Endoscopic third ventriculostomy in an infant with vein of Galen aneurysmal malformation treated by endovascular occlusion: Case report and a review of literature.

INTRODUCTION: Vein of Galen aneurysmal malformations (VGAMs) can, through multiple mechanisms, complicate with hydrocephalus (HCP). It is generally agreed that management strategies in this scenario should focus on endovascular embolizations. Treatment options for non-responders, however, have been only scarcely reported upon. CASE PRESENTATION: We present a nine-month-old boy with a mural type VGAM complicated by HCP. Despite endovascular occlusion of the sole feeder, the child exhibited hydrocephalus progression prompting an Endoscopic Third Ventriculostomy (ETV). This procedure restored a cerebrospinal fluid (CSF) circulation otherwise impaired by aqueduct obstruction. Later, a new feeder arose and a second embolization was ultimately needed in order to achieve VGAM regression. Throughout four years of follow up, the child attained all developmental marks. DISCUSSION/CONCLUSION: VGAMs are prone to hydrocephalus development as there is both an underlying venous congestion and a mechanical, obstructive component. Although there is a rationale for addressing both components, the underlying AV shunts and subsequent venous pressure elevations usually determine failure of traditional CSF shunting strategies. It is therefore challenging to manage HCP in patients who failed to improve following endovascular embolizations. For such cases, ETV stands as an elegant minimal invasive alternative with potential to provide a more physiologic drainage route and thus better allow for neurological development.

Minimally invasive endoscopically assisted remodelation (MEAR) of sagittal craniosynostosis: an alternative technique to open and endoscopic procedures with cranial orthosis time span reduction.

INTRODUCTION: Sagittal craniosynostosis represents the most frequent simplex skull suture pathology. There are currently several operative approaches to this defect. Minimally invasive techniques are preferred for young infants. Since July 2017, we have employed endoscopically assisted craniectomies followed by cranial orthosis. Gradually, we have developed our modified technique, the minimally invasive endoscopically assisted remodelation (MEAR). SURGICAL TECHNIQUE: MEAR is a combination of principles gained from classical cranial vault remodeling techniques and minimal invasive approaches. The long and wider lateral osteoectomies performed in the parietal and occipital bones along with loosening of the periosteum and dura adhesions at the lambdoid sutures lead to early correction of parieto-occipital dimensions. RESULTS: Thirty-one consecutive patients with scaphocephaly underwent MEAR. The median preoperative cephalic index of 67 units (P25:63.3, P75:70) was improved to a median postoperative cephalic index of 77 units (P25:75, P75: 81). Sufficient correction was achieved in all patients. Cranial orthosis was needed for a median of 1.5 months (P25:1, P75:2). We had no major surgical complications in this pilot series. CONCLUSIONS: With MEAR, we have achieved good cosmetic results. Duration of cranial orthosis was significantly shortened compared to conventional endoscopic-assisted procedures.

Torcular dural sinus malformations: a grading system proposal.

BACKGROUND: Torcular dural sinus malformations (tDSMs) are congenital complex vascular anomalies often referred as a single unit. Nevertheless, they possess distinct anatomical features, clinical diversity, and markedly different outcomes. OBJECTIVE: On the basis of our institutional experience and analysis of published data, we propose a grading system. METHODS: We have identified 44 papers to which we added our four institutional cases for a total of 126 patients. Eight predictor variables were studied. In order to assess their individual impact on mortality and possible correlations, a logistic regression model was constructed through a stepwise forward process. RESULTS: Overall mortality was 22.1%. Mortality was higher in tDSM patients diagnosed postnatally, 40.7% versus a 15.6% in prenatally found cases (p = 0.007). We divided the patients into four grades. Grade I comprised patients with no feeder evidence and possessed the best outcomes (mortality of 7.55%). Mortality rose for grades II and III defined respectively by scarce and multiple feeders. Brain damage was the defining feature of grade IV. A mortality of 75% could be observed within this grade. Grade IV was further divided into grades IVa (antenatal) and IVb (postnatal cases). Furthermore, our logistic regression model found that brain damage (OR 11.3, p < 0.001, 95% CI 2.97-42.91) and patent feeders (OR 4, p = 0.03, 95% CI 1.15-13.86) were major determinants of poor outcome (area under ROC curve of 81.44%). CONCLUSION: The grading system (tDSM-GS) streamlines classification into four different grades facilitating both diagnosis, clinical decision-making, and proper prognostication.

Intraoperative Thermography of the Electrical Stimulation Mapping: A Safety Control Study.

