Prepared by:

Galimyanova Elvira Ilgizarovna

 Congenital anomalies, also commonly referred to as
birth defects, congenital disorders, congenital
malformations, or congenital abnormalities, are
conditions of prenatal origin that are present at birth,
potentially impacting an infant's health, development
and/or survival.

 Congenital anomalies are persistent morphological

changes in organ, system or organism that go beyond
the variation of their structure, either genetically
determined, or as a result of impaired intrauterine
development.

 Congenital anomalies of the female genital tract are

developmental issues that form in the embryo. These
formations can occur in the vagina, ovaries, uterus or
cervix.
 I. Vaginal aplasia.
 1. Complete aplasia of the vagina and uterus:
 a) uterine rudiment in the form of two muscle
ridges;
 b) uterine rudiment in the form of one muscle
ridge (right, left, in center);
 c) muscle ridges are absent.
 2. Complete vaginal aplasia and a functioning
rudimentary uterus:
 a) a functioning rudimentary uterus in the form
of one or two muscle ridges;
 b) rudimentary uterus with cervical aplasia;
 c) rudimentary uterus with aplasia of cervical
channel.
 3. Partial vaginal aplasia with a functioning uterus:
 a) aplasia of the upper third;
 b) aplasia of the middle third;

 c) aplasia of the lower third

Vaginal aplasia

normal Complete lower third middle third

“double transverse
upper third vagina” or vaginal Uterus didelphys
longitudinal septum Vaginal agenesis
vaginal septum (TVS)
 II.Unicornuate uterus

 1. Unicornuate
uterus with a
rudimentary horn
communicating
with the cavity
 2. Non–
communicating
rudimentary horn.
 3. Rudimentary
horn without
cavity.
 4. Absence of
rudimentary horn.
Laparoscopic view reveals a round ligament on the left side and a normal
uterus, with no visible left tube or ovary, only a 2-cm tubal remnant. The
right fallopian tube and right ovary (with a corpus luteum) were
considered to be normal (arrow indicates tubal remnant).
 III.A double uterus and vagina.
 1. A double uterus and vagina without
disturbing the outflow of menstrual
blood.
 2. A double uterus and vagina with a
partially aplastic vagina.
 3. A double uterus and vagina with one
non-functioning uterus.
 • IV. Bicornuate uterus.
 1. Arcuate womb.
 2. Septate/subseptate womb
A bicornuate uterus with
pregnancy in the right horn
A 33–year–old G3 P2 was seen at the outpatient The Pfannenstiel’s
clinic at 27 weeks of gestation. All three incision was performed,
pregnancies were spontaneous. The and a baby girl
ultrasonography examination prior to the weighing 3,100 gr
cesarean section revealed a live fetus with (Apgar score = 8) was
breech presentation and an expected birth delivered at breech
weight of 3,300 gr. presentation, under
spinal anesthesia.
On inspection, the
uterus was seen to be
cylindrical in shape,
with a flattened left–
side wall and no left
tubal ostium, left tube,
or left ovary.
A rudimentary horn was
detected; it was fused
to the main cavity on
the left–posterior side
of the unicornuate
uterus. With it was an
attached normal right
tube and right ovary.
 V. Malformations of the fallopian tubes and
ovaries.
 1. Aplasia of the uterine appendages on one
side.
 2. Aplasia of fallopian tubes (one or both).
 3. The presence of additional fallopian tube.
 4. Aplasia of the ovary.
 5. Ovarian hypoplasia.
 6. The presence of accessory ovaries
Illustrating the abrupt discontinuation of the left fallopian
tube and the absence of the ipsilateral ovary
Partial accessory fallopian tube was observed on right side
which was arising from the ampullary portion of main
fallopian tube. Black arrow – main fallopian tube, white
arrow – accessory fallopian tube
 a congenital malformation characterized by a failure of
the Müllerian duct to develop, resulting in a missing uterus
and variable degrees of vaginal hypoplasia of its upper
portion.
 Müllerian agenesis (including absence of the uterus, cervix
and/or vagina) is the cause in 15% of cases of primary
amenorrhoea.
 A female with this condition is hormonally normal;
 development of secondary sexual characteristics including
thelarche and pubarche (pubic hair)
 karyotype will be 46,XX.
 one ovary is intact, if not both, and ovulation usually
occurs.
 Typically, the vagina is shortened and intercourse may be
difficult and painful.
 ultrasonography demonstrates a complete or partial
absence of the cervix, uterus, and vagina
 Classification
• Typical MRKH – Isolated
uterovaginal
aplasia/hypoplasia (64%)

