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Clinical manifestations and diagnosis of congenital anomalies of the uterus

Ronald E Iverson, Jr, MD
Alan H DeCherney, MD
Marc R Laufer, MD
Section Editor
Robert L Barbieri, MD
Deputy Editor
Sandy J Falk, MD, FACOG


Congenital anomalies of the uterus are often asymptomatic and therefore unrecognized. They may affect a young woman due to pain at the time of menarche, or a woman's obstetric and/or gynecologic health.

The classification, diagnosis, and clinical manifestations of congenital uterine anomalies will be reviewed here. Treatment of these anomalies and congenital cervical and vaginal abnormalities are discussed separately. (See "Surgical management of congenital uterine anomalies" and "Congenital cervical anomalies and benign cervical lesions" and "Diagnosis and management of congenital anomalies of the vagina".)


A classification system is an attempt to group by symptomatology, treatment, or prognosis. It is through use of standardized nomenclature that physicians can more accurately codify the symptomatology, treatment, and outcome of patients with uterine anomalies. Unfortunately, there is no universally accepted and interpreted classification system for uterine anomalies [1].

The European Society of Human Reproduction and Embryology (ESHRE) and the European Society for Gynaecological Endoscopy (ESGE) published a classification system in 2013 (figure 1A-B) [2].

The American Fertility Society in 1988 produced a standard form for classification of müllerian defects (table 1 and figure 2) [3]. This descriptive form focuses largely on vertical fusion defects and, therefore, on uterine dysgenesis. Associated anomalies in the vagina, cervix, fallopian tubes, and renal system should also be noted.

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Literature review current through: Nov 2017. | This topic last updated: Sep 26, 2017.
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