[Hepatopulmonary syndrome: a rare manifestation of cirrhosis in patient with diencephalic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma].

We describe a 15-year girl, who developed panhypopituitarism and diencephalic obesity after surgical excision of craniopharyngioma, followed by nonalcoholic fatty liver disease and cirrhosis 5 years after surgery. Cirrhosis in this case manifested by hypoxia due to hepatopulmonary syndrome, and despite cure of craniopharyngioma by surgery and radiosurgery treatment and adequate hormonal substitution therapy patient died 9 years after surgery. Growth hormone substitutional therapy in patients with hypopituitarism, and steatohepatitis may decrease liver triglyceride accumulation and prevent end-stage liver disease.

[Radiosurgical irradiation of patients with cerebral AVM on the Gamma knife].

UNLABELLED: Arterio-venous malformation (AVM) of the brain are congenital malformations of cerebral vessels in the embryonic period. Standards in AVM treatment is currently applying the endovascular technique, microneurosurgical removal and stereotactic radiation. MATERIALS AND METHODS: In the period from 2005 to 2011, in 139 patients with arteriovenous malformations of the brain were treated using "Gamma Knife". 89 (64%) of 139 patients suffered bleeding in anamnesis. Paroxysmal symptoms of varying severity dominated in the clinical picture of 35 (27%) patients. Previous microsurgical resection was performed in 10 patients. 28 patients experienced a partial embolization of vascular malformations before radiosurgery. A boundary value radiation dose ranged from 18 to 28 Gy (mean dose 20 Gy), day dose was in the range of 40-80% (average 50% isodose). Maximum dose ranged between 18-60 Gy (mean dose of 38 Gy.). RESULTS: Control angiography or spiral CT screening angiography was performed in all 85 patients who were treated with follow-up data for more than two years, showed that total obliteration occurred in 75% of cases. Frequency of symptomatic radiation reactions was less than 5%. After two years or more after treatment, complete obliteration was observed in 74% of patients. CONCLUSIONS: Higher frequency of AVM obliteration was seen with up to 2 cc. and the irradiation dose of at least 24 Gy.

[Microsurgical treatment of cerebral arteriovenous malformations in Burdenko Neurosurgical Institute (recent experience)].

Recently the number of AVM resections in Burdenko Neurosurgical Institute has been increased dramatically. Aim of this study was to assess the results of open surgery in our clinic in modern neurosurgical era. Consecutive series if 160 patients with AVM treated using microsurgical technique since 2009 till 2011 was analyzed. Spetzler-Martin score distribution was: grade I--29 (18.1%) cases, grade II--84 (52.5%), grade III--38 (23.8%), grade IV--9 (5.6%). Patients with grade V AVMs were not operated. Treatment options included: AVM resection in 143 (89.4%) cases, embolization followed by resection in 15 (9.3%) and clipping of afferents in 2 (1.3%). Glasgow outcome scale score distribution was the following: V (good recovery)--70 (43.7%), IV (moderate disability)--71 (44.4%), III (severe disability)--16 (10%), II (vegetative state)--1 (0.6%) and I (death)--2 (1.3%). Microsurgery remains the primary option for radical treatment of cerebral AVMs. Careful selection of patients and planning of surgery are crucial for good outcomes.

[Application of robotized radiosurgical system CyberKnife for the treatment of neurosurgical patients].

Robotized system for radiosurgery CyberKnife (Accuray Inc., USA) is the first device dedicated and optimized for advanced irradiation during 1-7 fractions (i.e. radiosurgery and hypofractionation). CyberKnife is characterized by elaborate guidance system, high precision of dose delivery, possibility of conformal dose distributions with high gradient of target borderline dose which is most important in proximity of critical structures. The first CyberKnife system in Russia was installed in Burdenko Neurosurgery Institute. The paper presents 2-year experience of treating patients using CyberKnife. From April 2009 till October 2011 896 patients were treated using CyberKnife. Mean age was 48 years. Overall number of sessions was 2626. Radiosurgical procedures were performed in 21.8% of patients. 91% of cases were treated for intracranial lesions. Limited follow-up period in all kinds of pathology demonstrated results consistent with standard fractionation or radiosurgery. The rates of observed complications were also comparable with accepted techniques. CyberKnife system plays significant role in everyday activity of department of radiation therapy. In careful and thorough selection of patients it allows efficient and high-quality treatment of patients with neurosurgical diseases.

