26 September 2022.
|
8 |
Roleplay. PMB. |
|
9 |
Write a ‘blurb’. General pre-pregnancy
counselling |
|
10 |
Role-play. Sterilisation request. |
|
11 |
EMQ. Mayer-Rokitansky-Küster-Hauser
syndrome |
8. 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.
9. Write
a ‘blurb’. General pre-pregnancy counselling.
Writing ‘blurbs’ is
an important activity for your study group. This is one of the most basic and
we can have a go at constructing a suitable version.
10. Role-play.
Sterilisation request.
Candidate’s instructions.
This is a roleplay station. Your tasks are to take a
history and discuss her request.
GP letter.
Castle Surgery,
Gambit Grove,
Chesstown. CHS1 U99.
Re Anne Jones.
25 Checkmate Street,
Chesstown. CHS7 Y86.
Dear Doctor,
Please see Mrs Jones who wishes to be sterilised. Our
family planning specialist is on leave and I know little about modern
contraception, so have not offered any advice.
Regards,
Dr. O.U.T. de Touche.
11. 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
