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Over decades now, laparoscopic Nissen fundoplication represents the treatment of choice for symptomatic children with gastroesophageal reflux disease (GERD) unresponsive to medication. Although the basic principles of Nissen's technique are still essential today, academic studies of long-Term results, complications, and patients benefits have fostered distinct modifications. Identification of surgical factors for wrap migration, dysphagia, and recurrent GERD led to recommendations for "short and floppy"wraps with minimal dissection of the phrenoesophageal membranes. This report summarizes up-To-date information from experts in the field on "how to wrap it right"followed by a critical discussion about long-Term benefits for children with GERD and future developments of laparoscopic Nissen fundoplication.

This study focuses on the impact of a prevention program regarding dog bites in children. As a consequence of our previous investigation in 2005, we have initiated a child safety program for primary school children starting January 2008 until present to teach children how to avoid dog attacks and how to behave in case of an attack. In our retrospective study, we analyzed all patients younger than 15 years presenting with dog-related injuries between 2014 and 2018. As the main indicator for success of the prevention measures taken, we have defined the severity of injury in comparison to our previous study. Out of 296 children with dog-related injuries, 212 (71.6%) had sustained a dog bite. In the vast majority (n = 195; 92%), these patients presented with minor injuries; the extremities were most commonly affected (n = 100; 47%). Injuries to the head (n = 95; 45%) and trunk (n = 18; 8%) were less frequent. The proportion of severe injuries (8%) was significantly lower compared to our previous study, where 26% of children presented with severe injuries necessitating surgical intervention, while the number of patients requiring in-hospital treatment declined from 27.5% in the period 1994–2003 to 9.0% in the period between 2014 and 2018 (p Conclusion: Teaching of primary school children may effectively reduce the injury severity of dog bites. What is Known:• Dog bites are a substantial healthcare problem especially in children. What is New:• This study shows that a broad-based prevention program for primary school children can effectively decrease the severity but not the frequency of dog bite injuries in children.

Background Patients following repair of an esophageal atresia (EA) or tracheoesophageal fistula (TEF) carry an increased risk of long-term cardiopulmonary malaise. The role of the airway microbiome in EA/TEF patients remains unclear. Methods All EA/TEF patients treated between 1980 and 2010 were invited to a prospective clinical examination, spirometry, and spiroergometry. The airway microbiome was determined from deep induced sputum by 16 S rRNA gene sequencing. The results were compared to a healthy age- and sex-matched control group. Results Nineteen EA/TEF patients with a mean age of 24.7 ± 7 years and 19 age- and sex-matched controls were included. EA/TEF patients showed a significantly lower muscle mass, lower maximum vital capacity (VCmax), and higher rates of restrictive ventilation disorders. Spiroergometry revealed a significantly lower relative performance capacity and lower peak VO2 in EA/TEF patients. Alpha- and beta-diversity of the airway microbiome did not differ significantly between the two groups. Linear discriminant effect size analysis revealed significantly enriched species of Prevotella_uncultured, Streptococcus_anginosus, Prevotella_7_Prevotella_enoeca, and Mogibacterium_timidum. Conclusion EA/TEF patients frequently suffer from restrictive ventilation disorders and impaired cardiopulmonary function associated with minor alterations of the airway microbiome. Long-term examinations of EA/TEF patients seem to be necessary in order to detect impaired cardiopulmonary function. Impact The key messages of the present study are a significantly decreased VCmax and exercise performance, as well as airway microbiome differences in EA/TEF patients. This study is the first to present parameters of lung function and exercise performance in combination with airway microbiome analysis with a mean follow-up of 24 years in EA/TEF patients. Prospective, long-term studies are needed to unravel possible interactions between alterations of the airway microbiome and impaired pulmonary function in EA/TEF patients.

Fusions involving NTRK1, NTRK2, and NTRK3 are oncogenic drivers occurring in a spectrum of mesenchymal neoplasms ranging from benign to highly malignant tumors. To gain further insights into the staining profile with the pan-TRK assay, we analyzed a large number of soft tissue sarcomas and correlated our findings with molecular testing. Additionally, we expand the spectrum of NTRK-fusion tumors by reporting a mesenchymal lesion in the lung as well as a mesenchymal skin lesion in the spectrum of benign fibrous histiocytoma with NTRK—fusion. We retrospectively reviewed soft tissue sarcomas diagnosed at the Diagnostic and Research Institute of Pathology, Medical University of Graz, between 1999 and 2019, and cases from the consultation files of one of the authors (BLA). In total, 494 cases were analyzed immunohistochemically with pan-TRK antibody (clone EPR17341, RTU, Roche/Ventana) and positive cases (defined as any cytoplasmic/nuclear staining in more than 1% of tumor cells) underwent next-generation sequencing (NGS). Immunohistochemical staining was observed in 16 (3.2%) cases. Eleven cases with focal weak and moderate cytoplasmic/membranous or focal moderate to strong nuclear staining did not harbor an NTRK-fusion (three synovial sarcomas, three leiomyosarcomas, two extraskeletal myxoid chondrosarcomas, and one each: dedifferentiated liposarcoma, pleomorphic liposarcoma, and myxofibrosarcoma). Four cases showed strong diffuse nuclear and/or cytoplasmatic staining, and one case showed diffuse, but weak cytoplasmic staining. All these cases demonstrated an NTRK-fusion (LMNA-NTRK1, IRF2BP2-NTRK1, TMB3-NTRK1, ETV6-NTRK3, RBPMS-NTRK3). Pan-TRK assay (clone EPR17341, RTU, Roche, Ventana) immunohistochemistry serves as a reliable diagnostic marker that can also be expressed in non-NTRK-rearranged mesenchymal neoplasms. It can be used as a surrogate marker for identification of NTRK fusion, nevertheless, an RNA-based NGS for detection of the specific fusion should be performed to confirm the rearrangement, if patients are undergoing targeted therapy. Additionally, we identified NTRK-fusion-positive, primary mesenchymal tumors of the lung and the skin.

Children born with esophageal atresia (EA) might suffer from significant oral feeding problems which could evolve into tube dependency. The primary aim of the study was to define the outcome of tube weaning in children after successful EA repair and to compare outcomes in children with short gap/TEF (tracheoesophageal fistula) and long-gap EA. Data of 64 children (28 with short-gap EA/TEF with primary anastomosis and 36 with long-gap EA with delayed surgical repair) who participated in a standardized tube weaning program based on the “Graz model of tube weaning” (in/outpatients in an intensive 3-week program, online coaching (Netcoaching) only, or a combined 2-week intensive onsite followed by online treatment “Eating School”) from 2009 to 2019 was evaluated. Sixty-one patients completed the program by transitioning to exclusive oral intake (95.3%). Three children (4.7%) were left partially weaned at the time of discharge. No significant differences could be found between short gap/TEF and long-gap EA group regarding outcomes.Conclusions: The study’s findings support the efficacy of tube weaning based on the published “Graz model of tube weaning” for children born with EA/TEF and indicate the necessity of specialized tube weaning programs for these patients. What is Known:• Children with esophageal atresia/tracheoesophageal fistula often suffer from feeding problems and tube dependency.• Different tube weaning programs and outcomes have been published, but not specifically for children with EA. What is New:• Evaluation of a large sample of children referred for tube weaning after EA repair.• Most children with EA can be weaned off their feeding tubes successfully after attending a specialized tube weaning program.

