Bladder Exstrophy: Causes, Symptoms, Risk Factors, Diagnosis, Treatment and Complications

Bladder exstrophy is an uncommon birth defect when the bladder grows outside of the baby. Because the open bladder cannot store pee or function properly, it spills urine (incontinence).

The problems caused by bladder exstrophy might range in severity. Intestinal and reproductive system issues, and bladder, genital, and pelvic bone anomalies are a few examples of these.

A basic ultrasound during pregnancy may show bladder exstrophy. Sometimes, though, the defect is not discovered until after the child is born. To correct birth defects brought on by bladder exstrophy in infants, surgery will be necessary.

Causes Of  Bladder Exstrophy

The cause of bladder exstrophy is unknown. Scientists think that a combination of hereditary and environmental factors likely has an impact.

The cloaca, a structure that houses the reproductive, urinary, and digestive apertures, is known to not develop normally as the foetus grows in infants with bladder exstrophy. Cloacal deformities can vary widely in severity depending on the age of the foetus at the time of the developmental mistake.

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Symptoms Of Bladder Exstrophy

Bladder exstrophy is the most common birth defect within the larger group of birth disorders known as the bladder exstrophy-epispadias complex (BEEC). Children impacted by BEEC have one of the following:

• Epispadias: This BEEC variant is the least severe since the urethra, the tube used to release urine, is not fully grown.
• Bladder exstrophy: This anomaly causes the bladder to originate outside of the body. The bladder is inside out as well. Bladder exstrophy traditionally affects the reproductive, digestive, and urinary tract organs. The anus, the aperture at the end of the large intestine, the bladder, the genitalia, the rectum, and the pelvic bones may all have anomalies.
• Children with congenital bladder exstrophy also suffer vesicoureteral reflux: With this condition, urine flows in the wrong direction from the bladder back up into the kidney connecting tubes, the ureters. Children with bladder exstrophy also experience epispadias.
• Exstrophy of the cloaca: Cloacal exstrophy is one of the most severe BEEC types. In this disorder, the genitalia, bladder, and rectum do not completely separate as the fetus develops. These organs may not have formed correctly, and the pelvic bones are also affected.
• The kidneys, backbone, and spinal cord could also be affected: Among children with cloacal exstrophy, spina bifida is one of the most prevalent spinal abnormalities. Children who have protruding abdominal organs are likely to experience bladder or cloacal exstrophy.

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Risk Factors Of  Bladder Exstrophy

Bladder exstrophy is more likely to occur in the following circumstances:

• History within the family: Firstborn children, children whose parents have the disorder, and siblings of a kid with the disorder are more likely to have bladder exstrophy.
• Ethnicity: White people have a higher incidence of bladder exstrophy than other racial groups.
• Sex: At birth, boys are more likely than girls to have bladder exstrophy.
• Incorporating assisted reproduction: It is more prevalent for children born with IVF or other assisted reproductive technologies to have bladder exstrophy.

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Diagnosis Of  Bladder Exstrophy

An unanticipated finding of bladder exstrophy occurs during a routine prenatal ultrasound. An earlier diagnosis can be made more definitively with MRI or ultrasonography. The following symptoms of bladder exstrophy may be seen during imaging tests:

• Difficult-to-fill or empty bladder
• Divided pubic bones, a part of the hipbones that comprise the pelvis
• Low-lying umbilical cord on the abdomen
• Smaller genitalia than normal

In some cases, the sickness is not noticeable until the baby is born. When doctors examine a newborn, they look for:

• The open portion of the bladder that is exposed to air and its size
• Where the testicles are situated, there is an intestinal hernia, or the stomach protruding through the abdominal wall
• surrounding anatomy of the navel
• The place of the rectum's terminal opening
• The ease with which the pelvis can move and the distance between the pubic bones.

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Treatment Of  Bladder Exstrophy

After delivery, a translucent plastic covering keeps the bladder safe.

Babies with bladder exstrophy are treated with reconstructive surgery. The major goals of reconstruction are as follows:

• Ensure that the space designated for holding urine is sufficient.
• Develop sex organs, or external genitalia, that are both functional and aesthetically pleasing.
• Ensure bladder control and continence.
• Sustain renal function

Although it's not obvious whether strategy is significantly better, there are two main ways to approach surgery. To enhance the surgeries and look at their long-term effects, more research is being done. The two surgical repair types are as follows:

• Complete repair: The procedure known as bladder exstrophy complete primary repair. The bladder and abdomen are closed, the urethra and external sex organs are healed, and the entire repair procedure is completed in a single session. This procedure can be done soon after delivery or when the child is two to three months old.

During pediatric surgery, the pelvic bones will usually need to be reconstructed. In cases where the baby is less than 72 hours old, the pelvic gap is small, and the baby's bones are malleable, the surgeons may elect not to proceed with the procedure.

• Partial repair: Officially, this procedure is referred to as "modern staged repair of bladder exstrophy." Staging a repair involves three steps. Three are finished six to twelve months after birth, four to five years after birth, and within 72 hours of birth. In the first procedure, the abdomen and bladder are closed; in the second, the urethra and sex organs are healed. When the child is old enough to participate in potty training, surgeons proceed to repair the bladder neck.

Standard postoperative care after surgery includes:

• Stillness: Following surgery, babies need to be in traction to recover. The minimum immobilization period for a child is four to six weeks, though this can change.
• Pain management: Painkillers can be delivered precisely where they are needed during surgery by having surgeons implant a tiny tube into the spinal canal. This makes it possible to control pain more effectively and prescribe fewer narcotic medications.

After surgery, most children, but not all of them, will be able to regain their bladder control. The procedure of catheterization involves the insertion of a tube to empty a child's bladder. Your child's growth can necessitate further operations.

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Complications Of  Bladder Exstrophy

Without treatment for urinary incontinence, children with bladder exstrophy will not be able to contain their urine. They also have an increased risk of sexual dysfunction and bladder cancer.

With surgery, complications can be reduced. The procedure's success depends on the severity of the deficit. Many children who have had surgical repairs can hold their urine. Young children with bladder exstrophy may walk with their legs partially bent outward due to the separation of their pelvic bones.

Even yet, people with bladder exstrophy at birth can procreate and participate in regular sexual activity. However, during the pregnancy, the woman and the unborn child will be in great danger, and an elective cesarean birth might be necessary.

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