6. Müllerian duct anomalies are often first detected
on hysterosalpingography (HSG) during the
work-up of infertility.
HSG has a limited role in the diagnosis of MDA's
as it only gives information about the uterine
cavity and not about the external contour of the
uterus.
The next step in the diagnosis is often ultrasound
or MRI.
HSG showing abnormal uterine cavity. Differentiation
between septate and bicorporeal uterus is not possible
HYSTEROSALPINGOGRAPHY
7. • Transabdominal and transvaginal ultrasound are often the first imaging
modalities used to evaluate the internal sex organs.
When indeterminate or complex, MR imaging is used.
The transabdominal ultrasound shows a uterus with normal external
contour of the fundus.
• MRI is considered the gold standard in the classification of MDAs due
to the detailed anatomic information provided.
ULTRASOUND
MRI
8. Early developmental failure of the Mullerian ducts at around 5 weeks gestation, results in
various degrees of agenesis or hypoplasia of the uterus, cervix and upper two-thirds of
the vagina.genesis
The Mayer–Rokitansky–Kuster–Hauser syndrome consists of a combined agenesis of the
uterus, cervix and upper portion of the vagina.
Symptoms may manifest at puberty as primary amenorrhea with normal secondary sexual
characteristics, as ovarian function is preserved.
CLASS I — AGENESIS/HYPOPLASIA
9. This anomaly results from complete or near-complete arrested development of one of
the Mullerian ducts
Four possible subtypes can develop:
(i) absent rudimentary horn
(ii) non-cavitary (non-functioning rudimentary horn
(iii) cavitary communicating rudimentary horn
(iv) cavitary non-communicating rudimentary horn
CLASS II — UNICORNUATE
10.
11.
12.
13. • This anomaly results from complete non-fusion of both Mullerian ducts.
• The individual horns are fully developed and almost normal in size.
• A deep fundal cleft and two cervices are present.
• A longitudinal or transverse vaginal septum may be present.
CLASS III — DIDELPHYS
14.
15.
16.
17.
18. • This class is characterized by partial non-fusion of the Mullerian ducts .
• This results in a central myometrium that may extend to the level of the internal cervical
os (bicornuate unicollis) or external os (bicornuate bicollis), with a fundal cleft 1 cm deep.
• The horns of the bicornuate uteri are not as fully developed and are smaller than those in
the didelphys uteri.
CLASS IV — BICORNUATE
19.
20.
21. This class of anomaly occurs when the final fibrous septum between the two Mullerian
ducts fails to resorb.
This results in the formation of a uterus that is completely or partially divided into two
cavities .
This class is associated with the poorest obstetrical outcomes . The fundal cleft is ,1 cm
deep and the intercornual distance is usually 4 cm.
The morphology of the outer fundal contour is the key to the diagnosis.
CLASS V — SEPTATE
22.
23. • Characterized by mild indentation of the endometrium at the uterine fundus
• It is the result of near complete resorption of the uterovaginal septum.
• Currently, no definitive depth has been established to differentiate the arcuate
configuration from the septate, it is generally thought that an arcuate uterus is compatible
with normal pregnancy and delivery.
CLASS VI — ARCUATE
24.
25. Several million women were treated with diethylstilbestrol (DES), a non-
steroidal estrogen, to prevent miscarriage between 1945 and 1970 .
The drug was promptly removed from the market when it was found that up
to 15% of newborn girls who were exposed to DES had uterine malformations
and an increased risk of vaginal clear cell carcinoma .
The uterine abnormalities include hypoplasia and a T-shaped uterine cavity .
Patients may also have abnormal transverse ridges, hoods and stenosis of the
cervix.
CLASS VII — DIETHYLSTILBESTROL RELATED
26.
27. • Septate uterus has a normal fundal contour but is characterized by a persistent
longitudinal septum that partially divides the uterine cavity The external uterine
fundal contour may be convex, flat, or mildly (< 1 cm) concave. Bicournuate
uterus has a deep > 1 cm fundal cleft in the outer uterine contour and
intercornual distance >4 cm.
• Septate uterus has acute angle <75° between uterine cavities while an angle
of more than 105° is more consistent with a bicornuate uterus.
