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1.
Clin Neurophysiol ; 163: 102-111, 2024 Apr 30.
Article in English | MEDLINE | ID: mdl-38729074

ABSTRACT

OBJECTIVE: We investigated the role of transverse temporal gyrus and adjacent cortex (TTG+) in facial expressions and perioral movements. METHODS: In 31 patients undergoing stereo-electroencephalography monitoring, we describe behavioral responses elicited by electrical stimulation within the TTG+. Task-induced high-gamma modulation (HGM), auditory evoked responses, and resting-state connectivity were used to investigate the cortical sites having different types of responses on electrical stimulation. RESULTS: Changes in facial expressions and perioral movements were elicited on electrical stimulation within TTG+ in 9 (29%) and 10 (32%) patients, respectively, in addition to the more common language responses (naming interruptions, auditory hallucinations, paraphasic errors). All functional sites showed auditory task induced HGM and evoked responses validating their location within the auditory cortex, however, motor sites showed lower peak amplitudes and longer peak latencies compared to language sites. Significant first-degree connections for motor sites included precentral, anterior cingulate, parahippocampal, and anterior insular gyri, whereas those for language sites included posterior superior temporal, posterior middle temporal, inferior frontal, supramarginal, and angular gyri. CONCLUSIONS: Multimodal data suggests that TTG+ may participate in auditory-motor integration. SIGNIFICANCE: TTG+ likely participates in facial expressions in response to emotional cues during an auditory discourse.

2.
Childs Nerv Syst ; 2024 Mar 15.
Article in English | MEDLINE | ID: mdl-38489033

ABSTRACT

PURPOSE: Rasmussen encephalitis (RE) is a very rare chronic neurological disorder of unilateral inflammation of the cerebral cortex. Hemispherotomy provides the best chance at achieving seizure freedom in RE patients, but with significant risks and variable long-term outcomes. The goal of this study is to utilize our multicenter pediatric cohort to characterize if differences in pathology and/or imaging characterization of RE may provide a window into post-operative seizure outcomes, which in turn could guide decision-making for parents and healthcare providers. METHODS: This multi-institutional retrospective review of medical record, imaging, and pathology samples was approved by each individual institution's review board. Data was collected from all known pediatric cases of peri-insular functional hemispherotomy from the earliest available electronic medical records. Mean follow-up time was 4.9 years. Clinical outcomes were measured by last follow-up visit using both Engel and ILAE scoring systems. Relationships between categorical and continuous variables were analyzed with Pearson correlation values. RESULTS: Twenty-seven patients met study criteria. No statistically significant correlations existed between patient imaging and pathology data. Pathology stage, MRI brain imaging stages, and a combined assessment of pathology and imaging stages showed no statistically significant correlation to post-operative seizure freedom rates. Hemispherectomy Outcome Prediction Scale scoring demonstrated seizure freedom in only 71% of patients receiving a score of 1 and 36% of patients receiving a score of 2 which were substantially lower than predicted. CONCLUSIONS: Our analysis did not find evidence for either independent or combined analysis of imaging and pathology staging being predictive for post peri-insular hemispherotomy seizure outcomes, prompting the need for other biomarkers to be explored. Our data stands in contrast to the recently proposed Hemispherectomy Outcome Prediction Scale and does not externally validate this metric for an RE cohort.

