In this article, we consider the influence of combined general anesthesia on the results of electrically-involved stapedial reflex threshold (esrt) registration. We pay a special attention to the anesthesia to exclude its influence on the esrt. This study included 52 patients with bilateral chronic neurosensory hearing impairment. We conducted a retrospective (from 2014 to 2016) and prospective (from 2017 to 2018) analysis of anesthesia protocols. Even though the inhaled anesthesia (sevoflurane in this case) has a depressive effect on esrt registration (the higher the minimal alveolar concentration of anesthetic agent, the higher the reflex threshold), our study shows a possibility of using it in an extended anesthesia monitoring. Inclusion of myorelaxants in cochlear implantation anesthesia protocol not only provides a safe anesthesia, but also does not prevent a timely intraoperative cochlear implant testing.

Introduction. Cochlear implantation is a recognized treatment method for patients with severe and deep neurosensory hearing loss. To adjust the parameters of the speech processor of a cochlear implant (CI), the implementation of subjective techniques is not always possible, especially for younger patients. A good starting point for determining patient comfort levels during the initial connection of a speech processor is the intraoperatively obtained data of objective methods, one of which is the registration of an electrically induced stapedial reflex (EISR). Due to the marked effect of general anesthesia on the results of testing of CI and the lack of a universal anesthesiological protocol for this surgical intervention, the purpose of the study is to study the effectiveness of using extended intraoperative monitoring during testing of CI. Material and methods. The study included two groups, each consisting of 26 patients aged from 1 year to 15 years with a diagnosis of chronic sensorineural hearing loss. All children were installed CI delivered by company Med El (Austria). In the 1st group, data from medical records were analyzed retrospectively (anesthetic patient card and ESRT threshold values registration card during anesthesia and standard anesthesia monitoring volume). In the 2nd group of patients, intraoperative monitoring of anesthesia was extended by continuous evaluation of EEG (BIS-index) and indices of the degree of myoplegia. CI testing in this group was carried out in the recovery phase of neuro-muscular conductivity (4 TOF responses) and with BIS-index values from 60 to 80, which corresponded to sedation during drug sleep. Results. In the course of the work done in two groups of patients, the average values of intermediate points of the ESRT were analyzed, a comparative assessment of hemodynamic parameters at the main stages of the operation was carried out, and the optimal values of the minimum alveolar concentration of sevoflurane anesthetic and BIS index were determined for timely registration of the movement of stapedial muscle by the surgeon.

Relevance. The hypoplastic left-heart syndrome at congenital heart disease has frequency of 261 cases out of 10,000 newborns. Children with hypoplastic left-heart syndrome can have comorbid congenital anomalies and acquired diseases that require treatment and are associated with high mortality risk.Description of a clinical case. Female patient, 6 years 4 months, with the hypoplastic left-heart syndrome was admitted to our hospital with the diagnosis «Sensorineural hearing loss, speech delay» for performing of cochlear implantation. The council of physicians was convoked before the surgery to coordinate patient management. Members of surgical, anesthesiology, laboratory and instrumental examination departments were involved into preoperative assessment and planning due to the high level of surgical and anesthetic risk. The patient was dismissed from hospital on the 10th day after cochlear implantation. The patient has undergone the course of auditory-verbal therapy a month after. The child was dismissed wit state improvement.Conclusion. The example of successfully performed surgery in the patient with hypoplastic left-heart syndrome and such severe comorbid pathology as sensorineural hearing loss is presented. Complete physical examination of the child in preoperative period and adequate preparation for the surgery were the key factors for patient successful management and further rehabilitation.

Relevance. The hypoplastic left-heart syndrome at congenital heart disease has frequency of 261 cases out of 10,000 newborns. Children with hypoplastic left-heart syndrome can have comorbid congenital anomalies and acquired diseases that require treatment and are associated with high mortality risk. Description of a clinical case . Female patient, 6 years 4 months, with the hypoplastic left-heart syndrome was admitted to our hospital with the diagnosis «Sensorineural hearing loss, speech delay» for performing of cochlear implantation. The council of physicians was convoked before the surgery to coordinate patient management. Members of surgical, anesthesiology, laboratory and instrumental examination departments were involved into preoperative assessment and planning due to the high level of surgical and anesthetic risk. The patient was dismissed from hospital on the 10th day after cochlear implantation. The patient has undergone the course of auditory-verbal therapy a month after. The child was dismissed wit state improvement. Conclusion. The example of successfully performed surgery in the patient with hypoplastic left-heart syndrome and such severe comorbid pathology as sensorineural hearing loss is presented. Complete physical examination of the child in preoperative period and adequate preparation for the surgery were the key factors for patient successful management and further rehabilitation.

