNA sequencing aided by a validated machine learning-based analytic approach to identify imGCs and quantify their abundance in the human hippocampus at different stages across the lifespan. We identified common molecular hallmarks of human imGCs across the lifespan and observed age-dependent transcriptional dynamics in human imGCs that suggest changes in cellular functionality, niche interactions and disease relevance, that differ from those in mice9. We also found a decreased number of imGCs with altered gene expression in Alzheimer's disease. Finally, we demonstrated the capacity for neurogenesis in the adult human hippocampus with the presence of rare dentate granule cell fate-specific proliferating neural progenitors and with cultured surgical specimens. Together, our findings suggest the presence of a substantial number of imGCs in the adult human hippocampus via low-frequency de novo generation and protracted maturation, and our study reveals their molecular properties across the lifespan and in Alzheimer's disease.
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Envelhecimento , Hipocampo , Longevidade , Neurogênese , Neurônios , Adulto , Envelhecimento/genética , Doença de Alzheimer/genética , Doença de Alzheimer/metabolismo , Doença de Alzheimer/patologia , Animais , Proliferação de Células , Giro Denteado/citologia , Giro Denteado/patologia , Perfilação da Expressão Gênica , Hipocampo/citologia , Hipocampo/patologia , Humanos , Longevidade/genética , Aprendizado de Máquina , Camundongos , Células-Tronco Neurais/citologia , Células-Tronco Neurais/metabolismo , Células-Tronco Neurais/patologia , Neurogênese/genética , Neurônios/citologia , Neurônios/metabolismo , Neurônios/patologia , Reprodutibilidade dos Testes , Análise de Sequência de RNA , Análise de Célula Única , Transcrição GênicaRESUMO
PURPOSE: Ventriculoperitoneal (VP) shunt placement is a common neurosurgical procedure performed in patients with early onset scoliosis (EOS). To provide insight into the risks of spine lengthening operations, we investigate the rate of VP shunt complications in patients with EOS undergoing spinal deformity correction interventions. METHODS: A retrospective review was performed of all patients with EOS at a single institution undergoing spinal deformity correction procedures from 2007 to 2018. Patients having undergone VP shunt implantation prior to deformity correction were included. A minimum of 2-year follow-up was required for inclusion. Clinical records and imaging studies were reviewed. RESULTS: Nineteen patients with VP shunts underwent Vertical Expandable Prosthetic Titanium Rib (VEPTR) implantation for treatment of early onset spinal deformity. The mean age at shunt placement and spine instrumentation surgery was 13.7 months (1 day to 13 years) and 6.1 years (0.5-15.1) respectively. The diagnoses associated with shunt implantation were: 12 spina bifida, 3 structural defects or obstructions, 2 intraventricular hemorrhage, 1 cerebral palsy, and 1 campomelic dwarfism. During the first 2 years following rib-based insertion, there was a mean of 2.5 expansion/revision procedures (0-5) with no shunt-related complications. The mean length of follow-up in this series was 7.0 years (2.6-13.2). A total of three (16%) patients required shunt revision following their rib-based device insertion, two patients with proximal shunt malfunctions and one with a mid-catheter breakage, at 2.4, 2.6, and 5.6 years, respectively, after rod implantation (Fig. 2). Each of these shunt revisions occurred more than 50 days following an expansion procedure (1.9, 2.9, and 5.7 months, respectively). CONCLUSION: Growing instrumentation procedures in EOS are associated with low risk for post-operative shunt complications in patients with ventriculoperitoneal shunts. There were no shunt revision procedures performed in the first 2 years following rib-based device insertion. Sixteen percent of patients went on to require a shunt revision at some point during their follow-up, which is comparable to the baseline rate of shunt revision in non-EOS patients. LEVEL OF EVIDENCE: IV, Case series.
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Escoliose , Derivação Ventriculoperitoneal , Humanos , Derivação Ventriculoperitoneal/efeitos adversos , Derivação Ventriculoperitoneal/métodos , Titânio , Resultado do Tratamento , Escoliose/cirurgia , Escoliose/etiologia , Estudos Retrospectivos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgiaRESUMO
Stereoelectroencephalography (SEEG) is an increasingly popular invasive monitoring approach to epilepsy surgery in patients with drug-resistant epilepsies. The technique allows a three-dimensional definition of the epileptogenic zones (EZ) in the brain. It has been shown to be safe and effective in adults and older children but has been used sparingly in children less than two years old due to concerns about pin fixation in thin bone, registration accuracy, and bolt security. As such, most current series of pediatric invasive EEG explorations do not include young participants, and, when they do, SEEG is often not utilized for these patients. Recent national survey data further suggests SEEG is infrequently utilized in very young patients. We present a novel case of SEEG used to localize the EZ in a 17-month-old patient with thin cranial bone, an open fontanelle, and severe drug-resistant epilepsy due to tuberous sclerosis complex (TSC), with excellent accuracy, surgical results, and seizure remission.
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OBJECTIVE: Single-ventricle congenital heart disease (CHD) in pediatric patients with Glenn and Fontan physiology represents a unique physiology requiring the surgical diversion of the systemic venous return from the superior vena cava (Glenn) and then the inferior vena cava (Fontan) directly to the pulmonary arteries. Because many of these patients are on chronic anticoagulation therapy and may have right-to-left shunts, arrhythmias, or lymphatic disorders that predispose them to bleeding and/or clotting, they are at risk of experiencing neurological injury requiring intubation and positive pressure ventilation, which can significantly hamper pulmonary blood flow and cardiac output. The aim of this study was to describe the complex neurological and cardiopulmonary interactions of these pediatric patients after acute central nervous system (CNS) injury. METHODS: The authors retrospectively analyzed the records of pediatric patients who had been admitted to a quaternary children's hospital with CHD palliated to bidirectional Glenn (BDG) or Fontan circulation and acute CNS injury and who had undergone intubation and mechanical ventilation. Patients who had been admitted from 2005 to 2019 were included in the study. Clinical characteristics, surgical outcomes, cardiovascular and pulmonary data, and intracranial pressure data were collected and analyzed. RESULTS: Nine pediatric single-ventricle patients met the study inclusion criteria. All had undergone the BDG procedure, and the majority (78%) were status post Fontan palliation. The mean age was 7.4 years (range 1.3-17.3 years). At the time of acute CNS injury, which included traumatic brain injury, intracranial hemorrhage, and cerebral infarct, the median time interval from the most recent cardiac surgical procedure was 3 years (range 2 weeks-11 years). Maintaining normocarbia to mild hypercarbia for most patients during intubation periods did not cause neurological deterioration, and hemodynamic profiles were more favorable as compared to periods of hypocarbia. Hypocarbia was associated with unfavorable hemodynamics but was necessary to decrease intracranial hypertension. Most patients were managed using low mean airway pressure (MAWP) in order to minimize the impact on preload and cardiac output. CONCLUSIONS: The authors highlight the complex neurological and cardiopulmonary interactions with respect to partial pressure of arterial CO2 (PaCO2) and MAWP when pediatric CHD patients with single-ventricle physiology require mechanical ventilation. The study data demonstrated that tight control of PaCO2 and minimizing MAWP with the goal of early extubation may be beneficial in this population. A multidisciplinary team of pediatric critical care intensivists, cardiac intensivists and anesthesiologists, and pediatric neurosurgeons and neurologists are recommended to ensure the best possible outcomes.