An analysis of factors affecting survival in prenatally diagnosed omphalocele

Işıl Ayhan; Oya Demirci; Lütfiye Uygur; Şahap Odacilar; Olga Devrim Özbay Ayvaz; Ali Karaman; Ayşenur Celayir

doi:10.1515/jpm-2023-0197

Article

An analysis of factors affecting survival in prenatally diagnosed omphalocele

Işıl Ayhan , Oya Demirci , Lütfiye Uygur , Şahap Odacilar , Olga Devrim Özbay Ayvaz , Ali Karaman and Ayşenur Celayir

Published/Copyright: August 3, 2023

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From the journal Journal of Perinatal Medicine Volume 51 Issue 9

Abstract

Objectives

To estimate factors affecting survival in prenatally diagnosed omphalocele, factors predicting genetic abnormalities, and association of omphalocele and specific groups of anomalies.

Methods

A retrospective observational study was performed, analyzing data of all omphalocele cases diagnosed prenatally in the perinatology clinic of a referral center. Demographic data, characteristics of the omphalocele (size, content, associated anomalies), results of genetic testing, pregnancy outcomes and postnatal outcomes were analyzed.

Results

Sixty-nine fetuses with omphalocele were included. The prevalence of omphalocele in livebirth was 0.007 %. Overall survival during the study period was 73.9 %. Twenty-eight (71.7 %) out of 39 cases with associated anomalies who were born live, survived, whereas survival was 85.7 % in the isolated cases. The most common anomaly associated with omphalocele were cardiac defects with 42 %; followed by placental or umbilical cord anomalies (28.9 %), skeletal defects (27.5), genitourinary anomalies (20.2 %), central nervous system (18.8 %) and facial anomalies (7.2 %), respectively. Eighty-five percent of the fetuses had at least one additional anomaly or ultrasound finding. Skeletal abnormalities and staged surgical repair of omphalocele were associated with survival. Associated skeletal anomalies and staged repair significantly increase the risk of postnatal death (OR: 4.6 95 % CI (1.1–19.5) and (OR: 10.3 95 % CI (1.6–63.9), respectively).

Conclusions

Associated skeletal abnormalities and staged surgical repair are negatively associated with postnatal survival.

Keywords: anomaly; omphalocele; omphalocele surgery; skeletal abnormalities

Corresponding author: Işıl Ayhan, Department of Perinatology, University of Health Sciences, Zeynep Kamil Women and Children’s Diseases Training and Research Hospital, Istanbul, Türkiye, E-mail: isil.ayhan@hotmail.com

Acknowledgments

The authors would like to thank Ms. Elif Delen Deveci for being an invaluable part of the perinatology team; arranging patient relations and helping authors in data collection.

Research ethics: Research involving human subjects complied with all relevant national regulations, institutional policies and is in accordance with the tenets of the Helsinki Declaration (as revised in 2013), and has been approved by the authors’ Institutional Review Board (Zeynep Kamil Women and Children’s Diseases Training and Research Hospital) (Decision-Nr.: 132/2022).
Informed consent: Informed consent was obtained from all individuals included in this study.
Author contributions: The authors have accepted responsibility for the entire content of this manuscript and approved its submission.
Competing interests: The authors state no conflict of interest.
Research funding: None declared.

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Received: 2023-05-09

Accepted: 2023-07-18

Published Online: 2023-08-03

Published in Print: 2023-11-27

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Articles in the same Issue

https://doi.org/10.1515/jpm-2023-0197

Keywords for this article

anomaly; omphalocele; omphalocele surgery; skeletal abnormalities