[Chordoid gliomas of the third ventricle]. / Opyt lecheniya khordoidnykh gliom III zheludochka.

BACKGROUND: Chordoid glioma is a rare slow-growing tumor of the central nervous system. Available world experience includes no more than 200 cases (lesion of the third ventricle in absolute majority of cases). Recognition and treatment of chordoid glioma are currently difficult problems due to small incidence of this disease. OBJECTIVE: To describe clinical manifestations and surgical treatment of chordoid glioma of the third ventricle considering literature data and own experience. MATERIAL AND METHODS: There were 12 patients (6 men and 6 women) with chordoid glioma between 2004 and 2023 (10 patients with lesion of the third ventricle, 1 - lateral ventricle, 1 - pineal region). Only patients with tumors of the third ventricle were analyzed. RESULTS: Total and subtotal resection was performed in 1 and 3 cases, respectively. Five patients underwent partial resection, 1 patient underwent biopsy. The follow-up data were available in 7 out of 10 patients (mean 25 months). Radiotherapy was performed in 4 patients (continued tumor growth in 2 cases). One patient died. CONCLUSION: Chordoid glioma is a benign tumor predominantly localized in the third ventricle. Preoperative MRI and CT in some cases make it possible to suspect chordoid glioma and differentiate this tumor from craniopharyngioma, meningioma and pituitary adenoma by such signs as isointense signal in T1WI, hyper- or isointense signal in T2WI, homogeneous contrast enhancement and edema of basal ganglia in T2 FLAIR images. The only effective treatment for chordoid glioma is surgery. Total resection is often impossible or extremely dangerous due to location of tumor, large size and invasion of the third ventricle. Postoperative mental disorders and diabetes insipidus, including severe hypernatremia, are common that requires mandatory monitoring of water and electrolyte balance.

[Keyhole surgery for giant basal brain tumors. Theoretical explanation with an example of trans-eyebrow subraorbital approach]. / Aktual'nost' keyhole-dostupov v khirurgii gigantskikh opukholei osnovaniya golovnogo mozga. Teoreticheskoe obosnovanie na primere chrezbrovnogo supraorbital'nogo dostupa.

BACKGROUND: Improvement of modern neurosurgical technology has given rise to keyhole surgery for a wide range of pathologies. Supraorbital trans-eyebrow approach has been described in detail for small tumors of anterior cranial fossa and suprasellar neoplasms. Can we use this small and less traumatic approach for a more complex tumor extending beyond the suprasellar region? OBJECTIVE: To analyze own experience of surgical treatment of large and giant parasellar tumors via supraorbital approach, to identify the principles of such operations, indications and possible limitations. MATERIAL AND METHODS: There were 58 resections of parasellar tumors via mini-supraorbital approach had been performed between 2016 and 2021 at the 2nd pediatric neurosurgical department. We selected 42 patients with large and giant tumors extending beyond the chiasmal-sellar zone and causing occlusive hydrocephalus (mean volume 44.2 cm3). Preoperative status of patients, postoperative clinical data and logistics of in-hospital treatment were analyzed. RESULTS: Total resection was performed in all cases. No redo surgeries or revisions were required. There were no cases of wound infection, CSF leakage and damage to large vessels. CONCLUSION: We standardized planning of surgeries for large neoplasms considering available knowledge about the nature of diseases, analysis of clinical and X-ray data, goals and objectives of surgery. The reader is offered a theoretical substantiation of applicability of keyhole surgery for chiasmal-sellar tumors on the example of well-known mini-supraorbital approach. We gave certain advices on planning such operations and illustrated clinical examples.

[Tubular retractors for transcranial approaches to intraaxial brain tumors in children]. / Opyt primeneniya tubulyarnykh retraktorov v transkranial'noi khirurgii intraaksial'nykh opukholei golovnogo mozga u detei.

