Congenital urethroperineal fistula, CUPF, is an extremely rare anomaly. A second urethral channel extends from the prostatic urethra to the perineal surface. The fistulous tract in CUPF opens ventrally and can be resected without loss of urinary function. In true urethral duplications, it is the ventrally positioned urethra that will always be the functional one and will need to be preserved for urinary purposes. Thus CUPF must be distinguished from true urethral duplication of the hypospadius variety. This requires clinical evaluation of voiding function and visualization of the urethra and fistula, and radiographic investigation with VCUG and injection of the perineal opening. With voiding, the predominant urinary stream will exit through the glands of the penis and only a few drops of urine will exit through the ventrally positioned fistula. The opposite is the case in duplication of the urethra of the hypospadius variety in which the majority of the urinary stream will exit at the perineal opening. Because voiding is predominately through the penile meatus in boys with CUPF, the clinical presentation is often delayed and may include slight urinary incontinence or urinary tract infection.

 

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