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DR.T.RAMANI DEVI MD DGO FICS FICOG
CONSULTANT OBSTETRICIAN AND GYNAECOLOGIST
RAMAKRISHNA NURSING HOME,
TRICHY
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INTRODUCTION MDA are fascinating disorders to obstetricians and
gynaecologists MD forms tubes, uterus, cervix and upper part of
vagina Ranges from agenesis to duplication. Associated with renal and axial skeletal systems
anomalies Has varying presentation ranging from primary
amenorrhea to menstrual disorders, infertility and pregnancy complications like BOH, PTL, Ectopic , etc
MDA has varying treatment from ability to have coitus to conceive and deliver normal babies.
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INCIDENCE
Dates back to 16th century a case utero vaginal agenesis
– Columbo et al (1600) General population – 0.1-3.5% - Byrene et al Fertile women – 4.3% Infertile women – 3.6% Sterile group - 2.4% Recurrent Aborters 5 - 13% - Grimbizis et al
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ETIOLOGY
Dysregulation occuring in differentiation, migration, fusion and canalisation
Associated with renal anomalies, axial skeletal anomalies and rarely cardiac and auditory anomalies
Probable causes: Intrauterine infection , genetic aberration, Teratogens like DES and Thalidomide.
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GENETICS OF MDA Sporadic Familial Multifactorial Autosomal dominant Autosomal recessive X linked Variants of GALT (Galactose 1 phosphate
uridyl transferase enzyme defect)
Genes Associated :- HOXA 9, 13 & WNT 4
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Embryogenesis of the Reproductive Tract
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CLASSIFICATION OF MDA
1979 – Buttram and Gibbons classification
Modified
1988 – American Fertility Society
classification
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American Fertility Society Classification of Mullerian Anomalies
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INCIDENCE OF MDA ACCORDING TO AFS Arcuate uterus 32.8% Septate uterus 33.6% Bicornuate uterus 20.0% DES exposed uterus 0.8% Unicornuate Uterine didelphys 33%
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EFFECT OF MDA UPON REPRODUCTION
Infertility Endometriosis Ectopic pregnancy Recurrent Pregnancy Loss Prematurity , IUGR , fetal malposition Uterine dysfunction Uterine rupture Increased perinatal morbidity and mortality
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DIAGNOSIS OF MDA
Clinical Hystero salphingogram Sonosalphingogram MRI – 100% accuracy Hystero laparoscopy Laparotomy or LSCS
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Vulvar Abnormalities
Vulval and lower 1/3rd vagina atresia
Labial Fusion Most commonly due to congenital adrenal
hyperplasia.
Imperforate hymen Persistence of the fusion between the sinovaginal
bulbs at the vestibuleAssociated with primary amenorrhea and
hematocolpos
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Vaginal Abnormalities
Developmental abnormalities of the normal single vagina include:
Vaginal agenesis Vaginal atresia Double vagina Longitudinal vaginal septum Transverse vaginal septum
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Obstetrical significance of vaginal abnormalities Complete mullerian agenesis – pregnancy is
impossible because uterus and vagina is absent
About one third of women with vaginal atresia have associated urological abnormalities
Complete vaginal atresia – precludes intercourse and then pregnancy
In most cases of partial atresia, because of pregnancy-induced tissue softening, obstruction during labor is gradually overcome. interferes with descent
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Obstetrical significance of vaginal abnormalities Complete longitudinal vaginal septum usually
does not cause dystocia because half of the vagina through which the fetus descends dilates satisfactorily.
Incomplete septum, however, occasionally interferes with descent.
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Cervical Abnormalities
Atresia. This may be combined with incomplete development of the upper vagina or lower
uterus
Double cervix. Each distinct cervix results from separate müllerian duct maturation. Both septate and true double cervices are frequently associated with a longitudinal
vaginal septum. Many septate cervices are erroneously classified as double.
Single hemicervix. This arises from unilateral müllerian maturation.
Septate cervix. This consists of a single muscular ring partitioned by a septum. The septum may be confined to the cervix, or more often, it may be the downward
continuation of a uterine septum or the upward extension of a vaginal septum.
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CLASS I- ROKITANSTY SYNDROME Primary amenorrhea Feminine patients Short vagina
DD: Testicular feminization syndrome
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Class I
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INVESTIGATIONS
Karyotyping USG/MRI Hormone assay IVP (associated vertebral anomalies can
be detected) and renal sonography Diagnostic Laparoscopy is not routinely
done.
