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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.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.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.

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.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, IA 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 IOur 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.

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.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.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.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.

OBJECTIVE Widespread use of modern neuroimaging has led to a surge in diagnosing pediatric brain incidentalomas. Thalamic lesions have unique characteristics such as deep location, surgical complexity, and proximity to eloquent neuronal structures. Currently, the natural course of incidental thalamic lesions is unknown. Therefore, the authors present their experience in treating such lesions. METHODS A retrospective, international multicenter study was carried out in 8 tertiary pediatric centers from 5 countries. Patients were included if they had an incidental thalamic lesion suspected of being a tumor and were diagnosed before the age of 20 years. Treatment strategy, imaging characteristics, pathology, and the outcome of operated and unoperated cases were analyzed. RESULTS Overall, 58 children (23 females and 35 males) with a mean age of 10.8 ± 4.0 years were included. The two most common indications for imaging were nonspecific reasons (n = 19; e.g., research and developmental delay) and headache unrelated to small thalamic lesions (n = 14). Eleven patients (19%) underwent early surgery and 47 were followed, of whom 10 underwent surgery due to radiological changes at a mean of 11.4 ± 9.5 months after diagnosis. Of the 21 patients who underwent surgery, 9 patients underwent resection and 12 underwent biopsy. The two most frequent pathologies were pilocytic astrocytoma and WHO grade II astrocytoma (n = 6 and n = 5, respectively). Three lesions were high-grade gliomas. CONCLUSIONS The results of this study indicate that pediatric incidental thalamic lesions include both low- and high-grade tumors. Close and long-term radiological follow-up is warranted in patients who do not undergo immediate surgery, as tumor progression may occur.

PurposeTo 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.MethodsBrain 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).ResultsBoth 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).ConclusionDecreased 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.

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.

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.

In this video, the authors present ultrasonic resection of calcified tumor of the third ventricle in a 12-year-old boy. He presented to the emergency department with a 1-week history of headache, drowsiness, and bilateral papilledema. Despite extensive calcification visible on a CT scan, a minimally invasive pure endoscopic approach was chosen. The use of an ultrasonic aspirator allows fast and safe removal of the tumor. The histological diagnosis was a low-grade glioneuronal tumor. In conclusion, the endoscopic ultrasonic aspirator is a useful tool to resect tumors in the ventricular system. The presence of calcifications within the tumor does not contraindicate an endoscopic approach. The video can be found here: https://stream.cadmore.media/r10.3171/2023.1.FOCVID22143

OBJECTIVE The authors’ objective was to measure the effect of posterior cranial vault distraction (PCVD) plus foramen magnum decompression (FMD) on dural sinus volume and venous flow in patients with syndromic craniosynostosis. METHODS The volumes of the sagittal, straight, transverse, and sigmoid sinuses of 5 consecutive patients with syndromic craniosynostosis who underwent PCVD+FMD were calculated in cubic centimeters with T2-weighted volumetric MRI sequences before surgery, immediately after surgery, and after the end of the distraction process. Tridimensional reconstructions of phase-contrast magnetic resonance angiography (PC-MRA) images were obtained with multiplanar reconstruction (MPR). RESULTS The average total volume of all dural sinuses increased immediately after surgery (from 10.06 cm3 to 12.64 cm3) and continued to increase throughout the 30-day distraction period (from 12.64 cm3 to 14.71 cm3) (p = 0.04), except that the right sigmoid sinus remained stable after the initial increase. The most important increases were observed for the left transverse sinus (+113.2%), right transverse sinus (+104.3%), left sigmoid sinus (+91.3%), and sagittal sinus (+41.8%). Less important modifications were evident for the right sigmoid sinus (+33.7%) and straight sinus (+23.4%). Significant improvements in venous flow were noted on the tridimensional reconstructions of the PC-MRA images. Venous obstruction grading score improved in 4 patients (average [range] 2.4 [ 2–5]) (p = 0.023) and remained stable in 1 patient. All patients had chronic tonsillar herniation (CTH) (mean [range] 16.6 [8–26] mm), and 3 had syringomyelia. CTH showed improvement on the last follow-up MRI evaluation in 4 patients (mean [range] 10.5 [0–25] mm) and worsened from 15 mm to 19 mm in 1 patient. Syringomyelia improved in 2 patients and remained unchanged in 1. CONCLUSIONS This study has provided the first radiological evidence of the impact of craniofacial surgery on dural sinus anatomy and venous drainage. The venous anomalies described in patients with syndromic craniosynostosis are not static, and PCVD+FMD triggers a dynamic process that can lead to significant modifications of intracranial venous drainage. The traction exerted by the distracted bone flap onto the occipitoparietal dura mater adherent to the inner calvaria may account for the enlargement of the dural sinus throughout the distraction period. The impact of these modifications on venous pressure, intracranial pressure, CTH, and hydrocephalus remains to be determined.

