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Background Laminotomy has been introduced in surgical practice to reduce complications of laminectomy after surgery of tumors in the spinal canal. However, the posterior ligament complex, which is routinely interrupted to remove the laminoplasty segment and gain access to the spinal canal, has a tendency not to heal and can lead to progressive kyphosis and collapse. Case presentation A 5-month-old boy affected by a thoracolumbar extradural tumor extending along seven spinal levels was operated on. The tumor was exposed and completely resected by a one-piece laminotomy with preservation of the integrity of the posterior tension band at both extremities. After 1-year radiological examination ruled out spinal deformity. Conclusion The technique herein presented, which we named in situ laminotomy, allows to fully preserve the posterior tension band without reducing the exposure of the spinal canal in multilevel tumors. Additionally, the technique makes also the reconstruction of the spine elements very easy and rapid. However, longer follow-up is necessary to prove the effectiveness of this procedure in preventing long-term deformity and instability.

Background The enlargement of the posterior cranial fossa volume is considered one of the main steps of the surgical management of children with multiple sutures craniosynostosis. Different management options have been proposed including fixed expansive craniotomy, free bone flap craniotomy, and distraction osteogenesis. Objectives To review indications to “free bone flap” craniotomy for the posterior fossa expansion, detailing advantages, disadvantages, and complications related to the technique. Results and conclusions A review of the literature shows that “free bone flap” posterior expansion cranioplasty still has a role, particularly in infants with thin and “honeycomb” structure of the bone, allowing to gain adequate intracranial volume increases and to postpone to a more adequate time surgery aimed at anterior cranial fossa expansion.

To investigate the current practice of neurosurgeons and their perception of complications related to the securement of external drainage (ED) to the skin.We created a 24-points English language questionnaire on Google Forms covering the five main domains of care. The survey was distributed among members of the European Society for Pediatric Neurosurgery (ESPN) in April 2020.The results were entirely self-reported, without any independent validation. Fifty-one neurosurgeons practising in different centres worldwide participated in this survey. Despite well-known complications and drawbacks, sutures are still the most commonly used method to secure cerebrospinal fluid (CSF) ventricular ED (49 out of 51 respondents) and spinal ED (37 out of 51) to the skin. Perception of the risk of pullout is estimated as1% by 25.5% of the respondents, 1-5% by 39.2%, 5-10% by 17.6% and 10% by 11.8%. Twenty out of fifty-one respondents acknowledge that their method of securement has drawbacks, and 49% believe that it may also affect the risk of infection. Factors eventually affecting the risk of pullout are young age (62.7%), aetiology (25.5%), neurological status (90.2%), occipital exit site (37.3%), inadequate length of the subcutaneous tunnel (58%), the duration of ED (70.6%), and hospital stay in service (84.3%). 39.2% of respondents agree that the paediatric population deserves a different device or technique to secure ED to the skin. 21.6% of respondents underestimate the risk of accidental pullout. 86.3% of respondents have never read about the 'sutureless subcutaneous anchoring device'.Complications associated with the securement method, such as the risk of pullout and infection, are most likely underestimated. More research is needed to implement effective guidelines in this field.

Background Gelfoam is a simple and effective hemostatic agent, which is utilized to seal brain corticotomies or skull burr holes too. Thanks also to its cheap costs, it is one of the most widely used tool in the neurosurgical daily practice. However, the migration of Gelfoam fragments can account for the occlusion of endoscopic third ventriculostomy (ETV) or shunt, leading to hydrocephalus recurrence. Case description The authors report on two cases of Galfoam migration causing recurrent hydrocephalus: a young girl, operated on by posterior fossa tumor removal and ETV for associated hydrocephalus, where part of the Gelfoam (used to seal the burr hole) migrated up to close the ETV; and a preterm boy, treated by endoscopic brain wash and, afterwards, by ventriculo-peritoneal shunt for post-hemorrhagic hydrocephalus, where all the Gelfoam used to close the corticotomy migrated into the lateral ventricle, thus reopening the corticotomy and releasing small fragments which finally obstructed the shunt. Both patients needed a new endoscopic procedure (the second one also a shunt revision). Conclusions The reviewed pertinent literature discloses other complications of Gelfoam migration (mass effect, granulomatous reaction) as well as other causes of uncommon ETV/shunt obstruction. Anyway, Gelfoam will remain an irreplaceable tool for neurosurgeons. The present paper just emphasizes the need of its correct use to avoid complications.

Background A comparison of clinical and radiological outcomes after two different variants of open door laminoplasty for cervical spondylotic myelopathy (CSM) is described. Methods Fifty-five patients underwent cervical laminoplasty for CSM. Between 2005 to 2009, 32 patients underwent open-door laminoplasty (Group A) performed with a monolateral exposure with contralateral "green stick" laminar fracture. A technical variant consisting in bilateral exposure, spinous process removal and symmetrical muscular closure was applied in the other 23 (Group B), from 2009 to 2016. A retrospective analysis of surgical results between the two groups was performed. Results In group B, the mean operative time was longer and the mean volume of intraoperative blood loss was much higher than in group A. Both differences were not significant (p=0.1601 and p=0.0884, respectively). The average hospitalization was nonsignificantly longer in group B (7.3±1.8 vs. 6.5±1.6 d; p= 0.0947). Postoperatively, axial neck pain was more severe in group A at last follow-up than preoperatively (P>0.05). mJOA scores and Nurick grades improved significantly in both groups at last follow-up; in group A, the mean recovery rates were 41.5 % by mJOA scores and 29.4 % by Nurick grades, whereas in group B they were 52.7 % and 36.8 %, respectively. Conclusions Applying some few variations to a standard monolateral approach, although slightly more time and blood consuming, could reduce the incidence of long term axial symptoms and, in our experience, are not burdened by worsening of outcomes. A prospective, multicentric study with a longer follow-up should be conducted in order to confirm our findings.

