|Year : 2018 | Volume
| Issue : 2 | Page : 194-201
Imaging of Müllerian duct anomalies
Ahmed F Yousef1, Hamada M Khater1, Ibrahim A Hussein2
1 Department of Radiology, Faculty of Medicine, Benha University, Benha, Egypt
2 Department of Radiology, Faculty of Medicine, Al-Anbar University, Ramadi, Iraq
|Date of Submission||05-Sep-2017|
|Date of Acceptance||10-Oct-2017|
|Date of Web Publication||17-Aug-2018|
Dr. Ibrahim A Hussein
Al-Anbar University, Ramadi, 31002
Source of Support: None, Conflict of Interest: None
Background Müllerian duct anomalies (MDAs) are relatively common disorders, although the actual incidence and prevalence of these disorders are difficult to assess.
Aim This work aimed to survey the role of imaging in determining the location and order of peculiarities of Müllerian conduits by various picture modalities.
Patients and methods The study included 37 patients with primary amenorrhea with suspected MDAs on the basis of a preliminary ultrasound study. Patients with primary infertility, secondary infertility, or recurrent abortions were investigated for possible MDA or patients with cyclic abdominal pain referred from the Gynecology Department to the private Radiology Department in Benha and Cairo City.
Results The study included 37 patients. All patients underwent pelvic ultrasound, hysterosalpingography, and pelvic MRI. Patients were further divided according to their final diagnosis by hystrosalpingography pelvic ultrasonography and pelvic MRI, which was correlated with clinical characteristics.
Conclusion MRI in our study showed excellent diagnosis performance compared with hysterosalpingography and ultrasound.
Recommendations Clinicians should be suspicious for Müllerian anomalies in cases of primary amenorrhea, pelvic pain, and certain adverse obstetrical outcomes.
Keywords: imaging, Müllerian duct anomalies, peculiarities
|How to cite this article:|
Yousef AF, Khater HM, Hussein IA. Imaging of Müllerian duct anomalies. Benha Med J 2018;35:194-201
| Introduction|| |
Developmental anomalies of the Müllerian duct system represent some of the most complex disorders that obstetricians and gynecologists encounter. Müllerian ducts are the primordial anlage of the female reproductive tract. They differentiate to form the Fallopian tube More Detailss, the uterus, the uterine cervix, and the superior aspect of the vagina. A wide variety of malformations can occur when this system is disrupted. They range from uterine and vaginal agenesis to duplication of the uterus and the vagina to minor uterine cavity abnormalities .
Patients suspected of having Müllerian duct anomaly (MDA) are often initially referred for pelvic ultrasonography (US). Ultrasound is quick, readily available, economical, and without risk of radiation. However, image degradation can occur in obese patients and in patients with overlying bowel gas, and the external contour may be difficult to visualize. MRI is currently considered the best imaging modality for MDA. It does not carry any risk of radiation and provides clear delineation of both the internal and the external uterine anatomy. MRI has been shown to have excellent agreement with the clinical diagnosis of the subtypes of MDA .
Although US remains the modality of choice for the initial study of patients who are suspected of having MDAs, in agreement with many authors in the literature, we should reserve MRI for patients with a technically inadequate or indeterminate ultrasound examination .
MRI should be considered an adjunct to transabdominal ultrasound to evaluate Müllerian anomalies .
MRI can be considered the imaging standard of reference because it is noninvasive, it does not carry any risk of ionizing radiation, has multiplanar capability, enables excellent soft tissue characterization, and has a greater field of interrogation than does US . It provides high-resolution images of the uterine body, fundus, and internal structure. In addition, it can enable evaluation of the urinary tract for concomitant anomalies .
Abnormalities in the external uterine fundal contour can be identified easily with MRI, allowing for clear differentiation between a fusion anomaly, such as a uterus didelphys or a bicornulate uterus, and a resorption anomaly, such as a septate uterus. Furthermore, MRI provides a clear depiction of a rudimentary uterine horn in a unicornuate uterus. An accurate differential diagnosis of MDAs on the basis of their characteristic MRI findings is crucial because the rates of gynecologic and obstetric complications vary considerably among MDAs .
Thus, the use of MRI for the evaluation of MDAs reduces the number of invasive procedures and related costs by guiding management decisions .
