3. 17 yr single femaleG0P0 presented for
evaluation of uterine and vaginal anomaly.
Her menarche started at age 12 ½ with
regular cycles lasting 5 days. Her pelvic
examination showed a longitudinal vaginal
septum with cervix on either side indicating a
didelphic vagina.An ultrasound showed ill
defined uterine anomaly.
4. MRI showed
1. Uterus has an external
arcuate configration
2. Complete uterine
septum extends from the
fundus to the level of the
cervix
3. Two separate cervix are
seen
5. Congenital uterine anomalies
are malformations of the uterus
that develop during embryonic life.
Congenital uterine anomalies
Occur in less than 5% of all
women, but have been noted in
up to 25% of women who have
had miscarriages and/or deliveries
of premature babies. When a
woman is in her mother’s womb,
her uterus develops as two separate
halves that fuse together before the
woman is born.The images
illustrate a normal and abnormal
uterus visualized by
hysterosalpingogram.
6. Uterine anomalies are quite often
asymptomatic and so are hard to recognize
Uterine anomalies are often an incidental
diagnosis while seeing the patient for a
different complaint
7.
8. Unicornuate: only one half of the uterus has developed
Didelphys: the two halves of the uterus remain separate
Bicornuate: an abnormal, indented external uterine surface and
two endometrial cavities
Septate: a normal external uterine surface but two endometrial
cavities
Arcuate: a normal external uterine surface with a 1 cm or less
indentation into the endometrial cavity
9.
10. In the majority of cases, the cause of a congenital uterine
anomaly is unknown. Most women with these
malformations (more than 90%) have a normal number of
chromosomes, 46 XX.
Between 1938 and 1971, some pregnant women were
treated with diethylstilbestrol (DES) which is a synthetic
estrogen to help prevent miscarriages and premature
deliveries.Women who were exposed to DES while in their
mother’s womb are at increased risk for having a
congenital uterine anomaly and vaginal adenosis
At this time, there are no well-established risk factors for
the development of a congenital uterine anomaly, and
there is no way to prevent development of a congenital
uterine anomaly.
11. Although congenital uterine anomalies are present at birth, these
malformations are usually without symptoms.. Congenital uterine
anomalies typically do not cause a woman to have difficulty
getting pregnant. However, these malformations are often
discovered during evaluations for infertility or pregnancy loss.
The Patient can present with:
Pelvic pain (cyclic or non-cyclic)
Dysmenorrhea
Abnormal vaginal bleeding
Uterine rupture during pregnancy
Recurrent pregnancy loss
Patient may have a concurrent renal abnormalities
12. Gynecologic Ultrasonography
Pelvic MRI :MRI is considered the preferred
modality due to its multiplanar capabilities as
well as its ability to evaluate the uterine
outline, junctional zone, and other pelvic
anatomy
Hysterosalpingography : Unable to outline
the exterior surface of the uterus.
Laparoscopy
Hysteroscopy
13. There are no non-surgical treatments for
congenital uterine anomalies.
Recommendations for surgical treatment of
congenital uterine anomalies depend on the
particular anomaly and the woman’s
reproductive history.
Surgical intervention should be considered when
a septate uterus is found in association with
adverse reproductive outcome.
Bicornuate, unicornuate and didelphic uteri
rarely require surgical treatment.
14. Many women with a congenital uterine
anomaly have no medical or reproductive
problems.
Septate uteri have some of the poorest
reproductive outcomes of müllerian duct
anomalies.
15. The septate uterus is a result of absent or incomplete
resorption of the intervening uterovaginal septum
following fusion of the müllerian ducts. It is the most
common congenital anomaly of the uterus,
comprising approximately 55% of all anomalies
A septum is primarily composed of fibromuscular
tissue that may project minimally from the uterine
fundus or may extend to the cervical os, almost
completely dividing the uterine cavity in two. Septa
also may be segmental, resulting in partial
communications between the two side
16. Complications: 90% miscarriage rate, a patient with a septate
uterus does not usually not have difficulty conceiving, but
a septate uterus is associated with the highest rate of pregnancy
loss of the Müllerian duct anomalies Raga et al reported a 25.5%
incidence of early miscarriage (< 13 weeks) and a 6.2% incidence
of late miscarriage (14 to 22 weeks) in women with septate
uterus. Premature birth rates are increased at approximately 21%
and fetal survival rates are estimated at 32%
Differential Diagnosis: Bicornuate uterus-- the shape of the
external uterine contour is crucial to differentiate a septate uterus
from a bicornuate uterus, because widely different clinical and
interventional approaches are assigned to each anomaly.
17. Treatment: Surgical intervention, Hysteroscopic
septal incision/hysteroscopic metroplasty. In septate
uterus, but not in bicornuate uterus, the septum can
be shaved off during hysteroscopy (metroplasty) to
form a single uterine cavity without perforating the
uterus
Prognosis: Reproductive outcome has been shown to
improve after resection of the septum, with reported
decreases in the spontaneous abortion rate from 88%
to 5.9% after hysteroscopic metroplasty.
18. Understanding the embryologic origin of the
defect of mullerian anomalies is the key to its
correct diagnosis
Women with a history of miscarriage, pre-
term deliveries, have a higher incidence of
anomalies
19. American Society of Reproductive Medicine
TheAmerican Congress of Obstetricians and
Gynecologists
Medscape
UptoDate
Notas del editor
Sexual differentiation begins early in the fetal period. Up until the sixth week of life the male and female genital systems are identical. There are two pairs of symmetrical genital ducts, the mesonephric (Wolffian) duct and the paramesonephric (müllerian) ducts. The müllerian ducts arise as coelomic invaginations in the mesonephros, and their formation is thought to be induced by the mesonephric duct. In the female embryo, in the absence of fetal testes, testosterone, and müllerian-inhibiting substance, the Wolffian ducts begin to degenerate and allow for the maturation of the müllerian ducts. The müllerian ducts grow caudally and become enclosed in peritoneal folds that later give rise to the broad ligaments of the uterus, to which the ovaries (mesovarium), fallopian tubes (mesosalpinx), and uterus (mesometrium) are attached. The müllerian ducts approach each other and begin to fuse. At 9 weeks gestation, the septum from the fused ducts begin to resorb, forming a tube with a single lumen called the uterovaginal canal. This becomes the uterus and upper portion of the vagina, whereas the unfused cranial portions of the müllerian ducts become the fallopian tubes. The lower portion of the vagina is formed from the sinovaginal bulb of the urogenital sinus. The uterovaginal canal elongates to eventually unite with the urogenital sinus, thus forming the completion of the female reproductive tract.
Müllerian tract anomalies result from failure to complete bilateral duct elongation, fusion, canalization, or septal resorption of the müllerian ducts. They may occur in any step during this developmental process. The etiologies of such disorders are still widely unknown.
in 1988 by the American Fertility Society (currently known as the America Society for Reproductive Medicine) and is now the most commonly accepted means of characterizing müllerian tract defects . Mayer-Rokitansky-Küster-Hauser
The septate uterus and bicornuate uterus are the most common congenital uterine anomalies. The arcuate uterus is considered a variation of normal uterine development by most obstetricians and gynecologists.
The mechanism by which the septate uterus causes pregnancy loss in not clearly understood. The conventional view is that the septum is rather avascular and this lack of vascularization may compromise decidual and placental growth. Also proposed is the idea that a uterine septum may impair fetal growth as a result of reduced endometrial capacity or a distorted endometrial cavity, and therefore result in second-trimester miscarriage and premature labor