DR SUNITA SUDHIR
PROFESSOR
Dept . of OBG
 DEVEOPMENT OF FEMALE
REPRODUCTIVE SYSTEM
 CLASSIFICATION OF MULLARIAN
ANAMOLIES
 CLINICAL FEATURES
 DIAGNOSIS
 TREATMENT IN EACH TYPE OF ANAMOLY
INTRODUCTION
 Mullarian duct forms fallopian tubes, uterus, cervix and
upper part of vagina
 Ranges from agenesis to duplication. May be associated
with renal and axial skeletal system anomalies
 Varying presentation ranging from primary amenorrhea
to menstrual disorders, infertility and pregnancy
complications like BOH, PTL, Ectopic , etc
 Treatment varies for each defect and the corrective
surgeries are planned ,sexual function to conceive and
deliver normal babies.
EMBRYO DEVELOPMENT AT 4TH
WEEK OF INTRAUTERINE LIFE
 Mesonephric Duct is extending from mesonephros to
the cloca
 5th week swelling on either side of dorsal mesentry
 Intermediate mesoderm forms urogenital ridge
 Gonad develop from somatic and germ cells.
 Y chromosome has testes determining factor and
SRY gene.
4TH WEEK(UNDIFFERENTIATED)
mesonephric duct
4th week to 6th week of intra
uterine life para mesonephric duct
starts developing laterally
Mesonephric duct
Paramesonephric
duct
The middle &caudal parts of the
Mullarian ducts undergoes medial
migration and fusion
7 TO 12 WEEKS development of
paramesonephric duct and formation
of uterus
DEVELOPMENT OF VAGINA
•Lateral fusion defects,
•Most common mullarian defects.
•failure of fusion of the two mullerian ducts or lack
of septal resorption after fusion has occurred.
•Vertical defects
•result in defective fusion of the caudal mu llerian
duct with the sinovaginal bulbs,
•Leading to cervical agenesis or vaginal septa.
LATERAL FUSION DEFECTS
Failure of fusion of mullarian ducts or failure of absorption of
the septum
EFFECT OF MULLARIAN DUCT
ANAMOLY ON REPRODUCTION
 Infertility
 Endometriosis
 Ectopic pregnancy
 Recurrent Pregnancy Loss
 Prematurity , IUGR , fetal malposition
 Uterine dysfunction
 Uterine rupture
 Increased perinatal morbidity and
mortality
DIAGNOSIS
Clinical
Hystero salphingogram
Ultra sonography
MRI – 100% accuracy
Hystero laparoscopy
Laparotomy or LSCS
AFS CLASSIFICATION
CLASS TYPE PENETRATION/ EXTENT
CLASS I HYPOPLASIA AND AGENESIS VAGINAL ; CERVICAL ; FUNDAL ;
TUBAL
CLASS II UNICORNUATE COMMUNICATING ; NON
COMMUNICATING ; NO CAVITY
; NO HORN
CLASS III DIDELPHYS
CLASS IV BICORNUATE PARTIAL ; COMPLETE
CLASS V SEPTATE PARTIAL ; COMPLETE
CLAS VI ARCUATE ---
CLASS VII DES DRUG RELATED ---
CLASS I- ROKITANSKY
SYNDROME(MRKH)
 Primary amenorrhea
 Phenotypically female(Normal secondary
sexual,external genital)
 Absent uterus ,cervix part of vagina but normal
ovaries
 Short vagina
 Normal testosterone levels of female
 Karyotyping 46xx
VAGINAL AGENESIS WITH
RUDIMENTARY UTERINE HORN(MRKH)
INVESTIGATIONS
 Karyotyping
 USG/MRI
 Hormone assay
 IVP (associated renal anomalies can be detected)

 Diagnostic Laparoscopy is not routinely done.
TREATMENT
 Psychological support
 Vaginal Reconstruction
Vagino plasty
Mac Indoes Vaginoplasty; Williams
vulvovaginoplasty, Vecchietti procedure
 Fertility – by surrogacy
UNICORNUATE UTERUS(CLASS II)
 Unilateral failure of development of MDA
Incidence: 2.5-13%
Types : Unicornuate uterus
Unicornuate with rudimentary horn
-NonCommunicating
-Communicating
- with endometrium
-without endometrium
Associated Renal anomalies like renal agenesis,
Horseshoe kidney and pelvic kidney44% (In the
presence of obstructed horn)
CLINICAL FEATURES
 Women with a unicornuate uterus may be
asymptomatic.