Reduction of surgical trauma associated with approach to deep brain structures including resection of tumors is an urgent direction in development of techniques and technology. OBJECTIVE: To analyze the efficacy and safety of tubular retractors in surgery of deep brain tumors in children. MATERIAL AND METHODS: The study included 17 children with deep brain tumors who underwent surgery between 2020 and 2021. Tubular retractors were used in all cases. The control group consisted of 15 children with a similar disease and standard intraoperative tissue traction technique. All patients underwent MRI of the brain on the first postoperative day. We analyzed severity of traction-induced damage to brain tissue in T2 FLAIR and DWI images. RESULTS: Clinical outcomes and structure of surgical complications were similar in both groups. Tubular retractor turned out to be a tool reducing traction injury. In the main group, postoperative MRI revealed significantly less damage to brain tissue along surgical approach in T2 (edema zone) and DWI (ischemic changes) images.

[Lessons learnt from 101 hemispheric pediatric epilepsy surgeries part ii: pitfalls and complications]. / Uroki 101 gemisferotomii u detei s polusharnoi simptomaticheskoi epilepsiei. Chast' II: oslozhneniya i oshibki.

OBJECTIVE: To evaluate the spectrum of pitfalls and complications after hemisherotomy basing on a retrospective study of a large consecutive pediatric cohort of patients from a single institution. MATERIAL AND METHODS: One hundred and one patients (med. age - 43 months) with refractory seizures underwent hemispherotomy. Developmental pathology was the anatomical substrate of disorder in 42 patients. The infantile post-stroke scarring and gliosis was the origin of epilepsy in the majority of 43 cases with acquired etiology. The progressive pathology (RE, S-W and TS) was the etiology in the rest of children (16 cases). The lateral periinsular technique was used to isolate the sick hemisphere in 55 patients; the vertical parasagittal approach was employed in 46 cases. Median perioperative blood loss constituted 10.5 ml/kg, but was markedly larger in kids with hemimegaly (52.8 ml/kg); 57 patients needed hemotransfusion during surgery. Median length of stay in ICU was 14.7 hours, and the length of stay in the hospital until discharge - 6.5 days. Eight patients underwent second-look surgery to complete sectioning of undercut commissural fibers. FU is known in 91 patients (med. length - 1.5 years). RESULTS: Major surgical complications with serious hemorrhage and/or surgery induced life-threatening events developed in 7 patients (one of them has died on the 5th day post-surgery for the causes of brain edema and uncontrolled hyponatremia). Various early and late infectious complications were noted in 4 cases. Ten patients experienced new not anticipated but temporary neurological deficit. Nine patients needed shunting for the causes of hydrocephalus within several first months post-hemispherotomy. Early seizure onset was associated with probability of all complications in general (p=0.02), and developmental etiology - with intraoperative bleeding and hemorrhagic complications (p=0.03). CONCLUSION: Children with developmental etiology, particularly those with hemimegalencephaly, are most challengeable in terms of perioperative hemorrhage and serious complications. Patients with relapse or persisting seizures should be evaluated for the possibility of incomplete hemispheric isolation and have good chances to become SF by re-doing hemispherotomy.

[Influence of resection quality on postoperative outcomes in children with atypical teratoid-rhabdoid tumor of the central nervous system]. / Vliyanie radikal'nosti operativnogo vmeshatel'stva na rezul'taty lecheniya atipichnoi teratoidno-rabdoidnoi opukholi tsentral'noi nervnoi sistemy u detei.

The purpose of this study was to assess the influence of resection quality on overall survival and disease-free survival in children with atypical teratoid-rhabdoid tumors (ATRT). The study included children younger than 18 years old for the period from 2008 to 2019. There were 134 interventions in 105 patients with ATRT including 11 redo resections («second-look¼ surgery) and 18 procedures for tumor recurrence. Age of patients ranged from 2 to 168 months (median 21 months). Patients with supratentorial tumors prevailed (50.5%), infratentorial neoplasms were diagnosed in 45.7% of patients, spinal cord lesion - 3.8% of cases. At the first stage, all patients underwent surgical treatment. Total resection was achieved in 34 (32.4%) patients, subtotal - 37 (35.2%) patients, partial resection - 30 (28.6%) patients. Biopsy was performed in 4 (3.8%) patients. Quality of resection and age at surgery significantly influenced overall and disease-free survival. Extended resection of tumor followed by adjuvant chemo- and radiotherapy are required to improve survival although ATRTs are high-grade neoplasms with poor prognosis.

