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Swallowing and feeding disorders are a major concern for children with oesophageal atresia (OA) after primary or staged OA repair. Primary OA repair is associated with higher rates of short-term complications in preterm infants with very low birth weight (VLBW) or extreme low birth weight (ELBW). On the other hand, primary repair may have the benefit of early commencement of oral feedings. We hypothesize that also in the medium-term, swallowing-related quality of life is better after primary oesophageal repair. We conducted a prospective cross-sectional study on swallowing quality in a national cohort of former VLBW and ELBW children with OA, using the structured paediatric swallowing quality of life (pedSWAL-QOL) questionnaire. Results were correlated with surgical approach and baseline clinical data. Principal component analysis of pedSWAL-QOL domains was performed. In total, 44 complete data sets of 78 children were available. The mean age of children was 8.5 years (SD = 7.4), and 23 children (52%) had primary OA repair. The overall median pedSWAL-QOL score was 2 (IQR = 0–3), representing a high swallowing-related quality of life, independent of surgical technique (p = 0.086). Children with a history of intracranial haemorrhage (ICH) (p = 0.002) and those with VACTERL association (p = 0.008) had significantly decreased enjoyment with eating. In addition, children with VACTERL association had problems to find suitable foods (p = 0.04). Conclusion: In this national cohort of VLBW and ELBW preterm-born children with OA, swallowing-related quality of life is good, mostly independent of initial surgery. Children with OA and ICH or VACTERL association may require more intense support with feeding. What is Known:• Dysphagia, resembling feeding and swallowing disorders, is common in children and adults with repaired oesophageal atresia. Nevertheless, dysphagia in children with oesophageal atresia decreases with age.• Parents of younger children suffer from increased anxiety and fear regarding eating and swallowing abilities of their children. What is New:• Swallowing-related quality of life in former preterm children with oesophageal atresia is good, independent of initial surgical approach (primary vs. staged repair), even in very low birth weight or extreme low birth weight infants. • Children suffering from VACTERL association or intracranial haemorrhage show decreased enjoyment with eating.

Introduction In pediatric Crohn's disease ileocecal resection is performed reluctantly as postoperative recurrence is frequent. Anti-tumor necrosis factor (TNF) therapy reduces postoperative recurrence rates but increases the risk for infections. Materials and Methods We retrospectively reviewed pediatric Crohn's disease patients who underwent ileocecal resection in our center. We compared disease activity and z-scores for height, weight, and body mass index of patients, who continuously received perioperative anti-TNF therapy (TNF + ), with those who did not (TNF–). Results Of 29 patients (48% females), 13 and 16 were grouped to TNF+ and TNF–, respectively. Patients' characteristics did not differ between groups, except a longer follow-up time in TNF–. We saw significant postoperative improvement but no normalization in z-scores for weight (1.78 vs. 0.77, p Conclusion In patients with localized Crohn's disease an ileocecal resection leads to short-term postoperative improvement of disease activity, body mass index, weight, and growth. For relevant catch-up growth an earlier intervention is necessary. Continuous perioperative anti-TNF therapy had no increased risk of perioperative infections.

Introduction The VACTERL association is a rare malformation complex, showing at least three anomalies of the following organ systems: vertebra, anorectum, heart and vessels, trachea and esophagus, genitourinary tract, and limbs. In addition to a multifactorial event, congenital vascular disorders are also discussed as triggers for the VACTERL association. The aim of this study was to determine whether there is a genetic background for vascular disorders triggering VACTERL association. Materials and Methods We performed a functional analysis on whole exome sequencing data of 21 patients with VACTERL or VACTERL-like phenotype using the online analysis tool “Database for Annotation, Visualization and Integrated Discovery (DAVID) v6.8.” The study was approved by the institutional ethics committee (approval no. 026–13). Written informed consent was obtained from all patients or their parents. Results We identified a total of 86 genetic variants (in 75 genes) classified as damaging (including probably damaging missense, nonsense, and frameshift variants), which are associated to cardiovascular development. Each investigated patient showed at least one damaging variant in genes associated to cardiovascular development. These variants were further reduced by significance in cardiovascular development to 39 genetic variants (in 33 genes). Of note, a pair of siblings, both presenting with cardiac and renal defects, had the same damaging variant in two different genes. Conclusion Our results indicate a genetic background for congenital vascular disorders in patients with VACTERL association. In line with the literature, our data suggest that genetic mutation led to vascular diseases, which in turn may cause malformations similar to the VACTERL association.

Background Foreign bodies lodged in the upper esophagus in children may result in life-threatening complications, especially with button batteries. Rapid removal is essential to prevent complications. Experts report that extraction with a suitable laryngoscope and a forceps is feasible under general anesthesia, but no further data had been available so far. Aims To study foreign body visualization and removal from the upper esophagus in children using a new optimized Miller size 3 blade video laryngoscope. Methods This prospective observational study was performed in three pediatric hospitals. The clinical observations were reported anonymously on an electronic spreadsheet after obtaining the informed consent from the parents or guardians. During the observational period from January 2019 to October 2020, all children with a foreign body lodged into the upper esophagus were eligible for participation and 22 cases were included. Main outcome measures were rates of successful removal and complications as well as duration of the procedure. Secondary outcome was subjective assessment regarding the quality of the visualization and the feasibility of the procedure. Results Success rate was 100% with no complications. Mean intervention and anesthesia times were 5 ± 4 minutes and 26 ± 25 minutes. Quality of visualization of the foreign body was judged as 'excellent' or 'good' in all cases and the feasibility of the procedure as 'without' or 'with little' effort in 95% of all cases. Conclusion The new Miller size 3 video laryngoscope enables rapid, easy, and reliable extraction of foreign bodies when they are located in the upper part of the esophagus. As early removal of esophageal foreign bodies, especially with button batteries, prevents life-threatening complications, we suggest this technique as the first choice of treatment.

