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Primary intramedullary spinal cord lymphoma (PISCL) is a rare cause of myelopathies. As PISCL is often underrecognized, delaying appropriate treatment, we sought to describe its presentation. We report two clinical cases of pediatric patients. The diagnosis of PISCL must be considered in a patient with symptoms of acute myelopathies.

Context Nationwide data on children diagnosed with craniopharyngioma (CP) are not available in Italy. Objective This work aimed to identify patients’ characteristics, type of surgical approach, complications and recurrences, number of pituitary deficits, and number of patients starting growth hormone (GH) treatment. Methods A retrospective multicenter collection took place of 145 patients aged 0 to 18 years who underwent surgery for CP between 2000 and 2018, and followed up in 17 Italian centers of pediatric endocrinology. Results Age at diagnosis was 8.4 ± 4.1 years. Duration of symptoms was 10.8 ± 12.5 months and headache was most frequent (54%), followed by impaired growth (48%) and visual disturbances (44%). Most lesions were suprasellar (85%), and histology was adamantinomatous in all cases but two. Surgical approach was transcranial (TC) in 67.5% of cases and transsphenoidal (TS) in 31.%. The TC approach was prevalent in all age groups. Postsurgery complications occurred in 53% of cases, with water-electrolyte disturbances most frequent. Radiotherapy was used in 39% of cases. All patients but one presented with at least one hormone pituitary deficiency, with thyrotropin deficiency most frequent (98.3%), followed by adrenocorticotropin (96.8%), arginine vasopressin (91.1%), and GH (77.4%). Body mass index (BMI) significantly increased over time. A hypothalamic disturbance was present in 55% of cases. GH therapy was started during follow-up in 112 patients at a mean age of 10.6 years, and 54 developed a recurrence or regrowth of the residual lesion. Conclusion CP is often diagnosed late in Italy, with TC more frequent than the TS surgical approach. Postsurgery complications were not rare, and hypopituitarism developed almost in all cases. BMI shows a tendency to increase overtime.

We describe a patient with focal epilepsy characterized by ictal asystole episodes and low-grade tumour over the left temporal neocortex. Non-invasive pre-surgical evaluation showed an epileptogenic zone extended beyond the low-grade tumour. This extension was confirmed by intraoperative electrocorticography. One-stage surgery with anterior temporal lobe resection was performed. The patient was seizure-free after one year of follow-up. Detailed electroclinical and therapeutic reasoning with hypotheses defining epileptogenic and symptomatogenic networks are discussed.

Craniopharyngioma (CP) is a rare epithelial low-grade tumour that develops in the sellar/suprasellar region of the brain, along the craniopharyngeal duct. It has a bimodal distribution and the first peak occurs in paediatric age almost exclusively consisting of the adamantinomatous subtype. A second peak occurs in adulthood after the fifth decade of life and is more likely to be of the papillary subtype. Therapy is based on surgical removal, so the best approach is the endonasal approach, which is sometimes associated with radiation therapy. Molecular target drugs are a promising novelty, indeed they are already in use in adults and are being tested in children. Although CP is considered a low malignancy tumour, its localization and close relationships with important structures such as the optical pathways, the hypothalamic-pituitary axis and the thalamus burden it with important complications (visual disturbances, central obesity, dysendocrinopathies) that can interfere with the patient’s quality of life.

Brainstem diffuse midline gliomas represent infiltrative and rare pediatric tumors with a dismal prognosis. Surgical biopsy is emerging as a valid technique to define diagnosis and molecular markers for future targeted therapies.We describe the key steps of an endoscopic trans-ventricular biopsy of a brainstem diffuse midline glioma and associated ventriculomegaly. The relevant surgical anatomy along with an illustrative video is described.The endoscopic third ventriculostomy combined with a punch biopsy of a brainstem diffuse midline glioma associated with ventriculomegaly represent a feasible and low-risk procedure to simultaneously treat incipient hydrocephalus and molecular diagnosis for future treatment and research.

Background: The link between the effects of recombinant human growth hormone (rhGH) therapy in patient with growth hormone deficiency (GHD) and Chiari malformation type I (CM-1) is controversial. Summary: We report the case of a patient with an unusual association of growth hormone deficiency (GHD) due to ectopic posterior pituitary and Chiari malformation type I (CM-1). Our patient developed headache and worsening of CM-1 after initiation of rhGH therapy. Following an atlo-occipital decompression surgery, the patient was able to resume therapy with a marked growth improvement. Based on this observation, we provide a systematic review of the current literature about these two pathologies. Key Messages: A careful follow-up of all patients with CM-1 treated with GH is mandatory, paying particular attention to the appearance of any neurological signs and symptoms.