Supplemental Digital Content is available in the text., IMPORTANCE: A Disintegrin and Metalloproteinase with Thrombospondin Motifs-1 is hypothesized to play a role in the pathogenesis of invasive infection, but studies in sepsis are lacking. OBJECTIVES: To study A Disintegrin and Metalloproteinase with Thrombospondin Motifs-1 protein level in pediatric sepsis and to study the association with outcome. DESIGN: Data from two prospective cohort studies. SETTING AND PARTICIPANTS: Cohort 1 is from a single-center study involving children admitted to PICU with meningococcal sepsis (samples obtained at three time points). Cohort 2 includes patients from a multicenter study involving children admitted to the hospital with invasive bacterial infections of differing etiologies (samples obtained within 48 hr after hospital admission). MAIN OUTCOMES AND MEASURES: Primary outcome measure was mortality. Secondary outcome measures were PICU-free days at day 28 and hospital length of stay. RESULTS: In cohort 1 (n = 59), nonsurvivors more frequently had A Disintegrin and Metalloproteinase with Thrombospondin Motifs-1 levels above the detection limit than survivors at admission to PICU (8/11 [73%] and 6/23 [26%], respectively; p = 0.02) and at t = 24 hours (2/3 [67%] and 3/37 [8%], respectively; p = 0.04). In cohort 2 (n = 240), A Disintegrin and Metalloproteinase with Thrombospondin Motifs-1 levels in patients within 48 hours after hospital admission were more frequently above the detection limit than in healthy controls (110/240 [46%] and 14/64 [22%], respectively; p = 0.001). Nonsurvivors more often had detectable A Disintegrin and Metalloproteinase with Thrombospondin Motifs-1 levels than survivors (16/21 [76%] and 94/219 [43%], respectively; p = 0.003), which was mostly attributable to patients with Neisseria meningitidis. CONCLUSIONS AND RELEVANCE: In children with bacterial infection, detection of A Disintegrin and Metalloproteinase with Thrombospondin Motifs-1 within 48 hours after hospital admission is associated with death, particularly in meningococcal sepsis. Future studies should confirm the prognostic value of A Disintegrin and Metalloproteinase with Thrombospondin Motifs-1 and should study pathophysiologic mechanisms.

Neonatal “surgical” malformations are associated with higher costs than major “non-surgical” birth defects. We aimed to analyze the financial burden on the Austrian health system of five congenital malformations requiring timely postnatal surgery. The database of the Austrian National Public Health Institute for the period from 2002 to 2014 was reviewed. Diagnosis-related group (DRG) points assigned to hospital admissions containing five congenital malformations coded as principal diagnosis (esophageal atresia, duodenal atresia, congenital diaphragmatic hernia, gastroschisis, and omphalocele) were collected and compared to all hospitalizations for other reasons. Out of 3,518,625 total hospitalizations, there were 1664 admissions of patients with the selected malformations. The annual mean number was 128 (SD 17, range 110–175). The mean cost of the congenital malformations per hospital admission expressed in DRG points was 26,588 (range 0–465,772, SD 40,702) and was significantly higher compared to the other hospitalizations (n = 3,516,961, mean DRG 2194, range 0–834,997, SD 6161, p &lt, 0.05). Surgical conditions requiring timely postnatal surgery place a significant financial burden on the healthcare system. The creation of a dedicated national register could allow for better planning of resource allocation, for improving the outcome of affected children, and for optimizing costs.

With the advances of neonatology, the survival rate for “live-born periviable fetuses” weighing In summary, minilaparotomy and loop ileostomy placement were effective to treat surgical MRI in Europe's tiniest male newborn. With the advances of neonatology, pediatric surgery reaches new frontiers as well.

In pediatric trauma, the kidney is the most commonly injured organ of the urinary tract. Renal trauma occurs in 10% to 20% of all pediatric blunt abdominal trauma cases. The vast majority of renal injuries can be treated conservatively. However, cases associated with hemodynamic instability require operative interventions. Injuries to the ureter, bladder or urethra are almost exclusively encountered in polytraumatized children. The aim of this article is to give an overview on traumatic injuries to the pediatric urinary system.

The dignity of adrenal masses in children varies from benign lesions like adenoma and ganglioneuroma to malignant tumors like adrenocortical carcinoma and neuroblastoma. Any surgical approach, especially minimally invasive surgery (MIS), requires careful risk stratification based on oncological and technical criteria. Herein, we present four patients who underwent MIS for adrenal masses. Laboratory testing differentiated between simple cysts and adenoma, but could not identify a child with adrenocortical tumor preoperatively. Analysis of image-defined risk factors excluded vascular encasement in all cases. All patients underwent laparoscopic adrenalectomy without complications. Histopathology revealed simple cyst, ganglioneuroblastoma, adenoma, and potentially malignant adrenocortical tumor in one patient/case each. All specimen showed clear margins and no recurrence was noted at a mean follow-up of 18 months.

Despite worldwide public attention and intense medical efforts, the prevalence of severe morbid obesity in children and adolescents is still rising. Similar to adults, excess adipose tissue triggers multiple immunological and metabolic pathways leading to serious co-morbidities such as impaired glucose tolerance or even type 2 diabetes (T2D), dyslipidemia, arterial hypertension, non-alcoholic fatty liver disease, and hyperuricemia. The management of severe childhood obesity requires a life-long multidisciplinary approach with a combination of lifestyle changes, nutrition, and medications. Standardized life-style intervention programs remain the first-line treatment for morbid obese children and adolescents, but unfortunately reveal limited long-term success. In such cases, metabolic bariatric surgery (MBS) has evolved from being a controversial issue to being included in distinct recommendations. According to the American Society for Metabolic and Bariatric Surgery (ASMBS) Pediatric Committee, indications for bariatric surgery in adolescence must follow very strict criteria. Adolescents with class II obesity (BMI > 120% of the 95th percentile) and a diagnosed co-morbidity or with class III obesity (BMI ≥ 140% of the 95th percentile) should be considered for MBS. These interventions represent high-risk operations, and adolescents should be treated in specialized, multidisciplinary high-volume obesity centers with long-term follow-up programs. The Roux-en-Y gastric bypass (RYGB) remains the gold standard of all malabsorptive procedures. Laparoscopic sleeve gastrectomy (LSG), which the authors pioneered as a stand-alone procedure in morbidly obese adolescents in 2008, has become the most commonly performed operation in morbidly obese adolescents at present. Recent literature proves that MBS is safe and effective in morbidly obese adolescents. Mid-term data have revealed significant improvement or even resolution of major co-morbidities. Thus, MBS for the treatment of morbidly obese adolescents has evolved from being a controversial issue to being included in distinct recommendations by several medical societies as a therapeutic strategy to reduce severe co-morbidities potentially causing end-organ damage in adulthood.

Nasal Glioma (NG) represents a rare congenital abnormality of the neonate, which can be associated with skull defects or even a direct communication to the central nervous system. MRI serves valuable information for differentiation from encephalocele, dermoid cyst and congenital hemangioma. Complete resection remains the treatment of choice. We present two cases of NG, which were both suspected during prenatal ultrasound and MRI. In the first case, postnatal MRI showed a transcranial continuity. Mass excision was performed and the defect was covered by a glabellar flap allowing a good cosmetic result. Postnatal MRI excluded a trans-glabellar communication in the second case. After surgical excision, the resulting skin defect was covered with a full thickness skin graft harvested from the right groin. In cases of NGs complete resection and cosmetic appealing results can be achieved and might necessitate a multidisciplinary approach.

Introduction: Embryologically, pheochromocytomas (PCCs), neuroblastomas, ganglioneuroblastomas (GNBs), and ganglioneuromas (GNs) share a common origin from neural crest cells.1,2 The concu...

Background Mesenteric lymphatic malformations (LMs) represent rare congenital anomalies that can include chylous or nonchylous content. The pathologic mechanisms explaining this phenomenon are poorly understood and not yet described. Furthermore, the current management approach does not consider the contents of the mesenteric LMs. In the present study, we have defined the relationship between the lymphatic mesenteric cyst content and the histologic evidence of LMs within the bowel wall. Methods We retrospectively investigated all patients with mesenteric LMs treated surgically at our department from 1999 to 2018. Results A total of 11 patients (6 girls and 5 boys) were included in our analysis. Seven patients had presented with LMs located in the jejunal mesentery, three in the ileocecal region, and only one in the mesocolon transversum and omentum. Of the 11 children, 7 had had LMs with nonchylous content and 4 had presented with chylous content LMs. Intestinal resection was performed in all 4 patients with chylous content LMs and 4 patients with nonchylous content LMs. Histopathologic evaluation of the surgical specimens determined that only the LMs with chylous content displayed malformed lymphatic channels throughout the bowel wall. The resected small bowel of four patients with nonchylous content showed no LM extension throughout the intestinal wall. Conclusions LMs with chylous content seem to develop from malformed lymphatic channels within the bowel wall. In such cases, segmental intestinal resection is mandatory. In contrast, mesenteric LMs with nonchylous content can potentially be treated without bowel resection if the blood supply can be preserved. This finding is, to the best of our knowledge, reported in the present study for the first time.

