Bladder exstrophy in Sweden--a long-term follow-up study

A retrospective review of all new cases of bladder exstrophy in Sweden in 1970 through 1989 is presented. The aim was to evaluate management of the malformation in Sweden and possibly identify factors that might improve the outlook. The incidence was 1:33,500 births. Records were reviewed of the 61 children (37 males and 24 females) treated at Sweden's four specialist centers of pediatric surgery. There was one perinatal death. Primary management was mainly early bladder closure (within 72 hours), late closure or perinatal urinary diversion. Subsequent measures included augmentation cystoplasty, bladder neck reconstruction, provision of continent urinary reservoir and penile reconstruction. The follow-up time was 4-19 years. In 15 cases renal scarring developed, though with impairment of total function in only three. Ten patients achieved continence, defined as 3-hour dry intervals, of whom four had urinary reservoirs with continent stoma. Urinary tract infections were recorded during follow-up in 43% of the surviving patients, though the true incidence of infection presumably was higher. To improve management of bladder exstrophy in sparsely populated Sweden, a nation-wide strategy of ongoing interhospital collaboration has been adopted. Results will be continuously presented.