Comprehensive Analysis of Hypermutation in Human Cancer.

We present an extensive assessment of mutation burden through sequencing analysis of >81,000 tumors from pediatric and adult patients, including tumors with hypermutation caused by chemotherapy, carcinogens, or germline alterations. Hypermutation was detected in tumor types not previously associated with high mutation burden. Replication repair deficiency was a major contributing factor. We uncovered new driver mutations in the replication-repair-associated DNA polymerases and a distinct impact of microsatellite instability and replication repair deficiency on the scale of mutation load. Unbiased clustering, based on mutational context, revealed clinically relevant subgroups regardless of the tumors' tissue of origin, highlighting similarities in evolutionary dynamics leading to hypermutation. Mutagens, such as UV light, were implicated in unexpected cancers, including sarcomas and lung tumors. The order of mutational signatures identified previous treatment and germline replication repair deficiency, which improved management of patients and families. These data will inform tumor classification, genetic testing, and clinical trial design.

Positional shunt assist for slit ventricle syndrome.

PURPOSE: Hydrocephalus is one of the most common pathologies in pediatric neurosurgery. One of the causes of recurring events of headaches among shunted children is "slit ventricle syndrome" (SVS). Several potential treatments have been proposed, yet SVS often represents a treatment challenge. The goal of the current series is to present our experience with adding a positional shunt-assist (SA) (Miethke, Aesculap) for the treatment of SVS. METHODS: Clinical data was retrospectively collected from all consecutive children with SVS that were treated with SA (Miethke, Aesculap) at our center. Surgical and clinical outcomes as expressed by hospital visits, or need for additional surgery, were evaluated. RESULTS: Nine cases were included. Hydrocephalus etiology included IVH (6), postinfectious (1), and congenital syndromes (2). Average age at first shunt was 4 months. Primary shunt type was differential-pressure-valve in all. Average age at SVS onset was 4 years. Average age at SA placement was 5.5 years. There were no perioperative complications besides a single stich abscess. A 6-month follow-up period after SA was compared to a 6-month period prior to the SA: average hospital visits decreased from 2.4 to 0.6 per patient (p < 0.0002). 4/9 patients needed an LP or shunt revision before the SA surgery, while no procedure was indicated during the immediate 6-month follow-up. At the last follow-up, there was a significant reduction in the rate of ER visits compared to prior to surgery; however, the number of neurosurgical procedures did not significantly differ. CONCLUSION: Using a SA for SVS was associated with a short-term improvement of symptoms in the majority of cases, reduction in hospital visits, and reduced need for SVS-related procedures.

The prepontine block and its relevance for the development and treatment of hydrocephalus.

OBJECTIVE: Pulsatile CSF flow patterns include flow through the ventricles to the subarachnoid space and cisterns and from the infra- to the supratentorial subarachnoid space. In this study, we demonstrate how an obstruction at the level of the prepontine space may lead to obstructive hydrocephalus with specific radiological characteristics, as well as the implications for treatment options. METHODS: We retrospectively collected data of patients who underwent surgery between February 2010 and December 2022 for hydrocephalus secondary to a suspected prepontine block. One additional patient diagnosed with prepontine block who did not undergo surgery was also included. We excluded patients with a background of previous unrelated neurosurgical procedures or CNS infections. RESULTS: Six children and two adults were included. Three presented with hydrocephalus on imaging, without any other underlying pathology. Five had a suprasellar arachnoid cyst, with its lower border abating the pons and occluding the spinal subarachnoid space (SAS). All cases had an open aqueduct on T2 sagittal sequences, as well as an infracerebellar or retrocerebellar CSF collection. In most cases, a horizontal web was identified in the prepontine region. Seven cases were treated with an endoscopic fenestration. One patient subsequently underwent a shunt surgery. All the operated children reached normal developmental milestones after surgery. CONCLUSIONS: This paper describes a rather small series of cases where clear obstruction was observed at the level of the prepontine subarachnoid space. We believe this anatomical subtlety adds to a better understanding of CSF pathways and the role of ETV in treating hydrocephalus, focusing on a small subgroup of patients without a clear obstruction.

