Vaginal agenesis is a rare mal-formation of the lower female genital tract, with a prevalence of 1 in 5000 female newborns. Mayer‑Rokitansky‑Kuster‑Hauser (MRKH) syndrome represents 90% of all causes and is caused by partial or complete aplasia of the Mullerian ducts. The majority of these women have complete uterine agenesis. Primary amenorrhea is the first clinical sign, in patients with 46 XX karyotype, functioning ovaries and normal external genitalia.
Creating a satisfactory neovagine when sexual activity is desired is primarily the goal of treatment. This ideal vaginal reconstruction should provide a long-lasting, functional passage for sexual intercourse that does not need maintenance with dilators or lubrication for sexual activity. At present, however, no standard treatment exists. Progressive dilatation is recommended by the American College of Obstetricians and Gynecologists as the first choice for creation of a neovagina, but this requires highly motivated patients and has a considerable failure rate. Surgical treatment usually shares a common step of tissue dissection between the urinary tract and the rectum.
