Website
|
19
|
EMQ. Pregnancy after
uterine transplant
|
|
20
|
EMQ. Mayer-Rokitansky-Küster-Hauser
syndrome
|
|
21
|
EMQ. Kallmann’s syndrome
|
|
22
|
EMQ. Montgomery &
consent
|
|
23
|
EMQ. Hepatitis B
|
19. EMQ. Pregnancy after uterine transplant.
Abbreviations.
ET: embryo
transfer.
UT: uterine
transplant
Scenario
1.
When was the 1st.
human uterine transplant performed?
Option list.
|
A
|
2000
|
|
B
|
2015
|
|
C
|
2010
|
|
D
|
2011
|
|
E
|
2012
|
|
F
|
2013
|
|
G
|
2014
|
|
H
|
2015
|
|
I
|
2016
|
|
J
|
2017
|
Scenario
2.
When was the 1st.
livebirth after human uterine transplant?
Option list.
|
A
|
2000
|
|
B
|
2015
|
|
C
|
2010
|
|
D
|
2011
|
|
E
|
2012
|
|
F
|
2013
|
|
G
|
2014
|
|
H
|
2015
|
|
I
|
2016
|
|
J
|
2017
|
Scenario
3.
How many live births had
occurred worldwide after UT up to the end of 2017?
Option list
|
A
|
< 5
|
|
B
|
5 - 10
|
|
C
|
11 - 20
|
|
D
|
21 - 50
|
|
E
|
51 - 100
|
|
F
|
> 100
|
Scenario
4.
For which of the following
conditions is UT a possible treatment?
Option list.
|
A
|
Androgen Insensitivity syndrome. AIS.
|
|
B
|
Congenital Adrenal hyperplasia. CAH.
|
|
C
|
Kallmann’s syndrome. KS.
|
|
D
|
Mayer-Rokitansky-Küster-Hauser syndrome. MRKH.
|
|
E
|
McCune-Albright syndrome. MCAS.
|
|
F
|
Swyer’s syndrome. SS.
|
|
G
|
Turner’s syndrome. TS.
|
Scenario
5.
Which, if any, of the following
are commonly used for donor selection?
Option list.
|
A
|
absence of adenomyosis
|
|
B
|
absence of fibroids
|
|
C
|
age < 65 years
|
|
D
|
good general health
|
|
E
|
negative cervical smear and no high-risk HPV
|
|
F
|
no cancer in past 5 years
|
|
G
|
parous
|
|
H
|
vaginal length > 7 cm.
|
Scenario
6.
What is the rate of graft
survival at 1 year, failure being the need for hysterectomy?
Option list.
|
A
|
< 10%
|
|
B
|
11 – 20%
|
|
C
|
21 – 30%
|
|
D
|
31 – 40%
|
|
E
|
41 – 50%
|
|
F
|
51 – 60%
|
|
G
|
> 60%
|
|
H
|
the figure is unknown
|
Scenario
7.
Which of the following
statements is correct?
Option list.
|
A
|
donor surgery is more extensive than recipient surgery
|
|
B
|
donor surgery is less extensive than recipient surgery
|
|
C
|
donor surgery is as extensive as recipient surgery
|
Scenario
8.
What are the risks to the donor
in addition to the usual ones of bleeding,
infection, haematoma and thrombosis? There is no option list.
Scenario
9.
Which condition has been the
reason for recipients needing uterine transplant and which complication is more
likely in addition to the usual ones of bleeding, infection, haematoma and thrombosis? There is
no option list.
Scenario
10.
When is IVF and
cryopreservation of eggs done?
Option list.
|
A
|
before uterine transplantation
|
|
B
|
at the time of uterine transplantation
|
|
C
|
12 months after uterine transplantation to ensure graft
rejection does not occur
|
|
D
|
when the recipient chooses
|
|
E
|
none of the above
|
Scenario
11.
Which maintenance therapy was
used immediately before embryo transfer in the first case resulting in
livebirth?
