A case of virilised female with urethral duplication and valve formation

We report the case of a female (chromosomal 46 XX) with a complex congenital urogenital malformation. Caesarian section was performed at the 30th gestational week due to hydrops fetalis and polyhydramnion. Clinical and radiological evaluation demonstrated a complex urogenital malformation with an enlarged penoclitoral organ, urethral duplication, and concomitant malformations. The girl presented with a female urethra with a vaginal orificium as well as a male urethra culminating in a microphallus. Additionally, a urethral valve formation just cranial to the bifurcation of the male and female urethra was also present. Due to concomitant diseases, a complicated clinical course and recurrent urinary tract infections, the final operative correction resulting in a female phenotype, was performed at the age of 2 (1/2) years as a single-stage procedure.