The association of feeding difficulties and generic health-related quality of life among children born with esophageal atresia.

BACKGROUND: Children born with esophageal atresia experience feeding difficulties. This study investigates the association of feeding difficulties and generic health-related quality of life among children aged 2-7 and 8-17 years, born with esophageal atresia. METHODS: 108 families (n = 36 aged 2-7 years; n = 72 aged 8-17) answered a survey regarding difficulties in their child's mealtimes and a validated generic health-related quality of life instrument(PedsQL 4.0). Clinical data was collected from hospital records. The association of feeding difficulties and health-related quality of life was analysed trough Mann-Whitney U-test. Linear regression determined whether the number of concurrent feeding difficulties in the child decreased the health-related quality of life scores. P < 0.05 was considered significant. RESULTS: In children aged 2-7 and 8-17 years, to have a gastrostomy, to use a food infusion pump, need for energy-enriched food and eating small portions were respectively significantly associated with lower total health-related quality of life scores in the parent-reports (p < 0.05). Most of the feeding difficulties had a negative significant relationship with the domains of physical and social functioning. Additionally, in the older age group, long mealtimes and adult mealtime supervision were associated with lower scores in both child and parent reports. In both age groups, an increased number of feeding difficulties in the child decreased the total generic health-related quality of life scores (p < 0.01). CONCLUSION: Specific feeding difficulties are associated with low health-related quality of life among children with esophageal atresia. An increasing number of feeding difficulties is associated to decreasing health-related quality of life-scores. Further research is needed to understand these associations.

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.

Postoperative morbidity and health-related quality of life in children with delayed reconstruction of esophageal atresia: a nationwide Swedish study.

BACKGROUND: In 10-15% of children with esophageal atresia (EA) delayed reconstruction of esophageal atresia (DREA) is necessary due to long-gap EA and/or prematurity/low birth weight. They represent a patient subgroup with high risk of complications. We aimed to evaluate postoperative morbidity and health-related quality of life (HRQOL) in a Swedish national cohort of children with DREA. METHODS: Postoperative morbidity, age-specific generic HRQOL (PedsQL™ 4.0) and condition-specific HRQOL (The EA-QOL questionnaires) in children with DREA were compared with children with EA who had primary anastomosis (PA). Factors associated with the DREA group's HRQOL scores were analyzed using Mann-Whitney U-test and Spearman's rho. Clinical data was extracted from the medical records. Significance level was p < 0.05. RESULTS: Thirty-four out of 45 families of children with DREA were included and 30 returned the questionnaires(n = 8 children aged 2-7 years; n = 22 children aged 8-18 years). Compared to children with PA(42 children aged 2-7 years; 64 children aged 8-18 years), there were no significant differences in most early postoperative complications. At follow-up, symptom prevalence in children aged 2-7 with DREA ranged from 37.5% (heartburn) to 75% (cough). Further digestive and respiratory symptoms were present in ≥ 50%. In children aged 8-18, it ranged from 14.3% (vomiting) to 40.9% (cough), with other digestive and airway symptoms present in 19.0-27.3%. Except for chest tightness (2-7 years), there were no significant differences in symptom prevalence between children with DREA and PA, nor between their generic or condition-specific HRQOL scores (p > 0.05). More children with DREA underwent esophageal dilatations (both age groups), gastrostomy feeding (2-7 years), and antireflux treatment (8-18 years), p < 0.05. Days to hospital discharge after EA repair and a number of associated anomalies showed a strong negative correlation with HRQOL scores (2-7 years). Presence of cough, airway infection, swallowing difficulties and heartburn were associated with lower HRQOL scores (8-18 years), p < 0.05. CONCLUSIONS: Although children with DREA need more treatments, they are not a risk group for postoperative morbidity and impaired HRQOL compared with children with PA. However, those with a long initial hospital stay, several associated anomalies and digestive or respiratory symptoms risk worse HRQOL. This is important information for clinical practice, families and patient stakeholders.

Factors of family impact in a Swedish-German cohort of children born with esophageal atresia.

