Congenital Anomalies of the Urethra

Abstract

Although usually diagnosed in childhood, they can occasionally present in adult life. The cause is unknown, and they may be classified anatomically (type 1-3) or according to the degree of upper tract dilatation (mild, moderate, or severe). Presentation is usually with symptoms of bladder outflow obstruction, but other described symptoms are recurrent infections, infertility, perineal pain, and postejaculatory dysuria. Anterior urethral valves are especially rare, and also most commonly recognized in children. These are found anywhere along the length of the urethra, with no particular preponderance. They may present in adulthood with symptoms of bladder outflow obstruction.

Diagnosis rests on the findings of a dilated posterior urethra on micturating cystourethrography (ureteric reflux may also be seen in approximately 50%). The valve is seen as a filling defect just above the point of transition between a dilated posterior urethra and a normal or attenuated anterior urethra (Fig. 10.1). Oblique views are important, and the filling catheter should be removed, as it may compress the flap and render it nonvisible. Sonography can also be used to diagnose valves, either by the transabdominal or transperineal route. In the adult, TRUS can also be used. Ascending urethrography, however, will not always show the valve, as it can be flattened by the jet of contrast.