Acute torsion of uterine remnant leiomyoma with Mayer-Rokitansky-Küster-Hauser syndrome.
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.


METHODS
Case report.


METHODS
Tertiary care center.


METHODS
A 40-year-old nulligravid woman with MRKH syndrome and a known history of bilateral uterine remnant leiomyomas with a surgical abdomen.


METHODS



METHODS
right salpingo-oopherectomy, excision of right and left hemiuteri with pedunculated leiomyomas, and left salpingectomy.


METHODS
Definitive therapy with preservation of premenopausal state.


RESULTS
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.


CONCLUSIONS
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.
Department of Obstetrics and Gynecology, Albert Einstein Medical Center, Philadelphia, Pennsylvania. Electronic address: kuhali@gmail.com.
Kuhali
K
Kundu
Department of Obstetrics and Gynecology, Albert Einstein Medical Center, Philadelphia, Pennsylvania.
Arnold W
AW
Cohen
Department of Obstetrics and Gynecology, Albert Einstein Medical Center, Philadelphia, Pennsylvania.
Jay
J
Goldberg
D058489:46, XX Disorders of Sex Development / Q000150:complications* / Q000175:diagnosis; D000006:Abdomen, Acute / Q000209:etiology; D000328:Adult; D000013:Congenital Abnormalities / Q000175:diagnosis; D005260:Female; D006801:Humans; D018231:Leiomyomatosis / Q000150:complications* / Q000473:pathology / Q000601:surgery; D009095:Mullerian Ducts / Q000002:abnormalities*; D010052:Ovariectomy; D058994:Salpingectomy; D014102:Torsion Abnormality / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D016896:Treatment Outcome; D014594:Uterine Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery
Mayer-Rokitansky-Küster-Hauser syndrome; acute abdomen; leiomyoma; ovarian preservation; torsion