Neuroendoscopic transventricular transchoroidal approach for access to the posterior zone of the third ventricle or pineal region.

The endoscopic transventricular transchoroidal approach facilitates entry into the posterior part of the third ventricle, allowing a visualization field from the foramen of Monro to the pineal region through this anatomical corridor. Combined surgery to treat the target lesion and possible endoscopic third ventriculostomy (ETV) can be performed through a single burr hole. A detailed description of this surgical technique is given, and a series of cases from our center is presented. This retrospective study included patients with lesions in the pineal region or posterior zone of the third ventricle who underwent surgery between 2004 and 2022 in our center for tumor biopsy or endoscopic cyst fenestration. In nine cases, the transchoroidal approach was performed. Demographic and clinical variables were collected: sex, age at diagnosis, clinical presentation, characteristics of the lesion, pathological diagnosis, characteristics of the procedure, complications, subsequent treatments, evolution, follow-up time, and degree of success of the endoscopic procedure. The mean and range of the quantitative variables and frequency of the qualitative variables were analyzed, together with the statistical significance (p < 0.05). Surgical planning was carried out by performing a preoperative MRI, calculating the ideal entry point and trajectory for each case. The preoperative planning of the surgical technique is described in detail. Of our sample, 55.6% were women, with a mean age of 35 years (7-78). The most common clinical presentation was intracranial hypertension (55.6%), with or without a focus. Eight patients presented hydrocephalus at diagnosis. The most frequent procedure was endoscopic biopsy with ETV (66.7%). The pathological diagnosis varied widely. Procedure-related complications included one case of self-limited bleeding of the choroidal fissure at its opening and one intraventricular hemorrhage due to tumor bleeding in the postoperative period. Non-procedure-related complications comprised two ETV failures and one case of systemic infection, while late complications included one case of disease progression and one case of radionecrosis. Four patients died, one due to poor neurological evolution after post-surgical tumor bleeding and three due to causes unrelated to the procedure. The rest of the patients had a favorable evolution and were asymptomatic or stable. The transchoroidal approach through a single burr hole is a feasible and safe option for access to the posterior part of the third ventricle. Proper planning of each case is necessary to avoid complications.

Life-threatening idiopathic intracranial hypertension: the role of venous sinus stenting.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a clinical syndrome characterised by raised intracranial pressure with no discernible aetiology. It is relatively rare in children and its demographic features may differ from those of adults. The relationship between IIH and venous sinus stenosis (VSS) is well known. As VSS plays an important role in the pathophysiology, treatments have been developed aimed at improving venous blood outflow in refractory IIH. In the last two decades, venous sinus stenting has emerged as a treatment option in cases where stenosis is documented. METHODS AND RESULTS: The scientific literature on paediatric cases of IIH and its treatment with venous sinus stenting was analysed. We present the case of a 6-year-old girl with a life-threatening presentation of IIH, who was treated with transverse sinus stenting and a lumboperitoneal shunt. We summarise the characteristic of paediatric stenting cases reported and review the literature focusing on the main aspects of venous sinus stenting. CONCLUSION: VSS stenting could be a treatment tool for the acute presentation of IIH with severe symptoms and VSS plus an elevated trans-stenotic pressure gradient. However, in some cases, additional surgical treatment may be necessary.

Re-Do endoscopic third ventriculostomy. Retrospective analysis of 13 patients.