Option list.
|
A
|
azathioprine + corticosteroids + tacrolimus
|
|
B
|
azathioprine + ciclosporin + corticosteroids + mycophenolate
mofetil
|
|
C
|
azathioprine + corticosteroids + mycophenolate mofetil
+ tacrolimus
|
|
D
|
azathioprine + corticosteroids + tacrolimus
|
|
E
|
ciclosporin + corticosteroids + mycophenolate mofetil +
tacrolimus
|
|
F
|
ciclosporin + mycophenolate mofetil + tacrolimus
|
|
G
|
corticosteroids + mycophenolate mofetil + tacrolimus
|
|
H
|
corticosteroids + tacrolimus
|
20. EMQ. Mayer-Rokitansky-Küster-Hauser syndrome.
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.
Lead-in.
What are the main features of
MRKH? There is no option list to make life harder.
Question
2.
Lead-in.
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.
Lead-in.
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.
Lead-in.
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.
Lead-in.
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.
Lead-in.
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.
Lead-in.
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.
Lead-in.
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-Fiel anomaly
|
|
E
|
ulnar hypoplasia
|
|
F
|
vertebral fusion
|
|
G
|
skeletal anomalies are not part of the syndrome
|
Question
9.
Lead-in.
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.
Lead-in.
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.
Lead-in.
What is 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.
Lead-in.
What is 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.
Lead-in.
What is 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.
Lead-in.
What is 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. T F
2. the phenotype and genotype are female. T F
3. studies have established a link between the syndrome
and the use of diethylstilboestrol in pregnancy. T F.
4. symmetrical uterovaginal aplasia is found in
type I disorders. T F
5. renal abnormalities are seen in more than half
of cases. T F
6. skeletal abnormalities are reported in up to
one-fifth of cases. T F
7. up to one-quarter of women have a malformed
ear or auditory canal. T F
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. T F
9. vaginal
agenesis is caused by failure of the caudal part of the Müllerian duct system
to develop. T F
10. magnetic resonance imaging is the gold standard
tool. T F
11. two-dimensional ultrasound scanning is not useful
for associated renal tract abnormalities. T F
12. complete androgen insensitivity syndrome is an
important differential diagnosis. T F
13. 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,
14. it is recommended that treatment is initiated as
soon as the diagnosis is made. F
15. psychological support to women undergoing this
procedure is of the utmost importance. T
16. vaginal dilators are acceptable as an option for
first-line therapy. T
17. Ingram’s modified Frank’s technique involves the
use of vaginal dilators. T
With regard to the surgical creation of a neovagina,
18. in the Davydov procedure the neovagina is lined
with peritoneum. T F
With regard to fertility in women with the MRKH syndrome,
19. transvaginal egg retrieval is recognised to be
difficult during in vitro fertilisation. T
20. the condition has been shown to be transmissible
to the offspring. T F
Mayer-Rokitansky-Küster-Hauser syndrome.
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
15.
Lead-in.
What are the main features of MRKH?
There is no option list to make life harder.
Question
16.
Lead-in.
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
17.
Lead-in.
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
18.
Lead-in.
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
19.
Lead-in.
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
20.
Lead-in.
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
21.
Lead-in.
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
22.
Lead-in.
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-Fiel anomaly
|
|
E
|
ulnar hypoplasia
|
|
F
|
vertebral fusion
|
|
G
|
skeletal anomalies are not part of the syndrome
|
Question
23.
Lead-in.
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
24.
Lead-in.
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
25.
Lead-in.
What is 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
26.
Lead-in.
What is 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
27.
Lead-in.
What is 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
28.
Lead-in.
What is 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. T F
2. the phenotype and genotype are female. T F
3. studies have established a link between the syndrome
and the use of diethylstilboestrol in pregnancy. T F.
