ABSTRACT
Growth promoting variants in PIK3CA cause a spectrum of developmental disorders, depending on the developmental timing of the mutation and tissues involved. These phenotypically heterogeneous entities have been grouped as PIK3CA-Related Overgrowth Spectrum disorders (PROS). Deep sequencing technologies have facilitated detection of low-level mosaic, often necessitating testing of tissues other than blood. Since clinical management practices vary considerably among healthcare professionals and services across different countries, a consensus on management guidelines is needed. Clinical heterogeneity within this spectrum leads to challenges in establishing management recommendations, which must be based on patient-specific considerations. Moreover, as most of these conditions are rare, affected families may lack access to the medical expertise that is needed to help address the multi-system and often complex medical issues seen with PROS. In March 2019, macrocephaly-capillary malformation (M-CM) patient organizations hosted an expert meeting in Manchester, United Kingdom, to help address these challenges with regards to M-CM syndrome. We have expanded the scope of this project to cover PROS and developed this consensus statement on the preferred approach for managing affected individuals based on our current knowledge.
Subject(s)
Class I Phosphatidylinositol 3-Kinases/genetics , Genetic Association Studies , Genetic Predisposition to Disease , Standard of Care , Consensus Development Conferences as Topic , Diagnosis, Differential , Disease Management , Genetic Association Studies/methods , Genetic Testing , Growth Disorders/diagnosis , Growth Disorders/genetics , Growth Disorders/therapy , Humans , Phenotype , Prenatal DiagnosisABSTRACT
OBJECTIVES: Despite converging basic scientific and clinical evidence of the link between chronic pain and depression, existing therapies do not often take advantage of this overlap. Here, we provide a critical review of the literature that highlights the intersection in brain networks between chronic low back pain (CLBP) and depression and discuss findings from previous deep brain stimulation (DBS) studies for pain. Based on a multidimensional model of pain processing and the connectivity of the subgenual cingulate cortex (SCC) with areas that are implicated in both CLBP and depression, we propose a novel approach to the treatment of CLBP using DBS of the SCC. MATERIALS AND METHODS: A narrative review with literature assessment. RESULTS: CLBP is associated with a shift away from somatosensory representation toward brain regions that mediate emotional processes. There is a high degree of overlap between these regions and those involved in depression, including the anterior cingulate cortex, medial prefrontal cortex, nucleus accumbens, and amygdala. Whereas target sites from previous DBS trials for pain were not anatomically positioned to engage these areas and their associated networks, the SCC is structurally connected to all of these regions as well as others involved in mediating sensory, cognitive, and affective processing in CLBP. CONCLUSIONS: CLBP and depression share a common underlying brain network interconnected by the SCC. Current data and novel technology provide an optimal opportunity to develop clinically effective trials of SCC DBS for CLBP.
Subject(s)
Chronic Pain , Deep Brain Stimulation , Low Back Pain , Brain , Brain Mapping , Chronic Pain/therapy , Gyrus Cinguli/diagnostic imaging , Humans , Low Back Pain/diagnostic imaging , Low Back Pain/therapyABSTRACT
BACKGROUND: Tuberous sclerosis complex (TSC) is a rare genetic disorder that commonly leads to drug-resistant epilepsy in affected patients. This study aimed to determine whether the underlying genetic mutation (TSC1 vs. TSC2) predicts seizure outcomes following surgical treatments for epilepsy. METHODS: We retrospectively assessed TSC patients using the TSC Natural History Database core registry. Data review focused on outcomes in patients treated with surgical resection or vagus nerve stimulation. RESULTS: A total of 42 patients with a TSC1 mutation, and 145 patients with a TSC2 mutation, were identified. We observed a distinct clinical phenotype: children with TSC2 mutations tended to be diagnosed with TSC at a younger age than those with a TSC1 mutation (p < 0.001), were more likely to have infantile spasms (p < 0.001), and to get to surgery at a later age (p = 0.003). Among this TSC2 cohort, seizure control following resective epilepsy surgery was achieved in less than half (47%) the study sample. In contrast, patients with TSC1 mutations tended to have more favorable postsurgical outcomes; seizure control was achieved in 66% of this group. CONCLUSION: TSC2 mutations result in a more severe epilepsy phenotype that is also less responsive to resective surgery. It is important to consider this distinct clinical disposition when counseling families preoperatively with respect to seizure freedom. Larger samples are required to better characterize the independent effects of genetic mutation, infantile spasms, and duration of epilepsy as they relate to seizure control following resective or neuromodulatory epilepsy surgery.