Background: Atlanta-Axial Rotatory Subluxation is an acquired condition, usually occurring in paediatric patients, because of either trauma or pharyngitis or ear-nose-throat (ENT) surgery or upper respiratory tract infection. Post-traumatic C1-C2 rotational subluxation in paediatric age is a very frequent event. It mimics benign torticollis but may result in permanent disability. The Atlanta-axial rotatory displacement, as defined initially by Fielding and Hawkins in 1977, is classified into four types: Type 1 is the most common form in children. It is a simple rotatory displacement without an anterior shift. Type 2 is potentially more dangerous. It is a rotatory displacement with an anterior shift of 5 mm or less. Type 3 is rotatory displacement with an anterior shift greater than 5 mm. Finally, type 4 is rotatory displacement with a posterior shift. Type 3 and 4 deformities are rare, but neurological involvement or even instant death may follow [Figure. 4] [18]. The condition requires prompt diagnosis by thorough examination to avoid any treatment delays. The difficulties for a timely and correct diagnosis and then the correct treatment is described. Case presentation: A 5-year-old girl comes to the emergency room with cervical pain and stiffness. This painful state, as mum reports, has been lasted for about ten days, after a mild trauma. The radiographic picture demonstrates a rotary subluxation C1-C2, type 1 according to Fielding and Hawkins classification. The treatment is head-halter traction with 2kg, and nonsteroidal anti-inflammatory drugs (NSAIDs). After 2 days the x-ray picture improves, but the painful symptoms persist. An in-depth diagnostic MRI of the cervical spine and brain revealed a medulloblastoma which made an immediate and effective neurosurgical intervention necessary. Conclusions: the C1-C2 rotatory dislocation must always be evaluated with extreme care, without neglecting any clinical and anamnestic information for a correct and timely differential diagnosis. Remember that children show very clearly what they feel, always. Keywords: AARS; Cervical spine dislocation; Rotatory subluxation; Differential diagnosis; Medulloblastoma

Objective: We describe a multicenter experience with vagus nerve stimulator implantation in pediatric patients with drug-resistant epilepsy. Our goal was to assess vagus nerve stimulation efficacy and identify potential predictors of favorable outcome. Methods: This is a retrospective study. Inclusion criteria: ≤18 years at time of vagus nerve stimulator implantation, at least 1 year of follow-up. All patients were previously found to be unsuitable for an excisional procedure. Favorable clinical outcome and effective vagus nerve stimulation therapy were defined as seizure reduction >50%. Outcome data were reviewed at 1, 2, 3, and 5 years after vagus nerve stimulator implantation. Fisher exact test and multiple logistic regression analysis were employed. Results: Eighty-nine patients met inclusion criteria. Responder rate (seizure frequency reduction >50%) at 1-year follow-up was 25.8% (4.5% seizure-free). At last follow-up, 31.5% had a favorable outcome and 5.2% were seizure free. The only factor significantly predicting favorable outcome was time to vagus nerve stimulator implantation, with the best outcome achieved when vagus nerve stimulator implantation was performed within 3 years of seizure onset. Implantation between 3 and 5 years after epilepsy onset correlated with better long-term seizure freedom (13.3% at T5). Overall, 65.2% of patients evidenced improved quality of life at last follow-up. However, 12.4% had adverse events, but most were mild and disappeared after 3-4 months. Conclusions: Early vagus nerve stimulator implantation within 5 years of seizure onset was the only predictor of favorable clinical outcome in pediatric patients. Improved quality of life and a low incidence of significant adverse events were observed.

Background: Augmented reality (AR) allows the overlapping and integration of virtual information with the real environment. The camera of the AR device reads the object and integrates the virtual data. It has been widely applied to medical and surgical sciences in recent years and has the potential to enhance intraoperative navigation. Materials and methods: In this study, the authors aim to assess the accuracy of AR guidance when using the commercial HoloLens 2 head-mounted display (HMD) in pediatric craniofacial surgery. The Authors selected fronto-orbital remodeling (FOR) as the procedure to test (specifically, frontal osteotomy and nasal osteotomy were considered). Six people (three surgeons and three engineers) were recruited to perform the osteotomies on a 3D printed stereolithographic model under the guidance of AR. By means of calibrated CAD/CAM cutting guides with different grooves, the authors measured the accuracy of the osteotomies that were performed. We tested accuracy levels of ±1.5 mm, ±1 mm, and ±0.5 mm. Results: With the HoloLens 2, the majority of the individuals involved were able to successfully trace the trajectories of the frontal and nasal osteotomies with an accuracy level of ±1.5 mm. Additionally, 80% were able to achieve an accuracy level of ±1 mm when performing a nasal osteotomy, and 52% were able to achieve an accuracy level of ±1 mm when performing a frontal osteotomy, while 61% were able to achieve an accuracy level of ±0.5 mm when performing a nasal osteotomy, and 33% were able to achieve an accuracy level of ±0.5 mm when performing a frontal osteotomy. Conclusions: despite this being an in vitro study, the authors reported encouraging results for the prospective use of AR on actual patients.

Medulloblastoma is the most common malignant brain tumor in children. Even if current treatment dramatically improves the prognosis, survivors often develop long-term treatment-related sequelae. The current radiotherapy standard for medulloblastoma is craniospinal irradiation with a boost to the primary tumor site and to any metastatic sites. Proton therapy (PT) has similar efficacy compared to traditional photon-based radiotherapy but might achieve lower toxicity rates. We report on our multi-centric experience with 43 children with medulloblastoma (median age at diagnosis 8.7 years, IQR 6.6, M/F 23/20; 26 high-risk, 14 standard-risk, 3 ex-infant), who received active scanning PT between 2015 and 2021, with a focus on PT-related acute-subacute toxicity, as well as some preliminary data on late toxicity. Most acute toxicities were mild and manageable with supportive therapy. Hematological toxicity was limited, even among HR patients who underwent hematopoietic stem-cell transplantation before PT. Preliminary data on late sequelae were also encouraging, although a longer follow-up is needed.

