eMedicine Specialties > Radiology > Obstetrics/Gynecology
Uterus, Mullerian Duct Abnormalities: Imaging
Updated: Oct 16, 2009
Radiography
Findings
Before the advent of MRI and US, the primary imaging modality for evaluating uterine anomalies was limited to fluoroscopy or HSG. Fluoroscopic spot films are obtained after the cervix is cannulated and radiopaque contrast is injected into the uterine cavity.
HSG provides high-resolution images of the contour of the uterine cavity and fallopian tubes and remains the key imaging test for assessing tubal abnormalities that may cause infertility. Typically, the question of müllerian duct anomaly arises during HSG examination if the typical trigone configuration of the cavity is not demonstrated.
A common finding is separation of the uterine cavity into right and left compartments. A divided uterine cavity can result from septate, bicornuate, or didelphys uterus. Certain criteria are used to increase confidence in diagnosing 1 of the 3 entities.
- Intercornual distance: If the distance between the distal ends of the horns (ends that are continuous with fallopian tubes) is less than 2 cm, the likelihood of septate uterus is increased. If the distance is greater than 4 cm, the likelihood of didelphys uterus is increased. Measurements of 2-4 cm (typical distance in a normal uterus) were indeterminate in an abnormal cavity configuration.
- Intercornual angle: This is the angle formed by the most medial aspects of the 2 uterine hemicavities. If the angle is less than 60°, septate uterus is more likely. For larger angles, the anomaly is more likely to be a bicornuate uterus (see Image below and Image 2 in Multimedia).
Uterus, müllerian duct abnormalities. Surgically proven case of bicornuate uterus. Correct diagnosis may be suggested based on hysterosalpingography findings, which are, most notably, the widened intercornual distance (>4 cm) and the widened intercornual angle (>60°).
- T-shaped cavity: A hypoplastic, irregular, T-shaped uterine cavity is pathognomonic for in utero DES exposure. The uterus typically is much smaller than average, and many forms of cavity appearance exist with varying, but usually symmetric, regions of narrowing or dilatation in the segments of the T. MRI and US examinations typically demonstrate uterine hypoplasia only (see Image below and Image 3 in Multimedia).
Uterus, müllerian duct abnormalities. T-shaped uterus. Classic configuration of the uterine cavity in a typical diethylstilbestrol-exposed uterus (American Fertility Society class VII). Uteri are typically hypoplastic. In this patient, no maternal history of diethylstilbestrol exposure was found.
Degree of Confidence
A large overlap exists between the subtypes when comparing uterine cavitary configuration, intercornual distance, and intercornual angle. In several studies, HSG had significantly less accuracy for diagnostic precision than MRI or US. In the studies, much of the final pathology was based on laparoscopic or surgical findings, primarily of the appearance of the uterine fundus, which HSG was not able to assess.
Since HSG techniques did not provide diagnoses with high degrees of confidence, US and MRI soon began to play a larger role in assessment and treatment of patients. Currently, HSG has been all but abandoned as a primary modality for workup of potential congenital uterine anomalies. Anomalies incidentally discovered on HSG are referred for further evaluation using MRI or US.
The only anomaly in which HSG plays a significant role in diagnosis is DES uterus (AFS class VII). The abnormal uterine cavity can be depicted clearly on HSG but often is visualized as only uterine hypoplasia on US or MRI.
False Positives/Negatives
HSG findings commonly allow misdiagnoses of partial septate versus bicornuate uteri or complete septate versus bicornuate bicollis versus didelphys uteri because of the large degree of overlap in the intercornual distances and angles in the entities. While the specific diagnosis may be uncertain, an abnormality usually is clearly present (see Image below and Image 1 in Multimedia).
Uterus, müllerian duct abnormalities. This was difficult to differentiate as septate or bicornuate uterus using hysterosalpingography. It was a surgically proven case of bicornuate uterus.
Magnetic Resonance Imaging
Findings
Examination protocol
MRI of the uterus (in benign conditions such as congenital anomalies or fibroid evaluation) is performed at the author's institution following administration of 1.0 mg IM of glucagon to decrease motion artifacts associated with bowel peristalsis. For the diagnosis of most anomalies, 5 main sequences are sufficient.
- Coronal single-shot fast spin-echo (FSE) images of the kidneys, ureters, and pelvis provide good localized and survey views of kidneys and ureters. This sequence is important because of the high incidence of associated renal anomalies (up to 50%), including agenesis, kidney duplication, or pelvic kidney.
- Axial T1 spin-echo (without fat-saturation pulse) images. Fat signal is useful to delineate pelvic structures. An anterior saturation band is used to attenuate ghosting artifact from respiration.
- Sagittal T2 FSE (without fat-saturation pulse) images. Fat signal is useful to delineate pelvic structures. An anterior saturation band is recommended. This sequence is useful for determining anterior versus retroverted uterus and to set up subsequent oblique scans along the long and short axes of the uterine fundus.