[First experience of radiosurgical treatment of arteriovenous malformations using CyberKnife].

Radiation treatment techniques are essential in complex management of selected neurooncological, neurovascular and functional brain pathology. Stereotactic radiotherapy provides sufficient damage to the target (tumor, AVM, functional structures) with minimal impact on surrounding brain tissue. Development of stereotactic methods with X-ray guidance lead to expansion of indications for irradiation of intracranial and extracranial lesions. This paper represents the first experience of treating patients with AVMs using CyberKnife system in Russia. The technique of stereotactic radiotherapy (SRT) and radiosurgery (SRS) is described in details, as well as indications for SRT and SRS in different regiment of fractionation, possibilities of planning systems, with additional emphasis on specific complications, radiation-induced reactions and capabilities of neurovisualization methods in evaluation of radiation-induced damage of brain tissue.

[Stereotactic radiotherapy and radiosurgery in treatment of patients with deep-seated pilocytic astrocytomas].

Pilocytic astrocytoma (PA) is a low-grade glial tumor (WHO grade I) with predominant occurrence in pediatric patients. According to many authors, stereotactic radiosurgery (SRS) and radiotherapy (SRT) promote long-term remission or retardation of tumor progression in patients with in inoperable lesions after incomplete resection or recurrence. Therefore it is essential to determine the role of SRS and SRT in complex management of patients with deep-seated PA. Since April 2005 till May 2010 101 patient with intracranial PA was treated in department for radiation therapy of Burdenko Neurosurgical Institute. The series consisted of 70 pediatric patients (below 17 years inclusively) and 31 adults, of them--51 male and 50 female patients. Mean age was 15.1 years (9.8 years in children and 28.7 in adults). In 90 patients (89.2%) tumors were previously histologically verified (tumor resection in 83 cases and biopsy in 7). In 11 (10.8%) patients diagnosis of PA was based on clinical and neurovisualization data. In most cases SRT (66 (66.3%) patients) was preformed, the rest 35 (34.7%) patients were treated by SRS. Median follow-up from the onset of disease reached 52 months (2-228 months). Catamnestic data were available in 88 (87%) patients. By the end of catamnestic follow-up (December 2010) 87 (98.8%) patients treated by SRS and SRT were alive. Median follow-up from the start of radiation treatment was 22.7 months (6-60 months). Progression of tumor was observed in 20 patients (22.7%), in 18 of them due to cyst growth. 18 patients were reoperated. In 12 operated patients histological examination and its comparative analysis were performed. We found that alterations in the tumor tissue, accompanied by regression of solid component and progression of cystic portion, represent reactive-degenerative changes in the tumor as a consequence of radiation-induced pathomorphism. SRS and STR are effective techniques for treatment of patients with primary and recurrent PA despite regardless of localization of the tumor. There procedures should be performed shortly after non-radical resection. Control of tumor growth by the present time (median follow-up is 22.7 months) reaches 98%. "Progression" of the tumor due to enlargement of cystic portion shortly after SRT and SRS represents reactive-degenerative alterations in the tumor tissue and should not be evaluated as true recurrence; without neurological deterioration these cases do not require special treatment.

[Linear accelerator-based stereotactic radiotherapy and radiosurgery in treatment of neurosurgical patients].