Background The impact of COVID-19 outbreak in the neurosurgical practice has been dramatic, imposing several limitations. The aim of this study is to present how the neurosurgical departments of Emilia-Romagna, a northern Italian region, have re-set their organization to maintain the higher standard of care as possible. Methods All OR and out-patients activities performed during the COVID-19 emergency in the neurosurgical department of Emilia-Romagna have been collected and compared to the means of the same timeframe in 2018 and 2019. Results In 2020, 205 surgical procedures and 466 out-patients consultations have been performed, representing respectively 28.8% and 26.4% of the previous biennium. The most of OR procedures had been emergencies/urgencies and oncological patients (respectively 113 and 66 vs 164.5 and 84.5 of the previous biennium), while elective surgeries decrease up to -97.1%, as for spinal nerves and endoscopic skull base procedures. The patients phone contacts and telemedicine evaluations of their examinations have permitted to reduce the hospital access for outpatients of 75.6%, but these modalities have, also, permitted to follow-up a large number of cases. Conclusions The outbreak of COVID-19 has imposed several limits to our current practice, however this should not represent an excuse to reduce the standard of care. In our experience, the net integration of different local centers has permitted for each of them to effectively cope the crisis, managing the local cases requiring a prompt surgery and keeping the care continuity with already discharged patients.

Meningiomas are an extremely rare histology among pediatric brain tumors, and there is a shortage of literature on their management. Proton therapy is currently used safely and effectively for many types of both pediatric and adult cancer, and its main advantage is the sparing of healthy tissues from radiation, which could translate in the reduction of late side effects. We review the literature on radiotherapy and proton therapy for pediatric meningiomas and report clinical outcomes for two aggressive pediatric meningiomas we treated with protons. Proton therapy might be a safe and effective therapeutic option for this rare subgroup of tumors.

Objective/Background The natural history and the outlook of patients with hydrocephalus are dramatically affected using cerebrospinal fluid shunts. The several issues related with a long-standing shunt should suffice to justify all the possible attempts to free the patient from it. This study focused on the role of secondary endoscopic third ventriculostomy (ETV) in the achievement of shunt removal in cases of shunt malfunction, and to identify patients who could benefit most from the procedure. Methods In the period of 2006–2015, ETV was attempted in 47 patients >6 months old with ventriculoperitoneal shunt malfunction who presented with increased ventricle size compared with the previous neuroradiological examinations; simultaneously the shunt was removed or ligated. Results The overall success rate of secondary ETV was 74% (shunt-free patients with normalized intracranial pressure and absence of symptoms attributable to hydrocephalus) in patients with a long shunt duration (up to 30 years). The number of previous shunt revision procedures (P = 0.026) and lower age (P = 0.017) correlate with the likelihood of secondary ETV failure, a score of 80 as ETV success score (calculated for both pediatric and adult patients, even if the score was meant for the pediatric population) correlates with secondary ETV success (P = 0.014). Conclusions Many patients with shunt malfunction can benefit from secondary ETV even after decades of shunting. Age at secondary ETV, the number of previous shunt revisions, and the ETV success score can help to better identify the best candidates for the procedure.

The paper descibes the cases of a 10-year-old girl and a 3-year-old boy with CNS neoplasia. The girl presented with acute vomiting and cervicalgia while in the boy vomiting persisted for three months as the only symptom and was iniatially misinterpreted as due to gastroesophageal reflux. The authors stress that in very young children (< 4 y) persistent vomiting may be the only symptom of a CNS neoplasia.

Background Due to the lack of high-quality evidence which has hindered the development of evidence-based guidelines, there is a need to provide general guidance on cranioplasty (CP) following traumatic brain injury (TBI), as well as identify areas of ongoing uncertainty via a consensus-based approach. Methods The international consensus meeting on post-traumatic CP was held during the International Conference on Recent Advances in Neurotraumatology (ICRAN), in Naples, Italy, in June 2018. This meeting was endorsed by the Neurotrauma Committee of the World Federation of Neurosurgical Societies (WFNS), the NIHR Global Health Research Group on Neurotrauma, and several other neurotrauma organizations. Discussions and voting were organized around 5 pre-specified themes: (1) indications and technique, (2) materials, (3) timing, (4) hydrocephalus, and (5) paediatric CP. Results The participants discussed published evidence on each topic and proposed consensus statements, which were subject to ratification using anonymous real-time voting. Statements required an agreement threshold of more than 70% for inclusion in the final recommendations. Conclusions This document is the first set of practical consensus-based clinical recommendations on post-traumatic CP, focusing on timing, materials, complications, and surgical procedures. Future research directions are also presented.

Background: Atlanto-Axial Rotatory Subluxation is an acquired condition, usually occurring in pediatric patients, because of either trauma or pharyngitis or ear-nose-throat ( ENT) surgery or upper respiratory tract infection. Post-traumatic C1-C2 rotational subluxation in pediatric age is a very frequent event. It mimics benign torticollis but may result in permanent disability. The atlanto-axial rotatory displacement, as defined initially by Fielding and Hawkins in 1977, is classified into four types: Type 1 is the most common form in children. It is a simple rotatory displacement without an anterior shift. Type 2 is potentially more dangerous. It is a rotatory displacement with an anterior shift of 5 mm or less. Type 3 is rotatory displacement with an anterior shift greater than 5 mm. Finally, type 4 is rotatory displacement with a posterior shift. Type 3 and 4 deformities are rare, but neurological involvement or even instant death may follow [Fig. 4] (18). The condition requires prompt diagnosis by thorough examination to avoid any treatment delays. The difficulties for a timely and correct diagnosis and then the correct treatment are described. Case presentation: A 5 year old girl comes to the emergency room with cervical pain and stiffness.This painful state, as mum reports, has been lasted for about ten days, after a mild trauma. The radiographic picture demonstrates a rotary subluxation C1-C2, type 1 according to Fielding and Hawkins classificationThe treatment is head-halter traction with 2kg, and nonsteroidal anti-inflammatory drugs (NSAIDs) . After 2 days the x-ray picture improves, but the painful symptoms persist. An in-depth diagnostic MRI of the cervical spine and brain revealed a medulloblastoma which made an immediate and effective neurosurgical intervention necessary. Conclusions: the C1-C2 rotatory dislocation must always be evaluated with extreme care, without neglecting any clinical and anamnestic information for a correct and timely differential diagnosis. Remember that children show very clearly what they feel, always .

