[Modern aspects of surgical treatment of nasal liquorrhea with localization of defect in frontal sinus]. / Sovremennye aspekty khirurgicheskogo lecheniia nazal'noi likvorei s lokalizatsiei defekta v lobnoi pazukhe.

Defects localized in the frontal sinus are difficult for surgical treatment, since there is a large number of anatomical variations in the structure of the naso-frontal canal and the sinus itself. With the development of endoscopic technology and paying attention to the modern tendency of minimal invasive surgery, new invasive approaches to reach frontal sinus, such as endoscopic approach according to Draf I-III and combined approaches were developed and put into practice. OBJECTIVE: To summarize and to analyze the results of treatment of nasal liquorrhea with localization of the defect in the frontal sinus using endoscopic endonasal and combined intra-extranasal approaches. MATERIAL AND METHODS: A retrospective analysis of a series of 43 cases of nasal liquorrhea with a frontal sinus defect being treated at the NMRCN Burdenko during the period from 2001 to 2017. To select access, a classification of frontal sinus defects according to their localization was developed. The analysis of demographic and clinical data of patients, as well as intraoperative and postoperative data analysis were done. RESULTS: In the series of 43 patients, endoscopic endonasal approach was performed in 28 (65%) cases. Combined approach was performed at 15 (35%) patients. The success rate of plastic surgery with endoscopic endonasal approach was 86% (24 of 28), with combined approach - 93% (14 of 15). CONCLUSION: Endoscopic endonasal and combined approaches are the methods of choice for plastic surgery of defects of the skull base in the frontal sinus as they are effective and safe. The choice of approach depends on anatomical features of the frontal sinus and on the localization of defect.

[Primary juvenile nasal angiofibroma in a 38 year-old male (case report)]. / Pervichno vyiavlennaia iunosheskaia angiofibroma osnovaniia cherepa u muzhchiny 38 let (klinicheskoe nabliudenie).

Juvenile nasal angiofibroma (JNA) is a benign, fibro-vascular, locally aggressive tumor with invasive growth patterns and high probability of recurrence. JNA present exclusively in adolescent boys between 9 and 19 years of age, total about 0.05% of head and neck tumors. 143 patients with JNA had been treated surgically from 2002 to August 2018 at N.N. Burdenko National Medical Research Center for Neurosurgery. All patients (primary and recurrences) were males of the age 7-38 years (16.1 [11.3; 19] years). The age of patients with primary JNA was less than 18-year old, of the exception of presenting case. There are more and more publications about case reports and successful treatment series of JNA. However, after detailed review the literature we revealed some kind of confusion: there are case reports of JNA in women and elderly people, JNA in patients with some systemic diseases, JNA with different places of origin and different morphological structure. In this paper we present a very rare case of 38 year-old male with pure primary JNA, accidentally diagnosed on CT and pathologically proven after endoscopic removal. This case confirms the possibility of detecting the JNA in adult men and explains why proper diagnosis of angiofibromas is necessary. The unique case and the literature review are presented.

[Management of complex skull base defects accompanied by pneumocephalus]. / Taktika lecheniia defektov osnovaniia cherepa, soprovozhdaiushchikhsia pnevmotsefaliei.

AIM: The study aim was to analyze our own experience in treating patients with complex skull base defects accompanied by pneumocephalus and, based on the findings, to develop an optimal treatment approach for this pathology. MATERIAL AND METHODS: We retrospectively reviewed a series of 30 patients with complex skull base defects accompanied by pneumocephalus who underwent inpatient treatment at the Burdenko Neurosurgical Institute in the period from 2001 to 2017. We analyzed demographic characteristics of patients (gender, age), clinical data (etiology, somatic and neuropsychiatric status, radiological data), and treatment aspects (conservative or surgical treatment, used approach, defect characteristics, reconstructive materials). Treatment outcomes were assessed based on analysis of changes in clinical manifestations, postoperative complications, and recurrences. The obtained data were compared to the results of literature review. RESULTS: In the series of 30 patients with complex skull base defects accompanied by pneumocephalus, the mean age was 41 years (range, 17-68 years); there were 17 (59%) males and 13 (41%) females. Etiologically, there were 17 (59%) traumatic cases, 11 (36%) iatrogenic cases, and 2 (5%) spontaneous cases. Clinically, the patients presented with the following manifestations: psycho-neurological symptoms (41%), nasal cerebrospinal fluid (CSF) leak (36%), impaired consciousness (27%), and meningitis (23%). Eight patients with acute injuries underwent complex conservative treatment that included infusion, and anti-edema, vascular, metabolic, anticonvulsant, and antibacterial therapy. In cases of conservative treatment failure or tension pneumocephalus accompanied by abrupt worsening of the patient's condition, surgical treatment was used. A total of 24 interventions (including revision surgery) were performed in 22 patients. All patients underwent endoscopic endonasal reconstruction of complex skull base defects. Tissues used for reconstruction included the fascia lata and adipose tissue (77%), fascia lata and cartilage/bone from the nasal septum (14%), and a pedicled nasoseptal flap (9%). The success rate of reconstructive interventions was 91%. There were 2 (9%) recurrences. Postoperative complications in the form of meningitis occurred in 4 (18%) patients. CONCLUSION: In the case of a nasal cerebrospinal leak history and worsening of the patient's condition accompanied by common cerebral and psychiatric symptoms, the development of pneumocephalus should be considered first, the early diagnosis of which will facilitate choosing the correct treatment approach. In the case of acute injury in patients with the established diagnosis of pneumocephalus caused by gas-forming infection, conservative therapy is indicated in the early period after neurosurgical interventions with opening of the meninges. Tension pneumocephalus together with a skull base bone defect (according to CT) and a nasal CSF leak history is the indication for surgical treatment. In this case, the endoscopic endonasal technique is the method of choice.

