Mullerian duct anomalies

1. Mullerian Ducts Anomalies
Dr Deepti Guntupalli

2. • Paired ducts derived from intermediate mesoderm
• Known as paramesonephric duct ( After named as Johannes peter
mullero (Described them in 1830)

3. EMBROLOGICAL DEVELOPMENT
• Appear between 5-6 wks
• Derived from intermediate mesoderm lateral to Wolffian duct as
invagination of dorsal coelomic epithelium
• Depends on absence of male determining factor which is present in Y-
Chromosome.
• Female development called Basic developmental path of the human
embryo requires not estrogen but the absence of testosterone
• Ambisexual period persist up to 8 wks, thereafter one type of duct
system persist and other disappears

4. Development of Genital Ducts
• Both male and female embryos have two pairs of genital ducts
• The mesonephric ducts ( Wolffian ducts) play an important role in the
development of the male reproductive system
• The paramesonephric ducts ( Mullerian ducts) have a leading role in
the development of the female reproductive system

6. Continuation..
• Till the end of sixth week, the development of the female
reproductive system.
• Mullerian ducts form as buds of coelomic epithelium.
• Grows downward and lateral to corresponding Wolffian ducts.
• Turn inwards and Crosses anterior to it joining its fellow from
opposite side.

8. Three Main principles governs the practical
approach to mullerian anomalies
1. Mullerian and Wolffian ducts are so closely related embryologically
that mullerian anomalies are commonly associated with anomalies of
kidney and ureter.
2. Development of gonads is separate from ducts so normal ovaries are
present in mullerian anomalies
3. Mullerian anomalies may be associated with anomalies in sex
chromosome make up of individual.

9. • Complete formation and differentiation of mullerian duct into female
reproductive system depends on completion of 3 phases of
development as follows
• 1 Organogenesis
• 2. Fusion
• 3. Septal resorption

10. • ORGANOGENESIS
• One or both mullerian duct may not develop fully- uterine agenesis or
hypoplasia unicornuate uterus
• FUSION
• LATERAL FUSION – Process during which lower segment of paired mullerian
duct fuse – Uterine didelphys or bicornuate uterus, Arcuate uterus.
• VERTICAL FUSION –fusion of ascending sinovaginal bulb with descending
mullerian duct- Transverse vaginal septum, imperforate hymen.
• SEPTAL RESORPTION
• After fusion central septum persist later resops to form single uterocervical
cavity – Septate uterus

11. DEVELOPMENT OF THE MULLERIAN DUCTS
• ETIOLOGY
• Although teratogenic exposures such as Thalidomide
• Diethylstilbestrol (DES)
• Radiation – have been linked with these abnormalities. The vast majority aare
likely related to
• Polygenetic and familial factors
• AGE
• Anomalies may be diagnosed in infancy, adolescence or young adulthood.

12. PREVALENCE
• True incidence and prevalence are difficult to assess
• ( Non standardized classification systems, Differences in diagnostic data
acquisition)
• Uncommon
• Most common in the female reproductive tract anomalies.
• General population : 0

13. MORBIDITY
1. OBSTETRICS
1. Repeated Abortion
2. Cervical incompetence
3. PTL
4. IUGR
5. Abnormal fetal life
6. Dystocia at delivery
7. Retained Placenta

14. II GYNECOLOGY
1. Infertility
2.Dysmenorrhea
3.obstructed or partially obstructed mullerian systems who
present with hematosalpinx, hematocolpos, retrograde
menses and endometriosis.
III RENAL
high association between mullerian duct anomalies and renal
anomalies such as unilateral agenesis

15. CLASSIFICATION
• 7 CLASSES ACCORDING TO THE American fertility society (AFS – 1988)
• CLASS I – HYPOPLASIA/AGENESIS
• The most common form - MAYER ROKITANSKY-KUSTER- HAUSER SYNDROME
• Combined agenesis of the uterus, cervix and upper portion of the vagina
• The patients have no reproductive potential aside from medical intervention in the form
of IVF of harvested Ova and implantation in a host uterus.
• DIFFERENTIAL DIAGNOSIS
• Testicular feminizing syndrome

17. • CLASS II – UNICORNUATE UTERUS
• Result from complete or almost complete arrest of development of 1
mullerian duct
• If the arrest is incomplete as in 90% of patient, A Rudimentary horn with or
without functioning endometrium is present
• If the rudimentary horn is obstructed it may come to surgical attention when
presenting as an enlarging pelvic mass
• If the contralateral healthy horn is almost fully developed, A full term
pregnancy is believed to be possible.

