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Posterior fossa syndrome (PFS) is a set of debilitating complications that can occur after surgery for posterior fossa tumors. This study aimed to assess the preoperative radiological and surgical risk factors for the onset of PFS in a histologically homogeneous population of children with medulloblastoma and compare it to a similar population of young adults.Included patients underwent posterior fossa surgery for medulloblastoma at 11 Italian neurosurgical wards (2003-2019) and were referred to Fondazione IRCCS Istituto Nazionale dei Tumori in Milan (INT) for postoperative treatments. We collected patients' pre- and post-operative clinical, surgical and radiological data from the INT charts. To compare the distribution of variables, we used the Mann-Whitney and Fisher tests for continuous and categorical variables, respectively.136 patients (109 children and 27 young adults) were included in the study. Among children, 29 (27%) developed PFS, and all of them had tumors at midline site with invasion of the fourth ventricle. Radiological evidence of involvement of the right superior (39% versus 12%; p = 0.011) or middle cerebellar peduncles (52% versus 18%; p = 0.002) seemed more common in children who developed PFS. Young adults showed an expected lower incidence of PFS (4 out of 27; 15%), that may be due to anatomical, physiological and oncological elements.This study confirmed some factors known to be associated with PFS onset and shed light on other debated issues. Our findings enhance an already hypothesized role of cerebellar language lateralization. The analysis of a population of young adults may shed more light on the often-neglected existence of PFS in non-pediatric patients.

(1) Background: The issue of unsuccessful surgery for Chiari-1 malformation (CM-1), as well as its potential causes and possible solutions, remains poorly documented and studied. (2) Methods: From a retrospective review of a personal series of 98 patients undergoing treatment for CM-1 during the past 10 years, we created two study groups. Group 1: 8 patients (8.1%) requiring additional surgeries owing to postoperative complications (7 cerebrospinal fluid leakage, 1 extradural hematoma); 7 patients (7.1%) undergoing reoperations for failed decompression during the follow-up. Group 2: During the same period, we also managed 19 patients who had previously been operated on elsewhere: 8 patients who required adequate CM-1 treatment following extradural section of the filum terminale; 11 patients requiring reoperations for failed decompression. Failed decompression was managed by adequate osteodural decompression, which was associated with tonsillectomy (6 cases), subarachnoid exploration (8 cases), graft substitution (6 cases), and occipito-cervical fixation/revision (1 case). (3) Results: There was no mortality or surgical morbidity in Group 1. However, one patient’s condition worsened due to untreatable syrinx. In Group 2, there were two cases of mortality, and surgical morbidity was represented by functional limitation and pain in the patient who needed revision of the occipitocervical fixation. Twenty patients improved (58.8%), 6 remained unchanged (32.3%), 1 worsened (2.9%) and 2 died (5.9%). (4) Conclusions: The rate of complications remains high in CM-1 treatment. Unfortunately, a certain rate of treatment failure is unavoidable, but it appears that a significant number of re-operations could have been avoided using proper indications and careful technique.

Complex craniofacial injuries (CFI) are induced by high-energy mechanisms of injury, resulting in the simultaneous breaking of many bones, bony displacement and often comminution, soft tissue lacerations or avulsions, and visceral consequences. They can be defined as facial injuries with a CFI score equal to or above 10. In the emergency setting, severe facial injuries can affect the patency of the airway and hemodynamic instability, so essential Damage Control procedures must be well known. These patients often have associated injuries, which can constitute real-life saving priorities. Among these, certainly the most frequent are traumatic brain injuries and cervical spine injuries. This type of patient generally requires long-lasting surgery, with a lack of a solid anatomical reference for reconstruction, a few weeks of hospitalization, and several days in ICU. The definitive treatment of complex craniofacial injuries, therefore, requires high technical skill, but mainly a solid organization of the reconstructive planning and multidisciplinary coordination of priorities and treatment sequences. Technologies and the heavy manipulation of images are nowadays indispensable tools, for a correct diagnosis and personalized surgical planning, and for increasing the level of safety and accuracy of the results. Concurrent skull base fractures and gunshot injuries represent the most complex expressions of severe craniofacial injuries, in which specific definitive treatment protocols are required for proper management.

Background: The aim of this study was to achieve a consensus on the minimum set of outcome measures and predictors to be used in the neurosurgical practice and on the timing of outcome assessment. Methods: A consensus building approach was employed. All neurosurgical departments in Lombardy (Italy) were invited to participate by the Carlo Besta Neurologic Institute IRCCS Foundation. Three workshops were organized during which a multidisciplinary group called Neurosurgical Outcome Network (NEON) was created and the methodology to select outcome measures, predictors, and timing of outcome assessment was established. Eight working groups were created for the different neurosurgical diseases (neuro-oncological, skull base, vascular, traumatic, spinal, peripheral nervous system, malformation, functional) and 8 workshops were organized to identify the outcome measures and predictors specific for each of the neurosurgical diseases based on the experts' clinical practice and the existing literature. Results: A total of 20 neurosurgical departments participated in this study. Specific outcome measures, predictors and the timing of outcome assessment were identified for each of the 8 neurosurgical diseases. Moreover, a list of variables common to all pathologies were identified by the NEON group as further data to be collected. Conclusions: A consensus on the minimum set of outcome measures and predictors and the timing of outcome assessments for 8 neurosurgical diseases was achieved by a group of neurosurgeons of the Lombardy region, called NEON. These sets could be used in future studies for a more homogeneous data collection and as a starting point to reach further agreement also at national and international level.

The insular cortex is considered one of the most complex regions of the brain, defined as the "hub" of somatosensory areas. Here, we examine the case of a surgically treated haemorrhagic cavernoma involving the middle and posterior insular cortex, presenting both sensory, gustative and speech symptoms. By reviewing the recent findings in humans' and primates' basic research, we illustrated clinical and radiological correlations of the reported case, confirming insular role in sensitive and gustatory functions.