A standard procedure for continuous intraoperative monitoring of the integrity of the corticospinal tracts by eliciting muscle responses is the electric stimulation mapping (ESM). However, standard ESM protocols are ineffective in 20% of young children. We have developed a novel, highly efficient paradigm consisting of short-time burst (30 ms) of high frequency (500 Hz) and high peak current (≤100 mA), which may cause local tissue overheating. The presented safety control study was therefore designed. The infrared thermography camera captured to-be-resected cortex of 13 patients in vivo during ESM. Thermograms were image processed to reveal discrete ESM thermal effect of currents from 10 to 100 mA. Peak 100 mA currents induced a maximal increase in temperature of 3.1 °C, 1.23±0.72 °C in average. The warming correlated with stimulating electrode resistance ( ). The measurement uncertainty was estimated ± 1.01 ºC for the most skeptical conditions. The histopathological evaluation of stimulated tissue (performed in all cases) did not show any destructive changes. Our study demonstrates the ability of the thermographic camera to measure the discrete thermal effect of the ESM. The results provide evidence for the safety of the proposed protocol for full range currents with minimal risk of brain tissue damage.

Two cases of improvement of smooth pursuit eye movements after selective posterior rhizotomy.

OBJECTIVE: Selective posterior rhizotomy (SPR) represents a standard neurosurgical approach in the treatment of spasticity in children with cerebral palsy (CP). Beside the reduction of spasticity in lower limbs, SPR may have suprasegmental effects, considerably above the surgery site. In this communication, we report on the improvement of smooth pursuit eye movements (SPEM) in two children after SPR. MATERIAL AND METHODS: Four children with CP underwent SPR. Eye movements were registered by infrared video-oculography before and after the surgery. RESULTS: The analysis of SPEM showed the improvement of the correlation coefficient of the eye response to the stimulus after SPR in two subjects. Improvement of SPEM performance was largely due to suppression of spontaneous fixation nystagmus. CONCLUSION: SPR may lead to the improvement of SPEM in children with CP. The influence of SPEM improvement on quality of life in a group of severely disabled nonambulant children with CP remains to be assessed.

BIR-1, the homologue of human Survivin, regulates expression of developmentally active collagen genes in C. elegans.

BIR-1 and Survivin are highly conserved members of the inhibitor of apoptosis protein family that regulate cell division in nematodes and mammals and inhibit apoptosis in mammals. In the C. elegans genome, bir-1 is organized in an operon together with transcription and splicing cofactor CeSKIP (skp-1) and is highly expressed during embryogenesis as well as in non-dividing cells during larval development. Previously we have shown that BIR-1 regulates transcription and development and its loss-of-function phenotype overlaps with loss of function of CeSKIP and nuclear hormone receptor CHR3 (NHR-23). Here we searched for genes whose expression is affected by BIR-1 loss of function using whole-genome microarray experiments and identified several collagen genes as candidate targets of bir-1 inhibition in L1 larval stage. The decreased expression of selected collagen genes in bir-l-inhibited larvae was confirmed by quantitative RT-PCR. Next, we generated transgenic lines expressing bir-1 mRNA under a heat shock-regulated promoter and tested whether bir-1 overexpression has the potential to augment the expression of genes that showed decreased expression in worms treated with bir-1 RNAi. Overexpression of bir-1 resulted in a pronounced increase (2 to 5 times) of the expression of these genes. Our findings support the concept that BIR-1, a protein generally regarded as a mitotic factor, is involved in the regulation of transcription during normal development of C. elegans and has a strong ability to affect transcription of developmentally active genes if overexpressed.

Elevated and deregulated expression of HDAC3 in human astrocytic glial tumours.

Abnormal expression of histone deacetylases may contribute to the establishment of a cancer specific transcription profile. We examined expression of HDAC3 in human non-malignant gliosis and glial astrocytic tumours. Samples from four non-malignant gliosis and 17 astrocytic gliomas (six of grade II, one of grade III and ten of grade IV) removed for therapeutic purposes were assayed for HDAC3 expression at mRNA and protein levels. HDAC3 mRNA was detected in non-tumorous gliosis as well as in all examined glial tumours. Seven out of eleven examined high-grade tumours showed an elevated number of copies of HDAC3 mRNA. Western blot analysis detected high levels of expression of HDAC3 in the majority of the examined tumours. Immunohistochemistry and immunofluorescence made on a collection of 35 astrocytic tumours detected nuclear as well as cytoplasmic HDAC3 expression in all of those tumours. While the distribution of HDAC3 was both nuclear as well as cytoplasmic and moderate in intensity in non-malignant tissues and low-grade gliomas, high-grade tumours expressed HDAC3 in a focally deregulated pattern that included strongly pronounced cytoplasmic localization. Confocal microscopy and additional co-localization analysis detected nuclear HDAC3 in all tumours examined. We conclude that HDAC3 expression is elevated in human astrocytic tumours and its expression pattern is deregulated at the cellular level in high-grade gliomas.