OBSTRUCTED HEMIVAGINA AND IPSILATERAL RENAL ANOMALY SYNDROME: A NONCLASSICAL PRESENTATION WITH AN INTERCERVICAL COMMUNICATION

Abstract

To describe a nonclassical presentation and infertility management of Obstructed Hemivagina with Ipsilateral Renal Agenesis Syndrome (OHVIRA), also known as Herlyn-Werner-Wunderlich Syndrome (HWWS). This is a case report of a 31-year-old female with OHVIRA syndrome who presented to a community-based medical center and fertility office. A 31-year-old female presented to her obstetric provider with a missed abortion (MAB) and decision was made for surgical management. Intraoperatively, a uterine didelphys was incidentally found due to an inability to evacuate the correct cavity during suction dilation and curettage. Additionally, the right cervix was dilated three centimeters, likely due to chronic hematocolpos and hematometria. MRI imaging suggested OHVIRA syndrome with a patent cervical connection at the level of the internal cervical os. Subsequently, she underwent complete vaginal septum excision and marsupialization. A prophylactic robotic-assisted transabdominal cerclage was then placed for concern of an incompetent cervix from chronic obstruction and intercervical communication. Finally, in vitro fertilization was undertaken, leading to a successful pregnancy outcome. OHVIRA syndrome classically presents in adolescence with nonspecific complaints of dysmenorrhea or pelvic pain. We report a nonclassical presentation of OHVIRA syndrome and illustrate a stepwise management strategy for women with this heterogenic Mullerian anomaly to achieve and maintain a pregnancy to term.