The Pediatric Epilepsy Surgery Interest Group (PESIG) under the auspice of the ISPN Research Committee: Availability of relevant technology and geographical distribution.

PURPOSE: Epilepsy surgery for pediatric drug-resistant epilepsy has been shown to improve seizure control, enhance patient and family QoL, and reduce mortality. However, diagnostic tools and surgical capacity are less accessible worldwide. The International Society Pediatric Neurosurgery (ISPN) has established a Pediatric Epilepsy Surgery Interest Group (PESIG), aiming to enhance global collaboration in research and educational aspects. The goals of this manuscript are to introduce PESIG and analyze geographical differences of epilepsy surgery and technology availability. METHODS: PESIG was established (2022) following an ISPN executive board decision. Using a standardized form, we surveyed the PESIG members, collecting and analyzing data regarding geographical distribution, and availability of various epilepsy treatment-related technologies. RESULTS: Two hundred eighty-two members registered in PESIG from 70 countries, over 6 continents, were included. We categorized the countries by GDP as follows: low, lower-medium, upper-medium, and high income. The most commonly available technology was vagus nerve stimulation 68%. Stereoelectroencephalography was available for 58%. North America had statistically significant greater availability compared to other continents. Europe had greater availability compared to Africa, Asia, and South (Latin) America. Asia had greater availability compared to Africa. High-income countries had statistically significant greater availability compared to other income groups; there was no significant difference between the other income-level subgroups. CONCLUSION: There is a clear discrepancy between countries and continents regarding access to epilepsy surgery technologies. This strengthens the need for collaboration between neurologists and neurosurgeons from around the world, to enhance medical education and training, as well as to increase technological availability.

Shifting Strategies in the Treatment of Pediatric Craniopharyngioma.

PURPOSE OF REVIEW: Craniopharyngiomas represent one of the most challenging diseases to treat. Despite their benign histology, and after many decades of surgical experience and technological advancements, there is still no clear consensus regarding the most effective management for this tumor. Due to their location and aggressive local characteristics, purely surgical approaches all too often result in unacceptable morbidity. RECENT FINDINGS: Partial resection combined with radiation therapy results in similar control rates when compared to aggressive surgery, while also minimalizing the neuro-endocrinological morbidity. In this manuscript, we describe the historical progression of the shifting strategies in the management of pediatric craniopharyngioma. Time has also altered our expectations for outcomes, evolving from purely morbidity and mortality to simple Glasgow Outcomes Scales, now to formal neuro-psychometric and quality of life data.

Postoperative coagulopathy among otherwise healthy pediatric patients undergoing open craniosynostosis repair: a retrospective study.

Significant blood loss and resultant transfusion may lead to coagulopathy. The need for routine transfusion of non-RBC blood products in healthy pediatric patients suffering significant, yet controlled, intra-operative blood loss is controversial. Open craniosynostosis surgery is often associated with significant intra-operative blood loss and transfusion, and routinely preformed on otherwise healthy pediatric patients. Therefore, we found it as a useful model for our study, which aimed to assess the need for routine transfusion of non-RBC blood products in healthy pediatric patients suffering significant intra-operative blood loss. We conducted a retrospective cohort study of otherwise healthy pediatric patients, undergoing open craniosynostosis surgery and transfused solely with packed red blood cells (pRBCs) in a single large-volume tertiary surgical center, between January 2010 and December 2021. Among 457 eligible patients, 34 (7.4%) developed significant postoperative coagulopathy. Median [IQR] intra-operative pRBC transfusion volume was 17.4 ml kg-1 [13.3, 23.1]. Patients who developed coagulopathy did not have higher postoperative pRBC transfusion rate (8.8% vs 3.8%, P = 0.16) or volume (median [IQR], 0 [0, 0] vs 0 [0, 0] ml, P = 0.15), nor higher hospital LOS (5 [4, 5] vs 5 [4, 5] days, P = 0.66). ICU LOS was 0.8 [0.7, 1] vs 0.7 [0.6, 0.8] days (P = 0.02), a difference of no clinical significance.  Conclusions: The incidence of significant coagulopathy after craniosynostosis surgery was low, and not associated with clinically important complications. In otherwise healthy pediatric patients, even significant intra-operative blood loss can be safely managed solely with intravenous fluids and pRBC transfusion. What is Known: • Significant intra-operative blood loss and resultant transfusion may lead to postoperative coagulopathy. • There are potential deleterious effects from both coagulopathy and administration of blood products. What is New: • Open craniosynostosis corrective surgery is a useful model for studying coagulopathy after significant intra-operative blood loss and transfusion in otherwise healthy children. • Under certain conditions, in otherwise healthy pediatric patients, even significant intra-operative blood loss can be safely treated with intravenous fluids and pRBC transfusion alone, with no clinically significant postoperative coagulopathy or its complications.

Chiari Type 1 Malformation and Syringomyelia in Children: Classification and Treatment Options.

Chiari type 1 malformation (CIM) is defined as tonsillar ectopia of >5 mm, while syringomyelia (SM) is defined as a cerebrospinal fluid (CSF)-filled cavity larger than 3 mm dissecting the spinal cord. Over the last decades, our understanding of these pathologies has grown; however, many controversies still exist almost in every aspect of CIM and SM, including etiology, indication for treatment, timing of treatment, surgical technique, follow-up regime, and outcome. This chapter provides a comprehensive overview on different aspects of CIM and SM and on the still existing controversies, based on the evidence presently available. Future directions for clinical research concerning CIM and SM treatment and outcome are elaborated and discussed as well.

The role of messaging services in day-to-day practice in pediatric neurosurgery, advantages of a bubble network, and an international survey.

INTRODUCTION: Messaging services (MS) are used widely worldwide. The implications of MS usage in daily hospital patient care have not been investigated. In this study, we discuss the extensive usage of MS in our Pediatric Neurosurgical Department, introduce our bubble algorithm, and provide additional input from an international survey. METHODS: WhatsApp activity in the department of pediatric neurosurgery at Dana Children's Hospital, Tel Aviv, Israel, was analyzed. We designed a graphic representation of the content of the different conversation bubbles and how they interact. We also described a survey evaluating MS use in daily neurosurgical practice was sent to 25 neurosurgeons around the world. Collected data included details on the usage of MS, the type of information being transferred, and the participants' opinion of the potential risks and benefits of these systems. We began collecting messaging data November 2018, before the COVID pandemic era. We continued to collect data over the course of almost 3 years. RESULTS: We identified a bubble network structure that reflects a logical method of communication between different segments of pediatric neurosurgical care in our institution. Additionally, we analyzed 22 survey responses, received from 14 different countries. The vast majority of centers with "department groups" use messaging services to transfer multimedia files of patient-related data. Nineteen responders believe that MS significantly improve overall patient care. CONCLUSION: MS has revolutionized and improved the patterns of communication in our department. The great benefits of quick, simple access to information strongly outweigh formality and the potential for medicolegal disadvantages (e.g., HIPAA).

Neurosurgical aspects of Noonan syndrome.

PURPOSE: Noonan syndrome (NS) is a rare neurodevelopmental syndrome characterized by dysmorphic features, congenital heart defects, neurodevelopmental delay, and bleeding diathesis. Though rare, several neurosurgical manifestations have been associated with NS, such as Chiari malformation (CM-I), syringomyelia, brain tumors, moyamoya, and craniosynostosis. We describe our experience in treating children with NS and various neurosurgical conditions, and review the current literature on neurosurgical aspects of NS. METHODS: Data were retrospectively collected from the medical records of children with NS who were operated at a tertiary pediatric neurosurgery department, between 2014 and 2021. Inclusion criteria were clinical or genetic diagnosis of NS, age < 18 years at treatment, and need for a neurosurgical intervention of any kind. RESULTS: Five cases fulfilled the inclusion criteria. Two had tumors, one underwent surgical resection. Three had CM-I, syringomyelia, and hydrocephalus, of whom one also had craniosynostosis. Comorbidities included pulmonary stenosis in two patients and hypertrophic cardiomyopathy in one. Three patients had bleeding diathesis, two of them with abnormal coagulation tests. Four patients were treated preoperatively with tranexamic acid, and two with Von Willebrand factor or platelets (1 each). One patient with a clinical bleeding predisposition developed hematomyelia following a syringe-subarachnoid shunt revision. CONCLUSIONS: NS is associated with a spectrum of central nervous system abnormalities, some of which with known etiology, while in others a pathophysiological mechanism has been suggested in the literature. When operating on a child with NS, a meticulous anesthetic, hematologic, and cardiac evaluation should be conducted. Neurosurgical interventions should then be planned accordingly.