For the time present, the cochlear implantation is a widely used method of the treatment of deafness and severe hearing impairment. The operation is time-consuming and requires a long stay of the child in the hospital. The placement of the cochlear implant (CI) is executed under general anesthesia through transosseous access. The executive work of the surgical and anesthetic teams is an integral part of the successful implementation of intraoperative CI testing, on the base of results of which, in the postoperative period, there will be carried out the initial adjustment of the speech processor. An anesthesiologist should create conditions that facilitate the use of nerve stimulators in the intraoperative period and in a timely manner prevent such common postoperative complications as nausea, vomiting, and dizziness. This article provides a review of the literature of domestic and foreign authors on the issues arising in anesthesiologists during the implementation of cochlear implantations and the ways of their solutions. It is considered what difficulties can arise during the installation and initial adjustment of CI, as well as what anesthesia complications are most frequent in operations of this type. After studying the results of the work of different authors, the initial adjustment of the CI in the anesthetic management under the control of BIS and TOF monitoring was concluded to be performed at a higher level, and the timely and targeted administration of various drugs, at certain stages of anesthesia, contributed to the decline of the complications rate in the postoperative period.

Data of tests on bearing segments supported by spheres and by flat elastic chambers without support disks are considered.

The article deals with data of cerebral oximeter INVOS Somanetics (US) used for comparison the degree of brain oxygenation reduction, ejection fraction and stroke volume relative to age norms in children with dilated cardiomyopathy.

Many orphan diseases in children require life-long and regular intravenous enzyme replacement therapy. The article describes the first Russian practice of implanting venous port systems in 12 patients with type I and II mucopolysaccharidosis and Pompe disease (6 months to 17 years old) to create long-term venous access. Currently, implantable venous port systems are used in 9 (75%) of 12 patients. 4 cases of thrombosis are observed in 3 patients. All of them have been successfully treated. 1 patient had a rotation of the port camera with a membrane facing downwards due to violation of an implantation technique. The camera was adjusted during the second operation.

The data presented suggest the possibility of using non-invasive cerebral oxymeter for the evaluation of changes of brain oxygenation during surgical intervention under sevoran anesthesia. The potential of cerebral oximetry for noninvasive postoperative monitoring tissue saturation with oxygen is evaluated.

Aim : to develop a system of evaluation of liver failure stage in children based on the International classification of functioning, disability and health (ICF). Patients and methods : based on the retrospective analysis of 14 biochemical markers, characterizing hepatic role in proteins, lipids and carbohydrates metabolism, of 115 children without liver diseases, 15 children who died of liver failure and 220 patients with various hepatic disorders, being followed-up in the SCCH of RAMS, a score system of evaluation of liver failure stage in children as an additional diagnostic tool was developed . Each of the biochemical markers was assessed according to the 5-point rating scale in dependence of its changes intensity. Results : the sum of points was considered to be a criterion of liver failure stage. According to the ICF recommendations, decrease of points on 0–4% (54–56 points) corresponds with absence of liver failure; 5–24% (43–53 points) — as mild dysfunction, 25–49% (29–42 points) — as moderate; 50-95% (3–28 points) — as severe; and 96–100% (0-2 points) — as absolute failure. Conclusions : score system of evaluation of liver failure stage can be applied at any step of diagnostics and treatment of children of any age, due to independence of the used markers from the age. It can be used in assessment of the severity of disorder in dynamics, in determination of the prognosis and as criterion of indications to liver transplantation, as well as during medico-social examination.

Aim: to develop a system of evaluation of liver failure stage in children based on the International classification of functioning, disability and health (ICF). Patients and methods: based on the retrospective analysis of 14 biochemical markers, characterizing hepatic role in proteins, lipids and carbohydrates metabolism, of 115 children without liver diseases, 15 children who died of liver failure and 220 patients with various hepatic disorders, being followed-up in the SCCH of RAMS, a score system of evaluation of liver failure stage in children as an additional diagnostic tool was developed. Each of the biochemical markers was assessed according to the 5-point rating scale in dependence of its changes intensity. Results: the sum of points was considered to be a criterion of liver failure stage. According to the ICF recommendations, decrease of points on 0–4% (54–56 points) corresponds with absence of liver failure; 5–24% (43–53 points) — as mild dysfunction, 25–49% (29–42 points) — as moderate; 50-95% (3–28 points) — as severe; and 96–100% (0-2 points) — as absolute failure. Conclusions: score system of evaluation of liver failure stage can be applied at any step of diagnostics and treatment of children of any age, due to independence of the used markers from the age. It can be used in assessment of the severity of disorder in dynamics, in determination of the prognosis and as criterion of indications to liver transplantation, as well as during medico-social examination.

Nutrition plays a key role in the growth and development of children. A present day balanced parenteral nutrition can adequately ensure the child’s organism with amino acids, carbohydrates, fats, and energy needed to maintain a basic energy level, physical activity, height, pre-correction of nutritional deficiency. The child’s body needs sufficient amounts of electrolytes, minerals, microelements and vitamins. Protein and energy homeostasis is the basis of the organism life, which determines the inflammatory response, the adequacy of the immune status, duration and severity of the disease. The paper presents recent data concerning the conduct of parenteral nutrition in children of different age groups.