Subject(s)
Tuberous Sclerosis , Humans , Mutation , Retrospective Studies , Seizures/genetics , Seizures/surgery , Tuberous Sclerosis/complications , Tuberous Sclerosis/genetics , Tuberous Sclerosis/surgery , Tuberous Sclerosis Complex 1 Protein/genetics , Tuberous Sclerosis Complex 2 Protein/geneticsABSTRACT
INTRODUCTION: Arachnoid membranes are well recognized as a cause of cerebrospinal fluid (CSF) flow impairment in disorders such as obstructive hydrocephalus and syringohydromyelia, but can be difficult to detect with standard noninvasive imaging techniques. True fast imaging with steady-state precession (TrueFISP) can exhibit brain pulsations and CSF dynamics with high spatiotemporal resolution. Here, we demonstrate the utility of this technique in the diagnosis and management of arachnoid membranes in the posterior fossa. CASE PRESENTATIONS: Three symptomatic children underwent cine TrueFISP imaging for suspicion of CSF membranous obstruction. Whereas standard imaging failed to or did not clearly visualize the site of an obstructive lesion, preoperative TrueFISP identified a membrane in all 3 cases. The membranes were confirmed intraoperatively, and postoperative TrueFISP helped verify adequate marsupialization and recommunication of CSF flow. Two out of the 3 cases showed a decrease in cerebellar tonsillar pulsatility following surgery. All children showed symptomatic improvement. CONCLUSION: TrueFISP is able to detect pulsatile arachnoid membranes responsible for CSF outflow obstruction that are otherwise difficult to visualize using standard imaging techniques. We advocate use of this technology in pre- and postsurgical decision-making as it provides a more representative image of posterior fossa pathology and contributes to our understanding of CSF flow dynamics. There is potential to use this technology to establish prognostic biomarkers for disorders of CSF hydrodynamics.
Subject(s)
Arnold-Chiari Malformation , Hydrocephalus , Arachnoid/diagnostic imaging , Arachnoid/surgery , Child , Humans , Hydrocephalus/diagnostic imaging , Hydrocephalus/surgery , Magnetic Resonance Imaging , Postoperative PeriodABSTRACT
INTRODUCTION: Down syndrome (DS) is the most common multiple malformation syndrome in humans and is associated with an increased risk of childhood malignancy, particularly leukemia. Incidence of brain tumors in patients with DS is limited to sporadic cases. We report the first case of a RELA fusion-positive ependymoma in a 3-year-old boy with DS. CASE PRESENTATION: Imaging prompted by new left-sided hemiparesis demonstrated an 8-cm hemorrhagic right temporal-parietal mass. Subsequent image-complete resection confirmed a RELA fusion-positive anaplastic ependymoma with 90% OLIG2 staining. Postoperatively, the patient, unfortunately, experienced fatal recurrence and drop metastases with leptomeningeal involvement. CONCLUSION: To our knowledge, this is the first reported case of a confirmed RELA fusion-positive ependymoma in a child with DS. We discuss this finding in the context of intracranial tumors in children with DS, as well as the finding of 90% positive OLIG2 expression and its potential as a prognostic marker.