Intraventricular hemorrhage and the subsequent development of posthemorrhagic hydrocephalus (PHH) is one of the most serious complication of prematurity, especially in extremely low birth weight infants. 1 Neurodevelopmental delay, epilepsy, and severe cognitive impairment represent common sequelae of PHH. 2 , 3 A ventriculoperitoneal shunt insertion in such premature infants is associated with higher rates of skin erosion, infection, and shunt failure. 4 One therapeutic option is represented by the use of temporary cerebrospinal fluid diversion procedures (such as external ventricular drainage, subcutaneous reservoir, and ventriculosubgaleal shunt) to gain time avoiding the PHH secondary damages. 5 , 6 An extremely low birth weight (birth weight = 653 g) infant at 24 + 4 gestational age weeks presented with a grade III intraventricular hemorrhage and periventricular hemorrhagic infarction 5 days after birth. Serial transfontanellar ultrasound disclosed a progressive PHH. Progressive symptomatic PHH, pulmonary hemodynamic instability, and suboptimal general prematurity conditions were the main factors that led to plan a percutaneous transfontanellar ultrasound-guided external ventricular drainage at the neonatal intensive care unit. The illustrated procedure represents a bedside minimally invasive, effective, reversible, and sparing-time choice alternative to other temporary cerebrospinal fluid diversion techniques. This edited, 2-dimensional operative video highlights the key surgical steps of the proposed procedure ( Video 1 ). All relevant patient identifiers have been removed from the video. Nevertheless, the parent’s consent was obtained regarding the procedure, video recording, and redistribution for educational purposes.

Background: In deep brain stimulation (DBS), tunneled lead and extension wires connect the implantable pulse generator to the subcortical electrode, but circuit discontinuity and wire revision compromise a significant portion of treatments. Objective: To identify factors predisposing to fracture or tethering of the lead or extension wire in patients undergoing DBS. Method: Retrospective review of wire-related complications was performed in a consecutive series of patients treated with DBS at a tertiary academic medical center over 15 yr. Results: A total of 275 patients had 513 extension wires implanted or revised. There were 258 extensions of 40 cm implanted with a postauricular connector (50.3%), 229 extensions of 60 cm with a parietal connector (44.6%), and 26 extensions 40 cm with a parietal connector (5.1%). In total, 26 lead or extension wires (5.1%) were replaced for fracture. Fracture rates for 60 cm extensions with a parietal connector, 40 cm wires with a postauricular connector, and 40 cm extensions with a parietal connector were 0.2, 1.4, and 12.9 fractures per 100 wire-years, significantly different on log-rank test. Total 16 (89%) 40 cm extension wires with a postauricular connector had fracture implicating the lead wire. Tethering occurred only in patients with 60 cm extensions with parietal connectors (1.14 tetherings per 100 wire-years). Reoperation rate correlated with younger age, dystonia, and target in the GPI. Conclusion: The 40 cm extensions with parietal connectors have the highest fracture risk and should be avoided. Postauricular connectors risk lead wire fracture and should be employed cautiously. The 60 cm parietal wires may reduce fracture risk but increase tethering risk.

Background In the last years, few reports have shown the feasibility of the endoscopic endonasal approach (EEA) for craniopharyngiomas in pediatric patients. For these tumors, recent studies have suggested less aggressive surgery, favoring the preservation of the patient's quality of life. Objective The aim of this study was to assess the outcome of the EEA in a large series with specific attention on the long-term functional sequelae. Materials All consecutive pediatric craniopharyngiomas operated on through this approach since 2000 were included in the study. Preoperative and postoperative operative clinical, radiologic, and pathologic features were retrieved from patient records (mean follow-up, 72 ± 67 months). Results The series included 25 patients (12 female; mean age, 8.9 ± 4.1 years). Most of the tumors presented with a supradiaphragmatic extension (88%). Removal was radical in 23 patients (92%). Complications consisted of 6 cerebrospinal fluid leaks (24%). One patient (4%) died of postoperative respiratory complications. Most patients (92%) developed panhypopituitarism and visual disturbances normalized or improved in 6 patients (43%). At follow-up, 9 patients (36%) were overweight/obese (6 were already overweight before surgery). The tumor recurrence rate was 19%. Conclusions EEA can be an effective approach for midline craniopharyngiomas in children older than 3 years. It gives a satisfactory exposure of the suprasellar region and an adequate assessment of the brain–tumor interface. Its main limitations are age-related anatomic features of nasal/paranasal sinuses and the risk of cerebrospinal fluid leak.

Patients with hypothalamic hamartomas (HHs) located at the level of the mammillary bodies may present with a peculiar epileptic syndrome characterized by gelastic seizures and, commonly, falls.(1-4) Associated symptoms can include developmental delay, cognitive deterioration, psychiatric disorders, central precocious puberty, and sleep disturbances.(1,4-6) Here, we report the case of an adult patient with HH and excessive daytime sleepiness (EDS) in whom CSF analysis disclosed low levels of hypocretin-1, a finding consistently observed in patients with type 1 narcolepsy.(7)

We report the case of a 14-year-old girl with a wide non-compressive pineal cyst, associated with the inability to control her sleep-wake schedule. Actigraphic monitoring showed a 24-hour free-running disorder (tau 26.96 hours). A 24-hour serum melatonin curve assay, with concomitant video-polysomnographic and body-core temperature monitoring, was performed. Melatonin curve showed a blunted nocturnal peak, lower total quantity of melatonin, and prolonged melatonin secretion in the morning, with normal temperature profile and sleep parameters. Treatment with melatonin up to 14 mg at bedtime was initiated with complete realignment of the sleep-wake rhythm (tau 23.93 hours). The role of the pineal cyst in the aforementioned alteration of melatonin secretion and free-running disorder remains controversial, but our case supports the utility of monitoring sleep/wake, temperature, and melatonin rhythms in the diagnostic work-up of pineal cysts associated with free-running disorder.