Radiation methods are essential in management of certain types of neurooncological, neurovascular and functional brain pathology. Application of stereotactic technique of irradiation allows sufficient damaging impact on target (tumor, AVM, functional structures) and maximal protection of surrounding brain tissues. Indications to radiation treatment of intracranial lesions are expanding with evolution of stereotactic methods. The paper deals with the first experience of linear accelerator-based treatment of patients with intracranial neurosurgical pathology in this country. Techniques of stereotactic radiotherapy (SRT) and radiosurgery (SRS), indications to SRS and SRT in different modes of fractioning are described in details. Additionally specific complications, radiation reactions and feasibility of neurovisualization in diagnosis of radiation-induced brain damage are discussed.

[First experience with a Gamma-knife unit used for radiosurgical treatment for intracranial space-occupying lesions].

A Gamma-Knife unit that is currently the gold standard in radiosurgery was first used in clinical practice in 1968. The essence of the radiosurgical treatment is to apply the stereotactic technique to high-precision irradiation of small intracranial targets by narrow beams of ionizing radiation from external sources. Three hundred and six patients with various intracranial diseases (137 with malignant tumors, 136 with benign tumors, and 33 patients with vascular diseases) underwent radiosurgery on a Gamma-Knife unit for over 1.5 years, from May 2005 to October 2006. A total of 329 irradiation sessions were performed. By summing up the first results, it can be noted that stereotactic radiosurgery using a Gamma-Knife unit is an effective and rather safe technique in the treatment of patients with various types of intracranial pathology, which maintains the high quality of life, reduces the likelihood of development of different neurological disorders after treatment and avoids the risk of operative complications. The possibility of performing outpatient treatment for a diversity of diseases without mandatory anesthesiological, ICU, and rehabilitative maintenance, determines the potential economic efficiency of this treatment.

[Results of surgical treatment in patients with neurocytomas of the brain].

Central neurocytoma (CN) is a rare brain neoplasm which is characterized by certain clinical, radiological, and morphological characteristics. This tumor was first described in 1982 by J. Hassoun et al. who found neurosecretory granules and microtubules while microscopically studying 2 patients with oligodendrogliomas of lateral ventricles and concluded that these neoplasms were of neuronal origin. Materials and methods. In 1992 to 2004, a total of 84 patients diagnosed as having CN were treated at the Academician N. N. Burdenko Research Institute of Neurosurgery, Russian Academy of Medical Sciences. In most cases, the tumor showed a clinical picture as elevated intracranial pressure that was detected in 80 (more than 90%) cases. In this series, all neurocytomas were located in the ventricles, mainly in the pellucid septal region; tumor infiltration of the lateral ventricular ependyma and medullary substance invasion into the region of the thalamus and corpus callosum. Computed tomographic scans displayed central neurocytomas as space-occupying lesions of mixed (heterogeneous) density, which frequently contained single and multiple petrificates and cysts. TI-weighted magnetic resonance imaging (MRI) indicated that in most cases (n=56, 66%), the intensity of a signal from the tumor was mixed and similar to that of the white matter of the brain. These masses were characterized by the presence of tumor structural cysts that were better visualized on T2-weighted tomograms. The tumor was removed in 83 cases; 2 out of them had previously undergone stereotactic biopsy (STB). STB was performed alone in one case. Transcallous, transcortical-through-premotor-area, combined, and transcallous-transcortical approaches were applied when the neurocytomas were removed. Results. Total removal of a tumor (when its remains were not visualized by postoperative MRI) was achieved in 11 (13%) patients. Its subtotal removal was accomplished in 48 (58%) patients. Partial removal was done in 24 (29%) patients. In the early postoperative period after tumor removal, there was a worse health status along with a transient progression of cerebral and focal symptoms in most patients with CN. The magnitude, pattern, and duration of these changes differed. Conclusion. The long (from several months to several years) history of the disease and young age (14 to 59 years) are typical of patients with CN. The tumors are located intraventricularly, more commonly along the midline, and they have well-defined X-ray signs: these are well-circumscribed tumors of heterogeneous density, which frequently contain single and/or multiple cysts and petrificates; there is a moderate contrast enhancement. The basic way of improving the results of treatment in patients with CN is the latter's early diagnosis and surgery when the tumor is locally advanced within the ventricular system.