The purpose of this review is to highlight some of the pertinent concepts and controversies surrounding the diagnosis and treatment of pediatric supratentorial high-grade gliomas. Unlike the adult counterparts, pediatric high-grade gliomas are likely derived from distinct cytogenetic and molecular alterations. Surgery has been shown to play a role in extending patient survival. Some success is associated with the provision of chemotherapy. Radiotherapy remains an important adjunct in children older than age 3 years. The challenges involved in improving the poor prognosis of children in whom these very aggressive tumors have been diagnosed will be discussed, as well as some of the novel approaches being investigated to improve patient survival and quality of life.
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Mandeep S. Tamber and Robert P. Naftel
OBJECTIVE
选择选项(并联或endo竞争copic third ventriculostomy) for the management of hydrocephalus requires patients and caregivers to make a subjective judgment about the relative importance of risks and benefits associated with each treatment. In the context of this particular decision, little is known about what treatment-related factors are important and how they are prioritized in order to arrive at a treatment preference.
开云体育世界杯赔率
The Hydrocephalus Association electronically distributed a survey to surgically treated hydrocephalus patients or their families. Respondents rated the importance of various surgical attributes in their decision-making about treatment choice, and also indicated their preference in hypothetical scenarios involving a trade-off between potential risks and benefits of treatment. Rank-order correlations were used to determine whether certain predictor variables affected the rating of factors or hypothetical treatment choice.
RESULTS
Eighty percent of 414 respondents rated procedural risks, minimizing repeat surgery, and improving long-term brain function as being very or extremely important factors when deciding on a treatment; 69% rated the need to implant a permanent device similarly. Parent-respondents rated procedural risks higher than patient-respondents. A majority of respondents (n = 209, 54%) chose a procedure with higher surgical risk if it meant that implantation of a permanent device was not required, and respondents were more likely to choose this option if they discussed both treatment options with their surgeon prior to their initial intervention (Spearman rho 0.198, p = 0.001).
Although only 144 of 384 total respondents (38%) chose a less established operation if it meant less repeat surgery, patient-respondents were more likely to choose this option compared to parent-respondents (Spearman rho 0.145, p = 0.005). Likewise, patient-respondents were more likely than parent-respondents to choose an operation that involved less repeat surgery and led to worse long-term brain function (Spearman rho 0.160, p = 0.002), an option that was chosen by only 23 (6%) of respondents overall.
CONCLUSIONS
This study is the first exploration of patient/parental factors that influence treatment preference in pediatric hydrocephalus. Procedural risks, minimizing repeat operations, and the desire to maximize long-term cognitive function appeared to be the most important attributes that influenced treatment decisions that the survey respondents had made in the past. Patients and/or their caregivers appear to see some inherent benefit in being shunt free. It appears that fear of multiple revision operations may drive treatment choice in some circumstances.
Ann Marie Flannery, Ann-Christine Duhaime, Mandeep S. Tamber, and Joanna Kemp
Object
This systematic review was undertaken to answer the following question: Do technical adjuvants such as ventricular endoscopic placement, computer-assisted electromagnetic guidance, or ultrasound guidance improve ventricular shunt function and survival?
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The US National Library of Medicine PubMed/MEDLINE database and the Cochrane Database of Systematic Reviews were queried using MeSH headings and key words specifically chosen to identify published articles detailing the use of cerebrospinal fluid shunts for the treatment of pediatric hydrocephalus. Articles meeting specific criteria that had been delineated a priori were then examined, and data were abstracted and compiled in evidentiary tables. These data were then analyzed by the Pediatric Hydrocephalus Systematic Review and Evidence-Based Guidelines Task Force to consider evidence-based treatment recommendations.
Results
The search yielded 163 abstracts, which were screened for potential relevance to the application of technical adjuvants in shunt placement. Fourteen articles were selected for full-text review. One additional article was selected during a review of literature citations. Eight of these articles were included in the final recommendations concerning the use of endoscopy, ultrasonography, and electromagnetic image guidance during shunt placement, whereas the remaining articles were excluded due to poor evidence or lack of relevance.
The evidence included 1 Class I, 1 Class II, and 6 Class III papers. An evidentiary table of relevant articles was created.
Conclusions
Recommendation: There is insufficient evidence to recommend the use of endoscopic guidance for routine ventricular catheter placement. Strength ofRecommendation: Level I, high degree of clinical certainty.
Recommendation: The routine use of ultrasound-assisted catheter placement is an option. Strength ofRecommendation: Level III, unclear clinical certainty.
Recommendation: The routine use of computer-assisted electromagnetic (EM) navigation is an option. Strength ofRecommendation: Level III, unclear clinical certainty.
