3. Congenital malformations of the female genital tract
may be the result of a clear disturbance in one stage of
embryonic development, or result from disturbances in
more than one stage of normal formation. There are
therefore extremely wide anatomical variations and a
large number of combinations of congenital
malformations of the female genital tract.
4. The human female reproductive system is divided
into two :- internal genital organs and external genital
organs.
Internal genital organs are
Vagina
Cervix
Uterus
Fallopian tubes
ovaries
5.
6. The external genital organs are
Mons pubis
Labia majora
Labia minora
Clitoris
Hymen
Vestibular gland (Bartholin’s glands)
Urethral orifice
Vaginal orifice
Perineum
Anus
7.
8. Vagina: The vagina is a canal that joins the cervix
(the lower part of uterus) to the outside of the body. It
also is known as the birth canal.
Uterus (womb): The uterus is a hollow, pear-shaped
organ that is the home to a developing fetus. The
uterus is divided into two parts: the cervix, which is
the lower part that opens into the vagina, and the
main body of the uterus, called the corpus. The
corpus can easily expand to hold a developing baby.
A channel through the cervix allows sperm to enter
and menstrual blood to exit.
9.
10. Ovaries: The ovaries are small, oval-shaped glands
that are located on either side of the uterus. The
ovaries produce eggs and hormones.
11. Fallopian tubes: These are narrow tubes that are
attached to the upper part of the uterus and serve as
tunnels for the ova (egg cells) to travel from the
ovaries to the uterus.
Conception, the fertilization of an egg by a sperm,
normally occurs in the fallopian tubes. The fertilized
egg then moves to the uterus, where it implants into
the lining of the uterine wall.
12.
13. Cervix: The Cervix (the lower part of the uterus that
protrudes into the vaginal canal) has an orifice that
allows passage of menstrual flow form the uterus and
passage of sperm into the uterus.
14. Genotype of embryo 46XX or 46XY is established at
fertilization.
At 1-6 wks it is sexually indifferent or
undifferentiated stage; that is genetically female and
male embryos are phenotypically indistinguishable.
AT Week 7 begins phenotypic sexual differentiation.
Week 12 female or male characteristics of external
genitalia can be recognized.
Week 20 phenotypic differentiation is complete.
15. In utero photograph of a 56-day embryo showing continued
growth of the genital tubercle and elongation of the urethral
folds that have not yet initiated fusion. The genital swellings
remain indistinct.
16. Both male and female embryos have two pairs of genital
ducts
The mesonephric ducts (wolffian ducts) play an important
role in the development of the male reproductive system
The paramesonephric ducts (mullerian ducts) have a
leading role in the development of the female
reproductive system
Till the end of sixth week, the genital system is in an
indifferent state, when both pairs of genital ducts are
present
17.
18. Mullerian ducts form as buds of coelomic epithelium .
Grows downward & lateral to corresponding wolffian
ducts.
Turn inwards & crosses anterior to it joining its fellow
from opposite side.
19. Consists of
• Upper vertical part lateral to
wolffian duct → fallopian
tube.
Middle horizontal part
crossing walffian duct →
remaining part of fallopian
tube.
Lower vertical part fusing to
opposite part → uterus,
cervix, upper 1/3rd of vagina.
In forming the uterus, the
mullerian ducts fuses from
below upwards
20. REABSORPTION OF SEPTUM
After the lower Mullerian ducts fuse, a central septum
is present, which subsequently must be reabsorbed to
form a single uterine cavity and cervix. Failure if
reabsorption between 14th and 18th week is the cause of
septate uterus.
21. VAGINA
Develops in 3rd month of embryonic life.
From lower end of uterovaginal canal (mullerian duct)
& urogenital sinus.
Uterovaginal canal fuses with sinovaginal bulb
(develops from posterior aspect of urogenital sinus)
forming vaginal plate.
Later canalizes to form vaginal canal.
22.
