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Andrew J. Dodgshun, Wirginia J. Maixner, Jordan R. Hansford, and Michael J. Sullivan

OBJECTIVE

Pilocytic astrocytomas (PAs) are common brain tumors in children. Optimal management of PA is gross-total resection (GTR), after which event-free survival (EFS) is excellent. The tempo of recurrences, when they do occur, is relatively sparsely reported, and there is no agreed upon surveillance recommendation for patients in this category. It has been suggested that surveillance MRI is performed too frequently and could be safely reduced in both frequency and duration. The authors conducted a retrospective review of pediatric patients with PA who underwent GTR at a single institution over an 18-year period and who had documented recurrences.

METHODS

All patients under 18 years of age who had undergone GTR of a PA between 1996 and 2013 were included in the study. Clinical, radiological, and tumor characteristics were recorded.

RESULTS

Sixty-seven patients met the criteria for GTR over the period studied. The 5-year EFS rate was 95% (95% CI 89%–100%) and overall survival was 100%. Recurrences showed a nonsignificant trend of occurring more commonly in patients with persistent nonenhancing FLAIR abnormalities after surgery, but there was no difference with regard to tumor location. All recurrences occurred before 3 years postresection, all were asymptomatic, and all patients were observed for at least one additional scan after the initial detection during routine surveillance MRI before further therapy was undertaken.

CONCLUSIONS

EFS and overall survival are excellent after GTR in this population with PAs. Progression after recurrence occurs slowly and is asymptomatic. A less intensive schedule of MRI surveillance in this group of patients would result in time and cost savings, without compromising safety. The authors suggest a schedule of 6 MRI scans to be obtained postoperatively, at 3–6 months, then at 1, 2, 3.5, and 5 years.

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Ronnie E. Baticulon, Michael C. Dewan, Nunthasiri Wittayanakorn, Philipp R. Aldana, and Wirginia J. Maixner

OBJECTIVE

There are limited data on the pediatric neurosurgical workforce in Asia and Australasia. The training and clinical practice of pediatric neurosurgeons need to be characterized in order to identify gaps in knowledge and skills, thereby establishing a framework from which to elevate pediatric neurosurgical care in the region.

METHODS

An online survey for pediatric neurosurgeons was created in REDCap (Research Electronic Database Capture), collecting demographic information and data on pediatric neurosurgical training and clinical practice. The link to answer the survey was sent to the mailing lists of the Asian Australasian Society for Pediatric Neurosurgery and the Japanese Society for Pediatric Neurosurgery, disseminated during the 2019 Asian Australasian Pediatric Neurosurgery Congress, and spread through social media. The survey was open to neurosurgeons who operated on patients ≤ 18 years old in Asian Australasian countries, whether or not they had completed fellowship training in pediatric neurosurgery. Descriptive statistics were computed and tabulated. Data were stratified and compared based on surgeon training and World Bank income group.

RESULTS

A total of 155 valid survey responses were analyzed, representing neurosurgeons from 21 countries. A total of 107 (69%) considered themselves pediatric neurosurgeons, of whom 66 (43%) had completed pediatric neurosurgery training. Neurosurgeons in East Asia commonly undergo a fellowship in their home countries, whereas the rest train mostly in North America, Europe, and Australia. A majority (89%) had operating privileges, and subspecialty pediatric training usually lasted from 6 months to 2 years. On average, trained pediatric neurosurgeons perform a higher number of pediatric neurosurgical operations per year compared with nonpediatric-trained respondents (131 ± 129 vs 56 ± 64 [mean ± SD], p = 0.0001). The mean number of total neurosurgical operations per year is similar for both groups (184 ± 129 vs 178 ± 142 [mean ± SD], p = 0.80). Respondents expressed the desire to train further in pediatric epilepsy, spasticity, vascular malformations, craniofacial disorders, and brain tumors.

CONCLUSIONS

Both pediatric and general neurosurgeons provide neurosurgical care to children in Asia and Australasia. There is a need to increase pediatric neurosurgery fellowship programs in the region. Skill sets and training needs in pediatric neurosurgery vary depending on the country’s economic status and between pediatric-trained and nonpediatric-trained surgeons.

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Joseph Yuan-Mou Yang, Richard Beare, Marc L. Seal, A. Simon Harvey, Vicki A. Anderson, and Wirginia J. Maixner

OBJECTIVE

Characterization of intraoperative white matter tract (WMT) shift has the potential to compensate for neuronavigation inaccuracies using preoperative brain imaging. This study aimed to quantify and characterize intraoperative WMT shift from the global hemispheric to the regional tract-based scale and to investigate the impact of intraoperative factors (IOFs).

