Accumulation of Postoperative Unexpected Events Assessed by the Comprehensive Complication Index® as Prognostic Outcome Parameters for Kasai Procedure.

Introduction The 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 complications and morbidity in surgical patients. We aimed to analyze the CCI® for the first time in BA infants and to correlate its association with outcomes. Material and Methods We conducted a retrospective review of medical records of infants with type III BA undergoing the 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, CCI®, and total event numbers per patient were correlated with one- and two-year outcomes post-surgery. Results A total of 131 events were identified in 101 patients (ranging 0−11 per patient). Forty-four Grade I (33.6%), 67 Grade II (51.1%), 18 Grade III (13.7%), and two sentinel events [>Grade IV] (1.5%) were documented according to Clavien−Dindo, including one death in a cardiac-associated BA patient. None of the complications significantly correlated with a poor outcome. Sixty-three (62.4%) CCI® scores were calculated (range 0−100). The mean CCI® score during the in-patient treatment post-surgery was significantly higher in patients with a poorer outcome than 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 were associated with a poorer outcome. Therefore, the CCI® is an excellent instrument for the postoperative morbidity assessment of BA patients.

The Role of Laparoscopic Treatment of Choledochal Malformation in Europe: A Single-Center Experience and Review of the Literature.

BACKGROUND: Numerous studies from Asian countries, including large collectives, have reported excellent results after laparoscopic resection of choledochal malformation (CM). However, the role of laparoscopic CM resection is still controversial outside Asia. We aimed to analyze the outcome of laparoscopic CM resection in our institution and to compare our outcome with the data reported in the literature. METHODS: All patients who underwent laparoscopic CM resection in our pediatric surgical department from 2002 to 2019 were retrospectively analyzed for surgical details and postoperative complications, which were graded according to the Clavien-Dindo classification. A systematic literature search identified all reports on over 10 cases of laparoscopic pediatric CM resection and surgical details, follow-up, and complication rates were extracted. RESULTS: Fifty-seven patients (72% female) with a mean age of 3.6 + 4.1 years underwent laparoscopic CM resection in our department. Conversion rate was 30%. Total complication rate was 28%. The rate of major complications (Clavien-Dindo grade III or more) was 16% and included stricture of the biliodigestive or enteric anastomosis (n = 4), adhesive ileus (n = 3), portal vein thrombosis (n = 1), and recurrent cholangitis with consecutive liver transplantation (n = 1). With increasing experience, complication rates decreased. The majority of publications on laparoscopic CM resections originated from Asia (n = 36) and reported on low complication rates. In contrast, publications originating from non-Asian countries (n = 5) reported on higher complications following laparoscopic CM resection. CONCLUSION: Our data indicate that laparoscopic CM resection can be safely performed. The learning curve in combination with the low incidence calls for a centralization of patients who undergo laparoscopic CM resection. There seems to be a discrepancy on complications rates reported from Asian and non-Asian countries following laparoscopic CM resection.

Kasai Procedure in Patients Older Than 90 Days: Worth a Cut.

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.

Province-Wide Stool Color Card Screening for Biliary Atresia in Lower-Saxony: Experiences with Passive Distribution Strategies and Results.

BACKGROUND: Stool color card (SCC) screenings for biliary atresia (BA) have shown to improve Kasai timing and outcome significantly. Both obligatory and non-obligatory screenings with passive distribution strategies have proven to be effective. Therefore, we have initiated a voluntary SCC program and aim to describe our experience. METHODS: Since 2017 we supply all maternity wards in Lower-Saxony with SCC. Attending pediatricians and parents of BA infants were contacted via questionnaires and asked for their evaluation of the SCC screening. RESULTS: 85.2% of attending pediatricians support the SCC screening, but only 78.1% considered the initiative useful. In their clinical routine, only 67% of visiting parents report to have received an SCC at the maternity hospital. In the group of parents of BA infants, only 54% (7/13) had received an SCC. Out of those seven parents, only one had referred their child to a children's hospital based on pathological SCC results. The lack of SCC education in the maternity hospitals was made responsible by parents. Within three years, only one infant with BA was identified through the SCC. CONCLUSIONS: Our voluntary SCC screening shows serious limitations with inacceptable distribution of SCCs and low acceptance of attending pediatricians. SCC programs in decentralized health care systems without educational campaigns, standardized diagnostic and treatment algorithms and the definition of reference centers are additional burdens for local health care providers without the promised benefit.

Need for transition medicine in pediatric surgery - health related quality of life in adolescents and young adults with congenital malformations.

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.

Ethical Publication Standards in Articles Reporting on Novel Surgical Methods: Analysis of Three Pediatric Surgical Journals.

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.

The Intestinal Flora at Kasai Procedure in Children with Biliary Atresia Appears Not to Affect Postoperative Cholangitis.

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.

Choledochal Cysts Resected during Childhood Show No Mutations of KRAS and BRAF as Early Markers of Malignancy in Cholangiocytes.

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.

Minimally Invasive Pediatric Surgery: The Learning Curve.

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.

Gravitational Autoreposition for Staged Closure of Omphaloceles.

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.