18. • (A)Unicornuate uterus
with no rudimentary horn
due to complete agenesis
of the contralateral duct
• (B)a rudimentary horn
without a uterine cavity.
• (C) A rudimentary horn
with a communicating
cavity to the normal side
• (D)A rudimentary horn
with a noncommunicating
cavity.

19. • CLASS III- DIDELPHYS UTERUS
• Results from complete nonfusion of both mullerian ducts.
• The individual horns are fully developed and almost normal in size
• Two cervices are inevitably present
• Didelphys uteri have the highest association with transverse vaginal septa
• Consider metroplasty; how ever, since each horn is almost a fully developed
uterus.
• Patients have been known to carry pregnancies to full term.

21. • CLASS IV- BICORNUATE UTERUS
• Results from – Partial nonfusion of the mullerian ducts
• SUBTYPES
• BICORNUATE UNICOLLIS
• The central myometrium extend to the level of the internal cervical OS
• BICORNUATE BICOLLIS
• The central myometrium extend to the level of external cervical OS
• Differential diagnosis of bicornuate bicollis from didelphys uterus
• 1. It demonstrates some degree of the fusion between the two horns, while in
classic didelphys uterus the two horns and cervices are separated completely
• 2. The horns of the bicornuate uteri are not fully developed. Typically they
are smaller than those of Didelphys uteri.
• Some patients are surgical candidates for metroplasty

24. • CLASSS V – SEPTATE UTERUS
• Results from failure of resorption of the septum between the two uterine
horns
• The septum can be partial or complete – in which case it extends to the
internal cervical OS
• Histologically composed of Myometrium or fibrous tissue.
• The uterine fundus is typically convex but may be flat or slightly concave
(<1cm fundal cleft)
• Women with septate uterus have the highest incidence of reproductive
complications
• Differential Diagnosis between a septate and a bicornuate uterus is
important ( Septate uteri are treated using Transvaginal hysteroscopic
resection of the septum, While if surgery is possible / or indicated for
the bicornuate uterus,
• An abdominal approach is required to perform metroplasty.

25. BICORNUAATE SEPTATE
Fundus indented Normal external surface
Variable degree of separation of uterine horns that
can be complete, partial or minimal
Need Laparoscopy to Dx,
Minimal reproductive problems, however can have
pregnancy loss, PTL
Septum can cause infertility, Recurrent Midtrimester
loss
HSG won’t Dx, Need Laparoscopy

28. • CLASS IV – ARCUATE UTERUS
• The endometrial cavity demonstrates a small fundal cleft or impression
(>/= 1.5 cm)
• The outer contour of the uterus is convex or flat
• This form is a normal variant (It is not significantly associated with the
increased risks of pregnancy loss and the other complications found in other
subtypes.)

29. • Arcuate Uterus
• Mild thickening
of the midline
fundal
myometrium
resulting in
fundal cavity
indentation but
normal outer
fundal contour.
• It is not
associated with
an increased
risk of Obstetric
/ gynaecologic
complications

30. • CLASS VII –
DIETHYLSTILBESTROL –
RELATED ANOMALY
• The uterine anomaly is
seen in the female
offspring of as many as
15% of women exposed to
DES during pregnancy.
• UTERUS : Hypoplasia and a
T-Shaped uterine cavity
• CERVIX : Abnormal
transverse ridges, hoods,
stenoses
• VAGINA : Adenosis with
increased risk of vaginal
clear cell carcinoma