Background: Histologically nephroblastomatosis consists of ≥2 microscopic foci of nephrogenic rests. In contrast, clinically apparent nephroblastomatosis consists of single, multiple or diffuse hyperplastic nephrogenic rests (HNR) that can be mistaken for nephroblastoma on imaging. Their treatment and outcome are as variable as published results.Methods: Analysis of 78 patients having clinically apparent uni- or bilateral nephroblastomatosis out of 2347 patients registered for a renal tumor in the consecutive International Society of Pediatric Oncology (SIOP)93-01/GPOH and SIOP2001/GPOH studies between 1993 and 2014. Results: Median age at diagnosis and follow-up was 13 months (0–12.7 years) and 9.2 years, respectively. 59% of patients were female, 57% had bilateral and 53% multifocal or diffuse nephroblastomatosis. Radiologic diagnosis was conclusive in 67% when reviewed centrally versus 30% without review (p=0.015). 27% of patients developed nephroblastoma(s). On histology 45% were high-risk, 30% intermediate-risk and 25% upfront-surgery-blastemal subtype. 5-year-event-free (EFS), nephroblastoma-free-(NFS) and overall-survival (OS) were 60.7%, 79.8% and 95.5%, respectively. Univariate risk factors for EFS/NFS were female gender (p), bilateral (p=0.002/p=0.03) and diffuse HNR at diagnosis (p), and non-complete remission (non-CR) at the end of first-line-treatment (both p). Gender and non-CR remained significant in multivariate COX regression analysis for EFS (p=0.005 and ), non-CR also for NFS (p=0.002). 34 of 74 evaluable patients achieved CR within 12 weeks after diagnosis, 25 of them had no further treatment and were followed, 9 received chemotherapy. Their EFS was 78% and 100%, respectively.Conclusion: Nephroblastomatosis confronts clinicians with a variety of pitfalls, but overall survival is excellent. However, patients need an extended follow-up for more than a decade. In patients who have achieved CR within 12 weeks, a relatively short treatment is sufficient. Contrary, the risk of progression for patients having bilateral and diffuse HNR is significantly increased, despite prolonged treatment. A clear common terminology is needed for future trials to facilitate true comparability of results.

Background Long segment laryngotracheoesophageal clefts (LTECs) are very rare large‐airway malformations. Over the last 40 years mortality rates declined substantially due to improved intensive care and surgical procedures. Nevertheless, long‐term morbidity, comorbidity, and clinical outcomes have rarely been assessed systematically. Methods In this retrospective case series, the clinical presentation, comorbidities, treatment, and clinical outcomes of all children with long‐segment LTEC that were seen at our department in the last 15 years were collected and analyzed systematically. Results Nine children were diagnosed with long segment LTEC (four children with LTEC type III and five patients with LTEC type IV). All children had additional tracheobronchial, gastrointestinal, or cardiac malformations. Tracheostomy for long‐time ventilation and jejunostomy for adequate nutrition was necessary in all cases. During follow‐up one child died from multiorgan failure due to sepsis at the age of 43 days. The clinical course of the other eight children (median follow‐up time 5.2 years) was stable. Relapses of the cleft, recurrent aspirations, and respiratory tract infections led to repeated hospital admissions. Conclusions Long‐segment LTECs are consistently associated with additional malformations, which substantially influence long‐term morbidity. For optimal management, a multidisciplinary approach is essential.

PURPOSE Rhabdoid tumor of the kidney (RTK) is one of the most aggressive childhood renal tumors. Overall survival ranges from 22% to 47%. The indication for radiation therapy (RT) in usually very young patients is an ongoing discussion. Recent protocols recommend RT independent of local stage, the latter being a good discriminator in other childhood kidney tumors. In this study, we analyze the evidence for RT in regard to risk factors, including tumor stage. METHODS AND MATERIALS This study analyzed 58 patients with RTK from Austria, Switzerland, and Germany treated in the framework of 4 consecutive, prospective renal/rhabdoid tumor studies from 1991 to 2014. All treatment protocols included multimodality treatment, including high-intensity chemotherapy, surgery, and RT. RESULTS Local stage distribution was not applicable, I, II, and III in 1, 6, 11, and 40, respectively. Twenty-nine (50%) patients had stage IV disease at diagnosis. Thirty-seven patients (64%) achieved complete remission, and 49% (18/37) relapsed. Thirty-four patients (60%) patients had progressive disease and died, 17 had local disease, 10 had combined disease, and 7 had distant disease; 2 treatment-related deaths were reported (3%). Twenty-one patients received RT during first-line treatment, 18 of them to all involved sites. Eight of the 34 cases of progressive disease occurred in irradiated patients. The local failure rate of treated patients with local stage II or III disease was 29% (6/18) in patients irradiated to all sites compared with 68% (15/22) in nonirradiated patients. One of 6 stage I patients received RT, and 1 patient experienced distant relapse (2-year progression-free and overall survival both 83% ± 15%). Progression-free survival for local stage II and III disease treated with RT, adjusted for early relapse or treatment abandonment, was 67% ± 11%, compared with 15% ± 7% without RT (P < .0001). CONCLUSION The 68% local failure rate in nonirradiated patients underlines the importance of local treatment. Our experience supports the use of RT for local control in higher stage disease. In contrast, no local relapse in 6 local stage I patients, including 5 nonirradiated patients, suggests omission of RT in this favorable subset of usually infant patients with RTK.

