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Goal: To understand endoscopic third ventriculostomy (ETV) surgical result in the hydrocephalus. Setting: Benha University Hospitals Department of Neurosurgery. Between January 2016 to February 2020. Materials and processes: A total of 58 hydrocephalus patients were included in this research, regardless of gender prejudice. CSF loculation or tumour hydrocephalus was excluded. Hydrocephalus CT scan brain and MRI were diagnosed. Information on patient specifics, causes of hydrocephalus and operation problems were recorded in Performa patient. The data were examined in version 26 of SPSS. Frequency and percentage for categorical variables were computed. Mean ± SD for age has been computed. Results were presented as tables. Results: The research comprised a total of 58 individuals with hydrocephalus. Of the 58 patients, 32 were male (55.2 per cent) and 26 were female (44.8 percent ). The average age was 22.86 months. The most frequent cause of hydrocephalus was congenital aqueductal stenosis. CSF leaking was observed in 20 individuals after surgery (34.5 percent ). 6 individuals have postoperative infection (10.3 percent ). Conclusions: third ventriculostomy endoscopic is safe and effective. Patients who fulfil the requirements are offered the option of freeing themselves from shunt dependence via endoscopic third ventriculostomy.

Spontaneous third ventriculostomy (STV) is characterized by the spontaneous rupture of one of the ventricle walls due to increased pressure in the third ventricle caused by obstructive hydrocephalus. Clinically, STV results in resolution of signs and symptoms of intracranial hypertension and head circumference stabilization. No spontaneous STV cases in patients with myelomeningocele have been reported in the literature. The objective of this study was to report three cases of STV in patients with type 2 Chiari malformation who underwent intrauterine treatment. All patients presented clinically with increased head circumference during outpatient follow-up. Only one patient required a ventriculoperitoneal shunt implantation. The other patients did not require further intervention. STV is a rare entity that is difficult to diagnose and should always be suspected in spontaneous hydrocephalus resolution, especially in early childhood. STV is not synonymous with hydrocephalus resolution.

Objective: To evaluate and discuss the outcomes of a combination of ventriculocystostomy (VC) and endoscopic third ventriculostomy (ETV) for obstructive hydrocephalus (HCP) due to ventricular/cisternal arachnoid cysts, and only ETV for obstructive HCP due to different etiologies. Methods: We retrospectively reviewed all 40 symptomatic patients (aged 4 months – 61 years) of obstructive HCP treated by ETV or VC+ETV during October 2014 – April 2019. VC+ETV was performed in 7 patients with intraventricular/cisternal arachnoid cyst and obstructive HCP. Only ETV was performed in 33 patients with obstructive HCP due to other etiologies. Results: Successful ETV or VC+ETV surgery was performed in 35 patients. The procedure failed in 5 patients aged < 1 year; all these 5 patients had a head circumference (HC) of > 90 percentile at the time of surgery. Another 5 patients aged < 1 year showed successful ETV, with a HC of 75–90 percentiles. Conclusion: ETV is a successful alternative treatment for obstructive HCP. The ventricular size may not demonstrate a remarkable reduction post-ETV than post-shunting. Accordingly, increased intracranial pressure may not effectively decrease during the early period post-ETV than post-shunting. Therefore, the success rates of VC and/or ETV are low in very young patients with very high HCs (> 90 percentile).

Endoscopic third ventriculostomy (ETV) is a hydrocephalus treatment procedure that involves opening the Liliequist membrane (LM). However, LM anatomy has not been well-studied neuroendoscopically, because approach angles differ between descriptive and microsurgical anatomical explorations. Discrepancies in ETV efficacy, especially among children age 2 and younger, may be due to incomplete LM opening. The objective of this study was to characterize the LM anatomically from a neuroendoscopic perspective to better understand the impact of anatomical features during LM ostomy and the ETV success rate. Additionally, the ETV success score was tested to predict patient outcome after the intraoperatively difficult opening of LM. Fifty-four patients who underwent ETV were prospectively analyzed with a mean follow-up of 53.1 months (1-90 months). The ETV technical parameters of difficulty were validated by seven expert neurosurgeons. The pediatric population (44) of this study represents the majority of patients (81.4%). The overall ETV success rate was 68.5%. Anomalies on the IIIVT floor resulted in an increased rate of ETV failure. The IIIVT was anomalous, and LM was thick in 33.3% of cases. Fenestration of LM was difficult in 39% of cases, and the LM and TC were opened separately in 55.6% of cases. The endoscopic third ventriculostomy success score (ETVSS) accurately predicted the level of difficulty opening the LM (p = 0.012), and the group with easy opening presented greater durability in ETV success. Neurosurgeons should be aware of the difficulty level of the overture of LM during ETV and its impact on long-term ETV effectiveness.

Background: Although generally a safe procedure, serious postoperative complications after endoscopic third ventriculostomy (ETV) for obstructive hydrocephaly have been rarely reported, such as del...

Background: Pineal region tumors often present with hydrocephalus. Endoscopic third ventriculostomy (ETV) and simultaneous tumor biopsy remain a minimally invasive procedure offering both diagnostic and therapeutic advantages in the management of these tumors. However, different operative techniques have been described in the literature. Aim: The aim is to study the ETV success rate, diagnostic rate of simultaneous tumor biopsy, complications, and follow-up of patients of pineal region tumors managed with ETV and simultaneous tumor biopsy using the single burr hole technique. Methods: The study was performed by retrospectively reviewing the records of patients of pineal region tumors managed by simultaneous ETV and tumor biopsy using a “single burr hole” technique from January 2012 to December 2019. Results: Thirty-four patients (22 males and 12 females) with a mean age of 28.7 years were analyzed. ETV was successful in relieving hydrocephalus in 29 (87.8%) patients. Three patients needed a ventriculoperitoneal shunt, and one required Ommaya reservoir placement for persistent hydrocephalus. Histological diagnosis was successfully established in 26 (78.8%) patients. There were two procedure-related mortalities. Two patients underwent craniotomy and tumor excision subsequently. Radiotherapy was given to 11 patients, and 9 patients were managed by observation alone. The mean follow-up of our study was 15.8 months. Conclusions: Simultaneous ETV and tumor biopsy using a single burr hole technique is a safe, minimally invasive procedure for the management of pineal region tumors.

