Experiences and satisfaction of video follow up of children with paediatric gastrointestinal conditions linking tertiary centre with guardians and clinicians at the local hospital: a cross-sectional study.

BACKGROUND: Children with complicated gastrointestinal conditions are dependent on follow up by tertiary care specialists throughout childhood to prevent and treat complications. In Sweden, paediatric surgical- and intestinal rehabilitation centres are centralised which means that many patients and guardians have to travel long distances to access tertiary referral centres. Our tertiary referral centre has developed a model of shared care with video conferences for follow up with our centre and the patient and guardians attending together with the responsible professionals at the local hospital. This study aimed to investigate the experiences and satisfaction with video follow-up visits (VFV) between a tertiary referral centre and guardians and clinicians at their local hospital. METHODS: Eligible participants were families with children with oesophageal atresia, intestinal failure and intestinal motility disorders and their local clinicians attending VFV with our tertiary referral centre from 2015 to 2020. Questionnaires included fixed-response alternatives, a 6-point Likert scale and open questions. RESULTS: Fifty-seven out of 102 families (56%) and 19 out of 27 local clinicians (70%) responded the questionnaires. In 68% of the VFV, two guardians attended compared to 35% in the physical visits. Of the guardians attending VFV, 82% lost ≤ half a working day and 91% attending physical visits lost ≥ one full working day. Median distance to the tertiary referral centre was 267 km and attending VFV avoided emissions of 7.2 metric tonnes of CO2. Of the guardians, 90% and of the clinicians 95% were satisfied with VFV. Advantages were avoidance of travelling and the participants shared the same information. CONCLUSIONS: VFV is an appropriate alternative to physical visits with a high grade of satisfaction among the guardians and clinicians. VFV was time-saving for the families and reduced CO2 emissions.

Swedish registry study showed that 50% of paediatric operations were performed in university hospitals and mortality was low.

AIM: To investigate the distribution of paediatric surgery in various hospitals and to study postoperative risk factors of mortality. METHODS: Retrospective registry-based cohort study of children aged 0-14 years undergoing surgery from 2017 to 2021. Data were extracted from the Swedish Perioperative Registry. A mixed logistic regression was applied for the all-risk mortality analysis. RESULTS: A total of 126 539 cases were identified, 50% in university, 36% in county and 14% in district hospitals. The dominating operations were appendectomy in 6667, orchidopexy in 5287, inguinal hernia repair in 4200 and gastrostomy in 1152 children. Among children under 1 year of age or American Society of Anesthesiologists Physical Status classification (ASA-PS-Class) 3-5, the majority underwent surgery in university hospitals. The 30-day mortality in university hospitals was 0.5% and in county hospitals 0%. The proportion of emergency surgery was similar in university and county hospitals. Independent risk factors of mortality were being under 1 year of age, ASA-PS-class 4-5, emergency surgery and surgery at university hospitals. CONCLUSION: Half of all operations in children were performed in university hospitals, with low postoperative mortality despite effective centralisation of high risk patients <1 year of age or ASA-PS-Class 3-5.

Reduced Expression of REG4 as a Sign of Altered Goblet Cell Function in Necrotizing Enterocolitis.

OBJECTIVE: Defective Goblet cells have been proposed to be involved in necrotizing enterocolitis (NEC). The aim was to study the expression of the Goblet cell marker REG4 and its potential involvement in NEC in preterm infants with and without NEC. STUDY DESIGN: Seventy histologically intact intestinal biopsies were studied: 43 were collected during surgery due to NEC (NEC group: 26.5 ± 3.0 weeks' gestational age [wGA]), and 27 from individuals who underwent surgery due to other conditions (Control group; 36.1 ± 4.5 wGA). The tissue samples were immunohistochemically stained for REG4. REG4 expression was quantified with a semiautomated digital image analysis and with clinical data compared between the groups. RESULTS: REG4 expression was lower in the NEC group than in the Control group (p = 0.035). Low REG4 expression correlated to the risk of NEC (p = 0.023). In a multivariable logistic regression analysis including GA and REG4 expression for NEC risk, only GA (p < 0.001) and not REG4 expression (p = 0.206) was associated with NEC risk. CONCLUSION: This study concludes that Goblet cell dysfunction may be involved in NEC development, as low expression of the Goblet cell marker REG4 was related to an increased NEC risk in preterm infants. Maturity could however not be excluded as a potential confounder for REG4 expression. KEY POINTS: · REG4 is a specific Goblet cell marker not yet studied in NEC.. · REG4 was quantified in intestinal biopsies from infants with and without NEC.. · REG4 expression was lower in infants with NEC, and expression seems to be maturity dependent..