23. Upper 1/3rd develops from mullerian duct –
mesodermal.
Lower 2/3rd develops from vaginal plate –
endodermal.
Incomplete breakdown of the junction between the
bulbs and the urogenital sinus proper leaves the
hymeneal membrane.
24.
25. By the fourth month:
Each germ cell, now become known as Oogonia, is
surrounded by a single layer of epithelial cells
The oogonia are transformed into primary oocytes as
they enter the 1st meiotic division and arrest in
prophase until puberty and beginning of ovulation.
Around the 20th week of gestation the ovary contains
about 7 million germ cells.
Degeneration and atresia begins around 20 weeks and
by birth approximately 20 million germ cells remain.
26. DEFINITION
Congenital malformations of the female genital tract
are defined as deviations from normal anatomy
resulting from embryonic mal development of the
Müllerian or paramesonephric ducts. Female genital
abnormalities often do not present until, or well after,
puberty.
27. DEFINITION
A uterine malformation is the result of an abnormal
development of the Müllerian ducts during
embryogenesis. Symptoms range from amenorrhea,
infertility, recurrent pregnancy loss and pain, normal
functioning depending on the nature of the defect.
28. Uterine malformation is estimated to be 6.7% in the
general population, slightly higher (7.3%) in the
infertility population and significantly higher in a
population of women with a history of recurrent
miscarriages (16%).
The septate uterus seems to be the most frequent
anomaly accounting for 30% to 50% of all the cases,
followed by the bicornuate uterus and unicornuate
uterus respectively.
29. Mullerian duct anomalies are categorized most
commonly into seven classes, according to the American
fertility society (AFS) classification.
HYPOPLASIA/ AGENESIS (CLASS I)
UNICORNUATE UTERUS (CLASS II)
DIDELPHYS UTERUS (CLASS III)
BICORNUATE UTERUS (CLASS IV)
SEPTATE UTERUS (CLASS V)
ARCUATE UTERUS (CLASS VI)
DIETHYLSTILBESTRIL
RELATED ANOMALY
(CLASS VII)
30. Hypoplasia includes entities such as uterine /cervical
agenesis or hypoplasia. The most common form is the
Mayer-Rokitansky-Kuster Hauser (MRKH) syndrome,
which is combined agenesis of the uterus, cervix and
upper portion of the vagina.
Patient have no reproductive potential aside from
medical intervention in the form of in vitro
fertilization of harvested ova and implantation in a
host uterus.
31.
32.
33. A unicornuate uterus is the result of complete or
almost complete arrest of development of one
mullerian duct.
If the arrest is incomplete, as in 90 % of patients,
a rudimentary horn with or without functioning
endometrium is present.
If the rudimentary horn is obstructed, it may come to
surgical attention when presenting as an enlarging
pelvic mass.
34.
35. If the contra lateral healthy horn is almost fully
developed, a full-term pregnancy is believed to be
possible.
Women with a unicornuate uterus have an increased
incidence of infertility, endometriosis, and
dysmenorrhea.
Implantation in the normal-sized hemiuterus is
associated with increased incidence of:
spontaneous abortion
preterm delivery
intrauterine fetal demise
36.
37. Didelphys uterus anomaly results from complete non-
fusion of both mullerian ducts and the individual
horns are fully developed and almost normal in size,
two cervices are inevitably present, a longitudinal or
transverse vaginal septum may be noted as well.
Each horn is almost a fully developed uterus, patient
is have been known to carry pregnancies to full term.
40. A bicornuate uterus results from partial non–fusion of
the mullerian ducts the central myometrium may
extend to the level of the internal cervical orifice of
the uterus (os) (bicornuate unicollis) or external
cervical os (bicornuate bicollis ).
The later is distinguished from didelphys uterus,
because it demonstrates some degree of fusion
between the two horns, while in classic didelphys
uterus, the two horns and cervices are separated
completely.
41.
