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Background Endoscopic endonasal odontoidectomy (EEO) has been described as a potential approach for craniovertebral junction (CVJ) disease which could cause anterior bulbomedullary compression and encroaching. Due to the atlantoaxial junction’s uniqueness and complex biomechanics, treating CVJ pathologies uncovers the challenge of preventing C1–C2 instability. A large series of patients treated with endonasal odontoidectomy is reported, analyzing the feasibility and necessity of whether or not to perform posterior stabilization. Furthermore, the focus is on the long-term follow-up, especially those whom only underwent partial C1 arch preservation without posterior fixation. Methods This study is a retrospective analysis of patients with ventral spinal cord compression for non-reducible CVJ malformation, consecutively treated with EEO from July 2011 to March 2019. Postoperative dynamic X-ray and CT scans were obtained in each case in order to document CVJ decompression as well as to exclude instability. The anterior atlas‐dens interval, posterior atlas‐dens interval and C1–C2 total lateral overhang were measured as a morphological criteria to determine upper cervical spine stability. Results Twenty-one patients (11:10 F:M) with a mean age of 60.6 years old at the time of surgery (range 34–84 years) encountered the inclusion criteria. For all 21 patients, a successful decompression was achieved at the first surgery. In 11 patients, the partial C1 arch integrity did not require a posterior cervical instrumentation on the bases of postoperative and constant follow-up radiological examination. In 13 cases, an improvement of motor function was recorded at the time of discharge. Only one patient had further motor function improvement at follow-up. Among the patients that did not show any significant motor change at discharge, 4 patients showed an improvement at the last follow-up. Conclusions The outcomes, even in C1 arch preservation without posterior fixation, are promising, and it could be said that the endonasal route potentially represents a valid option to treat lesions above the nasopalatine line.

Objective Evaluate the effectiveness of surgery for pituitary adenomas in the elderly and the reliability of the Trouillas score in patients with ≥ 65 years old. Methods A single center retrospective observational study was conducted for the period 2012–2020 recruiting all the patients over 65 years old underwent to primary endoscopic endonasal removal of both functioning and non-functioning pituitary adenomas. Radiological and histopathological features were examined for classification. Time-to-relapse was estimated using Kaplan-Meier product limit. Results 103 patients were recruited. Mean age at surgery was 72.2 years (range 65–83). The majority of tumors were non-functioning macroadenomas (83%). A gross total resection (GTR) was achieved in 46/73 (63%) of young elderly (age 65–74) and 14/30 (47%) of old elderly (age ≥ 75). According to Trouillas' classification criteria, 57 tumors resulted to be grade 1a, 8 grade 1b, 33 grade 2a, and 5 grade 2b. The mean TTR was 32.3 months ± 18.5. Mitotic index (p = 0.024) and a Trouillas grade (p = 0.048) remained statistically significant at multivariate analysis. Patients with Trouillas grade 1a/2a showed better rates of TTR than patients with Trouillas grade 1b/2b (mean DFS: 82 vs 49 months, p = 0.006) although the number of patients representing the second group (13) demands a critical interpretation of the result. Conclusions The possibility to predict tumor’s regrowth can define different timing for follow-up and could justify a more aggressive surgical treatment even among elderly patients, aiming to achieve GTR and significantly reduce the need for redo surgery.

The aim of this study was to analyse whether significant differences exist between free-hand 3D planning-guided screw placement and 3D printed template guided CBT screw positioning in terms of accuracy, size of screws and potential complications.Data were extracted from a prospectively collected database and resumed in a retrospective study. Adult patients fixed with CBT screws for lumbar degenerative pathologies were considered for this analysis. A group of patients in which screws were placed by using 3D planning guided free-hand technique (PG) were compared to a second group of patients that underwent screw positioning by using customised 3D printed guides (TG). Analysed parameters were size of the screws, accuracy, clinical outcomes and complications.A total number of 1004 screws and 251 patients was evaluated for this study. The PG included 158 patients (632 screws) while the TG evaluated 93 patients (372 screws). The TG group involved screws of larger size from L3 to S1. Differences between the two groups in terms of accuracy parameters reached statistical significance (p ≤ 0.05).The use of 3D patient-matched template guides was able to safely increase mean diameter and length of cortical bone trajectory screws, and also improved the accuracy of screw placement. Following previous evidence about CBT biomechanical properties, these advantages could allow to increase fixation strength over traditional convergent pedicle screw trajectories. Further biomechanics studies would be needed.

