Tutorial 27th. February 2023

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12	Role-play. Obstetric history APH. Take & present the history.  R-P: Shaymaa.
13	Role-play. Cystic fibrosis. Brother has cystic fibrosis.                  R-P:
14	EMQ. Mayer-Rokitansky-Küster-Hauser syndrome
15	MCQ. The Coombs test

 

 12.         Role-play. Obstetric history APH. Take & present the history.  R-P: Shaymaa.

Candidate’s instructions.

You are the SpR on call and are about to see Mary Smith who has been admitted with APH. Your tasks are to take a history and present it with a management plan to your consultant on the ward round which is due to start. The role-player is the patient, the examiner is the consultant.

 

13.         Role-play. Cystic fibrosis. Brother has cystic fibrosis.                  R-P: .

Candidate's Instructions.

This is a roleplay station. You are a year 4 SpR and are in the gynaecology clinic.

The consultant has just left you in charge as she is feeling unwell and has gone to lie down.

Your task is to deal with the patient as you would in real life.

The College would give you definite instructions such as take a history and discuss the management. I have made it harder in that you have to decide the key tasks.

GP referral letter.

Best Medical Centre,

High Road,

Anytown.

Re. Mrs. Bonnie Black,

25 Low Road,

Anytown.

Phone: 07889 888 132.

Dear Doctor,

Please see Mrs Black who is planning her first pregnancy. Her main concern is that her brother has cystic fibrosis.

This was the first time I had met her although she has been registered with us for 5 years – her health is good and she has no history of serious illness or surgery.

I have explained that I don’t know much about the implications of the brother’s cystic fibrosis for her potential pregnancies and that she needs to talk to an expert. I have stressed that the risk of her having a child with cystic fibrosis is high and that she needs to be aware that there is a distinct likelihood that any pregnancy would be likely to be affected and need TOP.

Yours sincerely,

John P. Clatter.

 

14.         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.

Don’t waste time looking up the answers. Just answer with what you already know and guess when you don’t know, but use ‘intelligent guessing’. This is what you have to do in the exam, so practise now. The same applies to the next question.

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.

MRKH:     Mayer-Rokitansky-Küster-Hauser 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

 

 

 

15.         The Coomb’s Test.

This is a MCQ from the days before EMQs and SBAs, but it covers the facts and I have not bothered to convert it. The answers are ‘true’ or ‘false’.

a.  the direct test detects maternal IgM on fetal cells.

b.  is used in the investigation of thrombocytopenia.

c.   is positive in the baby with jaundice due to spherocytosis.

d.  who wrote: “A flea hath smaller fleas that on him prey…..”

e.  what was the rest of the verse?

f.   what connection has this verse with the Coomb’s test?

g.  the indirect Coomb’s test is used to detect antibodies in maternal serum.

h.  the direct test uses anti IgG serum.