Background Chronic subdural hematoma associated with dural calcifications in previously pediatric shunted patients is a rare condition. The inner dural membrane opening can lead to progressive brain herniation into the subdural space due to brain reexpansion. Case Description A 15-year-old boy, previously shunted at birth for congenital hydrocephalus, presented with a giant chronic right hemispheric subdural hematoma. After 2 surgical procedures in which the subdural calcified neomembrane was opened and a subdural shunt was implanted, he developed a cortical brain herniation into the subdural space, resulting in brain ischemia and upper limb weakness and vomiting. The final surgical treatment consisted of an extensive wide peeling of visceral calcified membrane mutually to programmable valve placement along the subduroperitoneal shunt, in order to create a positive gradient between the subdural space and the ventricles. The patient experienced a prompt clinical improvement. Conclusions This case illustrates a rare complication of the treatment of a chronic subdural hematoma caused by insufficient opening of the calcified inner mambrane of the hematoma and encouraged by gradient pressure between the ventricular and subdural compartments. To avoid this complication, it is preferable to not open a thick, calcified, unelastic inner membrane. However, in case of lack of clinical and radiologic improvement, it may become necessary to open it: a wide opening should be attempted, in order to prevent brain strangulation.

We present an uncommon case of holocord, "rosary bead-like," multiloculated syringomyelia consistent with a fourth ventricle outlet obstruction in a 2-month-old infant who was previously shunted for posthemorrhagic hydrocephalus of prematurity. The shunt malfunction resulted in a syringomyelia because of the simultaneous obstruction of the 3 outlets of the fourth ventricle, resulting in an enlargement of the central canal of the spinal cord. The syringomyelia resolved with shunt revision, avoiding further complex surgical procedures.

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. 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. 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.

Introduction: Hydrocephalus is not usually part of Down syndrome (DS). Fourth ventricle outlet obstruction is a rare cause of obstructive hydrocephalus, difficult to diagnose, because tetraventricular dilatation may suggest a communicant/nonobstructive hydrocephalus. Case Presentation: We describe the case of a 6-year-old boy with obstructive tetraventricular hydrocephalus, caused by Luschka and Magen­die foramina obstruction and diverticular enlargement of Luschka foramina (the so-called fourth ventricle outlet obstruction) associated with DS. He was treated with endoscopic third ventriculostomy (ETV) without complications, and a follow-up MRI revealed reduction of the ventricles, disappearance of the diverticula, and patency of the ventriculostomy. Conclusion: Diverticular enlargement of Luschka foramina is an important radiological finding for obstructive tetraventricular hydrocephalus. ETV is a viable option in tetraventricular obstructive hydrocephalus in DS.

OBJECTIVE Colloid cysts (CCs) are rare at all ages, and particularly among children. The current literature on pediatric CC is limited, and often included in mixed adult/pediatric series. The goal of this multinational, multicenter study was to combine forces among centers and investigate the clinical course of pediatric CCs. METHODS A multinational, multicenter retrospective study was performed to attain a large sample size, focusing on CC diagnosis in patients younger than 18 years of age. Collected data included clinical presentation, radiological characteristics, treatment, and outcome. RESULTS One hundred thirty-four children with CCs were included. Patient age at diagnosis ranged from 2.4 to 18 years (mean 12.8 ± 3.4 years, median 13.2 years, interquartile range 10.3–15.4 years; 22% were < 10 years of age). Twenty-two cases (16%) were diagnosed incidentally, including 48% of those younger than 10 years of age. Most of the other patients had symptoms related to increased intracranial pressure and hydrocephalus. The average follow-up duration for the entire group was 49.5 ± 45.8 months. Fifty-nine patients were initially followed, of whom 28 were eventually operated on at a mean of 19 ± 32 months later due to cyst growth, increasing hydrocephalus, and/or new symptoms. There was a clear correlation between larger cysts and symptomatology, acuteness of symptoms, hydrocephalus, and need for surgery. Older age was also associated with the need for surgery. One hundred three children (77%) underwent cyst resection, 60% using a purely endoscopic approach. There was 1 death related to acute hydrocephalus at presentation. Ten percent of operated patients had some form of complication, and 7.7% of operated cases required a shunt at some point during follow-up. Functional outcome was good; however, the need for immediate surgery was associated with educational limitations. Twenty operated cases (20%) experienced a recurrence of their CC at a mean of 38 ± 46 months after the primary surgery. The CC recurrence rate was 24% following endoscopic resection and 15% following open resections (p = 0.28). CONCLUSIONS CCs may present in all pediatric age groups, although most that are symptomatic present after the age of 10 years. Incidentally discovered cysts should be closely followed, as many may grow, leading to hydrocephalus and other new symptoms. Presentation of CC may be acute and may cause life-threatening conditions related to hydrocephalus, necessitating urgent treatment. The outcome of treated children with CCs is favorable.

