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Choledochal malformation (CM) is a rare disease that can lead to malignancy and potential long-term sequelae despite surgical resection. There is no long-term follow-up data on patients after CM resection in Germany. We aimed to determine the long-term outcome of our patients with a duration of follow-up >10 years and focused on long-term sequelae and health-related quality of life (HRQOL).

Living donor liver transplantation (LDLT) is regularly performed in small-sized infants. Computed tomography (CT)-based donor liver volumetry is used to estimate the graft size. The aim of our study was to assess the results of CT liver volumetry and their impact on the clinical outcome after LDLT in extremely small-sized infants.

Purpose: To assess the number, characteristics, and functional short-, and midterm outcomes of patients with rectal atresia (RA) and stenosis (RS) in the ARM-Net registry. Methods: Patients with RA/RS were retrieved from the ARM-Net registry. Patient characteristics, associated anomalies, surgical approach, and functional bowel outcomes at 1 and 5-year follow-up were assessed. Results: The ARM-Net registry included 2619 patients, of whom 36 (1.3%) had RA/RS. Median age at follow-up was 7.0 years (IQR 2.3–9.0). Twenty-three patients (63.9%, RA n = 13, RS n = 10) had additional anomalies. PSARP was the most performed reconstructive surgery for both RA (n = 9) and RS (n = 6) patients. At 1-year follow-up, 11/24 patients with known data (45.8%, RA n = 5, RS n = 6) were constipated, of whom 9 required stool softeners and/or laxatives. At 5-year follow-up, 8/9 patients with known data (88.9%, RA n = 4, RS n = 4) were constipated, all requiring laxatives and/or enema. Conclusion: RA and RS are rare types of ARM, representing 1.3% of patients in the ARM-Net registry. Additional anomalies were present in majority of patients. Different surgical approaches were performed as reconstructive treatment, with constipation occurring in 46% and 89% of the patients at 1 and 5-year follow-up. However, accurate evaluation of long-term functional outcomes remains challenging. [ABSTRACT FROM AUTHOR]

Introduction Minimally invasive surgery (i.e., laparoscopy) and minimally invasive anesthesia (i.e., caudal anesthesia with spontaneous respiration) have separately shown benefits for inguinal hernia repair in infants, yet to what degree these techniques can be combined remains unknown. This study investigated whether laparoscopy impacts the feasibility of performing caudal anesthesia with spontaneous respiration in infants. Methods Prospectively collected data of all infants less than 12 months old and over 3 kg weight who underwent laparoscopic indirect hernia repair (LAP) at our department from 2019 to 2021 were compared with a historical control-matched group of infants who underwent open repair (OPEN) from 2017 to 2021. We assessed the patients' characteristics, anesthesia, and surgical data as well as intra- and postoperative complications. Results A total of 87 infants were included (LAP n = 29, OPEN n = 58). Caudal anesthesia with spontaneous respiration was feasible in 62.1% of cases (LAP n = 55.2%, OPEN n = 65.5%; nonsignificant). Neither group registered anesthetic intra- or postoperative complications. Sedatives were utilized in 97% of LAP patients versus 56.9% of OPEN patients (p < 0.00001). The airway was secured with a laryngeal mask in 89.7% of patients during LAP versus 41.4% during OPEN (p < 0.00001). No significant differences were found regarding the use frequency of opioids (48.3% LAP vs. 34.5% OPEN; nonsignificant) or neuromuscular blockers (6.9% LAP vs. 5.2% OPEN; nonsignificant). Conclusion This is the first comparative study on caudal anesthesia and spontaneous respiration in infants undergoing laparoscopic versus open inguinal hernia surgery. Laparoscopy increased the need for ventilatory support and sedatives but did not significantly impair the feasibility of caudal anesthesia and spontaneous respiration. [ABSTRACT FROM AUTHOR]

