Mortality and short‐term morbidity in infants with exomphalos

Purpose The increase in prenatal diagnosis together with the high rates of associated anomalies in omphalocele has led to increased rates of termination of pregnancies. The aim of this study was to examine the national Swedish birth prevalence and survival rates among these patients. Methods This study is based on a nationwide population-based cohort of all children born in Sweden between 1/1/1997 and 31/12/2016. All omphalocele cases were identified though the Swedish National Patient Register and the Swedish Medical Birth Register. Outcome of malformations and deaths were retrieved from the Swedish Birth Defects Register and the Swedish Causes of Death Register. Results The study included 207 cases of omphalocele (42% females). The birth prevalence for omphalocele was 1/10,000 live births. About 62% of the cases had associated malformations and/or genetic disorders; most common was ventricular septal defect. The mortality within the first year was 13%. The rate of termination of pregnancy was 59%. Conclusion The national birth prevalence for omphalocele in Sweden is 1/10,000 newborn, with high termination rates. Over half of the pregnancies with prenatally diagnosed omphalocele will be terminated. Among those who continue the pregnancy, 1-year survival rates are high. Type of study National register study Level of evidence III.

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“…An overall mortality of 14-30% has been reported [3,4,9,11,14]. In cases of isolated omphalocele, survival rate can be as high as 90% [9,15].…”

Section: Introductionmentioning

confidence: 99%

Omphalocele: national current birth prevalence and survival

et al. 2021

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“…Only a few population‐based studies have investigated early childhood mortality in omphalocele (Deng et al, 2014; Hijkoop et al, 2019; Marshall et al, 2015; Springett et al, 2014). Most studies conducted to date focused on clinical populations and inpatient mortality with a small numbers of cases (Akinkuotu et al, 2015; Conner et al, 2018; Corey et al, 2014; Fratelli et al, 2007; Raymond et al, 2018; Sakonidou, Ali, Farmer, Hickey, & Greenough, 2018). One USA study pooled data from several birth‐defect registries and reported infant mortality rates (Marshall et al, 2015), early and late neonatal mortality was studied in Europe (Groen et al, 2017), and a study from China reported perinatal mortality rates using data from a single registry (Deng et al, 2014).…”

Section: Introductionmentioning

confidence: 99%

A multi‐country study of prevalence and early childhood mortality among children with omphalocele

Nembhard

Bergman

Politis

et al. 2020

Birth Defects Research

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Background: Omphalocele is the second most common abdominal birth defect and often occurs with other structural and genetic defects. The objective of this study was to determine omphalocele prevalence, time trends, and mortality during early childhood, by geographical region, and the presence of associated anomalies. Methods: We conducted a retrospective study with 23 birth defect surveillance systems in 18 countries who are members of the International Clearinghouse for Birth Defects Surveillance and Research that submitted data on cases ascertained from 2000 through 2012, approximately 16 million pregnancies were surveyed that resulted in live births, stillbirths, or elective terminations of pregnancy for fetal anomalies (ETOPFA) and cases with omphalocele were included. Overall prevalence and mortality rates for specific ages were calculated (day of birth, neonatal, infant, and early childhood). We used Kaplan-Meier estimates with 95% confidence intervals (CI) to calculate cumulative mortality and joinpoint regression for time trend analyses. Results: The prevalence of omphalocele was 2.6 per 10,000 births (95% CI: 2.5, 2.7) and showed no temporal change from 2000-2012 (average annual percent change = −0.19%, p = .52). The overall mortality rate was 32.1% (95% CI: 30.2, 34.0). Most deaths occurred during the neonatal period and among children with multiple anomalies or syndromic omphalocele. Prevalence and mortality varied by registry type (e.g., hospital-vs. population-based) and inclusion or exclusion of ETOPFA. Conclusions: The prevalence of omphalocele showed no temporal change from 2000-2012. Approximately one-third of children with omphalocele did not survive early childhood with most deaths occurring in the neonatal period.

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“…A multivariable logistic regression model was then constructed to identify whether markers of RV function and pulmonary pressure were independently associated with death. This model also included previously reported factors associated with mortality and adverse cardiac function including gestational age at birth 26 and GO. 3,26 Indeed, gestational age and GO status may be associated with outcome of death and exposure status (PH or RV dysfunction), and as such were considered as important confounders.…”

Section: Discussionmentioning

confidence: 99%

“…This model also included previously reported factors associated with mortality and adverse cardiac function including gestational age at birth 26 and GO. 3,26 Indeed, gestational age and GO status may be associated with outcome of death and exposure status (PH or RV dysfunction), and as such were considered as important confounders. Statistical analyses were done with Stata SE (Version 14.2, College Station, TX) and R (Version 3.4.4)/ RStudio (Version 1.0.143).…”

Section: Discussionmentioning

confidence: 99%

Comprehensive Echocardiographic Assessment of Ventricular Function and Pulmonary Pressure in the Neonatal Omphalocele Population

et al. 2020

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Objective Pulmonary hypertension (PH) has been described in the neonatal omphalocele population. This study was aimed to describe cardiac function and PH severity using echocardiography in newborns with giant omphalocele (GO) and with non-GO and determine if right ventricular (RV) dysfunction is associated with mortality. Study Design Retrospective, single-center analysis of first echocardiography among neonatal omphalocele patients born between 2004 and 2017 was conducted. Multivariate logistic and univariate Cox's regression was constructed to measure hazard ratio (HR) for death outcome. Results There were 32 newborns, of whom 18 were GO and 7 died. GO had increased systolic pulmonary arterial to systolic systemic blood pressure ratio (97% [isosystemic] vs. 73% [three-fourths systemic] p = 0.03). RV performance parameters (tricuspid annular plane excursion, HR = 0.40; fractional area change, HR = 0.90; and RV peak global longitudinal strain, HR = 1.39) were associated with mortality. These RV performance parameters remained associated in a multiple logistic regression accounting for gestational age and GO status. The overall population had abnormal eccentricity index and pulmonary artery acceleration time to RV ejection time ratio, two markers of PH. Conclusion Patients with omphalocele have increased pulmonary pressure, with GO being worse than non-GO. RV dysfunction at initial echocardiography was significantly associated with mortality.

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Mortality and short‐term morbidity in infants with exomphalos

Abstract: Infants with exomphalos regardless of size can have chronic morbidity. Mortality is commonest in those with exomphalos major born at lower gestational age and birthweight.

Cited by 12 publications

References 13 publications

Omphalocele: national current birth prevalence and survival

Omphalocele: national current birth prevalence and survival

A multi‐country study of prevalence and early childhood mortality among children with omphalocele

Comprehensive Echocardiographic Assessment of Ventricular Function and Pulmonary Pressure in the Neonatal Omphalocele Population

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