Mullerian anomalies

MULLERIAN DUCTS
Paired ducts derived from intermediate
mesoderm.
Known as paramesonephric duct.
Named after Johannes peter mullero-described
them in 1830.

EMBRYOLOGICAL
DEVELOPMENT
Appear between 5-6 wks.
Derived from intermediate mesoderm lateral
to wolffian duct as invagination of dorsal
coelomic epithelium.
Depends on absence of male determining
factor which is present in Y-chromosome.

Cont.
Female development called Basic
developmental path of the human embryo –
requires not estrogen but the absence of
testosterone.
Ambisexual period persist upto 8 wks,
thereafter one type of duct system persist &
other disappears.

Development of Genital Ducts
• 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

Cont.
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.

 Consists of-
 Upper vertical part lateral to
wolffian duct → fallopian
tube.
 Middle horizontal part
crossing wolffian 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

Cont.
VAGINA
Develops in 3rd month of embryonic life.
From lower end of uterovaginal canal (mullarian
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.

Cont.
 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.
.

The Development of the Gonads
The phase of indifferent gonads (Genital Ridge):
The germ cells (primordial germ cells)
migrate to the genital ridge by the
6th week.
The germ cells influence development
of the gonad into ovary or testes
The covering epithelium proliferates
and sends cluster of cells into the
underlying mesoderm known as the
cells of the sex cords.
Differentiate into testis
or ovaries depending on
the presence or absence
of the Y chromosome in
the germ cells.
Ovarian
Differentiation
Occur in the the absence of Y
chromosome and SRY protein
it occurs two weeks later
(about the 8th week)

The cortical zone that contain the germ cells develop to a
much greater extent, while the medulla regress.
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.

Three main principles governs the practical
approach to mullerian anomalies.
1. Mullerian and wolffian ducts are so closely related
embryologically that mullerian anomalies are
commonly associated with anomalies of kidney and
ureter.
2. Development of gonads is separate from ducts so
normal ovaries are present in mullerian anomalies.
3. Mullerian anomalies may be associated with
anomalies in sex chromosome make up of individual.

Complete formation & differentiation of
mullerian duct into female reproductive
system depends on completion of 3 phases of
development as follows-
□ Organogenesis
□ Fusion
□ Septal resorption

ORGANOGENESIS
One or both mullerian duct may
not develop fully-Uterine
agenesis or hypoplasia
Unicornuate uterus.
 FUSION
Lateral fusion- process during
which lower segment of paired
mullerian duct fuse – Uterine
didelphys or Bicornuate
uterus,Arcuate uterus.

Cont.
 Vertical fusion- Fusion of ascending sinovaginal
bulb with descending mullerian duct – Transverse
vaginal septum, Imperforate hymen.
SEPTAL RESORPTION – after fusion central septum
persist later resorps to form single uterocervical
cavity – Septate uterus

INCIDENCE
There are irregularities in
incidence & prevalence rate because of –
– Non standardization of classification system.
– Non-uniform diagnostic modalities.
– Different study population.

Cont.
 Normal / Fertile women-1.5-4.5%
 Infertile patients – 3-6%
 Women with recurrent miscarriage -
 5-10%
DISTRIBUTION
 Septate uterus- 35%
 Bicornuate-26%
 Arcuate uterus-18%
 Unicornuate uterus-10%
 Didelphys uterus-8%

Clinical Presentation
Uterine anomalies are often an incidental diagnosis while
seeing the patient for a different complaint.
The Patient can present with:
 Pelvic pain (cyclic or non-cyclic)
Dysmenorrhea
Abnormal vaginal bleeding
 Vaginal pain
Uterine rupture during pregnancy
 Recurrent pregnancy loss
Patient may have a concurrent renal abnormalities

DIAGNOSIS
Physical examination
 Gynecologic Ultrasonography
 Pelvic MRI
 Hysterosalpinography
Laparoscopy and/or Hysteroscopy may be
indicated.

