BackgroundDespite improvements in the treatment of esophageal atresia with tracheoesophageal fistula (TEF) in recent decades, complications still exist. The rate of fistula recanalization after surgical repair is ~5% in large cohorts. However, there is controversy regarding the gold standard of treatment. This research aimed to evaluate the efficacy of treatment of pediatric patients with isolated (H-type) and recurrent TEF in our clinic.MethodsWe retrospectively analyzed 12 patients (7 boys, 5 girls) aged 35 days–14.6 years. The median age of our patients was 632 days [95% CI (confident interval) 120.1 to 2118.7]; the mean birth weight was 2713 g (95% CI 2258 to 3169; median: 2763 g); and the mean gestational age was 37.1 weeks (95% CI 35.4 to 38.8; median: 37 weeks). All patients were managed for isolated or recurrent TEF between January 1, 2015 and December 31, 2020 using endoscopy (laser de-epithelialization).ResultsLaser de-epithelialization alone was effective in 8 of 12 patients (66.67%), with a mean number of de-epithelializations of 2.25 (range: 1–4). After one attempt at de-epithelialization, success was achieved in only two patients (n=16.67%). The mortality rate was 0%. The median follow-up for patients who received endoscopic treatment exclusively (n=8) was 3.7 years (95% CI 1.38 to 4.87) after the last stage of de-epithelialization.ConclusionFlexible endoscopy is an alternative treatment to open surgical repair of isolated and recurrent TEF in children. The effectiveness of endoscopic laser de-epithelialization alone with subsequent fistula obliteration was 66.7%, with a median follow-up of 3.7 years.

BACKGROUND Esophageal atresia (EA) is the most common congenital anomaly of the gastrointestinal tract. Gastroesophageal reflux disease (GERD) is a frequent and lifelong problem in these patients. GERD can be asymptomatic and the incidence of esophageal gastric and intestinal metaplasia (Barrett’s esophagus) is increased in adults with EA compared with the general population. Timely and accurate diagnosis of GERD is important to reduce long-term problems and this may be achieved by pH-impedance testing. AIM To assess symptoms and pH-impedance data in children after EA, in order to identify their specific features of GERD. METHODS This study was conducted from November 2017 to February 2020 and involved 37 children who had undergone EA via open surgical repair (51.35% boys, 48.65% girls; age range: 1-14 years, median: 4.99 years). GERD diagnosis was made based on multichannel intraluminal impedance/pH study and two groups were established: EA without GERD, n = 17; EA with GERD, n = 20. A control group was established with 66 children with proven GERD (68.18% boys, 31.82% girls; median age: 7.21 years), composed of a nonerosive reflux disease (referred to as NERD) group ( n = 41) and a reflux esophagitis group ( n = 25). Upper gastrointestinal endoscopy with a mucosal esophageal biopsy was performed on all patients. RESULTS The most frequently observed symptom in EA patients with GERD and without GERD was cough (70% and 76.5% respectively). The number of patients with positive symptom association probability in the EA groups was significantly larger in the EA without GERD group ( P = 0.03). In the control reflux esophagitis group, prevalence of gastrointestinal symptoms was significantly higher than in the NERD group ( P = 0.017). For both EA groups, there was strong correlation with index of proximal events (IPE) and total proximal events (EA with GERD: 0.96, P < 0.001; EA without GERD: 0.97, P < 0.001) but level of IPE was significantly lower than in GERD patients without any surgical treatment ( P < 0.001). Data on distal mean nocturnal baseline impedance were significantly different between the EA with GERD group ( P < 0.001) and the two control groups but not between EA without GERD and the two control groups. CONCLUSION Mean nocturnal baseline impedance may have diagnostic value for GERD in EA children after open surgical repair. IPE might be an additional parameter of pH-impedance monitoring.

BACKGROUND Blue rubber bleb nevus syndrome (BRBNS) is a rare vascular disease, difficult to diagnose and choose a treatment method, especially in young children. There are several limiting factors to the use of enteroscopy for diagnostics and treatment in pediatric patients, in general. The literature on BRBNS cases is limited and presents various therapeutic approaches. CASE SUMMARY We present here a case of BRBNS involving a 4-year-old female, whose intestinal venous lesions were successfully treated by endoscopic sclerotherapy and aethoxysklerol foam. Skin lesions, typical for BRBNS, appeared on the 8 th d of the child’s life and their number increased over the next several months. The child also experienced episodes of critical decrease in hemoglobin level (by as much as 52 g/L) for several years, requiring iron supplementation and several blood transfusions. Video capsule endoscopy revealed numerous vascular formations in the small bowel. The combined findings of gastrointestinal venous formations and skin lesions prompted BRBNS diagnosis. Single-balloon enteroscopy was used to perform sclerotherapy, with aethoxysklerol foam. A positive effect was observed within 19 mo of follow-up. We continue to monitor the patient’s hemoglobin level, every 2 wk, and it has remained satisfactory (> 120 g/L). CONCLUSION Endoscopic sclerotherapy can be effective in the clinical management of gastrointestinal manifestations of BRBNS in young children.