Brain-wide structural and functional disruption in mice with oligodendrocyte-specific Nf1 deletion is rescued by inhibition of nitric oxide synthase.

Neurofibromin gene (NF1) mutation causes neurofibromatosis type 1 (NF1), a disorder in which brain white matter deficits identified by neuroimaging are common, yet of unknown cellular etiology. In mice, Nf1 loss in adult oligodendrocytes causes myelin decompaction and increases oligodendrocyte nitric oxide (NO) levels. Nitric oxide synthase (NOS) inhibitors rescue this pathology. Whether oligodendrocyte pathology is sufficient to affect brain-wide structure and account for NF1 imaging findings is unknown. Here we show that Nf1 gene inactivation in adult oligodendrocytes (Plp-Nf1fl/+ mice) results in a motor coordination deficit. Magnetic resonance imaging in awake mice showed that fractional anisotropy is reduced in Plp-Nf1fl/+ corpus callosum and that interhemispheric functional connectivity in the motor cortex is also reduced, consistent with disrupted myelin integrity. Furthermore, NOS-specific inhibition rescued both measures. These results suggest that oligodendrocyte defects account for aspects of brain dysfunction in NF1 that can be identified by neuroimaging and ameliorated by NOS inhibition.

[EPILEPSY SURGERY IN CHILDREN: A SUMMARY OF A DECADE AT THE SOURASKY TEL AVIV MEDICAL CENTER].

INTRODUCTION: Drug-resistant epilepsy in children is associated with morbidity, developmental regression and mortality. Over recent years, there is an increase in awareness regarding the role of surgery in the treatment of refractory epilepsy, both in the diagnostic phase and for treatment, reducing the number and magnitude of seizures. Technological advancements have enabled a minimalization of surgery, with reduction in surgical associated morbidity. METHODS: In this retrospective study, we review our experience with cranial surgery for epilepsy between the years 2011-2020. Collected data included information regarding the epileptic disorder, surgery, surgical-related complications and epilepsy outcome. RESULTS: A total of 93 children underwent 110 cranial surgeries over a decade. The main etiologies included cortical dysplasia (29), Rasmussen encephalitis (10), genetic disorders (9), tumors (7) and tuberous sclerosis (7). The main surgeries included lobectomies (32), focal resections (26), hemispherotomies (25), and callosotomies (16). Two children underwent MRI-guided laser interstitial thermal treatment (LITT). The most significant improvements following surgery were following hemispherotomy or tumor resection (100% of children, each). Following resections for cortical dysplasia led to a significant improvement in 70%. In 83% of children undergoing callosotomy, there were no additional drop seizures; 14% of the entire group underwent additional epilepsy surgery; 23% of children had an unexpected complication, in the vast majority with no permanent sequela. There was not mortality. CONCLUSIONS: Epilepsy surgery may lead to significant improvement and even cure of epilepsy. There is a wide span of epilepsy surgical procedures. Ealy referral of children with refractory epilepsy for surgical evaluation may significantly reduce the developmental injury, and improve functional outcomes.

Monogenic Causes of Apparently Idiopathic Perinatal Intracranial Hemorrhage.

