Exstrophy Bladder Repair

Bladder exstrophy is a complex and one of the rarest abnormalities that occur as the fetus develops in the womb. It’s whereby the urinary bladder forms outside the child’s body. This exposed bladder cannot function accordingly, including storing urine, which causes incontinence or urine leakage. Bladder exstrophy problems differ in severity and can include bladder defects, pelvic bones, and genital defects. It can also include the defects of the reproductive organs and intestines.

This condition can be diagnosed through regular ultrasound tests during pregnancy. However, the anomaly may sometimes be invisible until child delivery. If the child is born with bladder exstrophy, a surgical procedure is necessary to correct the problem. Exstrophy bladder repair surgery thus aims at repairing the defect and restoring the normal bladder function.

Causes of Bladder Exstrophy Disorder

The medical specialists are not certain of the actual causes of bladder exstrophy. However, it is believed to occur in the 11th week of pregnancy, when various organs are developing. According to some researchers, the bladder defect develops as the tissues of the lower wall of the abdomen expand. Simultaneously, the forming of pelvic bones and muscles are also affected.

The lower abdominal wall is usually protected by an impermanent tissue referred to as the cloacal membrane. It is replaced by the forming and maturing belly muscles. However, if the cloacal membrane ruptures before the abdomen muscles completely form, the bladder can be exstrophied. The urorectal septum is another tissue that serves to differentiate the enlarging bladder from the intestines or bowels.

Why is Exstrophy Repair Performed?

Babies born with bladder exstrophy should undergo repair surgery. This abnormality is more common among male children and is mostly associated with other congenital disabilities. Therefore, the exstrophy bladder repair is performed in order to help with the following;

Enable the infant to develop natural control of the urinary bladder
Prevent potential sexual function issues in the future
Enhance the physical look of the baby by making the genitals appear more normal
Avoid infections that might damage or affect the kidneys

At birth, the bladder can sometimes be too short. The operation will have to be postponed until the bladder has expanded in such a situation. The newborns are usually given antibiotics and sent home in the meantime. During this period, the bladder, which is located outside of the belly, has to remain moist at all times.

It might take several weeks to months before the bladder finally develops into the appropriate size. A pediatric staff will keep a close eye on the baby. This is until the surgical operation to repair the defect takes place.

How Exstrophy Bladder Repair is performed

There are two major approaches in which the physician can use to repair bladder exstrophy, both involving surgery. They include;

Complete primary repair of bladder exstrophy (CPRE)

CPRE is a treatment procedure to close the bladder and reconstruct the bladder neck, the transition area from the bladder to the urethra. It also involves repairing the epispadias (closing the whole urethra in a tube) all at the same time. When performing CPRE, the physician will create incisions in the pelvic bones or osteotomies. This is to give the standard anatomy of the pelvic bones and muscles. Children who have CPRE might need an additional operation in the future to treat vesicoureteral reflux and urinary incontinence.

The initial reconstruction or CPRE is normally done between the period of six and eight weeks. Postponing the CPRE procedure past the first two to three days of the child’s life may be beneficial in various ways. Such timing allows for the natural bonding of the infant and parents prior to the main reconstructive operation and long recovery time.

The period between the child’s birth and initial treatment provides an opportunity for development and physical growth. It also allows further maturation of certain organs and body systems before the complicated procedure. Generally, this contributes to more safety with both anesthesia and surgery.

Modern staged repair of bladder exstrophy (MSRE)

While most doctors often use the CPRE technique, the MSRE approach is also equally effective in addressing bladder exstrophy. MSRE consists of three surgical procedures for bladder exstrophy reconstruction. They include;

The first repair stage (closure of the bladder): This involves closing the bladder as well as the abdominal wall and reconstructing the belly button. Occasionally, it can include conducting an osteotomy, reformation of the pelvic bones to help during the repair. Normally, the initial procedure is done during the first two or three days of the child’s life.

The second stage (repair of epispadias): For male children, this involves closing the proximal portion of the urethra near the bladder together with the bladder. In females, the urethra is normally closed over its whole length. It will then stretch from the bladder to the skin's surface between the labia, as it should. This second phase of exstrophy bladder repair can occur between the ages of 6 and 12 months.

The third stage (reconstructing the bladder neck): This involves the reconstruction of the bladder neck as well as reimplantation of bilateral ureteral. The medical provider will perform this procedure once the bladder grows sufficiently to contain an adequate amount of urine. The operation is usually done on children aged 6 to 10 years old.

What Happens Before Exstrophy Bladder Repair?

Mostly,bladder exstrophy correction is performed when the baby is just some days old before being discharged from the hospital. With this situation, the medical team will start by preparing the child for the scheduled surgery.

If the operation was not performed when the baby was a newborn due to certain reasons, he or she might have to undergo some tests. These tests are essential in planning for the exstrophy bladder repair surgery at that particular time. They can thus include:

Urine examination, including urinalysis and urine culture. This helps check for the presence of bacteria in the baby’s urine and analyze the kidney function.
Examinations of the blood, including complete blood count, kidney tests, and electrolytes
Pelvic x-ray
Kidney ultrasound
Urine output record

Be sure to inform the medical provider of the medications that the child is currently using. In addition, tell them of any over-the-counter drugs or herbs that you purchased without a physician’s prescription.

It’s essential to stop giving your child medicines such as ibuprofen, aspirin, and warfarin. This also includes any other drugs from the tenth day before the operation. Such medications make it difficult for the blood to clot, resulting in severe blood loss. Inquire with the physician on which medications your child can continue taking on the day of the treatment procedure.

During the scheduled day of the operation, you will be asked not to feed the child with any food or drinks for some hours before treatment. However, if you have to give your child some drugs, ensure that he or she takes a small amount of water.

What to Expect After Exstrophy Bladder Repair

Following exstrophy bladder surgery, the doctor will attach a draining tube to the child’s bladder. This helps drain urine via the stomach wall and can also be referred to as a suprapubic catheter. The tube will remain in place for about three to four weeks.

If the child undergoes pelvic bone surgery, he or she will be required to remain in a sling or lower body case for four to six weeks. This promotes bone healing.

The child will also require comprehensive wound care, pain management, and the use of antibiotics. Before the health care provider permits your child to return home, they will teach you all the essential things.

Because of the high risk of infection, the baby will have to undergo urine culture and urinalysis during every appointment. These tests can be repeated if symptoms of an infection occur. Antibiotics are administered to some children on a daily basis to avoid chronic infection.

Possible Risks and Complications of Exstrophy Bladder Repair

As with any other surgical repair and reconstruction, exstrophy bladder repair also carries a few risks and complications. The possible risks associated with anesthesia and the operation can include;

Adverse reaction to anesthesia
Infection on the surgical site
Breathing difficulties
Bleeding
Blood clots formation

Additional risks associated with the procedure can include;

Sexual or erectile malfunction
Severe urinary tract infections
Kidney disorders
Incontinence (poor control of urine)
A need for additional surgeries in the future

Conclusion

Bladder exstrophy repair is a surgical procedure to correct a birth defect of the bladder. This is a disorder whereby the baby’s bladder develops before the abdominal wall forms fully. This leaves the pubic bone separated, hence exposing the bladder to the outer skin surface. This condition can be diagnosed during regular ultrasound scans or imaging tests like MRI or immediately after delivery.

While bladder exstrophy is a rare condition, prenatal care is essential for every pregnant mother and the developing fetus. Early diagnosis of bladder exstrophy provides enough time to study the condition and prepare for comprehensive expected care sufficiently.