Introduction. The authors present a clinical case of an 8-year-old patient with an extremely rare Allgrove’s syndrome and cardia achalasia. By various authors, in case of this syndrome, achalasia of the cardia is diagnosed in 75% of cases, along with other symptoms. Currently, there are a number of modalities for treating achalasia of the cardia. However, the most popular one is cardiomyotomy by Heller in combination with fundoplication by Dor which recently has been successfully performed with minimally invasive laparoscopic techniques.Clinical case. In present case, the patient had all possible minimally invasive surgical interventions. However, all attempts to apply organ-preserving techniques for treating achalasia of the cardia had failed .Results. Finally, the child had esophageal plastic surgery – coloesophagoplasty with good clinical outcomes.

Introduction. The achalasia cardia care in children has an extremely wide range of issues, many of which are still not completely solved. The variety of treatment options for achalasia cardia indicates the lack of an ideal technique as well as unsolved essence of this problem. Discussions on the choice of optimal treatment modality are still going on, and new minimally invasive techniques are being developed and implemented. Though there is a positive feedback on the results of per oral endoscopic myotomy (POEM) , one can face a number of problems concerning surgical techniques, effectiveness criteria for postoperative monitoring as well as development of secondary gastroesophageal reflux and ways how to combat its manifestations.Material and methods. The following databases were used: National Center for Biotechnology Information (https://www.ncbi.nlm.nih.gov/), scientific electronic library – eLIBRARY (https://elibrary.ru/defaultx.asp).Conclusion. This review is an attempt to systematize current material on existing treatment options for achalasia cardia – from endoscopic to surgical ones. The authors also present results of their comparative analysis on various curative techniques for achalasia of the cardia.

Purpose. A prospective study was carried out. It compared treatment outcomes in children who underwent esophagoplasty with stomach or colon esophagoplasty.Materials and methods. Clinical records of 172 patients who underwent esophagoplasty at N. F. Filatov Children’s Clinical Hospital No. 13 of Moscow from 2009 to 2015 were analyzed. The operated children were divided into two groups. 46 children from group 1 (basic group) aged 2 months to 13 years underwent esophagoplasty with stomach. 126 children aged 2 months to 18 years who had colon esophagoplasty were enrolled in group 2 (control group). In both groups, the majority was presented by children with atresia and corrosive strictures of esophagus. Clinical observation, esophagogastroduodenoscopy, contrast radiography, CT and MRI study, survey of patients were used to estimate direct and remote treatment outcomes.Results. The analyzed groups were comparable for the course of the early postoperative period. The early postoperative period had no complications in 54% of cases in group I and in 54.4% of cases in group II. Recurrent surgeries for complications were more frequent in group I (23.9%) as compared to group II until signifcant indices were reached (10.3%, p = 0.04). In the comparison groups, patients’ quality of life were signifcantly different by the rate of almost any complications in the remote period. They demonstrated better quality of life for children after colon esophagoplasty.Conclusion. Higher quality of life in children after colon esophagoplasty compared to children following esophagoplasty with stomach indicates that this is a surgery of choice in children.

Purpose. To estimate the results of surgical treatment of children with vasorenal hypertension. Materials and methods . 11 patients with renovascular hypertension were operated from 1999 to 2015 with 9 boys and 2 girls among them. Their age varied from 5 months to 14 years old. Their weight was 4.6 kg to 40 kg. All children had high blood pressure values (130/80 to 250/120 mmHg) in spite of antihypertension drug therapy given. Results . 10 patients had plasty of renal arteries affected both unilaterally (6) and bilaterally (4). An internal iliac artery was taken as a transplant in 10 cases whereas the great saphenous iliac vein was used in 2 cases. 1 child had undergone reimplantation of the renal artery. One patient in the group had the middle aortic syndrome (MAS) and pronounced hyperplasia of the renal arteries. Only abdominal aortic repair was performed in this case. In all cases a positive result was noted in the form of normalization of arterial blood pressure values until the complete withdrawal of antihypertensive agents (in 7 cases) or significant dosage reduction (4 patients). Conclusions . Surgery is the basic method in the treatment of children with renovascular hypertension. Using the internal ileac artery as a transplant is accompanied with good early and remote postoperation results. The internal ileac artery and great saphenous vein can be used in a bilateral lesion.

