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Congenital malformations are anomalies,
which may be either hereditary or occuring
during gestation and evident at the time of
birth. Development of the female genital tract
is a complex process depend upon a series of
event involving cellular differentiation ,
migration, fusion and canalization. Failure of
any one these processes result in a congenital
anomaly.
A Uterine malformation is the result
of an abnormal development of the Mullerian
ducts during embryogenesis.
 The most common types of uterine
abnormalities are caused by incomplete fusion
of mullerian ducts.
 Uterine anomalies are often associated with
vaginal maldevelopment.
American Fertility society (AFS) classification
of mullerian anomalies are :
 Class 1: Mullerian agenesis / hypoplasia-
segmental ( a. vaginal, b. cervical, c. uterine, d.
tubal, e. combined).
 Class 2 : Unicornuate with or without a
rudimentary horn.
 Class 3 : Uterus didelphys.
 Class 4 : Bicornuate utrerus (a.complete to
internal os, b.partial).
 Class 5: Septate uterus (complete incomplete )
 Class 6: Arcuate uterus.
 Class 7: Diethylstilbesterol (DES) –related
anomalies.
INCIDENCE
It varies between 3 and 4%.The
incidence is found to be high in women suffering
from recurrent miscarriage or pre term deliveries (5-
20%).
 Failure of development of one or both mullerian
ducts : The absence of both ducts leads to absence of
uterus , including oviducts . There is absence of
vagina as well . Primary amenorrhea is the chief
complaint.
 Failure of recanalization of the Mullerian ducts :
Agenesis of the upper vagina or of the cervix – this
may lead to hematometra as the uterus is
functioning.
 Failure of fusion of mullerian ducts: In majority , the
presence of deformity escapes attention. In some , the
detection is made accidently during investigation of
infertility or repeated pregnancy wastage . In others,
the diagnosis is made during D & E operation ,
manual removal of placenta or during cesarean
section.
 ARCUATE (18%):
The cornual parts of the uterus remains
separated . The uterine fundus looks concave with
heart shaped cavity outline .
 UTERINE DIDELHYS (8%):
There is complete lack of fusion
of the mullerian ducts with a double uterus, double
cervix and a double vagina.
 UTERUS BICORNIS (26%):
There is varying degree of fusion
of the muscle walls of the two ducts .
I. Uterus bicornis bicollis – There are two uterine
cavities with double cervix with or without
vaginal septum.
II. Uterus bicornis unicollis – There are two uterine
cavities with one cervix . The horns may be equal
or one horn may be rudimentary and have no
communication with the developed horns
 SEPTATE UTERUS (35%):
The two Mullerian ducts are
fused together but there is persistence of septum in
between the two partially or completely.
 UNICORNUATE UTERUS OR UTERUS
UNICORNIS(10%):
There is a failure of development of one
Mullerian duct . Only one side of the Mullerian
ducts forms and there is a single uterine cavity
with a cervix and one fallopian tube coming out of
the uterus.
 ABSENT UTERUS OR UTERINE AGENESIS:
This is the most severe kind of uterine
malformation . There is failure of uterus , cervix
and vagina to develop. A girl with this
malformation will experience puberty with the
absence of menstruation . The women will have a
small dimple in the place where the vagina should
be at.
 DES – RELATED ABNORMALITY:
It is due to DES exposure during
intrauterine life . Varieties of malformation are
included . Eg: Vagina –adenocarcinoma , adenosis.
Cervix – cockscomb cervix , cervical collar.
Uterus- hypoplasia , T-shaped cavity.
Fallopian tube – cornual budding , abnormal fimbrae.
 The condition may not produce
any clinical manifestation.
 Gynecological:
 Infertility and dyspareunia are often related in
association with vaginal septum.
 Dysmenorrhea in Bicornuate uterus or due to
cryptomenorrhea.
 Menstrual disorders (menorrhagia ,
cryptomenorrhea) are seen. Menorrhagia is due to
increased surface area in Bicornuate uterus.
 Obstetrical:
 Midtrimister miscarriage which may be recurrent .
 Rudimentary horn pregnancy may occur due to
transperitoneal migration of sperm or ovum from the
opposite side.
 Cervical incompetence.
 Increased incidence of malpresentation : tansverse lie in
arcuate or subseptate, breech in bicornuate,unicornuate
or complete septate uterus.
 Preterm labor,IUGR,IUD.
 Prolonged labor – Due to incordinate uterine action.