Paneth cell proteins DEFA6 and GUCA2A as tissue markers in necrotizing enterocolitis.

Previous studies suggest that Paneth cells are involved in NEC development. Defensin alpha 6 (DEFA6) and guanylate cyclase activator 2A (GUCA2A) are selective protein markers of Paneth cells. The objective was to explore DEFA6 and GUCA2A expression in intestinal tissue samples from newborn infants with and without NEC. Tissue samples from histologically intact intestine were analyzed from 70 infants: 43 underwent bowel resection due to NEC and 27 controls were operated due to conditions such as intestinal atresia, dysmotility, aganglionosis, pseudo-obstruction or volvulus. Each tissue sample was immunohistochemically stained for DEFA6 and GUCA2A. Semi-automated digital image analysis was performed to determine protein expression. Clinical data and protein expressions were compared between the groups. DEFA6 expression was lower in the NEC group (p = 0.006). Low DEFA6 correlated with risk of developing NEC in a logistic regression analysis, independently of gestational age and birth weight (OR 0.843 [CI 0.732-0.971]; p = 0.018). GUCA2A expression did not differ between the two groups. CONCLUSION: Lower expression of DEFA6 together with intact GUCA2A expression indicates that NEC patients have well-defined Paneth cells but diminished defensin activity. Our results suggest that DEFA6 could be used as a biomarker for NEC. WHAT IS KNOWN: • Previous studies of defensin activity in NEC have been inconsistent, showing that defensin levels may be increased or diminished in NEC. GUCA2A has to our knowledge never been studied in NEC. WHAT IS NEW: • This study benchmarks two specific Paneth cell markers (DEFA6 and GUCA2A) and their activity in individuals with and without NEC. • The key finding is that the NEC group had a lower DEFA6 expression compared to the Controls, while the expression of GUCA2A did not differ between the groups.

Surgical management of necrotising enterocolitis in Sweden: A national cohort study.

AIM: Necrotising enterocolitis (NEC) is the dominating surgical emergency in preterm neonates. The aims were to investigate indications, surgical management and mortality for surgically treated neonates with NEC. METHODS: Data were retrieved from the Swedish Neonatal Quality Register for Swedish neonates with surgically treated NEC from 1 January 2017 to 31 December 2021. Diagnosis was validated by surgical records and histopathology. Neonates with isolated spontaneous intestinal perforation were excluded. RESULTS: In total, 109 neonates were included. Median gestational age was 25 weeks (22-38), and median birth weight was 771 g (269-3920). Preoperative pneumoperitoneum was found in 32%, portal venous gas in 25% and clinical deterioration on conservative treatment in 26% of the neonates. Among the 97 neonates presenting with small bowel necrosis, single-focal NEC occurred in 38 (39%), multifocal NEC in 35 (36%) and panintestinal NEC in 24 (25%). A primary anastomosis was performed in 10/87 (11%) of the neonates with bowel resection at primary surgery. Clip-and-drop technique was applied in 24/87 (28%).Mortality rate was 37%. CONCLUSION: Mortality was well comparable with earlier reports considering exclusion of spontaneous intestinal perforation (SIP) and the low gestational age of the study population. Resection of necrotic bowel with stoma formation was the dominating surgical method.

The risk of autism spectrum disorder and intellectual disability but not attention deficit/hyperactivity disorder is increased in individuals with esophageal atresia.

