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Ian F. Pollack, Sameer Agnihotri and Alberto Broniscer

Brain tumors are the most common solid tumors in children, and, unfortunately, many subtypes continue to have a suboptimal long-term outcome. During the last several years, however, remarkable advances in our understanding of the molecular underpinnings of these tumors have occurred as a result of high-resolution genomic, epigenetic, and transcriptomic profiling, which have provided insights for improved tumor categorization and molecularly directed therapies. While tumors such as medulloblastomas have been historically grouped into standard- and high-risk categories, it is now recognized that these tumors encompass four or more molecular subsets with distinct clinical and molecular characteristics. Likewise, high-grade glioma, which for decades was considered a single high-risk entity, is now known to comprise multiple subsets of tumors that differ in terms of patient age, tumor location, and prognosis. The situation is even more complex for ependymoma, for which at least nine subsets of tumors have been described. Conversely, the majority of pilocytic astrocytomas appear to result from genetic changes that alter a single, therapeutically targetable molecular pathway. Accordingly, the present era is one in which treatment is evolving from the historical standard of radiation and conventional chemotherapy to a more nuanced approach in which these modalities are applied in a risk-adapted framework and molecularly targeted therapies are implemented to augment or, in some cases, replace conventional therapy. Herein, the authors review advances in the categorization and treatment of several of the more common pediatric brain tumors and discuss current and future directions in tumor management that hold significant promise for patients with these challenging tumors.

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Jennifer L. Quon, Lily H. Kim, Robert M. Lober, Maryam Maleki, Gary K. Steinberg and Kristen W. Yeom

OBJECTIVE

Moyamoya disease is a dynamic cerebrovascular condition that often requires vascular surveillance. Arterial spin labeling (ASL) is an MR perfusion method that is increasingly used for stroke and other various neurovascular pathologies. Unlike perfusion-weighted MRI, ASL uses endogenous water molecules for signal and therefore obviates gadolinium use; and provides direct, not relative, quantitative cerebral blood flow (CBF) measures. Presently, the potential role of ASL for evaluating postoperative pediatric moyamoya patients is relatively unexplored. This study investigated the role for ASL in evaluating cerebral hemodynamic changes in children who underwent revascularization surgery.

METHODS

This retrospective study examined 15 consecutive pediatric patients with moyamoya disease (n = 7) or moyamoya syndrome (n = 8) presenting between 2010 and 2014 who underwent revascularization and in whom 3T ASL was performed pre- and postoperatively. Postoperative MRI at least 3 months after revascularization procedure was used for analysis. Quantitative CBF in various vascular territories was interrogated: anterior, middle, and posterior cerebral arteries, and basal ganglia supplied by the lenticulostriate collaterals, resulting in evaluation of 20 brain regions.

RESULTS

After revascularization, CBF in the high middle cerebral artery territory significantly increased (p = 0.0059), accompanied by a decrease in CBF to the ipsilateral lenticulostriate-supplied basal ganglia (p = 0.0053). No perfusion changes occurred in the remaining cerebral vascular territories after surgery.

CONCLUSIONS

ASL-based quantitative CBF showed improved cerebral perfusion to the middle cerebral artery territory after revascularization in children with both moyamoya syndrome and disease. Reduced perfusion to the basal ganglia might reflect pruning of the lenticulostriate collaterals, potentially from effects of revascularization. ASL can quantitatively evaluate hemodynamic changes in children with moyamoya after revascularization, and it may be a useful adjunct to routine clinical MRI surveillance.

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Giulia Cossu, Mahmoud Messerer and Roy Thomas Daniel

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R. Michael Scott and Edward R. Smith

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Tryggve Lundar, Bernt Johan Due-Tønnessen, Radek Frič, Bård Krossnes, Petter Brandal, Einar Stensvold and Paulina Due-Tønnessen

OBJECTIVE

The authors conducted a study to delineate the long-term results of the surgical treatment of pediatric pleomorphic xanthoastrocytomas (PXAs).

METHODS

All consecutive children and adolescents (0–20 years) who underwent primary tumor resection for a PXA during the years 1972–2015 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index.