Background Causes of the nonaneurysmal subarachnoid hemorrhage (na-SAH) are still debated. Many studies confirmed the possible involvement of the intracranial venous system, in particular variants of the basal vein of Rosenthal. Study Object The aim of this study is to investigate the role of extracranial venous system, in particular the jugular drainage, in the na-SAH pathophysiology. Materials and Methods This is a multicenter retrospective study including patients suffering from na-SAH who were radiologically screened to exclude vascular malformations. The course of the internal jugular veins was evaluated to reveal any stenosis (caliber reduction >80%). Particular attention was paid at the passage between the styloid process and the arch of C1 to detect possible compression. As a control group, we enrolled patients who underwent CT angiograms and/or cerebral DSAs in the past 2 years. Results We included 194 patients. The na-SAH group consisted of 94 patients, whereas the control group consisted of 100 patients. Fifty patients of the control group underwent a CT angiography for an ischemic cerebrovascular disease or trauma and 50 patients for an SAH due to a ruptured aneurysm. A significant jugular stenosis was found in 49 (52.1%) patients in the na-SAH group and in 18 (18%) patients in the control group. At univariate and multivariate analysis, the difference was statistically significant (p Conclusions This is the first study investigating the correlation between jugular stenosis and the occurrence of na-SAH. The impaired venous outflow due to reduced venous caliber could result in an engorgement of the upstream intracranial veins with transient hypertensive phases facilitating ruptures. Further larger prospective studies are necessary to confirm these data.

In spite of literature data stating that children with single-suture craniosynostosis have an increased risk for neuropsychological deficits, no data are present clarifying the potential risk factors. All children with non-syndromic single-suture craniosynostosis operated on from January 2014 to January 2017 were enrolled. A comprehensive neurocognitive and neuro-ophthalmological evaluation was performed before surgery and 6 months after surgery. A further neurocognitive evaluation was performed 12 months after surgery. All children had a preoperative CT/MR study. One hundred forty-two patients were enrolled; 87 are affected by sagittal craniosynostosis, 38 by trigonocephaly, and 17 by plagiocephaly. A global neurocognitive impairment was documented in 22/87 children with scaphocephaly, 5/38 children with trigonocephaly, and 6/17 children with anterior plagiocephaly. There was a significant relationship between results of the ophthalmological evaluation, global IQ, and CT findings at diagnosis (r = 0.296, p

Purpose The assessment of Autism Spectrum Disorders (ASD) in childhood has two essential aspects: the identification of the risk (under 30 months of age) and the definition of a diagnosis that takes into account its core areas as well as further non-specific aspects. The purpose of this paper is to present an approach that considers the combination of clinical evaluation with the use of tools that analyse the various levels of the child’s functioning as fundamental. Design/methodology/approach The comprehensive assessment at the Institute of Ortofonologia in Rome provides the ADOS-2 and the Leiter-R for the evaluation of the symptomatology, the severity level, the non-verbal cognitive functioning and the fluid reasoning; the TCE and the UOI are used to identify, respectively, the child’s emotional skills and the ability to understand the intentions of others, as precursors of the theory of mind. Within this assessment, the Brief-P, the Short Sensory Profile and the RBS are also included for the evaluation of executive functions, sensory pattern and of restricted and repetitive behaviours, as observed by parents. Findings How to define a reliable development profile, which allows to plan a specific intervention calibrated on the potential of the child and on his development trajectory, is described. Two clinical cases are also presented. Originality/value The entire process is aimed both at a detailed assessment of the child’s functioning and at identifying a specific therapeutic project and predictive factors for achieving an optimal outcome.

Infection may complicate the outcome of cranial repair with significant additional morbidity, related to hospitalization, surgery and long antibiotic therapy, that may become even dramatic in case of multi-resistant germs and in particular in the paediatric population. Additionally, the economic costs for the health system are obvious. Moreover, surgical decisions concerning the timing of cranioplasty and choice of the material may be strongly affected by the risk of infection. Despite, management and prevention of cranioplasty infections are not systematically treated through the literature so far. We reviewed pertinent literature dealing with cranioplasty infection starting from the diagnosis to treatment options, namely conservative versus surgical ones. Our institutional bundle, specific to the paediatric population, is also presented. This approach aims to significantly reduce the risk of infection in first-line cranioplasty and redo cranioplasty after previous infection. A thorough knowledge and understanding of risk factors may lead to surgical strategies and bundles, aiming to reduce infectious complications of cranioplasty. Finally, innovation in materials used for cranial repair should also aim to enhance the antimicrobial properties of these inert materials.

