Urethral abnormalities in male neonates with VATER association

In the past 3 years, eight of 20 male neonates with stigmata of the VATER association were found to have significant urethral abnormalities. Three had megalourethra, two had duplication of the urethra, and one each had an anterior urethral valve, congenital stricture, and hypospadias. In only five of eight was the extent of the abnormality clinically apparent. All three infants with megalourethra and imperforate anus died because of the coexistence of other severe congenital anomalies. Prior literature has described but not stressed the increased prevalence of urethral abnormalities in children with the VATER association. Because of the high prevalence (eight of 20) of significant urethral anomalies, we perform voiding cystourethrography on all males with the stigmata of the VATER association, even in the absence of clinical symptoms.