The use of minimally invasive surgery (MIS) in pediatric patients has increased over the past decades. The process of mastering a new procedure is termed the learning curve, during which the ability to operate increases but poorer outcomes are produced. We aim to analyze the current evidence on learning curves in pediatric MIS and evaluate its impact on patient's clinical outcomes. A systematic literature search was performed for studies listed on PubMed that reported on the learning curve for MIS surgical procedures. Studies were included if they stated the number of procedures required to reach a consistency in outcomes or if they compared outcomes between early and late period of MIS experience regarding the endpoints operative time, conversions, and intra-/postoperative complications. A total of 22 articles reporting on 11 surgical procedures were included in the study. Most authors reported a significant decrease in operative time as well as peri- and postoperative complications with increasing experience of the surgeon. Complications ranged from minor to major, the latter being especially severe for patients receiving pyloromyotomy (5–7% higher risk of mucosal perforation), esophageal atresia repair (15% higher leakage rate and 19–77% higher stenosis rate), or Kasai portoenterostomy (26–35% more liver transplants in the first year after surgery) during the learning curve period. Pediatric MIS comes with a considerable learning curve that may have a significant impact on the patient's clinical outcomes. Efforts should be made to minimize the effect of the learning curve on the patients.

Introduction Management strategies for large omphaloceles remain controversial. In this study, we discuss the use of GRAVITAS (gravitational autoreposition sutures), the method used at our institution when successful primary closure is deemed questionable. Patient's primary clinical course and long-term outcomes were analyzed. Materials and Methods This is a single-center retrospective analysis of all consecutive patients with omphaloceles treated between 1997 and 2018. Decision for GRAVITAS was made when the defect was estimated too large for primary closure. Traction sutures were placed in the fascia surrounding the defect and then suspended from the top of the incubator to allow gravitational autoreposition of the herniated organs. Ventilation and muscle relaxation were maintained until secondary closure, which was performed after the obtruding viscera had been reduced by repeated adjustment of the suture's tension. Data are presented as mean ± standard deviation. Results Out of 49 patients with omphaloceles, 12 were treated with GRAVITAS, 33 underwent primary closure, and 4 were treated using Schuster's technique. Mean time to secondary closure after GRAVITAS was 7 ± 10 days. In nine of the patients who had isolated omphalocele, secondary closure was achieved after 4 ± 2 days. Ventilation time was 5 ± 2 days, and time to full feeds was 18 ± 16 days. In three patients (one with Fallot's tetralogy, one with Cantrell's pentalogy, and one with lung hypoplasia), abdominal closure was achieved after 17 ± 15 days. Due to cardiorespiratory comorbidity, ventilation time was >30 days. Five patients received initial closure of the skin and secondary fascial closure after 18 ± 15 months. One patient with prior fascial closure underwent later repair of an abdominal wall hernia. During follow-up (30 ± 35 months), one patient with gastrointestinal obstruction due to adhesions required laparotomy, and one patient with gastroesophageal reflux disease underwent fundoplication. Conclusion GRAVITAS is a feasible method for staged closure of large omphaloceles when successful primary closure is deemed questionable.

Introduction According to the Declaration of Helsinki, medical research and new therapeutic interventions involving human subjects require prior informed consent and ethical approval. In 2010, 46% of pediatric surgical publications lacked documentation of ethical approval and 84% lacked documentation of informed parental consent with lowest rates of ethical adherence found in articles concerning novel methods. The aim of this study was to investigate whether adherence to ethical standards has improved in pediatric surgical publications. Materials and Methods All 3,093 consecutive articles published in Journal of Pediatric Surgery, European Journal of Pediatric Surgery, and Pediatric Surgery International over the last 5 years were systematically reviewed for publications describing novel surgical methods. Novel methods were defined as surgical methods not published before or not considered common practice. The publications were reviewed as to whether ethical approval and informed consent to participate was documented. Results In total, 105 articles describing novel surgical methods were identified (61 Journal of Pediatric Surgery, 16 European Journal of Pediatric Surgery, and 28 Pediatric Surgery International). Authors reported on new operative techniques (62%), modified techniques (31%), or use of new materials (7%). Ethical approval was documented in 52% of the articles with almost half reporting approval for retrospective data analysis only but not the application of the novel method. Informed consent was documented in 21% of publications. Complications were reported in 48% of the studies, including recurrences and reinterventions for the unsuccessful novel methods. Two authors reported mortalities due to underlying disease, one of which failed to report prior ethical approval or informed consent. Conclusion Adherence to ethical publication principles in pediatric surgery has improved over the last years but is still lacking in many publications. When implementing new methods, prior ethical approval and informed consent and their documentation are mandatory, specifically in the light of potential hazard to patients.

Introduction Age at Kasai portoenterostomy (KPE) has been identified as a predictive factor for native-liver survival in patients with biliary atresia (BA). Outcomes of pediatric liver transplantation (LT) have improved over recent years. It has been proposed to consider primary LT as a treatment option for late-presenting BA infants instead of attempting KPE. We present our experience with patients older than 90 days undergoing KPE. Materials and Methods A retrospective chart review of patients with BA undergoing KPE at our institution between January 2010 and December 2020 was performed. Patients 90 days and older at the time of surgery were included. Patients' characteristics, perioperative data, and follow-up results were collected. Eleven patients matched the inclusion criteria. Mean age at KPE was 108 days (range: 90–133 days). Results Postoperative jaundice clearance (bilirubin < 2 mg/dL) at 2-year follow-up was achieved in three patients (27%). Eight patients (73%) received a liver transplant at a mean of 626 days (range: 57–2,109 days) after KPE. Four patients (36%) were transplanted within 12 months post-KPE. Two patients died 237 and 139 days after KPE due to disease-related complications. One patient is still alive with his native liver, currently 10 years old. Conclusion Even when performed at an advanced age, KPE can help prolong native-liver survival in BA patients and offers an important bridge to transplant. In our opinion, it continues to represent a viable primary treatment option for late-presenting infants with BA.