BACKGROUND: After repair of esophageal atresia (EA), childhood survivors commonly present with digestive and respiratory morbidity, and around 55% have associated anomalies. Although it is known that these problems can reduce health-related quality of life in children with EA, less is understood about the impact on the family. We aimed to identify factors related to family impact in children with EA. METHODS: One parent each of a child with EA (2-18 years) in 180 families from Sweden and Germany answered the PedsQL™ Family Impact Module as the dependent variable. The independent variables were the child's parent-reported health-related quality of life as measured by PedsQL™ 4.0, current symptoms, school situation, and parent/family characteristics together with child clinical data from the medical records. RESULTS: Stepwise multivariable regression analysis showed a multifactorial model of the total family impact scores (R2 = 0.60), with independent factors being the child's overall generic health-related quality of life, school-absence ≥ 1/month, severe tracheomalacia, a family receiving carer's allowance, and a parent with no university/college education, p < 0.05. Logistic regression analysis showed that an increased number of symptoms in the child the preceding 4 weeks lowered the family impact scores; however, the child's feeding (R2 = 0.35) and digestive symptoms (R2 = 0.25) explained more in the variation of scores than the child's respiratory symptoms (R2 = 0.09), p < 0.0001. CONCLUSIONS: Family functioning may be a contributing factor to the maintenance of child health. The study findings suggest multifactorial explanations to family impact in children with EA, which are essential when optimizing the support to these families in clinical and psychosocial practice. Future research should explore experiences of family impact from all family members' perspectives and multicenter studies are warranted to understand better the effectiveness of psychosocial-educational interventions to families of children with EA.

Linguistic and content validity of the Swedish version of the PedsQL™ gastrointestinal symptoms scales and symptoms module for paediatric patients.

AIM: To describe the process of linguistic and content validity of the Swedish version of the PedsQL™ Gastrointestinal Symptoms Scales and Symptoms Module, measuring health-related quality of life (HRQOL) in children with gastrointestinal (GI) disorders. METHODS: The establishment of linguistic and content validity was carried out in compliance with international standards on patient-reported outcome measurements. The process included forward translation, expert review and reconciliation, backward translation, backward translation review and interviews with 15 children aged 5-18 years with GI tract symptoms and 20 parents of children with GI tract symptoms aged 2-18 years. RESULTS: The Swedish version of the PedsQL™ Gastrointestinal Symptoms Scales and Symptoms Module (child report 5-18 years, parent report for children 2-18 years) was achieved without major difficulties. Eight issues needed discussion after forward translation, and there was one change after backward translation and three revisions following patient and parent testing. CONCLUSION: A conceptually equivalent Swedish language version of PedsQL™ Gastrointestinal Symptoms Scale and Symptoms Module for children aged 2-18 years old was developed. This enables improved HRQOL evaluations in children with GI disorders in Sweden. Future research using a larger sample is recommended to evaluate validity and reliability of the Swedish language version of the module.

Parent-child-agreement on health-related quality of life and its determinants in patients born with Esophageal Atresia: a Swedish-German cross-sectional study.

BACKGROUND: The aim was to compare parent and child-reported health-related quality of life (HRQOL) of children born with esophageal atresia (EA) and determine factors that affect the level of parent-child agreement. METHODS: We included 63 parent-child dyads of children born with EA aged 8-18 from Germany and Sweden. The generic PedsQL 4.0™ questionnaire and the condition-specific EA QOL questionnaire were used to assess children's HRQOL from parents' and children's perspectives. The PedsQL™ Family Impact Module was used to assess parental HRQOL and Family Functioning. RESULTS: On an individual level, intra-class correlation coefficients indicated strong levels of parent-child agreement (.61-.97). At the group level, the analyses showed no significant differences between the responses of parents and children. When a disagreement occurred, parents were more likely to rate generic HRQOL lower than the children (19-35%) and condition-specific HRQOL higher than the children (17-33%). Findings of the binary logistic regression analyzes showed that the child's age, gender, and country (Germany vs. Sweden) were significant predictors of parent-child agreement in condition-specific HRQOL. We did not identify any significant variables that explain agreement for the generic HRQOL. CONCLUSION: The parent-child agreement is mostly good, suggesting that parent-reports are a reliable source of information. However, discrepancies may occur and can be explained by the child's age, gender, and country (Sweden vs. Germany). Both perspectives are essential sources for treating EA patients and should not be considered right or wrong. Instead, this information broadens the perspective on pediatric EA patients.

