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Congenital uterine anomalies: a resource of diagnostic images, part 1: accurate diagnosis of such anomalies has prognostic importance for both obstetric and gynecologic outcomes.

Uterine malformations make up a diverse group of congenital anomalies that can result from various alterations in the normal development of the Mullerian ducts, including underdevelopment of one or both Mullerian ducts, disorders in Mullerian duct fusion, and alterations in septum reabsorption. How common are such anomalies, how are they classified, and what is the best approach for optimal visualization? Here, we explore these questions and offer an atlas of diagnostic images as an ongoing reference for your practice. Many of the images we offer will be found only online at

How common are congenital uterine anomalies?

The reported prevalence of uterine malformations varies among publications due to heterogeneous population samples, differences in diagnostic techniques, and variations in nomenclature. In general, they are estimated to occur in 0.4% (0.1% to 3.0%) of the population at large, 4% of infertile women, and between 3% and 38% of women with repetitive spontaneous miscarriage. (1)

Classical classification

A classification of the Mullerian anomalies was introduced in 1980 and, with few modifications, was adopted by the American Fertility Society (currently, ASRM). The Society identified seven basic groups according to Mullerian development and their relationship to fertility: agenesis and hypoplasias, unicornuate uteri (unilateral hypoplasia), didelphys uteri (complete nonfusion), bicornuate uteri (incomplete fusion), septate uteri (nonreabsorption of septum), arcuate uteri (almost complete reabsorption of septum), and anomalies related to fetal DES exposure. (2)

Anomalies also can be categorized in terms of progression along the developmental continuum, taking into account that many cases result from partial failure of fusion and reabsorption: agenesis (Types I and II), lack of fusion (Types III and IV), lack of reabsorption (Types V and VI), and lack of posterior development (Type VII). (3)

3D ultrasonography offers accurate, cost-efficient diagnosis

Using only 2D imaging, neither an unenhanced sonogram nor a sonohysterogram can provide definitive information regarding the possibility of a uterine anomaly. The fundal contour cannot be evaluated with 2D imaging; likewise, details regarding the configuration of the uterine cavity (or cavities) may not be appreciated with the use of 2D imaging (FIGURE 1).

To fully evaluate the uterine fundal contour and determine the type of uterine anomaly, it previously was necessary to obtain magnetic resonance imaging (MRI) or perform laparoscopy. Today, however, 3D coronal ultrasonography (US) can allow for accurate evaluation of fundal contour and diagnosis of uterine anomalies with lower cost and greater patient convenience. Several studies have confirmed the high accuracy of 3D US compared with MRI and surgical findings in the diagnosis of uterine anomalies (with 3D US showing 98% to 100% sensitivity and specificity). (4-6)

CASE Partial septate uterus

Upon 2D sagittal sonography (A), the uterus of a patient undergoing infertility evaluation shows a left lateral fibroid but otherwise appears normal. Transverse 2D view reveals 2 endometrial canals (hands) at the fundus and fibroid (arrows; B). 3D coronal imaging of the same patient demonstrates partial septate cavity and left lateral fibroid (C).


A special introduction from Steven R. Goldstein, MD, to the full offering of diagnostic images by Drs. Ozcan and Kaunitz, at


For the full offering of diagnostic images, including the ASRM classification of anomalies, normal endometrial cavity, arcuate uterus, incomplete (partial) uterine septum, and complete uterine septum, see the Web version of this article, at Look for Part 2 of this installment next month, when we will present images detailing the unicornuate, bicornuate, didelphic, and DES-exposed uterus.


(1.) Bermejo C, Martinez Ten P, Cantarero R, et al. Three-dimensional ultrasound in the diagnosis of Mullerian duct anomalies and concordance with magnetic resonance imaging. Ultrasound Obstet Gynecol. 2010;35(5):593-601.

(2.) The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, mullerian anomalies and intrauterine adhesions. Fertil Steril. 1988;49(6):944-955.

(3.) Acien P, Acien M. Updated classification of malformations. Hum Reprod. 2010;25(suppl I):i81-i82.

(4.) Deutch T, Bocca S, Oehninger S, Stadtmauer L, Abuhamad AZ. Magnetic resonance imaging versus three-dimensional transvaginal ultrasound for the diagnosis of Mullerian anomalies [abstract P-465]. Fertil Steril. 2006;86(suppl): S308.

(5.) Wu MH, Hsu CC, Huang KE. Detection of congenital Mullerian duct anomalies using three-dimensional ultrasound. J Clin Ultrasound. 1997;25(9):487-492.

(6.) Deutch TD, Abuhamad AZ. The role of 3-dimensional ultrasonography and magnetic resonance imaging in the diagnosis of Mullerian duct anomalies. J Ultrasound Med. 2008;27(3):413-423.

Michelle Stalnaker Ozcan, MD, and Andrew M. Kaunitz, MD

A special introduction from Steven R. Goldstein, MD, to the full offering of diagnostic images by Drs. Ozcan and Kaunitz, at

Dr. Stalnaker Ozcan is Assistant Professor and Associate Program Director, Obstetrics and Gynecology Residency, Department of Obstetrics and Gynecology, University of Florida College of Medicine-Jacksonville.

Dr. Kaunitz is University of Florida Research Foundation Professor and Associate Chairman, Department of Obstetrics and Gynecology, University of Florida College of Medicine-Jacksonville. He serves on the OBG Management Board of Editors.

The authors report no financial relationships relevant to this article.
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Author:Ozcan, Michelle Stalnaker; Kaunitz, Andrew M.
Publication:OBG Management
Date:Nov 1, 2014
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