Amino acid PET imaging has been used for a few years in the clinical and surgical management of gliomas with satisfactory results in diagnosis and grading for surgical and radiotherapy planning and to differentiate recurrences. Biological tumor volume (BTV) provides more meaningful information than standard MR imaging alone and often exceeds the boundary of the contrast-enhanced nodule seen in MRI. Since a gross total resection reflects the resection of the contrast-enhanced nodule and the majority of recurrences are at a tumor’s margins, an integration of PET imaging during resection could increase PFS and OS. A systematic review of the literature searching for “PET” [All fields] AND “glioma” [All fields] AND “resection” [All fields] was performed in order to investigate the diffusion of integration of PET imaging in surgical practice. Integration in a neuronavigation system and intraoperative use of PET imaging in the primary diagnosis of adult high-grade gliomas were among the criteria for article selection. Only one study has satisfied the inclusion criteria, and a few more (13) have declared to use multimodal imaging techniques with the integration of PET imaging to intentionally perform a biopsy of the PET uptake area. Despite few pieces of evidence, targeting a biologically active area in addition to other tools, which can help intraoperatively the neurosurgeon to increase the amount of resected tumor, has the potential to provide incremental and complementary information in the management of brain gliomas. Since supramaximal resection based on the extent of MRI FLAIR hyperintensity resulted in an advantage in terms of PFS and OS, PET-based biological tumor volume, avoiding new neurological deficits, deserves further investigation.

Lenz microphthalmia syndrome (LMS) is an allelic X-linked syndrome correlated to a null mutation of B cell lymphoma (BCL-6) corepressor (BCOR) gene, which is essential in the early embryonic development. Phenotypically, this rare hereditary syndrome is characterized by microphthalmia/anophthalmia and other eye disorders; mental disability; dental, ear, and digital abnormalities; and variable malformations affecting the heart, skeleton (limbs and/or spine), and genitourinary tract. In this paper, a case of a young adult with LMS affected additionally by immuno-hematological disturbances was treated with decompressive craniectomy after domestic accidental fall. Case description and a brief review of the current literature about this rare condition are presented here.

Purpose Congenital kyphosis (CK) is an uncommon condition that develops due to a defect of formation or segmentation of one or more vertebrae during the first weeks of embryonic life and can be frequently associated with abnormalities of the spinal cord. Meningocele manque (MM) is a rare congenital malformation consisting of intradural fibrous bands tethering the spinal cord, oftentimes in combination with a split cord malformation. The aim of this manuscript is to describe combined surgical management of a rare case of CK associated with double spinal cord tethering. Study design Case report. Methods We report the case of a 4-year-old boy presenting with a combination of congenital kyphosis due to L2 fully segmented posterior hemivertebra and double spinal cord tethering, at the level of the filum terminale and at L1-L2 due to an intradural ventral fibrous band. Results Spinal cord detethering and L2 hemivertebra resection with kyphosis correction were achieved with a single stage procedure. Segmental kyphosis was corrected from 45 degrees to 11 degrees post-operatively. Apart from a transitory sensory impairment, no immediate or delayed complications were noted and, at 2 years follow-up, correction and spinal alignment were stable. Conclusion To the best of our knowledge, this is the first case to report the simultaneous occurrence of congenital kyphosis and double spinal cord tethering due to MM and tethering of the filum terminale. Our case report shows that surgical correction of the deformity and spinal cord detethering can be safely and effectively achieved during the same surgical procedure.

Purpose To discuss a treatment algorithm for vertebral hemangioma in children. Methods Vertebral hemangioma (VH) is a rare cause of low back pain in children. In most cases, VHs present as incidental findings and do not require invasive diagnostic procedure. In case of symptomatic presentation, different approaches can be used. Over the years, we have developed a treatment algorithm for VH in children based on our clinical experience. In this manuscript, we propose a stepwise approach to treatment of VHs based on tumor extension and the degree of spinal cord/nerves compression with or without neurological deficit. Results According to the proposed protocol, we discuss two cases of aggressive VH treated at our institution by a multidisciplinary team. The first case is about a young girl treated with percutaneous one-level posterior instrumentation followed by medical adjuvant therapy for an L4 "Stage 3" VH. The second case is about an 8-year-old boy with rapidly progressive myelopathy due to T11 "Stage 4" VH treated with a combined anterior and posterior surgery (i.e., posterior decompression and fusion followed by vertebrectomy and expandable cage placement) after preoperative arterial embolization. Conclusion Given the lack of international guidelines and consensus with regard to treatment of VHs in children, we believe our proposal for a stepwise approach combining clinical and radiological characteristics of the lesion may help guide treatment of this condition in children.