[Hemangioblastomas of the filum terminale and cauda equina]. / Gemangioblastomy konechnoi niti i koreshkov konskogo khvosta.

Hemangioblastoma of the filum terminale and cauda equina is a rare tumor of nervous system. There are only few case reports in the literature. In this manuscript, the authors analyze the diagnosis and treatment of patients with this disease. MRI data and intraoperative images of stage-by-stage resection allow one to get an idea about radiological characteristics of hemangioblastoma of the filum terminale and surgical intervention. The authors discuss the world experience of surgical treatment and consider intraoperative complications.

[Perimedullary arteriovenous fistula. Case report and literature review]. / Perimedullyarnaya arteriovenoznaya fistula. Klinicheskoe nablyudenie i obzor literatury.

Arteriovenous fistula is a common vascular abnormality of spinal cord and meninges. This disease is more common in young men. Clinical manifestation includes progressive sensitive and motor disorders. However, acute symptoms including impaired consciousness, head or back pain are also possible. The authors describe a rare case. A 15-year-old boy experienced acute depression of consciousness accompanied by headache, vomiting, weakness in the upper limbs and sensitive disorders. The patient was hospitalized to the intensive care unit and examined for subarachnoid hemorrhage. MRI of the head and cervical spine and direct invasive angiography were performed. Perimedullary AVF of cervical spinal cord was diagnosed. Complete clinical regression was observed within a month. Microsurgical removal of AVF was performed in scheduled fashion. Postoperative follow-up period was over 6 months.

[Surgical treatment of epilepsy in children with gloneuronal brain tumors: morphology, MRI semiology and factors affecting the outcome]. / Khirurgicheskoe lechenie épilepsii u detei s glioneironal'nymi opukholiami mozga: morfologiia, magnitno-rezonansno-tomograficheskaia semiologiia i faktory, vliiaiushchie na iskhod.

INTRODUCTION: Glioneuronal tumors (GNT) are usually found in children (less than 1.5% of all neoplasms of the brain). With rare exceptions, they are benign and usually manifest only by epilepsy, which is quite often resistant to treatment with AE drugs. Tumor removal usually helps to cope with epileptic seizures, however, a number of issues regarding diagnosis and surgical treatment (interpretation of morphological data and classification, epileptogenesis and topography of the epileptogenic zone, the value of intraoperative invasive EEG and the optimal volume of resection) remain debatable. AIM: To describe the morphology, electro-clinical picture and MR-semiology in patients with gloneuronal brain tumors, as well as to analyse the results of their surgical treatment and the factors determining its outcome. MATERIAL AND METHODS: 152 children with a median age of 8 years were treated surgically (There were 64 gangliogliomas, 73 DNT, 15 cases where the tumor classification failed - GNT NOS). In children under 2 years of age, temporal localization of the tumor prevailed. In 81 cases, ECoG was used during the operation. Surgical treatment complications: transient neurological deficit (in 15 cases); hematomas removed without consequences (in 2 cases), infectious (osteomyelitis of bone bone flap in 2 cases). We analyzed: the age of the epilepsy onset (median - 4 years 7 months) and its duration (median - 23.5 months), the type of seizures, as well as the features of MR-semiology and morphology of tumors and adjacent areas of the brain. The volume of tumor resection was verified by MRI (in 101 cases) and CT (in each case). The follow-up was collected through face-to-face meetings, with repeated video EEG and MRI, as well as telephone interviews. We studied the effect of a number of parameters characterizing the patient and features of his/her operation on the outcome of treatmen. RESULTS: Among 102 patients in whom the follow-up history is one year or more (median - 2 years), a favorable outcome (Engel IA) was observed in 86 of them (84%); 55 of them (54%) at the time of the last examination stopped drug AE treatment. Radical tumor removal and younger age at the time of surgery were statistically significantly associated with a favorable result. CONCLUSION: In children with gloneuronal brain tumors, removal of the tumor is effective and relatively safe in the treatment of symptomatic epilepsy. Radical tumor resection and earlier intervention are the most important prerequisites for a favorable outcome and persistent remission of seizures.