METHODS

High angular resolution diffusion imaging (HARDI) diffusion-weighted data were acquired over 5 consecutive perioperative time points (MR1 to MR5) in 16 epilepsy patients (8 male; mean age 9.8 years, range 3.8–15.8 years) using diagnostic and intraoperative 3-T MRI scanners. MR1 was the preoperative planning scan. MR2 was the first intraoperative scan acquired with the patient's head fixed in the surgical position. MR3 was the second intraoperative scan acquired following craniotomy and durotomy, prior to lesion resection. MR4 was the last intraoperative scan acquired following lesion resection, prior to wound closure. MR5 was a postoperative scan acquired at the 3-month follow-up visit. Ten association WMT/WMT segments and 1 projection WMT were generated via a probabilistic tractography algorithm from each MRI scan. Image registration was performed through pairwise MRI alignments using the skull segmentation. The MR1 and MR2 pairing represented the first surgical stage. The MR2 and MR3 pairing represented the second surgical stage. The MR3 and MR4 (or MR5) pairing represented the third surgical stage. The WMT shift was quantified by measuring displacements between a pair of WMT centerlines. Linear mixed-effects regression analyses were carried out for 6 IOFs: head rotation, craniotomy size, durotomy size, resected lesion volume, presence of brain edema, and CSF loss via ventricular penetration.

RESULTS

The average WMT shift in the operative hemisphere was 2.37 mm (range 1.92–3.03 mm) during the first surgical stage, 2.19 mm (range 1.90–3.65 mm) during the second surgical stage, and 2.92 mm (range 2.19–4.32 mm) during the third surgical stage. Greater WMT shift occurred in the operative than the nonoperative hemisphere, in the WMTs adjacent to the surgical lesion rather than those remote to it, and in the superficial rather than the deep segment of the pyramidal tract. Durotomy size and resection size were significant, independent IOFs affecting WMT shift. The presence of brain edema was a marginally significant IOF. Craniotomy size, degree of head rotation, and ventricular penetration were not significant IOFs affecting WMT shift.

CONCLUSIONS

WMT shift occurs noticeably in tracts adjacent to the surgical lesions, and those motor tracts superficially placed in the operative hemisphere. Intraoperative probabilistic HARDI tractography following craniotomy, durotomy, and lesion resection may compensate for intraoperative WMT shift and improve neuronavigation accuracy.

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Ahmad R. Mohamed, Jeremy L. Freeman, Wirginia Maixner, Catherine A. Bailey, Jacquie A. Wrennall, and A. Simon Harvey

Object

Temporoparietooccipital (TPO) disconnection is described mainly in children with diffuse posterior quadrant lesions and concordant electroencephalography (EEG) findings. The authors report on 16 children who underwent TPO surgery, including 4 with no definite epileptogenic lesion and 8 with generalized electroclinical manifestations.

Methods

The authors conducted a retrospective review of clinical, neuropsychological, EEG, imaging, and histopathological data in 16 children with intractable epilepsy who underwent TPO disconnection and/or resection at their center between December 1998 and March 2010.

Results

Seizure onset occurred between the ages of 1 and 24 months, and TPO surgery was performed between the ages of 0.2 and 17 years. All children had refractory seizures, including epileptic spasms in 10 and tonic seizures in 7, and all had developmental delay. Twelve children had epileptogenic lesions on MR imaging, including 6 with posterior quadrant dysplasia. Four children had only subtle white matter signal change or unusual sulcation on MR imaging, associated with subtle but concordant EEG and functional imaging abnormalities. After a mean follow-up of 52 months (range 12–114 months), 9 children (56%) are seizure-free and 5 (31%) experienced seizure reduction of greater than 50%. Focal or regional background slowing on EEG was correlated with favorable seizure outcome. Five children showed developmental progress and 3 had acceleration in development following surgery. None of the children developed new motor deficits postoperatively.

Conclusions

Temporoparietooccipital disconnection is an effective, motor-sparing epilepsy surgery procedure for selected children with refractory focal or generalized seizures with localization to the posterior quadrant on 1 side, with or without a discrete lesion on MR imaging.

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Vivek Josan, Paul Smith, Andrew Kornberg, Christian Rickert, and Wirginia Maixner

✓Dysembryoplastic neuroepithelial tumors (DNETs) are benign supratentorial tumors based in the cerebral cortex. They usually are found in children and young adults with seizures that tend to become refractory to medical treatment. In the vast majority of cases resection results in good seizure control, and adjuvant therapy is not required. When tumors thought to be DNETs are not resected due to their proximity to eloquent cortex, lack of change in the clinical and neuro-imaging features over time supports the diagnosis of DNET.