4.
Eur J Neurol ; 30(10): 2986-2998, 2023 10.
Article in English | MEDLINE | ID: mdl-37329329

ABSTRACT

BACKGROUND AND PURPOSE: We analyzed the association of neuropsychological outcomes after epilepsy surgery with the intracranial electrode type (stereo electroencephalography [SEEG] and subdural electrodes [SDE]), and electrical stimulation mapping (ESM) of speech/language. METHODS: Drug-resistant epilepsy patients who underwent comprehensive neuropsychological evaluation before and 1 year after epilepsy surgery were included. SEEG and SDE subgroups were matched by age, handedness, operated hemisphere, and seizure freedom. Postsurgical neuropsychological outcomes (adjusted for presurgical scores) and reliable change indices were analyzed as functions of electrode type and ESM. RESULTS: Ninety-nine patients aged 6-29 years were included with similar surgical resection/ablation volumes in the SEEG and SDE subgroups. Most of the neuropsychological outcomes were comparable between SEEG and SDE subgroups; however, Working Memory and Processing Speed were significantly improved in the SEEG subgroup. Undergoing language ESM was associated with significant improvements in Spelling, Letter-Word Identification, Vocabulary, Verbal Comprehension, Verbal Learning, and Story Memory scores, but a decline in Calculation scores. CONCLUSIONS: Intracranial evaluations with SEEG and SDE are comparable in terms of long-term postsurgical neuropsychological outcomes. Our data suggest that SEEG may be associated with improvements in working memory and processing speed, representing cognitive domains served by spatially distributed networks. Our study also supports wider use of language ESM before epilepsy surgery, preferably using other language tasks in addition to visual naming. Rather than the type of electrode, postsurgical neuropsychological outcomes are driven by whether language ESM was performed or not, with beneficial effects of language mapping.


Subject(s)
Drug Resistant Epilepsy , Epilepsy , Humans , Stereotaxic Techniques , Electrodes, Implanted , Electroencephalography , Epilepsy/surgery , Drug Resistant Epilepsy/surgery
5.
Epilepsia ; 64(6): 1527-1540, 2023 06.
Article in English | MEDLINE | ID: mdl-36872854

ABSTRACT

OBJECTIVE: Electrical stimulation mapping (ESM) is the clinical standard for functional localization with subdural electrodes (SDE). As stereoelectroencephalography (SEEG) has emerged as an alternative option, we compared functional responses, afterdischarges (ADs), and unwanted ESM-induced seizures (EISs) between the two electrode types. METHODS: Incidence and current thresholds for functional responses (sensory, motor, speech/language), ADs, and EISs were compared between SDE and SEEG using mixed models incorporating relevant covariates. RESULTS: We identified 67 SEEG ESM and 106 SDE ESM patients (7207 and 4980 stimulated contacts, respectively). We found similar incidence of language and motor responses between electrode types; however, more SEEG patients reported sensory responses. ADs and EISs occurred less commonly with SEEG than SDE. Current thresholds for language, face motor, and upper extremity (UE) motor responses and EIS significantly decreased with age. However, they were not affected by electrode type, premedication, or dominant hemispheric stimulation. AD thresholds were higher with SEEG than with SDE. For SEEG ESM, language thresholds remained below AD thresholds up to 26 years of age, whereas this relationship was inverse for SDE. Also, face and UE motor thresholds fell below AD thresholds at earlier ages for SEEG than SDE. AD and EIS thresholds were not affected by premedication. SIGNIFICANCE: SEEG and SDE have clinically relevant differences for functional brain mapping with electrical stimulation. Although evaluation of language and motor regions is comparable between SEEG and SDE, SEEG offers a higher likelihood of identifying sensory areas. A lower incidence of ADs and EISs, and a favorable relationship between functional and AD thresholds suggest superior safety and neurophysiologic validity for SEEG ESM than SDE ESM.


Subject(s)
Drug Resistant Epilepsy , Electroencephalography , Humans , Electroencephalography/adverse effects , Electrodes, Implanted , Stereotaxic Techniques , Seizures , Brain Mapping , Electric Stimulation
6.
Neurocrit Care ; 39(3): 655-668, 2023 Dec.
Article in English | MEDLINE | ID: mdl-36539593