ObjectiveA single-institution cohort of 92 consecutive pediatric patients harboring tumors involving the fourth ventricle, surgically treatedviathe telovelar or transvermian approach, was retrospectively reviewed in order to analyze the impact of surgical route on surgery-related outcomes and cumulative survival.MethodsClinical, radiological, surgical, and pathology details were retrospectively analyzed. We selected n = 6 surgery-related clinical and radiological outcomes: transient and permanent neurological deficits, duration of assisted ventilation, postoperative new onset medical events, postoperative cerebellar mutism, and extent of resection. We built univariate and multivariate logistic models to analyze the significance of relationships between the surgical routes and the outcomes. Cumulative survival (CS) was estimated by the cohort approach.ResultsThere were 53 girls and 39 boys (mean age, 83 months). Telovelar approach was performed in 51 cases and transvermian approach in 41 cases. Early postoperative MRI studies showed complete removal in 57 cases (62%) and measurable residual tumor in 35 cases (38%). The average tumor residual volume was 1,316 cm3(range, 0.016–4.231 cm3; median value, 0.9875 cm3). Residual disease was more often detected on immediate postop MRI after telovelar approach, but the difference was not significant. Cerebellar mutism was observed in 10 cases (11%). No significant difference in the onset of cerebellar mutism was detected between telovelar and transvermian approach. The choice of surgical approach did not significantly modify any other postoperative outcome and 1-/3-year CS of high-grade surgically treated tumors.ConclusionsWith the limitation of a single-center, single-surgeon retrospective series, our findings offer significant data to reconsider the real impact of the choice of the surgical route to the fourth ventricle on the incidence of cerebellar mutism and surgery-related morbidity. This seems to be in line with some recent reports in the literature. Surgical approach to the fourth ventricle should be individualized according to the location of the tumor, degree of vermian infiltration, and lateral and upward extension. Telovelar and transvermian approaches should not be considered alternative but complementary. Pediatric neurosurgeons should fully master both approaches and choose the one that they consider the best for the patient based on a thorough and careful evaluation of pre-operative imaging.

Medulloblastoma (MB) is an embryonal tumor of the cerebellum constituting ~ 20% of pediatric brain tumors. To date, four MB molecular groups (further stratified in twelve subtypes) have been described. Among them, Groups 3 and Group 4 MB have the poorest prognosis due to their high metastatic potential. Recently, we have reported a metastatic axis driven by Prune1 overexpression in MB Group3 characterized by canonical TGF-β signaling enhancement and epithelial-mesenchymal transition. Here, we have developed a new not toxic pyrido-pyrimidine derivative with the ability to impair Prune-1-driven-axis, thus ameliorating the survival rate of a murine model of metastatic MB Group3 characterized by overexpression of human Prune1 gene in the cerebellum (under the control of MATH1 promoter). Of importance, this small molecule also is showing immunomodulatory functions thus inhibiting the conversion of tumor-infiltrating T lymphocytes (TILs) to immunosuppressive regulatory T cells (Tregs) in vivo via impairing the secretion of inflammatory cytokines from MB cells. Furthermore, this molecule can also act synergistically with the currently used modified-intensity chemotherapy (e.g. in PNET5 use of Vincristine) or potential in the combination with epigenetics drugs (e.g., LSD1/KDM1A inhibitors). Altogether these results are of importance for future targeted therapies of high-risk metastatic MB. Acknowledgments: We thank for funding support: the Italian Association for Cancer Research (AIRC) Grant IG no. 22129 (M.Z.) and Lazio Innova Grant n. 85-2017-14785 (FT; MZ)

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. 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. 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. Hydroxyapatite ceramic implants represent a valid alternative to other cranioplasty solutions. Where coaptation occurs correctly, with good osteointegration, implant mechanical resistance increases over time.

