Classical bladder eaxstrophy and epispadias comprise two of the malformations known as BEEC (bladder exstrophy-epispadias complex). The cause of these conditions is unknown, but although rare, there is a clear significant genetic component.
The goals of surgical management are prevention of kidney damage, continence and achievement of satisfactory functional genitalia.
There are various surgical approaches, which attempt to achieve volitional urethral voiding for the child. Three of these will be discussed in this chapter modern staged repair of bladder exstrophy (MSRE), radical soft tissue mobilisation (RSTM) and complete primary repair of bladder exstrophy (CPRE). Unfortunately, exstrophy reconstruction does not yet offer reliable continence for most children, and many require further procedures. Continence can be achieved in most patients following failure of reconstruction through augmentation cystoplasty, bladder neck tightening or closure and intermittent catheterisation through a Mitrofanoff channel.
Bladder exstrophy Epispadias Continence Incontinence Modern staged repair of bladder exstrophy (MRSE) Complete primary repair of bladder exstrophy (CPRE) Kelly repair Radical soft tissue mobilisation (RSTM) Bladder neck repair Bladder augmentation Mitrofanoff
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