Aim of the study. The massive liver resection in children is executed very seldom. The article analyzes the clinical cases of six patients aged from 3 to 14 years with benign and malignant volume neoplasm of the liver. Material and methods. Diagnosis is laborious enough and requires both, the application of routine methods and highly specialized equipment. All children underwent a typical or atypical liver resection through open access, using modern surgical techniques. Results. The length of hospitalization averaged 39 days. Postoperative complications, like bile duct damage, occurred in two cases. One child had a hematoma at the site of the operation. Conclusion. Extensive liver resections in children refer to comprehensive high-tech surgical interventions, in which the main complication is damaging of the bile duct. The early detection of complications contributes to the favorable course of the postoperative period.

Цель. Проведено проспективное исследование, целью которого являлось сравнение результатов лечения детей, которым была выполнена пластика пищевода желудком или колоэзофагопластика. Материалы и методы. Подвергнуты анализу истории болезней 172 пациентов, которым выполнена пластика пищевода в период с 2009 по 2015 гг. в ДГКБ № 13 им. Н. Ф. Филатова г. Москвы. Оперированные дети были разделены на 2 группы. Группе I (основной) – 46 детям в возрасте от 2 месяцев до 13 лет была выполнена пластика пищевода желудком. Группу II (контрольную) составили 126 детей в возрасте от 2 месяцев до 18 лет, которым была выполнена колоэзофагопластика. В обеих группах преобладали дети с атрезиями и рубцовыми стенозами пищевода. Для оценки непосредственных и отдаленных результатов лечения использованы: клиническое наблюдение, ФЭГДС, контрастная рентгеноскопия, КТ и МРТ исследование и анкетирование пациентов. Результаты. В целом анализируемые группы были сопоставимы по течению раннего послеоперационного периода. Без осложнений ранний послеоперационный период протекал в 54% случаев в I группе и в 54.4% случаев во II группе. Повторные хирургические вмешательства по поводу осложнений отмеченs больше в I группе (23.9%) по сравнению со II группой, до статистически значимых показателей (10.3%, р = 0.04). Качество жизни пациентов в группах сравнения имели статистически значимые различия по частоте практически всех осложнений отдаленного периода, что свидетельствовало о лучшем качестве жизни детей после колоэзофагопластики. Вывод. Более высокое качество жизни у детей после колоэзофагопластики в сравнении с детьми после пластики пищевода желудком позволяет нам считать эту операцию выбора для детей.

AIMTo evaluate complications and long-term results of delayed esophagoesophagostomy in children with esophageal atresia (EA).MATERIAL AND METHODS165 EA children were operated at the Filatov Municipal Children's Hospital #13 for the period 2006-2016. Primary esophageal anastomosis was performed in 136 (82.4%) children with tracheoesophageal fistula. In 5 (3%) neonates with non-fistulous EA esophago- and gastrostomy were made for further coloesophagoplasty. Other 24 (14.5%) children underwent gastrostomy for delayed esophagoesophagostomy. 6 (25%) of them died within 12 days after admission. 18 survivors with gastrostomy subsequently underwent delayed esophagoesophagostomy.RESULTSPostoperative complications occurred in 16 (88.9%) children. Esophageal anastomosis failure occurred in 4 (22.2%) patients, stenosis of anastomosis in 11 (61.1%) children, gastroesophageal reflux in 14 (77.8%) children. Early postoperative mortality was 16.7% (3 children). In remote period 92.3% of children were not adapted to normal diet and only in 7.7% of patients eating behavior corresponds to the age. 11 children underwent prolonged esophageal bougienage. 9 children underwent re-operation after delayed anastomosis. Esophageal extirpation was made in 4 children.CONCLUSIONEsophago- and gastrostomy provides 100% survival if primary esophageal anastomosis is impossible. Herewith, in children without esophagostomy mortality rate was 25%. We still can not confirm that delayed esophageal anastomosis is a good alternative for children with esophageal atresia. In view of our results the number of candidates for delayed esophageal anastomosis should be reduced.

