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Mony Benifla, Francesco Sala, John Jane Jr., Hiroshi Otsubo, Ayako Ochi, James Drake, Shelly Weiss, Elizabeth Donner, Ayataka Fujimoto, Stephanie Holowka, Elysa Widjaja, O. Carter Snead III, Mary Lou Smith, Mandeep S. Tamber and James T. Rutka

Object

The authors undertook this study to review their experience with cortical resections in the rolandic region in children with intractable epilepsy.

Methods

The authors retrospectively reviewed the medical records obtained in 22 children with intractable epilepsy arising from the rolandic region. All patients underwent preoperative electroencephalography (EEG), MR imaging, prolonged video-EEG recordings, functional MR imaging, magnetoencephalography, and in some instances PET/SPECT studies. In 21 patients invasive subdural grid and depth electrode monitoring was performed. Resection of the epileptogenic zones in the rolandic region was undertaken in all cases. Seizure outcome was graded according to the Engel classification. Functional outcome was determined using validated outcome scores.

Results

There were 10 girls and 12 boys, whose mean age at seizure onset was 3.2 years. The mean age at surgery was 10 years. Seizure duration prior to surgery was a mean of 7.4 years. Nine patients had preoperative hemiparesis. Neuropsychological testing revealed impairment in some domains in 19 patients in whom evaluation was possible. Magnetic resonance imaging abnormalities were identified in 19 patients. Magnetoencephalography was performed in all patients and showed perirolandic spike clusters on the affected side in 20 patients. The mean duration of invasive monitoring was 4.2 days. The mean number of seizures during the period of invasive monitoring was 17. All patients underwent resection that involved primary motor and/or sensory cortex. The most common pathological entity encountered was cortical dysplasia, in 13 children. Immediately postoperatively, 20 patients had differing degrees of hemiparesis, from mild to severe. The hemiparesis improved in all affected patients by 3–6 months postoperatively. With a mean follow-up of 4.1 years (minimum 2 years), seizure outcome in 14 children (64%) was Engel Class I and seizure outcome in 4 (18%) was Engel Class II. In this series, seizure outcome following perirolandic resection was intimately related to the child's age at the time of surgery. By univariate logistic regression analysis, age at surgery was a statistically significant factor predicting seizure outcome (p < 0.024).

Conclusions

Resection of rolandic cortex for intractable epilepsy is possible with expected morbidity. Accurate mapping of regions of functional cortex and epileptogenic zones may lead to improved seizure outcome in children with intractable rolandic epilepsy. It is important to counsel patients and families preoperatively to prepare them for possible worsened functional outcome involving motor, sensory and/or language pathways.

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Editorial

Achondroplasia

Arnold H. Menezes

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James A. J. King, Shobhan Vachhrajani, James M. Drake and James T. Rutka

Object

Achondroplasia is the most common form of human short-limbed dwarfism. The pediatric neurosurgeon is frequently required to treat children with achondroplasia who have hydrocephalus, cervicomedullary compression (CMD), and spinal canal stenosis. Accordingly, the authors have reviewed the experience of neurosurgery in children with achondroplasia at The Hospital for Sick Children.

Methods

The medical records and neurosurgery database at The Hospital for Sick Children were searched to identify all children with achondroplasia who underwent at least 1 neurosurgical procedure between 1956 and the present.

Results

Twenty-nine children with achondroplasia underwent 85 surgical procedures: 52 for CSF diversion in 12 patients, 20 for CMD in 18 patients, 8 for spinal disorders in 4 patients, and 5 for miscellaneous purposes in 4 patients. The CSF shunts were placed almost exclusively before 1990 and were associated with a significant number of complications. Patients undergoing CMD did very well, with only 1 patient failing to improve clinically.

Conclusions

This review provides a historical perspective on the evolution of treatment of pediatric patients with achondroplasia. The use of CSF diversion procedures, formerly fraught with complications, is now rare following the realization of the natural history of CSF space enlargement in these patients. Cervicomedullary compression is more commonly recognized due to better imaging. Central apnea is now better detected by routine sleep studies. Spine surgery, although rare, requires evaluation of both spinal stenosis and instability. These patients are best evaluated by a multidisciplinary team.

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Sasha C. Burn, Olaf Ansorge, Reinhard Zeller and James M. Drake

Object

Osteoid osteomas and osteoblastoma of the spine are rare lesions in childhood, and management strategies have changed. The authors reviewed their recent experience with these 2 types of lesions to elucidate current treatment modalities and outcomes.

Methods

Case records and radiographic images from all cases of osteoid osteoma and osteoblastoma diagnosed between 1993 and 2008 were retrospectively reviewed, including those managed nonsurgically.

