Mullerian Duct Anomalies

MULLERIAN DUCT ANOMALIES

Q. 1

Complete failure of mullerian duct fusion will re­sult in :

 A

Uterus didelphys

 B

Arcuate uterus

 C

Subseptate uterus

 D

Bicornuate uterus

Q. 1

Complete failure of mullerian duct fusion will re­sult in :

 A

Uterus didelphys

 B

Arcuate uterus

 C

Subseptate uterus

 D

Bicornuate uterus

Ans. A

Explanation:

Uterus didelphys

Index

Fig. Diagram of urogenital system : X – Intermediate cell mass.

Shaded areas is the genital ridge.

  1. Infundibulopelvic ligament.
  2. Ovary
  3. Ovarian ligament
  4. Round ligament.

Dotted outline is Wolffian duct (Gartner’s duct).

  1. Pronephros
  2. Epoophoron
  3. Mesonephros

Solid block is Mullerian ducts :

  1. Fimbria
  2. Fallopian tube
  3. Uterus
  4. Upper 3/4 vagina.

UGS : Urogenital sinus

Development of Mullerian ducts :

  • In the Th° week of intrauterine life of the embryo, an invagination of coelomic mesothelium occurs close to the primitive gonad, in the upper lateral part of the intermediate cell mass. This is called as Mullerian duct.
  • Two mullerian ducts develop, one on either side and grow caudally. They approach each other in the midline after crossing the Wolffian duct (see fig) and fuse.
  • The fused part projects into the urogenital sinus (UGS in figure).
  • The intervening portion is filled by proliferation of cells called as Sinovaginal bulb. These bulbs later canalize to form the lower vagina.

The Mullerian ducts form  :  

  • The Fallopian tubes° (develop from the cranial free part of the tube).
  • The uterus and cervix° (develops from middle part of the tube).
  • The upper three-fourth of the vagina° (develops from lower part of the tube).

–                     Initially when the two Mullerian ducts fuse, the intervening septum is present but later it disappears.

–                     When the two Mullerian ducts fail to fuse along the whole length, but develop normally and remain separate, the condition is called as Uterus Didelphys°.

–                     In uterus didelphys there are 2 vagina each having their own cervix and uterus i.e. in uterus didelphys there are two vagina, two cervix and two uterus°.


Q. 2

In complete mullerian duct aplasia all of the following are likely to be absent except:

 A

Ovaries

 B

Fallopian tubes

 C

Uterus

 D

Vagina

Q. 2

In complete mullerian duct aplasia all of the following are likely to be absent except:

 A

Ovaries

 B

Fallopian tubes

 C

Uterus

 D

Vagina

Ans. A

Explanation:

Ovaries

Friends here do not get confused – Ovaries are always normal in case of MRKH.

Theoretically both vagina and tubes are not found incase of MRKH but some part of tubes is always seen practically. For more details see answer 2


Q. 3

All of the following are features of mullerian agenesis except :

 A

46 XX karyotype

 B

Normal breast development

 C

Absent vagina

 D

Ovarian agenesis

Q. 3

All of the following are features of mullerian agenesis except :

 A

46 XX karyotype

 B

Normal breast development

 C

Absent vagina

 D

Ovarian agenesis

Ans. D

Explanation:

Ans:D.)Ovarian agenesis

Mullerian agenesis

  • It is Mayer Rokitansky-Kuster Hauser syndrome. .
  • Müllerian agenesis is a congenital malformation characterized by a failure of the Müllerian duct to develop, resulting in a missing uterus and variable degrees of vaginal hypoplasia of its upper portion. Müllerian agenesis (including absence of the uterus, cervix and/or vagina) is the cause in 15% of cases of primary amenorrhoea.
  • Because ovaries do not develop from the Müllerian ducts, affected women might have normal secondary sexual characteristics but are infertile due to the lack of a functional uterus.

Signs and Symptoms:

  • An individual with this condition is hormonally normal; that is, the person will enter puberty with development of secondary sexual characteristics including thelarche and adrenarche (pubic hair). The person’s chromosome constellation will be 46,XX. At least one ovary is intact, if not both, and ovulation usually occurs. Typically, the vagina is shortened and intercourse may, in some cases, be difficult and painful. Medical examination supported by gynecologic ultrasonography demonstrates a complete or partial absence of the cervix, uterus, and vagina.