Three-dimensional (3-D) endoscopes have been widely used for a large variety of approaches in neurosurgical practice. However, in the last decade, 3-D scopes were barely used for ventricular surgery. In this study, we illustrated our preliminary experience with a 3-D endoscope for third ventriculostomy using new dedicated endoscopic equipment.Over a 12-month period, a high-definition 3-D endoscopic third ventriculostomy (ETV) was performed in 14 patients with obstructive hydrocephalus. Patients were followed prospectively and compared retrospectively with a matched group of 16 similar patients who underwent ETV with a standard 2-dimensional (2-D) endoscope. Surgical outcome and intra- and postoperative course were retrospectively reviewed.3-D ETV provided excellent surgical results, and no significant difference was shown in terms of outcome, complication, and length of hospitalization between the 2 groups. Moreover, operative time (minutes) was significantly shorter in the 3-D group than the 2-D ETV group (19.9 ± 4.8 vs. 22.9 ± 1.4, respectively; P0.05), and the use of the 3-D endoscope provided subjective improvements of depth perception, hand-eye coordination, and surgeon comfort.Our preliminary study clearly demonstrated the effectiveness of 3-D ETV and provided a significant reduction of operative time. Depth information from the 3-D scope appears to facilitate rapid and stable ETV maneuvers, representing a critical development that may become a valuable tool for neuroendoscopy.

Spontaneous third ventriculostomies have been reported in relation to obstructive hydrocephalus and increased intracranial pressure and are most commonly seen as disruption of the floor of the third ventricle. Hydrocephalus has been reported in patients with Krabbe disease; however, it is clinically difficult to monitor for hydrocephalus in patients with Krabbe disease as symptoms of increased intracranial pressure may overlap with symptoms of Krabbe disease. We describe a case series of spontaneous third ventriculostomy and hydrocephalus, likely in response to increased intracranial pressure, in patients with infantile Krabbe disease.Brain magnetic resonance images of patients with infantile Krabbe disease were retrospectively analyzed to assess for ventricular size and presence of spontaneous third ventriculostomies. A brain atlas was used to standardize the calculation of ventricular size. Mid-sagittal, T2-weighted images around the third ventricle were assessed for spontaneous third ventriculostomies. Developmental outcomes were measured with a series of standardized and validated tests.Seventy-five patients with infantile Krabbe disease were evaluated. Twelve cases of spontaneous third ventriculostomies were identified. Head circumference (SE = 8.07; P 0.001) and average ventricular volume were greater (left: SE = 1.47, P 0.001) in patients with spontaneous third ventriculostomies when compared with patients without spontaneous third ventriculostomies. Patients with spontaneous third ventriculostomies also had more delayed development in adaptive (difference = 0.2, P 0.01), gross motor (difference = 0.0, P 0.01), and fine motor (difference = 0.1, P 0.001) function.Spontaneous third ventriculostomies, likely in the context of increased intracranial pressure, were identified in patients with Krabbe disease. Although difficult to assess, our study highlights the importance of monitoring for increased intracranial pressure, which can result in spontaneous third ventriculostomies, in patients with infantile Krabbe disease.

A 42-year-old male presented with diplopia, headache, and nausea. Magnetic resonance imaging (MRI) of the brain showed pineal tumor, and chest computed tomography (CT) demonstrated a lung tumor. Disorientation developed, with occurrence of hydrocephalus, and we performed neuroendoscopic surgery for biopsy of the pineal tumor and third ventriculostomy. The lung tumor was biopsied under bronchoscopic and CT guidance, and based on the pathological results, we diagnosed pineal metastasis of pulmonary sarcomatoid carcinoma (cT3N1M1b Stage IVA). Stereotactic radiotherapy for the metastatic pineal tumor and systemic chemotherapy (carboplatin + pemetrexed) were pursued, but hemorrhage of the tumor occurred, hydrocephalus worsened, and neoplastic meningitis was diagnosed by MRI. Therapy was switched to nivolumab, but without effect, and the patient succumbed. Even among lung tumors, sarcomatoid carcinoma is rare. There are also few reports of lung tumors metastasized to the pineal gland. Our case report of pineal tumor regarded as metastasis of pulmonary sarcomatoid carcinoma also includes a discussion of the literature.

The basal subarachnoid cisterns are expansions of the subarachnoid space and transmit cranial nerves and intracranial vessels. Providing neurosurgeons with key concepts, anatomical landmarks, and techniques can result in safer procedures and better patient outcomes. In this review, we discuss the major basal subarachnoid cisterns including their embryology, history, anatomical descriptions, and use during surgical approaches.

CSF flow disorders are frequently encountered in children. The advent of MR technology with the emergence of new pulse sequences allowed better understanding of CSF flow dynamics. In this pictorial review, we aim to conduct a comprehensive review of the MR protocol used to study CSF flow disorders and to discuss the utility of each pulse sequence in the adopted protocol. We will focus on the key anatomical structures that should be examined to differentiate hydrocephalus form ventricular dilatation ex-vacuo. The MR features of obstructive and communicating hydrocephalus will be discussed, in addition to the manifestations of CSF disorders associated with posterior fossa malformations (Dandy-Walker malformation, Chiari, and Blake’s pouch cyst). Moreover, the value of MRI in the assessment of patients following interventional procedures (ventriculoperitoneal shunt and third ventriculostomy) will be addressed. Electronic supplementary material The online version of this article (10.1186/s13244-019-0686-x) contains supplementary material, which is available to authorized users.