HYSTEROSALPINGOGRAM-
 Primary imaging modality.
 Normal uterus- typical trigone, configuration.
 Diagnostic criteria.
Intercornual distance
• Distance between the distal ends of horns.
• Septate uterus- <2 cm.
• Didelphys uterus- >4 cm.
• Normal uterus (indeterminate in an abnormal
cavity)- 2-4 cm

Cont.
Intercornual angle
• Angle formed by the most medial aspect of two
uterine hemicavities.
• Septate uterus- <60º
Bicornuate uterus- >105º
T- shaped uterus
• A hypoplastic, irregular, T shaped uterine cavity –
inutero DES exposure.
• Only anomaly in which HSG plays a significant
role- DES exposure.

Cont.
 Allows misdiagnosis between-
Partial septate & bicornuate uterus.
Complete septate & Uterus didelphys.

ULTRASONOGRAPHY
Most commonly 2D is used to evaluate.
( 75-100% sensitivity, upto 90% specificity).
Diagnose associated urological anomaly.
I Hypoplasia/Agenesis
• Absence of uterus & cervix- Agenesis.
• Hypoplastic uterus <2 cm intercornual distance-
Hypoplasia.

Cont.
Unicornuate uterus-
• Difficult to differantiate from normal.
• Banana shaped uterus.
• Laterally positioned.
• Rudimentary horn- soft tissue mass with
echogenicity similar to myometrium.
• Obstructed horn with functioning
endometrium-complex hemorrhagic cyst.

Cont.
Uterus didelphys-
• Two separate uterus with two cervix, separate
vagina difficult to see.
• Endometrial & myometrial zonal width are
preserved.
Bicornuate uterus-
• Two uterine cavity with normal endometrium.
• Reliable means of distinguisihing bicornuate
from septate uterus.

Cont.
• Concave fundus with fundal cleft >1 cm.
• Increased intercornual distance>4 cm.
• Intervening septum echogenicity similar to
myometrium.
Septate uterus
• Convex fundal contour.
• Intercornual distance <2 cm.
• Intervening septum composed of muscle or
fibrous tissue.

Cont.
Arcuate uterus
• May be detected.
• Fundal cleft <1 cm.
• Clinically not significant.
DES related
• Uterine hypoplasia.
• Diagnosed confidently by HSG.

3 D ULTRASONOGRAPHY
Permits accurate diagnosis.
Sensitivity & specficity-98.4%,100% resp.
It is best performed during the secretory phase
of the menstrual cycle so the endometrial
cavity is easier to outline.
The coronal plane is the most valuable in the
detection of uterine anomalies, shows the
entire endometrial canal & its relation to
myometrium and the uterine serosa. Multiple
planes can be constructed regardless of uterine
position.

Cont.
Accurately analyses uterine structure, contour
of fundus, muscular thickness, septum length.

MAGNETIC RESONANCE IMAGING
Gold standard for diagnosing uterine anomaly.
Evaluate concomitant urinary tract anomaly.
Hypoplasia/agenesis
• Absence of uterus, cervix & upper vagina.
• Hypoplastic uterus - <2 cm intercornual distance-
Hypoplasia.
• Zonal differentiation is poor.

Cont.
Unicoruate uterus-
• Banana or cigar shaped uterine cavity.
• Laterally deviated.
• Preserved zonal anatomy.
• Rudimentary horn- soft tissue mass with
intensity similar to myometrium.
• Obstructed horn with functioning
endometrium-distended with blood or blood
products.

Cont.
Uterus didelphys
• Two separated uterus & cervix.
• Septum of low signal intensity seen within
the upper vagina.
Bicornuate uterus
• Two uterine cavity with single cervix.

Cont.
• Concave fundus with fundal cleft >1 cm.
• Increased intercornual distance>4 cm.
• Intervening septum intensity similar to
myometrium.
Septate uterus
• Normal shaped uterus.
• Two separate uterine cavity.
• Fundal segment of septum with intensity similar
to myometrium.
• Fibrous segment with low intensity.

