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Developmental Anomalies Of Female Reproductive OrgansDevelopmental Anomalies Of Female Reproductive Organs

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.

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

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

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

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

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.

 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)

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

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

Failure Of Resorption Of The Median SeptumFailure Of Resorption Of The Median Septum

Total (Uterine septus).

Partial (Uterine sub-

septus)                

Arcuate.

Haemocolpos And Imperforate Hymen Haemocolpos And Imperforate Hymen

Hematocolpos secondery to imperforate hymenHematocolpos secondery to imperforate hymen

Haemometra And Imperforate Hymen Haemometra And Imperforate Hymen

Hematometra secondery to imperforate hymenHematometra secondery to imperforate hymen

Haematosalpinx And Imperforate Hymen Haematosalpinx And Imperforate Hymen

Anechoic tubular structures in the adnexa Anechoic tubular structures in the adnexa

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.

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.

Septate vagina Septate vagina

Sonohysterogram (saline infusion sonography)

Sonohysterogram (saline infusion sonography)

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)

Uterine HypoplasiaUterine Hypoplasia

Very reduced volume of uterus- hypoplastic uterusVery reduced volume of

uterus- hypoplastic uterus T-shaped uterusT-shaped uterus

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.

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.

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)..

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.

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

Bicornuate uterusBicornuate uterus

 Bicornuate Bicollis Uterus  Bicornuate Bicollis Uterus

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

Gravid bicornuate uterus, pregnancy in right horn (RH).  Gravid bicornuate uterus, pregnancy in right horn (RH). 

Bicornuate uterus

A transverse view of a bicornuate uterus. There is a single myometrial body with 2 distinct

endometrial cavities

Bicornuate uterus

Bicornuate uterus

Gravid bicornuate uterus, pregnancy in right horn (RH). 

Gravid bicornuate uterus, pregnancy in right horn (RH). 

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

Uterine Didelphis

Double cavity uterus completely separate right and left cavities

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.

Septate uterus

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

Septate uterus

A septate uterus showing two endometrial echoes

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

The fetal head situated in the right uterine horn.

Pregnant Septate uterus

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)

Anomalies of male reproductive organs

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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