Background Piezosurgery (PS) has gained increasing dispersion in neurosurgery. In pediatric neurosurgery, the experience is limited to craniosynostosis surgery. The present study assesses PS in the pediatric population, also considering outcomes and complications in cranial and spinal procedures. Methods All consecutive craniotomies and laminotomies, performed with PS (group A) or conventional osteotomes (group B) in the 2014–2017 period were reviewed. The following variables were analyzed: dural tears, estimated blood loss and need of transfusion, cosmetic outcome (Sloan score), and operative times. A review of the pertinent literature is included. Results 172 children were enrolled, 90 in group A and 82 in group B. The mean follow-up time was 2.1 years. A statistically significant difference in favor of group A was found about EBL (105 vs. 113 ml) and late outcome (Sloan class A 98.5% vs. 91.5%). PS also reduced the risk of dural tears (1 vs. 7 cases in groups A and B, respectively) and blood transfusion (52% vs. 55.5%) but without statistical significance. The operative times were significantly shorter in group B (13 vs. 23 minutes), although the newer PS plus (PSP) was demonstrated to significantly shorten these times compared with the traditional PS (3.5 vs. 6.5 minutes for orbitotomy and 7.5 vs. 9.5 minutes for hemicraniotomy). Conclusions PS is a safe and effective tool that can be specifically recommended for bone splitting and graft, laminotomy, and craniotomy in cosmetically eloquent areas. The limit of operation times can be overcome by a learning curve in neurosurgery and PSP.

Background The optimal management of neonatal post-hemorrhagic hydrocephalus (PHH) is still debated, though several treatment options have been proposed. In the last years, ventriculosubgaleal shunt (VSgS) and neuroendosdcopic lavage (NEL) have been proposed to overcome the drawbacks of more traditional options, such as external ventricular drainage and ventricular access device. Methods We retrospectively reviewed neonates affected by PHH treated at our institution since September 2012 to September 2020. Until 2017 patients received VSgS as initial treatment. After the introduction of NEL, this treatment option was offered to patients with large intraventricular clots. After NEL, VSgS was always placed. Primary VSgS was reserved to patients without significant intraventricular clots and critically ill patients that could not be transferred to the operating room and undergo a longer surgery. Results We collected 63 babies (38 males and 25 females) with mean gestational age of 27.8 ± 3.8SD weeks (range 23–38.5 weeks) and mean birthweight of 1199.7 ± 690.6 SD grams (range 500–3320 g). In 6 patients, hemorrhage occurred in the third trimester of gestation, while in the remaining cases hemorrhage complicated prematurity. This group included 37 inborn and 26 outborn babies. Intraventricular hemorrhage was classified as low grade (I–II according to modified Papile grading scale) in 7 cases, while in the remaining cases the grade of hemorrhage was III to IV. Mean age at first neurosurgical procedure was 32.2 ± 3.6SD weeks (range 25.4–40 weeks). Death due to prematurity occurred in 5 patients. First-line treatment was VSgS in 49 patients and NEL in the remaining 14 cases. Mean longevity of VSgS was 30.3 days (range 10–97 days) in patients finally requiring an additional treatment of hydrocephalus. Thirty-two patients required one to three redo VSgS. Interval from initial treatment to permanent shunt ranged from 14 to 312 days (mean 70.9 days). CSF infection was observed in 5 patients (7.9%). Shunt dependency was observed in 51 out of 58 surviving patients, while 7 cases remained shunt-free at the last follow-up. Multiloculated hydrocephalus was observed in 14 cases. Among these, only one patient initially received NEL and was complicated by isolated trapped temporal horn. Conclusions VSgS and NEL are two effective treatment options in the management of PHH. Both procedures should be part of the neurosurgical armamentarium to deal with PHH, since they offer specific advantages in selected patients. A treatment algorithm combining these two options may reduce the infectious risk and the risk of multiloculated hydrocephalus.

Introduction Electrical source imaging (ESI) and especially hdEEG represent a noninvasive, low cost and accurate method of localizing epileptic zone (EZ). Such capability can greatly increase seizure freedom rate in surgically treated drug resistant epilepsy cases. Furthermore, ESI might be important in intracranial record planning. Case report We report the case of a 15 years old boy suffering from drug resistant epilepsy with a previous history of DNET removal. The patient suffered from heterogeneous seizure semiology characterized by anesthesia and loss of tone in the left arm, twisting of the jaw to the left and dysarthria accompanied by daze; lightheadedness sometimes associated with headache and dizziness and at a relatively short time distance negative myoclonus involving the left hand. Clinical evidence poorly match scalp and video EEG monitoring thus requiring hdEEG recording followed by SEEG to define surgical target. Surgery was also guided by ECoG and obtained seizure freedom. Discussion ESI offers an excellent estimate of EZ, being hdEEG and intracranial recordings especially important in defining it. We analyzed our results together with the data from the literature showing how in children hdEEG might be even more crucial than in adults due to the heterogeneity in seizures phenomenology. The complexity of each case and the technical difficulties in dealing with children, stress even more the importance of a noninvasive tool for diagnosis. In fact, hdEEG not only guided in the presented case SEEG planning but may also in the future offer the possibility to replace it.

The space existing between the external and internal dural layer has been anatomically described as a virtual space; no clear clinical significance has been given to it to date. We hereby describe a case of a child with what was expected to be a purely epidural hematoma, at surgery, was found to be composed of two equally coexistent components, one in the epidural space and the second one between the two dural layers. The recognition of a possible involvement of the transdural space in the case of post-traumatic epidural hematomas is relevant for a correct conclusion of the surgical treatment of epidural hematomas, extensively considered basic practice among neurosurgical procedures.

Aim To analyze the current literature on neurocutaneous melanosis (NCM), as well as, our cases, in order to better define the prognosis and the presence of risk factors affecting it, thus, offering better information to the parents. Material and methods Two cases observed at the Pediatric Neurosurgery Unit of the Catholic University Medical School in Rome are described. Both of them had cutaneous stigmata and cerebral MR evidence of intracranial melanin deposits. These two children showed a very different clinical course. Results The present study enlighten the differences among the two cases and review the literature on the subject, with the attempt to understand which are clinical and disease related factors that might influence the prognosis. Conclusion Beside malignant features of cutaneous melanotic lesions, the presence of hydrocephalus at diagnosis and the early appearance of clinical symptoms, when appearing contemporarily, are predicting the rapid progression of the disease and a worse prognosis.

