The most common presentation of congenital absence of the vagina is known as Mayer-Rokitansky-Kuster-Hauser Syndrome, which is characterized by the absence of the vagina and uterus, the presence of apparently normal tubes and ovaries, feminine
appearance, normal female secondary sexual characteristics, a normal 46, XY karyotype, and a feminine psychosexual orientation. Absence of the vagina results from an embryological arrest in the development of the lower portion of the Mullerian
system.
Congenital absence of the vagina, first described in 1527 by Realdus Columbus, is a rare anomaly. It is encountered about once in every 4000 to 5000 gynecologic patient. We report the successful performance of McIndoe procedure and laparotomy in
one
case.
|