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Introduction:  The number of patients with necrotizing pneumonia has increased in recent years. The aim of this study is to review the incidence, management, and outcome of pediatric necrotizing pneumonia requiring surgical therapy and to prove that lung resection results in favorable development of patients. We hypothesize that overall lung function in children after lung resection does not differ from that of the healthy population., Materials and Methods:  A retrospective tertiary referral center study with a prospective follow-up spirometric study of patients with necrotizing pneumonia managed between January 2010 and December 2019 was performed., Results:  The study cohort consisted of 1,295 patients admitted to the pediatric department for community-acquired pneumonia; 47 patients developed necrotizing pneumonia, 36 of whom underwent parenchymal lung resection. A 5-year rise in the occurrence of necrotizing pneumonia requiring resection was 77%, with a significant increase in the last 3 years ( p  < 0.05). The median age at the time of surgery was 32.5 (interquartile range [IQR]: 32.25) months. Streptococcus pneumoniae was the most prevalent pathogen (83%), although 53.3% of these patients were vaccinated against the agent. In 67% of patients, preresection procedures were performed: drainage of pneumothorax (17%), drainage of empyema (46%), drainage of empyema with use of alteplase (25%), and thoracoscopic decortication (12%). Surgical procedures included lobectomy (72.2%), wedge resection (13.9%), bilobectomy (8.3%), and pneumonectomy (5.6%). The postoperative complication was bronchopleural fistula in three patients. There were two (5.5%) postoperative deaths due to multiple organ failure. The follow-up spirometry was performed 43.3 (median, IQR 23.8-66.7) months after surgical intervention. Normal lung function was detected in 35 (64.8%) patients, restrictive pattern in 6 (11.1%) patients, obstructive pattern in 11 (20.4%) patients, and combined in 2 (3.7%) patients., Conclusion:  The number of patients with necrotizing pneumonia requiring resection has increased significantly in the last 3 years ( p  < 0.05). Aggressive surgical treatment results in significant clinical improvement in most cases and favorable lung function outcome. Long-term follow-up showed normal spirometry in 64.8% of cases., Competing Interests: None declared., (Thieme. All rights reserved.)

Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is the most common congenital malformation of the upper digestive tract. This study represents the first genome-wide association study (GWAS) to identify risk loci for EA/TEF. We used a European case-control sample comprising 764 EA/TEF patients and 5,778 controls and observed genome-wide significant associations at three loci. On chromosome 10q21 within the gene CTNNA3 (p = 2.11 × 10 -8 ; odds ratio [OR] = 3.94; 95% confidence interval [CI], 3.10-5.00), on chromosome 16q24 next to the FOX gene cluster (p = 2.25 × 10 -10 ; OR = 1.47; 95% CI, 1.38-1.55) and on chromosome 17q12 next to the gene HNF1B (p = 3.35 × 10 -16 ; OR = 1.75; 95% CI, 1.64-1.87). We next carried out an esophageal/tracheal transcriptome profiling in rat embryos at four selected embryonic time points. Based on these data and on already published data, the implicated genes at all three GWAS loci are promising candidates for EA/TEF development. We also analyzed the genetic EA/TEF architecture beyond the single marker level, which revealed an estimated single-nucleotide polymorphism (SNP)-based heritability of around 37% ± 14% standard deviation. In addition, we examined the polygenicity of EA/TEF and found that EA/TEF is less polygenic than other complex genetic diseases. In conclusion, the results of our study contribute to a better understanding on the underlying genetic architecture of ET/TEF with the identification of three risk loci and candidate genes., Competing Interests: The co-author C.M.B. declares the following interests: Shire (grant recipient, Scientific Advisory Board member), Idorsia (consultant), Lundbeckfonden (grant recipient), Pearson (author, royalty recipient), and Equip Health Inc. (Clinical Advisory Board). All other co-authors declare no competing interests., (© 2022 The Authors.)

