MULLERIAN DUCT

ANAMOLIES
DR. SONAM PANDAY
1st YEAR RESIDENT
21st MAY 2023
 Objectives

- Introduction to development of gonads

- Fate of mullerian duct
- Classification of mullerian agenesis
- Surgical method of correction of mullerian agenesis
- Effects of mullerian agenesis in female reproductive life
 The epithelium of the genital ridge proliferates, and epithelial
cells penetrate the underlying mesenchyme. Here they form a
number of irregularly shaped cords, the primitive sex cords .
If the embryo is genetically male, the primordial
germ cells carry an XY sex chromosome complex

Under influence of the SRY gene on the Y

chromosome, which encodes the testis-
determining factor, the primitive sex cords continue
to proliferate and penetrate deep into the medulla
to form the testis or medullary cords.

Toward the hilum of the gland, the cords break up

into a network of tiny cells strands that later give
rise to tubules of the rete testis

During further development, a dense layer of

fibrous connective tissue, the tunica albuginea,
separates the testis cords from the surface
epithelium
• In female embryos with an XX sex chromosome
complement and no Y chromosome, primitive sex cords
dissociate into irregular cell clusters

• These clusters, containing groups of primitive germ cells,

occupy the medullary part of the ovary. Later, they
disappear and are replaced by a vascular stroma that
forms the ovarian medulla

• The surface epithelium of the female gonad, unlike that of

the male, continues to proliferate. In the seventh week, it
gives rise to a second generation of cords, cortical cords,
which penetrate the underlying mesenchyme but remain
close to the surface

• In the third month, these cords split into isolated cell

clusters. Cells in these clusters continue to proliferate and
begin to surround each oogonium with a layer of epithelial
cells called follicular cells. Together, the oogonia and
follicular cells constitute a primordial follicle
 Genital duct
• Initially, both male and female embryos have two pairs of genital ducts:
- mesonephric (Wolffian) ducts
- paramesonephric (Müllerian) ducts
• The genital ducts of male include vasa efferentia, duct of epididymis, vas deferens, and
ejaculatory duct
• The genital ducts of female include fallopian tube, uterus, and vagina
• In male the mesonephric ducts form the definitive genital ducts and paramesonephric
ducts mostly disappear, while in female paramesonephric ducts form the female genital
ducts and the mesonephric ducts mostly disappear.
• The paramesonephric duct arises as a longitudinal
invagination of the epithelium on the antero- lateral
surface of the urogenital ridge

• Cranially, the duct opens into the abdominal cavity

with a funnel-like structure Caudally, it first runs
lateral to the mesonephric duct, then crosses it
ventrally to grow caudomedially

• In the midline, it comes in close contact with the

paramesonephric duct from the opposite side.

• The caudal tip of the combined ducts projects into the

posterior wall of the urogenital sinus, where it causes
a small swelling, the sinus tubercle
• The paramesonephric duct is divided into three parts:(a) cranial vertical part (b)
middle horizontal part and (c) caudal vertical part.

• In females, the paramesonephric ducts form:

1. Uterine tubes
2. Uterus
3. Upper part of vagina

• 1. Development of uterine tubes: The uterine tube, on each side, is formed by

unfused part (the cranial vertical and middle horizontal part) of the paramesonephric
duct.
• The fallopian tube elongates and becomes tortuous.
• 2. Development of the uterus : The uterus is formed from the uterovaginal canal
(fused caudal vertical parts of the paramesonephric ducts)

• The primitive uterus soon differentiates into two parts: (a) body and (b) cervix.
• In fetus, cervix is larger than the body of uterus.
• The initial angular junction between the two paramesonephric ducts becomes a
convex dome and forms fundus of uterus
3. Development of the vagina :
• The vagina develops - As solid caudal tip of the uterovaginal canal comes in contact
with urogenital sinus, it induces formation of two outgrowths (evaginations) from the
dorsal wall of the definitive urogenital sinus called sinovaginal bulbs.
• The endodermal cells of the sinovaginal bulbs proliferate rapidly and form solid plate
of cells called vaginal plate.
• The cells from tip of the uterovaginal canal (mesodermal) also proliferate and add to
the vaginal plate.
• The central cells of vaginal plate breakdown and by the fifth month the plate is
completely canalized to form lumen of the vagina