Endoscopic third ventriculostomy (ETV) is a hydrocephalus treatment procedure that involves opening the Liliequist membrane (LM). However, LM anatomy has not been well-studied neuroendoscopically, because approach angles differ between descriptive and microsurgical anatomical explorations. Discrepancies in ETV efficacy, especially among children age 2 and younger, may be due to incomplete LM opening. The objective of this study was to characterize the LM anatomically from a neuroendoscopic perspective to better understand the impact of anatomical features during LM ostomy and the ETV success rate. Additionally, the ETV success score was tested to predict patient outcome after the intraoperatively difficult opening of LM. Fifty-four patients who underwent ETV were prospectively analyzed with a mean follow-up of 53.1 months (1–90 months). The ETV technical parameters of difficulty were validated by seven expert neurosurgeons. The pediatric population (44) of this study represents the majority of patients (81.4%). The overall ETV success rate was 68.5%. Anomalies on the IIIVT floor resulted in an increased rate of ETV failure. The IIIVT was anomalous, and LM was thick in 33.3% of cases. Fenestration of LM was difficult in 39% of cases, and the LM and TC were opened separately in 55.6% of cases. The endoscopic third ventriculostomy success score (ETVSS) accurately predicted the level of difficulty opening the LM (p = 0.012), and the group with easy opening presented greater durability in ETV success. Neurosurgeons should be aware of the difficulty level of the overture of LM during ETV and its impact on long-term ETV effectiveness.

It is difficult to demonstrate the success of the procedure in patients with third ventriculostomy. We evaluated that optic nerve sheath diameter (ONSD) measurement, which can reflect intracranial pressure, may be a criterion for decision of endoscopic third ventriculostomy (ETV) success.28 adult patients suffering long overt standing ventriculomegaly (LOVA) who performed ETV were included in this retrospective study. The patients were divided into two groups as successful (group A) and failed ETV group (group B) according to their postoperative evaluation. ONSD was measured on pre- and post-operative computed tomography (CT) and Evan's index (EI), diameter of third ventricule (V3), the patency of ETV stoma and periventricular edema were evaluated by magnetic resonance imaging (MRI).The mean ONSD was measured as 6.39±0.92mm for the right eye, 6.50±0.91mm for the left eye on preoperative CT. The mean ONSD by CT (after surgery) was 4.89±0.87mm for the right eye, 5.02±0.1mm for the left eye (p0.05). Postoperative group A and group B were compared according to ONSD measurement; mean ONSD in group A was 4.52±0.69mm for the right and 4.59±0.9mm for the left, mean ONSD in group B was 5.82±0.51mm for the right and 6.1±0.32mm for the left (p0.05). The best ONSD value for detecting failed ETV was 5.40mm (sensitivity 90%, specifity 75%, AUROC 0.938) for right and 5.91mm (sensitivity 90%, specifity 75%, AUROC 0.950) for left. EE was measured as 0.39±0.12mm on preoperative MRI and 0.39±0.12mm on postoperative MRI (p=0.3). V3 was measured as 14.7±2.47mm on preoperative MRI and 10.47±1.99mm on postoperative MRI (p0.05).The statistical values obtained from study show that the ONSD measurement can help in the postoperative evaluation of patients, who had a ETV surgery.

Background: Modern technical capabilities have made minimally invasive surgery increasingly popular. Small incisions can reduce surgical duration and the degree of tissue trauma, which reduces the risk of complications. Burr hole microsurgery is a relatively new minimally invasive technique used in neurosurgery. The objective of this study was to assess the feasibility and outcomes of using burr hole microsurgery for the management of intracranial lesions. Methods: Forty-four adults were treated with burr hole microsurgery. Patients were divided into groups according to the presence of (1) brain tumors (n = 20); (2) congenital brain cysts (n = 16); (3) cavernous angiomas (n = 3); and (4) neurovascular conflicts of the 5th cranial nerve (n = 5). All surgical interventions were performed using the “MARI” device. Results: The transcortical approach was used to remove 16 brain tumors, and 2 brain tumors were biopsied. In the two tumor biopsy cases, the parasagittal interhemispheric route was used. Gross total resection was achieved in 10 cases (62.5%) when tumor size reached up to 4 cm, subtotal resection was achieved in four cases (25%) in large tumors, and partial resection in two cases (12.5%). In patients with congenital cysts, cavernous angiomas, trigeminal neuralgia, and symptomatic regression were noted the postoperative period. The surgical duration was 30–180 min (median, 75 min). A hemorrhagic complication was observed in one case. Significant postoperative complications and mortality were not observed. Conclusion: Burr hole microsurgery can treat different intracranial lesions effectively. Despite a smaller craniotomy diameter of 11–14 mm compared with keyhole approaches, surgery was successful.