Knowledge of neurodevelopmental disorders such as attention deficit/hyperactivity disorder (ADHD), autism spectrum disorders (ASD) and intellectual disability (ID) in patients with esophageal atresia (EA) is scarce. The aims of this study were to investigate the prevalence and risk of ADHD, ASD and ID in individuals with EA. Data were obtained from four longitudinal population-based registries in Sweden and analyzed using Cox proportional hazards regression. Patients with EA born in Sweden in 1973-2018 were included together with five controls for each individual with the exposure matched on sex, gestational age at birth, birth year and birth county. Individuals with chromosomal aberrations and syndromes were excluded. In total, 735 individuals with EA and 3675 controls were included. Median age at time of the study was 20 years (3-48). ASD was found in 24 (3.9%), ADHD in 34 (5.5%) and ID in 28 (4.6%) individuals with EA. Patients with EA had a 1.66 times higher risk of ASD (95% confidence interval [CI], 1.05-2.64) and a 3.62 times higher risk of ID (95% CI, 2.23-5.89) compared with controls. The risk of ADHD was not significantly increased. ADHD medication had been prescribed to 88.2% of patients with EA and ADHD and to 84.5% of controls with ADHD. Individuals with EA have a higher risk of ASD and ID than individuals without the exposure. These results are important when establishing follow-up programs for children with EA to allow timely detection and consequentially an earlier treatment and support especially before school start.

Outcome and management in neonates with gastroschisis in the third millennium-a single-centre observational study.

Gastroschisis is one of the most common congenital malformations in paediatric surgery. However, there is no consensus regarding the optimal management. The aims of this study were to investigate the management and outcome and to identify predictors of outcome in gastroschisis. A retrospective observational study of neonates with gastroschisis born between 1999 and 2020 was undertaken. Data was extracted from the medical records and Cox regression analysis was used to identify predictors of outcome measured by length of hospital stay (LOS) and duration of parenteral nutrition (PN). In total, 114 patients were included. Caesarean section was performed in 105 (92.1%) at a median gestational age (GA) of 36 weeks (range 29-38) whereof (46) 43.8% were urgent. Primary closure was achieved in 82% of the neonates. Overall survival was 98.2%. One of the deaths was caused by abdominal compartment syndrome and one patient with intestinal failure-associated liver disease died from sepsis. None of the deceased patients was born after 2005. Median time on mechanical ventilation was 22 h. Low GA, staged closure, intestinal atresia, and sepsis were independent predictors of longer LOS and duration on PN. In addition, male sex was an independent predictor of longer LOS. CONCLUSION: Management of gastroschisis according to our protocol was successful with a high survival rate, no deaths in neonates born after 2005, and favourable results in LOS, duration on PN, and time on mechanical ventilation compared to other reports. Multicentre registry with long-term follow-up is required to establish the best management of gastroschisis. WHAT IS KNOWN: • Gastroschisis is one of the most common congenital malformations in paediatric surgery with increasing incidence. • There is no consensus among clinicians regarding the optimal management of gastroschisis. WHAT IS NEW: • Although primary closure was achieved in 82% of the patients, mortality rate was very low (1.8%) with no deaths in neonates born after 2005 following the introduction of measurement of intraabdominal pressure at closure. • Low gestational age, staged closure, intestinal atresia, sepsis, and male sex were independent predictors of longer length of hospital stay.

Vascular adhesion protein-1 expression is reduced in the intestines of infants with necrotizing enterocolitis: an observational research study.

