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Bladder Exstrophy

Conditions

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Overview 

Bladder exstrophy (BE) is a rare, complex birth defect in which the baby’s bladder protrudes through an opening in the lower abdomen. Bladder exstrophy occurs in early in fetal development and is more often in males than females. 

The condition occurs during fetal development when the baby’s lower abdominal wall and bladder do not close properly. The developing bladder forms inside out (“exstrophy”). It is also exposed to the outside of the body and visible at birth through a defect (hole) in the baby’s abdomen. The bladder is unable to store urine or function normally. It also causes the bladder and the pelvis to form abnormally. 

Bladder exstrophy typically involves additional defects in the urinary tract, reproductive organs, pelvic bones, lower abdominal wall muscles and skin, and rectum. 

Babies with bladder exstrophy require surgery after birth to repair the defects. 

Bladder exstrophy is a rare condition, it affects about 1 in every 40,000 babies

Bladder Exstrophy Causes & Risk Factors 

The cause of bladder exstrophy is not known. Some studies suggest that it is inherited from the child's parents. The risk of having a child with bladder exstrophy is 1 in 70 if one or both parents have had the condition themselves. However, the chance for parents to have more than one child with bladder exstrophy is less than 1%.

How does Bladder Exstrophy Affect Babies? 

Common complications include: 

  • Urinary incontinence (urine leakage) 
  • Vesicoureteral reflux (backward flow of urine from the bladder up into the ureters, the tubes that connect to the kidneys) 
  • The need for repeated reconstructive surgery 
  • Infertility 

In addition to physical complications, bladder exstrophy can impact the emotional well-being of the child and adolescent. 

Bladder Exstrophy Associated Defects 

Babies with bladder exstrophy also have a condition known as epispadias, where the urethra (the tube that carries urine out of the body) fails to close properly, leaving the inner lining exposed and the opening in the wrong place. 

Additional defects that often occur with bladder exstrophy include: 

  • Widened, shortened pelvic bones and pubic bones that are separated 
  • Weak, abnormally shaped muscles in the lower abdominal wall (spread to the side) 
  • Legs and feet that turn outwards 
  • A lower-than-normal umbilical cord, just above the bladder 
  • An umbilical hernia (bulging of intestine through a weak spot in the abdominal muscles) 
  • Anterior displacement of the anus, meaning the opening from the rectum is located closer to the scrotum in males and closer to the vagina in girls 
  • Genital abnormalities 
    • In males, a shortened penis 
    • In females, a short urethra, wide-spread labia, narrow vaginal opening, and clitoris divided into two parts 

Bladder Exstrophy Diagnosis  

Bladder exstrophy may be detected during a routine prenatal ultrasound if the fetal bladder is not visible or is not functioning normally. Bladder exstrophy can be diagnosed immediately at birth since the bladder is visible outside the body. Prenatal diagnosis of bladder exstrophy should be considered any time the bladder is not visible in the ultrasound, or if the fetus shows any of the following signs:

  • a bulge in the lower abdomen 
  • a low-set umbilical cord 
  • an abnormal widening of the curved ridge at the top of the pelvic bone (known as the iliac crest) 
  • in males, a small penis with a displaced scrotum

A diagnosis during pregnancy enables your family and your healthcare team to plan ahead for the specialized treatment and surgical expertise your baby will need at birth. 

If bladder exstrophy is not detected during pregnancy, the condition is diagnosed at birth when the bladder is visible on the outside of the child’s body.

Specialized Evaluation and Prenatal Care 

If bladder exstrophy is suspected or diagnosed during pregnancy, you may be referred to a fetal center for further evaluation and specialized care, to ensure proper treatment planning. 

At Texas Children’s Fetal Center, we arrange for you to visit as quickly as possible for a comprehensive assessment by a team of specialists experienced in diagnosing and treating these rare fetal conditions, including maternal-fetal medicine physicians (OB/GYNs specializing in high-risk pregnancies), fetal imaging experts, pediatric urologists, pediatric surgeons, and neonatologists. 

