Mullerian Duct Anomalies

Ans. is B. i.e. Mullerian duct

• Paired ducts derived from intermediate mesoderm.
• Known as a paramesonephric duct. 
• 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 the opposite side.
• Parts 
  1. Upper vertical part lateral to Wolffian duct → fallopian tube
  2. Middle horizontal part crossing Wolffian duct → remaining part of the fallopian tube
  3. Lower vertical part fusing to the opposite part → uterus, cervix, upper 1/3 rd. of the vagina
  4. In forming the uterus, the Mullerian duct fuses from below upwards.

Ans. is A. i.e. Ovaries

• Congenital absence of both the uterus and vagina due to absence of both Mullerian ducts are termed as Mullerian Agenesis or MRKH syndrome ( Mayer Rokitansky Kuster Hauser Syndrome)
• In classic Mullerian agenesis, patients have shallow vaginal pouch measuring only 1 or 2 inches deep.
• Also, the uterus, cervix and upper vagina are absent.
• Typically ovaries are normal as they arise from the genital ridge and a part of distal fallopian tubes is present.
• Karyotype = 46XX0
• Phenotype = Female

Ans. is A. i.e Uterus didelphys

• When the Mullerian ducts fail to fuse along their entire length, develop completely and separately the result is uterus didelphys.
• In this case, there will be two uterus and hence two cervices. It is also associated with two vaginas.
• It is associated with complete duplication of uterine horns as well as duplication of the cervix, with no communication between them.  
• It is mostly asymptomatic & there is a complete failure of Mullerian duct fusion.
• Dyspareunia may be seen as a result of vaginal septum.

Ans. is A. i.e. Urogenital sinus

• The major part of the female genital tract develops from The Mullerian ducts.
• Development of Mullerian Ducts/ Paramesonephric Ducts in Females in the 5th-6th week of intrauterine life of the embryo.
• Mullerian ducts develop as an invagination of intermediate cell mass. Two Mullerian ducts develop, one on either side and grow caudally. They approach each other in the midline after crossing the Wolffian duct and fuse.
• Fusion begins by 7-8 weeks and is completed by 12 weeks.
• Fusion proceeds in below upwards direction.
• Initially, when the two Mullerian ducts fuse, an intervening septum is present, but later by the 5th month of intrauterine life, it also disappears.
• Thus each side Mullerian duct forms each side Fallopian tube, each half of uterus, each half of Cervix, each half of the upper vagina.
• Whereby 5th month (20 weeks) septa disappear, a single uterine cavity is formed.
• Till now the fundus of the uterus is flat. In the last step fundus of the uterus becomes dome-shaped.

Development of Vagina:-

• Vagina develops from two sources:
• Mainly from the Mullerian duct which forms upper 2/3 part of the vagina (i.e. It is mesodermal in origin)
• Partly from the urogenital sinus/sinovaginal bulb which forms lower 1/3rd part of the vagina. The lower third thus is endodermal in origin
• Hymen represents the remnant of the sinovaginal bulb. Together they form a solid vaginal plate.
• Canalization of the solid vaginal plate occurs at 20 weeks
• If this canalization fails to occur it leads to Transverse Vaginal Septum.
• The mucous membrane or epithelium of the vagina is derived from the endoderm of urogenital sinus and muscles from mesoderm of Mullerian duct

Development of External Genitalia

• External genitalia is derived from the dorso somatic part of the lateral part mesoderm.

Ans. is B. i.e. Mullerian Duct

• The major part of the female genital tract develops from the Mullerian ducts.
• Development of Mullerian Ducts/ Paramesonephric Ducts in females occurs in the 5th-6th week of intrauterine life of the embryo.
• Mullerian ducts develop as an invagination of intermediate cell mass. Two Mullerian ducts develop, one on either side and grow caudally. They approach each other in the midline after crossing the Wolffian duct and fuse.
• Fusion begins by 7-8 weeks and is completed by 12 weeks.
• Fusion proceeds in below upwards direction.
• Initially, when the two Mullerian ducts fuse, an intervening septum is present, but later by the 5th month of intrauterine life, it also disappears.
• Thus each side Mullerian duct forms each side Fallopian tube, each half of uterus, each half of Cervix, each half of the upper vagina.
• Whereby 5th month (20 weeks) septa disappear, a single uterine cavity is formed.
• Till now the fundus of the uterus is flat. In the last step fundus of the uterus becomes dome-shaped.