Securing the catheter to the skin either with sutures or staples and to the skull with bolt system still represents the most common options in the management of CSF external drainage. However, these options bear an unavoidable risk of complications. This problem is common to vascular accesses and has been successfully overcome with the introduction of device for subcutaneous anchoring (SecurAcath®, Interrad Medical, Inc., Plymouth, Minnesota), which has rapidly become the standard of care in this field. We report our experience with the use of SecurAcath® to secure CSF drainage, either ventricular or spinal. Results were compared with literature data. Since 2015, SecurAcath® was used in 209 patients (mean age 7 years) to secure 195 external cranial catheters (either ventricular or subdural or intralesional) and 16 spinal drainages. Indwell time ranged from 5 to 30 days. No complication related to the use of the device was observed. In particular, there was no case of dislocation or accidental pullout of the catheter. Rate of infection, or superinfection in case of ventricular catheter implanted for CSF infection, was null. SecurAcath® is a safe and effective device to secure CSF external catheters, with several relevant advantages, including easy placement and maintenance. Moreover, it may stay in place for the whole duration of the catheter without any skin tissue trauma and allows a complete antisepsis of the exit site, thus reducing local skin complications. This factor has significant impact on the reduction of infection rate of external CSF catheters.

Purpose Sturge-Weber syndrome (SWS) is a neurocutaneous facomatosis characterized by facial and leptomeningeal angioma, glaucoma, seizures, and neurological disability. Therefore, a challenging multidisciplinary interaction is required for its management. The goal of this paper is to review the main aspects of SWS and to present an illustrative pediatric series. Methods The pertinent literature has been analyzed, focused mainly on etiopathogenesis, pathology, clinical features, diagnostic tools, management, and outcome of the disease. Moreover, a series of 11 children operated on for refractory epilepsy between 2005 and 2015 (minimum follow-up 5 years, mean follow-up 9.6 years) is reported. The series consists of six boys and five girls with 6.5-month and 16.2-month mean age at seizure onset and at surgery, respectively. Seizures affected all children, followed by hemiparesis and psychomotor delay (81%), glaucoma (54%), and other neurological deficits (45%). Results All children underwent hemispherectomy (anatomical in three cases, functional in two cases, hemispherotomy in six cases); one patient needed a redo hemispherotomy. Mortality was nil; disseminated intravascular coagulation and interstitial pneumonia occurred in one patient each; three children had subdural fluid collection. Eight patients (72%) are in the ILAE Class 1 (completely seizure and aura free), two in Class 2 (only auras, no seizure), and one in Class 3 (1-3 seizure days per year). AEDs discontinuation was possible in 73% of cases. The most important news from the literature concerned the pathogenesis (role of the mutation of the GNAQ gene in the abnormal SWS vasculogenesis), the clinical findings (the features and pathogenesis of the stroke-like episodes are being understood), the diagnostic tools (quantitative MRI and EEG), and both the medical (migraine, seizures) and surgical management (epilepsy). The epileptic outcome of SWS patients is very good (80% are seizure-free), if compared with other hemispheric syndromes. The quality of life is affected by the neurological and cognitive deficits. Conclusions SWS still is an etiological and clinical challenge. However, the improvements over the time are consistent. In particular, the neurosurgical treatment of refractory epilepsy provides very good results as long as the indication to treatment is correct.

CustomBone Service (CBS) is a patient-specific, biocompatible, and osteoconductive device made of porous hydroxyapatite, indicated for cranial reconstruction in adults and children. Adult literature data report a failure rate of about 8%. The aim of this Post-Marketing Surveillance study is to verify the hypothesis that CBS in children aged 7–13 years old shows a failure rate not superior to adults. Inclusion criteria were age at implantation ranging 7–13 years old, with at least 1 year elapsed from the date of surgery. The degree of satisfaction of surgeons and patients was assessed. Data about 76 implants in 67 patients (M:F = 41:26) were obtained from 28 centers across 7 European countries. The mean age at surgery was 10.03 ± 1.72 years, with age stratification almost equally distributed. Fifty-nine subjects received one CBS, 7 subjects two and one subject received three CBS. Main etiologies were trauma (60.5%), malformation (11.8%), bone tumor (10.5%), and cerebral tumor (7.9%). Main indications to CBS were decompression (47.4%), autologous bone resorption (18.4%), tumor resection (11.8%), malformation (9.2%), comminuted fracture (5.3%), and other materials rejection (5.3%). Main implantation sites were fronto-parieto-temporal (26.3%), parietal (23.7%), frontal (11.8%), fronto-temporal (10.5%), and parieto-temporal (7.9%). CBS was chosen as first line of treatment in 63.1% of the cases. Mean follow-up was about 36 months. Eleven adverse events (14.5%) were reported in nine devices. Five CBS required explantation (three cases of infection, one fracture, and one mobilization). Failure rate was 6.58%, which is statistically not superior to the explantation rate recorded in adults (two-sided 95%, CI 2.2–14.7%). Satisfaction of surgeons and patients was of about 95%. CBS is a safe and effective solution for cranial repair in pediatric patients. In particular, over the age of 7, CBS shows a rate of failure as low as in adults.

