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Background: Chiari malformation type 1 (CM1) remains a complex neurosurgical condition with ongoing debate regarding its optimal management. Methods: This narrative review examines key controversies surrounding the pathophysiology, surgical indications, and treatment strategies for CM1. Results: We highlight the challenges posed by the wide spectrum of CM variants and the evolving understanding of its association with syringomyelia, basilar invagination, and craniovertebral instability. Emerging surgical techniques, including minimally invasive approaches and the use of new technologies such as endoscopes and exoscopes, are evaluated for their potential to improve outcomes. Recent consensus guidelines are also discussed. Conclusions: The need for individualized treatment plans for CM1 is emphasized, with special focus put on the connection between novel pathophysiological insights, technological advancements and opportunities for a more nuanced surgical management. Further research is necessary to establish solid foundations for more individualized treatments.

BackgroundSyringomyelia 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.MethodsA 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 <75% were revised and entered round 2. Round 3 was face-to-face, during the Chiari Consensus Conference (Milan, November 2019).ResultsThirty-one out of 32 Jury members (6 neurologists, 4 neuroradiologists, 19 neurosurgeons, and 2 patient association representatives) participated in the consensus. After round 2, a consensus was reached on 57/63 statements (90.5%). The six difficult statements were revised and voted in round 3, and the whole set of statements was further discussed and approved.ConclusionsThe consensus document consists of 63 statements which benefited from expert discussion and fine-tuning, serving clinicians and researchers following adults with Chiari and syringomyelia.

Purpose: Cadaveric dissection is a cornerstone of neurosurgical education, providing trainees with a realistic 3D understanding of anatomy and a safe environment to practice surgical approaches. A preservation technique was developed that merges the advantages of fresh-frozen and embalmed cadavers, maintaining tissue realism while enhancing durability. This approach preserves flexibility and natural color, improves anatomical detail, and creates a safe, long-lasting model ideal for neurosurgical training. Methods: Four specimens were thawed, cannulated, and irrigated before implementing a protocol consisting of low concentration formaldehyde with glycerol and ethanol for extended preservation. The specimens were prepared for both neurosurgery training and educational purposes, and their condition was evaluated with a semi-quantitative scale. Each specimen was evaluated independently by two raters, blinded to the time-point, using a semi-quantitative scale anchored to predefined criteria (0–3 per domain). Inter-rater reliability was calculated using the intraclass correlation coefficient (ICC [2,k]) for continuous scores and Cohen’s κ for categorical agreement. Results: Over nine years of intermittent use, the specimens remained in good condition: tissues retained sufficient softness for dissection, injected vessels stayed vivid in color, and no foul odor or microbial growth was observed. The evaluation employed a semi-quantitative scale, with results ranging from 11/14 to 14/14. The mean values demonstrate stable tissue quality over time, with only minor variations in color and perfusion. The inter-rater reliability was high (ICC = 0.91; κ = 0.88). Conclusions: The preservation method leverages the strengths of both fresh-frozen and embalmed models. The results suggest feasibility of long-term reuse, although further quantitative validation is needed.

Background: Different lateral and postero-lateral cranial approaches to the petroclival region and to the mid-upper brain stem have been described so far, some of which require extensive osseous demolition and possible damage of neurovascular structures. Neuronavigational systems are now extensively available for preoperative planning and intraoperative navigation to assist the surgeons in choosing the optimally invasive approach for each pathology. Herein, we describe a detailed navigation-augmented morphometric analysis to bring insight into the usefulness of an anterior petrosectomy (AP) to handle lesions in the petroclival region. Methods: Eight cadaveric, silicone injected heads were used. A total of 14 approaches (AP, n = 8; retrosigmoid, RS, n = 6) using a standard microsurgical dissection technique were performed. All specimens had preoperative CT and MRI scans, as well as a post-dissection CT. The neuronavigational system was used for distance measurements, craniotomy sizes and surgical corridor volumes, for each approach. Results: The distance from the skull surface to the petrous apex was significantly shorter in the AP approach when compared with the RS (46.0 ± 1.9 mm versus 71.3 ± 1.8 mm, respectively, p < 0.001). Although the craniotomy size was not different, the volume of the surgical corridor was significantly larger with the AP approach (21.31 ± 1.91 cm3 vs. 13.39 ± 1.8 cm3). The AP approach increased the length of the basilar artery exposure from 6.9 ± 1.5 mm (obtained with a standard subtemporal approach) to 22.1 ± 1.7 mm (p < 0.0001). Conclusions: The surgical corridor to the petroclival region achieved by virtue of an AP was significantly larger and featured shorter working distances, resulting in a higher degree of surgical freedom. Although significant individual anatomical variations of fundamental neurovascular and bony structures were found, these difficulties were overcome by careful pre- and intraoperative use of neuronavigation.