Parent-Reported Feeding Difficulties among Children Born with Esophageal Atresia: Prevalence and Early Risk Factors.

INTRODUCTION: We aimed to describe the prevalence of observable feeding difficulties during mealtimes among children with repaired esophageal atresia (EA) and to determine their early predictors. MATERIALS AND METHODS: A survey, based on parents' reports and concerning difficulties in EA children's nutritional intake, was performed with the help of 114 families of 2 to 17-year-old EA patients. Neonatal and clinical/surgical data were collected from medical records. Comparisons were made of the prevalence n (%) of feeding difficulties between children in three age groups (2 to 7 years, 8 to 12 years, or 13 to 17 years of age) using the Mantel-Haenszel chi-square test. Logistic regression identified outcome predictors (odds ratio: 95% confidence interval). Predictors with p ≤ 0.1 in the univariable analysis were included in multiple regression analysis (p < 0.05). RESULTS: Seventy-five percent of the young children aged 2 to 7, (median number of feeding difficulties: 2), 61% of school-aged children aged 8 to 12 (median number of feeding difficulties: 1), and 60% patients in the teenage group, aged 13 to 17, (median number of feeding difficulties: 1), reported feeding difficulties. Surgical complications after EA repair independently predicted children having a gastrostomy (p ≤ 0.01), using a food infusion pump (p ≤ 0.01), taking small portions to facilitate eating (p = 0.01), and needing >30 minutes to finish a main meal (p = 0.02). Congenital independent predictors were VACTERL, low birth weight, and preterm birth. CONCLUSION: Parentally observed feeding difficulties were commonly reported during early childhood, although prevalence decreases in older age groups. Several congenital and surgical factors were identified as independent predictors of complicated nutritional intake patterns.

Partial Biliary Diversion May Promote Long-Term Relief of Pruritus and Native Liver Survival in Children with Cholestatic Liver Diseases.

INTRODUCTION: Rare cholestatic liver diseases may cause debilitating pruritus in children. Partial biliary diversion (PBD) may relieve pruritus and postpone liver transplantation which is the only other alternative when conservative treatment fails. The aim was to report long-term outcome after PBD in a population of 26 million people during a 25-year period. MATERIALS AND METHODS: This is an international, multicenter retrospective study reviewing medical journals. Complications were graded according to the Clavien-Dindo classification system. RESULTS: Thirty-three patients, 14 males, underwent PBD at a median of 1.5 (0.3-13) years at four Nordic pediatric surgical centers. Progressive familial intrahepatic cholestasis was the most common underlying condition. Initially, all patients got external diversion, either cholecystojejunostomy (25 patients) or button placed in the gallbladder or a jejunal conduit. Early complications occurred in 14 (42%) patients, of which 3 were Clavien-Dindo grade 3. Long-term stoma-related complications were common (55%). Twenty secondary surgeries were performed due to stoma problems such as prolapse, stricture, and bleeding, or conversion to another form of PBD. Thirteen children have undergone liver transplantation, and two are listed for transplantation due to inefficient effect of PBD on pruritus. Serum levels of bile acids in the first week after PBD construction were significantly lower in patients with good relief of pruritus than in those with poor effect (13 [2-192] vs. 148 [5-383] µmol/L; p = 0.02). CONCLUSION: PBD may ensure long-term satisfactory effect on intolerable pruritus and native liver survival in children with cholestatic liver disease. However, stoma-related problems and reoperations are common.

Clinical Factors Affecting Condition-Specific Quality-of-Life Domains in Pediatric Patients after Repair of Esophageal Atresia: The Swedish-German EA-QOL Study.

