MRCOG tutorial. 1st. February 2024

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1	How to prepare. Part 3.  Communication skills. Study partners. Importance of polished introduction to role-plays. ? adopt user-friendly name for role-play if you are ‘Clementina’, ‘Cressida’ ‘Maximilial’ or ‘Vladymir’. ‘Blurbs’ to deal with GP referral letter. Senior doctor thinking: staffing, training, audit, critical incident reporting and analysis etc. Avoiding medical jargon, abbreviations and acronyms ‘registrar’, ‘anaphylaxis’, PCOS, PMB – OK if explained first. Picking a course. Retaining Part 2 knowledge.
2	Part 3. The domains and creating an agenda. Safety must top your list.
3	Structured conversation.
4	Basic “blurbs” to write and practise. Setting the scene for breaking bad news, dealing with the GP referral letter, general pre-pregnancy counselling, recessive inheritance, x-linked inheritance, how to ask if the role-player has questions, dealing with information such as a relative with a serious problem. Normalising the abnormal before asking difficult questions – ‘many women in this situation find that they have scary thoughts about killing themself or the baby. Has this happened to you?’ Blurb to recognise the enormity of such an experience, but no reference to you – never ‘I feel your pain’ or its ilk. Dealing with an angry patient. Make a list.
5	Last-minute revision list. Resurrect your Part 2 list and amend it as the weeks go by. 1ry. amenorrhoea. Cancer staging. Explain balanced translocation.
6	Role-play. Woman attends for pre-pregnancy counselling as she plans her 1st. pregnancy. Her sister recently had a baby with Down’s syndrome..
7	EMQ. Mayer-Rokitansky-Küster-Hauser syndrome

 

1.     Part 3.  How to prepare.

 

2.     The domains and creating an agenda.

 

3.     Structured conversation. The examiner will ask a series of questions.

 

4.     Basic “blurbs” to write and practise.

 

5.     Last-minute revision list.

 

6.     Role-play. Candidate’s instructions will be emailed shortly before the tutorial begins.

 

7.     EMQ. Mayer-Rokitansky-Küster-Hauser syndrome.

Note. Some of the questions are not true EMQs as there may be more than one correct answer – this is me being lazy and saving typing.

Mayer–Rokitansky–K

¨

uster–Hauser

syndrome: diagnosis and management

With regard to the MRKH syndrome,

61. there is failure of development of the

mesonephric ducts. T F

62. the phenotype and genotype are female. T F

63. studies have established a link between the

syndrome and the use of diethylstilbestrol in

pregnancy. T F

With regard to the anatomical abnormalities seen in

MRKH syndrome,

64. symmetrical uterovaginal aplasia is found in

type I disorders. T F

65. renal abnormalities are seen in more than

half of cases. T F

66. skeletal abnormalities are reported in up to

one-fifth of cases. T F

67. up to one-quarter of women have a

malformed ear or auditory canal. T F

68. the close proximity of the m

¨

ullerian and

wolffian duct derivatives to the metanephric

duct in the developing embryo explains the

higher association of malformations of the

kidneys with this condition. T F

69. vaginal agenesis is caused by failure of the

caudal part of the m

¨

ullerian duct system to

develop. T F

Regarding the diagnosis of MRKH syndrome,

70. magnetic resonance imaging is the gold

standard tool. T F

71. two-dimensional ultrasound scanning is not

useful for associated renal tract

abnormalities. T F

72. complete androgen insensitivity syndrome is

an important differential diagnosis. T F

73. the presence of cyclical abdominal pain will

rule out the diagnosis, as it indicates the

presence of functioning endometrium. T F

With regard to the creation of a neovagina,

74. it is recommended that treatment is initiated

as soon as the diagnosis is made. T F

75. psychological support to women undergoing

this procedure is of the utmost importance. T F

76. vaginal dilators are acceptable as an option

for first-line therapy. T F

77. Ingram’s modified Frank’s technique involves

the use of vaginal dilators. T F

With regard to the surgical creation of a neovagina,

78. in the Davydov procedure the neovagina is

lined with peritoneum. T F

With regard to fertility in women with the MRKH

syndrome,

79. transvaginal egg retrieval is recognised to be

difficult during in vitro fertilisation. T F

80. the condition has been shown to be

transmissible to the offspring. T F

Abbreviations.

AIS:          androgen insensitivity syndrome

AMH:      anti- Müllerian hormone

MRKH:     Mayer-Rokitansky-Küster-Hauser syndrome

MURCS:  Müllerian duct aplasia, renal dysplasia and cervical somite anomaly syndrome.

 

Question 1.        What are the main features of MRKH? There is no option list to make life harder.

Question 2.        Which, if any, are the main secondary features associated with MRKH?

Option list.

A	anosmia
B	attention-deficit-hyperactivity syndrome
C	auditory anomalies
D	neural tube defects
E	renal anomalies
F	skeletal anomalies

Question 3.        How does MRKH syndrome usually present?

Option list.

A	cyclical pain due to haematometra
B	delayed puberty
C	precocious puberty
D	premature menopause
E	primary amenorrhoea
F	recurrent otitis media
G	recurrent urinary tract infection
H	secondary amenorrhoea

Question 4.        Which of the following chromosome patterns are typical of MRKH?

Option list.

A	45XO
B	45YO
C	46XX
D	46XY
E	47XXX
F	47XXY

Question 5.        What is the approximate incidence of MRKH in newborn girls?

Option list.