ABSTRACT

BACKGROUND: Spreading depolarizations (SDs) can be viewed at a cellular level using calcium imaging (CI), but this approach is limited to laboratory applications and animal experiments. Optical intrinsic signal imaging (OISI), on the other hand, is amenable to clinical use and allows viewing of large cortical areas without contrast agents. A better understanding of the behavior of OISI-observed SDs under different brain conditions is needed. METHODS: We performed simultaneous calcium and OISI of SDs in GCaMP6f mice. SDs propagate through the cortex as a pathological wave and trigger a neurovascular response that can be imaged with both techniques. We imaged both mechanically stimulated SDs (sSDs) in healthy brains and terminal SDs (tSDs) induced by system hypoxia and cardiopulmonary failure. RESULTS: We observed a lag in the detection of SDs in the OISI channels compared with CI. sSDs had a faster velocity than tSDs, and tSDs had a greater initial velocity for the first 400 µm when observed with CI compared with OISI. However, both imaging methods revealed similar characteristics, including a decrease in the sSD (but not tSD) velocities as the wave moved away from the site of initial detection. CI and OISI also showed similar spatial propagation of the SD throughout the image field. Importantly, only OISI allowed regional ischemia to be detected before tSDs occurred. CONCLUSIONS: Altogether, data indicate that monitoring either neural activity or intrinsic signals with high-resolution optical imaging can be useful to assess SDs, but OISI may be a clinically applicable way to predict, and therefore possibly mitigate, hypoxic-ischemic tSDs.


Subject(s)
Cortical Spreading Depression , Mice , Animals , Cortical Spreading Depression/physiology , Calcium Channels , Calcium , Brain , Ischemia
7.
Seizure ; 102: 105-112, 2022 Nov.
Article in English | MEDLINE | ID: mdl-36219913

ABSTRACT

OBJECTIVE: Seizures are known to occur with diurnal and other rhythms. To gain insight into the neurophysiology of periodicity of seizures, we tested the hypothesis that intracranial high-frequency oscillations (HFOs) show diurnal rhythms and sleep-wake cycle variation. We further hypothesized that HFOs have different rhythms within and outside the seizure-onset zone (SOZ). METHODS: In drug-resistant epilepsy patients undergoing stereotactic-EEG (SEEG) monitoring to localize SOZ, we analyzed the number of 50-200 Hz HFOs/channel/minute (HFO density) through a 24-hour period. The distribution of HFO density during the 24-hour period as a function of the clock time was analyzed with cosinor model, and for non-uniformity with the sleep-wake cycle. RESULTS: HFO density showed a significant diurnal rhythm overall and both within and outside SOZ. This diurnal rhythm of HFO density showed significantly lower amplitude and longer acrophase within SOZ compared to outside SOZ. The peaks of difference in HFO density within and outside SOZ preceded the seizures by approximately 4 hours. The difference in HFO density within and outside SOZ also showed a non-uniform distribution as a function of sleep-wake cycle, with peaks at first hour after arousal and ±2 hours around sleep onset. CONCLUSIONS: Our study shows that the diurnal rhythm of intracranial HFOs is more robust outside the SOZ. This suggests cortical tissue within SOZ generates HFOs relatively more uniformly throughout the day with attenuation of expected diurnal rhythm. The difference in HFO density within and outside SOZ also showed non-uniform distribution according to clock times and the sleep-wake cycle, which can be a potential biomarker for preferential times of pathological cortical excitability. A temporal correlation with seizure occurrence further substantiates this hypothesis.


Subject(s)
Drug Resistant Epilepsy , Electroencephalography , Humans , Circadian Rhythm , Seizures , Sleep
8.
Oper Neurosurg (Hagerstown) ; 23(6): 449-456, 2022 12 01.
Article in English | MEDLINE | ID: mdl-36227204

ABSTRACT

BACKGROUND: Posterior quadrant disconnection (PQD) has been described as a treatment for patients with refractory posterior quadrant subhemispheric epilepsy. Surgical outcomes are difficult to interpret because of limited literature. OBJECTIVE: To provide insight regarding the operative technique and postsurgical seizure freedom in young pediatric patients who underwent surgical disconnection for the treatment of posterior quadrant subhemispheric epilepsy at our institution. METHODS: The authors retrospectively analyzed a series of 5 patients who underwent PQD between 2019 and 2021. Charts were reviewed for preoperative workup including noninvasive/invasive testing, operative reports, and postoperative follow-up data which included degree of seizure freedom, completion of disconnection, and complications. RESULTS: Five patients were included in this series. The median age at seizure onset was 12 months (range 3-24 months), and the median age at surgery was 36 months (range 22-72 months). Histopathology confirmed focal cortical dysplasia in 3 of 5 patients (2 patients with type IB; 1 with type IIID). The average length of follow-up after surgery was 16.8 months (range 12-24 months). All patients underwent complete disconnection of the posterior quadrant without complications. Four of 5 patients (80%) had Engel score of I, while the remaining patient had an Engel score of IIB. CONCLUSION: Our early results demonstrate that complete PQD can be successful at providing excellent seizure freedom and functional outcomes in carefully selected young pediatric patients who have concordant seizure semiology, noninvasive/invasive testing, and imaging findings with primary seizure onset zone within the ipsilateral posterior quadrant. Meticulous surgical planning and thorough understanding of the surgical anatomy and technique are critical to achieving complete disconnection.