Nowadays laparoscopic interventions enable the collection of resident macrophage populations out of the human cavities. We employed this technique to isolate pleural monocytes/macrophages from healthy young adults who underwent a correction of pectus excavatum. High quality CD14+ monocytes/macrophages (plMo/Mφ) were used for RNA‐sequencing (RNA‐seq) in comparison with human monocyte‐derived macrophages (MDM) natural (MDM‐0) or IL‐4‐polarized (MDM‐IL4). Transcriptome analysis revealed 7166 and 7076 differentially expressed genes (DEGs) in plMo/Mφ relative to natural MDM‐0 and polarized MDM‐IL4, respectively. The gene set enrichment analysis, which was used to compare RNA‐seq data from plMo/Mφ with single‐cell (scRNA‐seq) data online from human bronchial lavage macrophages, showed that plMo/Mφs are characterized by a high expression of genes belonging to the metallothionein (MT) family, and that the expression of these genes is significantly higher in plMo/Mφ than in MDM‐0 or MDM‐IL4. Our results provide additional insights on high MTs‐expressing macrophage subsets, which seem to be present not only in bronchial lavage of healthy adults or in pleural exudates of lung cancer patients but also in pleural fluid of healthy young adults. Macrophage subsets expressing high MTs may have specific roles in lung defense, repair, and homeostasis, and require further investigations. [ABSTRACT FROM AUTHOR]

Introduction:  In Germany, pediatric surgery is organized in a decentralized manner. A nationwide clinical registry does not exist. The aim of this research is to analyze the current status of pulmonary surgery in the country in respect of children and adolescents.Materials and Methods:  Claims data have been extracted from two health insurance databases representing 34% of the German population. Operative diagnoses and procedures, as well as patients' characteristics, were recorded from 2016 to 2019.Results:  The incidence of the four index diagnoses investigated matched the current literature in three of four entities. The main indications <8 years were congenital lung malformations (61%) and spontaneous pneumothorax at age 8 to 17 years (77%). Furthermore, 1,668 pediatric pulmonary procedures (0-17 years) were identified (668 pulmonary resections). Two age peaks were identified: 13% of patients were operated on before 1 year old and 50% of patients were between 15 and 17 years old. Video-assisted-thoracoscopic-surgery (VATS) for pulmonary resections was applied in 62% of cases. 96% of pulmonary resections in patients <1 year and 70% of pulmonary resections in children between 1 and 14 years were performed by pediatric surgeons. Of patients between 15 and 17 years old, only 14% were operated on in pediatric surgery. The estimated average pulmonary resection caseload was 3.2 cases per year and per institution for pediatric surgery and 1.5 for adult surgery.Conclusion:  Indications for pulmonary surgery in children arise from rare diagnoses. In total, 66% of lung resections are performed in patients <1 year and >14 years. The majority of lung resections are performed as VATS. Patients ≤14 years are predominantly operated on by pediatric surgeons. The accessibility of pediatric pulmonary surgery is acceptable, but there is a low caseload per center. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction:  Despite its wide acceptance, the superiority of laparoscopic versus open pediatric surgery has remained controversial. There is still a call for well-founded evidence. We reviewed the literature on studies published in the last three decades and dealing with advantages and disadvantages of laparoscopy compared to open surgery.Materials and Methods:  Studies comparing laparoscopic versus open abdominal procedures in children were searched in PubMed/MEDLINE. Reports on upper and lower gastrointestinal as hepatobiliary surgery and on surgery of pancreas and spleen were included. Advantages and disadvantages of laparoscopic surgery were analyzed for different types of procedures. Complications were categorized using the Clavien-Dindo classification.Results:  A total of 239 studies dealing with 19 types of procedures and outcomes in 929,157 patients were analyzed. We identified 26 randomized controlled trials (10.8%) and 213 comparative studies (89.2%). The most frequently reported advantage of laparoscopy was shorter hospital stay in 60.4% of studies. Longer operative time was the most frequently reported disadvantage of laparoscopy in 52.7% of studies. Clavien-Dindo grade I to III complications (mild-moderate) were less frequently identified in laparoscopic compared to open procedures (80.3% of studies). Grade-IV complications (severe) were less frequently reported after laparoscopic versus open appendectomy for perforated appendicitis and more frequently after laparoscopic Kasai's portoenterostomy. We identified a decreased frequency of reporting on advantages after laparoscopy and increased reporting on disadvantages for all surgery types over the decades.Conclusion:  Laparoscopic compared with open pediatric surgery seems to be beneficial in most types of procedures. The number of randomized controlled trials (RCTs) remains limited. However, the number of reports on disadvantages increased during the past decades. [ABSTRACT FROM AUTHOR]

