ABSTRACT: Müllerian agenesis occurs in 1 out of every 4,000-10,000 females. The most common presentation of müllerian agenesis is congenital absence of the vagina, uterus, or both, which also is referred to as müllerian aplasia, Mayer-Rokitansky-Küster-Hauser syndrome, or vaginal agenesis. Satisfactory vaginal creation usually can be managed nonsurgically with successive vaginal dilation; however, there are a variety of surgical options for creation of a neovagina. Regardless of the treatment option selected, patients should be thoroughly counseled and prepared psychologically before the initiation of any treatment. Evaluation for associated congenital renal anomalies or other anomalies is also important. Although exact gynecologic screening recommendations are evolving, all women with a neovagina should undergo routine gynecologic care; however, vaginal cytologic screening is not indicated.
Müllerian agenesis: diagnosis, management, and treatment. Committee Opinion No. 562. American College of Obstetricians and Gynecologists. Obstet Gynecol 2013;121:1134–7.