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Thomas L. Beaumont, Jakub Godzik, Sonika Dahiya and Matthew D. Smyth

The authors report the case of a 14-year-old male with a subependymal giant cell astrocytoma (SEGA) that occurred in the absence of tuberous sclerosis complex (TSC). The patient presented with progressive headache and the sudden onset of nausea and vomiting. Neuroimaging revealed an enhancing left ventricular mass located in the region of the foramen of Monro with significant mass effect and midline shift. The lesion had radiographic characteristics of SEGA; however, the diagnosis remained unclear given the absence of clinical features of TSC. The patient underwent gross-total resection of the tumor with resolution of his symptoms. Although tumor histology was consistent with SEGA, genetic analysis of both germline and tumor DNA revealed no TSC1/2 mutations. Similarly, a comprehensive clinical evaluation failed to reveal any clinical features characteristic of TSC. Few cases of SEGA without clinical or genetic evidence of TSC have been reported. The histogenesis, genetics, and clinical approach to this rare lesion are briefly reviewed.

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Anna Jeong, Jennifer Strahle, Ananth K. Vellimana, David D. Limbrick Jr., Matthew D. Smyth and Mary Bertrand

OBJECTIVE

Electrical status epilepticus of sleep (ESES) is a rare electrographic pattern associated with global regression, which is often poorly responsive to traditional epilepsy treatments and can have a devastating and permanent neurocognitive outcome. The authors analyzed clinical, electroencephalographic, and neuropsychological outcomes in 9 patients with refractory ESES treated with functional hemispherotomy to illustrate the wide clinical spectrum associated with the disease and explore the role of hemispherotomy in its treatment.

METHODS

During the period between 2003 and 2015, 80 patients underwent hemispherotomy at the authors' institution. Video electroencephalography (EEG) reports were reviewed for ESES or continuous spikes and waves during sleep (CSWS). Patients with preoperative ESES (> 85% slow-wave sleep occupied by spike waves), a unilateral structural lesion amenable to surgery, and more than 6 months of follow-up data were included in the analysis. Clinical data, EEG recordings, neuropsychological testing, and parental and clinician reports were retrospectively reviewed.

RESULTS

Nine patients were eligible for study inclusion. Age at seizure onset ranged from birth to 4.2 years (mean 1.9 years), age at ESES diagnosis ranged from 3.5 to 8.8 years (mean 6.0 years), and age at hemispherotomy ranged from 3.7 to 11.5 years (mean 6.8 years). All patients had drug-resistant epilepsy. The duration of epilepsy prior to hemispherotomy ranged from 2.7 to 8.9 years (mean ± SD, 5.0 ± 2.2 years). Engel Class I seizure outcome was observed in all 9 children, with a mean follow-up of 3.0 years (range 0.5–6.1 years). Hemispherotomy terminated ESES in 6 of 6 patients with available postoperative sleep EEG. All children had preoperative neuropsychological impairments. Developmental regression was halted postoperatively, but none of the children returned to their original pre-ESES baseline. Four children demonstrated academic gains, 2 of whom transitioned to mainstream classes.

CONCLUSIONS

Children with drug-resistant ESES and a unilateral structural lesion should be evaluated for hemispherotomy as they may experience the cessation of seizures, termination of ESES, and improvement in neuropsychological status.

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Brandon A. Miller, Afshin Salehi, David D. Limbrick Jr. and Matthew D. Smyth

OBJECTIVE

The ROSA device is a robotic stereotactic arm that uses a laser system to register the patient’s head or spine with MR or CT images. In this study, the authors analyze their experience with this system in pediatric neurosurgical applications and present selected cases that exemplify the usefulness of this system.

METHODS

The authors reviewed all cases that utilized the ROSA system at their institution. Patient demographics, pathology, complications, electrode placement, laser ablation, and biopsy accuracy were analyzed. Patient disposition and condition at follow-up were also analyzed.

RESULTS

Seventeen patients underwent 23 procedures using the ROSA system. A total of 87 electroencephalography electrodes were placed, with 13% deviating more than 3 mm from target. Six patients underwent stereotactic needle biopsy, and 9 underwent laser interstitial thermotherapy (LITT). One patient who underwent LITT required a subsequent craniotomy for tumor resection. Another patient experienced an asymptomatic extraaxial hematoma that spontaneously resolved. No patient suffered neurological complications during follow-up. Follow-up from the last procedure averaged 180 days in epilepsy patients and 309 days in oncology patients.