ZusammenfassungDie Ösophagusatresie (ÖA) ist eine angeborene Kontinuitätsunterbrechung der Speiseröhre mit oder ohne Fistel zur Trachea (Tracheoösophageale Fistel, TÖF). Je nach Abstand zwischen den beiden Ösophagusenden unterscheidet man eine „kurzstreckige“ von einer „langstreckigen“ Form. Bis zu 50% der Neugeborenen weisen weitere Anomalien auf. Eine pränatale Diagnose der ÖA gelingt in 32–63% der Fälle. Die interdisziplinäre Betreuung dieser Kinder hat sich in den letzten Jahren gewandelt. In der folgenden Arbeit wird die aktuelle S2K-Leitlinie der Deutschen Gesellschaft für Kinderchirurgie zur Behandlung der ÖA mit unterer TÖF, die etwa 90% aller Fälle ausmacht, zusammengefasst. Hierzu gehören das präoperative Management sowie operative und anästhesiologische Aspekte (u. a. Thorakoskopie vs. Thorakotomie, präoperative Tracheobronchoskopie, intraoperative Hyperkapnie und Azidose). Ferner wird das postoperative Management insbesondere von relevanten Komplikationen wie der Anastomosenstenose beschrieben. Trotz Fortschritten in der Behandlung der ÖA ist die Langzeitmorbidität mit Motilitätsstörungen des Ösophagus, gastroösophagealer Refluxkrankheit, rezidivierenden Infektionen der oberen und unteren Atemwege, Tracheomalazie, mangelhaftem Gedeihen sowie orthopädischen Problemen nach Thorakotomie weiterhin hoch. Zum Erreichen einer guten Lebensqualität ist daher eine gute interdisziplinäre Nachsorge wichtig.

IntroductionThe purpose of this study was to determine the earliest timing of inguinal hernia repair under general anesthesia with minimized risk for respiratory complications during postoperative course.MethodsWe performed a monocentric analysis of patient records of premature and full-term infants undergoing inguinal hernia repair between 2009 and 2016. In addition to demographic and medical parameters, preexisting conditions and the perioperative course were recorded.ResultsThe study included 499 infants (preterm n = 285; full term n = 214). The number of subsequently ventilated patients was particularly high among preterm infants with bronchopulmonary dysplasia, up to 45.3% (p < 0.001). Less than 10% of subsequent ventilation occurred in preterm infants after 45 weeks of postmenstrual age at the time of surgery or in patients with a body weight of more than 4,100 g. Preterm infants with a bronchopulmonary dysplasia had an increased risk of apneas (p < 0.05). Only 10% of the preterm babies with postoperative apneas weighed more than 3,600 g at the time of surgery or were older than 44 weeks of postmenstrual age.ConclusionOur data indicate that after the 45th week of postmenstrual age and a weight above 4,100 g, the risk for respiratory failure after general anesthesia seems to be significantly decreased in preterm infants.

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

Zusammenfassung Hintergrund Die konservative Therapie ist der Standard bei Unter-4-jährigen mit Femurfraktur. Um die Zufriedenheit der Eltern mit dieser aufwändigen Behandlung zu verbessern haben wir wichtige Einflussfaktoren auf diesen wichtigen Behandlungsaspekt untersucht. Patienten: 42 Fälle von Kindern unter 4 Jahren, mit Femurfraktur und Overheadextension-Therapie (OHE) wurden analysiert. Methode Unser Standardverfahren beinhaltet die 10-tägige OHE mit anschließender Becken-Bein-Gips-Behandlung (BBG). Röntgenbilder wurden bezgl. des Heilverlaufs analysiert. Die Eltern wurden mittels eines pseudonymisierten Fragebogens über ihre Erfahrungen befragt. Ergebnisse Die Art der Fraktur wurde 73.3% der Eltern „verständlich und detailliert“ erklärt. 23.3% gaben an, dass „viele Fragen unbeantwortet” blieben. 70% der Eltern bemerkten Verhaltensauffälligkeiten bei Ihrem Kind, 63.3% berichteten von therapie-spezifischen Problemen. 86.7% der Eltern waren mit dem Behandlungserfolg zufrieden, 80% mit der Art der Therapie und 73.3% würden die OHE erneut wählen. Spearman-Korrelations-Analysen fanden eine signifikante Korrelation zwischen der generellen Elternzufriedenheit und einem hohen Grad an Aufklärung bzgl. der Art der Fraktur. Eltern, welche Probleme während der Visite ansprechen konnten zeigten eine signifikant höhere Zufriedenheit. Diskussion Die meisten Gründe für eine Unzufriedenheit der Eltern waren in Verbindung mit Kommunikationsproblemen zu sehen. Schlussfolgerung Eine gute Evaluierung während der Visite, gute Kommunikation mit den Ärzten sowie gute pflegerische Hilfestellung beeinflussen die Zufriedenheit der Familien erheblich.

