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Treatment of the disorders affecting skull base and craniovertebral junction (С1-С2, C1-C3) associated with craniovertebral instability is an unresolved issue of modern neurosurgery. Patients are often denied surgical care due to the disorder complexity, and treatment is limited to conservative methods (cervical collar, halo apparatus) that are palliative in nature. Patients with craniovertebral pathology have been considered to be radically inoperable until recently. Advanced methods for treatment of patients with various disorders affecting skull base and craniovertebral junction are used and developed in the Burdenko Neurosurgical Center.

Introduction The tumors of the diencephalon region (thalamic-hypothalamic-pituitary system) include a large group: pituitary adenomas, craniopharyngiomas, gliomas, and others. Tumors differ in the histological structure, and manifestations of the clinical symptoms; by hormonal data; by approaches and methods in treatment. Psychic symptoms are revealed in disease in addition to cerebral, neuroendocrine symptoms, neurological disorders. Psychoorganic syndrome is represented by emotional, motivational, personal, cognitive impairments, inversion of the sleep-wake cycle, seizures. Disorders of mental activity are detected in all tumors of this localization in varying degrees, according to the different authors from 20 to 100%; affective pathology varies from 2 to 80% by the literature. Objectives To study the emotional disorders in the structure of psychoorganic pathology in tumors of diencephalon region Methods 290 patients (18-78 years old, mean age 38+2): pituitary adenomas (PA), as the most common – 170 (58,6%), craniopharyngiomas (CG), as with the most varied manifestation of mental symptoms – 120 (41,4%). Methods: psychopathological, data from endocrinological, neurological, neuroimaging methods. Results Emotional disorders were detected in patients from 30 to 68% of cases, depending on the histology of the tumours: PA with excessive secretion of growth hormone - emotional disorders are in 60%; PA with excessive secretion of adrenocorticotropic hormone - in 50%; PA with excessive secretion of prolactin - in 30%; with excessive secretion of thyroid-stimulating hormone - in 40%; non-functioning PA - in 16%; CG - in 68%. Emotional disorders were more often represented by changeable mood, depression, apathy, sleep disturbance, and visceral symptoms. Symptoms differed depending on the histology of the tumor (type and level of hormones), the volume of the lesion and direction of growth, and concomitant hypertensive-hydrocephalic symptoms. Emotional disturbances often include memory impairment, personality and behavior changes. Conclusions Emotional disorders are detected in patients in 30-68% of cases in the structure of psychoorganic pathology with damage to the diencephalon region (in particular, with pituitary adenomas and craniopharyngiomas); are determined by the topography of the tumor and histology with the involvement of the corresponding structures and nuclei in the pathological process. Disclosure of Interest None Declared

Introduction. According to the recommendations of Russian and international professional associations, treatment of germinomas can be initiated without histological verification of the diagnosis, since it can be based on biochemical tumor markers. However, patients with brain germinomas usually have normal levels of these markers; therefore, histological verification is required. Stereotactic biopsy and transcranial biopsy are sometimes associated with a risk of damage to crucial anatomical structures. Currently, both biopsy and total removal of sellar and parasellar tumors can be performed via endoscopic endonasal approach. The study objective is to demonstrate the possibility of using endoscopic transsphenoidal approach for biopsy and total removal of chiasmosellar germinomas. Materials and methods. Thirteen patients with primary chiasmosellar germinomas underwent endoscopic endonasal interventions in N. N. Burdenko National Medical Research Center for Neurosurgery between 2010 and 2017. The “Germinoma-2008” protocol was used in the subsequent treatment of these patients. The male to female ratio was 2.25 : 1; mean age was 21.1 years (6–38 years).Results. The surgery volume varied between biopsy (n = 4) and partial (n = 5) or complete (n = 4) tumor removal. The diagnosis was histologically verified in all patients. None of the patients developed liquorrhea and / or meningitis in the postoperative period, which suggests that the surgery was effective and safe. Conclusion. The endoscopic endonasal approach for histological verification of the diagnosis and removal of chiasmosellar germinomas is safe and effective.

