Rectoanal Malformation with Rectovestibular Fistula vs. Testicular Feminization

Rectoanal Malformation with Rectovestibular Fistula vs. Testicular Feminization

Left picture: Here, the diagnosis is a low rectoanal malformation with an anovestibular fistula. Again, a precise clinical examination with spreading of the external genitals allows the determination of the cause of the conspicuous external genitals in this girl, which is demonstrated by the picture at the bottom of the same patient. Right picture: This toddler has a testicular feminization which is a type of male pseuodhermaphrodidism with XY-chromosomes, a peripheral resistance to testosterone, and female external genitals. In girls with recurrent protrusions of inguinal hernias this diagnosis must be considered as in the operative picture a the bottom of a girl with bilateral inguinal hernia and absent female internal genitals as ovary and fallopian tube.

Left picture: In this girl, the labia maiora which cover nearly completely the labia minora, form an apron with a central opening in the direction of the anus. Right picture: In this female toddler the labia minora are more visible than in the contralateral picture. The external genitals are inconspicuous except for a perianal pigmentation and an alteration like a scar on the relatively long perineum. During surgery, which was necessary because of repeated protrusion of inguinal hernia on both sides, a large hernia sac is visible with a wide neck and a protruded intestinal segment; instead of a left-sided adnex a gonad is recognizable with a double tuberosity in the figure at the top of the same side.