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Aim: The aims of this study were to evaluate health-related quality of life (HRQoL) in children with sacrococcygeal teratoma and to explore the effect of the scar on physical, emotional and behavioral aspects., Methods: A cohort of children operated on for sacrococcygeal teratoma between 2000 and 2013 at Lund University Hospital, Sweden, and their parents were interviewed. HRQoL was evaluated with PedsQL, and scar satisfaction was estimated through Patient Observer Scar Assessment Score (POSA)., Results: All eligible children (n = 17) were included (100% response rate). Median age was 7.3 years (range 3.5-16.0). Mean total PedsQL score was 92.3 (range 72.0 to 99.0). Patients with comorbidity scored lower (87.5) than those without (95.0) (p < 0.05). Pain during sitting down was reported by two (20%) patients, and itching was reported by another two patients (20%) aged > 8 years. No children reported that they avoided situations due to the scar, and most (80% of children and 90% of parents) reported absent or only mild negative emotions when considering the scar., Conclusion: Children with sacrococcygeal teratoma had a good overall HRQoL, but comorbidity reduced the outcome. A few children reported scar-related impact on physical, behavioral and emotional aspects.

Background: Incidence and long-term outcomes of choledochal malformations (CMs) in children remain unclear., Methods: Clinical characteristics, operative details, complications, and follow-up data were collected from eight pediatric surgical centers in Sweden, Norway, Denmark, and Finland, which also answered a questionnaire addressing management practices., Results: During 2000-2017, 126 pediatric CMs were diagnosed, corresponding an incidence of 1:37,400. Diagnostic, treatment, and follow-up practices varied markedly. Of patients with complete clinical data (n = 119), 85% and 11% had type I and IV CMs and were managed by open hepaticojejunostomy at median age of 2.5 (interquartile range 0.46-5.8) years. Associated malformations were more common in fusiform and type IV (23%) than cystic CMs (8%, p = 0.043). Pancreaticobiliary maljunction was more frequently confirmed in patients presenting with pancreatitis (26% vs. 7%, p = 0.005) and with fusiform CMs (56% vs. 25%, p = 0.001). Cholangitis/pancreatitis episodes, occurring in 12% during postoperative follow-up of 4.0 (2.0-7.9) years, associated with longer surveillance (OR 1.32, 95% CI 1.13-1.54, p < 0.001). However, only two thirds of centers continued follow-up until adulthood. No malignancies were reported., Conclusions: CM incidence was higher than traditionally reported among Western populations. Although open hepaticojejunostomy carries good short-term outcomes, long-term morbidity is noteworthy. Standardized evidence-based management strategies and long-term follow-up are encouraged., (Copyright © 2019 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.)

Purpose: To compare the two major complications, namely postoperative urethrocutaneous fistula and urethral stricture, between the Mathieu and tubularized incised plate (TIP) repair methods for distal hypospadias., Methods: In this meta-analysis, electronic databases were searched for comparative studies on the two techniques. The Oxford Centre for Evidence-based Medicine Levels of Evidence was used to evaluate the included studies. The main outcome measure was the frequency of postoperative fistula and urethral stricture. RevMan 5.3 was used for statistical analyses, with P < 0.05 indicating statistical significance., Results: A total of 17 studies, which included 1572 patients, met the inclusion criteria. The frequency of urethrocutaneous fistula did not differ between the Mathieu [115 (13%)] and TIP [90 (13%)] methods [odds ratio (OR) 1.1, 95% confidence intervals (CI) 0.6-1.9; P = 0.73)]. Urethral stricture was less frequent after the Mathieu [15 (2%)] method than after the TIP [37 (5%)] method (OR 0.5, 95% CI 0.3-0.8; P < 0.01), even after the subgroup analysis of eight randomized controlled trials was included. Overall, the quality of the included studies was determined to be satisfactory. The levels of evidence on which this review was based ranged from 1b to 2b using the CEBM Levels of Evidence., Conclusion: Compared with TIP repair, Mathieu repair for hypospadias had a significantly lower risk for urethral stricture; however, the risk for urethrocutaneous fistula was similar.