Chiari malformation type II (CM-II) is a hindbrain developmental malformation. Movement disorders are rarely described as associated with this condition. We describe the case of a newborn affected by CM-II presenting with head and neck tremors a few days after myelomeningocele repair surgery. Later, self-resolving episodes of expiratory apneas arose. Cranial ultrasounds showed progressive ventricular dilatation. She underwent ventriculoperitoneal shunt followed by craniocervical bony decompression. After surgery, both apnea and tremors disappeared. This particular presentation of CM-II is very rare and potentially difficult to diagnose.

Objective: To report the peculiarities of spinal epidural abscess in COVID-19 patients, because we observed an unusually high number of these patients following the outbreak of SARS-Corona Virus-2.Methods: We reviewed the clinical documentation of six consecutive COVID-19 patients with primary spinal epidural abscess that we had to surgically manage during a two-month period. These cases were analyzed for what concerns both the viral infection and the spinal abscess.Results: Abscess was primary in all cases meaning that no evident infective source was found. Primary abscess represents the rarest form of spinal epidural abscess, which is usually secondary to invasive procedures or spreading from adjacent infective sites, such as spondylodiscitis and generally occurs in patients with diabetes, obesity, cancer, or other chronic disease. In all cases, there was mild lymphopenia but the spinal abscess occurred regardless the severity of the viral disease, the immunologic state, and the presence of bacteremia. Obesity was the only risk factor and was reported just in two patients. All patients but one were hypertensive. The preferred localizations were cervical and thoracic, whereas the classic abscess generally occurs at lumbar level. No patient had history of pyogenic infection, even though previous asymptomatic bacterial contaminations were reported in three cases.Conclusion: We wonder about the concentration of this uncommon disease in a so short period. To our knowledge, cases of epidural spinal abscess in COVID-19 patients have been not yet reported. Accordingly, we ignore if the SARS-Corona Virus-2 may really predispose to spinal epidural abscesses. However, we hypothesize that, in our patients, the spinal infection could have depended on the coexistence of an initially asymptomatic bacterial contamination. The well-known COVID-19-related endotheliitis might have created the conditions for retrograde bacterial invasion of the correspondent spinal epidural space. Anyway, the spinal epidural abscess carries significantly high morbidity and mortality. It is difficult to diagnose, especially in compromised COVID-19 patients but it should be kept in mind because early diagnosis and treatment are crucial.

Chiari malformation type II is present in almost all patients with myelomeningocele but usually remains asymptomatic. Symptoms are generally more severe in neonates, who have the worst prognosis. The association symptoms/hydrocephalus is well known, and first treatment usually consists of ensuring adequate ventricular drainage. Craniovertebral decompression may be required in patients who do not improve after drainage. However, mechanisms of symptom development are not yet completely understood, timing and techniques of surgery are not codified, long-term evolution is poorly reported, and there are few paper reporting clinical onset and treatment in older patients. We reviewed our personal series of 42 consecutive symptomatic patients that required surgical treatment. Age at surgery ranged from 1 week to 44 years (mean 6.6 years). Surgical timing strictly depended on clinical conditions: urgent management in the more compromised patients (usually infants) and elective treatment before severe deterioration in patients with less severe conditions. All patients first underwent external ventricular drainage, which resolved the symptomatology in 17 cases (40%). Craniocervical decompression was required by 25 patients (60%) who received no benefit from the ventricular drainage. Early mortality (2 cases = 4.7%) occurred only in neonates. Clinical improvement was achieved in 37 of 40 survivors (92%). During a follow-up ranging from 2 to 20 years (mean 10.3 years), late mortality consisted of 4 cases (10%), mainly due to cardio-respiratory arrest. Twenty-two patients (55%) required surgery for shunt malfunction and 4 for cord detethering. Six patients (15%) required reoperation owing to symptom recurrence. Early treatment of symptomatic Chiari II malformations may warrant satisfactory results in a significant number of patients, even in neonates. Nevertheless, overall mortality remains relatively high, throughout the patient life. Formal transition programs and adult spina bifida care processes have become crucial.

To review the literature about the acute presentation of Chiari 1 malformation in children, with a focus on acute cervical cord syndromes with impairment of the cortico-spinal tract. To analyze the possible precipitating factors and the pathophysiology of the acute onset. Illustrative case presentation with literature review. The literature includes just a few dozens of children with acute presentation of Chiari 1 malformation. The more frequent presentations consist of oropharyngeal dysfunction, cranial nerve impairment, high intracranial pressure, peripheral motor deficit. Acute impairment of cervical long tracts is very rare and we could find only 16 previously reported cases of cervical cord impairment with quadriparesis or hemiparesis. Nowadays, a lot of asymptomatic Chiari 1 malformations are frequently observed owing to the wide diffusion of magnetic resonance imaging. This raised the question about the management of these patients. Despite severe and even lethal manifestations being reported in previously asymptomatic patients, the absolute rarity of acute deterioration does not justify prophylactic surgery. The diagnosis of Chiari malformation may be initially difficult in patients with isolated, acute, and unusual presentations but physicians should bear in mind its possibility, because prompt cranio-cervical decompression may be decisive.