BACKGROUND: Necrotizing enterocolitis (NEC) is an inflammatory bowel disease in preterm neonates with high morbidity and mortality. The only treatment available is supportive with broad-spectrum antibiotics and gastrointestinal rest. Better understanding of the pathogenesis is crucial for the development of new therapies. Vascular adhesion protein-1 (VAP-1), expressed in human blood vessels and lymphatic, plays a crucial role in the pathogenesis of inflammatory diseases in adults. The aim of the study was to investigate the VAP-1 expression in the intestines of infants affected by NEC. METHODS: Intestinal tissues from 42 preterm infants with NEC were examined with immunohistochemical staining using antibodies against VAP-1 and semi-automated digital image analysis was performed to determine tissue protein expression of VAP-1 in blood vessels located in the submucosa. Intestinal tissue from 26 neonates that underwent laparotomy and ileostomy due to other intestinal surgical conditions served as controls. Clinical data and protein expression were compared between the NEC-group and Controls. RESULTS: Mean gestational age was lower in NEC infants compared to controls, 26.6 ± 3.0 gestational weeks versus 36.5 ± 4.0 (p < 0.001) but without any significant difference in median postnatal age at surgery; for NEC 8 (5-27) days and for controls 3 (1-36) days (p = 0.6). Low VAP-1 correlated with increased risk for developing NEC in the logistic regression (p < 0.001). Multiple linear regression showed that both gestational age and NEC were independent predictors of VAP-1 expression. CONCLUSION: VAP-1 may play a role in the pathogenesis of NEC. Diminished expression of VAP-1 independent of maturation could indicate arrested vascular development in infants suffering from NEC. Further studies are needed to elucidate the role of VAP-1 in NEC.

Improved Outcome of Intestinal Failure in Preterm Infants.

OBJECTIVE: The aim of the study was to evaluate the outcome and to identify predictors for survival and enteral autonomy in neonatal intestinal failure (IF). METHODS: A retrospective observational study in a Swedish tertiary centre of children born between 1995 and 2016 with neonatal IF, defined as dependency on parenteral nutrition (PN) ≥60 days, starting with PN before the age of 44 gestational weeks. Data were extracted from medical records and predictors for survival and enteral autonomy were identified by the Cox regression model. Time to death and weaning off PN analysis were performed with Kaplan-Meier curves including log rank test. RESULTS: In total, 105 children were included. Median gestational age was 28 weeks (22-42), 50% were born extremely preterm (<28 gestational weeks). PN started at a median age of 2 days (0-147) with a median duration of 196 days (60-3091). Necrotizing enterocolitis was the dominating cause of IF (61%). Overall survival was 88%, 5 children died of sepsis and 4 of intestinal failure-associated liver disease. Survival increased from 75% during 1995 to 2008 to 96% during 2009 to 2016 (P = 0.0040). Age-adjusted small bowel length of >50% and birth 2009 to 2016 were predictors for survival. Enteral autonomy was achieved in 87%, with positive prediction by small bowel length of >25% of expected for gestational age and remaining ileocecal valve. CONCLUSIONS: Preterm neonates with IF, at high risk of IF-associated morbidity, showed a high overall survival rate. Small-bowel length and being born 2009 to 2016 were predictors for survival and remaining ICV and small-bowel length were predictors for enteral autonomy.

Gender and birth weight as risk factors for anastomotic stricture after esophageal atresia repair: a systematic review and meta-analysis.

BACKGROUND: Anastomotic stricture (AS) is the most frequently occurring complication that occurs after esophageal atresia (EA) repair. Nevertheless, the pathogenesis remains primarily unknown and there is inadequate knowledge regarding the risk factors for AS. Therefore, a systematic review of the literature and a meta-analysis was performed to investigate whether gender and birth weight were risk factors for the development of AS following EA repair. METHODS: The main outcome measure was the occurrence of AS. Forest plots with odds ratios (OR) and 95% confidence intervals (CI) were generated for the outcomes. Quality assessment was performed using the Newcastle-Ottawa scale. RESULTS: Six studies with a total of 495 patients were included; 59% males, and 37 and 63% of the patients weighed < 2500 g and ≥ 2500 g, respectively. Male gender (OR, 0.96; 95% CI, 0.66-1.40; p = 0.82) and birth weight < 2500 g (OR, 0.74; 95% CI, 0.47-1.15; p = 0.18) did not increase the risk of AS. The majority of the included studies were retrospective cohort studies and the overall risk of bias was considered to be low to moderate. CONCLUSION: Neither gender nor birth weight appear to have an impact on the risk of AS development following EA repair.

Risk factors for postoperative mortality in congenital diaphragmatic hernia: a single-centre observational study.

