Generally associated with bladder exstrophy (see this term) that combines a abnormal closure of the pubic symphysis, a significant defect of the abdominal wall and severe abnormalities of the terminal portion of the urinary and genital tract. There is a male prevalence.

Clinical presentation:

-        boy: the urethra opens at the upper face of the penis with an external urethral orifice located at the proximal part of it; urinary incontinence

-        girl: there is a wide open urethral canal between the 2 hemiclitoris; narrow, horizontalized vagina; urinary incontinence.

The epispadias associated with bladder exstrophy is repaired either during a third stage of the exstrophy reconstruction (in childhood) or in the neonatal period if reconstruction is done in one stage.

The two types of approaches currently used are:

1) 3-step reconstruction:

-        1st stage: in the neonatal period, closure of the abdominal wall, the bladder and the pubic symphysis (iliac osteotomies);

-        2nd stage: reconstruction of the urethra and anti-reflux surgery by the endovesical route (Cohen's intervention most often);

-        3rd stage: repair of the epispadias; sometimes associated with bladder augmentation surgery.

2) Reconstruction in a single procedure: it is a very long neonatal and hemorrhagic operation (6 hours) aiming to quickly create a functional bladder.

It includes 5 steps:

-        closure of the bladder and the bladder neck,

-        repair of the epispadias,

-        reconstruction of the pelvis and the pubic symphysis with bilateral iliac osteotomies (hemorrhagic stage),

-        bilateral inguinal herniorraphia,

-        closure of the abdominal wall with a muscle and a skin layer

In girls, the stage of epispadias repair consists in a clitoroplasty and there is an additional surgical step: a vaginoplasty.

Isolated epispadias is corrected in infancy.

Primary prevention of latex allergy is necessary from the first procedure.

Anesthetic implications:

check renal function; indication of a lumbar epidural block for perioperative analgesia.

References :

-        Ebert AK, Reutter H, Ludwig M, Rösch WH.
The exstrophy-epispadias complex.
Orphanet J Rare Dis 2009; 4:23.

-        Massanyi EZ, Gearhart JP, Kost-Byerly S.
Perioperative management of classic bladder exstrophy.
Research and Reports in Urology 2013; 5: 67-75

       

Updated: January 2020