[Place of ultrasound in the management of Mayer-Rokitansky-Kuster-Hauser syndrome. Observational study from 2000 to 2017 within university hospital of Strasbourg]

Objectives: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is the most common cause of congenital absence or severe hypoplasia of structures derived from Muller's canals including the upper vagina, uterus and fallopian tubes. The definition of this syndrome is the presence of normal female secondary sexual development criteria relating to the presence of functional ovaries associated with vaginal agenesis and uterine anomalies ranging from a rudimentary uterus to the total absence of uterus. The main clinical sign of MRKH is primary amenorrhea. Confirmation of diagnosis and identification of associated abnormalities are based primarily on imaging and Magnetic Resonance Imaging (MRI) is currently the gold standard in the comprehensive evaluation of MRKH syndrome. Therefore, this study evaluated the place of ultrasound in the diagnostic and therapeutic management of patients treated for MRKH syndrome.

Methods: This retrospective, single-center, observational study collected all patients in charge of diagnosis or treatment of MRKH Syndrome between January 2000 and June 2017 within the University Hospital Gynecology and Obstetrics Department of Strasbourg. The analysis of the medical files allowed the evaluation of ultrasound in the different stages of the patient's care.

Results: Twenty-one patients were included and 81% get an ultrasound, 38% of them had a referred ultrasound performed by a certified radiologist. Forty-eight percent of the patients had an MRI and every ultrasound provided a correct diagnosis. Sixteen patients received therapeutic management and only 50% of patients had preoperative MRI.

Conclusion: The role of medical imaging is to define the extent of uterovaginal abnormalities for accurate diagnosis, describe any coexisting abnormalities, and provide a roadmap for surgical planning. The first-line examination is transabdominal ultrasound, a simple, non-invasive procedure. The use of MRI in our series did not bring any diagnostic surplus value. Despite the faster and easier access to MRI; ultrasound remains an indispensable tool in the diagnostic and therapeutic management of MRKH patients.