[Liquorrhea after removal of fourth ventricle tumors in children. Retrospective analysis of a series of 211 primary surgeries]. / Likvoreia posle udaleniia opukholei IV zheludochka u detei. Retrospektivnyi analiz posledovatel'noi serii 211 pervichnykh operatsii.

Wound liquorrhea is a serious and dangerous complication developing after neurosurgical interventions. The highest risk group includes patients operated on using approaches to the skull base. In pediatric neurosurgery, approaches to the posterior cranial fossa are some of the most common ones, with wound liquorrhea amounting to 33%. Studies devoted to the wound liquorrhea problem have been performed in heterogeneous patient groups, which complicates evaluation of the results and reduces their practical value. MATERIAL AND METHODS: The analysis included pediatric patients operated on by a single surgeon for posterior cranial fossa (PCF) tumors in the period from 2009 to 2016. There were 472 patients. Criteria for inclusion in the study were as follows: age under 18 years; predominant tumor location in the fourth ventricle; primary surgery. According to the inclusion criteria, the study involved 211 patients. A search for the key words 'liquorrhea', 'pooling', 'meningocele', 'pseudomeningocele', and 'meningitis' was performed through electronic medical records of the involved patients. We analyzed the data on the total bed-day and the number of days spent in the critical care unit. Surgery protocols were the source of the following information: the presence of an external ventricular drain or ventriculoperitoneal shunt before tumor resection; information on DM closure; information on additional sealing of dura sutures. In addition, the patients were divided into two groups, the main difference between which was the use of additional sealing of dura sutures (Tachocomb, fibrin-thrombin glues, and their combination) to minimize the risk of wound liquorrhea. RESULTS: A total of 211 patients were included in the study. Postoperative wound liquorrhea occurred in 6 (2.8%) patients; another 5 (2.4%) patients had significant pseudomeningocele. Most children spent no more than 1 day in the critical care unit - totally 176 (83.4%) patients; the other 35 (16.6%) children spent more than 1 day in the critical care unit. A group of patients without sealing of dura sutures consisted of 144 (68.2%) patients. Liquorrhea occurred in 4 (2.8%) cases; in 3 (2.1%) cases, there was symptomatic pseudomeningocele without liquorrhea. In a group of patients with the use of suture sealing (Tachocomb, fibrin-thrombin glue in various combinations - 67 (31.8%) patients), there was liquorrhea in 2 (3%) cases and pseudomeningocele without liquorrhea in 2 (3%) cases. On the basis of PSM algorithm application, 2 groups of 67 patients each were formed: the group with sealing (A) and the group without sealing (B) of the DM. Of the 67 cases of sealing, Tachocomb was used in 64 (95.5%) patients, and fibrin-thrombin glue was applied in 53 (59.1%) patients. Differences between the groups in the rate of liquorrhea (2 (3.0%) patients in group A and 4 (6.0%) patients in group B) and pseudomenigocele (3 (4.5%) patients in group A and 5 (7.5%) patients in group B) were statistically insignificant (p=0.68 and p=0.58, respectively). CONCLUSION: Wound liquorrhea has remained a topical issue in surgery for posterior cranial fossa tumors and, if develops, doubles the postoperative bed-day. Our strategy of perioperative management of patients proved its efficacy in reducing the rate of postoperative liquorrhea up to 2.8%. A low percentage of liquorrhea did not allow unambiguous evaluation of the effect of additional sealing of dura sutures (Tachocomb and fibrin-thrombin glue).

