epispadias
Epispadias is a rare congenital anomaly that is almost always associated with bladder exstrophy.
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 penis, just behind the glans. In the penile form, the opening may be anywhere along penile shaft, however, it is most commonly seen near the base of the penis.
In females, three types have been described - 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.
See also
Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu epispadias: