Bladder exstrophy is an uncommon (3-4 children affected in every 100,000 births) congenital defect that occurs when the lower part of the abdominal wall does not form properly. The bony pelvis is open in the front, the urethra and the urinary bladder are split and exposed, the belly button is low, and the genitalia are also abnormal. The boys have a short and upwardly curved penis and girls have a narrow, short vagina and the clitoris is split into two parts. There are variations in the degree of severity of the condition. In a milder form, the bladder is intact, but the urethra is split (isolated epispadia).
The vast majority of children with bladder exstrophy are otherwise healthy and develop normally. The malformation, however, is associated with the risk of long-term problems including urinary incontinence and sexual dysfunction. The goal of treatment is to prevent urinary leakage and kidney damage and to allow normal sexual function, with the opportunity of becoming parents in adult life.
Children with bladder exstrophy need the care of a professional team which includes a pediatric urologist, orthopaedic surgeon, urotherapist and psychologist, at a pediatric urology or surgery centre. The condition is corrected in a series of surgical procedures as the child develops, with a reconstruction of the bladder, bony pelvis and genitalia, with or without continence enhancement surgery. Timing and surgical methods differ between centres in Europe. Continued treatment to prevent leakage and kidney damage comprises urotherapy, intermittent catheterization for emptying the bladder, and pharmacotherapy. It is important that the child and the family are offered continuous psychological support.