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TREATMENT
Vaginal Reconstruction
– Vagino plasty : Mac Indoes Vaginoplasty; Williams vulvovaginoplasty, Vecchietti procedure
Fertility – by surrogacy Psychological support
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Unicornuate Uterus (Class II) 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
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UNICORNUATE UTERUS Unilateral failure of development of MDAIncidence: 2.5-13% Types : Unicornuate
Unicornuate with rudimentary horn -Communicating -Non communicating
- with endometrium-without
endometrium
Associated Renal anomalies like renal agenesis, Horseshoe kidney and pelvic kidney44% (In the presence of obstructed horn)
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Class II
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CLINICAL FEATURES
Haematometra Endometriosis Preterm labour – 43% IUGR Mal presentation Ectopic -4.3% Pregnancy in accessory horn -2% Rupture uterus
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IMAGING MODALITIES IN UNICORNUATE UTERUSHSG 3D USG MRI
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DIAGNOSIS AND SURGICAL MANAGEMENT
HSG – non communicating horn cannot be diagnosed
USG – 3D or High Resolution MRI – banana shaped uterus Laparoscopy – indicated for excision of
rudimentary horn which has endometrium IVU or renal sonography
Cervical encirclage is mandatory if patient conceives
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REPRODUCTIVE OUTCOME IN UNICORNUATE UTERUS
Live birthrate 43.7% Abortion rate 35-43% Preterm delivery 27% Term delivery 31%
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NONCOMMUNICATING RUDIMENTARY UTERINE HORN * attached fallopian tube (arrow) was patent*
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UNICORNUATE UTERUS WITH RUDIMENTARY HORN
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Uterine Didelphys (Class III) This anomaly is distinguished from bicornuate
and septate uteri by the presence of complete nonfusion of the cervix and hemiuterine cavity
Except for ectopic and rudimentary horn pregnancies, problems associated with uterine didelphys are similar but less frequent than those seen with unicornuate uterus
Complications may include - preterm delivery (20%) - fetal growth restriction (10%)- breech presentation (43%)- cesarean delivery rate (82%)
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DI DELPHYS
Failure of midline fusion of MD either completely or partially
Incidence: 11% Types : Total Septum
Partial Septum
Transverse Septum
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Class III
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CLINICAL FEATURES
Asymptomatic – Failure of tampons to obstruct menstrual flow
Hematometrocolpos if there is Hematometra obstruction Hematosalpinx 20% renal anomalies Endometriosis Other associated anomalies : bladder exstrophy ,
congenital VVF, cervical agenesis
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IMAGING MODALITIES IN DIDELPHYS UTERUSHSG 3DUSG MRI
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DIAGNOSIS &SURGICAL MANAGEMENT
Clinical USG MRI- 2 widely separated uterine horns, 2
cervices are typical identified. Intercornual angle >60 degree
Laparoscopy IVP
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UTERUS DIDELPHYS
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SURGICAL MANAGEMENT
With obstruction - Excision of the horn Non obstruction- Strassmann metroplasty only in selected
cases
Cervical encirclage is mandatory if patient conceives
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REPRODUCTIVE OUTCOME INDI DELPHYS
Term delivery 20% Ectopic 2.3% Abortion 20% Live birth 68% Preterm delivery 24%
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Bicornuate and Septate Uteri (Classes IV and V) 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 by Buttram and Gibbons
70 percent for bicornuate 88 percent for septate uteri There also is an increased incidence of
preterm delivery, abnormal fetal lie, and cesarean delivery.
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BICORNUATE UTERUS
Incomplete fusion of MD at uterine fundus level
Incidence - 20% May be complete - bicornuate bicollis May be incomplete - bicornuate unicollis
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Class IV
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ULTRASOUND IMAGING OF SEPTATE AND BICORNUATE UTERUS
Anna Lev-Toaff, MD , Thomas Jefferson University, PA
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Clinical features
Asymptomatic Abortion 28% Preterm delivery 25% Live birth 63%
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IMAGING MODALITIES IN BICORNUATE UTERUSHSG 3D USG
MRI
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DIAGNOSIS To be differentiated from septate uterus HSG USG during luteal phase shows 2 endometrial
cavities with a deep dimple in the fundus. MRI – Ideal Intercornual distance is >105 degrees Myometrial tissue is seen in bicornuate uterus Vs
septum in septate uterus with angle of <75 degree
Laparoscopy
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SURGICAL MANAGEMENT
Metroplasty is reserved only in recurrent aborters
Strassmann procedure either by Laparoscopy or Laparotomy
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BICORNUATE UTERUS
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BICORNUATE UTERUS WITH OBSTRUCTION IN ONE HORN
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REPRODUCTIVE OUTCOME INBICORNUATE UTERUS Increased incidence in infertile
population. Term pregnancy rate 60% Live birth 65% Metroplasty is indicated only when other
causes are ruled out.
