Epispadias is a rare congenital anomaly almost always associated with bladder exstrophy.
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Epidemiology
It occurs in 1 in 30,000 births, with a male: female ratio of 3:1.
Clinical presentation
The roof of the urethra is absent, and the urethra opens anywhere between the base and the glans of the penis.
In males, three types are described - glandular, penile, and complete. The glandular form is most common, and the urethra opens on the dorsum of the penis, just behind the glans. In the penile form, the opening may be anywhere along the penile shaft. However, it is most commonly seen near the base of the penis.
In females, three types are clitoric, subsymphyseal, and complete.
In complete forms of epispadias, incontinence is always seen due to sphincter involvement and is also associated with small bladder capacity.
Pathology
Incomplete urethral tubularization.
Associations
exstrophy-epispadias complex (EEC): represents a spectrum of genitourinary malformations ranging in severity from epispadias to classical bladder exstrophy and exstrophy of the cloaca
Radiographic features
Epispadias is a clinical presentation, and does not need a radiological diagnosis. However, associated bladder exstrophy features need to be well understood in this regard.
Treatment and prognosis
A Mitrofanoff procedure in an option in selected cases.