The perinatal management of newborns with giant omphaloceles requires careful suspicion concerning ‘unexpected’ associated malformations. We describe a newborn with a combination of incomplete Pentalogy of Cantrell consisting of giant omphalocele, anterior congenital diaphragmatic hernia (CDH) and pericardial absence complicated by a hepatic rapidly involuting congenital hemangioma (RICH). A giant omphalocele was detected prenatally. Postnatally, staged closure of the omphalocele was planned. A mass of the liver was noted and (mis-)taken for a hematoma. In the further course, the baby developed cardiorespiratory insufficiency due to a central CDH which was excised. Subsequently, staged closure of the omphalocele became impossible. Specific workup revealed a mass in the left liver lobe. The mass was resected and the abdominal wall defect repaired. Histology confirmed the diagnosis of an RICH. The surgical treatment of newborns with giant omphaloceles requires a multidisciplinary neonatal support and an elaborate pediatric surgical armamentarium to cope with additional malformations.

Neonatologia; Cirurgia pediàtrica; Medicina toràcica pediàtrica Neonatología; Cirugía pediátrica; Medicina torácica pediátrica Neonatology; Paediatric surgery; Paediatric thoracic medicine Introduction A worldwide lack of consensus exists on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) even though the incidence is increasing. Either a surgical resection is performed or a wait-and-see policy is employed, depending on the treating physician. Management is largely based on expert opinion and scientific evidence is scarce. Wide variations in outcome measures are seen between studies making comparison difficult thus highlighting the lack of universal consensus in outcome measures as well. We aim to define a core outcome set which will include the most important core outcome parameters for paediatric patients with an asymptomatic CPAM. Methods and analysis This study will include a critical appraisal of the current literature followed by a three-stage Delphi process with two stakeholder groups. One surgical group including paediatric as well as thoracic surgeons, and a non-surgeon group including paediatric pulmonologists, intensive care and neonatal specialists. All participants will score outcome parameters according to their level of importance and the most important parameters will be determined by consensus. Ethics and dissemination Electronic informed consent will be obtained from all participants. Ethical approval is not required. After the core outcome set has been defined, we intend to design an international randomised controlled trial: the COllaborative Neonatal NEtwork for the first CPAM Trial, which will be aimed at determining the optimal management of patients with asymptomatic CPAM. The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

The aim of this study was to analyze the exhaled volatile organic compounds (VOCs) profile, airway microbiome, lung function and exercise performance in congenital diaphragmatic hernia (CDH) patients compared to healthy age and sex-matched controls. A total of nine patients (median age 9 years, range 6&ndash, 13 years) treated for CDH were included. Exhaled VOCs were measured by GC&ndash, MS. Airway microbiome was determined from deep induced sputum by 16S rRNA gene sequencing. Patients underwent conventional spirometry and exhausting bicycle spiroergometry. The exhaled VOC profile showed significantly higher levels of cyclohexane and significantly lower levels of acetone and 2-methylbutane in CDH patients. Microbiome analysis revealed no significant differences for alpha-diversity, beta-diversity and LefSe analysis. CDH patients had significantly lower relative abundances of Pasteurellales and Pasteurellaceae. CDH patients exhibited a significantly reduced Tiffeneau Index. Spiroergometry showed no significant differences. This is the first study to report the VOCs profile and airway microbiome in patients with CDH. Elevations of cyclohexane observed in the CDH group have also been reported in cases of lung cancer and pneumonia. CDH patients had no signs of impaired physical performance capacity, fueling controversial reports in the literature.

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung's disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung's disease) from 264 hospitals (89 in high-income countries, 166 in middle-income countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p

Abdominal lymphatic malformations in neonates require sophisticated management. In symptomatic cases, acute complications may necessitate immediate surgery. We present the case of a giant abdominal lymphatic malformation diagnosed in the 18th gestational week. Sonographic concerns about intestinal hypoperfusion in the 33rd week of gestation indicated caesarean section. Postnatal imaging confirmed a macrocystic lymphatic malformation occupying almost the complete abdominal cavity; the intestinal perfusion was normal. Clinical deterioration on Day 13 of life required laparotomy. Intraoperatively, the lymphatic mass was located in the ileocecal mesentery. Two major cysts showed recent hemorrhage explaining the onset of abdominal compartment syndrome. The malformation was completely removed. An ileocecal resection with an ileocolic anastomosis was performed. The postoperative course was uneventful. In neonates with abdominal lymphatic malformations, an onset of abdominal compartment syndrome requires surgical exploration. If feasible, the complete removal of the lesion represents a curative option.

Gut hyperpermeability can be caused by either apoptosis of the intestinal epithelium or altered status, permeability or porosity of tight junctions. This project aims to elucidate these mechanisms in the early phase of sepsis. Eighteen male wild type mice were randomized to two groups. All mice received one single gavage of fluorescein isothiocyanate (FITC) dextran 30 min before intervention. One group (n = 10) underwent cecal ligation and puncture to induce sepsis. The other group (n = 8) was sham operated. Septic animals exhibited significantly increased permeability for FITC 8 h post-operatively. Significantly increased serum interleukin-6, tumor-necrosis-factor-alpha and interleukin-1-beta confirmed sepsis. Septic animals showed significant bowel wall inflammation of ileum and colon samples. PCR revealed significantly increased expression of claudin-2 and decreased expressions of claudin-4, tight-junction-protein-1 and occludin-1 resembling increased permeability of tight junctions. However, these alterations could not be confirmed at the protein level. Light microscopy revealed significant dilatation of intercellular spaces at the basal sections of intestinal epithelial cells (IEC) in septic animals confirmed by increased intercellular spaces at the level of tight junctions and adherens junctions in electron microscopy (TEM). In small angle X-ray scattering no increase in number or size of nanopores could be shown in the bowel wall. HOECHST staining and PCR of ileum samples for apoptosis markers proofed no relevant differences in intestinal epithelial cell apoptosis between the groups. Intestinal hyperpermeability in septic animals was most likely caused by alterations of the intercellular contacts and not by apoptosis or increased size/number of nanopores of intestinal epithelial cells in this murine model of early sepsis.

Malignant diseases can cause tumor-associated cachexia (TAC). Supplementation with prebiotic non-digestible carbohydrates exerts positive metabolic effects in experimental oncologic diseases. The aim of this project was to assess the effect of prebiotic supplementation with OMNi-LOGiC&reg, FIBRE on intestinal microbiome, bacterial metabolism, gut permeability, and inflammation in a murine model of neuroblastoma (NB)-associated TAC. For this study, 2,000,000 NB cells (MHH-NB11) were implanted into athymic mice followed by daily supplementation with water or 200 mg prebiotic oligosaccharide (POS) OMNi-LOGiC&reg, FIBRE (NB-Aqua, n = 12, NB-POS, n = 12). Three animals of each tumor group did not develop NB. The median time of tumor growth (first visibility to euthanasia) was 37 days (IQR 12.5 days) in the NB-Aqua group and 37 days (IQR 36.5 days) in the NB-POS group (p = 0.791). At euthanasia, fecal microbiome and volatile organic compounds (VOCs), gut permeability (fluorescein isothiocyanate-dextran (FITC-dextran), and gut barrier markers were measured. Values were compared to sham animals following injection of culture medium and gavage of either water or OMNi-LOGiC&reg, FIBRE (SH-Aqua, n = 10, SH-POS, n = 10). Alpha diversity did not differ significantly between the groups. Principal coordinate analysis (PCoA) revealed clustering differences between Aqua and POS animals. Both NB and POS supplementation led to taxonomic alterations of the fecal microbiome. Of 49 VOCs, 22 showed significant differences between the groups. NB animals had significantly higher gut permeability than Aqua animals, POS did not ameliorate these changes. The pore and leak pathways of tight junctions did not differ between groups. In conclusion, our results suggest that NB-induced TAC causes increased gut permeability coupled with compositional changes in the fecal microbiome and VOC profile. Prebiotic supplementation with OMNi-LOGiC&reg, FIBRE seemed to induce modifications of the fecal microbiome and VOC profile but did not improve gut permeability.