A seven years-old boy presented with headache and progressive drowsiness. MRI demonstrated obstruction at the foramen of Magendie and foramina of Luschka. Child underwent an endoscopic third ventriculostomy (ETV). The postoperative course was uneventful. MRI scan showed a marked decrease in the size of the ventricles, showing therefore that cerebrospinal fluid flow through the ventricular system and subarachnoid space was restored de Oliveira RS and Ballestero MFM contributed equally to this manuscript

Hydrocephalus is a major cause of patient decreased quality of life and high health care financial burden in the United States and throughout the world. The placement of ventricular shunts (ventriculoperitoneal shunt) has proven to be a safe treatment for hydrocephalus, but it is associated with a high complication rate leading to a lower quality of life and continued financial burden for patients, their families, and society as a whole. The endoscopically assisted third ventriculostomy (ETV) has been practiced as an alternative to ventricular shunting since the 1990s. Success rates vary widely and there are many factors which contribute to the varying success rates. The ETV procedure has the potential to alleviate much of the overall quality of life issues and some of the financial burdens associated with hydrocephalus provided success rates can be increased and the procedure and management techniques are adopted more widely. Common techniques have been published in the past which report associated improvements in success rates amongst individual surgeons. Here, we report a novel perioperative technique and management strategy that displays a higher than reported success rate. Our methods and results show potential to significantly improve overall ETV success rates if reproduced and subsequently adopted widely. We retrospectively studied records of 24 adult patients with hydrocephalus who were treated with an ETV procedure. Routinely, we placed an external lumbar drain postoperatively which was continued for a minimum of 2 days. There was a 95.8% success rate at 30 days. The overall success rate was 83.3%. This is significantly higher than the average of the predicted success scores calculated by the ETV success scoring system (71.8%). It is also significantly higher than previous studies' reported ETV success rates in adults. We propose additional similar studies to be performed to test the reproducibility of increased success rates using our technique, ideally through a prospective, randomized, multicenter trial.

We report a case of headache due to a hydrocephalus with associated syringomyelia. Magnetic resonance imaging showed a fourth ventricular outlet obstruction. An endoscopic third ventriculostomy (ETV) was performed with successful clinical and radiologic outcome. Fourth ventricular outlet obstruction is an uncommon cause of obstructive hydrocephalus, but it must be kept in mind. As far as we know, there are few reports that propose ETV as treatment. Therefore our case supports that ETV could be a successful option for the management of this condition.

To present an attainable and realistic model for neuroendoscopic simulation which replicates exercises of tissue biopsy and coagulation and membrane fenestration.We presented a stepwise method to create a neuroendoscopic simulation model using bovine brain and membrane units made by a soda cup covered by an amniotic membrane inside an expanded polystyrene spherical container. We used face validation for preliminary evaluation. We also rated the students before and after training with the NEVAT global rating scale (GRS) and recorded the time required to complete all 3 procedures (third ventriculostomy, tissue biopsy, and coagulation). The total cost of the model was $5.The experts consider this new model as capable of reproducing real surgical situations with great similarity to the human brain. We tested the model in 20 trainees. The median GRS score before the training was 9 (range, 7-12). After repeated training and performance feedback, the final median GRS score was 41 (range, 37.5-45; P0.0001). The time needed to finish the exercises before training was 33 minutes (range, 30.5-42.5 minutes), and after using the model the final median time was 20 minutes (range, 17.5-22 minutes; P 0.0001).Simulators for neuroendoscopy described so far are reliable, but they entail a high cost. Models with live animals, although of lower cost, are questioned from an ethical point of view. In the current work, we describe a high fidelity ventricular neuroendoscopic simulator model that, because of its low cost, can be replicated in any training center that has a neuroendoscope.

A 4-year-old boy with macrocephaly (+3 SD) was evaluated with MRI, which showed a subdural hygroma secondary to rupture of a left temporal arachnoid cyst and triventricular enlargement. Two years later, we repeated imaging to explore the possibility of a third ventriculostomy. There was a flow void across the floor of the third ventricle (figure). By using 3D cardiac-gated dynamic imaging, we demonstrated that a spontaneous ventriculostomy had already occurred (video, [links.lww.com/WNL/B217][1]). Spontaneous third ventriculostomy is rare and can occur in children and adults with chronic hydrocephalus.1 [1]: http://links.lww.com/WNL/B217

Objectives Endoscopic third ventriculostomy (ETV) emerged as an effective alternative to shunting devices in patients with obstructive hydrocephalus. When ETV fails, neurosurgeons must choose between applying a shunting device or performing a repeat ETV (re-ETV) and attempt a shunt independent outcome. In this series, clinical, surgical and follow-up data from six patients who underwent a second ETV were reviewed. Patients and methods Between January 2005 and June 2015, six patients underwent re-ETV, with four being children. Causes of obstructive hydrocephalus included idiopathic aqueduct stenosis, congenital aqueduct stenosis, neonatal intraventricular haemorrage, hypothalamic glioma and post-meningitis aqueductal stenosis. Success of the procedure was defined by clinical improvement and shunt independence. Results Overall success rate of this series was 83.3%, with re-ETV being effective in five of the six patients. The single case of re-ETV failure was observed in the pediatric population and was due to late stoma obstruction by tumoral growth, with a ventriculo-peritoneal shunt (VPS) being placed 6 months after re-ETV. In this series, no mortality and no major permanent morbidity were observed following re-ETV. Conclusion Repeat ETV is a safe and effective procedure and should be an option for treatment of recurrent obstructive hydrocephalus if stoma closure or obstruction is present. Younger age and the presence of a previous VPS should not discourage this procedure.

Aplasia cutis congenita of scalp (ACCS) is a rare developmental anomaly. It has presented in children who have many concomitant anomalies. Large, deep defects can complicate by repeat local and systemic sepsis and life-threatening hemorrhage. In this paper, we describe, to the best of our knowledge, the first case of a newborn boy with ACCS and myelomeningocele whose evolution with hydrocephalus has brought us a serious paradigm of using a shunt in the presence of tissue expanders. The treatment of hydrocephalus with third ventriculostomy associated with good aesthetic final result show an alternative to the use of shunt in this scenario, even in infants of young age. We review here therapeutic strategies and challenges with this disease.

