Введение. Сохранившаяся клоака — особый вид аноректальных аномалий. Сочетание урологической, генитальной и ректальной аномалий затрудняет выполнение радикальной реконструкции. Материалы и методы. В настоящем исследовании изучены результаты выполнения операций 50 пациентам с персистирующей клоакой, находившихся на лечении с 2010 по 2021 г. Представлено две группы: первая — с коротким каналом (менее 3 см) — 35 детей, вторая — с длинным каналом (более 3 см) — 15 детей. Мы изучили прогноз для кишечного контроля, тип реконструкции, необходимость реконструкции влагалища, осложнения после операции и дни пребывания в стационаре. Результаты. Аномалии мюллеровых протоков во второй группе выше, чем в первой — 36 против 94 % ( р < 0,001). Сакральный индекс и случаи миелодисплазии не различались в группах. Сакральный индекс в первой группе составил 0,62 ± 0,14, во второй — 0,58 ± 0,14 ( p = 0,520). Миелодисплазия в первой группе 33 %, во второй — 38 % ( р = 0,744). В первой группе использовали тотальную урогенитальную мобилизацию (51 %), во второй — абдоминальную реконструкцию (54 %). Реконструкция влагалища была необходима 28 % пациентов первой группы, 60 % — второй. Осложнения в 3,5 раза чаще возникли в первой группе (60 против 17 % во второй) ( р = 0,003). Продолжительность пребывания в медицинском учерждении пациентов второй группы больше, чем у пациентов первой группы. Заключение. Данные нашего исследования демонстрируют, что реконструкция персистирующей клоаки нуждается в индивидуальном планировании операции с учетом длины канала, а также состояния всех структур, формирующих клоаку.

The review of topical issues of diagnostics and treatment of malformation of the anterior abdominal wall - omphalocele in newborns is presented. Currently, there are no specific, recognized criteria acceptable for the maintaining and further managing pregnancy, delivery and treatment of children with omphalocele. Prenatally, the possibility of predicting the tactics of surgical treatment and postoperative management of a newborn with omphalocele is not taken into account. In the choice of tactics of treatment of this category of patients there is a need to consider many factors, namely: the data for the antenatal diagnosis of the defect, duration of respiratory support at the stage of preparation for surgery, age at the time of surgical treatment, the size of the hernia SAC, and the size of the liver in the hernia SAC. These criteria are necessary to determine the possibility of radical surgery for the anterior abdominal wall or stage-by-stage treatment with the creation of a temporary abdominal cavity, with preliminary gradual immersion of the evented organs into the abdominal cavity under the control of the child’s hemodynamic and respiratory parameters, with the dynamic control of the occurrence and progression of pulmonary hypertension under the ultrasound control. To date, a unified approach has not been developed in the surgical treatment and postoperative care of newborns with omphalocele. The use of a variety of methods of surgical correction and their modifications reveals extensive potential for the treatment of different form of exomphalos, but leads to the fact that some of these techniques find their applications in a single universally accepted treatment of this condition. The lack of unified standards of prevention and organization of treatment approaches for newborns with various forms of omphalocele makes this problem very relevant. When preparing a literary review, such databases as PubMed, MedLine, CyberLeninka, and RSCI were used.

Introduction. Surgical treatment of intestinal atresia with distal tracheoesophageal fistula involves resection of fistula and intestinal primary anastomosis. However, premature children often have complications associated with delayed anastomosis. Thus, an optimal surgical approach is not determined. Purpose. Analyzing treatment results in newborns with intestinal atresia and very low weight. Materials and methods. Treatments outcomes in infants with intestinal atresia and very low weight (less than 1500 g) from 2008 to 2017 were assessed retrospectively. The patients were divided into 2 groups: (1) fistula dressing and crossing with subsequent delayed anastomosis reconstruction and (2) primary anastomosis. Demographic, surgical and postoperative complications were compared. Results. 23 preterm children with IA/TEF were operated. Twelve patients (52%) underwent primary anastomosis, whereas 11 (48%) of them had a stepwise surgery. Anastomotic leak confirmed by esophagram was similar in both groups (17% and 18%). Stenosis was more common in step-wise surgery (83%) as compared to the group of primary anastomosis (27%) (р<0.05). The esophagus was preserved in two patients who underwent step-wise surgery. 4 patients had coloesophagoplasty. The postoperative period was similar in both groups. 6 patients (50%) from the step-wise group and 5 patients (45%) from the group of primary anastomosis died. Conclusion. Staging plasty of IA/TEF in newborns does not improve the quality of life. In this group, gastro- and esophagostomy exteriorization is a preferable surgical approach.