Results

Thirty cases were identified; 22 were treated surgically and 8 were managed nonsurgically. The patients' mean age at presentation was 13 years (range 3–17 years). Of 30 patients, 29 (97%) presented with pain; 7 (23%) had scoliosis at presentation; 12 (40%) experienced relief with nonsteroidal antiinflammatory medication. Osteoid osteoma was diagnosed in 7 (32%) of the 22 patients who underwent surgery, and osteoblastoma in 15 (68%). Nine (41%) of the 22 surgically treated patients underwent fusion procedures (bone onlay or instrumentation) at the time of surgery. Pain freedom without medication had been achieved in 16 (73%) of the 22 surgically treated patients at a mean follow-up of 28 months (range 2–75 months) and was confirmed in 3 (38%) of the 8 nonsurgically treated patients at a mean follow-up of 33 months (range 24–51 months).

Conclusions

Osteoid osteomas and osteoblastomas can present challenging management problems in pediatric patients. In the majority of cases in which conservative therapy fails or pathological diagnosis is required, surgery using modern intraoperative imaging and spinal instrumentation can provide symptom relief and tumor control.

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Ratan D. Bhardwaj, Kurtis I. Auguste, Abhaya V. Kulkarni, Peter B. Dirks, James M. Drake and James T. Rutka

Object

Because of their location and biological behavior, brainstem cavernous malformations (CMs) pose a formidable clinical challenge to the neurosurgeon. The optimal management of these lesions requires considerable neurosurgical judgment. Accordingly, the authors reviewed their experience with the management of pediatric brainstem CMs at the Hospital for Sick Children.

Methods

The authors performed a retrospective chart review of pediatric patients who had received diagnoses of a brainstem CM at the Hospital for Sick Children over the past 20 years.

Results

Twenty patients were diagnosed with brainstem CMs. The mean age at diagnosis was 10.1 ± 5.4 years, and the patients included 13 boys and 7 girls. The mean maximal diameter of the CM was 14.3 ± 11.2 mm. The lesions were evenly distributed on the right and left sides of the brainstem with 4 midbrain, 13 pontine, and 3 medullary lesions. Seven patients underwent surgery for the management of their CMs, with a mean age at presentation of 5.2 years, and a mean CM size of 21.0 mm. Of note from the surgical group, 2 patients had a family history of CMs, 2 lesions were medullary, the CM reached a pial surface in 6 of 7 patients, and 6 of 7 lesions were located on the right side. The mean age at presentation among the 13 patients in the nonsurgical group was 12.7 years, and the mean CM size was 10.6 mm. Seven of these patients had a prior history of radiation for tumor, and only 3 had lesions that reached a pial surface.

Conclusions

The management of brainstem CMs in children is influenced by multiple factors. The majority of patients received conservative management and tended to be asymptomatic with smaller lesions. Patients with larger lesions and direct pial contact, in whom symptoms arose at a younger age were more likely to undergo surgical management. A history of familial CM was also a predictor for receiving surgical treatment. No patients with a prior history of radiation therapy underwent surgery for CMs. The presence of multiple lesions seemed to have no impact on the type of management chosen. Patients who underwent surgery did suffer morbidity related to the procedure, and tended to improve clinically over time. Conservative management was associated with new deficits arising in children, some of which improved with time. Consideration of many clinical and radiological parameters is thus prudent when managing the care of children with brainstem CMs.

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Ichiro Sugiyama, Katsumi Imai, Yu Yamaguchi, Ayako Ochi, Yoko Akizuki, Cristina Go, Tomoyuki Akiyama, O. Carter Snead III, James T. Rutka, James M. Drake, Elysa Widjaja, Sylvester H. Chuang, Doug Cheyne and Hiroshi Otsubo

Object

Magnetoencephalography (MEG) has been typically used to localize epileptic activity by modeling interictal activity as equivalent current dipoles (ECDs). Synthetic aperture magnetometry (SAM) is a recently developed adaptive spatial filtering algorithm for MEG that provides some advantages over the ECD approach. The SAM-kurtosis algorithm (also known as SAM[g2]) additionally provides automated temporal detection of spike sources by using excess kurtosis value (steepness of epileptic spike on virtual sensors). To evaluate the efficacy of the SAM(g2) method, the authors applied it to readings obtained in children with intractable epilepsy secondary to tuberous sclerosis complex (TSC), and compared them to localizations obtained with ECDs.

Methods

The authors studied 13 children with TSC (7 girls) whose ages ranged from 13 months to 16.3 years (mean 7.3 years). Video electroencephalography, MR imaging, and MEG studies were analyzed. A single ECD model was applied to localize ECD clusters. The SAM(g2) value was calculated at each SAM(g2) virtual voxel in the patient's MR imaging–defined brain volume. The authors defined the epileptic voxels of SAM(g2) (evSAM[g2]) as those with local peak kurtosis values higher than half of the maximum. A clustering of ECDs had to contain ≥ 6 ECDs within 1 cm of each other, and a grouping of evSAM(g2)s had to contain ≥ 3 evSAM(g2)s within 1 cm of each other. The authors then compared both ECD clusters and evSAM(g2) groups with the resection area and correlated these data with seizure outcome.

