Multiple Fibroids in a Case of MRKH Syndrome with Absent Uterus: Recommendations for Management.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital anomaly characterized by aplasia or hypoplasia of uterus and vagina in women with normal development of secondary sex characteristics. It affects one in 4,000-5,000 female births. Women with this syndrome present in their teens with primary amenorrhea. MRKH syndrome may be associated with renal, skeletal, cardiac and auditory anomalies. Although, very rare women with MRKH syndrome may develop leiomyomas from a rudimentary uterus. Initial investigation in women having MRKH syndrome with leiomyoma is ultrasonography (USG). However, magnetic resonance imaging (MRI) is more accurate for diagnosis. Complete removal of the masses with the uterine remnant is recommended.