Mandeep S. Tamber, Paul Klimo Jr., Catherine A. Mazzola, and Ann Marie Flannery
Object
The objective of this systematic review was to answer the following question: What is the optimal treatment strategy for CSF shunt infection in pediatric patients with hydrocephalus?
开云体育世界杯赔率
The US National Library of Medicine and the Cochrane Database of Systematic Reviews were queried using MeSH headings and key words relevant to the objective of this systematic review. Abstracts were reviewed, after which studies meeting the inclusion criteria were selected and graded according to their quality of evidence (Classes I–III). Evidentiary tables were constructed that summarized pertinent study results, and based on the quality of the literature, recommendations were made (Levels I–III).
Results
A review and critical appraisal of 27 studies that met the inclusion criteria allowed for a recommendation for supplementation of antibiotic treatment using partial (externalization) or complete shunt hardware removal, with a moderate degree of clinical certainty. However, a recommendation regarding whether complete shunt removal is favored over partial shunt removal (that is, externalization) could not be made owing to severe methodological deficiencies in the existing literature. There is insufficient evidence to recommend the use of intrathecal antibiotic therapy as an adjunct to systemic antibiotic therapy in the management of routine CSF shunt infections. This also holds true for other clinical scenarios such as when an infected CSF shunt cannot be completely removed, when a shunt must be removed and immediately replaced in the face of ongoing CSF infection, or when the setting is ventricular shunt infection caused by specific organisms (for example, gram-negative bacteria).
Conclusions
Supplementation of antibiotic treatment with partial (externalization) or complete shunt hardware removal are options in the management of CSF shunt infection. There is insufficient evidence to recommend either shunt externalization or complete shunt removal as the preferred surgical strategy for the management of CSF shunt infection. Therefore, clinical judgment is required. In addition, there is insufficient evidence to recommend the combination of intrathecal and systemic antibiotics for patients with CSF shunt infection when the infected shunt hardware cannot be fully removed, when the shunt must be removed and immediately replaced, or when the CSF shunt infection is caused by specific organisms. The potential neurotoxicity of intrathecal antibiotic therapy may limit its routine use.
Recommendation: Supplementation of antibiotic treatment with partial (externalization) or with complete shunt hardware removal is an option in the management of CSF shunt infection. Strengthof Recommendation: Level II, moderate degree of clinical certainty.
Recommendation: There is insufficient evidence to recommend either shunt externalization or complete shunt removal as a preferred surgical strategy for the management of CSF shunt infection. Therefore, clinical judgment is required. Strengthof Recommendation: Level III, unclear degree of clinical certainty.
Recommendation: There is insufficient evidence to recommend the combination of intrathecal and systemic antibiotics for patients with CSF shunt infection in whom the infected shunt hardware cannot be fully removed or must be removed and immediately replaced, or when the CSF shunt infection is caused by specific organisms. The potential neurotoxicity of intrathecal antibiotic therapy may limit its routine use. Strength of Recommendation: Level III, unclear degree of clinical certainty.
Joanna Kemp, Ann Marie Flannery, Mandeep S. Tamber, and Ann-Christine Duhaime
Object
The objective of this guideline was to answer the following question: Do the entry point and position of the ventricular catheter have an effect on shunt function and survival?
开云体育世界杯赔率
Both the US National Library of Medicine/MEDLINE database and the Cochrane Database of Systematic Reviews were queried using MeSH headings and key words specifically chosen to identify published articles detailing the use of CSF shunts for the treatment of pediatric hydrocephalus. Articles meeting specific criteria that had been delineated a priori were then examined, and data were abstracted and compiled in evidentiary tables.
Results
The search yielded 184 abstracts, which were screened for potential relevance to the clinical question of the effect of ventricular catheter entry site on shunt survival. An initial review of the abstracts identified 14 papers that met the inclusion criteria, and these were recalled for full-text review. After review of these articles, only 4 were noted to be relevant for an analysis of the impact of entry point on shunt survival; an additional paper was retrieved during the review of full-text articles and was included as evidence to support the recommendation. The evidence included 1 Class II paper and 4 Class III papers. An evidentiary table was created including the relevant articles.
Conclusion
Recommendation: There is insufficient evidence to recommend the occipital versus frontal point of entry for the ventricular catheter; therefore, both entry points are options for the treatment of pediatric hydrocephalus. Strength ofRecommendation: Level III, unclear degree of clinical certainty.