Background The aim of the present study was to assess whether paediatric intensive care units (PICUs) in three central European countries comply with guidelines concerning infrastructure provided by the European Society of Intensive Care Medicine (ESICM). Between July 2016 and May 2017, a survey was conducted based on the ESICM guidelines. The questionnaire was structured into four categories: structural quality, diagnostic/therapeutic equipment, personnel and organization. All PICUs treating paediatric patients in the D–A–CH region [Germany (D), Austria (A) and Switzerland (CH)] were researched through the national societies. A total of 126 PICUs were contacted (D: 106; A: 12; and CH: 8). Results Eighty-five of 126 PICUs responded (D: 67%; A: 61%; and CH: 100%). A median of 500 patients was treated annually (D: 500; A: 350; and CH: 600) with a median of 12 beds (D: 12; A: 8; and CH: 12). Recommendations regarding infrastructure were met as follows: structural quality 62% in D, 71% in A and 75% in CH; diagnostic/therapeutic equipment: 87% in D, 91% in A and 89% in CH; personnel: 65% in D, 87% in A and 85% in CH; and organization: 75% in D, 73% in A and 88% in CH. Conclusions This survey reveals deficits concerning structural quality in all countries. Furthermore, shortcomings regarding personnel were found in Germany and for organization in Germany and Austria. These issues need to be addressed urgently to further improve treatment quality and patient safety in the future. Electronic supplementary material The online version of this article (10.1186/s13613-018-0451-1) contains supplementary material, which is available to authorized users.

The aim of this study was to determine the diagnostic capacity of hepcidin in pediatric acute appendicitis and its accuracy as a predictor of the severity of appendicitis.In children with appendicitis (n = 39), leukocytes, platelet count, and the serum levels of C-reactive protein (CRP) and hepcidin were compared to a control group (n = 25) of patients with unspecific abdominal pain. Additionally, parameters were compared between children with simple (n = 17) and complicated appendicitis (n = 22). Receiver operation characteristic analyses of the different parameters were performed and the areas under the curve (AUCs) calculated.Leukocytes and serum hepcidin levels were significantly higher in children with acute appendicitis versus control group (13.7 ± 5.7 versus 9.8 ± 3.9 G/L, P = 0.005 and 31.3 ± 21.7 versus 20.4 ± 14 ng/mL, P = 0.039). AUCs for hepcidin, leukocytes, and CRP were 0.654, 0.711, and 0.619, respectively. Complicated appendicitis was associated with significantly higher hepcidin concentrations compared to simple appendicitis (38.5 ± 17.6 ng/mL versus 21.6 ± 23.4 ng/mL, P 0.001). A combination of leukocytes, CRP, and hepcidin had the highest AUC (0.914) to predict complicated appendicitis.Increased serum levels of hepcidin were found in children with appendicitis compared to controls. While hepcidin was useful to identify patients with complicated appendicitis as it does not seem appropriate to distinguish between simple appendicitis and other causes for acute abdominal pain.

Highlights • Laparoscopic repair of intermediate anorectal malformation (ARM) lead to full-thickness rectal prolapse (RP) in a boy. • As conservative treatment modalities failed, the parents opted for surgical treatment at the patient’s age of 4 years. • Intractable RP of 2 cm with ulcerations was successfully treated by Well’s laparoscopic posterior mesh-rectopexy. • The mid-term outcome was favorable with no prolapse recurrence. • Well’s procedure represents a feasible treatment option for RP secondary to ARM repair in children., Introduction Intractable full-thickness rectal prolapse (IRP) unresponsive to conservative treatment remains a major problem after anorectoplasty for high or intermediate anorectal malformation (ARM). Surgical management must aim for a permanent fixation of the rectum to the presacral fascia. While in children with IRP following ARM repair the optimal procedure has not been established yet, laparoscopic posterior mesh-rectopexy (Well’s procedure) has demonstrated efficacy in adults. Presentation of case A male infant with intermediate ARM received laparoscopic-assisted anorectal pull-through at the age of 4 months. Three months later he developed mucosal prolapse and received multimodal conservative treatment. Because of progression into a full-thickness RP with ulcerations, the parents opted for surgical management. Well’s procedure was performed at the age of 4 years. Using four ports, the rectum was circumferentially mobilized down to the pelvic floor and pulled inside. A 5 × 5 cm prolene mesh was tacked to the sacrum, enveloped posteriorly 270° around the rectum, fixed with interrupted prolene sutures on both edges and carefully covered with peritoneum. Any redundant external mucosa was excised from a perineal approach. There were no intra- and postoperative complications. Within 1.5 years of follow-up the boy had voluntary bowel movements and was toilet trained. No prolapse recurrence could be observed nor provoked. Discussion We present the first pediatric case of IRP secondary to laparoscopic ARM repair which has been successfully treated by combined Well’s procedure and perineal mucosal resection. Conclusion Well’s procedure is a successful technique and should be further explored in children with ARM and IRP.

Since the introduction of thoracoscopic repair for congenital diaphragmatic hernia (CDH) by Van der Zee and Bax in 1995 [1], this approach has always been one of the most challenging in neonatal MIS. For more than two decades, technology improved and made it increasingly feasible, but surgical complications like recurrence rate never reached open standards. It seemed that minimal invasive surgery (MIS) of CDH had a performance problem [2]. Furthermore observations of intraoperative hypercapnia and severe acidosis raised medical concerns [3]. Thus today, unlike other neonatal MIS procedures, CDH repair has not yet reached the status of a “gold standard” for the complete spectrum of patients [4]. Instead the academic discussion has identified key points of success calling for a “smart” selection of neonates and specialized teams. The following chapter will deal with such details to improve overall success rate in this very delicate group of patients.

Neuroblastoma (NB) is the most common extracranial pediatric solid tumor. Children suffering from high-risk and/or metastatic NB often show no response to therapy, and new therapeutic approaches are urgently needed. Malignant tumor development has been shown to be driven by the dysregulation of eukaryotic initiation factors (eIFs) at the translation initiation. Especially the activity of the heterotrimeric eIF4F complex is often altered in malignant cells, since it is the direct connection to key oncogenic signaling pathways such as the PI3K/AKT/mTOR-pathway. A large body of literature exists that demonstrates targeting the translational machinery as a promising anti-neoplastic approach. The objective of this study was to determine whether eIF4F complex members are aberrantly expressed in NB and whether targeting parts of the complex may be a therapeutic strategy against NB. We show that eIF4AI is overexpressed in NB patient tissue using immunohistochemistry, immunoblotting, and RT-qPCR. NB cell lines exhibit decreased viability, increased apoptosis rates as well as changes in cell cycle distribution when treated with the synthetic rocaglate CR-1-31-B, which clamps eIF4A and eIF4F onto mRNA, resulting in a translational block. Additionally, this study reveals that CR-1-31-B is effective against NB cell lines at low nanomolar doses (≤20 nM), which have been shown to not affect non-malignant cells in previous studies. Thus, our study provides information of the expression status on eIF4AI in NB and offers initial promising insight into targeting translation initiation as an anti-tumorigenic approach for NB.

One-trocar laparoscopic appendectomy (OTA) is routinely adopted in children with acute appendicitis. In case of a difficult appendectomy, it is necessary to add additional trocar/s to safely complete the procedure. This technique is called multiport hybrid laparoscopic appendectomy (HLA). We aimed to compare the outcome of multiport HLA versus OTA. METHODS: We retrospectively reviewed the data of 1,092 patients underwent LA in 5 European centers of pediatric surgery in the last 5 years. We compared 2 groups: G1 of 575 patients (52.6 %) (average age 10 years) underwent OTA and G2 of 517 patients (47.4 %) (average age 8.2 years) underwent multiport HLA. RESULTS: No intra-operative complications occurred in both groups. An additional pathology was treated in 12 cases (8 Meckel's diverticulum, 2 carcinoids, 2 ovarian cysts) in G2. Operative time was significantly shorter in G2 compared to G1 (47.8 vs 58.6 min; p < .001). The average analgesic requirement was significantly shorter in G2 compared to G1 (44 vs 56 h; p < .001). As for postoperative complications, the incidence of port-site infections was similar between the two groups, while the incidence of postoperative abdominal abscesses (PAA) was significantly higher in G1 compared to G2 (4.7 vs 0.2 %; p < .001). The cosmetic outcome was excellent in all patients of both groups. A subgroup analysis between complicated and uncomplicated appendicitis showed that only in complicated cases, the average operative time, the average VAS pain score, the average analgesic requirements and the incidence of PAA were significantly higher in OTA group compared to multiport HLA group (p < .001). CONCLUSIONS: Our results suggest that OTA is a valid and safe procedure for the uncomplicated cases, while additional trocars are required in case of complicated appendicitis. Multiport HLA significantly reduces the operative time, the incidence of abdominal abscesses and the analgesic requirements compared to OTA.