 Implantation in the normal-sized
hemiuterus is associated with increased
incidence of:
 spontaneous abortion
 preterm delivery
 intrauterine fetal demise
 Ruptered uterus
Class II
CLINICAL FEATURES
 Haematometra
 Endometriosis
 Preterm labour – 43%
 IUGR
 Mal presentation
 Ectopic -4.3%
 Pregnancy in accessory horn -2%
 Rupture uterus
IMAGING MODALITIES IN
UNICORNUATE UTERUS
HSG 3D USG MRI
DIAGNOSIS AND SURGICAL
MANAGEMENT
 MRI – unicornuate uterus with non
communicating horn can be diagnosed
 IVU or renal sonography
 Laparoscopy – indicated for excision of
rudimentary horn which has endometrium
Cervical encirclage is mandatory if patient
conceives
REPRODUCTIVE OUTCOME IN
UNICORNUATE UTERUS
 Live birthrate 43.7%
 Abortion rate 35-43%
 Preterm delivery 27%
 Term delivery 31%
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
CLINICAL FEATURES
 Asymptomatic –
- preterm delivery (20%)
- fetal growth restriction (10%)
- breech presentation (43%)
- cesarean delivery rate (82%)
 Other associated anomalies : bladder exstrophy ,
congenital VVF, cervical agenesis
IMAGING MODALITIES IN
DIDELPHYS UTERUS
HSG 3DUSG MRI
DIAGNOSIS &SURGICAL
MANAGEMENT
 Clinical
 USG
 MRI- 2 widely separated uterine horns, 2
cervices are typical identified. Intercornual
angle >60 degree
 Laparoscopy
 IVP
SURGICAL MANAGEMENT
 Cervical encirclage is mandatory if patient
conceives
 If Present With obstruction :
- Excision of the horn
REPRODUCTIVE OUTCOME IN
DI DELPHYS
 Term delivery 20%

 Ectopic 2.3%
 Abortion 20%
 Preterm delivery 24%
BICORNUATE UTERUS(CLASS IV)
 Incomplete fusion of MD at uterine fundus
level
 Incidence - 20%
 May be complete - bicornuate bicollis
 May be incomplete - bicornuate unicollis
Bicornuate and Septate Uteri
 Marked increase in miscarriages due to
abundant muscle tissue in the septum
 Pregnancy losses in first 20 weeks
70 % in Bicornuate
88 % in Septate uteri.
 Increased incidence of preterm delivery,
abnormal fetal lie, and cesarean delivery.
Clinical Features
 Asymptomatic
 Abortion 28 %
 Preterm delivery 25 %
 Live birth 63 %
IMAGING MODALITIES IN BICORNUATE
UTERUS
HSG 3D USG MRI
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
Bicornuate uterus
SURGICAL MANAGEMENT
 Metroplasty is reserved only in recurrent aborters
 Strassmann procedure either by Laparoscopy or
Laparotomy
REPRODUCTIVE OUTCOME IN
BICORNUATE UTERUS
 Term pregnancy rate 60%
 Live birth 65%
 Metroplasty is indicated only when other causes
are ruled out.
Acien , 1993
SEPTATE UTERUS
 Incomplete resorption of medial septum
 Incidence : 33.6%
 Types: Complete
Incomplete
Class V
CLINICAL FEATURES
 Dysmenorrhoea
 Primary or secondary infertility
 Poor reproductive performance
 Recurrent miscarriages
 Preterm delivery
IMAGING MODALITIES IN SEPTATE
UTERUS
HSG USG 3DUSG
MRI
Septate uterus
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
REPRODUCTIVE OUTCOME IN
SEPTATE 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
COMPLICATION
 Uterine perforation
 Hemorrhage
 Cervical incompetence
 Residual septum
Class VI
Arcuate Uterus
 This malformation is only a mild deviation from
the normally developed uterus.
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%
IMAGING MODALITIES IN ARCUATE
UTERUS
HSG 3D USG MRI
REPRODUCTIVE OUTCOME IN
ARCUATE UTERUS
 Preterm delivery 05.1%
 Live birth 66.2%
 Ectopics 03.6%
 Spontaneous abortion 20.0%
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
Mullarian anamolies

Mullarian anamolies

  • 1.
  • 2.
