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Purpose: Selective dorsal rhizotomy (SDR) and intrathecal baclofen (ITB) pump placement are two surgical options in children affected by spasticity secondary to cerebral palsy 1 . The latest literature is an enlarging indication for SDR in case of ITB failure in non-ambulant patients as an alternative to pump reimplantation to reduce spasticity and facilitate patients' care., Methods: A retrospective single-center study has screened all children diagnosed with spastic tetraparesis who underwent in the last 10 years SDR and had previously ITB pump implanted. A cohort of six patients was pooled out. Furthermore, pertinent literature has been reviewed., Results: Indication for pump removal was pump pocket infection, parents' decision, and poor response to ITB. Patients' amount of lifetime with the pump implanted has been 6.9 years. The preoperative and postoperative Ashworth scores were statistically different in both procedures (p = 0.005 and p = 0.,02)., Conclusions: Only two studies investigated the pediatric population undergoing SDR in the occurrence of ITB pump removal. Authors are offering SDR to all children, regardless of GMFCS groups and previous ITB pump placement. In conclusion, SDR represents a valid tool in neurosurgeon's hands to help ameliorate patients' long-lasting quality of life, reducing the severity of the spasticity and leading to better management by caregivers., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: The perivascular spaces of the brain are also known as Virchow-Robin spaces (VRSs). Dilated Virchow-Robin spaces in the brainstem are rare and mainly cause symptoms due to obstructive hydrocephalus, less frequently because of their size, mass effect, and impact on eloquent structures., Case Illustration: We present a patient with giant tumefactive VRS with hydrocephalus and neurological symptoms who was treated with endoscopic third ventriculostomy (ETV) followed by microscopic cyst fenestration. On the basis of this observation, we performed a thorough review of the literature to evaluate different treatment options., Results: An 11-year-old girl presented with a headache for 3 months. The patient had a giant tumefactive mesencephalothalamic VRS with triventricular hydrocephalus. She was initially treated with endoscopic third ventriculostomy and multiple cyst fenestration. Symptomatic cyst regrowth required multiple cyst fenestrations via transcallosal transchoroidal (N = 2) and subtemporal approaches (N = 1) at the 2- and 4-year follow-ups. A literature review of these conditions allowed the detection of 12 cases (including our index case), and only 25% (3/12) of the patients underwent cyst fenestration 16.7% (2/12) required endoscopic fenestration and 8.3% (1/12) required microscopic fenestration., Conclusion: Giant mesencephalothalamic dVRSs are rare in the pediatric population. These patients are usually symptomatic due to obstructive hydrocephalus. Surgical options are endoscopic third ventriculostomy, ventricular shunt procedures, or direct cyst fenestration (microscopic or endoscopic). Close follow-up is mandatory owing to the risk of progression of the disease. Cyst fenestration resolves symptoms immediately, as it addresses both hydrocephalus and mass effects due to the cystic lesion in the same setting., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Craniosynostosis (CRS) is a rare congenital cranial malformation in which 1 or more cranial or facial sutures are fused in utero or rapidly fused in early infancy. The cranial sutures separate the skull bone plates and enable rapid growth of the skull in the first 2 years of life, in which growth is largely dictated by growth of the brain. CRS is a rare disease that occurs in 1 in 2100 to 1 in 2500 births and may be either nonsyndromic (also referred to as isolated) or syndromic. In syndromic CRS, other birth defects are present next to the CRS. The distinction between nonsyndromic and syndromic manifestations is made on the basis of dysmorphologic evaluation and genetic evaluation. Owing to advances in genetic diagnostics, nonsyndromic patients are increasingly recognized as syndromic patients. CRS treatment is almost entirely surgical and is sometimes paired with postoperative helmet therapy for maintenance. Corrective procedures are complex, long, and associated with the risk of numerous complications, including heavy blood loss and its sequelae. Although surgery may restore a normal appearance, even in nonsyndromic patients, patients may experience persistent deficits in intellectual ability and cognitive function. The European Commission (EC) has prioritized rare diseases in recent horizon European research programs; indeed, collections or even individual samples may be extremely valuable for research., Methods and Results: Here, we present a study protocol in which the combined expertise of clinicians and researchers will be exploited to generate a biobank dedicated to CRS. The generation of the CRS biobank presented in this study will include the collection of different types of biological materials as well as advanced radiological images available to the scientific community., Conclusion: The activation of a CRS biobank will provide an opportunity to improve translational research on CRS and to share its benefits with the scientific community and patients and their families., (© 2024. The Author(s).)