Subject(s)
Brain Neoplasms , Down Syndrome , Ependymoma , Glioma , Supratentorial Neoplasms , Brain Neoplasms/diagnostic imaging , Brain Neoplasms/genetics , Brain Neoplasms/surgery , Child , Child, Preschool , Down Syndrome/complications , Ependymoma/complications , Ependymoma/diagnostic imaging , Ependymoma/genetics , Humans , Male , Transcription Factor RelAABSTRACT
The role of cervicomedullary decompression (CMD) in the care of hydrocephalic achondroplastic children who present with simultaneous foramen magnum stenosis is not well understood. We sought to determine the percentage of symptomatic achondroplastic children with foramen magnum stenosis who had stabilization or improvement in ventriculomegaly following CMD. The authors retrospectively reviewed the records of pediatric patients at Cedars-Sinai Medical Center with achondroplasia and signs of progressive ventriculomegaly who underwent CMD for symptomatic foramen magnum stenosis between the years 2000 and 2018. Clinical outcomes included changes in fontanelle characteristics, head circumference (HC) percentile, and incidence of ventriculoperitoneal (VP) shunting. Radiographic outcomes measured included changes in Evans ratio. We excluded individuals who were shunted before CMD from our study. Sixteen children presented with symptomatic foramen magnum stenosis and full anterior fontanelle or jump in the HC percentiles. Two children underwent placement of a VP shunt before decompressive surgery and were excluded from further analysis. Of the remaining 14 children who underwent CMD, 13 (93%) showed softening or flattening of their fontanelles post-operatively. Ten of these 14 children had both pre- and post-operative HC percentile records available, with 8 showing increasing HC percentiles before surgery. Seven of those eight children (88%) showed a deceleration or stabilization of HC growth velocity following decompression of the foramen magnum. Among 10 children with available pre- and post-operative brain imaging, ventricular size improved in 5 (50%), stabilized in 2 (20%), and slightly increased in 3 (30%) children after decompression. Two children (14%) required a shunt despite decompression of the foramen magnum. A significant proportion of children with concomitant signs of raised intracranial pressure or findings of progressive ventriculomegaly and foramen magnum stenosis may have improvement or stabilization of these findings following CMD. CMD may decrease the need for VP shunting and its associated complications in the select group of hydrocephalic children with achondroplasia presenting with symptomatic foramen magnum stenosis.
Subject(s)
Achondroplasia/surgery , Foramen Magnum/surgery , Hydrocephalus/surgery , Nervous System Malformations/surgery , Achondroplasia/physiopathology , Adolescent , Cephalometry/methods , Cervical Vertebrae/physiopathology , Cervical Vertebrae/surgery , Child , Child, Preschool , Constriction, Pathologic/physiopathology , Constriction, Pathologic/surgery , Cranial Fontanelles/physiopathology , Cranial Fontanelles/surgery , Female , Foramen Magnum/physiopathology , Humans , Hydrocephalus/physiopathology , Infant , Male , Nervous System Malformations/physiopathology , Spinal Cord Compression/physiopathology , Spinal Cord Compression/surgeryABSTRACT
OBJECTIVE: The purpose of this study was to establish prognostic factors in fetuses diagnosed with periventricular pseudocysts (PVPCs) without known congenital infection, between 28 and 37 weeks of gestation. METHODS: This retrospective study included cases of fetal PVPC from 2008 to 2018. PVPCs were classified according to location, number, extension, morphology, and size. Additional findings, MRI and genetic studies were recorded. Pregnancy outcome, postnatal, or postmortem results were obtained. Images from patients with normal (Group 1) and abnormal postnatal development (Group 2) were compared for analysis of factors predictive of outcome. RESULTS: One-hundred and fifteen pseudocysts were observed in 59 patients. In 34 fetuses (57%), the PVPC was an isolated finding. Thirty-nine patients delivered live newborns, 27% opted for termination of pregnancy, and 4 patients were lost to follow-up. Eighty-four percent of the liveborns had normal development. When assessing for the influence of pseudocyst characteristics, a wide CSP, or large head circumference, neither of these affected the outcome. The presence of additional anomalies was the only positive predictor for abnormal development regradless of specific PVPC characteristics (P = .002). CONCLUSIONS: In fetuses with PVPCs, the presence of additional anomalies was the only predictor for adverse postnatal outcome. No association between cystic characteristics and adverse outcome was observed.