[The causes of late diagnostics of vestibular shwannomas]. / Prichiny pozdnei diagnostiki vestibuliarnykh shvannom.

AIM: The objective of the present study was to elucidate the causes of late diagnostics of vestibular shwannomas based on the results of the analysis of the medical histories of the patients admitted to the Academician N.N. Burdenko National Medical Research Centre of Neurosurgery for the management of this condition. MATERIAL AND METHODS: We undertook the retrospective review of a series of 192 cases of vestibular shwannomas in the patients treated based at the N.N. Burdenko National Medical Research Centre of Neurosurgery in 2015. The study included the analysis of demographic characteristics of the patients as well as their anamnestic and clinical data. RESULTS AND DISCUSSION: A total of 55 (29%) patients were found to have small tumours (Samii T1-T3) whereas 137 (71%) ones presented with giant neoplasms including T4a and T4b tumours (in accordance with the Samii classification) in 63 (33%) and 74 (38%) patients respectively. The duration of the disease from the appearance of its first clinical symptoms up to the establishment of the definitive diagnosis was 41 months on the average. 37 (19%) patients appeared to have paid no attention to the unilateral impairment of hearing. 46 (24%) patients applied for the medical assistance to the therapists and neurologists. 56 (29%) patients had remained for a long time under the supervision of the ENT specialists and surdologists based at the local outpatient facilities. CONCLUSION: More than 2/3 of the examined patients presenting with vestibular shwannomas apply for the medical assistance at the late stages of the disease. Such situation can be attributed to the low level of oncological alertness among the general population and health providers, the poor awareness of the general practitioners and otorhinolaryngologists about this pathology, the unavailability or inefficient application of the modern neurovisualization technologies, in the first place magnetic resonance imaging. The problems encountered in connection with the performance of screening studies give evidence of the necessity of the introduction of up-to-date otoneurological and audiological investigations as well as the neurovisualization techniques into the everyday clinical practice for the comprehensive examination of all the patients suffering from unilateral hearing loss.

[A modern approach to the diagnosis of nasal liquorrhea]. / Sovremennyi podkhod k diagnostike nazal'noi likvorei.

Nasal liquorrhea is cerebrospinal fluid leakage from cerebrospinal fluid spaces of the cerebral cavity into the nasal cavity or paranasal sinuses due to congenital or acquired abnormalities of the skull base bones and meninges of various etiologies. The severity of liquorrhea varies from hidden manifestations to profuse leakage of cerebrospinal fluid from the nasal cavity. The diagnosis of overt nasal liquorrhea is not problematic, but the diagnosis of latent liquorrhea is a challenge. In this case, the disease leads to potentially fatal complications, such as meningitis (the risk amounts to 10-37%), pneumocephaly, pneumonia, etc. These peculiarites give rise to two main tasks: early diagnosis confirming liquorrhea and accurate identification of the CSF fistula location when planning further surgical management. PURPOSE: The study purpose was to review and comparatively analyze all modern methods of diagnosing nasal liquorrhea as well as to substantiate the most effective and promising approaches and algorithms. MATERIAL AND METHODS: The study included papers in English and Russian found in the Pubmed database and related to the diagnosis of basal liquorrhea of different etiology and localization. RESULTS: This review demonstrates that diagnostic tests vary widely in sensitivity, specificity, accuracy, invasiveness, and cost. Given all the criteria, detection of beta-2 transferrin or beta-trace protein is the best method for confirming nasal liquorrhea, and high-resolution computed tomography is the best technique for localization of the abnormality. CONCLUSION: Based on the review, we suggest a diagnostic algorithm for nasal liquorrhea. However, the evidence presented in this review is unfortunately not very reliable, which indicates the existing need for more accurate studies.