     DEVEOPMENT OFFEMALE REPRODUCTIVE SYSTEM  CLASSIFICATION OF MULLARIAN ANAMOLIES  CLINICAL FEATURES  DIAGNOSIS  TREATMENT IN EACH TYPE OF ANAMOLY
  • 3.
    INTRODUCTION  Mullarian ductforms fallopian tubes, uterus, cervix and upper part of vagina  Ranges from agenesis to duplication. May be associated with renal and axial skeletal system anomalies  Varying presentation ranging from primary amenorrhea to menstrual disorders, infertility and pregnancy complications like BOH, PTL, Ectopic , etc  Treatment varies for each defect and the corrective surgeries are planned ,sexual function to conceive and deliver normal babies.
  • 4.
    EMBRYO DEVELOPMENT AT4TH WEEK OF INTRAUTERINE LIFE  Mesonephric Duct is extending from mesonephros to the cloca
  • 5.
     5th weekswelling on either side of dorsal mesentry  Intermediate mesoderm forms urogenital ridge  Gonad develop from somatic and germ cells.  Y chromosome has testes determining factor and SRY gene.
  • 7.
  • 8.
    4th week to6th week of intra uterine life para mesonephric duct starts developing laterally Mesonephric duct Paramesonephric duct
  • 9.
    The middle &caudalparts of the Mullarian ducts undergoes medial migration and fusion
  • 10.
    7 TO 12WEEKS development of paramesonephric duct and formation of uterus
  • 11.
  • 12.
    •Lateral fusion defects, •Mostcommon mullarian defects. •failure of fusion of the two mullerian ducts or lack of septal resorption after fusion has occurred. •Vertical defects •result in defective fusion of the caudal mu llerian duct with the sinovaginal bulbs, •Leading to cervical agenesis or vaginal septa.
  • 13.
    LATERAL FUSION DEFECTS Failureof fusion of mullarian ducts or failure of absorption of the septum
  • 14.
    EFFECT OF MULLARIANDUCT ANAMOLY ON REPRODUCTION  Infertility  Endometriosis  Ectopic pregnancy  Recurrent Pregnancy Loss  Prematurity , IUGR , fetal malposition  Uterine dysfunction  Uterine rupture  Increased perinatal morbidity and mortality
  • 15.
    DIAGNOSIS Clinical Hystero salphingogram Ultra sonography MRI– 100% accuracy Hystero laparoscopy Laparotomy or LSCS
  • 16.
    AFS CLASSIFICATION CLASS TYPEPENETRATION/ EXTENT CLASS I HYPOPLASIA AND AGENESIS VAGINAL ; CERVICAL ; FUNDAL ; TUBAL CLASS II UNICORNUATE COMMUNICATING ; NON COMMUNICATING ; NO CAVITY ; NO HORN CLASS III DIDELPHYS CLASS IV BICORNUATE PARTIAL ; COMPLETE CLASS V SEPTATE PARTIAL ; COMPLETE CLAS VI ARCUATE --- CLASS VII DES DRUG RELATED ---
  • 17.
    CLASS I- ROKITANSKY SYNDROME(MRKH) Primary amenorrhea  Phenotypically female(Normal secondary sexual,external genital)  Absent uterus ,cervix part of vagina but normal ovaries  Short vagina  Normal testosterone levels of female  Karyotyping 46xx
  • 18.
  • 19.
    INVESTIGATIONS  Karyotyping  USG/MRI Hormone assay  IVP (associated renal anomalies can be detected)   Diagnostic Laparoscopy is not routinely done.
  • 20.
    TREATMENT  Psychological support Vaginal Reconstruction Vagino plasty Mac Indoes Vaginoplasty; Williams vulvovaginoplasty, Vecchietti procedure  Fertility – by surrogacy
  • 21.
    UNICORNUATE UTERUS(CLASS II) Unilateral failure of development of MDA Incidence: 2.5-13% Types : Unicornuate uterus Unicornuate with rudimentary horn -NonCommunicating -Communicating - with endometrium -without endometrium Associated Renal anomalies like renal agenesis, Horseshoe kidney and pelvic kidney44% (In the presence of obstructed horn)
  • 22.
    CLINICAL FEATURES  Womenwith a unicornuate uterus may be asymptomatic.  Implantation in the normal-sized hemiuterus is associated with increased incidence of:  spontaneous abortion  preterm delivery  intrauterine fetal demise  Ruptered uterus
  • 23.
  • 24.
    CLINICAL FEATURES  Haematometra Endometriosis  Preterm labour – 43%  IUGR  Mal presentation  Ectopic -4.3%  Pregnancy in accessory horn -2%  Rupture uterus
  • 25.