Additional testing may include a high-resolution anatomy ultrasound, an ultra-fast MRI and a fetal echocardiogram of your baby’s heart, conducted by experts in the evaluation of fetal abnormalities. Using state-of-the-art imaging technologies and techniques, we obtain detailed views of your child’s condition, enabling the most accurate diagnosis and a treatment plan tailored to your baby’s unique needs, optimizing outcomes. 

Our specialists will meet with you about the results, discuss treatment recommendations and answer any questions your family has, to help you make the most informed decisions regarding your baby’s care and treatment. 

Bladder Exstrophy Diagnosis - Pregnancy and Delivery 

Mother and baby will be closely monitored throughout pregnancy to assess fetal growth and development and watch for signs of complications. 

In some cases, special delivery planning, such as induction of labor, may be recommended. Delivery at a center with the expertise and resources to treat babies with bladder exstrophy may also be advised, including the highest level neonatal intensive care unit (NICU), to avoid transferring your newborn.

Bladder Exstrophy Diagnosis - Treatment after Birth 

Babies with bladder exstrophy require surgery after birth to repair the defect.  

The type and timing of surgery varies based on the child’s defect and overall health. The timing may be immediate, with initial reconstructive surgery performed a few days after birth, or it may be best for the child to postpone or delay surgery for several weeks. 

Surgical strategies include a staged repair, where a series of three surgeries are performed over time, or a “complete primary repair” that combines the first two procedures in one surgery.  

The goals of surgery include: 

  • Closing the bladder and placing it back inside the body
  • Closing the urethra (epispadias repair)
  • Achieving normal bladder function and urinary control
  • Preservation of renal (kidney) function
  • Closing the abdominal wall muscles and skin
  • Repairing the function and appearance of genitalia, for normal sexual and reproductive function
  • Attaching the pelvic bones (osteotomy) 

Children with bladder exstrophy require lifelong care by urologists experienced in the treatment of this rare and complex birth defect. Additional surgeries are often required throughout the child’s life to optimize bladder function and control. Ongoing emotional support is also critical to helping children and adolescents cope with the challenges of this condition. 

Even with successful surgery, patients may have long-term problems with the following: 

  • ability to hold in urine
  • urinary reflux (back-up of urine to the kidneys)
  • repeated urinary tract infections (UTIs)
  • bladder or colonic adenocarcinoma (cancer that develops in the glandular tissues of the body)
  • uterine prolapse (the uterus slips down into or protrudes out of the vagina)
  • self-image 

Fortunately, sexual function and libido remain normal in bladder exstrophy patients. Successful pregnancies and delivery are possible. 

Postnatal Care Team 

A unique and distinct advantage for mothers delivering at Texas Children’s Pavilion for Women is our location inside one of the largest and highest ranked children’s hospitals in the world, for seamless access to the critical care services and specialists your child may need. 

Our Fetal Center team works closely with Texas Children’s Urology Division for specialized expertise in the treatment of these rare birth defects, carefully planning and coordinating your baby’s postnatal care. This means no transfers after delivery. It also means the pediatric specialists responsible for treating your child have been an integral part of their care team since before birth. 

Depending on your baby’s symptoms, his or her postnatal care team may include: 

Why Texas Children’s Fetal Center? 

  • A single location for expert maternal, fetal and pediatric care. At Texas Children’s Hospital, mother and baby receive the specialized care required for the diagnosis and treatment of bladder exstrophy all in one location, for highly coordinated care and treatment planning. 
  • A skilled, experienced team with proven outcomes. We have a dedicated team of maternal-fetal medicine specialists, fetal imaging experts, neonatologists, and pediatric urologists, surgeons, and anesthesiologists who work in concert to care for you and your baby every step of the way, using proven protocols we’ve developed over the years. With their combined expertise and unified approach, this team offers the best possible care for pregnancies involving bladder exstrophy. 
  • We care for your child’s needs at every stage of life. Our comprehensive approach starts with your first prenatal visit and continues through delivery, postnatal care, and throughout childhood and adolescence, thanks to one of the nation’s leading teams of fetal and pediatric specialists for the care and treatment of rare birth defects. 

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