Background Sepsis and severe focal infections represent a substantial disease burden in children admitted to hospital. We aimed to understand the burden of disease and outcomes in children with life-threatening bacterial infections in Europe. Methods The European Union Childhood Life-threatening Infectious Disease Study (EUCLIDS) was a prospective, multicentre, cohort study done in six countries in Europe. Patients aged 1 month to 18 years with sepsis (or suspected sepsis) or severe focal infections, admitted to 98 participating hospitals in the UK, Austria, Germany, Lithuania, Spain, and the Netherlands were prospectively recruited between July 1, 2012, and Dec 31, 2015. To assess disease burden and outcomes, we collected demographic and clinical data using a secured web-based platform and obtained microbiological data using locally available clinical diagnostic procedures. Findings 2844 patients were recruited and included in the analysis. 1512 (53·2%) of 2841 patients were male and median age was 39·1 months (IQR 12·4–93·9). 1229 (43·2%) patients had sepsis and 1615 (56·8%) had severe focal infections. Patients diagnosed with sepsis had a median age of 27·6 months (IQR 9·0–80·2), whereas those diagnosed with severe focal infections had a median age of 46·5 months (15·8–100·4; pInterpretation Mortality in children admitted to hospital for sepsis or severe focal infections is low in Europe. The disease burden is mainly in children younger than 5 years and is largely due to vaccine-preventable meningococcal and pneumococcal infections. Despite the availability and application of clinical procedures for microbiological diagnosis, the causative organism remained unidentified in approximately 50% of patients.

Respiratory manifestations of gastroesophageal reflux disease (GERD), particularly chronic cough, are being recognized with increased frequency in children. This survey aimed to investigate the efficacy of laparoscopic Nissen fundoplication for treatment of GERD-related respiratory symptoms not responsive to medical therapy in neurological normal children.We collected data of children with GERD-related respiratory complaints not responsive to medical therapy who underwent laparoscopic Nissen fundoplication in four European centers of Pediatric Surgery over a 10-year period. We excluded children with neurological impairment.A total of 220 laparoscopic Nissen procedures were performed in the period 2005-2015. Twenty-four (12 boys and 12 girls, average age 9.5 years) out of the 220 patients (10.9%) presented with chronic cough and other respiratory manifestations, including asthma, reactive airway disease, and recurrent pneumonia. Average operative time was 65 minutes (range 45-100). As for postoperative complications, two tight wraps requiring endoscopic dilatation (IIIb Clavien) and two relapses of GERD for slipped Nissen requiring reoperation (IIIb Clavien) were recorded. None of these complications occurred in the group of patients with GERD-related respiratory symptoms. At follow-up evaluation, respiratory symptoms disappeared with a significant improvement of quality of life scoring (I Grade Visick) in 22/24 patients (91.6%).Our results confirm that GERD should be investigated as one of the possible etiologic factors in any child with persistent respiratory complaints. In patients with symptoms not responsive to medical therapy, laparoscopic Nissen fundoplication is the treatment of choice with a very high success rate (90% in our series), a very low morbidity, a significant improvement in airway symptoms, and a marked reduction in the need for medications.

BACKGROUND: There is a limited and conflicting evidence about the most appropriate method for appendiceal stump closure during laparoscopic appendectomy (LA). We aimed to compare endoloop (EL) versus endostapler (ES) for stump closure during LA for complicated perforated appendicitis in children. METHODS: We retrospectively reviewed the records of 708 patients (463 boys and 245 girls with an average age of 9.8 years) who underwent LA for complicated appendicitis in 5 international centers of Pediatric Surgery over a 5-years period (January 2011-December 2016). The appendix was perforated with localized peritonitis in 470 cases and diffuse peritonitis in 238 patients. EL was used in 374 cases (G1), whereas ES was adopted in 334 cases (G2). RESULTS: No intra-operative complication occurred in both groups but 5 conversions to open surgery were reported in G1 (1.3%) and 4 in G2 (1.1%) (OR 1.1; 95% CI 0.30-4.19). Use of EL was significantly associated with higher incidence of intra-abdominal abscess (OR 1.36; 95% CI 0.84-2.18), postoperative ileus (OR 3.61; 95% CI 0.76-17.11), and re-operations/readmissions (OR 6.46; 95% CI 1.46-28.62) compared to ES. The average cost of supplies for LA was significantly higher in G2 (€ 915.60) compared to G1 (€ 578.36) (p = 0.0001). The average cost of re-operations/readmissions was significantly higher in G1 (€ 4.091,39) compared to G2 (€ 2.127,88) (p = 0.0001) (OR 1.72; 95% CI 1.47-2.01). CONCLUSIONS: Our study is the first in the pediatric population to demonstrate that the method used for appendiceal stump closure may influence the outcome of LA in complicated appendicitis. Although ES is more expensive compared to EL, our results demonstrated that appendix stump closure should be performed using ES rather than EL in complicated perforated appendicitis since its use was associated with a lower incidence of postoperative intra-abdominal abscess and postoperative ileus and lower re-operations and readmissions rates and costs.

Aim of the Study Severe lung hypoplasia and persistent pulmonary hypertension are the main determining factors of survival in infants with congenital diaphragmatic hernia (CDH). The oxygenation index (ratio of delivered oxygen and its arterial level) closely reflects lung function. Single-institution studies have reported that best oxygenation index on day 1 of life (BOI-d1) is the most reliable postnatal predictor of survival in CDH. The aim of this study was to evaluate the predictive value of BOI-d1 in four disparate high volume centers in Europe. Methods A retrospective, multicenter study of infants with CDH born between 2000 and 2009 in four European tertiary institutions was conducted. Ethical approval was obtained from institutional review boards. Centers no. 1 and. 4 offered extracorporeal membrane oxygenation (ECMO), whereas center no. 3 offered fetal endoluminal tracheal occlusion (FETO) in fetuses defined as poor prognosis (lung-to-head ratio [LHR] ≤ 1.0 and “liver-up” position). Prenatal LHR and perinatal variables, including gestational age, birth weight, defect side, liver position, BOI-d1, and patch requirement, were analyzed. Receiver operating characteristic curves were used to determine cutoff values for continuous variables. Comparison was made between survivors and nonsurvivors using univariate analysis and logistic regression analysis, p Results A total of 235 infants (center no. 1, n = 29; no. 2, n = 64; no. 3, n = 113; and no. 4, n = 29) were included. One infant required (2%) ECMO and 66 (28%) had FETO. LHR was available in 83 patients (36%). Overall survival (discharge from hospital) and 28-day survival were 67.6% (n = 159) and 72.3% (n = 170), respectively. Univariate analysis showed that significant categorical predictors of 28-day survival were liver-down position (p 1 (p = 0.003), and primary repair (p = 0.02) but not defect side (p = 0.83). Area under the receiver operating characteristic (AUROC) curve for continuous variables; gestational age, birth weight, and BOI-d1 were 0.70, 0.68, and 0.88, respectively. AUROC for BOI-d1 (28-day survival) was 0.91 and had sensitivities (73 and 91%) and specificities (92 and 80%) for cutoffs of 40 and 82, respectively. Conclusion This multicenter study showed, that except from the defect side, all the prenatal variables studied have predictive value but the most useful is BOI-d1. This is simple to calculate and represents an excellent marker for lung function and a reliable early postnatal predictor of survival.