Introduction:  Human peritoneal macrophages are resident in the abdominal cavity where they support the specific microenvironmental regulation. We have previously observed a phenotypic switch of murine macrophages during infancy that was associated with a functional development. To investigate the age related changes in human peritoneal macrophages, we analyzed peritoneal macrophages of children undergoing laparoscopic procedures.Materials and Methods:  Immunologically healthy children who received minimally invasive surgery in our department were included in this study. In all cases, the written consent was obtained. At the beginning of laparoscopy, physiologic NaCl-solution was instilled and manually removed through the umbilical trocar to gain macrophages. Lavage cells were processed for flow cytometry analysis. CD14+ myeloid cells were monitored for specific lineage marker expression.Results:  A total of 21 donors (age: 7 days-18 years) were included and divided into three groups. In all age groups, 97% of myeloid cells expressed CD11b. 70% of these expressed CD14. Three subsets of CD14 cells were detected on the basis of CD14/CD16 expression (CD14 + CD16dim, CD14 + CD16inter, and CD14 + CD16high). In neonates, >80% belonged to the CD14 + CD16high subset, reducing to 30% in adolescents. In none of the cases, the M2 markers CD23 and CD25 were expressed.Conclusion:  This is the first study showing that lineage marker expression of peritoneal macrophages in neonates differs from that in adults. The knowledge about neonatal tissue resident macrophages might help to understand their complex interaction and to use specific macrophage properties for therapeutic approaches. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction:  The pros and cons of video-assisted thoracoscopic versus conventional thoracic surgery in infants and children are still under debate. We assessed reported advantages and disadvantages of video-assisted thoracoscopy in pediatric surgical procedures, as well as the evidence level of the available data.Materials and Methods:  A systematic literature search was performed to identify manuscripts comparing video-assisted thoracoscopic and the respective conventional thoracic approach in classic operative indications of pediatric surgery. Outcome parameters were analyzed and graded for level of evidence (according to the Oxford Centre of Evidence-Based Medicine).Results:  A total of 48 comparative studies reporting on 12,709 patients, 11 meta-analyses, and one pilot randomized controlled trial including 20 patients were identified. More than 15 different types of advantages for video-assisted thoracoscopic surgery were described, mostly with a level of evidence 3b or 3a. Most frequently video-assisted thoracoscopic surgery was associated with shorter hospital stay, shorter postoperative ventilation, and shorter time to chest drain removal. Mortality rate and severe complications did not differ between thoracoscopic and conventional thoracic pediatric surgery, except for congenital diaphragmatic hernia repair with a lower mortality and higher recurrence rate after thoracoscopic repair. The most frequently reported disadvantage for video-assisted thoracoscopic surgery was longer operative time.Conclusion:  The available data point toward improved recovery in pediatric video-assisted thoracoscopic surgery despite longer operative times. Further randomized controlled trials are needed to justify the widespread use of video assisted thoracoscopy in pediatric surgery. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction:  In the last three decades, minimally invasive surgery (MIS) has been widely used in pediatric surgery. Meta-analyses (MAs) showed that studies comparing minimally invasive with the corresponding open operations are available only for selected procedures. We evaluated all available MAs comparing MIS with the corresponding open procedure in pediatric surgery.Materials and Methods:  A literature search was performed on all MAs listed on PubMed. All analyses published in English, comparing pediatric minimally invasive with the corresponding open procedures, were included. End points were advantages and disadvantages of MIS. Results of 43 manuscripts were included. MAs evaluating the minimally invasive with the corresponding open procedures were available for 11 visceral, 4 urologic, and 3 thoracoscopic types of procedures. Studies included 34 randomized controlled trials. In 77% of MAs, at least one advantage of MIS was identified. The most common advantages of MIS were a shorter hospital stay in 20, a shorter time to feeding in 11, and a lower complication rate in 7 MAs. In 53% of MAs, at least one disadvantage of MIS was found. The most common disadvantages were longer operation duration in 16, a higher recurrence rate of diaphragmatic hernia in 4, and gastroesophageal reflux in 2 MAs. A lower native liver survival rate after laparoscopic Kasai-portoenterostomy was reported in one MA.Conclusion:  In the available MAs, the advantages of MIS seem to outnumber the disadvantages. However, for some types of procedures, MIS may have considerable disadvantages. More randomized controlled trials are required to confirm the advantage of MIS for most procedures. [ABSTRACT FROM AUTHOR]