Background/purpose: The available literature on congenital isolated H-type tracheoesophageal fistula (TEF) is limited, and preferred approach varies among centers (cervicotomy, thoracotomy, thoracoscopy). We aimed to present one of the biggest case series of thoracoscopic approach for congenital isolated TEF and to assess the method's feasibility and outcomes., Methods: Retrospective review of thoracoscopic TEF repair experiences at 2 European university pediatric surgery centers., Results: 9 boys and 3 girls were involved in the study (age 5 days-4 years), 8 of them were newborns (mean birth weight 3013 g, mean gestational age 39 weeks). The most common presenting symptoms were desaturations on feeding in neonates and recurrent respiratory tract infections in older children. The diagnoses were established on contrast study and confirmed with rigid bronchoscopy. The fistulas were located at or below the thoracic inlet; the fistulas were 2 cm above the carina to half the height of the trachea. All patients underwent successful thoracoscopic TEF repair. There were no conversions. The postoperative course was uneventful in all but one who had rethoracoscopy for prolonged postoperative chylothorax. All patients had satisfactory vocal cord function. One patient required reoperation for fistula recurrence 8 months after primary surgery., Conclusion: Thoracoscopic approach for isolated H-type TEF seems to be possible as a procedure of choice with satisfactory results and all benefits of minimally invasive procedure., Level of Evidence: IV (case series)., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Over a twenty-year period, we performed 255 ERCP procedures in infants aged up to 1 year. ERCP was indicated in cholestatic infants with suspicion of biliary obstruction. The most common diagnosis was biliary atresia (48%), choledochal cysts (13%), and choledocholithiasis (4%). The procedure complication rate was 13.7%. Hyperamylasemia occurred in 12.9%. More severe complications were rare-0.8% of ERCP procedure. There were no cases of postprocedural pancreatitis or death. Our study has proved that ERCP is a safe and reliable method in this age group. Its high specificity and negative predictive value for extrahepatic biliary atresia can prevent unnecessary surgeries in patients with normal bile ducts or endoscopically treatable pathologies., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2021 Jan Stovicek et al.)

Introduction: Sinus pilonidalis (SP) is an acquired inflammatory disease, which is relatively common in the paediatric population. Surgery is indicated in symptomatic patients. In 2017, minimally invasive pilonidal sinus treatment (EPSiT) was adapted to the paediatric population., Aim: To evaluate the first experience with minimally invasive endoscopic treatment of SP (PEPSiT) in children and adolescents in the Czech Republic., Material and Methods: A retrospective review of all consecutive paediatric patients who underwent PEPSiT from November 2018 to February 2020. The monitored parameters were demographics, perioperative course of the disease, surgery, length of hospitalisation, postoperative complications, healing, disease recurrence, and follow-up., Results: Seventeen patients were enrolled in the study. The median age at surgery was 17.1 years (range: 12.5-18). The subjects comprised 76% males, and the median body mass index was 25.6 kg/m 2 (range: 17-30.3 kg/m 2 ). Thirteen patients underwent previous surgical treatment (76%) under local anaesthesia. The median duration of PEPSiT was 50 min (range: 30-85 min). The subjective evaluation of pain by patients on the VAS scale was 0 on the day of discharge. There were no postoperative complications up to the 30 th postoperative day. Two disease recurrences were successfully managed by re-PEPSiT. By the end of follow-up, 14/15 patients had healed. Two patients are still within 3 months of surgery, which is too soon to definitively evaluate possible recurrence of the disease., Conclusions: These preliminary results show that PEPSiT is a highly promising method. It is safe and well-tolerated by patients (short hospital stay, quick return to normal life, low pain and analgesic consumption). Two recurrences of disease were treated by re-PEPSiT., Competing Interests: The authors declare no conflict of interest., (Copyright: © 2020 Fundacja Videochirurgii.)