4. symmetrical uterovaginal aplasia is found in
type I disorders. T F
5. renal abnormalities are seen in more than half
of cases. T F
6. skeletal abnormalities are reported in up to
one-fifth of cases. T F
7. up to one-quarter of women have a malformed
ear or auditory canal. T F
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. T F
9. vaginal
agenesis is caused by failure of the caudal part of the Müllerian duct system
to develop. T F
10. magnetic resonance imaging is the gold standard
tool. T F
11. two-dimensional ultrasound scanning is not useful
for associated renal tract abnormalities. T F
12. complete androgen insensitivity syndrome is an
important differential diagnosis. T F
13. 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,
14. it is recommended that treatment is initiated as
soon as the diagnosis is made. F
15. psychological support to women undergoing this
procedure is of the utmost importance. T
16. vaginal dilators are acceptable as an option for
first-line therapy. T
17. Ingram’s modified Frank’s technique involves the
use of vaginal dilators. T
With regard to the surgical creation of a neovagina,
18. in the Davydov procedure the neovagina is lined
with peritoneum. T F
With regard to fertility in women with the MRKH syndrome,
19. transvaginal egg retrieval is recognised to be
difficult during in vitro fertilisation. T
20. the condition has been shown to be transmissible
to the offspring. T F
Mayer-Rokitansky-Küster-Hauser syndrome.
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
29.
Lead-in.
What are the main features of
MRKH? There is no option list to make life harder.
Question
30.
Lead-in.
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
31.
Lead-in.
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
32.
Lead-in.
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
33.
Lead-in.
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
34.
Lead-in.
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
35.
Lead-in.
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
36.
Lead-in.
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-Fiel anomaly
|
|
E
|
ulnar hypoplasia
|
|
F
|
vertebral fusion
|
|
G
|
skeletal anomalies are not part of the syndrome
|
Question
37.
Lead-in.
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
38.
Lead-in.
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
39.
Lead-in.
What is 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
40.
Lead-in.
What is 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
41.
Lead-in.
What is 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
42.
Lead-in.
What is 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. T F
2. the phenotype and genotype are female. T F
3. studies have established a link between the syndrome
and the use of diethylstilboestrol in pregnancy. T F.
4. symmetrical uterovaginal aplasia is found in
type I disorders. T F
5. renal abnormalities are seen in more than half
of cases. T F
6. skeletal abnormalities are reported in up to
one-fifth of cases. T F
7. up to one-quarter of women have a malformed
ear or auditory canal. T F
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. T F
9. vaginal
agenesis is caused by failure of the caudal part of the Müllerian duct system
to develop. T F
10. magnetic resonance imaging is the gold standard
tool. T F
11. two-dimensional ultrasound scanning is not useful
for associated renal tract abnormalities. T F
12. complete androgen insensitivity syndrome is an
important differential diagnosis. T F
13. 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,
14. it is recommended that treatment is initiated as
soon as the diagnosis is made. F
15. psychological support to women undergoing this
procedure is of the utmost importance. T
16. vaginal dilators are acceptable as an option for
first-line therapy. T
17. Ingram’s modified Frank’s technique involves the
use of vaginal dilators. T
With regard to the surgical creation of a neovagina,
18. in the Davydov procedure the neovagina is lined
with peritoneum. T F
With regard to fertility in women with the MRKH syndrome,
19. transvaginal egg retrieval is recognised to be
difficult during in vitro fertilisation. T
20. the condition has been shown to be transmissible
to the offspring. T F
Mayer-Rokitansky-Küster-Hauser syndrome.
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
43.
Lead-in.
What are the main features of
MRKH? There is no option list to make life harder.
Question
44.
Lead-in.
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
45.
Lead-in.
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
46.
Lead-in.
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
47.
Lead-in.
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
48.
Lead-in.
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
49.
Lead-in.
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
50.
Lead-in.
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-Fiel anomaly
|
|
E
|
ulnar hypoplasia
|
|
F
|
vertebral fusion
|
|
G
|
skeletal anomalies are not part of the syndrome
|
Question
51.
Lead-in.
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
52.
Lead-in.
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
53.
Lead-in.
What is 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
54.
Lead-in.
What is 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
55.
Lead-in.
What is 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
56.
Lead-in.
What is 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. T F
2. the phenotype and genotype are female. T F
3. studies have established a link between the syndrome
and the use of diethylstilboestrol in pregnancy. T F.