Introduction: The use of neuroendoscopic for the management of myelomeningocele-associated hydrocephalus is still controversial. Anatomic variations and abnormalities of the ventricular system and the confusing topography of the floor of the third ventricle are of utmost importance in this approach. Case presentation: The present case video displayed a case of a 4-year-old girl with myelomeningocele related hydrocephalus presented with a closure of third ventriculostomy after tethered cord release. Several neuroendoscopic options were depicted such as third ventriculostomy using bipolar diathermia, ventriculocystostomy of a quadrigeminal arachnoid cyst and a choroid plexus cauterization as a strategy to improve the success rate of this procedure and handle the difficulties presented in this form of hydrocephalus. Discussion: This video warned the young neurosurgeons regarding ventricular anatomic challenges presented in this case as well as the need of customization of neuroendoscopic approach. Conclusion: Neuroendoscopic approach is an effective strategy to manage myelomeningocele-associated-hydrocephalus.

Hypothalamic lesions can compromise its essential regulatory roles resulting in critical disruption of temperature and blood pressure homoeostasis. We present the case of a 55-year-old woman who had been previously submitted to several neurosurgical procedures aimed at treating idiopathic hydrocephalus. She presented to our department with recurring episodes of hypothermia and wide blood pressure variations, which had been worsening over the last few years. After extensive complementary workup, which excluded new neurological lesions or endocrinological conditions, hypothalamic dysfunction was assumed to be the cause of this syndrome. She was successfully treated with midodrine and on-demand captopril, which resulted in adequate control of her blood pressure. This case highlights the rare and unpredictable consequences of damage to the hypothalamus, depicting the favourable result of a heretofore unpublished medical approach.

Dear Reader, You are receiving the second volume of Archives of Pediatric Neurosurgery. This is an electronic journal from the Brazilian Society of Pediatric Neurosurgery, which is published quarterly. We are dedicating this volume to hydrocephalus-related topics. Hydrocephalus is one of the most important topics in pediatric neurosurgery and is related to up to 40% of the activities performed in many specialized services. With its multiple etiologies and variety of proposed treatment modalities, this disease presents a huge challenge, and many questions have not been answered. When focusing on fetal hydrocephalus, we still cannot determine which fetuses will benefit from treatment during the fetal period. The answers are also unclear as to why surgery for myelomeningocele correction performed under 27 weeks of gestation decreases the prevalence of hydrocephalus. What is the best treatment for intraventricular hemorrhage in premature infants: Brain Washing, external ventricular drainage, ventriculosubgaleal shunt, reservoir placement and repeated punctures or lumbar punctures? Furthermore, what is the best treatment for hydrocephalus in cases of posterior fossa tumor: Placing an external ventricular drain and resecting the tumor, performing a third ventriculostomy or placing a ventriculoperitoneal shunt, considering that in cases of giant tumors in each of these situations, ascending herniations can occur? Of course, the procedure selected will be related to the dynamics, volume and expertise of the treatment center. Regarding valves, we know that the number of shunts implanted annually is enormous and that the market for valves is divided among several companies that manufacture these prostheses. Which valve is the best? An anti-gravitational one, a programmable one, one with an anti-siphon mechanism? Fixed-pressure valves, valves that produce fewer MRI artifacts, those that do not experience pressure changes due to MRI, or those impregnated with antibiotics? How can a simple valve work as well as a valve with a complex system? Why does a valve system cost $1,000 in one country but costs ten times more in another country? Why are some types of valves sold only in underdeveloped countries? How can we avoid or minimize biological and mechanical complications after valve placement? Even neuroendoscopic procedures that have been performed for more than 25 years in Brazil are not free of complications such as bleeding, infection and technical incapacity of the professional. Nevertheless, we believe that all services that treat children with hydrocephalus should be able to provide neuroendoscopic procedures, thus decreasing the number of shunts placed. We hope that this volume can answer some of these questions. I invite you to participate in Archives of Pediatric Neurosurgery by contributing scientific articles focusing on pediatric neurosurgery. This volume was compiled during the coronavirus (COVID-19) pandemic. We hope that this disease will be controlled quickly, allowing us to return to our normal activities. We also hope that pregnant women will not transmit the disease to their fetuses, causing hydrocephalus, as was observed in the last Zika virus epidemic. Sincerely, Sergio Cavalheiro Editor-in-chief, Archives of Pediatric Neurosurgery

Introduccion La hidrocefalia de inicio fetal, perinatal y neonatal representa un gran reto terapeutico y a menudo cursa con un pronostico neurologico pobre, debido a su etiologia heterogenea, que incluye alteraciones del neurodesarrollo. Objetivo Realizar una recopilacion de los avances en terapia celular como propuesta que permite ampliar el espectro de tratamiento en la hidrocefalia congenita-neonatal. Desarrollo Las intervenciones terapeuticas disponibles actualmente, como la derivacion ventriculo-peritoneal y la tercera ventriculostomia, son insuficientes para resolver por completo la hidrocefalia y para prevenir o revertir los danos neurologicos asociados. Es por esto por lo que ha surgido la necesidad de crear nuevas alternativas terapeuticas a partir del conocimiento de los mecanismos fisiopatologicos que participan en el desarrollo de esta condicion. Particularmente, la terapia celular con celulas madre neuronales y celulas madre mesenquimales ha demostrado, en estudios con animales y en estudios preclinicos con humanos, ser eficiente y segura para prevenir la hidrocefalia originada a partir de la disrupcion de la zona ventricular y secundaria a la hemorragia intraventricular, con la consiguiente prevencion de las secuelas neurologicas sensoriomotoras y cognitivas. Conclusiones Hasta el momento no tenemos un tratamiento eficiente que ofrezca calidad de vida a los pacientes con hidrocefalia, y que esa alternativa terapeutica sea efectiva. Introduction Fetal hydrocephalus, perinatal and neonatal represents a major therapeutic challenge and often has with poor neurological prognosis, due to its heterogeneous aetiology, including neurodevelopmental disorders. Objective To make a collection of advances in cell therapy as a proposal that can extend the spectrum of treatment in congenita-neonatal hydrocephalus. Development Therapeutic interventions available at present as the ventricle-peritoneal shunt and third ventriculostomy, are insufficient to fully resolve the hydrocephalus and to prevent or reverse the associated neurological damage. This is why what has emerged the need for new therapeutic alternatives based on the knowledge of physiopathological mechanisms involved in the development of this condition. In particular, cell therapy with neural stem cells and mesenchymal stem cells has proven in animal studies and preclinical studies with humans, efficiently and safely to prevent hydrocephalus originated from the disruption of the ventricular zone and secondary to intraventricular hemorrhage, with the consequent prevention of neurological sequelae sensorimotor and cognitive. Conclusions So far, we do not have an efficient treatment that offers quality of life to patients with hydrocephalus, and that alternative therapy to be effective.