Subject(s)
Cysts/diagnosis , Cysts/epidemiology , Nervous System Malformations/diagnosis , Nervous System Malformations/epidemiology , Adult , Cysts/congenital , Female , Fetal Diseases/diagnosis , Fetal Diseases/epidemiology , Humans , Infant, Newborn , Israel/epidemiology , Magnetic Resonance Imaging , Male , Pregnancy , Pregnancy Outcome/epidemiology , Prenatal Diagnosis/methods , Prenatal Diagnosis/statistics & numerical data , Prognosis , Retrospective Studies , Risk Factors , Ultrasonography, Prenatal , Young AdultABSTRACT
BACKGROUND: Symptomatic pneumocephalus is an uncommon complication of cranial surgery. Reports of symptomatic pneumocephalus in deep brain stimulation (DBS) surgery are lacking, due to the rarity of this condition. The -authors describe 2 patients who experienced clinically significant intraparenchymal pneumocephalus as a consequence of DBS surgery and report their clinical presentations, treatments, and outcomes. Cases Descriptions: The first patient was a 69-year-old woman with Parkinson disease and the second was a 73-year-old woman with medically refractory essential tremor. Both patients underwent DBS implantation and developed focal neurological deficits in the days after surgery. In each case, immediate postoperative head computed tomography scans showed extra-axial pneumocephalus which redistributed on subsequent imaging along the dorsal length of the lead. For each patient, a second surgery was carried out to evacuate the pneumocephalus without lead removal. Clinical symptoms and radiological signs of intracranial air were resolved on the last follow-up. CONCLUSION: Symptomatic intraparenchymal pneumocephalus is a rare complication of DBS surgery which can be treated with surgical evacuation.
Subject(s)
Deep Brain Stimulation/adverse effects , Essential Tremor/therapy , Parkinson Disease/therapy , Pneumocephalus/etiology , Aged , Deep Brain Stimulation/trends , Essential Tremor/diagnostic imaging , Female , Humans , Parkinson Disease/diagnostic imaging , Pneumocephalus/diagnostic imaging , Tomography, X-Ray Computed/trendsABSTRACT
OBJECTIVE: Deep brain stimulation (DBS) studies have shown that stimulation of the motor segment of the thalamus based on probabilistic tractography is predictive of improvement in essential tremor (ET). However, probabilistic methods are computationally demanding, requiring the need for alternative tractography methods for use in the clinical setting. The purpose of this study was to compare probabilistic vs deterministic tractography methods for connectivity-based targeting in patients with ET. METHODS: Probabilistic and deterministic tractography methods were retrospectively applied to diffusion-weighted data sets in 36 patients with refractory ET. The thalamus and precentral gyrus were selected as regions of interest and fiber tracking was performed between these regions to produce connectivity-based thalamic segmentations, per prior methods. The resultant deterministic target maps were compared with those of thresholded probabilistic maps. The center of gravity (CG) of each connectivity map was determined and the differences in spatial distribution between the tractography methods were characterized. Furthermore, the intersection between the connectivity maps and CGs with the therapeutic volume of tissue activated (VTA) was calculated. A mixed linear model was then used to assess clinical improvement in tremor with volume of overlap. RESULTS: Both tractography methods delineated the region of the thalamus with connectivity to the precentral gyrus to be within the posterolateral aspect of the thalamus. The average CG of deterministic maps was more medial-posterior in both the left (3.7 ± 1.3 mm3) and the right (3.5 ± 2.2 mm3) hemispheres when compared to 30 %-thresholded probabilistic maps. Mixed linear model showed that the volume of overlap between CGs of deterministic and probabilistic targeting maps and therapeutic VTAs were significant predictors of clinical improvement. CONCLUSIONS: Deterministic tractography can reconstruct DBS thalamic target maps in approximately 5 min comparable to those produced by probabilistic methods that require > 12 h to generate. Despite differences in CG between the methods, both deterministic-based and probabilistic targeting were predictive of clinical improvement in ET.
