Eur J Pediatr Surg 2021; 31(02): 172-176
DOI: 10.1055/s-0040-1703012
Original Article

Omphalocele in Finland from 1993 to 2014: Trends, Prevalence, Mortality, and Associated Malformations—A Population-Based Study

1   Department of Paediatric Surgery and Orthopaedics, Turku University Hospital, Turku, Finland
2   Department of Paediatric Surgery and Orthopaedics, University of Turku Faculty of Medicine, Turku, Finland
,
Asta Tauriainen
3   Department of Paediatric Surgery, KYS, Kuopio, Finland
,
Johanna Syvänen
1   Department of Paediatric Surgery and Orthopaedics, Turku University Hospital, Turku, Finland
2   Department of Paediatric Surgery and Orthopaedics, University of Turku Faculty of Medicine, Turku, Finland
,
Teemu Kemppainen
4   Department of Biostatistics, University of Turku, Turku, Finland
5   Department of Biostatistics, Turku University Hospital, Turku, Finland
,
Eliisa Löyttyniemi
4   Department of Biostatistics, University of Turku, Turku, Finland
5   Department of Biostatistics, Turku University Hospital, Turku, Finland
,
Ulla Sankilampi
6   Department of Paediatrics, Kuopio University Hospital, Kuopio, Finland
,
Kari Vanamo
7   Department of Paediatric Surgery, Kuopio University Hospital, Kuopio, Pohjois-Savo, Finland
,
Mika Gissler
8   Department of Information Services, National Institute for Health and Welfare, Helsinki, Finland
9   Department of Neurobiology, Karolinska Institute, Stockholm, Stockholm County, Sweden
,
Anna Hyvärinen
10   Department of Paediatric Surgery, Tampere University Hospital, Tampere, Finland
11   Department of Medicine, Tampere University Faculty of Medicine, Tampere, Finland
,
Ilkka Helenius
1   Department of Paediatric Surgery and Orthopaedics, Turku University Hospital, Turku, Finland
2   Department of Paediatric Surgery and Orthopaedics, University of Turku Faculty of Medicine, Turku, Finland
› Author Affiliations
Funding This study was funded by Clinical Research Institute HUCH.

Abstract

Inroduction The aim of this study is to assess the changes in prevalence, mortality and termination pregnancy of omphalocele, and to identify associated anomalies.

Materials and Methods A population-based nationwide register study. All cases with omphalocele were identified in the Finnish Register of Congenital Malformations and the Care Register for Health Care from 1993 to 2014 including live births, stillbirths, and terminations of pregnancy due to fetal anomalies. Associated anomalies were recorded and analyzed, and perinatal and infant mortality and prevalence were calculated.

Results There were 600 cases with omphalocele including 229 live births, 39 stillbirths, and 332 (55%) abortions. Birth prevalence in Finland was 1.96 per 10,000 births with no consistent trend over time. However, total prevalence was much higher (4.71/10,000) because more than half of these families chose option for the termination of pregnancy. Omphalocele is often complicated with other anomalies; most commonly chromosomal abnormalities (9.3%), heart defects (6.3%), central nervous system anomalies (3.0%), gastrointestinal, and urogenital malformations (both 2.0%). Proportion of chromosomal and central nervous system abnormalities were even higher in terminated pregnancies. Overall infant mortality was 22%. Total 1-year survival rates for isolated omphalocele, cases with multiple anomalies and neonates with chromosomal defects were 80, 88, and 17%, respectively.

Conclusion Omphalocele is a rare congenital anomaly, often associated with other malformations. Our data suggest that isolated cases may be more common than previously thought. In the absence of chromosomal defects, survival is reasonably good. Regardless, more than half of these pregnancies are often terminated.



Publication History

Received: 17 October 2019

Accepted: 27 January 2020

Publication Date:
04 March 2020 (online)

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