Müllerian Anomalies: Revisited

Abstract The Müllerian structures are developed from paired embryologic ducts that undergo fusion and resorption in utero to form the uterus, fallopian tubes, cervix, and upper two-thirds of the vagina. Any disruption of the normal embryological development of the Müllerian structure results in Müllerian anomalies. Müllerian anomalies are a complex spectrum of abnormalities that are usually associated with primary amenorrhea, infertility, and obstetric complications. Obstructed anomalies can be associated with endometriosis due to retrograde menstruation and sometimes endometriosis. Frequently, Müllerian anomalies are associated with renal anomalies; therefore, identification of both kidneys is important. Ovaries are normal in patients with Müllerian anomalies. Hysterosalpingography is routinely used in the evaluation of infertility and can demonstrate Müllerian anomalies, but has its limitations in characterizing the external fundal contour, which is a key component in categorizing the Müllerian anomaly. Two-dimensional ultrasound is the first-line modality for evaluating the uterus and adnexa. Three-dimensional ultrasound or magnetic resonance imaging may help to visualize the external uterine fundal contour and internal indentation of the endometrial cavity, which are two morphologic characteristics that are keys to the diagnosis of congenital uterine anomalies. Three-dimensional ultrasound can be used to evaluate uterine morphology and endometrial cavity configuration. The American Society for Reproductive Medicine (ASRM) and European Society of Human Reproduction and Embryology classifications are commonly used to categorize the Müllerian anomaly. The New ASRM classification is more practical as it appears simpler and more user-friendly, as it uses drawings with clear descriptions rather than symbols, and gives us a whole view of the anomaly. Correct diagnosis of Müllerian anomalies and their classification is critical for surgical and clinical management.