OBJECTIVES Smartphones have become an integral part of daily life, often grabbing full attention of its user. We hypothesized that smartphone-associated trauma in children and adolescents has increased in the last decade. The objective of this study was to analyze smartphone-related injuries in children at two German centers for pediatric emergency care. METHODS Smartphone-related injuries were recorded between January 2008 and March 2018 at two centers of pediatric surgery in Germany. Data were assessed for patient demography, cause of accident, type of injury, treatment, and outcome. RESULTS Ten children (8 girls, 2 boys; mean ± SD age, 10.6 ± 6.0 years; range, 10 weeks to 17 years) were included. Two patients were injured in 2008 to 2015, eight in 2016 to 2018, of which three required hospital admissions. Six accidents happened in public spaces, and four within domestic environments. Eight children (mean ± SD age, 13.3 ± 2.4 years; 7 girls) were injured while using their smartphone, therefore being distracted. Two children (mean ± SD age, 6.5 ± 6.4 months) were involuntarily hurt by the smartphone of their caregivers. The causes of accident and related injuries were highly variable and ranged from minor trauma (mild head injury [n = 3], abrasions [n = 2], bruises of fingers [n = 2]/hand [n = 1]/ankle [n = 2]) to major injuries requiring intensive care treatment (pelvic [n = 1] or vertebral body fractures [n = 1]). CONCLUSIONS Smartphone-associated injuries mainly caused by distraction gain increasing importance in pediatric traumatology. The frequency is higher in females compared with their male counterparts. The prevention of these accidents should become part of educational programs for children and adolescents.

Introduction: Since early 2020 the COVID-19 pandemic and statutory preventive reorganization of treatment capacities with cancellation of elective surgery as well as curfew regulations led to vastly decreased utilization of primary health care.Materials and Methods: To assess whether there are negative effects on pediatric acute care in Bavaria during the spring 2020 lockdown a state-wide retrospective multi-center study was performed to analyze the rate of perforated appendicitis during lockdown. Children who have been operated on during the corresponding period in 2018/19 served as control group.Results: Overall, 514 patients (292 boys, 222 girls) were included (2020: 176 patients; 2019: 181 patients; 2018: 157 patients). Median age was 11.2 years. Four hundred thirty-nine patients (85.4%) underwent laparoscopic surgery, 69 (13.4%) open surgery and 1.2% underwent conversion from laparoscopic to open surgery. In 2020 a perforation rate of 27.8% (49/176 patients) was found, in 2018–2019 perforation rate was 20.7% (70/338 patients, p = 0.0359, Cochran-Mantel-Haenszel-Test). Subgroup analysis showed that in younger patients (≤ 11.2 years), in 2020 perforation rate was significantly higher with 37.6% (32/85 patients), while 22.2% (39/176) in 2018/2019 (p = 0.014, Fisher's exact test).In boys perforation rate was significantly higher in 2020 with 35.0% (35/100 patients) compared to 21.4% in 2018–2019 (p = 0.0165, Fisher's exact test).Conclusion: During the period of curfew regulations in Bavaria the rate of perforated appendicitis in childhood increased significantly, especially in younger children and boys. Potentially this has to be attributed to delayed presentation to pediatric surgery care. Because of potential long-term sequelae of perforated appendicitis these adverse effects during curfew have to be taken into account for future political decision making to ensure reasonable patient care and avoid collateral damage in near-future or on-going pandemic situations.

Background Children with hepatoblastoma (HB) are at risk of sarcopenia due to immobility, chemotherapy, and malnutrition. We hypothesized that children with HB have a low preoperative total psoas muscle area (tPMA), reflecting sarcopenia, which negatively impacts outcome. Procedure Retrospective study of children (1-10 years) with hepatoblastoma treated at a large university children's hospital from 2009 to 2018. tPMA was measured as the sum of the right and left psoas muscle area (PMA) at intervertebral disc levels L3-4 and L4-5. z-Scores were calculated using age- and gender-specific reference values and were compared to anthropometric measurements, clinical variables, and outcomes. Sarcopenia was defined as a tPMA z-score below -2. Results Thirty-three children were included. Mean tPMA z-score was -2.18 ± 1.08, and 52% were sarcopenic. A poor correlation between tPMA and weight was seen (r = 0.35; confidence interval [CI] 0.01, 0.62; P = .045), and most children had weights within the normal range (mean z-score -0.55 ± 1.39). All children categorized as high risk with relapse (n = 5/12) were sarcopenic before surgery. Relapse was significantly higher in the high-risk sarcopenic group compared to the nonsarcopenic group (P = .008). The change in tPMA z-score 1-4 months after surgery did not improve in patients with relapse, but did improve in 75% of children without relapse. Conclusions The majority of children with HB were sarcopenic prior to surgery. Especially in children with high-risk hepatoblastoma, sarcopenia is an additional risk factor for relapse. Large multicenter studies are needed to confirm these preliminary results.

Introduction Pelvic neuroblastoma (NB) is a rare entity and occurs in 2 to 5% of all NBs. Surgery in the pelvic area is—even for the experienced oncological surgeon—technically challenging, as injuries of bladder and/or rectal innervation may carry lifelong consequences for the patient. Several studies have proven the impact of image-defined risk factors (IDRFs) for outcome, complications and extent of resection in NB; however, the specific role of IDRF in pelvic NB has not been investigated yet. Materials and Methods Patient charts were retrospectively evaluated for International Staging System stage, IDRF status, MYCN amplification, and outcome parameters. Results Between 2003 and 2019, 277 NBs were surgically resected in the department of pediatric surgery of Dr. von Hauner Children's Hospital. Out of these, 11 patients (3.9%) had pelvic NB. Evaluation of the preoperative imaging showed two patients without IDRF (stage L1) and eight patients in stage L2. One patient had stage M according to distant metastasis. Patients without IDRF underwent complete macroscopical resections, whereas complete tumor removal was not possible without mutilation in patients with IDRF. At time point of diagnosis, only patients with IDRF had functional neurological problems. Three patients developed perioperative complications; all of them had at least one IDRF. Three patients developed local recurrence during the course of the disease, all of them had at least one IDRF. Conclusion Our results indicate on a preliminary level the importance of IDRF as a prognostic tool for surgical removal of pelvic NB.

