Objective: To report a case of acute abdomen secondary to torsion of uterine remnant leiomyoma and ipsilateral adnexa in a woman with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and known history of bilateral uterine remnant leiomyomas.
Design: Case report.
Setting: Tertiary care center.
Patient(s): A 40-year-old nulligravid woman with MRKH syndrome and a known history of bilateral uterine remnant leiomyomas with a surgical abdomen.
Surgery: right salpingo-oopherectomy, excision of right and left hemiuteri with pedunculated leiomyomas, and left salpingectomy.
Main Outcome Measure(s): Definitive therapy with preservation of premenopausal state.
Result(s): First known case of acute surgical presentation secondary to torsion of uterine remnant leiomyoma and ipsilateral adnexa in a woman with MRKH syndrome and bilateral voluminous uterine remnant leiomyomas without any plan for elective surgical intervention.
Conclusion(s): Elective surgical removal of uterine remnant leiomyoma for women with MRKH syndrome can prevent the complication of torsion as well as prevent ovarian resection in premenopausal women. Torsion should be considered in the differential diagnosis in a woman with MRKH syndrome and known history of leiomyomas who presents with acute abdomen.
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