[Radicalness and complications of repeated surgery for malignant neuroepithelial tumors of the posterior cranial fossa in children]. / Radikal'nost' i oslozhneniia povtornykh khirurgicheskikh vmeshatel'stv pri zlokachestvennykh neiroépitelial'nykh opukholiakh zadnei cherepnoi iamki u detei.

Treatment of primary malignant neuroepithelial tumors of the posterior cranial fossa (PCF) in childhood includes surgical resection, radiation therapy (RT), and chemotherapy (CT). The radicalness of surgery is one of the most important prognostic factors of survival. Despite the significant advances in treatment, many of these tumors recur. Today, oncologists are increasingly recommending repeated surgery for recurrent malignant neuroepithelial tumors of the PCF to achieve gross total resection (GTR). Patients undergo this surgery after RT and palliative CT, which may increase surgical risks. OBJECTIVE: The study objective was to assess the resection extent of recurrent malignant neuroepithelial tumors of the PCF in children as well as the risk and structure of postoperative complications. MATERIAL AND METHODS: The prospective study included 50 patients under the age of 18 who underwent surgery for recurrent malignant neuroepithelial tumors of the PCF at the Neurosurgical Institute (NSI) in the period between 2002 and June 2015. Anaplastic ependymomas were present in 37 patients, and medulloblastomas were detected in 13 patients. A total of 58 repeated surgeries were performed. RESULTS: GTR was achieved in 53 (91.4%) cases, near total resection (NTR) was achieved in 2 (3.4%) cases, and subtotal resection (STR) was achieved in 3 (5.2%) cases. The mean bed-day after surgery was 12 (4-47) days, and the mean critical care stay was 3.2 (0-23) days. Seven patients required tracheostomy; meningitis developed in 3 patients; liquorrhea occurred in 2 cases. Ventriculoperitoneal shunting was used in 8 (13.8%) cases. One (1.7%) patient died in the early postoperative period. CONCLUSION: Our results demonstrate that resection of recurrent malignant neuroepithelial tumors in children can be performed with high radicalness (90%) and acceptable risks.

[Implantation of VPS in the early postoperative period following removal of posterior cranial fossa tumors in children]. / Neobkhodimost' implantatsii VPSh v rannem posleoperatsionnom periode posle udaleniia opukholei zadnei cherepnoi iamki u detei.

One of the main manifestations of posterior cranial fossa (PCF) tumors is the development of hydrocephalus (HC) symptoms that in most cases are the reason for examination and diagnosis [1, 2]. According to the literature, the rate of shunt surgery after removal of PCF tumors in the children's population is 18-40% [3-9]. Hydrocephalus remaining after removal of PCF tumor and requiring further treatment is called persistent hydrocephalus. The above figures are based on the treatment outcomes in children, which were obtained as early as in the 1990s. Given these facts, we conducted a modern study to assess the risk of persistence/development of persistent hydrocephalus in children after removal of posterior cranial fossa tumors and to explore factors affecting this risk. MATERIAL AND METHODS: We performed a retrospective analysis of the treatment outcomes in 155 children operated on at the Burdenko Neurosurgical Institute between 2012 and 2014. All children were operated on by a single surgeon. Factors, such as the patient age, histological pattern of tumor, and primary/repeated surgery, were analyzed. RESULTS: A total of 13 (8.4%) patients needed shunt surgery. An analysis demonstrated no need for shunt surgery in children with piloid astrocytomas. In children with medulloblastoma, the need for a permanent shunt occurred in 5 (9.1%) cases; all these children were older than 3 years. A group of anaplastic ependymoma included 23 children; of these, 13 children were older than 3 years, and 10 children were under the age of 3 years. Five (21.7%) patients required shunt surgery. Shunting was needed in 3 children from group 4. In one case, a child younger than 3 years of age who was operated on for a dermoid cyst of the PCF, and in 2 children older than 3 years of age who were operated on for ganglioastroscytoma and atypical teratoid-rhabdoid tumor (ATRT). CONCLUSION: At present, the risk of persistent hydrocephalus in children after removal of PCF tumors is lower than that reported in the literature. In our study, it was 8.4%. The age of children under 3 years and repeated operations do not statistically significantly increase the risk of persistent hydrocephalus. Only tumor histology is statistically significant: the highest risk of the need for implantation of VPS was found in the group with anaplastic ependymomas, and the minimal risk was in the group of piloid astrocytomas.