Background: The surgical repair of long-gap esophageal atresia (LGEA) is still a challenge and there is no consensus on the preferred method of reconstruction. We performed a systematic review of the surgical treatment of LGEA Gross type A and B with the primary aim to compare the postoperative complications related to the different methods within the first postoperative year., Methods: Systematic literature review on the surgical repair of LGEA Gross type A and B within the first year of life published from January 01, 1996 to November 01, 2016., Results: We included 57 articles involving a total of 326 patients of whom 289 had a Gross type A LGEA. Delayed primary anastomosis (DPA) was the most applied surgical method (68.4%) in both types, followed by gastric pull-up (GPU) (8.3%). Anastomotic stricture (53.7%), gastro-esophageal reflux (GER) (32.2%) and anastomotic leakage (22.7%) were the most common postoperative complications, with stricture and GER occurring more often after DPA (61.9% and 40.8% respectively) compared to other methods (p < 0.001)., Conclusion: The majority of patients in this review were managed by DPA and postoperative complications were common despite the surgical method, with anastomotic stricture and GER being most common after DPA., Level of Evidence: Systematic review of case series and case reports with no comparison group (level IV)., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background/purpose: Urinary tract function in children with Hirschsprung disease (HD) is rarely considered., Aim: to evaluate the prevalence of urinary tract anomalies and dysfunction in children with HD compared to controls., Methods: This was an observational cross sectional case-control study. Children with HD who underwent transanal endorectal pull-through technique (TERPT) from 2005 to 2017 were invited to participate. Ultrasound of the urinary tract was performed postoperatively. Children >4 years were asked to answer a urinary tract function questionnaire. Controls were age-matched healthy children. Ethical approval was obtained., Results: Seventy two children with HD and TERPT were included. Ultrasound was performed in 58 children (83%) post-TERPT. Ten anomalies were diagnosed in six children (10%). Structural anomalies included abnormal kidney size (7%), renal agenesis (2%), prominent calyces (2%) and renal pelvis anomaly (25). Probable acquired anomalies included hydronephrosis (2%), hydroureter (2%) and parenchymal damage (2%). One child had a prior nephrectomy owing to a Wilms' tumor. All 37 children >4 years (27 boys and 10 girls), median aged 8 years (range 4-12), answered the questionnaire as did 284 healthy controls (144 boys and 140 girls). Boys with HD reported a higher frequency of enuresis: 65% versus 9% (p = 0.001) and urinary tract infections: 18% versus 3% (p = 0.012). Girls with HD reported enuresis more frequently (60%) than healthy girls (7%) (p = 0.001). Children with HD with constipation reported enuresis more frequently (p = 0.038)., Conclusions: Urinary tract anomalies and dysfunction deserve attention in the follow-up of children with HD. We suggest screening for urinary tract anomalies and urinary tract symptoms in follow-up of children with HD., Type of Study: Treatment study., Level: III., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background  Gastrostomy placement in children is one of the most frequently performed pediatric surgical procedures and laparoscopic-assisted gastrostomy (LAG) is the preferred technique. Wound infection after LAG has become a significant concern due to the emergence of antibiotic resistance. The aim of this study was to describe the frequency of wound infection after LAG in children younger than 2 years of age and to identify the associated risk factors and the bacterial species involved. Methods  Information about wound infection, results from bacterial cultures, and type of antibiotic treatment used within 30 postoperative days after LAG were compiled for infants who underwent LAG from 2010 to 2017. A retrospective chart review was performed. Data was compiled from charts and from an electronic database containing prospectively collected data. A multivariate logistic analysis was used to explore potential risk factors. Preoperative antibiotic prophylaxis and postoperative local wound care were conducted according to standard procedures. Results  The 141 included infants underwent surgery at a median age of 10 months (range: 1-24). Thirty-eight (27%) patients had a clinically determined wound infection, bacteria were cultured from 26/38 (69%), and 30/38 (79%) received antibiotic treatment. The median interval from surgery to detection of a clinical wound infection was 14 days (range: 4-30). The most common microbes discovered were skin bacteria Staphylococcus aureus or Streptococcus pyogenes , but respiratory and intestinal bacteria were also found. Multivariate logistic regression analysis revealed no independent risk factors for infection such as age, gender, or underlying diagnosis. Conclusion  Infants have a high rate of postoperative clinical wound infection after LAG despite the use of preoperative antibiotic prophylaxis and intense local wound care. Gender, age at operation, and previous diagnoses were not found to be independent risk factors for wound infection.

Introduction  To assess differences in initial symptoms, treatments, and bowel function between children with Hirschsprung's disease (HD) with or without a cognitive dysfunction (CD). Materials and Methods  The study included children with HD who underwent transanal endorectal pull-through. A retrospective chart review was performed to collect data on patient characteristics, diagnosis, and treatment. Data on bowel symptoms in children older than 4 years without a colostomy were compiled during a cross-sectional, patient-reported follow-up. Results  Fifty-three children with HD were included; of these, 12 (23%) had CD. The median birth weight was lower, frequency of vomiting as the presenting symptom was lower, and time until the first contact with a pediatric surgeon was higher in children with CD than in those without (3,295 vs. 3,623 g, p  = 0.013; 28 vs. 66%, p  = 0.02; and 4 days vs. 1 day, p  = 0.048, respectively). At follow-up, 5 (15%) of 33 children aged over 4 years had CD. More children without CD had some ability to hold back defecation and sense the urge to defecate than those with CD ( p  = 0.002 and p  = 0.001, respectively). Conclusion  HD children who have CD present with different initial symptoms, have a delay in the first consultation with a pediatric surgeon, and experience poorer bowel function outcomes than HD children without CD. Therefore, HD children with CD should receive special attention in both clinical practice and research.