4. symmetrical uterovaginal aplasia is found in
type I disorders. T F
5. renal abnormalities are seen in more than half
of cases. T F
6. skeletal abnormalities are reported in up to
one-fifth of cases. T F
7. up to one-quarter of women have a malformed
ear or auditory canal. T F
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. T F
9. vaginal
agenesis is caused by failure of the caudal part of the Müllerian duct system
to develop. T F
10. magnetic resonance imaging is the gold standard
tool. T F
11. two-dimensional ultrasound scanning is not useful
for associated renal tract abnormalities. T F
12. complete androgen insensitivity syndrome is an
important differential diagnosis. T F
13. 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,
14. it is recommended that treatment is initiated as
soon as the diagnosis is made. F
15. psychological support to women undergoing this
procedure is of the utmost importance. T
16. vaginal dilators are acceptable as an option for
first-line therapy. T
17. Ingram’s modified Frank’s technique involves the
use of vaginal dilators. T
With regard to the surgical creation of a neovagina,
18. in the Davydov procedure the neovagina is lined
with peritoneum. T F
With regard to fertility in women with the MRKH syndrome,
19. transvaginal egg retrieval is recognised to be
difficult during in vitro fertilisation. T
20. the condition has been shown to be transmissible
to the offspring. T F
Mayer-Rokitansky-Küster-Hauser syndrome.
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
57.
Lead-in.
What are the main features of
MRKH? There is no option list to make life harder.
Question
58.
Lead-in.
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
59.
Lead-in.
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
60.
Lead-in.
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
61.
Lead-in.
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
62.
Lead-in.
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
63.
Lead-in.
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
64.
Lead-in.
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-Fiel anomaly
|
|
E
|
ulnar hypoplasia
|
|
F
|
vertebral fusion
|
|
G
|
skeletal anomalies are not part of the syndrome
|
Question
65.
Lead-in.
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
66.
Lead-in.
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
67.
Lead-in.
What is 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
68.
Lead-in.
What is 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
69.
Lead-in.
What is 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
70.
Lead-in.
What is 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. T F
2. the phenotype and genotype are female. T F
3. studies have established a link between the syndrome
and the use of diethylstilboestrol in pregnancy. T F.
4. symmetrical uterovaginal aplasia is found in
type I disorders. T F
5. renal abnormalities are seen in more than half
of cases. T F
6. skeletal abnormalities are reported in up to
one-fifth of cases. T F
7. up to one-quarter of women have a malformed
ear or auditory canal. T F
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. T F
9. vaginal
agenesis is caused by failure of the caudal part of the Müllerian duct system
to develop. T F
10. magnetic resonance imaging is the gold standard
tool. T F
11. two-dimensional ultrasound scanning is not useful
for associated renal tract abnormalities. T F
12. complete androgen insensitivity syndrome is an
important differential diagnosis. T F
13. 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,
14. it is recommended that treatment is initiated as
soon as the diagnosis is made. F
15. psychological support to women undergoing this
procedure is of the utmost importance. T
16. vaginal dilators are acceptable as an option for
first-line therapy. T
17. Ingram’s modified Frank’s technique involves the
use of vaginal dilators. T
With regard to the surgical creation of a neovagina,
18. in the Davydov procedure the neovagina is lined
with peritoneum. T F
With regard to fertility in women with the MRKH syndrome,
19. transvaginal egg retrieval is recognised to be
difficult during in vitro fertilisation. T
20. the condition has been shown to be transmissible
to the offspring. T F
Mayer-Rokitansky-Küster-Hauser syndrome.
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
71.
Lead-in.
What are the main features of
MRKH? There is no option list to make life harder.
Question
72.
Lead-in.
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
73.
Lead-in.
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
74.
Lead-in.
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
75.
Lead-in.
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
76.
Lead-in.
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
77.
Lead-in.
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
78.
Lead-in.
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-Fiel anomaly
|
|
E
|
ulnar hypoplasia
|
|
F
|
vertebral fusion
|
|
G
|
skeletal anomalies are not part of the syndrome
|
Question
79.
Lead-in.
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
80.
Lead-in.
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
81.
Lead-in.
What is 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
82.
Lead-in.
What is 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
83.
Lead-in.
What is 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
84.
Lead-in.
