(135) Preconception Counseling in the Setting of Unilateral Cervical Atresia in a Patient with a Bicornuate Bicollis Uterus: A Case Study

Background: Obstructive müllerian anomalies are rare and can be associated with pelvic pain, adverse pregnancy outcomes, and infertility. We describe the first known case of a patient with a bicollis bicornuate uterine anomaly with unilateral cervical atresia and communication between the two cervices, an Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA) anomaly variant.

Case: A 21-year-old nulligravid woman presented for deep dyspareunia and a suspected diagnosis of OHVIRA based on her initial outside evaluation. Initial MRI was interpreted to show widely divergent uterine horns and two cervices—possibly representing a didelphys versus bicornuate bicollis uterus with no hematocolpos or obvious vaginal septum, as well as left renal agenesis. Physical exam demonstrated a normal vaginal canal without bulge and a single palpable cervix on digital and speculum exam. A repeat MRI (Figure 1) demonstrated a bicornuate bicollis uterus with two separate uterine cavities and cervices. Additionally, there was noted to be a blind-ending left cervix and a communicating channel between the distal cervices leading to a single vagina were detected. No hematocolpos or obstruction was appreciated. Based on updated imaging, surgical intervention was not recommended. After conferring with a reproductive endocrinologist, we counseled the patient regarding the sequelae of conceiving a pregnancy in the left uterine horn. In the absence of a direct communication between the left uterine cavity and the vagina, a hysterotomy would be required for either miscarriage management or for a pregnancy termination. Furthermore, pregnancy management would be more complicated in that horn and a cesarean birth would be required. For these reasons, it was strongly recommended that she consider in vitro fertilization to optimize implantation in the right uterine cavity for any future pregnancy.

Comments: We report the first known incidence of a rare müllerian anomaly, thought to be a likely variant of OHVIRA. The communication between cervices, in addition to a blind-ending unilateral cervix is an anatomical variant not previously described in the literature. This patient is at risk for unique adverse pregnancy outcomes due to her theoretical ability to conceive a pregnancy in the uterine horn that does not have a direct connection to the vagina. We recommend the use of reliable contraception until she is ready to conceive under the care of a reproductive endocrinologist.