Congenital malformations of the female genital tract consist of a group of miscellaneous deviations from the normal anatomy. Although certain types of congenital malformations are a result of a clear disturbance in one stage of embryologic development, others are a result of disturbances in more than one stage of normal formation .
| Aim|| |
This work aimed to survey the part of imaging in terms of the location and order of peculiarities of Müllerian conduits by various picture modalities.
| Patients and methods|| |
The study included 37 patients. Their ages ranged from 15 to 40 years (mean age: 21.78 years). The study was approved by Scientific Research Committee of Benha University An informed consent is taken from all participants.
The patients were referred from the Gynecology Department to the private Radiology Department in Benha and Cairo City:
- Patients with suspected MDAs on the basis of a preliminary ultrasound study.
- Patients with primary amenorrhea, primary infertility, secondary infertility, or recurrent abortions investigated for possible MDA.
- Patients with cyclic abdominal pain.
- Patients contraindicated for MRI, e.g. cardiac pacemaker, aneurismal clips, bone growth stimulators, electrically programmed infusion pump, bullets and sharpnels, orthopedic implants and devices, intravascular stents, coils, and filters.
- Overweight patients, weighing more than 150 kg (cannot fit in the MRI machine).
- Patients with claustrophobia.
All patients were subjected to the following:
- Demographic and clinical data collection including patient’s name, age, marital status, complaint, and duration of illness.
- Imaging procedure: all patients underwent pelvic ultrasound (transabdominal and/or transvaginal), hysterosalpingography (HSG), and pelvic MRI.
| Methods|| |
- Pelvic ultrasound technique for MDA:
- All patients underwent a preliminary pelvic ultrasound examination. The examination was performed on ultrasound machine GE logic 7 (GE Healthcare, Chicago, USA).
- Transabdominal examination was performed through a moderately full bladder, which lifted gassy loops of the bowel out of the way and provided an acoustic window through which pelvic organs were visualized. We performed the examination using a 3–4 MHz transducer; it was placed on the abdomen using a conductive gel.
- Transvaginal ultrasound was performed by the introduction of a 7–8 MHz probe into the vagina. The bladder was empty.
- Sagittal images were obtained by scanning in the same plane as the uterus parallel to its long axis.
- No specific patient preparation is required for HSG.
- The examination should be scheduled during days 7–12 of the menstrual cycle (day 1 being the first day of menstrual bleeding).
- Using an aseptic technique, a speculum is used to distend the vagina and an 8 F Foley catheter is inserted into the uterine cavity. A diluted, water-soluble, hyperosmolar iodinated contrast agent is then hand injected into the uterine cavity through the Foley catheter.
- MRI technique for MDA:
- 1.5 T magnets were used. All patients were imaged in the supine position.
- Patients were instructed to have a full bladder before examination.
- Also, before the examination, patients were questioned routinely about any contraindications for MRI examination and instructed to put off any metal objects.
- An inversion-recovery image of the uterus in the sagittal plane was obtained initially to determine uterine lie. Fast spin echo T2-weighted images were then acquired parallel to the long axis of the uterus to characterize the external uterine contour in the coronal plane depending on uterine lie.
- For the purpose of MDA classification, oblique coronal T2-weighted images of the uterus were the most critical as these were necessary for proper assessment of the uterine fundal contour ([Table 1]).
- Finally, a coronal fast spoiled gradient-echo image or a single-shat fast spin echo T2-weighted image was obtained using a body coil with a large field of view to enable assessment of the kidney.
The following was assessed in MRI: uterus, vagina, gonads, and associated pelvic lesions and renal anomalies ([Figure 1] and [Figure 2]).
|Figure 1 Sagittal-weighted MRI show normal bladder and rectum, but absence of the uterus and proximal vagina.|
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|Figure 2 Coronal and sagittal-weighted MRI of the same case and diagnosed as agenesis of the uterus.|
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- Size: uterine length was measured; it normally measures 6–9 cm and the uterine body to cervical ratio is 2 : 1 when measured in the sagittal plane.
- Intercornual distance: the distance between the distal ends of the horns was measured in the oblique long-axis images and it is normally 2–4 cm.
- External fundal contour: the external contour of the uterus was normally convex and was best detected in long-axis oblique images.
- Zonal anatomy: zonal anatomy is the differentiation between the high signal intensity endometrium, the low-intensity junctional zone (inner myometrium), and the intermediate intensity outer myometrium as depicted in T2-weighted images. It is normally observed in the reproductive age group.
- Uterine septum: a uterine septum sought; if present, its signal intensity and extent are assessed.