• The wing-like expansion of vagina around the cervix forms fornices of vagina
• The vagina remains separated from the phallic part of definitive urogenital sinus by a
thin plate of tissue called hymen, which consists of a thin layer of vaginal cells
superiorly and epithelial lining of urogenital sinus inferiorly

• The hymen usually develops a small opening in its center during perinatal life
 Vestigeal remnants of mesonephric duct
(a) Epoophoron: longitudinal duct running parallel to the uterine tube (persistent cranial
part of the mesonephric duct) are present vertically above the ovary in the mesovarium
(b) Paroophoron: A few mesonephric tubules are detached from the mesonephric duct and
persist as small blind tubules between the ovary and uterus
(c) Gartner’s duct: A part of the mesonephric duct persists and lies between the two layers
of broad ligament by the side of the body of uterus which open into the cervix or vagina. A
collection in upper lateral vaginal wall is a gartner cyst
A gartner cyst is mostly asymptomatic ,simple cyst and is
Treated by simple excision

This corresponds to the vas deferens in males

Mullerian duct anamolies
INCIDENCE
• Normal /fertile women:1.5-4.5%
• Infertile women:3-6%
• Women with recurrent miscarriage:5-10%
Distribution
• Bicornuate: 39%
• Septate:34%
• Didelphic:11%
• Arcuate:7%
• Unicornuate :5%
• Hypoplastic/Aplastic/solid and other forms:4%
 Absence or Incomplete Development of Both
Müllerian Ducts (Class I)
• Pathology
- Complete failure in development of the Müllerian ducts results in absence of the
fallopian tubes, uterus, cervix and 2/3rd of the vagina
- vulva is likely to be normal and there may be a depression of variable depth
representing the lower (urogenital sinus) part of the vagina
- Karyotype :- normal
Clinical features
• Patients usually present between 15 and 18 years of age with primary amenorrhoea
• Growth and secondary sexual characteristics are normal

• On local examination :- patients may have a small vaginal pouch developed

• On rectoabdominal examination:- uterus cannot be palpated

• Ultrasound is inconclusive shows absence of the uterus and demonstrates the ovaries
• MRI or laparoscopy is required. MRI is the ideal method for demonstrating uterine
malformations.
 Mullerian Agenesis/Hypoplasia
• Absence or hypoplasia of uterus proximal to vagina and in some cases
fallopian tube too
• Two variants
- Partial :rare
- Complete : more common (MAYER-ROKITANSKY-KUSTER-HAUSER
SYNDROME)
Mayer-Rokitansky-Kuster-Hauser
Syndrome

• Congenital absence of uterus & vagina, small rudimentary uterus

may be present
• Normal ovarian function including ovulation
• Genotype - 46xx
• Phenotype: female
• Associated with other congenital anomalies - (skeletal, renal)
• Usually diagnosed at puberty with c/o primary amenorrhea

• Age appropriate secondary sexual character

• Normal development of breast, body, proportionate hair distribution,

external genitalia

• Vaginal vault can be either absent completely or short vaginal port

can be present

• Hormonal profile : Normal

• USG
- Absence of uterus & fallopian tube with normal ovaries

• MRI
- Uterus & vagina absent
- Rudimentary uterus can be seen
- Coexisting renal abnormality identified
 Treatment
Aim:- To create a neovagina

1. Non surgical
Frank (1938) described non surgical method to create neovagina using
sequential application of wider & longer dilator
- Series of graduated dilator dilate vaginal space
- Creates a functional vagina within 3-6 months
2. SURGICAL
- Without dissecting cavity-
• 1. Williams vulvovaginoplasty(1964)
• 2. Vecchietti procedure(1965)