[Surgery of posterior cranial fossa tumors in children in the prone position. The surgical technique features]. / Khirurgiia opukholei zadnei cherepnoi iamki u detei v polozhenii 'lezha na zhivote'. Osobennosti khirurgicheskoi tekhniki.

Posterior cranial fossa tumors are the most common neuro-oncological pathology of childhood. More than half of them are located along the midline, occupying the cerebellar vermis and 4th ventricle cavity. Historically, most of these tumors were operated on with the patient in sitting position. This tendency has significantly changed in the last 30 years. For example, 95% of all operations in Japan are now performed with the patient in lying position; for the US and Europe, these figures are 80 and 60%, respectively. This global tendency of switching to the lying position is mainly associated with a high risk of venous air embolism in the sitting position. In the period between 1999 and 2013, the first author used only the sitting position for resection of PCF tumors. During this period, he performed 606 operations. In patients with large/giant tumors (usually, these were piloid astrocytomas with cysts), the surgeon often faced the problem of excessive retraction of the cerebellum and rupture of the bridging veins, sometimes outside the surgical approach area. This situation led either to massive blood loss or to venous air embolism. MATERIAL AND METHODS: Therefore, beginning at 2013, we started to selectively use the prone position in cases of hemispheric piloid astrocytomas of the cerebellum. This initial experience allowed us to assess the surgical features of the procedure and use the experience in more complex interventions. Since the middle of 2016, given the tendency of using key-hole approaches, we have increasingly used the prone position in surgery of PCF tumors, sometimes removing tumors even through the burr hole. Since the end of 2016, we have routinely used the prone position for various tumors of the 4th ventricle. Between November 2016 and September 2017, the first author performed 113 surgeries for PCF tumors in children; of these, only 4 operations were performed in the sitting position. Thus, in less than a year, the prone position has become the main one in surgery for all PCF tumors in our practice. In this article, we would like to share our practical suggestions both about using the prone position and about its advantages and disadvantages that should be considered by a doctor who does not have experience of PCF surgery with the patient in prone position.

[Spinal cord epidermoid cysts]. / Épidermoidy spinnogo mozga.

The article describes in detail a large modern clinical series of patients operated on for spinal cord epidermoid cysts. AIM: the study aim was to comprehensively investigate the epidemiological and clinical peculiarities of spinal cord epidermoid cysts and evaluate immediate and long-term results of their surgical treatment. MATERIAL AND METHODS: We describe and analyze surgical outcomes of 20 patients with spinal cord epidermoid cysts. This sample was selected from 554 patients with intramedullary tumors and 365 patients with spinal dysraphism (aged from 2 months to 72 years) who were operated on by the first author in 2002-2017. RESULTS: Our findings confirm a high efficacy and low risk of surgical treatment of patients diagnosed with spinal epidermoid cysts. Severe complications of disease progression serve as indications for 'preventive' surgery even in asymptomatic cases. Complete resection of the tumor capsule without deterioration of the functional surgical result is the main goal of surgical treatment. Incomplete resection is associated with a high risk of recurrence. The most thorough follow-up with annual MRI examination is necessary for the first 6 years after surgery.

[Expansive suboccipital cranioplasty in Chiari 1 malformation (a case report and technical notes)]. / Ékspansivnaia kranioplastika zadnei cherepnoi iamki pri anomalii Kiari 1 (klinicheskoe nabliudenie i opisanie khirurgicheskoi tekhniki).

In this case report, we describe the use of expansive suboccipital cranioplasty in Chiari-1 malformation. The technique improves the efficacy and safety of treatment for Chiari-1 malformation. The technique can be used as an adjunct treatment together with any variant of posterior fossa decompression, including duroplasty and extradural decompression.