42. A septate uterus results from failure of reabsorption
of the septum between the two uterine horns. The
septum can be partial or complete in which case it
extends to the internal cervical os. Histologically, the
septum may be composed of myometrium or fibrous
tissue.
Differentiation between a septate and a bicornuate
uterus is important, because septate uteri are treated
by using transvaginal hysteroscopy resection of the
septum.
43. Whereas if surgery is possible and indicated for the
bicornuate uterus, an abdominal approach is required
to perform metroplasty.
44. Marked increase in miscarriages that is likely due to
the abundant muscle tissue in the septum
Pregnancy losses in the first 20 weeks were reported
from the case studies
70 percent for bicornuate
88 percent for septate uteri
There is also an increased incidence of preterm
delivery, abnormal fetal lie, and cesarean delivery.
45.
46. An arcuate uterus has a single uterine cavity with a
convex or flat uterine fundus, the endometrial cavity,
which demonstrates a small fundal cleft or
impression.
The outer contour of the uterus is convex or flat this
form is often considered a normal variant, because it
is not significantly associated with the increased risks
of pregnancy loss and the complications found in
other subtypes.
47.
48. Several million women were treated with
diethylstilbestrol (DES) an estrogen analogue
prescribed to prevent miscarriage from 1945 to
1971.
The drug was withdrawn once its teratogenic effects on
the reproductive tracts of male and female fetuses were
understood.
The uterine anomaly is seen in the female offspring
of as many as 15 % of women exposed to DES
during pregnancy.
49. Female fetuses, which are affected, have a variety of
abnormal findings that include uterine hypoplasia and
a T- shaped uterine cavity.
Increased risk of developing
cervical intraepithelial neoplasia
small-cell cervical carcinoma
vaginal adenosis,
non-neoplastic structural abnormalities
50. Patient’s history and physical examination.
Pelvic ultrasound (us) with transabdominal.
Transvaginal imaging specially newer three –dimensional
(3D) sonographic technique offer relatively higher
sensitivity and specificity.
Hysterosalpingography under fluroroscopy to evaluate
the uterine cavity and tubal patency.
Magnetic resonance imaging (MRI).
According to a research study MRI is considered the
criterion standard for imaging uterine anomalies
51. Gynecological impacts
Infertility and dyspareneunia are often seen in vaginal
septum.
Dysmenorrhea in bicornuate uterus or crptomenorrhea.
Menorrhagia may also occur in bicornuate uterus.
Obstetrical impacts
More than 50% of women with malformed uterus will stay
completely asymptomatic from the obstetrical point of view.
Anatomical aspect and clinical outcomes can be different
and influenced by other associated anomalies like anomalies
of endometrium, vascularization, myometrial compliance,
cervical competence and others.
52. • The uterine malformation
are not the only factor
responsible for the
infertility, but it should be
taken into account that
some of them can increase
the risk of endometriosis.
INFERTILITY
54. Before pregnancy
Before pregnancy comprises the surgical treatment, if it
is possible and necessary.
Some of the malformations remain out of surgical
capabilities (unicornuate uterus, didelphys uterus) but
surgical procedures can prevent ectopic pregnancy.
The septate uteri (type V) are the only uterine
malformations, whose surgical treatment is relatively
simple by hysteroscopic excision. This treatment is
addressed to the symptomatic patient that had an
obstetrical complication before.
55. Hysteroscopic treatment can be proposed to patients
with some types of the bicornuate uteri and arcuate
uteri.
Two important facts must be taken into account in the
surgical treatment of uterine malformations.
The restoration of normal cavity anatomy is not
guarantee of a good obstetrical prognosis.
The number of pregnancies does not improve
obstetrical prognosis in women with untreated uterine
malformations.
56. During pregnancy
When the diagnosis of uterus malformation is made at
the beginning of pregnancy, the treatment can be only
preventive (setting at rest, sonographic monitoring of
the fetal growth and the cervical competence observed).
