Epispadia

In male epispadias, the urethra opens as an epithelial-lined groove on the dorsal aspect of the penis, proximal to the glans pubis. Depending on the location of the opening, male epispadias may be classified as glandular (or balanic; opening in the glans penis), penile (opening in the penile shaft) or penopubic (opening at the penopubic junction). The penopubic form is the most severe, and the most likely to be associated with separation of the pubic bones and disruption of the bladder neck resulting in incontinence.

In female epispadias, the clitoris is usually bifid, the mons is flattened, the labia are separated, and there is a variable urethral defect. In the mildest form, the urethral opening is merely potulous. In intermediate forms, there is a dorsal split along most of the urethra. In the most severe form, the cleft extends to involve the external sphincter, resulting in incontinence. In females, epispadias is usually of the severe (or complete) form and bone changes are usually present.

Management of epispadias is surgical, and aimed at reconstruction of the urethral opening, correction of the dorsal chordee (which is almost invariably co-existent in males), and correction of incontinence (where possible). Repair of the associated bone malfunction may also be required.

Plain radiographs may show widening of the symphysis pubis. Voiding cystourethrography may reveal reflux, which is common in the more severe forms of epispadias due to a small bladder with abnormally located ureteric orifices. MRI may be helpful in assessing the urethral defect.

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