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

Syringomyelia and Chiari malformation are classified as rare diseases on Orphanet, but international guidelines on diagnostic criteria and case definition are missing. Aim of the study: to reach a consensus among international experts on controversial issues in diagnosis and treatment of Chiari 1 malformation and syringomyelia in adults. A multidisciplinary panel of the Chiari and Syringomyelia Consortium (4 neurosurgeons, 2 neurologists, 1 neuroradiologist, 1 pediatric neurologist) appointed an international Jury of experts to elaborate a consensus document. After an evidence-based review and further discussions, 63 draft statements grouped in 4 domains (definition and classification/planning/surgery/isolated syringomyelia) were formulated. A Jury of 32 experts in the field of diagnosis and treatment of Chiari and syringomyelia and patient representatives were invited to take part in a three-round Delphi process. The Jury received a structured questionnaire containing the 63 statements, each to be voted on a 4-point Likert-type scale and commented. Statements with agreement

Ventricular shunting procedures represent the classical surgical treatment for hydrocephalus. Slit ventricle syndrome (SVS) with craniocerebral disproportion (CCD) and secondary craniosynostosis (SCS) is a well-known but uncommon complication following cerebrospinal fluid (CSF) shunting in children. Its general management includes shunt upgrade or revision, placement of anti-siphon devices, lumbo-peritoneal shunting, and endoscopic third ventriculostomy. Cranial expansion is generally considered a major procedure and is indicated when less invasive treatments fail. In these cases, SVS and associated SCS have been usually managed through anterior cranial vault expansion. This procedure aims to decrease the risk of further shunt revisions and to improve neurologic symptoms, but it takes the disadvantage of considerable iatrogenic alterations of the facial proportions. The authors report a case of a 6-year old boy with SVS and SCS, who maintained eumorphic face and was treated by an innovative post-coronal vault expansion ensuring a wide volume increase while avoiding any change of facial conformation.

Ligamentous cyst is a cystic formation arising from degeneration of ligamentous structures all around the spinal cord. They can cause spinal cord compression, like synovial cyst. Unlike synovial cyst, there is no spinal instability in pathogenesis of ligamentous cyst. Differential diagnosis through pre-operative MRI is difficult and intraoperative findings plus histopathology are crucial to achieve a diagnosis. In this case report, we deal with a rare case of cervical junction ligamentous cyst. A 59-year-old Caucasian female was admitted in our ward with left-sided hemiparesis, cervical pain, and upper limb diffused paresthesias, due to an oval-shaped formation into ALL, of 13 mm in maximum diameter, with peripheral contrast enhancement. The patient underwent, under general anesthesia, a surgery through a posterolateral suboccipital approach which aimed to decompress the spinal cord and to drain the cyst with total removal of the compression by emptying the cyst and fulfilling it with muscle graft and glue. No posterior fixation was needed. After the surgery, symptoms improved and a post-operative MRI scan demonstrated the good result of the surgery.

Dural arteriovenous fistulas (dAVFs) account for 10-15% of all intracranial arteriovenous lesions. Different classification strategies have been proposed in the course of the years. None of them seems to guide the treatment strategy.We expose the experience of the vascular group at Niguarda Hospital and we propose a very practical classification method based on the location of the shunt. We divide dAVF in sinus and non-sinus in order to simplify our daily practice, as this classification method is simply based on the involvement of the sinuses.477 intracranial dural arteriovenous fistulas have been treated. 376 underwent endovascular treatment and 101 underwent surgical treatment. Cavernous sinus DAVFs and Galen ampulla malformations have been excluded from this series as they represent a different pathology per se. 376 dAVFs treated by endovascular approach: 180 were sinus and 179 were non-sinus. 101 dAVFs treated with surgical approach: 15 were sinus and 86 were non-sinus.Of the 477 intracranial dAVF the recorded mortality and severe disability was 3% and morbidity less than 4%. All patients underwent a postoperative DSA with nearly 100% of complete occlusion of the fistula. At a mean follow-up of 5 years in one case there was a non-sinus fistula recurrence, due to the presence of a partial clipping of "piè" of the vein.The sinus and non-sinus concept has guided our institution for years and has led to good clinical results. This paper intends to share this practical classification with the neurosurgical community.

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.

The increased life expectancy of patients with myelomeningocele led to the necessity of new management for addressing complex spine deformities with severe pelvic obliquity. Tethered cord release (TCR) and spinal fusion were described as treatment options. However, nowadays, the surgical strategy is not well defined and high rates of mechanical failures and complications are reported. Our aim was to propose a new two-stage surgical pathway to treat a selected group of severe myelomeningocele scoliosis. This is a retrospective series of myelomeningocele scoliosis in paralytic adolescent patients treated with concurrent TCR and posterior spinal fusion (PSF), followed by delayed anterior fusion (ASF) through minimally invasive lateral approach. Inclusion criteria were as follows: evolutive scoliosis in tethered cord syndrome and paraplegia, main curve >70°, pelvic obliquity >20°, and age between 10 and 18 years. Clinical, surgical, and radiographic parameters were evaluated preoperatively and at a mean follow-up (FU) of 2.8 years. Six patients out of 58 met the inclusion criteria and were included in the study (mean age 12.3 years old). The mean curve and pelvic obliquity correction were 68.8% and 82%, respectively. No loss of correction was recorded at FU. One case of cerebrospinal fluid leakage requiring revision surgery was recorded. To the best of our knowledge, this is the first study proposing a two-stage surgical pathway for severe MMC spinopelvic deformity treatment. Concurrent TCR and PSF with delayed minimally invasive ASF permitted to minimize complications providing good fusion rates without loss of correction and implant failure.

