Bladder exstrophy is rare, occurring about once in 30 to 50,000 live births. Despite a devastating initial appearance, because associated defects are few, many with bladder exstrophy lead near normal successful lives following reconstruction.
Goals for repair include providing urinary continence in both sexes and reconstructing the penis in males to provide for standing micturition and vaginal penetration.
Two approaches, the staged (Hopkins) repair and the complete repair (Mitchell) are currently in use.
If newborn closure is done in early infancy, iliac osteotomy is not always required.
A dedicated, experienced, consistent team is required to provide optimal results.
Children with bladder exstrophy are born with the bladder exposed (Fig. 67-1). The defect is obvious. The exposed umbilicus is at the midline. Just inferior to the umbilicus is the exposed bladder, which is surrounded by shiny, transitional para-exstrophy skin. The defect extends from the dome of the bladder to the tip of the urethra in classic bladder exstrophy. In epispadias, the bladder is closed, and only the urethra is exposed. The pubic bones are variably separated, further in bladder exstrophy than in epispadias. The umbilical to anal distance is shortened. If the child is older, the rectum may be prolapsed. In the male, the penis is short. The corporal bodies are separated, drawn apart a variable distance as they pass inferior to the pubic bones. The clitoris in the female is bifid. The vagina may be duplicated and the vaginal introitus may be stenotic.
Typical appearance of the bladder in classic bladder exstrophy. The bladder has a polypoid appearance with the detrusor prolapsed and the urothelium exposed. The urethral plate is shortened and open. The phallus is small in the male. The umbilical–anal distance is shortened.
Despite these dramatic defects, children with bladder exstrophy have surprisingly few other problems. Presuming a successful modern reconstruction is accomplished, most will live rewarding lives and contribute greatly to society.
The treatment of children with bladder exstrophy has evolved from supportive treatment in primitive times until the early to middle nineteenth century when initial surgical attempts were made to divert the urine to the rectum. The early attempts at ureterosigmoidostomy were fraught with complication, usually infection, and most patients died. A few scattered attempts at bladder closure were made in the middle of the 1800s. One attempt by Richard was made in 1853, and another was performed by Pancoast in 1858 at the Gross Clinic in Philadelphia. These early attempts at bladder closure, coming as they did in the era before antibiotics and suitable upper urinary diversion, failed. Most patients died of overwhelming wound infection or pyelonephritis caused by infection in a poorly emptying upper tract. Because of the severe morbidity of the primitive bladder closures ...