The authors report on a patient in whom a pilocytic astrocytoma developed within a DNET, raising questions regarding the classification of these lesions and the need for lifelong clinical and imaging surveillance. This paper adds to the growing body of literature about the biological behavior of these lesions.

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Shaun P. Appaduray, James A. J. King, Alison Wray, Patrick Lo, and Wirginia Maixner

Pediatric dural arteriovenous malformations (dAVMs) are rare lesions that have a high mortality rate and require complex management. The authors report 3 cases of pediatric dAVMs that presented with macrocrania and extracranial venous distension. Dural sinus thrombosis developed in 2 of the cases prior to any intervention, which is an unusual occurrence for this particular disease. All 3 cases were treated using staged endovascular embolization with a favorable outcome in 1 case and a poor outcome in the other 2 cases. Complications developed in all cases and included dural sinus thrombosis, parenchymal hemorrhage, intracranial venous hypertension, and seizures. The strategies and challenges used in managing these patients will be presented and discussed, along with a review of the literature. While outcomes remain poor, the authors conclude that prompt treatment with endovascular embolization provides the best results for children with these lesions. A well-established venous collateral circulation draining directly to the internal jugular veins may further improve the rate of favorable outcome after embolization.

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Marie Bourgeois, Christian Sainte-Rose, Giuseppe Cinalli, Wirginia Maixner, Conor Malucci, Michel Zerah, Alain Pierre-Kahn, Dominique Renier, Elisabeth Hoppe-Hirsch, and Jean Aicardi

The incidence of epilepsy among children with hydrocephalus and its relation to shunts and their complications, raised intracranial pressure (ICP), and developmental outcome are explored in a retrospective study.

The authors studied a series of 802 children with hydrocephalus due to varying causes, who were treated by ventriculoperitoneal shunt placement between 1980 and 1990, with a mean follow-up period of 8 years. Patients who had tumoral hydrocephalus and those whose files lacked significant data were excluded. Data extracted from medical records, including history of the hydrocephalus and history of seizures, if any, were analyzed.

Thirty-two percent of the children had epilepsy, the onset of which frequently occurred at approximately the same time that the diagnosis of hydrocephalus was made. The majority of the affected children had severe uncontrolled epilepsy. The incidence of epilepsy was significantly affected by the original cause of the hydrocephalus. The presence of radiological abnormalities was also found to be a significant predictor of epilepsy. Similarly, shunt complications predisposed to epilepsy. Episodes of raised ICP related to hydrocephalus or in association with shunt malfunction may also predispose to epileptic seizures. Furthermore, the presence of a shunt by itself seems able to promote an epileptogenic focus. Finally, epilepsy appears to be an important predictor of poor intellectual outcome in hydrocephalic children with shunts.

A prospective study is needed to identify clearly and confirm avoidable factors predisposing to seizures in these children so that we can strive to reduce the incidence of these seizures and, subsequently, improve quality of life.

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Marie Bourgeois, Christian Sainte-Rose, Giuseppe Cinalli, Wirginia Maixner, Conor Malucci, Michel Zerah, Alain Pierre-Kahn, Dominique Renier, Elisabeth Hoppe-Hirsch, and Jean Aicardi

Object. The incidence of epilepsy among children with hydrocephalus and its relation to shunts and their complications, raised intracranial pressure (ICP), and developmental outcome are explored in a retrospective study.

Methods. The authors studied a series of 802 children with hydrocephalus due to varying causes, who were treated by ventriculoperitoneal shunt placement between 1980 and 1990, with a mean follow-up period of 8 years. Patients who had tumoral hydrocephalus and those whose files lacked significant data were excluded. Data extracted from medical records, including history of the hydrocephalus and history of seizures, if any, were analyzed.

Thirty-two percent of the children had epilepsy, the onset of which frequently occurred at approximately the same time that the diagnosis of hydrocephalus was made. The majority of the affected children had severe uncontrolled epilepsy. The incidence of epilepsy was significantly affected by the original cause of the hydrocephalus. The presence of radiological abnormalities was also found to be a significant predictor of epilepsy. Similarly, shunt complications predisposed to epilepsy. Episodes of raised ICP related to hydrocephalus or in association with shunt malfunction may also predispose to epileptic seizures. Furthermore, the presence of a shunt by itself seems able to promote an epileptogenic focus. Finally, epilepsy appears to be an important predictor of poor intellectual outcome in hydrocephalic children with shunts.