Background: The impact of perioperative care interventions on postreconstructive complications and short-term colorectal outcome in patients with anorectal malformation (ARM) type rectovestibular fistula is unknown., Methods: An ARM-Net consortium multicenter retrospective cohort study was performed including 165 patients with a rectovestibular fistula. Patient characteristics, perioperative care interventions, timing of reconstruction, postreconstructive complications and the colorectal outcome at one year of follow-up were registered., Results: Overall complications were seen in 26.8% of the patients, of which 41% were regarded major. Differences in presence of enterostomy, timing of reconstruction, mechanical bowel preparation, antibiotic prophylaxis and postoperative feeding regimen had no impact on the occurrence of overall complications. However, mechanical bowel preparation, antibiotic prophylaxis ≥48 h and postoperative nil by mouth showed a significant reduction in major complications. The lowest rate of major complications was found in the group having these three interventions combined (5.9%). Multivariate analyses did not show independent significant results of any of the perioperative care interventions owing to center-specific combinations. At one year follow-up, half of the patients experienced constipation and this was significantly higher among those with preoperative mechanical bowel preparation., Conclusions: Differences in perioperative care interventions do not seem to impact the incidence of overall complications in a large cohort of European rectovestibular fistula-patients. Mechanical bowel preparation, antibiotic prophylaxis ≥48 h, and postoperative nil by mouth showed the least major complications. Independency could not be established owing to center-specific combinations of interventions., Type of Study: Treatment study., Level of Evidence: III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background/purpose: The aims of this study were to evaluate bowel and urinary tract function, to identify predictors for dysfunctional outcome and to evaluate health-related quality-of life (QoL) in patients treated for sacrococcygeal teratomas (SCT)., Methods: Medical records of patients with SCT born between 1985 and 2015 treated at three Swedish pediatric surgical centers were reviewed. Questionnaires regarding urinary tract function, bowel function and QoL were sent to patients and parents. Different QoL instruments were used for the different age groups., Results: Totally 85 patients were identified. Four patients died in the neonatal period. Forty-nine patients answered the questionnaires (60%). Median age at follow-up was 8.9 years (range 3.6-28.8). Bowel dysfunction was reported by 36% and urinary tract dysfunction by 46% of the patients. Univariate analysis revealed that urinary tract dysfunction correlated with gestational age (p = 0.018) and immature histology (p = 0.008), and bowel dysfunction correlated with gestational age (p = 0.016) and tumor size (p = 0.042). Low gestational age was an independent predictor for both urinary tract and bowel dysfunction. Good or very good QoL was reported by 56% of children aged 4-7 years, 90% of children aged 8-17 years and 67% of the adults., Conclusion: Although a considerable proportion of bowel and urinary tract dysfunction was found, the reported QoL was good in a majority of the patients with SCT. Low gestational age was found to be a predictor for bowel- and urinary tract dysfunction., Level of Evidence: Level III., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Several surgical procedures have been described in the reconstruction of long-gap esophageal atresia (LGEA). We reviewed the surgical methods used in children with LGEA in the Nordic countries over a 15-year period and the postoperative complications within the first postoperative year., Methods: Retrospective multicenter medical record review of all children born with Gross type A or B esophageal atresia between 01/01/2000 and 12/31/2014 reconstructed within their first year of life., Results: We included 71 children; 56 had Gross type A and 15 type B LGEA. Delayed primary anastomosis (DPA) was performed in 52.1% and an esophageal replacement procedure in 47.9%. Gastric pull-up (GPU) was the most frequent procedure (25.4%). The frequency of chromosomal abnormalities, congenital heart defects and other anomalies was significantly higher in patients who had a replacement procedure. The frequency of gastroesophageal reflux (GER) was significantly higher after DPA compared to esophageal replacement (p = 0.013). At 1-year follow-up the mean body weight was higher after DPA than after organ interposition (p = 0.043)., Conclusion: DPA and esophageal replacement procedures were equally applied. Postoperative complications and follow-up were similar except for the development of GER and the body weight at 1-year follow-up. Long-term results should be investigated., Type of Study: Treatment study., Level of Evidence: Level III., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: A meta-analysis was performed to compare the rates of the major complications associated with two gastrostomy tube placement techniques in a pediatric population: laparoscopy-assisted gastrostomy (LAG) and percutaneous endoscopic gastrostomy (PEG)., Methods: The PubMed electronic database was queried for comparative studies of the two insertion techniques. The Newcastle-Ottawa scale (NOS) was used for the assessment of the quality and risk of bias in the included studies. The main outcome measure was the frequency of major complications defined as the need for reoperation within 30 days or death. RevMan 5.3, was used, with a p < 0.05 indicating statistical significance., Results: Eight studies including 1550 patients met the inclusion criteria. The risk for major complications was higher in PEG than in LAG 3.86 (95% confidence interval 1.90-7.81; p < 0.0002). The number needed to treat to reduce one major complication by performing LAG instead of PEG was 23. There were no randomized-controlled trials. Overall, the quality of the included studies was determined to be unsatisfactory., Conclusions: PEG placement was associated with a significantly higher risk of major complications compared to LAG placement. Therefore, LAG should be the preferred method for gastrostomy tube placement in children.

Aim of the Study: To assess the frequency of and identify contributing factors to wound dehiscence after posterior sagittal anorectoplasty (PSARP) in children born with anorectal malformations (ARM)., Methods: Ethical approval was obtained (DNR 2017/191). Charts of all children with anorectal malformations (ARM) reconstructed with PSARP, limited PSARP, or PSARVUP at a tertiary centre of paediatric surgery between 2001 and 2016 were reviewed. Wound dehiscence within 30 days postoperatively was analysed regarding gender, prematurity, birth weight, type of ARM, other congenital malformations, single- or multistaged reconstruction, age and weight at reconstruction, postoperative antibiotics, and fasting. Multiple regression analysis was performed for risk factors in single-stage PSARP or limited PSARP, presented as odds ratio (OR) with 95% confidence interval (CI)., Main Results: Ninety patients were included, of which 53 (59%) were males. Single-staged PSARP was performed in 40 (44%) patients and 50 (56%) had a multistaged reconstruction with a colostomy. Wound dehiscence was significantly more common among patients without a colostomy; 17 (43%) vs. 11 (22%) (p=0.043). In patients with single-stage PSARP, no single factor was identified to increase the risk for wound dehiscence: cardiac malformations (OR 3.73) (95% CI 0.78-17.88), low weight at surgery (OR 1.56) (95% CI 0.36-6.99), antibiotics < 1 day (OR 1.6) (95% CI 0.43-5.94), or short fasting 0-3 days (OR 4.44) (95% CI 0.47-42.18)., Conclusions: A divided colostomy protected against wound dehiscence after PSARP. No risk factor for wound dehiscence after single-staged PSARP was identified. Further studies are needed to establish contributing factors to uncomplicated wound healing after PSARP.

Herein, we present the first evaluation of cationic dendrimers based on 2,2-bis(methylol)propionic acid (bis-MPA) as nonviral vectors for transfection of short interfering RNA (siRNA) in cell cultures. The study encompassed dendrimers of generation one to four (G1⁻G4), modified to bear 6⁻48 amino end-groups, where the G2⁻G4 proved to be capable of siRNA complexation and protection against RNase-mediated degradation. The dendrimers were nontoxic to astrocytes, glioma (C6), and glioblastoma (U87), while G3 and G4 exhibited concentration dependent toxicity towards primary neurons. The G2 showed no toxicity to primary neurons at any of the tested concentrations. Fluorescence microscopy experiments suggested that the dendrimers are highly efficient at endo-lysosomal escape since fluorescently labeled dendrimers were localized specifically in mitochondria, and diffuse cytosolic distribution of fluorescent siRNA complexed by dendrimers was observed. This is a desired feature for intracellular drug delivery, since the endocytic pathway otherwise transfers the drugs into lysosomes where they can be degraded without reaching their intended target. siRNA-transfection was successful in C6 and U87 cell lines using the G3 and G4 dendrimers followed by a decrease of approximately 20% of target protein p42-MAPK expression.