Primary central nervous system (CNS) neuroblastoma is a rare intracranial tumor affecting children mainly in the first years of life. It is usually a supratentorial tumor with a wide spectrum of clinical presentation, seizures, and focal neurological deficits being the most common presenting signs. A 2-year-old child was admitted to our ward after a generalized seizure. Neurological examination was normal. Radiological studies showed a small DWI hyperintense lesion of the right rectus gyrus. Follow-up brain MRI 8 months later showed a huge growth of the tumor (90 × 80 × 65 mm) with polycyclic and apparently defined margins, cystic components, and diffuse contrast enhancement. Complete tumor removal was performed in two planned surgical steps. Histological diagnosis was CNS neuroblastoma. At a follow-up of 8 months, the child is in good clinical and neurological condition and is completing chemotherapy treatment according to the SIOP PNET 4 protocol. A thorough review of the literature confirms that primary CNS neuroblastoma has to be considered a distinct entity. The disease related mortality is 12.5%, lower than the one usually reported for other previously described as PNETs tumors. The most relevant factors influencing prognosis are the possibility of obtaining a complete tumor removal and age more than 3 years, which allows to include radiotherapy among treatment options.

Residual and recurrent intracranial aneurysms after surgical clipping present a persistent risk of bleeding. Secondary coiling after incomplete clipping represents a strategy to occlude the residual sac: feasibility, bleeding risk and outcome were evaluated through a systematic review of literature along with the series of two tertiary referral neurovascular centres. Demographics, ruptured status, aneurysm morphology, topography, exclusion at surgery, timing of secondary coiling, complications, occlusion rate and outcome were analysed. Percentage of incidence and 95% CI were calculated for all variables. T test was used for continue variables, whereas Fisher's test (two-sided) is for categorical ones. Overall, 102 patients (92 cases from literature and 10 cases from institutional series) were included. Mean age at diagnosis was 52.94 ± 12.17 years, and male/female ratio 0.5; 3/4 of aneurysms involved the anterior circulation, whereas ¼ the posterior circulation. An aneurysmal neck remnant was described in 58.43% of cases, an aneurysmal sac remnant in 29.21% and a regrowth in 12.36%. Residual aneurysm rupture was reported in 22% of cases. Complete/near-complete occlusion after secondary coiling was observed in 70% of cases, a partial in 25.56% and a failure in 4.44%. Only one case of perforation was reported. Complications were comparable to standard endovascular procedures. Aneurysms remnants after clipping are often observed in cases difficult anatomical locations. Their bleeding risk is not negligible. Secondary coiling is a rescue strategy to effectively and safely secure the aneurysm remnant. Only in a minority of cases, it is a staged treatment after 'remodelling' of the aneurysm neck.

Background Postoperative seizures (PSs) after neurosurgical operations are common but little is known about the role of surgical brain incision on their genesis. This topic has not been addressed so far. Objective To verify if the corticotomy affects the risk of PSs and postoperative epilepsy (PE) in children. Methods One hundred forty-three consecutive pediatric cases operated on for supratentorial lesions at the same institution in the last 15 yr have been retrospectively reviewed by dividing them into group A, 68 children who required brain corticotomy mainly for hemispheric tumors, and group B, 75 children treated through extracortical approaches mainly for suprasellar and optic tumors. Patients with possible "epileptic" biases, like preoperative seizures, were excluded. Results No significant differences have been found between group A and B as far as incidence of PSs (11.7% vs 14.5%) and PE (4.5% vs 6.5%), timing, and type of seizures are concerned after a mean 6.8 yr follow-up. The size of corticotomy in group A ( 3 cm2) had no impact on epileptogenesis as well as the other variables considered in both groups (age, sex, extent of lesion resection). Conclusion This study shows that the surgical cortical "trauma" would not represent a risk factor for PSs and PE. According to the present analysis and the literature, other causes seem to be involved (namely, electrolytic imbalance and brain gliosis). This information is important for preoperative surgical planning and postoperative management. A validation by both adult series and prospective studies is needed.

Postnatal growth of neurocranium is prevalently completed in the first years of life, thus deeply affecting the clinical presentation and surgical management of pediatric neurosurgical conditions involving the skull. This paper aims to review the pertinent literature on the normal growth of neurocranium and critically discuss the surgical implications of this factor in cranial repair. A search of the electronic database of Pubmed was performed, using the key word “neurocranium growth”, thus obtaining 217 results. Forty-six papers dealing with this topic in humans, limited to the English language, were selected. After excluding a few papers dealing with viscerocranium growth or pathological conditions not related to normal neurocranium growth 18 papers were finally included into the present review. The skull growth is very rapid in the first 2 years of life and approximates the adult volume by 7 years of age, with minimal further growth later on, which is warranted by the remodeling of the cranial bones. This factor affects the outcome of cranioplasty. Thus, it is essential to consider age in the planning phase of cranial repair, choice of the material, and critical comparison of results of different cranioplasty solutions.