- Dissecting cavity & lining with graft-

• 1. Mc Indoe operation
- Dermis graft
- Amnion graft
Mc Indoe operation
• Most common

• Most satisfactory results - Procedure of choice

• Three important principles-

- Dissection of adequate space between bladder & rectum
- Inlay a split thickness skin graft
- Prolonged dilatation during the contractile phase of healing
Technique
• Taking the graft :- After a careful pelvic examination is performed under
anesthesia to verify previous findings, the patient is positioned for taking a skin
graft from the buttocks

• Skin graft taken of 0.018 inch thick, 8-9 cm wide, 16-20 cm in length

• The graft is placed between two layers of moist gauze and the donor sites are
dressed
Creating the neovaginal space
• The patient is placed in the lithotomy position, and a
transverse incision is made through the mucosa of the
vaginal vestibule

• The space between the urethra and bladder anteriorly

and the rectum posteriorly is dissected until the
undersurface of the peritoneum is reached

• With blunt dissection, on each side of a median raphe

channel is created and then dilating each channel
with Hegar dilators or with finger dissection

• The median raphe is then divided, thus joining the

two channels.
• The dissection should be carried as high as
possible without entering the peritoneal
cavity and without cleaning away all tissue
beneath the peritoneum

• A split-thickness skin graft will not take well

when applied against a base of thin
peritoneum

• All bleeders should be ligated by clamping

and tying them with very fine sutures as it is
essential that the vaginal cavity be dry to
prevent bleeding, beneath the graft
Preparing the vaginal form
• Vaginal form is mold shaped obtained from
a foam rubber block for the vaginal cavity
and covered with a condom

• The foam rubber is gas sterilized in blocks

measuring approximately 10 × 10 × 20 cm
The block is shaped with scissors to
approximately twice the desired size,
compressed into a condom and placed into
the neovagina
• The form is left in place for 20 to 30
seconds with the condom open to allow
the foam rubber to expand and conform
to the neovaginal space

• The condom is then closed, and the

form is withdrawn.

• The external end is tied with 2-0 silk,

and an additional condom is placed over
the form and tied securely
Post operative management

• Bed rest in upright and flat position for one week

• Antibiotics
• Low residue diet
• Replacing with new form
- Removed after 7-10 days. vaginal cavity irrigated with warm saline
- Inspection of cavity to determine the take of graft
- New form applied
- Applied continuously for 6 wks except at the time of urination &
defecation
• Next 12 months-new form applied only during night
Complication

• Post operative infection

• Intra/post-op hemorrhage
• Post operative fistula formation with solid mold(due to pressure
necrosis)
- rectovaginal-more common
• Failure of graft taken up
• Later granulation formation
• Malignant transformation
Williams Vulvo vaginoplasty
• Construction of a perineal bridge to help contain the vaginal mold was
a routine part of the operation described by McIndoe

• Advantages-
- Technique is simple
- Less local complication
- Early recovery

• Operation of choice for unsatisfactory Mc Indoe operation

• A horseshoe-shaped incision is made in the
vulva to extend across the perineum and
up the medial side of the labia to the level
of the external urethral meatus

• The initial mucosal incisions are made as

close to the hairline as possible and
approximately 4 cm from the midline

• After complete mobilization, the inner skin

margins are sutured together with knots
tied inside the vaginal lumen
• A second layer of sutures approximates
subcutaneous fat and perineal muscles for
support

• The external skin margins are approximated

with interrupted sutures

• The procedure is performed properly, it should

be possible to insert two fingers into the pouch
to a depth of 3 cm

• The patient is confined to bed for 1 week to

avoid tension on the suture line

• Examinations are avoided for 6 weeks, at which

time the patient is instructed in the use of
dilators
 VECCHIETTI OPERATION
• The Vecchietti procedure is a surgical technique for the treatment of vaginal
agenesis that constructs a dilatation-type neovagina in 7 to 9 days

