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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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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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Francesco T. Mangano and Matthew D. Smyth

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

Object. The literature contains scant data regarding variations in anatomy at the level of the foramen of Magendie in patients with Chiari I malformation and syringomyelia.

Methods. Based on their operative experience and hospital data, the authors detailed the incidence of arachnoid veils found in juxtaposition to the foramen of Magendie in patients with hindbrain herniation. Additionally, radiological studies were retrospectively reviewed in cases in which such an anomaly was noted intraoperatively.

Of 140 patients with Chiari I malformation who underwent decompressive surgery, an associated syrinx was demonstrated in 80 (57%). The foramen of Magendie was obstructed by an arachnoid veil in 10 (12.5%) of these patients; once the lesion was punctured, the cerebrospinal fluid drained freely from this median aperture. On retrospective review of imaging studies, none of these anomalous structures was evident. In all patients with an arachnoid veil and syringomyelia resolution of syringomyelia was revealed on postoperative imaging.

Conclusions. In the absence of a clear pathophysiology of syrinx production, the authors would recommend that patients with syringomyelia and Chiari I malformation undergo duraplasty so that, if present, these veils can be fenestrated.

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Laleh Jalilian, David D. Limbrick Jr., Karen Steger-May, Jim Johnston, Alex K. Powers and Matthew D. Smyth

Object

The goal of this study was to evaluate the efficacy of anterior versus complete sectioning of the corpus callosum in children suffering from medically refractory epilepsy. The authors report seizure outcome in patients who underwent anterior two-thirds or complete corpus callosotomy (CC) during the period 1995–2008 at St. Louis Children's Hospital.

Methods

The medical records of 27 children and adolescents with a minimum follow-up of 6 months were retrospectively evaluated with respect to seizure status, anticonvulsant outcomes, and subjective results. Preoperatively, patients suffered from a variety of seizure types that occurred daily, weekly, or episodically. The male/female ratio was 19:8, and patients ranged in age between 3 and 19 years (mean 9.93 years). Seizure outcome, parental assessment of daily function, and changes in the number of prescribed antiepileptic drugs were all assessed.

Results

Fifteen patients underwent an initial anterior two-thirds CC, and 12 underwent a complete CC. Of the 15 patients who underwent an anterior CC, 7 went on to receive a posterior CC. Seizure control was superior in children undergoing a complete CC (91%, Class I–III) versus an anterior two-thirds CC (75%, Class I–III). Seizure types most affected by CC included atonic, myoclonic, and absence. The number of postoperative antiepileptic drugs did not significantly change following CC in either the anterior only or complete groups. One patient experienced a transient disconnection syndrome that resolved within 4 weeks, and 4 patients experienced mild hemiparesis and speech delays that resolved with therapy. Three patients experienced surgical complications requiring a second operation. The overall daily function and attentiveness of the patients improved.

Conclusions

A complete CC should be considered as the initial procedure in lower-functioning children afflicted by absence, atonic, or myoclonic seizures. Severely affected higher-functioning children may also benefit from a complete CC, without clinically significant disconnection syndromes. A completion posterior CC may benefit patients in whom a prior anterior CC has failed.

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Manish N. Shah, Alex A. Kane, J. Dayne Petersen, Albert S. Woo, Sybill D. Naidoo and Matthew D. Smyth

Object

This study investigated the differences in effectiveness and morbidity between endoscopically assisted wide-vertex strip craniectomy with barrel-stave osteotomies and postoperative helmet therapy versus open calvarial vault reconstruction without helmet therapy for sagittal craniosynostosis.

Methods

Between 2003 and 2010, the authors prospectively observed 89 children less than 12 months old who were surgically treated for a diagnosis of isolated sagittal synostosis. The endoscopic procedure was offered starting in 2006. The data associated with length of stay, blood loss, transfusion rates, operating times, and cephalic indices were reviewed.

Results

There were 47 endoscopically treated patients with a mean age at surgery of 3.6 months and 42 patients with open-vault reconstruction whose mean age at surgery was 6.8 months. The mean follow-up time was 13 months for endoscopic versus 25 months for open procedures. The mean operating time for the endoscopic procedure was 88 minutes, versus 179 minutes for the open surgery. The mean blood loss was 29 ml for endoscopic versus 218 ml for open procedures. Three endoscopically treated cases (6.4%) underwent transfusion, whereas all patients with open procedures underwent transfusion, with a mean of 1.6 transfusions per patient. The mean length of stay was 1.2 days for endoscopic and 3.9 days for open procedures. Of endoscopically treated patients completing helmet therapy, the mean duration for helmet therapy was 8.7 months. The mean pre- and postoperative cephalic indices for endoscopic procedures were 68% and 76% at 13 months postoperatively, versus 68% and 77% at 25 months postoperatively for open surgery.