Background:Children with intracranial hypertension are at risk for visual loss and their visual function must be closely monitored. Surgery with the insertion of a ventriculoperitoneal shunt is imperative when vision is threatened.Case Description:Herein, we report a case of a 5-year-old boy whose refractory intracranial hypertension and severe, progressive visual loss (secondary to a chronic, otogenic, right sigmoid sinus thrombosis, and a contralateral sinus tight stenosis) were resolved by a combination of continuous (6 h), locoregional, infusion of recombinant tissue plasminogen activator (rt-PA), and mechanical thrombectomy.Conclusion:The association of in loco and continuous infusion of recombinant tissue plasminogen activator (rt- PA) with mechanical thrombectomy resulted in effective in partially reopening the occluded sinus and facilitating a good clinical recovery. This combined endovascular approach may represent an alternative, less invasive, therapeutic option to surgery in children with intracranial hypertension caused by chronic cerebral venous sinus thrombosis.

The Raphe Pallidus (RPa) is a region of the brainstem that was shown to modulate the sympathetic outflow to many tissues and organs involved in thermoregulation and energy expenditure. In rodents, the pharmacological activation of RPa neurons was shown to increase the activity of the brown adipose tissue, heart rate, and expired CO2, whereas their inhibition was shown to induce cutaneous vasodilation and a state of hypothermia that, when prolonged, leads to a state resembling torpor referred to as synthetic torpor. If translatable to humans, this synthetic torpor-inducing procedure would be advantageous in many clinical settings. A first step to explore such translatability, has been to verify whether the neurons within the RPa play the same role described for rodents in a larger mammal such as the pig. In the present study, we show that the physiological responses inducible by the pharmacological stimulation of RPa neurons are very similar to those observed in rodents. Injection of the GABAA agonist GABAzine in the RPa induced an increase in heart rate (from 99 to 174bpm), systolic (from 87 to 170mmHg) and diastolic (from 51 to 98mmHg) arterial pressure, and end-tidal CO2 (from 49 to 62 mmHg). All these changes were reversed by the injection in the same area of the GABAA agonist muscimol. These results support the possibility for RPa neurons to be a key target in the research for a safe and effective procedure for the induction of synthetic torpor in humans.

To evaluate cystic dynamics in two pediatric low grade gliomas during proton beam therapy (PBT) by weekly MRI and in the follow up (FU) time. Early late toxicity was also reported. Both solid and cystic tumor’s components were drown in weekly MRI performed during proton radiation and in the FU phase. P1 case was a multiple partially resected 5 yo girl with hypothalamic pylocitic Astrocytoma no-responding after second line of chemotherapy, who acutely lost visual acuity due to cystic tumor’s progression. P2 case was a 13 yo boy with progressive mesencephalic pylocitic Astrocytoma previously treated with multi-agents chemotherapy and several surgeries. After cystic drainage both were treated with PBT at 54 Gy GCE. During proton therapy # 7 MRI for P1 and # 6 MRI for P2 patient were performed and didn’t shown cyst growth which required intervention; P1 developed two episodes of severe acute headache and both developed partial alopecia. In P1 patient both cystic and solid component progressively reduced (cyst/solid-1w +2/-15%, cyst/solid-2w -9/-22%, cyst/solid-3w -17/-28%, cyst/solid-4w -18/-38%, cyst/solid-5w -30/-43%, cyst/solid-6w -34/-44%, cyst/solid-1FU -43/-50%, cyst/solid-2FU -51/-63%). In P2 patient only cyst part progressively increased (cyst/solid-2w +7/-4%, cyst/solid-3w +36/-10%, cyst/solid-4w +58/-12%, cyst/solid-5w +73/-15%, cyst/solid-6w +90/-15%, cyst/solid-7w +94/-16%, cyst/solid-1FU +126/-37%, cyst/solid-2FU -18/-45%). P2 had transient cyst growth after PT with significant volume reduction on the second FU. After 10 months of median FU, no late side effects were noted however P1 improved visual acuity.

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

Object Low-grade tumor (LGT) is an increasingly recognized cause of focal epilepsies, particularly in children and young adults, and is frequently associated with cortical dysplasia. The optimal surgical treatment of epileptogenic LGTs in pediatric patients has not been fully established. Methods In the present study, the authors retrospectively reviewed 30 patients (age range 3–18 years) who underwent surgery for histopathologically confirmed LGTs, in which seizures were the only clinical manifestation. The patients were divided into 2 groups according to the type of surgical treatment: patients in Group A (20 cases) underwent only tumor removal (lesionectomy), whereas patients in Group B (11 cases) underwent removal of the tumor and the adjacent epileptogenic zone (tailored surgery). One of the patients, who underwent 2 operations, is included in both groups. Follow-up ranged from 1 to 17 years. Results Sixteen (80%) of 20 patients in Group A had an Engel Class I outcome. In this group, 3 of 4 patients who were in Engel Classes II and III had temporomesial lesions. All patients in Group B had temporomesial tumors and were seizure free (Engel Class I). In this series, in temporolateral and extratemporal tumor locations, lesionectomy yielded a good seizure outcome. In addition, a young age at seizure onset (in particular < 4 years) was associated with a poor seizure outcome. Conclusions Tailored resection in temporomesial LGTs was associated with excellent seizure outcome, indicating that an adequate presurgical evaluation including extensive neurophysiological evaluation (long-term videoelectroencephalography monitoring) to plan appropriate surgical strategy is advised.