Cranioplasty aims at restoring the physiological integrity and volume of the skull. Any disproportion between the intracranial content and the volume of the container may favor the occurrence of complications. A classification of volume mismatches is proposed. A negative mismatch, consisting of intracranial content minor to skull volume, is well represented by the sinking flap. On the other side, a positive mismatch, consisting of intracranial content higher than skull volume, usually depends on CSF collection or hydrocephalus once the brain edema is regressed. Though, in children, this condition may result from physiological brain growth after decompressive craniectomy. Treatment algorithm based on this classification is presented. A 1-year-old boy with a severe traumatic brain injury underwent right decompressive craniectomy, evacuation of subdural hematoma, and dural expansion at another institution. After failure of autologous bone-assisted cranioplasty for infection, a helmet was recommended in order to postpone the cranial repair. Patient was admitted to our institution 3 years later. CT scan showed brain herniation through the cranial defect, associated to a condition of acquired craniocerebral disproportion, due to the condition of “open skull”. Augmented hydroxyapatite cranioplasty (CustomBone, Finceramica, Faenza, Italy) was performed in order to manage this rare condition of positive volume mismatch. Subsequent course was uneventful and no complication was recorded at 30-month follow-up. This illustrative case highlights the possible occurrence of a positive structural mismatch between the skull and the intracranial content after decompressive craniectomy, thus configuring a condition of acquired craniocerebral disproportion, aside of other brain or CSF complications. We firstly recognize this condition in the literature and propose it as a possible factor affecting the outcome of cranioplasty in infants and young children.

Desmoplastic infantile gangliogliomas (DIGs) and desmoplastic infantile astrocytomas (DIAs) are tumors typical of the infantile age. A large size, with a mixed solid and cystic component, clinical presentation with progressing signs of increased intracranial pressure, a prominent benign desmoplastic structure at histological examination, and a favorable clinical course in the majority of cases are the prominent features of these tumors. The objective of the present paper was to review the pertinent literature on the topic together with our personal experience, with the aim of an updated review of the subject. Only 28 papers are present in the literature devoted to DIGs and DIAs, most of them reporting on single cases or small series, with a total of 107 patients aged from 5 days to 48 months with a slight male prevalence. Most of the reported cases refer to supratentorial and hemispheric locations, a few cases involving the hypothalamic region, the posterior fossa, and the spinal cord. The typical MRI appearance is of large mixed solid and cystic tumors with a spontaneous hyperintense T2 appearance of the solid part which also shows a strong contrast enhancement. Mixed ganglionic and astrocytic cells are identifiable in DIGs, whereas DIAs are typically featured by the exclusive presence of glial cells. In both cases, more primitive cells may be observed, which present a higher number of mitoses and these areas can mimic the features of malignant astrocytomas. Surgery represents the treatment of choice; however, radical removal has been reported as possible only in around 30 % of the cases: the low age of the patients together with their low weight and the large size of and the hyper-vascularized structure of the tumors represent the main factors limiting surgery. Pure observation is considered as first choice in children undergoing a partial/subtotal tumor resection, chemotherapic regimens being considered in cases of recurrences after a second look surgery. Long-term prognosis is favorable with mortality being related mostly to the rare midline (i.e., hypothalamic) locations, which beyond the functionally relevant site, tend to have an unusually more aggressive histological behavior.

OBJECT Only a few published studies of the surgical treatment of benign peripheral nerve sheath tumors (BPNSTs), malignant peripheral nerve sheath tumors (MPNSTs), and peripheral non–neural sheath tumors (PNNSTs) have analyzed the results and possible prognostic factors using multivariate analysis. The authors report on their surgical series of cases of BPNSTs, MPNSTs, and PNNSTs with long-term follow-up and analyze the role of selected factors with respect to the prognosis and risk of recurrence of these tumors using multivariate analysis. They also review the pertinent literature and discuss their results in its context. METHODS The authors retrospectively reviewed data from cases involving patients who underwent resection of a peripheral nerve tumor between January 1983 and December 2013 at their institution. Of a total of 200 patients, 150 patients (with 173 surgically treated tumors) had adequate follow-up data available for analysis. Pain was assessed using a visual analog scale (VAS), and motor and sensory function were assessed by means of the Louisiana State University grading system. They also analyzed the relationship between tumor recurrence and patient sex, patient age, diagnosis of neurofibromatosis (NF), tumor histopathology, tumor size, tumor location, and extent of resection (subtotal vs gross-total resection), using univariate and multivariate analyses. RESULTS There was a statistically significant improvement in the mean VAS pain score (preoperative 3.96 ± 2.41 vs postoperative 0.95 ± 1.6, p = 0.0001). Motor strength and sensory function were significantly improved after resection of tumors involving the brachial plexus (p = 0.0457 and p = 0.0043, respectively), tumors involving the upper limb (p = 0.0016 and p = 0.0016, respectively), BPNSTs (p = 0.0011 and p < 0.0001, respectively), and tumors with dimensions less than 5 cm (motor strength: p = 0.0187 and p = 0.0021 for ≤ 3 cm and 3–5 cm tumors, respectively; sensory function: p = 0.0003 and p = 0.0001 for ≤ 3 cm and 3–5 cm tumors, respectively). Sensory function showed a statistically significant improvement also in patients who had undergone resection of tumors involving the lower limb (p = 0.0118). Total resection was associated with statistically significant improvement of motor strength (p = 0.0251) and sensory function (p < 0.0001). In univariate analysis, a history of NF (p = 0.0034), a diagnosis of MPNST or PNNST (p < 0.0001), and subtotal resection (p = 0.0042) were associated with higher risk of tumor recurrence. In multivariate analysis (logistic regression analysis), a history of NF (OR 9.28%, 95% CI 1.62–52.94, p = 0.0121) and a diagnosis of MPNST (OR 0.03%, 95% CI 0.002–0.429, p = 0.0098) or PNNST (OR 0.081%, 95% CI 0.013–0.509, p = 0.0077) emerged as independent prognostic factors for tumor recurrence. CONCLUSIONS A total resection should be attempted in all cases of peripheral nervous system tumors (irrespective of the supposed diagnosis and tumor dimensions) because it is associated with better prognosis in term of functional outcome and overall survival. Moreover, a total resection predicts a lower risk of tumor recurrence. Patients with a history of NF and tumors with malignant histology remain a challenge both for neurosurgeons and oncologists due to higher recurrence rates and the lack of standardized adjuvant therapies.