CONCLUSIONS

The precision, ease of use, and versatility of the ROSA system make it well suited for pediatric neurosurgical practice. Further work, including long-term analysis of results and cost-effectiveness, will help determine the utility of this system and if its applications can be expanded.

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David Y. A. Dadey, Ashwin A. Kamath, Eric C. Leuthardt and Matthew D. Smyth

Subependymal giant cell astrocytoma (SEGA) is a rare tumor occurring almost exclusively in patients with tuberous sclerosis complex. Although open resection remains the standard therapy, complication rates remain high. To minimize morbidity, less invasive approaches, such as endoscope-assisted resection, radiosurgery, and chemotherapy with mTOR pathway inhibitors, are also used to treat these lesions. Laser interstitial thermal therapy (LITT) is a relatively new modality that is increasingly used to treat a variety of intracranial lesions. In this report, the authors describe two pediatric cases of SEGA that were treated with LITT. In both patients the lesion responded well to this treatment modality, with tumor shrinkage observed on follow-up MRI. These cases highlight the potential of LITT to serve as a viable minimally invasive therapeutic approach to the management of SEGAs in the pediatric population.

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Chester K. Yarbrough, Jacob K. Greenberg, Matthew D. Smyth, Jeffrey R. Leonard, Tae Sung Park and David D. Limbrick Jr.

Object

Historically, assessment of clinical outcomes following surgical management of Chiari malformation Type I (CM-I) has been challenging due to the lack of a validated instrument for widespread use. The Chicago Chiari Outcome Scale (CCOS) is a novel system intended to provide a less subjective evaluation of outcomes for patients with CM-I. The goal of this study was to externally validate the performance of the CCOS.

Methods

Patients undergoing surgery for CM-I between 2001 and 2012 were reviewed (n = 292). Inclusion criteria for this study were as follows: 1) patients receiving primary posterior fossa decompression; 2) at least 5.5 months of postoperative clinical follow-up; and 3) patients ≤ 18 years of age at the time of surgery. Outcomes were evaluated using the CCOS, along with a “gestalt” impression of whether patients experienced significant improvement after surgery. A subgroup of 118 consecutive patients undergoing operations between 2008 and 2010 was selected for analysis of interrater reliability (n = 73 meeting inclusion/exclusion criteria). In this subgroup, gestalt and CCOS scores were independently determined by 2 reviewers, and interrater reliability was assessed using the intraclass correlation coefficient (ICC) and kappa (κ) statistic.

Results

The median CCOS score was 14, and 67% of patients had improved gestalt scores after surgery. Overall, the CCOS was effective at identifying patients with improved outcome after surgery (area under curve = 0.951). The interrater reliability of the CCOS (ICC = 0.71) was high, although the reliability of the component scores ranged from poor to good (ICC 0.23–0.89). The functionality subscore demonstrated a low ICC and did not add to the predictive ability of the logistic regression model (likelihood ratio = 1.8, p = 0.18). When analyzing gestalt outcome, there was moderate agreement between raters (κ = 0.56).

Conclusions

In this external validation study, the CCOS was effective at identifying patients with improved outcomes and proved more reliable than the authors' gestalt impression of outcome. However, certain component subscores (functionality and nonpain symptoms) were found to be less reliable, and may benefit from further definition in score assignment. In particular, the functionality subscore does not add to the predictive ability of the CCOS, and may be unnecessary. Overall, the authors found the CCOS to be an improvement over the previously used assessment of outcome at their institution.

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Timothy W. Vogel, Albert S. Woo, Alex A. Kane, Kamlesh B. Patel, Sybill D. Naidoo and Matthew D. Smyth

Object

The surgical management of infants with sagittal synostosis has traditionally relied on open cranial vault remodeling (CVR) techniques; however, minimally invasive technologies, including endoscope-assisted craniectomy (EAC) repair followed by helmet therapy (HT, EAC+HT), is increasingly used to treat various forms of craniosynostosis during the 1st year of life. In this study the authors determined the costs associated with EAC+HT in comparison with those for CVR.

Methods

The authors performed a retrospective case-control analysis of 21 children who had undergone CVR and 21 who had undergone EAC+HT. Eligibility criteria included an age less than 1 year and at least 1 year of clinical follow-up data. Financial and clinical records were reviewed for data related to length of hospital stay and transfusion rates as well as costs associated with physician, hospital, and outpatient clinic visits.