AIM:To present an overall experience of endoscopic operations in children with neurogenic tumors in two medical institutions.MATERIAL AND METHODS:Thoracoscopic excision of tumors was performed in 19 children aged 1 month-7.5 years (mean 1.5 years) in two clinics for the period 2010-2014. In 9 children (47.3%) age did not exceed 1 year. In our study ganglioneuroma was diagnosed in 9 cases, ganglioneuroblastoma--in 2 patients, neuroblastoma stage I--in 8 cases. All patients were under observation and treated according to NB2004 protocol. Tumor's diameter ranged from 1 to 6 cm (mean 4.9±1.9 cm). Mean duration of surgery was 62±22 minutes. There were no any intraoperative complications. Early postoperative period in all patients after endoscopic surgery was more favorable than in those after open operations. Any local recurrences were not observed for the follow-up period.RESULTS:Thoracoscopic operations can become more preferable method in treatment of children with neurogenic tumors if great vessels are not involved into neoplastic process and limited volume of tumor is absent.

The spectrum of pulmonary surgical pathology in newborns and infants is mainly presented by congenital diseases. Surgery is the main radical method of treatment of children with congenital lung diseases. By this time in the N.F.Filatov City Children's Hospital No13 it is performed more than 1000 endoscopic operations on the thoracic cavity including more than 300 lung resections. More than 190 cases were caused by congenital lung disease. The results of treatment of 194 infants with congenital lung disease for the period 2005-2014 are analyzed in this article. 14 (22%) patients were operated urgently. 8 patients (12%) with compensated respiratory failure underwent surgery at the age of 8 to 46 days. Children with absence of clinical manifestations underwent thoracoscopic lung resection at the age of 3 to 8 months. Thoracoscopic lobectomy was performed in the 40 cases (62%). There were no intraoperative complications. Thoracoscopic lung resection for malformations in newborns and infants is currently the method of choice. It allows to visualize pathological focus intraoperatively and to perform precise dissection of pulmonary root structures using modern equipment. Thus, it improves course of postoperative period and reduces duration of hospital-stay. Repeated lung resection in patients with recurrent tuberculosis of operated lung (literature review and own data).

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.

Relapses after laparoscopic fundoplication for the treatment of gastroesophageal reflux in children are a serious challenge to pediatric surgery. Modified Nissen laparoscopic fundoplication developed and implemented in N.F.Filatov Children’s City Hospital ensures a two-fold decrease in the frequency of repeated surgery and increases treatment efficacy up to 92%. We undertook a retrospective study of 345 patients with the statistical analysis of its results. The results of modified surgery and evaluation of statistically significant risk factors of relapses were confirmed by multifactorial logistic regressive analysis. It was shown that the choice of surgery is an independent risk factor of relapses. Other factor influencing the outcome of primary surgery is hiatal hernia. Such patients make up a group at risk of relapse.

The authors' experience of surgical treatment of myasthenia gravis in children was analyzed. 32 patients were operated on, mean age was 13±3 (5-21 years); girls were 26, boys were 6 (19%). Disease severity was IIB-IVB by the MGFA classification, all patients were on anticholinesterase and pednisolone therapy. The operation was indicated by certified neurologists, specialized in myasthenia. All patient were thoracoscopically thymectomized using left- or rightside access. There were no conversions and intraoperative complications. The operation duration was 77±23 min. The postoperative hospital stay was 6.6±3.6 (3-9) days. Excellent long-term results were achieved in 6 (25%) patients, good - in 20 (62.5%) children, no changes were registered in 2 and deterioration of myasthenia in 2 (6.25%) patients. The study proved that thoracoscopic thymectomy obtains the radicalism of open technique, proposing the easier postoperative period and being the method of choice for the surgical treatment of myasthenia gravis in children.