 Obstructed labor – obstruction by the non-gravid horn of
the bicornuate uterus or rudimentary horn.
 Retained placenta and postpartum hemorrhage where
the placenta is implanted over the uterine septum.
 Ultrasonography.
 Hysterosalpingography which allows
evaluation of the uterine cavity and tubal
pregnancy.
 MRI Scan.
 Hysteroscopy.
 Dysmenorrhea.
 Hematometra.
 Complications during pregnancy and labor –
late miscarriage, preterm labor, ssuterine
rupture, malpresentation, obstructed labor.
 Fertility is uneffected except for uterine
agenesis.
 Surgical intervention depending on the type of
abnormality or enabling a viable pregnancy.
 Rudimentary horn should be excised to reduce the
risk of ectopic pregnancy.
 Unification operation (bicornuate or septate uterus)
is,therefore,indicated in unexplained cases with
uterine malformation. Abdominal metroplasty could
be done either by excising or incising the septum.
 Hysteroscopic metroplasty is more commonly
done.Resection of the septum can be done either by
resectoscope or by laser.
contd…..
Advantages are-
i. High success rate(80-89%)
ii. Short hospital stay.
iii. Reduced postoperative morbidity.
iv. Subsequent chance of vaginal delivery is high
compared to abdominal metroplasty where C-
section is mandatory.
v. Unicornuate uterus has very poor outcome for
pregnancy (40%).No treatment is generally effective.
vi. Uterus didelphys has best possibility of successful
pregnancy(64%).
 Assess the condition of the women by collecting
health history , menstrual history and obstetrical
history .
 Based on the severity of the condition plan for
interventions.
 Educate the couple about the best possible treatment
of uterine malformation.
 Assure the couple about chances of fertility or
pregnancy except for the case of uterine agenesis.
 Allow the client exploring all possible options for
family.
 Help the couples in overcoming delimmas , deciding
the right fertility treatment.
 Counsel and encourage the couple for child adoption
in case of infertility.
 Help the couple to deal with emotional stress.
 Advice the mother to avoid fertility impairing
medication.
 To relieve dysmenorrhea , provide hot application.
 Administer analgesics to relieve pain.
 Educate the woman to practice light exercise and
maintain nutritious diet such as fibre diet after
surgical treatment.

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Uterine malformation

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  • 2. Congenital malformations are anomalies, which may be either hereditary or occuring during gestation and evident at the time of birth. Development of the female genital tract is a complex process depend upon a series of event involving cellular differentiation , migration, fusion and canalization. Failure of any one these processes result in a congenital anomaly.
  • 3. A Uterine malformation is the result of an abnormal development of the Mullerian ducts during embryogenesis.  The most common types of uterine abnormalities are caused by incomplete fusion of mullerian ducts.  Uterine anomalies are often associated with vaginal maldevelopment.
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  • 6. American Fertility society (AFS) classification of mullerian anomalies are :  Class 1: Mullerian agenesis / hypoplasia- segmental ( a. vaginal, b. cervical, c. uterine, d. tubal, e. combined).  Class 2 : Unicornuate with or without a rudimentary horn.  Class 3 : Uterus didelphys.  Class 4 : Bicornuate utrerus (a.complete to internal os, b.partial).
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  • 8.  Class 5: Septate uterus (complete incomplete )  Class 6: Arcuate uterus.  Class 7: Diethylstilbesterol (DES) –related anomalies. INCIDENCE It varies between 3 and 4%.The incidence is found to be high in women suffering from recurrent miscarriage or pre term deliveries (5- 20%).
  • 9.  Failure of development of one or both mullerian ducts : The absence of both ducts leads to absence of uterus , including oviducts . There is absence of vagina as well . Primary amenorrhea is the chief complaint.  Failure of recanalization of the Mullerian ducts : Agenesis of the upper vagina or of the cervix – this may lead to hematometra as the uterus is functioning.
  • 10.  Failure of fusion of mullerian ducts: In majority , the presence of deformity escapes attention. In some , the detection is made accidently during investigation of infertility or repeated pregnancy wastage . In others, the diagnosis is made during D & E operation , manual removal of placenta or during cesarean section.
  • 11.  ARCUATE (18%): The cornual parts of the uterus remains separated . The uterine fundus looks concave with heart shaped cavity outline .  UTERINE DIDELHYS (8%): There is complete lack of fusion of the mullerian ducts with a double uterus, double cervix and a double vagina.