BACKGROUND: The management of congenital diaphragmatic hernia (CDH) is a major challenge. The mortality is dependent on associated malformations, the severity of pulmonary hypoplasia, pulmonary hypertension and iatrogenic lung injury associated with aggressive mechanical ventilation. The aims of the study were to investigate the mortality over time in a single paediatric surgical centre, to compare the results with recent reports and to define the risk factors for mortality. METHODS: The medical records of infants with CDH from two time periods: 1995-2005 and 2006-2016 were reviewed. Cox regression was used for statistical analysis. RESULTS: The study included 113 infants. The mortality rate was significantly decreased in the later time period, compared to the earlier, 4.4 and 17.9%, respectively. At the early time period five patients (7.5%) were treated with ECMO and in the later time period ECMO was used in three patients (6.5%). The mortality in ECMO-treated patients was 50% in both time periods. Prenatal diagnosis, intrathoracic liver, low Apgar score and low birth weight were defined as independent risk factors for mortality. CONCLUSION: Despite no significant differences in the incidence of independent risk factors and the use of ECMO between the two time periods, mortality decreased over time. The mortality was lower than previously reported. The results indicate that there are many important factors involved in a successful outcome after CDH repair. Large multicentre studies are necessary to define those critical factors and to determine optimal treatment strategies.

Outcome of laparoscopic versus open gastrostomy in children.

PURPOSE: Laparoscopic gastrostomy (LAPG) has gained popularity in children. The aim of this study was to compare the outcome of LAPG versus open gastrostomy (OG) in children with focus on complications, operative times and postoperative length of stay. METHODS: Retrospective study of children who had gastrostomies inserted at our tertiary Pediatric Surgery Center from 2000 until 2013. The indications for a gastrostomy were an anticipated need for enteral support for at least 6 months. Totally 243 children were included in the study, 83 with LAPG and 160 with OG. RESULTS: We found a significant difference in postoperative length of stay, 3 days in the LAPG group versus 4 days in the OG group but no difference in a sub-group analysis from 2010 to 2013 when both techniques were used. There was no difference in median operative time or complications rates. Granuloma was the dominating complication in both groups. CONCLUSION: These two feeding-access techniques are comparable regarding complications, operative times and postoperative length of stay. The choice of surgical method should be individualized based on the patient's characteristics and the experience of the surgeon. The favorable results with LAPG in adults are not necessarily transferable to children since there are physiological and anatomical differences.

The Incidence of Necrotizing Enterocolitis and Late-Onset Sepsis during the COVID-19 Pandemic in Sweden: A Population-Based Cohort Study.

INTRODUCTION: The effect of the pandemic restrictions in the NICUs is not well studied. Necrotizing enterocolitis (NEC) is characterized by intestinal inflammation and bacterial invasion. This study aimed to investigate whether the incidence of NEC has changed during the COVID-19 pandemic in Sweden and whether it was associated with a change in the frequency of extremely preterm births. METHODS: Data were retrieved from the Swedish Neonatal Quality Register (SNQ) for infants registered between January 2017 and December 2021 born below a gestational age of 35 weeks. The registry completeness is 98-99%. The diagnosis of NEC was the primary outcome. Generalized linear model analysis was used to calculate the risk ratio for NEC. RESULTS: Totally 13,239 infants were included. 235 (1.8%) infants developed NEC, out of which 91 required surgical treatment. 8,967 infants were born before COVID-19 pandemic and 4,272 during. Median gestational age at birth was 32.8 weeks in both periods. The incidence of NEC was significantly lower during COVID-19 pandemic compared to the prior period (1.43 vs. 1.94%, p 0.037), but not the incidence of surgical NEC. The crude risk ratio of developing NEC during COVID-19 pandemic was 0.74 (95% CI: 0.55-0.98). The incidence of late-onset sepsis with positive culture was also declined during COVID-19 (3.21 vs. 4.15%, p value 0.008). CONCLUSION: While we found significant reduction in the incidence of NEC and culture-positive late-onset sepsis during the COVID-19 pandemic, the number of extremely preterm births was unchanged.

Intestinal microbial profiles in extremely preterm infants with and without necrotizing enterocolitis.