Background Ethiopia is a rapidly developing country in Eastern Africa. 43.2% of the population is younger than 15. In contrast, until a few years ago, pediatric surgery was only available in Addis Ababa. Now, Ethiopia is making great efforts to improve the care of surgical ill children. JimmaChild was established to set up a pediatric surgery in Jimma. Methods JimmaChild developed from a scientific collaboration between Jimma University (JU) and Ludwig-Maximilians-University. The project was developed and realized by Ethiopian and German colleagues. A curriculum was written for this purpose. The pediatric surgical training of the fellows was carried out on-site by German pediatric surgeons. Results A new pediatric surgery was established at JU with its own operating room, ward, and staff. After two and a half years, two fellows completed their final examinations as pediatric surgeons. Among others, 850 elective surgeries were performed, 82% assisted by the German colleagues. The German colleagues rated the preparation for the trip, the on-site support, and the professional progress of the fellows mostly as good to very good. Reported problems in the program flow were also recognized and solved in part. Conclusions Best possible integration of the project into existing structures was achieved by close cooperation of Ethiopian and German colleagues during the project development. Problems were identified and addressed early on by external monitoring. As the project responsibility was mainly with the Ethiopian colleagues, a department was created that now exists independent of external funding and trains its own fellows.

Esophageal atresia (EA) is a congenital anomaly that entails an interrupted esophagus with or without tracheoesophageal fistula (TEF). Depending on the distance of the two esophageal pouches a "short-gap" is distinguished from a "long-gap" variant. Up to 50% of newborns have additional anomalies. EA is prenatally diagnosed in 32-63% of cases. Recently, the interdisciplinary care in these children underwent substantial changes. Therefore, we summarize the current guideline of the German society of pediatric surgery for the treatment of patients with EA and distal TEF (Gross Type C). Controversies regarding the perioperative management include surgical-technical aspects, such as the thoracoscopic approach to EA, as well as general anesthesia (preoperative tracheobronchoscopy, intraoperative hypercapnia and acidosis). Moreover, postoperative complications and their management like anastomotic stricture are outlined. Despite significant improvements in the treatment of EA, there is still a relevant amount of long-term morbidity after surgical correction. This includes dysmotility of the esophagus, gastroesophageal reflux disease, recurrent respiratory infections, tracheomalacia, failure to thrive, and orthopedic complications following thoracotomy in the neonatal age. Therefore, close follow-up is mandatory to attain optimal quality of life.Die Ösophagusatresie (ÖA) ist eine angeborene Kontinuitätsunterbrechung der Speiseröhre mit oder ohne Fistel zur Trachea (Tracheoösophageale Fistel, TÖF). Je nach Abstand zwischen den beiden Ösophagusenden unterscheidet man eine „kurzstreckige“ von einer „langstreckigen“ Form. Bis zu 50% der Neugeborenen weisen weitere Anomalien auf. Eine pränatale Diagnose der ÖA gelingt in 32–63% der Fälle. Die interdisziplinäre Betreuung dieser Kinder hat sich in den letzten Jahren gewandelt. In der folgenden Arbeit wird die aktuelle S2K-Leitlinie der Deutschen Gesellschaft für Kinderchirurgie zur Behandlung der ÖA mit unterer TÖF, die etwa 90% aller Fälle ausmacht, zusammengefasst. Hierzu gehören das präoperative Management sowie operative und anästhesiologische Aspekte (u. a. Thorakoskopie vs. Thorakotomie, präoperative Tracheobronchoskopie, intraoperative Hyperkapnie und Azidose). Ferner wird das postoperative Management insbesondere von relevanten Komplikationen wie der Anastomosenstenose beschrieben. Trotz Fortschritten in der Behandlung der ÖA ist die Langzeitmorbidität mit Motilitätsstörungen des Ösophagus, gastroösophagealer Refluxkrankheit, rezidivierenden Infektionen der oberen und unteren Atemwege, Tracheomalazie, mangelhaftem Gedeihen sowie orthopädischen Problemen nach Thorakotomie weiterhin hoch. Zum Erreichen einer guten Lebensqualität ist daher eine gute interdisziplinäre Nachsorge wichtig.

Introduction Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) occurs approximately 1 in 3.500 live births representing the most common malformation of the upper digestive tract. Only half a century ago, EA/TEF was fatal among affected newborns suggesting that the steady birth prevalence might in parts be due to mutational de novo events in genes involved in foregut development. Methods To identify mutational de novo events in EA/TEF patients, we surveyed the exome of 30 case-parent trios. Identified and confirmed de novo variants were prioritized using in silico prediction tools. To investigate the embryonic role of genes harboring prioritized de novo variants we performed targeted analysis of mouse transcriptome data of esophageal tissue obtained at the embryonic day (E) E8.5, E12.5, and postnatal. Results In total we prioritized 14 novel de novo variants in 14 different genes (APOL2, EEF1D, CHD7, FANCB, GGT6, KIAA0556, NFX1, NPR2, PIGC, SLC5A2, TANC2, TRPS1, UBA3, and ZFHX3) and eight rare de novo variants in eight additional genes (CELSR1, CLP1, GPR133, HPS3, MTA3, PLEC, STAB1, and PPIP5K2). Through personal communication during the project, we identified an additional EA/TEF case-parent trio with a rare de novo variant in ZFHX3. In silico prediction analysis of the identified variants and comparative analysis of mouse transcriptome data of esophageal tissue obtained at E8.5, E12.5, and postnatal prioritized CHD7, TRPS1, and ZFHX3 as EA/TEF candidate genes. Re-sequencing of ZFHX3 in additional 192 EA/TEF patients did not identify further putative EA/TEF-associated variants. Conclusion Our study suggests that rare mutational de novo events in genes involved in foregut development contribute to the development of EA/TEF.

