Postoperative morbidity and health-related quality of life in children with delayed reconstruction of esophageal atresia: a nationwide Swedish study. Dellenmark-Blom, M., Örnö Ax, S., Öst, E., Svensson, J. F., Kassa, A., Jönsson, L., Abrahamsson, K., Gatzinsky, V., Stenström, P., Tollne, A., Omling, E., & Engstrand Lilja, H. Orphanet Journal of Rare Diseases, 17(1):239, June, 2022.
doi  abstract   bibtex   
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 \textless 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 \textgreater 0.05). More children with DREA underwent esophageal dilatations (both age groups), gastrostomy feeding (2-7 years), and antireflux treatment (8-18 years), p \textless 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 \textless 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.
@article{dellenmark-blom_postoperative_2022,
	title = {Postoperative morbidity and health-related quality of life in children with delayed reconstruction of esophageal atresia: a nationwide {Swedish} study},
	volume = {17},
	issn = {1750-1172},
	shorttitle = {Postoperative morbidity and health-related quality of life in children with delayed reconstruction of esophageal atresia},
	doi = {10.1186/s13023-022-02381-y},
	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 {\textless} 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 {\textgreater} 0.05). More children with DREA underwent esophageal dilatations (both age groups), gastrostomy feeding (2-7 years), and antireflux treatment (8-18 years), p {\textless} 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 {\textless} 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.},
	language = {eng},
	number = {1},
	journal = {Orphanet Journal of Rare Diseases},
	author = {Dellenmark-Blom, Michaela and Örnö Ax, Sofie and Öst, Elin and Svensson, Jan F. and Kassa, Ann-Marie and Jönsson, Linus and Abrahamsson, Kate and Gatzinsky, Vladimir and Stenström, Pernilla and Tollne, AnnaMaria and Omling, Erik and Engstrand Lilja, Helene},
	month = jun,
	year = {2022},
	keywords = {Child, Cough, Delayed reconstruction, Esophageal Atresia, Esophageal atresia, Health-related quality of life, Heartburn, Humans, Long-gap esophageal atresia, Long-term morbidity, Morbidity, Postoperative outcomes, Quality of Life, Sweden, Treatment Outcome},
	pages = {239},
}

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