Background/purpose: Biliary atresia is the most common reason for newborn cholestasis and pediatric liver transplantation. Even after normalization of serum bilirubin after portoenterostomy, most patients require liver transplantation by adulthood due to expanding fibrosis. We addressed contemporary outcomes of biliary atresia in the Nordic countries., Methods: Data on center and patients characteristics, diagnostic practices, surgical treatment, adjuvant medical therapy after portoenterostomy, follow-up and outcomes were collected from all the Nordic centers involved with biliary atresia care during 2005-2016., Results: Of the 154 patients, 148 underwent portoenterostomy mostly by assigned surgical teams at median age of 64 (interquartile range 37-79) days, and 95 patients (64%) normalized their serum bilirubin concentration while living with native liver. Postoperative adjuvant medical therapy, including steroids, ursodeoxycholic acid and antibiotics was given to 137 (93%) patients. Clearance of jaundice associated with young age at surgery and favorable anatomic type of biliary atresia, whereas annual center caseload >3 patients and diagnostic protocol without routine liver biopsy predicted early performance of portoenterostomy. The cumulative 5-year native liver and overall survival estimate was 53% (95% CI 45-62) and 88% (95% CI 83-94), respectively. Portoenterostomy age <65days and annual center caseload >3 patients were predictive for long-term native liver survival, while normalization of serum bilirubin after portoenterostomy was the major predictor of both native liver and overall 5-year survival., Conclusions: The outcomes of biliary atresia in the Nordic countries compared well with previous European studies. Further improvement should be pursued by active measures to reduce patient age at portoenterostomy., Retrospective Prognosis Study: Level II., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: The aim was to explore if severe congenital heart disease (CHD) influenced the need for dilatation of anastomotic strictures (AS) after the repair of esophageal atresia (EA)., Methods: A retrospective case-control study was conducted examining AS in children with EA and Gross type C. The spectra of CHD and cardiac interventions were reviewed. The frequency of dilatations of AS during the first year following EA reconstruction was compared between children with and without severe CHD requiring cardiac surgery during their first year of life. Endoscopic signs of stricture were an indication for dilatation., Results: Included in the follow-up for AS were 94 patients who had EA reconstructions, of whom 10 (11%) children had severe CHD requiring surgery during the first year including 19 different cardiac interventions. In total, 38 patients needed dilatation of esophageal AS, distributed as six (60%) with severe CHD and 32 (38%) without severe CHD ( p = 0.31)., Conclusion: Severe CHD was present in 11% of children with EA. Esophageal AS developed in 60% children with concomitant CHD, but although high, it did not reach statistical difference from children without CHD (38%).

Aim: We evaluated bowel function in healthy children with regard to gender and age., Methods: The study was carried out in 2016 at a tertiary children's hospital. Healthy children aged 3.5 years to 15 years who were admitted to the hospital, siblings to patients or offspring of staff members were included. Validated self-report questionnaires and internally developed questions regarding obstructive outlet- and gas-related symptoms were used., Results: A total of 310 participants (50% girls) were included, which corresponded to a 94% answer frequency. Respondents were divided into a younger age group (3.5 years to seven years), consisting of 135 children, and an older age group (eight years to 15 years), consisting of 175 children. Younger children reported more foul odours than older children (50% vs. 29%, p = 0.001) and more obstructive symptoms (21% vs. 10%, p = 0.01). There was no difference between the age groups regarding constipation (19% vs 16%, NS). Overall, 55% of those with constipation had no treatment for the condition, although they reported abdominal pain (51%) and problems with foul odours (57%)., Conclusion: Healthy children frequently reported constipation, abdominal pain and gas-related problems, but treatment was rare. Overall, bowel function seemed to improve during childhood, although constipation remained largely untreated., (©2018 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Background: Topical oestrogen and manual separation are the main treatments for labial adhesions. The aim was to evaluate treatment of labial adhesions and compare the outcome of topical oestrogen treatment with that of manual separation., Method: All girls aged 0-12 years admitted to a tertiary centre for paediatric surgery for labial adhesions were included. The study design was dual: The first part was a retrospective chart review of the treatment success according to the medical charts. The second part was a cross-sectional parent-reported long-term outcome study (> 6 months after last treatment finished)., Results: In total 71 patients were included and the median follow-up time for the chart study was 84 (6-162) months after treatment with oestrogen or manual separation. Oestrogen was the first treatment for 66 patients who had an initial successful rate of 62% but this was followed by recurrences in 44%. Five patients had manual treatment as their first treatment and they had a 100% initial success rate followed by recurrences in 20%. Therefore, for the first treatment course there was a final success rate of 35% for oestrogen and 80% for manual separation (p = 0.006). Corresponding final success rates including all consecutive treatments over the study period were 46/130 (35%) for oestrogen and 21/30 (70%) for manual separation (p = 0.001). The success rate for oestrogen did not differ if treatment was given in a course length of 0-4 weeks (39% success) or > 4 weeks (32% success) (p = 0.369). In the parent-reported long-term outcome study the response rate was 51% (36/71). Parents reported that recurrences of adhesions after last prescribed/performed treatment were frequent: in total 25% of patients still had adhesions corresponding to 8/29 (29%) of those whose last treatment was oestrogen and 1/9 (11%) of those whose last treatment was manual separation., Conclusion: Due to the results recurrences are common after both oestrogen and manual separations. However, the overall final outcome after manual separation seems to be more successful when compared to that of topical oestrogen treatment.