Pallister-Killian syndrome (PKS; OMIM #601803) is a rare genetic disorder typically characterized by developmental delay, seizures, sparse temporal hair, and facial dysmorphisms. PKS is most frequently caused by mosaic supernumerary isochromosome 12p. Here, we report a 27-month-old girl with a prenatal diagnosis of PKS and a histopathological diagnosis of pineocytoma., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To measure the size of jugular foramina in infants affected by external hydrocephalus (EH) and in a control group, to support the hypothesis that a jugular foramen (JF) stenosis may determine dural venous sinus alterations and increased venous outflow resistance as main pathophysiological factor., Methods: Minimum, maximum, and mean values of JF areas were measured in a series of phase-contrast magnetic resonance venous angiography (angio MRV PCA3D) performed on 81 infants affected by EH. Results were compared with a group of 54 controls., Results: Smaller JF area was significantly smaller in patients versus controls (43.1 ± 14.6 vs. 52.7 ± 17.8; p < 0.001) resulting in a significantly smaller mean JF areas in patients vs. controls (51.6 ± 15.8 vs. 57.0 ± 18.3; p = 0.043). In patients, smaller JF areas were significantly associated with higher venous obstruction grading score (VOGS) both on the right (p = 0.018) and on the left side (p = 0.005). Positional plagiocephaly (cranial vault asymmetry index > 3.5%) was more frequent among EH patients than controls (38/17) but the difference was not significant (p = 0.07). In the 38 plagiocephalic patients, JF area was smaller on the flattened side than the contralateral in a significant number of cases both in right (21/7) and left (9/1) plagiocephaly (p < 0.0005) as well as the mean area (48.2 + 16.4 mm 2 vs. 57.5 + 20.7 mm 2 , p = 0.002) and VOGS was significantly higher on the plagiocephalic side than on the contralateral side (1.6 ± 1.1 vs. 1.1 ± 0.9, p = 0.019)., Conclusion: In this series of infants affected by EH, the mean size of the ostium of both JF resulted significantly smaller than controls. JF stenosis was significantly associated with higher degrees of venous obstruction on both sides, suggesting a direct extrinsic effect of JF size on dural sinus lumen and possible consequent effect on venous outflow resistance. Positional plagiocephaly, when present, was associated with a decreased JF area and increased VOGS on the flattened side., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background and Objectives: Robot-assisted biopsies have gained popularity in the last years. Most robotic procedures are performed with a floor-based robotic arm. Recently, Medtronic Stealth Autoguide, a miniaturized robotic arm that work together with an optical neuronavigation system, was launched. Its application in pediatric cases is relatively unexplored. In this study, we retrospectively report our experience using the Stealth Autoguide, for frameless stereotactic biopsies in pediatric patients., Methods: Pediatric patients who underwent stereotactic biopsy using the Stealth Autoguide cranial robotic platform from July 2020 to May 2023 were included in this study. Clinical, neuroradiological, surgical, and histological data were collected and analyzed., Results: Nineteen patients underwent 20 procedures (mean age was 9-year-old, range 1-17). In four patients, biopsy was part of a more complex surgical procedure (laser interstitial thermal therapy - LITT). The most common indication was diffuse intrinsic brain stem tumor, followed by diffuse supratentorial tumor. Nine procedures were performed in prone position, eight in supine position, and three in lateral position. Facial surface registration was adopted in six procedures, skull-fixed fiducials in 14. The biopsy diagnostic tissue acquisition rate was 100% in the patients who underwent only biopsy, while in the biopsy/LITT group, one case was not diagnostic. No patients developed clinically relevant postoperative complications., Conclusion: The Stealth Autoguide system has proven to be safe, diagnostic, and highly accurate in performing stereotactic biopsies for both supratentorial and infratentorial lesions in the pediatric population., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: COVID-19 pandemic is thought to have changed the epidemiology of some pediatric neurosurgical disease: among them are the intracranial complications of sinusitis and otitis (ICSO). According to some studies on a limited number of cases, both streptococci-related sinusitis and ICSO would have increased immediately after the pandemic, although the reason is not clear yet (seasonal changes versus pandemic-related effects). The goal of the present survey of the European Society for Pediatric Neurosurgery (ESPN) was to collect a large number of cases from different European countries encompassing the pre-COVID (2017-2019), COVID (2020-2021), and post-COVID period (2022-June 2023) looking for possible epidemiological and/or clinical changes., Material and Methods: An English language questionnaire was sent to ESPN members about year of the event, patient's age and gender, presence of immune-deficit or other favoring risk factors, COVID infection, signs and symptoms at onset, site of primary infection, type of intracranial complication, identified germ, type and number of surgical operations, type and duration of medical treatment, clinical and radiological outcome, duration of the follow-up., Results: Two hundred fifty-four cases were collected by 30 centers coming from 14 different European countries. There was a statistically significant difference between the post-COVID period (129 children, 86 cases/year, 50.7% of the whole series) and the COVID (40 children, 20 cases/year, 15.7%) or the pre-COVID period (85 children, 28.3 cases/year, 33.5%). Other significant differences concerned the presence of predisposing factors/concurrent diseases (higher in the pre-COVID period) and previous COVID infection (higher in the post-COVID period). No relevant differences occurred as far as demographic, microbiological, clinical, radiological, outcome, morbidity, and mortality data were concerned. Paranasal sinuses and middle ear/mastoid were the most involved primary site of infection (71% and 27%, respectively), while extradural or subdural empyema and brain abscess were the most common ICSO (73% and 17%, respectively). Surgery was required in 95% of cases (neurosurgical and ENT procedure in 71% and 62% of cases, respectively) while antibiotics in 99% of cases. After a 12.4-month follow-up, a full clinical and radiological recovery was obtained in 85% and 84% of cases, respectively. The mortality rate was 2.7%., Conclusions: These results suggest that the occurrence of ICSO was significantly increased after the pandemic. Such an increase seems to be related to the indirect effects of the pandemic (e.g., immunity debt) rather than to a direct effect of COVID infection or to seasonal fluctuations. ICSO remain challenging diseases but the pandemic did not affect the management strategies nor their prognosis. The epidemiological change of sinusitis/otitis and ICSO should alert about the appropriate follow-up of children with sinusitis/otitis., (© 2024. The Author(s).)

Background: Colloid cysts are benign tumors usually located on the roof of the third ventricle. Cyst removal is the treatment of choice. It can be accomplished microsurgically through a transcortical- or transcallosal approach, or endoscopically. There is a lack of consensus regarding the best strategy for cyst removal. One of the challenges of the traditional endoscopic technique is dealing with the cyst content density. Hyperdensity on computed tomography scan and low signal on T2-weighted magnetic resonance imaging (MRI) cyst are correlated with high viscosity cystic content., Case Reports: We present a case of a colloid cyst of the third ventricle in a 15-year-old boy removed through a pure endoscopic transventricular approach. The cyst presented a low signal on T2 MRI; nevertheless, it was easily removed with the help of an endoscopic ultrasonic aspirator., Discussion and Conclusion: The colloid cyst of the third ventricle can be safely treated by a purely endoscopic approach. The rationale of the use of the ultrasonic aspirator relies on the facilitation of aspiration of the content even when the consistency is extremely firm., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Tumors of the choroid plexus of the third ventricle are uncommon. Surgical excision is technically challenging because of the rich vascularisation, central location, and high incidence in young children. Open microsurgical resection is considered the standard treatment. However, attempts at purely endoscopic removal of choroid plexus tumors of the third ventricle have also been made in the past, with encouraging results., Case Reports: We report our experience with endoscopic ultrasonic removal of two cases of tumors of the third ventricular choroid plexus. The first case was a large atypical choroid plexus papilloma (WHO grade 2) in the anterior third ventricle associated with hydrocephalus; the second case was a smaller choroid plexus papilloma (WHO grade 1) in the middle/posterior third ventricle without overt hydrocephalus requiring a more anterior neuronavigation guided approach., Discussion and Conclusion: Choroid plexus papillomas of the third ventricle can be safely treated by a purely endoscopic approach because they are usually smaller than their counterparts in the lateral ventricle and often have a recognizable vascular pedicle. Early detection and control of the vascular pedicle at the choroidal border is key to success. The use of ultrasonic aspirator facilitates and expedites endoscopic access. By alternating surface coagulation with fragmentation and aspiration with the ultrasonic aspirator, the tumor can be removed without difficult dissection maneuvers., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