OBJETIVES: Indication for endoscopic third ventriculostomy (ETV) in the treatment for noncommunicating hydrocephalus is widely accepted. There is controversy regarding the indication of a second procedure (re-ETV) when the first has failed. The objective of this work is to revise ETV failures in a series in which re-ETV was performed and to describe the factors related to its prognosis. METHOD: Retrospective study of pediatric patients with ETV failure treated by re-ETV between 2003 and 2018. Gender, age in first and second ETV, time to failure of first ETV, etiology of hydrocephalus, previous presence of shunt, ETV-SS in the first and second ETV, intraoperative findings, success of the second procedure and follow-up were collected. The ETV-SS result was grouped into high (≥ 80), moderate (50-70) or low (≤ 40) scores. Endoscopic procedure failure was considered clinical worsening or the absence of radiological criteria for improvement (reduction in ventricular size or presence of ETV flow artifact in the floor of third ventricle). RESULTS: Of 97 ETV carried out in this period, 47 failures were registered, with 13 re-ETV performed. Of these, 8 were classified as successful (61.53%). Re-ETV was successful in 4/4 cases in which etiology was tectal tumor or aqueduct stenosis. In the group with a high ETV-SS score there was a higher rate of success (75%) than in the group with a moderate score (40%). 9 patients presented shunt prior to first ETV and in them, success was 66.6% compared to 50% in the group without prior shunt. All re-ETV were performed without complications. In 11 of the 13 procedures a closed stoma was found and the remaining 2 cases, we found a punctate opening. The mean follow-up after re-ETV was 61.23 months. CONCLUSION: The selection of patients for re-VET should be cautious. Factors such as age, etiology, and previous shunt (ETV-SS factors) have prognostic influence. However, there are specific factors which indicate favorable prognostic for re-VET such as a longer time to failure of the first procedure, the finding of a closed/punctate stoma or the loss of flow artifact in the follow-up MRI.

Analysis of complications in intraventricular neuroendoscopy in children: proposal for a standardization system.

Although intraventricular neuroendoscopy is considered a minimally invasive technique with good results, there is nevertheless a risk of developing certain complications. As no agreement apparently exists concerning the classification of these complications, we aim to propose a form of classification based on the results of our series, comparing them with recent publications. We undertook a retrospective study of 170 children who underwent intraventricular neuroendoscopy between 2003 and September 2020 at our center. Data were recorded on demographic and clinical variables: age, gender, presenting symptoms, etiology, number of procedures, type of procedure, and complications. Complications were divided into two main groups, intraoperative and postoperative, and in subgroups. The intraoperative complications included one group of systemic alterations and another group of surgical problems. The postoperative complications were divided into six groups: systemic, neurologic, hormone, fluid, hemorrhagic and death. A total of 202 neuroendoscopic procedures were performed in 170 children. The mean age at first surgery was 71 months (22-122). The most common etiology of the hydrocephalus was intraventricular tumors (32.9%), followed by aqueductal stenosis (13.5%). The most usual presenting sign was intracranial hypertension. The procedure most used was third ventriculostomy (62.9%). During the procedures, we experienced 5 surgical intraoperative complications (2.47% per procedure). In the postoperative period, there were 23.7% systemic complications per procedure, 12.87% neurologic, 8.41% hormone, 10.9% fluid, 0.5% hemorrhagic, and 0.99% for postoperative death. The rate of complications associated with intraventricular neuroendoscopy was similar in our series to those already published. Comparative studies require standardization for the analysis of neuroendoscopic complications.

Endoscopic third ventriculostomy in children with chronic communicating congenital hydrocephalus: a single-center cohort retrospective analysis.

OBJECTIVE: The aim of this study was to analyze the role of endoscopic third ventriculostomy (ETV) in the treatment of pediatric chronic communicating congenital hydrocephalus (CCCH). MATERIAL AND METHODS: This retrospective study comprised a series of 11 children with CCCH treated with ETV. Data were recorded on gender, history, presenting symptoms, age at surgery, complications during surgery, clinical evolution, ETV survival, and follow-up period. Radiological variables including ventricular and cephalic diameters were also recorded to determine a series of ventricular indexes in magnetic resonance imaging (MRI) before and after the ETV procedure. The procedure was considered to be successful when there was clinical stability or improvement accompanied by a reduction in the radiological indexes in the postoperative control images, such that there was no need to place an extrathecal cerebrospinal fluid shunt. RESULTS: Over a mean follow-up period of 35.8 months (range: 6-108 months) from the ETV procedure, three patients required shunt placement; one of these was due to early failure in an 8-month old girl, the only patient younger than 12 months in our series. The radiological indexes were reduced in all patients except for one of the cases of ETV failure. The mean ETV survival among the successful cases was 32.1 months (range: 6-108 months), whilst that of the failed cases was 16 months (range: 6-108 months). CONCLUSION: Although studies with larger sample sizes are needed, ETV appears to be a promising option for the treatment of this type of patient with CCCH.