Classification:

  • Typical MRKH – Isolated uterovaginal aplasia/hypoplasia
    • Prevalence – 64 percent
  • Atypical MRKH – Uterovaginal aplasia/hypoplasia with renal malformation or uterovaginal aplasia/hypoplasia with ovarian dysfunction
    • Prevalence – 24 percent
  • MURCS syndrome – Uterovaginal aplasia/hypoplasia with renal malformation, skeletal malformation, and cardiac malformation
    • Prevalence – 12 percent

Q. 4

The most important indication for surgical repair of a Bicornuate Uterus is :

 A

Infertility

 B

Dysmenorrhoea

 C

Menorrhagia

 D

Habitual abortion

Q. 4

The most important indication for surgical repair of a Bicornuate Uterus is :

 A

Infertility

 B

Dysmenorrhoea

 C

Menorrhagia

 D

Habitual abortion

Ans. D

Explanation:

Habitual abortion

Bicornuate uterus is caused by incomplete lateral fusion of the mullerian ducts is (only the lower parts of the duct fuse, leaving the cornua separate). it is characterized by 2 separate but communicating endometrial cavities and a single uterine cervix.

Failure of fusion may extend to the cervix resulting in a complete bicornuate uterus or may be partial causing a milder form.

Bicornuate uterus causes :

  • Menorrhagia° (because of larger bleeding surface)
  • Spasmodic dysmenorrhea.°

But most of the cases are first recognized during pregnancy as a result of pregnancy mishap like :

  • Abortions and premature labour.°
  • Malpresentations – Transverse lie is favoured.°
  • Insufficient uterine action leading to retained placenta and PPH.°

Diagnosis — On bimanual examination it may be possible to feel the two separate uterine horns or uterus may appear unusually wide.°

  • HSG is the initial diagnostic step in evaluating a possible bicornuate uterus but HSG does not differentiate between a bicornuate and septate uterus.
  • To distinguish a septate uterus from bicornuate uterus — USG should be done.

Management : Bicornuate uterus re•uires sur al treatment onl h- it causes habitual abortions.°

“When a bicornuate or se tate uterus has caused not less than three Miscarriages and no pregnancy has resulted in a viable child, surgery may be indicated.”  

“Surgical reconstruction of the bicornuate uterus has been advocated in women with multiple spontaneous abortions and in whom no other causative factors are identified.” 

Surgery done is : Stressmann Metroplasty°  where an incision is made over the uterus and the two horns are sutured together to form a single cavity.

After such a surgery : if woman conceives she should be taken up forEleativelaCaat 38 weeks of Gestation.°


Q. 5

Bicornuate uterus is due to :

 A

Incomplete fusion of uterine cavity

 B

Incomplete fusion of paramesonephric duct

 C

Incomplete fusion of mesonephric duct

 D

Incomplete formation of vagina

Q. 5

Bicornuate uterus is due to :

 A

Incomplete fusion of uterine cavity

 B

Incomplete fusion of paramesonephric duct

 C

Incomplete fusion of mesonephric duct

 D

Incomplete formation of vagina

Ans. B

Explanation:

Incomplete fusion of paramesonephric duct

Mullerian duct is synonymous to paramesonephric duct whereas wolffian duct is synonymous of Mesonephric duct.

Bicornuate uterus is due to incomplete fusion of Mullerian / Paramesonephric ducts.

Extra Edge

Fate of Para mesonephric Ducts (Mullerian ducts)o

In Females                                                 Males    

It forms                                                     They remain rudimentary

  • Fallopian tubes                             • The cranial end of each duct persists as appendix of testis.
  • Uterus                                           • It forms the prostatic utricle corresponding to vagina & uterus
  • Upper 3/4 of the vagina

Q. 6

MC congenital abnormality of uterus is :

 A

Uterus didelphys

 B

Arcuate

 C

Unicornuate

 D

Septate

Q. 6

MC congenital abnormality of uterus is :

 A

Uterus didelphys

 B

Arcuate

 C

Unicornuate

 D

Septate

Ans. D

Explanation:

Septate

Sorry for this one friends, 2 very reliable textbooks quote different incidences of different malformations.                                                                            

Anomaly

r

Per cent

Anomaly

Per cent’

Bicornuate uterus

37%

Septate uterus

35%

Arcuate uterus

15%.

Bicornuate uterus

26% I

Incomplete septum

13%’

Arcuate uterus

18%

Uterus didelphys

11%

Unicornuate uterus

10%

Complete septum

9%      –

Uterus didelphys

8%

Unicornuate uterus

4%

 

 

 

Now decide for yourself which book would you like to follow.