- Intercornual angle: the intercornual angle is the angle between the most medial aspects of the two uterine hemi cavities.
- Obstruction: distended blood-filled uterus (hematometra) and blood-filled fallopian tubes (hematosalinx) show the characteristic signal pattern of altered blood and blood products. The level of obstruction was determined.
The vagina was normally seen as a tube of intermediate signal intensity between the bladder base and the urethra anteriorly and the anal canal posteriorly. The direction and extent of a vaginal septum − if present − were assessed. The obstruction site of a blood-filled vagina (hematocolpos) was estimated.
Both ovaries were identified. Any associated lesions such as endometriosis or teratoma were noted.
Associated pelvic lesions or renal anomalies
Any associated pelvic lesions or renal anomalies were reported.
| Results|| |
The study included 37 patients. All patients underwent pelvic ultrasound, HSG, and pelvic MRI.
The ages of the patients ranged from 15 to 40 years, with a mean age of 21.78±4.6 years. This is shown in [Table 2].
Cases were classified according to the clinical presentation as shown in [Table 3]:
- 16/37 (43%) cases presented with amenorrhea.
- 18/37 (49%) cases presented with secondary infertility.
- 3/37 (8%) cases presented with cyclic pain.
Cases were classified according to modified American Fertility Score (modified Rock and Adams classification) as shown in [Table 4]:
- 8/37 (43%) cases were classified as class I.
- 2/37 (5%) cases were classified as class II.
- 17/37 (46%) cases were classified as class III.
- 5/37 (13.5%) cases were classified as class IV.
- 5/37 (13.5%) cases did not have MDA.
|Table 4 Distribution of cases according to the modified Rock and Adams classification|
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Cases were further divided according to their final diagnosis by hystrosalpingography pelvic US and pelvic MRI, which was correlated with clinical characteristics.
According to imaging findings, class I included 12 cases, eight true cases and four false cases, as shown in [Table 5].
- 6/12 (50%) cases had pure Mayer–Rokitansky–Küster–Hauser (MRKH).
- 2/12 (17%) cases had MRKH with rudimentary horns.
- 4/12 (33%) cases did not have MDA (androgen insensitivity syndrome).
- 10/12 (84%) cases were diagnosed correctly on ultrasound.
- 12/12 (100%) cases were diagnosed correctly on MRI.
|Table 5 Distribution of cases within class I according to their final diagnosis|
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Included 2/37 (5%) cases: one case of vaginal and another case of cervical atresia.
Both US and MRI showed 100% sensitivity and specificity in the diagnosis of the presence and level of obstruction.
According to imaging findings, class III included 18 cases: 17 true cases and one false case.
- 17/18 (94.5%) cases were class III MDA.
- 1/18 (5.5%) case did not have MDA (misdiagnosed as having double uterine cavities; however, pelvic MRI showed a single uterine cavity and the false cavity was thick-walled endometrioma).
- 12/18 (67%) cases were diagnosed correctly on ultrasound.
- 18/18 (100%) cases were diagnosed correctly on MRI.
This final diagnosis of class III as shown in [Table 6].
- 4/17 (24%) cases had unicornuate uterus.
- 5/17 (29%) cases had bicornuate uterus.
- 7/17 (41%) cases had septate uterus.
- 1/17 (6%) case had didelphys uterus.
|Table 6 Distribution of cases within class III according to their final diagnosis|
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According to imaging findings, 5/32 (15.6%) cases were classified as class IV.
- 3/5 (60%) cases were diagnosed with a didelphys uterus with obstructed hemivaginal and absent kidney on the obstructed side.
- 1/5 (20%) case was diagnosed with a septate uterus with pelvic right kidney.
- 1/5 (20%) case was diagnosed with MRKH with a left rudimentary horn and absent right kidney.
- Both US and MRI showed 100% sensitivity and specificity in diagnosis.
Comparison of the overall diagnostic indices of ultrasound and MRI:
- 29/37 (78.3%) cases were diagnosed correctly by US.
- 37/37 (100%) cases were diagnosed correctly by MRI.
| Discussion|| |
MDAs represent a wide spectrum of developmental abnormalities related to various gynecologic and obstetric complications, including primary amenorrhea, infertility, and endometriosis. The use of diverse imaging modalities, in conjunction with clinical information, provides important clues for the diagnosis of MDAs .
A wide variety of malformations can occur when this system is disrupted. They range from uterine and vaginal agenesis to duplication of the uterus and vagina to minor uterine cavity abnormalities .