Introduction  Proton pump inhibitors (PPIs) are used as prophylaxis, guarding against anastomotic stricture (AS) in the aftermath of reconstructed esophageal atresia (EA). The incidence of stricture formation was studied in this setting, comparing outcomes of 3- and 12-month PPI prophylactic regimens. Patients and Methods  Patient characteristics (gestational age, birth weight, prevalence of chromosomal aberrations, and other malformations), as well as rates of survival, AS formation, and required balloon dilation, were recorded in the following therapeutic subsets: (1) all infants undergoing primary surgical anastomosis for EA in years 2010-2014 and given postoperative PPI prophylaxis for 12 months and (2) all infants similarly treated for EA in years 2001-2009 but given postoperative PPI prophylaxis for 3 months only. Duration of follow-up was 1 year in each group. Results  Patient characteristics and survival rates in 12-month ( n  = 33) and in 3-month ( n  = 30) treatment groups did not differ significantly. The prevalence of AS was 42%/43% in each group (12 months, 14/33; 3 months, 13/30; p  = 1). Median number of dilations required was 3 (range, 1-9) per patient in each group ( p  = 0.69). Median age at initial dilation was 163 days and 63 days in 12- and 3-month groups, respectively ( p  = 0.04). Conclusion  Development of AS in the first year after reconstruction of EA was not reduced by prolonged PPI prophylaxis (12 vs. 3 months), but initial balloon dilation procedures were performed later in infants who were treated longer., (Georg Thieme Verlag KG Stuttgart · New York.)

Aim of the Study: Anastomotic strictures commonly occur in patients undergoing surgery for esophageal atresia (EA). The primary aim of this study was to determine the age distribution of dilation procedures for anastomotic strictures over the patient's childhood after reconstruction of EA. The secondary aim was to evaluate the effect of postoperative proton pump inhibitors (PPIs) on the frequency of dilations., Methods: This observational study was conducted at a single tertiary center of pediatric surgery. The times that dilations of strictures were performed were assessed during three study periods: 1983-1995, 2001-2009, and 2010-2014. PPIs were not used during the first period, and then, respectively, for 3 and 12 months postoperatively. The indications for dilation were signs of obstruction and/or radiological signs of stricture., Primary Results: A total of 131 children underwent esophageal reconstruction, and of those, 60 (46%) required at least 1 dilation procedure for strictures. There were no differences in the frequencies of dilation procedures between the three study periods (28/66, 18/32 and 14/33, respectively; P = 0.42). The overall median number of dilations per patient was 3 (range 1-21) with no differences between the study periods. The differences between ages at which the first dilation was performed during each study period were significant, as follows: 7, 2, and 8 months, respectively (P = 0.03). Fiftyone percent of all dilation procedures were performed during the first year of life, 16% during the second year, and 33% during years 2-15. Four children (2%) underwent >12 dilations., Conclusion: The first year of life was the time of greatest need for dilation of AS after reconstruction of EA; however, dilations were also performed several years later. PPIs did not affect the frequency of dilations during the first year of life.

Background/aim: The role of the microbiome has been widely discussed in the etiology of appendicitis. The primary aim was to evaluate the microbiome in the normal appendix and in appendicitis specifically divided into the three clinically and histopathologically defined grades of inflammation. Secondary aims were to examine whether there were any microbiome differences between proximal and distal appendices, and relate the microbiome with histopathological findings., Methods: A prospective pilot study was conducted of children undergoing appendectomy for appendicitis. The diagnosis was based on histopathological analysis. Children with incidental appendectomy were used as controls. The proximal and distal mucosa from the appendices were analyzed with 16S rRNA gene sequencing., Results: A total of 22 children, 3 controls and 19 appendicitis patients; 11 phlegmonous, 4 gangrenous, and 4 perforated appendices, were prospectively included. The amount of Fusobacterium increased and Bacteroides decreased in phlegmonous and perforated appendicitis compared to controls, but statistical significance was not reached, and this pattern was not seen in gangrenous appendicitis. No relation could be seen between different bacteria and the grade of inflammation, and there was a wide variation of abundances at phylum, genus, and species level within every specific group of patients. Further, no significant differences could be detected when comparing the microbiome in proximal and distal mucosa, which may be because the study was underpowered. A trend with more abundance of Fusobacteria in the distal mucosa was seen in appendicitis patients with obstruction (25 and 13 %, respectively, p = 0.06)., Conclusion: The pattern of microbiome differed not only between groups, but also within groups. However, no statistically significant differences could be found in the microbiome between groups or clinical conditions. No correlation between a specific bacteria and grade of inflammation was found. In the vast majority of cases of appendicitis, changes in microbiome do not seem to be the primary event. Since there seem to be differences in microbiome patterns depending on the sample site, the exact localization of biopsy sampling must be described in future studies., Competing Interests: Compliance with ethical standardsFunding sourceMag-TarmFonden, Swedish Gastroenterology Society, and Development Foundation, Region Skåne, Sweden.Financial disclosureAll authors have no financial relationships relevant to this article to disclose.Conflict of interestThe authors have no conflicts of interest to disclose.