Subject(s)
Deep Brain Stimulation , Essential Tremor , Humans , Essential Tremor/diagnostic imaging , Essential Tremor/therapy , Deep Brain Stimulation/methods , Retrospective Studies , Thalamus/diagnostic imaging , TremorABSTRACT
BACKGROUND: Achieving safe, maximal tumor resection in gliomas can be challenging due to the tumor's intricate relationship with surrounding structures. Tubular retractors offer a minimally invasive approach, preserving functional pathways and reducing complications. To assess their efficacy and safety, we conducted a systematic review and meta-analysis. METHODS: A search across databases identified 26 studies meeting inclusion criteria, encompassing 106 patients with various glioma types and tumor locations. RESULTS: Among 26 eligible studies, 15 provided sufficient data on 106 patients (median age: 50.5 years). Glioblastoma multiforme constituted 52.4â¯% of tumors, followed by IDH-mutant astrocytomas at 31.0â¯%. Tumor locations varied, with intraventricular and thalamic involvement in 16.3â¯% (16/98) of cases, followed by temporal (12.2â¯%), frontal and occipital (each 8.16â¯%), basal ganglia (8.16â¯%), parietal (7.14â¯%), optic pathway (2.04â¯%), and caudate nucleus (1.02â¯%) involvement. VyCor and Brainpath retractors were most used (22.6â¯% and 21.7â¯%, respectively). Tubular retractors were often combined with the exoscope (35.9â¯%). Gross total resection (GTR) was achieved in 69.4â¯% of cases, near-total resection (NTR) in 5.1â¯%, and subtotal resection/partial resection (STR/PR) in 25.5â¯%. Mean extent of resection (EOR) significantly differed between GTR and STR/NTR/PR groups (p<0.001). Postoperative complications included visual deficits (6.38â¯%), hemiparesis or weakness (2.13â¯%), multiple complications (1.06â¯%), and other unspecified complications (3.19â¯%). CONCLUSION: Tubular retractors are a valuable intraoperative adjunct and component of the surgical armamentarium for glioma surgery allowing bimanual operative techniques to manage hemostasis directly with excellent surgical outcomes and an acceptable complication profile.
Subject(s)
Brain Neoplasms , Glioma , Neurosurgical Procedures , Humans , Glioma/surgery , Brain Neoplasms/surgery , Neurosurgical Procedures/methods , Surgical Instruments , Postoperative Complications/epidemiologyABSTRACT
BACKGROUND AND OBJECTIVES: ChatGPT is a natural language processing chatbot with increasing applicability to the medical workflow. Although ChatGPT has been shown to be capable of passing the American Board of Neurological Surgery board examination, there has never been an evaluation of the chatbot in triaging and diagnosing novel neurosurgical scenarios without defined answer choices. In this study, we assess ChatGPT's capability to determine the emergent nature of neurosurgical scenarios and make diagnoses based on information one would find in a neurosurgical consult. METHODS: Thirty clinical scenarios were given to 3 attendings, 4 residents, 2 physician assistants, and 2 subinterns. Participants were asked to determine if the scenario constituted an urgent neurosurgical consultation and what the most likely diagnosis was. Attending responses provided a consensus to use as the answer key. Generative pretraining transformer (GPT) 3.5 and GPT 4 were given the same questions, and their responses were compared with the other participants. RESULTS: GPT 4 was 100% accurate in both diagnosis and triage of the scenarios. GPT 3.5 had an accuracy of 92.59%, slightly below that of a PGY1 (96.3%), an 88.24% sensitivity, 100% specificity, 100% positive predictive value, and 83.3% negative predicative value in triaging each situation. When making a diagnosis, GPT 3.5 had an accuracy of 92.59%, which was higher than the subinterns and similar to resident responders. CONCLUSION: GPT 4 is able to diagnose and triage neurosurgical scenarios at the level of a senior neurosurgical resident. There has been a clear improvement between GPT 3.5 and 4. It is likely that the recent updates in internet access and directing the functionality of ChatGPT will further improve its utility in neurosurgical triage.