Uterine abnormalities are frequently unrecognized at birth and are unreported. However, during childbearing years, when reproductive malfunctions occur, they are over reported. Other factors that affect reporting of irregularities in the incidence and prevalence rates include non standardization of classification systems, nonuniform diagnostic modalities, and different study populations of women .
In women with fertility problems, the incidence of MDAs is slightly higher at 3–6%. The most commonly reported MDAs are septate, arcuate, didelphys, unicornuate, or hypoplastic uteri. The exact distribution depends on the study and on the geographic location .
Grimbizis et al.  reported that the mean incidence of uterine malformations was 4.3% for the general population and/or for fertile women. This rate was determined by reviewing data compiled from five studies that included ∼3000 women with uterine malformations.
Grimbizis et al.  also reported on prevalence, which was about 4.3% for the general population and/or fertile women, about 3.5% in infertile women, and about 13% in women with recurrent pregnancy losses.
MDA are uncommon, but are a treatable form of infertility. Patients with MDA are known to have higher incidences of infertility and repeated first-trimester spontaneous abortions. The role of imaging is to detect and classify these MDA so that appropriate treatment can be provided .
The role of imaging is to help detect, diagnose, and distinguish surgically correctable forms of MDAs from inoperable forms. In some correctable lesions, the surgical approach is altered on the basis of imaging findings .
Diagnostic imaging work-up for MDAs often begins with HSG and/or US. Although HSG and/or US may be sufficient to detect the presence of a uterine abnormality, MRI is generally needed to classify the abnormality into a specific MDA category .
MRI has become increasingly popular for use in evaluating MDAs because of its noninvasiveness, lack of ionizing radiation, and capability for multiplanar imaging and soft tissue characterization .
Abnormalities in the external uterine fundal contour are readily recognized with MRI, allowing for clear differentiation between a fusion anomaly, such as a uterus didelphys or a bicornulate uterus, and a resorption anomaly, such as a septate uterus .
This study included 37 cases; 32/37 (86.5%) patients had actual MDA and 5/37 (13.5%) received a false diagnosis on the basis of clinical and ultrasound findings.
The age of presentation of the cases ranged from 15 to 40 years, with a mean age of 21.78±4.6 years.
Out of the 37 cases, 16/37 (43%) cases presented with primary amenorrhea, normal external genitalia, and age appropriate developmental milestones, 18/37 (49%) cases presented with secondary infertility, and 3/37 (8%) cases presented with cyclic pain. All cases underwent pelvic US and MRI.
Lalwani et al.  reported that most MDAs are associated with functioning ovaries and age appropriate external genitalia. These abnormalities are often recognized after the onset of puberty.
In the prepubertal period, normal external genitalia and age appropriate developmental milestones often mask abnormalities of the internal reproductive organs. After the onset of puberty, young women often present to the gynecologist with menstrual disorders. Late presentations include infertility and obstetric complications .
The 32/37 patients diagnosed with MDA were classified according to the Modified Rock and Adams (American Fertility Score) classification. Class I (uterine agenesis) included 8/32 (22%) cases, class II (cervical and vaginal atresia) included 2/32 (5%) cases, class III (septate, didelphys, unicornuate, and bicornulate uterus) included 17/32 (46%) cases, and class IV (combinations of defects) included 5/32 (13%) cases.
Out of the 32 cases diagnosed with MDA, 9/32 (28%) cases were diagnosed with MRKH and another 9/32 (28%) cases were diagnosed with septate uterus; these two conditions showed the highest incidence in our study.
Troiano and McCarthy  reported that a septate uterus is one of the most common MDAs. It accounts for ∼55% of MDAs.
Lalwani et al.  reported that complete Müllerian aplasia (MRKH syndrome) is the most common variant encountered and it is characterized by the congenital absence of the vagina and the uterus in 90–95% of cases. The fallopian tubes are normal and the ovaries have normal endocrine and oocyte functions.
MRHK is the second most common cause of primary amenorrhea in adolescents . US findings can confirm the clinical findings suggesting the absence of uterus and fallopian tubes in the presence of normal ovaries .
Class I included 12 cases: eight true cases and four false cases: 6/12 (50%) cases had pure MRKH with complete absence of the uterus and the cervix. A total of 2/12 (17%) cases had MRKH with rudimentary horns. Totally, 4/12 (33%) cases did not have MDA, and presented with primary amenorrhea misdiagnosed on ultrasound as MRKH. On MRI, undescended tests were identified on MRI and were further confirmed after revision of the hormonal profile (androgen insensitivity syndrome). Totally, 10/12 (84%) cases were diagnosed correctly on ultrasound. Totally, 12/12 (100%) cases were diagnosed correctly on MRI.
