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

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Matthew D. Smyth, R. Shane Tubbs, E. Martina Bebin, Paul A. Grabb and Jeffrey P. Blount

Object. The aim of this study was to define better the incidence of surgical complications and untoward side effects of chronic vagus nerve stimulation (VNS) in a population of children with medically refractory epilepsy.

Methods. The authors retrospectively reviewed the cases of 74 consecutive patients (41 male and 33 female) 18 years of age or younger (mean age 8.8 years, range 11 months–18 years) who had undergone implantation of a vagal stimulator between 1998 and 2001 with a minimum follow up of 1 year (mean 2.2 years). Of the 74 patients treated, seven (9.4%) had a complication ultimately resulting in removal of the stimulator. The rate of deep infections necessitating device removal was 3.5% (three of 74 patients who had undergone 85 implantation and/or revision procedures). An additional three superficial infections occurred in patients in whom the stimulators were not removed: one was treated with superficial operative debridement and antibiotic agents and the other two with oral antibiotics only. Another four stimulators (5.4%) were removed because of the absence of clinical benefit and device intolerance. Two devices were revised because of lead fracture (2.7%). Among the cohort, 11 battery changes have been performed thus far, although none less than 33 months after initial implantation. Several patients experienced stimulation-induced symptoms (hoarseness, cough, drooling, outbursts of laughter, shoulder abduction, dysphagia, or urinary retention) that did not require device removal. Ipsilateral vocal cord paralysis was identified in one patient. One patient died of aspiration pneumonia more than 30 days after device implantation.

Conclusions. Vagus nerve stimulation remains a viable option for improving seizure control in difficult to treat pediatric patients with epilepsy. Surgical complications such as hardware failure (2.7%) or deep infection (3.5%) occurred, resulting in device removal or revision. Occasional stimulation-induced symptoms such as hoarseness, dysphagia, or torticollis may be expected (5.4%).

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

Object

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.

Methods

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.

Results

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.

Conclusions

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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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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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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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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John Persing

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Matthew D. Smyth, Marissa J. Tenenbaum, Christian B. Kaufman and Alex A. Kane

Object

Although most patients with sagittal craniosynostosis are recognized and treated in infancy, some children are not referred to craniofacial centers until later in childhood. In this paper the authors describe a novel operative technique for calvarial reconstruction in older children with previously untreated sagittal craniosynostosis.

Methods

The authors report a clinical series of eight patients who were treated using novel single-stage calvarial reconstruction, and they assess the complications and outcomes. The patient is placed supine for the procedure, which consists of a coronal incision, bifrontal craniotomy without orbital osteotomy, and multiple interlocking midline parietooccipital osteotomies and recontouring. Fixation is achieved using a bioabsorbable plate system. Cranial indices were calculated from measurements obtained before and after the reconstructive procedures. Preoperative, intraoperative, and postoperative photographs and three-dimensional computed tomography scans are presented for review.

Between November 2003 and April 2005, the authors treated seven boys (age range ~ 1–10 years, mean age 4.2 years) with uncorrected sagittal craniosynostosis and one with bicoronal and sagittal synostosis. The mean operating time was 5.13 hours (range 4.3–8 hours), with a mean blood loss of 425 ml (range 200–800 ml). As a percentage of the estimated circulating blood volume, the mean operative blood loss was 33.5% (range 17–57%). The mean hospital stay was 4.9 days. The cranial index significantly improved from a mean of 65.6 to 71.3% (p = 0.001). No acute or delayed complications have been noted. Follow-up examinations performed at an average of 12 months (range 1–17 months) have confirmed early patient and family satisfaction.

Conclusions

An approach of aggressive calvarial reconstruction with multiple interleaving osteotomies crossing the midline achieves improvements in biparietal narrowing. Combined with a bifrontal reconstruction, early outcomes are excellent, with an acceptable amount of intraoperative blood loss and no significant complications.

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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.