BACKGROUNDClassic treatment of Chiari malformation type 1 consists of foramen magnum decompression. Selected patients may require occipitocervical fixation, transoral odontoidectomy, tonsillectomy, and so forth. Treatment standardization does not yet exist, and some patients risk being overtreated.OBSERVATIONSA 20-year-old man with headache and Chiari malformation type 1 underwent extradural bone decompression. One year later, he was managed with the extradural section of his filum terminale. Eighteen months later, the patient underwent monitoring of intracranial pressure, occipitocervical stabilization, transoral odontoidectomy, minimally invasive subpial tonsillectomy, and occipital cranioplasty. His headache never changed, and he progressively developed hemiparesis and swallowing and respiratory disturbances. Two years later, a new magnetic resonance imaging scan showed extended syringomyelia with scarce peritonsillar subarachnoid space. The umpteenth operation consisted of the removal of a constricting epidural scar, arachnoid dissection, total tonsillectomy, creation of a wide subarachnoid space, and dural sac augmentation. The patient’s initial postoperative course was smooth, and his headache improved. However, 8 days after surgery, the patient acutely presented with vegetative disturbances and died because of malignant brainstem edema of unknown origin.LESSONSThe story of this patient is not so uncommon. He underwent all the possible surgical treatments rather than a timely adequate osteodural decompression. Probably, he received less with more.

Introduction: Dural arteriovenous fistulas (dAVFs) account for 10–15% of all intracranial arteriovenous lesions. Different classification strategies have been proposed in the course of the years. None of them seems to guide the treatment strategy. Objective: We expose the experience of the vascular group at Niguarda Hospital and we propose a very practical classification method based on the location of the shunt. We divide dAVF in sinus and non-sinus in order to simplify our daily practice, as this classification method is simply based on the involvement of the sinuses. Material and Methods: 477 intracranial dural arteriovenous fistulas have been treated. 376 underwent endovascular treatment and 101 underwent surgical treatment. Cavernous sinus DAVFs and Galen ampulla malformations have been excluded from this series as they represent a different pathology per se. 376 dAVFs treated by endovascular approach: 180 were sinus and 179 were non-sinus. 101 dAVFs treated with surgical approach: 15 were sinus and 86 were non-sinus. Discussion: Of the 477 intracranial dAVF the recorded mortality and severe disability was 3% and morbidity less than 4%. All patients underwent a postoperative DSA with nearly 100% of complete occlusion of the fistula. At a mean follow-up of 5 years in one case there was a non-sinus fistula recurrence, due to the presence of a partial clipping of “piè” of the vein. Conclusions: The sinus and non-sinus concept has guided our institution for years and has led to good clinical results. This paper intends to share this practical classification with the neurosurgical community.

Background Giant calcified thoracic disk herniation (GCTD) is an uncommon event, which requires surgical treatment in less than 1% of patients. GCDTs are a specific subgroup of herniated thoracic disks occupying more than 40% of the spinal canal showing calcifications associated with a certain degree of ossification. In this paper, we are reporting our whole experience in the surgical management of GCTDs through anterior approaches. We believe that they present characteristics that associate them to the circumscribed type of Ossified Posterior Longitudinal Ligament (OPLL) with a possible common pathophysiology consisting in the dural violation. Methods Twenty-three consecutive patients with GCDTs were managed through anterior approaches during the period 1996-2019 at the Niguarda Hospital - Milan, Italy. Clinical data, radiological features, surgical reports, histological findings, and outcomes were reviewed. Results There was no mortality, whereas permanent morbidity consisted of 1 cases of worsened paraparesis due to accidental spinal cord contusion. One patient required reoperation to repair a postoperative cerebrospinal fluid (CSF) leakage. All patients underwent postoperative MRI which showed excellent decompression of cord and dural sac in all cases. Histological study of en-bloc removed GCTD showed typical calcification patterns of the PLL. Conclusions GCDTs may be assimilated to the so-called "circumscribed type" of OPLL. The GCDTs may show the same radiological CT and MRI pattern of OPLL. The anterior accesses now represent the standard of care for GCTDs. The use of operative microscope and intraoperative monitoring is mandatory. The risk of CSF leakage can be markedly reduced by meticulous reconstruction of the dura and the placement of spinal drainage. Adequate exposition may sometimes require one or two levels of corpectomy with consequent vertebral body reconstruction and fixation of anterior column of the spine.

The halo vest is widely used throughout the world to manage craniovertebral and cervical instabilities. It can be used for postoperative immobilization or as an alternative to surgical fixation.In this paper we present some cases of severe complications from our own practice and review the literature on halo complications.Like any therapeutic manoeuvre, halo placement may be followed by various complications. In the meantime, modern techniques of fixation offer safe and immediate stabilization.The halo vest remains a formidable method for cervical immobilization. However, it should not be used a priori instead of surgery.

Background: The halo vest is widely used throughout the world to manage craniovertebral and cervical instabilities. It can be used for postoperative immobilization or as an alternative to surgical fixation.

Endoscopic third ventriculostomy (ETV) is now widely used to manage ventriculoperitoneal shunt (VPS) malfunctions, but outcome predictors are still debated. Different opinions exist about the prognostic factors, but shunt duration is generally considered unimportant although its influence remains poorly investigated.A total of 139 patients undergoing ETV for VPS malfunction were reviewed. Successful ETV was defined as shunt independence. There were 56 children and 83 adults; hydrocephalus had different causes. The mean VPS-ETV interval was 8.1 years.Ninety patients (64.7%) became shunt free. Children and adults achieved comparable success rates (64.3% and 65.1%, respectively). Age, gender, and cause of shunt malfunction were scarcely significant. The success rates were 86.8% (33/38 patients) in obstructive hydrocephalus (OH), 55.2% (36/67) in communicating hydrocephalus, and 58.8% (21/34) in myelomeningocele-related hydrocephalus, which was significant (P = 0.02). History of multiple revisions was a negative predictor (P 0.001): success rate, 39% (16/48) versus 81.3% (74/91). Any individual time step increase reduced the odds of ETV success by 34% (P = 0.014). In OH, the results were good regardless of any other factor, including shunt duration. In communicating hydrocephalus, short VPS-ETV intervals correlated with better outcome (P = 0.021), although they were irrelevant in perinatal posthemorrhagic hydrocephalus. In myelomeningocele-related hydrocephalus, shunt duration had intermediate effects.ETV is the first option for shunt malfunctions in OH and perinatal posthemorrhagic hydrocephalus, regardless of other factors. Conversely, in other types of hydrocephalus, the chances of shunt independence are lower and shunt duration and history of multiple shunt revisions are significant.