AIM: Necrotizing enterocolitis (NEC) represents one of the gravest complications in premature infants. The suggested role of intestinal microbiota in the development of NEC needs to be elucidated. METHODS: This prospective single-centre case-control study applied barcoded pyrosequencing to map the bacterial composition of faecal samples from extremely preterm infants. Ten patients were diagnosed with NEC and matched to healthy controls with regard to sex, gestational age and mode of delivery prior to analysis of the samples. RESULTS: Enterococcus, Bacillales and Enterobacteriaceae dominated the flora. Although not statistically significant, a high relative abundance of Bacillales and Enterobacteriaceae was detected at early time points in patients developing NEC, while healthy controls had a microbiota more dominated by Enterococcus. A low diversity of intestinal microbial flora was found without any differences between NEC patients and controls. In 16 healthy controls, Firmicutes (Enterococcus and Bacillales) dominated the faecal flora during the first weeks after birth and were then succeeded by Enterobacteriaceae. CONCLUSION: No significant differences in the composition of intestinal microbiota of patients developing NEC were detected; however, some findings need to be scrutinized in subsequent studies.

Neurodevelopmental outcomes in individuals with VACTERL association. A population-based cohort study.

BACKGROUND: Studies on neurodevelopmental outcomes in individuals with congenital anomalies who undergo neonatal surgery are scarce and have reported contradictory findings based on small study groups. The congenital condition VACTERL association includes at least three malformations: vertebral anomalies, anorectal malformations, cardiac defects, tracheoesophageal fistula with or without esophageal atresia, renal anomalies and limb deformities. Most of these patients undergo surgery during their first days of life. Neurodevelopmental disorders include a broad group of disabilities involving some form of disruption to brain development. Attention deficit hyperactivity disorder (ADHD), autism spectrum disorders (ASD) and intellectual disability (ID) are diagnoses included in this group. The aim of the study was to investigate the risk of ADHD, ASD and ID in a cohort of individuals with VACTERL association. METHOD: Data was obtained from four Swedish national health registers and analyzed using the Cox proportional hazards model. Patients born 1973-2018 in Sweden with the diagnosis of VACTERL association were included in the study. For each case five healthy controls matched for sex, gestational age at birth, birth year and birth county were obtained. RESULTS: The study included 136 individuals with VACTERL association and 680 controls. Individuals with VACTERL had significantly higher risk of ADHD, ASD and ID than the controls; 2.25 (95% CI, 1.03-4.91), 5.15 (95% CI, 1.93-13.72) and 8.13 (95% CI, 2.66-24.87) times respectively. CONCLUSIONS: A higher risk of ADHD, ASD and ID was found among individuals with VACTERL association compared to controls. These results are of importance to caregivers and to professionals participating in follow ups of these patients in providing early diagnosis and support, aiming to optimize the quality of life of these patients.

Paediatric Adhesive Small Bowel Obstruction is Associated with a Substantial Economic Burden and High Frequency of Postoperative Complications.

BACKGROUND: Intra-abdominal adhesions can lead to adhesive small bowel obstruction (ASBO). The incidence of ASBO is higher in paediatric surgery than in adult surgery. However, ASBO related complications, economic burden and clear management guidelines in the treatment of ASBO are lacking. The aims of this study were to investigate underlying diagnoses, treatments, complications and costs in paediatric ASBO. METHOD: An observational retrospective study in children 0-15 years, hospitalised for ASBO during 2000-2020. Data were extracted from the medical records. Complications were classified based on Clavien Dindo Classification of Surgical Complications. Descriptive statistics were presented as median, continuous variables and categorical variables summarised with frequencies. Time to ASBO was presented as a Kaplan-Meier estimate. RESULTS: In total, 101 patients with 137 episodes of ASBO were included whereof 58.4% underwent first (index) surgery during the neonatal period. Median follow-up was 11.3 (0.6-19) years and median time to the first ASBO was 3.76 months (95%CI 2.23-12.02). The most common diagnoses at index surgery were necrotising enterocolitis, duodenal obstruction and primary ASBO. In 86.6% of the patients, first ASBO did not resolve with conservative treatment and a laparotomy was needed. Postoperative complications were found in 52%. Median cost for one episode of acute ASBO was 36 236 USD (1629-236 159). CONCLUSION: Neonatal surgery was the dominating cause of ASBO and surgical intervention the most common treatment with a high frequency of postoperative complications and significant healthcare costs. Future studies are needed to develop safe management guidelines for the treatment of paediatric ASBO. LEVELS OF EVIDENCE: III.

