Abstract
We report the case of an unusual genitourinary anomaly in a 5-year-old patient. The malformation consisted of a para-anal persistent urogenital sinus in a 46XX patient associated with a scrotum-like structure in the presumed clitoral location. A duplicate urethra arising from the anterior aspect of the bladder neck drained into the scrotal pouch. Two hemivaginas drained into the urogenital sinus. The patient was totally continent through the urogenital sinus but the duplicate urethra was incontinent. No hormonal problems were encountered. The girl underwent total urogenital sinus mobilization, scrotal pouch ablation, and duplicate urethra resection.
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