Subject(s)
Drug Resistant Epilepsy , Epilepsy , Malformations of Cortical Development , Child , Humans , Infant , Child, Preschool , Drug Resistant Epilepsy/diagnostic imaging , Drug Resistant Epilepsy/surgery , Drug Resistant Epilepsy/complications , Retrospective Studies , Treatment Outcome , Epilepsy/diagnostic imaging , Epilepsy/surgery , Malformations of Cortical Development/complications , Seizures/etiology , Seizures/surgery
9.
Neurosurg Focus ; 53(4): E5, 2022 10.
Article in English | MEDLINE | ID: mdl-36183179

ABSTRACT

OBJECTIVE: Patients with tuberous sclerosis complex (TSC) epilepsy present with unique clinical challenges such as early seizure onset and high rates of intractability and multifocality. Although there are numerous studies about the safety and efficacy of stereoelectroencephalography (SEEG), this topic has not been studied in TSC patients who have distinct epilepsy profiles. The authors investigated subdural grid (SDG) and SEEG monitoring to determine whether these procedures lead to similar seizure and safety outcomes and to identify features unique to this pediatric population. METHODS: TSC patients who underwent SDG or SEEG placement and a second epilepsy surgery during the period from 2007 to 2021 were included in this single-center retrospective cohort analysis. Various patient, hospitalization, and epilepsy characteristics were collected. RESULTS: A total of 50 TSC patients were included in this study: 30 were included in the SDG cohort and 20 in the SEEG cohort. Baseline weekly seizure count did not significantly differ between the 2 groups (p = 0.412). The SEEG group had a greater mean baseline number of antiepileptic drugs (AEDs) (3.0 vs 2.0, p = 0.003), higher rate of previous surgical interventions (25% vs 0%, p = 0.007), and larger proportion of patients who underwent bilateral monitoring (50% vs 13.3%, p = 0.005). Despite this, there was no significant difference in seizure freedom between the SDG and SEEG cohorts. The mean reduction in seizure count was 84.9% and 47.8% of patients were seizure free at last follow-up (mean 79.4 months). SEEG trended toward being a safer procedure than SDG monitoring, with a shorter mean ICU stay (0.7 days vs 3.9 days, p < 0.001), lower blood transfusion rate (0% vs 13.3%, p = 0.140), and lower surgical complication rate (0% vs 10%, p = 0.265). CONCLUSIONS: In the comparison of the SDG and SEEG cohorts, the SEEG group included patients who appeared to receive more aggressive management and have a higher rate of multifocality, more prior surgical interventions, more AEDs at baseline, and a higher rate of bilateral invasive monitoring. Despite this, the SEEG cohort had similar seizure outcomes and a trend toward increased safety. Based on these findings, SEEG appears to allow for monitoring of a wider breadth of TSC patients given its minimally invasive nature and its relative simplicity for monitoring numerous regions of the brain.


Subject(s)
Drug Resistant Epilepsy , Epilepsy , Tuberous Sclerosis , Child , Humans , Anticonvulsants/therapeutic use , Drug Resistant Epilepsy/surgery , Electrodes, Implanted , Electroencephalography/methods , Epilepsy/surgery , Retrospective Studies , Seizures/surgery , Stereotaxic Techniques , Treatment Outcome , Tuberous Sclerosis/complications , Tuberous Sclerosis/surgery
10.
Clin Neurophysiol ; 142: 199-208, 2022 10.
Article in English | MEDLINE | ID: mdl-36063669

