Congenital uterine anomaly

1. Narmeen Hassan MS5
Final year medical student
Dow Medical College, Pakistan

2.  Case
 Introduction
 Müllerian Development
 Congenital MüllerianAnommalities
 Causes
 Clinical Presentation
 Diagnosis
 Treatment
 Prognosis

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

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

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