Intraoperative Measurement of Intracranial Pressure During Cranial Vault Remodeling in Children with Craniosynostosis

Purpose In this study, we analyzed the utility of intracranial pressure (ICP) monitoring intraoperatively for deciding height reduction and need for cerebrospinal fluid (CSF) diversion during cranial vault remodeling in children with multisutural craniosynostosis (CS). Methods This is a retrospective observational study of children who underwent surgery for CS and ICP monitoring during surgery. The ICP was monitored using an external ventricular drainage catheter. The ICP monitoring was continued during the entire procedure. Results A total of 28 (19 boys) children with the involvement of two or more sutures underwent ICP monitoring during surgery. The commonest pattern of suture involvement was bicoronal seen in 16 (57.1%) children followed by pancraniosynostoses in eight (28.6%) cases. The mean opening ICP was 23 mm Hg, which dropped to 10.9 mm Hg after craniotomy. The ICP increased transiently to 19.5 mm Hg after height reduction, and the mean ICP at closure was 16.2 mm Hg. The ICP recordings helped in undoing the height reduction in two children and ventriculoperitoneal shunt after surgery in two children. Conclusions Intraoperative monitoring of ICP helps in deciding the type of cranial vault remodeling and the need for CSF diversion after surgery.

Chiari malformation and spinal interdural cyst: A proposed association and review of the literature

Background: Interdural cysts are rare meningeal cysts with an unclear etiology. They are often mistaken for other mass lesions, including arachnoid cysts and tumors. Correctly identifying and classifying these cysts, as well as how they have formed in individual patients, are crucial to providing effective treatment options for patients. Case Description: We report a case of a patient with shunted idiopathic intracranial hypertension who developed a symptomatic Chiari malformation and was subsequently discovered to have a spinal interdural cyst. The Chiari malformation was likely due to intracranial hypotension secondary to lumbar cerebrospinal fluid (CSF) diversion. Once the shunt was removed, a spinal interdural cyst became clinically and radiographically evident, and the Chiari resolved, suggesting that both entities were effects of shared CSF flow dynamics. Conclusion: This cyst likely originated due to the trauma from remote repeated lumbar punctures and lumboperitoneal shunt placement, allowing CSF to enter the interdural space after the catheter was removed.

Clinical and Molecular Features of Disseminated Pediatric Low Grade Glioma - A Systematic Review of Literature

INTRODUCTIONGliomas account for approximately 46% of all pediatric CNS tumors. There is growing awareness of pediatric low-grade gliomas (PLGG) that disseminate to distant parenchymal or leptomeningeal locations either at the time of initial diagnosis or upon disease surveillance. Disseminated PLGGs (dPLGGs) are associated with a poorer prognosis than non-disseminated PLGGs. To date there is no comprehensive report characterizing the genome profile of dPLGGs and their associated management. This systematic review aims to identify the pattern of genetic alterations and treatment outcomes described for dPLGG.METHODSA systematic review of the literature was performed to identify relevant articles. A quality and risk of bias assessment of articles was done using the GRADE framework and ROBINS-I tool, respectively.RESULTSFifty-two studies published from 1994 to 2020 were included in this review with 368 cases reported. There was sporadic reporting of genetic alterations. The most common genetic alteration observed among study subjects was 1p deletion (76%) and BRAF-KIAA1549 fusion (55%). BRAF p.V600E mutation was found in 7% of subjects. A higher proportion of cases demonstrated primary dissemination compared to secondary dissemination (65% vs 25%). First-line chemotherapy consisted primarily of an alkylation-based regimen and vinca alkaloids. Surgical intervention ranged from biopsy alone to surgical resection and CSF diversion, and depended largely upon tumor location and timing of dissemination. Overall, 73% of cases were alive at last follow-up (median, 40.2 months). All studies reviewed either ranked low or moderate for both quality and risk of bias assessments. CONCLUSIONWhile 1p deletion and BRAF-KIAA1549 fusion are the most commonly described molecular alterations in dPLGG, these tumors appear to express heterogeneous molecular and biological characteristics distinct from non-disseminated PLGGs. Additional studies on the molecular and biological features of these tumors are needed to better understand the pathogenesis of dPLGG and to inform the development of additional targeted regimens.