Cont.
Arcuate uterus
• Normal shaped uterus with single uterine
cavity.
• Slightly concave or flat external contour.
DES related
• Small uterus with T-shaped cavity.

CLASSIFICATION
AMERICAN FERTILTY SOCIETY CLASSIFICATION (1988)
I. Segmental or complete agenesis or hypoplasia
Agenesis and hypoplasia may involve the vagina, cervix, fundus, tubes,
or any combination of these structures. Mayer-Rokitansky-Kuster-Hauser
(MRKH) syndrome is the most common example in this category.
II. Unicornuate uterus
 With rudimentary horn –
□ With endometrial cavity-
-Communicating
-Non communicating
□ Without endometrial cavity
 Without rudimentary horn

Cont.
III. Didelphys uterus
• Complete or partial duplication of the vagina, cervix,
and uterus characterizes this anomaly.
IV. Bicornuate uterus
• Complete bicornuate uterus -uterine septum that
extends from the fundus to the cervical os.
• Partial bicornuate uterus - septum, which is located at
the fundus.
• In both variants, the vagina and cervix each have a
single cavity.

Cont.
V.Septate uterus
• A complete or partial midline septum is
present within a single uterus.
VI. Arcuate uterus
• A small septate indentation is present at the
fundus.
VII. DES-related abnormalities
• T-shaped uterine cavity with or without
dilated horns is evident. Not associated with
renal anomalies.

Mullerian Agenesis
Classified as:
Lateral Fusion Defects 1:
• most common type of uterine anomalies.
• Usually a failure of formation of one Mullerian duct
• failure of migration of a duct
• failure of fusion of a duct
 Septate Uterus: Contains 2 endometrial cavities but with a normal external surface.
 Arcuate Uterus:It has a small Septum which can be seen externally as a small
indentation at the uterine fundus .
An asymmetrical lateral fusion defect
 Unicornate Uterus. The affected mullerian duct does not develop at all or may
become a horn of the uterus ( communicating or non-communicating)
 Can be associated with an an ectopic ovary. This is important with women who are
being induced to ovulate.

• Bicornate Uterus :Has two cavities with an indented fundus greater than or
equal to 1 cm
•. Caused by the partial fusion of the mullerian ducts
• Can exist a varying degree of separation
• Didelphys: Considered a “double uterus” in which 2 mullerian ducts fail to fuse
forming 2 uterine cavities.
• Limited to the uterus and cervix (no vaginal involvement)
•15-20% of patients also have other unilateral anomalies eg; Ipsilateral renal agenesis
Vertical Fusion Defects:
• defective fusion of the caudal mullerian duct and urogenital sinus
• Abnormal vaginal canalization
• Symptoms will depend on degree of obstruction of the defect
Diethylstilbestrol:
It is a synthetic estrogen that was used from 1949-1971
Caused Vaginal adenosis among others… DES induced anomalies of the uterus

Mullerian Agenesis/Hypoplasia
Recently termed- mullerian aplasia.
C/by absence or hypoplasia of uterus proximal to
vagina & in some cases fallopian tubes.
 TWO VARIANT
□ Partial- rare
□ Complete- more common
eg: 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).

Cont.
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.

Cont.
USG
• Absence of uterus & fallopian tube with
normal ovaries.
MRI
• Uterus & vagina absent.
• Rudimentary uterus can be seen.
• Coexisting renal abnormality identified.