- Oblique long-axis T2 FSE (without fat-saturation pulse) images are similar to sagittal T2 FSE sequences but obtained along the long axis of the uterus (perpendicular to the sagittal plane, usually a slightly oblique coronal). This plane is ideal for visualization of the uterine cavity and uterine fundal contour.
- Oblique short-axis T2 FSE (without fat-saturation pulse) images are perpendicular to the long axis and sagittal planes (usually oblique axial), providing short-axis (target) views of the uterine cavity useful for visualizing a transverse septum if present.
MRI appearance of müllerian anomalies (AFS classification system)
Class I (hypoplasia/agenesis): Findings of agenesis include absence of the uterus, cervix, and/or upper two thirds of the vagina. In uterine agenesis, no identifiable uterine tissue is noted. Partial agenesis of müllerian duct derivatives also can be visualized. In uterine hypoplasia, the endometrial cavity is small, with a reduced intercornual distance (<2 cm). When uterine hypoplasia is associated with hormonal dysfunction (infantile uterus), not only is the uterus small, but the zonal anatomy is differentiated poorly on T2-weighted images.
Class II (unicornuate uterus): Unicornuate uterus appears banana shaped without the usual rounded fundal contour and triangular appearance of the fundal cavity (see Image below and Image 9 in Multimedia). Uterine zonal anatomy is normal. If present, a rudimentary horn can be observed as a soft-tissue mass with signal intensity similar to that of myometrium. If obstructed, a rudimentary horn with functioning endometrium may be distended by blood or blood products.
Uterus, müllerian duct abnormalities. Unicornuate uterus. Patient has full development of a single uterine horn and a normal-appearing cervix. This anomaly was one of many in this patient with Goldenhar syndrome.
Class III (didelphys uterus): Two separate normal-sized uteri and cervices are seen (see Image below and Image 8 in Multimedia). A septum may be visualized extending into the upper vagina. The 2 uterine horns are usually widely splayed, and endometrial and myometrial zonal widths are preserved. Vaginal septa are most commonly associated with this type but can be seen in the other anomalies.
Uterus, müllerian duct abnormalities. Didelphys uterus. Complete separation and full development of both müllerian ducts is noted. (a) Two vaginas and 2 cervices; (b) 2 distinct cervices; (c) 2 uterine horns are widely splayed; (d) cross section of uterine bodies and cervices.
Class IV (bicornuate uterus): Two uterine cavities are seen with normal endometrium (see Image below and Image 7 in Multimedia). The most important imaging finding is a concave fundus with a fundal cleft greater than 1 cm. This has been shown to be a reliable means of distinguishing bicornuate uterus from septate uterus. An increased intercornual distance (>4 cm) may be observed. The cleft is visualized best on oblique coronal images in the plane of the long axis of the uterus. The tissue separating the 2 horns demonstrates signal intensity identical to myometrium on all pulse sequences. The inferior portion of the septum (extending for a variable length inferiorly) may be fibrous, with low T1 and T2 signal intensity.
Class V (septate uterus): The outer fundal contour is convex, flattened, or mildly concave (fundal cleft <1 cm; see Images below and Images 4-6 in Multimedia). The intercornual distance is usually normal (<4 cm), and each uterine cavity is usually small. The septum may be composed of muscle or fibrous tissue and is not a reliable means of distinguishing septate uterus from bicornuate uterus. A more reliable means for differentiating the 2 is to examine the fundal contour (see class IV). Differentiation between a septate and a bicornuate uterus is important because septate uteri are treated with transvaginal hysteroscopic resection of the septum, whereas if surgery is possible or indicated for the bicornuate uterus, an abdominal approach is required for metroplasty.
Uterus, müllerian duct abnormalities. T2 fast spin-echo MRI image of septate uterus acquired in the oblique plane along the long axis of the uterus. Note that the outer fundal contour (superior border) is flat or slightly concave, which is sufficient to make the diagnosis of septate uterus.
Uterus, müllerian duct abnormalities. Septate uterus. Note that a longer septum divides the uterine cavity. Outer fundal contour is flat.
Uterus, müllerian duct abnormalities. MRI image of septate uterus. The patient has a thin, fibrous septum that cannot be resolved distally at the fundus. More importantly, the outer fundal contour remains convex, thus excluding a bicornuate uterus.
Class VI (arcuate uterus): MRI may detect this abnormality, but typically, it is not clinically significant because arcuate uterus has no significant negative effects on pregnancy outcome.
Class VII (DES related): MRI may detect this abnormality as a hypoplastic uterus. Typically, the DES-related anomaly is diagnosed confidently using HSG (see Radiograph).