Bowel Control, Bladder Function, and Quality of Life in Children with Cloacal Malformations.

INTRODUCTION: Long-term outcomes of cloacal malformations remain unclear. We evaluated postoperative bowel control, bladder function and quality of life in patients under 18 years of age with cloaca. MATERIALS AND METHODS: This was a multi-center cross-sectional observational study accomplished by the Nordic Pediatric Surgery Research Consortium. Patients with a cloacal malformation, 4-17 years of age, were eligible. Data including patient characteristics, surgical procedures, and complications were retrieved from case records. Established questionnaires with normative control values evaluating bowel function, bladder function, and health-related quality of life (HRQoL) were sent to the patients and their caregivers. The study was approved by the participating center's Ethics Review Authorities. RESULTS: Twenty-six (67%) of 39 eligible patients with median age 9.5 (range, 4-17) years responded. Twenty-one (81%) patients had a common channel ≤3 cm. Imaging confirmed sacral anomalies in 11 patients and spinal cord abnormalities in nine. Excluding patients with stoma (n = 5), median bowel function score was 12 [7-19], and 5 patients (20%) reported a bowel function score ≥17, approaching normal bowel control level. Bowel management increased proportion of socially continent school-aged children to 52%. Six (23%) patients had a permanent urinary diversion or used clean intermittent catheterization (CIC), while majority (70%) of the remaining patients were urinary continent. The reported HRQoL was comparable to healthy Swedish children. CONCLUSION: Whilst well-preserved spontaneous bowel control was rare, a majority of patients were dry for urine without any additional procedures. Few patients experienced social problems or negative impact on HRQoL due to bladder or bowel dysfunction. LEVEL OF EVIDENCE: Level IV.

Prevalence of Mental Health Problems, Associated Factors, and Health-Related Quality of Life in Children with Long-Gap Esophageal Atresia in Sweden.

BACKGROUND: Children with long-gap esophageal atresia (LGEA) face a high risk of digestive and respiratory morbidity, but their mental health outcomes have not been investigated. We aimed to identify the prevalence of mental health problems in children with LGEA, associated factors and health-related quality of life (HRQOL). METHODS: Twenty-six children with LGEA aged 3-17 were recruited nationwide in Sweden. One of their parents and adolescents aged 11-17 completed information on the child's mental health (Strength and Difficulties Questionnaire), generic (PedsQL 4.0) and condition-specific HRQOL (EA-QOL). Parents gave information on current child symptomatology. Mental health level was determined using validated norms; abnormal≥90 percentile/borderline≥80 percentile/normal. Elevated levels were considered borderline/abnormal. Data were analyzed using descriptives, correlation and Mann-Whitney-U test. Significance level was p < 0.05. RESULTS: Twelve children with LGEA aged 3-17 (46%) had elevated scores of ≥1 mental health domain in parent-reports, whereas 2 adolescents (15%) in self-reports. In parent-reports, 31% of the children had elevated levels of peer relationship problems, with associated factors being child sex male (p = 0.037), airway infections (p = 0.002) and disturbed night sleep (p = 0.025). Similarly, 31% showed elevated levels of hyperactivity/inattention, and associated factors were male sex (p = 0.005), asthma (p = 0.028) and disturbed night sleep (p = 0.036). Elevated levels of emotional symptoms, seen in 20%, were related to swallowing difficulties (p = 0.038) and vomiting problems (p = 0.045). Mental health problems correlated negatively with many HRQOL domains (p < 0.05). CONCLUSIONS: Children with LGEA risk mental health difficulties according to parent-reports, especially peer relationship problems and hyperactivity/inattention, with main risk factors being male sex, airway problems and sleep disturbances. This should be considered in follow-up care and research, particularly since their mental health problems may impair HRQOL. LEVELS OF EVIDENCE: Prognosis study, LEVEL II.