• Atypical MRKH –
Uterovaginal
aplasia/hypoplasia with
renal malformation or
uterovaginal
aplasia/hypoplasia with
ovarian dysfunction – 24%

MRKH, vaginal agenesis • MURCS syndrome –

Treatment Uterovaginal
• create a functioning vagina, but in the aplasia/hypoplasia with
absence of a uterus no surgery is renal malformation,
currently available to make pregnancy skeletal malformation,
possible. and cardiac malformation
• Standard approaches use vaginal (12%)
dilators and/or surgery to develop a
functioning vagina to allow for
penetrative sexual intercourse.
 Isolated vaginal aplasia is a rare pathological
entity due to abnormal development of the
terminal part of Müller's canals.
 The vagina is formed during the third month
of the embryo's life
 Usually, vaginal aplasia is partial and the
distal, medial or cranial third is affected.
 Vaginal aplasia may also be associated with
cervical atresia.
 In patients with vaginal aplasia and a functioning
uterus, the there are no menstrual periods, but
every 3-4 weeks intervals appear pain in the
lower abdomen - constant aching or
severe,bursting, radiating to the lumbar region
and lower limbs.

 Pain is sometimes accompanied by vomiting,

fever.

 There may be complaints of frequent and painful

urination, difficulty in defecation.

 depending on the block level hematocolpos or

hematometer, the formation of hematosalping is
possible.
 vaginoplasty is performed, puncturing
the hematocolpos
 puncture needle and dissection of the entire
thickness of tissue with a scalpel along the needle in
transverse direction 3-4 cm long.
 Hematokolpos is emptied,the vaginal cavity is washed
with an antiseptic solution, the cervix is ​exposed.
 The next stage of the operation consists in stitching
the overlying departments vagina with underlying. A
tampon with vaseline oil is inserted into the vagina
for 2 days
 after removing it on 3rd day, the cavity is treated
with a solution of antiseptics, and the line of sutures
in the vagina with a solution potassium
permanganate.
 In case of complete aplasia of
the vagina and a functioning
rudimentary uterus -total laparoscopic
extirpation of a functioning rudimentary
uterus and colpopoiesis from the pelvic
peritoneum with laparoscopic assistance.

 In case of vaginal aplasia and a functioning

uterus with cervical aplasia or the cervical canal
(cervicovaginal aplasia) -reconstructive plastic
surgery by laparovaginal access:
 creation of the uterovaginal (utero-perineal)
anastomosis, with the introduction a polymer
stent that creates an anastomosis between a
functioning uterus and the vagina (or the
vestibule of the vagina)
 isa malformation in which the
paramesonephral ducts do not merge during
their development to form one uterus and
one vagina, but develop independently with
the formation of two uterus and two vaginas.
 Development options:
 doubling of the uterus and vagina without
disturbing the outflow of menstrual blood;
 doubling of the uterus and vagina with a
partially aplastic one vagina;
 doubling of the uterus and vagina with one
non-functioning uterus.
 Clinical feature in case of doubling of the
uterus and vagina without disturbance outflow
of menstrual blood:
 1) discomfort during sexual activity;
 2) sometimes after prolonged sexual intercourse
in one vagina, sexual contact in another vagina
can be very painful
 doubling of the uterus and partially aplastic
one vagina:
 1) painful menstruation after a few months or
 1-2 years after menarche due to the accumulation
of blood in the aplastic vagina;
 2) pain in the lower abdomen of a bursting
character;
 3) pus-like vaginal discharge due to the formation
of a fistulous tract between the vaginas -addition
of a secondary infection.
 In case of normal outflow of menstrual blood, a
patient may not present any complaints, the defect
is detected during gynecological examination, in
childbirth, during abortion, during examination
 Patients complaints of infertility in cases of sexual
intercourse in the vagina,communicating with a non-
functioning uterus