Subject(s)
Neurosurgery , Triage , Humans , Triage/methods , Neurosurgery/education , Natural Language Processing , Neurosurgical Procedures/methods , Internship and ResidencyABSTRACT
BACKGROUND: Cerebral hemispherectomy can effectively treat unihemispheric epilepsy. However, posthemispherectomy hydrocephalus (PHH), a serious life-long complication, remains prevalent, requiring careful considerations in technique selection and postoperative management. In 2016, we began incorporating open choroid plexus cauterization (CPC) into our institution's hemispherectomy procedure in an attempt to prevent PHH. OBJECTIVE: To determine whether routine CPC prevented PHH without exacerbating hemispherectomy efficacy or safety. METHODS: A retrospective review of consecutive patients who underwent hemispherectomy for intractable epilepsy between 2011 and 2021 was performed. Multivariate logistic regression was used to identify factors independently associated with PHH requiring cerebrospinal fluid (CSF) shunting. RESULTS: Sixty-eight patients were included in this study, of whom 26 (38.2%) underwent CPC. Fewer patients required CSF shunting in the CPC group (7.7% vs 28.7%, P = .033) and no patients who underwent de novo hemispherectomy with CPC developed PHH. Both cohorts experienced seizure freedom (65.4% vs 59.5%, P = .634) and postoperative complications, including infection (3.8% vs 2.4%, P = .728), hemorrhage (0.0% vs 2.4%, P = .428), and revision hemispherectomy (19.2% vs 14.3%, P = .591) at similar rates. Patients without CPC had greater odds of developing PHH requiring CSF shunting (odds ratio = 8.36, P = .026). The number needed to treat with CPC to prevent an additional case of PHH was 4.8, suggesting high effectiveness. CONCLUSION: Preventing PHH is critical. Our early experience demonstrated that routinely incorporating CPC into hemispherectomy effectively prevents PHH without causing additional complications, especially in first-time hemispherectomies. A multicenter randomized controlled trial with long-term follow-up is required to corroborate the findings of our single-institutional case series and determine whether greater adoption of this technique is justified.
Subject(s)
Hemispherectomy , Hydrocephalus , Humans , Infant , Choroid Plexus/surgery , Hemispherectomy/adverse effects , Ventriculostomy/methods , Hydrocephalus/etiology , Hydrocephalus/prevention & control , Hydrocephalus/surgery , Cautery/methods , Treatment OutcomeABSTRACT
Achondroplasia is the most common of skeletal dysplasias and is caused by a defect in endochondral bone formation. In addition to skeletal deformities, patients with achondroplasia possess significant abnormalities of the axial skeleton, including small skull base with a narrowed foramen magnum and small vertebral bodies with shortened pedicles. Consequently, patients with achondroplasia are at risk of several severe neurologic conditions, such as cervicomedullary compression, spinal stenosis, and hydrocephalus, which frequently require the attention of a neurosurgeon. This article provides an updated review on the neurosurgical evaluation and care of children with Achondroplasia.
Subject(s)
Achondroplasia , Hydrocephalus , Spinal Stenosis , Achondroplasia/surgery , Child , Constriction, Pathologic , Foramen Magnum , Humans , Hydrocephalus/surgery , InfantABSTRACT
In recent years, the hunt for objective biomarkers in chronic pain has intensified, as interest has grown in precision medicine techniques, and the global opioid crisis has underscored the need to accelerate the pace of pain research. A growing body of neuroimaging literature suggests that chronic pain is associated with various alterations in regional brain areas as well as whole-brain networks, which may represent unique radiological pain signatures or biomarkers to guide diagnosis, response, and treatment. Here, we provide a comprehensive and updated literature review on investigative efforts to identify neuroimaging biomarkers for pain.
Subject(s)
Chronic Pain , Magnetic Resonance Imaging , Biomarkers , Brain/diagnostic imaging , Chronic Pain/diagnostic imaging , Humans , Magnetic Resonance Imaging/methods , NeuroimagingABSTRACT
Much of the current medical discussion for within centers for skeletal dysplasia and specifically patients with achondroplasia focuses on infancy and early childhood. Most neurosurgical concerns arise due to a defect in the endochondral ossification, resulting on early fusion of the synchondrosis. As patients age, the neurosurgical focus shifts from primarily cranial to spinal concerns. Often pediatric neurosurgeons may continue to follow their patients with skeletal dysplasia. However, general adult neurosurgeons and orthopedic surgeons may see these graduated adults in their practice. This article provides a review of the common neurosurgical concerns for patients with achondroplasia.