Purpose . Congenital heart disease (CHD) is reported to be associated with Hirschsprung disease (HD). The aim was to evaluate any differences between children with HD with and without CHD, respectively, with regard to patient characteristics, medical care, and patient reported bowel function. Method . This is a retrospective chart study and a cross-sectional long-term follow-up of patients older than 4 years old, including all children with HD operated on with transanal endorectal pull-through (TERPT) at a tertiary center of pediatric surgery. Information about patient characteristics, diagnostics, surgery, and medical care was compiled. At long-term follow-up, bowel function was assessed by Bowel Function Score. Results . Included were 53 HD-patients, 13 with CHD and 40 without CHD. Children with CHD more commonly presented with failure to thrive; 4 (23%) compared to those without CHD (0%) ( p < 0.01). In the long-term follow-up, including 32 patients (6 with CHD), constipation was more commonly reported by children with CHD 5 (83%) than by children without CHD 4 (27%) ( p = 0.01). No differences were shown in the other parameters such as fecal control and incontinence. Conclusion . HD-patients with CHD more commonly presented with failure to thrive and more frequently reported constipation than HD-patients without CHD. The findings indicate that HD-patients with CHD might need special consideration in their initial care and long-term follow-up.

Purpose: The aims of this study were to assess the short- and long-term complication rates after video-assisted gastrostomy (VAG), the effects of age and gender on long-term complications and the effect of duration of gastrostomy tube retention on the need for gastroraphy when the gastrostomy device was removed., Methods: This was a retrospective study of children undergoing VAG at a single institution. Children who died or moved from the area were excluded. The rates of short- and long-term complications developing at 3-6 months or 2 or more years, respectively, were compared., Results: A total of 170 children were studied, out of a cohort of 303 children. The median age at surgery was 2 years. The median duration of postoperative long-term follow-up was 5 years (2-9 years). The complications at the respective short and long-term follow-ups were as follows: granulation tissue, leakage, infection and vomiting. There were no differences in the short- versus long-term complication rates for gender and age. Children needing gastroraphy had used a gastrostomy device significantly longer compared with children with spontaneous closure., Conclusion: Complications after VAG decrease over time. A longer duration of gastrostomy device retention leads to increased need for gastroraphy.

Background: The aim of the study was to describe the technique of two-trocar laparoscopic appendectomy and compare the outcome between two- and three-trocar techniques in children., Methods: All children who underwent laparoscopic surgery for suspected appendicitis from 2006 to 2014 in a center for pediatric surgery were included in the study. Converted surgeries and patients with appendiceal abscess or concomitant intestinal obstruction were excluded. A total of 259 children underwent appendectomy with either two (35 %) or three (65 %) laparoscopic trocars according to the surgeons' preference and intraoperative judgment. Patient demographics, clinical symptoms, surgery characteristics, and complications were reviewed., Results: The mean age of the children was 10.4 years (range, 1-14 years). The mean follow-up time was 41.2 months (SD ± 29.2). No significant differences in age, gender, weight, or signs and symptoms were found between the two- and three-trocar groups. The mean surgery time was significantly shorter in the two-trocar group (47 min) than in the three-trocar group (66 min; p < 0.001). The rates of surgical complications were 2 % vs. 4 %, (p = 0.501), and the rates of postoperative complications were 0 % vs. 5 % (p = 0.054), in the two- and three-trocar groups. The overall incidence of postoperative wound infection was low (<1 %) and did not differ between groups., Conclusions: Two-trocar laparoscopic appendectomy seems to be a safe and feasible technique with a low rate of postoperative wound infections. The present findings demonstrate that when the two-trocar technique could be applied, it is a good complement to the conventional three-trocar technique.

Purpose: The diagnosis of pediatric appendicitis is still a challenge, resulting in perforation and negative appendectomies. The aim of this study was to evaluate novel biomarkers in urine and to use the most promising biomarkers in conjunction with the Pediatric Appendicitis Score (PAS), to see whether this could improve the accuracy of diagnosing appendicitis., Methods: A prospective study of children with suspected appendicitis was conducted with assessment of PAS, routine blood tests, and measurements of four novel urinary biomarkers: leucine-rich α-2-glycoprotein (LRG), calprotectin, interleukin 6 (IL-6), and substance P. The biomarkers were blindly determined with commercial ELISAs. Urine creatinine was used to adjust for dehydration. The diagnosis of appendicitis was based on histopathological analysis., Results: Forty-four children with suspected appendicitis were included, of which twenty-two (50 %) had confirmed appendicitis. LRG in urine was elevated in children with appendicitis compared to children without (p < 0.001), and was higher in children with gangrenous and perforated appendicitis compared to those with phlegmonous appendicitis (p = 0.003). No statistical significances between groups were found for calprotectin, IL-6 or substance P. LRG had a receiver operating characteristic area under the curve of 0.86 (95 % CI 0.79-0.99), and a better diagnostic performance than all routine blood tests. LRG in conjunction with PAS showed 95 % sensitivity, 90 % specificity, 91 % positive predictive value, and 95 % negative predictive value., Conclusion: LRG, adjusted for dehydration, is a promising novel urinary biomarker for appendicitis in children. LRG in combination with PAS has a high diagnostic performance.

Bifunctional dendrons based on 2,2-bis(methylol)propionic acid (bis-MPA) are highly desirable scaffolds for biomedical applications. This is due to their flawless nature and large and exact number of functional groups as well as being biodegradable and biocompatible. Herein, we describe a facile divergent growth approach to their synthesis from monobenzylated tetraethylene glycol and post functionalization utilizing fluoride-promoted esterification (FPE) chemistry protocols. The scaffolds, presenting selectively deprotectable hydroxyls in the periphery and at the focal point, were isolated on a multigram scale with excellent purity up to the fourth generation dendron with a molecular weight of 2346 Da in seven reactions with a total yield of 50%. The third generation dendron was used as a model compound to demonstrate its functionalizability. Selective deprotection of the dendron's focal point was achieved with an outstanding yield of 94%, and biotin as well as azido functionalities were introduced to its focal point and periphery, respectively, through FPE chemistry. Bulky disperse red dyes were clicked through CuAAC to the dendron's azido groups, giving a biotinylated dendron with multivalent dyes with a molecular weight of 6252 Da in a total yield of 37% in five reactions with an average yield of 82% starting from the third generation focally and peripherally protected dendron. FPE chemistry proved to be a superb improvement over previous protocols towards bis-MPA dendrons as high purity and yields were obtained with less toxic solvents and greatly improved monomer utilization.