The treatment of hydrocephalus has changed in recent years with better imaging and introduction of endoscopic procedures as well as enhanced shunts. Indications of endoscopic third ventriculostomy (ETV) are now more refined with better quantification of outcome. This article reviews the current state of neuroendoscopy for infective hydrocephalus in children. The roles of third ventriculostomy as a primary procedure or after shunt malfunction, endoscopic interventions in multiloculated hydrocephalus and introduction of intraventricular lavage to salvage severely infected children are evaluated.

Objective: The term “hydrocephalus” encompasses a range of disorders characterised by clinical symptoms, abnormal brain imaging and derangement of cerebrospinal fluid (CSF) dynamics. The ability to elucidate which patients would benefit from CSF diversion (a shunt or third ventriculostomy) is often unclear. Similar difficulties are encountered in shunted patients to predict the scope for improvement by shunt re-adjustment or revision.

The efficacy of endoscopic third ventriculostomy (ETV) in childhood strongly correlates with age. Reasons for the higher failure rate in infants are under debate but the “distensible” head with its immature skull (which would prevent an adequate flow through the ETV and/or a sufficient pressure gradient between the cortical subarachnoid spaces and the venous sinuses) has been suggested as a possible cause. Our evidence of spontaneous conversion of a failed, but always patent, third ventriculostomy into an effective one occurring in a child after maturation of the skull supports this hypothesis.

Summary Objective To investigate the safe displacement range of the foramen of Monro (FM) during single burr hole rigid endoscopic third ventriculostomy (ETV) and endoscopic tumor biopsy (ETB). Methods Eleven patients who received ETV/ETB for third ventricular and pineal region tumor were reviewed. The burr-hole location, the size, and the virtual displacement of FM were measured using neuronavigation software. Results Hydrocephalus was resolved, and no subsequent cerebrospinal fluid (CSF) shunting was required in all cases. Histological diagnosis was established in 11 patients. Ten cases received instrumental cognitive and memory assessment postoperatively. The results were within the normal range for eight cases. The mean burr-hole location was 1.7 cm anterior to coronal suture and 3 cm from the midline. The mean diameters of FM measured on the axial, coronal, sagittal, and views were 5.7, 7.8, and 5.6 mm, respectively. The mean virtual displacements of the FM were 1.9 ± 2.0 mm (range = 0–4.8) for ETV and 2.4 ± 2.1 mm (range = 0–5.5) for ETB. The maximum displacements were 4.8 mm anteriorly for ETV and 5.5 mm posteriorly for ETB. Conclusion Single burr hole rigid ETV/ETB is likely to be safe within maximum FM displacements of 4.8 mm anterior for ETV and 5.5 mm posterior for ETB. Preoperative trajectory planning using neuronavigation software is recommended.

Neuroendoscopy has established itself as a routine neurosurgical procedure in pediatric population. To have an uneventful smooth perioperative course, it is important for anesthesiologist to know about the key steps, possible complications, and specific anesthetic requirements. Among neuroendoscopies, endoscopic third ventriculostomy is the commonly performed one.

Hydrocephalus should be seen as a condition rather than a disease. Since different conditions can lead to hydrocephalus, the treatment has to be adapted and tailored to each patient. Whenever possible, the cause of hydrocephalus should be directly addressed. Tumor resection and endoscopic procedures for third ventriculostomy or cyst fenestration can often prevent the insertion of cerebrospinal fluid (CSF) shunts. There is no ideal shunt so far; therefore, every effort and measures should be taken to avoid shunt insertion. It is so important to treat every hydrocephalous patient as individual case, and strategic planning should be made for a lifelong management.

The major advantages of three-dimensional sampling perfection with application optimized contrasts using different flip-angle evolution (3D-SPACE) technique are its high resistance to artifacts that occurs as a result of radiofrequency or static field, the ability of providing images with sub-millimeter voxel size which allows obtaining reformatted images in any plane due to isotropic three-dimensional data with lower specific absorption rate values. That is crucial during examination of cerebrospinal-fluid containing complex structures, and the acquisition time, which is approximately 5 min for scanning of entire cranium. Recent data revealed that T2-weighted (T2W) 3D-SPACE with variant flip-angle mode (VFAM) imaging allows fast and accurate evaluation of the hydrocephalus patients during both pre- and post-operative period for monitoring the treatment. For a better assessment of these patients; radiologists and neurosurgeons should be aware of the details and implications regarding to the 3D-SPACE technique, and they should follow the updates in this field. There could be a misconception about the difference between T2W-VFAM and routine heavily T2W 3D-SPACE images. T2W 3D-SPACE with VFAM imaging is only a subtype of 3D-SPACE technique. In this review, we described the details of T2W 3D-SPACE with VFAM imaging and comprehensively reviewed its recent applications. © 2017 Belgian Neurological Society