Objective To refine the surgical indications of surgery for life-threatening cerebral hematomas caused by aneurysm rupture, through the analysis of possible outcome predictors. Methods Forty-nine consecutive patients requiring prompt clot evacuation were retrospectively reviewed. In all cases, the hematoma was equal to or greater than 60 mL. The bleeding aneurysm was located on the middle cerebral artery in 26 cases, on the internal carotid artery in 10 cases, and on the anterior cerebral artery in 13 cases; four aneurysms were giant. Six patients underwent aneurysm coiling followed by clot removal, whereas 43 patients were managed by concomitant clot evacuation and aneurysm clipping. The main clinical and radiologic features, the management paths and the treatment modalities were correlated with the outcomes. A statistical analysis was conducted. Results Overall mortality was 32.6%, severe morbidity was 22.4% and 22 patients (44.8%) achieved favorable results. The short-term results were more significantly influenced by the radiological parameters than by the initial clinical conditions. The prognostic weight of the radiologic findings was partially lost for six-month results, whereby management factors gained in importance. Conclusions The bleeding severity was strongly determinant for early mortality. However, if patients can survive the initial crucial phase, their chances of a favorable outcome are not negligible. Further improvement may be possible through better patient selection and the identification of nonsalvageable subjects.

Large cranial defects in very young patients are challenging. The ideal material for cranioplasty in this age group has not yet been identified. Cryopreserved autologous bone presents very high rates of failures, acrylic resins pose a number of compatibility problems, bioceramics may be contraindicated, and autografts may be not adequate for repair of large defects. We present an 18-month-old baby with a large post-traumatic cranial defect which was repaired by assembling a new bone flap on a sterile stereolithographic 3-D model. This customized newly assembled flap consisted of a scaffold of autologous bone (from vault duplication) sustaining 2 large grafts of homologous cadaveric bone. It was adequately modeled and contoured on the 3-D model using metallic plates and screws. Immediately after implantation on the skull, the metallic devices were progressively replaced by reabsorbable material, thus maintaining the previously obtained flap profile. In this paper we detail this original technique which was developed to manage this specific patient.

Purpose: Autogenous bone represents the best material in pediatric reconstructive cranioplasty because of its compatibility with growth; however, its availability is limited. Alternative use of alloplastic materials would have the advantage of unlimited amount, but shows an increased risk of complications and incompatibility with growth. Fresh frozen banked cadaveric bones could be ideal for the reconstruction of large cranial defects in growing patients, because it offers unlimited amount of structural grafts with biomechanics properties quite similar to the autologous bones. Results: The authors report 2 cases of growing patients (13 months female and 9 years old male) undergoing the reconstruction of large (about 70 cm2 and 50 cm2 respectively) cranial vault defects, by using structural homologous banked bone grafts. Analytic main reconstruction materials risks/benefits evaluation, literature review of few previously reported pediatric cranioplasty with homologous bone, and both clinical cases satisfactory radiologic long-term results (beyond 4 years of F.U.) are reported. Conclusion: Structural homologous banked bone grafts appear as a valid solution for pediatric canioplasty reconstruction of wide defects, entailing the advantages of available without limitations and compatibility with future growth. The risk of transmissible infections seems minimal and is totally counterbalanced by the stability of long-term results.

Traumatic injuries of the craniovertebral junction (CVJ) area are common and frequently the outcome of motor vehicle accidents, falls, and diving accidents. To define and characterize CVJ traumatic injuries, some international classifications are currently in use, and they are thought and focused on junction bone fracture. However, recent data point out a major important role of the CVJ ligaments and membranes in traumatic injuries with a secondary function of the osseous structures. Emphasizing the correct role of the ligaments and membranes is extremely important for determining appropriate medical or surgical planning for patients and also to design new CVJ injury classifications. We reviewed every recent major publication on the ligaments and membranes of the CVJ area. We divided the information into sections concerning anatomy, embryology, biomechanics, trauma, and CVJ bone fractures. A role of the ligaments and membranes in the traumatic injuries of the CVJ area has often been recognized; but only recently, with the increase in the knowledge of the anatomic and biomechanical junction area, supported by neuroradiological tools (magnetic resonance imaging) and a more detailed traumatic injuries assessment, has the role of the ligaments and membranes been highlighted. Ligaments and membranes have a pivotal role in each junctional ability and are the key to orienting any medical or surgical indications in this unique area of the spine.

Object The goal of this study was to compare long-term results of surgery with the outcomes of conservative treatment in patients with asymptomatic lipomas of the conus medullaris. Methods The parents of 56 consecutive children with a diagnosis of asymptomatic lipoma of the conus medullaris underwent detailed neurosurgical consultation. The pros and cons of both prophylactic surgery and conservative treatment were carefully presented. Both options were offered, and the parents were free to choose the preferred management. A total of 32 children underwent surgical treatment, and 24 were conservatively treated. Afterward, all patients entered the same protocol of serial neurological and urological follow-up at the Centro Spina Bifida. The mean follow-up periods were 9.7 years in the surgical treatment group and 10.4 years in the conservative treatment group. Results Permanent surgical morbidity was 3.1% (1 patient). During follow-up, tethered cord syndrome occurred in 9.7% of the surgically treated patients (3 of 32 patients) and in 29.1% of the conservatively managed children (7 of 24 patients). This difference did not result in statistical significance, but a clear trend in favor of surgery emerged. Young age at surgery and a cord/sac ratio < 50% appeared to be determining factors in the prevention of subsequent tethered cord syndrome. Conclusions The small size of this series does not provide enough statistical evidence that surgical treatment can really improve the natural history of asymptomatic lipomas of the conus medullaris. Nevertheless, surgery appears at least advisable since it reduces by 75% the odds of TCS (p = 0.067), which is quite close to statistical significance.