OBJECTIVE: Perinatal intracranial hemorrhage (pICH) is a rare event that occurs during the fetal/neonatal period with potentially devastating neurological outcome. However, the etiology of pICH is frequently hard to depict. We investigated the role of rare genetic variations in unexplained cases of pICH. METHODS: We performed whole-exome sequencing (WES) in fetuses and term neonates with otherwise unexplained pICH and their parents. Variant causality was determined according to the American College of Medical Genetics and Genomics (ACMG) criteria, consistency between suggested genes and phenotypes, and mode of inheritance. RESULTS: Twenty-six probands (25 families) were included in the study (9 with a prenatal diagnosis and 17 with a postnatal diagnosis). Intraventricular hemorrhage (IVH) was the most common type of hemorrhage (n = 16, 62%), followed by subpial (n = 4, 15%), subdural (n = 4, 15%), and parenchymal (n = 2, 8%) hemorrhage. Causative/likely causative variants were found in 4 subjects from 3 of the 25 families (12%) involving genes related to the brain microenvironment (COL4A1, COL4A2, and TREX-1). Additionally, potentially causative variants were detected in genes related to coagulation (GP1BA, F11, Von Willebrand factor [VWF], FGA, and F7; n = 4, 16%). A potential candidate gene for phenotypic expansion related to microtubular function (DNAH5) was identified in 1 case (4%). Fifty-five percent of the variants were inherited from an asymptomatic parent. Overall, these findings showed a monogenic cause for pICH in 12% to 32% of the families. INTERPRETATION: Our findings reveal a clinically significant diagnostic yield of WES in apparently idiopathic pICH and support the use of WES in the evaluation of these cases. ANN NEUROL 2021;89:813-822.

Fetal cerebral ventriculomegaly: What do we tell the prospective parents?

Fetal cerebral ventriculomegaly is a relatively common finding, observed during approximately 1% of obstetric ultrasounds. In the second and third trimester, mild (≥10 mm) and severe ventriculomegaly (≥15 mm) are defined according to the measurement of distal lateral ventricles that is included in the routine sonographic examination of central nervous system. A detailed neurosonography and anatomy ultrasound should be performed to detect other associated anomalies in the central nervous system and in other systems, respectively. Fetal MRI might be useful when neurosonography is unavailable or suboptimal. The risk of chromosomal and non-chromosomal genetic disorders associated with ventriculomegaly is high, therefore invasive genetic testing, including microarray, is recommended. Screening for prenatal infections, in particular cytomegalovirus and toxoplasmosis, should also be carried out at diagnosis. The prognosis is determined by the severity of ventriculomegaly and/or by the presence of co-existing abnormalities. Fetal ventriculoamniotic shunting in progressive isolated severe ventriculomegaly is an experimental procedure. After delivery, ventricular-peritoneal shunting or ventriculostomy are the two available options to treat hydrocephalus in specific conditions with similar long-term outcomes. A multidisciplinary fetal neurology team, including perinatologists, geneticists, pediatric neurologists, neuroradiologists and neurosurgeons, can provide parents with the most thorough prenatal counseling. This review outlines the latest evidence on diagnosis and management of pregnancies complicated by fetal cerebral ventriculomegaly.

Factors That Influence Intraoperative Decision-Making among Pediatric Neurosurgeons: A Grounded Theory Study.

INTRODUCTION: Pediatric neurosurgery is a subspecialty of medicine that is responsible for diagnosing, managing, and treating neurological disease in children with the use of surgery. Good intraoperative decision-making is critical to ensuring patient safety, yet almost nothing is known about what factors play a role in intraoperative decisions. As such, the purpose of this paper was to explore the factors that influence intraoperative decisions when pediatric neurosurgeons encounter something unexpected or uncertain during surgery. METHODS: The study utilized the grounded theory method of data collection and analysis. Twenty-six pediatric neurosurgeons from 12 countries around the world were interviewed between June and October 2020 about the factors that go into making intraoperative decisions. Data were analyzed line by line and constant comparison was used to examine relationships within and across codes and categories. RESULTS: Pediatric neurosurgeons reflected on 6 factors while operating in order to come to a decision about how to proceed when they encountered an uncertainty or complication. The study findings resulted in a conceptual model that describes how concrete data including biological and technological factors and contextual data including emotional/relational factors, surgeon factors, and cultural factors influence risk assessment when making an intraoperative decision during surgery. CONCLUSIONS: The findings from this research can be used for training and educating surgeons about intraoperative decision-making processes. Pedagogical modules can be developed that include training sessions on factors that may implicitly and explicitly influence thinking processes during an operation. Surgeons may also benefit from having open discussions with surgical colleagues about the rich, emotional, intellectual scope of the work that they do with all the challenges that these relationships can bring into decision-making in the operating room.