Re-Do endoscopic third ventriculostomy. Retrospective analysis of 13 patients. / Re-ventriculostomía endoscópica de tercer ventrículo. Análisis retrospectivo de una serie de 13 casos.

OBJETIVES: Indication for endoscopic third ventriculostomy (ETV) in the treatment for noncommunicating hydrocephalus is widely accepted. There is controversy regarding the indication of a second procedure (re-ETV) when the first has failed. The objective of this work is to revise ETV failures in a series in which re-ETV was performed and to describe the factors related to its prognosis. METHOD: Retrospective study of pediatric patients with ETV failure treated by re-ETV between 2003 and 2018. Gender, age in first and second ETV, time to failure of first ETV, etiology of hydrocephalus, previous presence of shunt, ETV-SS in the first and second ETV, intraoperative findings, success of the second procedure and follow-up were collected. The ETV-SS result was grouped into high (≥80), moderate (50-70) or low (≤40) scores. Endoscopic procedure failure was considered clinical worsening or the absence of radiological criteria for improvement (reduction in ventricular size or presence of ETV flow artifact in the floor of third ventricle). RESULTS: Of 97 ETV carried out in this period, 47 failures were registered, with 13 re-ETV performed. Of these, 8 were classified as successful (61.53%). Re-ETV was successful in 4/4 cases in which etiology was tectal tumor or aqueduct stenosis. In the group with a high ETV-SS score there was a higher rate of success (75%) than in the group with a moderate score (40%). 9 patients presented shunt prior to first ETV and in them, success was 66.6% compared to 50% in the group without prior shunt. All re-ETV were performed without complications. In 11 of the 13 procedures a closed stoma was found and the remaining 2 cases, we found a punctate opening. The mean follow-up after re-ETV was 61.23 months. CONCLUSION: The selection of patients for re-VET should be cautious. Factors such as age, etiology, and previous shunt (ETV-SS factors) have prognostic influence. However, there are specific factors which indicate favorable prognostic for re-VET such as a longer time to failure of the first procedure, the finding of a closed/punctate stoma or the loss of flow artifact in the follow-up MRI.

Quality of life in children with hydrocephalus treated with endoscopic third ventriculostomy.

OBJECTIVE: The objectives of this study were to determine the quality of life of a pediatric cohort with hydrocephalus treated by endoscopic third ventriculostomy (ETV), using the Hydrocephalus Outcome Questionnaire-Spanish version (HOQ-Sv), and study the clinical and radiological factors associated with a better or worse functional status. METHODS: This cross-sectional study was undertaken between September 2018 and December 2019. It comprised a series of 40 patients ranging from 5 to 18 years old with hydrocephalus treated by ETV. ETV was considered to be successful if there was no need for surgery for the treatment of hydrocephalus after a minimum follow-up of 6 months. The clinical variables included gender, age at hydrocephalus diagnosis, age at the time of ETV, age at completion of the questionnaire, etiology and type of hydrocephalus (communicating or not), prior shunt, repeat ETV, number of neurosurgical procedures, number of epileptic seizures, presenting signs, and follow-up duration until last office revision. The radiological variables were the Evans Index and the pre- and posttreatment frontooccipital horn ratio. An analysis was conducted of the association between all these variables and the various dimensions on the HOQ-Sv, completed by the parents of the patients via telephone or in the outpatient offices. RESULTS: The mean age of the children at ETV was 7 years (range 7-194 months), and on completing the questionnaire was 12 years (range 60-216 months). The mean HOQ scores were as follows: overall 0.82, physical domain 0.86, social-emotional (SE) domain 0.84, cognitive domain 0.75, and utility score 0.90. A history of epileptic crises was a predictive factor for a worse score overall and in the SE and cognitive domains. Factors related to a worse score in the physical domain were a previous shunt, the number of procedures, and the etiology and type of hydrocephalus. The mean follow-up duration from ETV to the last office visit was 5 years (64.5 months). No association was found between the degree of ventricular reduction and the quality of life. CONCLUSIONS: The factors related to a worse score in the different dimensions of the HOQ were a history of epileptic seizures, the number of procedures, communicating hydrocephalus, and having had a previous valve. No association was found between the reduction in ventricular size and the quality of life as measured on the HOQ-Sv.

