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Generic and condition-specific health-related quality of life in symptom subgroups of children with delayed reconstruction of esophageal atresia
Source publication
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 Swedi...
Context in source publication
Context 1
... age group 8-18, there were no significant differences in EA-QOL scores between children with DREA and PA (p > 0.05), or as viewed in Fig. 6a-b between those with DPA, ER or PA (p > 0.05). Table 3 presents generic and condition-specific HRQOL in children aged 8-18 with DREA with and without digestive and respiratory symptoms (in subgroups with ≥ 5 observations). Swallowing difficulties, heartburn, cough or airway infections were significantly associated with lower generic and/or condition-specific HRQOL scores, p < 0.05. ...Similar publications
Introduction:
Surgical repair of recurrent tracheoesophageal fistula has a high risk of complications. Therefore, various endoscopic techniques have been used to avoid complications.
Objective:
To understand the usefulness of trichloroacetic acid endoscopic application for the treatment of recurrent tracheoesophageal fistula.
Materials and meth...
Citations
... or full enteral tube feeding, which in turn can cause severe strictures [35]. It has also been shown that digestive problems have a negative impact on health-related quality of life [36]. In our study parents expressed a belief that sham feeding had normalized the child's eating behavior and that the child was eager to start eating after reconstructive surgery. ...
Purpose
This study aimed to explore parents’ experience of sham feeding their baby born with esophageal atresia at home, waiting for reconstructive surgery.
Method
Semi-structured interviews were conducted with parents of six children born with esophageal atresia waiting for delayed reconstruction. The interviews were analyzed using qualitative content analysis.
Results
Parents experienced that sham feed reinforced the healthy abilities in their baby. They had faith in their own ability as parents to care for their child as well as to see to their baby’s strength to cope with difficulties. Parents expressed that the health care system can hinder as well as be a major support on their way to a more normal life at home while waiting for reconstructive surgery.
Conclusion
The experience of sham feeding at home while waiting for reconstructive surgery is characterized by positive aspects both for children born with esophageal atresia and their parents.
... Under this definition, some cases with a very short upper esophageal segment and distal tracheoesophageal fistula or those where the distal fistula is connected to the left or right bronchus (as opposed to the trachea) would be included in this surgical definition of LGEA. Moreover, many extremely premature EA patients may also be difficult to repair at birth, regardless of the specific anatomy, due to the fragility of the esophagus in very low birthweight infants (4). ...
... The diagnosis of LGEA is synonymous with a prolonged hospitalization and worse long-term outcomes when compared to those without EA (4,13,14). There have been several expert opinion consensus guidelines established by the American Pediatric Surgical Association (APSA), as well as the European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) and the International Network of Esophageal Atresia (INoEA) (2,3,15). ...
A uniquely challenging subset of infants diagnosed with esophageal atresia (EA) are those born with long-gap EA (LGEA). The common unifying feature in infants with LGEA is that the proximal and distal segments of the esophagus are too far apart to enable primary anastomosis via a single operation in the newborn period. Although any type of EA can technically result in a long gap, the Gross type A variant occurs in 8% of all EA cases and is most commonly associated with LGEA. In this review, we provide an evidence-based approach to the current challenges and management strategies employed in LGEA. There are fortunately a range of available surgical techniques for LGEA repair, including delayed primary repair, staged repair based on longitudinal traction strategies to lengthen both ends (e.g., Foker procedure, internal traction), and esophageal replacement using other portions of the gastrointestinal tract. The literature on the management of LGEA has long been dominated by single-center retrospective reviews, but the field has recently witnessed increased multi-center collaboration that has helped to increase our understanding on how to best manage this challenging patient population. Delayed primary repair is strongly preferred as the initial approach in management of LGEA in the United States as well as several European countries and is supported by the American Pediatric Surgery Association recommendations. Should esophageal replacement be required in cases where salvaging the native thoracic esophagus is not possible, gastric conduits are the preferred approach, based on the relative simplicity of the operation, low postoperative morbidity, and long-term durability. Long-term followup for monitoring of swallowing function, nutritional status, aspiration/respiratory illnesses, gastroesophageal reflux, and associated comorbidities is essential in the comprehensive care of these complex patients.