Background: Gliomas are among the most challenging pathologies for neurosurgeons due to their infiltrative and recurrent nature in functionally relevant regions. Current knowledge confirms that gross total resection highly influence survival in patient with glioma. However, surgery performed in eloquent brain area, could seriously compromise the quality of life in patient with reduced life expectancy even more if it concerns the language function. Methods: 18 right-handed patients with perisylvian gliomas on the left hemisphere were prospectively analyzed over a period of 12 months. Standardized preoperative Diffusion-Tensor-Imaging based tractography of the five main language Tracts (Arcuate Fasciculus, Frontal Aslant Tract, Inferior Fronto-Occipital Fasciculus, Inferior Longitudinal Fasciculus, Uncinate Fasciculus) was navigated during the surgical procedure. Using a validated method, correlations were made between the pre-operative fascicles and their possible infiltration and surgical damage. The language status was assessed using the Aachen Aphasia Test. Results: In all nine patients who developed a permanent disorder there was pre-operative involvement of at least one fascicle and resection of at least one of these. In this way, areas of high risk of permanent language damage have emerged as a result of surgical injury: the temporoparietal junction, the middle portion of the FAT and the temporal stem. Conclusions: Navigated tractography has proven to be a user-friendly tool that can assess perioperative risk, guide surgical resection, and help the neurosurgeon to find that balance between tumor resection and function preservation.

Introduction Post-operative CSF leak still represents the main drawback of Endoscopic Endonasal Approach (EEA), and different reconstructive strategies have been proposed in order to decrease its rate. Objective To critically analyze the effectiveness of different adopted reconstruction strategies in patients that underwent EEA. Materials and methods Adult patients with skull base tumor surgically treated with EEA were retrospectively analyzed. Data recorded for each case concerned patient demographics, type of surgical approach, histotype, anatomical site of surgical approach, intra-operative CSF leak grade (no leak (INL), low flow (ILFL), high flow (IHFL)), reconstructive adopted strategy, Lumbar Drain positioning, post-operative CSF leak rate and intra/post-operative complications. Results A total number of 521 patients (January 2012-December 2019) was included. Intra-operative CSF leak grade showed to be associated with post-operative CSF leak rate. In particular, the risk to observe a post-operative CSF leak was higher when IHFL was encountered (25,5%; Exp(B) 16.25). In particular, vascularized multilayered reconstruction and fat use showed to be effective in lowering post-operative CSF leaks in IHFL (p 0.02). No differences were found considering INL and ILFL groups. Yearly post-operative CSF leak rate analysis showed a significative decreasing trend. Conclusion Intra-operative CSF leak grade strongly affected post-operative CSF leak rate. Multilayer reconstruction with fat and naso-septal flap could reduce the rate of CSF leak in high risk patients. Reconstructive strategies should be tailored according also to the type and the anatomical site of the approach.

Deep Brain Stimulation (DBS) of the subthalamic nucleus (STN) is a well-recognized intervention for Parkinson’s Disease (PD). We used LEAD-DBS, a toolbox facilitating DBS electrode reconstructions and computer simulations based on postoperative MRI and CT imaging, to investigate the interaction and followed benefits of electrical field generated by STN-DBS and surrounding areas, such as caudal Zona Incerta (cZI). Thirty-two PD patients, treated with directional STN-DBS in the period 2016–2018 at the Walton Center NHS Foundation Trust, were retrospectively recruited. Their MRI and CT imaging were analyzed with LEAD-DBS to measure the volume of tissue activated (VTA). Considering the clinical outcome based on the UPDRS III score improvement of 62.65% at 6 months follow up, we found a VTA intersection of 21.5% with motor STN and 61.7% with cZI. These observations may support the contribution of cZI deep stimulation to improve clinical outcome of PD patients treated with DBS, promoting the intriguing path of dual targeting.