The aim is to evaluate clinical data and data of intraesophageal 24-hour pH/impedance measurement in pediatric patients with corrected esophageal atresia. Material and methods. A retrospective analysis was carried out on the basis of inpatient records of 43 pediatric patients with corrected esophageal atresia (CEA) aged from 1 to 14 years, who were examined at the State Institution «Republican Scientific and Practical Center of Pediatric Surgery» from November 2017 to March 2020. Average age of the group: 5.09±1.2 years, of which 23 boys (53.5%), 20 girls (46.5%). All patients with CEA underwent esophagogastroduodenoscopy (EGD) and intraesophageal 24-hour pH/impedance measurement while off acid-suppressive therapy. Results. Depending on the results of pH/impedance measurement, the patients with CEA were divided into two groups: CEA with gastroesophageal reflux disease (GERD) – 20 (54.1%) and CEA without GERD – 17 (45.9%). The most common symptom in patients with CEA was cough in both groups, in 26 (70.27%) children. Only in 4 out of 37 patients with CEA no symptoms during the time of the study were registered. Upper gastrointestinal tract endoscopy showed that 16 (43.2%) children with CEA had grade A esophagitis (according to the Los Angeles classification), 1 (2.7%) had gastric metaplasia of the esophageal mucosa, and 6 (16.2%) had chronic gastritis. EGD data did not reveal any significant differences between CEA patients with GERD and CEA patients without GERD (P=0.819). When comparing pH/impedance parameters, a significant difference (P<0.005) was obtained for the following parameters: reflux index, number of reflux episodes, distal mean nocturnal baseline impedance (MNBI) and duration of the longest reflux episode. In addition, the positive association of symptoms with episodes of refluxes (>95%) in the group of CEA patients without GERD was significantly higher than in CEA patients with GERD: 3/20 (15%) versus 8/17 (47.06%). Conclusions. The prevalence of GERD in patients with CEA in this study was 54.06%. Extraesophageal symptoms (in particular, cough) are prevalent in patients with CEA (70.3%). GERD can be asymptomatic in patients with CEA. In this study, 15% of CEA patients with confirmed GERD were asymptomatic. Patients with CEA in the study groups rarely had typical GERD symptoms (heartburn, regurgitation, chest pain and belching). The research was carried out in accordance with the principles of the Declaration of Helsinki. The research protocol was approved by the Local Ethics Committee of the institutions indicated in the article. Informed consent of parents and children was obtained for the research. The authors declare no conflicts of interest. Key words: corrected esophageal atresia, gastroesophageal reflux disease, esophagitis, children, esophageal 24-hour pH/impedance measurement.

Purpose. To determine the diagnostic significance of blood citrulline level when estimating the short bowel syndrome (SBS) in children under 5 years old. To determine the blood citrulline level in children of different age. Methods. The examined group (N=31) included children under 5 who underwent small or small and large intestine resection and developed (15 children) or not developed (16 children) clinical signs of SBS. The control group included children who underwent no surgery on the GIT and had no congenital malformations and clinical signs of enteropathy (N=42). To determine the level of citrulline, overpressured high performance liquid column chromatography was used. Statistical analysis of the results was done with MedCalc® version 18.11.3. Results. Mean level of blood citrulline is 28.9 µmol/l in the examined group (95% CI 23.2–34.6) and 30.5 µmol/l in the control group (95% CI 25.0–35.1). Reliable data of the ratio between blood level citrulline and age of the child were obtained both among operated and non-operated children. The odds ratio of determining citrulline in blood was below 24.4 µmol/l in development of SBS clinical picture and amounted to 4.08 р < 0,05. Additional use of ROC analysis confirms the presence of interrelation between citrulline level and absence or presence of SBS clinical signs with 100% specificity and 43% sensitivity. The area under curve (categorical variable showing whether SBS is present or lacking) is 0,746, р = 0,005. Conclusion. Determination of serum citrulline as a diagnostic marker of short bowel syndrome in children under 5 found low sensitivity but high specificity. Thus, blood citrulline index can be used as a biomarker to diagnose SBS, to describe the course of SBS in detail and determine the risk of severe forms of this pathology in children under 5.