Long-term followup of complete primary repair of exstrophy: the Seattle experience

Since 1989, we have used the complete primary repair of exstrophy surgical technique to reconstruct the genitourinary system of children born with the exstrophy-epispadias complex based on the assumption that this complex represents a malformation. We initially reported using this technique in 1999. We now report a longer term followup of this initial group as well as surgical outcomes in a larger group of children who have undergone this repair for classic bladder exstrophy.

Since 1989, we have prospectively followed 39 children who underwent the complete primary repair of exstrophy technique to construct classic bladder exstrophy. Median followup in the original group of patients that we reported in 1999 is 106 months. Median followup in the entire series is 58 months.

Of boys and girls 4 years or older 74% have achieved daytime continence with volitional voiding. Of boys and girls 20% and 43%, respectively, have achieved primary urinary continence without the need for bladder neck reconstruction. An additional 18% of boys and girls achieved continence with only bladder neck injection following complete primary repair of exstrophy. Complications developed in 7 of the 39 children (18%) in the entire series.

Urinary continence has been consistently achieved with this form of exstrophy repair. Primary continence without the need for further reconstruction is possible. The results of this patient series have caused us to modify the complete primary repair of exstrophy technique in an effort to improve the rate of primary urinary continence.