    IMAGING MODALITIES IN UNICORNUATEUTERUS HSG 3D USG MRI
  • 26.
    DIAGNOSIS AND SURGICAL MANAGEMENT MRI – unicornuate uterus with non communicating horn can be diagnosed  IVU or renal sonography  Laparoscopy – indicated for excision of rudimentary horn which has endometrium Cervical encirclage is mandatory if patient conceives
  • 27.
    REPRODUCTIVE OUTCOME IN UNICORNUATEUTERUS  Live birthrate 43.7%  Abortion rate 35-43%  Preterm delivery 27%  Term delivery 31%
  • 28.
    Uterine Didelphys (ClassIII)  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
  • 29.
    CLINICAL FEATURES  Asymptomatic– - preterm delivery (20%) - fetal growth restriction (10%) - breech presentation (43%) - cesarean delivery rate (82%)  Other associated anomalies : bladder exstrophy , congenital VVF, cervical agenesis
  • 30.
    IMAGING MODALITIES IN DIDELPHYSUTERUS HSG 3DUSG MRI
  • 31.
    DIAGNOSIS &SURGICAL MANAGEMENT  Clinical USG  MRI- 2 widely separated uterine horns, 2 cervices are typical identified. Intercornual angle >60 degree  Laparoscopy  IVP
  • 32.
    SURGICAL MANAGEMENT  Cervicalencirclage is mandatory if patient conceives  If Present With obstruction : - Excision of the horn
  • 33.
    REPRODUCTIVE OUTCOME IN DIDELPHYS  Term delivery 20%   Ectopic 2.3%  Abortion 20%  Preterm delivery 24%
  • 34.
    BICORNUATE UTERUS(CLASS IV) Incomplete fusion of MD at uterine fundus level  Incidence - 20%  May be complete - bicornuate bicollis  May be incomplete - bicornuate unicollis
  • 35.
    Bicornuate and SeptateUteri  Marked increase in miscarriages due to abundant muscle tissue in the septum  Pregnancy losses in first 20 weeks 70 % in Bicornuate 88 % in Septate uteri.  Increased incidence of preterm delivery, abnormal fetal lie, and cesarean delivery.
  • 36.
    Clinical Features  Asymptomatic Abortion 28 %  Preterm delivery 25 %  Live birth 63 %
  • 37.
    IMAGING MODALITIES INBICORNUATE UTERUS HSG 3D USG MRI
  • 38.
    DIAGNOSIS  To bedifferentiated 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
  • 39.
  • 40.
    SURGICAL MANAGEMENT  Metroplastyis reserved only in recurrent aborters  Strassmann procedure either by Laparoscopy or Laparotomy
  • 41.
    REPRODUCTIVE OUTCOME IN BICORNUATEUTERUS  Term pregnancy rate 60%  Live birth 65%  Metroplasty is indicated only when other causes are ruled out. Acien , 1993
  • 42.
    SEPTATE UTERUS  Incompleteresorption of medial septum  Incidence : 33.6%  Types: Complete Incomplete
  • 43.
  • 44.
    CLINICAL FEATURES  Dysmenorrhoea Primary or secondary infertility  Poor reproductive performance  Recurrent miscarriages  Preterm delivery
  • 45.
    IMAGING MODALITIES INSEPTATE UTERUS HSG USG 3DUSG MRI
  • 46.
  • 47.
    SURGICAL MANAGEMENT  HysteroscopicSeptal 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
  • 48.
    REPRODUCTIVE OUTCOME IN SEPTATEUTERUS 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
  • 49.
    COMPLICATION  Uterine perforation Hemorrhage  Cervical incompetence  Residual septum
  • 50.
    Class VI Arcuate Uterus This malformation is only a mild deviation from the normally developed uterus.
  • 51.
    ARCUATE UTERUS  Nearcomplete resorption of the uterovaginal septum.  Small intrauterine indentation shorter than 1cm and located in the fundal region diagnosed by HSG.  Incidence : 32.8%
  • 52.
    IMAGING MODALITIES INARCUATE UTERUS HSG 3D USG MRI
  • 53.
    REPRODUCTIVE OUTCOME IN ARCUATEUTERUS  Preterm delivery 05.1%  Live birth 66.2%  Ectopics 03.6%  Spontaneous abortion 20.0%
  • 54.
    CONCLUSION  MDA arenot 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