Treatment of intraosseous meningiomas is a two-staged procedure including tumor resection and cranial reconstruction. Several are the potential pitfall of this kind of surgery such as the conspicuous dimensions usually reached by the tumor and the peculiar involvement of irregular and deep bony structures. For these reasons, a surgical accurate virtual planning and a careful removal followed by tailored reconstruction are mandatory to achieve satisfactory results. We analyzed six patients operated on for intraosseous meningiomas between September 2014 and June 2018. Resection strategy was planned beforehand and shared with the manufacturer who provided the PEEK cranioplasty used in the reconstructive phase. Between September 2014 and April 2018, six patients affected by intraosseous meningioma were operated on. Female/male ratio was 5:1 and mean age was 54 ± 10.8 years. Mean FU was 20.3 ± 16.4 months. Mean dimension was 73.9 ± 24.8 mm × 69.2 ± 16.2 mm. Mean surgical time was 5.1 ± 1.1 h. The resection of intraosseous meningiomas requires the earliest and finest reconstructive phase. Custom-made implants should be considered the gold-standard for cranioplasty, especially in large skull and in frontal or hairless areas of the skull. The described technique is simple, accurate, and effective in achieving good results in disease control as well as cosmetic and functional restoration.

The aim of this paper is to review the current knowledge on the management of local infections in posterior cranial fossa (PCF) and spinal surgery; the center of our paper will be the diagnosis and management of infections of the wound, the subcutaneous tissue, and muscle strata. Very few papers address the problem of local infections in posterior cranial fossa and spinal surgery. The largest amount of the literature data is indeed centered on how to deal with deep infections, CSF leak, and secondary meningitis or hardware infection. Concerning PCF surgery, a higher rate of local infections has been reported, compared with supratentorial surgery. To prevent such complications, prophylaxis remains the most important tool in dealing with local infections; a careful surgical planning and patient selection are also important. On the other hand, in dealing with local infection in spinal setting, there are several evidences that local vancomycin might help reducing them in adults as well as in children. The treatment protocol consists in antibiotic therapy and, whenever needed, wound revision to remove infectious material or to obtain a microbiological diagnosis. Prognosis has overall to be considered favorable both in the short term as well as in the long term.

The management of obstructive hydrocephalus associated to posterior cranial fossa tumor remains a controversial issue. In this context, the role of endoscopic third ventriculostomy (ETV) performed before tumor removal (the so-called preoperative ETV) is debated and its success rate is reported as being largely variable in the literature. We describe two children who experienced the closure of a preoperatively performed ETV after the surgical resection of their posterior fossa tumor. In both cases tumor removal was performed in the prone position. Radiological investigations and intraoperative evidence documented that the failure of the ETV was due to a blood clot which directly occluded the stoma. We speculate that the prone position favored the migration of blood, through the aqueduct, thereafter, clotting on the most declivous part of the third ventricle once the patient assumed the upright position, with the consequent occlusion of the stoma. Based on a gravitational hypothesis, we named this mechanism the "snow globe effect."

Unfortunately in the original publication, the affiliation provided for the author G. Tamburrini was incorrect. The correct affiliation for G. Tamburrini should read as follows.

Unfortunately in the original publication, the affiliation provided for the author G. Tamburrini was incorrect. The correct affiliation for G. Tamburrini should read as follows: Fondazione Policlinico Universitario A. Gemelli IRCSS, Universita Cattolica del Sacro Cuore, Rome, Italy.

Shunt malfunction represents one of the most common problem to deal with in the daily neurosurgical practice. Among the variety of possible presentations, the cyst-like dilatation of the brain around the proximal catheter is one of the most rare and misleading type. The case of a boy with a 'tumour-like' proximal shunt malfunction is described and the pertinent literature is analysed.

Objective Duraplasty can be performed both by means of autologous tissues (such as galea-pericranium, temporal muscle, fascia lata) or by commercially available dural patches. Nowadays many neurosurgeons consider galea-pericranium duraplasty time-consuming, technically demanding or not adequate, thus dural surrogates are increasingly popular. In this prospective research we compared duraplasty using autologous galea-pericranium vs. dural patches in terms of postoperative long term results, ease/time required and costs. Patients and methods Research has been designed as prospective cohort study, that included 185 patients undergoing supratentorial elective neurosurgery with galea-pericranium or non-autologous duraplasty (minimum follow-up 12 months). Variables taken into account were: wound infection, CSF fistula, subcutaneous CSF collection, bone flap osteitis, brain abscess, empyema and tardive wound dehiscence (particularly after postoperative radiotherapy). Time for galea-pericranium collection, size of galea-pericranium harvest and dural defects were recorded in each case. Costs for non-autologous duroplasty were calculated. Results No statistically significant differences were evident in long term postoperative results between the two groups. Mean time of galea-pericranium collection is less than 2 min and enough galea-pericranium can be harvested in supratentorial approach to cover almost any dural defect. The only difference between the two groups is costs: an average of 268.7€/patient spent just for duraplasty. This figure is surely substantial if considered for the entire amount of surgeries performed in a department. Conclusions Considering that in our study long term results are equivalent, that galea-pericranium duraplasty is feasible and rapid, our indications are in favor of saving a considerable amount of money since an ideal autologous dural substitute is available and “free”.