BACKGROUND: Intracranial cysts containing cerebrospinal fluid (CSF) may be developmental or acquired. OBJECTIVE: To analyze the results of endoscopic neurosurgery in the management of intracranial CSF cysts. METHODS: In a 7-year period, 64 consecutive patients underwent endoscopic neurosurgery for CSF cysts. Group 1 consisted of 13 patients with acquired cysts; group 2 included 51 patients with developmental cysts. In all cases, the cyst walls were fenestrated through small burr holes with frameless guided operative endoscopes. Follow-up ranged from 1 to 6 years (mean, 3.4 years). RESULTS: There were no mortality and no permanent morbidity, apart from a patient (1.6%) who remained neurologically intact but required ventriculoperitoneal shunting because of intraoperative hemorrhage. The planned fenestrations could be performed in all patients except 2, owing to thick, opaque cyst walls. In group 1, 6 patients fully recovered and remained intact throughout the follow-up, whereas 7 improved but had various degrees of neurological disabilities that were related to their initial diseases. Radiological results were excellent in all cases. In group 2, there were 7 asymptomatic patients who remained unchanged and 44 "symptomatic" patients: 40 (91 %) clinically improved, 4 (9%) remained unchanged, and none worsened. Cyst size decreased in 37 patients (74%) and remained unchanged in 13 (26%). CONCLUSION: In this series, patients of different ages, harboring cysts of various sizes and locations, could be satisfactorily treated with endoscopic neurosurgery.

The possibility of an association between Dandy-Walker malformation and occipital meningocele is well-known. However, just an overall number of about 40 cases have been previously reported. Giant occipital meningocele has been described only in three newborns. Incidence, pathology, clinical presentation, and proper management of this association are still poorly defined.An 8-year-old boy with Dandy-Walker malformation and giant (25 cm in diameter) occipital meningocele is presented. This boy was born without any apparent occipital mass and harbored no other significant malformations including hydrocephalus. On admission, he was neurologically intact and the giant occipital mass presented partially calcified cyst walls. Treatment consisted of the excision of the occipital malformation, cranioplasty, and cysto-peritoneal shunt. Outcome was excellent.To the best of our knowledge, among the few reported patients with Dandy-Walker malformation associated to occipital meningocele, this is the oldest one and the one with the largest occipital meningocele; he is unique with calcified walls of the occipital meningocele and the only one who survived the repair of the giant malformation. In Dandy-Walker malformation, occipital meningocele may develop and grow regardless of hydrocephalus. Giant size may be reached and the cyst may become calcified. Surgical repair may warrant favorable outcome.

ObjectThe authors report on a series of 145 consecutive patients with different types of spine lesions surgically treated via an anterior approach (AA) at the thoracic and lumbar levels during the past 10 years. Indications, techniques, and surgical results are described.MethodsThis series included 92 patients with fractures, 30 with neoplasms, 13 with thoracic disc hernias, and 10 with spinal infections. Based on the lesion to be addressed, the AA was used for lesion excision, corpectomy, vertebral body reconstruction with cages, realignment, and/or plating or screwing. The approach was extracavitary in 55 patients and intracavitary in 90. In 126 patients (86.8%), neural decompression and spine stabilization were achieved via a stand-alone AA (SA-AA), whereas 19 patients (13.1%) were treated using a 2-stage anteroposterior approach. This circumferential approach was reserved for select cases of severe traumatic dislocation, particular types of tumors, or specific anatomical locations. The authors developed a simple neuronavigation-based method of identifying the severely injured patients who were eligible for the SA-AA by evaluating the angle of lateral dislocation.ResultsThere were no deaths and no instances of major surgery-related morbidity. Minor morbidity was almost always transitory and was reported in 13 patients (8.9%). Neurological improvement was reported in 20% of injured patients with a preoperative incomplete lesion. Postoperatively, all patients were able to stand or at least sit without load pain. During the follow-up (mean ± standard deviation 3.8 ± 2.4 years), there were no cases of failure, fracture, dislocation, or bending of the anterior instrumentation, and the rate of pseudarthrosis was 0%.ConclusionThe anterior route provides direct access to most spine diseases and allows optimal neural decompression and the possibility of adequate realignment and strong reconstruction/fixation. Stability of the vertebral column is achieved, resolution of clinical pain is rapid and almost complete, and the rate of surgical complications is very low. The authors assert that the SA-AA offers so many advantages and has such good results that the 2-stage anteroposterior approach can be reserved for a minority of select cases and that the time for using the posterior approach alone is over.

Midline congenital abnormalities of the skin may be related to subjacent visceral developmental defects. We report on three unrelated children presenting a small midline pit on the bridge of the nose from which a small tuft of hair was emerging. This presentation is the hallmark of a nasal dermoid sinus cyst. In one of the patients the sinus tract was connected to the anterior cerebral fossa and required neurosurgical management. Although midline cutaneous abnormalities may look trivial, dermatologists should keep a high index of suspicion for possible association with more severe underlying defect. The diagnosis of nasal dermoid sinus cyst requires appropriate imaging studies to determine the extension of the sinus tract thus allowing proper management.

Treatment of cerebral aneurysm has changed greatly over the last several years. Although surgery was the treatment of choice for decades, coiling is coming into more prevalent use now. This article highlights when each modality should be used.

