Urodynamic evaluation of children with the caudal regression syndrome (caudal dysplasia sequence)

A total of 50 children with the caudal regression syndrome (caudal dysplasia sequence) underwent urodynamic evaluation of lower urinary tract function. Of the patients 15 had isolated sacral agenesis, 13 had sacral agenesis in combination with an anorectal malformation and 22 had an anorectal malformation without sacral anomaly. Neurogenic bladder-sphincter dysfunction was observed in 11 of the 15 children with isolated sacral agenesis (73%) and in 10 of the 13 children with combined sacral agenesis and anorectal malformation (77%). The pattern of neurogenic bladder-sphincter dysfunction in both groups did not differ significantly. In the group of 22 children with isolated anorectal malformation only 1 had signs of neurogenic bladder-sphincter dysfunction. These results indicate that anorectal malformation per se does not influence lower urinary tract function and that the sacral anomaly is the determining factor for neurogenic bladder-sphincter dysfunction in children with anorectal malformation.