Female epispadias: are we missing the diagnosis?

To describe three of four female patients with occult epispadias, as usually epispadias is reported to be a rare condition in females (1/480,000), and obvious and easy to diagnose, but less severe variants may still involve urethral sphincter incompetence in the absence of obvious clinical signs.

The hospital records over a 10-year period (1 January 1991 to 31 December 2000) were reviewed to find all female patients diagnosed with epispadias. Data were collected from the Consultative Council on Obstetric and Paediatric Mortality and Morbidity for the number of live births per year for the decade studied, and the incidence of female epispadias determined.

Over the 10-year period there were 13 patients with a diagnosis of epispadias, from 636,698 total live births; four were female and nine male. Each of the female patients had varying degrees of epispadias; three had subtle signs with apparently normal external genitalia, and only one had a bifid clitoris. However, even the most minor variant case had a palpable notch in the symphysis pubis, and an 'oblong' external urethral meatus. Thus the incidence of female epispadias is at least 1/160,000.

Epispadias in females may be up to three times more common than previously expected, but even experienced clinicians can miss the diagnosis. The importance of a careful examination should be emphasized, especially in female patients with chronic wetting of unknown cause. A bifid clitoris is an important sign but not always present. Key diagnostic features include drug-resistant wetting, a very low leak pressure on cystometrography, a palpable gap in the pubic symphysis and an abnormal oval-shaped external urethral meatus.