Purpose: Analysis of surgical management and survival of pediatric patients with gastric tumors treated at our institution., Methods: A retrospective study of patients with primary gastric tumors treated between 1993 and 2018 was conducted., Results: Eight patients, five girls and three boys, were diagnosed with gastric tumors at an average age of 10.4 years (1 day-15.4 years). Surgical management included Billroth type I procedure in five and tumor excision in three patients. Histology revealed gastrointestinal stromal tumor (GIST) in four patients and one of each of schwannoma, myofibroblastic tumor, hamartoma and teratoma. Microscopically clear margins were reported in six patients. Repeated local recurrence occurred in three patients (2 × GIST, 1 × myofibroblastic tumors) who consequently underwent three, four and six reoperations. One of these patients had liver metastases, which were managed with ligation of the hepatic arteries. This patient was also diagnosed with a lung hamartoma, which was treated with a lobectomy. Survival rate was 100% with a median follow-up of 8.6 years (7 months-25.5 years)., Conclusions: Gastric tumors are rare in children and represent a management challenge. Repeated recurrence of GISTs and myofibroblastic tumors remains frequent even after complete resection and may necessitate multiple surgeries, therefore patients require a lifelong follow-up.

Background: Appendicitis is one of the most common diagnoses in pediatric populations. Although new recommendations for the treatment of pediatric appendicitis were published, management varies among different institutions., Objectives: To determine current practices in 4 (n = 4) representative pediatric surgical departments in Central Europe., Material and Methods: One department from each of the 4 countries was surveyed using an online questionnaire. Questions focused on preoperative, operative and postoperative practices in 2018, particularly those related to antibiotic (ATB) therapy and laparoscopy., Results: A total of 519 appendectomies were performed, among which 413 (79.6%) were laparoscopic appendectomies (LAs), with a conversion rate of 5.1%. Appendectomy, as an elective procedure, was performed in 43 (8.3%) patients. One-quarter (129 patients) had complex appendicitis and 72.3% of these were operated laparoscopically. In 3 departments, ATB prophylaxis was administered, based on the decisions of the operating surgeon. One department used standard ATB prophylaxis (metronidazole). Whenever phlegmonous appendicitis was detected, ATB were administered therapeutically in 2 departments. Two other departments administered ATB based on surgeon decision. The choice of ATB was not standardized. If complex appendicitis was detected, all sites administered ATB therapeutically. The type of ATB treatment was standardized in complex cases in 2 departments. Thirty-four complications (6.6%) at surgical sites were recorded - 4.1% (16/390) after uncomplicated and 14% (18/129) after complex appendicitis. Thirty-two occurred after acute surgeries and 26 of these followed laparoscopic procedures. Postoperatively, intra-abdominal abscesses occurred in 3.5% of laparoscopic and in 2.9% of open appendectomy (OA) cases., Conclusions: This questionnaire study showed that treatment outcomes for appendicitis in children in Central Europe are comparable with data reported in the literature. Laparoscopic appendectomy is the predominant surgical method, but there is a little consensus for ATB treatment in the management of appendicitis at our 4 pediatric surgical departments.