Septate vs bicornuate
28. SUMMARY OF THE THERAPEUTIC INTERVENTIONS PERFORMED FOR THE DIFFERENT CLASSES
OF MULLERIAN DUCT ANOMALIES : CLASS TREATMENT
I – Hypoplasia/agenesis No reproductive potential; medical intervention in the form of in vitro fertilisation
of harvested ova and implantation in a host uterus needed
II – Unicornuate Non-communicating, cavitary horn Always surgically resected, as it is associated with
dysmenorrhoea, haematometra, endometriosis and ectopic pregnancy
Non-communicating, non-cavitary horn Surgery not currently recommended. No complications of
endometriosis etc, as there is no endometrium
Communicating, cavitary horn Also surgically removed because pregnancy that implants in the rudimentary
horn rarely is viable . No horn No treatment. Reproductive potential is possible
III – Didelphys May consider metroplasty; however, full-term pregnancies have occurred
IV – Bicornuate Surgical intervention rarely needed; may consider metroplasty
V – Septate Often treated with transvaginal hysteroscopic resection of the septum. Conception is
possible 2 months after surgery
30. Presentation
Patient with primary infertility, presents with severe right
adnexal tenderness and pelvic pain.
Patient Data
Age: 30 years
Gender: Female
The female reproductive tract develops from a pair of Müllerian ducts that form the fallopian tubes, uterus, cervix and the upper two-thirds of the vagina.The ovaries and lower third of the vagina have a different embryological origin (genital ridge and urogenital sinus, respectively).
First there is formation of the paired Müllerian ducts, followed by fusion of the two ducts into a single uterus, cervix and upper vagina.
Finally resorption of the septum will lead to a normal cavum.Failure of formation of the Müllerian ducts can result in an aplastic or hemi-uterus.Failure or incomplete fusion of the ducts can result in a bicorporeal uterus.
Non or incomplete septal resorption results in a septate uterus.
The table shows the European classification system ESHRE/ESGE from 2013.
Class U0 is a normal uterus
Class U1 is a dysmorphic shaped uterus either as a T-shaped cavum due to abnormally thick uterine walls or as a T-shaped cavum due to an abnormal outer contour (infantilis).
Class U2 is the result of failure of resorption of the septum. There is an internal indentation. The outer contour of the uterus is normal and this differentiates the septate uterus from the bicorporeal uterus.
Class U3 is a bicorporeal uterus with a left and right corpus as a result of failure of fusion. The outer contour is abnormal with an external cleft of the fundus. A bicorporeal septate uterus has both an external cleft and a septum.
Class U4 is a hemi-uterus as a result of unilateral failure of formation of the Müllerian duct.
Class U5 is an aplastic uterus as a result of bilateral failure of formation of the Müllerian ducts.
Class U6 are unclassified cases
. The ovaries
originate within the primitive ectoderm and not from
the paramesonephros, and thus these patients have
normal female sexual development. In uterine hypoplasia,
a small but fully differentiated organ is present.
The last one may obstruct and present
with abdominal pain, subsequently requiring surgical
intervention.
Unicornuate uterus with no rudimentary horn. (a) Illustration shows a right unicornuate uterus with no rudimentary horn. (b) HSG image shows a small, oblong uterine cavity (*) deviated to the right of midline with a single fallopian tube (arrowhead). (c) Axial T2-weighted MR image shows a single uterine horn (*) and cervix (arrowhead). (d) Coronal T2-weighted MR image shows absence of soft tissue adjacent to the right unicornuate cervix (arrowhead), a finding indicating absence of a rudimentary horn. (Fig 7a courtesy of Joanna Culley, BA.)
Unicornuate uterus with an obstructed noncommunicating rudimentary horn. Axial T2- weighted MR images show a normal-appearing left unicornuate uterus (arrow in a) and an obstructed noncommunicating right rudimentary horn with layering debris (* in b).
Ultrasound of Unicornuate Uterus with Rudimentary Horn.
• Images :
1- Abdominal Axial section.
2 - TVS Sagittal section.
3- TVS Axial section.
Uterine Duplication Anomaly !
• HOW to reach the main type ?
* Two separated uterine horns (may be didelphys, bicornuate or unicornuate).
* Asymmetric endometrial stripe - Asymmetric horn sizes (so it is unicornuate).
• HOW to reach the sub-type ?
* The Rudimentary horn (left side) has endometrial stripe (so it is functioning / cavitary).
* Although definite communication between the rudimentary horn and the cervix could note be determined on usg but there is NO obstruction / NO retained fluid, so it is most likely communicating yet confirmation is needed by MRI
.
Image showing two separate endocervical canals that open into separate fusiform endometrial cavities, with no communication between the two horns. Each endometrial cavity ends in a solitary fallopian tube.
ReplyForward
Figure 12. Transverse transabdominal US image shows a uterus didelphys, with two uterine horns (u) separated by echogenic fat (*). There is a viable embryo (arrow) in the left uterine horn.