Degree of Confidence
MRI has consistently demonstrated high sensitivity and specificity for evaluation of uterine anomalies. Pellerito et al found MRI capable of helping correctly diagnose 24 of 24 anomalies (100% accuracy), compared to 11 of 12 anomalies (92%) detected on endovaginal sonography (EVS).29 For anomalies requiring surgery (unicornuate or bicornuate uteri), MRI demonstrated 100% sensitivity and specificity, compared to 67% sensitivity and 100% specificity for EVS. For nonsurgical lesions, both MRI and EVS had 100% sensitivity and specificity.
Pellerito et al also noted that MRI had the added advantage of detecting other incidental abnormalities, including a dermoid and submucosal leiomyoma, found on EVS to be indeterminate and nonvisualized, respectively.29
False Positives/Negatives
Data suggest very low false-negative and false-positive rates.
Ultrasonography
Findings
Examination protocol
Most commonly, 2D EVS is used to help evaluate uterine anatomy. Transabdominal 2D imaging may be performed, ideally through a distended bladder, but offers reduced sensitivity and specificity because of increased distance from the uterus and, often, intervening bowel. Preliminary studies indicate that 3D techniques in sonography may offer improved sensitivity and specificity for detection of uterine anomalies. Image quality for all techniques is highly operator dependent.
US appearance of müllerian anomalies (AFS classification system)
- Class I (hypoplasia/agenesis): Findings of agenesis include absence of the cervix and/or uterus with a blind-ending vagina. In uterine agenesis, no identifiable uterine tissue is present. Partial agenesis of müllerian duct derivatives also can be observed. In uterine hypoplasia, the endometrial cavity is small with a reduced intercornual distance (<2 cm).
- Class II (unicornuate uterus): The unicornuate uterus appears banana shaped without the usual rounded fundal contour and triangular appearance of the fundal cavity. Uterine zonal anatomy is normal. If present, a rudimentary horn can be observed as a soft-tissue mass with echogenicity to that of myometrium. If obstructed, a rudimentary horn with functioning endometrium may present as a complex hemorrhagic cystic structure.
- Class III (didelphys uterus): Two separate normal-sized uteri and cervices are observed. A vaginal septum may be difficult to visualize. The 2 uterine horns are usually widely splayed, and endometrial and myometrial zonal widths are preserved.
- Class IV (bicornuate uterus): US may demonstrate 2 uterine cavities with normal endometrium. The most important imaging finding is a concave fundus with a fundal cleft greater than 1 cm. This has been shown to be a reliable means of distinguishing bicornuate from septate uteri. 3D US may play a useful role in making this diagnosis but, as of yet, is not widely available. An increased intercornual distance (>4 cm) may be observed. The cleft is visualized best on oblique coronal images in the plane of the long axis of the uterus. The septum separating the 2 horns demonstrates echogenicity identical to that of myometrium. The inferior portion of the septum (extending for a variable length inferiorly) may be fibrous.
- Class V (septate uterus): US may demonstrate a convex or flattened fundal contour. The intercornual distance usually is normal or decreased (<4 cm), and each uterine cavity is usually small. The septum may be composed of muscle or fibrous tissue and is not a reliable means of distinguishing septate from bicornuate uteri. A more reliable means to differentiate the 2 involves examining the fundal contour (see class IV). Confirm ambiguous US findings using MRI.
- Class VI (arcuate uterus): US may detect the abnormality, but typically, it is not clinically significant because arcuate uterus has no significant negative effects on pregnancy outcome.
- Class VII (DES related): US may detect the abnormality as uterine hypoplasia. Typically, DES-related uterus is diagnosed confidently using HSG.
Degree of Confidence
Some studies have found that 3D US is highly sensitive (up to 100%) and specific (up to 100%) in helping diagnose major müllerian anomalies.30 Studies have also found 2D transvaginal sonography to be a highly effective means of diagnosis, with 75-100% sensitivity and up to 95% specificity. Positive predictive value was higher with 3D scanning than with 2D scanning (100% vs 50%, respectively).
Nicolini et al found that transabdominal 2D US failed to visualize the uterine cavity adequately in as many as 35% of patients although it adequately imaged the uterine fundus in 90% of patients.31
False Positives/Negatives
In a study by Raga et al, 3D US detected 12 of 12 congenital uterine anomalies and correctly classified the anomalies according to AFS class in 11 of 12 patients. One false-negative result involved bicornuate uterus misdiagnosed as septate uterus because of a leiomyoma that caused the fundal contour to appear convex.32
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 Imaging: Uterus, Mullerian Duct Abnormalities |
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Further Reading
Related eMedicine topics
Mullerian Duct Anomalies
Malposition of the Uterus
Surgical Management of Mullerian Duct Anomalies
Infertility
Intrauterine Growth Retardation
Keywords
müllerian duct abnormalities of the uterus, uterine, female reproductive tract, müllerian congenital uterine anomalies, müllerian anomalies, müllerian duct anomalies, Mayer-Rokitansky-Kuster-Hauser syndrome, female reproductive tract developmental abnormalities, paramesonephric developmental abnormalities
