Dataset Information

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

ABSTRACT:

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.

SUBMITTER: Dellenmark-Blom M

PROVIDER: S-EPMC9207832 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature

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Publications

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

Dellenmark-Blom Michaela M Örnö Ax Sofie S Öst Elin E Svensson Jan F JF Kassa Ann-Marie AM Jönsson Linus L Abrahamsson Kate K Gatzinsky Vladimir V Stenström Pernilla P Tollne AnnaMaria A Omling Erik E Engstrand Lilja Helene H

Orphanet journal of rare diseases 20220620 1

<h4>Background</h4>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.<h4>Methods</h4>Postoperative morbidity, age-specific generic HRQOL (PedsQL<sup>™</sup> 4.0) and condit ...[more]

PMID: 35725462

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Project description:BackgroundChildren 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.MethodChildren 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.ResultsFormal 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".ConclusionsChildren 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.

Project description:The ideal approach to long gap esophageal atresia is still controversial. On one hand, preserving a patient's native esophagus may require several steps and can be fraught with complications. On the other hand, most replacement procedures are irreversible and disrupt gastrointestinal physiology. The purpose of this study was to evaluate the short- and medium-term outcome of electively delayed esophageal elongation procedures before esophageal reconstruction in patients with long-gap esophageal atresia. Since the neonatal esophagus grows over-proportionally and can increase its wall thickness in the first few months of life, we hypothesized that postponing the elongation steps until 3 months of age would lead to a lower complication rate. We thus retrospectively recorded complications such as mediastinitis, anastomotic leakage, stricture formation, or gastroesophageal reflux requiring surgery, and compared it to reported outcomes. In our treatment protocol, patients born with long-gap esophageal atresia underwent gastrostomy placement and were sham fed until 3 months of age. We then assessed the gap between the esophageal ends and started serial elongation procedures. We only proceeded to the reconstruction of the esophagus when its length allowed a tension-free anastomosis. From April 2013 to April 2019, we treated 13 Patients with long-gap esophageal atresia. Nine patients without prior surgical procedures underwent Foker procedures. Four patients arrived with a pre-existing cervical esophagostomy and thus underwent Kimura's procedure, two of them with a concomitant Foker elongation of the lower pouch. Esophageal reconstruction was feasible in all patients, while none of them developed mediastinitis at any point in their treatment. We managed the only anastomotic leak conservatively. Almost half of the patients did not require any further intervention following reconstruction, while three patients required multiple (≥5) anastomotic dilatations. All but one patient achieved full oral nutrition. Only one child required a fundoplication to manage gastroesophageal reflux symptoms. Electively delayed esophageal elongation procedures in patients with long-gap esophageal atresia allowed preservation of the native esophagus in all patients. The approach had low peri-procedural morbidity, and patients enjoy favorable functional outcomes. Therefore, we suggest considering this method in the management of patients with long-gap esophageal atresia.

Project description:ObjectiveTo investigate health-related quality of life (HRQOL) in patients after surgical repair for esophageal atresia (EA) and identify its potential influencing factors.MethodsA total of 102 EA children who had previously visited our hospital participated in this cross-sectional study. Basic data and disease data of the patients were collected. The HRQOL was measured with the Pediatric Quality of Life Inventory™4.0 (PedsQL™4.0) and EA-QOL questionnaire and ranked on a reverse 0-100 scale, with a higher number indicative of a better HRQOL perception. The scores of PedsQL™4.0 in children with EA were collected and compared with that of the demographically matched healthy control group. Meanwhile, the condition-specific HRQOL of EA was analyzed by the EA-QOL questionnaire, and the potential clinical factors that influenced the HRQOL were determined by the generalized linear model.ResultsThe group of EA and control reached a similar score in the generic PedsQL™4.0 (EA group: 86.55 ± 9.69; control group: 89.41 ± 6.54; p = 0.670). There was no significant difference between the EA group and the control group in other domains except the school functioning. Condition-specific HRQOL in the 2-7-year-old group had the highest score in social isolation and stress domain and the lowest score in the physical health and treatment domain, with an overall quality of life score of 83.48 ± 10.22. The scores of the 8-17-year-old group were relatively high in social relationships and health and well-being and lowest in the eating domain, with an overall quality of life score of 89.43 ± 8.57. Heart malformation, complicated esophageal surgery history, respiratory symptoms,and digestive symptoms in the past 1 month were the main factors affecting the HRQOL of children aged 2-7 years. Complicated esophageal surgery history, respiratory symptoms, and digestive symptoms in the past 1 month were the main factors affecting the HRQOL of children aged 8-17 years.ConclusionsThe findings suggest that patients with EA generally had a good HRQOL. However, EA children with postoperative complications and associated symptoms have lower scores in the EA-QOL questionnaire.

Project description:BackgroundEsophageal atresia (EA) is a rare congenital anomaly characterized by a discontinuity of the esophagus. Following surgical repair, survival rates have improved dramatically the past decenniums and today exceed 90%, but the children commonly present with esophageal and respiratory morbidity. In 2018, a condition-specific quality-of-life questionnaire for children with esophageal atresia (EA) aged 2-7 in Sweden-Germany was finalized (The EA-QOL questionnaire). The study aim was to describe the evaluation of the new translations across 12 new countries in Europe, Asia, Africa, Central-and North America.MethodsFollowing forward-backward translation into the new languages, the 17-item EA-QOL questionnaire was tested in cognitive debriefing interviews with parents of children with EA aged 2-7. Parents rated if each item was easy to understand (clarity) and sensitive to answer (interference with personal integrity). They could skip responding to a non-applicable/problematic item and give open comments. Predefined psychometric criteria were used; item clarity ≥80%/item sensitive to answer ≤20%/item feasibility ≤5% missing item responses. The decision to modify the translation was based on native expert, patient stakeholder, and instrument developer review, and the need for harmonization between translations.ResultsSimilar to findings in the Swedish-German cognitive debriefing, the cross-cultural analysis of input from 116 parents from 12 new countries (4-14 parents, median 9 parents/country) showed that all items in the EA-QOL questionnaire fulfilled the criteria for item clarity ≥80% and sensitive to answer (ranging from 1%-4.5%), although results varied between countries. Four items had missing responses between 5.2% and 13.4%, three within the same domain and were in line with parents' explanations. Poor translations and feasibility were improved.ConclusionsBased on parent input, the collaboration between native experts, patient stakeholders, and instrument developers, a linguistic version of the EA-QOL questionnaire for children aged 2-7 for use in and across 14 countries has been established. These efforts have set the conditions for a cross-cultural field test of the EA-QOL questionnaire and will open the doors for a new chapter in outcome research, registries, and clinical practice concerning children with EA. In the long-term, this will help increase knowledge of the disease's burden, promote patient-centeredness, exchange of information between nations, and strengthen evidence-based treatments for children born with EA.

Dataset Information

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

Background

Methods

Results

Conclusions

Publications

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

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