During the last 30 years, the neurosurgeons have witnessed a revolution in the practice of interventricular surgery. The advent of neuroendoscopy at the end of the 1980s has allowed a minimally invasive management of a very large series of pathologies in pediatric neurosurgery ranging from hydrocephalus to arachnoid cyst to intraventricular tumors. The progresses in the management of hydrocephalus, intracranial cyst, and the fluid filled collection nevertheless has been more rapid and radical due to the simpler equipment that is necessary to perform this kind of surgery. The intraventricular tumors instead have been addressed in a slower way, and for many years, the only endoscopic procedure that was allowed on interventricular tumors was a biopsy associated with the management of hydrocephalus. Only very small tumors have been considered operable for complete removal during many years due to the limitations of the neuroendoscopic equipment and to the small calibers of the working channel. More recently, the advent of new devices and new surgical techniques are offering new perspectives on the possibility of intraventricular tumor surgery in children. In this review, we describe the historical perspective of the learning curve of intraventricular tumor surgery under neuroendoscopic control and try to offer a view of the future perspective in the removal of larger intraventricular tumors, analyzing the main indications for intraventricular endoscopic tumor surgery. We offer as well an historical perspective of the evolution of skull base surgery and endonasal transsphenoidal approach for skull-based tumors in children. This kind of surgery that has acquired widespread acceptance for many pathologies in adult age has diffused more slowly in pediatric neurosurgery due to the anatomical limitation observed in these age range. Also in this field, the slow evolution of the technique and of the technology available to neurosurgeons has allowed a very significant expansion of indication for the minimally invasive removal of skull base tumors in children., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Meningiomas are relatively rare in children and tend to be intraventricular and cystic, with often malignant behavior. Complete excision is associated with the most favorable outcome; moreover, the size and extent of these lesions often make complete excision in one step impossible because of the risk of intraoperative death from uncontrollable hemorrhage., Case Presentation: A 10-year-old girl was admitted for headache in the last 3 months and was found to have a giant left intraventricular lesion with a volume of 166.63 cm 3 , which caused hydrocephalus and significant mass effect. Very large draining veins were evident within the tumor, draining into the thalamostriates and internal cerebral veins. Cerebral angiography showed multiple feeders originating mainly from branches of the posterior left choroidal artery with distal afferents that could not be embolized. Therefore, a left parietal transcortical approach was chosen. Given the vascularity of the tumor, saline-cooled radiofrequency coagulation (Aquamantys ® ) was used to reduce blood loss intraoperatively. Gross total resection (GTR) was achieved with an estimated blood loss of 640 mL. Pathology analysis was consistent with WHO grade 1 transitional meningioma. Postoperatively, the patient was neurologically intact, and MRI confirmed complete resection., Conclusion: Aquamantys ® is a novel bipolar coagulation device that employs a new bipolar coagulation technique combining radiofrequency energy and saline to achieve hemostatic sealing by denaturing collagen fibers. This offers the possibility of achieving adequate hemostasis even in giant intraventricular tumors in infants to obtain GTR resection with minimal blood loss., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Dandy-Walker malformation (DWM) is a posterior fossa malformation characterized by a huge posterior fossa cyst in communication with the fourth ventricle. Hydrocephalus is associated with more than 80% of cases and is usually treated by shunting. Despite infection being a common complication of the shunt, abscess formation within the cyst was reported only once., Case Report: A neonate affected by DWM developed a posterior fossa abscess following a shunt infection. The purulent collection was refractory to standard treatment (antibiotics and burr hole drainage); therefore, an endoscopic approach was performed in order to remove the purulent collection under direct vision. This material was aspirated with the help of an endoscopic ultrasonic aspirator. The outcome was favorable, with a resolution of infection and re-implantation of the ventriculo-peritoneal shunt. Surprisingly, post-operative radiological examination showed substantial modification of the anatomy of the posterior fossa with disappearing of the Dandy-Walker cyst. To the best of our knowledge, this is the first documented report of a true Dandy-Walker malformation that modified its anatomical appearance over time., Discussion and Conclusion: Endoscopic aspiration of intracranial purulent collection should be considered a valid option to manage complicated cases. An endoscopic ultrasonic aspirator may make the procedure more effective and faster., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Optic pathway gliomas (OPGs) presenting with acute hydrocephalus represent a true neurosurgical and neurooncological challenge. Two main strategies are currently used: microsurgical removal of the tumor, restoring CSF pathways, and endoscopic biopsy associated with ventriculo-peritoneal shunt implantation. Since the availability of an ultrasonic aspirator handpiece, that can be used inside the working channel of a neuroendoscope, a different less invasive surgical strategy can be used., Methods: Four pediatric patients were treated by endoscopic ultrasonic aspiration, in order to remove the upper pole of the tumor, obtaining tissue for diagnosis and restoring CSF pathways as initial treatment of OPG invading the third ventricle and causing hydrocephalus due to simultaneous blockage of both Monro foramina and of the Sylvian aqueduct. Surgical technique is described. Pre-operative and post-operative volumes were calculated on magnetic resonance imaging., Results: In all cases, the surgical procedure was uneventful, the biopsy was diagnostic, and CSF pathways were restored. The amount of tumor removed ranged between 31 and 76%. All patients underwent oncological treatment of their tumors. One patient received V-P shunt only 1 year after endoscopic decompression due to tumor progression., Conclusion: Our preliminary results show that the use of endoscopic cavitron aspirator is safe and effective to obtain CSF flow restoration and tumor biopsy (for histological and molecular purposes), avoiding CSF shunt implant in the acute phase and offering the chance to obtain a consistent tumor debulking in a minimally invasive fashion. Neuroendoscopic approach together with modern target therapy offers the opportunity to avoid or delay major surgery., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Cerebrospinal fluid (CSF) diversion for the treatment of hydrocephalus is one of the most common neurosurgical procedures. Over the years, the development of the neuronavigation system has allowed the surgeon to be guided in real time during the procedures. Nevertheless, to date, the revision rate remains as high as 30-40%. The aim of this study was to investigate the role of intraoperative image guidance in the prevention of shunt failure. We herein report the first literature meta-analysis of image guidance and shunt revision rate in the pediatric population., Methods: Principal online databases were searched for English-language articles published between January, 1980, and December, 2021. Analysis was limited to articles that included patients younger than 18 years of age at the time of primary V-P shunt. Articles reporting combined results of free-hand and image-guided placement of ventricular catheter (VC) were included. The main outcome measure of the study was the revision rate in relation to the intraoperative tools. Secondary variables collected were the age of the patient and ventricle size. Statistical analyses and meta-analysis plots were done via R and RStudio. Heterogeneity was formally assessed using Q, I 2 , and τ 2 statistics. To examine publication bias was performed a funnel plot analysis., Result: A total of 9 studies involving 2017 pediatric patients were included in the meta-analysis. 55.9% of procedures were carried out with the aid of intraoperative tools, while 44.1% procedures were conducted free hand. The intraoperative tools used were ultrasound (9.1%), electromagnetic neuronavigation (21.07%), endoscope (67.32%), and combined images (2.4%).The image-guided placement of VC was not statistically associated with a lower revision rate. The pooled OR was 0.97 [CI 95% 0.88-1.07] with an I 2 statistics of 34%, t 2 of 0.018 and a p-value of 0.15 at heterogeneity analysis., Conclusion: Our analysis suggest images guidance during VC shunt placement does not statistically affect shunt survival. Nevertheless, intraoperative tools can support the surgeon especially in patients with difficult anatomy, slit ventricles or complex loculated hydrocephalus., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Infantile myofibromatosis is a rare and nonmalignant pediatric tumor of myofibroblastic origin that may occur in solitary or multifocal forms. Soft tissue of the head and neck, trunk, and extremities, skeleton, and viscera are usually involved. Intracranial involvement is reported to be extremely rare, and its clinical picture has been poorly characterized. We present two cases of giant infantile myofibromatosis of the skull base with intracranial involvement. The first case with prenatal diagnosis involved extensively the extradural space of the occipital region and was previously treated by chemotherapy for a previous diagnosis of hemangioperycitoma. Tumor was removed at the age of 5 months and no recurrence was observed during the 3-year follow-up. The second case in a 2-year-old baby involved the anterior cranial base, the nasal cavity, the right orbit, and presented massive involvement of the anterior cranial fossa. Surgery allowed complete removal and a recurrence-free period of 7 years after surgery. Treatment options for these unusual cases are presented and details of histological diagnosis are discussed., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Medulloblastoma (MB) is the most common malignant pediatric brain tumor, accounting for 40% of childhood tumors in posterior fossa. Metastatic disease, occurring in 20-30% of all medulloblastoma cases at diagnosis, is largely exclusive to the leptomeninges. On the contrary, primary leptomeningeal medulloblastoma or so-called chameleon medulloblastoma, defined by the absence of a detectable intraparenchymal lesion with a widespread diffusion along leptomeninges, is a rare entity of difficult diagnosis with only a few cases reported in literature., Methods and Results: A comprehensive literature search of three databases (PubMed, Ovid Medline, and Ovid Embase) have been conducted to identify pertinent papers focusing on the diagnostic process, management, and treatment of primary leptomeningeal medulloblastoma and its peculiar features. To our knowledge, only eight cases are described in literature, including five pediatric patients and three adults, two of which with the initial involvement of the spinal cord. In addition, we report another two pediatric cases, showing widespread primary diffusion along leptomeninges of brain and spinal cord. Finally, we analyze in-depth the peculiar morphological MRI features of this tumor., Conclusion: The classification and treatment of medulloblastomas are likely to change in the coming years due to new insights into the molecular biology of medulloblastoma. Primary leptomeningeal medulloblastoma could represent another potential challenge for biologists to start exploring the underlying mechanisms of this different clinical and pathological entity, with different implications for diagnosis and its management., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Neurological manifestations, such as encephalitis, meningitis, ischemic, and hemorrhagic strokes, are reported with increasing frequency in patients affected by Coronavirus disease 2019 (COVID-19). In children, acute ischemic stroke is usually multifactorial: viral infection is an important precipitating factor for stroke. We present a case of a child with serological evidence of SARS-CoV-2 infection whose onset was a massive right cerebral artery ischemia that led to a malignant cerebral infarction. The patient underwent a life-saving decompressive hemicraniectomy, with good functional recovery, except for residual hemiplegia. During rehabilitation, the patient also developed a lower extremity peripheral nerve neuropathy, likely related to a long-Covid syndrome., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: The TROPHY registry has been established to conduct an international multicenter prospective data collection on the surgical management of neonatal intraventricular hemorrhage (IVH)-related hydrocephalus to possibly contribute to future guidelines. The registry allows comparing the techniques established to treat hydrocephalus, such as external ventricular drainage (EVD), ventricular access device (VAD), ventricular subgaleal shunt (VSGS), and neuroendoscopic lavage (NEL). This first status report of the registry presents the results of the standard of care survey of participating centers assessed upon online registration., Methods: On the standard of treatment forms, each center indicated the institutional protocol of interventions performed for neonatal post-hemorrhagic hydrocephalus (nPHH) for a time period of 2 years (Y1 and Y2) before starting the active participation in the registry. In addition, the amount of patients enrolled so far and allocated to a treatment approach are reported., Results: According to the standard of treatment forms completed by 56 registered centers, fewer EVDs (Y1 55% Y2 46%) were used while more centers have implemented NEL (Y1 39%; Y2 52%) to treat nPHH. VAD (Y1 66%; Y2 66%) and VSGS (Y1 42%; Y2 41%) were used at a consistent rate during the 2 years. The majority of the centers used at least two different techniques to treat nPHH (43%), while 27% used only one technique, 21% used three, and 7% used even four different techniques. Patient data of 110 infants treated surgically between 9/2018 and 2/2021 (13% EVD, 15% VAD, 30% VSGS, and 43% NEL) were contributed by 29 centers., Conclusions: Our results emphasize the varying strategies used for the treatment of nPHH. The international TROPHY registry has entered into a phase of growing patient recruitment. Further evaluation will be performed and published according to the registry protocol., (© 2021. The Author(s).)