Lalwani et al.  reported that the hormonal profile is that of a normal female with age appropriate luteinizing hormone, follicle-stimulating hormone, estradiol, and testosterone levels. This profile helps distinguish the MRKH syndrome from androgen insensitivity syndromes in which postpubertal testosterone is elevated.
Brown and Badawy  reported that MRI is extremely useful; absence of the vagina and the uterus on a technically adequate image confirms the diagnosis of agenesis or hypoplasia. This modality can also depict a rudimentary uterus and any coexisting renal abnormalities .
Class III included 18 cases; 7/18 (39%) cases were septate, 4/18 (22.5%) cases unicornuate, and 1/18 (5.5%) case had a thick-walled endometrioma and was misdiagnosed as a double uterine cavity by pelvic US. A total of 12/18 (67%) cases were correctly diagnosed on ultrasound. A total of 18/18 (100%) cases were correctly diagnosed on MRI.
Out of 32 cases diagnosed with MDA, 4/32 (12.5%) cases were diagnosed with unicornuate uterus; 2/4 (50%) cases were diagnosed with a single uterine horn, and 2/4 (50%) cases were diagnosed with unicornuate uterus with a rudimentary horn. Some of these cases presented with cyclic pain and others presented with secondary infertility.Unicornuate uterus accounts for ∼2.4–13% of all Müllerian anomalies . In a retrospective longitudinal review, the incidence of unicornuate uterus was 0.06% for the examined population, which included more than 3000 women of reproductive age who desired conception .
The unicornuate uterus may occur on its own, but it is frequently associated with a rudimentary horn .
Women with noncommunicating, functioning rudimentary horns may present with pelvic pain usually secondary to hematometra or endometriosis .
Our study was compared with another prospective study  that was carried out on 22 female patients aged between 13 and 45 years, mean age 29 years; they were investigated by HSG, ultrasound, and MRI.
MRI yielded a correct diagnosis of 21 uterine anomalies, with accuracy more than 93% better than both HSG and ultrasound.
The MRI is a very accurate imaging modality, and provides a very accurate diagnosis of MDA that can enable planning of treatment .
Deutch and Abuhamad  reported that although US remains the modality of choice for the initial study of patients who are suspected of having MDAs, this study proposed that, in agreement with many authors in the literature, MRI should be reserved for patients with technically inadequate or indeterminate ultrasound examination.
Deutch and Abuhamad  also reported that MRI showed the highest sensitivity; thus, its use in cases with suspected MDA reduces false-negative results. Also, it enabled better assessment of combined anomalies than other imaging modalities.
Studies have also found two-dimensional transvaginal sonography to be a highly effective means of diagnosis, with 75–100% sensitivity and up to 95% specificity .
Pellerito et al.  found that MRI could enable correct diagnosis of 24 of 24 anomalies (100% accuracy) compared with 11 of 12 anomalies (92%) detected by endovaginal sonography (EVS). For anomalies requiring surgery (unicornuate or bicornulate uteri), MRI showed 100% sensitivity and specificity compared with 67% sensitivity and 100% specificity for EVS. For nonsurgical lesions, both MRI and EVS had 100% sensitivity and specificity.
This study assessed the role of MRI and ultrasound. US showed two false-positive and six false-negative results, whereas MRI showed no false-positive or false-negative results, thus leading to a more accurate diagnosis.
| Conclusion|| |
MRI proved to be very useful in the differentiation of uterine anomalies with its ability to provide information on the external and internal uterine anatomy and tissue composition; it represents a powerful tool in the diagnosis and classification of patients with MDAs, and thereby provides a strong basis for deciding on further treatment options.
MRI readily recognized abnormalities in the external uterine fundal contour, allowing for a clear differentiation between a fusion anomaly, such as a uterus didelphys or a bicornulate uterus, and a resorption anomaly, such as a septate uterus.
Furthermore, MRI enabled a clear depiction of a rudimentary uterine horn in a unicornuate uterus.
MRI in our study showed excellent diagnosis performance compared with HSG and ultrasound.
Clinicians should be suspicious for Müllerian anomalies in cases of primary amenorrhea, pelvic pain, and certain adverse obstetrical outcomes.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6]