INTRODUCTION: We aimed to identify clinical factors affecting condition-specific health related quality of life (HRQOL) domains in children born with esophageal atresia (EA). This can facilitate preventive care to risk groups of HRQOL impairments. MATERIALS AND METHODS: A total of 124 Swedish and German families of EA children answered the validated EA-QOL questionnaires (response rate 68%), for evaluation of three HRQOL domains in children 2 to 7 years old (53 parents) and four HRQOL domains in children 8 to 17 years old (62 children/71 parents). Clinical data were collected through medical records and a questionnaire. Statistics included between-group analysis, univariable and stepwise multivariable regression analysis, p < 0.05. RESULTS: Between 2 to 7 years, no primary anastomosis (p = 0.022) and female gender (p = 0.026) predicted worse scores related to "physical health and treatment," and gastrostomy insertion related to "eating" (p = 0.0001), and "social isolation and stress" (p = 0.001). Between 8 to 17 years, no primary anastomosis (child report), prematurity, esophageal dilatation (parent report) predicted poor HRQOL related to "eating" (p < 0.05), associated anomalies to "body perception" (p = 0.031, parent report), female gender (p = 0.018, child report) and severe EA (p = 0.011 child report, p = 0.004 parent report) to "social relationships," and severe EA predicted worse "health and well-being" scores (p = 0.004, parent report). An increased number of digestive symptoms (difficulty swallowing food, heartburn, and vomiting), lowered all EA-QOL domain scores in both age groups (p < 0.001). An increased number of respiratory problems (cough, wheezing, airway infections. breathlessness, and chest tightness), lowered scores in two HRQOL domains among children 2 to 7 years (p < 0.05). CONCLUSION: Impairments within condition-specific HRQOL domains in EA children are found in congenital and surgical subgroups, and notably related to digestive symptoms throughout childhood.

Management strategies and treatment results of pediatric choledochal malformations in the Nordic countries.

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.

Long-Term Effectiveness of Antireflux Surgery in Esophageal Atresia Patients.

INTRODUCTION: Gastroesophageal reflux disease (GERD) is a common morbidity after esophageal atresia (EA) repair, and the antireflux procedure (ARP) is a way of treating GERD symptoms. The aim of this study was to evaluate whether reflux index (Ri) and growth were improved by ARP. MATERIALS AND METHODS: Ninety-nine individuals with EA treated at the Queen Silvia Children's Hospital in Gothenburg, Sweden, between 1997 and 2010 were followed prospectively according to a structured care program. Twenty-four-hour pH-metry (Ri) and growth were studied at birth, then at 1, 7, and 15 years of age. All the patients included had reached 7 years of age. RESULTS: Preoperatively, Ri was significantly higher (32%) in the ARP than the non-ARP group (10%). Postoperatively, no difference was seen between the groups. However, at 7 and 15 years of age, Ri was significantly higher in the ARP group than in the non-ARP group. Weight (standard deviation scores) was significantly lower in the ARP group at 1 and 7 years of age when compared with the non-ARP group, but these differences were not seen at birth and at 15 years of age. In a multivariate analysis, only birth weight remained a significant factor for low weight at 7 years of age. At 15 years of age, no risk factors for low body weight were found. CONCLUSION: In the long term, ARP is not effective in reducing GERD as measured as Ri in EA patients. The ARP group had significantly lower weight at 1 and 7 years of age than the non-ARP group, but this was not the case at the age of 15.

Health-related quality of life experiences in children and adolescents born with esophageal atresia: A Swedish-German focus group study.

BACKGROUND: Esophageal atresia (EA) is a rare malformation of the esophagus, which needs surgical treatment. Survival rates have reached 95%, but esophageal and respiratory morbidity during childhood is frequent. Child and parent perspectives and cultural and age-specific approaches are fundamental in understanding children's health-related quality of life (HRQoL) and when developing a pediatric HRQoL questionnaire. We aimed to increase the conceptual and cross-cultural understanding of condition-specific HRQoL experiences among EA children from Sweden and Germany and investigate content validity for an EA-specific HRQoL questionnaire. METHODS: Eighteen standardized focus groups (FGs) with 51 families of EA children aged 2-17 years in Sweden (n = 30 families) and Germany (n = 21 families) were used to explore HRQoL experiences, which were content analyzed into HRQoL domains. The Swedish HRQoL domains were analyzed first and used as framework to evaluate HRQoL content reported in the German FGs. HRQoL experiences were then categorized as physical, social, and emotional HRQoL burden or resource. RESULTS: One thousand nine hundred eight HRQoL statements were recorded. All nine EA-specific HRQoL domains identified in the Swedish FGs (eating, social relationships, general life issues, communication, body issues, bothersome symptoms, confidence, impact of medical treatment, and additional difficulties due to concomitant anomalies) were recognized in the FGs held in Germany, and no additional EA-specific HRQoL domain was found. The HRQoL dimensions referenced physical burden (n = 655, 34.5%), social burden (n = 497, 26.0%), social resources (n = 303, 15.9%), emotional burden (n = 210, 11.0%), physical resources (n = 158, 8.3%), and emotional resources (n = 85, 4.5%). CONCLUSION: This first international FG study to obtain the EA child and his or her parents' perspective on HRQoL suggests Swedish-German qualitative comparability of the HRQoL domains and content validity for a cross-cultural EA-specific HRQoL questionnaire. EA children make positive and negative HRQoL experiences, but prominently related to physical and social burden, which underlines appropriate follow-up care and future research.

