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


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.


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.


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.


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


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.


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.


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.


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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Matthew D. Smyth, Lawrence Pitts, Robert K. Jackler and Kenneth D. Aldape

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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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James T. Rutka

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Michael Anthony Ciliberto, David Limbrick, Alexander Powers, Jeffrey B. Titus, Rebecca Munro and Matthew D. Smyth


Intractable epilepsy is a significant burden on families and on the cognitive development and quality of life (QOL) of patients. Periinsular hemispherotomy (PIH) for medically intractable epilepsy can benefit patients who qualify for this procedure. The ideal hemispherotomy candidate has ipsilateral ictal and interictal epileptiform activity, unilateral MR imaging abnormalities, contralateral hemiplegia, and a normal contralateral hemisphere. However, certain patients present with a mixed picture of bilateral electroencephalography (EEG) findings and severe intractable epilepsy, prompting consideration of a more aggressive treatment approach. This report introduces the possibility of surgery for patients who normally would not meet criteria for this treatment modality.


In this retrospective chart review, the authors report on 7 patients with bilateral seizure onset noted on routine or video-EEG monitoring. A QOL phone questionnaire, based on the Quality of Life in Childhood Epilepsy tool, was administered to a parent. The authors reviewed each patient's chart for surgical complications, changes in examination, QOL, limited neuropsychological outcomes, and seizure outcomes. They also investigated each chart for MR imaging and EEG findings as well as the patient's epilepsy clinic notes for seizure semiology and frequency.


All patients enjoyed a decrease in seizure frequency and a subjective increase in QOL after PIH. Five patients (71%) achieved Engel Class I or II seizure control. The mean follow-up was 3.64 years (2–5.3 years). One patient is now off all antiseizure medication. No patient had a decrement in Full Scale IQ on postsurgical testing, and 2 (28.5%) of 7 individuals had increased adaptive and social functioning. Postsurgical examination changes included hemiplegia and homonymous hemianopia.


Hemispherotomy in patients with intractable epilepsy is generally reserved for individuals with unilateral epileptiform abnormalities or lesions on MR imaging. Seven patients in this study benefited from surgery despite bilateral seizure onset with improvement in seizure control and overall QOL. Thus, bilateral ictal onset does not necessarily preclude consideration for hemispherotomy in selected patients with severe medically refractory epilepsy.

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R. Shane Tubbs, Daniel Webb, Matthew D. Smyth and W. Jerry Oakes

Object. The authors hypothesized that children with preoperative Chiari I malformation and an absent gag reflex may harbor pharyngeal musculature atrophy identifiable on magnetic resonance (MR) imaging.

Methods. Thirty patients with preoperative Chiari I malformation and a functioning gag reflex, five patients with preoperative Chiari I malformation and complete absence of gag reflex, and 50 control individuals underwent radiological measurement of the posterior pharyngeal wall thickness.

The thickness of the posterior pharyngeal wall in age-matched controls was significantly thinner (p < 0.0001) than that in patients with Chiari I malformation and no functioning gag reflex. Additionally, in patients with hindbrain herniation and absent gag reflex, the posterior pharyngeal wall thicknesses were comparable to or thinner than those in age-matched controls. A general decrease in the thickness of the posterior pharyngeal wall was found in control individuals who were older compared with patients with Chiari I malformation and a preserved gag reflex in whom the prevertebral soft tissues were found to increase in thickness with age.

Analysis of these data showed that in children with a nonfunctioning gag reflex the prevertebral soft tissue, composed primarily of the superior constrictor muscle, is statistically thinner compared with that in age-matched controls and age-matched children with Chiari I malformation and a functioning gag reflex. The authors theorize that the discrepancy between this measurement in controls and patients with hindbrain herniation is due to the thickening of the craniocervical ligaments that is known to occur in this clinical entity.

Conclusions. The finding that prevertebral soft tissue thickens with age in patients with Chiari I malformation and functioning gag reflex alone may aid in the interpretation of soft-tissue injury following cervical spine injuries in this group.

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Matthew D. Smyth, Jason T. Banks, R. Shane Tubbs, John C. Wellons III and W. Jerry Oakes

Object. The authors performed a study to evaluate the efficacy of a regimen of scheduled minor analgesic medications in managing postoperative pain in children undergoing intracranial procedures.

Methods. Postoperative pain scores were analyzed in two groups of children who underwent decompressive surgery for Chiari malformation: Group A underwent a scheduled regimen of minor oral analgesic medications (standing doses of acetaminophen [10 mg/kg] and ibuprofen [10 mg/kg] alternating every 2 hours) and Group B received analgesic medication when requested.

Fifty children underwent a standard occipital craniectomy (25 in each group). The pain scores were significantly lower in Group A during most of the postoperative period. Length of stay (LOS) was shorter (2.2 compared with 2.8 days), and narcotic and antiemetic requirements were also lower in Group A patients than in Group B patients. Patients with spinal cord syringes exhibited a similar postoperative status to those without, and similar improvements in pain scores with scheduled minor analgesic medications were also evident.

Conclusions. A regimen of minor analgesic therapy, given in alternating doses every 2 hours immediately after craniotomy and throughout hospitalization, significantly reduced postoperative pain scores and LOS in children in whom suboccipital craniotomy was performed. Narcotic and antiemetic requirements were also decreased in association with this regimen. Application of this postoperative analgesia protocol may benefit children and adults in whom various similar neurosurgical procedures are required.

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Matthew D. Smyth, Eric E. Klein, W. Edwin Dodson and David B. Mansur

✓The authors report the successful use of radiosurgery in a child for posterior corpus callosotomy; the early results are good and the patient has not suffered any morbid conditions. The relevant literature pertaining to the use of radio-surgery for treating epilepsy is reviewed. Details of the radiosurgical techniques and prescription dose used are presented, along with 1-year serial neuroimaging results.

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Giant intracranial teratoma with epignathus in a neonate

Case report and review of the literature

James M. Johnston, Nilesh A. Vyas, Alex A. Kane, David W. Molter and Matthew D. Smyth

✓Epignathus, a congenital oropharyngeal teratoma, is a rare clinical entity with variable clinical outcomes described in the literature. Even fewer cases of epignathus with intracranial extension have been reported, all with poor outcomes. In this manuscript, the authors present a case of epignathus with intracranial extension, emphasizing clinical presentation, imaging findings, a staged surgical approach, multidisciplinary management, and outcome.