A membrana de Liliequist, de maneira simplista e sucinta, pode ser entendida como uma projeção formada pela aracnoide que se estende do dorso da sela turca aos corpos mamilares. Apesar de se tratar de uma estrutura anatômica bem conhecida pelos neurocirurgiões, muitos radiologistas não a conhecem ou não valorizam seu estudo. A avaliação por imagem desta membrana é factível e pode ser interessante para o melhor planejamento pré-operatório, avaliação pós-operatória de terceiro-ventriculostomias e entendimento dos cistos aracnoides suprasselares e hemorragias perimesencefálicas. Ilustramos neste artigo os aspectos anatômicos da membrana, sobretudo do ponto de vista de imagem, além de suas possíveis implicações clinicocirúrgicas. In a simplistic and succinct way, Liliequist membrane may be understood as a projection formed by an arachnoid membrane extending from the dorsum sellae to the mammillary bodies. In spite of being well known to neurosurgeons, many radiologists neither know this anatomical structure nor give importance to its study. The imaging evaluation of this membrane is feasible and may be interesting for a better preoperative planning; postoperative evaluation of third ventriculostomies; and understanding of suprasellar arachnoid cysts and perimesencephalic hemorrhage. The present article illustrates the anatomy of the membrane, with emphasis on imaging findings, besides describing its possible clinical and surgical implications.

Resumen Introduccion El abordaje endoscopico habitual para el manejo diagnostico y terapeutico de los tumores pineales es a traves del asta frontal del ventriculo lateral introduciendo el endoscopio por el agujero de Monro. Presentamos el caso de un paciente con esta patologia en el que se realizo toma de biopsia de la lesion a traves de la fisura coroidea mediante un acceso endoscopico al atrio ventricular. Caso clinico Paciente varon de 25 anos de edad que comienza con cefalea y diplopia. Al examen fisico se constata signo de Parinaud y papiledema. La resonancia magnetica demostro una lesion ocupante de la region pineal asociada a hidrocefalia. Inicialmente por via endoscopica habitual (acceso frontal) se trato la hidrocefalia y se tomo biopsia del tumor. El especimen tisular no fue util, por lo que se planifico un segundo procedimiento endoscopico para la toma de tejido tumoral a traves de la fisura coroidea introduciendo el endoscopio a traves del atrio. Conclusiones El abordaje a los tumores pineales a traves del atrio ventricular, mediante un agujero parietal, realizando apertura de la fisura coroidea permite la toma directa de biopsia, con una trayectoria endoscopica unica, sin necesidad de atravesar otras estructuras neurales.

The management of pineal cysts is still debatable, especially for asymptomatic incidental ones. For symptomatic cysts associated with hydrocephalus, the surgical management is mandatory and may include either classical microsurgical approaches to the pineal region or endoscopic trans-ventricular approaches in a minimally invasive philosophy. The authors expose a stepwise technique to treat a pineal cyst associated with an obstructive hydrocephalus in one procedure gathering a third ventriculostomy followed by an intraventricular marsupialisation of the pineal cyst. This endoscopic approach allows the treatment of the hydrocephalus and the pineal cyst in one short minimally invasive procedure.

Introduction Liliequist membrane (LM) is the most important anatomic structure for the success of endoscopic third ventriculostomy (ETV). Identification of this membrane is difficult with conventional MRI techniques. The purpose of this retrospective study is to determine the impact of threedimensional sampling perfection with application-optimized contrasts using different flip-angle evolutions (3D-SPACE) sequence with variant flip-angle mode (VFAM) in the assessment of LM at 3-T MRI devices. Methods 3D-SPACE with VFAMimages were obtained in 445 patients. LM visibility and integrity were scored as 0 (good), 1 (moderate), and 2 (poor) on these images for each parts (sellar, diencephalic, and mesencephalic) and overall of the membrane. Results According to the LMoverall integrity scores, 11%(48 cases) of the patients had perforated membrane. According to subsegmental integrity scores, sellar part was completely intact in 63 % of patients, diencephalic segment was completely intact in 60 % of the patients, and mesencephalic segment was completely intact in 95 % of the patients. Visibility scores of the third ventricle inferior wall were significantly higher in the patients with intact LM (p = 0.001). There was not any statistically significant relationship between LM pattern and overall integrity (p = 0.352). LM attachment sites could be detected easier in the patients who had better visibility of third ventricle inferior wall or intact LM (p < 0.001 for both). Conclusion 3D-SPACE technique is a useful alternative for the evaluation of morphology, integrity, individual variations, topographic relationships, and visibility of LM since it has some advantages including lower SAR values, fewer artifacts, and high-resolution images.

Object Endocrine dysfunction following endoscopic third ventriculostomy (ETV) is rare, but it has been reported. In the present study the authors sought to determine the histological nature of the floor of the third ventricle in hydrocephalic brains to better elucidate this potential association. Methods Five adult cadaveric brains with hydrocephalus were examined. Specifically, the floors of the third ventricle of these specimens were studied histologically. Age-matched controls without hydrocephalus were used for comparison. Results Although it was thinned in the hydrocephalic brains, the floor of the third ventricle had no significant difference between the numbers of neuronal cell bodies versus nonhydrocephalic brains. Conclusions Although uncommon following ETV, endocrine dysfunction has been reported. Based on the present study, this is most likely to be due to the injury of normal neuronal cell bodies found in this location, even in very thinned-out tissue.