'Every patient is like my child': pediatric neurosurgeons' relational and emotional bonds with their patients and families.

PURPOSE: To explore the relational and emotional components of the surgeon-patient relationship from the perspective of practicing pediatric neurosurgeons in the field. MATERIALS AND METHODS: The study utilized the Grounded Theory Method of data collection and analysis. 26 pediatric neurosurgeons from 12 countries were interviewed using video-conferencing technology. RESULTS: Pediatric neurosurgeons find meaning, joy and pleasure in the relationships they form with their patients and their families, while also experiencing difficult and painful emotions when these patients do not do well. Four themes emerged from the analysis that include having a relational attachment to patients, forming bonds with the parents/caregivers of these patients, dealing with patient suffering, death and complications, and communicating bad news to parents. CONCLUSIONS: Pediatric neurosurgeons develop deep and enduring bonds with their patients and their families. These relationships are an integral part of what brings meaning and joy to their work, and simultaneously, are one of the most significant emotional challenges of their careers. . Training neurosurgical fellows should include pedagogical modules about the relational and emotional dimensions of their work, with a specific and dedicated focus on communicating bad news.

Epilepsy surgery in infants up to 3 months of age: Safety, feasibility, and outcomes: A multicenter, multinational study.

OBJECTIVE: Drug-resistant epilepsy (DRE) during the first few months of life is challenging and necessitates aggressive treatment, including surgery. Because the most common causes of DRE in infancy are related to extensive developmental anomalies, surgery often entails extensive tissue resections or disconnection. The literature on "ultra-early" epilepsy surgery is sparse, with limited data concerning efficacy controlling the seizures, and safety. The current study's goal is to review the safety and efficacy of ultra-early epilepsy surgery performed before the age of 3 months. METHODS: To achieve a large sample size and external validity, a multinational, multicenter retrospective study was performed, focusing on epilepsy surgery for infants younger than 3 months of age. Collected data included epilepsy characteristics, surgical details, epilepsy outcome, and complications. RESULTS: Sixty-four patients underwent 69 surgeries before the age of 3 months. The most common pathologies were cortical dysplasia (28), hemimegalencephaly (17), and tubers (5). The most common procedures were hemispheric surgeries (48 procedures). Two cases were intentionally staged, and one was unexpectedly aborted. Nearly all patients received blood products. There were no perioperative deaths and no major unexpected permanent morbidities. Twenty-five percent of patients undergoing hemispheric surgeries developed hydrocephalus. Excellent epilepsy outcome (International League Against Epilepsy [ILAE] grade I) was achieved in 66% of cases over a median follow-up of 41 months (19-104 interquartile range [IQR]). The number of antiseizure medications was significantly reduced (median 2 drugs, 1-3 IQR, p < .0001). Outcome was not significantly associated with the type of surgery (hemispheric or more limited resections). SIGNIFICANCE: Epilepsy surgery during the first few months of life is associated with excellent seizure control, and when performed by highly experienced teams, is not associated with more permanent morbidity than surgery in older infants. Thus surgical treatment should not be postponed to treat DRE in very young infants based on their age.

Dynamic mapping using an electrified ultrasonic aspirator in lipomyelomeningocele and spinal cord detethering surgery-a feasibility study.

BACKGROUND: Intraoperative neurophysiologic monitoring (IONM) is an established technique and adjunct of brain and spinal lesion resection surgery. In spina bifida syndrome surgery, mapping of the surgical wound is a common and accepted method in determining the position and functionality of nerve roots of the cauda equina (CE), especially when the anatomy is not straightforward and roots are splayed across or entangled within the lesion. Here, we describe a novel technique of continuous CE mapping using an electrified cavitron ultrasonic aspirator (eCUSA) in children with lipomyelomeningocele (LMMC) lesions. METHODS: We assessed a method of dynamic CE mapping using an eCUSA as a stimulation probe. Twenty children (0.5-18 years) were included in this study, diagnosed with occult spina bifida LMMC in which the eCUSA stimulator was applied. IONM data and 2-weeks post-operative data were collected. RESULTS: LMMC lesions were located in the lumbar, sacral, and lumbosacral spine. eCUSA stimulation at 0.3-3.0 mA intensities elicited positive lower extremity muscle responses in 12 of the 20 patients included in the study. These responses allowed the surgeon real-time identification of the nerve roots tangent at the LMMC-cauda equina structure and intensive removal of the fat tissue in the area non-responding to the eCUSA stimulation. CONCLUSION: Continuous eCUSA-based stimulation of the cauda equina during LMMC resection is a feasible mapping technique with potential added value improving safety of untethering. Future studies evaluating extension of untethering, as well as the rates of retethering and long-term neurological and urological outcomes, are warranted.