Cavernous malformations of the third ventricle are uncommon vascular lesions. Evidence suggests that cavernous malformations in this location might have a more aggressive natural history due to their risk of intraventricular hemorrhage and hydrocephalus.1 The gold standard of treatment is considered to be microsurgical gross total resection of the lesion. However, with progressive improvement in endoscopic capabilities, several authors have recently advocated for the role of minimally-invasive neuroendoscopy for resecting intraventricular cavernous malformations.2-4 In this timely intraoperative video, we demonstrate the gross total resection of a third ventricle cavernous malformation that presented with hemorrhage via a right-sided trans-frontal neuroendoscopic approach.

Area V6A, a functionally defined region in the anterior bank of the parietooccipital sulcus, has been subdivided into dorsal and ventral cytoarchitectonic fields (V6Ad and V6Av). The aim of this study was to define the cortical connections of the cytoarchitectonic field V6Ad. Retrograde and bidirectional neuronal tracers were injected into the dorsal part of the anterior bank of parietooccipital sulcus of seven macaque monkeys (Macaca fascicularis). The limits of injection sites were compared with those of cytoarchitectonic fields. The major connections of V6Ad were with areas of the superior parietal lobule. The densest labeling was observed in the medial intraparietal area (MIP). Areas PEc, PGm, and V6Av were also strongly connected. Labeled cells were found in medial parietal area 31; in cingulate area 23; in the anterior (AIP), ventral (VIP), and lateral (LIP) intraparietal areas; in the inferior parietal lobule (fields Opt and PG); and in the medial superior temporal area (MST). In the frontal lobe, the main projection originated from F2, although labeled cells were also found in F7 and area 46. Preliminary results obtained from injections in nearby areas PEc and V6Av revealed connections different from those of V6Ad. In agreement with functional data, the strong connections with areas where arm-reaching activity is represented suggest that V6Ad is part of a parietofrontal circuit involved in the control of prehension, and connections with AIP specifically support an involvement in the control of grasping. Connections with areas LIP and Opt are likely related to the oculomotor activities observed in V6Ad. J. Comp. Neurol. 513

Objective: The main clinico-diagnostic features, risk factors and associated diseases of cavernous malformations (CMs), also called cavernous angiomas or cavernomas, of the central nervous system (CNS) in children are described, and the most relevant differences compared to the affected adult population are pointed out, focusing on the management of pediatric patients harboring cranial and spinal CMs. Materials: This was a retrospective study of a series of 42 children symptomatic for CMs of the cranial and spinal compartments (35 supratentorial brain lesions, 5 infratentorial and 2 in the spinal region) operated on between 1975 and 2005, with a clinical follow-up ranging from 12 to 192 months. The results were compared with those found in the most recent literature dealing with pediatric CMs of the CNS. Results: Surgical treatment produced excellent or good results in 69% of our 42 children. Unchanged neurological deficits were observed in 23.8% of cases, while morbidity from surgical procedures was 7.14%. Mortality was absent in this series. These surgical results are comparable with those found in the literature, where morbidity and mortality rates from surgery are 8.8 and 1.13%, respectively, and are mostly associated with procedures for the excision of deep, critically located cavernomas. Conclusion: CMs represent the most common CNS vascular lesion in children, although their incidence is 4 times lower than that of the adult population. The natural history of pediatric CMs throughout the neuraxis seems to be more aggressive than in adult patients; these lesions have higher rates of growth and hemorrhage, larger dimensions and often atypical radiological pictures at diagnosis. Beside the familial form of the disease, which is more often associated with multiple lesions and an earlier age of clinical presentation, the major risk factor for CMs in children seems to be radiotherapy for CNS tumors. Furthermore, a greater number of CMs coexistent with mixed angiomatous lesions have been reported in children than in adults. Surgical results are related to the preoperative neurological status of the children; symptomatic patients who are operated on early, before they develop severe neurological deficits or long-standing seizures, may achieve the best clinical outcome. Radiosurgery does not seem to be advisable in children as an alternative treatment for deep CMs or those causing epilepsy.

Background: The most appropriate management of recurrent glioblastoma is still controversial. In particular, the role of surgery at recurrence remains uncertain. Patients & methods: From our Institutional data warehouse we analyzed 270 consecutive patients who received second surgery for recurrent glioblastoma, to assess survival after second surgery, and to evaluate prognostic factors. Results: Complete resection was found in 128 (47.4%) and partial resection in 142 patients (52.6%). Median survival from second surgery was 11.4 months (95% CI: 10.0–12.7). Multivariate analysis showed that age (p = 0.001), MGMT methylation (p = 0.021) and extent of surgery (p < 0.001) are associated with better survival. Conclusion: A complete resection should be the goal for second resection and younger age and MGMT methylation status might be considered in the selection of patients.