Results

The average age of patients who underwent CVR was 6.8 months compared with 3.1 months for those who underwent EAC+HT. Patients who underwent EAC+HT most often required the use of 2 helmets (76.5%), infrequently required a third helmet (13.3%), and averaged 1.8 clinic visits in the first 90 days after surgery. Endoscope-assisted craniectomy plus HT was associated with shorter hospital stays (mean 1.10 vs 4.67 days for CVR, p < 0.0001), a decreased rate of blood transfusions (9.5% vs 100% for CVR, p < 0.0001), and a decreased operative time (81.1 vs 165.8 minutes for CVR, p < 0.0001). The overall cost of EAC+HT, accounting for hospital charges, professional and helmet fees, and clinic visits, was also lower than that of CVR ($37,255.99 vs $56,990.46, respectively, p < 0.0001).

Conclusions

Endoscope-assisted craniectomy plus HT is a less costly surgical option for patients than CVR. In addition, EAC+HT was associated with a lower utilization of perioperative resources. Theses findings suggest that EAC+HT for infants with sagittal synostosis may be a cost-effective first-line surgical option.

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David Y. A. Dadey, Ashwin A. Kamath, Matthew D. Smyth, Michael R. Chicoine, Eric C. Leuthardt and Albert H. Kim

OBJECTIVE

The precision of laser probe insertion for interstitial thermal therapy of deep-seated lesions is limited by the method of stereotactic guidance. The objective of this study was to evaluate the feasibility of customized STarFix 3D-printed stereotactic platforms to guide laser probe insertion into mesiotemporal and posterior fossa targets.

METHODS

The authors conducted a retrospective review of 5 patients (12–55 years of age) treated with laser interstitial thermal therapy (LITT) in which STarFix platforms were used for probe insertion. Bone fiducials were implanted in each patient's skull, and subsequent CT scans were used to guide the design of each platform and incorporate desired treatment trajectories. Once generated, the platforms were mounted on the patients' craniums and used to position the laser probe during surgery. Placement of the laser probe and the LITT procedure were monitored with intraoperative MRI. Perioperative and follow-up MRI were performed to identify and monitor changes in target lesions.

RESULTS

Accurate placement of the laser probe was observed in all cases. For all patients, thermal ablation was accomplished without intraoperative complications. Of the 4 patients with symptomatic lesions, 2 experienced complete resolution of symptoms, and 1 reported improved symptoms compared with baseline.

CONCLUSIONS

Customized stereotactic platforms were seamlessly incorporated into the authors' previously established LITT workflow and allowed for accurate treatment delivery.

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Thomas J. Wilson, Kathleen E. McCoy, Wajd N. Al-Holou, Sergio L. Molina, Matthew D. Smyth and Stephen E. Sullivan

OBJECTIVE

The aim of this paper is to compare the accuracy of the freehand technique versus the use of intraoperative guidance (either ultrasound guidance or frameless stereotaxy) for placement of parietooccipital ventricular catheters and to determine factors associated with reduced proximal shunt failure.

METHODS

This retrospective cohort study included all patients from 2 institutions who underwent a ventricular cerebrospinal fluid (CSF) shunting procedure in which a new parietooccipital ventricular catheter was placed between January 2005 and December 2013. Data abstracted for each patient included age, sex, method of ventricular catheter placement, side of ventricular catheter placement, Evans ratio, and bifrontal ventricular span. Postoperative radiographic studies were reviewed for accuracy of ventricular catheter placement. Medical records were also reviewed for evidence of shunt failure requiring revision. Standard statistical methods were used for analysis.

RESULTS

A total of 257 patients were included in the study: 134 from the University of Michigan and 123 from Washington University in St. Louis. Accurate ventricular catheter placement was achieved in 81.2% of cases in which intraoperative guidance was used versus 67.3% when the freehand technique was used. Increasing age reduced the likelihood of accurate catheter placement (OR 0.983, 95% CI 0.971–0.995; p = 0.005), while the use of intraoperative guidance significantly increased the likelihood (OR 2.809, 95% CI 1.406–5.618; p = 0.016). During the study period, 108 patients (42.0%) experienced shunt failure, 79 patients (30.7%) had failure involving the proximal catheter, and 53 patients (20.6%) had distal failure (valve or distal catheter). Increasing age reduced the likelihood of being free from proximal shunt failure (OR 0.983, 95% CI 0.970–0.995; p = 0.008), while both the use of intraoperative guidance (OR 2.385, 95% CI 1.227–5.032; p = 0.011), and accurate ventricular catheter placement (OR 3.424, 95% CI 1.796–6.524; p = 0.009) increased the likelihood.

CONCLUSIONS

The use of intraoperative guidance during parietooccipital ventricular catheter placement as part of a CSF shunt system significantly increases the likelihood of accurate catheter placement and subsequently reduces the rate of proximal shunt failure.