Abdominal metroplasty could be done either by
existing the septum or by incising the septum.
The success rate of abdominal metroplasty in terms of
live birth is 5% to 75%. Nowadays, hysteroscopic
metroplasty is done for this condition.
57. The fallopian tubes develop from the unpaired distal
ends of the mullerian ducts and extend outward from
the superolateral portion of the uterus.
The fallopian tubes are between 10 and 14 cm long
and normally end by curling around the ovary.
Disease may be asymptomatic or may be linked to
infertility.
58. Congenital anomalies of the fallopian tube include
Aplasia
Atresia
Hypoplasia ( very long or thin)
Accessory horn or ostia and tubal diverticulum
Complete absence of the fallopian tube
A number of embryonic cystic remnants.
It may cause infertility or ectopic pregnancy
59. The congenital anomaly of the ovaries include
congenital absence of ovary and the development
ovarian cyst.
Accessory ovary (division of the original ovary into
two) also comes under this condition.
Rarely, supernumerary ovaries may be found in the
broad ligament or elsewhere .
60. Para ovarian cysts are not actually ovarian, they are
usually located alongside the ovaries or on the fallopian
tubes, but they are often hard to distinguish from the
ovarian cysts.
The cyst can grow to be very big and even extend to the
upper abdomen. Their size and systems do not correspond
to the hormonal cycle like other ovarian cysts do.
Para ovarian cysts can tear, bleed, rupture and become
infected. It account for 10% to 20% of all adenexal masses
and are relatively uncommon in children. They are more
common in women 30-40 years of age.
61. Paramesonephric cyst
Hydatid cyst of morgagni
Wolffian cyst
Kobelt cyst
Cyst of the organ of rosenmuller
62.
63. The outer end of the wolffian (Gartner’s) duct may be
pea sized, cystic and pedunculated, and attached to
the outer end of the vaginal tube.
Gartner’s duct cyst are the remnants of the wolffian
duct and they are rarely seen in adulthood .
64.
65. The paramesonephric duct (or mullerian duct) forms
the fallopian tube at about 9 weeks of gestation.
Multiple invaginations near the ostium of the tube
become the fimbriae. Any secondary invegination that
does not connect may form a blind sac and this enlarges
to form a Paramesonephric cyst.
69. Narrow introitus condition is revealed after the
marriage. The patient complains about dyspareunia.
Treatment is done by manual stretching under general
anaesthesia or by perineoplasty.
70. Transverse vaginal septum (TVS) is formed when the
tissue between the vaginal plate and fused mullerian
ducts fail to reabsorb.
This anomaly divides the vagina into two segments,
reducing its functional length. The most common
locations are the midvagina at rate of 40% and the
inferior vagina at a rate of 14%.
The TVS is one of the most rare mullerian duct
anomalies, with an appropriate frequency of one
case in 70,000 females.
71.
72. Diagnosed in utero during third trimester with
transabdominal sonography.
Abdominal ultrasonography of the pelvis can also
detect hydro/mucocolpos.
MRI should also be performed to make a definitive
diagnosis.
73. Surgical management of TVS fetus, neonates and
infants –
When third trimester ultrasonography finding lead to
the diagnosis, early delivery and drainage of the
obstructed vagina and uterus are indicated.
In infant, vaginal septum is usually thin and can
corrected without extensive procedures. Surgical
excision of the obstructed septum through a perineal
approach.
75. Initial and ongoing assessment of the patient includes-
Level of consciousness
Vital signs
Oxygen saturation
Skin color and temperature
Comfort
Fluid balance
Dressings and drains
76. Ongoing postoperative nursing interventions includes
Managing pain
Appropriate positioning
Encouraging deep breathing and coughing exercise
Promoting leg excise and ambulation
Maintain adequate hydration
Promoting urine elimination
Provide bowel care.
Surgical aseptic technique is used when changing
dressing on surgical wound.