Q. 7

To diagnose uterus didelphys, procedure of choice is :

 A

Laparoscopy

 B

IVP

 C

HSG

 D

USG

Q. 7

To diagnose uterus didelphys, procedure of choice is :

 A

Laparoscopy

 B

IVP

 C

HSG

 D

USG

Ans. C

Explanation:

HSG

A didelphic uterus results when there is failed fusion of the paired mullearian ducts. This anomaly is characterised by the presence of two endometrial cavities each with a uterine cervix. A longitudnal vaginal septum runs between the two cervices in most of the cases.

“To confirm the diagnosis (of didelphic uterus) HSG is recommedned to identity the possibility of communication between the uterus.” …Williams Gynae. lle, p 417 As far as other options are concered –

Laparascopy can diagnose uterine didelphys accurately but is not the procedure of choice as it is an invasive procedure.

Option ‘b’ role of IVP in uterine anomalies is only to rule out associated renal anomalies.

Option ‘cl’ role of USG in uterine anomalies – For identificatiion of congenital malformation, standard two -dimensional transvaginal ultrasound compliments HSG and improves diagnostic accuracy in differentiating septate and bicornuate uteri by revealing the shape of the fundal contour.                                                                . 

Also know :

–  Of all the major uterine malformations, the didelphic uterus has the best reproducjtive prognosis.

–  Surgery should be reserved for highly selected women in whom repeated late trimester losses or premature delivery has occured with no apparent etiology.


Q. 8

Complete failure of mullerian duct fusion will result in :

 A

Uterus didephys

 B

Arcuate uterus

 C

Subseptate uterus

 D

Unicornuate uterus

Q. 8

Complete failure of mullerian duct fusion will result in :

 A

Uterus didephys

 B

Arcuate uterus

 C

Subseptate uterus

 D

Unicornuate uterus

Ans. A

Explanation:

Uterus didephys


Q. 9

In cases of recurrent abortions, most common uterine malformation seen is?

 A Mullerian fusion defects
 B

Uterine syncytium

 C Unicornuate uterus
 D

Uterine agenesis

Q. 9

In cases of recurrent abortions, most common uterine malformation seen is?

 A Mullerian fusion defects
 B

Uterine syncytium

 C Unicornuate uterus
 D

Uterine agenesis

Ans. A

Explanation:

Mullerian fusion defects REF: William’s 22′ ed chapter 9

Abnormal Mullerian duct formation or fusion defects may develop spontaneously or may follow in utero exposure to diethylstilbestrol is the commonest cause of uterine factors leading to recurrent spontaneous abortions.


Q. 10 The most important indication for surgical repair of a double uterus, such as a  septate or bicornuate uterus, is
 A Habitual abortion          
 B Dysmenorrhea
 C Menometrorrhagia
 D Dyspareunia
Q. 10 The most important indication for surgical repair of a double uterus, such as a  septate or bicornuate uterus, is
 A Habitual abortion          
 B Dysmenorrhea
 C Menometrorrhagia
 D Dyspareunia
Ans. A

Explanation:

Habitual abortion is the most important indication for surgical treatment of women who have a double uterus. The abortion rate in women who have a double uterus is to three times greater than that of the general population. Therefore, women who present with habitual abortion should be evaluated to detect a possible double uterus.  Hysterosalpingography, hysteroscopy, ultrasound, CT,  and  magnetic resonance imaging  (MRI)  are  all potentially useful imaging modalities in this investigation. Dysmenorrhea, premature delivery, dyspareunia, and menometrorrhagia are other, less important indicators for surgical intervention.


Q. 11

Complete failure of Mullerian duct fusion will result in:

 A

Uterus didelphys

 B

Arcuate uterus

 C

Subseptate uterus

 D

Unicornuate uterus

Q. 11

Complete failure of Mullerian duct fusion will result in:

 A

Uterus didelphys

 B

Arcuate uterus

 C

Subseptate uterus

 D

Unicornuate uterus

Ans. A

Explanation:

In uterus didelphys there is complete lack of fusion of Mullerian ducts with a double uterus, double cervix, and a double vagina.