Purpose: To evaluate the anatomical variations of dural venous sinuses in children with external hydrocephalus, proposing a radiological grading of progressive anatomic restriction to venous outflow based on brain phase-contrast magnetic resonance venography (PC-MRV); to evaluate the correlation between positional plagiocephaly and dural sinuses patency; and to compare these findings with a control group to ascertain the role of anatomical restriction to venous outflow in the pathophysiology of external hydrocephalus., Methods: Brain MRI and PC MRV were performed in 97 children (76 males, 21 females) diagnosed with external hydrocephalus at an average age of 8.22 months. Reduction of patency of the dural sinuses was graded as 1 (stenosis), 2 (complete stop) and 3 (complete agenesis) for each transverse/sigmoid sinus and sagittal sinus. Anatomical restriction was graded for each patient from 0 (symmetric anatomy of patent dural sinuses) through 6 (bilateral agenesis of both transverse sinuses). Ventricular and subarachnoid spaces were measured above the intercommissural plane using segmentation software. Positional plagiocephaly (PP) and/or asymmetric tentorial insertion (ATI) was correlated with the presence and grading of venous sinus obstruction. These results were compared with a retrospective control group of 75 patients (35 males, 40 females)., Results: Both the rate (84.53% vs 25.33%) and the grading (mean 2.59 vs mean 0.45) of anomalies of dural sinuses were significantly higher in case group than in control group. In the case group, sinus anomalies were asymmetric in 59 cases (right-left ratio 1/1) and symmetric in 22. A significant association was detected between the grading of venous drainage alterations and diagnosis of disease and between the severity of vascular anomalies and the widening of subarachnoid space (SAS). Postural plagiocephaly (39.1% vs 21.3%) and asymmetric tentorial insertion (35.4% vs 17.3%) were significantly more frequent in the case group than in the control group. When sinus anomalies occurred in plagiocephalic children, the obstruction grading was significantly higher on the flattened side (p ≤ 0.001)., Conclusion: Decreased patency of the dural sinuses and consequent increased venous outflow resistance may play a role in the pathophysiology of external hydrocephalus in the first 3 years of life. In plagiocephalic children, calvarial flattening may impact on the homolateral dural sinus patency, with a possible effect on the anatomy of dural sinuses and venous drainage in the first months of life., (© 2021. The Author(s).)

Background: Desmoplastic infantile astrocytomas and gangliogliomas (DIA/DIG) usually present with a large size, large cystic component, large dural implant, encasement of big vessels, clinical presentation within 18 months of life, high incidence of seizures and overall good prognosis, even if tumour surgery can be very challenging at first procedure., Methods: We retrospectively reviewed clinical and radiological data of patients diagnosed with desmoplastic infantile tumours who were surgically treated between 2008 and 2019., Results: The series included 12 patients. The median age at surgery was 91 days. The average tumour volume was 212 cm 3 . Cystic components were predominant ranging from 0 to 295 cm 3 . Active hydrocephalus was pre-operatively evident in 5 cases. Eight patients (66.6%) received total or subtotal removal, three of them (25%) underwent partial removal, and one patient (8.3%) received a biopsy. One patient died within 24 h after surgery due to severe hypotension, as a consequence of significant intraoperative blood loss. Overall, seven (58.3%) patients were reoperated on the tumour after the first procedure: 4 patients were operated twice; 3 patients were operated 3 times. Two patients presented remote localizations and underwent chemotherapy. At last follow-up, 7 patients were tumour-free, 2 are alive with stable disease, and 2 are alive with progressive disease (leptomeningeal seeding)., Conclusion: Desmoplastic infantile tumours are rare giant neonatal tumours. Total removal is the goal of treatment, but prognosis remains good even if total removal is not achieved. In case of tumour progression or epilepsy from residual tumour, reoperation is the first option, with chemotherapy reserved to unresectable or disseminated cases with mixed results, while, to date, radiotherapy still plays no role.