Background:  Esophageal atresia (EA) is a rare disease requiring surgical repair, usually within the first days of life. Patients with EA require intensive postoperative care and often have comorbidities. There is a lack of data on the costs incurred by patients with EA during the first year of life., Methods:  Anonymized claims data were provided by the Techniker Krankenkasse (∼10.8 million clients). Data were extracted for patients who had an inpatient diagnosis of EA (International Classification of Diseases [ICD]: Q39.0 or Q39.1) and a reconstruction of the esophageal passage in case of atresia (Operationen-und Prozedurenschlüssel [German version of ICPM, International Classification of Procedures in Medicine; OPS] 5-428.0 to 5-428.7, 5-316.1 or 5-431.0) during their first hospital stay. All patients were in their first year of life at initial hospitalization (2016-2020) and were followed up for 1 year. Costs, length of hospital stay, and duration of mechanical ventilation and differentiated OPS services were analyzed using descriptive statistics. Multiple linear regression was used to analyze the determinants of hospital costs., Results:  A total of 119 patients with EA were included (55.5% male). The mean cost of the 1-year observation period was €89,736 ± 97,419 (range €12,755-640,154). The increasing costs of the initial hospitalization led to a disproportionate increase in the costs of the 1-year observation period. The presence of an associated malformation combined with surgical complications was associated with almost five-fold higher costs than in patients without an associated malformation and an uncomplicated course (€193,103 ± 157,507 vs. €39,846 ± 33,473). The mean duration of mechanical ventilation was 23.2 ± 43.1 days and the mean length of hospital stay was 80.3 ± 77.2 days., Conclusion:  To our knowledge, this is the first study to investigate the costs of EA patients in the first year of life. The presence of an associated malformation combined with surgical complications was associated with almost five-fold higher costs than in patients without an associated malformation and an uncomplicated course., Competing Interests: None declared., (Thieme. All rights reserved.)

Background/purpose:  Gastroesophageal reflux disease (GERD) after lung transplantation (LuTx) can lead to chronic lung allograft dysfunction. Our aim was to assess the prevalence of GERD in pediatric LuTx recipients and to investigate the impact of medical and surgical GERD treatment on lung function., Methods:  Ethical approval was obtained. Data of all consecutive patients who underwent LuTx from 2013 to 2023 and aged < 18 years at the time of the study were prospectively collected. A GERD diagnostic algorithm was established, including assessment of symptoms (vomiting, heartburn, regurgitation, cough, recurrent airway infections), bronchoscopy, forced expiratory volume in 1 second, and pH impedance. Further investigations included upper gastrointestinal series and esophagogastroduodenoscopy. Patients with GERD underwent medical treatment or fundoplication. Lung function was monitored., Results:  Thirty-six patients (2 months-18 years, 50% male) were included. Twenty-nine (80%) underwent spirometry, 16 (45%) pH impedance study, and 14 (39%) esophagogastroduodenoscopy. Twenty-two (61%) had no GERD symptoms and 12 (33%) showed normal pH impedance study or esophagogastroduodenoscopy. Fourteen (39%) patients had GERD symptoms, all 9 tested symptomatic patients (25%) had pathological GERD-specific diagnostics. Three (8%) patients underwent fundoplication after a median of 1.6 years (range 1.1-5.7 years) post-LuTx without surgical complications. After a median follow-up of 2.3 years (range 1.3-2.8 years) post-fundoplication, all ( n  = 3) had complete remission of GERD symptoms and lung function improvement. Lung function decline was observed in 6 (67%) of the tested symptomatic patients on proton-pump inhibitors (PPIs) treatment., Conclusion:  Over one-third of our patients presented with GERD symptoms after LuTx. Symptoms and lung function may be reliable GERD indicators. Given the high prevalence of GERD, we suggest a routine posttransplant diagnostic algorithm including pH impedance study. Eighty percent of all symptomatic patients had a lung function decline despite PPI. Fundoplication is safe and may improve long-term outcome in pediatric LuTx recipients., Competing Interests: None declared., (Thieme. All rights reserved.)