The bladder exstrophy–epispadias complex (BEEC)

Abstract Cloacal exstrophy, bladder exstrophy, and epispadias are a spectrum of rare embryological malformations known as bladder exstrophy–epispadias complex (BEEC). Their exact aetiology remains unknown, but there is evidence of a significant genetic component. They represent the most challenging cases that paediatric urologists manage. There are various surgical approaches, but many children require multiple reconstructive procedures and have long-term adjustment issues. Successful initial bladder closure is the most important factor for achieving urinary continence and sufficient bladder capacity. In most patients, continence can be achieved following failure of bladder neck reconstruction by augmentation cystoplasty and revision of the bladder neck reconstruction or bladder neck closure. The main goals of treatment are prevention of kidney deterioration, and achievement of social continence and satisfactory sexual function.