• The procedure uses specialized equipment including a traction device, a ligature

carrier, and an acrylic shaped olive

• The operative phase involves positioning the olive at the perineum and the traction
sutures extraperitoneally. Classically performed through a Pfannenstiel incision, the
ligature carrier introduces the suture into a newly dissected vesicorectal space. The
olive is threaded with suture at the perineum, and the suture is reintroduced at the
abdomen
Absence or Incomplete Development of One Müllerian
Duct (Class II)
Pathology
• Absence of one Müllerian duct results in a unicornuate uterus with only one
fallopian tube (Class II)
• cervix and vagina may be normal in appearance and function
Symptoms

• A unicornuate uterus causes few symptoms and is usually only discovered by

chance or as a result of pregnancy complications.
• If it happens that the patient has dysmenorrhea UNILATERAL
• This type has the worst pregnancy outcome and is associated with abortions and
preterm labour
• Non communicating rudimentary horn leads to retrograde menstruation causing
endometriosis
 Diagnostic Signs
• Uterus leans well to one side of the pelvis which cannot easily be straightened should
always be suspected as being unicornuate
• Hysterosalpingographs are helpful but do not always distinguish between true and
apparent unicornuate deformity

Treatment
• No treatment is indicated for the true unicornuate
uterus
• The rudimentary horn of an apparent unicornuate
uterus may have to be excised if it causes symptoms
UNICORNUATE UTERUS

• Indication of operation- Presence of endometrium in the accessory

horn

• Treatment of choice- Laproscopic hemihysterectomy of rudimentary

horn

• Surgical reconstructive procedures do not improve obstetrical

outcome
 Uterus Didelphys (Class III)
Pathology

• If the two Müllerian ducts remain separate, the two halves of the uterus remain
distinct and each has its own cervix
DIDELPHYS
Usually associated with good reproductive outcome

In pregnancy , however there is increased incidence of breech

presentation and preterm labour
Increased risk of retrograde menstruation leading to increased risk of
endometriosis

Requires no treatment
 Bicornuate Uterus (Class IV)
Pathology
• In this condition only the lower parts of the ducts fuse, leaving the cornua separate
• The cervix and vagina may be single or double
MRI SHOWING BICORNUATE UTERUS
BICORNUATE UTERUS
• This is associated with recurrent abortions , which forms an indication for
unification surgery
• In pregnancy it is associated with breech/ transverse lie and preterm labour
• Treatment :unification surgery
- strassman’s{TREATMENT OF CHOICE}
- jone’s metroplasty
STRASSMANN METROPLASTY

• Incision given in medial side of each hemicorpus, deep enough to enter

the endometrial cavity

• The incision extends from the superior aspect of each horn, near the
interstitial region of the fallopian tubes, to the inferior aspect of the
uterus

• The goal is to achieve a single endocervical canal. If 2 cervices are

present, their unification is not recommended
• Apposition of the myometrium

• After resecting the wedge, the myometrial edges naturally evert.

Apposition of the opposing myometrium is achieved using interrupted
vertical figure-8 sutures along the posterior and anterior uterine walls

• The final layer is closed using continuous subserosal sutures, without

exposing any suture material to the peritoneal cavity

• Transvaginal dilatation of the cervix is performed, assuring proper

endometrial cavity drainage
Jones Metroplasty

• Abdominal approach
• Wedge shaped incision given at the top of fundus within 1 cm of
insertion of tubes
• Uterine septum excised as wedge
• Unification of two halves of uterus is done in three layers with
interrupted stitches
Septate and Subseptate Uterus (Class V)

• Pathology:-
The uterus is outwardly normal but contains a complete or incomplete septum
which reflects a failure in breakdown of the walls between the two ducts
MRI SHOWING SEPTATE UTERUS
• On hysterosalpingography , both septate and bicornuate uterus looks
similar.
septate Bicornuate
Angle <75 deg Angle >105 deg:obtuse
Fundus flat Fundus concave
Distance b/w horns<4cm Distance b/w horns> 4cm
Endometrium to fundus >5mm Endometrium to fundus< 5mm