Object The evaluation of third ventriculostomy function in hydrocephalic patients is challenging. The utility of the constant flow infusion test in predicting response to shunt insertion in normal-pressure hydrocephalus, as well as in identifying shunt malfunction, has been previously demonstrated. The object of this study was to evaluate its usefulness in determining whether a revision CSF diversion procedure was indicated in patients presenting with recurring symptoms and persisting ventriculomegaly after endoscopic third ventriculostomy (ETV). Methods The authors conducted a prospective study of all patients who, after undergoing ETV at their institution, presented postoperatively with recurring symptoms and persisting ventriculomegaly. Results Forty-six patients (mean age 40.7 years, including 11 patients younger than 18 years) underwent 56 constant flow ventricular infusion tests (VITs) at a mean of 24.7 months post-ETV. Thirty-three patients with resistance to CSF outflow (Rout) less than 13 mm Hg/ml/min underwent follow-up (median 17 months) and experienced resolution of symptoms. In 10 episodes Rout was greater than 13 mm Hg/ml/min; the patients in these cases underwent revisional CSF diversion. Two patients demonstrated high and frequent B (slow) waves despite a low Rout; these patients also underwent successful revisions. Patients who improved after surgery had increased B wave activity in the plateau phase of the VIT (p = 0.01). Thirty-four patients underwent MR imaging at the same time; 4 had high Rout despite evidence of flow across the stoma. These 4 patients underwent surgery and experienced resolution of symptoms. Of 9 patients without flow, Rout was less than 13 mm Hg/ml/min in 4; these patients were successfully treated conservatively. Conclusions The VIT is a useful and safe adjunct to clinical and MR imaging evaluation when ETV failure is suspected.

Hydrocephalus is a complex pathophysiology with disturbed cerebrospinal fluid (CSF) circulation. There are numerous numbers of classification trials published focusing on various criteria, such as associated anomalies/underlying lesions, CSF circulation/intracranial pressure patterns, clinical features, and other categories. However, no definitive classification exists comprehensively to cover the variety of these aspects. The new classification of hydrocephalus, “Multi-categorical Hydrocephalus Classification” (Mc HC), was invented and developed to cover the entire aspects of hydrocephalus with all considerable classification items and categories. Ten categories include “Mc HC” category I: onset (age, phase), II: cause, III: underlying lesion, IV: symptomatology, V: pathophysiology 1—CSF circulation, VI: pathophysiology 2—ICP dynamics, VII: chronology, VII: post-shunt, VIII: post-endoscopic third ventriculostomy, and X: others. From a 100-year search of publication related to the classification of hydrocephalus, 14 representative publications were reviewed and divided into the 10 categories. The Baumkuchen classification graph made from the round o’clock classification demonstrated the historical tendency of deviation to the categories in pathophysiology, either CSF or ICP dynamics. In the preliminary clinical application, it was concluded that “Mc HC” is extremely effective in expressing the individual state with various categories in the past and present condition or among the compatible cases of hydrocephalus along with the possible chronological change in the future.

This study evaluates the safety, efficacy, and indications for continuous lumbar drainage (CLD) in patients following endoscopic third ventriculostomy (ETV).We retrospectively reviewed the clinical data of 22 consecutive patients treated between 1996 and 2010 with CLD after ETV. The decision to insert a CLD was made in selected patients only. CLD was inserted in cases of high measured intracranial pressure (12 patients), clinical symptoms indicative of continuing hydrocephalus (2 patients), and "prophylactically" in 8 patients, based either on the clinical condition of patients before ETV or on technical difficulties during the ETV procedure, which seemed to increase the risk of ETV failure. CLD insertion took place either in the operating room immediately following the ETV procedure or under very specific conditions and with close patient monitoring in an ICU setting. Only four patients eventually required shunting, all within 1 month after ETV. Therefore, the overall ETV success rate was 81.8% (18/22 patients). Of the 14 patients suffering from measured or clinically observed continuing hydrocephalus, 12 (85%) ultimately recovered without the need for a permanent shunt. Without the CLD, some of these patients would probably have been declared "failures" and referred for a standard shunt. CLD provided a time window following ETV for the absorption system to recover and return to full functionality.Selective usage of CLD is a reasonable and safe method to gain time and possibly facilitates the recovery of absorption capacity following ETV. CLD should be considered before conceding a post-ETV patient as a failure.

The aim of this study was to assess the mid-term results, success rates, and time-to-failure of secondary endoscopic third ventriculostomy (secondary ETV), as well as the complex management of preoperative and postoperative cares. To this purpose, a retrospective analysis of a pediatric population of 22 children who underwent endoscopic third ventriculostomy (ETV) after shunt malfunction (secondary ETV) was performed. The failure rate, given by the percentage of new shunt replacement in the first 3 months after ETV, was 36%, with a mean time to failure of 14.3 days. All the failures were evident within 1 month after the ETV. Despite the small number of patients in our series, we found no significant correlation between ETV failure and both patient age and hydrocephalus etiology (p = 0.47 and p = 0.78, respectively). In our experience, ETV secondary to shunt malfunction in pediatric patients has a success rate of 64%. As it is a safe and rapid treatment option even in emergency conditions, it is worth performing this procedure in previously shunted children.

Chronic obstructive hydrocephalus is known to cause ventricular diverticula and, rarely, spontaneous ventriculostomy. The authors present the case of a patient in whom a spontaneous third ventriculostomy was identified with long-standing hydrocephalus secondary to aqueductal stenosis. To their knowledge, this is the first report in which a spontaneous stoma in the floor of the third ventricle was evaluated using endoscopy and cerebrospinal fluid dynamics studies. Both studies confirmed that the spontaneous stoma is similar in structure and function to surgical third ventriculostomy.