RESULTS

Of the 12 patients, 8 patients were in the 1st decade of life and 4 in the 2nd. The male/female ratio was 6:6. No patient was lost to follow-up. One patient presented with severe progressive tumor disease and died within 3 months after repeated resection. Another child died 3 days following a second surgical procedure involving gross-total resection (GTR) 8 years after the initial operation. The other 10 patients were alive at the latest follow-up when they reached the median age of 34 years (range 11–60 years). The median follow-up duration was 22 years (range 2–41 years). Barthel Index score was 100 in all 10 survivors. A total 18 tumor resections were performed. Five patients underwent a second tumor resection after MRI/CT confirmed recurrent tumor disease, from 6 months up to 17 years after the initial operation. Only one of our patients received adjuvant therapy: a 19-year-old male who underwent resection (GTR) for a right-sided temporal tumor in 1976. This particular tumor was originally classified as astrocytoma WHO grade IV, and postoperative radiotherapy (54 Gy) was given. The histology was reclassified to that of a PXA. Seven of 8 children whose primary tumor resection was performed more than 20 years ago are alive as of this writing—i.e., 88% observed 20-year survival. These are long-term survivors with good clinical function and all are in full- or part-time work.

CONCLUSIONS

Pediatric patients with PXA can be treated with resection alone with rewarding results. Recurrences are not uncommon, but repeated surgery is well tolerated and should be considered in low-grade cases before adjuvant therapy is implemented. Follow-up including repeated MRI is important during the first postoperative years, since individual patients may have a more aggressive tumor course.

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Peter A. Ferrazzano, Bedda L. Rosario, Stephen R. Wisniewski, Nadeem I. Shafi, Heather M. Siefkes, Darryl K. Miles, Andrew L. Alexander, Michael J. Bell and for the Investigators of the ADAPT Trial

OBJECTIVE

There is no consensus on the optimal timing and specific brain MRI sequences in the evaluation and management of severe pediatric traumatic brain injury (TBI), and information on current practices is lacking. The authors performed a survey of MRI practices among sites participating in a multicenter study of severe pediatric TBI to provide information for designing future clinical trials using MRI to assess brain injury after severe pediatric TBI.

METHODS

Information on current imaging practices and resources was collected from 27 institutions participating in the Approaches and Decisions after Pediatric TBI Trial. Multiple-choice questions addressed the percentage of patients with TBI who have MRI studies, timing of MRI, MRI sequences used to investigate TBI, as well as the magnetic field strength of MR scanners used at the participating institutions and use of standardized MRI protocols for imaging after severe pediatric TBI.

RESULTS

Overall, the reported use of MRI in pediatric patients with severe TBI at participating sites was high, with 40% of sites indicating that they obtain MRI studies in > 95% of this patient population. Differences were observed in the frequency of MRI use between US and international sites, with the US sites obtaining MRI in a higher proportion of their pediatric patients with severe TBI (94% of US vs 44% of international sites reported MRI in at least 70% of patients with severe TBI). The reported timing and composition of MRI studies was highly variable across sites. Sixty percent of sites reported typically obtaining an MRI study within the first 7 days postinjury, with the remainder of responses distributed throughout the first 30-day postinjury period. Responses indicated that MRI sequences sensitive for diffuse axonal injury and ischemia are frequently obtained in patients with TBI, whereas perfusion imaging and spectroscopy techniques are less common.

CONCLUSIONS

Results from this survey suggest that despite the lack of consensus or guidelines, MRI is commonly obtained during the acute clinical setting after severe pediatric TBI. The variation in MRI practices highlights the need for additional studies to determine the utility, optimal timing, and composition of clinical MRI studies after TBI. The information in this survey describes current clinical MRI practices in children with severe TBI and identifies important challenges and objectives that should be considered when designing future studies.

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Cuiping Xu, Tao Yu, Guojun Zhang, Gary B. Rajah, Yuping Wang and Yongjie Li

OBJECTIVE

The aim of this study was to evaluate the electro-clinical features, etiology, treatment, and postsurgical seizure outcomes in patients with intractable epileptic spasms (ESs).

METHODS

The authors retrospectively studied the medical records of all patients who had presented with medically intractable ESs and had undergone surgery in the period between October 2009 and August 2015. The interictal electroencephalography (EEG) pattern, MRI studies, magnetoencephalography findings, and postsurgical seizure outcomes were compared.

