Obstetrics and gynecology · 2012
Müllerian remnant leiomyomas in women with Mayer-Rokitansky-Küster-Hauser syndrome.
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Fletcher Horace Melford, Campbell-Simpson Karen, Walcott Dwight, Harriott John
Аннотация
BACKGROUND: Müllerian remnant leiomyomas occur in women with Mayer-Rokitansky-Küster Hauser syndrome in which leiomyomas arise in absence of a uterus. This is a rare condition and we present two unusual cases.
CASES: In the first case, a woman with Mayer-Rokitansky-Küster-Hauser syndrome diagnosed was found to have a pelvic mass and was scheduled for laparotomy. A few days before the surgery, acute abdominal pain developed. At surgery she was found to have a twisted adnexum including a Müllerian remnant leiomyoma. This was untwisted successfully; the leiomyoma was excised and the ovary was saved. In the second case, one member of a pair of monozygotic twins was found to have leiomyomas at age 33 after having an absent uterus diagnosed at laparoscopy 8 years previously. She had successful removal of the leiomyoma at laparotomy. Her sister had normal development and had two children.
CONCLUSION: Mayer-Rokitansky-Küster-Hauser syndrome leading to Müllerian remnant leiomyomas is uncommon but should be suspected in women in whom a pelvic mass develops after the confirmation of the Müllerian defect.
CASES: In the first case, a woman with Mayer-Rokitansky-Küster-Hauser syndrome diagnosed was found to have a pelvic mass and was scheduled for laparotomy. A few days before the surgery, acute abdominal pain developed. At surgery she was found to have a twisted adnexum including a Müllerian remnant leiomyoma. This was untwisted successfully; the leiomyoma was excised and the ovary was saved. In the second case, one member of a pair of monozygotic twins was found to have leiomyomas at age 33 after having an absent uterus diagnosed at laparoscopy 8 years previously. She had successful removal of the leiomyoma at laparotomy. Her sister had normal development and had two children.
CONCLUSION: Mayer-Rokitansky-Küster-Hauser syndrome leading to Müllerian remnant leiomyomas is uncommon but should be suspected in women in whom a pelvic mass develops after the confirmation of the Müllerian defect.