ABSTRACT

OBJECTIVE: To clinically validate the connectivity-based magnetoencephalography (MEG) analyses to identify seizure onset zone (SOZ) with comparing to equivalent current dipole (ECD). METHODS: The ECD cluster was quantitatively analyzed by calculating the centroid of the cluster and maximum distance (the largest distance between all dipoles). The "primary hub" was determined by the highest eigencentrality. The distribution of nodes in the top 5% of eigenvector centrality values was quantified by generating the convex hull between each node. RESULTS: Thirty-one patients who underwent MEG, stereotactic-EEG, and focal surgery were included. The primary hub was significantly closer to the sEEG-defined SOZ compared to ECD (p = 0.009). The seizure freedom positive and negative predictive values of complete ECD cluster and primary hub resections did not significantly differ, although complete resection of the primary hub showed slightly better negative predictive value (ECD: 50.0% NPV, hub: 64.7% NPV). Both quantitative ECD and functional connectivity analyses suggested that spatially restricted dipole distributions and higher connectivity in a smaller region correlate with better seizure outcomes. CONCLUSIONS: Our findings suggest that MEG network analysis could be a valuable complement to the ECD methods. SIGNIFICANCE: The results of this study are an important step towards using non-invasive neurophysiologic recordings to accurately define the epileptic network.


Subject(s)
Epilepsy , Magnetoencephalography , Electroencephalography , Epilepsy/diagnostic imaging , Epilepsy/surgery , Humans , Magnetic Resonance Imaging , Magnetoencephalography/methods , Neurologic Examination , Predictive Value of Tests , Seizures
11.
J Neurosurg Pediatr ; : 1-8, 2022 Aug 26.
Article in English | MEDLINE | ID: mdl-36029267

ABSTRACT

OBJECTIVE: Pediatric epilepsy is characterized as drug resistant in 20%-30% of patients and defined as persistent seizures despite adequate treatment with two first-line antiepileptic medications. The American Academy of Neurology advocates surgical options earlier in the treatment of epilepsy to provide long-term seizure reduction. The new development of minimally invasive approaches has recently allowed for surgical options to patients not previously deemed surgical candidates. These may include patients with bilateral, deep, eloquent, or poorly localizing epileptogenic foci. To this end, responsive neurostimulation (RNS) is an FDA-approved closed-loop neuromodulation device for adjuvant treatment of adults with medically intractable epilepsy arising from one or multiple foci. METHODS: In this study, the authors describe their initial institutional experience with the use of RNS in pediatric patients with drug-resistant epilepsy. An IRB-approved retrospective review was conducted of 8 pediatric patients who underwent RNS implantation at Cincinnati Children's Hospital Medical Center between 2019 and 2021. RESULTS: Eight patients met the inclusion criteria for the study. The average age at the time of surgery was 14.7 years (range 8-18 years) with a mean follow-up of 16.5 months. All patients underwent invasive monitoring with stereo-EEG, subdural grid placement, or a combination of both. All patients had either bilateral or eloquent cortex targets. Trajectories were based on noninvasive (phase 1) and invasive (phase 2) seizure onset zone localization data. Four (50%) of the 8 patients underwent surgical intervention for epilepsy prior to RNS placement. RNS electrodes were placed with robot-assisted guidance in a hybrid operating room with intraoperative CT and electrocorticography. The authors demonstrated individualized RNS electrode trajectory and placement with targets in the amygdala/hippocampus, bilateral insula, bilateral parietal and occipital targets, and frontoparietal regions for a total of 14 implanted electrodes. One adverse event occurred, a wound infection requiring return to the operating room for removal of the RNS implant. All patients demonstrated a reduction in seizure frequency. All patients achieved > 50% reduction in seizure frequency at last follow-up. CONCLUSIONS: RNS implantation in carefully selected pediatric patients appears safe and efficacious in reducing seizure burden with a low rate of operative complications.

