Prevalence, Mortality, and Associated Anomalies in Esophageal Atresia: A Retrospective Study of Finnish Population Data (2004–2017)

Suvi Alikärri; Ilkka Helenius; Susanna Heiskanen; Johanna Syvänen; Teemu Kemppainen; Eliisa Löyttyniemi; Mika Gissler; Arimatias Raitio

doi:10.1055/a-2423-0188

European Journal of Pediatric Surgery, Table of Contents

Eur J Pediatr Surg 2025; 35(03): 180-186
DOI: 10.1055/a-2423-0188

Original Article

Prevalence, Mortality, and Associated Anomalies in Esophageal Atresia: A Retrospective Study of Finnish Population Data (2004–2017)

Authors

Suvi Alikärri

¹Department of Paediatric Surgery, Orthopaedics and Traumatology, University of Turku and Turku University Hospital, Turku, Finland

²Department of Surgery, Kanta-Häme Central Hospital, Hämeenlinna, Finland
Ilkka Helenius

¹Department of Paediatric Surgery, Orthopaedics and Traumatology, University of Turku and Turku University Hospital, Turku, Finland

³Department of Orthopaedics and Traumatology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
Susanna Heiskanen

¹Department of Paediatric Surgery, Orthopaedics and Traumatology, University of Turku and Turku University Hospital, Turku, Finland
Johanna Syvänen

¹Department of Paediatric Surgery, Orthopaedics and Traumatology, University of Turku and Turku University Hospital, Turku, Finland
Teemu Kemppainen

⁴Department of Biostatistics, University of Turku and Turku University Hospital, Turku, Finland
Eliisa Löyttyniemi

⁴Department of Biostatistics, University of Turku and Turku University Hospital, Turku, Finland
Mika Gissler

⁵Knowledge Brokers, Finnish Institute of Health and Welfare, Helsinki, Finland

⁶Department of Molecular Medicine and Surgery, Karolinska Institute, Stockholm, Sweden and Academic Primary Health Care Centre, Region Stockholm, Stockholm, Sweden
Arimatias Raitio

¹Department of Paediatric Surgery, Orthopaedics and Traumatology, University of Turku and Turku University Hospital, Turku, Finland

Abstract

Introduction The aim of the study was to investigate the prevalence of esophageal atresia (EA), frequency of associated anomalies, and mortality from 2004 to 2017 in the Finnish population. We hypothesized the Spitz classification and the presence of other congenital malformations would predict mortality in patients with EA as well as assumed the survival to be high among patients with EA.

Materials and Methods This retrospective, population-based study was based on the registries maintained by THL Finnish Institute for Health and Welfare and Statistics Finland. The cases were identified and classified according to the World Health Organization's International Classification of Diseases and Health Related Problems (ICD) revisions 9 and 10 (ICD-9 and ICD-10) codes and accompanying written diagnoses. Associated anomalies were classified based on the EUROCAT criteria, and minor anomalies were excluded. All statistical tests were performed as a two-sided significance level set at p < 0.05. The chi-square or Fisher's exact test was utilized for categorical variables. The change in prevalence rates during the study period was evaluated with linear regression.

Results In total, 337 cases with EA were identified including 295 (87.5%) live births, 17 (5.0%) stillbirths, and 25 (7.4%) terminations of pregnancy. The total prevalence for EA in Finland was 4.17/10,000 births with no significant change during the study period, p = 0.35. Neonatal mortality was 5% (n = 15) and 1-year survival was 91.5%. Mortality was associated with syndromic cases (p = 0.002). The Spitz classification predicted neonatal mortality better than cardiac anomalies alone (p < 0.001 and p = 0.6, respectively). Type C was the most common atresia type (65.9%) followed by type A (14.8%) and B (6.8%). The most common group of associated malformations were heart defects (35.0%) followed by other gastrointestinal tract malformations (15.3%) and limb anomalies (12.2%). Syndromic cases (12.2%) were associated with type A and B atresias (p = 0.001). VACTERL association was observed in 16.6% of the cases.

Conclusion The overall prevalence of EA remains stable and relatively high in Finland. Despite the high prevalence of co-occurring malformations, the overall survival rate is high. Spitz classification predicted neonatal survival well.

Keywords

esophageal atresia - prevalence - mortality - associated anomalies - Spitz classification

Full Text

References

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