[A case of Mayer-Rokitansky syndrome]

The Mayer-Rokitansky syndrome has been generally defined as a congenital absence of the vagina in genotypic and phenotypic female subjects with normal endocrine status. Many authors have reported that urological anomalies associate commonly with this syndrome, but recently a new concept of this syndrome was proposed by Tarry and associates. They considered the embryogenesis of the Müllerian system and recognized a spectrum of Müllerian anomalies without total vaginal agenesis as this syndrome. They also proposed a new classification of this syndrome (Müllerian grade) considering grade of Müllerian anomaly. We experienced a 14-year-old girl who had uterus didelphys, left hematocolpos due to ipsilateral vaginal agenesis and agenesis of the left kidney, and herein report this case as Mayer-Rokitansky syndrome. In addition to Tarry's classification, we propose another embryologic classification for Müllerian anomalies considering the period of faulty differentiation in this syndrome: type A-faulty differentiation of the mesonephros (before week 4), type B-faulty differentiation of the mesonephric duct (week 4 to 5), type C- faulty differentiation of the paramesonephric duct only (after week 6).