Necrotizing enterocolitis (NEC) is a severe gastrointestinal disease affecting mainly preterm newborns. It is characterized by unexpected onset and rapid progression with specific diagnostic signs as pneumatosis intestinalis or gas in the portal vein appearing later in the course of the disease. Therefore, we analyzed diagnostic and prognostic potential of the markers of early NEC pathogenesis, such as excessive inflammatory response (serum amyloid A (SAA)) and gut epithelium damage (intestinal and liver fatty acid-binding protein (I-FABP and L-FABP, respectively) and trefoil factor-3 (TFF-3)). We used ELISA to analyze these biomarkers in the urine of patients with suspected NEC, either spontaneous or surgery-related, or in infants without gut surgery (controls). Next, we compared their levels with the type of the disease (NEC or sepsis) and its severity. Already at the time of NEC suspicion, infants who developed NEC had significantly higher levels of all tested biomarkers than controls and higher levels of I-FABP and L-FABP than those who will later develop sepsis. Infants who will develop surgery-related NEC had higher levels of I-FABP and L-FABP than those who will develop sepsis already during the first 6 hours after the abdominal surgery. I-FABP was able to discriminate between infants who will develop NEC or sepsis and the SAA was able to discriminate between medical and surgical NEC. Moreover, the combination of TFF-3 with I-FABP and SAA could predict pneumatosis intestinalis , and the combination of I-FABP, L-FABP, and SAA could predict gas in the portal vein or long-term hospitalization and low SAA predicts early full enteral feeding. Thus, these biomarkers may be useful not only in the early, noninvasive diagnostics but also in the subsequent NEC management., Competing Interests: The authors declare that they have no conflicts of interests., (Copyright © 2020 Stepan Coufal et al.)

Introduction:  The incidence of Crohn's disease (CD) within the pediatric population is increasing worldwide. Despite a growing number of these patients receiving anti-tumor necrosis factor α therapy (anti-TNF-α), one-third of them still require surgery. There is limited data as to whether anti-TNF-α influences postoperative complications. We evaluated postoperative complications in patients who were or were not exposed to anti-TNF-α therapy in our institutional cohort., Materials and Methods:  A retrospective review of CD patients who underwent abdominal surgery between September 2013 and September 2018 was performed. The patients were divided into two groups based on whether they were treated with anti-TNF-α within 90 days before surgery. Thirty-day postoperative complications were assessed using Clavien-Dindo classification (D-C); this examination included surgical site infections (SSIs), stoma complications, intra-abdominal septic complications, non-SSIs, bleeding, ileus, readmission rate, and return to the operating room. Mann-Whitney U -test, Fisher's exact test, and multivariate logistic regression analyses were used for statistical analysis., Results:  Sixty-five patients (41 males) with a median age of 16 years (range: 7-19) at the time of operation were identified. The most common surgery was ileocecal resection in 49 (75%) patients. Forty-three (66.2%) patients were treated with anti-TNF-α preoperatively. Seven patients (11%) experienced postoperative complications. There was no statistically significant difference in postoperative complication in patients who did or did not receive anti-TNF-α before surgery (D-C minor 2.3% vs. 4.6%, p  = 1; D-C major 7% vs. 9.1%, p  = 1)., Conclusion:  The use of anti-TNF-α in pediatric CD patients within the 90 days prior to their abdominal surgery was not associated with an increased risk of 30-day postoperative complications., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Aim of Study: Duodenum-preserving resection of the pancreatic head (DPRPH) with Roux-en-Y pancreatojejunostomy is a procedure used to remove focal pathological lesions of the pancreatic head. Although predominantly used in adult patients, it is both safe and effective in children. The aim of this study was to review our experience with this procedure, with focus on its indications, complications and long-term outcomes., Methods: A retrospective analysis of pediatric patients who underwent DPRPH between 1994 and 2015 was performed. Patient files were reviewed for demographic, diagnostic, operative and histological details, postoperative complications. Patients were contacted telephonically and sent questionnaires to determine long-term outcomes., Results: The study cohort consists of 21 patients, 14 girls and 7 boys, with an average age of 11.72 years (range 3 months to 18.6 years), who underwent DPRPH with end-to-end anastomosis of the jejunum to the pancreatic body (Roux-en-Y anastomosis). In four cases the head and also part of the body of the pancreas was resected. In the remaining 17 cases, only the head of the pancreas was resected. Indications for DPRPH were solid pseudopapillary tumor of the pancreas (n = 10), trauma (n = 8), pancreas divisum (n = 1), focal congenital hyperinsulinism (n = 1) and pancreatic cyst (n = 1). The length of follow-up ranged from 1 to 22 years (average 9.66). One patient developed a biliary fistula, which closed spontaneously within 2 weeks after stent insertion. A recurrence of abdominal pain was reported in two patients, occurring at 7 months after the operation in one patient and at 1 year in the other. Pancreatic endocrine insufficiency did not occur in any of the 21 patients. Seven patients currently require a low fat diet, five of which need pancreatic enzyme supplementation. An additional two patients need enzyme supplementation without dietary restriction., Conclusion: DPRPH is a safe and effective procedure for the treatment of large focal pathological lesions of the pancreatic head in children. As a less invasive procedure than pancreatoduodenectomy, it is more appropriate for the developing child.

