Epispadias is a rare congenital anomaly that is almost always associated with bladder exstrophy.
It occurs in 1 in 30,000 births, with a male: female ratio of 3:1.
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.
Incomplete urethral tubularization.
- exstrophy-epispadias complex (EEC): represents a spectrum of genitourinary malformations ranging in severity from epispadias to classical bladder exstrophy and exstrophy of the cloaca.
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.
- 1. Ryu J, Kim B. MR imaging of the male and female urethra. Radiographics. 21 (5): 1169-85. Radiographics (full text) - Pubmed citation
- 2. Ebert AK, Reutter H, Ludwig M, Rösch WH. The exstrophy-epispadias complex. Orphanet journal of rare diseases. 4: 23. doi:10.1186/1750-1172-4-23 - Pubmed
- 3. Ulman I, Ergün O, Avanoğlu A, Gökdemir A. The place of Mitrofanoff neourethra in the repair of exstrophy-epispadias complex. European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie. 8 (6): 352-4. doi:10.1055/s-2008-1071231 - Pubmed