OBJECTIVE:To improve postoperative outcomes in newborns and infants with choledochal cysts and to determine the indications for surgery.MATERIAL AND METHODS:There were 13 children aged 0-3 months with choledochal cyst who underwent reconstructive surgery between 2019 and 2023. In all children, choledochal cyst was associated with cholestasis. Acholic stool was observed in almost half of the group (n=7). All children underwent cyst resection and Roux-en-Y hepaticoenterostomy.RESULTS:Symptoms of cholestasis regressed in all patients. Mean surgery time was 128±27 min. There were no complications. Enteral feeding was started after 1-2 postoperative days, abdominal drainage was removed after 6.2±1.6 days. Mean length of hospital-stay was 16±3.7 days. Adequate bile outflow is one of the main principles. For this purpose, anastomosis with intact tissues of hepatic duct should be as wide as possible. Roux-en-Y loop should be at least 40-60 cm to prevent postoperative cholangitis.CONCLUSION:Drug-resistant cholestasis syndrome and complicated choledochal cysts (cyst rupture, bile peritonitis) are indications for surgical treatment in newborns and infants. When forming Roux-en-Y hepaticoenterostomy, surgeon should totally excise abnormal tissues of the biliary tract to prevent delayed malignant transformation.

Introduction. Lymphatic malformations (LM) are a congenital pathology of lymphatic vessels that arose during embryogenesis. The relevance of the research topic is due to the rarity, variety of clinical manifestations, and the lack of clinical recommendations for treatment. Aim. To evaluate our experience of surgical and conservative treatment of LM in the surgical department for newborns and infants. Materials and methods. We performed a retrospective analysis of infants with various forms of LM treated at our department from 2017 to 2022. The study protocol was approved by the local ethics committee. The patients’ parents gave written voluntary informed consent to participate in the study. Results. Depending on the form of a LM, the infants underwent conservative therapy, sclerotherapy or surgical resection of the LM. Conclusions. Genetic tests for the spectrum of overgrowth syndromes are necessary for all LM patients to clarify the etiology of the process, prevent recurrence and complications.

Chyloperitoneum and chylothorax are rare conditions with high mortality rates whose optimal treatment strategy remains unclear. The aim of the study was to evaluate the results of chyloperitoneum and chylothorax treatment with a synthetic somatostatin analogue (octreotide) and immunosuppressive therapy with sirolimus. The study was approved by the Independent Ethics Committee and the Scientific Council of the National Medical Research Center for Children’s Health of Ministry of Healthсare of Russia. The patients' parents gave their consent to the use of their children's data, including photographs, for research purposes and in publications. We conducted a retrospective study of nine children diagnosed with congenital chyloperitoneum and chylothorax who had been treated from 2018 to 2022. All the children received either abdominal or pleural drainage, parenteral nutrition, and conservative therapy with drugs. The first line of therapy was octreotide for 14–20 days that was then switched to sirolimus if there had been no effect. The effectiveness of conservative therapy with octreotide at a dose of 5–10 µg/kg/hour was observed in 5 cases. If there had been no effect by day 14, the patients were started on sirolimus at a dose of 0.05–0.2 mg/day which proved to be effective in all the patients (n = 4). Our study showed that sirolimus is effective in complex cases of chyloperitoneum and chylothorax in newborns and infants. Because of the rarity of these disorders, our conclusions were based on the analysis of a small cohort. To confirm our results and develop uniform diagnostic and treatment guidelines, further, more targeted multicenter research is needed. Until such guidelines are adopted, decisions on the treatment of chyloperitoneum and chylothorax should be made on an individual basis and approved by the medical committee of a treatment center.

The esophageal lung is a rare congenital malformation of the foregut, characterized by the existence of the lung tissue, a segment of the lung, and the main bronchus between the esophagus and the part of respiratory system. Because of the rare occurrence of this defect, examples of the treatment of this disease can be found only in foreign literature, in Russian-language sources, there are no described cases of treatment of the esophageal lung. In this regard, the literature data was analyzed and there was presented, clinical picture, classification, diagnosis, and treatment of this defect, as well as a case from practical activities was carried out: diagnosis and treatment of a premature one month baby with an esophageal lung.