Uterus didelphys. (a) Illustration shows a uterus didelphys with a left transverse vaginal septum. (b) Coronal T2-weighted image of a uterus didelphys, obtained in plane with the uterus, shows two widely divergent uterine horns (arrows) separated by a deep fundal cleft. Two separate cervices are present (arrowheads). (c) Axial image caudal to b shows two vaginas (arrows). Fig 9a courtesy of Joanna Culley, BA.)
Axial spoiled gradient-echo fat-saturated T1-weighted MR image shows hyperintense blood products within an obstructed horn of a uterus didelphys (*). The hyperintense blood products decompress through the fallopian tube (arrows) into a large hematosalpinx (H).
Uterus didelphys with an obstructed hemivagina. (a) Coronal single-shot fast spin-echo T2-weighted MR image shows widely separated horns of a uterus didelphys (white arrows). Two hemivaginas are present (arrowheads). Note the absent left kidney (black arrow) with bowel in the renal fossa, which is ipsilateral to the obstructed hemivagina. k = normal right kidney. (b) Axial T2-weighted image shows the two hemivaginas (arrowheads); the obstructed, dilated left hemivagina contains heterogeneous debris (*).
Figure 2. Classification criteria for US differentiation of bicornuate from septate uteri. (a) When the apex of the fundal contour is more than 5 mm (arrow) above a line drawn between the tubal ostia, the uterus is septate. (b, c) When the apex of the fundal contour is below (arrow in b) or less than 5 mm above (arrow in c) a line drawn between the tubal ostia, the uterus is bicornuate. (Figs 2a–2c courtesy of Joanna Culley, BA.)
Figure 13. Bicornuate uterus. (a) Illustration shows a bicornuate unicollis uterus. (b) HSG image shows a bicornuate bicollis uterus with two HSG cannulas due to two cervices. There is a fundal linear defect (arrow) with filling of two symmetric uterine horns through the right cannula (arrowhead) due to communication in the lower uterine segment. (c) Axial T2-weighted MR image shows a uterine fundal cleft (arrow) greater than 1 cm with soft tissue separating the two symmetric uterine cavities. This finding is critical for distinction from uterus didelphys. Arrowhead = communication between the two cavities. (d) Coronal 3D US image shows the prominent uterine fundal cleft (arrow), which represents the presence of a fusion anomaly, and uterine fundal soft tissue (*) separating into the symmetric uterine cavities (U), which communicate at the level of the uterine isthmus. (Fig 13a courtesy of Joanna Culley, BA.)
The septum may be muscular fibrous or a combination of both.
It is important to distinguish a fibrous septum from a muscular septum, as the former can be repaired by a hysteroscopic approach, whereas the latter may require a transabdominal surgical approach
Figure 14. Septate uterus. (a) Illustration shows a complete septate uterus. (b) HSG image of a partial septate uterus shows a thin linear filling defect (arrow) extending from the uterine fundus, separating the uterine cavity into two symmetric cavities. (c) Coronal 3D US image of a partial septate uterus shows the isoechoic muscular septum and hypoechoic fibrous septum (*), which extends just proximal to the internal cervical os (arrowhead). The apex of the fundal contour (arrow) is more than 5 mm above a line drawn between the tubal ostia (white line), a finding compatible with a septate uterus. (d) Axial T2-weighted MR image of a complete septate uterus shows a normal external uterine contour (black arrow). The hypointense fibrous septum (white arrows) originates from the isointense muscular septum and extends into the cervical os (arrowhead). A hypointense uterine fundal fibroid (f) is also present. (Fig 14a courtesy of Joanna Culley, BA.)
Figure 15. Arcuate uterus. (a) HSG image shows a broad-based uterine fundal filling defect (black arrowhead). White arrowheads = patent fallopian tubes. (b) Coronal 3D US image shows the broad-based fundal myometrial prominence (*) and a convex external uterine contour (arrowheads). (c) Axial gadolium-enhanced T1-weighted fat-saturated MR image shows the convex external uterine contour (arrow) and the broad-based prominent fundal myometrium (*).
DES uterus. HSG image shows the classic T-shaped uterine cavity due to DES exposure.
Axial images show a bicornuateCoronal image shows a solitary kMullerian anomalies are frequently associated with renal tract anomalies. This case shows a similar presentation of bicornuate uterus associated with solitary kidneyidney.
uterus.
Axial C+ portal venous phase SHOWS Complete septate uterus with septum seen to extend down to the internal os, and convex fundal contour.
Coronal C+ portal venous phase SHOWS Dysplastic, malrotated, and pelvic location of right kidney. Unicornuate configuration of the uterus toward the left.