[Ischemic complications following surgical treatment of moyamoya disease: risk factors and prevention]. / Ishemicheskie oslozhneniya khirurgicheskogo lecheniya patsientov s bolezn'yu moiya-moiya: faktory riska i metody profilaktiki.

BACKGROUND: One of the most difficult problems in surgical treatment of moyamoya disease is prevention of ischemic perioperative complications. The risk of these events is significantly higher compared to other cerebrovascular diseases (up to 30%). OBJECTIVE: To identify unfavorable prognostic factors of perioperative cerebral ischemic complications, to determine the group of high-risk patients and to develop the guidelines for perioperative management of these patients. MATERIAL AND METHODS: We analyzed clinical and diagnostic data and postoperative outcomes in 80 patients with various forms of moyamoya disease. These patients underwent 134 different interventions. Staged revascularization of both hemispheres was performed in 40 patients (80 surgeries). Most patients (n = 55) underwent combined brain revascularization (79 surgeries). RESULTS: Persistent postoperative complications (ischemic stroke) developed in 7 cases (5.3%). Transient neurological impairment was observed in 36 cases (27%). Statistical analysis revealed the following risk factors of perioperative complications: critical stenosis/occlusion of posterior cerebral artery (OR 9.704), severe perfusion deficit (OR 5.393) and previous TIA or ischemic stroke within 3 months prior to surgery (OR 6.433). If at least two of these signs are present, sensitivity of prognosis for postoperative complications is 80.7% and 88.6%, respectively. CONCLUSION: Patients with moyamoya disease are at high risk of perioperative complications due to complex rearrangement of collateral cerebral circulation and high sensitivity of brain to local and systemic hemodynamic changes. Patients with risk factors require careful perioperative management to exclude pathogenetic factors provoking ischemia. Early surgical treatment is advisable to reduce the risk of ischemic and hemorrhagic lesions following natural course of disease and severe perioperative complications.

[Combined cerebral revascularization for moyamoya disease]. / Kombinirovannaya revaskulyarizatsiya golovnogo mozga v lechenii bolezni moiya-moiya.

INTRODUCTION: Combined cerebral revascularization with direct and indirect components is recognized as the most appropriate method for treating patients with Moyamoya disease all over the world, however, large studies on its effectiveness in Russia have not yet been conducted. THE AIM OF THE STUDY: Was to evaluate the results of combined cerebral revascularization in patients with Moyamoya disease with an analysis of the clinical state and perfusion and angiographic features of neoangiogenesis. MATERIAL AND METHODS: For the period from 2013 to 2020 in N.N. Burdenko National Medical Research Center of Neurosurgery 79 combined revascularizations were performed in 55 patients with Moyamoya disease. The average age was 13.9±10.11 years. All patients were examined according to a single protocol, including a comprehensive assessment of the clinical symptoms, the state of the brain tissue, the cerebral vascular system, and cerebral blood flow. The results of surgical treatment were evaluated in the early postoperative and long-term periods. The average follow-up period was 14.66±13.17 months. In the postoperative period, the dynamics of neurological status and cerebral blood flow were assessed and the features of neoangiogenesis from various components of revascularization were studied. RESULTS: In the early postoperative period, in 77.2% of cases, a good treatment result was observed, with no negative dynamics of the neurological status. Ischemic stroke in the operated hemisphere developed in 4 cases (5.1%). In the follow-up period, an improvement in neurological symptoms was observed in 76.6%. Improvement of cerebral blood flow was noted in 92.4% of cases, direct anastomoses functioned in 94.3%, and signs of neovascularization in the area of indirect synangiosis were observed in 80.0%. At the same time, a complementary influence of direct and indirect components of revascularization was revealed in ensuring good general angiographic and perfusion results. CONCLUSIONS: Combined revascularization is a highly effective method of surgical treatment of all patients with Moyamoya disease.

[Discussion of the manuscript Combined double-barrel direct and indirect bilateral cerebral revascularization in the treatment of moyamoya disease. Discussion and literature review]. / K obsuzhdeniyu stat'i «Sochetanie kombinirovannoi dvustvol'noi pryamoi i nepryamoi revaskulyarizatsii golovnogo mozga s dvukh storon v lechenii bolezni moyamoya¼. Diskussiya i obzor literatury.

First of all, I would like to thank my colleagues for their interest in this article [1] and note the relevance of the problem of surgical treatment of moyamoya disease, which provoked this discussion. Moyamoya disease is a rare, atypical pathology for our country, the diagnosis and treatment of which is controversial among specialists, and the lack of agreed protocols is the cause of differences in the interpretation of the results of surgical treatment of such patients.

[Modern trends in diagnosis and surgical treatment of moyamoya disease]. / Sovremennye tendentsii diagnostiki i khirurgicheskogo lecheniya bolezni moiyamoiya.