Neuroplasticity and Epilepsy Surgery in Brain Eloquent Areas: Case Report.

Introduction: Neuronal plasticity includes changes in any component of the central nervous system in response to intrinsic or extrinsic stimuli. Brain functions that depend on the epileptogenic cortex pose a challenge in epilepsy surgery because many patients are excluded from pre-surgical evaluation for fear of the possible sequelae. Some of these patients may be rescued by enhancing neuronal plasticity with brain neuromodulation techniques. Case Report: We describe a 6-year-old child with refractory focal motor seizures symptomatic to a neuroepithelial dysembryoblastic tumor in the left temporo-parietal region. He underwent limited resection of the lesion in order to avoid sequelae in his language function. A functional study at age of 17 years revealed an overlap of Wernicke's area with the tumor and areas of incipient language reorganization in the contralateral hemisphere. An invasive neuromodulation procedure was designed to enhance neuroplasticity. After craniotomy, he underwent language training and simultaneous electrical inhibition of language using an electrode grid placed over the lesion. The intensity of the language inhibitory stimulus was increased every day to force the use of accessory language areas in the right hemisphere by neuroplasticity. Results: The language of the patient improved for six consecutive days until he was able to speak and understand while undergoing maximum electrical inhibition. The tumor was resected using a cortical mapping guide. Discussion: Application of direct cortical stimulation techniques and language pre-habilitation before epilepsy surgery can be useful to rescue patients excluded from resective surgery, especially young patients with long-term lesions.

Neuroendoscopic treatment of arachnoid cysts in the paediatric population. Series results for 20 patients. / Tratamiento neuroendoscópico de quistes aracnoideos en edad pediátrica. Resultados de una serie de 20 pacientes.

INTRODUCTION: Neuroendoscopy has become an effective and safe treatment for arachnoid cysts in the paediatric population. We review the paediatric patients with arachnoid cysts treated by neuroendoscopy in our hospital and analyse the results. MATERIAL AND METHODS: A retrospective analysis of 20 patients operated on from 2005 to 2018. The variables assessed are: gender, age, clinical presentation, cyst site, presence of hydrocephalus and/or extra-axial collections, endoscopic procedures and complications. Procedure success is defined as an improvement in symptoms and reduction in cyst size until end of follow-up. RESULTS: Our series comprised 13 males and 7 females (mean age: 64.6 months, range: 4-172 months). The most frequent site was suprasellar-prepontine (7), followed by intraventricular (6), quadrigeminal (3), interhemispheric (2) and Sylvian (2). A total of 70% (14/20) of patients had hydrocephalus at diagnosis, which increased to 85% in suprasellar-prepontine cysts and 100% in quadrigeminal cysts. Only 4/14 patients with required a ventriculoperitoneal shunt (median age at diagnosis: 12.5 months). Of these 4 patients, 3 developed severe shunt overdrainage. The procedure was successful in 60% (12/20) of the patients in the series. Success by location was 57% (4/7) in suprasellar cysts, 33% (1/3) in quadrigeminal cysts, 66% (4/6) in intraventricular cysts, 100% (2/2) in interhemispheric cysts and 50% (1/2) in Sylvian cysts. Treatment thus failed in 8 cases, with a mean time to failure of 12.12 months (range: 0-45 months). A new neuroendoscopic procedure was performed in 4 of these 8 cases (success in 2/4), a ventriculoperitoneal shunt was placed in 2 cases, a cystoperitoneal shunt was placed in 1 case and the remaining case was managed conservatively. Mean follow-up time was 52.45 months (range: 3-129 months). CONCLUSIONS: Neuroendoscopy is an effective and safe treatment for arachnoid cysts in paediatric patients that also enables managing associated hydrocephalus in most cases. The choice of neuroendoscopic procedure and success rate depend on cyst location. Younger patients have been found to have a higher shunt dependency rate. In these cases, measures to prevent shunt overdrainage are recommended.

Neuroendoscopic biopsy: analysis of a series of 80 patients.

