MONOZYGOTIC TWINS CONCORDANT FOR TRACHEO-ESOPHAGEAL FISTULA AND DISCORDANT FOR THE VATER ASSOCIATION

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Abstract
Monozygotic female twins were concordant for tracheo-esophageal fistula (TEF); and 1 twin also manifested other anomalies of the VATER [vertebral anomalies, anal atresia, tracheo-esophageal fistula, renal/radial atresia] association, including agenesis of the female reproductive tract. Review of the literature discloses a familial tendency for recurrence of TEF and a high concordance rate in monozygotic twins, indicating a significant genetic influence for the isolated anomaly. In the case of the VATER association, the sporadic occurrence of affected individuals and discordance in twins implicates the effect of non-genetic factors.