This review is devoted to moyamoya disease. It is a rare chronic steno-occlusive cerebrovascular disease. However, moyamoya disease is increasingly diagnosed by neurosurgeons in our country. Unlike atherosclerotic lesions of cerebral arteries, pathogenesis and course of this disease are much more complex and variable. Therefore, specialists often have certain difficulties in diagnosis, management and treatment of these patients. To date, a large number of surgical interventions have been proposed for the treatment of moyamoya disease. Revascularization approaches include direct procedures (extra-intracranial microanastomoses), indirect methods (synangioses) and combined revascularization. The purpose of the review is to systematize current literature data on the pathogenesis, diagnosis, clinical patterns and surgical treatment of patients with moyamoya disease. results Outcomes of surgical revascularization and the role of its various components in combined approach are under particular attention.

[Combination of double and indirect two-sided revascularization of the brain in the treatment of moyamoya disease]. / Sochetanie kombinirovannoi dvustvol'noi pryamoi i nepryamoi revaskulyarizatsii golovnogo mozga s dvukh storon v lechenii bolezni moyamoya.

An adult patient with progressive chronic cerebral ischemia associated with moyamoya disease who underwent combined revascularization of both cerebral hemispheres in step-by-step fashion is reported in the article. The feature of this case is a large volume of revascularization procedures with double-barrel extra-intracranial anastomoses combined with indirect synangioses. This surgical approach ensured early postoperative development of extensive collateral network and complete compensation of impaired cerebral circulation. Particular attention is paid to description and discussion of preoperative diagnosis, the choice of surgical treatment, as well as the features of surgical technique. The advantages and disadvantages of this approach are discussed in comparison with literature data.

[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.

[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.

[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.

[Surgical treatment of cerebral vascular diseases].

Theoretical and practical aspects of the complex treatment of brain and spinal vascular diseases using microsurgical, endovascular and radiosurgical methods are considered. Authors present the data demonstrating that, due to the implementation of the program of development of vascular centers in the Russian Federation, considerable progress was made in the treatment of cerebral aneurisms and hemorrhagic stroke. In author's opinion, wide introduction of surgical methods in the treatment of occlusive lesions of the blood vessels supplying the brain is needed.

[A new entity in WHO classification of tumors of the central nervous system--embryonic tumor with abundant neuropil and true rosettes: case report and review of literature].

Embryonic tumor with abundant neuropil and true rosettes (ETANTR) is a very aggressive rare tumor with unique histologic and molecular features occurring in very young children. At present approximately 80 cases of ETANTR have been documented in the literature since first description in 2000. We report seven patients with ETANTR below 4 years of age who underwent surgical resection in the Burdenko Neurosurgery Institute between 2005 and 2010. Four children have received different modality chemotherapy and radiotherapy and two patients were treated by chemotherapy alone. One child did not receive any adjuvant treatment. All children had local relapses, two of them were operated twice. A 2 year old girl underwent subtotal resection thrice. Histological examination showed that all tumors were composed of true multilayered rosettes admixed with large areas of paucicellular neuropil. By analysis of recurrences we have found that large areas of neuropil and number of true rosettes were lost and tumors acquired a resemblance to central nervous system primitive neuroectodermal tumors. In four cases frozen tumor material was available for array-based comparative genomic hybridization, which discovered trisomy of chromosome 2 and amplification at the 19q13.42 chromosome locus. Fluorescence in situ hybridization revealed amplification at the 19q13.42 chromosome locus in all cases.

[Physiology of CSF system and pathophysiology of hydrocephalus].

In the paper author briefly and distinctly presents basic physiological and pathophysiological concepts used in diagnosis and treatment of hydrocephalus, using modern literature data and his own experience.

[Successful treatment of moya-moya disease using encephalo-duro-arteriosynangiosis].

Aim of the study was to demonstrate the possibility of effective surgical revascularization of the brain in children with moya-moya disease. 3 year old girl with moya-moya disease underwent encephalo-duro-arteriosynangiosis. At 14 months follow-up signs and symptoms of cerebral ischemia totally resolved. Angiography confirmed brain supply from external carotid artery via the newly formed anastomotic network. Authors emphasize the necessity to seek moyamoya disease in children with symptoms of brain ischemia to allow timely and effective surgical treatment.

[Endoscopic ventriculostomy of the third ventricle in infants of less than 2 years of age].

The efficiency of endoscopic ventriculostomy (EVS) of the third ventricle is noticeably lower in small children than in adults and many surgeons prefer to shunt them despite the fact that revision has to be resorted to in half of the cases. Recent reports on the successful use of EVS in infants under 2 years of age have made the authors to revise traditional views. A sample of 20 babies (mean age 9.7 +/- 4.3 months) with obstructive triventricular hydrocephalus (HC) undergone EVS at the Academician N. N. Burdenko Research Institute of Neurosurgery, Russian Academy of Medical Sciences, in 1999 to 2006. The cause of HC was intracranial infection (n = 7), hemorrhage (n = 4), aqueduct stenosis (n = 4), Galen's vein aneurysms (GVA) (n = 3), and posterior third ventricular tumors (n = 2). Postoperative follow-ups averaged 15 months. There were no deaths; overall morbidity was 10%. By and large, the efficiency of EVS was 47%. At the same time, the best results were observed in babies with GVA (100%) and aqueduct stenosis (75%). The results were significantly worse in patients with the postinflammatory (33%) and posthemorrhagic (0%) etiology of HC. In addition to the etiology, the efficiency of EVS correlated with factors, such as prolapse of the third ventricular floor into the interpeduncular cistern, the thickened and rigid floor of the third ventricle, and an analysis excluding 3 babies with GVA established a direct correlation between the dysfunction of EVS and the early manifestation of HC. Postoperative clinical improvement was attended by decreases in the anterior-posterior horn ratio in all cases and in the sizes of the third ventricle.

