title: Mayer-rokitansky-kuster-hauser syndrome associated with severe inferior vena cava stenosis.
abstract: Precis. The postoperative course of a neovagina creation procedure in a young woman with Meyer-Rokitansky-Kuster-Hauser syndrome was complicated, despite prophylaxis, by extensive pelvic deep venous thrombosis secondary to unsuspected severe inferior vena cava stenosis. Background. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital vaginal agenesis and an absent or rudimentary uterus in genotypical females. Malformations of the inferior vena cava (IVC) are not commonly associated with MRKH syndrome. We report a case of a patient with MRKH syndrome with severe IVC stenosis that was diagnosed when the patient presented with extensive pelvic deep venous thrombosis (DVT) during the postoperative course of a neovagina creation. Case. A 19-year-old female underwent a McIndoe procedure. Despite DVT prophylaxis, extensive pelvic DVT of the femoral vein was diagnosed on postoperative day 7. Therapeutic anticoagulation was initiated, and pharmacological and mechanical thrombolysis were performed. During these procedures, a hypoplastic IVC was noted. Conclusion. MRKH syndrome can be associated with IVC malformations, which constitute an anatomical risk factor for postoperative DVT.
authors.affiliation: Division of Reproductive Endocrinology and Infertility, Department of Gynecology and Obstetrics, Johns Hopkins University School of Medicine, 10751 Falls Road, Suite 280, Lutherville, MD 21093, USA.
authors.forename: Laura
authors.identifier: 0000-0002-8926-2407
authors.initials: L
authors.lastname: Londra
authors.affiliation: Division of Reproductive Endocrinology and Infertility, Department of Gynecology and Obstetrics, Johns Hopkins University School of Medicine, 10751 Falls Road, Suite 280, Lutherville, MD 21093, USA.
authors.forename: Kyle
authors.identifier: 0000-0002-7566-9919
authors.initials: K
authors.lastname: Tobler
authors.affiliation: Division of Reproductive Endocrinology and Infertility, Department of Gynecology and Obstetrics, Johns Hopkins University School of Medicine, 10751 Falls Road, Suite 280, Lutherville, MD 21093, USA.
authors.forename: John
authors.initials: J
authors.lastname: Wu
authors.affiliation: Division of Reproductive Endocrinology and Infertility, Department of Gynecology and Obstetrics, Johns Hopkins University School of Medicine, 10751 Falls Road, Suite 280, Lutherville, MD 21093, USA.
authors.forename: Lisa
authors.initials: L
authors.lastname: Kolp