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MULLERIAN DUCT ANOMALIES
1.
2. EMBRYOLOGY
Two paired müllerian ducts ultimately develop
into: fallopian tubes, uterus, cervix, and the
upper two thirds of the vagina.
3 phases of development as follows:
1. Organogenesis:
One or both müllerian ducts may not develop
fully, resulting in:
uterine agenesis or hypoplasia (bilateral) or
unicornuate uterus (unilateral).
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4. 2. Fusion:
a.Lateral fusion of the lower segments of
the paired müllerian ducts form the
uterus, cervix, and upper vagina
Failure of fusion results in bicornuate or
didelphys uterus.
b. Vertical fusion: fusion of the ascending
sinovaginal bulb with the descending
müllerian system forms a normal patent
vagina
incomplete vertical fusion results in an
imperforate hymen.ABOUBAKR ELNASHAR
5. c. Septal resorption:
After the lower müllerian ducts fuse, a central
septum is present, which subsequently must be
resorbed to form a single uterine cavity and
cervix. Failure of resorption is the cause of
septate uterus.
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7. ETIOLOGY
Although teratogenic exposures such as
thalidomide,
diethylstilbestrol (DES), and
radiation have been linked with these
abnormalities, the vast majority are likely
related to
polygenetic and
familial factors.
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8. PREVALENCE
•True incidence and prevalence are difficult
to assess:
{Nonstandardized classification systems.
Differences in diagnostic data acquisition}
•Uncommon
•Most common of the female reproductive
tract anomalies
General population: 0.5%
Fertile women: 2-3%
Infertile women: 3%
Repeated miscarriages: 5-10%
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12. II. Gynecology:
1. Infertility
2. Dysmenorrhea
3. Obstructed or partially obstructed müllerian
systems who present with
hematosalpinx, hematocolpos, retrograde
menses, and endometriosis.
III. Renal:
High association between müllerian duct
anomalies and renal anomalies such as
unilateral agenesis.
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13. CLASSIFICATION
7 classes according to the American Fertility
Society (AFS) (1988):
Class I (hypoplasia/agenesis):
The most common form is the Mayer-
Rokitansky-Kuster-Hauser syndrome:
Combined agenesis of the uterus, cervix,
and upper portion of the vagina.
Patients have no reproductive potential
aside from medical intervention in the form
of IVF of harvested ova and implantation in
a host uterus.
DD: Testicular fiminization syndrome
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15. Class II (unicornuate uterus):
• result from complete, or almost complete,
arrest of development of 1 müllerian duct
•If the arrest is incomplete, as in 90% of
patients, 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
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16. Unicornuate uterus. Note the failure of the development
of one half of the uterus. This form may be associated
with a rudimentary horn arising from the contralateral
müllerian duct.
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20. 1.Unicornuate with uterine horn (no with uterine horn
(no endometrial endometrial cavity) fused to
unicornuate uterus
2.Unicornuate uterus with noncommunicating horn
containing endometrial cavity not fused
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21. Class III (didelphys uterus):
•results from complete nonfusion of both
müllerian 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; however, since each
horn is almost a fully developed uterus,
patients have been known to carry
pregnancies to full term.
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22. Didelphys uterus. Note
the complete separation
but full development of
each müllerian duct.
Uterine didelphys with
complete vaginal
septum
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27. Class IV (bicornuate uterus):
• Results from: partial nonfusion of the müllerian 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.
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28. • DD of bicornuate bicollis from didelphys uterus
1.it demonstrates some degree of 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.
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29. Bicornuate uterus. Note the partial fusion of the lower uterine
segment and persistently separated upper uterine segments.
Of key importance is the prominent fundal cleft (>1 cm), which
distinguishes the anomaly from septate uterus.
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33. Class 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 (<1-cm fundal cleft).
•Women with septate uterus have the highest
incidence of reproductive complications.
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34. •DD 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 and/or indicated for the
bicornuate uterus, an abdominal approach is required
to perform metroplasty}.