Ref: Shaw’s Textbook of Gynaecology, 12th Edition, Page 62 ; Textbook of Gynecology By D C Dutta, 4th Edition, Page 42


Q. 12

In complete mullerian duct aplasia all of the following are likely to be absent, EXCEPT:

 A

Ovaries

 B

Fallopian tubes

 C

Uterus

 D

Vagina

Q. 12

In complete mullerian duct aplasia all of the following are likely to be absent, EXCEPT:

 A

Ovaries

 B

Fallopian tubes

 C

Uterus

 D

Vagina

Ans. A

Explanation:

The ovaries develop from the gonadal ridge, where as the the uterus, vagina and fallopian tubes develop from the mullerian duct. Hence development of ovaries is not affected by mullerian duct agenesis.

Ref: Embryology By Ronald W. Dudek, James D. Fix, pages 148-54; Knobil and Neill’s Physiology of Reproduction, Volume 1 By Ernst Knobil, Jimmy D. Neill, pages 327-31


Q. 13

Which of the following condition present with absence of both Mullerian and Wolffian duct structures:

 A

Antimullerian Hormone deficiency

 B

Androgen Insensitivity Syndrome

 C

FSH Receptor defect

 D

Ovotesticular syndrome

Q. 13

Which of the following condition present with absence of both Mullerian and Wolffian duct structures:

 A

Antimullerian Hormone deficiency

 B

Androgen Insensitivity Syndrome

 C

FSH Receptor defect

 D

Ovotesticular syndrome

Ans. B

Explanation:

Both Wolffian duct structures and Mullerian duct structures do not develop in individuals with Androgen Insensitivity Syndrome.
 
Ref: Robboy’s Pathology of the Female Reproductive Tract, 2008, 2nd Edition, Chapter 1; Blaustein’s Pathology of the Female Genital Tract, 2002, 5th Edition, Chapter 1; Pediatric Endocrinology’ By Sperling, 3rd Edition, Chapter 5; Textbook of Gynecology By Sudha Salhan, 2012, Page 198

Q. 14

Which of the following Mullerian duct anomaly is associated with the presence of two cervixes?

 A

Uterus didelphys

 B

Uterus bicornis unicollis

 C

Uterus subseptus

 D

Uterus unicornis with a rudimentary horn

Q. 14

Which of the following Mullerian duct anomaly is associated with the presence of two cervixes?

 A

Uterus didelphys

 B

Uterus bicornis unicollis

 C

Uterus subseptus

 D

Uterus unicornis with a rudimentary horn

Ans. A

Explanation:

When the 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 cervixes. It is also associated with two vaginas.

Ref: Shaw’s Textbook of Gynaecology, 12th Edition, Page 62.

Q. 15

Normal development of ovaries in a female with absent uterus and vagina indicates which of the following genetic conditions?

 A

Turner’s syndrome

 B

Testicular feminizing syndrome

 C

Mullerian agenesis

 D

Gonadal dysgenesis

Q. 15

Normal development of ovaries in a female with absent uterus and vagina indicates which of the following genetic conditions?

 A

Turner’s syndrome

 B

Testicular feminizing syndrome

 C

Mullerian agenesis

 D

Gonadal dysgenesis

Ans. C

Explanation:

Mullerian agenesis is the failure of the paramesonephric ducts to develop. In müllerian agenesis, the ovaries are not affected and thus ovarian function is normal.

Secondary sexual development and height are in a normal range. The condition is also called MRKH or Mayer-Rokitansky-Kustner-Hauser Syndrome.

Ref: Clinical Reproductive: Medicine and Surgery By Tommaso Falcone, William W. Hurd, William W. Hurd, 2007, Page 237 ; Shaw, 13th ed, Page 87


Q. 16

A 19 year old patient came to the out patient department with complaints of primary amenorrhea. She had well developed breast and pubic hair. However there was absence of vagina and uterus. Likely diagnosis is:

 A

XYY

 B

Mullerian agenesis

 C

Gonadal dysgenesis

 D

Klinefelter’s syndrome

Q. 16

A 19 year old patient came to the out patient department with complaints of primary amenorrhea. She had well developed breast and pubic hair. However there was absence of vagina and uterus. Likely diagnosis is:

 A

XYY

 B

Mullerian agenesis

 C

Gonadal dysgenesis

 D

Klinefelter’s syndrome

Ans. B

Explanation:

Müllerian agenesis (Mayer-Rokitansky-Küster-Hauser syndrome) is the second most common cause of primary amenorrhea. These individuals have normal ovarian development, normal endocrine function, and normal female sexual development. The physical findings are a shortened or absent vagina in addition to absence of the uterus, although small masses resembling a rudimentary uterus may be noted.

