congenital anomelies of reproductive organs
TRANSCRIPT
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Developmental Anomalies Of Female Reproductive OrgansDevelopmental Anomalies Of Female Reproductive Organs
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Embryology
The uterus develops from the two
parameso
nephric ducts (Mullerian ducts).
The caudal two thirds of these ducts give
rise to the uterus and the upper
third become the Fallopian tubes.
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6-9 weeks: the appearance of Müllerian ducts and their caudal
midline fusion
and connection with urogenital sinus;
10-13 weeks: the upward fusion of the caudal parts of the Müllerian
ducts;
14-18 weeks: the resorption of the medial septum initially separating
the caudal
parts of the Müllerian ducts to form the utero-vaginal
channel as
the origin of both uterine cavity and superior 2/3 of the
vagina.
The development of the uterus is divided into the three
stages
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As the superior 2/3 of the vagina has the same embryologic
origin as the
uterus, uterine malformations are often associated with upper
vaginal
malformations;
Genital anomalies are often associated with urinary tract
anomalies
(kidneys, urethra) due to close embryological interactions;
The independent ovarian and Müllerian ducts
development explains the
generally normal ovarian morphology and function in the cases
of
uterovaginal developmental anomalies;
The uterine malformations are usually not associated with
chromosomal
or sexual differentiation anomalies.
Four important facts
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Developmental Anomalies Of Female Reproductive OrgansDevelopmental Anomalies Of Female Reproductive Organs
Congenital vesico-vaginal fistula /Perineal / vestibular anus
Congenital recto-vaginal fistula / ectopic ureter
Developmental Anomalies Of The External GenitaliaDevelopmental Anomalies Of The External Genitalia
Vaginal AbnormalityVaginal Abnormality
Imperforate hymen
Septate vaginaCongenital atresia of vaginaNarrow introitus
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Developmental Anomalies Of Female Reproductive OrgansDevelopmental Anomalies Of Female Reproductive Organs
Unduly elongated
Accessory cilia or diverticula
Developmental Anomalies Of The Felopian TubesDevelopmental Anomalies Of The Felopian Tubes
Anomelies of the ovariesAnomelies of the ovaries
Streak gonads or gonadal dysgenesis Accessory ovarySuspensory ovariesEctopic ovaries
May be absent unilateraly
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Congenital Uterine AbnormalitiesCongenital Uterine Abnormalities
Uterine malformation may be due to
Arrested development of the mullerian ducts;
Failure of development of the mullerian ducts;
Failure of resorption of the median septum.
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Embryological mechanism
Classification after Musset (1964)
Classification after American Fertility
Society “AFS” (1988)
Aplasia or agenesis of the Mülleri
an ducts
BilateralComplete – lethalIncomplete – Mayer-Rokitansky-Küster- Hauser syndrome
Type I
UnilateralComplete – true unicornuate uterusIncomplete – pseudo unicornuate uterus
Type IIUnicornuate uterusII a: with communicating rudimentary hornII b: with noncommunicating rudimentary hornII c: rudimentary horn without cavityII d: without rudimentary horn
Anomaly of the fusion
Didelphys uterus or bicornuate bicervical uterus
Type IIIUterus didelphys
Bicornuate unicervical uterusTotalSubtotalFundic
Type IVBicornuate uterusIV a: completeIV b: partial
Anomaly of the resorption
Septate uterusTotalSub-totalCorporal (only the uterine body)Fundal
Type VUterus septateV a: completeV b: partial
Arcuate uterusType VIArcuate uterus according with AFS this form can be considered as a partially septate uterus (fundal)
HypoplasiaUterine hypoplasiaDES (diethylstilbestrol) syndrome
Type VIIDES (diethylstilbestrol) uterus (iatrogenic)
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Developmental Anomalies Of The UterusDevelopmental Anomalies Of The Uterus
Arrested Development Of The Mularian DuctArrested Development Of The Mularian Duct
Bilateral - Uterine aplasia.
