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.
Highlights
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare phenomenon characterized by congenital absence of the vagina with variable uterine development
MRKH syndrome is classified as either type I, with isolated Mullerian anomalies, or type II, with associated malformations in other systems
We report the case of a 19-year-old woman who initially presented with type I MRKH syndrome
Summary
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare phenomenon characterized by congenital absence of the vagina with variable uterine development. We report the case of a 19-year-old woman who initially presented with type I MRKH syndrome. During the postoperative course of a neovagina procedure, the patient developed extensive pelvic deep venous thrombosis (DVT) and was diagnosed with a previously unsuspected inferior vena cava (IVC) malformation.
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