UNICORNUATE UTERUS
 One mullerian duct develops normally while
opposite fails to develop or develop incompletely.
 With rudimentary horn-
□ With endometrial cavity-
-communicating
-non communicating
□ Without endometrial cavity-
 Without rudimentary horn

Cont.
Associated with urological anomalies-44%.
 Poorest fetal survival among all mullerian
anomalies b/c of –
□ Insufficient muscular mass.
□ Decreased uterine volume.
□ Decreased ability to expand.
 Non communicating accessory horn having
endometrial cavity- most common & most
clinically significant.
Can cause- hematometra , endometriosis

Cont.
 Ectopic pregnancy in horn f/by rupture can
happen-should be removed before pregnancy.

HSG
• Useful for diagnosis,
• Can not detect non
communicating horn.
MRI
• Reliable.
• Only one fallopian tube
identified.
• Uterine volume reduced
• Accessory horn appears
solid
• Opacified when
endometrium is absent.

USG
• Useful for identifying rudimentary horn.
• Can identify communication with main uterine
cavity.

Cont
Laproscopy
Rarely
indicated.
Additional :
IVP, Renal
USG
 Indication of surgery-Presence of accessory horn with
endometrium.
 Laparoscopic hemi hysterectomy of rudimentary
horn is treatment of choice.

UTERINE DIDELPHYS
 Failure of medial fusion of two
mullerian ducts.
 C/by 2 hemiuterus, 2 endocervical
canal, 2 cervix, vagina can be single
or double.
Each hemiuterus having one
fallopian tube.
Renal agenesis most commonly
associated with uterine didelphys.

Cont.
 Simultaneous pregnancy can occur
in each uterus –are always dizygotic.
 Usually asymptomatic (non
obstructive) – diagnosed during
pelvic examination- two cervix seen.
 Associated with best possibility of
successful pregnancy after arcuate
uterus.
 May have history of recurrent
second trimester abortion.

 Unilateral vaginal obstruction can cause
hematocolpos, hemetometra, endometriosis- can be
overlooked as there is cyclical menstruation from opp.
Side.

Cont.
HSG
• Two uterine cavity with two cervix with
two vagina.
• Intercornual distance-> 4 cm.
MRI
• Two widely separeted uterus.
• Two cervix, two vagina.
• Associated renal anomaly identified.

Cont.
Indication of vaginal septum resection-
Obstructed unilateral hemivagina to preserve
fertility.
In non obstructive vagina – surgical correction
is limited.(severe dyspareunia).
 Metroplasty – Rarely indicated.
Results are disappointing.

BICORNUATE UTERUS
 Incomplete fusion at the level of uterine
fundus.
 Lower uterus & cervix are fused completely.
 Two separate but commmunicating
endometrial cavity.
 Important to differentiate bicornuate uterus
from septate uterus –different reproductive
outcome & treatment strategies.

Cont.
Subclassification : depending on septum length.
 COMPLETE : Septum upto cervix.
BICORNUATE UNICOLLIS : Septum extends to
internal os.
BICORNUATE BICOLLIS : Septum extends to
external os.
PARTIAL : Septum confined to fundal region.

Cont.
• Usually don’t have reproductive associated
problem.
• Depends on length of septum.
USG
 Should be done in luteal phase-endometrial
echo complex is
better identified.
 Not useful for distinguising
bicornuate uterus form septate
uterus.

Cont.
HSG
• Not reliable.
• Two chambered uterine cavity.
• Septal thickness is important.
MRI
• Two uterine body with single
cervix.
• Myometrial tissue separating
two cavity has intensity identical
to that of myometrium.

Cont.
LAPROSCOPY
Can distinguish.
Surgery seldom required.
Preserved for pts. with recurrent spontaneous
abortion, preterm birth.
Straussmann procedure –treatment of choice.

SEPTATE UTERUS
Failure of resorption of medial septum after
complete fusion of mullerian duct.
 Most common.
Subclassification- depending on septal length.
COMPLETE : Septum upto os.( double vagina)
PARTIAL : Septum does’t extend upto os.

