What is Bladder Exstrophy?
Bladder Exstrophy is an abnormality of the formation of bladder and the bony pelvis.
The bladder does not form into its normal round shape but instead is flattened and exposed on the abdominal wall. The pelvic bones are also widely separated. The remaining part of the lower urinary tract can also be flattened and exposed with abnormal formation of the prostate and penis.
In a family who is having a child with exstrophy, the likelihood of a second child being born with exstrophy is 1 in 100. The risk of having a child with exstrophy is 1 in 70, if the parents have exstrophy.
Diagnosis of Bladder Exstrophy
Diagnosis of bladder exstrophy can be made on careful repeated ultrasound which is done before delivery, but usually the diagnosis is not made until the baby is born. The finding of the exposed bladder is typical.
Treatment of Bladder Exstrophy
Advancement in surgery in the past 15 years have allowed reconstruction of the penis and bladder so that a more "normal" and functional lifestyle can be maintained by the patient and family.
Current state of the art treatment for bladder exstrophy involves reconstruction of the various aspects of the deformity.
Initial Closure: This is generally done soon after the baby is born. At this first operation, the pelvic bones are reformed into their normal ring shape, the bladder, abdominal wall, and posterior urethra are closed and the belly button is reconstructed. This procedure takes between 4-6 hours.
After the surgery, baby is placed with the lower legs in traction to prevent separating the pelvic bones. Babies are observed carefully and may be in the Intensive Care Unit to be monitored for the first day or two. Generally the baby stays in the hospital for 3-4 weeks of healing.
Antibiotics are given after the operation to prevent further infection. The tube in the bladder is removed 4 weeks after the surgery. Gradually, the size of the bladder increases over time.
In very special instances with an excellent bladder plate and good sized penis, bladder exstrophy closure and epispadias repair can be combined. Though, this is only for very experienced exstrophy surgeons.
Epispadias repair: This repair occurs around 6-12 months of age. Time of surgery and the extent of the surgery depend on the size of the bladder and the problem of the penis. At this stage, the urethra on top of the flattened penis is closed and transferred to below the corporal bodies as in the normal penis.
Continence Procedure: The control of urine leakage is repaired and then further enlargement of the bladder is preformed if necessary. The timing of this procedure depends totally on the capacity of the bladder and the child's emotional status. The child must "want to be dry" and able to participate in a voiding program.