Giant Omphalocele: Systematic Review of Pulmonary Complications and Implications for Neonatal Care
Joana Peixoto1, Joana Neto1,2, Susana Pissarra3
1Department of Obstetrics, Gynecology, and Pediatrics, Faculty of Medicine, Universidade do Porto, Porto, Portugal.
Insights
Newborn infants with giant omphalocele (GO) face high risks of pulmonary hypoplasia (PH), persistent pulmonary hypertension (PPHN), and mortality. Associated congenital anomalies (SCA) further increase these dangers, highlighting the need for better management.
Area of Science:
- Neonatalogy
- Pediatric Surgery
- Congenital Anomalies
Background:
- Giant omphalocele (GO) is a severe congenital anomaly with significant neonatal implications.
- Pulmonary hypoplasia (PH), persistent pulmonary hypertension of the newborn (PPHN), and associated congenital anomalies (SCA) are critical concerns in infants with GO.
- High mortality rates underscore the severity of this condition.
Purpose of the Study:
- To determine the prevalences of pulmonary hypoplasia (PH), persistent pulmonary hypertension of the newborn (PPHN), and mortality in newborns with giant omphalocele (GO).
- To assess the prevalence of syndromes and other congenital anomalies (SCA) associated with GO.
- To highlight the need for standardized diagnostic criteria and improved management strategies.
Main Methods:
- A systematic review was conducted following PRISMA guidelines.
- Databases searched included MEDLINE/PubMed, Google Scholar, and Scopus up to May 22, 2023.
- Included studies comprised 14 observational studies and 9 case reports.
Main Results:
- Prevalence of PH ranged from 18.5-54% (median 26%) and PPHN from 13-100% (median 38.5%) in observational studies.
- Mortality rates varied between 4-50% (median 23.5%) in observational studies and 55.5% in case series.
- Associated congenital anomalies (SCA) were reported in 58% of patients, significantly increasing mortality risk, especially with preterm birth.
Conclusions:
- The prevalences of PH, PPHN, mortality, and SCA in neonates with GO are alarmingly high.
- Heterogeneity in diagnostic criteria limits definitive prevalence estimates.
- Urgent need for standardized diagnostic criteria, enhanced prenatal counseling, and specialized center management is evident.
Abstract:
The primary objectives were to know the prevalences of pulmonary hypoplasia (PH), persistent pulmonary hypertension of the newborn (PPHN), and mortality in newborn infants with giant omphalocele (GO). A secondary aim was to assess the prevalence of syndromes and other congenital anomalies (SCA) associated with GO.A systematic review according to PRISMA guidelines using MEDLINE/PubMed, Google Scholar, and Scopus platforms was performed. The search strategy combined indexed and in-process citations up to May 22, 2023.Out of 651 articles, 23 met the inclusion criteria, comprising 14 observational studies and 9 case reports. There was no uniformity in the criteria for diagnosing PH and PPHN among the studies. In the observational studies, the prevalence of PH varied between 18.5 and 54% (median: 26%); the prevalence of PPHN varied between 13 and 100% (median: 38.5%); and mortality varied between 4 and 50% (median 23.5%). SCA were reported in 58% of patients. In the case series, PH was present in 27.8% of the neonates, PPHN in 50%, and mortality occurred in 55.5%. About 55.5% had associated SCA. The association of SCA and preterm birth contributed significantly to increase the mortality rate.The reported prevalences of PH, PPHN, mortality, and SCA associated with GO are alarmingly high. Although the heterogeneity and limitations of the included studies prevent definitive prevalence estimates, the findings underscore the urgent need for standardized diagnostic criteria, better prenatal counseling, and management in highly specialized centers.
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