 Fertility is not compromised yet has poorest reproductive
outcome.
 once pregnant, the greater the septum , greater the risk of
miscarriage esp. in 2nd trimester.
 Decreased intrauterine space for fetal growth.
 Implantation of placenta on poorly vascularized septum.
Decision to prefer surgical correction of septum
should be based on poor reproductive outcome
rather than on presence of septum alone.

Cont.
INDICATION FOR SURGERY
• Recurrent spontaneous abortion.
• Single 2nd trimester abortion.
• Preterm delivery.
HSG
• Two chambered uterine cavity.
• Length & thickness of septa should be
assessed.

Cont.
LAPROSCOPY
• Best for distinguishing septate from
bicornuate uterus.
• Normal fundal contour.
MRI
• Normal fundal contour with outward
convexity.
• Low signal intensity for septum.

Cont.
Surgical procedure of choice :
Hysteroscopy metroplasty with
concurrent laparoscopy. (outcomes are
excellent)
Post t/t miscarriage rates are 10% in
contrast to 90% pre t/t rates with complete
septum.

ARCUATE UTERUS
Near complete resorption of uterovaginal
septum.
C/by small intrauterine indentation <1 cm in
fundal region.
Considered as mild form of bicornuate uterus.
 Clinically benign.

HSG
• Single uterine cavity with
saddle shaped fundal
indentation.
MRI
• Convex or flate contour.
• Cavity with broad & smooth
indentation similar to
myometrium.

TRANSVERSE VAGINAL SEPTUM
 Formed when tissue between vaginal plate
& caudal aspect of fused mullerian ducts fail
to reabsorb.
 Develop at all levels.
a) Superior vagina-46%
b) Mid vagina-40%
c) Inferior vagina-14%

Cont.
NEONATES & INFANTS
 Rarely diagnosed in neonates & infants – hydromucocolpos.
 Unlike imperforate hymen bulging vagina is not seen with
valsalva maneuvre.
 USG is initial study, MRI is more useful as it clearly defines
the length of atretic segment between the upper and lower
vagina.
 Management : surgical excision of septum.

Cont.
POST MENARCHAE
 Symptoms depends on whether
septum is complete or incomplete.
 Presents with primary amenorrhea.
 Cyclical pelvic pain.
O/E
Palpable central lower abdominal
or pelvic mass secondary to
hematometra, hematocolpos,
hematosalpinx.

Cont.
 Local ex.- no bulging membrane.
 Incomplete TVS allows menstrual flow to
escape periodically but hematometra,
hematocolpos can later develop.
 Temptation to insert a needle for diagnostic
purpose must be resisted to avoid the risk of
converting a hematocolpos into a pyocolpos
Chronic retrograde menstruation frequently
result in pelvic endometriosis and adhesions,
which can be severe.

Imperforate hymen
Imperforate hymen is embryologically not of
mullerian origin although clinically have a similar
presentation
Imperforate:[8][9] hymenal opening nonexistent;
will require minor surgery if it has not corrected
itself by puberty to allow menstrual fluids to
escape.
Cribriform, or microperforate: sometimes
confused for imperforate, the hymenal opening
appears to be nonexistent, but has, under close
examination, small openings.
Septate: the hymenal opening has one or more
bands of tissue extending across the opening

Presenting symptoms
Surgical repair after the onset of puberty but before menarche is
optimal. The most common scenario is that in which a young woman
presents with increasingly severe intermittent abdominal and pelvic
pain due to a large hematocolpos and hematometra. This situation is
preventable, as routine examinations of the genitalia can detect this
obstruction and allow correction before menarche
Urinary pressure and even retention, with hydroureter and/or
hydronephrosis, may occur due to the mass effect and resultant
obstruction. Vaginal and rectal pressure is typically present. Severe
constipation and low-back pain are described as presenting symptoms.
The laborlike menstrual cramps may be severe and cyclic, although the
cyclic nature of the symptoms may not be easily or immediately
appreciated by the young woman or her family.

