The gynecologic needs of individuals with anorectal malformations (ARMs) are complex and vary throughout the life course. It is well known that Müllerian anomalies are common in this population, particularly uterus didelphys which is reported in up to 60% of patients with cloacal anomalies.1,2 A concurrent Mullerian anomaly is less common with other anorectal malformations. For example a didelphys uterus is only identified in 5% of patients with a rectovestibular fistula.3 Obstruction resulting in hematometra/colpos has been observed in 26-41% of patients with cloaca. Obstruction can result from acquired causes such as vaginal stenosis or congenital causes such as obstructing septa or rudimentary uterine remnants.[4], [5], [6]

Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) is a well described constellation of anomalies that includes didelphys uterus, vaginal septum obstructing menstrual outflow of one uterus, and an ipsilateral renal anomaly. While an obstructed hemivagina can occur in patients both with and without ARM, the anatomy has potential to be more complex and varied in patients with ARM. The following is a case series of patients with didelphys uterus and obstructed hemivagina that compares the anatomic variation that exists in patients with concurrent ARM to those without ARM.