We report a case of a 9-year-old female who required surgical treatment and resuscitation after severe transoesophageal bleeding of a right aberrant subclavian artery (RASA). Bleeding of this RASA was caused by a mechanical irritation due to an oesophageal stent. The stent was placed weeks before to dilate the oesophagus after accidental ingestion of a caustic agent. Although conservative management of benign oesophageal stenosis in children is highly recommended, there are still some major complications to be considered. To avoid erosion of aberrant subclavian artery vascular rings and slings, as described in several case reports, these vessels should be excluded by computed tomography (CT) or magnetic resonance imaging (MRI) scans before placement of oesophageal stents. This case suggests that management of caustic ingestion in children is still a major challenge in paediatric surgical departments. Keywords: Paediatric surgery, Chemical Burn, Oesophageal stenosis, Oesophageal stenting, Vascular abnormalities, Right aberrant subclavian artery

Introduction. Patellar instability (PI) is a common finding in children. Current parameters describing patellofemoral joint alignment do not account for knee size. Additionally, most parameters utilize joint-crossing tibiofemoral landmarks and are prone to errors. The aim of the present study was to develop a knee size-independent parameter that is suitable for pediatric or small knees and determines the malpositioning of the distal patellar tendon insertion solely utilizing tibial landmarks. Methods. Sixty-one pediatric knees were included in the study. The tibial tubercle posterior cruciate ligament distance (TTPCL) was measured via magnetic resonance imaging (MRI). The tibial head diameter (THD) was utilized as a parameter for knee size. An index was calculated for the TTPCL and THD (TTPCL/THD). One-hundred adult knees were analyzed to correlate the data with a normalized cohort. Results. The THD was significantly lower in healthy females than in males (69.3 mm ± 0.8 mm vs. 79.1 mm ± 0.7 mm; p<0.001) and therefore was chosen to serve as a knee size parameter. However, no gender differences were found for the TTPCL/THD index in the healthy adult study cohort. The TTPCL/THD was significantly higher in adult PI patients than in the control group (0.301 ± 0.007 vs. 0.270 ± 0.007; p=0.005). This finding was repeated in the PI group when the pediatric cohort was analyzed (0.316 ± 0.008 vs. 0.288 ± 0.010; p=0.033). Conclusion. The TTPCL/THD index represents a novel knee size-independent measure describing malpositioning of the distal patellar tendon insertion determined solely by tibial landmarks.

Conservative options are the standard for treatment of femoral fractures for patients under the age of 4 years. We investigated possible factors influencing the satisfaction of the parents in order to improve their experience.We analyzed 42 cases of children under the age of 4 years treated with overhead extension therapy (OHE) for femoral fracture.Our protocol requires transition to spica cast treatment (SCT) and discharge from the hospital after the initial 10-day OHE. X-rays were analyzed for fracture healing. A pseudonymized questionnaire was sent to the parents in order to evaluate their experiences during the treatment.The nature of the fracture was explained to 73.3% of the parents in a way that was "understandable and in detail". However, 23.3% stated that "many questions were not answered". Seventy percent of parents noticed behavioral abnormalities during treatment, and 63.3% reported treatment-specific problems. Additionally, 86.7% of parents were satisfied with the therapy outcome, 80% were content with the type of therapy, and 73.3% would choose OHE again. We found a high Spearman correlation between general satisfaction and parents who felt well informed. There was also a strong correlation between high general satisfaction and the opportunity for any problems to be discussed and addressed during the medical rounds.The reasons for nonsatisfaction were primarily due to communication problems.Thorough evaluation during medical rounds, communication with healthcare professionals and care provided by the nurses affects the contentment of the families significantly.Die konservative Therapie ist der Standard bei Unter-4-jährigen mit Femurfraktur. Um die Zufriedenheit der Eltern mit dieser aufwändigen Behandlung zu verbessern haben wir wichtige Einflussfaktoren auf diesen wichtigen Behandlungsaspekt untersucht. Patienten: 42 Fälle von Kindern unter 4 Jahren, mit Femurfraktur und Overheadextension-Therapie (OHE) wurden analysiert.Unser Standardverfahren beinhaltet die 10-tägige OHE mit anschließender Becken-Bein-Gips-Behandlung (BBG). Röntgenbilder wurden bezgl. des Heilverlaufs analysiert. Die Eltern wurden mittels eines pseudonymisierten Fragebogens über ihre Erfahrungen befragt.Die Art der Fraktur wurde 73.3% der Eltern „verständlich und detailliert“ erklärt. 23.3% gaben an, dass „viele Fragen unbeantwortet” blieben. 70% der Eltern bemerkten Verhaltensauffälligkeiten bei Ihrem Kind, 63.3% berichteten von therapie-spezifischen Problemen. 86.7% der Eltern waren mit dem Behandlungserfolg zufrieden, 80% mit der Art der Therapie und 73.3% würden die OHE erneut wählen. Spearman-Korrelations-Analysen fanden eine signifikante Korrelation zwischen der generellen Elternzufriedenheit und einem hohen Grad an Aufklärung bzgl. der Art der Fraktur. Eltern, welche Probleme während der Visite ansprechen konnten zeigten eine signifikant höhere Zufriedenheit.Die meisten Gründe für eine Unzufriedenheit der Eltern waren in Verbindung mit Kommunikationsproblemen zu sehen.Eine gute Evaluierung während der Visite, gute Kommunikation mit den Ärzten sowie gute pflegerische Hilfestellung beeinflussen die Zufriedenheit der Familien erheblich.