What is 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. T F
2. the phenotype and genotype are female. T F
3. studies have established a link between the syndrome
and the use of diethylstilboestrol in pregnancy. T F.
4. symmetrical uterovaginal aplasia is found in
type I disorders. T F
5. renal abnormalities are seen in more than half
of cases. T F
6. skeletal abnormalities are reported in up to
one-fifth of cases. T F
7. up to one-quarter of women have a malformed
ear or auditory canal. T F
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. T F
9. vaginal
agenesis is caused by failure of the caudal part of the Müllerian duct system
to develop. T F
10. magnetic resonance imaging is the gold standard
tool. T F
11. two-dimensional ultrasound scanning is not useful
for associated renal tract abnormalities. T F
12. complete androgen insensitivity syndrome is an
important differential diagnosis. T F
13. 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,
14. it is recommended that treatment is initiated as
soon as the diagnosis is made. F
15. psychological support to women undergoing this
procedure is of the utmost importance. T
16. vaginal dilators are acceptable as an option for
first-line therapy. T
17. Ingram’s modified Frank’s technique involves the
use of vaginal dilators. T
With regard to the surgical creation of a neovagina,
18. in the Davydov procedure the neovagina is lined
with peritoneum. T F
With regard to fertility in women with the MRKH syndrome,
19. transvaginal egg retrieval is recognised to be
difficult during in vitro fertilisation. T
20. the condition has been shown to be transmissible
to the offspring. T F
21. EMQ. Kallmann’s
syndrome
Kallmann’s syndrome.
Abbreviations.
Ks: Kallmann’s syndrome
Scenario
1.
Which of the following might be
included in descriptions of Kallmann’s syndrome?
Option list.
|
A
|
hypogonadotrophic hypogonadism
|
|
B
|
hypogonadotrophic hypogonadism + anosmia
|
|
C
|
hypogonadotrophic hypogonadism + anosmia +
colour-blindness.
|
|
D
|
hypogonadotrophic hypogonadism due to uterine agenesis
|
Scenario
2.
Lead in.
Which, if any, of the following are features of the Kallmann
phenotype?
|
A
|
absent or minimal breast development
|
|
B
|
aortic stenosis
|
|
C
|
blue eyes
|
|
D
|
blue hair
|
|
E
|
hot flushes
|
|
F
|
short stature
|
|
G
|
tall stature
|
|
H
|
vaginal agenesis
|
|
I
|
none of the above
|
Scenario
3.
How common is Kallmann’s syndrome and what is the
female: male ratio?
|
A
|
1 in 1,000 and F:M ratio 1:1
|
|
B
|
1 in 5,000 and F:M ratio 1:1
|
|
C
|
1 in 10,000 and F:M ratio 1:4
|
|
D
|
1 in 50,000 and F:M ratio 1:4
|
|
E
|
1 in 100,000 and F:M ratio 1:8
|
|
F
|
1 in 250,000 and F:M ration 1:10
|
Scenario
4.
What is the most common mode of
inheritance of Ks?
Option list.
|
A
|
hypogonadotrophic hypogonadism
|
|
B
|
hypogonadotrophic hypogonadism + anosmia
|
|
C
|
hypogonadotrophic hypogonadism due to uterine agenesis
|
|
D
|
autosomal dominant
|
|
E
|
autosomal recessive
|
|
F
|
X-linked recessive
|
|
G
|
new mutation of the ANOS1 gene
|
|
H
|
the most common mode of inheritance is not known
|
Scenario
5.
How is Kallmann’s syndrome
diagnosed?
|
A
|
abdominal and pelvic
ultrasound scan
|
|
B
|
cell-free fetal DNA
|
|
C
|
chromosome analysis
|
|
D
|
CT scan of hypothalamus /
pituitary
|
|
E
|
MR scan of hypothalamus /
pituitary
|
|
F
|
none of the above.
|
Scenario
6.
How is Kallmann’s syndrome
treated initially?
Which of the following
statements are true?
Option list.
|
A
|
GnRH analogue depot
|
|
B
|
pulsatile GnRH therapy
|
|
C
|
combined oral contraceptive
|
|
D
|
counselling & education
re gender re-assignment
|
|
E
|
depot progestogen
|
|
F
|
none of the above
|
Scenario
7.
