A rare case of Mayer-Rokitansky-Küster-Hauser syndrome with right ovarian torsion and hypoplasia of the left adnexa

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder caused by the abnormal absence of paramesonephric ducts. The main characteristics of this syndrome include hypoplasia or aplasia of the uterus, absence of the cervix and upper part of vagina. Otherwise, ovaries and fallopian tubes have normal functions. A 9-year-old girl presented to the emergency department by acute abdominal pain. Based on a high probable diagnosis of ovarian torsion, surgical exploration was accomplished demonstrating rudimentary or aplastic uterus, hypoplasia of the left adnexa and torsion of the right ovary. In consideration of MRKH syndrome, further assessments were done and the diagnosis was confirmed. As this syndrome is rare and there is a probability of ovarian torsion caused by malformation of the ovarian ligament, physicians should be aware of this syndrome to diagnose it earlier and preserve the ovarian tissue.