... Similarly, a large multicentre study of management and outcomes of isolated LGOA treatment using DPA revealed a high rate of successful reconstructions, but prolonged LOHS (median 125 days) with a median age at repair of 87 days [23]. A long initial hospital stay, as well as significant associated anomalies, and persistent digestive or respiratory symptoms may negatively influence on health related quality of life (HRQOL) [24]. Other studies have also remarked that a prolonged hospital stay may be related to suboptimal long-term morbidity and neurodevelopment outcomes [25,26]. ...
Objective Management of long gap esophageal atresia (LGOA) is controversial. This study aims at comparing the management of LGOA between two high-volume centers.
Methods We included patients with LGOA (type A and B) between 2008 and 2022. Demographics, surgical methods, and outcomes were collected and compared.
Results The study population involved 28 patients in center A and 24 patients in center B. A surgical approach was thoracoscopic in center A, only for one patient was open for final procedure. In center B, 3 patients were treated only thoracoscopically, 2 converted to open, and 19 as open surgery. In center A primary esophageal anastomosis concerned 1 case, two-staged esophageal lengthening using external traction 1 patient, and 26 were treated with the multistaged internal traction technique. In 24 patients a full anastomosis was achieved: in 23 patients only the internal traction technique was used, while 1 patient required open Collis–Nissen procedure as final management. In center B primary anastomosis was performed in 7 patients, delayed esophageal anastomosis in 8 patients, esophageal lengthening using external traction in 1 case, and 9 infants required esophageal replacement with gastric tube. Analyzed postoperative complications included: early mortality, 2/28 due to accompanied malformations (center A) and 0/24 (center B); anastomotic leakage, 4/26 (center A) treated conservatively—all patients had a contrast study—and 0/24 (center B), 1 case of pleural effusion, but no routine contrast study; recurrent strictures, 13/26 (center A) and 7/15 (center B); and need for fundoplication, 5/26 (center A) and 2/15 (center B). Age at esophageal continuity was as a median of 31 days in center A and 110 days in center B. Median time between initial procedure and esophageal anastomosis was 11 days in center A and 92 days in center B.
Conclusion Thoracoscopic internal traction technique reduces time to achieve esophageal continuity and the need for esophageal substitution while maintaining a similar early complication rate.
... Demographic and clinical data were collected from a standardized questionnaire and hospital records respectively. At our institution patients attended the Swedish national standardized follow-up program for children with EA, [27] and additionally if clinical problems arose that needed attention. ...
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.
... does not yet formally include a standardized provision of psychological consultation to the child [44], but the pediatric surgeon can mandate the child to have school-based support, which seems to work adequately in most cases of children with LGEA in Sweden. ...
Background: Children with long-gap esophageal atresia (LGEA) risk living with aerodigestive morbidity and mental health difficulties. No previous study has investigated their experiences of schooling, despite the importance of schools in children’s development, learning and social relationships. We aimed to describe experiences of schooling in children with LGEA in Sweden.
Method: Twenty-six children with LGEA aged 3-17 were recruited nationwide in Sweden. One parent completed a survey on their child’s school-based supports (according to definitions from the Swedish National Agency for Education), school absence, school satisfaction, school functioning (PedsQL 4.0), mental health (Strength and Difficulties Questionnaire) and current symptomatology. School data were compared to that from 95 children with EA who had primary anastomosis (PA), a hypothesized milder affected group. Mental health level was determined using validated norms; abnormal≥90 percentile. Data were analyzed using descriptives, correlation and Mann-Whitney-U test. Significance level was p<0.05.
Results: Seventeen children with LGEA (65.4%) had formal school-based support concerning nutritional intake (60%), educational needs (50%) and medical/special health needs (35%), which was significantly more frequent compared to children with PA overall (36.8%, p=0.013) and regarding nutritional intake support in school (20%, p< 0.001). In children with LGEA, school-based support was related to low birth weight (p=0.036), young child age (p=0.014), height ≤-2SD for age/sex (p=0.024) and an increased number of aerodigestive symptoms (p<0.05). All children with LGEA who had abnormal scores of mental health, except for one child, had school-based support. Nine children with LGEA (36%) had school absence ≥1times/month the past year, more frequently because of colds/airway infections (p=0.045) and gastro-intestinal related problems compared to PA (p=0.003). School functioning scores were not significantly different from children with PA (p=0.34) but correlated negatively with school-based support (<0.001) and school absence (p=0.002). One parent out of 26 reported their child’s school satisfaction as “not good”.