Cervical Atresia
• It is rare
• Often presents with normal vagina or absence
of lower vagina.
• Management of obstruction at the level of
cervix is most controversial
• conservative approach: uterovaginoplasty
• Radical approach: hysterectomy

a, Isolated congenital cervical atresia with normal vaginal
development. b, Congenital cervical atresia with complete
vaginal agenesis

PREGNANCY AND LABOUR IN UTERINE
MALFORMATION:
•Minor degrees of developmental defects of the uterus do not
usually interfere with pregnancy and labour.
•In most cases of uterine deformities, the two portions of the
uterus and a false decidua may form in the other half.
•Abortion does not occur infrequently – in some cases premature
labour is the result
• In the minor malformations, the pregnancy often goes on to term
and ends normally.

UTERUS BICORNIS BICOLLIS
• Pregnancy may occur in either half of the uterus, and when it occurs in
one horn of a bicornuate uterus, the other undergoes some degree of
hypertrophy and a decidua is formed in its cavity.
• Repeated abortions and breech presentations may occur more
frequently than in a normal uterus.
• Usually, the pregnancy takes a normal course and the delivery is
spontaneous
•In rare instances, the non pregnancy horn of the uterus may impede
the progress of labour by obstructing the passage of the head in the
pelvic cavity.
• With uterus bicornis unicollis, the difficulty of diagnosis is even
greater, and some cases are not diagnosed till after delivery.

UTERUS DIDELPHYS:
• Pregnancy may take a normal course. Occasionally, delivery
may be impeded by the septum in the vagina
• In the early weeks of pregnancy the presence of the non-gravid
uterus may give rise to the mistaken diagnosis of an
extrauterine gestation
• In some of these cases menstruation may occur throughout
the course of pregnancy form the non pregnant uterus.
• The pregnancy may be complicated by abortion, preterm
delivery, fetal growth restriction and malpresentation.

UTERUS SEPTUS AND SUBSEPTUS:
• In some, pregnancy and labour proceed normally but sometimes
repeated abortion may occur
• Malpresentations, specially breech, may be more frequent.
•In some cases, the placenta may be retained or adherent and it is
during the manual removal of the placenta that the condition is
generally diagnosed.
• In a few cases, the head or part of the fetus may pass through the
septum
UTERUS UNICOLLIS :
• Pregnancy is extremely rare in this type of uterine deformity.
Pregnancy in the rudimentary horn is attended with grave risks.
• Increased Incidence of abortion, preterm labour, IUGR, breech
presentation, uterine dysfunction in labour and caesarean section occur.
ARCUATE UTERUS :
There is depression at fundus. Fundal notching can be detected at late
pregnancy. This results in transverse lie.

EFFECTS OF ABNORMALITY ON PREGNANCY:
•When pregnancy occurs in the woman with an abnormal uterus, the
outcome depends on the ability of the uterus to accommodate the
growing fetus
• If there is insufficient hypertrophy, the possible difficulties are aborton,
premature labour and abnormal lie of the fetus.In labour, poor uterine
function may be experienced.
• Minor defects of structure cause little problem and might pass
unnoticed with the woman having a normal outcome to her pregnancy.
•Occasionally problems arise when a fetus is accommodated in one horn
of a double uterus and the empty horn has filled the pelvic cavity. In this
situation the empty horn had grow owing to the hormonal influences of
the pregnancy, and its size and position will cause obstruction during
labour. Caesarean section would be the method of delivery.

CLINICAL OUTCOME
Gynecological:
• Infertility and dyspareunia are often related in association
with vaginal septum
•Dysmenorrhoea in bicornuate uterus or due to
cryptomenorrhoea (pent up menstrual blood in rudimentary
horn)
• Menorrhagia – due to increased surface area in bicornuate
uterus.

