Malrotation in conjunction with esophageal atresia/tracheo-esophageal fistula

Esophageal atresia or tracheoesophageal fistula (EA/TEF) often occurs in as sociation with a well-defined group of other anomalies. We report the preva lence of malrotation and other intestinal anomalies in a large data series comprising 632 nontrisomic infants with EA/TEF ascertained by the Californi a Birth Defects Monitoring Program from January 1, 1983 to December 31, 199 4. Consistent with findings reported previously in smaller case series, our findings showed a notable prevalence of imperforate anus (9.0%) and duoden al atresia (5.2%), among other gastrointestinal defects. They also showed a previously unrecognized high prevalence of intestinal malrotation (4.4%). Compared with other infants studied, the infants with EA/TEF and malrotatio n of the intestine had a higher proportion of other associated anomalies (i n particular intestinal, central nervous system, vertebral and rib, renal a nd genital anomalies). These findings indicate that intestinal malrotation is more common in infants with EA/TEF than is generally perceived, and that intestinal malrotation in an infant with EA/TEF is associated with a highe r burden of additional congenital anomalies, suggesting that this group of infants may have more pervasive developmental deficits and poorer prognosis than has previously been recognized. Teratology 60:114-116, 1999. (C) 1999 Wiley-Liss, Inc.