31. CLINICAL PRESENTATION
• 1. IN THE NEWBORN/ INFANT
• Obstructed system: A palpable abdominal, pelvic or vaginal mass ( mucocolpos)
• 2. IN ADOLESCENT GIRL
• Delayed menarche / or an obstructed system presenting as an intra- abdominal mass (
hematocolpos) Cyclical pain.
• 3. CHILDBEARING AGE
• Infertility, repeated spontaneous abortions or PTL

32. INVESTIGATION
• 1. US
• TAS and if feasible, TVS
• TVS 2D: uterine anomalies may not be excluded on the basis of negative US findings.
• 3D : Higher sensitivity and specificity
• 2. HSG Performed under Fluoroscopy
• Allows evaluation of the UT cavity and tubal patency- Anomalies may be suggested
• HSG is the least accurate :
• 1. Positive findings often are nonspecific for precise diagnosis
• 2. Visualization of 2 UT cavities on HSG does not aid in distinguishing septate, didelphys &
bicornuate uteri
• It may not be possible to perform if there is a lower abnormality prohibiting UT entry from
the vagina

33. • 3. MRI
• Standard for imaging UT anomalies
• 1 High resolution images
• 2. Evaluate the urinary tract for concomitant anomalies. In the past,
intravenous urography was used for this purpose.
• Most types of UT anomalies can be diagnosed
• MRI is the most accurate – followed by TVS and HSG
• 4. HYSTEROSCOPY AND LAPAROSCOPY
• Employed to help with the diagnosis as well as potential treatments with
similar shortcomings related to hysteroscopy to those seen with HSG
• It would be necessary to employ both methods to differentiate a septum from
a bicornuate uterus.

34. MANAGEMENT
• Depend on
• 1. The presence and severity of menstrual, fertility and sexual function
problems
• 2. The type of anomaly
• The mere presence of an abnormality does not necessitate treatment
unless thee patient is symptomatic as a result of it
• MENSTRUAL DISTURBANCES
• Most commonly represented by
• 1. A transverse or blocking septum,
• 2. May be absence of the vagina or cervical anomalies

35. INFERTILITY
• Etiology
• Problems relating to fertilization (Due to blockage of the sperm’s path
• Implantation or pregnancy maintenance
• The type of abnormality will guide the approach treatment
• Many assisted reproductive techniques are now available
• Uterine septum : Hysteroscopic resection

36. SEXUAL FUNCTION
• Can be affected in couple of ways
• 1. A Complete absence of the vagina. In this circumstance, normal intercourse
would be impossible and creation of a neovagina may be appropriate
• 2. In the case of both a transverse and longitudinal septum, a physical barrier may
make intercourse difficult, painful, or even impossible
• Vaginal septum, if the patient is symptomatic can usually be treated with a simple
resection if small.
IN CASE OF ABSENCE OF VAGINA
1. If the uterus is present: Creation of the neovagina with a communication to the
cervix
2. If these do not exist or if the there is no uterus
1. Nonsurgical method should be employed initially ( the use of subsequently larger vaginal
dilators to stretch the area where the vagina is created.

37. • 2. SURGICAL PROCEDURES
A Mclndoe:
• A space is dissected between the rectum and the bladder
• A split- thickness skin graft from the buttocks is used to form the
vagina
• dilator at the time of the procedure creates continuous dilation
of the vagina while the graft heals

38. • B. OTHER PROCEDURES
• WILLIAMS VULVOVAGINOPLASTY
• It uses full thickness skin flaps from the labia majora to create a vaginal pouch
which axis is directly posterior and horizontal to the perineum; however the
vagina is functional and well received by patients
• Musculocutaneous flaps and free intestinal grafts
• The decision of which approach to take is dictated by the patients characteristic's and
needs.
• C IVF CYCLE
• Through the myometrial wall – a direct connection of the uterus cavity to the
vagina through the cervix may not be an issue when considering fertility
problems
• When fertility is not an issue and the patient is suffering from the menstrual
problems, hysterectomy can be consideration.

39. • THANKING YOU