 In case of doubling of the uterus and vagina with a

partially aplastic one vagina, the percentage of
diagnostic errors is very high:
 despite pronounced clinical features, doctors are
confused by presence of menstruation, so pain during
menstruation are regarded as dysmenorrhea.
 Bursting pains below abdomen are the cause of
unjustified and ineffective surgical interventions:
appendectomy, ovarian resection, removal of the
fallopian tube, removal of the uterine appendages,
removal of one of the uterus, metroplasty.
 Treatment depends on the option of doubling the
uterus and vagina and the presence of complaints.
 Dissection of the septum in the vagina with doubling
of the uterus and vagina is performed according to
the following indications: dyspareunia, infertility
(after exclusion other factors).
 Doubling of the uterus and vagina with a partially
aplastic one vagina is an indication for opening the
hematocolpos, emptying and rinsing vaginal cavity
with an antiseptic solution, maximum excision of the
closed vaginal wall adjacent to a functioning vagina.
 In the postoperative period - prevent draining of the
walls of the opened vagina
 In the embryological sense, unicorniate
uterus is half a normal uterus,which can be
in two versions:
 it is presented in the form of one horn,
 the uterus is one-horned with an additional
rudimentary horn
 Distinctive features are an absence of
uterine fundus (half uterus), in the presence
of a rudimentary horn - aplasia of the kidney
on its side.
 The clinical symptoms are
manifested by primary
dysmenorrhea, tumor-like
formation in the small
pelvis, often - infertility,
miscarriage, adenomyosis
in the rudimentary horn
(even without endometrial
lining), a hematometer in
the rudimentary horn.

 An extremely formidable
complication of
unicornuate uterus with a
rudimentary horn can be
an ectopic pregnancy.
 Diagnosisis based on ultrasound of the pelvic
organs and kidney, if necessary, excretory
urography is performed. Final diagnosis is
established with simultaneous laparoscopy
and hysteroscopy.

 When a rudimentary horn is detected, it

should be removed together with the
fallopian tube on the side, if necessary -
correction of concomitant diseases.
 Planning pregnancy in 6 months.
 Bicornuate uterus - a developmental defect
in which splitting of the uterus is noted into
two horns or parts due to insufficient fusion
of paramesonephralducts.
 The division into horns can start from the
top, middle or bottom one third of the body
of the uterus, depending on the shape of the
bicornuate uterus, the horns always merge.
 A distinctive feature of the bicornuate uterus
is the presence of only one cervix.
 Clinical features
 In case of a saddle and
incomplete form there
may be no
complaints,malformation
of the uterus is detected
by chance during
examination and
treatment for
gynecological diseases.
 Typical complaints (20–
68% of cases) are
miscarriage, isthmic-
cervical insufficiency,
pelvic proposal of the
fetus, abnormalities of
labor.
 surgical correction only in case of impaired
reproductive function after excluding other
possible causes of miscarriage, infertility.
 - laparoscopic metroplasty
 a single uterine cavity is created with careful
layer-by-layer suturing of the walls of the
uterus.
 An anti-adhesion barrier is applied to the
seam area.
 Pregnancy is allowed no earlier than 8-10
months after surgery.
 is a form of a congenital malformation where
the uterine cavity is partitioned by a longitudinal
septum; the outside of the uterus has a normal
typical shape.
 Various options for an intrauterine septum:
 • full - up to the internal or external os(with the
formation of two cervical canals and two
hemicavities) - up to 4 cm long;
 • thin or thick;
 • on a wide base (in the form of a triangle);
 • one hemicavity is longer than the other.
 It is extremely rare - longitudinal septum of
upper third of vagina or full longitudinal septum.
 primaryor secondary infertility, miscarriage
pregnancy.

 Diagnostics
 Accurate diagnosis of the intrauterine
septum is difficult and is only possible with
combining modern diagnostic methods, when
the results are interpreted in the context of
a clinical situation: TVU and MRI,
hysteroscopy,laparoscopy
 - dissection of the intrauterine septum
 In the presence of a thin or incomplete or on a narrow base
septum is dissected in the first phase of the menstrual
cycle.

 In cases of a thick septum, on a broad base and / or other

unfavorable factors, hormonal preparation with a-GRh or
antigonadotropins is necessary for 1–2 menstrual cycles

 The intrauterine septum is dissected along its center from

apex to the base

 Resectoscope- sequentially, in short movements, with a

monopolar current of 60–80 W in cutting mode. The septum
is dissected until a single uterine cavity is formed.
 Important!
constant visualization of the orifices of the uterine tubes.
 Inthe postoperative period, patients are
prescribed COC contraception within 2-3
months (the time required for full
regeneration of the endometrium), after
which pregnancy is allowed.