A woman was diagnosed with
Kallmann’s syndrome at 16 and had successful initial treatment. She is now 25,
married and wishes to have a pregnancy. She has had pre-pregnancy assessment
and counselling. Which of the following can be considered?
|
A
|
GnRH analogue depot
|
|
B
|
induction of ovulation with
clomiphene
|
|
C
|
gonadotrophin therapy
|
|
D
|
pulsatile GnRH therapy
|
|
E
|
none of the above
|
22. EMQ.
Montgomery Ruling.
Abbreviations.
BMA: British Medical Association.
GMC: General Medical Council.
Question
1.
Which, if
any, of the following statements are true?
Lead-in
|
A.
|
The
Montgomery ruling largely replaces the Bolam ruling
|
|
B.
|
The Montgomery ruling largely replaces the Chester
ruling
|
|
C.
|
The Montgomery ruling largely replaces the Sidaway
ruling
|
|
D.
|
The Montgomery ruling is being contested in the
European Court by the GMC as it infringes the rights of doctors
|
|
E.
|
The Montgomery ruling is being contested in the
European Court by the BMA as it infringes the rights of doctors
|
Question
2.
Which, if
any, of the following statements are true?
Lead-in
|
A.
|
the
level of risk, however small, must be disclosed if a patient requests it
|
|
B.
|
the level of risk of damage from a procedure need not
be disclosed if < 1%
|
|
C.
|
the level of risk of damage from a procedure need not
be disclosed if < 10%
|
|
D.
|
a material risk is one that would be reflected in
damages > £100,000 if negligence were proved in court
|
|
E.
|
a material risk is one that would be reflected in
damages > £1,000,000 if negligence were proved in court
|
|
F.
|
a material risk is one that involves anatomical damage,
not emotional or psychological
|
|
G.
|
a material risk is one that a reasonable person in the
patient’s situation would be likely to regards as significant
|
23. EMQ. Hepatitis B.
Abbreviations.
HAV: hepatitis
A virus
HBcAg: hepatitis
B core antigen
HBeAg: hepatitis
B e antigen
HBsAg: hepatitis
B surface antigen
HBcAb: antibody
to hepatitis B core antigen
HBeAb: antibody
to hepatitis B e antigen
HBsAb: antibody
to hepatitis B surface antigen
HBIG: hepatitis
B immunoglobulin
HBV: hepatitis
B virus
HBcAg: hepatitis
B core antigen
HBeAg: hepatitis
B e antigen
HBsAg: hepatitis
B surface antigen
HBcAb: antibody
to hepatitis B core antigen
HBeAb: antibody
to hepatitis B e antigen
HBsAb: antibody
to hepatitis B surface antigen
HBIG: hepatitis
B immunoglobulin
HCV: hepatitis
C virus
HEV: hepatitis
E virus
HSV: herpes
simplex virus
VT: vertical
transmission
Option list.
|
A.
|
acyclovir
|
|
B.
|
divorce
|
|
C.
|
HBcAg
+ve
|
|
D.
|
HBeAg
+ve
|
|
E.
|
HbsAg
+ve
|
|
F.
|
HBsAg
+ve; HBsAb –ve; HBcAb –ve; HBeAg +ve
|
|
G.
|
HBsAg
+ve; HBsAb –ve on two tests six months apart
|
|
H.
|
HBsAg
-ve; HBsAb -ve on two tests six months apart
|
|
I.
|
HBsAg
-ve; HBsAb +ve; HBcAb –ve
|
|
J.
|
HBsAg
-ve; HBsAb +ve; HBcAb +ve
|
|
K.
|
HBsAg
-ve; HBsAb +ve
|
|
L.
|
HBsAg
+ve; HBcAg +ve
|
|
M.
|
HBV
vaccine
|
|
N.
|
HBIG
|
|
O.
|
HBV
vaccine + HBIG
|
|
P.
|
immune
as a result of infection
|
|
Q.
|
immune
as a result of vaccination
|
|
R.
|
not
immune
|
|
S.
