Duplicate bladder exstrophy: a unique approach to initial repair

To describe a unique approach to the management of duplicate bladder exstrophy combining initial bladder closure and epispadias repair. Bladder exstrophy has been successfully managed by staged surgical repair with early bladder closure, subsequent epispadias repair, and, finally, bladder neck reconstruction. Duplicate bladder exstrophy is a rare variant of the exstrophy complex with fewer than 20 cases reported.

A male newborn presented with the appearance of both bladder exstrophy and a ruptured omphalocele. Repair of the omphalocele and bilateral orchiopexy was performed shortly after birth, but bladder closure was delayed until there was complete healing of the omphalocele defect, and the investigators believed the infant was ready for abdominal wall and bladder exstrophy closure. At age 8 months, bladder closure was performed with the intraoperative finding of a duplicate bladder lying posterior to the exstrophied bladder. The left ureter drained on the exstrophic bladder plate, and the right ureter drained into the posterior (internalized) duplicate bladder. After anterior innominate and vertical iliac osteotomies, the left ureter was reimplanted into the posterior bladder. A portion of the exstrophied bladder was then tubularized to construct a neourethra of the epispadic penis.

The child has done well with an excellent cosmetic appearance of the abdominal wall and a straight phallus. The bladder subsequently required a Mitrofanoff-type continent stoma along with bladder augmentation, which was performed at the time of his Young-Dees-Leadbetter bladder neck reconstruction (age 3.5). The child is currently continent but requires intermittent clean catheterization through his appendicovesicostomy.

The investigators report a unique and unexpected variant of bladder exstrophy and its successful management.