Prevalence of esophageal atresia among 18 international birth defects surveillance programs

Natasha Nassar, Emanuele Leoncini, Emmanuelle Amar, Jazmín Arteaga-Vázquez, Marian K. Bakker, Carol Bower, Mark A. Canfield, Eduardo E. Castilla, Guido Cocchi, Adolfo Correa, Melinda Csáky-Szunyogh, Marcia L. Feldkamp, Babak Khoshnood, Danielle Landau, Nathalie Lelong, Jorge S. López-Camelo, R. Brian Lowry, Robert Mcdonnell, Paul Merlob, J. MétnekiMargery Morgan, Osvaldo M. Mutchinick, Miland N. Palmer, Anke Rissmann, Csaba Siffel, Antonin Sìpek, Elena Szabova, David Tucker, Pierpaolo Mastroiacovo

Research output: Contribution to journalArticle

41 Citations (Scopus)

Abstract

BACKGROUND: The prevalence of esophageal atresia (EA) has been shown to vary across different geographical settings. Investigation of geographical differences may provide an insight into the underlying etiology of EA. METHODS: The study population comprised infants diagnosed with EA during 1998 to 2007 from 18 of the 46 birth defects surveillance programs, members of the International Clearinghouse for Birth Defects Surveillance and Research. Total prevalence per 10,000 births for EA was defined as the total number of cases in live births, stillbirths, and elective termination of pregnancy for fetal anomaly (ETOPFA) divided by the total number of all births in the population. RESULTS: Among the participating programs, a total of 2943 cases of EA were diagnosed with an average prevalence of 2.44 (95% confidence interval [CI], 2.35-2.53) per 10,000 births, ranging between 1.77 and 3.68 per 10,000 births. Of all infants diagnosed with EA, 2761 (93.8%) were live births, 82 (2.8%) stillbirths, 89 (3.0%) ETOPFA, and 11 (0.4%) had unknown outcomes. The majority of cases (2020, 68.6%), had a reported EA with fistula, 749 (25.5%) were without fistula, and 174 (5.9%) were registered with an unspecified code. CONCLUSIONS: On average, EA affected 1 in 4099 births (95% CI, 1 in 3954-4251 births) with prevalence varying across different geographical settings, but relatively consistent over time and comparable between surveillance programs. Findings suggest that differences in the prevalence observed among programs are likely to be attributable to variability in population ethnic compositions or issues in reporting or registration procedures of EA, rather than a real risk occurrence difference.

Original languageEnglish
Pages (from-to)893-899
Number of pages7
JournalBirth Defects Research Part A - Clinical and Molecular Teratology
Volume94
Issue number11
DOIs
Publication statusPublished - Nov 2012

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Esophageal Atresia
Parturition
Stillbirth
Live Birth
Fistula
Confidence Intervals
Population
Pregnancy

Keywords

  • Congenital anomalies
  • Epidemiology
  • Esophageal atresia
  • Prevalence

ASJC Scopus subject areas

  • Developmental Biology
  • Pediatrics, Perinatology, and Child Health
  • Embryology

Cite this

Nassar, N., Leoncini, E., Amar, E., Arteaga-Vázquez, J., Bakker, M. K., Bower, C., ... Mastroiacovo, P. (2012). Prevalence of esophageal atresia among 18 international birth defects surveillance programs. Birth Defects Research Part A - Clinical and Molecular Teratology, 94(11), 893-899. https://doi.org/10.1002/bdra.23067

Prevalence of esophageal atresia among 18 international birth defects surveillance programs. / Nassar, Natasha; Leoncini, Emanuele; Amar, Emmanuelle; Arteaga-Vázquez, Jazmín; Bakker, Marian K.; Bower, Carol; Canfield, Mark A.; Castilla, Eduardo E.; Cocchi, Guido; Correa, Adolfo; Csáky-Szunyogh, Melinda; Feldkamp, Marcia L.; Khoshnood, Babak; Landau, Danielle; Lelong, Nathalie; López-Camelo, Jorge S.; Lowry, R. Brian; Mcdonnell, Robert; Merlob, Paul; Métneki, J.; Morgan, Margery; Mutchinick, Osvaldo M.; Palmer, Miland N.; Rissmann, Anke; Siffel, Csaba; Sìpek, Antonin; Szabova, Elena; Tucker, David; Mastroiacovo, Pierpaolo.

In: Birth Defects Research Part A - Clinical and Molecular Teratology, Vol. 94, No. 11, 11.2012, p. 893-899.

Research output: Contribution to journalArticle

Nassar, N, Leoncini, E, Amar, E, Arteaga-Vázquez, J, Bakker, MK, Bower, C, Canfield, MA, Castilla, EE, Cocchi, G, Correa, A, Csáky-Szunyogh, M, Feldkamp, ML, Khoshnood, B, Landau, D, Lelong, N, López-Camelo, JS, Lowry, RB, Mcdonnell, R, Merlob, P, Métneki, J, Morgan, M, Mutchinick, OM, Palmer, MN, Rissmann, A, Siffel, C, Sìpek, A, Szabova, E, Tucker, D & Mastroiacovo, P 2012, 'Prevalence of esophageal atresia among 18 international birth defects surveillance programs', Birth Defects Research Part A - Clinical and Molecular Teratology, vol. 94, no. 11, pp. 893-899. https://doi.org/10.1002/bdra.23067
Nassar, Natasha ; Leoncini, Emanuele ; Amar, Emmanuelle ; Arteaga-Vázquez, Jazmín ; Bakker, Marian K. ; Bower, Carol ; Canfield, Mark A. ; Castilla, Eduardo E. ; Cocchi, Guido ; Correa, Adolfo ; Csáky-Szunyogh, Melinda ; Feldkamp, Marcia L. ; Khoshnood, Babak ; Landau, Danielle ; Lelong, Nathalie ; López-Camelo, Jorge S. ; Lowry, R. Brian ; Mcdonnell, Robert ; Merlob, Paul ; Métneki, J. ; Morgan, Margery ; Mutchinick, Osvaldo M. ; Palmer, Miland N. ; Rissmann, Anke ; Siffel, Csaba ; Sìpek, Antonin ; Szabova, Elena ; Tucker, David ; Mastroiacovo, Pierpaolo. / Prevalence of esophageal atresia among 18 international birth defects surveillance programs. In: Birth Defects Research Part A - Clinical and Molecular Teratology. 2012 ; Vol. 94, No. 11. pp. 893-899.
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abstract = "BACKGROUND: The prevalence of esophageal atresia (EA) has been shown to vary across different geographical settings. Investigation of geographical differences may provide an insight into the underlying etiology of EA. METHODS: The study population comprised infants diagnosed with EA during 1998 to 2007 from 18 of the 46 birth defects surveillance programs, members of the International Clearinghouse for Birth Defects Surveillance and Research. Total prevalence per 10,000 births for EA was defined as the total number of cases in live births, stillbirths, and elective termination of pregnancy for fetal anomaly (ETOPFA) divided by the total number of all births in the population. RESULTS: Among the participating programs, a total of 2943 cases of EA were diagnosed with an average prevalence of 2.44 (95{\%} confidence interval [CI], 2.35-2.53) per 10,000 births, ranging between 1.77 and 3.68 per 10,000 births. Of all infants diagnosed with EA, 2761 (93.8{\%}) were live births, 82 (2.8{\%}) stillbirths, 89 (3.0{\%}) ETOPFA, and 11 (0.4{\%}) had unknown outcomes. The majority of cases (2020, 68.6{\%}), had a reported EA with fistula, 749 (25.5{\%}) were without fistula, and 174 (5.9{\%}) were registered with an unspecified code. CONCLUSIONS: On average, EA affected 1 in 4099 births (95{\%} CI, 1 in 3954-4251 births) with prevalence varying across different geographical settings, but relatively consistent over time and comparable between surveillance programs. Findings suggest that differences in the prevalence observed among programs are likely to be attributable to variability in population ethnic compositions or issues in reporting or registration procedures of EA, rather than a real risk occurrence difference.",
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T1 - Prevalence of esophageal atresia among 18 international birth defects surveillance programs

AU - Nassar, Natasha

AU - Leoncini, Emanuele

AU - Amar, Emmanuelle

AU - Arteaga-Vázquez, Jazmín

AU - Bakker, Marian K.