Obstetrical
Obstetrical Midtrimester abortion which may be recurrent.
 Cornual pregnancy – with inevitable rupture around 16 th week – if pregnancy
occurs in the rudimentary horn
Increased incidence of malpresentation – transverse lie in arcuate or subseptate.,
breech in biconuate, unicornuate or complete septate uterus
Preterm labour
 Prolonged labour – due to incordinate uterine action
 Obstructed labour – 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.

VAGINAL AGENESIS
Aim:- to create a neovagina.
Non surgical
Initial therapy.
• 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.
• Patient instructed to sit on racing type bicycle seat for
at least 2 hrs/day at interval of 15-30 min.(to apply
pressure by leaning forward with dilator in place)

Cont.
SURGICAL
Non surgical treatment fails-considered.
Without use of abdominal contents-
Without dissecting cavity-
1. Williams vulvovaginoplasty(1964)
2. Vecchietti procedure(1965)
Dissecting cavity & lining with graft-
1. Mc Indoe operation
2. Dermis graft
3. Amnion graft
4. Flaps-Musculocutaneous, fasciocutaneous
With use of abdominal contents-
• Peritoneum
• Intestinal contents.

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.

Cont.
Technique
Skin graft taken.
0.018 inch thick, 8-9 cm wide, 16-20 cm in
length.
Vaginal form of adequate size is prepared by
applying skin graft over it.
Neo vaginal space is created.
Form placed in neo vagina, edge of graft
sutured with skin edge.

Cont.
Post operative management
• Bed rest in upright and flat position for one week.
• Antibiotics.
• Low residue diet.
• Replacing with new form-o
Removed after 7-10 days. vaginal cavity irrigated with
warm saline.
o Inspection of cavity to determine the take of graft.
o New form applied.
o Applied continuously for 6 wks except at the time of
urination & defecation.
o Next 12 months-new form applied only during night.

Cont.
Complication
• Post operative infection.
• Intra – post-op hemorrhage.
• Post operative fistula formation with solid
mould(due to pressure necrosis).
rectovaginal-more common
• Failure of graft taken up.
• Later granulation formation.
• Malignant transformation.

Williams Vulvo vaginoplasty
Full thickness skin flap of labia majora to
create a vaginal pouch.
Advantages-
• Technique is simple.
• Less local complication.
• Early recovery.
• Operation of choice for unsatisfactory Mc
Indoe operation.
• After extensive pelvic surgery or radiation
therapy.

• The Williams vulvovaginoplasty. A horseshoe shaped incision
deepened to expose superficial perineal muscle. B:Inner skin margins
united progressively with fine sutures. C:vaginal tube completed,
admits two fingers and 10-12 cm in length D: The entrance to the
pouch should not cover the external urethral meatus.

VECCHIETTI OPERATION
An olive-shaped device placed at vaginal
opening and with laparoscopic guidance
connected to a traction device on lower
abdomen. The traction device is tightened
every day, gradually pulling the olive-shaped
device inward to create a vagina over about a
week. Removal needs further manual dilation.

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;
however cervical cerclage may be
beneficial when indicated.

UTERUS DIDELPHYS
Uterus didelphys with obstructed unilateral
vagina- Full excision and marsupalization of
vaginal septum.
Non obstructed- Surgery rarely indicated.
Recommended procedure is Strassmann
metroplasty in pts with repeated poor
obstetrical outcomes

BICORNUATE UTERUS
Seldom requires surgical reconstruction.
Treatment of choice – Strassmann procedure.
Older procedures –
• Jones metroplasty
• Thompkins 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.

Cont.
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.

The Strassmann metroplasty with modification. A: If a rectovesical ligament is found, it should be
removed. B: An incision is made on the medial side of each hemicorpus and carried deep enough
to enter the uterine cavity. The edges of the myometrium will evert to face the opposite side. C
and D: The myometrium is approximated by use of interrupted vertical figure-of-eight 3—0
polyglycolic acid sutures. One should avoid placing sutures too lose to the interstitial portion of
the fallopian tubes. E: A continuous 3—0 polyglycolic acid subserosal suture is used as a final layer.
Tourniquets are removed, and defects in the broad ligament are closed.

