Bladder Exstrophy

The bladder is a balloon-shaped organ located in the lower belly. the bladder stores the urine produced by the kidney and then it goes out through the urethra. Bladder exstrophy is a complex are rare birth disorders that occur when the baby is in the womb. Bladder exstrophy is diagnosed before birth during in routine pregnancy ultrasound. This can include exposed bladder, defects in the bladder, genitals, urinary tract, abdominal muscles, pelvic bones, and reproductive organs. The skin, abdominal wall and underlying organs, and pelvic bones at the lower part of the belly are not fused properly so the bladder is exposed outside of the skin. This bladder cannot store urine because it is separated from the urethra.

Sometimes the condition is not clear in ultrasound. The disorder may involve other organs at various levels. Doctor checks the new-born for:

• Size and potion of exposed bladder
• Testicles' position
• Bulging intestine
• position of rectum opening
• External genitals
• Separation of pubic bones

The signs and symptoms of bladder exstrophy depend on the conditions of disease the child is born with.

• The bladder is exposed to the abdominal wall and abnormally developed. It is small in size with an undeveloped neck. Urinating is done in an open area as the bladder cannot hold urine. It involves the urinary tract, digestive and reproductive systems. Children can have vesicoureteral reflux disorder too, where urine flows back up to the kidney.
• Bladder exstrophy most often has epispadias. The urethra that expels urine from the bladder to the outside of the body, is not fully developed. The urethra is exposed on the top surface of the penis in boys, and in girls, the urethral opening may be positioned between the clitoris.
• Pubis bones are separated and wide opened
• Vesicoureteral reflux (VUR) condition may be created and urine flows back up to the kidney.
• Abnormal development of genitals in boys and girls
• Cloacal exstrophy is the most complicated disorder where the rectum, bladder, and genitals are not correctly formed and joined together. This affects pelvic bones, kidneys, backbone, and spinal cord.

There is no clear cause of bladder exstrophy. Popular thinking is bladder defects could occur at the times of tissue development in the lower wall of the abdomen or cloacal membrane overdevelop in the fetus and prevent tissue development, growth of organs, and separation of the supportive lower abdominal wall structure. Some factors can also increase the risk of this disorder:

• A family history of bladder exstrophy may cause a child to born with this condition.
• More common in whites than in other races.
• Male genders are more affected by bladder exstrophy
• Children born through assisted reproduction such as IVF are at high risk

Bladder exstrophy is diagnosed during a fetal ultrasound or other imaging tests. Ultrasound shows if the baby's bladder is not filling and emptying. If bladder exstrophy is not clear in the unborn stage, it is visible after birth.

Bladder exstrophy is treated with reconstructive surgery and treatment started after the birth. The goals of reconstruction surgery are to:

• Preserve normal kidney function
• Repair the bladder and moving it inside the body
• Close the back of the urethra and separated the pelvis
• Bladder neck reconstruction
• Pelvic osteotomy
• Develop adequate bladder function and anterior abdominal wall and construct a urinary reservoir to be operated for some times
• Complete repair surgery closes the bladder, abdomen, pelvis bones and repairs the urethra and outer sex organs in a single procedure.
• Modern staged repair of bladder exstrophy is performed in 3 stages for the reconstruction of bladder exstrophy.

After surgical repair of bladder exstrophy, long time follow-up care is necessary to ensure the bladder and kidneys are functioning normally and developing in a healthy way. Surgery is performed to optimize the child's outcome and improve quality of life.