[Nasal schwannoma with skull base destruction in a child (a case report and literature review)]. / Nazal'naia shvannoma s destruktsiei osnovaniia cherepa u rebenka (sluchai iz praktiki i obzor literatury).

Schwannomas (neurinomas) are benign, encapsulated, and slow-growing neoplasms developing from any peripheral, cranial, or vegetative nerve that has the Schwann sheath. Schwannomas of the nasal cavity and paranasal sinuses in adults occur rarely, less than 4% of cases. The world literature lacks similar data for the pediatric age group because these tumors in children are unique and extremely rare. The most common clinical symptoms of sinonasal schwannoma are nasal breathing disorders, hyposmia or anosmia, and snoring at night. Other symptoms are associated with intranasal schwannoma invasion of neighboring organs and tissues and include exophthalmos, epiphora (lacrimation), headaches, and anesthesia in the facial area. In this article, we describe a rare case of diagnosis and successful surgical treatment of a child with intranasal schwannoma destructing the skull base.

[Experience of treating patients with lateral sphenoid sinus recess CSF leak]. / Nash opyt lecheniia patsientov s nazal'noi likvoreei iz lateral'nogo karmana klinovidnoi pazukhi.

According to various authors, the rate of sphenoid sinus defects ranges from 15 to 26%. In the sphenoid sinus, the most common defect localization is the lateral recess - 35.3%. Regardless of the etiology and pathogenesis of lateral sphenoid sinus recess cerebrospinal fluid (CSF) leak, the treatment approach is aimed at plastic closure of the defect. Surgery in this area is associated with a high rate of recurrences (25%) due to a complex anatomical landscape and inapplicability of a direct approach. PURPOSE: The study purpose was to analyze our own experience in treating patients with CSF leak and meningoencephalocele in the lateral sphenoid sinus recess and, based on the obtained results, develop an algorithm for the treatment of this pathology. MATERIAL AND METHODS: We retrospectively reviewed a series of 89 patients with CSF leak and meningoencephalocele in the lateral sphenoid sinus recess who were treated at the Burdenko Neurosurgical Institute in the period between 2001 and 2017. The demographic characteristics of patients, clinical data, and aspects of surgical treatment were analyzed. Treatment outcomes were assessed based on analysis of recurrences and postoperative complications. The obtained data were compared to the literature data. RESULTS: In the series of 89 patients with CSF leak and meningoencephalocele in the lateral sphenoid sinus recess, the mean age was 49 years. Surgery through the transsphenoid approach was performed in 89 (74%) patients. There were 25 (28%) recurrences in this group. Nine (7%) patients underwent repair of a complex skull base defect using the transethmoidal approach; there were 3 (33%) recurrences. The transpterygoid approach was used in 22 (17%) patients; there were 3 (14%) recurrences in this group. CONCLUSION: Endoscopic endonasal repair is an effective technique for treating complex skull base defects in the lateral sphenoid sinus recess. The choice of an approach to the defect depends on the degree of pneumatization of the sphenoid sinus, surgeon's experience, and availability of the necessary equipment and tools. The endoscopic transpterygoid approach is advisable to use in cases of excessive pneumatisation of the sinus with developed lateral processes and recurrent nasal CSF leak. This enables visualization of the defect edges in this hard-to-reach area and adequate repair.

[Modern diagnosis and treatment in children with congenital basal encephalocele]. / Sovremennye metody diagnostiki i lecheniia detei s vrozhdennymi bazal'nymi cherepno-mozgovymi gryzhami.

Basal encephalocele is a rare disease that predominantly occurs in children. Its most common symptoms include nasal liquorrhea, difficulty in nasal breathing, and deformity of the naso-orbital region. MATERIAL AND METHODS: The study group included 19 patients with basal encephalocele, aged 2 months to 18 years. Ten (59%) patients were operated on through a transnasal endoscopic approach; 3 (17.5%) patients were operated on through a transcranial approach; 4 (23.5%) patients were operated on using a combined approach: the patients underwent simultaneous elimination of a cranio-orbital region deformity using the basal transcranial approach as well as hernial sac resection and hernioplasty using the transnasal endoscopic approach. Two children had no surgery due to minimal symptoms and a lack of cerebrospinal fluid leak. RESULTS: Application of the algorithms for diagnosis and treatment of encephalocele, suggested by the authors, enabled making the timely diagnose, defining the optimal surgical tactics, and achieving good treatment results. CONCLUSION: A differentiated approach to the choice of a surgical technique for basal encephalocele, the use of auto-tissues for skull base reconstruction, intraoperative and postoperative lumbar drainage, and simultaneous elimination of deformity of the fronto-naso-orbital region enable avoiding complications and achieving good functional and aesthetic results.

