Abstract
Purpose
To estimate the incidence, mortality and cancer risk of the congenital malformation esophageal atresia (EA) in a population-based investigation.
Methods
A population-based cohort study of EA patients registered in three nationwide registers in Sweden in 1964–2007. The incidence of EA per total number of live births was assessed. Mortality and cancer occurrence were expressed as standardized mortality ratio (SMR) and standardized incidence ratio (SIR) with 95% confidence intervals (CI). Mortality was further analyzed by Cox regression and expressed as hazard ratio with 95% CI.
Results
The EA cohort comprised 1,126 patients. The mean incidence was 3.16 per 10,000 live births, without any temporal changes (p for trend =0.94). Associated anomalies were present in 42% and chromosomal abnormalities in 5%. EA patients had an almost 12 times higher risk of mortality compared to the background population (SMR 11.8, 95% CI 10.3–13.5). The mortality increase was most pronounced during the first 5 years after birth. Survival improved during the study period (p for trend =0.0001). EA did not entail a strongly increased cancer risk (SIR 0.9; 95% CI 0.2–2.6).
Conclusions
EA has a stable incidence, the survival has improved substantially during recent decades, and the cancer risk might not be increased.
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Acknowledgments
This work was financially supported by The Swedish Research Council, Stockholm, Sweden.
Conflict on interest
The authors declare that they have no conflict of interest.
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Appendices
Appendix A- Associated malformations
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ICD7 (1964–1968) 750,00-759,99 + 325,40, 275,10, 276,10
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Malformations of the circulatory system 754,00-754,79
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Chromosomal abnormalities 325,40 Down’s syndrome, 275,10 Turner, 276,10 Klinefelter
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ICD8 (1969–1986) 740,99-759,99
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Malformations of the circulatory system 746,09 - 747,99
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Chromosomal abnormalities 759,30 - 759,99
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ICD9 (1987–1996) 740-759
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Malformations of the circulatory system 745-747
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Chromosomal abnormalities 758
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ICD10 (1997–2007) Q00-Q99
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Malformations of the circulatory system Q20-Q28
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Chromosomal abnormalities Q90-Q99
Appendix B- International Classification of Diseases (ICD) codes used to identify the diagnosis of cancer
ICD-7 (1964–1968)
-
1, Esophageal cancer
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150,91
-
2, Regional cancers
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151,91
-
161,91
-
162,01
-
231,07
-
162,11
-
162,17
-
231,08
-
162,81
-
231,10
-
3, All other malignant cancers (esophageal or regional tumors excluded)
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”140.01”,”140.11”,”140.81”,”140.91”,”141.01”,”141.71”,”141.81”,”141.91”,”142.01”,”142.71”,”142.81”,”142.91”,”143.91”,”144.91”,”145.01”,”145.71”,”145.81”,”145.91”,”146.91”,”147.91”,”148.91”,”150.91”,”151.91”,”152.01”,”152.71”,”152.81”,”152.91”,”152.92”,”153.01”,”153.11”,”153.21”,”153.31”,”153.71”,”153.81”,”153.91”,”154.01”,”155.01”,”155.11”,”155.21”,”155.81”,”156.91”,”157.91”,”158.91”,”159.91”,”160.01”,”160.11”,”160.21”,”160.71”,”10.81”,”160.91”,”161.91”,”162.01”,”162.11”,”162.17”,”162.21”,”162.81”,”163.91”,”164.91”,”165.91”,”170.91”,”171.01”,”171.11”,”172.11”,”173.01”,”174.01”,”175.01”,”175.11”,”175.81”,”175.91”,”176.01”,”176.11”,”176.71”,”176.81”,”176.91”,”177.91”,”178.91”,”179.01”,”179.11”,”179.71”,”179.81”,”179.91”,”180.01”,”181.01”,”181.21”,”181.71”,”181.81”,”190.01”,”190.11”,”190.21”,”190.31”,”190.41”,”190.51”,”190.61”,”190.71”,”190.81”,”190.91”,”191.01”,”191.11”,”191.21”,”191.31”,”191.41”,”191.51”,”191.61”,”191.71”,”191.81”,”191.91”,”192.01”,”192.11”,”192.21”,”192.31”,”192.41”,”192.51”,”199.11”,”193.01”,”193.21”,”193.31”,”193.41”,”193.81”,”193.91”,”194.01”,”195.01”,”195.11”,”195.21”,”195.31”,”195.41”,”195.51”,”195.61”,”196.01”,”196.11”,”196.21”,”196.31”,”196.41”,”196.51”,”196.61”,”196.71”,”196.81”,”196.91”,”197.01”,”197.11”,”197.21”,”197.31”,”197.81”,”197.91”,”198.01”,”198.11”,”198.21”,”198.31”,”198.41”,”198.71”,”198.81”,”198.91”,”199.01”,”200.00”,”200.10”,”200.28”,”201.99”,”202.00”,”202.10”,”202.11”,”202.12”,”202.19”,”203.99”,”204.00”,”204.05”,”204.10”,”204.15”,”204.20”,”204.30”,”204.40”,”204.48”,”205.99”
ICD-8 (1969–1986)
-
1, Esophageal cancers
-
150,XX
-
151,01
-
2, Regional cancers
-
151,11
-
151,81
-
151,87
-
151,99
-
161,01
-
161,88
-
161,89
-
161,99
-
162,01
-
162,11
-
162,12
-
162,13
-
162,14
-
162,17
-
162,18
-
162,19
-
3, All other malignant cancers (esophageal or regional tumors excluded)
-
140-199
ICD-9 (1987–1996)
-
1, Esophageal cancers
-
150
-
150D
-
150E
-
150F
-
50W
-
150X
-
151A
-
2, Regional cancers
-
151B
-
151C
-
151D
-
151E
-
151F
-
151G
-
151W
-
151X
-
161
-
161A
-
161B
-
161C
-
161D
-
161W
-
161X
-
162
-
162A
-
162C
-
162D
-
162E
-
162F
-
162W
-
162X
-
3, All other malignant cancers (esophageal or regional tumors excluded)
-
140-195
-
199-208
ICD-10 (1997-)
-
1, Esophageal cancers
-
C15.3
-
C15.4
-
C15.5
-
C15.8
-
C15.9
-
C16.0
-
C16.0A
-
C16.0B
-
C16.0C
-
C16.0X
-
2, Regional cancers
-
C16.1
-
C16.2
-
C16.3
-
C16.4
-
C16.5
-
C16.6
-
C16.8
-
C16.9
-
C32.0
-
C32.1
-
C32.2
-
C32.3
-
C32.8
-
C32.9
-
C33.9
-
C34.0
-
C34.1
-
C34.2
-
C34.3
-
C34.8
-
C34.9
-
C34.9A
-
C34.9B
-
C34.9C
-
C34.9D
-
C34.9E
-
C34.9F
-
C34.9G
-
C34.9H
-
C34.9J
-
C34.9K
-
C34.9X
-
3, All other malign cancers (esophageal or regional tumors excluded)
-
C00-C14
-
C15-C26
-
C30-C39
-
C40-C41
-
C43-C44
-
C45-C49
-
C50
-
C51-C58
-
C60-C63
-
C64-C68
-
C69-C72
-
C73-C75
-
C76-C80
-
C81-C96
-
C97
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Oddsberg, J., Lu, Y. & Lagergren, J. Aspects of esophageal atresia in a population-based setting: incidence, mortality, and cancer risk. Pediatr Surg Int 28, 249–257 (2012). https://doi.org/10.1007/s00383-011-3014-1
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DOI: https://doi.org/10.1007/s00383-011-3014-1