Pineal region tumors: an entity with crucial anatomical nuances.

PURPOSE: Intra-axial "pineal region" tumors include pineal, tectal, and aqueductal tumors. All three tumor subgroups cause obstruction of the aqueduct; however, they differ in radiological nuances, pathology, differential diagnosis, and treatment. The goal of this manuscript is to describe the radiological, clinical, and pathological nuances that differentiate between these subgroups. METHODS: All patients with intra-axial pineal region tumors were analyzed retrospectively, including demographics, radiological characteristics, pathology, treatment, and outcome. RESULTS: Forty-nine patients (1-69 years of age) were included: 19 pineal, 10 tectal, 10 aqueductal, 4 periaqueductal, and 6 complex. The 3 main subgroups differed in various radiological and anatomical nuances. Age and gender did not differ between groups. Other factors that did not differ between groups included T1 and T2 signals, presence of blood products, a normally located (non-displaced) tectum, anterior tectal displacement, thalamic involvement, and presence of hydrocephalus. The pathological spectrum differed between the 3 main subgroups, as well as the surgical treatment, and outcome. CONCLUSIONS: Despite sharing a close anatomical location, as well as all causing obstruction of the aqueduct with secondary hydrocephalus, the differential diagnosis, diagnostic methods, and possible treatment and surgical options differ between the various subgroups. Anatomical nuances are described to better delineate the various tumor subgroups and recommend specific treatment approaches.

Peritoneal insertion of shunts in children: comparison between trocar and laparoscopically guided insertion.

PURPOSE: Ventriculo-peritoneal shunts are commonly used for treating hydrocephalus for all age groups. There are 3 main methods for shunt placement into the peritoneum: mini laparotomy, laparoscopically guided, or percutaneously with a trocar. There is limited literature comparing between these techniques in the pediatric population, and specifically-the trocar has not been compared with laparoscopy. The goal of this study is to compare trocar and laparoscopy use, with respect to safety and potential need for future shunt revisions. METHODS: Data was retrospectively collected from 2 centers for children (< 18 years old) who underwent a primary insertion of a CSF shunt to the peritoneum, and had no prior abdominal surgery or significant abdominal disease. One center used a trocar, and the other laparoscopic guidance. Demographics, surgical time, and shunt complications were analyzed. Primary endpoint was distal shunt malfunction, either technique-related or non-technique-related. RESULTS: Two hundred fifty-seven children (220 trocar, 37 laparoscopy) were included. The groups were similar with regard to age at surgery and etiology of hydrocephalus. Trocar use was associated with a slightly higher, although statistically insignificant, rate of technique-related distal complications (4.1% vs 0, p = 0.37). Following propensity score matching, there was no statistically significant difference in any shunt complication between both groups. Trocar use was associated with shorter surgery, and less surgical personnel. CONCLUSIONS: In primary shunt surgery in children, abdominal placement of the catheter using a trocar or laparoscopic guidance is safe, and associated with a low distal malfunction rate, with no statistically significant differences between both techniques.

MRI-based diagnosis and treatment of pediatric brain tumors: is tissue sample always needed?

Traditional management of newly diagnosed pediatric brain tumors (PBTs) consists of cranial imaging, typically magnetic resonance imaging (MRI), and is frequently followed by tissue diagnosis, through either surgical biopsy or tumor resection. Therapy regimes are typically dependent on histological diagnosis. To date, many treatment regimens are based on molecular biology. The scope of this article is to discuss the role of diagnosis and further treatment of PBTs based solely on MRI features, in light of the latest treatment protocols. Typical MRI findings and indications for surgical biopsy of these lesions are described.