Postsurgical handover of paediatric patients from operating rooms to intensive care units is a critical moment. This process is susceptible to errors and inefficiencies particularly if poor teamwork in this multidisciplinary and ad hoc collaboration occurs. Through combining provider- and observer-rated team performance, we aimed to determine agreement levels on team performance and associations with mental demands, disruptions, and stress. An observational and multisource study of provider and concomitant expert-observer ratings was established. In an Academic Paediatric Hospital, we conducted standardized observations of postsurgical handovers to PICU. We applied established observational and self-reported teamwork tools. Nested fixed and mixed models were established to estimate agreement within teams, between providers' and observer's ratings, as well as for estimations between team performance and mental demands, disruptions, and stress outcomes. Thirty-one postsurgical patient handovers were included with overall 109 ratings of involved providers. Provider-perceived team performance was rated high. Within the receiving sub-team, situation awareness was perceived lower compared to the handoff sub-team [F(df = 1) = 4.41, p = .04]. Inter-provider agreement on handover team performance was low for the overall team yet higher within handover sub-teams. We observed that high level of distractions during the handover was associated with inferior team performance rated by observers (B = - 0.72, 95% CI = - 1.44, - 0.01).Conclusion: We observed substantial disagreements on how involved professionals as well as observers rated teamwork during patient transfers. Investigations into paediatric teamwork and particular team-based handovers should carefully consider if concurrent provider and observer assessments are a valid and reliable way to evaluate teamwork in paediatric care. Common handover language should be established and mandatory before jointly evaluating this process. Our findings advocate also that handovers should be performed under low levels of distractions.What is Known:• Efficient teamwork during transfers of critically ill children is fundamental to quality and safety of handover practice.• Postoperative handovers are often performed by ad hoc teams of caregivers with multiple backgrounds and are prone to suboptimal team performance, communication, and information transfer.What is New:• Our provider and expert evaluations of team performance during OR-PICU handovers showed poor agreement for team performance. Our findings challenge previous results drawing upon single source assessments and inform future studies to carefully consider what approach of team performance assessments is required.• We further demonstrate that high levels of disruptions are associated with poor team performance during patient handovers and that efforts to ensure undisrupted handover practices in clinical care are necessary.

Introduction: Both a Hickman catheter (HC) and port catheter (Port) can be inserted either percutaneously by the Seldinger technique or by surgical venous cut-down. Catheters are inserted with a vessel-sparing technique when they are placed in the internal jugular vein (IJV) by venous cut-down. Although this technique is common, data are sparse regarding the vessel's state at long-term follow-up. This study was aimed at determining the flow pattern and constitution of the IJV after vessel-sparing implantation of an HC or Port and comparing the outcomes to those of implantation with the Seldinger technique. Methods: One hundred children (58 boys, 42 girls) between 33 days and 18 years of age who underwent a vessel-sparing implantation of an HC or Port in the IJV were prospectively included. All patients underwent surgical venous cut-down at a single institution. Patency and shape of the IJV were determined by ultrasound and categorized according to 2 possible outcomes: relevant alteration (including occlusion of the IJV) and no relevant alteration, with relevant alteration defined as changes that caused an altered flow pattern. Results: Median age was 6 years at the time of operation, and the median indwelling time of catheters was 271 days. Twenty-two of our patients (22%) showed relevant alterations. These changes included high-grade stenosis or lesion in 13 patients (13%) and occlusion in 9 patients (9%). There were no operation-associated complications, such as pneumothorax, hematopericardium, or accidental puncture of the carotid artery. Statistical analysis did not reveal any specific parameter as a risk factor for relevant structural abnormalities. Discussion: In a comparison of our data to the literature, venous cut-down showed an alteration rate of 26% and a patency rate of 85%, whereas the Seldinger technique was found to cause alteration in 15%, with a patency rate of 97% but a successful placement rate of only 90.3-91.6%. Conclusion: The indication for long-term catheter placement may determine which method is preferable. A child who is likely to need more catheters in the future might benefit from the Seldinger technique, since there is a higher chance of long-term patency of the vessel. A patient undergoing chemotherapy might benefit more from the surgical venous cut-down with less placement-associated complications.

Chirurgische Erkrankungen der Trachea betreffen osophagotracheale Fisteln, Tracheomalazien und Stenosen. Die Anlage eines Tracheostomas ist eine der haufigsten kinderchirurgischen Eingriffe an der Trachea und dient der Sicherung des Atemwegs.

Chirurgische Erkrankungen der Schilddruse im Kindesalter sind selten und sollten daher in entsprechenden Zentren behandelt werden.

Fehlbildungen des Halses betreffen von den Kiemengangen und dem Ductus thyreoglossus ausgehend Fisteln und Zysten sowie die auserst selten auftretenden medianen Halsspalten und Flugelfellbildungen (Pterygium colli). Pathologien der Gefassysteme werden in dieser Region haufiger gesehen und imponieren als Gefastumoren, wie Hamangiome, und kongenitale Gefasmalformationen, wie Lymphangiome und Blutgefasdysplasien.