Development of Vagina:-

• Vagina develops from two sources:
• Mainly from the Mullerian duct which forms upper 2/3 part of the vagina (i.e. It is mesodermal in origin)
• Partly from the urogenital sinus/sinovaginal bulb which forms lower 1/3rd part of the vagina. The lower third thus is endodermal in origin
• Hymen represents the remnant of sinovaginal bulb. Together they form a solid vaginal plate.
• Canalization of the solid vaginal plate occurs at 20 weeks
• If this canalization fails to occur it leads to Transverse Vaginal Septum.
• The mucous membrane or epithelium of the vagina is derived from the endoderm of urogenital sinus and muscles from mesoderm of Mullerian duct

Development of External Genitalia

• External genitalia is derived from the dorso somatic part of the lateral part mesoderm.

Ans. is A. i.e Uterus didelphys

• When the Mullerian ducts fail to fuse along their entire length, develop completely, and separately the result is uterus didelphys.
• In this case, there will be two uteri and hence two cervices. It is also associated with two vaginas.
• It is associated with complete duplication of uterine horns as well as duplication of the cervix, with no communication between them.  
• It is mostly asymptomatic & there is a complete failure of Mullerian duct fusion.
• Dyspareunia may be seen as a result of vaginal septum.

Ans. is C. i.e. Mullerian agenesis

Clinical features of MRKH/ Mullerian agenesis

• The patient presents between 15-18 years of age with primary amenorrhea (due to the absence of uterus).
• Secondary sexual characteristics are normal as ovaries are normal (because ovaries do not develop from Mullerian duct but genital ridge, so ovulation is also normal) i.e. breast, pubic hair and axillary hair all are normal.
• Per Rectal examination: Vagina is felt like a blind pouch and uterus is absent.
• Although in MRKH syndrome ( Mayer Rokitansky Kuster Hauser Syndrome)  fallopian tube should be absent, typically a part of the distal tube is present (distal 1/3^rd present).
• Findings are confirmed by USG

Ans. is A. i.e. Mullerian fusion defects

• Mullerian duct anomaly is an important cause of recurrent miscarriage in early and mid-trimester.
• Septate or arcuate uterus is the most common uterine anomaly associated with Mullerian fusion defects and it is the most common defect associated with repeated pregnancy loss.
• In septate uterus: both Mullerian duct fuse & form septa but septa fails to resolve. So the uterus is outwardly normal but contains a complete or incomplete septum inside.
• In the arcuate uterus, the fundus of the uterus remains flat & does not become dome-shaped.
• on HSG in the septate uterus, an angle of less than 75° between the uterine horns is suggestive of a septate uterus, and an angle of more than 105° is more consistent with bicornuate uteri.
• Management for septate uterus:

1. Earlier: Jones/Tompkins Metroplasty was done.
2. Nowadays: Hysteroscopic resection of septa is being done after inducing endometrial atrophy by administering the GnRH analogue for 2 months.

• Main complications: Uterus perforation and fluid overload.

Ans. is A. i.e. Mullerian duct

• The Upper three-quarter of the vagina is developed from the fusion of the paramesonephric (Mullerian) duct.
• Lower one fourth is developed by the canalization of the sinovaginal bulbs which form by the proliferation of the urogenital sinus. 
• Structures developing from the Mullerian duct are:

1. Fallopian tubes
2. Uterus and cervix
3. Upper three fourth of vagina

Ans. is A. i.e. Mullerian agenesis

Clinical features of MRKH/ Mullerian agenesis

• The patient presents between 15-18 years of age with primary amenorrhea (due to the absence of uterus).
• Secondary sexual characteristics are normal as ovaries are normal (because ovaries do not develop from Mullerian duct but the genital ridge, so ovulation is also normal) i.e. breast, pubic hair and axillary hair all are normal.
• Per Rectal examination: Vagina is felt like a blind pouch and uterus is absent.
• Although in MRKH syndrome ( Mayer Rokitansky Kuster Hauser Syndrome)  fallopian tube should be absent, typically a part of the distal tube is present (distal 1/3^rd present).
• Findings are confirmed by USG.

Ans. is C. i.e. Mullerian duct

• Mesonephric duct (Wolffian duct) is the main genital duct in males as it gives rise to mainly the male genital system.
• Paramesonephric duct (Mullerian duct) gives rise to the mainly female genital tract.
• The major part of the female genital tract develops from The Mullerian ducts.
• Development of Mullerian Ducts/ Paramesonephric Ducts in Females is in the 5th-6th week of intrauterine life of the embryo.
• Mullerian ducts develop as an invagination of intermediate cell mass. Two Mullerian ducts develop, one on either side and grow caudally. They approach each other in the midline after crossing the Wolffian duct and fuse.
• Fusion begins by 7-8 weeks and is completed by 12 weeks.
• Fusion proceeds in below upwards direction.
• Initially, when the two Mullerian ducts fuse, an intervening septum is present, but later by the 5th month of intrauterine life, it also disappears.
• Thus each side Mullerian duct forms each side Fallopian tube, each half of uterus, each half of Cervix, each half of the upper vagina.
• When by 5th month (20 weeks) septa disappear, a single uterine cavity is formed.
• Till now the fundus of the uterus is flat. In the last step fundus of the uterus becomes dome-shaped.