Conclusions. A prospective study is needed to identify clearly and confirm avoidable factors predisposing to seizures in these children so that we can strive to reduce the incidence of these seizures and, subsequently, improve these children's quality of life.

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Philippe Pencalet, Wirginia Maixner, Christian Sainte-Rose, Arielle Lellouch-Tubiana, Giuseppe Cinalli, Michel Zerah, Alain Pierre-Kahn, Elisabeth Hoppe-Hirsch, Marie Bourgeois, and Dominique Renier

Object. Cerebellar astrocytomas are benign tumors of childhood known to be associated with excellent long-term survival in patients in whom complete surgical resection is possible. However, the roles of other factors—clinical, radiological, histological, and therapeutic—in the survival of the patient, tumor recurrence, and long-term patient outcome remain imprecise. The goal of this study was to examine these factors and their relationships.

Methods. To clarify these issues a retrospective review was conducted of 168 children who were surgically treated for a cerebellar astrocytoma at Hôpital Necker—Enfants Malades between 1955 and 1995. These patients' clinical files were examined, the histological characteristics of their tumors were reviewed, and their outcomes were assessed according to Bloom's scale and the Wechsler intelligence quotient test.

Of the 168 patients in the study, 91 were male and 77 were female with a mean age of 6.9 years and a mean follow up lasting 7.7 years. Tumors were identified as being strictly located in the cerebellum in 76.2% of the patients and as involving the brainstem (referred to as the “transitional form”) in 23.8% of the patients. Complete surgical excision was possible in 88.7% of cases. There was a total mortality rate of 4.2% and a tumor recurrence rate of 9.5%. Fifty-eight percent of the patients had no neurological sequelae at follow-up evaluation.

Pejorative factors that were discovered by multivariate analysis to be important included: a long preoperative duration of symptoms and the transitional form of tumor with respect to survival; incomplete tumor excision with respect to an increased risk of recurrence; and a long preoperative duration of symptoms, an early epoch during which surgery was performed (1955–1974), severe ventricular dilation, and the transitional form of tumor with respect to a poorer long-term patient outcome.

Conclusions. The presence of brainstem involvement (tumor in the transitional form) emerged as a significant negative prognostic factor and should be treated as a distinct nosological entity. The extent of surgical excision has a significant bearing on the risk of tumor recurrence.

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Marie Bourgeois, Christian Sainte-Rose, Arielle Lellouch-Tubiana, Conor Malucci, Francis Brunelle, Wirginia Maixner, Giuseppe Cinalli, Alain Pierre-Kahn, Dominique Renier, Michel Zerah, Jean-François Hirsch, Francoise Goutières, and Jean Aicardi

Object. Surgery in children with epilepsy is a new, evolving field. The important practical issues have been to define strategies for choosing the most suitable candidates and the type and optimal timing of epilepsy surgery. This study was undertaken to elucidate these points.

Methods. To identify the factors that correlated with outcome, the authors analyzed a series of 200 children (aged 1–15 years (mean 8.7 years) who underwent surgery between 1981 and 1996 at the Hôpital Necker—Enfants Malades. In 171 cases (85.5%) the epilepsy was medically refractory and was associated with focal cortical lesions. Surgery consisted of resection of the lesion without specifically attempting to identify and remove the “epileptogenic area.”

In the group of children whose seizures were medically refractory, the mean follow-up period was 5.8 years. According to Engel's classification, 71.3% of these children became seizure free (Class Ia,) whereas 82% were in Class I. A multivariate statistical analysis revealed that among all the factors studied, the success of surgery in a patient in whom there was a good clinical/electroencephalogram/imaging correlation depended on the patient's having undergone a minimally traumatic operation, a complete resection of the lesion, and a short preoperative seizure duration.

After the surgical control of epilepsy, behavior disorders were more improved (31% of all patients) than cognitive function (25%). The patient age at onset, duration and frequency of seizures, intractability of the disease to therapy, and seizure characteristics were correlated with cognitive, behavioral, and academic performance pre- and postoperatively. Multivariate statistical analysis revealed that cognitive dysfunction correlated highly with the duration of epilepsy prior to surgery, whereas behavioral disorders correlated more with seizure frequency.

Conclusions. These data must be taken into account when selecting patients for surgical treatment and when deciding the timing of surgery. Early surgical intervention allows for optimum brain development.