Generic Health-Related Quality of Life after Repair of Esophageal Atresia and Its Determinants within a German-Swedish Cohort.

AIM: Despite advances of outcomes of esophageal atresia (EA), knowledge on patients' health-related quality of life (HRQoL) is sparse. Due to the heterogeneity of EA, larger cohorts need to be investigated to ensure reliability of data. Aim was to determine generic HRQoL after EA repair in a Swedish-German cohort. PATIENTS AND METHODS: Ethical approval was obtained. A total of 192 patients (2-18 years; 134 Swedish; 58 German) were included. Clinical data were reviewed. EA was classified in "severe" and "mild/moderate." Pediatric Quality of Life Inventory 4.0 Generic Core Scale (PedsQL 4.0) was used in appropriate versions (2-7 years; 8-18 years; self- [SR] and proxy report [PR]) to determine generic HRQoL. RESULTS: Swedish and German samples were clinically and demographically comparable. HRQoL was lower in "severe EA" versus "mild/moderate" (2-18 years; total score; PR 85.6 vs. 73.6; p < 0.001) and Gross A versus Gross C type EA (2-7 years; total score; PR 61.0 vs. 79.3; p = 0.035). Total HRQoL was higher in the Swedish versus German sample (2-18 years; total score; PR 82.3 vs. 72.7; p = 0.002). HRQoL was impaired in the German sample versus healthy population (2-18 years; total score; PR 72.7 vs. 82.7; p = 0.001). In German patients (8-18 years), HRQoL was higher in SR versus PR (80.7 vs. 74.7; p = 0.044). Patients' age and presence of VACTERL association or isolated anorectal malformations did not affect HRQoL. Various differences were detected regarding different dimensions of PedsQL 4.0. CONCLUSION: In this first international study, we found several differences in perception of generic HRQoL. HRQoL appears to be determined by the type of EA and severity rather than patients' age or the presence of typical associated malformations. Country-specific differences may be culturally dependent, but further investigations are suggested. A condition-specific instrument validated for EA may provide additional insights.

The Esophageal-Atresia-Quality-of-life Questionnaires: Feasibility, Validity and Reliability in Sweden and Germany.

OBJECTIVES: Esophageal atresia (EA) is a rare malformation characterized of discontinuity of the esophagus, concurrent with or without a tracheoesophageal fistula (TEF). We report the feasibility validity and reliability of a condition-specific quality-of-life (QOL) tool for EA/TEF children, the age-adapted EA-QOL-questionnaires, when used in Sweden and Germany. METHODS: A total of 124 families of children with EA/TEF participated in the study; 53 parents completed the EA-QOL-questionnaire for children aged 2 to 7 years; 62 children/71 parents the EA-QOL-questionnaire for children 8 to 17 years. Feasibility was determined from the percentage of missing item responses. Based on clinical data and previously validated generic QOL-instruments (PedsQL 4.0, DISABKIDS-12), the final EA-QOL scores were evaluated against hypotheses of validity (known-groups/concurrent/convergent) and reliability (internal consistency/retest reliability of scores for 3 weeks). Significant level was P < 0.05. RESULTS: In the questionnaire for EA/TEF children aged 2 to 7 years, 16/18 items were completed with missing values <6% (range 0%-7.5%), and in the questionnaire for 8 to 17-year-olds, 24/24 child-reported items (range 0%-4.8%) and 21/24 parent-reported items (range 0%-7.0%). In both age-specific EA-QOL-questionnaires, desirable standards for known-groups and concurrent validity were fulfilled; digestive symptoms and feeding difficulties negatively impacted EA-QOL-Total-scores (P < 0.001), and as hypothesized, in 2 to 7-year-olds, respiratory symptoms decreased EA-QOL-Total-scores (P = 0.002). Correlations between the EA-QOL and generic QOL questionnaires supported convergent validity. Internal consistency reliability was satisfactory. The level of agreements of EA-QOL-scores between the field- and retest study were good to excellent. CONCLUSIONS: The overall psychometric performance of the EA-QOL-questionnaires for EA/TEF children is satisfactory and can enhance outcome evaluations in future research and clinical practice.