Only ten cases of neonatal congenital segmental dilatation (CSD) of the colon have been described so far. We present a full-term female newborn with trisomy 21, ventricular septal defect, and gross abdominal distension. Plain abdominal radiographs revealed a huge cystic lesion occupying the left hemiabdomen. Upon laparotomy on day 4 a CSD of the distal sigmoid and proximal rectum was confirmed and resected. The proximal colon was exteriorized and the distal part closed as a Hartmann pouch. Histology confirmed a huge segmental dilatation of the sigmoid without dysganglionosis or pseudodiverticula, but normal intestinal architecture. After correction of the ventricular septal defect a low rectal end-to-end anastomosis could be performed at an age of 5 months. The postoperative course was uneventful. CSD of the sigmoid colon is extremely “rare to meet” and a “challenge to treat” in the newborn period, but clinical awareness of this entity prompts pediatric surgical success.

Proton pump inhibitors (PPIs) are the standard therapy for gastroesophageal reflux disease. In adults, PPI treatment is associated with Clostridium difficile infections (CDI). In contrast to adults the microbiome of infants develops from sterility at birth toward an adult-like profile in the first years of life. The effect of PPIs on this developing microbiome has never been studied. The aim of the present study was to determine the effect of oral PPIs on the fecal microbiome in infants with gastroesophageal reflux disease (GERD). In this prospective longitudinal study 12 infants with proven GERD received oral PPIs for a mean period of 18 weeks (range 8–44). Stool samples were collected before (“before PPI”) and 4 weeks after initiation of PPI therapy (“on PPI”). A third sample was obtained 4 weeks after PPI discontinuation (“after PPI”). The fecal microbiome was determined by NGS based 16S rDNA sequencing. This trial was registered with clinicaltrials.gov (NCT02359604). In a comparison of “before PPI” and “on PPI” neither α- nor β-diversity changed significantly. On the genus level, however, the relative abundances showed a decrease of Lactobacillus and Stenotrophomonas and an increase of Haemophilus. After PPI therapy there was a significant increase of α- and β-diversity. Additionally, the relative abundances of the phyla Firmicutes, Bacteroidetes, and Proteobacteria were significantly changed and correlated to patients' age and the introduction of solid foods. PPI treatment has only minor effects on the fecal microbiome. After discontinuation of PPI treatment the fecal microbiome correlated to patients' age and nutrition.

Plexiform fibromyxoma is a very rare mesenchymal tumor of the stomach, found almost exclusively in the antrum/pylorus region. The most common presenting symptoms are anemia, hematemesis, nausea and unintentional weight loss, without sex or age predilection. We describe here two cases of plexiform fibromyxoma, involving a 16-year-old female and a 34-year-old male. Both patients underwent complete resection (R0) by distal gastrectomy and retrocolic gastrojejunostomy (according to Billroth 2); for both, the postoperative course was uneventful. Histology showed multiple intramural and subserosal nodules with characteristic plexiform growth, featuring bland spindle cells situated in an abundant myxoid stroma with low mitotic activity. Immunohistochemistry showed α-smooth muscle actin-positive spindle cells, focal positivity for CD10, and negative staining for KIT, DOG1, CD34, S100, β-catenin, STAT-6 and anaplastic lymphoma kinase. One of the cases showed focal positivity for h-caldesmon and desmin. Upon follow-up, no sign of disease was found. In the differential diagnosis of plexiform fibromyxoma, it is important to exclude the more common gastrointestinal stromal tumors as they have greater potential for aggressive behavior. Other lesions, like neuronal and vascular tumors, inflammatory fibroid polyps, abdominal desmoid-type fibromatosis, solitary fibrous tumors and smooth muscle tumors, must also be excluded.

Congenital infantile fibrosarcoma (CIF) is a rare malignant mesenchymal tumor and only 14 cases have been reported with gastrointestinal manifestation. We report about a female newborn delivered per emergency cesarean section at 34 weeks of gestation. Postnatally, she rapidly developed an acute abdomen and sonographic evidence of intestinal perforation requiring laparotomy on the first day of life. A perforated 2 × 3 cm sized spherical tumorous structure of the jejunum was identified. Due to unknown histopathology at this point and unclear resectional margins, she received a temporary ileostomy, which was closed two months later. Histopathology revealed a congenital intestinal fibrosarcoma without the characteristic ETV6-NTRK3 fusion transcript. In conclusion, this rare tumor must be considered as differential diagnosis of intestinal perforations in newborns.

Introduction The “metabolic competition” for nutrients between cancer cells and the patient has emerged as an important research area. For pediatric oncology, it remains unclear whether the neuroendokrine regulation of appetite by gastrointestinal hormones such as ghrelin “eat”, GLP-1 (glucagon-like peptide, “do not eat”), and PYY (peptide tyrosine–tyrosine, “do not eat”) is influenced by tumor growth. Material and Methods In a prospective randomized study, human hepatoblastoma (HB) and neuroblastoma (NB) cells (3 × 106) were transplanted into the abdominal wall of immune-incompetent (nu/nu) rats (ethic committee approval: TVV43/11). Sham-operated animals received cell culture medium only. Tumor growth was allowed for 8 weeks. Then, all the animals underwent a 2-hour oGTT (oral glucose tolerance test) and were assessed for serum levels of glucose, insulin, ghrelin, GLP-1, and PYY. Finally, all tumor masses and adipose tissues were excised and calculated. Results Total body weight (including tumor masses) differed for HB (329 + 31 g), but not for NB (358 + 22 g) compared with Sham (361 + 35 g). Subcutaneous adipose tissue was significantly decreased for both the tumor groups (HB = 2.6 g, NB = 2.1 g, and Sham = 3.5 g). Only for NB, fasting glucose (3.4 + 0.6 mmol/L) and insulin (0.89 + 0.11 ng/mL) levels were significantly decreased compared with Sham (4.4 + 0.6 mmol/L; 1.19 + 0.36 ng/mL) only. During the oGTT (all data calculated as area under the curve, AUC) glucose levels were significantly increased for HB (104 ± 10) and NB (102 ± 13) compared with Sham (84 ± 3), but insulin levels remained similar for either group. Triglyceride levels were increased for HB (0.51 mmol/L) and especially NB (0.73 mmol/L) compared with Sham (0.34 mmol/L). Inflammatory parameters did not differ between the groups. Total ghrelin levels were significantly increased for NB (111 ± 10) and altered for HB (102 ± 15) compared with Sham (84 ± 8). Vice versa GLP-1 was statistically decreased in HB (92 ± 7) and NB (88 ± 12) compared with Sham (127 ± 13). Finally, PYY levels were nonsignificantly reduced for HB (117 ± 5) and NB (120 ± 4) compared with Sham (146 ± 12).

Objective Serum uric acid (sUA) is believed to contribute to the pathogenesis of metabolic comorbidities like hypertension, insulin-resistance (IR) and endothelial dysfunction (EDF) in obese children. The present pilot study investigated the association between sUA concentrations and loss of body weight following laparoscopic sleeve gastrectomy (LSG) or laparoscopic Roux-en-Y-gastric bypass (RYGB) in severely obese adolescents. Materials/Methods 10 severely obese adolescents underwent either LSG (n = 5) or RYGB (n = 5). 17 normal weight, healthy, age- and gender-matched adolescents served as a normal weight peer group (NWPG). Pre- and 12 months postoperatively, sUA and relevant metabolic parameters (glucose homeostasis, transaminases, lipids) were compared. Results Preoperatively, sUA was significantly elevated in patients with severe obesity compared to NWPG. Twelve months after LSG and RYGB, a significant decrease in sUA, BMI, CVD risk factors, hepatic transaminases, and HOMA-IR was observed. Reduction in SDS-BMI significantly correlated with changes in sUA. Conclusions sUA levels and metabolic comorbidities improved following bariatric surgery in severely obese adolescents. The impact of changes in sUA on long-term clinical complications of childhood obesity deserves further study.