Background: Chronic leg ulcers remain a challenge to the treating physician. Such wounds often need skin grafts to heal. This necessitates a readily available, fast, simple, and standardized procedure for grafting., Objectives: The aim of this work was to test a novel method developed for outpatient transplant procedures., Methods: The procedure employs a handheld disposable dermatome and a roller mincer that cut the skin into standardized micrografts that can be spread out onto a suitable graft bed. Wounds were followed until healed and photographed., Results: The device was successfully used to treat and close a traumatic lower limb wound and a persistent chronic venous leg ulcer. The donor site itself healed by secondary intent with minimal cosmetic impairment., Conclusion: The method was successfully used to graft 2 lower extremity wounds.

Introduction: In children treated surgically for first-time perianal abscesses, discovery and excision of concomitant fistulas may also be warranted., Aim: To evaluate children of varying age after incision and drainage of first-time perianal abscesses, examining recurrences rates with and without search for a fistula., Method: A retrospective review was conducted, analyzing children (ages 0-15 years) treated for first-time perianal abscesses at a tertiary pediatric surgical center, with a minimum follow-up of 6 months., Results: A total of 104 patients subjected to 112 treatments for first-time perianal abscesses were eligible. Surgical procedures constituted 84 (75%) of treatments, searching for fistulas in 49 (58%). In 34 (69%), fistulas were confirmed and treated. In the surgically treated subset, the recurrence rate was higher if no attempt was made to exclude a fistula (46%), as opposed to confirmed absence of a fistula (27%) or concurrent fistulotomy (9%; p = 0.02). Younger patients showed a higher recurrence rate (12/26; 46%), compared with older counterparts (11/58; 19%) (p = 0.002)., Conclusion: In children surgically treated for first-time perianal abscess, recurrence rates appear to be lowered by locating and treating coexisting fistulas.

Background: The incidence in appendicitis and the appendectomy rate are different between boys and girls. This study aimed to further evaluate appendicitis in children from a gender perspective. Gender differences in presentation, perioperative care, and outcome after appendectomy were examined., Method: A single, institution-based, retrospective study was conducted at a tertiary pediatric surgery center. All children <15 years of age who underwent appendectomy for suspected appendicitis or who were conservatively treated for an appendiceal abscess, from 2006 to 2014, were included. Patient demographics, symptoms, preoperative management including time to appendectomy, operative characteristics, and postoperative course including complications and length of hospital stay were evaluated., Results: The study included 427 children: 183 girls and 244 boys. The median postoperative follow-up time was 40 (range 1-106) months. The genders did not differ significantly in age, mean weight, symptoms, laboratory results, time to appendectomy, postoperative complications, postoperative pain management, antibiotic treatment, and length of hospital stay. The frequencies of hopping/percussion/coughing tenderness in the right lower quadrant (61 and 51%, p = 0.042), phlegmonous appendicitis (56 and 45%, p = 0.032), perforated appendicitis (18 and 10%, p = 0.043), and open appendectomy (57 and 28%, p = 0.048) were significantly higher in boys. Girls had significantly higher frequencies of preoperative imaging (50 and 38%, p = 0.021), negative appendectomy (18 and 7%, p = 0.005), gangrenous appendicitis (18 and 11%, p = 0.049), and operative complications (7 and 2%, p = 0.015). In girls, the Pediatric Appendicitis Score (PAS) was more specific (59 and 41%, respectively, p = 0.032) and had a greater negative predictive value (33 and 11%, respectively, p = 0.003) than in boys., Conclusion: In children with appendicitis, there are some gender differences. Girls had negative appendectomies more often, despite having more preoperative imaging and they had operative complications more frequently, despite having less frequent perforations. Boys had a higher frequency of perforation even though their time to appendectomy equaled that of girls. These findings and the reasons behind them must be further studied.

Background: The transanal endorectal one-stage pull-through (TERPT) procedure in children with Hirschsprung disease (HD) is frequently used worldwide. To give the children's families realistic expectations and to plan the medical care for the period after TERPT, the outcome is of great importance., Aim: The aim of this article is to collect information on the number of stools passed daily after one-stage TERPT procedure for HD. Patients and, Methods: A prospective follow-up study for collecting information on the outcome of planned TERPT from 2005 through 2012 was performed. A control group consisting of age and gender matched children was used., Results: The results show an initial high frequency of daily stools, median 12 stools/day (range, 3-30 stools/day), reaching an acceptable situation with median 4 stools/day (range, 0-10 stools/day) after 1 year. After 4 years, the number of stools did not differ significantly from healthy controls., Conclusion: This study shows that it takes 4 years after TERPT before the number of stools becomes normalized. To compare the long-term outcome, it would be desirable to have uniform regular reports on the daily frequency of passed stools, incontinence, and constipation during the years after TERPT., (Georg Thieme Verlag KG Stuttgart · New York.)

Background. The aim of this study was to map gastroscopies performed at a single tertiary pediatric surgery centre to investigate indications, complications, outcomes, and ethical aspects. Material and Methods. A retrospective study of gastroscopies performed during two time periods (2001-2004 and 2011-2014) was conducted. Data regarding indications, outcomes, and complications of pediatric gastroscopies were analysed from a prospectively collected database. Results. The indications for gastroscopies changed over time. Therefore, 376 gastroscopies performed from 2011 through 2014 were studied separately. The median patient was four years old. The predominant indications were laparoscopic gastrostomy (40%), investigation of gastroenterological conditions (22%), obstruction in the upper gastrointestinal tract (20%), gastroesophageal reflux disease (GERD) (15%), and other indications (3%). Percentages of gastroscopies with no positive findings for each condition were laparoscopic gastrostomy, 100%; gastroenterological conditions, 46%; obstruction in the upper gastrointestinal tract, 36%; GERD, 51%. Furthermore, gastroscopies did not lead to any further action or change in treatment in 45% of gastroenterological conditions and 72% of GERD cases. The overall complication rate was 1%. Conclusion. The results are valuable to educate pediatric surgeons and to inform health care planning when including gastroscopy within clinical practice.

