What is Bladder Exstrophy?
An abnormality of formation of the 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 remainder of the lower urinary
tract may also be flattened and exposed, with abnormal formation of
the prostate and penis. This
congenital birth defect is seen in one of 10,000 to 50,000 live births. In a family with a child with exstrophy, the likelihood
of a second child being born with exstrophy is one in 100. The risk
of having a child with exstrophy is one in 70, if the parents have
exstrophy. Major genetic studies are currently underway at Johns Hopkins
involving the exstrophy-epispadias complex.
Diagnosis
Diagnosis can be made on careful repeated ultrasounds
done before delivery, but usually the diagnosis is not made until
the baby is born. The finding of the exposed bladder is typical.
Treatment
Advances in surgery in the last 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 exstrophy involves reconstruction
of the various aspects of the deformity (i.e. closing the bladder,
repair of the penis, and prevention of urine leakage.
This usually involves separate operations at various times in the
life of the child to obtain the best results.
- Initial Closure: This is usually done soon after the baby
is born. At this first operation, the pelvic bones are reformed
into their normal ring shape (by a Pediatric Orthopedic surgeon),
the bladder, abdominal wall, and posterior urethra are closed and
the belly button is reconstructed. This procedure takes between
4-6 hours. Following surgery, the 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. The baby usually stays in
the hospital for 3-4 weeks of healing. Antibiotics are given following
the operation to prevent infection. The tube in the bladder is removed
four weeks following surgery. The size of the bladder gradually
increases over time. In very special circumstances with an excellent
bladder plate and good sized penis, bladder exstrophy closure and
epispadias repair can be combined.
However, 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 is dependent
on the size of the bladder and the deformity 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: At this time 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
and developmental status. The child must "want to be dry" and able
to participate in a voiding program.
Special Voiding Improvement Program
for the Exstrophy - Epispadias Patient
Children with bladder exstrophy face a combination of medical and
emotional challenges as they work with their urology team. Developing
a continence management program is an ongoing process that is often
stressful for children and their families. The voiding improvement
program provides hands-on one-on-one assistance to the child and family
before and after bladder neck repair.
The clinic is staffed by a specially trained, multidisciplinary team
including a pediatric urologist, pediatric nurse practitioner, pediatric
behavioral psychologist, and pediatric clinical nurse.
The Voiding Improvement Team also assists the pediatric urologist
in the evaluation of readiness for bladder neck reconstruction to
help the child and family prepare for the post-surgical work that
will allow for favorable continence outcome.
After bladder neck reconstruction surgery we work with children and
families using both behavior modification and muscle retraining procedures
to teach the child, family, and the child's bladder musculature to
function at their maximum potential for long-term continence.
After bladder neck surgery, frequent daily phone consultations occur
until the child is voiding well and tube free.
Visit usually last one hour and may include the following treatment
components:
- bladder ultrasound
- urine culture
- medication management
- biofeedback for bladder muscle retraining
- establishment of a voiding urine
- assessment of barrier to adherence
- nutrition education
- behavior therapy
- relapse prevention recommendations
Progress is evaluated at each visit and communicated to the pediatric
urologist.
Follow-up
Experience at the Johns Hopkins Hospital indicate
that 72-75% of patients are free of urine leakage following reconstruction
by the above-mentioned stages. The deformity of the penis was corrected
to the satisfaction of the patient and the family in most instances.
This, however, requires dedicated and intensive treatment and long-term
follow up into adolescence and adulthood by the exstrophy team.
Reference:- John P. Gearhart, Robert D. Jeffs: Exstrophy
of the Bladder, Epispadias and other Bladder Anomalies in Campbell's
Urology, Sixth Edition. Eds. Walsh PC, Retik AB, Stamey TA, Darracott
Vaughan E, Jr., WB Saunders Co. Vol. 2 1772-1821.
- John P. Gearhart: The bladder exstrophy-epispadias
complex. In pediatric Urology. Es Gearhart JP, Rink RR, and Mouriquand
P. Saunders, Philadelphia. Chapter 32, p 511-546.