Subject(s)
Achondroplasia , Foramen Magnum , Achondroplasia/surgery , Adult , Child , Child, Preschool , Constriction, Pathologic , Humans , InfantABSTRACT
OBJECTIVES: The aim of this study is to assess the procedure-to-delivery interval (PDI), the obstetric complications, and the early neonatal outcome in patients that did or did not receive glucocorticosteroids (GCSs) before third-trimester amniocentesis (TTA). METHODS: A retrospectively analysis of 445 TTA procedures divided into two groups based on the administration (study group = 220 patients) or not (control group = 225 patients) of GCSs before TTA. The PDI was calculated for all patients. Obstetric and neonatal outcomes were compared between the groups. RESULTS: The rate of procedure-associated complications was similar between the groups. The mean PDI was 47.2 ± 16.8 days. The overall incidence of preterm birth was 11.7%; 9% delivered between 34 and 37 weeks and 2.7% between 28 and 34 weeks. Only nine patients (2%) delivered within seven days following TTA. The incidence of respiratory distress syndrome in the study and control groups was 1.8% and 1.3%, p = .71, respectively. There were no significant differences in other neonatal outcomes in term and preterm deliveries between the study and control groups. CONCLUSIONS: In the present study, the administration of glucocorticoids prior to TTA did not reduce the rates of neonatal complications, which was similar in both groups and not higher than the general population.
Subject(s)
Premature Birth , Respiratory Distress Syndrome, Newborn , Adrenal Cortex Hormones/adverse effects , Amniocentesis/adverse effects , Female , Gestational Age , Humans , Infant, Newborn , Pregnancy , Premature Birth/epidemiology , Prenatal Care/methods , Respiratory Distress Syndrome, Newborn/epidemiology , Respiratory Distress Syndrome, Newborn/prevention & control , Retrospective StudiesABSTRACT
BACKGROUND: When evaluating deep brain stimulation (DBS) for newer indications, patients may benefit from trial stimulation prior to permanent implantation or for investigatory purposes. Although several case series have evaluated infectious complications among DBS patients who underwent trials with external hardware, outcomes have been inconsistent. OBJECTIVE: To determine whether a period of lead externalization is associated with an increased risk of infection. METHODS: We conducted a Preferred Reporting Items for Systematic Reviews and Meta-Analyses compliant systematic review of all studies that included rates of infection for patients who were externalized prior to DBS implantation. A meta-analysis of proportions was performed to estimate the pooled proportion of infection across studies, and a meta-analysis of relative risks was conducted on those studies that included a control group of nonexternalized patients. Heterogeneity across studies was assessed via I2 index. RESULTS: Our search retrieved 23 articles, comprising 1354 patients who underwent lead externalization. The pooled proportion of infection was 6.9% (95% CI: 4.7%-9.5%), with a moderate to high level of heterogeneity between studies (I2 = 62.2%; 95% CI: 40.7-75.9; P < .0001). A total of 3 studies, comprising 212 externalized patients, included a control group. Rate of infection in externalized patients was 5.2% as compared to 6.0% in nonexternalized patients. However, meta-analysis was inadequately powered to determine whether there was indeed no difference in infection rate between the groups. CONCLUSION: The rate of infection in patients with electrode externalization is comparable to that reported in the literature for DBS implantation without a trial period. Future studies are needed before this information can be confidently used in the clinical setting.
Subject(s)
Deep Brain Stimulation , Brain , Electrodes , HumansABSTRACT
Deep brain stimulation (DBS) of the ventral intermediate nucleus (VIM-DBS) is a highly successful treatment for medication-refractory essential tremor (ET). Clinical outcomes are dependent on accurate targeting. Here, we aim to develop a framework for connectivity-guided DBS targeting by evaluating probabilistic tractography and clinical response at both initial programming (IP) and clinical follow-up (CF). Magnetic resonance imaging and clinical outcomes were evaluated in 23 ET patients who were treated by VIM-DBS at the University of California Los Angeles (20 at IP, 18 at CF, 14 at both). Lead-DBS was used to model the volume of tissue activated tissue (VTA) based on programming configurations at both IP and CF. Probabilistic tractography, calculated in FSL, was used to evaluate 1) clinically weighted whole brain connectivity of VTA; 2) connectivity between VTA and freesurfer-derived target regions of interest (ROI) including primary motor, premotor, and prefrontal cortices, and cerebellum; and 3) volume of intersection between VTA and probabilistic tractography-based segmentation of the thalamus. At IP, individual contacts were scored as high or low efficacy based on acute tremor improvement. At CF, clinical response was measured by percent of change of the Clinical Rating Scale for Tremor (CRST) compared to preoperative scores. Contributions from each target ROI to clinical response was measured using logistic regression for IP and linear regression for CF. The clinically weighted map of whole brain connectivity of VTA shows preferential connectivity to precentral gyrus and brainstem/cerebellum. The volume of intersection between VTA and thalamic segmentation map based on probabilistic connectivity to primary motor cortex was a significant predictor of contact efficacy at IP (OR = 2.26 per 100 mm3 of overlap, p = .04) and percent change in CRST at CF (ß = 14.67 per 100 mm3 of overlap, p = .003). Targeting DBS to the area of thalamus most connected to primary motor cortex based on probabilistic tractography is associated with superior outcomes, providing a potential guide not only for lead targeting but also therapeutic programming.