Background/purpose: We analyzed the capacity of urinary Intestinal fatty acid-binding protein (I-FABP) to quantify the degree of mucosal injury in neonates with gastroschisis (GS) and to predict the speed of their clinical recovery after surgery., Methods: In this prospective study, we collected urine during the first 48h after surgery from neonates operated between 2012 and 2015 for GS. Neonates with surgery that did not include gut mucosa served as controls for simple GS and neonates with surgery for intestinal atresia served as control for complex GS patients. The I-FABP levels were analyzed by ELISA., Results: Urinary I-FABP after the surgery is significantly higher in GS newborns than in control group; I-FABP in complex GS is higher than in simple GS. I-FABP can predict subsequent operation for ileus in patients with complex GS. Both ways of abdominal wall closure (i.e. primary closure and stepwise reconstruction) led to similar levels of I-FABP. None of the static I-FABP values was useful for the outcome prediction. The steep decrease in I-FABP after the surgery is associated with faster recovery, but it cannot predict early start of minimal enteral feeding, full enteral feeding or length of hospitalization., Conclusion: Urinary I-FABP reflects the mucosal damage in gastroschisis but it has only a limited predictive value for patients' outcome., Competing Interests: The authors have declared that no competing interests exist.

Introduction:  A retrospective study was performed to evaluate the clinical features, diagnostic methods, and treatment alternatives in children with pancreas divisum (PD)., Materials and Methods:  Patients who underwent treatment for PD between 1999 and 2014 at our department were evaluated for sex, age, presenting symptoms, physical examination findings, biochemical markers, diagnostic methods, treatment modalities, and results of treatment during follow-up., Results:  Seven patients who underwent treatment of symptomatic PD were included in the study. The median for follow-up period was 8 years (from 26 months to 16 years). Male-to-female ratio was 4:3 and the median age at presentation was 11 years (2-14 years). Presenting symptoms were recurrent episodic epigastric pain. Pancreatitis was documented by elevated amylase or lipase levels. Endoscopic retrograde cholangiopancreatography (ERCP) was the method of diagnosis of PD in all patients. Five patients had complete PD and two had incomplete variants. Three patients improved after ERCP papillotomy. In three patients, papillotomy was unsuccessful but they have only mild episodes of pancreatitis. One patient presented at the age of 4 years with recurrent pancreatitis. She was treated surgically by duodenum-preserving resection of the pancreatic head (DPRPH) because of severe recurrent pancreatitis occurring even after ERCP papillotomy. The patient is 26 months after operation without any reported problems., Conclusion:  Patients with symptomatic PD are indicated for ERCP papillotomy attempt. If there is not improvement after ERCP, then recurrent bouts of severe pancreatitis are considered as an indication for surgical procedure. DPRPH is a safe and feasible surgical alternative., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: To compare anthropometric data (body mass index [BMI]) in patients without lithiasis to patients with symptomatic simple cholelithiasis or choledocholithiasis., Methods: We retrospectively reviewed data from 147 patients undergoing laparoscopic cholecystectomy between 2001-2015. Complete growth data from 98 patients was compared with anthropometric data from the population of the Czech Republic and a control group (BMI of 100 consecutive patients without biliary stones in abdominal ultrasound who were admitted to a surgical department for suspected appendicitis)., Results: The BMI of 75 children with simple cholelithiasis and 23 with choledocholithiasis was compared to the standard Czech pediatric population and to the control group. The median age (simple cholelithiasis and choledocholithiasis) was 16 years, and 35 patients (24%) had a family history of gallstones. Types of lithiasis included multiple (n = 120), solitary (n = 11), and sludge (n = 10). Five cases had polyps and one had gallbladder dysplasia. Patients with simple cholelithiasis had significantly higher BMI compared to the control group without cholelithiasis (p<0.0001) and the standard Czech population (p = 0.03). Patients with choledocholithiasis had a mean BMI significantly higher than that of the general population (p = 0.001) and the control group (p = 0.0001). Patients with choledocholithiasis had significantly higher BMI than those with simple cholelithiasis (p = 0.03)., Conclusion: Patients with cholelithiasis had significantly higher BMI than the general population, and patients with choledocholithiasis had significantly higher BMI than patients with simple lithiasis. Elevated BMI is a risk factor for developing choledocholithiasis. ERCP and early laparoscopic cholecystectomy in patients with choledocholithiasis offer equivalent outcomes in patients with simple cholelithiasis.