Spinal cord injury (SCI) is a devastating condition with a significant medical and socioeconomic impact. To date, no effective treatment is available that can enable neuronal regeneration and recovery of function at the damaged level. This is thought to be due to scar formation, axonal degeneration and a strong inflammatory response inducing a loss of neurons followed by a cascade of events that leads to further spinal cord damage. Many experimental studies demonstrate the therapeutic effect of stem cells in SCI due to their ability to differentiate into neuronal cells and release neurotrophic factors. Therefore, it appears to be a valid strategy to use in the field of regenerative medicine. This review aims to provide an up-to-date summary of the current research status, challenges, and future directions for stem cell therapy in SCI models, providing an overview of this constantly evolving and promising field.

Background: Ventriculoperitoneal shunting (VPS) is the mainstay of treatment for most forms of hydrocephalus; VPS infection (VPSI) is a leading cause of shunt-related morbidity and mortality. A meta-analysis of the existing literature on risk factors for VPSI is currently lacking. Herein, the authors performed a systematic review and meta-analysis to evaluate the role of different clinical factors in the development of VPSI. Methods: A systematic search in the PubMed, Scopus, and Cochrane databases was performed to identify studies comparing patients developing VPSI to controls. The following data were extracted where available: number of patients who developed VPSI vs. number of patients with a regular course, demographics (gender, age at VPS insertion, age > 18 years), average length of hospital stay before shunt implant (days), aetiology of hydrocephalus (tumour-associated hydrocephalus; post-haemorrhagic hydrocephalus; congenital hydrocephalus; spinal dysraphism-associated hydrocephalus; post-infectious hydrocephalus; post-traumatic hydrocephalus; post-craniotomy hydrocephalus), and hydrocephalus type (obstructive hydrocephalus, communicating hydrocephalus, normal-pressure hydrocephalus—NPH). Results: Five studies including 2333 patients (225 with VPS infection) were analysed. Tumour-related hydrocephalus was linked to a lower infection risk (OR 0.418; p < 0.001), while congenital hydrocephalus (OR 2.502; p < 0.001) and spinal dysraphism (OR 2.359; p < 0.001) increased the risk. Conclusions: VPSI represents a serious complication after shunt surgery. Our meta-analysis identifies three key factors influencing the risk of VPSI. VPS-centred, large multicentre prospective studies are needed to possibly confirm the role of the factors we identified and to identify additional ones, enabling earlier detection of VPSI and allowing for better patient care.

Purpose Retro-odontoid pseudotumor, not related to inflammatory or traumatic conditions, is an uncommon pathology. Atlanto-axial instability has been advocated to explain the pathophysiology of retro-odontoid pseudotumor’s formation and growth. Despite pseudotumor direct removal through transoral or lateral approach represented the main surgical strategy for a long time, in the last decade several authors highlighted the possibility to treat retro-odontoid pseudotumor by occipito-cervical or C1–C2 fixation without removal of the intracanalar tissue. The goal of this study is to analyze the data collected in a series of patients suffering from cervical myelopathy due to non-inflammatory, degenerative retro-odontoid pannus and treated by posterior C1–C2 fixation. The relevant literature is also reviewed. Methods Five patients, not suffering from inflammatory diseases, were treated between 2009 and 2012. Abnormalities of cranio-cervical junction and/or lower cervical spondylotic degeneration were observed in all patients. No evidence of atlanto-axial instability was demonstrated. Clinical and radiological evaluation included pre- and post-operative Nurick score as well as pre- and post-operative X-rays, CT and MRI. In one case, CT scan highlighted an eggshell calcification of the pannus. All patients underwent either a C1–C2 fixation (C1 lateral mass and C2 isthmus-pedicle screws) or occipito-cervical fixation (2 patients) in cases of C0–C1 fusion. Results Follow-up ranges from 22 to 45 months (mean 32) in four patients. One patient died of surgery-unrelated disease. Nurick score changes suggest a clinical improvement in four cases. Neuro-radiological evaluation shows a progressive but incomplete reduction of thickness of retro-odontoid pseudotumor in one patient, and its disappearance in the other three cases. A second-stage transoral or posterior lateral approach was not required. Conclusion Although the etiopathogenesis of non-inflammatory, i.e., degenerative, retro-odontoid pseudotumor is still controversial, our series (the second largest on degenerative retro-odontoid pannus in the literature) confirms that a posterior approach may be sufficient and transoral surgery is not required.