Background Midbrain and surrounding areas are among the most difficult regions to surgically approach in neurosurgery. The subtemporal approach is a well-known neurosurgical approach that is rarely described in children. Objective To assess the feasibility, advantages, and disadvantages of subtemporal approach in children. Methods A total of 20 pediatric patients with intra-axial tumors involving the lateral midbrain or superior cerebellar peduncle or with extra-axial tumors in the middle incisural space/Meckel cave underwent subtemporal transtentorial approaches with the aim to remove (16 cases) or biopsy (4 cases) their lesions. These cases were retrospectively reviewed and the surgical approach described. Results Total resection was achieved in 11 patients. In the 4 patients who underwent biopsies, a diagnosis was obtained. A total of 5 patients presented non-neoplastic lesions (dermoid tumors or cavernomas). A total of 9 patients harbored low-grade tumors and 6 high-grade tumors. Patients with low-grade tumors and non-neoplastic lesions are all alive with no evidence of disease or stable residue. Four patients affected by high grade tumors died. No patient suffered permanent neurological deficits related to the surgical approach. Three patients presented temporary neurological deficits following the procedure: one case of strabismus, one case of aphasia and hemiparesis, secondary to delayed, transient thrombosis of the Labbe vein, and 1 case of trigeminal neuralgia. Conclusion The subtemporal approach represents a feasible approach for mesencephalic and perimesencephalic lesions in children. It provides an ample and direct access, with excellent outcomes and acceptable postoperative morbidity.

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. 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. 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.

Background Interhypothalamic adhesions (IHAs) are horizontally oriented parenchymal bands of tissue connecting the medial hypothalamic regions across the third ventricle. They can be assessed with high-resolution magnetic resonance (MR) techniques. Case Description We report MR and neuroendoscopic features of IHA in a 3-year-old boy without symptoms referable to the hypothalamus. He presented with obstructive hydrocephalus secondary to posterior fossa tumor. An endoscopic third ventriculostomy (ETV) was attempted but not performed because of the presence of a thick IHA, which prevented an approach to the floor of the third ventricle. During the procedure the patient also experienced supraventricular tachycardia. The procedure was aborted, and an external ventricular drainage was left in the ventricles until resolution of hydrocephalus after posterior fossa surgery. To the best of our knowledge, no study has previously described in detail endoscopic images of IHA. Conclusions MR imaging allows one to preoperatively identify most anatomic anomalies of the ventricular system and the floor of the third ventricle. However, an IHA that may prevent an approach to the floor of the third ventricle due to his thickness may be missed. This can be recognized only on direct vision, underlining the importance of endoscopy in neurosurgery.

Background: Patients with brain vascular disease and hydrocephalus may be predisposed to acute ischemic stroke in case of shunt dysfunction and subsequent increased intracranial pression. Patients with brain tumor may develop hydrocephalus as a consequence of obstruction of cerebrospinal fluid pathways and radiation-induced moyamoya syndrome secondary (RIMS) to radiotherapy (RT). Case Description: A 15-year-old male patient, affected by hydrocephalus and RIMS, presented acute cerebral ischemia after an episode of shunt malfunction. The shunt was promptly revised and the areas of ischemia visible at magnetic resonance imaging significantly decreased. Conclusion: Children who receive RT for brain tumor, particularly if the circle of Willis region is involved, require close surveillance for the development of vasculopathy and consequent stroke. This surveillance must be even tighter if the patient has been treated with ventricular shunt for the possible synergistic interaction between the two causes on reducing cerebral perfusion and increasing the risk of acute ischemic events.

BACKGROUND Cavum trigeminale cephaloceles (CTCs) are characterized by a cystic appearance and extension of the posterolateral aspect of Meckel’s cave into the superomedial portion of the petrous apex. A possible mechanism is a transient or sustained increase of intracranial pressure transmitted to Meckel’s cave, leading to its secondary herniation, in the presence of predisposing local factors. The majority of CTCs are incidental findings. Among symptomatic cases, headache, trigeminal neuralgia, cerebrospinal fluid leak, vertigo, ataxia, facial numbness, hearing loss, diplopia, and other visual disturbances have been described. OBSERVATIONS The authors describe a case of an 18-month-old male patient referred to their hospital with drowsiness, vomiting, left cranial nerve VI palsy, and papilledema. Neuroradiological investigation revealed the presence of a CTC with a swollen aspect of the left pontine hemisection and indirect signs of intracranial hypertension. The patient was managed with implantation of a ventriculoperitoneal shunt. LESSONS CTCs are rare lesions that are difficult to recognize on neuroimaging, in which they can be mistaken for tumoral or inflammatory lesions. The most frequent asymptomatic forms should be considered as “leave-me-alone” lesions. In cases of intracranial hypertension, according to a supposed theory on the origin of CTCs, the authors recommend treating only the intracranial hypertension instead of performing more invasive surgical procedures, especially in pediatric patients.