Successful management of symptomatic hydrocephalus using a temporary external ventricular drain with or without endoscopic third ventriculostomy in pediatric patients with germinoma

OBJECTIVE The objective of this study was to describe the management of hydrocephalus in a cohort of pediatric patients with germinoma. METHODS The authors conducted a retrospective chart review of patients with germinoma and symptomatic hydrocephalus treated at the Hospital for Sick Children between 2002 and 2020. Descriptive data included tumor location, CSF diversion procedure (external ventricular drain [EVD], endoscopic third ventriculostomy [ETV], ventriculoperitoneal [VP] shunt) and outcomes. The frontooccipital horn ratio (FOR) method was used to determine the presence of ventriculomegaly. RESULTS Of 39 patients with germinoma, 22 (73% male) had symptomatic hydrocephalus at diagnosis (11 pineal, 4 suprasellar, and 7 bifocal). Management of hydrocephalus included EVD (n = 5, 22.7%), ETV (n = 5, 22.7%), and combination ETV and EVD (n = 7, 31.8%), whereas 5 patients (22.7%) did not undergo surgical intervention. The median FOR at diagnosis was 0.42 (range 0.38–0.58), which correlated with moderate to severe ventriculomegaly. Carboplatin and etoposide–based chemotherapy induced fast tumor shrinkage, avoiding CSF diversion (n = 5) and resolving hydrocephalus with a transient EVD (n = 5). The median duration until EVD removal was 7 days (range 2–10 days). Two of 12 patients with EVD ultimately required a VP shunt. Kaplan-Meier overall survival was 100% and progression-free survival was 96.4% at a median follow-up of 10.4 years. CONCLUSIONS Timely initiation of chemotherapy is imperative to rapidly reduce tumor bulk in children with germinoma and limits the need for VP shunt insertions. In children in whom CSF diversion is required, hydrocephalus may be successfully managed with a temporary EVD ± ETV.

Delayed presentation of syringomyelia after retrosigmoid craniectomy and resection of vestibular schwannoma: discussion of mechanisms

We present a case of syringomyelia following retrosigmoid craniectomy and resection of vestibular schwannoma complicated by Cerebrospinal fluid (CSF) leak, multiple infections and CSF diversion procedures. We believe this is the first reported case of syrinx formation after this type of surgery. Potential mechanisms by which the syrinx may have developed are discussed.

Torkildsen’s Ventriculocisternostomy First Applications: The Anthropological Evidence of a Young Slavic Soldier Who Died in the Torre Tresca Concentration Camp (Bari, Italy) in 1946

Human skeletal remains are considered as real biological archives of each subject’s life. Generally, traumas, wounds, surgical interventions, and many human pathologies suffered in life leave identifiable marks on the skeleton, and their correct interpretation is possible only through a meticulous anthropological investigation of skeletal remains. The study here presented concerns the analysis of a young Slavic soldier’s skeleton who died, after his imprisonment, in the concentration camp of Torre Tresca (Bari, Italy), during the Second World War (1946). In particular, the skull exhibited signs of surgical activity on the posterior cranial fossa and the parieto-occipital bones. They could be attributed to surgical procedures performed at different times, showing various degrees of bone edge remodeling. Overall, it was possible to correlate the surgical outcomes highlighted on the skull to the Torkildsen’s ventriculocisternostomy (VCS), the first clinically successful shunt for cerebrospinal fluid (CSF) diversion in hydrocephalus, which gained widespread use in the 1940s. For this reason, the skeleton we examined represents a rare, precious, and historical testimony of an emerging and revolutionary neurosurgical technique, which differed from other operations for treating hydrocephalus before the Second World War and was internationally recognized as an efficient procedure before the introduction of extracranial shunts.

From research to clinical practice: long-term impact of randomized clinical trial examining the effect of lumbar drains on cerebrospinal fluid leak rates following endonasal skull base surgery.