Objective. To assess capabilities and advantages of endoscopic transnasal removal of pathological foci in the region of the C2 odontoid process. Material and Methods. The study included 3 patients who underwent endoscopic transnasal removal of the invaginated odontoid process accompanied by simultaneously (2 cases) or previously performed (1 case) occipitospondylodesis. Results. The pathological focus was totally removed in all cases. Postoperative complication occurred in one case – wound liquorrhea with subsequent development of meningitis which required performing plastic surgery of the CSF fistula. At follow-up examination, all patients presented with complete regression of symptoms. Conclusion. Endoscopic transnasal access allows for radical removal of pathological foci of the craniovertebral junction. Endoscopic trans- nasal approach will not be able to completely replace a transoral one, but it is a reasonable alternative in experienced hands.

A clinical case of a single-step transoral removal of C2 and C3 vertebral bodies with subsequent stabilization of the C1–C4 spinal segment through the same approach (with additional submandibular approach to insert screws into the C4 vertebra body) without dissection of the mandible and tongue is presented. The first experience of anterior stabilization of the C1–C4 spinal segment using a custom-made instrumentation system demonstrated its effectiveness. It was shown that, taking into account the length of the cervical spine resection, the anterior stabilization with a custom-made plate should be combined with standard methods of posterior fixation. Innovative surgical technologies allow optimizing the surgical technique of cervical spine stabilization and provide earlier rehabilitation of a patient.

The paper presents a review of three clinical cases of treating patients with chronic fractures of the odontoid process of C2 vertebra using various combinations of surgical techniques. In all cases, complete decompression of the spinal cord and stabilization of the upper cervical segment of the spine were achieved. A decrease in the range of motion in the cervical spine was observed, which did not affect patient satisfaction. A gradual regression of the neurological status and increase in muscle strength were recorded. Comparative analysis of the neurological status was carried out before and after surgical treatment. When assessing the general condition of patients using the index of disability due to pain in the neck (NDI), an improvement in the quality of life was noted. These data show the opportunities and peculiarities of methods for treating patients with chronic C2 odontoid fractures. Type of publication: description of clinical cases. Evidence level: IV.

Abstract Background Preservation of anatomic integrity and function of the cranial nerves during the removal of skull base tumors is one of the most challenging procedures in endoscopic endonasal surgery. It is possible to use intraoperative mapping and identification of the cranial nerves in order to facilitate their preservation. The purpose of this study was to evaluate the effectiveness of intraoperative trigger electromyography in prevention of iatrogenic damage to the cranial nerves. Methods Twenty three patients with various skull base tumors (chordomas, neuromas, pituitary adenomas, meningiomas, cholesteatomas) underwent mapping and identification of cranial nerves during tumor removal using the endoscopic endonasal approach in Department of Neurooncology of Federal State Autonomous Institution “N.N. Burdenko National Medical Research Center of Neurosurgery” of the Ministry of Health of the Russian Federation from 2013 to 2018. During the surgical interventions, mapping and identification of the cranial nerves were carried out using electromyography in triggered mode. The effectiveness of the method was evaluated based on a comparison with a control group (41 patients). Results In the main group of patients, 44 nerves were examined during surgery using triggered electromyography. During the study, the III, V, VI, VII, and XII cranial nerves were identified intraoperatively. Postoperative cranial nerve deficiency was observed in 5 patients in the study group and in 13 patients in the control group. The average length of hospitalization was 9 days. Conclusion We did not receive statistically significant data supporting the fact that intraoperative identification of cranial nerves using trigger electromyography reduces the incidence of postoperative complications in the form of cranial nerve deficits (p = 0.56), but the odds ratio (0.6) suggests a less frequent occurrence of complications in the study group. Based on our experience, the trigger electromyography methodology appears quite promising and requires further research.

