Aspects of esophageal atresia in a population-based setting: incidence, mortality, and cancer risk.
OBJECTIVE
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.
Upper Gastrointestinal Research, Department of Molecular Medicine and Surgery, Karolinska Institutet, 171 76, Stockholm, Sweden. jenny.oddsberg@karolinska.se
Jenny
J
Oddsberg
Yunxia
Y
Lu
Jesper
J
Lagergren
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D016001:Confidence Intervals; D004933:Esophageal Atresia / Q000150:complications / Q000453:epidemiology*; D004938:Esophageal Neoplasms / Q000453:epidemiology* / Q000209:etiology; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011159:Population Surveillance; D011379:Prognosis; D016016:Proportional Hazards Models; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D013548:Sweden / Q000453:epidemiology; D055815:Young Adult