Introduction Evidence supports long-term oral antibiotic prophylaxis to prevent cholangitis after Kasai procedure. Data regarding perioperative intravenous prophylaxis are lacking. Ascending pathogens from the intestine are made responsible for recurrent cholangitis. Therefore, we analyzed the flora in the upper jejunum during the Kasai procedure and their potential impact on postoperative cholangitis. Materials and Methods In 26 patients, swabs were taken at the bowel prepared for the Roux-en-Y-loop. Our postoperative protocol includes intravenous third-generation cephalosporins for 2 weeks and rectal steroids starting at day 4. Cholangitis was defined as the postoperative reappearance of acholic stools or increase of serum bilirubin in combination with fevers or increase of inflammatory parameters. In this scenario, Tazocin was administered for another 2 weeks. Results Swabs remained sterile in nine patients (34.6%). In 17 patients (65.4%), gram-positive and gram-negative pathogens were identified; all belonging to physiological intestinal flora. A total of 96.2% pathogens were covered by the antibiotic prophylaxis. The cholangitis incidence was 55.6% in the sterile cohort, and 23.5% in the gram-positive and gram-negative cohort (p = 0.06). In the cholangitis cohort, no significant differences were detected for the age at Kasai and the pre- and postoperative total bilirubin. Conclusion We found that our antibiotic regiment covered bacteria in the upper gastrointestinal (GI) tract in the majority of our patients at the time of Kasai. Nonetheless, a significant proportion of patients developed signs of cholangitis. There was no higher rate of cholangitis in patients with resistant bacteria. Thus, our data do not support the hypothesis of extended postoperative intravenous antibiotics to prevent ascending cholangitis.

Survival rates of patients with visceral congenital malformations have increased considerably. However, long-term morbidity in these patients is high. In the last decades, these circumstances have led to a shift in goals of caretakers and researchers with a new focus on patients’ perspectives and long-term morbidity. Health-related quality of life (HrQoL) is the most commonly used patient-reported outcome measure to assess the impact of chronic symptoms on patients’ everyday lives. Most pediatric surgical conditions can cause a significantly decreased HrQoL in affected patients compared to the healthy population. In order to guarantee life-long care and to minimize the impact on HrQoL a regular interdisciplinary follow-up is obligatory. The period of transition from child-centered to adult-oriented medicine represents a critical phase in the long-term care of these complex patients. This scoping review aims to summarize relevant pediatric surgical conditions focusing on long-term-morbidity and HrQoL assessment in order to demonstrate the necessity for a well-structured and standardized transition for pediatric surgical patients.

Introduction In patients with choledochal cysts (CDC), a hyperplasia-dysplasia-carcinoma sequence can lead to biliary tract malignancy. The limited data available suggest that the risk decreases considerably after excision in childhood. We analyzed samples of resected CDC from pediatric patients histologically and performed mutational analysis of the proto-oncogenes KRAS and BRAF as early markers of malignant alteration in cholangiocytes. Materials and Methods After institutional review board approval, patients undergoing resection for CDC in our center from 2011 to 2019 were retrospectively identified. Histopathological reports were searched for inflammation and endothelial alteration. Cases with sufficient tissue specimen were tested for KRAS codon 12/13 and BRAF codon 600 mutations by pyrosequencing. Results In total, 42 patients underwent resection for choledochal cyst in the study period. Median age at surgery was 2.4 years (range = 18 days–18 years). Histopathological analysis showed no malignancy, but various degrees of inflammation or fibrosis in approximately 50% of the patients and in all age groups. Sufficient tissue for mutation analysis was available for 22 cases, all of which tested negative for KRAS or BRAF mutation. Conclusion In our series, chronic inflammatory changes were frequently present in CDC of infants and children. However, the lack of KRAS and BRAF mutations suggests that no malignant changes have been initiated in this group of European patients undergoing early resection.

Introduction Kasai procedure in children with biliary atresia (BA) is associated with several complications in the short-term. The Comprehensive Complication Index (CCI) is a validated metric in adult surgery for the analysis of complication and morbidity in surgical patients. We aimed to analyze the CCI for the first time in BA infants and to corelate its association with the outcome. Material and Methods Retrospective review of medical records of infants with type III BA undergoing Kasai procedure between January 2011 and December 2021 at our institution. All unexpected events were ranked according to the Clavien-Dindo classification and the CCI per patient was subsequently calculated. Clavien-Dindo grades, individual events, the CCI and the total event number per patient were correlated with the one- and two-year outcome post Kasai. Results 131 events were identified in 101 patients (range 0 &ndash; 11 per patient). Forty-four Grade I (33.6%), 67 Grade II (51.1%), 18 Grade III (13.7%) and two sentinel events [&gt; Grade IV] (1.5%) were documented according to Clavien-Dindo, including one death in a cardiac-associated BA patient. None of the complications correlated significantly with a poor outcome. Sixty-three (62.4%) CCI were calculated (range 0 &ndash; 100). The mean CCI during the in-patient treatment post Kasai, was significantly higher in patients with a poorer outcome compared to patients with native liver survival at one- and two-year follow-up (22.7 21.7 vs 13.2 18.1; p=0.02). Conclusion Not the severity of complications, but the accumulation of numerous events related to Kasai procedure are associated with a poorer outcome. Therefore, the CCI is an excellent instrument for the postoperative morbidity assessment of BA patients.