Intraoperative cerebrospinal fluid (CSF) leaks are associated with increased risk of post-operative CSF leaks despite multilayered reconstruction with vascularized tissue. A recent randomized controlled trial (RCT) examining the use of peri-operative lumbar drains (LD) in high-risk skull base defects identified a significant reduction in post-operative CSF leak incidence (21.2% vs. 8.2%; p=0.017). This study was conducted to assess the efficacy of the selective use of CSF diversion, for patients with intraoperative CSF leaks involving endoscopic endonasal approaches (EEA) to the skull base. Method: Consecutive endoscopic endonasal surgeries of the skull base from a pre-RCT cohort and post-RCT cohort were compared. The following case characteristics between the two cohorts were examined: patient age, body mass index (BMI), rate of revision surgery, tumor histology, use of CSF diversion, and vascularized reconstruction. The primary measured outcome was post-operative CSF leak. Results: The pre-RCT cohort included 76 patients and the post-RCT cohort, 77 patients, with dural defects in either the anterior or posterior cranial fossa (pituitary and parasellar/suprasellar surgeries excluded). There was a significant reduction in the incidence of post-operative CSF leak in the post-RCT cohort (27.6% vs. 12.9%; p=0.04). On subgroup analysis, there was a trend toward improvement in CSF leak rate of the anterior cranial fossa (19.2% vs 10.5%; p=0.27) whereas CSF leak rates of the posterior cranial fossa were significantly reduced compared to the pre-RCT cohort (41.4% vs 12.8%; p=0.02). Conclusion This study demonstrates that the integration of selective CSF diversion into the reconstructive algorithm improved post-operative CSF leak rates.

Management strategies for pediatric patients with tectal gliomas: a systematic review

Pediatric tectal gliomas generally have a benign clinical course with the majority of these observed radiologically. However, patients often need treatment for obstructive hydrocephalus and occasionally require cytotoxic therapy. Given the lack of level I data, there is a need to further characterize management strategies for these rare tumors. We have therefore performed the first systematic review comparing various management strategies. The literature was systematically searched from January 1, 2000, to July 30, 2020, to identify studies reporting treatment strategies for pediatric tectal gliomas. The systematic review included 355 patients from 14 studies. Abnormal ocular findings—including gaze palsies, papilledema, diplopia, and visual field changes—were a common presentation with between 13.6 and 88.9% of patients experiencing such findings. CSF diversion was the most performed procedure, occurring in 317 patients (89.3%). In individual studies, use of CSF diversion ranged from 73.1 to 100.0%. For management options, 232 patients were radiologically monitored (65.4%), 69 received resection (19.4%), 30 received radiotherapy (8.4%), and 19 received chemotherapy (5.4%). When examining frequencies within individual studies, chemotherapy ranged from 2.5 to 29.6% and radiotherapy ranged from 2.5 to 28.6%. Resection was the most variable treatment option between individual studies, ranging from 2.3 to 100.0%. Most tectal gliomas in the pediatric population can be observed through radiographic surveillance and CSF diversion. Other forms of management (i.e., chemotherapy and radiotherapy) are warranted for more aggressive tumors demonstrating radiological progression. Surgical resection should be reserved for large tumors and/or those that are refractory to other treatment modalities.

Ventriculoperitoneal Shunt versus Endoscopic Third Ventriculostomy in the treatment of Obstructive Hydrocephalus in Pediatric Midline Posterior Fossa tumors

