What is Bladder Exstrophy?
Occurring in 1 in 30,000 births, bladder exstrophy is a major abnormality of the abdominal wall, pelvis and bladder. The pelvic ring fails to form properly and the abdominal wall muscles are divided below the level of the umbilicus. Within this defect, lies the bladder, which is a flat plate on the abdominal wall. The urine drains directly onto this. In addition, there are genital anomalies. In boys, the urethra is open on the back of the penis – epispadias. In females, the urethra is also open, and the clitoris is divided to each side of it – female epispadias.
Surgery for bladder exstrophy consists of a closure of the bladder, performed in the first few days of life. This brings the pelvic ring together in front of the bladder, and reconstructs the anterior abdominal wall. Following this successful closure, there will remain issues with incontinence, and secondary surgery is required towards the end of the first year, or in the second year, of life. Current procedures performed are at Great Ormond Street and originated with Prof Cuckow; they are very successful in creating continence in about 70% of patients who have this major abnormality.