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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 transoral endoscopic surgery performed through the natural orifices of the human body can reduce the rate of complications associated with surgical procedures and general anesthesia.The aim of the present study was to reveal potentials in restoring the duodenal patency using NOTES technologies.Material and methods. The researchers assessed outcomes of surgical treatment of 4 patients with partial congenital duodenal obstruction who were operated on in two medical institutions of the Siberian Federal District: Ivano-Matreninsky Children’s Clinical Hospital in Irkutsk 2 patients and Maternity and Childhood Center in Krasnoyarsk 2 patients. The trial period lasted over the past 5 years, since January 2015. Two endoluminal techniques for restoring the duodenal patency were used balloon dilatation of stenosis and endoscopic membranotomy. In the final part of the study, demographic data, intraand postoperative parameters and treatment outcomes were analyzed.Results. All endoscopic procedures were successfully completed without conversion to laparotomy. In average, it took 25 minutes to restore the lumen of the duodenum (range 20-30 minutes). One patient showed moderate bleeding from the destroyed membrane after balloon dilatation which was stopped by electrocoagulation. Patients’ follow-up revealed that in one of them the disease returned one month later after the balloon dilatation. Repeated balloon dilation relieved the patient of the disease symptoms.Conclusion. Endoluminal techniques for treating congenital partial duodenal obstruction are feasible and can be successfully performed in most patients. Our experience has shown that this surgery can be done by both balloon dilatation and by endoscopic membranotomy.

Etiological treatment of esophageal achalasia are absent. None of the currently available treatment methods can restore normal esophageal peristalsis and the functionality of the lower esophageal sphincter, thereby alleviating dysphagia and other symptoms of achalasia. Therapeutic procedures currently used for the effective treatment of achalasia of the esophagus are represented by laparoscopic Heller's myotomy and endoscopic balloon dilatation of the esophagus. According to the literature, Heller's myotomy is considered as a method of choice in children, since it provides more reliable results in comparison with pneumatic expansion during prolonged observation. Peroral endoscopic myotomy (POEM) is a new endoscopic method for the treatment of esophageal achalasia, first reported by P. Pasricha in experiment, and then H. Inoue performed this operation in adult patients. There are only a few studies on the use of POEM in children and adolescents. Existing studies have demonstrated the feasibility of this procedure in children, low level of complications and excellent results with short-term observation. Several studies have reported high efficacy in the use of POEM in children, ranging from 90% to 100%. Reports of the occurrence of complications after POEM in children are sporadic and are represented by the formation of subcutaneous emphysema, carboperitoneum, retroperitoneum and mediastinal emphysema. Obviously, detailed studies with longer observation are required before the role of POEM in the treatment of esophageal achalasia in children can be established. If preliminary results are confirmed, POEM can be a real alternative to Heller's myotomy in pediatric patients. This study is intended to explore the potential and safety of the use of POEM for the treatment of achalasia in children based on a review of existing data in the scientific literature.