Subject(s)
Deep Brain Stimulation , Essential Tremor , Motor Cortex , Diffusion Tensor Imaging , Essential Tremor/diagnostic imaging , Essential Tremor/therapy , Humans , Thalamus/diagnostic imaging , Treatment Outcome , TremorABSTRACT
BACKGROUND: Maximal, aggressive resection of diffuse low-grade gliomas (DLGG) is well established as the standard of care in neuro-oncology. The role of repeat resection for tumor progression is unclear. OBJECTIVE: To assess the role of repeated operation for DLGG, and the effect on malignant transformation and survival. METHODS: We conducted a historical cohort study in which all patients undergoing multiple resections of DLGG between the years 1995-2019 were evaluated for overall survival (OS) and time to transformation (TTT). We then compared the outcome of this group with that of a matched control group comprised of patients who underwent only one operation despite being eligible for repeat surgery at tumor progression, but had received non-surgical oncological therapy or declined additional treatment. RESULTS: Of 607 patients in our departmental DLGG database, 93 patients underwent 2 or more surgeries and had sufficient follow-up and imaging data to be included in the study group. Thirty-eight patients were included in the matched control group. Early (less than 1 year) progression was associated with decreased survival and shorter TTT in the study group. Patients undergoing multiple resections had significantly longer TTT and OS compared to patients who underwent a single surgery. This effect was especially noted in patients who had radiological evidence of tumor transformation. CONCLUSIONS: Repeated resections of LGG are safe and offer survival benefit in select patients. Early progression following resection is associated with worse prognosis. Patients with evidence of radiological transformation may benefit the most from re-resection.
Subject(s)
Brain Neoplasms/surgery , Glioma/surgery , Neurosurgical Procedures/adverse effects , Reoperation/adverse effects , Adult , Brain Neoplasms/mortality , Brain Neoplasms/pathology , Cell Transformation, Neoplastic/pathology , Combined Modality Therapy , Disease Progression , Female , Glioma/mortality , Glioma/pathology , Humans , Kaplan-Meier Estimate , Male , Middle Aged , Neurosurgical Procedures/methods , Prognosis , Registries , Retrospective StudiesABSTRACT
BACKGROUND: Deep brain stimulation (DBS) has been used for chronic pain for decades, but its use is limited due to a lack of reliable data about its efficacy for specific indications. OBJECTIVE: To report on 9 patients who underwent DBS for facial pain, with a focus on differences in outcomes between distinct etiologies. METHODS: We retrospectively reviewed 9 patients with facial pain who were treated with DBS of the ventral posteromedial nucleus of the thalamus and periventricular gray. We report on characteristics including facial pain etiology, complications, changes in pain scores using the visual analog scale (VAS), and willingness to undergo DBS again. RESULTS: Nine patients underwent DBS for either poststroke, post-traumatic, postherpetic, or atypical facial pain. Eight patients (89%) were permanently implanted. Seven patients had sufficient follow-up (mean 40.3 mo). Of these 7 patients, average VAS scores decreased from 9.4 to 6.1 after DBS. The average decrease in VAS was 55% for post-traumatic facial pain (2 patients), 45% for poststroke (2 patients), 15% for postherpetic neuralgia (2 patients), and 0% for atypical facial pain (1 patient). Three of the 8 implanted patients (38%) had complications which required removal of hardware. Only 2 of 7 (29%) patients met classical criteria for responders (50% decrease in pain scores). However, among 4 patients who were asked about willingness to undergo DBS again, all expressed that they would repeat the procedure. CONCLUSION: There is a trend towards improvement in pain scores following DBS for facial pain, most prominently with post-traumatic pain.