Background Treatment of secondary hydrocephalus due to posterior fossa tumors in these children is still a matter of controversy, although preoperative ventriculo-peritoneal shunt (VP shunt) insertion before tumor excision is widely accepted among neurosurgeons but many attempts are rising to minimize permanent VP shunt insertion and associated complications and introducing third endoscopic ventriculostomy (ETV) as one of the options of 2ry hydrocephalus. Objectives Comparing the post-operative clinical success with resolution of the manifestations and post-operative complications between endoscopic third ventriculostomy and ventriculoperitoneal shunt as different modes of CSF diversion in children with 2ry hydrocephalus due to midline posterior fossa tumors. Methods The following electronic databases were searched from June 2009 to june2019: PubMed, Google scholar search engine. Cochrane database of systematic reviews, EMBASE and science Direct, using the keywords ―hydrocephalus; posterior fossa tumors; pediatrics; ventriculoperitoneal shunt; endoscopic third ventriculostomy‖. Studies were eligible if they contain the target keywords in title or abstract, addressing the Pediatric age group with 2ry hydrocephalus due to de novo posterior fossa tumor manifested by signs of increase the intra cranial tension including persistent headache and vomiting, blurred vision, 6th nerve palsy, papilledema in fundus examination, acute DCL and 2ry hydrocephalus confirmed by brain imaging. Exclusion criteria included studies including age group below 1yr or above 18 yr, or patients with recurrent post fossa tumors and operated before or patients presented by failed previously attempt of CSF diversion. Results: A total of 1255 citations were screened for eligibility,6 studies were included in our systematic review discussing, comparing and evaluating the durability of ETV versus VP shunt in treatment the 2ry hydrocephalusdue to pediatric posterior fossa tumor.. Overall study population reached 474 patients. the overall clinical findings at presentation and postoperative outcomes regarding the clinical findings improvement, radiological improvement and postoperative complications between ETV and VP shunt are compared and showing that ETV should be considered as an alternative procedure to VP shunt in controlling severe hydrocephalus related to posterior fossa tumors to relieve symptoms quickly during the preoperative period when patients should wait for their definite tumor excision. Conclusion The shorter duration of surgery, the lower incidence of morbidity, the absence of mortality, the lower incidence of procedure failure of endoscopic third ventriculostomy as compared to ventriculoperitoneal shunt, and the significant advantage of not becoming shunt dependent make endoscopic third ventriculostomy to be recommended as the first choice in the treatment of pediatric patients with marked obstructive hydrocephalus due to posterior fossa tumors. It is a preliminary, simple, safe, effective, physiological, minimally invasive procedure for the relief of elevated intracranial pressure before direct tumor removal.

Epilepsy surgery in children under 3 years of age: surgical and developmental outcomes

OBJECTIVE Pediatric epilepsy surgery is known to be effective, but early surgery in infancy is not well characterized. Extensive cortical dysplasia, such as hemimegalencephaly, can cause refractory epilepsy shortly after birth, and early surgical intervention is indicated. However, the complication rate of early pediatric surgery is significant. In this study, the authors assessed the risk-benefit balance of early pediatric epilepsy surgery as relates to developmental outcomes. METHODS This is a retrospective descriptive study of 75 patients who underwent their first curative epilepsy surgery at an age under 3 years at the authors’ institution between 2006 and 2019 and had a minimum 1-year follow-up of seizure and developmental outcomes. Clinical information including surgical complications, seizure outcomes, and developmental quotient (DQ) was collected from medical records. The effects of clinical factors on DQ at 1 year after surgery were evaluated. RESULTS The median age at surgery was 6 months, peaking at between 3 and 4 months. Operative procedures included 27 cases of hemispherotomy, 19 cases of multilobar surgery, and 29 cases of unilobar surgery. Seizure freedom was achieved in 82.7% of patients at 1 year and in 71.0% of patients at a mean follow-up of 62.8 months. The number of antiseizure medications (ASMs) decreased significantly after surgery, and 19 patients (30.6%) had discontinued their ASMs by the last follow-up. Postoperative complications requiring cerebrospinal fluid (CSF) diversion surgery, such as hydrocephalus and cyst formation, were observed in 13 patients (17.3%). The mean DQ values were 74.2 ± 34.3 preoperatively, 60.3 ± 23.3 at 1 year after surgery, and 53.4 ± 25.1 at the last follow-up. Multiple regression analysis revealed that the 1-year postoperative DQ was significantly influenced by preoperative DQ and postoperative seizure freedom but not by the occurrence of any surgical complication requiring CSF diversion surgery. CONCLUSIONS Early pediatric epilepsy surgery has an acceptable risk-benefit balance. Seizure control after surgery is important for postoperative development.