Duplicate bladder exstrophy: A unique approach to initial repair

Objectives. To describe a unique approach to the management of duplicate bl adder exstrophy combining initial bladder closure and epispadias repair. Bl adder exstrophy has been successfully managed by staged surgical repair wit h early bladder closure, subsequent epispadias repair, and, finally, bladde r neck reconstruction. Duplicate bladder exstrophy is a rare variant of the exstrophy complex with fewer than 20 cases reported. Methods. A male newborn presented with the appearance of both bladder exstr ophy and a ruptured omphalocele. Repair of the omphalocele and bilateral or chiopexy was performed shortly after birth, but bladder closure was delayed until there was complete healing of the omphalocele defect, and the invest igators believed the infant was ready for abdominal wall and bladder exstro phy closure. At age 8 months, bladder closure was performed with the intrao perative finding of a duplicate bladder lying posterior to the exstrophied bladder. The left ureter drained on the exstrophic bladder plate, and the r ight ureter drained into the posterior (internalized) duplicate bladder. Af ter anterior innominate and vertical iliac osteotomies, the left ureter was reimplanted into the posterior bladder. A portion of the exstrophied bladd er was then tubularized to construct a neourethra of the epispadic penis. Results. The child has done well with an excellent cosmetic appearance of t he 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 reconst ruction (age 3.5). The child is currently continent but requires intermitte nt clean catheterization through his appendi-covesicostomy. Conclusions. The investigators report a unique and unexpected variant of bl adder exstrophy and its successful management.(C) 1999, Elsevier Science In c. All rights reserved.