Purpose of review: To review the Mayer-von Rokitansky-Küster-Hauser syndrome and to address means of diagnosis, patient education and counselling. The timing of, and vast options for, creation of a functional vaginal are also discussed.
Recent findings: The diagnosis of Mayer-von Rokitansky-Küster-Hauser usually occurs during an evaluation of primary amenorrhea. Counselling and support are of great importance for affected young women and their families. Educational materials have increased with the availability of Internet web sites and there is a vast number of options for creation of a functional vagina; most international centers promote the utilization of vaginal dilators.
Summary: Young girls, adolescents and women with Mayer-von Rokitansky-Küster-Hauser should be offered a comprehensive evaluation, and presented with information regarding all options for management and support. Ongoing psycho-social and educational support is extremely important. International centers that focus on congenital anomalies of the reproductive tract should be developed. These centers of excellence will facilitate long-term follow up studies to improve patient care and evidence based medical options.