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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.

External ventricular drainage is often considered a life-saving treatment in acute hydrocephalus. Given the large number of discussion points, the ideal management of EVD has not been completely clarified. The objective of this study was to review the most relevant scientific evidence about the management of EVD in its main clinical scenarios. We reviewed the most recent and relevant articles about indications, timing, management, and complications of EVD in neurocritical care, with particular interest in patients with subarachnoid hemorrhage (SAH), severe traumatic brain injury (TBI), and intraventricular hemorrhage (IVH) using the following keywords alone or matching with one another: intracranial pressure, subarachnoid hemorrhage, traumatic brain injury, intraventricular hemorrhage, external ventricular drainage, cerebrospinal shunt, intracranial pressure monitoring, and ventriculoperitoneal shunt. In the management of EVD in SAH, the intermittent drainage strategy is burdened with an elevated risk of complications (e.g., clogged catheter, hemorrhage, and need for replacement). There seems to be more ventriculoperitoneal shunt dependency in rapid weaning approach-managed patients than in those treated with the gradual weaning approach. Although there is no evidence in favor of either strategy, it is conventionally accepted to adopt a continuous drainage approach in TBI patients. Less scientific evidence is available in the literature regarding the management of EVD in patients with severe TBI and intraparenchymal/intraventricular hemorrhage. EVD placement is a necessary treatment in several clinical scenarios. However, further randomized clinical trials are needed to clarify precisely how EVD should be managed in different clinical scenarios.

Introduction: Traumatic brain injury (TBI) represents a severe pathology with important social and economic concerns, decompressive craniectomy (DC) represents a life-saving surgical option to treat elevated intracranial hypertension (ICP). The rationale underlying DC is to remove part of the cranial bones and open the dura mater to create space, avoiding secondary parenchymal damage and brain herniations. The scope of this narrative review is to summarize the most relevant literature and to discuss main issues about indication, timing, surgical procedure, outcome, and complications in adult patients involved in severe traumatic brain injury, underwent to the DC. The literature research is made with Medical Subject Headings (MeSH) terms on PubMed/MEDLINE from 2003 to 2022 and we reviewed the most recent and relevant articles using the following keywords alone or matched with each other: decompressive craniectomy; traumatic brain injury; intracranial hypertension; acute subdural hematoma; cranioplasty; cerebral herniation, neuro-critical care, neuro-anesthesiology. The pathogenesis of TBI involves both primary injuries that correlate directly to the external impact of the brain and skull, and secondary injuries due to molecular, chemical, and inflammatory cascade inducing further cerebral damage. The DC can be classified into primary, defined as bone flap removing without its replacement for the treatment of intracerebral mass, and secondary, which indicates for the treatment of elevated intracranial pressure (ICP), refractory to intensive medical management. Briefly, the increased brain compliance following bone removal reflects on CBF and autoregulation inducing an alteration in CSF dynamics and so, eventual complications. The risk of complications is estimated around 40%. The main cause of mortality in DC patients is due to brain swelling. In traumatic brain injury, primary or secondary decompressive craniectomy is a life-saving surgery, and the right indication should be mandatory in multidisciplinary medical–surgical consultation.

IntroductionAggressive osteoblastoma (AO) represents a rare tumor with borderline features between benign osteoblastoma and osteosarcoma. Having a local aggressive behavior without metastasizing attitude, radical excision is a mainstay treatment. Conversely, spine fusion technique is still debated. We report a rare case of cervicothoracic junction (CTJ) AO and the tailored decision-making process to choose the best treatment.Case presentationA 34-year-old man complaining of neck pain was admitted to our department. Cervicothoracic MRI revealed a well-circumscribed lesion involving C7 left lamina with cortical erosion and mild spinal canal invasion. Additionally, STIR sequences exhibited a bright signal spreading through the posterior third of the C7 and T1 vertebrae which on T1-weighted and T2-weighted sequences appeared isointense and hyperintense, respectively. Therefore, the patient underwent a C7 laminotomy. Histology revealed an aggressive variant of osteoblastoma. Therefore, tumor was classified as AO and surgical management was reconsidered. A combined anterior and posterior approach was recommended to reach oncological radicality and spinal stability. At 6-years follow-up, patient remained neurologically intact without signs of recurrence and/or of instability.DiscussionDue to its rarity and mimicking features, diagnosis of AO results challenging. Due to its aggressive behavior, radical surgery is the mainstay treatment. Conversely, the most suitable fusion technique is still debated. A proper surgical management should be focused on oncological radicality to guarantee the total tumoral removal avoiding progression or recurrences. Similarly, a proper evaluation of the long-term spinal balance should be assessed to avoid developing of spinal deformities or instrumentation failures.