12.
Surg Neurol Int ; 13: 281, 2022.
Article in English | MEDLINE | ID: mdl-35855169

ABSTRACT

Background: The management of complicated symptomatic pineal cysts in the pediatric population is challenging and variable. Surgical management may include treatment of hydrocephalus alone, or direct treatment of the cyst with or without direct hydrocephalus management. This is typically done through craniotomy-based microsurgical approaches to the pineal region or an endoscopic transventricular approach. Methods: We present a stepwise minimally invasive technique to treat complicated pineal cysts in young children associated with an obstructive hydrocephalus in a single procedure through third ventriculostomy combined with an intraventricular marsupialization of the pineal cyst through a single burr-hole using stereotactic navigation. Results: Two young patients with over 2 years of follow-up have done well without complication using this technique. Other literature reports for complex pineal cysts in pediatric patients are reviewed and this technique is not previously described for this population. Conclusion: Endoscopic third ventriculostomy and cyst marsupialization using a single burr-hole and stereotactic navigation for symptomatic or enlarging pineal cysts in children allow for minimally invasive management, a rapid recovery, short hospital stay, and durable outcome owed to redundant CSF flow pathways.

13.
Front Neurosci ; 16: 867021, 2022.
Article in English | MEDLINE | ID: mdl-35663562

ABSTRACT

Background: Improvement in visual naming abilities throughout the childhood and adolescence supports development of higher-order linguistic skills. We investigated neuronal circuits underlying improvement in the speed of visual naming with age, and age-related dynamics of these circuits. Methods: Response times were electronically measured during an overt visual naming task in epilepsy patients undergoing stereo-EEG monitoring. Coherence modulations among pairs of neuroanatomic parcels were computed and analyzed for relationship with response time and age. Results: During the overt visual naming task, mean response time (latency) significantly decreased from 4 to 23 years of age. Coherence modulations during visual naming showed that increased connectivity between certain brain regions, particularly that between left fusiform gyrus/left parahippocampal gyrus and left frontal operculum, is associated with improvement in naming speed. Also, decreased connectivity in other brain regions, particularly between left angular and supramarginal gyri, is associated with decreased mean response time. Further, coherence modulations between left frontal operculum and both left fusiform and left posterior cingulate gyri significantly increase, while that between left angular and supramarginal gyri significantly decrease, with age. Conclusion: Naming speed continues to improve from pre-school years into young adulthood. This age-related improvement in efficiency of naming environmental objects occurs likely because of strengthened direct connectivity between semantic and phonological nodes, and elimination of intermediate higher-order cognitive steps.

14.
J Clin Neurophysiol ; 39(3): 240-246, 2022 Mar 01.
Article in English | MEDLINE | ID: mdl-35255008

ABSTRACT

PURPOSE: Magnetoencephalography (MEG) defines the spike-generating zone and provides targets for invasive monitoring with stereotactic electroencephalography. This retrospective, blinded, cross-sectional study determined whether MEG virtual sensors could identify hippocampal epileptiform activity. METHODS: Using MEG beamformer analysis, virtual sensors were manually placed in bilateral hippocampi and corresponding virtual sensor waveforms were analyzed for the presence of epileptiform activity. These findings were compared with hippocampal stereotactic electroencephalography in the same patients. Concordance was determined using sensitivity and specificity. RESULTS: Thirty patients (mean age 12.5 ± 5.9 years) and 35 hippocampi were included. Patients were also placed into subgroups based on conventional MEG analysis: temporal (n = 19), extratemporal (n = 10), and normal (n = 1). Overall, sensitivity and specificity were 57.9% and 50.0%, respectively (n = 35). Patients with temporal sources based on conventional MEG analysis had sensitivity and specificity of 80.0% and 36.4%, respectively (n = 21). Those with extratemporal sources based on conventional MEG had sensitivity and specificity of 42.9% and 80.0%, respectively (n = 12). CONCLUSIONS: When grouped by conventional MEG analysis, virtual sensors can be useful to confirm mesial temporal dipoles seen with conventional analysis. SIGNIFICANCE: This work may help support the use of MEG for the detection of epileptiform activity in the hippocampus and influence the planning of invasive electrode placement.