Clinical follow–up
77. As the vagina is largely derive from the mullerian
ducts, lack of fusion of the two ducts can lead to the
formation of a vaginal duplication.
Lack of absorption of the wall between the two ducts
will leave a residual septum, leading to a ‘double
vagina’
78.
79. Physical examination.
Gynecologic ultrasonography.
Pelvic MRI or HSG.
Laparoscopy and /or hysteroscopy may be indicated in
some patients, the vaginal development may be
affected.
80. Surgical intervention depends on the extent of the
individual problem with a didelphic uterus.
With this a uterine septum can be resected in a simple
outpatient procedure that combines laparoscopy and
hysteroscopy.
This procedure greatly decreases the rate of miscarriage
for women with this anomaly.
81. IMPERFORATE HYMEN
When no hymeneal opening is present, a membrane
covers the area of the hymen and is called an
imperforate hymen.
An imperforate hymen needs to be surgically
corrected.
Diagnosis is either in the newborn baby or at the time
of menarche (the first period).
82. It is an obstructive anomaly of the female genital tract,
but estimates of its frequency vary from 1 case per
1000 population.
ETIOLOGY
Imperforate hymen result from abnormal or incomplete
embryologic development.
PATHOPHYSIOLOGY
By the 5th month of gestation, the canalization of the
vagina is complete. The hymen itself is formed from
the proliferation of the sinovaginal bulbs becoming
perforate before or shortly after birth.
83. Careful physical examination.
Abdominal and pelvic ultrasonography and MRI.
Transrectal ultrasonography may help in delineating
complex anatomy.
Laparoscopy has been recommended to evacuate
pelvic and intra-abdominal endometrial material
generated because of retrograde menstruation.
84. Surgical intervention for imperforate hymen should
require only one definitive procedure to evacuate the
retained secretions and to ensure the maintenance of
patency.
An elliptical excision of the membrane is performed
of choice.
An elliptical excision of the membranes is performed
close to the hymenal ring , followed by evacuation of
the obstructed material ,this technique is considered
to be most effective in definitive treatment .
85.
86. After the appropriate diagnostic studies are
performed , an outpatient procedure to be done under
general anaesthesia is scheduled .
Distinguishing an imperforate hymen from a
transverse vaginal septum is important , because
later it requires a relatively extensive procedure to
reconstruct a functional vaginal tract and because it
has complications in terms of reduced fertility,
87. The retained secretion are typically sterile unless
previous manipulation like needle aspiration has
resulted in infection.
Therefore ,prophylactic antibiotics are not usually
required.
88. For postoperative analgesia, acetaminophen or non-
steroidal anti-inflammatory drugs (NSAIDS), such as
ibuprofen, are given .
Follow up for 6 to 8 weeks to allow the patient to re-
establish a menstrual cycle .Finding on evaluation of
the patient’s menstrual cycle determine the need for
further evaluation .
89. Microperforate hymen is essentially an imperforate
hymen with a very small hole within it.
The hole may be large enough for mucus or blood to
come through the hymnal opening, but instead of
having a regular menstrual period lasting 4 to 7 days.
The women may have a period, which lasts longer. This
is due to the fact that the blood cannot come out at a
normal rate.
90. Septate hymen refers to a band extra hymenal tissue,
running vertically in the area of the normal hymen.
A hymenal septum may interfere with a women’s
ability to insert a tampon or she may find that she can
insert the tampon.
Once it expends with blood, she cannot remove the
tampon.
91. Vaginal agenesis (agenesis means failure to develop
or grow) is a congenital disorder of the reproductive
system, which affects a relatively small proportion of
women, one in 5,000 to 7,000 or about 0.025 %.
It occurs when the vagina, the muscular canal
connecting the cervix of the uterus to the vulva, stops
developing during pregnancy.
92. Sufferers have a short vagina of may be 3.5cm or 1.5
inch length, but there might be no vagina at all.