A nordic multicenter study on contemporary outcomes of pediatric short bowel syndrome in 208 patients.

BACKGROUND & AIMS: Despite advances in the management of short bowel syndrome related intestinal failure (SBS-IF), large-scale contemporary pediatric studies are scarce. The aim of this multicenter study was to assess key outcomes and clinical prognostic factors in a recent Nordic pediatric SBS-IF population. METHODS: Patients with SBS-IF treated during 2010-2019, whose parenteral support (PS) started at age <1 year and continued >60 consecutive days were included and retrospectively reviewed. All six participating centers followed multidisciplinary SBS-IF management. Risk factors for PS dependency, intestinal failure associated liver disease (IFALD) and mortality were assessed with Cox regression and Kaplan Meier analyses. IFALD was defined with serum liver biochemistry levels. RESULTS: Among 208 patients, SBS-IF resulted from NEC in 49%, gastroschisis w/wo atresia in 14%, small bowel atresia in 12%, volvulus in 11%, and other diagnoses in 14%. Median age-adjusted small bowel length was 43% (IQR 21-80%). After median follow up of 4.4 years (IQR 2.5-6.9), enteral autonomy was reached by 76%, none had undergone intestinal transplantation, and overall survival was 96%. Half of deaths (4/8) were caused by septic complications. Although biochemical cholestasis occurred only in 3% at latest follow-up and none of deaths were directly caused by IFALD, elevated liver biochemistry (HR 0.136; P = 0.017) and shorter remaining small bowel (HR 0.941; P = 0.040) predicted mortality. Shorter remaining small bowel and colon, and presence of end-ostomy were the main predictors of PS dependency, but not IFALD. Patients with NEC reached enteral autonomy more efficiently and had decreased incidence of IFALD compared to other etiologies. CONCLUSIONS: Although with current multidisciplinary management, prognosis of pediatric SBS is encouraging, septic complications and IFALD still associated with the remaining low mortality rate.

Predictors of histopathological esophagitis in infants and adolescents with esophageal atresia within a national follow-up programme.

PURPOSE: Esophageal atresia (EA) is a congenital anomaly of the foregut. Although the survival has improved over the years there is a significant gastrointestinal morbidity affecting physical function and health-related quality of life. The aims of the study were to identify and evaluate predictors of histopathological esophagitis in infants and adolescents with EA. METHODS: Single centre, cross-sectional study including one and 15-year-old patients operated for EA that participated in the national follow-up programme between 2012 and 2020 according to a pre-established protocol including upper endoscopy with oesophageal biopsies and 24h-pH-test. Data was collected from patients' medical records and pH-analysis software. Regression models were used to identify predictors of histopathological oesophagitis. Possible predictors were abnormal reflux index, endoscopic esophagitis, hiatal hernia, symptoms of gastroesophageal reflux (GER) and age. RESULTS: 65 patients were included, 47 children and 18 adolescents. All children were treated with PPI during their first year of life. Symptoms of GER were reported by 13 (31.7%) of the infant's caregivers, 34 of the children (72.3%) had abnormal reflux index and 32 (68.1%) had histopathological esophagitis. The corresponding numbers for adolescents were 8 (50%), 15 (83.3%) and 10 (55.6%). We found no significant associations between histopathological esophagitis and endoscopic esophagitis, symptoms of GER, hiatus hernia or age group. Abnormal reflux index was an independent predictor of histopathological esophagitis. Seven patients with normal reflux index had histopathological esophagitis, all grade I. CONCLUSIONS: We found a high prevalence of histopathological esophagitis despite PPI treatment in accordance with recommendations. No significant difference between the two age groups was seen. Abnormal reflux index was an independent predictor of histopathological esophagitis. However, we cannot recommend the use of pH-metry as a substitute for esophageal biopsies; future studies are needed to elucidate if esophageal biopsies might be postponed in infants with normal reflux index.