The aim of this paper is to discuss the problems of craniocervical instability and craniocervical fusion in infancy. Despite the relative frequency of carniovertebral joint malformations, actual instability is quite rare in infancy. An infant 8 months of age presented with tetraparesis and sleep apnea due to a complex malformation of her craniovertebral joint. An initial attempt at conservative treatment using a rigid neck collar failed, so the patient was surgically managed by the onlay placement of two autologous rib grafts. The rigid collar was maintained for 5 months. Both rib grafts progressively reabsorbed within a few months, while the clinical deficits recurred. Reoperation consisted of occipitocervical interposition of two robust struts of banked cadaveric adult fibula. This time, the skull appeared mature enough to allow immobilization by the halo system. Adequate occipitocervical fusion was eventually achieved, and the patient fully recovered. To the best of our knowledge, there is no other reported case of an infant undergoing craniovertebral fusion using cadaveric adult bone. When screw placement is not considered advisable to manage small infants, appropriate stability may be obtained using struts of robust cadaveric bone. A meticulous carpentry technique with graft interposition under compression and adequate postoperative immobilization remains mandatory.

Occipital anomalies (in particular flattening) may be related to several conditions including craniosynostosis. In general, there are three main groups of occipital flattening: 1) positional plagiocephaly, 2) lambdoid craniosynostosis, and 3) alterations related to other conditions (i.e. other types of craniosynostosis, cervical malformations, and so forth). Proper differential diagnosis is mandatory. Physical examination often allows the diagnosis, but there may be cases requiring radiological assessments.Positional forms are the most common and usually need no surgical correction, even though severe deformities that persist beyond the year of age may require surgery. Lambdoid craniosynostoses are quite rare and do require surgical correction. Different operative techniques have been proposed, but the intervention usually consists of a posterior cranial expansion. Occipital anomalies related to other conditions usually require a tailored treatment addressed to manage the alterations responsible for the deformity.

Objective This report focuses on the surgical management of aggressive intracranial dural arteriovenous fistulae (d-AVFs), which are defined as fistulae with arterialized leptomeningeal veins (red veins). Particular attention is paid to the accurate identification of the venous drainage pattern and to the choice of the proper treatment strategy. Methods Thirty-four consecutive patients with aggressive intracranial d-AVFs were treated between 1994 and 1998. Angiographic studies allowed the identification of two main types of aggressive lesions, i.e., d-AVFs with sinus drainage and reflow into leptomeningeal veins (12 patients), which we designated sinus fistulae, and d-AVFs drained exclusively by leptomeningeal veins without sinus interposition (22 patients), which we designated nonsinus fistulae. All patients underwent surgical treatment, which consisted of resection of the fistulous sinus tract in 12 cases of sinus fistulae and interruption of the draining veins at their dural origin in 22 cases of nonsinus fistulae. Surgical preparation via multistage transarterial embolization was required in all 12 cases of sinus fistulae and in 4 of 22 cases of nonsinus fistulae. Results The mortality rate was 0%, and there were no instances of lasting morbidity. Radioanatomic cures were achieved in all cases. There was no case of venous hypertension or venous infarction after resection of the affected sinus or interruption of the draining veins. No arteriovenous shunts recurred during the follow-up period. Conclusion Careful preoperative identification of the venous drainage pattern seems critical for planning of the correct surgical strategy to treat aggressive intracranial d-AVFs. If the fistula exhibits sinus drainage with reflow into leptomeningeal veins, surgical excision of the fistulous sinus segment represents a safe and definitive treatment option. In these cases, the affected sinus may be safely removed, provided that preoperative angiograms demonstrate participation of the sinus in drainage of the lesion, indicating that the sinus is nonfunctional. Conversely, if the fistula exhibits pure leptomeningeal drainage, the sinus does not participate in drainage of the lesion and cannot be excised. In these cases, the best treatment involves interruption of the draining veins at the point at which they exit the dural wall of the sinus. This simple easy treatment has been proven to be safe and highly effective in permanently eliminating arteriovenous shunts.

Aneurysms of the vertebro-basilar junction area have been considered the most difficult to be surgically treated because of their deep location, the proximity of the brain stem and the cranial nerves. However, at present, new endovascular techniques and new transbasal surgical approaches offer valuable management strategies. This paper concerns six consecutive patients whom we managed either endovascularly or surgically during a period of eighteen months. Direct surgical treatment was offered to two young patients with relatively small aneurysms in good neurological condition, whereas the other four patients initially underwent an endovascular attempt at aneurysm obliteration using the Guglielmi detachable coil system. Unfortunately, interventional neuroradiology failed in three cases, and surgery had to be re-considered. Accordingly, a total of five patients underwent surgical clipping through the combination of a transmastoid retrolabyrinthine approach with the suboccipital lateral approach. This combination of approaches provided a good control of both vertebral arteries and basilar artery, and allowed the aneurysm to be correctly clipped in all cases. Good long-term results were achieved in all cases but one. Based on this preliminary experience, we would stress the importance of a multidisciplinary approach with a treatment calibrated for each single case. Furthermore, if surgery is to be performed, the combination of transmastoid-retrolabyrinthine and suboccipital lateral approaches provides a wide exposure of the whole vertebro-basilar junction area and allows good access to the lesion.

Neuroendoscopy is presently a routine procedure in most neurosurgical services. It was originally developed to deal with obstructive hydrocephalus, but modern technical advancements have made possible to face also other neurological diseases including brain tumors. Presently, small endoscopes with exquisite detail visualization, high resolution screens, 3D cameras, dedicated ultrasonic aspirators and laser beams offer therapeutic options which could be just dreamed a few years ago. The friendly interface with image-guided systems further improves the technique. Nowadays, the golden standard treatment of ventricular tumors includes biopsy under endoscopic control and (if necessary) treatment of the associated hydrocephalus during the same procedure. Endoscopic biopsy may be followed (if indicated) by traditional microsurgery, but it may also remain the only surgical procedure needed since several midline tumors either do not require surgical excision or may be managed by radiosurgery, chemotherapy, and/or radiation-therapy. Another endoscopic option is the fenestration/aspiration of cystic space occupying lesions. This technique may result very useful in selected cases of craniopharyngioma, metastases, abscesses and cystic tumors in general. This treatment aims to the endoscopic cyst shrinkage followed by radiosurgery or other adjunctive therapies. Moreover, there are particular expansive lesions which may be even endoscopically excised. This is especially the case of 3rd ventricle colloid cysts, for which endoscopy has become the first choice treatment in a lot of neurosurgical units. Finally, most CSF-cysts (arachnoid, choroid, ependymal, post-surgical, post-hemorrhagic) may be endoscopically treated thus avoiding open surgery or cysto-peritoneal shunting.