[Intramedullary spinal cord tumors and hydrocephalus: an analysis of the results of surgical treatment in 541 patients]. / Intramedulliarnye opukholi spinnogo mozga i gidrotsefaliia: analiz rezul'tatov khirurgicheskogo lecheniia 541 bol'nogo.

The article addresses the problem of intramedullary tumors (IMTs) combined with hydrocephalus (HC). PURPOSE: The study purpose was to explore, based on large clinical material, the occurrence of hydrocephalus combined with intramedullary tumors, possible pathogenetic mechanisms of its development, effect of tumor resection on the course of hydrocephalus, and need and timing of shunting surgery. MATERIAL AND METHODS: We present and analyze the data of the largest individual series of patients of all age groups operated on for IMTs of the spinal cord: 541 patients; 586 operations; age from 2 months to 72 years. RESULTS AND CONCLUSION: Our findings confirm a potential pathogenetic relationship between IMT and HC. The overall occurrence rate of HC in IMT patients was 6.3%. In patients with benign tumors (WHO Grade 1-2; 449 patients), HC developed in 25 (5.6%) cases; in patients with malignant tumors (WHO Grade 3-4; 84 patients), HC developed in 7 (8.3%) cases. A statistically significant prevalence of cervico-medullary tumors was found in HC patients: 19 (59.4%) cases. According to our data, dissemination of the tumor process is a potential factor of HC development.

[Posterior decompression of the craniovertebral junction in syringomyelia combined with Chiari-1 malformation in children]. / Rezul'taty zadnei dekompressii kraniovertebral'nogo perekhoda pri siringomielii s anomaliei Kiari-1 u detei.

OBJECTIVE: to develop the algorithm for defining the amount of posterior decompression of the craniovertebral junction in children with syringomyelia combined with Chiari-1 malformation. MATERIAL AND METHODS: Sixty eight children with syringomyelia and Chiari-1 malformation, under age of 18 years, underwent posterior decompression of the craniovertebral junction (PDCVJ) in the period from January 2001 to June 2016. Seven (10%) patients underwent extradural decompression (EDD), 16 (24%) patients underwent extra-arachnoid duraplasty (EAD), 25 (37%) patients underwent intra-arachnoid dissection (IAD) and duraplasty, and 20 (29%) patients underwent PDCVJ and placement of a fourth ventricle-subarachnoid shunt. RESULTS: Clinical improvement occurred in 85% of patients, and stabilization was observed in 11% of patients. Syringomyelia regressed in 78% of cases. There were no complications associated with EDD; however, re-operation was required in 3 (43%) cases. In the case of EAD, treatment results were satisfactory in 11 (79%) patients; re-operation was required in 2 (12.5%) cases; there were no complications in the early postoperative period. The highest complication rate of 6 (30%) cases was associated with shunt placement and duraplasty. However, long-term results in this group of patients were satisfactory in 16 (94%) cases, and MRI-based positive changes were observed in 100% of cases. CONCLUSION: According to our analysis, EAD is the method of choice for PDCVJ in children with syringomyelia and Chiari-1 malformation without myelopathy symptoms. In the presence of myelopathy symptoms, intra-arachnoid dissection (with or without shunting) is an acceptable alternative. To our opinion, the use of EDD in syringomyelia is unadvisable.

[Intramedullary spinal cord tumors and neurofibromatosis]. / Intramedullyarnye opukholi spinnogo mozga i neirofibromatoz.

The article addresses the issue of a combination and relationship between intramedullary spinal cord tumors (IMSCTs) and neurofibromatosis (NF). AIM: To study, based on large clinical material, features of the prevalence and biological nature of intramedullary spinal cord tumors in neurofibromatosis patients in different age groups. MATERIAL AND METHODS: We analyzed the data of the largest series of patients from all age groups who underwent surgery for spinal cord intramedullary tumors (541 patients; 586 surgeries; age, 2 months to 72 years). RESULTS: Our findings support a potential pathogenetic relationship between intramedullary tumors and neurofibromatosis. Astrocytoma was a predominant intramedullary tumor in patients with NF-1, while ependymoma predominated in patients with NF-2. IMSCTs combined with NF-1 occur predominantly in children and adolescents, while a combination with NF-2 is typical of young adults. Our findings confirm the fact that IMSCT surgery is required for a small number of NF patients.