These individuals have a 46,XX karyotype. 

Ref: Rosen M.P., Cedars M.I. (2011). Chapter 13. Female Reproductive Endocrinology and Infertility. In D.G. Gardner, D. Shoback (Eds), Greenspan’s Basic & Clinical Endocrinology, 9e.

Q. 17

Which is the most common uterine malformation seen in cases of recurrent abortions?

 A

Mullerian fusion defects

 B

Uterine syncytium

 C

Unicornuate uterus

 D

Uterine agenesis

Q. 17

Which is the most common uterine malformation seen in cases of recurrent abortions?

 A

Mullerian fusion defects

 B

Uterine syncytium

 C

Unicornuate uterus

 D

Uterine agenesis

Ans. A

Explanation:

Mullerian duct anomaly is an important cause of recurrent miscarriage in early and midtrimester. 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.


Q. 18

Which among the following is the MOST common congenital uterine malformation that causes recurrent abortions?

 A

Mullerian fusion defects

 B

Uterine syncytium

 C

Cervical incompetence

 D

Uterine agenesis

Q. 18

Which among the following is the MOST common congenital uterine malformation that causes recurrent abortions?

 A

Mullerian fusion defects

 B

Uterine syncytium

 C

Cervical incompetence

 D

Uterine agenesis

Ans. A

Explanation:

Anatomical abnormalities are responsible for 10-15% of recurrent abortion.

Congenital and acquired anomalies.

Congenital anomalies are due to defects in the Mullerian duct fusion or resorption (e.g. unicornuate, bicornuate, septate or double uterus). This causes about 12% cases of recurrent abortions.

Acquired anomalies are,
  • Intrauterine adhesions
  • Uterine fibroids
  • Endometrosis
  • Cervical incompetence
Ref: Textbook of Obstetrics by D C Dutta, 6th edition, Page 169.

Q. 19

A 19 year old patient presents with primary amenorrhoea. She has well developed breasts and axillary and pubic hair. Uterus and vagina are absent. The diagnosis is most likely:

 A

XYY syndrome

 B

Gonadal dysgenesis

 C

Mullerian agenesis

 D

Klinefelter’s syndrome

Q. 19

A 19 year old patient presents with primary amenorrhoea. She has well developed breasts and axillary and pubic hair. Uterus and vagina are absent. The diagnosis is most likely:

 A

XYY syndrome

 B

Gonadal dysgenesis

 C

Mullerian agenesis

 D

Klinefelter’s syndrome

Ans. C

Explanation:

Müllerian agenesis (Mayer-Rokitansky-Küster-Hauser syndrome) is the second most common cause of primary amenorrhea.

Clinical features:

  • Normal ovarian development
  • Normal endocrine function
  • Normal female sexual development
  • Shortened or absent vagina in addition to absence of the uterus
  • Individuals have 46,XX karyotype

Ref: Rosen M.P., Cedars M.I. (2011). Chapter 13. Female Reproductive Endocrinology and Infertility. In D.G. Gardner, D. Shoback (Eds),Greenspan’s Basic & Clinical Endocrinology, 9e.


Q. 20

A 7-year-old child presents to the paediatric clinic with amiguous genitalia increasing with age. On examination her height, weight and blood pressure were recorded within normal limits. Labia appeared bifid with 2 separate perineal openings, phallic length was 2.5 cm and no palpable gonads were noted in the inguinal region. USG shows presence of mullerian structures. The most probable diagnosis is-

 A

Classic Salt-Wasting 21 Hydroxylase deficiency

 B

Simple virilizing congenital adrenal hyperplasia

 C

Complete Androgen Insensitivity Syndrome

 D

5-Alpha Reductase Deficiency

Q. 20

A 7-year-old child presents to the paediatric clinic with amiguous genitalia increasing with age. On examination her height, weight and blood pressure were recorded within normal limits. Labia appeared bifid with 2 separate perineal openings, phallic length was 2.5 cm and no palpable gonads were noted in the inguinal region. USG shows presence of mullerian structures. The most probable diagnosis is-

 A

Classic Salt-Wasting 21 Hydroxylase deficiency

 B

Simple virilizing congenital adrenal hyperplasia

 C

Complete Androgen Insensitivity Syndrome

 D

5-Alpha Reductase Deficiency

Ans. B

Explanation:

Ans. is ‘b’ i.e., Simple virilizing congenital adrenal hyperplasia

  • Among the given options only 21 hydroxylase is the common cause of ambiguous genitalia.
  • Now the confusion arises, because 21-hydroxylase deficiency typically presents with salt wasting and hypotension, whereas the child in question is having normal BP.
  • Actually, some forms (simple virilizing form) of 21-hydroxylase deficiency have normal BP and no salt loss. 

