Omphalocele: clinical and epidemiological profile of patients born in a tertiary care center in Rio de Janeiro
Abstract Background The objective of this paper is to describe the clinical and epidemiological profile and the early outcomes of patients with omphalocele born in a fetal medicine, pediatric surgery, and genetics reference hospital in Rio de Janeiro - Brazil. To determine its prevalence, describe t...
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| Format: | Article |
| Language: | English |
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BMC
2023-06-01
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| Series: | BMC Pregnancy and Childbirth |
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| Online Access: | https://doi.org/10.1186/s12884-023-05741-z |
| _version_ | 1797806450220728320 |
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| author | Matheus Sarabion Vilela Pereira Daniela Koeller Rodrigues Vieira Maria de Fátima M. P. Leite Maria Auxiliadora Monteiro Villar Carla Verona Barreto Farias |
| author_facet | Matheus Sarabion Vilela Pereira Daniela Koeller Rodrigues Vieira Maria de Fátima M. P. Leite Maria Auxiliadora Monteiro Villar Carla Verona Barreto Farias |
| author_sort | Matheus Sarabion Vilela Pereira |
| collection | DOAJ |
| description | Abstract Background The objective of this paper is to describe the clinical and epidemiological profile and the early outcomes of patients with omphalocele born in a fetal medicine, pediatric surgery, and genetics reference hospital in Rio de Janeiro - Brazil. To determine its prevalence, describe the presence of genetic syndromes, and congenital malformations, emphasizing the characteristics of congenital heart diseases and their most common types. Methods Using Latin-American Collaborative Study of Congenital Malformations (ECLAMC) database and records review, a retrospective cross-sectional study was performed, including all patients born with omphalocele between January 1st, 2016, and December 31st, 2019. Results During the period of the study, our unity registered 4,260 births, 4,064 were live births and 196 stillbirths. There were 737 diagnoses of any congenital malformation, among them 38 cases of omphalocele, 27 were live born, but one was excluded for missing data. 62.2% were male, 62.2% of the women were multiparous and 51.3% of the babies were preterm. There was an associated malformation in 89.1% of the cases. Heart disease was the most common (45.9%) of which tetralogy of Fallot was the most frequent (23.5%). Mortality rate was 61.5%. Conclusions Our data showed a good correspondence with the existing literature. Most patients with omphalocele had other malformations, especially congenital heart disease. No pregnancy was interrupted. The presence of concurrent defects showed a huge impact on prognosis, since, even if most survived birth, few remained alive and received hospital discharge. Based on these data, fetal medicine and neonatal teams must be able to adjust parents counseling about fetal and neonatal risks, especially when other congenital diseases are present. |
| first_indexed | 2024-03-13T06:07:30Z |
| format | Article |
| id | doaj.art-92a570f4215b45758d618fd092decd1e |
| institution | Directory Open Access Journal |
| issn | 1471-2393 |
| language | English |
| last_indexed | 2024-03-13T06:07:30Z |
| publishDate | 2023-06-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pregnancy and Childbirth |
| spelling | doaj.art-92a570f4215b45758d618fd092decd1e2023-06-11T11:28:06ZengBMCBMC Pregnancy and Childbirth1471-23932023-06-012311610.1186/s12884-023-05741-zOmphalocele: clinical and epidemiological profile of patients born in a tertiary care center in Rio de JaneiroMatheus Sarabion Vilela Pereira0Daniela Koeller Rodrigues Vieira1Maria de Fátima M. P. Leite2Maria Auxiliadora Monteiro Villar3Carla Verona Barreto Farias4National Institute of Women, Children and Adolescents Health Fernandes Figueira/Oswaldo Cruz Foundation (IFF/FIOCRUZ)National Institute of Women, Children and Adolescents Health Fernandes Figueira/Oswaldo Cruz Foundation (IFF/FIOCRUZ)National Institute of Women, Children and Adolescents Health Fernandes Figueira/Oswaldo Cruz Foundation (IFF/FIOCRUZ)National Institute of Women, Children and Adolescents Health Fernandes Figueira/Oswaldo Cruz Foundation (IFF/FIOCRUZ)National Institute of Women, Children and Adolescents Health Fernandes Figueira/Oswaldo Cruz Foundation (IFF/FIOCRUZ)Abstract Background The objective of this paper is to describe the clinical and epidemiological profile and the early outcomes of patients with omphalocele born in a fetal medicine, pediatric surgery, and genetics reference hospital in Rio de Janeiro - Brazil. To determine its prevalence, describe the presence of genetic syndromes, and congenital malformations, emphasizing the characteristics of congenital heart diseases and their most common types. Methods Using Latin-American Collaborative Study of Congenital Malformations (ECLAMC) database and records review, a retrospective cross-sectional study was performed, including all patients born with omphalocele between January 1st, 2016, and December 31st, 2019. Results During the period of the study, our unity registered 4,260 births, 4,064 were live births and 196 stillbirths. There were 737 diagnoses of any congenital malformation, among them 38 cases of omphalocele, 27 were live born, but one was excluded for missing data. 62.2% were male, 62.2% of the women were multiparous and 51.3% of the babies were preterm. There was an associated malformation in 89.1% of the cases. Heart disease was the most common (45.9%) of which tetralogy of Fallot was the most frequent (23.5%). Mortality rate was 61.5%. Conclusions Our data showed a good correspondence with the existing literature. Most patients with omphalocele had other malformations, especially congenital heart disease. No pregnancy was interrupted. The presence of concurrent defects showed a huge impact on prognosis, since, even if most survived birth, few remained alive and received hospital discharge. Based on these data, fetal medicine and neonatal teams must be able to adjust parents counseling about fetal and neonatal risks, especially when other congenital diseases are present.https://doi.org/10.1186/s12884-023-05741-z“Omphalocele”“Congenital heart defects”“Epidemiology” |
| spellingShingle | Matheus Sarabion Vilela Pereira Daniela Koeller Rodrigues Vieira Maria de Fátima M. P. Leite Maria Auxiliadora Monteiro Villar Carla Verona Barreto Farias Omphalocele: clinical and epidemiological profile of patients born in a tertiary care center in Rio de Janeiro BMC Pregnancy and Childbirth “Omphalocele” “Congenital heart defects” “Epidemiology” |
| title | Omphalocele: clinical and epidemiological profile of patients born in a tertiary care center in Rio de Janeiro |
| title_full | Omphalocele: clinical and epidemiological profile of patients born in a tertiary care center in Rio de Janeiro |
| title_fullStr | Omphalocele: clinical and epidemiological profile of patients born in a tertiary care center in Rio de Janeiro |
| title_full_unstemmed | Omphalocele: clinical and epidemiological profile of patients born in a tertiary care center in Rio de Janeiro |
| title_short | Omphalocele: clinical and epidemiological profile of patients born in a tertiary care center in Rio de Janeiro |
| title_sort | omphalocele clinical and epidemiological profile of patients born in a tertiary care center in rio de janeiro |
| topic | “Omphalocele” “Congenital heart defects” “Epidemiology” |
| url | https://doi.org/10.1186/s12884-023-05741-z |
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