Xiao Zhu, Michael M. McDowell, William C. Newman, Gary E. Mason, Stephanie Greene, and Mandeep S. Tamber
Nivolumab is an immune checkpoint inhibitor (ICI) currently undergoing Phase III clinical trials for the treatment of glioblastoma. The authors present the case of a 10-year-old girl with glioblastoma treated with nivolumab under compassionate-use guidelines. After the first dose of nivolumab the patient developed hemiparesis, cerebral edema, and significant midline shift due to severe tumor necrosis. She was managed using intravenous dexamethasone and discharged on a dexamethasone taper. The patient's condition rapidly deteriorated after the second dose of nivolumab, demonstrating hemiplegia, seizures, and eventually unresponsiveness with a fixed and dilated left pupil. Computed tomography of her brain revealed malignant cerebral edema requiring emergency decompressive hemicraniectomy. Repeat imaging demonstrated increased size of the lesion, reflecting immune-mediated inflammation and tumor necrosis. The patient remained densely hemiplegic, but became progressively more interactive and was ultimately extubated. She resumed nivolumab several weeks later, but again her condition deteriorated with headache, vomiting, swelling at the craniectomy site, and limited right-sided facial movement following the sixth dose. MRI demonstrated severe midline shift and uncal herniation despite her craniectomy. Her condition gradually declined, and she died several days later under “do not resuscitate/do not intubate” orders. To the authors' knowledge, this represents the first case of malignant cerebral edema requiring operative intervention following nivolumab treatment for glioblastoma in a pediatric patient.
Saman Fouladirad, Alexander Cheong, Ashutosh Singhal, Mandeep S. Tamber, and Patrick J. McDonald
OBJECTIVE
Hydrocephalus is one of the most common condition treated by pediatric neurosurgeons. Many neurosurgeons are unable to continue to care for patients after they become adults. Although significant gaps in care are believed to exist for youth transitioning from pediatric to adult care, very little is known about how patients and their caregivers feel about the process. This qualitative study sought to examine the perceptions of adolescents, young adults, and their caregivers regarding transitioning from pediatric to adult care at a single Canadian center.
开云体育世界杯赔率
The authors explored the perceptions of patients with treated hydrocephalus and their caregivers using semistructured interviews and qualitative research methodologies. A convenience sample was recruited, composed of adolescent patients and their caregivers at the neurosurgery clinic of BC Children’s Hospital, and patients and caregivers recently transitioned to adult care from the clinic. Interviews were transcribed verbatim and coded, with common themes identified.
RESULTS
Four overarching themes relating to the process of transitioning from pediatric to adult hydrocephalus care for patients and their caregivers were identified from the data: 1) achieving independence, 2) communication gaps, 3) loss of significant relationships and environment, and 4) fear of uncertainty.
CONCLUSIONS
Overall, patients with hydrocephalus and their families are dissatisfied with the process of transitioning. This study identified common themes and concerns among this cohort that may form the basis of an improved transition model for youth with hydrocephalus as they become adults.
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.
开云体育世界杯赔率
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
小儿神经外科手术的并发症s 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.
Ann Marie Flannery, Catherine A. Mazzola, Paul Klimo Jr., Ann-Christine Duhaime, Lissa C. Baird, Mandeep S. Tamber, David D. Limbrick Jr., Dimitrios C. Nikas, Joanna Kemp, Alexander F. Post, Kurtis I. Auguste, Asim F. Choudhri, Laura S. Mitchell, and Debby Buffa
Abhaya V. Kulkarni, Jay Riva-Cambrin, Jerry Butler, Samuel R. Browd, James M. Drake, Richard Holubkov, John R. W. Kestle, David D. Limbrick, Tamara D. Simon, Mandeep S. Tamber, John C. Wellons III, William E. Whitehead, and for the Hydrocephalus Clinical Research Network
Object
The Hydrocephalus Clinical Research Network (HCRN), which comprises 7 pediatric neurosurgical centers in North America, provides a unique multicenter assessment of the current outcomes of CSF shunting in nonselected patients. The authors present the initial results for this cohort and compare them with results from prospective multicenter trials performed in the 1990s.
开云体育世界杯赔率
Analysis was restricted to patients with newly diagnosed hydrocephalus undergoing shunting for the first time. Detailed perioperative data from 2008 through 2012 for all HCRN centers were prospectively collected and centrally stored by trained research coordinators. Historical control data were obtained from the Shunt Design Trial (1993–1995) and the Endoscopic Shunt Insertion Trial (1996–1999). The primary outcome was time to first shunt failure, which was determined by using Cox regression survival analysis.
Results
Mean age of the 1184 patients in the HCRN cohort was older than mean age of the 720 patients in the historical cohort (2.51 years vs 1.60 years, p < 0.0001). The distribution of etiologies differed (p < 0.0001, chi-square test); more tumors and fewer myelomeningoceles caused the hydrocephalus in the HCRN cohort patients. The hazard ratio for first shunt failure significantly favored the HCRN cohort, even after the model was adjusted for the prognostic effects of age and etiology (adjusted HR 0.82, 95% CI 0.69–0.96).
Conclusions
Current outcomes of shunting in general pediatric neurosurgery practice have improved over those from the 1990s, although the reasons remain unclear.