Introduction. This study describes authors’ experience in performing laparoscopic pyeloplasty in infants whose age does not exceed 3 months of life, and compares pre- and postoperative results of these surgeries in order to answer the question - does the laparoscopic technology for the treatment of hydronephrosis in newborns and infants provide acceptable results based on improving kidney morphology and function? Material and methods. 105 children, aged 3 months, were enrolled into this retrospective study who for 8 years, since 2012, have been laparoscopically operated on for congenital hydronephrosis. Because of bilateral renal damage, a total of 110 surgeries were performed, including simultaneous pyeloplasty in 5 patients. All patients had dismembered pyeloplasty with the Anderson-Hynes technique via transparietal laparoscopic access. Indications for surgery were: decreased renal function with obstruction signs of urine outflow from the pelvis confirmed by radioisotope renography; a combination of reduction of parenchyma thickness and increase of pelvis diameter at serial ultrasound studies; or a combination of these pathologies with urinary tract infection. During the trial, the following parameters were recorded: demographic data, findings of perioperative diagnostic examinations, surgical details, recovery process after surgery and long-term consequences. Results. The average age of patients was 50.24 days. Of 105 patients, 60 (57.14%) were less than 1 month old, and the remaining 45 (42.86%) were 1-3 months old. Gender distribution in groups was as follows: m / f = 73:32. Unilateral operations on the left kidney were in 61.82% patients, on the right kidney - in 33.64% patients. 4.54% patients had bilateral pyeloplasty. Average duration of surgery was 73.07 minutes (40-120 minutes). The average length of hospital stay was 4.19 days (2-9 days). At the early postoperative period, 4 (3.6%) patients developed postoperative complications - urinoma formation. A comparison of pre- and postoperative renal morphometry findings by ultrasound and Doppler ultrasound examination showed a significant decrease of renal pelvis dimensions and improved renal blood flow. The pelvis size decreased in average from 23.5 to 5.5 mm (p = 0.001), and RI from 0.72 to 0.64 (p = 0.001). Functioning of the operated kidney, as showed by the radioisotope renography, improved from 34 to 45.27 (p = 0.001). Long-term follow-up observation revealed one relapse of the disease (0.9%) - stenosis of pyeloureteral anastomosis which required a repeated laparoscopic pyeloplasty. One patient (0.9%)/ who was under follow-up observation for 36 months, had deterioration and loss of kidney function without signs of renal obstruction at the level of pyeloureteral segment which required laparoscopic nephrectomy. Thus, taking into account one relapse and loss of kidney function in one patient, we can state that the effectiveness of laparoscopic pyeloplasty in our study was 98.2%. Conclusion. Summarizing results of the applied laparoscopic pyeloplasty in the youngest group of patients whose age does not exceed 3 months of life, it can be stated that the minimally invasive approach contributes to normalizing morphological and functional parameters of kidney.

To analyze 20-year experience in surgical treatment of cardiac achalasia.A retrospective analysis included 35 children (20 boys and 15 girls) who underwent surgical treatment of cardiac achalasia at the Filatov Children's Clinical Hospital No. 13 for the period 2001-2020.Heller laparoscopic cardiomyotomy with Douro fundoplication was followed by favorable outcomes in 33 (94.3%) children. We also observed significant decrease of postoperative Eckardt score compared to baseline values.Methods of cardiodilation (balloon dilatation, bougienage) ensure a short-term effect and may be only used in addition to surgical treatment. Heller laparoscopic cardiomyotomy with Douro fundoplication results favorable outcome in more than 92% of patients and can be considered as a standard for the treatment of this disease in children. Oral endoscopic myotomy cannot be considered as an alternative to Heller laparoscopic cardiomyotomy with Douro fundoplication due to high risk of secondary gastroesophageal reflux and its complications. Therefore, additional fundoplication is required that makes treatment of cardiac achalasia more invasive and expensive compared to Heller laparoscopic cardiomyotomy with Douro fundoplication.Ахалазия кардии (АК) — это идиопатическое нейромышечное заболевание, характеризуемое функциональным нарушением проходимости кардиального отдела пищевода и, как следствие, расширением вышележащих его отделов. Среди существующего многообразия хирургического лечения АК наибольшую популярность получила кардиомиотомия по Геллеру (ЛКМГ) в сочетании с фундопликацией по Дору (ФД), которую в последние годы успешно выполняют минимально инвазивным лапароскопическим способом. Однако общепринятых рекомендаций по лечению АК у детей пока нет. Представляем 20-летний опыт клиники детской хирургии РНИМУ им. Н.И. Пирогова по лечению АК.Проведен ретроспективный анализ результатов лечения АК у 35 детей (мальчиков 20, девочек 15) получивших лечение в ДГКБ №13 им. Н.Ф. Филатова за период с 2001 по 2020 г.В общей сложности после однократной ЛКМГ + ФД получен хороший результат у 33 (94,3%) детей. Отмечено также отчетливое уменьшение суммы балов после операции по шкале Eckardt по сравнению с исходной.Методы кардиодилатации (балонная диалатация, бужирование) дают краткосрочный эффект и могут быть использованы только в дополнение к оперативному лечению; ЛКМГ + ФД позволяет получить хороший результат более чем в 92% случаев и может быть рассмотрена как стандарт лечения у детей; пероральная эндоскопическая миотомия не может рассматриваться как альтернатива ЛКМГ + ФД из-за высокого риска возникновения вторичного гастроэзофагельного рефлюкса и его осложнений, что требует дополнительного проведения гастрофундопликации и делает лечение АК более инвазивным и затратным, чем ЛКМГ + ФД.

