Tutorial 21 July 2022

 

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21	Roleplay. PMB.
22	EMQ. Mayer-Rokitansky-Küster-Hauser syndrome
23	EMQ. Noonan syndrome
24	EMQ. Stilboestrol
25	EMQ. ‘CAESAR’ trial.
26	EMQ. Ulipristal

 

21.   Roleplay. PMB.

Candidate’s Instructions.

You are an SpR in the “one-stop” PMB clinic. Mary Smith, 55 years old, has been referred by her General Practitioner. She has had some bleeding since the menopause.

Your task is to take an appropriate history and advise her about the investigations you feel are appropriate and why.

 

22.   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

 

23.   Noonan syndrome.

Abbreviations.

NS:  Noonan syndrome.

TS:   Turner syndrome.

Question 1.       Why ‘Noonan’?

Option list.

A	the first case was diagnosed in the Noonan family in Wichita, Kansa in 1953.
B	the first case was described by Jacqueline A. Noonan.
C	it is named after Dr Theodore X. Dalry who had the condition. He was a preacher on USA TV in the 1950s and particularly railed against onanism, acquiring the soubriquet ‘Dr. Noonan’.
D	none of the above.

Question 2.       Which, if any, of the following have been used as names for the condition?

Option list.

A	familial Turner syndrome
B	female pseudo-Turner syndrome
C	male Turner syndrome
D	Noonan-Ehmke syndrome
E	Noonan's syndrome
F	NS
G	pseudo-Ullrich-Turner syndrome
H	Turner phenotype with normal karyotype
I	Turner syndrome in female with X chromosome
J	Turner-like syndrome
K	Ullrich-Noonan syndrome
L	all of the above
M	none of A-K

Question 3.       What is the approximate incidence of NS in newborns?

Option list.

A	1 in 2,000
B	1 in 5,000
C	1 in 10,000
D	1 in 50,000
E	1 in 100,000

Question 4.       Which, if any, of the following is true of NS?

Option list.

A	it is an autosomal dominant condition
B	it is an autosomal recessive condition
C	it is an X-linked dominant condition
D	it is an X-linked recessive condition
E	it is due to loss of part of an X chromosome
F	it is due to loss of part of chromosome 5
G	none of the above

Question 5.       Which if any of the following are features of NS?

Option list.

A	bicuspid aortic valve
B	bleeding disorders
C	coarctation oi the aorta
D	cryptorchidism
E	furrowed philtrum
F	hypertelorism, epicanthic folds, ptosis
G	hypertrophic  cardiomyopathy
H	leukaemia
I	low occipital hairline
J	low-set, retro-rotated ears
K	micrognathia
L	obesity
M	pectus excavatum or carinatum (Latin: ‘keel-shaped’)
N	pulmonary stenosis
O	scoliosis
P	‘shield’ chest
Q	short stature
R	significant intellectual impairment
S	streak gonads
T	‘striking’ blue or blue/green eyes
U	tall stature
V	thin philtrum
W	thrombophilia
X	webbed neck

Question 6.       Which, if any, of the following are common in NS and TS?

Option list.

A	bicuspid aortic valve
B	bleeding disorders
C	coarctation of the aorta
D	coeliac disease
E	cryptorchidism,
F	cubitus valgus
G	diabetes
H	epicanthic folds
I	gonadal dysgenesis
J	hypertelorism
K	hypothyroidism
L	hypertrophic cardiomyopathy
M	increased risk of leukaemia
N	low occipital hairline
O	pectus excavatum or carinatum (Latin: ‘keel-shaped’)
P	pulmonary stenosis
Q	red-green colour blindness
R	short stature
S	short, webbed neck

 

24.   Stilboestrol.

Stilboestrol. EMQ. Questions.

DOS:        ‘daughter(s) of stilboestrol’. Daughters of WSIP.

FDA:        US Food and Drug Administration.

GDOS:     ‘granddaughter(s)  of stilboestrol’’. Granddaughters of WSIP.

GSOS:      ‘grandson(s)  of stilboestrol’’. Grandsons of WSIP.

SOS:        ‘son(s)  of stilboestrol’. Sons of WSIP.

WSIP:     women who took stilboestrol in pregnancy.

Question 1.            When was stilboestrol first described?

Option list.

A	1938
B	1940
C	1950
D	1961
E	1970
F	1971
G	1973
H	1984
I	2005
J	2019

Question 2.       When did Herbst describe the risk of cancer for DOS?

Option list.     Use the list for question 1.

Question 3.       Which cancer did he refer to?

Option list.     Use the list for question 7.

Question 4.       When did the FDA and CSM issue warnings about the use of DES in pregnancy?

Option list.     Use the list for question 1.

Question 5.       The Kefauver-Harris Amendments to the 1938 Food, Drug, and Cosmetic Act were a response to the thalidomide tragedy / scandal in the USA. When were they enacted?

Option list.     Use the list for question 1.

Question 6.       When was the  US National Cancer Institute’s “DES Third Generation Study” published?

Option list.     Use the list for question 1.

Question 7.       Which, if any, of the following are more common in women exposed to DES in

 pregnancy?

Option list.

A	amenorrhoea
B	menstrual irregularity
C	infertility
D	polycystic ovary syndrome
E	breast cancer
F	cervical cancer
G	ovarian cancer
H	miscarriage
I	ectopic pregnancy
J	pre-eclampsia
K	premature delivery
L	IUGR
M	neural tube defect
N	uterine malformation
O	cervical malformation
P	abnormal cervical cytology
Q	vaginal adenosis
R	vaginal adenocarcinoma
S	vaginal squamous carcinoma
T	vaginal melanoma
U	ADHD
V	depression

Question 8.       Which, if any, of the following are more common in DOS?