Background: Endoscopic aqueductoplasty with aqueductal stenting is an effective surgical procedure for the treatment of isolated fourth ventricle (IFV). Due to the rarity of the underlying pathology, it can be considered a rare procedure that can be performed with different surgical techniques and approaches., Objectives: To assess long-term functioning of permanent aqueductal stents implanted in children affected by hydrocephalus and IFV and to describe some variations of the same procedure., Methods: We reviewed retrospectively all the patients presenting at our institution in the years 1999-2019 for symptoms of isolated fourth ventricle who underwent a surgical procedure of endoscopic aqueductoplasty and/or aqueductal stent. Surgical reports, radiological images, and surgical videos were retrospectively analyzed., Results: Thirty-three patients with symptomatic isolated fourth ventricle (IFV) underwent fifty (50) neuroendoscopic procedures in the period observed. The median age of the patients at the time of first surgery was 7 months, with 22 premature babies. In twenty-nine patients (87.8%), a precoronal approach was performed, while four patients received a suboccipital burr hole. Ten patients were never reoperated since. Twenty-three patients underwent further surgeries: a new aqueductoplasty with aqueductal stent was performed in 13 cases. The remaining 10 patients required a combination of other procedures for management of hydrocephalus. Long-term follow-up showed a permanent stent functioning rate of 87% at 2 years and 73% at 4 years, remaining stable afterwards at very long term (20 years)., Conclusion: Endoscopic aqueductoplasty and stenting is a reliable procedure in the long-term management of isolated fourth ventricle.

Introduction: Neuroendoscopic biopsy represents the procedure of choice for pure intraventricular lesions. Instead, in case of deep-seated paraventricular tumors, with intact ependyma, the advantage of neuroendoscopy over stereotactic biopsy is not so evident, because the lesion is not under direct vision; the tissue sample may be limited to more superficial ependymal layer, and bleeding may obscurate vision. Also, stereotactic biopsy may reserve additional problems for these lesions: inaccuracy caused by leak of cerebrospinal fluid and increased risk of severe hemorrhage due to damage of the ependymal vessels., Case Reports: We report two cases of young children affected by thalamic tumors that were biopsied using a modification of a recently proposed technique: endoscopic visual control, neuronavigated needle biopsy., Conclusion: This technique may combine the accuracy of a stereotactic needle biopsy with the advantage of visual control on site of ependymal puncture and possibility of immediate bleeding control.

Lumbar pseudomeningocele is a common complication of spinal surgery. It is caused by dural defect, and it consists of extradural collection of cerebrospinal fluid communicating with subarachnoid space. Usually it is small, asymptomatic, and self-limiting. In case of persistence, common treatments include bedrest, puncture and compressive dressing, subcutaneous or subarachnoid lumbar drainage, surgical re-exploration for durotomy closure, and blood patch. Fast-resorbing polymer mesh has been recently used for closure osseous gap in adult patients who developed giant pseudomeningocele following laminectomy. We report the case of a 16-year-old girl, affected by spinal dysraphism, who developed a persistent giant lumbar pseudomeningocele after repeated surgery for detethering of the spinal cord and removing of spinal lipomyelomeningocele. She was finally treated with fast-resorbing polymer mesh, to fill the gap of the posterior osseous defect and to provide support for the thecal sac. For our best knowledge, this is the first pediatric case reported in literature on this kind of reconstruction. In complex cases of pseudomeningocele, implant of fast-resorbing polymer mesh may represent an option also in pediatric patients.

Background: Pediatric spinal epidural abscess is a major suppurative infection of the central nervous system. It is an extremely rare pathology carrying serious risk of permanent neurological sequelae if is not properly treated., Methods and Results: All the pertinent literature was analyzed, focused on pediatric cases of spinal epidural abscess and its peculiar features. Two illustrative cases are also presented. The first case is that of a 9-year old girl who took medical attention, when she was already paraplegic. Despite prompt surgical evacuation and good neuroradiological outcome and intensive rehabilitation, motor deficits did not recover after surgery. The second case was that of a 14-year old girl who presented with fever, neck pain, and torticollis. Prompt diagnosis, decompressive surgery, and 6 weeks of antibiotics allowed good neurological outcome., Conclusions: The management of spinal epidural abscess includes evacuation of the abscess with decompression of the spinal cord and prolonged antibiotic treatment. The presence of neurological deficit and the delay in the initiation of proper treatment are the two factors that more worsen prognosis.

Introduction: Cranioplasty in children is a controversial and challenging issue, since there is still no consensus on the ideal material. Main problems in paediatric age are represented by the child's growing skull, the lower bone thickness and the high incidence of cerebrospinal fluid (CSF) disorders or brain swelling. Autologous bone is still considered the "gold standard". When it is not available, a wide range of alloplastic materials have been proposed. Hydroxyapatite, a ceramic-based derivative, bears a chemical composition very similar to the human natural bone, making this material a valuable alternative to other cranioplasty solutions., Methods: All patients implanted with a custom-made porous hydroxyapatite device at Santobono-Pausilipon Hospital in Naples were retrospectively reviewed. A follow-up CT scan of the skull was performed from 1 up to 48 months postoperatively to document the bone ingrowth as well as the osteointegration process. The bone density was measured as according to the Hounsfield scale at the bone-implant interface., Results: Between 2014 and 2018, 11 patients (7 males, 4 females) underwent cranioplasty with hydroxyapatite ceramic implants (HAP). Patients' age ranged between 3 and 16 years old. Initial aetiology was trauma in most cases. Two subjects were implanted with HAP as primary cranioplasty, 9 as revision surgery following previous cranioplasty failure. Sites of the cranial defect were unilateral fronto-temporo-parietal (N = 8), unilateral frontal (N = 1) and bifrontal (N = 2). Two patients with large bilateral defects received two prostheses. In one of these, the two prostheses were explanted and replaced with two back-up implants (accounting for a total of 15 implants in 11 patients). Osteointegration was measurable for 12 out of 15 implanted devices. The mean percentage was about 51%. There were six asymptomatic prosthesis fractures (40%), all occurring within 6 months from implant. In one case, the bifrontal prostheses were explanted and replaced. This was the only patient who underwent revision surgery., Conclusion: Hydroxyapatite ceramic implants represent a valid alternative to other cranioplasty solutions. Where coaptation occurs correctly, with good osteointegration, implant mechanical resistance increases over time.

Introduction: Post-traumatic hydrocephalus following head injury is a well-known entity. Most cases occur in patients with severe head injuries, often following decompressive craniectomy. On the contrary, acute post-traumatic hydrocephalus, caused by aqueductal obstruction by a blood clot, following mild head injury is uncommon., Clinical Material: Six patients aged between 6 and 15 months presented hydrocephalus secondary to a blood clot in the aqueduct. Because of intracranial hypertension at presentation, 4 patients were urgently treated with external ventricular drains (EVDs). Post-operative course was uneventful. In 2 cases, EVDs were removed without further treatments. In 2 cases, hydrocephalus recurred. These patients were successfully treated with endoscopic third ventriculostomy. The remaining two patients developed symptoms a few days after the trauma. One, that presented hydrocephalus at imaging, was managed with a ventriculo-peritoneal shunt; the other, that presented subdural hygroma, was managed with subduro-peritoneal shunt that was removed later. All patients had complete recovery., Discussion and Conclusion: Hydrocephalus secondary to clot in the aqueduct may rarely be the result of mild head injury in young children. Usually, prompt surgical management warrants a very good outcome. Most children may be treated without a permanent shunt, by using external drains and endoscopic third ventriculostomy.