Advances in comprehension of molecular biology of glioblastoma (GBM) have led to the development of targeted therapies. The aim of the present study was to evaluate the efficacy and safety of a targeted therapeutic approach in which administration of bevacizumab and erlotinib was tailored on the molecular profile of recurrent GBM. We prospectively enrolled ten adult patients suffering from recurrent GBM who had undergone surgical resection and standard chemo-radiotherapy. Tumor tissue was assessed for the expression of EGFRvIII and MGMT promoter methylation by RT-PCR, and for PTEN and VEGF expression by immunohistochemistry. Normal PTEN status was required for inclusion. Patients with VEGF overexpressing tumors (10/10) were treated with bevacizumab (10 mg/kg iv every 2 weeks in 6-week cycles); patients whose tumor expressed EGFRvIII (4/10) added erlotinib (150 mg/day orally; 300 mg/day if on enzyme-inducing antiepileptic drugs). Therapy was continued until disease progression or unacceptable toxicity. Primary endpoints of the study were response rate (RR), 6-month progression-free survival (PFS-6), and safety profile. The RR and PFS-6 were 100 % (4/4) and 50 % (3/6) in patients treated with bevacizumab+erlotinib (n = 4) and bevacizumab (n = 6), respectively. In the whole cohort (n = 10), RR and PFS-6 were both 70 % (7/10); median PFS and overall survival (OS) were 8.0 (3.0–31.0) and 9.5 (5.0–31.0) months, respectively. No grade 3/4 adverse events were observed; three patients treated with bevacizumab+erlotinib displayed grade 1/2 rash not requiring dose reduction; one patient treated with bevacizumab developed intratumoral hemorrhage requiring treatment discontinuation. To our knowledge, this is the first study on recurrent GBM in which administration of bevacizumab and erlotinib was tailored on the molecular profile of the patient’s tumor. Although we treated a limited number of patients, we obtained significantly higher RR and PFS-6 than those reported in a previous trial lacking molecular tumor analysis.

Decompressive hemicraniectomy (DHC) is performed to treat a variety of conditions, such as traumatic brain injury, subarachnoid hemorrhage, and brain swelling following middle cerebral artery (MCA) infarction. The rationale for this treatment is to remove a portion of bone and open the dura mater in order to decrease intracranial pressure (ICP) and prevent brain shift. However, the usefulness of routine hemicraniectomy for MCA infarction is still unclear because some patients may not progress to cerebral herniation [1]. We report on a man submitted to DHC for brain swelling and midline shift following right MCA infarction, whose clinical conditions rapidly ameliorated, despite an increase of cerebral herniation on post-operative CT scan. We, also, review the pertinent literature.

Primary empty sella syndrome (ESS) shares with idiopathic intracranial hypertension (IIH) high intracranial pressure (ICP) and association with obesity. Bariatric surgery is effective in IIH treatment; no data, however, exist about ESS. We report two overweight ESS patients in whom bariatric surgery resolved obesity but failed in curing ESS, requiring cerebrospinal fluid (CSF) shunt placement.

Object Communicating hydrocephalus is an uncommon complication in patients treated for glioblastoma multiforme (GBM). Its pathogenesis remains unclear. The authors evaluated the clinical and radiological factors associated with the onset of communicating hydrocephalus and the impact of ventriculoperitoneal (VP) shunt surgery on the outcome of these patients. Methods One hundred twenty-four patients harboring GBM, who had undergone craniotomy for tumor resection and adjuvant radiochemotherapy, were retrospectively assessed. Seven of them developed communicating hydrocephalus and were treated with VP shunt surgery. Clinical and radiological estimates included Karnofsky Performance Scale (KPS) score, previous surgery, overall survival (OS), CSF pressure and components, tumor location, and leptomeningeal dissemination. Results All 7 patients who developed communicating hydrocephalus had undergone at least 2 craniotomies for tumor resection before the onset of hydrocephalus (p = 0.0006; Fisher exact test). Six cases showed high levels of CSF proteins. There was a highly significant relationship between ventricular opening at surgery for tumor recurrence and onset of hydrocephalus (p = 0.0002; Fisher exact test). In these patients, VP shunt surgery was followed by a significant improvement of KPS score (p = 0.0180; Wilcoxon signed-rank test). The median OS after VP shunt insertion was 5 ± 2.9 months. Conclusions Ventricular opening after radiochemotherapy and high CSF protein levels are significant predictors of communicating hydrocephalus in patients with GBM. The VP shunt surgery improves quality of life in these patients.

BACKGROUND: Giant cysts of the midline, not associated to a tumor, are exceptional finding in the brain of adults. Here we present two cases of symptomatic giant cerebral cysts of the midline occurred in an elderly and in a young adult patients both treated with mini-invasive unilateral neuroendoscopic procedure. In the recent literature (since 1999) similar cases have not been reported. Beside the clinical report, review of literature and major anatomical features of the region are described. CASE DESCRIPTION: These two adults (82 and 41 years old respectively) had a slow progressive development of headache, gait disturbances, memory impairment and urinary incontinence. Magnetic resonance imaging showed giant cyst of the midline and hydrocephalus. Surgery with the endoscopic procedure, through a right frontal burr hole, was followed by clinical and radiological improvement. CONCLUSION: Giant cerebral cysts of the midline in adults can be successfully treated through a neuroendoscopic monolateral approach that comprehends multiple openings, diffuse coagulation of the capsule, and careful releasing of capsule-ependyma adherences. Knowledge of major anatomical and developmental details of the septal region is necessary to avoid complication in a mini-invasive surgical procedure.