Conclusions: Children with LGEA commonly receive school-based support, reflecting multifaceted daily needs and disease severity. School absence is frequent and related to poorer school functioning. Collaborative strategies between specialized health care providers and schools are needed to accommodate for their disease-specific needs in school. Future research focusing on academic achievement in children with EA is needed.
... Recent reports suggest that mental health and HRQoL is only partially affected in children and adolescent with EA patients. More precise, EA patient seem to have emotional and behavioral problems when compared to the normative population [15][16][17][18]. No differences in the child's HRQoL, between short and long gaps, have been reported [11,19,20]. ...
Introduction: Pediatric surgeons have yet to reach a consensus whether a gastric sleeve pull-up or delayed primary anastomosis for the treatment of esophageal atresia (EA), especially of the long-gap type (LGEA) should be performed. Thus, the aim of this study was to evaluate clinical outcome, quality of life (QoL), and mental health of patients with EA and their parents.
Methods: Clinical outcomes of all children treated with EA from 2007 to 2021 were collected and parents of affected children were asked to participate in questionnaires regarding their Quality of Life (QoL) and their child’s Health-Related Quality of Life (HRQoL), as well as mental health.
Results: A total of 98 EA patients were included in the study. For analysis, the cohort was divided into two groups: (1) primary versus (2) secondary anastomosis, while the secondary anastomosis group was subdivided into (a) delayed primary anastomosis and (b) gastric sleeve pull-up and compared with each other. When comparing the secondary anastomosis group, significant differences were found between the delayed primary anastomosis and gastric sleeve pull-up group; the duration of anesthesia during anastomosis surgery (478.54 vs 328.82 min, p < 0.001), endoscopic dilatation rate (100% vs 69%, p = 0.03), cumulative time spent in intensive care (42.31 vs 94.75 days, p = 0.03) and the mortality rate (0% vs 31%, p = 0.03). HRQoL and mental health did not differ between any of the groups.
Conclusion: Delayed primary anastomosis or gastric sleeve pull-up appear to be similar in patients with long-gap esophageal atresia in many key aspects like leakage rate, strictures, re-fistula, tracheomalacia, recurrent infections, thrive or reflux. Moreover, HrQoL was comparable in patients with (a) gastric sleeve pull-up and (b) delayed primary anastomosis. Future studies should focus on the long-term results of either preservation or replacement of the esophagus in children.
... Demographic and clinical data were collected from a standardized questionnaire and hospital records respectively. At our institution patients attended the Swedish national standardized follow-up program for children with EA, [27] and additionally if clinical problems arose that needed attention. ...
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 questions about 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: Parent-reported feeding difficulties are associated with low health-related quality of life among children with esophageal atresia. A high number of feeding difficulties is associated to lower health-related quality of life. Further research is needed to understand these associations.
Introduction Patient-reported outcome measures (PROMs) can be employed in both research and clinical care to enhance our understanding of outcomes that matter to patients. This narrative review aims to describe PROM use in recent pediatric surgical research, identify and describe psychometrically robust PROMs, providing an overview of those derived from pediatric patient input, and make recommendations for future research.
Materials and Methods A search was conducted to identify articles published from 2021 to August 2023 describing the availability and/or use of at least one valid or reliable PROM in children with conditions including anorectal malformations, biliary atresia, congenital diaphragmatic hernia, duodenal atresia, esophageal atresia, abdominal wall defects, Hirschsprung's disease, sacrococcygeal teratoma, and short bowel syndrome. Articles were categorized based on their objectives in applying PROMs. Psychometrically robust PROMs were identified and described.
Results Out of the 345 articles identified, 49 met the inclusion criteria. Seventeen focused on esophageal atresia and 14 on Hirschsprung's disease. Twenty-nine PROMs were identified, with 12 deemed psychometrically robust. Seven psychometrically robust PROMs were developed using patient input in the primary item generation. Most PROMs were applied to advance understanding of conditions and/or treatment and fewer were developed or psychometrically evaluated. No PROMs were assessed for their impact or incorporated into an implementation study.