Introduction: Intrathecal baclofen (ITB) is an effective treatment for managing primary and secondary dystonia. Intraventricular baclofen (IVB) was first developed to allow treating patients in which the use of ITB was difficult due to anatomic anomalies. After that, several studies indicate that intraventricular administration of baclofen, is more effective than ITB in refractory dystonia., Clinical Material: We report three cases of children with acute dystonic and dysautonomic storm, treated with IVB. The clinical outcome was satisfactory. The response to the treatment continued after the pump disconnection, suggesting that in this kind of cerebral dysregulations, short-term IVB is an effective treatment., Conclusion: Early treatment with IVB may be an effective option in patients with post-anoxic dysautonomic and dystonic storm.

Background: Intracerebral abscess is a rare, but very serious complication of neonatal sepsis. Aggressive medical and surgical strategy is warranted in patients with large abscesses., Case Report: A neonate with bilateral large frontal abscesses, caused by Serratia marcescens, was operated using a neuroendoscope coupled with an ultrasonic aspiration device. To our knowledge, this is the first report of such utilization of this new tool. The left abscess was surgically drained through endoscopic aspiration using ultrasonic aspirator. Clinical condition rapidly improved, and postoperative MRI of the brain revealed an almost complete resolution of the left abscess. A second endoscopic procedure was performed 1 week later, and the right abscess was endoscopically drained with the same technique. The remaining clinical course was uneventful., Discussion and Conclusion: Ultrasonic aspiration is effective in obtaining complete removal of the purulent material and of the dense fibrin layer adherent to the capsule. However, further studies are warranted to determine its real effectiveness, compared with the classic technique. In our opinion, it should be considered an option in more difficult cases, such as abscesses caused by aggressive germs in young or immunocompromised patients, or in case of recurrent lesions, in order to avoid craniotomy and open surgery.

Introduction: Acute basilar artery occlusion (ABAO) is an infrequent but potentially fatal cause of strokes in both adults and children, and it is usually due to vertebral artery dissection (VAD). VAD has been found to be usually a consequence of traumatic vertebral artery injury. ABAO usually presents with symptoms of acute ischemic stroke (AIS) of the posterior circulation or transient ischemic attack (TIA). It may lead to death or long-term disability if not promptly recanalized. Basilar artery recanalization in children can be achieved safely and with excellent clinical outcome using endovascular thrombectomy with the new generation self-expanding and retrievable stents., Clinical Presentation: We report the case of a 23-month old baby that came to the emergency room of our hospital for progressive impairment of consciousness associated with widespread stiffness and plaintive cry, appeared after accidental fall from stroller. An emergency brain CT scan was obtained showing multiple infarction lesions in the brainstem and left cerebellum suggestive of acute stroke in posterior circulation territories. An MR scan with angiography and diffusion-weighted sequences confirmed the multiple infarction lesions and demonstrated poor representation of the flow signal at the V3 segment of the left vertebral artery and absent representation of the flow signal at the distal segment of the basilar artery suggestive of acute thrombotic occlusion. The patient was immediately referred to interventional neuroradiology unit, and digital subtraction angiography showed complete basilar artery occlusion and left vertebral artery dissection at extracranial V2-V3 segment. The patient underwent intra-arterial thrombectomy using stent retrievers and occlusion of the V2-V3 segment of the left vertebral artery. The patient survived and long-term outcome was excellent., Conclusions: To our knowledge, only nine cases of ABAO in children treated with intra-arterial thrombectomy have been previously reported in the literature. In only three cases, the Solitaire stent was applied. Our case is the first case of basilar artery occlusion treated with Solitaire stent, in a child under 24 months. The reports that are available so far indicate that basilar artery recanalization in children can be achieved safely and with excellent clinical outcome using endovascular thrombectomy with the new generation self-expanding and retrievable stents.

Chiari malformation type I (CM-I) and hydrocephalus are often associated with complex craniosynostosis. On the contrary, their simultaneous occurrence in monosutural synostosis is extremely rare. The pathophysiological hypothesis is that they may alter posterior fossa growth and lead to cerebellar tonsil herniation also without skull base primary involvement. Hydrocephalus is multifactorial and may be secondary to fourth ventricle outlet obstruction. The management of these cases is quite complex and not well defined. Cranial vault remodeling should be the only treatment when CM-I is asymptomatic and not related to syringomyelia. Suboccipital decompression should be reserved only in complicated CM-I, usually as a second surgical step following the correction of the supratentorial deformity. In our opinion, the associated hydrocephalus should be treated first in order to normalize intracranial hypertension before opening the cranial sutures. We report the case of a 26-month-old child that presented with sagittal craniosynostosis, hydrocephalus, and CM-I. He was managed by performing endoscopic third ventriculostomy (ETV) first and cranial vault remodeling thereafter. Clinico-radiological outcome was very satisfying. Concerning literature is reviewed; physiopathology and surgical management are discussed.

Introduction: Management of posterior fossa tumors in infants and neonates is challenging. The characteristics of the young babies make surgery very difficult, sometimes precluding a safe complete removal., Methods: A review of the literature was undertaken to examine the incidence, histology, surgical aspects, and prognosis of posterior fossa tumors in the first year of life. Therapeutical strategies of the most frequent tumor types are also discussed in detail., Results: Histology is dominated by tumors with aggressive behavior, such as medulloblastomas, atypical teratoid/rhabdoid tumors, and anaplastic ependymomas. The most important surgical considerations in small children are the small circulating blood volume; the poor thermoregulation; and incomplete maturation of the brain, of the skull, and of the soft tissue. Treatment toxicity is inversely related to the age of the patients. Radiation therapy is usually considered as contraindicated in young children, with few exceptions. Proton therapy is a promising tool, but access to this kind of treatment is still limited. The therapeutic limitations of irradiation render resection of this tumor and adjuvant chemotherapy often the only therapeutic strategy in many cases., Conclusions: The overall prognosis remains dismal because of the prevalent aggressive histologies, the surgical challenges, and the limitations of adjuvant treatment. Nevertheless, the impressive improvements in anesthesiology and surgical techniques allow, in the vast majority of the cases, complete removal of the lesions with minor sequelae in high-volume referral pediatric centers.

