Aspects of esophageal atresia in a population-based setting: incidence, mortality, and cancer risk

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.