Pediatric Bladder Exstrophy

Bladder exstrophy is a rare congenital condition in which the bladder and parts of the urinary tract develop outside the body. Instead of forming as a closed, internal organ, the bladder protrudes through an opening in the lower abdomen. The condition is also associated with abnormalities of the pelvic bones, external genitalia and often the urethra. Bladder exstrophy is part of a broader group of conditions called the bladder exstrophy-epispadias complex (BEEC), which includes:

• Epispadias – the urethra does not fully close, leaving it exposed
• Classic bladder exstrophy – the bladder is open on the surface of the abdomen
• Cloacal exstrophy – a more severe condition involving the intestines and reproductive organs

Bladder exstrophy occurs in approximately 1 in 30,000 to 50,000 live births and affects males more often than females.

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The condition is typically diagnosed at birth because of its visible presentation. Symptoms and signs of bladder exstrophy include:

• Visible bladder on the lower abdomen, open and exposed
• Constant leakage of urine (inability to store urine in the bladder)
• Widened pubic bones
• Epispadias (incomplete closure of the urethra)
• Genital malformations (e.g., a shortened penis in boys, narrow vaginal opening/separated clitoris in girls)
• Umbilical abnormalities or low-set belly button
• Short urethra or missing urethral opening

Associated issues can include vesicoureteral reflux (backward flow of urine from the bladder to the kidneys), kidney damage or infections.

The exact cause of bladder exstrophy is unknown, but it is believed to result from an error during fetal development when the lower abdominal wall and urogenital structures fail to close properly. This typically occurs early in pregnancy, between the fourth and sixth weeks of gestation.

Bladder exstrophy is usually a sporadic condition, meaning it most often appears unexpectedly in a child with no family history of the disorder. However, there may be a slightly increased risk in families with a history of the condition. Researchers are currently studying both environmental and genetic factors as possible contributors.

Bladder exstrophy is often diagnosed at birth based on the appearance of the exposed bladder and genital anatomy. In some cases, the condition may be suspected prenatally if an ultrasound shows:

• Absence of a visible bladder
• Lower abdominal wall defect
• Widened pubic bones
• Genital abnormalities

After birth, additional evaluation helps define the full extent of the condition and plan for treatment:

• Physical examination – To assess the anatomy of the bladder, urethra and genitals
• Ultrasound – Evaluates the kidneys, bladder and urinary tract
• X-rays – Assess pelvic bone separation and spine
• MRI or CT scan – May be used for surgical planning and evaluation of associated anomalies
• Echocardiogram – Screens for congenital heart defects (especially if BEEC is suspected)

Infants with bladder exstrophy may also have other congenital anomalies involving the spine or gastrointestinal system, which are evaluated as part of their initial workup.

Treatment for bladder exstrophy involves staged surgical reconstruction, ideally beginning in the newborn period. The goals of treatment are to reconstruct the bladder and abdominal wall, preserve kidney function and achieve urinary continence, and healthy genital development.

The Comprehensive Congenital Colorectal Program at Children’s Memorial Hermann Hospital collaborates with affiliated pediatric specialists from UTHealth Houston, including urologists, orthopedic surgeons, nephrologists and rehabilitation specialists, to deliver the complex, coordinated care these patients require.

Treatment often involves multiple surgeries tailored to the patient’s needs at various stages of life from infancy through adolescence.

Surgical Treatment Stages

1. Bladder and abdominal wall closure (usually within the first 2 to3 days of life)
   • Closes the bladder and brings together the pubic bones (often with pelvic osteotomies)
   • Reconstructs the abdominal wall and protects internal organs
2. Epispadias repair (typically between 6 to12 months of age)
   • Reconstructs the urethra and genitals to improve function and appearance
3. Continence surgery (later childhood or school age)
   • Depending on bladder function and capacity, some children may require procedures such as bladder neck reconstruction, bladder augmentation or the creation of a catheterizable channel (Mitrofanoff).
   • Some children may require catheterization to empty the bladder and prevent urinary tract infections or kidney damage.
     

With specialized care, many children with bladder exstrophy go on to lead full and active lives. Long-term outcomes depend on the severity of the condition and how well the bladder responds to reconstruction. Key factors influencing long-term outcomes include:

• Urinary continence – Achieved in many patients, but some require additional surgeries or catheterization.
• Kidney function – Regular monitoring is essential to prevent infection or damage.
• Sexual and reproductive function – Most children with bladder exstrophy can achieve normal puberty and, with medical guidance, can pursue parenthood later in life.
• Psychosocial support – Because of the condition’s complexity and visible differences, emotional and social support is an important part of care.

Children with bladder exstrophy benefit greatly from long-term follow-up with a multidisciplinary team to monitor growth, development and functional outcomes.

At Children’s Memorial Hermann Hospital, we provide compassionate, personalized care for children with bladder exstrophy and related urologic conditions. Through the Comprehensive Congenital Colorectal Program, families have access to the latest surgical techniques, advanced bowel/bladder management and coordinated, long-term support.

Through our affiliation with UTHealth Houston¹, we bring together a skilled multidisciplinary team with deep experience in treating colorectal conditions. Every child’s treatment plan is personalized to their anatomy, needs and long-term goals from infancy through adolescence and beyond.

If you have any questions, use the online tool below to help us connect with you. To refer a patient or schedule an appointment, please contact our clinic using the information below.

¹ Affiliated physicians evaluate patients at UT Physicians clinic locations and perform all inpatient procedures and treatments at Children’s Memorial Hermann Hospital.