Abstract Background Until recently, tumors of the clivus and the anterior region of the posterior cranial fossa were considered extremely difficult to access and often inoperable using standard transcranial approaches. With the introduction into the neurosurgical practice of minimally invasive methods utilizing endoscopic techniques, it became possible to effectively remove hard-to-reach tumors, including central tumors of the anterior region of the posterior cranial fossa. Methods From 2008 to the present time, the inpatient institution has operated on 140 patients with various tumors of the base of the skull, localized to the clivus and anterior region of the posterior cranial fossa (65 men and 75 women). The age of patients ranged from 3 to 74 years. Tumor distribution according to the histopathological features was as follows: chordomas, 103 (73.57%); meningiomas, 12 (8.57%); pituitary adenomas, 9 (6.43%); fibrous dysplasia, 4 (2.86%); cholesteatoma, 3 (2.14%); craniopharyngiomas, 2 (1.43%); plasmacytomas, 2 (1.43%); and other tumors (giant cell tumor, neurohypophyseal glioma, osteoma, carcinoid, chondroma), 5 (3.57%). The tumors had the following size distribution: giant (more than 60 mm), 35 (25%); large (35–59 mm), 83 (59.3%); medium (21–35 mm), 21 (15%); and small (less than 20 mm), 1 (0.7%). In 11 cases, intraoperative monitoring of the cranial nerves was performed (21 cranial nerves were identified). Results Upper, middle, and lower transclival approaches provide access to the anterior surface of the upper, middle, and lower neurovascular complexes of the posterior cranial fossa. The chordoma cases were distributed as follows according to extent of removal: total removal, 68 (66.02%); subtotal removal, 25 (24.27%); and partial removal, 10 (9.71%). The adenomas of the pituitary gland were removed totally in 6 cases, subtotally in 1 case and partially in 2 cases. The meningiomas were removed totally in 1 case, subtotally in 5 cases, and partially in 5 cases, with less than 50% of the tumor removed in 1 case. Other tumors (cholesteatoma, craniopharyngioma, fibrous dysplasia, giant cell tumor, glioma of the neurohypophysis, osteoma, plasmacytoma, carcinoid, and chondroma) were removed totally in 9 cases and subtotally in 7 cases. Postoperative CSF leaks occurred in 9 cases (6.43%) and meningitis in 13 cases (9.29%). Oculomotor disorders developed in 19 patients (13.57%), 12 of which regressed during the period from 4 to 38 days after surgery, and 7 of which were permanent. In 2 cases, surgical treatment had a lethal outcome (1.43%). Conclusion The endoscopic endonasal transclival approach can be used to obtain access to the centrally located tumors of the posterior cranial fossa. It is an alternative to transcranial approaches in the surgical treatment of tumors of the clivus. The results of using this approach are comparable with the results of transcranial and transfacial approaches and, in some cases, surpass them in effectiveness. The extended endoscopic endonasal posterior (transclival) approach, considering its minimally invasive nature, allows fora radical and low-risk (in terms of postoperative complications and lethality) removal of various skull base tumors of central localization with the involvement and without the involvement of the clivus, which, until recently, were considered to be almost inoperable.