Clinical predictors and prevalence of receiving special preschool/school support in children with repaired esophageal atresia.

BACKGROUND/PURPOSE: In a sparsely investigated field, we aimed to evaluate the use of special preschool/school support among children with repaired esophageal atresia (EA) and/or tracheoesophageal fistula (TEF), the predicting clinical factors for this support, and level of school absence. METHODS: Data on 119 EA/TEF children 2-17years old were collected through medical records and questionnaires (response rate 95%). Logistical regression analysis identified clinical predictors of special preschool/school support in the population without genetic disorders (n=105). Nominal hypothesis testing was performed using Fisher's exact test (p<0.05). RESULTS: Of the 119 children, 35.3% received special preschool/school support; 26.8% educational support, 21.8% support with nutritional intake issues and 13.4% received both types of support. Educational support was independently predicted by birth weight<2500g (p=0.026) and associated anomalies (p=0.049), nutritional intake support by gastrostomy insertion (p=0.0028), and both types of supports by major revisional surgery (p=0.0081). School absence ≥1month/year, present in 25.5% of the children, was more frequently reported in children receiving preschool/school support, in preschoolers and in those with persistent respiratory problems (p<0.05). CONCLUSIONS: Special preschool/school support is provided for approximately one-third of EA/TEF children. In EA/TEF children without genetic disorders, use of this support is predicted by congenital and surgical factors, and related to frequent school absence.

Outcomes of biliary atresia in the Nordic countries - a multicenter study of 158 patients during 2005-2016.

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.

Macrophage Phenotype Is Associated With the Regenerative Response in Experimental Replacement of the Porcine Esophagus.

A porcine model for bridging circumferential defects in the intrathoracic esophagus has been developed in order to improve the treatment of children born with long-gap esophageal atresia. The aim of this study was to identify factors beneficial for tissue regeneration in the bridging area in this model and to describe the histological progression 20 days after replacement with a silicone-stented Biodesign mesh. Resection of 3 cm of intrathoracic esophagus and replacement with a bridging graft was performed in six newly weaned piglets. They were fed through a gastrostomy for 10 days, and then had probe formula orally for another 10 days prior to sacrifice. Two out of six piglets had stent loss prior to sacrifice. In the four piglets with the stent in place, a tissue tube, with visible muscle in the wall, was seen at sacrifice. Histology showed that the wall of the healing area was well organized with layers of inflammatory cells, in-growing vessels, and smooth muscle cells. CD163+ macrophages was seen toward the esophageal lumen. In the animals where the stent was lost, the bridging area was narrow, and histology showed a less organized structure in the bridging area without the presence of CD163+ macrophages. This study indicates that regenerative healing was seen in the porcine esophagus 20 days after replacement of a part of the intrathoracic esophagus with a silicone-stented Biodesign mesh, if the bridging graft is retained. If the graft is lost, the inflammatory pattern changes with invasion of proinflammatory, M1 macrophages in the entire wall, which seems to redirect the healing process toward scar formation.

Added Value of pH Multichannel Intraluminal Impedance in Adults Operated for Esophageal Atresia.