Introduction: We performed a retrospective study on clinical assessment, tumor location, radiological imaging, histopathological characteristics, and therapeutic management of 7 patients affected by choroid plexus carcinoma (CPC) or atypical choroid plexus papilloma (ACPP) who have been observed in the last 12 years., Methods: Four patients fulfilled the criteria for classification as ACPP and three cases as CPC. The median age of the patients at the diagnosis was 42 months (range 3-190 months). Except one older patient (15 years old), all patients were younger than 3 years of age. In all patients affected by ACPP, a total surgical resection was achieved. Two children relapsed 12 and 8 months following radical removal. Both of them underwent adjuvant chemotherapy (carboplatin, cyclophosphamide, etoposide, doxorubicin, and methotrexate); a complete remission was maintained in all cases. In all three patients with CPC, it was impossible to achieve complete resection at first surgery. The response to chemotherapy was variable: in one case, it was complete with complete remission following 6 months; in one case, it was partial with reduction on volume (the patient underwent second-look surgery with complete resection); in the third case, there was no response and the patient progressed and finally died with metastatic disease, 8 months after chemotherapy was started. For children with CPC, the OS was 75% at 6 years., Results: In our series, surgery associated with chemotherapy led to long-term survival in 4/4 patients affected by ACPP and 2/3 patients affected by CPC. Clinical results achieved in our series confirm that our therapeutic regimen is feasible and efficient as a possible adjuvant treatment for both CPC and ACPP. It also suggests that surgery has a pivotal role in the management of most children affected by CPTs.

Introduction: Subdural fluid collections following intraventricular and/or paraventricular procedures in pediatric neurosurgery are common and can be hard to treat. We describe our technique to close cortical defects by the aid of a fibrin adhesive and subsequent Ringer inflation with the aim to avoid cortical mantle collapse and to prevent the development of subdural fluid collections., Materials and Methods: We report the preliminary results of a prospective study on a consecutive series of 29 children who underwent 37 transcortical or transcallosal surgical procedures since 2008 in our department., Results: In 17 procedures, we performed a transcortical approach on lesions, and in other 19 operations, we operated by a transcallosal. In 5/17 transcortical approaches (29%) and in 3/20 transcallosal approaches (15%), we observed a 5-mm-thick subdural fluid collection of the 5 patients with subdural fluid collections in the transcortical group, 3 patients (17%) underwent surgery for symptomatic or progressive subdural fluid collections. Of the 3 patients in the transcallosal group, a subduro-peritoneal shunt was necessary only for 1 patient (5%). At the very end of the treatment (including chemotherapy and radiotherapy), it was possible to remove the subduro-peritoneal shunt in all these patients because of disappearance of the subdural fluid collections., Conclusion: In pediatric patients after transcortical or transcallosal procedures, the use of a fibrin adhesive to seal surgical opening and subsequent inflation of the residual cavity with Ringer lactate solution to avoid cortical mantle collapse seems safe and appears to prevent the development of subdural fluid collections.

Introduction: The sylvian aqueduct is the most common site of intraventricular blockage of the cerebro-spinal fluid. Clinical aspects, neuroradiological appearance, and treatment of hydrocephalus secondary to aqueductal stenosis are specific., Methods: An extensive literature review concerning etiologic, pathogenetic, clinical, and neuroradiological aspects has been performed. Therapeutic options, prognosis, and intellectual outcome are also reviewed., Conclusion: The correct interpretation of the modern neuroradiologic techniques may help in selecting adequate treatment between the two main options (third ventriculostomy or shunting). In the last decades, endoscopic third ventriculostomy has become the first-line treatment of aqueductal stenosis; however, some issues, such as the cause of failures in well selected patients, long-term outcome in infant treated with ETV, and effect of persistent ventriculomegaly on neuropsychological developmental, remain unanswered.

Introduction: Even if the first description of Dandy-Walker dates back 1887, difficulty in the establishment of correct diagnosis, especially concerning differential diagnosis with other types of posterior fossa CSF collection, still persists. Further confusion is added by the inclusion, in some classification, of different malformations with different prognosis and therapeutic strategy under the same label of "Dandy-Walker"., Methods: An extensive literature review concerning embryologic, etiologic, pathogenetic, clinical and neuroradiological aspects has been performed. Therapeutic options, prognosis and intellectual outcome are also reviewed., Conclusion: The correct interpretation of the modern neuroradiologic techniques, including CSF flow MR imaging, may help in identifying a "real" Dandy-Walker malformation. Among therapeutical strategies, single shunting (ventriculo-peritoneal or cyst-peritoneal shunts) appears effective in the control of both ventricle and cyst size. Endoscopic third ventriculostomy may be considered an acceptable alternative, especially in older children, with the aim to reduce the shunt-related problems. Prognosis and intellectual outcome mostly depend on the presence of associated malformations, the degree of vermian malformation and the adequate control of hydrocephalus.

Introduction: Hydrocephalus secondary to intraspinal tumors is a well-known but rare condition since about 1% of patients with spinal cord tumors have various degrees of hydrocephalus at initial presentation., Discussion: The mechanism of development of intracranial hypertension and hydrocephalus in patients with spinal cord tumor is not exactly known. The problematic aspects of this condition, with regard to clinical presentation and pathophysiology, are discussed and the relevant literature is reviewed. This uncommon association should always be kept in mind in the differential diagnosis of hydrocephalus of unknown etiology for three main reasons: the possibility of neurological deterioration if the patient is shunted prior tumor removal, the possibility to treat the hydrocephalus without shunting by simply removing the tumor, and the possible role of hydrocephalus as an early sign of intracranial metastasis in patients previously operated upon for removal of intramedullary gliomas. Due to the very slow evolution of the disease, a careful and close clinical and neuroradiological follow-up are essential for many years afterward. The presence of intracranial hypertension in a patient previously operated for a spinal tumor should be considered and investigated as an early sign of neoplastic intracranial seeding.

Introduction: Pseudotumor cerebri is a condition characterized by raised intracranial pressure, normal CSF contents, and normal brain with normal or small ventricles on imaging studies. It affects predominantly obese women of childbearing age; however, its incidence seems to be increasing among adolescent and children. While among older children the clinical picture is similar to that of adults, younger children present demographic and clinical peculiarities. Different diagnostic criteria for adults and pre-pubertal children have been proposed. Etiology and pathogenesis are still unclear, particular concerning the role of obstruction to venous outflow., Methods: An extensive literature review concerning all the aspects of pseudotumor cerebri has been performed, both among adults and pre-pubertal children., Conclusion: Pseudotumor cerebri is an avoidable cause of visual loss, both in adults and children. Few diagnostic measures are usually sufficient to determine the correct diagnosis. Since pseudotumor cerebri is a diagnosis of exclusion, the differential diagnosis work out is of special importance. Modern neuroimaging techniques, especially magnetic resonance imaging and magnetic resonance venography may clarify the role of obstruction to venous outflow in each case. Various therapeutic options are available: medical, surgical, and endovascular procedures may be used to prevent irreversible visual loss. Treatment is usually effective, and most patients will experience complete resolution of symptoms without persistent deficits.

Introduction: Migration of the distal catheter into the heart is an extremely rare complication of ventriculoperitoneal shunt, with only 11 cases reported in literature; in only four of them, there was also the migration into the pulmonary artery. The authors report the case of a young patient with this rare complication that was managed simply converting the ventriculoperitoneal shunt into a ventriculoatrial shunt., Discussion: The migration of the distal catheter occurred through the internal jugular vein probably as the consequence of direct damage of the vessel during tunneling. Venous flow and negative intrathoracic pressure may have gradually pulled the catheter up into the heart; the upward migration may also be encouraged by the continuous movements of the patient that was agitated in the postoperative period as the consequence of her psychiatric disorder. The authors review the literature regarding migration of distal tubing into the heart and pulmonary artery. Diagnosis and management of this complication is discussed.

