Esophageal atresia and tracheo-esophageal fistula in Western Australia: prevelence and trends

Abstract

Objectives A recent international study reported a higher prevalence of esophageal atresia with or without tracheo-esophageal fistula (EA±TEF) in Western Australia (WA). The aim of this study was to examine the prevalence and trends of EA and/or TEF in WA; determine the proportion of cases with associated anomalies; and explore the impact of time of diagnosis. Methods The study population comprised all infants born in WA, 1980-2009 and registered with EA and/or TEF on the WA Register of Developmental Anomalies (WARDA). Results EA±TEF and TEF alone affect, on average, 1 in every 2,927 births in WA, with a total prevalence of 3.00 and 0.42 per 10,000 births, respectively. The prevalence of EA±TEF increased by 2.0% per annum, with only cases with associated anomalies (64% of cases) demonstrating an increase. TEF rates were stable. Among EA±TEF infants, the proportion of live births, stillbirths and elective terminations of pregnancy for fetal anomaly (TOPFA) was 79%, 6% and 15%, respectively; while the majority (94%) of TEF only cases were live births. In 2000-2009 there was 30% fall in EA±TEF live births with 61 (58%) cases diagnosed in first week of life, 10 (9%) prenatally and 34 (32%) at postmortem only. Conclusions A higher prevalence of EA±TEF in WA was observed with increase over time attributable to increase with associated anomalies. Consistent reporting, availability of prenatal diagnosis and ascertainment of cases following TOPFA or postmortem examinations can significantly affect prevalence of EA and/or TEF.