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This further divides into the Gonadal or Genital Ridge, which will become
the ovary, and into the nephrogenic cord
Mllerian Ducts become the fallopian tubes, uterus, and upper vagina
Derive from coelomic epithelium covering the nephrogenic cord
This separate gonadal and mllerian derivation that women
with mllerian defects typically have functionally normal
ovaries and are phenotypic females.
Urinary tract develops from the Mesonephros or Wolffian Ducts situated within
each nephrogenic cord and connects the mesonephric kidney to the cloaca
Each mesonephros degenerates near the end of the first trimester, and
without testosterone, the mesonephric ducts regress as well.
Fusion begins in the middle and then extends caudally and cephalad.
As the distal end of the fused mllerian ducts contacts the urogenital
sinus, this induces endodermal outgrowths (Sinovaginal Bulbs).
These bulbs proliferate and fuse to form the vaginal plate, which
later resorbs to form the vaginal lumen.
This vaginal canalization is generally completed by the 20th week.
Lumen remains separated from the urogenital sinus by the
hymeneal membrane.
When these are identified, the urinary system can be evaluated with
magnetic resonance (MR) imaging, sonography, or intravenous
pyelography
MESONEPHRIC REMNANTS
Mesonephric ducts usually degenerate, however, persistent remnants may
become clinically apparent.
Mesonephric or Wolffian vestiges can persist as Gartner Duct Cysts.
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Urethra and vagina are typically short, and the vaginal orifice is frequently
stenotic and displaced anteriorly.
Clitoris is duplicated or bifid, and the labia, mons pubis, and clitoris are
divergent.
Uterus, fallopian tubes, and ovaries are typically normal except for
occasional mllerian duct fusion defects.
Due to the extensive adhesions from prior repair and altered anatomy
typically encountered, some recommend planned cesarean delivery at a
tertiary center
Epispadias without Bladder Exstrophy includes
In some preterm neonates, the clitoris may appear large, but then
regresses as the infant grows.
Mllerian Agenesis
Class I segmental defects can be caused by mllerian hypoplasia or agenesis
MLLERIAN ABNORMALITIES
There are four principal deformities that arise from defective mllerian duct
embryological steps:
1)
Agenesis of both
ducts, either focally or
along the entire duct
length
2)
Unilateral maturation
of one mllerian duct
with incomplete or
absent development
of the opposite side
3)
Absent or faulty
midline fusion of the
ducts
4)
Defective
canalization.
American Fertility Society
separates anomalies into groups with similar clinical characteristics, prognosis
for pregnancy, and treatment
Rem Alfelor
Chapter 3: Congenital Genitourinary Abnormalities
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may be necessary.
Cervical Abnormalities
Developmental abnormalities of the cervix include
1)
Partial or Complete Agenesis
2)
Duplication
3)
Longitudinal Septa
Uncorrected complete agenesis is incompatible with pregnancy, and IVF with
gestational surrogacy is an option.
Surgical correction by uterovaginal anastomosis has resulted in successful
pregnancy
There are significant complications with this corrective surgery, and the
need for clear preoperative anatomy delineation has been emphasized
Uterine Abnormalities
Accurate population prevalences of these are difficult to assess because the
best diagnostic techniques are invasive
Population prevalence ranges from 0.4-5%, and rates in women with recurrent
miscarriage are significantly higher
Distribution of uterine anomalies as follows:
Bicornuate,
39%
Septate
34%
Didelphic
11%
Arcuate
7%
Unicornuate
5%
Hypo- Or Aplastic
4%
Mllerian anomalies may be discovered at routine pelvic examinations,
cesarean delivery, during laparoscopy for tubal sterilization, or during infertility
evaluation.
Depending on clinical presentation, diagnostic tools may include
Hysterosalpingography
Sonography
MR imaging
Laparoscopy
Hysteroscopy
Poorly defines the external uterine contour and can delineate only patent
cavities.
Transabdominal views may help to maximize the viewing field, but transvaginal
sonography (TVS) provides better image resolution.
Contraindicated in pregnancy.
Three-dimensional (3-D) sonography is more accurate than 2-D sonography
because it provides uterine images from virtually any angle.