OBJECTIVE Hydrocephalus treatment in extremely low-birth-weight (ELBW) infants still represents a challenge for the pediatric neurosurgeon, particularly when the patient weighs far less than 1000 g. In such cases, the benefits in terms of neurological outcome following early treatment do not always outweigh the surgical risks, especially considering the great difference in the surgical risk before patient weight increases. To assess the efficacy and reliability of a percutaneous-tunneled, transfontanellar external ventricular drain (PTTEVD) in ELBW infants, the authors started a new protocol for the early surgical treatment of hydrocephalus. METHODS Ten cases of posthemorrhagic hydrocephalus (PHH) in ELBW infants (5 cases < 700 g, range for all cases 550–1000 g) were treated with a PTTEVD that was implanted at bedside as the first measure in a stepwise approach. RESULTS The average duration of the procedure was 7 minutes, and there was no blood loss. The drain remained in place for an average of 24 days (range 8–45 days). In all cases early control of the hydrocephalus was achieved. One patient had a single episode of CSF leakage (due to insufficient CSF removal). In another patient Enterococcus in the CSF sample was detected the day after abdominal surgery with ileostomy (infection resolved with intrathecal vancomycin). One patient died of Streptococcus sepsis, a systemic infection existing prior to drain placement that never resolved. One patient had Pseudomonas aeruginosa sepsis prior to drain insertion; a PTTEVD was implanted, the infection resolved, and the hydrocephalus was treated in the same way as with a traditional EVD, while the advantages of a quick, minimally invasive, bedside procedure were maintained. Once a patient reached 1 kg in weight, when necessary, a ventriculoperitoneal shunt was implanted and the PTTEVD was removed. CONCLUSIONS The introduction of PTTEVD placement in our standard protocol for the management of PHH has proved to be a wise option for small patients.

Background Lymphangioleiomyomatosis is a progressive interstitial lung disease that affects young women. It has been suggested that estrogens play a role in its evolution, and progesterone therapy is often provided in these cases. Case Description We present a case of a postmenopausal woman with LAM treated with progesterone; subsequently, rapid growth of multiple intracranial meningiomas was observed. One prominent lesion was excised, and 3 other lesions regressed spontaneously over 2 years. Conclusions This is a rare case of a non–pregnancy-related regression of meningiomas in a woman affected by LAM. The significance of this association and the hormonal treatment of the disease are discussed.

Medulloblastoma belongs to the group of highly malignant neuroepithelial tumours and is the commonest tumour in childhood (average age nine years) followed by astrocytoma1. Medulloblastoma usually arises in the posterior fossa, namely the cerebellar vermis, and more seldom in the fourth ventricle, supratentorium and spinal cord. We describe the 3 Tesla magnetic resonance (MR) features of a medulloblastoma located in the right cerebellar hemisphere adhering to the tentorium in a 16-year-old male.

Low-grade tumor (LGT) is an increasingly recognized cause of focal epilepsies, particularly in children and young adults, and is frequently associated with cortical dysplasia. The optimal surgical treatment of epileptogenic LGTs in pediatric patients has not been fully established.In the present study, the authors retrospectively reviewed 30 patients (age range 3-18 years) who underwent surgery for histopathologically confirmed LGTs, in which seizures were the only clinical manifestation. The patients were divided into 2 groups according to the type of surgical treatment: patients in Group A (20 cases) underwent only tumor removal (lesionectomy), whereas patients in Group B (11 cases) underwent removal of the tumor and the adjacent epileptogenic zone (tailored surgery). One of the patients, who underwent 2 operations, is included in both groups. Follow-up ranged from 1 to 17 years.Sixteen (80%) of 20 patients in Group A had an Engel Class I outcome. In this group, 3 of 4 patients who were in Engel Classes II and III had temporomesial lesions. All patients in Group B had temporomesial tumors and were seizure free (Engel Class I). In this series, in temporolateral and extratemporal tumor locations, lesionectomy yielded a good seizure outcome. In addition, a young age at seizure onset (in particular4 years) was associated with a poor seizure outcome.Tailored resection in temporomesial LGTs was associated with excellent seizure outcome, indicating that an adequate presurgical evaluation including extensive neurophysiological evaluation (long-term videoelectroencephalography monitoring) to plan appropriate surgical strategy is advised.

Object The four types of cerebrovascular malformations may sometimes be combined and more often occur in pairs; triads are exceptional. The authors present six patients with the clinicoradiographic profile of mixed vascular malformations of the brainstem, including cavernous malformation (CM), capillary telangiectasia, and developmental venous anomaly (DVA). Methods Five patients (one of whom was a child) suffered from hemorrhage, suggesting that this complex association has a high bleeding potential. Progressive growth, rebleeding, and de novo occurrence of the associated CM were documented in three cases. Magnetic resonance imaging of the brain was obtained in all patients by using one or more of the following modalities: T1-weighted sequences before and after gadolinium administration; T2-weighted sequences; T2-weighted fluid attenuated inversion recovery; T1-weighted fast spin echo; and diffusion weighted, diffusion tensor, and perfusion imaging in three cases. Results Three patients were surgically treated with the intention of excising the hemorrhagic lesion, but only two patients had their malformations successfully removed. In the third case, diffuse pontine telangiectasia precluded the safe excision of the CM. Histological examination demonstrated a blended pathological milieu characterized by coalescent telangiectasia and venules associated with loculated endothelial chambers resembling an immature or de novo CM. Three patients were treated conservatively; recurrent minor hemorrhage occurred in one case. The authors found these malformations to be arranged in two basic relationships: CM inside the telangiectasia and CM in the radicles of the DVA. Stenosis of the main venous collector and dilation of the medullary veins were important findings. Conclusions The pathogenesis of this malformation may be referred to a developmental deviance of the brainstem capillary–venous network associated with transitional vessels and loculated endothelial vascular spaces related to genetic and acquired origins, probably in a restrictive venous outflow milieu.