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.

THOMPKINS METROPLASTY
• Abdominal approach.
• Single median incision given- divides uterine
corpus in half.
• Each lateral half incised to within 1 cm of
tubes.
• Myometrium is reapproximated.
• Leaves the uterotubal junction in a more
normal and lateral position.
• Less traumatic and cause less bleeding

SEPTATE UTERUS
Indication of surgery-
• Recurrent spontaneous abortion
• History of preterm labour.
Procedure of choice- Transcervical hysteroscopic
lysis of uterine septum with concurrent
laproscopy.
Preoperatively GnRH analogues for 2 months are
given to induce endometrial atrophy.

Cont.
• The laparoscope is placed.
• Hysteroscopy commences,and 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,
– and bleeding occurs from small vessels at the
fundal myometrium.(septum is relatively avascular)

Cont.
Main complication is
 Uterine perforation
 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.

TRANSVERSE VAGINAL SEPTUM
Cont.
Excision Of septum followed by vaginoplasty.
Depends on location & thickness of septum.
High TVS
Surgical correction is more difficult.
Transverse incision made in vaginal vault.
Dissection done between bladder & rectum upto
cervix.
Lateral margin of septum excised.
Hematocolpos drained.
And form channel for drainage, placed in vagina- allows
epithelialization & maintains patency.

Cont.
LOW, MID, THIN TVS
 Excised with multiple radial incision.
Upper & lower segments joined with multiple
delayed absorbable sutures.
Because septa that appears relatively thin may be
significantly larger after decompression of the
proximal haematcolpus, consider the possibility
that a graft may be required.

• Surgical correction of transverse vaginal septum. A: The upper end of a short vagina. The small
sinus tract opening, through which the patient menstruated, is shown. The line of incision is drawn
through the mucous membrane between the vaginal dimple and the sinus. B: Areolar tissue is
dissected through to the pocket of mucosa that covered the cervix. The mucosa is incised. C: An
anastomosis is made between the lower vagina and the upper vagina. 0: Completed vagina. It is
slightly shorter than normal but of normal caliber.

Hymenotomy
The objective of a hymenotomy procedure is to open the hymenal membrane in
such a way as to leave a normally patent vaginal orifice that does not scar
The hymenal orifice is enlarged using a circular incision following the lines of
the normal annular hymenal configuration. Alternatively, a cruciate incision along
the diagonal diameters of the hymen, rather than anterior to posterior, avoids
injury to the urethra and can be enlarged by removal of excess hymenal tissue. In
either approach, hemostasis is required using interrupted stitches with fine
absorbable suture (eg, 4-0 polyglycolic acid suture).
The surgical procedure of hymenotomy and evacuation of hematocolpos is
performed in an outpatient setting. The patient and family should be instructed to
expect continued drainage of dark, thick, old blood for several days to a week after
the procedure. Mild cramping may occur as the hematometra resolves and
evacuates.

COMPLICATIONS of
MULLERIAN ANOMALIES
Abortion
 Weak uterine action
Post partum hemorrhage
Adhesion of the placenta
Malpresentations
 Prolonged or obstructed labour
Uterine rupture due to its poor development.
 The placenta, if it is formed on the septum, may be
adherent and may cause post partum hemorrhage.

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.

oUterine malformations like absence, rudimentary or
infantile are not amenable to treatment.
o Hypoplastic uterus in young girls may gradually develop
with advance of age.
o Oestrogen therapy may be temporarily given for
amenorrhea, oligomenorrhoea
o dilation and curettage may be helpful in some cases with
dysmenorrhoea.
oChance pregnancy may develops in the hypoplastic uterus
oCervical cerclage is indicated in women with uterine
didelphys, unicornuate or bicornuate uterus and poor
reproductive performance.

Mullerian anomalies

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Mullerian anomalies