Type of 2 I -hydroxylase deficiency

(A) Classic salt wasting form

o This is a severe form of deficiency characterized by features of both glucocorticoid (cortisol)/mineralocorticoid

deficiency (salt wasting) and androgen excess. (Ambiguous genitalia in females). Males may be normal at birth. o This form typically manifests early, between 7-21 days of life and is a potentially life threatening condition. Classic simple virilizing form

o Patients with classic simple virilizing form have impaired cortisol biosynthesis but do not develop salt loss. o Clinical features are caused solely by overproduction of androgens.

o The only manifestation is ambiguous genitalia in a female child with varying degrees of clitoral enlargement and labial fusion.

o Depending on severity of clitoral enlargement and labial fusion, the diagnosis may be established at birth. However such patients may present in early childhood.

(C) Non-classic (aquired/late) from

o Patients with non-classical form or late onset form produce normal amount of cortisol and/or aldosterone, but at the expense of producing excess androgens.

o These usually present in childhood or early adulthood with premature pubarche & symptoms/signs of PCOD. Hirsuitism, Acne and Oligomenorrhea are the most common presenting features.


Q. 21

Spigelian hernia is:      

March 2005

 A

Hernia passing through the obturator canal

 B

Hernia occurring through the linea alba

 C

Hernia through the triangle of petit

 D

Hernia occurring at the level of arcuate line

Q. 21

Spigelian hernia is:      

March 2005

 A

Hernia passing through the obturator canal

 B

Hernia occurring through the linea alba

 C

Hernia through the triangle of petit

 D

Hernia occurring at the level of arcuate line

Ans. D

Explanation:

Ans. D: Hernia occurring at the level of arcuate line

Spigelian hernia is an acquired ventral hernia through the linea semilunaris, the line where the sheaths of the lateral abdominal muscles fuse to form the lateral rectus sheath, at the level of arcuate line.

Spigelian hernias are nearly always found above the level of the inferior epigastric vessels.

Commonly, the patient is over the age of 50 years. Men and women are equally affected.

The patient presents with pain that is localised to the hernial site and is aggravated by any movement that raises intra­abdominal pressure. Later, the pain becomes more dull, constant, and diffuse.

A soft, reducible mass may be present in the lower abdominal area which disappears on pressure. When the mass is reduced, the hernial orifice can usually be palpated.

Diagnosis is more difficult when the hernia dissects within the layers of the abdominal wall – internal and external obliques – or may be located at a distance from the linea semilunaris.

Ultrasound and CT scan may help to confirm the diagnosis.


Q. 22

A 16-year-old female with normal pubic hair and breast development, presented with complaints of primary amenorrhea. Investigation shows normal karyotype. Most likely diagnosis is:

AFMC 12; JIPMER 12; AIIMS 12, 13

 A

Mullerian agenesis

 B

Turner syndrome

 C

Testicular feminization

 D

Kallmann syndrome

Q. 22

A 16-year-old female with normal pubic hair and breast development, presented with complaints of primary amenorrhea. Investigation shows normal karyotype. Most likely diagnosis is:

AFMC 12; JIPMER 12; AIIMS 12, 13

 A

Mullerian agenesis

 B

Turner syndrome

 C

Testicular feminization

 D

Kallmann syndrome

Ans. A

Explanation:

Ans. Mullerian agenesis


Q. 23

Structure developing from Mullerian duct in males‑

 A

Seminal vesicle

 B

Epididymis

 C

Prostatic utricle

 D

Ureter

Q. 23

Structure developing from Mullerian duct in males‑

 A

Seminal vesicle

 B

Epididymis

 C

Prostatic utricle

 D

Ureter

Ans. C

Explanation:

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

i) Appendix of testis (Hydatid of Morgagni)

ii) Prostatic utricle.


Q. 24

The most common congenital anomaly of an organ as marked by an arrow shown in the photograph below is ? 

 A

Unicornuate.

 B

Bicornuate.

 C

Septate.

