Bladder Exstrophy in Children

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Overview

Bladder exstrophy is a rare and complex congenital condition where a child is born with the bladder and surrounding structures improperly formed, resulting in the bladder being exposed outside the body. This condition occurs when the abdominal wall and the bladder fail to close during fetal development, causing the bladder to be exposed and inverted on the outside of the abdomen. Bladder exstrophy can also affect the urinary tract, reproductive organs, pelvic bones, and digestive system. Early diagnosis and surgical repair are essential to correct this condition and improve the child’s quality of life.

Symptoms

Bladder exstrophy is usually evident at birth due to the visible abnormality of the bladder and lower abdominal wall. The symptoms of bladder exstrophy include:

Visible bladder outside the abdomen: The bladder is exposed on the outside of the abdomen, which is the most noticeable sign.
Flattened, split genitalia: In boys, the penis may appear flattened and split, while in girls, the clitoris may be divided, and the urethra is often malformed.
Urethral abnormalities: The urethra (the tube that carries urine out of the body) may be shorter or improperly formed.
Urinary incontinence: Since the bladder cannot hold urine, children with bladder exstrophy continuously leak urine.
Widened pelvic bones: Children with bladder exstrophy often have a wider than normal distance between their pelvic bones, affecting walking and mobility as they grow.
Displaced belly button or umbilical hernia: The belly button may be positioned unusually, and some children may develop a hernia in this area.
Vesicoureteral reflux (VUR): This is when urine flows backward from the bladder into the kidneys instead of draining down. This condition can develop after the bladder is surgically closed.
Abnormal genital development:
- Boys: The penis may appear shorter and curve upwards. The testicles may not be in their usual place, and a hernia might be present.
- Girls: The clitoris and labia are split and spread apart, while the vagina and urethra may be shorter than usual. However, the internal reproductive organs, like the uterus and ovaries, are typically normal

Causes

Bladder exstrophy is a congenital condition, meaning it occurs during fetal development. The exact cause of bladder exstrophy is not fully understood, but it is believed to result from a combination of genetic and environmental factors that affect the development of the urinary system. It is not typically inherited and usually occurs sporadically. However, having a family history of bladder exstrophy may increase the likelihood of recurrence in future pregnancies.

Diagnosis

Bladder exstrophy is usually diagnosed shortly after birth due to the obvious physical appearance of the exposed bladder. In some cases, it may be detected during a prenatal ultrasound if the bladder and abdominal wall abnormalities are visible. The diagnostic process may include:

Prenatal ultrasound:
- A detailed ultrasound during pregnancy may show signs of bladder exstrophy, such as an absent bladder or abnormal positioning of the pelvic bones.
Physical examination:
- After birth, a physical exam will confirm the diagnosis based on the visible abnormalities of the bladder, genitalia, and abdomen.
Imaging tests:
- Additional imaging tests, such as an X-ray or MRI, may be used to assess the extent of the condition and plan for surgical repair.
- These tests help identify any associated abnormalities in the urinary tract, pelvic bones, or reproductive organs.

Treatment Options

Treatment for bladder exstrophy typically involves surgical correction to reconstruct the bladder, close the abdominal wall, and restore urinary function. Multiple surgeries may be required to fully repair the condition. At Gertrude’s Children’s Hospital, we offer expert surgical care and long-term follow-up for children with bladder exstrophy. Treatment options include:

Initial surgery (bladder closure):
The first step in treating bladder exstrophy is surgery to close the bladder and abdominal wall. This surgery is usually performed within the first few days of life. The goal is to place the bladder back inside the abdomen and reconstruct the abdominal muscles to provide protection and support for the bladder.
Pelvic bone realignment:
In some cases, the child’s pelvic bones may need to be realigned during surgery to provide a stable framework for the bladder and urinary tract. This procedure, known as pelvic osteotomy, helps to improve bladder function and prevent complications as the child grows.
Urinary continence surgery:
After the initial bladder closure, additional surgeries may be required to improve urinary continence. These surgeries focus on strengthening the bladder and urethral muscles, allowing the child to control urine flow more effectively. Procedures such as bladder neck reconstruction or the creation of a catheterizable stoma (an opening in the abdomen to empty the bladder) may be performed.
Reconstructive surgery for genitalia:
In children with bladder exstrophy, reconstructive surgery may also be necessary to repair and reconstruct the external genitalia. This is especially important for boys with epispadias or other genital abnormalities.
Management of kidney function:
Children with bladder exstrophy may require long-term monitoring of kidney function to ensure that there are no complications, such as kidney damage or infections. Regular follow-up visits with a pediatric urologist and periodic imaging tests are essential to monitor bladder and kidney health.
Physical therapy and rehabilitation:
Children with bladder exstrophy may benefit from physical therapy to help with mobility and walking, especially if pelvic bone realignment was performed. Rehabilitation can help improve strength and coordination as the child grows.

Patient Success Stories

At Gertrude’s Children’s Hospital, we are committed to providing the best possible care for children with Bladder Exstrophy. Here’s why families trust us:

“Our child was diagnosed with Bladder Exstrophy, and we didn't know where to turn. The team at Gertrude's Children's Hospital was amazing - they worked with us to develop a personalized treatment plan that helped our child manage their symptoms and thrive.”
Sarahparent of a child with Bladder Exstrophy

Bladder Exstrophy in Children

The exact cause of bladder exstrophy is not fully understood, but it is believed to result from a combination of genetic and environmental factors that affect the development of the urinary system during fetal growth.

Bladder exstrophy is treated through a series of surgeries to close the bladder, repair the abdominal wall, and reconstruct the urinary and genital structures. Multiple surgeries may be needed over time.

With early and appropriate treatment, most children with bladder exstrophy can lead healthy, active lives. Ongoing medical care, including follow-up visits and possible additional surgeries, is important for maintaining good bladder and kidney function.

Bladder exstrophy is often part of a broader condition known as the exstrophy-epispadias complex, which can include genital and pelvic abnormalities. Your child’s doctor will evaluate for any associated conditions and develop a comprehensive treatment plan.

Yes, children with bladder exstrophy require regular follow-up care to monitor bladder function, kidney health, and urinary continence. Long-term care ensures that any complications are detected and treated early.

Contact Us

If your child has been diagnosed with bladder exstrophy, Gertrude’s Children’s Hospital is here to provide expert care. Our pediatric urology specialists will develop a personalized treatment plan to ensure your child’s health and quality of life.

Contact us today to learn more about our Bladder Exstrophy in Children treatment options.