Option list.     Use the option list for Question 7.

Question 9.       Which, if any, of the following have been described as risks for SOS?

Option list.

A	ADHD
B	cryptorchidism
C	depression
D	hypospadias
E	infertility
F	prostate cancer
G	suicide

Question 10.    Which, if any, of the following have been described as risks for GDOS?

Option list.     Use the option list for Question 7.

Question 11.    Which, if any, of the following have been described as risks for GSOS?

Option list.     Use the list for question 9.

 

25.   ‘CAESAR’ trial.

Abbreviations.

ECV:           external cephalic version.

SSI:             surgical site infection.

Question 1.    What was the CAESAR trial? Which, if any, of the following statements are true?

Statements

A	a prospective, cohort study
B	a randomised, controlled trial
C	a comparison of selected techniques used during Caesarean section
D	a study of the risks of Caesarean section on maternal request without medical grounds
E	a study of the outcomes of C section performed after failed instrumental delivery

Question 2.    Where did the questions addressed by the trial come from?

Option list

A	the RCOG council
B	the RCOG exam committee
C	a survey of UK obstetricians asking what questions they would like to have answered
D	Dr. Johnstone, Consultant Obstetrician, Falkirk
E	National Childbirth Trust

Question 3.        The questionnaire also asked about the issues that the respondents would like to see addressed in a research programme. What issues were include in the CAESER trial?

Statements

A	outcome of C. section depending on aqueous versus alcohol-based skin preparation
B	cord traction versus manual removal of the placenta
C	digital versus ‘swab on a holder’ exploration of the uterine cavity to exclude RPOC
D	Joel-Cohen compared with Pfannenstiel incision
E	elective C. section at 38 versus 39 weeks
F	elective C. section with staff wearing masks versus not wearing masks
G	prophylactic antibiotics versus no prophylactic antibiotics
H	pre-op vaginal antiseptic “painting” versus none
I	blunt v. sharp opening of the lower segment
J	manual versus forceps delivery of the fetal head in cephalic presentations
K	single v double closure of the lower segment
L	closure v non-closure of parietal & pelvic peritoneum
M	liberal v restricted use of pelvic drains
N	glue v subcuticular suturing of the skin
O	none of the above

Question 4.    Which of the following statements is true of the definition of the 1ry. outcome?

Option list

A	use of antibiotics for maternal infectious morbidity during the hospital stay
B	use of antibiotics for maternal infectious morbidity during the 1st. six weeks
C	duration of postnatal hospital stay
D	abdominal and pelvic pain as measured on an analogue scale at 6 weeks
E	none of the above.

Question 5.        Which, if any, of the following describe the 2ry. outcomes? > 1 may be correct.

Statements:

A	additional treatments to the abdominal wound
B	haematoma formation
C	pain
D	breast feeding at discharge
E	breast feeding at 6 weeks
F	unexpected maternal morbidity
G	postnatal depression at 6 weeks
H	puerperal psychosis

Question 6.    Which if any of the following statements are true of the findings of the study?

Statements

A	there were no significant differences for any outcome
B	there was more endometritis after non-closure of the pelvic peritoneum
C	there was more 2ry. bleeding after interrupted-suture closure of the lower segment
D	there was more evidence of pelvic infection with liberal use of pelvic drains
E	none of the above.

Question 7.    When does the WHO recommend that prophylactic antibiotics be given at C section.

Option list

A	4 hours before the incision
B	2 hours before the incision
C	1 hour before the incision
D	with the incision
E	just before the incision

Question 8.        What did the paper by Sommerstein et al of December 2020 add to the debate on timing of administration of prophylactic antibiotics at C section?

Option list.

A	prophylactic antibiotics are most effective if given 1 hour before incision
B	prophylactic antibiotics are most effective if given ½ hour before incision
C	prophylactic antibiotics are most effective if given at the time of incision
D	prophylactic antibiotics are less effective if given after cord clamping
E	prophylactic antibiotics are equally effective if given after cord clamping
F	prophylactic antibiotics are most effective if given at incision and continued for 48 hours
G	none of the above

 

26.   Ulipristal.

Abbreviations.

EC:              emergency contraception

eMC:          electronic medicines compendium

CYP450:     cytochrome P450

UPA            ulipristal acetate

Question 1.           What type of drug is ulipristal?

Question 2.           How does ulipristal prevent conception as an emergency contraceptive?

Question 3.           How is ulipristal broken down / excreted?

Question 4.           What is the half-life of ulipristal?

Question 5.            Which drug (erythromycin, phenobarbitone, valium)  may prolong the half-life of ulipristal?

Question 6.            Which drugs may reduce the half-life of ulipristal? There is no option list. Just write down as many drugs as you can think of that induce the CYP450 enzmes.

Question 7.           What is the main use of ulipristal?

Question 8.           What is the dose of ulipristal?

Question 9.           What time-scale applies to the licensed use of ulipristal?

Question 10.        What contraceptive advice is given to those using ulipristal?

Question 11.        What advice is given to women who are breast-feeding?

Question 12.        Can treatment with ulipristal be repeated within 1 month?

Question 13.        Which medical conditions are contraindications to ullipristal use ? These are not on the option list.

Question 14.        What is the current situation re prescribing ulipristal? Write the key facts.