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Deanna Sasaki-Adams, Abhaya Kulkarni, James Rutka, Peter Dirks, Michael Taylor and James M. Drake

✓Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by disruption of normal collagen formation resulting in varying degrees of skeletal vulnerability, ligamentous laxity, and scleral discoloration. Children with OI may suffer from complex neurosurgical problems affecting the brain and spine. The authors sought to determine the neurosurgical implications of OI in a cohort of patients treated at a quaternary care center for pediatrics.

The authors reviewed the case histories of 10 children with OI treated by the neurosurgical service at the Hospital for Sick Children in Toronto between January 1988 and March 2007. The cases of 4 of these children are highlighted in the article. The most common neurosurgical conditions encountered in this cohort included macrocephaly in 5 patients, subdural hematoma in 3 patients, epidural hematoma in 2 patients, and hydrocephalus in 3 patients. Basilar invagination and spinal fractures were observed in 20% of the cohort. Although some patients could be treated nonoperatively, several required craniotomy for clot evacuation, decompression, and spinal fixation for fracture or basilar invagination, and cerebrospinal fluid shunt insertion.

Neurosurgical conditions affecting patients with OI include macrocephaly, the development of an acute intracranial hematoma after often minimal trauma, the development of chronic subdural fluid collections that may require drainage, hydrocephalus (both communicating and noncommunicating), basilar invagination, and subaxial spinal fractures. Surgery may be complicated in some children because of the underlying bone fragility and bleeding diathesis commonly observed in patients with OI.

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Niketa C. Shah, Amit Ray, Ute Bartels, James Rutka, Eric Bouffet, James Drake, Cynthia E. Hawkins and Annie Huang

✓ The authors report on 2 newborn infants with the unusual presentation of intrinsic brainstem tumors. Both nondysmorphic, full-term neonates had cranial nerve palsies and hypotonia. Diagnoses of diffuse intrinsic brainstem gliomas were made on the basis of magnetic resonance imaging, which showed large expansive, nonenhancing intrinsic pontine masses. Intrinsic pontine tumors, characteristically seen in school-age children, are most often high-grade gliomas that are almost invariably fatal. However, the microanatomy and natural history of pontine tumors in neonates are unknown. With parental consent, both newborns were treated expectantly with supportive care but died of progressive disease by 2 weeks of age. In one child, postmortem examination revealed a primary brainstem primitive neuroectodermal tumor. The authors conclude that, as in older children, neonatal intrinsic brainstem tumors may be of a highly malignant nature. The rapid tumor progression in both cases indicates that where a diagnostic procedure may pose significant risks, supportive observation can aid in distinguishing malignant from benign tumor growth.

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Benjamin W. Y. Lo, Abhaya V. Kulkarni, James T. Rutka, Andrew Jea, James M. Drake, Maria Lamberti-Pasculli, Peter B. Dirks and Lehana Thabane

Object

Cephaloceles represent primary axial mesodermal defects, occurring in 0.8–4 per 10,000 live births. Prior studies have reported posterior location, hydrocephalus, microcephaly, seizure, and presence of brain tissue as poor prognostic markers for neurological outcome. However, these studies were small and the results were analyzed using univariate tests. The purpose of this study was to investigate the potential risk factors for the occurrence of developmental delay in patients with cephaloceles, using both univariate and multivariable regression techniques.

Methods

This is a retrospective cohort study of cephalocele cases treated at the Hospital for Sick Children between 1990 and 2006. Two independent investigators collected the data from the Hospital for Sick Children Encephalocele Database and hospital charts. Developmental assessments were made by general pediatricians and neuropsychologists. Both univariate analysis (α = 0.10) and multivariable logistic regression analysis (α = 0.05) were performed.

Results

Eighty-five cases of cephaloceles were identified. The patient group consisted of 48 boys and 37 girls. Sixty-eight lesions were encephaloceles and 17 were meningoceles. The distribution was as follows: frontal (40 lesions), occipital (33), and parietal (12). Associated conditions included hydrocephalus (23), seizure disorder (17), microcephaly (6), corpus callosal abnormalities (15), heterotopias (9), cerebral dysgenesis (11), and myelomeningocele (1). Evaluation of long-term development revealed that 41 patients (48%) had normal development, 9 (11%) had mild delay, 14 (16%) had moderate delay, and 21 (25%) had severe delay. Hydrocephalus, seizure disorder, microcephaly, presence of associated intracranial abnormalities, and presence of brain tissue were significantly associated with poor outcome on univariate analysis. Multivariable analysis revealed hydrocephalus and presence of intracranial abnormalities to be statistically significant predictors of developmental delay.

Conclusions

To the authors' knowledge, this is one of the largest North American cephalocele series documented. Unlike prior studies, location of the cephaloceles is not a significant predictor of outcome. The multivariable regression analysis demonstrates hydrocephalus and the presence of associated intracranial abnormalities as variables with cumulative predictive effects for developmental delay.

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James M. Drake and Jay Riva-Cambrin

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James A. J. King, William Halliday and James M. Drake

The authors report on a child with known neurofibromatosis Type 1 who developed high-grade diffuse leptomeningeal gliomatosis, without a known primary glioma. To the authors' knowledge, this is the first report of the coexistence of these conditions in a child.