Subject(s)
Electroencephalography , Magnetoencephalography , Adolescent , Child , Cross-Sectional Studies , Hippocampus , Humans , Retrospective Studies
15.
Am J Med Genet A ; 188(4): 1280-1286, 2022 04.
Article in English | MEDLINE | ID: mdl-34964243

ABSTRACT

Costello syndrome (CS) is an autosomal dominant disorder caused by pathogenic variants in HRAS. Craniosynostosis is a known feature of other RASopathies (Noonan and cardiofaciocutaneous syndromes) but not CS. We describe four individuals with CS and craniosynostosis and present a summary of all previously reported individuals with craniosynostosis and RASopathy.


Subject(s)
Costello Syndrome , Craniosynostoses , Ectodermal Dysplasia , Noonan Syndrome , Costello Syndrome/diagnosis , Costello Syndrome/genetics , Craniosynostoses/diagnosis , Craniosynostoses/genetics , Facies , Failure to Thrive , Humans
17.
Neurosurg Focus ; 50(4): E6, 2021 04.
Article in English | MEDLINE | ID: mdl-33794501

ABSTRACT

OBJECTIVE: Patients presenting with head shape changes phenotypical for craniosynostosis may have incomplete fusion of the involved sutures. The surgical literature is lacking in appropriate management strategies for these patients. In this paper, the authors evaluate their experience with a novel treatment strategy: suturectomy of only the fused portion followed by helmeting therapy in patients with skull deformity secondary to incomplete suture synostosis. METHODS: Patients with craniosynostosis with incomplete suture fusion requiring operative intervention between 2018 and 2020 were included for evaluation. Patients were selected for partial suturectomy if the patent portion of the suture had a normal appearance. All patients underwent craniectomy of the involved portion of the synostosed suture. Intraoperative ultrasound was used to reassess the degree of fusion at the time of surgery and incision planning. A 2- to 3-cm strip craniectomy was performed under direct visualization through a single minimal access incision. Postoperative helmeting was utilized for all patients. Demographic and perioperative data were collected, including laser scan data in the form of cranial index (CI) and cranial vault asymmetry (CVA), defined as the difference between two diagonal measurements, from the frontozygomaticus to the opposite eurion. RESULTS: Four males and 1 female with a mean age of 2.8 months (range 1.1-3.9 months) at presentation were included. All patients had incomplete sagittal synostosis (one patient also had an incomplete left lambdoid synostosis and another had an incomplete left coronal synostosis). The mean age at surgery was 3.5 months (range 2.0-4.7 months) without any major complications. All patients were compliant with postoperative helmeting. The average age at the last follow-up was 12.8 months (range 5.3-23.7 months) with a mean follow-up duration of 9.3 months (range 0.5-19.6 months). Final laser scan evaluations were available for 3 patients and showed an improvement of the CI from an average of 71.3 (range 70-73) to 84.3 (range 82-86). The CVA improved from an average of 9.67 mm (range 2-22 mm) to 1.67 mm (range 1-2 mm). CONCLUSIONS: Minimally invasive direct excision of the involved portion of fused cranial sutures followed by helmet therapy for phenotypical craniosynostosis is a safe and effective treatment strategy. This technique is suitable for very young patients and appears to offer similar outcomes to complete suturectomy. Further studies are required to see if this approach reduces the deformity severity for patients requiring vault remodeling later in life.


Subject(s)
Cranial Sutures , Craniosynostoses , Child, Preschool , Cranial Sutures/diagnostic imaging , Cranial Sutures/surgery , Craniosynostoses/diagnostic imaging , Craniosynostoses/surgery , Craniotomy , Female , Humans , Infant , Male , Postoperative Period , Retrospective Studies , Skull/surgery
18.
J Neurosurg Pediatr ; 27(6): 629-636, 2021 Mar 26.
Article in English | MEDLINE | ID: mdl-33770756

