Müllerian Duct Anomalies

Published on 10/03/2015 by admin

Filed under Obstetrics & Gynecology

Last modified 22/04/2025

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Müllerian Duct Anomalies

Synonyms/Description

Congenital uterine anomalies

Etiology

Congenital anomalies of uterine shape occur in 3% to 4% of all women. The prevalence in women with infertility and early miscarriage is up to 10%, and as high as 25% in those with midtrimester pregnancy losses. Patients with uterine shape abnormalities have an increased incidence of congenital renal anomalies such as unilateral renal agenesis. The American Society for Reproductive Medicine has developed the following classification of uterine anomalies.

Class I: Agenesis of the Uterus, Cervix, and/or Upper Vagina

Includes women with Mayer-Rokitansky-Küster-Hauser syndrome (incidence 1/5000). Patients with this condition are born with no uterus, cervix, or upper vagina.

Class II: Unicornuate Uterus (20% of Uterine Anomalies)

This anomaly results from a lack of or incomplete development of one of the two Müllerian tubercles. This gives rise to only one complete horn of the uterus with either total absence or hypoplasia of the contralateral horn (rudimentary horn). In 35% of cases, the unicornuate uterus is isolated, but most are associated with variable development of a contralateral rudimentary uterine horn. Thirty-three percent of women with a unicornuate uterus have a noncavitary rudimentary horn (without endometrium), whereas 32% have a rudimentary horn with endometrium present. This rudimentary horn may or may not communicate with the “normal” hemiuterus.
Unicornuate uteri are associated with an early pregnancy loss rate of 41% to 62%, especially when a rudimentary horn is present. They are also associated with preterm delivery.

Class III: Didelphic Uterus (5% of Uterine Anomalies)

These patients have a complete lack of fusion of the bilateral Müllerian ducts, resulting in two totally separate uterine horns, each with its own endometrial cavity and cervix. The vagina also contains a septum in 75% of cases. Uterine didelphys is associated with spontaneous miscarriage rates of 32% to 52% and premature birth rates of 20% to 45%.

Class IV: Bicornuate Uterus (10% of Uterine Anomalies)

Incomplete fusion of the two Müllerian ducts leads to a concave dip in the serosal surface of the uterine fundus as well as a division of the endometrial cavity into two horns that connect near the cervix. Bicornuate uteri are associated with spontaneous abortion rates of 28% to 35% and premature birth rates of 14% to 23%.

Class V: Septate Uterus (55% of Uterine Anomalies)

When there is absence of normal resorption of the uterine septum after the two Müllerian ducts have fused, the uterine cavity will be septate. The septum may be complete or partial (more common), but the serosal surface of the uterus remains normal. A uterine septum is associated with spontaneous abortion rates ranging from 26% to 94% and premature birth rates ranging from 9% to 33%.

Class VI: Arcuate Uterus

This is considered a normal variant. With a normal serosal surface, these uteri have a slight indentation of the fundal portion of the uterine cavity that measures less than 1 cm in depth.

Ultrasound Findings

Before 3-D ultrasound, 2-D ultrasound was the first imaging modality to suspect a Müllerian duct anomaly; however, MRI was needed to display the coronal view of the uterine cavity to make the correct diagnosis. Although the hysterosalpingogram accurately depicts the shape of the uterine cavity, it provides no information about the outer contour of the uterus. A hysterosalpingogram cannot distinguish between a septate and a bicornuate uterus.
Currently, 3-D ultrasound can easily provide a reconstructed coronal view of the uterus, demonstrating both the shape of the endometrial cavity and the outer serosal/myometrial contour of the uterus. MRI is no longer necessary to diagnose the vast majority of uterine malformations. The accuracy of both 3-D ultrasound and MRI for diagnosing specific uterine malformations is 90% to 95%.
Once a 3-D image of the coronal view of the uterine cavity is obtained, the anatomy of the uterus can be observed easily. The uterus is septate if the septum extends 10 mm or more into the cavity from the midcornual line and the serosal surface is normal. A septum that is shallow and extends less than 10 mm caudally defines an arcuate uterus. A bicornuate uterus has a serosal indentation extending 10 mm or more caudally from the normal serosal surface, thus creating two horns. The bicornuate uterus also has an obligatory septum or partial septum, called a subseptum. Patients with Müllerian duct anomalies may have two cervices (bicollis), which are visible using 3-D reconstruction of the cervix. A uterine didelphys has two completely separate uterine horns that are located at opposite sides of the pelvis. These two horns are typically hard to image simultaneously because of their distance from each other.

Differential Diagnosis

The 3-D coronal view of the uterus is very accurate, and there is usually no differential diagnosis when the image is adequate. It is sometimes difficult to decide whether the uterus is arcuate or partially septate, and admittedly the criteria are rather arbitrary, probably with some overlap clinically. The same is true for the borderline bicornuate uterus in which the serosal dip may not quite reach 10 mm.
The unicornuate uterus may have a bizarre-looking rudimentary horn, which can mimic a solid mass such as a fibroid. In such cases, the presence of the unicornuate anatomy of the uterus should suggest the correct diagnosis of a rudimentary horn.

