The short bowel syndrome (SBS, or simply short gut) is the most common cause for the chronic intestinal failure (CIF). The purpose of the study was to present the results of treatment of infants at risk for the development of post-resection SBS and the formation of CIF. Research materials and methods: 22 children threatened by the formation of post-resection SBS were treated in the Surgical Department of Newborns and Infants of the National Medical Research Center for Children’s Health (Moscow, Russia) from 2017 to 2021. Results: All patients have undergone the surgical treatment. The scope of the surgical intervention included adhesiolysis with the imposition of an interintestinal anastomosis. Simultaneous imposition of multiple interintestinal anastomoses (more than 4) was performed in 7 patients (32%). As a result, the CIF formation was avoided in 8 patients (36%) by maintaining the total length of the remaining small intestine. The follow-up period ranged from 12 to 46 months. Currently, 2 patients (9%) receive partial parenteral nutrition, and in 17 patients (77%) it was possible to achieve completely autonomous enteral nutrition. A lethal outcome was stated in 3 patients (14%) due to the development of a catheter-associated infection. Conclusion: in the course of this work, the principles of surgical treatment of patients threatened by the development of post-resection SBS and the formation of CIF were developed. Adequate parenteral and enteral nutrition can optimize intestinal adaptation and improve the nutritional status of the pediatric patient.

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.

A unique clinical case of a combination of malformations of the anterior abdominal wall (omphalocele of large size) and malformation of the lymphatic system (congenital chyloperitoneum) is presented. Each of these defects can lead to a fatal outcome, and the combination of them has greatly increased the risk of developing an unfavourable outcome. The use of immunosuppressive therapy with Sirolimus used for the first time during the newborn period made it possible to completely stop the chyloperitoneum. This clinical example shows that in the treatment of surgical patients with multiple congenital malformations, a multidisciplinary approach and observation is necessary for timely response to the patient’s condition.

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.

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.

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.