Unilateral - Unicornuate uterus (uterus unicornis unicollis). Solitary uterine cavity and single fallopian tube
communicating rudimentary horn
without rudimentary horn noncommunicating rudimentary hornrudimentary horn without cavity
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Failure Of Development Of The Mullerian DuctsFailure Of Development Of The Mullerian Ducts
Uterine didelphis (complete duplication) 2 uteri, 2 cervices, 2 vaginas.
Bicornuate uterus – Bicollis- 2 uterine horns, 2 cervices and 1 vagina Unicollis- 2 uterine horns, 1 cervix and 1 vagina
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Failure Of Resorption Of The Median SeptumFailure Of Resorption Of The Median Septum
Total (Uterine septus).
Partial (Uterine sub-
septus)
Arcuate.
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Haemocolpos And Imperforate Hymen Haemocolpos And Imperforate Hymen
Hematocolpos secondery to imperforate hymenHematocolpos secondery to imperforate hymen
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Haemometra And Imperforate Hymen Haemometra And Imperforate Hymen
Hematometra secondery to imperforate hymenHematometra secondery to imperforate hymen
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Haematosalpinx And Imperforate Hymen Haematosalpinx And Imperforate Hymen
Anechoic tubular structures in the adnexa Anechoic tubular structures in the adnexa
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Haemocolpos And Imperforate Hymen Haemocolpos And Imperforate Hymen
Young female patient presented with primary
amenorrhoea and abdominal mass.
Ultrasound images reveal hypoechoic
contents
s/o fluid distending the uterus and vagina.
Fine particulate debris is seen within the fluid
(blood).
These findings suggest vaginal outflow
obstruction due to imperforate hymen.
Young female patient presented with primary
amenorrhoea and abdominal mass.
Ultrasound images reveal hypoechoic
contents
s/o fluid distending the uterus and vagina.
Fine particulate debris is seen within the fluid
(blood).
These findings suggest vaginal outflow
obstruction due to imperforate hymen.
The vagina distended with hemorrhagic contents.
However the uterus does not appear to be involved.
The vagina distended with hemorrhagic contents.
However the uterus does not appear to be involved.
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HaemometraHaemometra
A large amount of hypoechoic blood (clot) is seen within the uterus. This an be seen with an imperforate lymen or as a
postoperative complication of gynecologic surgery.
A large amount of hypoechoic blood (clot) is seen within the uterus. This an be seen with an imperforate lymen or as a
postoperative complication of gynecologic surgery.
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Septate vagina Septate vagina
Sonohysterogram (saline infusion sonography)
Sonohysterogram (saline infusion sonography)
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Vaginal AtresiaVaginal Atresia
Ultrasound of a newborn girl with vaginal atresia and hydrometrocolpos.
Longitudinal image of the uterus with a dilated endometrial canal (hydrometra)
Ultrasound of a newborn girl with vaginal atresia and hydrometrocolpos.
Longitudinal image of the uterus with a dilated endometrial canal (hydrometra)
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Uterine HypoplasiaUterine Hypoplasia
Very reduced volume of uterus- hypoplastic uterusVery reduced volume of
uterus- hypoplastic uterus T-shaped uterusT-shaped uterus
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Uterine HypoplasiaUterine Hypoplasia
Longitudinal US scan shows a minute uterus (arrow).
Transverse US scan shows no visible ovarian tissue.
Longitudinal US scan shows a minute uterus (arrow).
Transverse US scan shows no visible ovarian tissue.
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Unicornuate uterusUnicornuate uterus
Difficult to differentiate from the normal uterus by USG.
Suspected when the uterus appears small & laterally positioned.
Difficult to differentiate from the normal uterus by USG.
Suspected when the uterus appears small & laterally positioned.
