Bladder Exstrophy

Bladder exstrophy is discovered as soon as the baby is born since the bladder is open with a bulging mucous membrane on the lower part of the abdomen, causing urine to constantly seep out of ureter openings on the bladder plate. The baby is most often healthy otherwise. 

Bladder exstrophy is a severe form of congenital abnormality resulting in a closure defect in the lower part of the abdomen that affects the pelvic ring, bladder, urethra, and external genitalia. There are both milder and more severe variants, from epispadias to cloacal exstrophy. Approximately one out of 30,000 children are born with classic bladder exstrophy. The malformation can be detected when the bladder fails to fill, and there is a low-set umbilicus.

The bladder, pelvic ring, and abdominal wall are surgically closed at 1-2 months of age. In girls, the urethra and external genitalia are reconstructed at the same time. In boys, surgery on the urethra and penis is usually postponed until one year of age. The lifetime  consequences include urinary incontinence, recidivating urinary tract infections, and sexual dysfunction. 
 
The surgery for bladder exstrophy, including genital surgery and later continence surgery in children, is centralized and takes place at Karolinska. We provide highly specialized care on behalf of the Swedish National Board of Health and Welfare. This centralization contributes to increased knowledge, quality, and patient safety. Our bladder exstrophy and epispadias team is also part of the European Reference Network (ERN) for eUROGEN, enabling us to exchange expertise with other specialists in Europe.  
 
Children with bladder exstrophy and epispadias and their families are managed by a multidisciplinary team with the goal of offering excellent care. The children are routinely checked during their childhood in order to detect and treat any problems that may arise. The most common problems are urinary tract infections, incontinence due to poor emptying of the bladder and sphincteric incompetence.  
 
In adulthood, continued follow-up and treatment are carried out by a specialized urological team in cooperation with the pediatric team (including urologists, urotherapists, gynecologists, plastic surgeons, counselors, and psychologists), holding special knowledge and experience regarding congenital malformations of the urinary tract.