[Endoscopic endonasal diagnosis and treatment of skull base meningoencephalocele]. / Éndoskopicheskaia éndonazal'naia diagnostika i lechenie meningoéntsefalotsele osnovaniia cherepa.

PURPOSE: The study purpose was to determine the successful outcome rate of endoscopic endonasal reconstruction of skull base CSF fistulas with removal of meningocele as well as analyze disease relapses. The second purpose of the study was to describe the treatment outcomes, depending on the type, location, and size of meningocele as well as on a technique used to reconstruct the skull base defect. MATERIAL AND METHODS: Three hundred and eleven patients diagnosed with cerebrospinal fluid (CSF) rhinorrhea who underwent surgery at the Burdenko Neurosurgical Institute in the period between 2007 and 2014 were retrospectively analyzed. Meningocele was detected in 141 (45.3%) of all patients with CSF rhinorrhea. The diagnosis was made before surgery and verified during endoscopic endonasal reconstructive closure of skull base defects. Therefore, the analysis group included all cases of meningocele and meningoencephalocele verified during surgery. The study analyzed the gender, age of surgery, follow-up period, comorbidities, use of lumbar drainage, etiology of the defect, localization, type of reconstructive material, complications, and disease relapses. We defined the efficacy of endoscopic endonasal meningocele resection with fistula reconstruction as the absence of signs of CSF rhinorrhea and a hernia sac based on the data of control CT and endoscopy performed in the late postoperative period. The follow-up period ranged from 1 month to 5 years. RESULTS: All patients underwent endoscopic endonasal reconstruction of the skull base defect. The treatment efficacy was 110 (78%) patients for primary surgery and 26 (84%) patients for repeated surgery. CONCLUSION: Endoscopic endonasal surgery is a safe, effective, and minimally invasive technique for treatment of meningocele and meningoencephalocele, which enables resection of a hernia sac and reconstruction of dura mater and skull base defects.

[First experience of CyberKnife stereotactic radiotherapy for glomus jugulare tumors].

Glomus jugulare tumors (GJT) (AKA paraganglioma or chemodecroma) make 0.6% of head and neck tumors and 80% of jugular foramen lesions. Recently different types of stereotactic radiotherapy are actively used in their treatment. The given paper summarizes our first experience of using CyberKnife system in management of patients with paragangliomas. Since April 2009 till September 2011 34 patients with GJT were treated in department for radiation therapy of Burdenko Neurosurgical Institute using CyberKnife system. Mean age was 50.5 years (range 26-75 years). Mean volume of treated lesions was 14.6 +/- 10.96 cm3 (range--1.04-37.4 cm3). Stereotactic radiosurgery was performed in 4 patients. Mean prescribed dose was 17 +/- 3.1 Gy (13.7-22 Gy). 30 patients received hypofractionation treatment (3-7 fractions with total dose 18-35 Gy). Mean follow-up period in our series was 8 months (1-20 months). Control of tumor growth reached 100% because none of the patients needed any additional therapy (microsurgical resection or repeated radiotherapy). Persistent trigeminal neuropathy was observed in 1 case (5%). There were no constant impairment of VII, IX, X, XII and XIII cranial nerves in the series. Stabilization of auditory function was present in 9 (82%) of 11 patients with initially preserved hearing. Preservation of effective hearing was observed in 75% of patients. Stereotactic radiosurgery and hypofractionation using CyberKnife system are effective modalities in management of such complex neurosurgical disease as GJT. Neurological deterioration after radiation treatment is extremely rare and in most cases is transient.

[Computed tomography of the temporal bone for the diagnosis of facial nerve neurinoma].

The present retrospective study of 6,200 subjects with temporal bone pathology allowed for the identification of a group of 12 patients aged between 12 and 59 years in whom the presence of facial nerve neurinoma diagnosed by computed tomography was confirmed during surgical intervention and by histological methods. The patients were allocated to three groups depending on the localization of neurinoma. Patients of group 1 (n=8) had neurinoma of the mastoidal segment, those of group 2 presented with neurinoma of the tympanic segment, and patients of group 3 showed combined lesions of the tympanic and labyrinthine segments. Clinical and CT characteristics of each group are presented. CT of the temporal bone is shown to provide a tool of high informative value for the diagnosis of facial nerve neurinoma. It is suggested that CT should be used to examine patients with facial nerve paresis or conductive and mixed hearing loss of unspecified etiology for the early diagnosis of facial nerve neurinoma.