Malpresentation
Fetal-growth restriction
Fetal demise
Preterm delivery
Abnormal uterine blood flow, cervical incompetence, and diminished
cavity size and muscle mass of the hemi-uterus are postulated to
underlie these risks
Rudimentary horns also increase the risk of an ectopic pregnancy within the
remnant, which may be disastrous.
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Term was once used to refer to all marsupials, but now only to one genus
that includes the American possum (Didelphys virginiana) one of several
mammalian species in which the female has a double uterus, cervix, and
vagina.
These anomalies may be suspected on pelvic examination by identification of a
longitudinal vaginal septum and two cervices.
During HSG for fertility evaluation, contrast shows two separate endocervical
canals.
Separate divergent uterine horns with a large intervening fundal cleft are
seen.
Miscarriage
Preterm Birth
Malpresentation
Metroplasty for either uterine didelphys or bicornuate uterus involves resection
of intervening myometrium and fundal recombination
Miscarriage
Preterm Birth
Malpresentation
Surgical correction by Metroplasty is reserved for highly selected patients.
60% pregnancy rate and 45% live birth rate in those so treated.
Some women with uterine anomalies and repetitive pregnancy losses may
benefit from transvaginal or transabdominal cervical cerclage women with
partial cervical atresia or hypoplasia may also benefit
Diethylstilbestrol Reproductive Tract Abnormalities (Class VII)
During the 1960s, a synthetic nonsteroidal estrogen diethylstilbestrol (DES)
was used to treat pregnant women for threatened abortion, preterm labor,
preeclampsia, and diabetes.
It was later discovered that women exposed as fetuses had increased risks of
developing a number of specific reproductive-tract anomalies.
These included
Vaginal Clear Cell Adenocarcinoma
Cervical Intraepithelial Neoplasia
Small-Cell Cervical Carcinoma
Vaginal Adenosis
A fourth of affected women had identifiable structural variations in the cervix and
vagina to include
Transverse Septa
Circumferential Ridges
Cervical Collars
Even more anomalies were smaller uterine cavities, shortened upper uterine
segments, T-shaped and other irregular cavities, and fallopian tube
abnormalities
These women also had fertility issues that included impaired conception rates
and higher rates of miscarriage, ectopic pregnancy, and preterm delivery,
especially in those with structural abnormalities
Now, more than 50 years after DES use was proscribed, most affected women
are past childbearing age, but higher rates of earlier menopause and breast
cancer have been reported in exposed women
Fallopian Tube Abnormalities
Fallopian tubes develop from the unpaired distal ends of the mllerian ducts.
Congenital anomalies include
Accessory Ostia
Particularly when the abdominal wall is lax such as with diastasis recti or
ventral hernia, the uterus may fall forward.
This may be so extreme that the fundus lies below the lower margin of the
symphysis.
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Abdominal discomfort
Pelvic pressure
After bladder catheterization, the uterus can usually be pushed out of the
pelvis when the woman is placed in the knee-chest position.
Rem Alfelor
Sacculation
Persistent entrapment of the pregnant uterus in the pelvis may lead to extensive
An elongated vagina passing above the level of a fetal head that is deeply
placed into the pelvis suggests a Sacculation or an abdominal
pregnancy.
Uterine Torsion
It is common during pregnancy for the uterus to rotate to the right side.
Rarely, uterine rotation exceeds 180 degrees to cause torsion.
Most cases of torsion result from
Uterine leiomyomas
Mllerian anomalies
Fetal malpresentation
Pelvic adhesions
Obstructed labor
Abdominal pain
Uterine hypertonus
Vaginal bleeding
Hypotension
Both maternal and fetal complications were more common with early gestation
and with greater degrees of torsion.
Most cases of uterine torsion are found at the time of cesarean delivery.
In some women, torsion can be confirmed preoperatively with MR imaging,
which shows a twisted vagina that appears X-shaped rather than its normal Hshape
As with uterine incarceration, during cesarean delivery, a severely displaced
uterus should be repositioned anatomically before hysterotomy.
In some cases, an inability to reposition may require that a posterior
hysterotomy incision be done
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