Results

Seizures started when patients were between 6 weeks and 8 years of age (median 6 months), and became intractable secondary to multiple tubers in all cases. Ictal onset on scalp video electroencephalography was lateralized in 8 patients (62%). The MEG studies showed multiple ECD clusters in 7 patients (54%). The SAM(g2) method showed multiple groups of epileptic voxels in 8 patients (62%). Colocalization of grouped evSAM(g2) with ECD clusters ranged from 20 to 100%, with a mean of 82%. Eight patients underwent resection of single (1 patient) and multiple (7 patients) lobes, with 6 patients achieving freedom from seizures. Of 8 patients who underwent surgery, in 7 the resection area covered ECD clusters and grouped evSAM(g2)s. In the remaining patient the resection area partially included the ECD cluster and grouped evSAM(g2)s. Six of the 7 patients became seizure free.

Conclusions

The combination of SAM(g2) and ECD analyses succeeded in localizing the complex epileptic zones in children with TSC who had intractable epilepsy secondary to multiple cortical tubers. For the subset of children with TSC who present with early-onset and nonlateralized seizures, MEG studies in which SAM(g2) and ECD are used might identify suitable candidates for resection to control seizures.

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Sasha C. Burn, Reinhard Zeller and James M. Drake

Object

Intrathecal baclofen is an effective treatment for spasticity in patients with cerebral palsy. There has been increasing concern, however, that intrathecal baclofen may accelerate the development of scoliosis in this population. To this end, the authors reviewed their population of pediatric patients with baclofen pumps to assess the incidence of scoliosis.

Methods

This was a retrospective chart and radiology review of all pediatric patients with baclofen pumps. Cobb angles were measured preoperatively and on follow-up images.

Results

Of 38 patients identified, 32 had adequate data available for inclusion in the study (16 with cerebral palsy, 7 with dystonic cerebral palsy, 4 with head injury, and 5 with other diagnoses). The mean age at pump insertion was 10.6 years and the mean follow-up period was 31 months (range 1–118 months). The mean annual Cobb angle progression was 19° (range 0–68°, median 12°).

Conclusions

In the authors' group of patients there was notable development and progression of scoliosis at a greater than previously reported rate for the same patient population, and also greater than previously reported patients with intrathecal baclofen pumps. The largest possible confounding factor in this study was the insertion of the pump before skeletal maturity and therefore coinciding with the time when scoliosis may be developing naturally. A prospective study is recommended to gather further data on the development of scoliosis in this particular population with intrathecal baclofen pumps.

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James A. J. King, Kurtis I. Auguste, William Halliday, James M. Drake and Abhaya V. Kulkarni

Hydrocephalus secondary to giant retrocerebellar cysts in infancy is a challenging condition and many treatment options exist. The authors report on 3 consecutive cases involving infants under the age of 6 months treated successfully with ventriculocystostomy in combination with direct hydrocephalus treatment (endoscopic third ventriculostomy or shunt placement). They describe the operative procedure, the surgical morbidity, and outcome in each case and review the literature regarding surgical approaches to this condition.

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James M. Drake, Jay Riva-Cambrin, Andrew Jea, Kurtis Auguste, Mandeep Tamber and Maria Lamberti-Pasculli

Object

Complications of specific pediatric neurosurgical procedures are well recognized. However, focused surveillance on a specific neurosurgical unit, for all procedures, may lead to better understanding of the most important complications, and allow targeted strategies for quality improvement.

Methods

The authors prospectively recorded the morbidity and mortality events at a large pediatric neurosurgical unit over a 2-year period. Morbidity was defined as any significant adverse outcome or death (for obstructive shunt failure, within 30 days). Multiple and unrelated complications in the same patient were recorded as separate events.

Results

There were 1082 surgical procedures performed during the evaluation period. One hundred seventy-seven complications (16.4%) occurred in 147 patients. By procedure, the most common complications occurred in vascular surgery (41.7%) and brain tumor surgery (27.9%). The most common complications were CSF leakage (31 cases), a new neurological deficit (27 cases), early shunt or endoscopic third ventriculostomy obstruction (27 cases), and shunt infection (24 cases). Meningitis occurred in 19 cases: in 58% of shunt infections, 13% of CSF leaks, and 10% of wound infections. Sixty-four percent of adverse events required a second procedure, most commonly an external ventricular drain placement or shunt revision.

Conclusions

Complications in pediatric neurosurgical procedures are common, result in significant morbidity, and more than half the time require a repeat surgical procedure. Targeted strategies to prevent common complications, such as shunt infections or CSF leaks, might significantly reduce this burden.

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Editorial

Prediction, with restriction

Alan R. Cohen