Development of Vagina:-

• Vagina develops from two sources:
• Mainly from the Mullerian duct which forms upper 2/3 part of the vagina (i.e. It is mesodermal in origin).
• Partly from the urogenital sinus/sinovaginal bulb which forms lower 1/3rd part of the vagina. The lower third thus is endodermal in origin.
• Hymen represents the remnant of the sinovaginal bulb. Together they form a solid vaginal plate.
• The canalization of the solid vaginal plate occurs at 20 weeks.
• If this canalization fails to occur it leads to Transverse Vaginal Septum.
• The mucous membrane or epithelium of the vagina is derived from the endoderm of the urogenital sinus and muscles from the mesoderm of the Mullerian duct.

Development of External Genitalia

• External genitalia is derived from the dorso somatic part of the lateral part mesoderm.

 Ans. is D. i.e. Mullerian duct

• The appendix testis (or hydatid of Morgagni) is a vestigial remnant of the Mullerian duct, present on the upper pole of the testis and attached to the tunica vaginalis.
• It is present about 90% of the time.
• The appendix of the testis can, occasionally, undergo torsion (i.e. become twisted), causing acute one-sided testicular pain and may require surgical excision to achieve relief.
• One-third of patients present with a palpable “blue dot” discoloration on the scrotum.
• Another structure developing from the Mullerian duct in males is a Prostatic utricle.

Ans. is C. i.e.  Prostatic utricle

Remnants of Mullerian duct (paramesonephric duct) in males are:-

• Appendix of the testis (Hydatid of Morgagni) & Prostatic utricle.
• The development of the paramesonephric (Müllerian) ducts is controlled by the presence or absence of anti- Mullerian hormone (AMH).
• In males: The developing testes produce AMH causing regression of paramesonephric ducts.

Ans. is A. i.e. Mullerian duct

• Organ shown in the picture above represents uterus.
• Uterus develops from the Mullerian duct.
• The Mullerian ducts are the primordial anlage of the female reproductive tract.
• They differentiate to form the fallopian tubes, uterus, the uterine cervix, and the superior aspect of the vagina

Parts formed by Mullerian duct

• Upper vertical part lateral to Wolffian duct → fallopian tube
• Middle horizontal part crossing Wolffian duct → remaining part of the fallopian tube
• Lower vertical part fusing to the opposite part → uterus, cervix, upper 1/3 rd. of the vagina
• In forming the uterus, the Mullerian duct fuses from below upwards.

Ans.B. Mullerian agenesis

Clinical features of MRKH/ Mullerian agenesis

• The patient presents between 15-18 years of age with primary amenorrhea (due to the absence of uterus).
• Secondary sexual characteristics are normal as ovaries are normal (because ovaries do not develop from Mullerian duct but genital ridge, so ovulation is also normal) i.e. breast, pubic hair and axillary hair all are normal.
• Per Rectal examination: Vagina is felt like a blind pouch and uterus is absent.
• Although in MRKH syndrome ( Mayer Rokitansky Kuster Hauser Syndrome)  fallopian tube should be absent, typically a part of the distal tube is present (distal 1/3^rd present).
• Findings are confirmed by USG

Ans. is ‘A’ i.e., Mullerian duct

• Known as a paramesonephric duct.
• Grows downward & lateral to corresponding Wolffian ducts.
• The development of the paramesonephric (Müllerian) ducts is controlled by the presence or absence of Anti- Mullerian Hormone (AMH).

Parts 

• Upper vertical part lateral to Wolffian duct → fallopian tube
• Middle horizontal part crossing Wolffian duct → remaining part of the fallopian tube.
• Lower vertical part fusing to the opposite part → uterus, cervix, upper 1/3 rd. of the vagina.
• In forming the uterus, the Mullerian duct fuses from below upwards.

Ans. is A. Retention cyst in remnants of Wolffian duct

• Gartner’s cyst is cysts of the remnants of Wolffian duct.
• Main location: Anterolateral wall of the vagina, hence are often confused with cystocele.
• Rugosities of the overlying vaginal mucosa is lost and vaginal mucosa over it becomes tense and shiny.
• Margins are well defined. Not reducible & no impulse on coughing

Ans. is D. i.e.  Septate uterus

• Septate uterus is the most common Mullerian anomaly.
• In this condition; both Mullerian duct fuse & form septa but septa fail to resolve.
• So the uterus is outwardly normal but contains a complete or incomplete septum inside.
• Increased rate of pregnancy loss. 
• On HSG: An angle of less than 75° between the uterine horns is suggestive of a septate uterus, and an angle of more than 105° is more consistent with bicornuate uteri.