[Posterior decompression of the craniovertebral junction in children with Chiari malformation: a surgery extent issue]. / Zadnyaya dekompressiya kraniovertebral'nogo perekhoda pri anomalii Kiari-1 u detei: vybor ob''ema operatsii.

AIM: The study objective was to develop a rational approach for defining the extent of posterior decompression in children with Chiari 1 malformation. MATERIAL AND METHODS: Posterior decompression was performed in 76 children with Chiari 1 malformation, under 18 years of age, in the period between 2001 and 2015. Fifty two (68%) children had syringomyelia. Extradural decompression (EDD) was performed in 14 (18%) cases, extra-arachnoid duraplasty (EAD) in 21 (28%) cases, intra-arachnoid dissection and duraplasty in 21 (28%) cases, and foramen of Magendie stenting and duraplasty in 20 (26%) cases. RESULTS: Complications occurred in 15 (20%) patients, with one of them being fatal (case fatality rate, 1.3%). The complication rate was higher after (1) intra-arachnoid dissection (p=0.0009) and stenting (p=0.02). Re-operation was required in 8 (11%) patients. The overall rate of complications and re-operations was lowest after EAD (10%). CONCLUSION: EAD is the method of choice for Chiari 1 malformation in children. EDD can be adopted as a primary option, but it requires selection of relevant patients. Intra-arachnoid dissection, with/without stenting, is not advisable as a primary intervention, but may be inevitable in the re-operation case.

[Intramedullary melanocytoma: a clinical case report and literature review]. / Intramedullyarnaya melanotsitoma: opisanie klinicheskogo nablyudeniya i obzor literatury.

The paper describes a rare clinical case of intramedullary melanocytoma, provides a detailed description of a pathomorphological study, and addresses the issues of differential diagnosis and surgical treatment.

[Hemispherectomy in the treatment of pediatric symptomatic epilepsy of children]. / Gemisferektomiya v lechenii simptomaticheskoi epilepsii u detei.

INTRODUCTION AND PURPOSE: Hemispherectomy is a recognized option in the treatment of symptomatic forms of intractable focal epilepsy in patients with developmental brain malformations and some acquired lesions of one the hemispheres. The prognosis for an outcome of the technique is important in terms of the indications for surgical treatment. MATERIAL AND METHODS: We described the hemispherectomy technique and its variants and analyzed our own experience of surgery in 40 children. The most common (27 cases) brain pathology was extended unilateral cortical dysplasia with polymicro- or pachygyria and consequences of perinatal stroke. Six children had Rasmussen encephalitis; 6 patients had hemimegalencephaly; 1 child with Sturge-Weber syndrome had angiomatosis of the soft meninges. The patients' mean age was 3 years. Functional hemispherectomy (hemispherotomy) was used in most cases (37); 3 patients underwent anatomical hemispherectomy. RESULTS: At the time of discharge, seizures resolved in all patients; later, no seizure recurrence was observed in 25 out of 29 cases with known follow-up (the follow-up median was 2.5 years), which corresponded to class 1 outcomes on the ILAE scale (86%). Serious complications developed in 2 cases; 1 patient died; hydrocephalus and the need for bypass surgery occurred in other 2 children. These results are discussed along with the literature data, and the indications for hemispherectomy are provided. CONCLUSION: Hemispherectomy is a reliable and effective technique for treatment of symptomatic hemispheric forms of epilepsy in children. More than in 80% of patients with congenital or acquired pathology of one of the cerebral hemispheres, its deafferentation or resection leads to persistent elimination of seizures. Children with severe forms of intractable epilepsy should be promptly referred to dedicated centers to address the issue of advisability of surgical treatment.