Objective of the study: to evaluate the effect of extra-abdominal liver location on the postnatal status of a newborn with omphalocele. Materials and methods of research: a single-center retrospective-prospective cohort continuous non-randomized controlled study was carried out for the period from September 2017 to December 2020. The study involved 110 newborns diagnosed with omphalocele and underwent surgical treatment from August 2007 to April 2019. 2 groups were formed from this cohort of patients: the 1st main group (63 children) – newborns with liver in omphalocele, the 2nd control group (47 children) – newborns without liver in omphalocele. Results: the prevalence of viscero-abdominal disproportion in newborns with omphalocele was 27% (p=0.001) in the 1st group of children, statistically significant compared with the 2nd group. In the 1st group, the duration of stay in the intensive care unit (ICU) significantly exceeded the periods in the 2nd group – 6 (4; 15) versus 3 (0.5; 6) days, p=0.01. In the postoperative period, the development of pulmonary hypertension prevailed in the 1st group – 22% of cases, in the 2nd group – 6% (p=0.032). The average time of transition to full enteral nutrition in both groups was statistically significantly different (11 [8; 19] versus 10.5 [6; 12] days, p=0.012). The cosmeticity of the abdominoplasty results was assessed as good in two groups – 80% and 94%, respectively, p=0.171. Conclusion: when choosing the tactics of surgical treatment, it is necessary to take into account the effect of the extra-abdominal location of the liver in the postoperative period. In the presence of liver in omphalocele, in a greater percentage of cases, a viscero-abdominal imbalance, and a pulmonary hypertension develop, the duration of the postoperative period in the ICU and in the hospital and the period of transition to enteral autonomy increase.

The article presents a modern view on the prognostic factors of spontaneous resolution and effectiveness of treatment of children with vesicoureteral reflux (VUR). The most frequently used predictors of successful treatment of VUR (grades of reflux, distal ureteral diameter ratio to the distance of the lumbar vertebrae, reflux induction phase on mictional cystourthrography), delayed removal of contrast from the upper urinary tract, gender, age, antenatal hydronephrosis). Methodology, statistical significance, advantages, disadvantages of the described prognostic factors are analyzed.

Predicting the outcome of endoscopic correction of vesicoureteral reflux (VUR) is an urgent problem in pediatric urology because the identification of predictors for the effectiveness of endoscopic treatment of VUR will optimize the strategy for examination and treatment of the disease. The purpose of the research was to evaluate the statistical impact of various predictors on the success of endoscopic correction of VUR; to predict the clinical outcome by creating a mathematical model. Materials and methods used: a single-center experimental uncontrolled study was conducted in Sept. 2017 - Feb. 2022. The results of treatment of 150 pediatric patients (240 renal units) aged from 1 month till 7 years old who underwent endoscopic correction of VUR are presented. The following indicators were evaluated as predictors: gender, age of the disease manifestation, bilateral/unilateral VUR, grade of VUR, ureteral diameter ratio (UDR), cystography initial confirmation of VUR, expansion of the pelvicalyceal system and ureter according to ultrasound, the presence of urinary tract infection. Results: according to the data obtained, when comparing the grade of VUR, cystography initial confirmation of VUR, UDR, expansion of the pelvis and ureter depending on the result of treatment, the statistically significant differences have been found (p<0.001). A prognostic model has been developed to determine the probability of the outcome of endoscopic correction of VUR using the binary logistic regression method. The area under the ROC curve was 0.985±0.007 with 95% CI: 0.967-1.000. The resulting regression model was statistically significant (p<0.001). The sensitivity and specificity of the model were 96.4% and 95.3%, respectively. Conclusion: the results of the research show that UDR and the initial confirmation of VUR have greater predictive power for the success of the endoscopic urethroplasty compared to the grade of VUR alone. The use of the predictors and predictive modeling can help improving the selection of candidates for the endoscopic correction of VUR and developing personalized treatment tactics.

In this article, we report three cases of Zinner syndrome (renal agenesis or renal dysplasia, ipsilateral seminal vesicle cyst, and seminal duct obstruction) in children of different age and describe their diagnosis and treatment. All patients have undergone kidney and bladder ultrasonography, renal scintigraphy, and cystoscopy. Two children have also undergone contrast-enhanced computed tomography; one child has undergone magnetic resonance imaging. A 6-month-old patient with a large cyst and clinical manifestations of infravesical obstruction and chronic constipation was operated on. He has undergone diagnostic laparoscopy and cyst removal; seminal duct atresia was confirmed intraoperatively. Key words: Zinner syndrome, renal agenesis, seminal vesicle cyst