[Stereotactic radiotherapy and radiosurgery in treatment of patients with intracranial schwannomas].

Aim of this study is to assess the role of stereotactic radiosurgery (SRS) and radiotherapy (SRT) in management of cranial nerves schwannomas by analysis of tumor control, clinical response and variables affecting treatment outcomes. Between April 2005 and January 2009 patients with schwannomas of VIII (63), V (14) and caudal nerves (2) were treated in Burdenko Moscow Neurosurgical Institute using linear accelerator. Mean age was 49 years (13-82). In 42 cases radiation treatment was preceded by surgical resection. 13 patients had type I or II neurofibromatosis. Mean volume of the tumor was 3.9 cm3 (0.5-14.4 cm3) and 13.4 cm3 (2.8-41.3 cm3) for SRS and SRT, respectively. Mean SRS dose was 12 Gy (10.8-14.4 Gy) for vestibular schwannomas and 15 Gy (13.2-18 Gy) for schwannomas of other nerves. In hypofractionated SRT the dose of 35 Gy was delivered in 7 fractions or 30 Gy in 6 fractions. In cases of classical fractioning total dose of 50-60 Gy was divided into daily fractions of 1.8-2.0 Gy. Radiographic tumor control rate reached 97.5% at the last follow-up. 5 patients experienced trigeminal dysfunction, it was transient in 3 cases and persistent in 2. Permanent decline in House-Brackmann facial nerve scale developed in 2 of 79 patients. After treatment effective hearing (class I-II) was preserved in 7 of 9 patients (67%) who had same level of hearing before SRS. Linear accelerator-based stereotactic radiation treatment provides long-term tumor control associated with high rates of preservation of neurological functions. No further tumor surgery was necessary in 100% of cases with solitary tumors with a minimal follow-up of 5 years.

[Endoscopic rhinosurgery: from simple intranasal surgery to interventions on orbital apex and base of the skull].

This paper is focused on the main methods and indications for endoscopic intranasal surgery, from conventional infundibulotomy for the management of chronic rhinosinusutis resistant to conservative therapy to up-to-date endoscopic techniques for the treatment of lacrimal duct obstruction, nasal liquorrhea, endocrine ophthalmopathy, benign and malignant tumours of paranasal sinuses, and skull base lesions. A review of the literature data is supplemented by original clinical observations.

[Surgical treatment of extensive craniofacial juvenile angiofibromas].

Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumor occurring almost exclusively in adolescent and young adult males. The tumor is characterized by slow progression, aggressive growth, high vascularization and increased rate of persistence and recurrence. From 2000 till 2008 29 consecutive male patients with JNA Fisch grade III and IV (intracranial extradural or intradural extension) were operated in Burdenko Neurosurgical Institute (Moscow, Russia). Most patients received different kinds of treatment before admission to the Institute. All patients underwent surgical resection using predominantly orbitozygomatic approach. Preoperative endovascular embolization was applied. Total removal was achieved in 86% of cases. Postoperative complications included osteomyelitis of the bone flap (4 cases), nasal CSF leak (1 case) etc. Recurrences were observed in 3 patients, all of them underwent repeated surgeries. 4 cases are presented (3 patients with Fisch grade IV tumor and 1 with grade III). Surgical treatment is the basic tactics in management of extensive JNAs. The authors recommend to use orbitozygomatic approach and endoscopic assistance.

[Endoscopic surgical treatment of vascular tumors of the paranasal sinuses and base of the skull].

Fourteen endonasal endoscopic operations for vascular tumors of the paranasal sinuses, mainly of stage II-III, were performed in 1997-2006. In one case intraoperative navigation was used. Preoperative angiography was made in 4 patients with a definite diagnosis of hemangioma. Out of them, in three cases preoperative embolisation of the afferent vessel was conducted; apparent vascular bed in tumor stroma was not found. Follow-up from 1 to 8 years registered no further growth of the tumor in patients operated endoscopically.

[Rhinological aspects of endoscopic endonasal removal of hypophysial adenomas].

The authors analyse rhinological aspects of endoscopic endonasal transsphenoidal adenomectomy (EETA): a nasal operation, intra- and postoperative liquorrhea, liquorrhea-associated meningitis, postoperative nasal hemorrhage, local changes of nasal cavity anatomic structures; provide practical recommendations on management of the conditions many of which are life-threatening.