All of the following are true about MRKH (Mayer – Rokitansky – Kuster – Hauser) syndrome, EXCEPT:

Correct Answer: Absent ovary
Description: Congenital absence of both the uterus and vagina is termed mullerian aplasia, mullerian agenesis, or Mayer-Rokitansky-Kuster-Hauser syndrome. In classic mullerian agenesis, patients have a shallow vaginal pouch, only measuring 1 to 2 inches deep. In addition, the uterus, cervix, and upper pa of the vagina are absent. Typically, a poion of the distal fallopian tubes are present. Also, normal ovaries are expected, given their separate embryonic source. Most patients with mullerian agenesis have only small rudimentary mullerian bulbs without endometrial activity. However, in 2 to 7 percent of women with this condition, active endometrium develops and patients typically present with cyclic abdominal pain. Ref: Hoffman B.L., Schorge J.O., Schaffer J.I., Halvorson L.M., Bradshaw K.D., Cunningham F.G., Calver L.E. (2012). Chapter 18. Anatomic Disorders. In B.L. Hoffman, J.O. Schorge, J.I. Schaffer, L.M. Halvorson, K.D. Bradshaw, F.G. Cunningham, L.E. Calver (Eds), Williams Gynecology, 2e.
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