AU - Bower, Carol

AU - Canfield, Mark A.

AU - Castilla, Eduardo E.

AU - Cocchi, Guido

AU - Correa, Adolfo

AU - Csáky-Szunyogh, Melinda

AU - Feldkamp, Marcia L.

AU - Khoshnood, Babak

AU - Landau, Danielle

AU - Lelong, Nathalie

AU - López-Camelo, Jorge S.

AU - Lowry, R. Brian

AU - Mcdonnell, Robert

AU - Merlob, Paul

AU - Métneki, J.

AU - Morgan, Margery

AU - Mutchinick, Osvaldo M.

AU - Palmer, Miland N.

AU - Rissmann, Anke

AU - Siffel, Csaba

AU - Sìpek, Antonin

AU - Szabova, Elena

AU - Tucker, David

AU - Mastroiacovo, Pierpaolo

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N2 - BACKGROUND: The prevalence of esophageal atresia (EA) has been shown to vary across different geographical settings. Investigation of geographical differences may provide an insight into the underlying etiology of EA. METHODS: The study population comprised infants diagnosed with EA during 1998 to 2007 from 18 of the 46 birth defects surveillance programs, members of the International Clearinghouse for Birth Defects Surveillance and Research. Total prevalence per 10,000 births for EA was defined as the total number of cases in live births, stillbirths, and elective termination of pregnancy for fetal anomaly (ETOPFA) divided by the total number of all births in the population. RESULTS: Among the participating programs, a total of 2943 cases of EA were diagnosed with an average prevalence of 2.44 (95% confidence interval [CI], 2.35-2.53) per 10,000 births, ranging between 1.77 and 3.68 per 10,000 births. Of all infants diagnosed with EA, 2761 (93.8%) were live births, 82 (2.8%) stillbirths, 89 (3.0%) ETOPFA, and 11 (0.4%) had unknown outcomes. The majority of cases (2020, 68.6%), had a reported EA with fistula, 749 (25.5%) were without fistula, and 174 (5.9%) were registered with an unspecified code. CONCLUSIONS: On average, EA affected 1 in 4099 births (95% CI, 1 in 3954-4251 births) with prevalence varying across different geographical settings, but relatively consistent over time and comparable between surveillance programs. Findings suggest that differences in the prevalence observed among programs are likely to be attributable to variability in population ethnic compositions or issues in reporting or registration procedures of EA, rather than a real risk occurrence difference.

AB - BACKGROUND: The prevalence of esophageal atresia (EA) has been shown to vary across different geographical settings. Investigation of geographical differences may provide an insight into the underlying etiology of EA. METHODS: The study population comprised infants diagnosed with EA during 1998 to 2007 from 18 of the 46 birth defects surveillance programs, members of the International Clearinghouse for Birth Defects Surveillance and Research. Total prevalence per 10,000 births for EA was defined as the total number of cases in live births, stillbirths, and elective termination of pregnancy for fetal anomaly (ETOPFA) divided by the total number of all births in the population. RESULTS: Among the participating programs, a total of 2943 cases of EA were diagnosed with an average prevalence of 2.44 (95% confidence interval [CI], 2.35-2.53) per 10,000 births, ranging between 1.77 and 3.68 per 10,000 births. Of all infants diagnosed with EA, 2761 (93.8%) were live births, 82 (2.8%) stillbirths, 89 (3.0%) ETOPFA, and 11 (0.4%) had unknown outcomes. The majority of cases (2020, 68.6%), had a reported EA with fistula, 749 (25.5%) were without fistula, and 174 (5.9%) were registered with an unspecified code. CONCLUSIONS: On average, EA affected 1 in 4099 births (95% CI, 1 in 3954-4251 births) with prevalence varying across different geographical settings, but relatively consistent over time and comparable between surveillance programs. Findings suggest that differences in the prevalence observed among programs are likely to be attributable to variability in population ethnic compositions or issues in reporting or registration procedures of EA, rather than a real risk occurrence difference.

KW - Congenital anomalies

KW - Epidemiology

KW - Esophageal atresia

KW - Prevalence

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