Background: The significant technological improvement of endoscopic instrumentation has allowed, in the last 10 years, a widespread diffusion of neuroendoscopic procedures. Nevertheless, severe, sometimes life-threatening, complications may occur during neuroendoscopic surgery, and the incidence and age specificity of complications in children have been underdescribed so far., Materials and Methods: Complications recorded in a prospectively collected database of pediatric patients undergoing neuroendoscopic procedures were analysed; the medical histories of the patients and the surgical procedures were reviewed., Results: Complications occurred in 32 out of 231 (13.8%) procedures performed for the management of obstructive hydrocephalus (137), multiloculated hydrocephalus (53), arachnoid cysts (29) and intraventricular tumors (12). Subdural hygroma occurred in 11 cases, seven requiring subdural shunting. In one of these cases, infection of the subdural space occurred and required a craniotomy. Cerebrospinal fluid (CSF) infection occurred in 11 cases. In one case, a frontal abscess developed and was managed with craniotomy. CSF leak occurred in nine cases, intraventricular haemorrhages in two, technical failures in seven, subcutaneous CSF collection (managed with lumbo-peritoneal shunt) in one, thalamic contusion and post-operative transient akinetic mutism in one. This patient suddenly died 6 months later, probably as a consequence of closure of the stoma. Two patients developed secondary compartmentalisation of the ventricles after complicated endoscopic third ventriculostomy. In nine cases, these complications were associated. Overall, no patient died after the procedure (operative mortality 0), one patient died 6 months after the procedure for unexplained events (sudden death rate 0.4%), and three patients presented permanent disability as a consequence of surgical complication (permanent morbidity 1.3%)., Conclusions: Complication rate of neuro-endoscopic procedures is not negligible even in experienced hands. The majority are minor complications which do not affect the final outcome, but sporadically major events may occur, leading to significant problems in surgical management and, occasionally, to permanent disabilities. Careful selection of patients on pre-operative imaging studies and intensive training of surgeons are mandatory to improve results.

Introduction: Chiari malformation (CM) is a frequent finding in multisutural and syndromic craniosynostosis, occurring in 70% of patients with Crouzon's syndrome, 75% with oxycephaly, 50% with Pfeiffer's syndrome and 100% with the Kleeblattschädel deformity. The pathogenesis of this condition and rationale for treatment are still controversial., Discussion: Since its first description in 1972, several factors have been cited to play a role in inducing CM. In the light of recent publications, the roles of premature fusion of cranial vault and cranial base sutures, of congenital anomalies of the cerebellum and brain stem, of raised intracranial pressure, of venous hypertension and of hydrocephalus are reviewed. Evaluation and management of CM are also discussed., Conclusion: Chiari malformation appears to be an acquired and progressive condition that develops in the first months of life, because of a disproportion between hindbrain growth and an abnormally small posterior fossa, a consequence of the premature fusion of lambdoid and cranial base sutures. Venous hypertension caused by stenosis of the jugular foramen can also be present in these patients, resulting in intracranial hypertension and/or hydrocephalus. Careful MRI evaluation is recommended for the forms of craniosynostosis at a high risk of developing hindbrain herniation. The selection of posterior cranial vault expansion as the first surgical procedure is advocated. In selected cases, treatment of the posterior cranial deformity by occipital vault remodelling and treatment of the Chiari-like deformity by suboccipital decompression can be carried out using the same surgical procedure.

Case Reports: Treatment of isolated fourth ventricle syndrome is difficult and there is no widely agreed method. Fourth ventriculo-peritoneal shunting is the most commonly utilized procedure for the management of this syndrome. Complications from shunting are common and are usually related to malfunction, infection, dislocation and overdrainage. We present two unusual cases in which both patients developed bilateral abducens and facial nerve palsies following shunting of an isolated fourth ventricle. Magnetic resonance imaging (MRI) in both cases revealed collapse of the fourth ventricles with downward displacement of the brain stem. In the first case the trans-tentorial pressure difference was equilibrated with the aid of a "Y" connector between the supratentorial and infratentorial shunts, with full recovery of the neurological deficits; in the second case this approach failed and following a complicated neurosurgical course successful endoscopic aqueductal stenting was performed., Discussion: Pathogenesis of cranial nerve palsies following fourth ventricle shunting and the rationale of treatment are discussed and the literature is reviewed.

Object: The purpose of the present study is to assess the effectiveness of endoscopic third ventriculostomy (ETV) in children with hydrocephalus related to posterior fossa tumors., Methods: Between September 1999 and December 2002, 63 children with posterior fossa tumors were treated at Santobono Hospital in Naples, Italy. Twenty-six patients had severe hydrocephalus. In order to relieve intracranial hypertension before tumor removal, 20 were treated with ETV, and 6 with ventriculo-peritoneal (VP) shunts. Twenty patients with mild hydrocephalus were treated with diuretics, corticosteroid agents, and early posterior fossa surgery, and 17 patients who did not have hydrocephalus were treated by elective posterior fossa surgery. Another 4 ETV were performed in the management of postoperative hydrocephalus., Results: Preoperative ETV procedures were technically successful. One was complicated by intraventricular bleeding. The successful 19 preoperative ETV resolved intracranial hypertension before posterior fossa surgery in all cases. Three of these 19 patients developed postoperative hydrocephalus and were treated by VP shunt insertion after posterior fossa surgery. Out of the 4 ETV performed after posterior fossa surgery, only 2 were successful, both when the shunt malfunctioned., Conclusions: Endoscopic third ventriculostomy should be considered as an alternative procedure to ventriculo-peritoneal shunting and external ventricular draining for the emergency control of severe hydrocephalus caused by posterior fossa tumors, since it can quickly eliminate symptoms, and hence, can delay surgery scheduling if required. Even though ETV does not prevent postoperative hydrocephalus in all cases, it does protect against acute postoperative hydrocephalus due to cerebellar swelling. In addition, it eliminates the risks of cerebrospinal fluid (CSF) infection related to external drainage and minimizes the risk of overdrainage because it provides more physiological CSF drainage than the other procedures. Since postoperative hydrocephalus is very often physically obstructive, ETV should always be considered a possible treatment procedure.

Introduction: Hydrocephalus due to aqueductal stenosis following mumps meningoencephalitis is a rare condition, reported only in 16 cases in the literature. The pathogenetic role of the mumps virus in inducing aqueductal stenosis has been demonstrated experimentally in animal models and clinically proven in a few cases. Although obstructive in nature, the post-infectious etiology raises the question as to whether third ventriculostomy is the appropriate treatment., Patient: We report a case of hydrocephalus due to pure aqueductal stenosis occurring in an 11-year-old boy who suffered from mumps meningoencephalitis 9 years previously. The boy was successfully treated by endoscopic third ventriculostomy., Conclusions: The present case offers further evidence of the purely obstructive nature of the hydrocephalus induced by paramyxovirus meningoencephalitis, even if it did occur several years after the infectious disease. The pathogenesis of mumps-induced hydrocephalus and the rationale of treatment are discussed, and the literature is reviewed.