RESULTS

Twenty-six patients, 12 boys and 14 girls (age range 3–22 years), were eligible for study inclusion. Of these 26 patients, 84.6% (22) presented with multiple seizure types including partial seizures (PSs) independent of the ESs (30.8%); ESs followed by tonic seizures (30.8%); myoclonic seizures (19.2%); tonic seizures (19.2%); ESs followed by PSs (19.2%); focal seizures with secondary generalization (15.4%); atypical absence (11.5%); PSs followed by ESs (7.7%); and myoclonic followed by tonic seizures (7.7%). Seventeen patients underwent multilobar resection and 9 underwent unilobar resection. At the last follow-up (mean 36.6 months), 42.3% of patients were seizure free (outcome classification [OC] 1), 23.1% had > 50% reduction in seizure frequency (OC2–OC4), and 34.6% had < 50% reduction in seizure frequency or no improvement (OC5 and OC6). Predictors of favorable outcomes included an interictal focal EEG pattern and concordance between interictal EEG and MRI-demonstrated lesions (p = 0.001 and 0.004, respectively).

CONCLUSIONS

A favorable surgical outcome is achievable in a highly select group of patients with ESs secondary to structural lesions. Interictal EEG can help in identifying patients with the potential for favorable resective outcomes.

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Angela W. Palmer and Gregory W. Albert

OBJECTIVE

Various surgical techniques have been described to treat subdural fluid collections in infants, including transfontanelle aspiration, burr holes, subdural drain, subduroperitoneal shunt, and minicraniotomy. The purpose of this study was to describe a modification of the minicraniotomy technique that avoids the implantation of external drainage catheters and potentially carries a higher success rate.

METHODS

In this retrospective study, the authors describe 11 cases involving pediatric patients who underwent parietal minicraniotomies for the evacuation of subdural fluid collections. In contrast to cases previously described in the literature, no patient received a drain; instead, a subgaleal pocket was created such that the fluid could flow from the subdural to the subgaleal space. Preoperative and postoperative data were reviewed, including neurological examination findings, radiological findings, complications, hospital length of stay, and findings on follow-up examinations and imaging. The primary outcome was failure of the treatment strategy, defined as an increase in subdural fluid collection requiring further intervention.

RESULTS

Eleven patients (8 boys and 3 girls, median age 4.5 months) underwent the described procedure. Eight of the patients had complete resolution of the subdural collection on follow-up imaging, and 2 had improvement. One patient had a new subdural collection due to a second injury. Only 1 patient underwent aspiration and subsequent surgical repair of a pseudomeningocele after the initial surgery. Notably, no patients required subduroperitoneal shunt placement.

CONCLUSIONS

The authors describe a new surgical option for subdural fluid collections in infants that allows for more aggressive evacuation of the subdural fluid and eliminates the need for a drain or shunt placement. Further work with more patients and direct comparison to other alternative therapies is necessary to fully evaluate the efficacy and safety of this new technique.

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Sarah Jernigan, Armide Storey, Christine Hammer, Coleman Riordan, Darren B. Orbach, R. Michael Scott and Edward Smith

OBJECTIVE

PHACE syndrome (PHACES) has been linked to cervical and cerebral vascular anomalies, including persistent embryonic anastomoses and progressive steno-occlusive disease. However, no prior studies have documented the long-term response of PHACES patients with moyamoya disease to surgical revascularization with pial or myosynangiosis. The authors present their experience with 8 consecutive patients with PHACES and moyamoya disease.

METHODS

Retrospective review of patients who underwent pial synangiosis revascularization for moyamoya disease with concurrent diagnosis of PHACES.

RESULTS

A total of 8 patients out of 456 surgically treated moyamoya patients had a diagnosis of PHACES. All patients were female, and their average age at the time of surgical treatment was 9.3 years (range 1.8–25.8 years). Five patients had associated basilar artery anomalies or stenosis. All patients had symptomatic narrowing of the petrous segment of the internal carotid artery with tortuous collateralization. Three patients underwent unilateral pial or myo-synangiosis and 5 underwent bilateral procedures. The average hospital length of stay was 5.0 days (range 3–7 days). There were no postoperative complications. Follow-up ranged from 8 to 160 months (average 56 months). Seven of 8 patients have had follow-up angiograms and all had Matsushima grade A or B collateralization without progression of stenosis in other locations. All patients had reduced cortical FLAIR signal on 6-month follow-up MRI and no evidence of new radiographic or clinical strokes.

CONCLUSIONS

Patients with moyamoya disease and PHACES had an intracranial arteriopathy characterized by ectactic anterior vasculature with concomitant basilar artery stenosis, and were all female. The patients had both radiographic and clinical responses to pial synangiosis. The surgical treatment of these patients can be challenging given facial hemangiomas located near the surgical field. Patients with unilateral disease did not have evidence of progression in other cerebral circulation during the given follow-up period.