Qiguang Wang and Yan Ju
Eelco W. Hoving, Mehrnoush Rahmani, Leonie I. Los and Victor W. Renardel de Lavalette
A serious ophthalmological complication of an endoscopic third ventriculostomy that created an iatrogenic Terson syndrome is described. A patient with an obstructive hydrocephalus was treated endoscopically, but due to the inadvertent use of a pressure bag during rinsing, in combination with a blocked outflow channel, a steep rise in intracranial pressure occurred. Postoperatively the patient experienced disturbed vision caused by bilateral retinal hemorrhages, and an iatrogenic Terson syndrome was diagnosed. The pathogenesis of Terson syndrome is discussed based on this illustrative case.
Eelco W. Hoving, Johan M. Kros, Ellis Groninger and Wilfred F. A. den Dunnen
✓Desmoplastic infantile gangliogliomas (DIGs) are rare supratentorial tumors that arise in infancy. Despite the large size of these lesions, the prognosis is generally considered favorable after gross-total resection (GTR); however, in incidental cases tumor progression has been described. The authors report on a child harboring a DIG with a high-grade primitive cell population and glial differentiation. The progressive clinical course was determined by this cell component in spite of GTR and adjuvant chemotherapy. The significance of the presence of a high-grade primitive tumor component in the context of DIG is discussed.
Michiel Wagemakers, Mariska Sie, Eelco W. Hoving, Grietje Molema, Eveline S. J. M. de Bont and Wilfred F. A. den Dunnen
This study aimed to characterize the pediatric intracranial ependymoma vasculature in terms of angiogenic activity and maturation status so as to provide indications for the applicability of vessel-targeted therapy in cases of pediatric intracranial ependymoma.
Tumor samples obtained in patients with ependymomas were immunohistochemically (double) stained for Ki 67/CD34, caspase 3a/CD34, vascular endothelial growth factor (VEGF)–A, –B, –C, –D, collagen Type IV, and smooth muscle actin to determine microvessel density, tumor and endothelial cell proliferation and apoptotic fraction, the relative expression of VEGF family members, and the coverage of the tumor endothelial cells by basal membrane and pericytes. Messenger RNA expression of angiopoietin-1 and -2 was analyzed by real-time reverse transcriptase polymerase chain reaction. These data were compared with those obtained in a glioblastoma series.
Despite a low endothelial cell turnover, the microvessel density of ependymomas was similar to that of glioblastomas. In ependymomas the expression of VEGF-A was within the range of the variable expression in glioblastomas. The staining intensities of VEGF-B, -C, and -D in ependymomas were significantly lower (p < 0.001). The expression of angiopoietin-1 was higher in ependymomas than in glioblastomas (p = 0.03), whereas angiopoietin-2 expression was similar. The coverage of tumor endothelial cells with basal membrane and pericytes was more complete in ependymomas (p = 0.009 and p = 0.022, respectively).
The ependymoma vasculature is relatively mature and has little angiogenic activity compared with malignant gliomas. Therefore, the window for vessel normalization as a therapeutic aim might be considered small. However, the status of the tumor vasculature may not be a reliable predictor of treatment effect. Therefore, possible benefits of antiangiogenic treatment cannot be excluded beforehand in patients with ependymomas.
Mark ter Laan and Eelco W. Hoving
Marike L. D. Broekman, Eelco W. Hoving, Kuan H. Kho, Lucienne Speleman, K. Sen Han and Patrick W. Hanlo
✓ Beckwith–Wiedemann syndrome (BWS) is a rare congenital syndrome characterized by gigantism, macroglossia, exophthalmos, postpartum hypoglycemia, and multiple midline defects such as omphalocele. The authors describe, to the best of their knowledge, the first case of a child in whom BWS was diagnosed and who was subsequently treated for a nasal encephalocele.
Because the authors believe that this feature might not be an incidental finding in patients with BWS, intranasal masses in these patients should be carefully differentiated, as complications might be severe.
Gerben E. Breimer, Ruben Dammers, Peter A. Woerdeman, Dennis R. Buis, Hans Delye, Marjolein Brusse-Keizer and Eelco W. Hoving
After endoscopic third ventriculostomy (ETV), some patients develop recurrent symptoms of hydrocephalus. The optimal treatment for these patients is not clear: repeat ETV (re-ETV) or CSF shunting. The goals of the study were to assess the effectiveness of re-ETV relative to initial ETV in pediatric patients and validate the ETV success score (ETVSS) for re-ETV.
Retrospective data of 624 ETV and 93 re-ETV procedures were collected from 6 neurosurgical centers in the Netherlands (1998–2015). Multivariable Cox proportional hazards modeling was used to provide an adjusted estimate of the hazard ratio for re-ETV failure relative to ETV failure. The correlation coefficient between ETVSS and the chance of re-ETV success was calculated using Kendall’s tau coefficient. Model discrimination was quantified using the c-statistic. The effects of intraoperative findings and management on re-ETV success were also analyzed.
The hazard ratio for re-ETV failure relative to ETV failure was 1.23 (95% CI 0.90–1.69; p = 0.20). At 6 months, the success rates for both ETV and re-ETV were 68%. ETVSS was significantly related to the chances of re-ETV success (τ = 0.37; 95% bias corrected and accelerated CI 0.21–0.52; p < 0.001). The c-statistic was 0.74 (95% CI 0.64–0.85). The presence of prepontine arachnoid membranes and use of an external ventricular drain (EVD) were negatively associated with treatment success, with ORs of 4.0 (95% CI 1.5–10.5) and 9.7 (95% CI 3.4–27.8), respectively.
Re-ETV seems to be as safe and effective as initial ETV. ETVSS adequately predicts the chance of successful re-ETV. The presence of prepontine arachnoid membranes and the use of EVD negatively influence the chance of success.