Bladder Exstrophy and Epispadias
What Is Bladder Exstrophy?
Bladder Exstrophy is a congenital birth defect that is the malformation of the bladder and urethra, in which the bladder is turned "inside out". The bladder does not form into its normal round shape but instead is flattened and exposed outside the body. The lower portion of the bladder, a funnel-shaped bladder neck, made up of muscles that open and close the bladder, fails to form correctly. The urethra and genitalia are not formed completely (epispadius) and the anus and vagina appear anteriorly displaced. Additionally, the pelvic bones are widely separated (diastasis).
It is unknown what causes Bladder Exstrophy. The problem occurs somewhere between 4-10 weeks of pregnancy when various organs, tissues and muscles begin to form layers that separate, divide and fold. Bladder Exstrophy is NOT inherited and did NOT occur because of anything the mother did or did not do during pregnancy!
Bladder Exstrophy is a Surgically Correctable Birth Defect. You Must be Aware that Each Child With Exstrophy is Uniquely Different and the Care and Treatment Prescribed by Your Urologist Will be Specific to Your Child’s Needs.
Bladder Exstrophy is noted in 1 of 30,000 to 50,000 live births!
Bladder Exstrophy is more likely to occur in males than females by approximately 2 to 1
The risk of having a 2nd child with bladder exstrophy is about 1 in 100 and 1 in 70 if one of the parents has bladder exstrophy.
How is Bladder Exstrophy treated?
Bladder Exstrophy requires surgical repair usually involving staged "reconstruction". The primary goals for reconstruction are: closure of the bladder and urethra; closure of the abdominal wall; preservation of kidney and sexual function; improved appearance of genitalia; and urinary continence. There are usually three stages of reconstruction (varies for each child and surgical strategy chosen by your surgeon):
1st Stage - Closure of bladder and abdomen (24-48 hours of life)
2nd Stage - Epispadius repair (2-3 yrs old)
3rd Stage - Achieve urinary continence (4-5 yrs old)
Other procedures your child may require Bladder Augmentation, Reimplantation of Ureters or Mitrofanoff Stoma.
Disorders Associated With Bladder Exstrophy
Epispadius - The urethra, which carries urine out of the body, has not formed completely. In boys, the penis is flattened and is pulled up towards the abdomen with the urethra open on the upper surface of the penis. In girls, the urethral opening is located between a divided clitoris and labia minora.
Vesicoureteral Reflux - Urine is made by the kidneys and travels down tubes called ureters to be stored in the bladder. Normally, urine flows one way. Reflux is a condition where urine can back up from the bladder into the kidneys. Reflux becomes serious when bacteria infected urine in the bladder travels to the kidneys, which can lead to kidney damage or worse, loss of kidney function. Many children outgrow this condition, but some require surgery to repair this problem. Some children are placed on a daily low-dose antibiotic for a period of time.
Diastasis - Separation of the front pubic bones which does not allow the bladder to remain inside the body.
Small Bladder Capacity - All exstrophy bladders are small at birth, some smaller than others. The extent to which the bladder will grow cannot be definitely determined. Successful bladder closure and epispadius repair provide conditions for the bladder to grow. Good urethral resistance can help "stretch" the bladder. Time will tell.
Missing Bladder Neck & Sphincter - The lower portion of the bladder, a funnel-shaped bladder neck and sphincter, consists of muscles that open and close the bladder outlet to control urine flow.
Will Bladder exstrophy affect my child's health and development?
In the majority of cases the children are healthy with normal intelligence, and normal physical and social development. Your child may have a waddling gait which will become less obvious as he/she gets older. Extra care will be needed for urinary control.
A parent of a bladder exstrophy child described it best: "Your child's future is as bright as any other child's".
The normal urinary system
The urinary system consists of two kidneys, two ureters, the bladder, the bladder sphincter (the muscle that acts as a binder to hold urine in the bladder), and the urethra. The kidneys clean waste and remove excess fluids from the blood, and help to manage blood pressure. The ureters are the tubes that drain the waste, the urine, into the bladder. The urine is stored in the bladder. When the bladder is full, it contracts and the sphincter opens allowing the drainage of urine into the urethra to the outside of the body.
What surgeries will my child need?
Please be aware that each case is different and your doctor will decide which surgery and treatment plan is best for your child. In most cases more than one surgery will be necessary and your doctor will discuss this with you. The following information is generalized and not specific to your child.
The first surgery is primiary closure of the exstrophied bladder and proper placement of the pubic bones. This is best done within the first seventy-two hours after birth. After the bladder closure the infant will be placed in a position called modified Bryant's traction. This is a position in which the hips have 90 degrees of flexion, the knees and ankles are held together, and the buttocks are slightly elevated off the bed. This traction may be needed for three to four weeks. In some cases more time may be necessary.
The bladder size may be very small. Surgery will be needed to increase the size of the bladder. This will allow the bladder to hold an ample volume of urine. Most patients will need to empty the bladder of urine with a tube/catheter. The catheter is inserted into the bladder, the urine is drained and then the catheter is removed. This procedure is called clean intermittent urinary catheterization. This will be done several times a day. This will be taught to the patients by the pediatric urology nurse or another qualified professional.
The Male patient:
The male may have a short, curved penis which may appear somewhat flat at the top. The urethral opening is epispadiac (on the upper surface of the penis). There is usually a space between the base of the penis and the scrotum. The patient may have bilateral inguinal hernias. The testes may be undescended (not in the scrotum) or retractile (capable of going back into the scrotum). If hernias are present they will be repaired. Reconstructive surgery will be done to repair the penis. This surgery will result in functional and cosmetically acceptable genitalia.
The Female patient:
The female patient usually presents with a normal uterus, fallopian tubes, and ovaries. The vagina may be slightly higher in placement and somewhat narrowed. The clitoris is separated into two parts, the labia and mons pubis (hair bearing skin) are spread apart. Reconstructive surgery will be preformed to bring the clitoris, mons pubis, and labia (if necessary) together. This surgery will provide functional and cosmetically acceptable genitalia.
The adult female patient will be able to have normal sexual intercourse. Almost all females are able to have children. When ready to start a family her doctor should be consulted since she will be prone to prolapse of the uterus. This is not dangerous when monitored carefully. Her doctor will determine if a cesarean section will be necessary.
Potential Issues Facing Exstrophy
What to Expect in the Hospital:
Tri-State Bladder Exstrophy Support Group
Westchester Medical Center
Contact: Dr. Edward Reda
19 Bradhurst Avenue, Suite 2575
Hawthorne, NY 10532
Phone (914) 493-8628 FAX (914) 493-8564 or email info@pedsurology.com