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Rudimentary Uterine HornRudimentary Uterine Horn
Transvaginal ultrasonographic diagnosis of the rudimentary uterine horn. (A) The right-sided uterus (U) and the large left pelvic mass (M) with hypo-echoic content. (B) The distended ovoid pelvic mass (M)..
Transvaginal ultrasonographic diagnosis of the rudimentary uterine horn. (A) The right-sided uterus (U) and the large left pelvic mass (M) with hypo-echoic content. (B) The distended ovoid pelvic mass (M)..
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Bicornuate uterusBicornuate uterus
The main sonographic feature of the uterine duplications is an invagination of the uterine fundus, well seen on coronalviews (or by 3D sonography). The spacing between the horns with the urinary bladder in the gap between the horns can be recognized as a "V sign" of the bladder.
The differential diagnosis can be difficult with a septate uterus when the spacing between the horns is small. The fundal invagination bigger than 1cm of depth and a distinct vascularization on color Doppler images testify the bicornuate uterus.
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Bicornuate uterusBicornuate uterus
Endometrial cavities are widely separeted & deep indentation on the fundal
contour.
Endometrial cavities are widely separeted & deep indentation on the fundal
contour.
Two divergent uterine horns along with their endometrial plate and a large fundal cleft
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Bicornuate uterusBicornuate uterus
Bicornuate Bicollis Uterus Bicornuate Bicollis Uterus
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3D image which demonstrates a “heart” shaped bicornuate uterus. Fundal indentation is well seen (arrow) as well as the widely divergent horns (asterisks) with single cervix
Two Divergent Uterine Horns Along With Their Endometrial Plate And A
Large Fundal Cleft
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Gravid bicornuate uterus, pregnancy in right horn (RH). Gravid bicornuate uterus, pregnancy in right horn (RH).
Bicornuate uterus
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A transverse view of a bicornuate uterus. There is a single myometrial body with 2 distinct
endometrial cavities
Bicornuate uterus
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Bicornuate uterus
Gravid bicornuate uterus, pregnancy in right horn (RH).
Gravid bicornuate uterus, pregnancy in right horn (RH).
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Uterine Didelphis
Sonography of the
uterus shows 2
separate uteri, 2
separate cervices and
separate vagina in this
female patient.
This is an extreme
example of complete
failure of fusion of the
2 halves of the uterus
(Mullerian ducts)
during the fetal stage
Sonography of the
uterus shows 2
separate uteri, 2
separate cervices and
separate vagina in this
female patient.
This is an extreme
example of complete
failure of fusion of the
2 halves of the uterus
(Mullerian ducts)
during the fetal stage
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Uterine Didelphis
Double cavity uterus completely separate right and left cavities
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Uterine septum completely divides cavity from fundus to cervix
Septate uterus
Uterine out line is relatively normal.
Two endometrail cavity close together
And separated by a thin, fibrous septum.
The septum has poor blood supply & contain little myometrium if any.
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Septate uterus
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Fundal septum is seen in this patient with bicornuate uterus. The placenta is implanted posteriorly within the
right horn and attaches onto the septal wall.
Pregnant Septate uterus
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Septate uterus
A septate uterus showing two endometrial echoes
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Longitudinal section of the fetus showing its location belong the uterine septum within the amniotic cavity that had spread
to the both uterine horns.
Septate uterus
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The fetal head situated in the right uterine horn.
Pregnant Septate uterus
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Arcuate uterus
Transvaginal view of arcuate uterus with a small indentation in the fundal endometrium (large arrow). The outer contour of the uterus is smooth and normal appearing (small arrows)
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Anomalies of male reproductive organs
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Anomalies of male reproductive organs
Absence of prostate
Absence & duplication of seminal vesicle & vas deferens
Cysts of utricle
Atresia, stenoses & uplication of penile urethra
Agenesis & duplication of penis
Hypospadias & epispadias
Defects of erectile tissue
Mullerian & paramesonephric ducts remnants.
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