Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: Common presentation of an unusual variation

Abstract

Mullerian duct abnormalities are congenital malformations that are easily missed and can lead to incorrect diagnosis and further unnecessary operative procedures.  In this case, a young female presented with cyclic pelvic pain that continued after previous surgical resection of an ovarian cyst. Further investigation with clinical examinations and multimodality imaging demonstrated ipsilateral renal agenesis and a Class III Mullerian duct anomaly (MDA) requiring a second operative procedure.  It is believed that this case is a variant of the described obstructed hemi-vagina with ipsilateral renal agenesis (OVIRA) anomaly as pathologically there was ipsilateral renal agenesis and complete vaginal agenesis in our case.   It is imperative to have a high clinical suspicion of mullerian duct abnormalities when encountering a patient with other urogenital anomalies. This will decrease the amount of misdiagnoses, guide appropriate surgical intervention, and decrease the risk of future reproductive complications.