Introduction

The epispadias/exstrophy complex encompasses a spectrum of rare midline defects in the pelvis and genitalia. Classic bladder exstrophy is a severe congenital malformation affecting approximately 1 in 40000 live births. Although the exact etiology remains unknown, current evidence seems to implicate poor mesenchymal ingrowth with subsequent deleterious effects on the timing and position of rupture of the cloacal membrane.

The birth of a child with bladder exstrophy marks the start of a lifelong journey. The severity of the defect promises an extensive surgical undertaking that starts most frequently during the first days of life and often extends through adolescence. Components of surgical correction include: closure of the bladder, posterior urethra, pelvis and abdominal wall at birth, genital reconstruction, and some form of continence procedure. The approach pioneered at the senior author's institution addresses these components in a modern, staged fashion. Alternatively, there are also proponents of complete primary closure, which attempts to address all components in a single operation. However, recent data from the American Academy of Pediatrics reveals inferior continence rates when this technique is used. Longer follow-up is needed to ascertain the applicability of this repair, as the associated complications are not insignificant.

With respect to outcomes, the goals of reconstruction in the exstrophy patient are urinary continence and satisfactory genital cosmesis and function. Although most children prefer to achieve continence with voiding per urethra, some will accomplish this same outcome with intermittent catheterization.