Cavernomas and cortical dysplasia are well-known causes of partial epilepsy. The association between cavernoma and cortical dysplasia in an epileptic patient has not yet been sufficiently documented. We report a case of long-term, drug-resistant, partial left temporal epilepsy associated with a cavernoma. According to the neurophysiological non-invasive presurgical study, the patient was submitted to a tailored left temporal resection. The histopathological study showed the coexistence of cavernoma and cortical dysplasia. This is an interesting combination of epilepsy-related pathologies that have so far not been documented.

Calcification of the ligamentum flavum is a rare manifestation of the calcium pyrophosphate dihydrate deposition disease (CPPD). In CPPD deposition disease, spinal involvement is rare. Until now, thoracic spine CPPD causing thoracic cord compression has been reported in only sporadic cases. We report a new case of thoracic calcification of the ligamentum flavum. In our case, similar to the other reported cases, an affected middle-aged woman despite the clinical and MRI signs of myelopathy had an unexpected important and rapid improvement of the neurological picture. This condition should be considered in differential diagnosis of thoracic cord compression to offer the patient an early and useful surgical treatment.

✓The risk of hemorrhage in cavernous malformations (CMs) depends on lesion, host, and environmental factors. Anticoagulation therapy is a well-known risk factor for intracerebral bleeding, but the occurrence of hemorrhages in patients with CMs has not been reported. Low molecular weight heparin therapy is generally considered to be safe, although significant hemorrhagic complications have recently been reported. The authors report a case of intralesional bleeding in a CM after prophylactic anticoagulation therapy was administered in a patient with the familial form of the disease. The leaky endothelial structure of CMs may constitute an unexpected target of the vascular effects of heparin.

Annulotomy is a mandatory step to perform intradiscal decompression to resolve a disco radicular conflict. However, this manoeuvre can lead to post surgical complications such as vertebral instability and back pain. Coblation assisted microdiscectomy (CAM procedure) allows a quoted removal of disc without anulus damage.

Object. Glioneuronal tumors (ganglioglioma, dysembryoplastic neuroepithelial tumors [DNTs]) are commonly associated with partial seizures. The optimal surgical treatment of such tumors, however, has not been fully established; it is still unclear whether lesionectomy itself can be used to control seizures or if epileptogenic areas adjacent to the tumor should also be removed. To address this uncertainty, the authors analyzed seizure outcome in a series of children with epileptogenic glioneuronal tumors that had been treated only by lesionectomy. Methods. The authors retrospectively reviewed 15 children surgically treated for glioneuronal tumors associated with epilepsy. Patients ranged in age from 3 to 18 years (mean 12.6 years); there were 12 boys and three girls. The interval between onset of seizures and surgery ranged from 0.5 to 16 years (mean 6.1 years). Ten patients (66.6%) suffered complex partial seizures and five (33.3%) simple partial seizures. Seizure frequency varied from several per day to one per month. Nine tumors (60%) were temporal and six extratemporal; in all patients resection was limited to the tumor. The follow-up duration ranged from 1 to 11 years (mean 5.6 years). Gross-total removal was achieved in 13 patients and subtotal in two. The histological diagnosis was ganglioglioma in 11 cases and DNT in four. At last follow up 13 patients (86.6%) were Engel Class I, one was Engel Class II, and one was Engel Class III. Conclusions. The results of this study indicate that lesionectomy may provide good long-term seizure control in the majority of children with epileptogenic glioneuronal tumors.

We retrospectively analysed seizure outcome and surgical results in a series of 21 patients with ganglioglioma treated with lesionectomy. The 21 patients (13 males, eight females) had a history of epilepsy longer than 1 year and post-operative follow up of at least 1 year. Information on the duration of the seizures, type and frequency was retrieved from medical records. In all patients, surgery was limited to the tumour. The interval between onset of seizures and surgery ranged from 1 to 35 years (mean 11). Patient age ranged from 6 to 61 years (mean 27.5). Fifteen patients (71.4%) had complex partial seizures and six had simple partial seizures. Secondary generalisation was present in 10 patients (47.6%). Seizure frequency varied from several per day to one per month. Sixteen tumours were temporal (76.1%; 11 temporo-mesial, five temporo-lateral), and five were extratemporal (23.8%). The mean follow-up period was 5.4 years (range: 1.25–10 years). No tumour progression was observed. No patient died during surgery or the post-operative course. Fourteen patients (66.6%) were Engel class I (five temporo-mesial, five temporo-lateral, four extratemporal) and seven (33.3%) were Engel class II. Post-operative complications were observed in six patients (28.6%), two of whom had cerebellar haemorrhage, possibly due to increased transmural venous pressure. In our patients with temporal neocortical and extratemporal ganglioglioma, lesionectomy allowed good seizure control. The unsatisfactory outcome for mesiotemporal gangliogliomas might indicate the need for extensive neurophysiological presurgical evaluation in order to perform tailored surgery. To avoid cerebellar haemorrhage, attention should be paid to those factors involved in transmural venous pressure increases.

Resume La calcification du ligament jaune est une des manifestations rares de la chondrocalcinose. L'atteinte rachidienne est rare au cours de la chondrocalcinose. Jusqu'a present, seuls quelques cas sporadiques de chondrocalcinose thoracique responsables de compression medullaire, avaient ete rapportes. Nous rapportons un nouveau cas de calcification thoracique du ligament jaune. Ce cas est similaire aux autres cas decrits. Il s'agit d'une femme d'âge moyen qui a presente une amelioration importante, rapide et inattendue de son tableau neurologique malgre la presence de signes cliniques de myelopathie et de signes a l'IRM. Ce diagnostic differentiel doit donc etre evoque pour pouvoir proposer un traitement chirurgical precoce et utile aux patients.