• Best way to distinguish b/w septate and bicornuate uterus: Diagnostic

laparoscopy + hysteroscopy
• (MRI :Best imaging method)
SEPTATE UTERUS
• This type requires surgery more commonly than bicornuate as it is
associated with infertility and recurrent abortions
• In pregnancy it is associated with transverse lie
 SEPTATE UTERUS
• Indication of surgery-
• Recurrent spontaneous abortion
• History of preterm labour

• Procedure of choice- Transcervical hysteroscopic lysis of uterine septum

with concurrent laparoscopy
• Preoperatively GnRH analogues for 2 months are given to induce
endometrial atrophy.
• The laparoscope is placed
• The hysteroscope is inserted to the level of the external os
• Using an Operating continous flow hysteroscope, the septum may be divided
with diathermy scissors, a rectoscope wire or knife electrode or the Nd YAG laser
• Dissection of the septum is complete when :-
- the hysteroscope can be moved freely from 1 tubal ostium to the other
- the tubal ostia are visualized simultaneously
- bleeding occurs from small vessels at the fundal myometrium is visible(septum is
relatively avascular)
• Main complication is Uterine perforation and Fluid overload
• Post op management-
• Placement of intrauterine device for a month -controversial.
• Conjugated estrogen and progesteron added to facilitate
epithilelization.
• After one month follow with HSG & Hysteroscopy
Arcuate Uterus (Class VI)

Pathology
This is a flat-topped uterus in which the fundal bulge has not developed after fusion
of the ducts
If only the exterior of the uterus is affected, the fundal myometrium is abnormally
thin.
DES-related Anomalies (Class VII)

Pathology:-
• Several characteristic anomalies have been described in women who were
exposed to diethylstilboestrol (DBS) in utero.
- Benign vaginal adenosis and cervical hoods
- T-shaped uteri,
- wide lower segments and constriction bands
- Vaginal clear cell adenocarcinoma
- Unlike all other congenital uterine malformations, the DES uterus is not
associated with an increase in renal anomalies.
COMPLICATIONS OF MULLERIAN
ANOMALIES
1. DURING PREGNANCY

• Abortion
• Cornual pregnancy - with inevitable rupture around 16 th week - if
pregnancy occurs in the rudimentary horn
• Malpresentations (transverse lie in arcuate or subseptate., breech in
biconuate, unicornuate or complete septate uterus)
• Preterm labour
2. DURING LABOR

- Weak uterine action (Prolonged or obstructed labour)

- Post partum hemorrhage
- Adhesion of the placenta
(The placenta, if it is formed on the septum, may be adherent and may cause post
partum hemorrhage)

- Uterine rupture due to its poor development

- when a fetus is accommodated in one horn of a double uterusand the empty horn had
grow owing to the hormonal influences of the pregnancy, and its size and position will
cause obstruction during labour so Caesarean section would be the method of
delivery
•
3. Gynecological:

• Infertility
• Dyspareunia are often related in association with vaginal septum
• Dysmenorrhoea in bicornuate uterus due to
• cryptomenorrhoea (pent up menstrual blood in rudimentary horn)
• Menorrhagia - due to increased surface area in bicornuate uterus.
CONCLUSION
• Müllerian anomalies are a morphologically diverse group of developmental
disorders that involves the internal female reproductive tract

• Establishing an accurate diagnosis is essential for planning treatment and

management strategies

• The surgical approach for correction of müllerian duct anomalies is specific

to the type of malformation and may vary in a specific group

• For most surgical procedures, the critical test of the procedure's value is the
patient's postoperative ability to have healthy sexual relations and achieve
successful reproductive outcomes
REFERENCES
• Sadler, T. and Langman, J., 2004. Langman's medical embryology 13th edition
• Inderbir Singh’s Human Embryology 11th Edition
• Te Linde's Operative Gynecology. Philadelphia :Lippincott, 1977, 10th edition
• Jeffcoate's Principles of Gynecology 8th deiction
THANKYOU!!!