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Jay Riva-Cambrin, Allan S. Detsky, Maria Lamberti-Pasculli, Michael A. Sargent, Derek Armstrong, Rahim Moineddin, D. Douglas Cochrane and James M. Drake

Object

Approximately 30% of children with posterior fossa tumors exhibit hydrocephalus after tumor resection. Recent literature has suggested that prophylactic endoscopic third ventriculostomy diminishes the risk of this event. Because the majority of patients will not have postoperative hydrocephalus, a preoperative clinical prediction rule that identifies patients at high or low risk for postresection hydrocephalus would be helpful to optimize the care of these children.

Methods

The authors evaluated a derivation cohort of 343 consecutive children with posterior fossa tumors who underwent treatment between 1989 and 2003. Multivariate methods were used on these data to generate the Canadian Preoperative Prediction Rule for Hydrocephalus. The rule's estimated risk of postresection hydrocephalus was compared with risk observed in 111 independent patients in the validation cohort.

Results

Variables identified as significant in predicting postresection hydrocephalus were age < 2 years (score of 3), papilledema (score of 1), moderate to severe hydrocephalus (score of 2), cerebral metastases (score of 3), and specific estimated tumor pathologies (score of 1). Patients with scores ≥ 5 were deemed as high risk. Predicted probabilities for the high- and low-risk groups were 0.73 and 0.25, respectively, from the derivation cohort, and 0.59 and 0.14 after prevalence adjustment compared with the observed values of 0.42 and 0.17 in the validation cohort.

Conclusions

A patient's score on the Preoperative Prediction Rule for Hydrocephalus will allow improved patient counseling and surgical planning by identifying patients at high risk of developing postresection hydrocephalus. These patients might selectively be exposed to the risks of preresection CSF diversion to improve outcome.

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John C. Wellons III, Chevis N. Shannon, Abhaya V. Kulkarni, Tamara D. Simon, Jay Riva-Cambrin, William E. Whitehead, W. Jerry Oakes, James M. Drake, Thomas G. Luerssen, Marion L. Walker, John R. W. Kestle and for the Hydrocephalus Clinical Research Network

Object

The purpose of this study was to define the incidence of permanent shunt placement and infection in patients who have undergone the 2 most commonly performed temporizing procedures for posthemorrhagic hydrocephalus (PHH) of prematurity: ventriculosubgaleal (VSG) shunt placement and ventricular reservoir placement for intermittent tapping.

Methods

The 4 centers of the Hydrocephalus Clinical Research Network participated in a retrospective chart review of infants with PHH who underwent treatment at each institution between 2001 and 2006. Patients were included if they had received a diagnosis of Grade 3 or 4 intraventricular hemorrhage, weighed < 1500 g at birth, and had received surgical intervention. The authors determined the incidence of conversion from a temporizing device to a permanent shunt, the incidence of CSF infection during temporization, and the 6-month CSF infection rate after permanent shunt placement.

Results

Thirty-one (86%) of 36 patients who received VSG shunts and 61 (69%) of 88 patients who received ventricular reservoirs received permanent CSF diversion with a shunt (p = 0.05). Five patients (14%) in the VSG shunt group had CSF infections during temporization, compared with 11 patients (13%) in the ventricular reservoir group (p = 0.83). The 6-month incidence of permanent shunt infection in the VSG shunt group was 16% (5 of 31), compared with 12% (7 of 61) in the reservoir placement group (p = 0.65). For the first 6 months after permanent shunt placement, infants with no preceding temporizing procedure had an infection rate of 5% (1 of 20 infants) and those who had undergone a temporizing procedure had an infection rate of 13% (12 of 92; p = 0.45).

Conclusions

The use of intermittent tapping of ventricular reservoirs in this population appears to lead to a lower incidence of permanent shunt placement than the use of VSG shunts. The incidence of infection during temporization and for the initial 6 months after conversion appears comparable for both groups. The apparent difference identified in this pilot study requires confirmation in a more rigorous study.

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Ibrahim Ahmed, Kurtis I. Auguste, Shobhan Vachhrajani, Peter B. Dirks, James M. Drake and James T. Rutka

Object

Epidermoid tumors are benign lesions representing 1% of all intracranial tumors. There have been few pediatric series of intracranial epidermoid tumors reported previously. The authors present their experience in the management of these lesions.

Methods

The neurosurgical database at the Hospital for Sick Children was searched for children with surgically managed intracranial epidermoid tumors. The patients' charts were reviewed for demographic data, details of clinical presentation, surgical therapy, and follow-up. Ethics board approval was obtained for this study.

Results

Seven children, all girls, were identified who met the inclusion criteria between 1980 and 2007. The average age at surgery was 11.2 years (range 8–15 years), and the mean maximal tumor diameter was 2.1 cm. Headache was the most common presenting symptom, and 1 tumor was found incidentally. Most patients had normal neurological examinations, but meningism was found in 2 cases. There were 3 cerebellopontine angle lesions, 1 pontomedullary lesion, and 3 supratentorial tumors. Hydrocephalus developed in 1 patient after aseptic meningitis, and she underwent shunt placement. There were no operative deaths. Complete resection could be performed in 2 patients. One patient experienced a small recurrence that did not require a repeated operation, while 1 subtotally resected lesion recurred and the patient underwent a second operation.

Conclusions

Intracranial epidermoid tumors are rare in the pediatric population. Total resection is desirable to minimize the risk of postoperative aseptic meningitis, hydrocephalus, and tumor recurrence. Aggressive neurosurgical resection may be associated with cranial nerve or ischemic deficits, however. In these cases, neurosurgical judgment at the time of surgery is warranted to ensure maximum resection while minimizing postoperative neurological deficits.

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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