Acien , 1993
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SEPTATE UTERUS
Incomplete resorption of medial septum Incidence : 33.6% Types: Complete
Incomplete
DD: Uterus didelphys
Renal tract anomalies are rare
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Class V
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CLINICAL FEATURES
Dyspareunia Dysmenorrhoea Primary or secondary infertility Poor reproductive performance
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IMAGING MODALITIES IN SEPTATE UTERUSHSG USG 3DUSG
MRI
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SURGICAL MANAGEMENT Hysteroscopic Septal Resection under
Laparoscopic guidance using microscissors, electro cautery, laser, Versa point
Stop dissecting - When both cornuae are seen in the
same plane- Appearance of vascularity- Move the scope from one side to other
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SEPTATE UTERUS
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REPRODUCTIVE OUTCOME INSEPTATE UTERUS Spontaneous abortion 33-75% Live birth 62% Term deliveries 51% Preterm labour 10% Ectopic 2%Metroplasty increases the incidence of live
birth to 82%
Acien , 1993
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POST OPERATIVE MANAGEMENT Estrogens may be used
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COMPLICATION
Uterine perforation Hemorrhage Cervical incompetence Residual septum
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Class VI
Arcuate Uterus This malformation is only a mild
deviation from the normally developed uterus.
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ARCUATE UTERUS
Near complete resorption of the uterovaginal septum.
Small intrauterine indentation shorter than 1cm and located in the fundal region diagnosed by HSG.
Incidence : 32.8%
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IMAGING MODALITIES IN ARCUATE UTERUS HSG 3D USG MRI
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DIAGNOSIS & TREATMENT HSG MRI IVP and renal ultrasound Hysteroscopy Resection indicated in poor performers
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REPRODUCTIVE OUTCOME INARCUATE UTERUS
Preterm delivery 05.1% Live birth 66.2% Ectopics 03.6% Spontaneous abortion 20.0%
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Diethylstilbestrol-Induced Reproductive Tract Abnormalities Development of rare vaginal clear cell
adenocarcinoma. Increased risk of developing cervical intraepithelial neoplasia small-cell cervical carcinoma vaginal adenosis, non-neoplastic structural abnormalities
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Diethylstilbestrol-Induced Reproductive Tract AbnormalitiesStructural Abnormalities: transverse septa, circumferential ridges involving the
vagina and cervix cervical collars smaller uterine cavities shortened upper uterine segments T-shaped and irregular oviduct abnormalities
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Diethylstilbestrol-Induced Reproductive Tract Abnormalities
Their incidences of miscarriage,
ectopic pregnancy, and preterm delivery are also increased, especially in women with structural abnormalities
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T SHAPED UTERUS
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MANAGEMENT OF T SHAPED UTERUS
Lateral metroplasty
Encerclage is mandatory in the event of pregnancy
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CONCLUSION MDA are not so uncommon Presents at varying stages of life as
primary amenorrhoea , infertility, Recurrent abortion, preterm labour,
MRI helps in accurate diagnosis DHL is indicated only when intervention
is needed. Corrective surgery improves pregnancy
outcome
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DEVELOPMENT OF THE OVARY- The primitive sex cords degenerate & become replaced by
vascular fibrous tissue which forms the permanent medulla.- The epithelium of the celomic cavity proliferates & become
thicker. It forms columns of cells known as cortical cords.- The cortical cords split into separate follicular cell clusters
surrounding germ cells & form together primordial follicles.
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DEVELOPMENT OF THE DUCTS OF THE GONADS
2 ducts are formed in male & female embryos: mesonephric (Wolffian ) & paramesonephric (Mullerian) duct.
In male embryo:- Mullerian duct degenerate (except
the uppermost part which forms appendix testis & lowermost part which forms prostatic utricle).
Wolffian duct:- Its upper part becomes markedly
convoluted forming the epididymis.- The middle part forms the vas
deferens.- The lower part forms a small pouch
which forms the seminal vesicle.- The terminal part forms the
ejaculatory duct.- (The upper most part of the duct forms
appendix epididymis).- Mesonephric tubules opposite the
developing testis forms efferent ducts which become connected to rete testis.