Introduction In the literature, there are conflicting data regarding the recovery of mental disorders, in particular, pathologies of the emotional, personality, behavioral and cognitive spheres, in patients after surgical treatment of tumors of the diencephalic region. Objectives To evaluate the dynamics of psychopathological disorders after removal of a craniopharyngioma. Methods 45 patients (18–68 y.o.), operated through transcranial access. The follow-up period ranged from 3 months to 9 years (on average 2.8 + 0.4). The main method is psychopathological, supplemented by rating scales and questionnaires. Results In the late postoperative period, mental disorders were detected in 75% of patients (Table 1). Table 1. Dynamics of the main psychopathological symptom complexes (n = 45). Disorders (may be a combination) Before surgery (n,%) 2 weeks after (n,%) 18 months after (n,%) Emotional and volitional 27 (60%) 27 (60%) 15 (33%) Cognitive - Korsakov syndrome 18 (40%) 4 (9%) 27 (59%) 8 (18%) 18 (40%) 7 (15%) Personality 21 (46%) 25 (55%) 23 (51%) The table shows that emotional-volitional disorders have a clear positive dynamics by 18 months after surgery compared with the preoperative level. Korsakov’s syndrome and personality disorders are less favorable. 23 patients (52%) returned to their previous profession; 22 (48%) stopped working due to a severe degree of disability, of which 7 (15%) need constant supervision. Conclusions The positive dynamics of psychopathological symptoms is observed only within 1.5 years after the removal of the craniopharyngioma, in the future they remain without a tendency to improve. 22 patients (48%) stopped working. The most severe degree of disability is 15% patients. Disclosure No significant relationships.

Background: Cholesteatomas of the temporal bone pyramid can invade the clivus through the petrous apex. As these cases are extremely scarce in world literature, an optimal approach to their treatment has not been determined. Methods: We present two clinical cases: a patient with an apical cholesteatoma of the temporal bone pyramid extending to the clivus and a patient with a massive cholesteatoma of the temporal bone pyramid, also extending to the clivus according to the classification proposed by Sanna. Results: The apical cholesteatoma was removed totally, while the massive cholesteatoma was subjected to subtotal removal. In the second case, the duration of postoperative hospital stay was 7 days, while in the first case, hospitalization time was extended to 12 days as a result of postoperative cerebrospinal fluid leakage (due to intraoperative dura mater damage during the removal of the cyst capsule) and consequent revision duraplasty, further substantiating the need for the most delicate separation of the tumor capsule from the underlying dura mater. Conclusion: As a result of the development of modern endoscopic endonasal technologies in skull base surgery, effective removal of cholesteatomas of temporal bone pyramid with extension to the clivus has become possible using minimally invasive and organ-saving methods.

27 patients aged 2,5-61 years with skull base and C1-C2 vertebral body tumours and nontumorous pathology in the craniocervical junction underwent surgery. All patients revealed craniovertebral instability. To perform OSD we used autobone and metallic wire in 1 case, "Ventrofix" - 2; "CCD" - 9, "Vertex" - 15. In 26 cases OSD was followed by transoral tumor removal; in 1 - removal of the skull base chordoma spreading into C1-C2 segments was followed by OSD. In our practice we used original patent instruments, devices and surgical techniques. After the tumor has been removed, the skull defect hermetic closure and plasty were performed using the original patent technique for preventing postoperative CSF leakage as well as different glue compositions. This technique proved to shorten hospitalization period and reduce treatment costs as well as launch an early rehabilitation programme - on the 3d-4th day after operation. Use of new technologies in surgical treatment of skull base tumors invading upper cervical spinal segments accompanied by craniovertebral instability allowed to improve surgical outcome and start up early rehabilitation.

One of the most serious/potentially fatal complications of transsphenoidal surgery (TSS) is internal carotid artery (ICA) injury. Of 6230 patients who underwent TSS, ICA injury occurred in 8 (0.12%). The etiology, possible treatment options, and avoidance of ICA injury were analyzed. ICA injury occurred at two different stages: (1) during the exposure of the sella floor and dural incision over the sella and cavernous sinus and (2) during the resection of the cavernous sinus extension of the tumor. The angiographic collateral blood supply was categorized as good, sufficient, and nonsufficient to help with the decision making for repairing the injury. ICA occlusion with a balloon was performed at the injury site in two cases, microcoils in two patients, microcoils plus a single barrel extra-intracranial high-flow bypass in one case, stent grafting in one case, and no intervention in two cases. The risk of ICA injury diminishes with better preoperative preparation, intraoperative navigation, and ultrasound dopplerography. Reconstructive surgery for closing the defect and restoring the blood flow to the artery should be assessed depending on the site of the injury and the anatomical features of the ICA.