Right-sided (right, in dextral position) aortic arch (RAA) is recorded in approximately 5% of esophageal atresia (EA) patients. This vascular abnormality may complicate the surgical treatment of EA and is still an unsolved problem in pediatric surgery. Conventional methods for determining the anatomy of the aortic arch do not always provide accurate data, which can lead to thoracotomy on the side of the aortic arch. Skills and preferences of the surgeon still determine the choice of surgical approach in EA patients with RAA. For most experienced surgeons, to make anastomosis of the esophagus in patients with RAA from right access is not so difficult. However, in EA patients with RAA, combined with the vascular ring, left-sided thoracotomy can make the final combined reconstruction technically simpler. In this scientific review, we sought to determine the prevalence rate of RAA in the population of EA patients, the level of pre-operative evaluation of this vascular anomaly, and the existing surgical strategies that consist in choosing the optimal side for access to the esophagus.

To summarize data of a multiple-center study of the treatment of duodenal atresia in 3 children's hospitals of the Siberian Federal District.We analyzed postoperative outcomes in 211 patients with duodenal atresia. All patients underwent surgery at various hospitals of the Siberian Federal District: Ivano-Matreninsky Children's Clinical Hospital in Irkutsk (expert hospital) - 120 patients; Center for Maternal and Child Welfare in Krasnoyarsk (learning hospital No. 1) - 51 patients; Kemerovo Regional Children's Clinical Hospital (learning hospital No. 2) - 40 patients. The study has been carried out for 15 years (from January 2005 and to December 2019). Patients were divided into 2 cohorts: group I - 88 patients (laparoscopic formation of duodenal anastomosis); group II - 123 patients (surgery via laparotomy). Demographic data, intra- and postoperative parameters and complications were analyzed.Preoperative parameters were similar in both groups. Significant between-group differences were found for surgery time (70 vs. 90 min;Laparoscopic correction of duodenal atresia can be safely performed by experienced endoscopic surgeons from different centers united by the same ideology of endoscopic surgery. Laparoscopy ensures less duration of surgery, faster postoperative recovery, less mortality, incidence of early and late postoperative complications.Представлены обобщенные данные мультицентрового лечения дуоденальной атрезии в 3 детских госпиталях Сибирского федерального округа.Изучены итоги хирургического лечения 211 больных дуоденальной атрезией из различных лечебных учреждений Сибирского федерального округа: Ивано-Матренинская детская клиническая больница Иркутска (экспертный госпиталь) — 120 пациентов; Центр материнства и детства Красноярска (обучающийся госпиталь №1) — 51 пациент; областная детская клиническая больница г. Кемерово (обучающийся госпиталь №2) — 40 пациентов. Исследование проводили на протяжении 15 лет, с января 2005 г. по декабрь 2019 г. Больных распределили на две группы: в 1-й — анастомоз двенадцатиперстной кишки выполнили лапароскопически (Сравниваемые группы имели одинаковые дооперационные параметры. Обнаружены статистически значимые различия в длительности операции между лапароскопическими и открытыми процедурами (70 мин против 90 мин;Лапароскопическая реконструкция дуоденальной атрезии может быть безопасно выполнена опытными эндоскопическими хирургами из разных центров, объединенных одной идеологией выполнения эндохирургических операций. При сопоставлении итогов лечения лапароскопический доступ демонстрирует результаты, превосходящие результаты открытой хирургии, включая сокращение длительности операции, ускоренное послеоперационное восстановление пациентов, снижение летальности, а также частоты ранних и поздних послеоперационных осложнений.