Conclusions

Endoscopically assisted strip craniectomy offers a safe and effective treatment for sagittal craniosynostosis that is comparable in outcome to calvarial vault reconstruction, with no increase in morbidity and a shorter length of stay.

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Chester K. Yarbrough, Alexander K. Powers, Tae Sung Park, Jeffrey R. Leonard, David D. Limbrick and Matthew D. Smyth

Object

A subset of patients with Chiari malformation Type I (CM-I) presented with acute onset of a neurological deficit. In this study the authors summarize their experience with these patients' clinical presentation, imaging results, timing of surgery, and outcome following decompression.

Methods

The authors reviewed clinical records, imaging studies, and operative notes from all patients undergoing posterior fossa decompression for CM-I at St. Louis Children's Hospital from 1990 to 2008. Of the 189 patients who underwent surgery, 6 were identified with the acute onset of a neurological deficit at presentation.

Results

All 6 children (age range 3–14 years, 3 boys and 3 girls) had either syringomyelia (5 patients) or T2 signal changes in the spinal cord (1 patient) and CM-I on initial MR imaging. Three patients presented after minor trauma (1 with paraparesis, 2 with sensory deficits). Three patients presented without a clear history of trauma (1 with abrupt onset of spontaneous dysphagia and ataxia, 2 with sensory deficits). Decompression was performed at a mean 7.7 ± 4.9 days after symptom onset (7.0 ± 1.6 days after neurosurgical evaluation). In 1 patient, symptoms had resolved by the time of surgery; in the remainder of the patients, clear improvements were noted within 2 weeks of surgery, with complete resolution of symptoms by 12 months postoperatively. Follow-up MR images were obtained in 4 patients, demonstrating improvement in the extent of the syrinx in each patient.

Conclusions

Children with CM-I and syringomyelia can develop acute spinal cord or bulbar deficits with relatively minor head or neck injuries. The prognosis for symptomatic improvement in the observed deficit is good, with each patient in our series showing resolution of deficits over time. However, based on this relatively limited experience, the authors suggest that patients who present with an acute neurological deficit and are found to have CM-I be managed with early posterior fossa decompression. Patients with CM-I and syringomyelia may be at higher risk of acute neurological deficit than those without a syrinx.

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Ian G. Dorward, Jeffrey B. Titus, David D. Limbrick, James M. Johnston, Mary E. Bertrand and Matthew D. Smyth

Object

Patients undergoing epilepsy surgery without evidence of a lesion on MR imaging and without a temporal source for seizure onset generally have less favorable outcomes than patients with structural lesions or temporal onset. However, many of these patients are viable candidates for invasive monitoring and subsequent resection or multiple subpial transections (MSTs). The purpose of this study was to evaluate the surgical treatment of pediatric patients with extratemporal, nonlesional epilepsy in order to better understand the clinical and neuropsychological outcomes expected in this patient group.

Methods

Forty-three pediatric patients with negative results on MR imaging and lateralized, extratemporal findings on electroencephalography underwent invasive monitoring with grid and/or strip electrodes. Thirty-three subsequently had resection of an epileptogenic focus and/or MSTs.

Results

Outcome was classified as Engel class I or II in 54.5% of the patients who underwent resection/MSTs and Engel class III or IV in 45.5%. Use of MSTs was associated with poor outcome. Neuropsychological evaluation showed significant improvement in immediate auditory attention following surgery and revealed several significant results on subgroup analysis. Complications occurred in 14% of patients (a 7% rate per procedure). Ten patients (23%) underwent invasive monitoring without proceeding to therapeutic surgery because no epileptogenic region was amenable to resection. Neuropsychological outcomes were generally stable.

Conclusions

Patients with extratemporal, nonlesional seizures are viable candidates for invasive monitoring with grid/strip electrodes, and good outcomes can be obtained with resective surgery. The use of MSTs may correlate with worse outcome. This study also provides additional data to assist in counseling patients on the risks of negative invasive monitoring, deficits resulting from resection/MSTs, and possible operative complications.