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.

Keywords: Delayed reconstruction; Esophageal atresia; Health-related quality of life; Long-gap esophageal atresia; Long-term morbidity; Postoperative outcomes.

Fig. 1

Presentation of the Swedish follow-up program at a tertiary pediatric surgical center for children born with esophageal atresia. The visits include a check-up and multidisciplinary monitoring of digestive and airway problems, growth, development of winged-scapula and scoliosis. At 1 and 15 years of age, patients are at a minimum offered physiological examination of the esophagus (e.g. 24-Hour pH-Impedance, gastroscopy with biopsy and at 15 years, test of the airways (e.g. spirometry) and exercise bicycle test

Fig. 2

Symptom prevalence and treatments at follow up in children aged 2–7 (Fig. 2a) and in children aged 8–18 (Fig. 2b) with delayed reconstruction of esophageal atresia compared to children with esophageal atresia Gross type C who underwent primary anastomosis. The statistical comparison was performed using Fisher’s exact test. Significant level was p < 0.05. Significant p-values are marked with bold text

Fig. 3

The PedsQL 4.0 scores in children aged 2–7 (a) and children aged 8–18 (b–c) with delayed reconstruction of esophageal atresia (including both delayed primary anastomosis and esophageal replacement) compared to children with primary anastomosis of the same age group and gender distribution

Fig. 4

The PedsQL 4.0 scores in children aged 8–18 with delayed primary anastomosis, esophageal replacement and primary anastomosis, self-report (a) and parent-report (b)

Fig. 5

The EA-QOL scores in children aged 2–7 (a) and children aged 8–18 (b–c) with delayed reconstruction of esophageal atresia (including both delayed primary anastomosis and esophageal replacement) compared to children with primary anastomosis of the same age group and gender distribution

Fig. 6

The EA-QOL scores in children aged 8–18 with delayed primary anastomosis, esophageal replacement and primary anastomosis, self-report (a) and parent-report (b)