  • 12.  UTERUS BICORNIS (26%): There is varying degree of fusion of the muscle walls of the two ducts . I. Uterus bicornis bicollis – There are two uterine cavities with double cervix with or without vaginal septum. II. Uterus bicornis unicollis – There are two uterine cavities with one cervix . The horns may be equal or one horn may be rudimentary and have no communication with the developed horns
  • 13.  SEPTATE UTERUS (35%): The two Mullerian ducts are fused together but there is persistence of septum in between the two partially or completely.  UNICORNUATE UTERUS OR UTERUS UNICORNIS(10%): There is a failure of development of one Mullerian duct . Only one side of the Mullerian ducts forms and there is a single uterine cavity with a cervix and one fallopian tube coming out of the uterus.
  • 14.  ABSENT UTERUS OR UTERINE AGENESIS: This is the most severe kind of uterine malformation . There is failure of uterus , cervix and vagina to develop. A girl with this malformation will experience puberty with the absence of menstruation . The women will have a small dimple in the place where the vagina should be at.  DES – RELATED ABNORMALITY: It is due to DES exposure during intrauterine life . Varieties of malformation are included . Eg: Vagina –adenocarcinoma , adenosis. Cervix – cockscomb cervix , cervical collar. Uterus- hypoplasia , T-shaped cavity. Fallopian tube – cornual budding , abnormal fimbrae.
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  • 18.  The condition may not produce any clinical manifestation.  Gynecological:  Infertility and dyspareunia are often related in association with vaginal septum.  Dysmenorrhea in Bicornuate uterus or due to cryptomenorrhea.  Menstrual disorders (menorrhagia , cryptomenorrhea) are seen. Menorrhagia is due to increased surface area in Bicornuate uterus.
  • 19.  Obstetrical:  Midtrimister miscarriage which may be recurrent .  Rudimentary horn pregnancy may occur due to transperitoneal migration of sperm or ovum from the opposite side.  Cervical incompetence.  Increased incidence of malpresentation : tansverse lie in arcuate or subseptate, breech in bicornuate,unicornuate or complete septate uterus.  Preterm labor,IUGR,IUD.  Prolonged labor – Due to incordinate uterine action.  Obstructed labor – obstruction by the non-gravid horn of the bicornuate uterus or rudimentary horn.  Retained placenta and postpartum hemorrhage where the placenta is implanted over the uterine septum.
  • 20.  Ultrasonography.  Hysterosalpingography which allows evaluation of the uterine cavity and tubal pregnancy.  MRI Scan.  Hysteroscopy.
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  • 22.  Dysmenorrhea.  Hematometra.  Complications during pregnancy and labor – late miscarriage, preterm labor, ssuterine rupture, malpresentation, obstructed labor.  Fertility is uneffected except for uterine agenesis.
  • 23.  Surgical intervention depending on the type of abnormality or enabling a viable pregnancy.  Rudimentary horn should be excised to reduce the risk of ectopic pregnancy.  Unification operation (bicornuate or septate uterus) is,therefore,indicated in unexplained cases with uterine malformation. Abdominal metroplasty could be done either by excising or incising the septum.  Hysteroscopic metroplasty is more commonly done.Resection of the septum can be done either by resectoscope or by laser.
  • 24. contd….. Advantages are- i. High success rate(80-89%) ii. Short hospital stay. iii. Reduced postoperative morbidity. iv. Subsequent chance of vaginal delivery is high compared to abdominal metroplasty where C- section is mandatory. v. Unicornuate uterus has very poor outcome for pregnancy (40%).No treatment is generally effective. vi. Uterus didelphys has best possibility of successful pregnancy(64%).
  • 25.  Assess the condition of the women by collecting health history , menstrual history and obstetrical history .  Based on the severity of the condition plan for interventions.  Educate the couple about the best possible treatment of uterine malformation.  Assure the couple about chances of fertility or pregnancy except for the case of uterine agenesis.
  • 26.  Allow the client exploring all possible options for family.  Help the couples in overcoming delimmas , deciding the right fertility treatment.  Counsel and encourage the couple for child adoption in case of infertility.  Help the couple to deal with emotional stress.  Advice the mother to avoid fertility impairing medication.  To relieve dysmenorrhea , provide hot application.  Administer analgesics to relieve pain.  Educate the woman to practice light exercise and maintain nutritious diet such as fibre diet after surgical treatment.