Neuroendoscopy enables diagnostic biopsy of intraventricular and/or paraventricular tumors and the simultaneous treatment of associated hydrocephalus in selected cases. The objective of this paper was to analyze the effectiveness and safety of this procedure. This retrospective study included 80 patients between 2 months and 78 years old diagnosed with intraventricular and/or paraventricular expansive lesion who underwent neuroendoscopic biopsy from 2004 to 2016. Collected variables were gender, age at diagnosis, clinical presentation, tumor location, surgical technique, management of hydrocephalus, pathological findings, procedure-related complications, and follow-up time. Neuroendoscopic biopsy was performed in 80 patients. Mean age at diagnosis was 27 years, and 52.5% were men. According to the Depreitere Classification, 71 were level I (conclusive diagnosis), 1 level III (problematic categorization), and 8 level IV (non-interpretable diagnosis). The most frequent diagnosis was grade I astrocytoma (14%). Diagnostic success per patient was 88.7%. Sixty-nine patients had hydrocephalus at diagnosis, 37 of whom were treated with endoscopic third ventriculostomy (ETV), with septostomy (SPT) in 14, and only SPT in 4. Twenty-eight patients underwent ventricular peritoneal shunt (VPS), with SPT in 20. The ETV success rate was 70.9%. The complication rate per patient was 11%: five patients presented intraventricular hemorrhage, three of whom died; one patient presented cerebrospinal fluid fistula; three presented transient oculomotor impairment. Postoperative follow-up was from 1 month to 12.4 years (mean 45 months). Neuroendoscopy is an effective procedure for the pathological diagnosis of intraventricular and paraventricular tumors, allowing the treatment of associated hydrocephalus. Nevertheless, it is not exempt from serious complications and requires proper training.

Neuroendoscopic treatment for hydrocephalus associated to midline arachnoid cysts in a series of nine pediatric patients.

BACKGROUND: Arachnoid cysts are extra-axial cerebrospinal fluid (CSF) collections surrounded by a membrane. Occasionally, hydrocephalus is associated due to a change in CSF circulatory dynamics. Neuroendoscopic treatment has been recommended for patients who develop symptoms resulting from the cyst location. METHODS: We retrospectively evaluate the results in our series of 9 patients with hydrocephalus associated to midline arachnoid cysts treated endoscopically. Success was rated on a scale of five degrees of neuroendoscopical success. RESULTS: We performed endoscopic third ventriculostomy (ETV) in three cases; ETV was associated to ventriculocystostomy (VC) in three cases; ETV, VC and septostomy (SPT) were performed in one patient; neuroendoscopic Monro foraminoplasty (NEFPMO) plus SPT were associated in one case; last patient was performed ETV, VC and cystocysternostomy (CC). For first procedures, 6 patients completed permanent Success (grade I). In one case success was transitory (grade II) and required a second procedure (ETV). In one patient VC success and ETV failure implied partial success (grade III). One patient's early failure (grade V) required a second procedure (ETV + NEFPMO). Success in second procedures was grade I in both patients. Follow-up period was over 12 months and altogether success was grade I in 8/9 patients and grade III in 1/9 patients. Shunt independency went over 88%. CONCLUSIONS: Endoscopy allows a solution avoiding the implantation of cerebrospinal fluid shunt devices. When possible, we likely approach both, hydrocephalus and arachnoid cyst, with different endoscopic maneuvers in a single procedure. It is important to expand the usage of success classifications for combined procedures.

[Arachnoid cysts of the central nervous system. Algorithms and recommendations for management]. / Quistes aracnoideos del sistema nervioso central. Algoritmos y recomendaciones generales de manejo.

The symptoms related to the presence of arachnoid cysts in the Central Nervous System depend on the size of the cyst and its growth rate, its location and, in some cases, the associated CSF dynamic disorder. Sometimes there is acute clinical presentation due to cyst rupture or acute bleeding. Although it is generally accepted that asymptomatic or paucisymptomatic cysts do not require surgical treatment, there is no consensus on the therapeutic approach of choice in symptomatic cases. The aim of this paper is to review the literature, analyzing the pros and cons of the three main surgical options (microsurgery, neuroendoscopy, and CSF shunt) based primarily on the location of the cyst. Although treatment must be always individualized, basic management recommendations may be offered.

[Microsurgical treatment of intracraneal arachnoid cysts]. / Tratamiento microquirúrgico de los quistes aracnoideos intracraneales.