A study shows 30% of patient with vaginal agenesis
have kidney abnormalities. Approximately 12% of
patients with vaginal agenesis have skeletal
abnormalities that affect the spine, ribs or limbs.
93. Absent vagina
Absence of menstrual period
Absent uterus and other reproductive organs
Kidney abnormalities
Skeletal abnormalities
Hearing loss
94. Since outward genital appear normal ,vaginal
agenesis is not usually diagnosed until puberty.
Typically ,an adolescent girl 15 to 18 years old,
consult a pediatrician or gynaecologist when her
period does not start.
The condition may also be discovered in infancy or
childhood while investigating kidney, skeletal or
other abnormalities ,such as the absence of an anal
opening .
95. Ultrasound (US)-reveals if the uterus and ovaries are
present and the presence and location of kidneys.
MRI – shows a more detailed picture of the
reproductive tract and kidneys.
96. Most young women are treated in their late teens or early
20s .others may wait until they are older and sexually
active.
Treatment is not urgent, but it is usually necessary before
sexual intercourse.
Self –dilation
Surgery (vaginoplasty)
Skin graft
Bowel vaginoplasty
Counseling
97. Self –dilation
Patient presses a small rod (dilator ) against the skin
or the small vagina for 15 to 20 minutes per day.
This is often done after bathing, when skin is more
pliable progressively larger dilation are used to
expand the vagina.
Several months may be required to obtain the desired
result.
98. Vaginoplasty
Are used to create a functional vagina. These
treatment are usually delayed until the patient
possesses the maturity to handle follow –up dilation.
Skin graft
mclndoe procedure - Most commonly performed
procedure uses a skin graft from the buttocks the
surgeon makes an incision ,where the vagina would
normally develop and inserts the graft to create a
vagina .
99. A mold is placed in the newly formed vagina for 7 days.
Following surgery, patients use a vaginal dilator, which is
like a solid ,large tampon ,it is removed for urination,
bowel movements or sexual intercourse .
After a time , patients use the dilator only at night.
Bowel vaginoplasty
In bowel vaginoplasty, a portion of the sigmoidal colon is
diverted to an opening in the genital area. Creating a new
vagina. The remaining colon is then reconnected and
potential complications include bowel leakage at the
attachment site and mucus drainage for up to 1 year after
the surgery .
100. • Bed rest in upright and flat position for one week.
• Antibiotics.
• Low residue diet.
o Vaginal cavity irrigated with warm saline.
o Inspection of cavity to determine the take of graft.
101. Fear related to possible outcome of infertility.
Situational low-esteem related to inability to
conceive or feeling of failure.
Altered sexuality patterns related to structured
efforts to conceive or loss of spontaneity.
Ineffective coping related to unmet expectations or
feeling of loss
Deficient knowledge related to diagnostic and
treatment procedure
102. Reproductive outcome of septate uterus following
hysteroscopic septum resection
A retrospective study to evaluate the reproductive
outcome following hysteroscopic septum resection in
patients with primary and secondary (recurrent
pregnancy loss [RPL] and bad obstetric history [BOH])
infertility.
103. Hysteroscopic septum resection was performed on 26
patients with a history of either recurrent pregnancy
loss, BOH or infertility.
The septum resection was performed using a bipolar
versa point system. Reproductive performance of these
patients after septum resection was analyzed. The main
outcome measures were clinical pregnancy and live
birth rates.
104. Results: Hysteroscopic septum resection was
performed on seven patients with the history of
secondary infertility. Post operatively, the pregnancy
rate was 86%, and the live birth rate was 67%. After
septum resection in 19 primary infertile patients,
(32%) patients conceived which resulted in live birth
rates of 67% .
105. Conclusion: Hysteroscopic septum resection using
bipolar versa point system is an effective and safe
approach for the removal of septum. Hysteroscopic
septum resection in women with septate uterus
significantly improves the live birth rates and future
fertility is not impaired