Clinical Aspects and Recommendations

All types of uterine anomalies are associated with renal anomalies. Women with a unicornuate uterus have the highest prevalence of renal anomalies, occurring in approximately 40%. Although all uterine anomalies are associated with a multitude of reproductive complications, the only type amenable to definitive treatment is the septate uterus. Hysteroscopic resection of a uterine septum is a relatively successful procedure in expert hands. Removal of a uterine septum is not always necessary and should be based on the patient’s reproductive history and desires, the size of the septum, and consultation with experts in this area. Because all forms of uterine anomalies are associated with certain types of obstetric complications, these patients require differing degrees of surveillance during pregnancy. Consultation or management by maternal-fetal medicine experts should be considered.

Figures

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Figure M3-1 The normal uterine cavity seen using the 3-D coronal view. Note the triangular shape of the uterine cavity and the rounded outer myometrial wall or serosal surface of the uterus.

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Figure M3-2 Mayer-Rokitansky-Küster-Hauser syndrome. Note the absence of the uterus and cervix in the pelvis. The vaginal probe could only be inserted part of the way into the vagina. The rest of the vagina is not developed.

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Figure M3-3 A and B, Two different patients with arcuate uteri. B shows the proper way to measure the depth of the myometrial indentation. If the measurement is 10 mm or more, then it is a septum. The measurement was less than 10 mm, indicating an arcuate uterus.

 

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Figure M3-4 Bicornuate uterus seen in 2-D and 3-D in two different patients. The 2-D image is a transverse view through the fundus of the uterus, showing two islands of endometrium and indicating a Müllerian anomaly. The 3-D image shows the typical bicornuate uterus with a deep indentation at the fundus, dividing the uterus into two distinct horns. These horns merge in the lower uterine segment. Figures A and B are one patient and C and D are another.

 

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Figure M3-5 A and B, Uterus didelphys. Note that the two uterine horns are widely separated and take on the appearance of floppy rabbit ears on this 3-D coronal view.

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Figure M3-6 3-D coronal view of a typical partial septum or subseptum. The calipers demonstrate the method of measuring the depth of the septum.

 

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Figure M3-7 A to D, Four different patients with partially septate uteri. Note the very different widths and depths of the septae when comparing the appearance of these uteri. The smallest septum (A) measured 12 mm and has an appearance similar to the arcuate uterus in Figure M3-3.

 

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Figure M3-8 A partially septate or subseptate uterus. A is a 2-D transverse view, showing the typical two islands of endometrium consistent with a Müllerian anomaly. B shows the preoperative 3-D coronal view of the partial septum. C shows the postoperative result of the septal excision.

 

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Figure M3-9 Partially septate/subseptate uterus seen using 3-D and sonohysterography. A and B show the 3-D volume displaying the partial septum. C to E show the sonohysterogram images of the same septum (C is a parallel tomographic cut through the uterus). Note that the catheter is visible inside the left endometrial cavity.

 

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Figure M3-10 Unicornuate uterus. A shows a unicornuate uterus seen with 2-D ultrasound. The anomaly is very hard to detect on this view. B and C show the single uterine horn using 3-D ultrasound. The 3-D rendered coronal view makes the anomaly obvious.

 

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Figure M3-11 A and B, Unicornuate uterus with a noncommunicating rudimentary horn. A, The 2-D image suggests a septate uterus; however, the 3-D coronal view (B) demonstrates the rudimentary horn with an island of endometrium, which does not connect with the rest of the cavity.

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Figure M3-12 Unicornuate uterus with a small rudimentary horn. Note that the rudimentary horn contains a small cavity that connects to the rest of the uterus but is tiny.

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Figure M3-13 Obstructed hemivagina. This patient had a didelphic uterus, and there was a fluid collection in the vaginal region. This is a view from the perineum looking up into the vagina. Note the fluid collection (arrows), indicating an obstructed hemivagina.

 

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Figure M3-14 Complete septate uterus with an early pregnancy in the left horn

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Figure M3-15 Shallow partial septum/subseptum in a uterus that also contains a set of twins in the right horn.

 

Suggested Reading

Deutch T.D., Abuhamad A.Z. The role of 3-dimensional ultrasonography and magnetic resonance imaging in the diagnosis of Müllerian duct anomalies: a review of the literature. J Ultrasound Med. 2008;27:413–423.

Faivre E., Fernandez H., Deffieux X., Gervaise A., Frydman R., Levaillant J.M. Accuracy of three-dimensional ultrasonography in differential diagnosis of septate and bicornuate uterus compared with office hysteroscopy and pelvic magnetic resonance imaging. J Minim Invasive Gynecol. 2012;19:101–106.

Ghi T., Casadio P., Kuleva M., Perrone A.M., Savelli L., Giunchi S., Meriggiola M.C., Gubbini G., Pilu G., Pelusi C., Pelusi G. Accuracy of three-dimensional ultrasound in diagnosis and classification of congenital uterine anomalies. Fertil Steril. 2009;92:808–813.

Homer H.A., Li T.C., Cooke I.D. The septate uterus: a review of management and reproductive outcome. Fertil Steril. 2000;73:1–14.

Khati N.J., Frazier A.A., Brindle K.A. The unicornuate uterus and its variants: clinical presentation, imaging findings, and associated complications. J Ultrasound Med. 2012;31:319–331.

Troiano R.N., McCarthy S.M. Müllerian duct anomalies: imaging and clinical issues. Radiology. 2004;233:19–34.

Woelfer B., Salim R., Banerjee S., Elson J., Regan L., Jurkovic D. Reproductive outcomes in women with congenital uterine anomalies detected by three-dimensional ultrasound screening. Obstet Gynecol. 2001;98:1099–1103.