A previously healthy man underwent endoscopic biopsy for a suspected pineal germinoma. Histology and immunohistochemistry did not confirm the preoperative diagnosis, and neurosarcoidosis was hypothesised because of the presence of granulomatous reaction. The patient remained in good health for 3 years and was still asymptomatic when a control MRI showed metastasis implantation along the endoscopic route. A redobiopsy provided the diagnosis of germinoma, but was complicated by severe ventricular haemorrhage requiring emergency clot excision. Postoperative clinical conditions were so severe that the treatment of germinoma was postponed. Three months later, repeated MRI showed tumour progression. Chemotherapy promoted good tumour regression so that the treatment was completed by radiation therapy. The tumour completely disappeared on MRI, but the patient remained severely disabled because of the haemorrhage.

Vittorio Marchi was an Italian neuroscientist who lived during the second half of the 19th century. He is generally remembered for developing a novel staining method to trace the degeneration of nervous fibers in lesions of the central nervous system. This osmium-based method was used worldwide for approximately a century and continues to be cited in modern textbooks. Despite several important original scientific contributions, Marchi was never awarded a university chair. Discouraged, he left to practice neurology in a rural area but continued to write outstanding articles, and in 1897, he became a pioneer of meningioma surgery. Today, he is remembered only in specialized contexts. We hope this article can provide the recognition that Marchi deserves.

STUDY DESIGN Retrospective study. OBJECTIVE The aim of this study is to show that a single traumatic fracture of the atlas is unusual but not rare and requires specific management strategies, which can be highlighted by the study of the junctional ligaments and membranes. SUMMARY OF BACKGROUND DATA A single traumatic fracture of the atlas arch is considered a rare event and has been analyzed in few case reports. Ligaments and membranes play a primary role in providing stability to the craniovertebral junction area. METHODS Here, we report 10 cases of a single traumatic fracture of the atlas arch: 4 cases were part of our series of spine injuries and were studied by magnetic resonance imaging during the acute phase to assess the junctional ligaments. The remaining 6 cases were obtained from the Literature. RESULTS Indirect signs of a traumatic ligamentous injury were found only in 1 patient. However, in all cases, these fractures were considered biomechanically stable and all patients were discharged with a rigid collar. During the follow-up, all patients reported an improvement in their neck pain and tenderness, with radiologic signs of bone healing. CONCLUSIONS A single fracture of the atlas arch is an unusual traumatic event that results from slow-force impact. Medical experience in treating this specific fracture subtype is nowadays poor because of the paucity of the literature; thus, this unusual condition may either be underestimated or even overstudied and overtreated. Nowadays, it seems reasonable to assume that the management should be conservative in all cases and that magnetic resonance imaging often plays only a marginal role.

The craniovertebral junction is a specific region of the spine with unique anatomical and biomechanical properties that yields a wide variety of injury patterns. Junctional traumatic fractures and/or dislocations are widely reported in clinical practice, but we could identify only a subgroup of upper cervical spine traumatic injuries with very few cases reported in the literature, and for this reason may be considered rare. In some of these cases, the absence of spinal biomechanical instability, in association with moderate clinical symptoms (neck stiffness and pain) and the difficulty in fracture identification through standard cervical radiographs, leads to a high percentage of missed injuries. In other cases, traumatic events have been commonly described only in autopsy series due to the high degree of spinal biomechanical instability. Herein, we have summarized all the relevant literature concerning this issue and also included our cases, with the aim of emphasizing prompt diagnosis and correct management. We provide a guide for correctly identifying “rare” craniovertebral junction traumatic injuries.

Objective This study aims to identify the angiographic parameters that could help predict the risks of hyperemic complications (HCs) in the treatment of cerebral arteriovenous malformations (AVMs). Methods The charts and the pre- and post-treatment angiographies of 400 consecutively treated patients with cerebral AVMs were retrospectively reviewed. Several parameters were analyzed: grade, size, drainage depth, number of the “recruited veins” (veins coming from the brain and joining the main AVM drainage), and “venous times” (the times of visualization of the nidus and the main drainage, of the “recruited veins,” and finally of the normal cerebral veins). On this basis, two groups were identified: group 1, consisting of 309 patients (77.3%) with normal/subnormal venous times, and group 2, consisting of 91 patients (22.7%) with altered venous times and venous engorgement. Finally, the weight of the various parameters was measured by univariate and multivariate analyses. Results An overall number of 245 patients (61.2%) achieved favorable outcomes. HCs occurred in a total of 28 patients (7%): No patient belonged to group 1, so that these adverse effects were reported in 30.7% of group 2 patients. The presence of deep drainage and the number of recruited veins resulted in statistically significantly different impacts on the risk of the different grades. Conclusions The statistical analysis allowed the identification of three different risk scores, which were named Nig-scores (Niguarda scores). Nig-score 0 means no risk of HCs and concerns patients without altered venous times; Nig-score 1 represents patients with intermediate risk, that is, with moderately altered venous times and few recruited veins; Nig-score 2 indicates high risk of HCs and refers to patients with markedly altered venous times.