Abstract
Background: The occurrence of an extra-uterine leiomyoma, arising from the intra-peritoneal portion of the round ligament in a lady with Mullerian agenesis diagnosed at the age of forty is extremely rare. We report a case of this rare combination in a Middle Eastern woman. Case: A 40 years old lady, primarily amenorrheic, presented to our clinic for an infertility consultation. The work-up showed features suggestive of Mayer-Rokitansky- Kuster-Hauser (MRKH) syndrome with a leiomyoma arising from the intra-peritoneal part of the round ligament.
Highlights
The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome or Müllerian agenesis is characterized by the congenital absence of the upper vagina, cervix and uterus
Girls with Müllerian agenesis have normal childhood and they usually do not seek professional advice until puberty when the syndrome presents as primary amenorrhea, otherwise they have normal development of secondary sexual characteristics, normal external genitalia, functional ovaries, and a normal karyotype of 46, XX (Morcel and Camborieux, 2007)
We present a case of intraperitoneal leiomyoma of the round ligament in a patient diagnosed, for the first time at age of forty, to have the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome
Summary
The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome or Müllerian agenesis is characterized by the congenital absence of the upper vagina, cervix and uterus. We present a case of intraperitoneal leiomyoma of the round ligament in a patient diagnosed, for the first time at age of forty, to have the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. She was scheduled for a diagnostic laparoscopy as being part of primary amenorrhea work up and for the pelvic mass assessment.
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