BACKGROUND: Gastroesophageal reflux (GER) and dysphagia are common following repaired esophageal atresia (EA). The risk of esophagitis and Barrett esophagus is increased compared with the general population. As yet, the causes are not fully explained. PURPOSE: The aim of this study was to investigate how GER, measured by pH multichannel intraluminal impedance (pH-MII), is correlated to the esophageal symptoms and histological findings. METHODS: Twenty-nine adult subjects operated for EA in Gothenburg from 1968 to 1983 were evaluated with pH-MII, manometry, and gastroscopy. RESULTS: pH-MII was performed in 15, manometry in 19, and gastroscopy in 24 subjects. Eleven subjects displayed pathological reflux parameters of any kind, mainly nonacid reflux (10/15). Dysphagia correlated to the number of weakly acidic reflux episodes. Lower esophageal sphincter (LES) incompetence, which correlated to a pathological number of acid reflux episodes (p = 0.012), was noted in 21/24 subjects, but the majority had a normal resting pressure. Esophagitis was present in 14/24, two of whom had Barrett esophagus. Histological changes correlated to the reflux index and the number of weakly acidic reflux episodes (p = 0.028 and 0.040) and tended to correlate to dysphagia (p = 0.052). CONCLUSION: pH-MII adds further information when it comes to explaining what causes symptoms and esophageal histological changes in adults operated for EA.

Treatment and Follow-Up of Patients with Long-Gap Esophageal Atresia: 15 Years' of Experience from the Western Region of Sweden.

OBJECTIVE: This retrospective study aims to report treatment results in patients with long-gap esophageal atresia (LGEA), gross A + B type, and discuss the value of different clinical findings and physiological tests in the follow-up. METHODS: This retrospective observational study comprises all patients with LGEA admitted to our department between 1995 and 2010. RESULTS: A total of 16 patients were included. Their mean gestational age was 35(+2) weeks and their mean birth weight was 1,945 g (-2.5 standard deviation scores). No catch-up growth in height could be seen and they remained smaller than the average population during the study period. Gastrostomy was performed as the first surgical procedure. Overall, 11 of the 16 patients had a delayed primary anastomosis. Elongation of the distal esophageal segment was required in 3 of the 16 patients and a colonic interposition in 2 of the 16 patients. The median age at definitive surgery was 150 days. All the patients had gastroesophageal reflux after their definitive surgery. Three of the 16 patients required surgery due to aspiration and all 3 had a pathological lung clearance index (LCI) at multiple-breath washout (MBW). At the age of 1 or 7 years, the LCI was pathological in 4 of the 14 patients, and spirometry showed an obstruction in 9 of the 14 patients. CONCLUSION: LGEA is a severe congenital malformation, with severe morbidity. No mortality was seen. MBW could be a useful tool for the early detection of progressive pulmonary damage.

Health-related quality of life experiences among children and adolescents born with esophageal atresia: Development of a condition-specific questionnaire for pediatric patients.

BACKGROUND/PURPOSE: The aims were to present the framework for the development a condition-specific health-related quality-of-life (HRQOL) questionnaire for children with esophageal atresia (EA) and to describe HRQOL experiences reported by children and by their parents. METHODS: Utilizing the well-established DISABKIDS methodology, standardized focus group discussions were held and transcribed. HRQOL experiences were identified, content analyzed and evaluated using descriptive statistics. RESULTS: 30 families (18 children 8-17years, 32 parents of children 2-17years) participated in ten focus group discussions. 1371 HRQOL experiences were identified referencing social, emotional and physical aspects of eating and drinking (n=368), relationships with other people (n=283), general life issues; physical activity, sleep and general health (n=202), communicative/interactive processes of one's health condition (n=161), body issues (n=109), bothersome symptoms (n=81), impact of health care use/medical treatment (n=78), confidence in oneself and in the future (n=65) and difficulties because of concomitant anomalies (n=24). A basis of two age-related HRQOL questionnaires for children with EA (2-7years, 8-17years) was subsequently constructed. CONCLUSIONS: EA interacts with various aspects of the child's life. In addition to HRQOL issues of eating and drinking, social dimensions like relationships and interactions with other people seem to be prominent condition-specific HRQOL parameters. The settings for the development of the first condition-specific HRQOL questionnaires for patients with EA are established.