Aim: To evaluate long-term quality of life and somatic growth of patients with gastroschisis and compare them with the general population., Methods: We performed a questionnaire survey of the quality of life of our patients treated between 2004-2012., Results: A questionnaire was sent to our 56 patients with gastroschisis, 38 mothers of patients (68%) responded to the questionnaire. 33 of 38 mothers claim that the quality of life of their child is very good, 4 of them responded that it is good. 1 mother confessed that the quality of life was very poor. Anthropometric data show comparable results with the standard population except for patients of 1 year of age who still have lower weight (P<0.001) and body height in the 5th percentile and patients of 3 years of age who are also significantly thinner. 13% of patients in our study group have gastrointestinal problems. 9 patients (24%) attend follow-up at the neurological center (Attention Deficit Hyperactivity Disorder n=6, mental retardation n=1, dysarthria n=2), however, overall intellectual abilities are within normal range. 7 patients underwent surgery for umbilical (n=3) or inguinal hernia (n=4), 2 patients were operated on for undescended testicles, 3 patients were operated on for an adhesive ileus. 92% of mothers are very satisfied with the cosmetic result of the scar., Conclusion: The study has shown that the majority of patients after operation of gastroschisis have a very good quality of life without limitation in comparison with the general population. The presented anthropometric data confirm that the development of patients with gastroschisis is favourable.

Purpose: The aim of the study was to identify which prenatal ultrasonographic findings in fetuses with gastroschisis correlate with complicated postnatal outcome., Methods: Ultrasound findings at the 30th week of pregnancy and medical reports were statistically analyzed to identify independent prenatal ultrasonographic predictors of postnatal outcome., Results: Completed prenatal data were gathered from 64 pregnancies. Prenatal intra-abdominal bowel dilatation (cutoff 10 mm) correlated with the presence of atresia (p < 0.01), longer administration of parenteral nutrition, extended hospital stay (median 53 vs. 21 days; 68 vs. 36 days, both p < 0.05), and greater number of additional surgical procedures (p < 0.05). Infants with antenatal presence of thickened bowel wall (greater than or equal to 3 mm) required longer administration of parenteral nutrition (median 34 vs. 20 days; p < 0.01) and prolonged stay (median 44 vs. 37 days; p < 0.05). Presence of oligohydramnion (amniotic fluid index below 8 cm) was connected with longer administration of parenteral nutrition in newborns (median 30 vs. 16 days; p < 0.05)., Conclusion: The isolated presence of oligohydramnion with amniotic fluid index below 8 cm, thickened bowel wall equal to or more than 3 mm and the prenatal intra-abdominal dilatation with 10 mm cutoff had significant predictive value for the adverse postnatal outcome of patients with gastroschisis.