Introduction. Malformations of the anterior abdominal wall in newborns is one of the actual problems in neonatal surgery. Antenatal diagnostics of such defects as omphalocele and gastroschisis allows to diagnose and perform necessary surgical interventions in time. Often, outcomes of treatment of newborns with embryonic hernia depend not only on the form of the defect, but also on the accompanying abnomalies. Purpose. To present outcomes of surgical treatment of newborns with anterior abdominal wall malformations. Material and methods. From December 2017 till May 2019, 34 pregnant women with fetal anterior abdominal wall malformations were consulted; 17 out of them with omphalocele and gastroschisis were treated. Results. In December 2017, the surgical department for newborns and infants at the National Medical Research Center for Children’s Health successfully helped children with malformations of the anterior abdominal wall. 16 children were discharged home in a satisfactory condition; there was one unsatisfactory outcome. 11 patients were treated for gastroschisis; 6 patients were treated for omphalocele. 3 patients had repeated surgeries for concomitant pathologies. There were no any adhesive processes and commissural intestinal obstruction. Conclusions. In Russia, there are two groups of newborns with anterior abdominal wall defects: children with isolated defects and children with concomitant pathologies who have an increased risk of unsatisfactory outcomes. The technique developed by us for treating newborns with anterior abdominal wall malformations and concomitant pathologies has very good cosmetic and functional results and reduces the number of postoperative complications and unsatisfactory outcomes.

Congenital short bowel syndrome is a rare condition of the newborn, with several reports demonstrating high mortality. For the first time in Russia, we report a case of treatment of a newborn girl with genetically confirmed congenital short bowel syndrome, and also provide a review of the literature on this syndrome. After birth, the child experienced constant vomiting of bile with a progressive decrease in body weight. The laparotomy for congenital adhesions between the loops of the small intestine with severe violations of the evacuation function revealed that the small bowel was 50 cm in length, confirming the diagnosis of congenital short bowel syndrome. The genetic test, using whole exome sequencing, identified a homozygous mutation in the CLMP gene in this patient. A positive result in the postoperative period was achieved using the protocol for the management of patients with short bowel syndrome. Currently, the girl is 11 months old, body weight is 9 kg, development is harmonious. Long-term survival of children with congenital short bowel syndrome is now possible if enteral feeds are introduced early to promote intestinal adaptation, with subsequent weaning off parenteral nutrition.

Purpose. Presentation of clinical cases of rare combination of omphalocele with pylorostenosis in the postoperative period and additional liver lobe. Materials and methods. In National Medical Research Center for Childrens Health of health surgical ward of newborns and infants for the 2019 us operated 2 children who performed surgery involving intra-operative decision making about further surgical tactics. Results. the results of surgical treatment of newborns with omphalocele combined with hypertrophic pylorostenosis and extra liver lobe are presented. Conclusions. In patients with malformations of the anterior abdominal wall in the postoperative period, when regurgitation syndrome appears, it is necessary to make a differential diagnosis between the functional and organic causes of obstruction. When confirming the organic nature of the obstruction, surgical intervention is indicated. It is necessary to be able to timely and objectively assess the risks and expediency of the approach when choosing surgical tactics in each individual case.

Actuality. To date, there are few publications focused on pneumovesicoscopic ureteral reimplantation in young children. The article is devoted to surgical interventions in this age group. Material and methods. Surgical interventions on small volume bladder have significant technical difficulties. The authors assessed potentials for performing pneumovesicoscopic ureteral reimplantation by the Cohen technique in 14 children under 2 with various obstructive uropathies. Results. In all children, surgical interventions were performed according to preoperative plans. There were no conversions to an open procedure. As it has been found out, patient’s age and bladder volume were not limiting factors for this type of intervention. Conclusion. The pneumovesicoscopic ureteral reimplantation in young children is a hi-tech procedure which requires a unique equipment and a highly qualified surgical team. To reduce the procedure duration, further experience and more information are needed.

In January 2009 to December 2013, the N.F. Filatov Children’s City Clinical Hospital (CCCH) Thirteen gave 357 consultations to pregnant women with fetal urinary tract abnormalities and identified the signs of infravesical obstruction in 27 fetuses of 28–32 weeks of gestation. During the first 24 hours of life, these babies were urgently admitted from maternity hospitals to the Unit for Surgery of Neonates and Premature Babies for further examination and treatment. The diagnosis was verified by miction cystourethrography that showed posterior urethral enlargement. Comprehensive X-ray urological examination and blood biochemical tests were carried out in the unit to evaluate renal function. For additional assessment of the latter, urinary enzymes and β2-microglobulins were examined and biochemical urinalysis was performed from September 2012. All the infants underwent transurethral resection of a posterior urethral valve. Later on, the babies were followed up and treated in an outpatient setting in the Nephrourological Center, N.F. Filatov CCCH Thirteen. The duration of the follow-up was 1 to 4 years. Analysis of the long-term results of endoscopic correction of ureterovesical segment abnormality shows that the use of mini-invasive treatment options in neonates and infants makes it possible to recover urinary tract urodynamics and bladder evacuation function, to reduce the number of infectious complications, and to prevent reflux nephropathy and chronic kidney disease.