Craniotomy and fenestration of membranes is one of the main treatment options for symptomatic arachnoid cysts. Open surgery advantages include, direct inspection of the cyst, biopsy sampling, fenestration in multilocular cysts and, in certain locations, cyst communication to basal cisterns. The aim of this paper is to review the advantages and disadvantages of this treatment modality for arachnoid cysts taking into account the different anatomical locations.

Prognosis of atypical teratoid rhabdoid tumors (AT/RT) treated with multimodal therapy protocols. Report of our series.

OBJECTIVE: Atypical teratoid rhabdoid tumors (AT/RT) of the central nervous system are rare, very aggressive embryological tumors, typically diagnosed in young patients and having a low survival rate after diagnosis. The aim of this study was to emphasize, based on the latest results in the literature, the need for protocols for multidisciplinary treatment in these patients. MATERIAL AND METHODS: We report our series of 3 cases treated, diagnosed and followed up between 2009 and 2014. They were treated with multimodal therapy protocols (Rhabdoid SIOP-2007 and European Rhabdoid Registry EU-RHAB-2010). In addition, we carried out a literature review. RESULTS: Two of our 3 cases (supratentorial and spinal tumors) did not show any progression of the disease after long follow-up, in contrast with most of the cases available in the literature. The second patient had a shorter survival. CONCLUSIONS: Patient age at the time of diagnosis, supratentorial location of the mass and fewer complications with adjuvant treatments seem to be factors yielding good prognosis for AT/RT tumors. In agreement with the latest international protocols, multidisciplinary treatment is the ideal treatment, consisting of radiotherapy and chemotherapy after complete tumor resection.

Ethmoidal meningoencephalocele and CSF leak after posthaemorrhagic ventricular dilatation in a newborn child.

BACKGROUND: Rhinoliquorrhoea suggests a communication between the subarachnoid space and the sinonasal tract. Clinical presentation includes clear nasal discharge, headache, pneumocephalus, meningitis or brain abscess. Cerebrospinal fluid (CSF) leaks are mostly of traumatic origin (skull base fractures), iatrogenic (secondary to endoscopic endonasal surgery) or associated with tumour aetiology. Occasionally, hydrocephalus has been the cause of rhinoliquorrhoea in adults, presumably secondary to the chronically raised intracranial pressure with skull base erosion and meningocele. To our knowledge, the association of hydrocephalus and ethmoid meningoencephalocele/CSF leak has not been previously reported in a newborn child. CASE PRESENTATION: We present the case of a 9-month-old girl who was referred for rhinorrhoea. She had a history of posthaemorrhagic ventricular dilatation. Brain computed tomography (CT) and magnetic resonance imaging (MRI) showed a left ethmoidal meningoencephalocele and small ventricular size. The meningoencephalocele was surgically repaired using an intradural subfrontal approach. During the postoperative period, after the transient lumbar drain was withdrawn, she developed symptomatic hydrocephalus. Ventriculoperitoneal shunting was required. CONCLUSION: Progressive ventricular dilatation may arise from a meningoencephalocele/CSF leak in paediatric patients. Early identification and repair of the meningoencephalocele are critical to avoid development of complications.

[Cranial fasciitis: a case report and review of the literature]. / Fascitis craneal: caso clínico y revisión de la literatura.

Cranial fasciitis is an uncommon, rapidly-growing, benign, non-tumoural, myofibroblastic lesion of the skull, found mainly among young children in their first year of life. It is histologically similar to nodular fasciitis and pseudosarcomatous fasciitis. It may mimic more aggressive pathologies, such as sarcomatosis or histiocytosis, due to its rapid, nodular growth in subcutaneous tissue. Complete resection is considered curative and, therefore, entails a low risk of metastases or malignant recurrences. We present the clinical, radiological and pathological findings in a 4-year-old boy with cranial fasciitis in the deep, subcutaneous, soft tissue, with erosion of the outer table of the cranium, which also produced periosteal reaction, while respecting the inner table and meninges. The objective of this article is to highlight the absence of radiotherapeutic or chemotherapeutic adjuvant treatment. In addition, an exhaustive review of the literature is also presented.