Objective: We studied the short-term response of autonomic control to delivery in normal pregnancies and pregnancies with preeclampsia (PE). Methods: Fourteen healthy pregnant women and 13 women with PE were monitored within four days before and four days after delivery and compared to values of 14 non-pregnant women as controls using high-resolution electrocardiogram and noninvasive continuous blood pressure monitoring. Results: In PE, blood pressure remained elevated four days postpartum, but markers for arterial stiffness normalized. In contrast, none of heart rate variability and baroreflex sensitivity parameters, altered due to either pregnancy or disease, were normalized 96 h after delivery. Conclusion: Four days after delivery, the maternal cardiovascular system is still strongly affected by pregnancy independent of the health status.

Objective HDL regulates endothelial function via stimulation of nitric oxide production. It is documented that endothelial function is impaired in obese adolescents, and improved by lifestyle interventions (LI). Design and Methods HDL function in obese adolescents and the impact of LI or Roux-en-Y gastric bypass surgery (RYGB) was assessed. HDL was isolated from 14 adolescents with normal body mass index (HDLcontrol), 10 obese (HDLobese) before and after 6 month LI, and five severe obese adolescents before and one year after RYGB. HDL-mediated phosphorylation of endothelial nitric oxide synthase (eNOS)-Ser1177, eNOS-Thr495, and PKC-sII was evaluated. In addition the HDL proteome was analyzed. Results HDLobese-mediated eNOS-Ser1177 phosphorylation was reduced, whereas eNOS-Thr495 phosphorylation increased significantly when compared to HDLcontrol. No impact of obesity was observed on PKC-sII phosphorylation. LI and RYGB had no impact on HDL-mediated phosphorylation of eNOS and PKC-sII. A principle component plot analysis of the HDL particle separated controls and severe obese, whereas the interventions did not trigger sufficient differences to the HDL proteome to permit distinction. Conclusion These results demonstrated that HDL-function is impaired in obese adolescents, and that LI or RYGB did not correct this dysfunction. This might be an argument for developing earlier prevention strategies in obese adolescents to avoid HDL dysfunction.

Objective Data on safety, effectiveness and amelioration of metabolic comorbidities are scarce for adolescents undergoing laparoscopic sleeve gastrectomy (LSG). Methods Data on a 17 year-old female who underwent LSG due to morbid obesity and quantification of visceral adipose tissue (VAT)/hepatic fat (HF) by MRI scan are presented. In addition, a review of the literature related to LSG in adolescent obesity is provided [search terms: laparoscopic sleeve gastrectomy, adolescence, obesity]. Results Pre-existing comorbidities in our patient included insulin resistance, NASH (HF: 28%) and hyperuricemia. BMI dropped from 52.9 kg/m2 to 40.4 and 35.0 kg/m2 after 6 and 24 months, respectively, following LSG. VAT dropped by 49.2% and by 71.5% within 6 and 24 months, accompanied by a decrease of HF to 1.53% and to 0.85%, respectively. Improvements of metabolic parameters towards normal range, which were reached within 6 months following LSG, could be maintained for 2 years. The review of the literature revealed that 9 original papers on LSG in adolescent obesity are available to date, ranging from single case presentations to case series of 108 patients and including children and adolescents 8–18 years. Conclusions LSG is a safe and – in the short term – effective bariatric procedure for adolescent obesity (follow up 24 months). Long-term results with larger numbers of patients and involving additional features, such as quantification of VAT and HF as markers of metabolic risk and insulin resistance, are warranted to further understand the effectiveness and safety and to further explore the long-term benefits of this procedure in adolescence.

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) represent major therapeutic challenges, frequently associated with serious morbidities following surgical repair. The aim of this longitudinal study was to assess temporal changes in morbidity and mortality of patients with EA/TEF treated in a tertiary-level center, focusing on postoperative complications and their impact on long-term gastroesophageal function. One hundred nine consecutive patients with EA/TEF born between 1975 and 2011 were followed for a median of 9.6 years (range, 3–27 years). Comparative statistics were used to evaluate temporal changes between an early (1975–1989) and late (1990–2011) study period. Gross types of EA were A (n = 6), B (n = 5), C (n = 89), D (n = 7), and E (n = 2). Seventy (64.2%) patients had coexisting anomalies, 13 (11.9%) of whom died before EA correction was completed. In the remaining 96 infants, surgical repair was primary (n = 66) or delayed (n = 25) anastomosis, closure of TEF in EA type E (n = 2), and esophageal replacement with colon interposition (n=2) or gastric transposition (n=1). Long-gap EA was diagnosed in 23 (24.0%) cases. Postoperative mortality was 4/96 (4.2%). Overall survival increased significantly between the two study periods (42/55 vs. 50/54; P = 0.03). Sixty-nine (71.9%) patients presented postoperatively with anastomotic strictures requiring a median of 3 (range, 1–15) dilatations. Revisional surgery was required for anastomotic leakage (n = 5), recurrent TEF with (n = 1) or without (n=9) anastomotic stricture, undetected proximal TEF (n = 4), and refractory anastomotic strictures with (n = 1) or without (n = 2) fistula. Normal dietary intake was achieved in 89 (96.7%) patients, while 3 (3.3%) remained dependent on gastrostomy feedings. Manometry showed esophageal dysmotility in 78 (84.8%) infants at 1 year of age, increasing to 100% at 10-year follow-up. Fifty-six (60.9%) patients suffered from dysphagia with need for endoscopic foreign body removal in 12 (13.0%) cases. Anti-reflux medication was required in 43 (46.7%) children and 30 (32.6%) underwent fundoplication. The rate of gastroesophageal reflux increased significantly between the two study periods (29/42 vs. 44/50; P = 0.04). Twenty-two (23.9%) cases of endoscopic esophagitis and one Barrett’s esophagus were identified. Postoperative complications after EA/TEF repair are common and should be expertly managed to reduce the risk of long-term morbidity. Regular multidisciplinary surveillance with transitional care into adulthood is recommended in all patients with EA/TEF.

Helium is used as an insufflation gas to avoid the negative properties of carbon dioxide (CO(2)), such as CO(2) accumulation, acidosis, and tachycardia, particularly in the case of insufficient respiratory function, seen also in infancy. Any laparoscopic procedure carries the risk of a gas embolism.Seven anesthetized piglets (weighing 9.9-12.8 kg), randomized into three groups, served as models for pre-teenage children. Three piglets received a CO(2) embolism, followed by a helium embolism of 2 mL/kg, respectively. Helium was administered to three piglets, whereas both gases were repeatedly administered alternately to one piglet. The embolisms were administered for 30 seconds via a central venous line. Cardiac output was measured using the thermodilution method. The observation period for each embolism was 60 minutes in Groups 1 and 2 and 15 minutes in Group 3.All animals survived CO(2) embolisms. Four of the six piglets died after helium embolisms. Following helium embolisms there was a prompt initial decrease in the end-tidal CO(2) pressure and an initial increase in the pulmonary arterial pressure. A further decrease in arterial blood pressure was prevented by a compensatory increase in the heart rate and appeared just before death. After only 5 minutes cardiac output showed a 25% decline from the initial value. Helium embolisms led to a severe increase in the pulmonary dead space.Embolisms with the smallest amounts of helium administered via direct venous puncture have an immediate lethal impact. Extended perioperative monitoring and trocar placement under vision should be performed.

Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy and its prognosis has considerably improved over the past 2 decades due to new therapeutic approaches. In some cases, however, it can develop very rapidly and cause possibly fatal complications. We report on the case of an 11-year-old boy with ALL, who rapidly developed severe lactic acidosis and abdominal compartment syndrome. He died of multiorgan failure only 5 days after diagnosis of ALL had been established. Autopsy revealed systemic leukemic infiltrations. We suppose that the mass of tumor cells induced a cascade of metabolic and endocrine reactions, which not only triggered the rapid progression of the disease but were also accountable for the lack of response to treatment. The pathophysiology of abdominal compartment syndrome as a rare and in our case ultimately fatal complication of ALL is described.