ABSTRACT Context: Diffuse idiopathic skeletal hyperostosis (DISH) or Forestier's syndrome may reduce vertebral mobility, thus affecting the stability of adjacent vertebral segments and promoting spinal stenosis, vertebral dislocation, and unstable fracture secondary to low-energy trauma. Aims: This study aimed to contribute with a case series of three patients affected by DISH undergone surgery with occipitocervical fixation for craniovertebral junction (CVJ) instability since the poor literature about CVJ instability and surgery in patients affected by DISH. Settings and Design: This was a multicentric case series. Subjects and Methods: Literature about CVJ instability and surgery in patients affected by DISH is poor. Thus, we present a case series of three patients affected by DISH, who underwent surgery with occipitocervical fixation with different clinical and radiological patterns. Results: CVJ represents one of the most mobile joints of the spine and is at greater risk for instability. Moreover, instability itself may act as primum movens for several degenerative conditions such as cervical spondylosis, ossification of the posterior longitudinal ligament, and cervical deformities. On the contrary, DISH itself may worsen CVJ instability because of subaxial spine stiffness. In case of DISH, the rigid unit formed by several ossified vertebral bodies acts as a long lever arm, increasing the forces applied to the hypermobile CVJ and reducing the dynamic buffer capability of ossified spine. On the other hand, vertebral instability increases the odds of fractures. In such cases, CVJ posterior instrumentation and fusion is an effective and feasible surgical technique, aimed to restore vertebral stability and to halt the progression of spinal stenosis. Conclusions: Due to the altered dynamics cervical spine along with the possible comorbidities, treatment indication and surgery for patients affected by DISH must be tailored case by case.

Background: Spontaneous intracranial hypotension (SIH) is a rare and frequently misdiagnosed disorder characterized by a low volume of cerebrospinal fluid (CSF) caused by the leakage of CSF through the spinal dural membrane. Patients with Marfan Syndrome (MS) and other connective tissue disorders are at an increased risk for dural ectasia, which may predispose them to spontaneous CSF leaks due to the structural weakness of their dural membranes. The management of SIH in MS patients is debated. Conservative measures, an epidural blood patch (EBP), and surgical treatments are the options generally provided. Methods: Herein, we report on the case of a 52-year-old female affected by MS, genetically confirmed, with a two-month history of sudden-onset, “thunderclap” headache, worsened in an upright position and horizontal diplopia. A Computed Tomography (CT) scan of the brain showed a bilateral chronic subdural hematoma, slit ventricles, and a caudal descent of the brainstem without overt tonsillar herniation. The Magnetic Resonance Imaging (MRI) scan of the whole spine revealed dural ectasia in the lumbosacral area and presacral perineural cyst without extradural CSF collection. The case was successfully managed with bed rest and high-dose corticosteroid therapy. Then, we discuss the pertinent literature, consisting of 25 papers dealing with the treatment of SIH in patients affected by MS. Results: The literature review yielded 25 papers dealing with SIH management in patients with MS, including 28 patients overall; 21 patients underwent EBP, of whom 7 patients had multiple procedures. Overall, in 23 cases (82%), the symptoms improved. In three cases, the patients were managed conservatively with bed rest. In three of these cases, there was an improvement. In one case, the surgical fenestration of two lumbar intradural spinal meningeal cysts was performed and the patient improved after the procedure. Our patient underwent 15 days of steroid therapy (dexamethasone iv 12 mg/day for 7 days, then reduced to 4 mg/day) and intravenous hydration (Ringer lactate 1500 mL/day). In ten days, the symptoms disappeared. At the 6-month follow-up, the patient was in good clinical condition, and a CT scan showed an almost complete regression of the bilateral subdural hematoma. Conclusions: The management of SIH in MS patients is still challenging. Patients with connective tissue disorders such as MS are at an increased risk for SIH. Few studies have assessed the management of these patients and different strategies. Our case and the available literature provide further data for this type of case.