In this video, the authors present an interhemispheric transcallosal transchoroidal approach to a pineal mass in a 15-year-old boy. He received emergency endoscopic third ventriculostomy (ETV), then an endoscopic biopsy that revealed an immature teratoma. Surgical removal was selected. The mass was located very high in the posterior third ventricle, hidden behind the splenium of the corpus callosum and the vein of Galen, so an interhemispheric transcallosal approach followed by a complete dissection of the whole choroidal fissure was chosen and allowed complete removal of the tumor. Microsurgical dissection is presented, showing clearly in detail all the neurovascular structures encountered. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2126.

The authors present a pediatric case of a pineoblastoma treated with gross-total removal through an occipital interhemispheric transtentorial approach (OITA). The child presented with acute hydrocephalus that was treated by endoscopic third ventriculostomy (ETV) and tumor biopsy through a single burr hole. Histology revealed a pineoblastoma. Microsurgical total removal was performed 3 months after neoadjuvant chemotherapy. OITA was chosen on the basis of the tumor’s location below the Herophilus-Galen line of sight. In this video, the authors show the positioning, the operating devices, the approach, and the microsurgical dissection, indicating all the neurovascular structures encountered. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2122.

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. We retrospectively reviewed clinical and radiological data of patients diagnosed with desmoplastic infantile tumours who were surgically treated between 2008 and 2019. The series included 12 patients. The median age at surgery was 91 days. The average tumour volume was 212 cm3. Cystic components were predominant ranging from 0 to 295 cm3. 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). 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.

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.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.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.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).Endoscopic aqueductoplasty and stenting is a reliable procedure in the long-term management of isolated fourth ventricle.

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. 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. 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.

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.

s: The natural history of non-optic central nervous system (CNS) tumors in neurofibromatosis type 1 (NF1) is largely unknown. Here, we describe prevalence, clinical presentation, treatment, and outcome of 49 non-optic CNS tumors observed in 35 pediatric patients (0&ndash, 18 years). Patient- and tumor-related data were recorded. Overall survival (OS) and progression-free survival (PFS) were evaluated. Eighteen patients (51%) harbored an optic pathway glioma (OPG) and eight (23%) had multiple non-optic CNS lesions. The majority of lesions (37/49) were managed with a wait-and-see strategy, with one regression and five reductions observed. Twenty-one lesions (42.9%) required surgical treatment. Five-year OS was 85.3%. Twenty-four patients progressed with a 5-year PFS of 41.4%. Patients with multiple low-grade gliomas progressed earlier and had a lower 5-year PFS than those with one lesion only (14.3% vs. 57.9%), irrespective of OPG co-presence. Non-optic CNS tumors are common in young patients with NF1. Neither age and symptoms at diagnosis nor tumor location influenced time to progression in our series. Patients with multiple lesions tended to have a lower age at onset and to progress earlier, but with a good OS.

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. 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. Early treatment with IVB may be an effective option in patients with post-anoxic dysautonomic and dystonic storm.

Objective Recently, the neuronavigation system (NS) has become an essential intraoperative tool for many neurosurgical procedures, allowing for precise lesion localization. It is particularly important to avoid errors during the navigation process. Here we report a novel technique using palatal positioning of the patient tracker to ensure optimal accuracy during magnetic navigation in various neurosurgical procedures. Methods This retrospective study included a total of 34 patients treated in our institution between June 2017 and January 2018. The patients were split into 2 groups who underwent surgery under general anesthesia: a microscopic transcranial group and an endoscopic endonasal group. Preoperative and postoperative navigation accuracy was assessed by 2 neurosurgeons. Results After our surgical planning navigation protocol was applied, both transcranial and endonasal procedures were successfully performed under navigation guidance in all but 1 patient. There were no intraoperative or postoperative complications related to the tracker mounted under the hard palate. In 33 cases a maximal tracking view and optimal navigation accuracy was achieved, for a success rate of 97%. Conclusions The positioning of the patient tracker under the hard palate proved safe, accurate, and feasible in 97% of our patients. In our case series, it met the main goal of avoiding device displacement without a sense of invasiveness and postoperative patient discomfort.

Intracerebral abscess is a rare, but very serious complication of neonatal sepsis. Aggressive medical and surgical strategy is warranted in patients with large abscesses. 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. 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.