Синдром Гольденхара представляет собой врожденное заболевание, при котором поражаются структуры, развивающиеся из первой и второй жаберных дуг. У детей с синдромом Гольденхара могут наблюдаться сопутствующие аномалии развития легких. Эти особенности должны быть учтены при подготовке пациентов к общей анестезии. Детям с синдромом Гольденхара необходимо выполнение рентгенографии грудной клетки при планировании оперативных вмешательств в условиях общей анестезии. При обнаружении на рентгенограмме особенностей строения легких желательно выполнение дополнительного обследования (в объеме компьютерной томографии). Особенности строения легких требуют повышенной настороженности анестезиолога относительно трудностей при интубации трахеи и создания возможности проведения фиброоптической интубации.

AIM To improve the results of surgical treatment of children with extended tracheal stenosis. MATERIAL AND METHODS Since 2013 slidingtraсheal plasty under extracorporeal membrane oxygenation was performed in 4 children aged 1 year 2 months - 4.5 years with extended tracheal stenosis in Children's City Clinical Hospital №13. Patients' weight was from 10,5 to 16 kg. Extended tracheal stenosis and complete cartilag inousrings were confirmed in all patients after peoperative survey. Indications for surgery were based on medical history data, the severity of respiratory failure and survey data. Sliding traсheal plasty by different approach esunder extracorporeal membrane oxygenation was applied in all patients. RESULTS There were no intraoperative complications. In the study group 1 patient died in remote postoperative period after discharge due to deterioration of his condition caused by upper respiratory tract infection and acute respiratory failure. In immediate postoperative period 1 patient had pronounced growth of granulation tissue in the area of anastomosis followed laser photocoagulation and prolonged intubation. The average ICU-stay was 24 days, the duration of mechanical ventilation - 11 days. Follow-up carefor patients was conducted on terms of 4-6 and 12 months after surgery. In 2 patients moderate complaints of recurrent bronchitis without signs of respiratory insufficiency during 6-8 months postoperatively were observed. In one child complaints were completely absent.

UNLABELLED:Extracorporeal life support (ECLS) is used in pediatric patients with ARDS in recent 20 years with survival rate from 50 to 67% Venovenous ECLS was used in 1 year 2 months old patient with ARDS and pneumonia developed in postoperative period after gastral esophagoplasty. Purposes of ECLS use were stabilization child's condition and normalization of gas composition of blood with relative lungs repose. Indications for ECLS were increasing respiratory failure, hypoxemia, low respiratory index (PaO2/FiO2 ratio 47.3), alveolar-arterial gradient of oxygen (A-aDO) 630 mmHg and absence of positive effect from high frequency oscillation (HFO).MATERIALS AND METHODS:ECLS was used in 1 year 2 months old patient with ARDS and bilateral pneumonia developed in postoperative period after gastral esophagoplasty. Deltasteam system (Medos Medizintechnik AG, Germany) with centrifugal pump and servoregulation of blood flow pressure was used for ECLS. Double-lumen cannula with size 12 French was used ECLS was instituted via right internal jugular vein.RESULTS:The patient did not have expressed heart failure. Thus preference was given to venovenous ECLS and not to venoarterial ECLS. Duration of ECLS use was 72 hours. Auscultation parameters and gas exchange improved haemodynamics stabilized parameters of biochemical and haematological analysis normalized and the dynamics x-ray examination was positive after the ECLS use. Patient was decannulated and extubated.CONCLUSIONS:Venovenous ECLS was an only way of life support in child with heavy ARDS and pneumonia developed in postoperative period More observations are needed for more thorough analysis and recommendations.

Extracorporeal life support (ECLS) is used in pediatric patients with ARDS in recent 20 years with survival rate from 50 to 67 %. Venovenous ECLS was used in 1 year 2 months old patient with ARDS and pneumonia developed in postoperative period after gastral esophagoplasty. Purposes of ECLS use were stabilization child’s condition and normalization of gas composition of blood with relative lungs repose. Indications for ECLS were increasing respiratory failure, hypoxemia, low respiratory index (p aO/FiO 2 ratio 47.3), alveolar-arterial gradient of oxygen (A-aDO 2) 630 mmHg and absence of positive effect from high frequency oscillation (HFO). Materials and methods: ECLS was used in 1 year 2 months old patient with ARDS and bilateral pneumonia developed in postoperative period after gastral esophagoplasty. Deltasteam system (Medos Medizintechnik AG, Germany) with centrifugal pump and servoregulation of blood flow pressure was used for ECLS. Double-lumen cannula with size12 French was used. ECLS was instituted via right internal jugular vein. Results: The patient did not have expressed heart failure. Thus preference was given to venovenous ECLS and not to venoarterial ECLS. Duration of ECLS use was 72 hours. Auscultation parameters and gas exchange improved, haemodynamics stabilized, parameters of biochemical and haematological analysis normalized and the dynamics x-ray examination was positive after the ECLS use. Patient was decannulated and extubated. Conclusions: Venovenous ECLS was an only way of life support in child with heavy ARDS and pneumonia developed in postoperative period. More observations are needed for more thorough analysis and recommendations.