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35. Bicornuate
•Fundus indented
•Variable degree of
separation of uterine
horns that can be
complete, partial or
minimal
•Minimal reproductive
problems, however can
have pregnancy loss, PTL
•HSG won’t dx, need
laparoscopy
Septate
•Normal external surface,
•need laparoscopy to dx
•Septum can cause
infertility,
recurrent midtrimester
loss
Septate or bicornuate?
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36. Septate uterus. Midline
septum can be of
variable length and can
be muscular or fibrous. In
the diagram, the septum
is shown as an extension
of the uterine
myometrium.
Septate uterus. The midline
septum can extend for a variable
length and can be muscular or
fibrous. In the diagram, the
septum is thin and linear as
expected in the fibrous type. Since
the composition of the septum
varies, whether it is composed of
muscle or fibrous tissue is not a
means to distinguish septate from
other forms of uterine anomalies.
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40. Class VI (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}.
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41. Arcuate uterus. Mild thickening of the midline fundal
myometrium resulting in fundal cavity indentation but
normal outer fundal contour. Some authors consider it a
normal variant. It is not associated with an increased
risk of obstetric/gynecologic complications.
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42. 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.
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45. TeLinde's Modification of the American Fertility
Society Classification of Uterovaginal Anomalies[3]
Class I. Dysgenesis of the Mullerian Ducts
Class II. Disorders of Vertical Fusion of the Mullerian Ducts
Class III. Disorders of Lateral Fusion of the Mullerian Ducts
Class IV. Unusual Configuration of Vertical-Lateral Fusion Defects
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46. CLINICAL PRESENTATION
Suggestion:
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.
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47. 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
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48. 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.
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49. This was difficult to
differentiate as septate or
bicornuate uterus using
hysterosalpingography. It was
a surgically proven case of
bicornuate uterus.
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°).ABOUBAKR ELNASHAR
50. 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.ABOUBAKR ELNASHAR
51. 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.
3. Most types of ut anomalies can be diagnosed
• MRI is the most accurate, followed by TVS and
HSG.
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52. Septate uterus :
outer fundal contour
(superior border) is flat or
slightly concave
Septate uterus:
a longer septum divides the
uterine cavity.
Outer fundal contour is flat.
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53. Didelphys uterus:
a. Complete
separation and full
development of
both müllerian
ducts
b. Two vaginas
and 2 cervices
c. 2 distinct
cervices
d. 2 uterine horns
are widely
splayed; cross
section of
uterine bodies
and cervices.
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54. Unicornuate uterus:
Full development of a single uterine horn and a
normal-appearing cervix. This anomaly was one
of many in this patient with Goldenhar syndrome.ABOUBAKR ELNASHAR
55. Septate uterus:
Thin, fibrous septum that cannot
be resolved distally at the
fundus.
Outer fundal contour is convex,
thus excluding a bicornuate
uterus.
Bicornuate bicollis:
The midline uterine external fundal cleft (superior
border) has a depression >1 cm, excluding septate
uterus
2 cervices are present.
Not didelphys uterus because some degree of fusion
has occurred between the lower uterine segments
(ie, they are fused, although the cavities are not
communicating).
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56. 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.
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58. 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 the patient is
symptomatic as a result of it.
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59. Menstrual disturbances:
most commonly represented by
1. a transverse or blocking septum, but also 2.
absence of the vagina or cervical anomalies.
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61. 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 to treatment.
Many assisted reproductive techniques are
now available.
Uterine septum: hysteroscopic resection
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62. Sexual function
can be affected in a 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.
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63. In cases of absence of the vagina:
I. If the uterus is present: creation of the
neovagina, with a communication to the cervix
II. If these do not exist, or if there is no uterus:
1. Nonsurgical methods should be employed
initially (the use of subsequently larger vaginal
dilators to stretch the area where the vagina is
to be created).
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64. 2. Surgical procedures.
a. McIndoe:
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.
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65. 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 patient's
characteristics and needs.
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66. c. IVF cycle through the myometrial wall. Therefore,
a direct connection of the uterine 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 menstrual problems, hysterectomy
can be a consideration.
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