[Treatment of bladder exstrophy. Reconstruction or urinary diversion]

Abstract

Bladder exstrophy is seen in 1 of 30,000-40,000 live births, and is seldom treated in many urological departments. Treatment options for children with exstrophy are upper urinary tract diversion or reconstruction of the bladder and plastic surgery of the bladder neck to gain urinary continence by the age of 4-7 years. Historical reviews report continence rates of 10-30% after a staged approach with primary reconstruction and secondary bladder neck repair. This formerly meant upper urinary tract diversion as a third stage in 70-90%. Multiple operative procedures could be avoided when primary diversion was done. The best results were reported following antirefluxive implantation of ureters into the sigmoid colon (ureterosigmoidostomy). In boys, the base of the bladder was removed, leaving a small residual bladder which together with the reconstructed epispadias served as a "seminal tract". Total removal of the bladder was performed in girls. Long-term follow up of upper urinary tract diversion showed disturbances of serum electrolytes, urinary tract infections and stone formation, and after ureterosigmoidostomies an increased rate of colon carcinomas was documented. These results led to renewed interest in reconstruction. The technique of bladder neck reconstruction was changed, resulting in a higher rate of late urinary continence: augmentation cystoplasties, clean intermittent catheterization and the artificial sphincter help to achieve a continence rate of more than 90%. This goal was reached only after multiple operations and without knowledge of the long-term sequelae of augmentation cystoplasties. The years to come will show whether new concepts of ureterosigmoidotomies, such as the sigma-rectum pouch, will be preferable, or a late urinary tract diversion after failed reconstruction. Most centers are now agreed that primary reconstruction of bladder exstrophy should be attempted in the newborn child.