The issue of surgical treatment of children with incarcerated inguinal hernias is presented. The evolution of views on the incarceration pathogenesis in the presence of a non-obliterated Nuck’s diverticulum with subsequent ischemia and threatening necrosis of internal organs in the hernia sac is reviewed. Authors who are in favor of early correction of inguinal hernias in young children present their arguments. Techniques of the strategy that allows for preliminary conservative invagination and subsequent delayed surgical reconstruction are presented. Detailed advantages of endosurgical interventions and possible complications in the treatment of children with incarcerated inguinal hernias including the reasons for blood flow reduction in the testes following hernioherniorrhaphy are presented.

Purpose of the study: to estimate the efficacy of treatment and remote results of delayed esophago-esophageal anastomosis in children with EA. Material and methods. We analyzed 18 case histories of children with delayed esophago-esophageal anastomosis performed at N. F. Filatov Children’s Municipal Clinical Hospital No. 13 from 2006 to 2016. Results. Postoperative complications occurred in 16 children out of 18 (88.9%). Esophageal anastomotic leakage was developed in 4 patients (22.2%), anastomotic stenosis in 11 patients (61.1%) and gastroesophageal reflux in 14 children (77.8%). Lethality amounted to 16.7% (3 children) in the early postoperative period. As per the analysis of delayed results, dysphagia was noted in 92.3% of children. After delayed anastomosis 9 children underwent recurrent surgery, 4 of them had subsequent esophageal extirpation. Conclusions. Considering the high rate of complications (88.9%) and bad delayed results, our observations do not allow us to recommend the delayed esophago-esophageal anastomosis as the surgery of choice for children with EA.

Extracorporeal membrane oxygenation (ECMO) was firstly used in 1977 for the treatment of congenital diaphragmatic hernia (CDH) in a neonate. The first time ECMO was administered in our country to a child with CDH on the 10th of January, 2013, at the intensive care department of Municipal Pediatric Hospital No. 13 named after N.F. Filatov. However, the first patient with CDH who had received ECMO, had survived decannullation and had been discharged from the hospital arrived to this hospital only on the 12th of October, 2015. The aim of ECMO was to stabilize the child’s condition and to obtain an opportunity of surgical correction of the congenital malformation. Indications included increasing cardiopulmonary failure, hypoxemia (РаО 2 50, the alveolar-arterial oxygen gradient> 600 for 8 hours), combined acidosis, hypotension, suprasystemic pulmonary hypertension (main pulmonary artery pressure was higher than 100 mm Hg). There were no technical issues or complications during the procedure. Hemostatic therapy under the Activated Clotting Time (ACT) supervision, artificial lung ventilation (ALV), intravenous fluid and transfusion therapy were carried out. Cardiotonic therapy was canceled after the beginning of veno-arterial ECMO due to hemodynamic stabilization. Protective artificial ventilation was applied: VR-20; Pin-18mbar; PEEP-7mbar; FiO 2 -0,21. ACT was kept at 203 seconds in average at the average heparin infusion speed of 4 unit/kg/hour. Acidbase balance indicatiors: рН-7,38; РаО 2 -121,7 mm Hg, РаСО 2 -44,0 mm Hg, ВЕ-3,3, lactate 1,3. Average central venous pressure was 6,6 mm Hg., average invasive blood pressure - 51 mm Hg. After 72 hours of artificial circulatory support ECMO was canceled and surgical intervention was started after gas exchange and hemodynamic stabilization.

Urologic malformations are the most wide spread pathologythat are founded in 5-14% of newborns. Thefrequencyis 6—8 casesin 1000 newborns. Among all prenatally detected defects, the kidneys and urinary system malformations is 26 to 28%.The prenatal consulting center was formed in the Filatov’s Children Hospital. The postnatal management protocol for children with urologic malformations identified prenatally was set up.The results of treatment more than 1500 patients allow recommendingthree-stage management for children with obstructive uropathy. The introduction in the perinatal urology and empowers outpatient medical record technology, allow to obtain good results of treatment and reduce the number of organ removal procedures.