Orphanet Journal of Rare Diseases (Jun 2022)

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

  • Michaela Dellenmark-Blom,
  • Sofie Örnö Ax,
  • Elin Öst,
  • Jan F. Svensson,
  • Ann-Marie Kassa,
  • Linus Jönsson,
  • Kate Abrahamsson,
  • Vladimir Gatzinsky,
  • Pernilla Stenström,
  • AnnaMaria Tollne,
  • Erik Omling,
  • Helene Engstrand Lilja

DOI
https://doi.org/10.1186/s13023-022-02381-y
Journal volume & issue
Vol. 17, no. 1
pp. 1 – 16

Abstract

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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). 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.

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