[Cerebrospinal fluid dynamics in chronic obstructive hydrocephalus before and after successful endoscopic third ventriculostomy].

OBJECTIVE: to describe cerebrospinal fluid dynamics in chronic obstructive hydrocephalus before and after successful endoscopic third ventriculostomy (ETV). MATERIALS AND METHODS: 8 patients (7-21 years old) with chronic obstructive hydrocephalus due to tectal plate glioma were investigated before and after successful ETV. Apart from clinical and MRI assessment ICP-monitoring (ICP0) and intraventricular infusion studies were performed as well as upright ICP (ICP90) was investigated preoperatively and on 1st and 7th postoperative days (POD). Dynamic changes were assessed using Wilcoxon matched pairs test. RESULTS: There were no complications. MRI demonstrated functional ventriculostomy and reduced ventricle size in all cases. At follow-up all ETV's were considered clinically successful. By the 7th POD ICP0 showed tendency to reduction (p = 0.07) and ICP90 reduced significantly (p = 0.02). Significant reduction of Rcsf was evident by the 1st POD (p = 0.03) and was maintained until the 7th POD (p = 0.02). Elastance coefficient and compliance (as measured during infusion study) didn't change significantly in relation to ETV. In all the 3 cases when computerized ICP monitoring was used reduction of ICP pulse amplitude (AMP), AMP/ICP slope and RAP was noted by the 7-th POD which most probably reflects reduction of intracranial elastance. CONCLUSION: Effect of ETV in chronic obstructive hydrocephalus cannot be explained exclusively by its influence on ICP0; clinical improvement can possibly be attributed also to normalization of ICP90 and Rcsf, reduction of intracranial elastance and increase of cerebral blood flow reserve capabilities. There may be a pathophysiological ground for ETV in obstructive hydrocephalus with normal ICP.

[Complications and failures of endoscopic ventriculostomy of the third ventricle]. / Oslozhneniia i neudachi éndoskopicheskoi ventrikulostomii III zheludochka.

The study was undertaken to elucidate the poor outcomes of endoscopic ventriculostomy of the third ventricle (EVTV), including complications and dysfunctions. A series of 249 consecutive EVTV (the mean age of 15 years in 247 patients) made at the Institute of Neurosurgery in 1995 to 2003 was analyzed. The causes of hydrocephalus were benign tumors in 95 (38%) patients, stenosis of the aqueduct of the cerebrum in 73 (29%), malignant tumors in 63 (25%), and other causes in 18 (7%) cases. Obstruction in the posterior cranial fossa was present in 12 (5%); prior to EVTV, 24 (10%) and 36 (15%) patients had undergone craniotomy and bypass surgery, respectively; 12 (5%) patients had sustained subarachnoidal or intraventricular hemorrhage, 21 (8%) had intracranial infections. The follow-up averaged 16 months. Its results showed that 40 complications occurred in 34 (14%) patients. Seven (3%) patients required unplanned operations; transient and persistent neurological deficits were present in 15 (6%) and 2 (1%), respectively; there were no surgery-related deaths. Meningitis occurred in 14 (6%) cases; its risk was higher in patients having a history of intracranial infections (p = 0.02); meningitis was absent in patients with benign tumors (p < 0.01). Intracranial hemorrhages occurred in 7 (3%) cases; 2 of them required surgical treatment. Moreover, there were 6 (2%) wound complications (4 cases of wound cerebrospinal fluid discharge); 5 (2%) EVTVs were prematurely discontinued; isolated neurological deficit occurred in 6 (2%), single seizures were observed in 2 (1%). There were early dysfunctions in 16 (6%) cases; their risk was associated with obstruction in the posterior cranial fossa (p = 0.04) and with the technical result of an operation (p < 0.01). Late dysfunctions occurred in 21 (8%) cases, on the average, after 12 months of EVTV; their risk was higher in patients with malignant tumors (p = 0.04). It is concluded that indications for EVTV should be substantiated by the good chance of having a steady-state surgical success, by an overall prognosis, and staffs experience. The history of intracranial infections requires careful preoperative studies. Obstruction at the level of the posterior intracranial fossa is a relative contraindication to EVTV due to a low chance of having a success. The high risk for late dysfunctions in patients with malignant tumors requires a more strict choice of indications in this group. If there is anastomotic dysfunction, EVTV may be a good alternative to shunt revision.