A	~ 1 in 1,000
B	~ 1 in 2,000
C	~ 1 in 4,000
D	~ 1 in 6.000
E	~ 1 in 8,000
F	~ 1 in 10,000
G	~ 1 in 100,000
H	the figure is unknown
I	it does not occur

Question 6.        What is the approximate incidence of MRKH in newborn boys?

Option list.

A	~ 1 in 1,000
B	~ 1 in 2,000
C	~ 1 in 4,000
D	~ 1 in 6.000
E	~ 1 in 8,000
F	~ 1 in 10,000
G	~ 1 in 100,000
H	the figure is unknown
I	it does not occur

Question 7.             Which of the following statements are correct in relation to urinary tract anomalies associated with MRKH?

Option list.

A	absent bladder
B	absent kidney
C	ectopic ureter
D	horseface kidney
E	hypospadias
F	urinary tract anomalies are not part of the syndrome

Question 8.             Which of the following statements are correct in relation to skeletal anomalies associated with MRKH?

Option list.

A	absent thumb
B	absent big toe
C	developmental dysplasia of the hip
D	Klippel-Feil anomaly
E	ulnar hypoplasia
F	vertebral fusion
G	skeletal anomalies are not part of the syndrome

Question 9.             Which of the following statements are correct in relation to auditory anomalies associated with MRKH?

Option list.

A	absent ear
B	absent stapes
C	acoustic neuroma
D	conductive deafness
E	inductive deafness
F	stapedial ankylosis
G	auditory anomalies are not part of the syndrome

Question 10.    What is the recommended first-line management for creation of a neovagina.

Option list.

A	digital dilatation
B	marriage to a virile husband
C	vaginal balloons
D	vaginal dilators
E	vaginoplasty
F	there is no recommended 1st. line management

Question 11.     What are the key features of Davydov vaginoplasty?

Option list.

A	horseshoe perineal incision with labial flaps used to create a pouch
B	creation of space between bladder and rectum and lining it with amnion
C	creation of space between bladder and rectum and lining it with skin graft
D	creation of space between bladder and rectum and lining it with sigmoid colon
E	creation of space between bladder and rectum and lining it with peritoneum
F	traction via threads running to the abdomen from a vaginal bead

Question 12.    What are the key features of McIndoe vaginoplasty?

Option list.

A	horseshoe perineal incision with labial flaps used to create a pouch
B	creation of space between bladder and rectum and lining it with amnion
C	creation of space between bladder and rectum and lining it with skin graft
D	creation of space between bladder and rectum and lining it with sigmoid colon
E	creation of space between bladder and rectum and lining it with peritoneum
F	traction via threads running to the abdomen from a vaginal bead

Question 13.    What are the key features of Vecchietti vaginoplasty?

Option list.

A	horseshoe perineal incision with labial flaps used to create a pouch
B	creation of space between bladder and rectum and lining it with amnion
C	creation of space between bladder and rectum and lining it with skin graft
D	creation of space between bladder and rectum and lining it with sigmoid colon
E	creation of space between bladder and rectum and lining it with peritoneum
F	traction via threads running to the abdomen from a vaginal bead

Question 14.    What are the key features of Williams vaginoplasty?

Option list.

A	horseshoe perineal incision with labial flaps used to create a pouch
B	creation of space between bladder and rectum and lining it with amnion
C	creation of space between bladder and rectum and lining it with skin graft
D	creation of space between bladder and rectum and lining it with sigmoid colon
E	creation of space between bladder and rectum and lining it with peritoneum
F	traction via threads running to the abdomen from a vaginal bead

TOG CPD questions.

With regard to the MRKH syndrome.

1.     there is failure of development of the mesonephric ducts.                                     True / False

2.     the phenotype and genotype are female.                                                                   True / False

3.     studies have established a link between the syndrome and the use of diethylstilboestrol in pregnancy.                                                                                                                              True / False

With regard to the anatomical abnormalities seen in MRKH syndrome.

4.     symmetrical uterovaginal aplasia is found in type I disorders.                                True / False

5.     renal abnormalities are seen in more than half of cases.                                         True / False

6.     skeletal abnormalities are reported in up to one-fifth of cases.                              True / False

7.     up to one-quarter of women have a malformed ear or auditory canal.                    True / False

8.     the close proximity of the Müllerian and Wolffian duct derivatives to the duct in the developing embryo explains the higher association of malformations of the kidneys with this condition.

True / False

9.     vaginal agenesis is caused by failure of the caudal part of the Müllerian duct system to develop.

True / False

Regarding the diagnosis of MRKH syndrome,

10.   magnetic resonance imaging is the gold standard tool.                                            True / False

11.   two-dimensional ultrasound scanning is not useful for associated renal tract abnormalities.

True / False

12.   complete androgen insensitivity syndrome is an important differential diagnosis.  True / False

13.   the presence of cyclical abdominal pain will rule out the diagnosis, as it indicates the presence of functioning endometrium.                                                                                          True / False

With regard to the creation of a neovagina,

14.   it is recommended that treatment is initiated as soon as the diagnosis is made.     True / False

15.   psychological support to women undergoing this procedure is of the utmost importance.

True / False

16.   vaginal dilators are acceptable as an option for first-line therapy.                         True / False

17.   Ingram’s modified Frank’s technique involves the use of vaginal dilators.                    True / False

With regard to the surgical creation of a neovagina,

18.   in the Davydov procedure the neovagina is lined with peritoneum.                      True / False

With regard to fertility in women with the MRKH syndrome,

19.   transvaginal egg retrieval is recognised to be difficult during in vitro fertilisation.             True / False

20.   the condition has been shown to be transmissible to the offspring.                      True / False