True hermaphroditism. A case report with observations on its bizarre presentation

A true XX hermaphrodite presenting in adulthood with male sex gender identity was found to have separate vaginal and urethral openings in the perineum. A total vaginectomy was performed at the same time as total abdominal hysterectomy and gonadectomy because the vaginal opening would interfere with a urethroplasty to repair the hypospadias and chordee phallus. During this procedure prostatic tissue was excised in the area of the anterior vaginal wall adjacent to the urethra. Recommendations regarding this rare XX true hermaphroditism are put toward and observations on some bizarre features of the entity are made. The authors plead for the formation of a national register to study this interesting condition.