Background. This study aimed to evaluate Pediatric Appendicitis Score (PAS), diagnostic delay, and factors responsible for possible late diagnosis in children <4 years compared with older children who were operated on for suspected appendicitis. Method. 122 children, between 1 and 14 years, operated on with appendectomy for suspected appendicitis, were retrospectively analyzed. The cohort was divided into two age groups: ≥4 years (n = 102) and <4 years (n = 20). Results. The mean PAS was lower among the younger compared with the older patients (5.3 and 6.6, resp.; P = 0.005), despite the fact that younger children had more severe appendicitis (75.0% and 33.3%, resp.; P = 0.001). PAS had low sensitivity in both groups, with a significantly lower sensitivity among the younger patients. Parent and doctor delay were confirmed in children <4 years of age with appendicitis. PAS did not aid in patients with doctor delay. Parameters in patient history, symptoms, and abdominal examination were more diffuse in younger children. Conclusion. PAS should be used with caution when examining children younger than 4 years of age. Diffuse symptoms in younger children with acute appendicitis lead to delay and to later diagnosis and more complicated appendicitis.

Background: Patients with Hirschsprung disease lack the normal rectoanal inhibitory reflex, which can be studied with anorectal manometry or US., Objective: To see whether the rectoanal inhibitory reflex could be visualised with a modified contrast enema, thereby increasing the diagnostic accuracy of the contrast enema and reducing the number of rectal biopsies., Materials and Methods: Fifty-nine boys and 42 girls (median age, 12 months) with suspected Hirschsprung disease were examined with a modified contrast enema, supplemented with two injections of cold, water-soluble contrast medium, to induce the reflex. Two paediatric radiologists evaluated the anonymised examinations in consensus. The contrast enema findings were correlated with the results of rectal biopsy or clinical follow-up., Results: Five boys and one girl (median age, 7.5 days) were diagnosed with Hirschsprung disease. The negative predictive value of the rectoanal inhibitory reflex was 100%. A contrast enema with signs of Hirschsprung disease in combination with an absent rectoanal inhibitory reflex had the specificity of 98% and sensitivity of 100% for Hirschsprung disease., Conclusion: The modified contrast enema improves the radiological diagnosis of Hirschsprung disease. By demonstrating the rectoanal inhibitory reflex in children without Hirschsprung disease, we can reduce the proportion of unnecessary rectal biopsies.

Aim: The aim of this study is to evaluate postoperatively bowel symptoms of antegrade colon enema through appendicostomies in preschool children with anorectal malformation (ARM)., Method: 164 children with ARM operated on with posterior sagittal anorectal plasty were included. The malformations were classified according to Krickenbeck classification. Seventeen children in preschool age had an appendicostomy. The bowel symptoms according to the Krickenbeck follow-up were analysed pre- and postoperatively. All complications were registered. A questionnaire on the use of the appendicostomy was answered., Results: The median age (range) at the time of the appendicostomy was 4 (1-6) years. The observation time was 5 (0.5-14) years. The main indications for appendicostomy were incontinence and noncompliance to rectal enemas. Postoperatively there was a significant decrease in soiling and constipation (P < 0.001). The total complication rate was 43% with infections (29%), stenosis (12%), and retrograde leakage (0). The median time required for giving enema in the appendicostomy was 45 minutes (range: 15-120) once a day varying from 2 times/week to 3 times/day. And: complications are less frequent than in older children., Conclusion: Appendicostomy in preschool children with ARM is a way to achieve fecal cleanness before school start. The infection rate was high, but other complications are less frequent than in older children.

Introduction: A wireless BRAVO (Medtronic, Shoreview, MN) capsule for pH measurement in the diagnosis of gastroesophageal reflux disease (GERD) is intended to be less uncomfortable, and facilitates activity during the measuring period, compared to the usual method with a naso-esophageal catheter. The aim of this study was to report on our experience with the wireless system in children. A secondary aim was to see if there was any cut-off level for esophageal acid exposure causing esophagitis as verified by pathologic examination., Materials and Methods: A total of 62 wireless 24-hour pH measurements with the BRAVO capsule were carried out over a period of 2 years in 58 children with symptoms of GERD. The median age of the children was 8 +/- 4 years (range, 1-15). They underwent upper endoscopies and the placement of the capsule under general anesthesia. Correlations between endoscope findings and pathologic diagnosis were done., Results: In 10 children, the endoscopies showed esophagitis. The median percent time of pH <4 was 7.0 +/- 9.6% (range, 0-61). The DeMeester score was abnormally high in 33 children. Three children described dysphagia during the measuring time. In 3 patients, we experienced technical problems with the wireless system. Biopsies were taken in 49 children, of which 18 showed esophagitis, with no correlation to the 24-hour pH-measurement findings., Conclusions: Ambulatory pH monitoring, using the wireless pH system, is feasible and was well tolerated by the children. No cut-off level for the acid exposure in correlation to pathologic diagnosis of esophagitis could be found. We recommend the use of the wireless pH-measurement system in children.

Quality assurance of drug prescription is a pre-requisite for rational drug use. From 22 health-care centres in the south-western area of the Stockholm County Council region, drug-prescription data were obtained from the patients' computerised medical recordings. This could be done with the aid of a specially designed database program. The drug-prescription data from the 22 health-care centres were collected and compiled in a central unit. Thereafter the results were brought back to the health-care centres, in which the quality assurance of drug prescription could be started.