Background: The Critical Incident Reporting System (CIRS) is a tool for employees to report anonymously of near misses. Its efficiency and improvement of safety is proved by many studies. Our department introduced CIRS in 2009 and it is used frequently. As the number of reports decreased over time we asked for factors responsible for the reduced use. Methods: All employees had access to CIRS and have been trained in several courses of instruction. Accomplished results and consequences were published in biannual newsletters. In 2014 we initiated an anonymous employee attitude survey to ask for their experience and satisfaction with CIRS. Results: 88 near misses were reported since 2009. 44 (50%) reports were classified as RS1, 34 (38.6%) as RS2, and 10 (11.4%) as RS3. No RS4 reports were notified. Most reports concerned problems with administration of medication (n=26; 29.5%) and problems with technical devices (n=18; 20.5%). 75 (83%) of our employees participated in the survey. 64 (86.5%) discerned that CIRS is anonymous. 31 (41.9%) reported already a near miss. Of note, two-third didn’t realize an improvement following their report. On the other hand, only half of the pollees stated to read the newsletter. Conclusion: Even if efficiency and advantages of CIRS are proved and undeniable, sufficient and perpetual feedback of results and improvements developed by the CIRS team and regular trainings of the employees are mandatory for the success of CIRS.

Der fulminante Verlauf einer nekrotisierenden Enterokolitis (NEC) kann zu einem Kurzdarmsyndrom („short bowel syndrome“, SBS) fuhren. Im Rahmen der multidisziplinaren Fallbesprechung kommt dem Kinderchirurgen eine zentrale Rolle zu, da er durch seine Interventionen den Krankheitsverlauf der NEC, aber auch des SBS entscheidend mitbeeinflusst. Um fur den Patienten die bestmoglichen Entscheidungen zu treffen, ist die genaue Kenntnis beider Krankheitsbilder und der jeweiligen Therapieoptionen unabdingbar.

Wilms tumor (WT) is the most common pediatric renal malignancy. A recent ontogenic model suggests that undifferentiated tumor state, and hence poor prognosis, in WT is determined by stabilization of β-catenin in the nucleus. Forkhead box M1 (FOXM1) is a downstream component of the Wnt pathway and promotes nuclear localization of β-catenin. As elevation of FOXM1 gene expression is prognostic in various types of malignancy, we hypothesized that high FOXM1 expression in WT is associated with undifferentiated histology and thus poor prognosis. In the current study, the expression of FOXM1 mRNA was determined in 46 WT specimens and 11 renal tissue controls from patients undergoing tumor nephrectomy, and these data were assessed with regard to clinicopathological parameters. The results demonstrated an upregulation of FOXM1 in WT by 10-fold compared to normal tissue. Expression differed significantly between controls and tumors of intermediate- and high-risk histopathology (P

Entzundliche Veranderungen betreffen uberwiegend Lymphknoten; hier sei insbesondere auf den Befall mit atypischen Mykobakterien hingewiesen.

The option of nephron sparing surgery for unilateral Wilms tumor has been debated in the recent literature. This procedure is being used increasingly to preserve kidney tissue and function. However, nephron sparing surgery is feasible only for selected cases, and a higher local relapse rate has been observed. Moreover, a significant reduction of nephrons is associated with development of renal hypertension and progressive renal failure. We analyzed outcomes after bilateral partial nephrectomy and unilateral partial plus contralateral total nephrectomy in patients with bilateral Wilms tumor.We analyzed data from the Society of Pediatric Oncology and Hematology database on 22 patients with bilateral Wilms tumor. Kidney size was measured using volumetric analysis of magnetic resonance imaging. Patients were matched with children who had undergone magnetic resonance imaging of the abdomen for other malignancies.Mean kidney volumes after unilateral partial plus total contralateral nephrectomy (66.9 cm(3)) were significantly greater than the reference kidneys (p = 0.028), whereas controls were equal to the bilateral partial nephrectomy group (49.7 cm(3), p = 0.959). Total kidney volume was significantly larger after bilateral partial nephrectomy (102.1 cm(3)) vs unilateral partial plus total contralateral nephrectomy (66.9 cm(3), p = 0.0338). Eight patients (66.7%) had renal hypertension after unilateral partial plus total contralateral nephrectomy but only 2 (20%) after bilateral partial nephrectomy (p = 0.043). Overall survival and relapse rates were equal between the groups and did not correlate with unfavorable histology.Our findings suggest that patients with bilateral Wilms tumor benefit from bilateral nephron sparing surgery. Hypertension is less common after bilateral partial nephrectomy, and rates of local relapse or disease associated death are distributed equally between the groups.

The Wilms tumor 1 (WT1) gene plays an essential role in early development and differentiation of the urinary tract, particularly the kidneys. Aberrant transcriptional activity of WT1 is a key finding in the genesis of Wilms tumors (WTs). However, the mechanisms responsible for this alteration remain poorly understood. In the present study, we examined the methylation pattern of a putative CCCTC-binding factor (CTCF) binding site downstream of the WT1 gene as a potential cause of WT1 misregulation in 44 native WT specimens. We found that 16 WT cases exhibited a much higher WT1 expression compared to normal kidney tissue, and that the high mRNA expression of WT1 is strongly correlated with a high degree of DNA methylation of the CTCF binding site near the WT1 promoter. However, there was no correlation between the KTS+/KTS- splicing variants of WT1 and the methylation status of the CpGs of the CTCF binding site. Our results demonstrated an aberrant methylation pattern at a CTCF binding site downstream the WT1 gene, which is associated with an elevated WT1 transcriptional activity. Thus, methylation of the CTCF binding site may be partially responsible for the transcriptional activation of the WT1 locus and hypermethylation of this site may be an important oncogenic mechanism in the genesis of WT.