ABSTRACT

OBJECTIVE: External magnetic forces can have an impact on programmable valve mechanisms and potentially alter the opening pressure. As wearable technology has begun to permeate mainstream living, there is a clear need to provide information regarding safety of these devices for use near a programmable valve (PV). The aim of this study was to evaluate the magnetic fields of reference devices using smartphone-integrated magnetometers and compare the results with published shunt tolerances. METHODS: Five smartphones from different manufacturers were used to evaluate the magnetic properties of various commonly used (n = 6) and newer-generation (n = 10) devices using measurements generated from the internal smartphone magnetometers. PV tolerance testing using calibrated magnets of varying field strengths was also performed by smartphone magnetometers. RESULTS: All tested smartphone-integrated magnetometers had a factory sensor saturation point at around 5000 µT or 50 Gauss (G). This is well below the threshold at which a magnet can potentially deprogram a shunt, based on manufacturer reports as well as the authors' experimental data with a threshold of more than 300 G. While many of the devices did saturate the smartphone sensors at the source, the magnetic flux density of the objects decreases significantly at 2 inches. CONCLUSIONS: The existence of an upper limit on the magnetometers of all the smartphones used, although well below the published deprogramming threshold for modern programmable valves, does not allow us to give precise recommendations on those devices that saturate the sensor. Based on the authors' experimental data using smartphone-integrated magnetometers, they concluded that devices that measure < 40 G can be used safely close to a PV.


Subject(s)
Cerebrospinal Fluid Shunts , Magnetometry/instrumentation , Proof of Concept Study , Smartphone , Wearable Electronic Devices/adverse effects , Cerebrospinal Fluid Shunts/instrumentation , Electromagnetic Phenomena , Humans , Magnetic Fields
19.
Eye Brain ; 13: 29-40, 2021.
Article in English | MEDLINE | ID: mdl-33542671

ABSTRACT

Craniosynostosis, a premature fusion of cranial sutures that can be isolated or syndromic, is a congenital defect with a broad, multisystem clinical spectrum. The visual pathway is prone to derangements in patients with craniosynostosis, particularly in syndromic cases, and there is a risk for permanent vision loss when ocular disease complications are not identified and properly treated early in life. Extensive advancements have been made in our understanding of the etiologies underlying vision loss in craniosynostosis over the last 20 years. Children with craniosynostosis are susceptible to interruptions in visual input arising from strabismus, refractive errors, and corneal damage; any of these aberrations can result in understimulation of the visual cortex during childhood neurodevelopment and permanent amblyopia. Elevated intracranial pressure resulting from abnormal cranial shape or volume can lead to papilledema and, ultimately, optic atrophy and vision loss. A pediatric ophthalmologist is a crucial component of the multidisciplinary care team that should be involved in the care of craniosynostosis patients and consistent ophthalmologic follow-up can help minimize the risk to vision posed by such entities as papilledema and amblyopia. This article aims to review the current understanding of neuro-ophthalmological manifestations in craniosynostosis and explore diagnostic and management considerations for the ophthalmologist taking care of these patients.

20.
Oper Neurosurg (Hagerstown) ; 19(6): 735-740, 2020 11 16.
Article in English | MEDLINE | ID: mdl-32710764

ABSTRACT

BACKGROUND: Fascia lata remains a popular and robust graft to repair osteodural defects in endoscopic neurosurgery. Classically, this graft is obtained via a large incision in the thigh that is prone to pain and muscle herniation after surgery. OBJECTIVE: To present a novel technique for harvesting fat and fascia lata graft and reapproximating the edges via an endoscopic approach through the thigh using an "outside-in" technique to prevent muscle herniation. METHODS: Initially our technique was performed in cadavers and includes the following: small 2 cm incision in the lateral thigh to accommodate the endoscope, use of blunt dissection and endoscopic tools to obtain the graft, and reapproximation of the fascia via an outside-in technique using conventional sutures with endoscopic visualization to retrieve the sutures beneath the skin and tie them. We then applied the technique to a patient undergoing transsphenoidal tumor resection. RESULTS: This technique was trialed in 3 cadaver specimens (6 limbs) and was used successfully in a patient with excellent cosmetic results seen in follow-up. CONCLUSION: Endoscopic retrieval of fascia lata is feasible via a very small incision. Reapproximation of the cut fascial edges to minimize muscle herniation can quickly and easily be performed with an outside-in technique detailed here. Additional case series may help to solidify the endoscopic retrieval as a preferred technique for fascia lata graft.


Subject(s)
Endoscopy , Fascia Lata , Cadaver , Dissection , Humans
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