|
chronic
carrier of HBV infection
|
|
T.
|
10%
|
|
U.
|
30%
|
|
V.
|
50%
|
|
W.
|
60%
|
|
X.
|
70-90%
|
|
Y.
|
soap
and boiling water
|
|
Z.
|
10%
dilution of bleach in water
|
|
AA.
|
10%
dilution of formaldehyde in alcohol
|
|
BB.
|
ultraviolet
irradiation
|
|
CC.
|
yes
|
|
DD.
|
no
|
|
EE.
|
HAV
|
|
FF.
|
HBV
|
|
GG.
|
HCV
|
|
HH.
|
HEV
|
|
II.
|
HSV
|
|
JJ.
|
none
of the above
|
Scenario 1.
An
asymptomatic primigravida books at 10 weeks. Her partner had an acute HBV
infection 4 months ago. What results on routine blood testing would indicate
that she has an acute HBV infection?
Scenario 2.
An
asymptomatic primigravida books at 10 weeks. Her partner had an acute HBV
infection 4 months ago. What results on routine blood testing would indicate
that she is immune to the HBV as a result of infection?
Scenario 3.
An
asymptomatic primigravida books at 10 weeks. Her partner had an acute HBV infection
4 months ago. What results on routine blood testing would indicate that she is
immune to the HBV as a result of HBV vaccine?
Scenario 4.
An
asymptomatic primigravida books at 10 weeks. Her partner had an acute HBV
infection 9 months ago. What results on routine blood testing would show that
she is a chronic carrier of HBV infection?
Scenario 5.
Testing shows
that he is positive for HBsAg, positive for HBcAb but negative for IgM HBcAb.
What does this mean in relation to his HBV status?
Scenario 6.
Testing shows
that he is negative for HBsAg, positive for HBcAb and positive for HBsAb.
What does this
mean in relation to his HBV status?
Scenario 7.
How
common is chronic HBV carrier status in UK pregnant women?
Scenario 8.
What
is the risk of death from chronic HBV carrier status?
Scenario 9.
A
primigravid woman at 8 weeks gestation is found to be non-immune to HBV. She
has recently married and her husband is a chronic carrier. What should be done
to protect her from infection?
Scenario 10.
A
woman is a known carrier of HBV. What is the risk of vertical transmission in
the first trimester?
Scenario 11.
What
is the risk of the neonate who has been infected by vertical transmission
becoming a carrier without treatment?
Scenario 12.
Should
antiviral maternal therapy in the 3rd. trimester be considered for
women with HBeAg or high viral load?
Scenario 13.
How
effective is hepatitis B prophylaxis for the neonate in preventing chronic
carrier status as a result of vertical transmission?
Scenario 14.
Can
a woman who is a chronic HBV carrier breastfeed safely?
Scenario 15.
Hepatitis B
infection is the most dangerous of the viral hepatitis infections in pregnancy.
Scenario 16.
A
pregnant woman who is not immune to HBV has a partner who is a chronic carrier.
Can HBV vaccine be administered safely in pregnancy?
Scenario 17.
A
pregnant woman who is not immune has a partner with acute hepatitis due to HBV.
He cuts his hand and bleeds onto the kitchen table. How should she clean the
surface to ensure that she gets rid of the virus?
Scenario 18.
Is it true
that the presence of HBeAg in maternal blood is a particular risk factor for
vertical transmission? Not really a scenario, but never mind!
Scenario 19.
Does
elective Cs before labour and with the membranes intact reduce the vertical
transmission rate?
Scenario 20.
Which
hepatitis virus normally produces a mild illness, but represents a major risk
to pregnant women, with a mortality rate of up to 5%?
Scenario 21.
A
pregnant woman has a history of viral hepatitis and informs the midwife at
booking that she is a carrier and that she has a significant risk of cirrhosis
and has been advised not to drink alcohol. Which is the most likely hepatitis
virus?
Scenario 22.
Which
hepatitis virus is an absolute contraindication to breastfeeding after
appropriate treatment of the infected mother and prophylaxis for the baby?
Scenario 23.
Which
hepatitis virus is linked to an increased risk of obstetric cholestasis?
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