Objective We conducted a meta-analysis to assess the correlation of lung volume and liver position measured by magnetic resonance imaging (MRI) with survival until discharge in fetuses with isolated congenital diaphragmatic hernia (CDH). Method Systematic searches of MEDLINE and EMBASE from 1 January 1980 to 10 December 2010 were performed. Studies correlating total fetal lung volumes (TFLV, observed/expected (O/E) TFLV) and/or liver position by fetal MRI to survival in expectantly managed fetuses with CDH were included. Data on the side of the defect, position of the liver, TFLV, O/E TFLV, gestational age (GA) at MRI, GA and weight at birth were collected. Odds ratio (OR) for dichotomous data, mean differences (MD) or standardized mean differences (SMD) for continuous variables were determined using RevMan 5.0 software. Results Nineteen studies (n = 602 fetuses) were included. Survival was associated with left-sided defects (OR 2.52; p = 0.01), “liver down” (OR 0.18; p

Neuroblastomas are malignant tumors of the sympathetic nervous system. Areas of manifestation most commonly involve the abdomen, neck, thorax and pelvis. Primary renal neuroblastomas are extremely rare, only a few case reports exist worldwide, and even those are discussed controversially. We present the case of a 6-year-old girl with a renal tumor and a tumor thrombus extending into the right atrium, which radiologically appeared to be a Wilms tumor. Since the lesion did not respond to nephroblastoma-specific therapy, a biopsy from one of the liver metastases was taken, revealing the revised diagnosis of a clear cell renal cell carcinoma. Histopathology of the reference center, however, described a primary renal neuroblastoma. After adjusting the chemotherapy tumornephrectomy including the complete venous thrombus could be performed without any complications. Neuroblastoma originating from a kidney is an absolute rarity that can easily be misdiagnosed as Wilms tumor, especially, if a typical tumor thrombus with extension into the inferior vena cava is seen. Therefore neuronspecific enolase in serum as well as vanillylmandelic acid and homovanillic acid in the urine should be determined in all patients when Wilms tumor is assumed. To the best of our knowledge, this is the first published case of a primary renal neuroblastoma with a tumor thrombus extending into the right atrium.

Object Many ophthalmological abnormalities are described in conjunction with hydrocephalus. The results of visual field diagnosis remain a matter of further discussion. The aim of this study was to investigate visual field deficits in children with shunt-treated hydrocephalus. Methods All children over 6 years of age treated for hydrocephalus at the authors' institute between December 2007 and December 2008 were included in the study. The children underwent an ophthalmological investigation for strabismus and binocular function, ophthalmoscopy, visual acuity, and refraction. The special focus was the visual field diagnosis, which the authors established in all children with cognitive conditions. The investigation was made by using the Goldmann visual field examination (kinetic perimetry). Children with and without visual field defects were compared concerning age at the time of ophthalmological examination, genesis of hydrocephalus, and frontooccipital horn ratio measured on current CT or MR images. Results Complete investigations were undertaken in 56 children (24 girls and 32 boys, mean age 15.1 years). The following orthoptic pathological entities were diagnosed: 29 children have a strabismus in 29 cases, 17 of these have an exotropia, 12 an esotropia, 4 children a hypotropia, 2 a hypertropia and 3 children a heterophoria. A nystagmus was found in 10 children. The ocular fundus investigation showed 13 children with an optic nerve atrophy. A visual field diagnosis was possible in 44 of the 56 patients and was incomplete in 12 patients with cognitive deficits or inadequate compliance. In 24 of 42 children there was a concentric visual field constriction between 10° and 50° out of the center. Children with visual field deficits were older than those with a normal visual field (p = 0.051). Nine of 10 children with postmenigitic hydrocephalus had a visual field defect (p = 0.025). In children with visual field defects the frontooccipital horn ratio was significantly higher (p = 0.013). Conclusions The results suggest that children with shunt-treated hydrocephalus have a higher risk of having ophthalmological abnormalities. Visual field deficits are often a problem in these patients. A diagnostic visual field examination can complete the ophthalmological monitoring in patients with hydrocephalus, especially in patients with large ventricles. Children with postmeningitic hydrocephalus should be ophthalmologically monitored more frequently and intensively.

Congenital diaphragmatic hernia (CDH) occurs sporadically with an incidence of 1 : 2 500 live births. Despite the progress in neonatal intensive care, CDH remains associated with a mortality of at least 30 % in isolated cases. The in essence surgically correctable defect of the diaphragm en-ables the prenatal herniation of abdominal organs into the thoracic cavity. The resulting abnormal development of the airways and pulmonary vessels causes neonatal respiratory insufficiency and persistent pulmonary hypertension. The condi-tion can be diagnosed prenatally and the degree of pulmonary hypoplasia, which determines the postnatal course, can be measured to make an -individual prognosis. In severely affected patients, prenatal surgery may improve neonatal outcome by reversing pulmonary hypoplasia. This is currently implemented by percutaneous fetoscopic endoluminal tracheal occlusion (FETO) to trigger fetal lung growth. Although there are no maternal complications, preterm rupture of the membranes remains the major drawback of the procedure (20 % < 34 weeks). However, as compared to historical controls of a similar severity, survival as well as early neonatal morbidity are significantly improved by FETO. As a consequence, a multicentre randomised-controlled trial in fe-tuses with mod-erate hypoplasia on FETO com-pared to expectant management has been started ( www.totaltrial.eu ). Primary outcome measure is survival without chronic lung disease (i. e., with-out bronchopulmonary dysplasia). A trial in severely affected -fetuses with survival as main outcome is cur-rently under review by ethics committee. A standardised neonatal management en-ables optimal treatment and multicentre com-patibility. It remains to be proven if fetoscopic surgery can maintain a solid position in the prenatal treatment of CDH to improve both mortality and morbidity of the affected children.

Object During recent years, several biomarkers have been introduced for use in the diagnosis of traumatic brain injury (TBI). The primary objective of this investigation was to determine if S100B (or S100 calcium-binding protein B) and neuron-specific enolase (NSE) serum concentrations can effectively be used to discriminate between symptomatic and asymptomatic children with minor head trauma. Methods The authors conducted a prospective clinical study that involved patients age 6 months to 15 years who had sustained minor head trauma. Children with concomitant extracranial injuries were excluded. Blood samples were obtained within 6 hours of injury to measure S100B and NSE levels in serum. The authors defined 2 diagnostic groups: a mild TBI group (patients with Glasgow Coma Scale [GCS] scores of 13–15) in whom there were clinical signs of concussion (short loss of consciousness, amnesia, nausea, vomiting, somnolence, headache, dizziness, or impaired vision) and a head contusion group (patients with a GCS score of 15) in whom symptoms were absent. Both S100B and NSE concentrations were compared between the 2 groups. Secondary end points were defined as follows: correlation of S100B/NSE and a) the presence of scalp lacerations, b) GCS score, c) age, and d) correlation between S100B and NSE. Results One hundred forty-eight patients were enrolled (53 in the contusion group, 95 in the mild TBI group). After adjusting for differences in age and time of injury to blood sample withdrawal, there was no significant difference in S100B or NSE between patients in the 2 groups. Scalp lacerations and GCS score had no affect on posttraumatic S100B or NSE concentrations. The correlation between S100B and NSE was significant. Both markers showed a significant negative correlation with age. Conclusions The authors demonstrated that S100B and NSE do not discriminate between symptomatic and asymptomatic children with minor head injury. There seem to be limitations in marker sensitivity when investigating pediatric patients with mild TBI.

Bardet-Biedl syndrome (BBS) is a rare genetic disorder characterized by a wide range of phenotypic variability and associated with the development of life-threatening obesity. Birth weight tends to be normal, but rapid weight gain begins after the first year, probably due to polyphagia rather than abnormalities in energy metabolism. A morbidly obese 16-year-old male patient with BBS was referred to our institution, after nonsurgical methods of weight control had failed, for surgical treatment of his obesity. His preoperative body mass index (BMI) was 52.28 kg/m(2) (height, 1.84 m; weight, 177 kg) and was above the 99th centile for age and gender. The patient underwent laparoscopic Roux-en-Y gastric bypass (RYGBP). The postoperative period was uneventful. Three and a half years after the operation, the patient's weight has decreased to 118 kg (BMI, 34.85 kg/m(2)), while significant improvement in his hypertension, hyperuricemia, and mobility has been noted. In our BBS patient, RYGBP proved to be safe and effective; nevertheless, longer follow-up is required to evaluate the weight loss durability and to assess the lasting beneficial effect of surgical intervention on genetically determined co-morbidities.