Background Questions remain on the optimal management of subarachnoid hemorrhage (SAH) patients once they are admitted to the referring center, before and after the aneurysm treatment. To address these issues, we created a consensus of experts endorsed by the Italian Society of Anesthesia and Intensive Care (SIAARTI) to provide clinical guidance regarding this topic. Specifically, in this manuscript (part 2), we aim to provide a list of experts’ recommendations regarding the management of SAH patients in a center with neurosurgical/neuroendovascular facilities after aneurysm treatment. Methods A multidisciplinary consensus panel composed by 24 physicians selected for their established clinical and scientific expertise in the acute management of SAH patients with different specializations (anesthesia/intensive care, neurosurgery, and interventional neuroradiology) was created. A modified Delphi approach was adopted. Results A total of 33 statements were discussed, voted, and approved. Consensus was reached on 30 recommendations (28 strong and 2 weak). In 3 cases, where consensus could not be agreed upon, no recommendation was provided. Conclusions This consensus provides practical recommendations (and not mandatory standard of practice) to support clinician’s decision-making in the management of SAH patients in centers with neurosurgical/neuroendovascular facilities after aneurysm securing.

Background Issues remain on the optimal management of subarachnoid hemorrhage (SAH) patients once they are admitted to the referring center, before and after the aneurysm treatment. To address these issues, we created a consensus of experts endorsed by the Italian Society of Anesthesia and Intensive Care (SIAARTI). In this manuscript, we aim to provide a list of experts’ recommendations regarding the early management of SAH patients from hospital admission, in a center with neurosurgical/neuro-endovascular facilities, until securing of the bleeding aneurysm. Methods A multidisciplinary consensus panel composed of 24 physicians selected for their established clinical and scientific expertise in the acute management of SAH patients with different background (anesthesia/intensive care, neurosurgery, and interventional neuroradiology) was created. A modified Delphi approach was adopted. Results Among 19 statements discussed. The consensus was reached on 18 strong recommendations. In one case, consensus could not be agreed upon and no recommendation was provided. Conclusions This consensus provides practical recommendations for the management of SAH patients in hospitals with neurosurgical/neuroendovascular facilities until aneurysm securing. It is intended to support clinician’s decision-making and not to mandate a standard of practice.

BackgroundThe immediate management of subarachnoid hemorrhage (SAH) patients in hospitals without neurosurgical/neurointerventional facilities and their transfer to a specialized center is challenging and not well covered in existing guidelines. To address these issues, we created a consensus of experts endorsed by the Italian Society of Anesthesia and Intensive Care (SIAARTI) to provide clinical guidance.MethodsA multidisciplinary consensus panel composed by 19 physicians selected for their established clinical and scientific expertise in the acute management of SAH patients with different specializations (anesthesia/intensive care, neurosurgery and interventional neuroradiology) was created. A modified Delphi approach was adopted.ResultsA total of 14 statements have been discussed. Consensus was reached on 11 strong recommendations and 2 weak recommendations. In one case, where consensus could not be agreed upon, no recommendation could be provided.ConclusionsManagement of SAH in a non-specialized setting and early transfer are difficult and may have a critical impact on outcome. Clinical advice, based on multidisciplinary consensus, might be helpful. Our recommendations cover most, but not all, topics of clinical relevance.

BackgroundInsular cavernous malformations (iCMs) are very rare vascular lesions. Their surgical management is challenging, due to their complex functional and vascular relationship. The continuous improvement of intra-operative tools and neuroimaging techniques has progressively enhanced the safety of iCM surgery. Nevertheless, the best surgical approach remains controversial.ObjectiveTo analyze the potential role of an anatomo-functional classification to guide the iCMs’ management.MethodsThe study included patients affected by iCMs and referred to the Senior Author (FA). All cases were divided in 2 groups, according to a mainly pial growth pattern (exophytic group) or a subcortical one (endophytic group). Endophytic iCM was further subdivided in 3 subgroups, based on the insular gyri involved. According to this classification, each patient underwent a specific additional neuroimaging investigation and surgical evaluation.ResultsA total of 24 patients were included. In the surgical group, trans-sylvian (TS) approach was used in 6 patients with exophytic or Zone I endophytic iCMs. The transcortical (TC) approach with awake monitoring was used in 6 cases of Zone II endophytic vascular lesions. Both TS and trans-intraparietal sulcal (TIS) approach were used for 3 cases of Zone III endophytic iCM. At follow-up, 3 patients were fully recovered from a transient speech impairment while a permanent morbidity was observed in one case.ConclusionsICMs represent a single entity with peculiar clinical and surgical aspects. The proposed iCM classification focuses on anatomical and functional concerns, aiming to suggest the best pre-operative work-up and the surgical evaluation.

Background The early management of polytrauma patients with traumatic spinal cord injury (tSCI) is a major challenge. Sparse data is available to provide optimal care in this scenario and worldwide variability in clinical practice has been documented in recent studies. Methods A multidisciplinary consensus panel of physicians selected for their established clinical and scientific expertise in the acute management of tSCI polytrauma patients with different specializations was established. The World Society of Emergency Surgery (WSES) and the European Association of Neurosurgical Societies (EANS) endorsed the consensus, and a modified Delphi approach was adopted. Results A total of 17 statements were proposed and discussed. A consensus was reached generating 17 recommendations (16 strong and 1 weak). Conclusions This consensus provides practical recommendations to support a clinician’s decision making in the management of tSCI polytrauma patients.