Conclusions This review reveals gaps in the application of PROMs in recent pediatric surgical research. Emphasis should be placed on the development and utilization of psychometrically robust PROMs, broadening the scope of covered diseases, conducting impact assessments, and evaluating implementation strategies.
Background
Children with long-gap esophageal atresia (LGEA) risk living with aerodigestive morbidity and mental health difficulties. No previous study has investigated their experiences of schooling, despite the importance of schools in children’s development, learning and social relationships. We aimed to describe experiences of schooling in children with LGEA in Sweden in comparison with children with EA who had primary anastomosis.
Method
Children with LGEA aged 3–17 were recruited nationwide in Sweden. One parent completed a survey on their child’s school-based supports (according to definitions from the Swedish National Agency for Education), school absence, school satisfaction, school functioning (PedsQL 4.0), mental health (Strength and Difficulties Questionnaire) and current symptomatology. School data were compared between 26 children with LGEA to that from 95 children with EA who had PA, a hypothesized milder affected group. Mental health level was determined using validated norms; abnormal ≥ 90 percentile. Data were analyzed using descriptives, correlation and Mann–Whitney- U test. Significance level was p < 0.05.
Results
Formal school-based support was reported in 17 (65.4%) children with LGEA and concerned support with nutritional intake (60%), education (50%) and medical/special health needs (35%). The prevalence of school-based support was significantly higher compared to children with PA overall (36.8%, p = 0.013) and regarding nutritional intake support (20%, p < 0.001). In children with LGEA, school-based support was related to low birth weight ( p = 0.036), young child age ( p = 0.014), height ≤ −2SD for age/sex ( p = 0.024) and an increased number of aerodigestive symptoms ( p < 0.05). All children with LGEA who had abnormal mental health scores had school-based support, except for one child. Nine children with LGEA (36%) had school absence ≥ 1times/month the past year, more frequently because of colds/airway infections ( p = 0.045) and GI-specific problems compared to PA ( p = 0.003). School functioning scores were not significantly different from children with PA ( p = 0.34) but correlated negatively with school-based support (< 0.001) and school absence ( p = 0.002). One parent out of 26 reported their child’s school satisfaction as “not good”.
Conclusions
Children with LGEA commonly receive school-based support, reflecting multifaceted daily needs and disease severity. School absence is frequent and related to poorer school functioning. Future research focusing on academic achievement in children with EA is needed.
Background:
This study aimed to evaluate the contemporary surgical management of long-gap esophageal atresia, a rare and challenging problem managed by pediatric general surgeons.
Methods:
A retrospective review of the Pediatric Health Information System database for infants who underwent neonatal gastrostomy, followed by surgical reconstruction for long-gap esophageal atresia (2014-2021). Patients with birthweight less than 1.5 kg and those who received neonatal cardiac surgery were excluded. Outcomes were analyzed, including the need for further procedures, length of stay, and mortality.
Results:
Of 1,346 infants who underwent repair across 47 major children's hospitals, 100 (7%) met the inclusion criteria for long-gap esophageal atresia. Cardiac anomalies were identified in 43% of patients. The median age at repair was 87 days (interquartile range, 62-133). Ten percent of patients had a planned or unplanned reoperation ≤30 days after index surgery, and 4% underwent reoperation at >30 days. The median time to reoperation was 9 days (interquartile range, 7-60). Mortality during index admission was 5%, and the median hospital length of stay was 143 days (interquartile range, 101-192). Length of stay was significantly longer in patients with cardiac anomalies (cardiac: 179 days, non-cardiac: 125 days; P < .001), and 52% of patients required at least 1 postoperative dilation. The median time to the first dilation was 70 days (interquartile range, 42-173).
Conclusion:
This large multicenter study highlights the challenges of infants with long-gap esophageal atresia but suggests a high rate of successful delayed primary reconstruction. Hospitalizations are prolonged, and anastomotic stricture rates remain high. These data are useful for pediatric surgeons in counseling families on surgical repair strategy, timing, and postoperative outcomes.
