|
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 |
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.
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