 D

Arcuate.

Q. 24

The most common congenital anomaly of an organ as marked by an arrow shown in the photograph below is ? 

 A

Unicornuate.

 B

Bicornuate.

 C

Septate.

 D

Arcuate.

Ans. C

Explanation:

Organ marked in the picture above represents uterus.

septate uterus is a common type of congenital uterineanomaly, and it may lead to an increased rate of pregnancy loss. The main imaging differential diagnoses are arcuateuterus and bicornuate uterus.


Q. 25

 Diagnose the Uterine condition shown on Hysterosalpingogram 

 A

 Unicornuate uterus 

 B

 Bicornuate uterus 

 C

Arcuate uterus 

 D

Normal uterus

Q. 25

 Diagnose the Uterine condition shown on Hysterosalpingogram 

 A

 Unicornuate uterus 

 B

 Bicornuate uterus 

 C

Arcuate uterus 

 D

Normal uterus

Ans. B

Explanation:

Bicornuate uterus 

It is possible to diagnose a bicornuate uterus using gynecologic ultrasonography, specifically sonohysterography, and MRI.However, as there is no indication to do such procedures on asymptomatic women, the presence of a bicornuate uterus may not be detected until pregnancy or delivery. In a C-section (usually done due to malpresentation), the irregular shape of the uterus will be apparent.

Other less reliable diagnostic imaging methods include hysterosalpingography and hysteroscopy; these procedures are typically done during the course of an infertility investigation.


Q. 26

ldentify the uterine anomaly shown in the photograph below ? 

 A

Hypoplasia uterus.

 B

Unicornuate uterus.

 C

Bicornuate uterus.

 D

Uterus didelphus.

Q. 26

ldentify the uterine anomaly shown in the photograph below ? 

 A

Hypoplasia uterus.

 B

Unicornuate uterus.

 C

Bicornuate uterus.

 D

Uterus didelphus.

Ans. D

Explanation:

 The uterine anomaly shown in the photograph above represents Uterus didelphus.

Uterus didelphus is a type of  mullerian duct anomaly (class III) where there is a complete duplication of uterine horns as well as duplication of the cervix, with no communication between them.  Didelphic uteri account for approximately ~8% (range 5-11%) of Müllerian duct anomalies.

It results from failed ductal fusion that occurs between the 12th and 16th week of pregnancy and is characterised by two symmetric, widely divergent uterine horns and two cervices. The uterine volume in each duplicated segment is reduced. As with most uterine anatomical anomalies, there is an increased incidence of fertility issues, and Müllerian abnormalities, in general, are over-represented in infertile women. The chance of seeing a pregnancy to term is significantly reduced, down to only 20%, with a third of pregnancies ending in abortion and over half in premature deliveries. Only 40% of pregnancies resulted in living children.



Q. 27

Identify the uterine anomaly shown in the photograph below ? 

 A

Unicornuate.

 B

Hypoplasia uterus.

 C

Bicornuate uterus.

 D

Uterus didelphus.

Q. 27

Identify the uterine anomaly shown in the photograph below ? 

 A

Unicornuate.

 B

Hypoplasia uterus.

 C

Bicornuate uterus.

 D

Uterus didelphus.

Ans. C

Explanation:

The uterine anomaly shown in the photograph above represents Bicornuate uterus.

bicornuate uterus or bicornate uterus , commonly referred to as a “heart-shaped” uterus, is a uteruscomposed of two “horns” separated by a septum. In humans, a bicornuate uterus is a type of uterine malformation.


Q. 28

A 19 years old patient came to the out patient department with complaints of primary amenorrhea. She had well-developed breast and pubic hair. However there was absence of vagina and uterus but normal ovaries. Likely diagnosis is:

 A

XYY

 B

Mullerian agenesis

 C

Gonadal dysgenesis

 D

Klinefelter’s syndrome

Q. 28

A 19 years old patient came to the out patient department with complaints of primary amenorrhea. She had well-developed breast and pubic hair. However there was absence of vagina and uterus but normal ovaries. Likely diagnosis is:

 A

XYY

 B

Mullerian agenesis

 C

Gonadal dysgenesis

 D

Klinefelter’s syndrome

Ans. B

Explanation:

Ans. b. Mullerian agenesis

  • Mullerian agenesis is the cause of primary amenorrhea, which is characterized by absence of uterus /vagina, also known as Rokitansky-Kuster- Hausen syndrome.


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