Bladder exstrophy is a rare developmental abnormality that is present at birth in which the bladder and related structures are turned inside out.Bladder exstrophy-epispadias-cloacal exstrophy complex is a spectrum of anomalies involving the urinary tract, genital tract, musculoskeletal system and sometimes the intestinal tract.
The bladder exstrophy-epispadias-cloacal exstrophy complex is caused by a developmental abnormality that occurs 4-5 weeks after conception in which the cloacal membrane is not replaced by tissue that will form the abdominal muscles.
Bladder exstrophy is the most common in the larger group of birth defects called the bladder exstrophy-epispadias complex (BEEC). If your child has BEEC, he or she will have one of the following:
Epispadias. This is the least severe form of BEEC, in which the tube to expel urine (urethra) doesn't fully develop.
Bladder exstrophy. This defect causes the bladder to form on the outside of the body. The bladder is also turned inside out. Usually, bladder exstrophy will involve organs of the urinary tract, as well as the digestive and reproductive systems. Defects of the abdominal wall, bladder, genitals, pelvic bones, final section of the large intestine (rectum) and opening at the end of the rectum (anus) can occur
Children with bladder exstrophy also have vesicoureteral reflux. This condition causes urine to flow the wrong way — from the bladder back up into the tubes that connect to the kidneys (ureters). Children with bladder exstrophy also have epispadias.
Cloacal exstrophy. Cloacal exstrophy (kloe-A-kul EK-stroh-fee) is the most serious form of BEEC. In this condition, the rectum, bladder and genitals don't fully separate as the fetus develops. These organs may not be correctly formed, and the pelvic bones are affected as well.