Bladder exstrophy is a rare and complex condition involving anomalies of the urinary tract, genital tract, the musculoskeletal system and sometimes the intestinal tract. The abdominal layers do not form properly, leaving the bladder open and exposed on the lower abdominal wall. The urethral opening in boys is often on top of the penis and not at the tip (epispadias). The urethra is usually open like a plate instead of closed like a tube. In girls, the urethral opening can occur between a divided clitoris, and the vagina is often shorter than normal. The pubic bones are spread wider and are shorter than usual to varying degrees and hips are turned outwards.
Bladder exstrophy occurs in about 2.2 cases per 100,000 live births and is more common in boys. There are no identifiable causes and currently no chromosomal findings identified, although clustering in some families has been observed.
During a prenatal ultrasound, findings of a low-lying umbilical cord, lack of a bladder and ambiguous genitalia are indicators of bladder exstrophy. Bladder exstrophy alone is not an indication for early delivery. If bladder exstrophy is not identified prenatally, it is apparent immediately following birth.
Once the baby is born, plastic wrap is placed on the bladder to protect it. Ultrasounds are done to evaluate the kidneys. A whole team of specialists will work with your family. The size of the bladder is one factor deciding when the surgery will occur. Surgical correction often occurs within the first few weeks of life. The goals of the surgery are to close the bladder and place it inside the body, behind the abdominal wall, in some cases correct the epispadias, reconstruct the genitalia, and pull the pelvic bones together. After surgery, babies are placed in a hip cast for about four weeks and stay in the hospital for most of that time.
Close and lifelong follow up is needed in these children to assess their ability to hold their urine (continence), evaluate for urine backflow to the kidneys (vesicoureteral reflux), monitor bladder size and pressure to protect the kidney, and to optimize the function and appearance of the external genitalia. Multiple surgeries are often needed to achieve these goals. Each child with exstrophy is different, and treatment decisions are made on an individual basis and the needs of that child.