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352 Appendix I
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4 A female infant has an antenatally detected
unilateral MCDK. Her contralateral kidney is normal and she is otherwise healthy.
What is her approximate risk of developing a
Wilms tumour in the MCDK?
a) Less than 0.1%
b) 0.5% to 1%
c) 1% to 1.5%
d) 1.5% to 2%
e) Greater than 2%
5 A postnatal ultrasound in a newborn infant
reveals ndings consistent with a diagnosis
of unilateral multicystic dysplastic kidney.
Which of the following would be the most
appropriate next investigation?
a) 99mTC DMSA scan
b) IVU
c) Measurement of glomerular ltration rate
(GFR)
d) CT scan
e) MRI
2 Which of the following statements best
describes the role of percutaneous nephro
lithotomy (PCNL) in children?
a) PCNL is unsuitable for the treatment of
staghorn calculi
b) PCNL can be performed under sedation in
older children
c) Stones are most commonly disintegrated
with an ultrasound probe
d) Stones are most commonly extracted
intact from the kidney using a modied
stone basket
e) None of the above
3 A 10-year-old girl is referred with a provi-
sional diagnosis of xanthogranulomatous
pyelonephritis? Which of the following fea-
tures of her presentation and investigation is
not consistent with this diagnosis?
a) Dierential function 20% on DMSA scan
b) Elevated inammatory markers
c) Haemoglobin 8.5 g/dl
d) Palpable mass
e) Presence of calculi on CT scan
CHAPTER 11: URINARY TRACT
CALCULI
1 A 9-year-old boy presents with a 1.1 cm
stone in the distal le ureter. ere is moderate dilatation of the ureter and collecting
systems on ultrasound. He is pain free but
the appearances on further imaging aer
2weeks are unchanged. Which of the following is now the optimal management of this
young patient?
a) External shockwave lithotripsy (ESWL)
b) Conservative – re-assess with further
imaging aer 12 months
c) Open ureterolithotomy
d) Basket extraction
e) Ureteroscopy and laser lithotripsy
4 Which of the following is the most common
cause of metabolic stones in children?
a) Hyperoxaluria
b) Cystinosis
c) Hypercalciuria
d) Uric acidaemia
e) Xanthine oxidase deciency
5 A 2-year-old boy presents with haematuria.
An ultrasound scan on admission demon-
strates a ‘staghorn’ calculus in the le kid-
ney. Urine culture is positive. Which of the
following is most likely to be the infecting
organism in this case?
a) E. coli
b) Bacteroides
c) Enterococcus
d) Staph aureus
e) Proteus mirabilis
Self-Assessment Section: Questions 353
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CHAPTER 12: URINARY
INCONTINENCE
1 During the process of normal voiding,
detrusor contraction is initiated in response
to stimulation via which of the following
pathways?
a) Voluntary somatic S2-4 pathways
b) Parasympathetic S 2 to 4 pathways
c) T 10 to 12 lumbar sympathetic pathways
d) T 10 to 12 lumbar parasympathetic
pathways
e) None of the above
2 A 6-year-old girl is referred with a history
of diurnal and nocturnal incontinence. She
came out of nappies at 2½ years of age and
was dry during the daytime (but not at night),
until around 3 years of age. Since then, she has
been wetting on an almost daily basis. Which
of the following is the most likely diagnosis
based upon this history?
a) Occult spinal dysraphism
b) Dysfunctional voiding
c) Sacral agenesis
d) Pelvic oor weakness (stress incontinence)
e) Ectopic ureter
4 A 12-year-old girl is referred with a history
of incontinence which only occurs when she
is laughing and joking with friends. She is
dry at other times. Which of the following
forms of treatment would have the highest
chance of providing symptomatic relief in
this patient?
a) Pelvic oor exercises
b) Treatment with Methylphenidate
c) Referral to a child psychologist
d) Treatment with Desmopressin
e) Avoiding so drinks which contain caeine
5 Which of the following anatomical causes of
urinary incontinence in girls is least likely
to be detected on visual examination of the
perineum?
a) Congenital cloacal anomaly
b) Urogenital sinus anomaly
c) Primary epispadias
d) Ectopic ureter
e) Labial adhesions
CHAPTER 13: NEUROGENIC
BLADDER
3 A 5-year-old boy is referred with a lifelong
history of nocturnal enuresis. He wets the
bed virtually every night but has been consistently dry during the daytime since the age of
three. Physical examination is normal. What
should be done next?
a) Commence treatment with oxybutynin
b) Check urine osmolality on early morning
urine sample
c) Videourodynamics
d) MRI scan of the spine
e) None of the above
1 Which of the following statements are most
applicable to the neurology and pathophysi-
ology of neurogenic bladder dysfunction?
a) Neurologic lesions in the lumbosacral
region account for 80% of cases
b) e neurologic picture (upper or lower
motor) in children with myelomeningocele is closely correlated with the level of
the spinal abnormality
c) e urodynamic nding of detrusor/
sphincter dyssynergia denotes a ‘safe’ type
of neurogenic bladder
d) Sphincter weak ness incontinence is usually
a feature of a lower motor neuron lesion
e) Sacral agenesis is accompanied by the
presence of a cutaneous lesion in 80% of
cases
354 Appendix I
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2 Which of the following structures is usually
used to create the Monti continent catheterisable channel?
a) Ileum
b) Caecum
c) Colon
d) Appendix
e) Rectus sheath
3 Using the formula provided in the chapter,
what is the calculated functional bladder
capacity of a normal 6-year-old child?
a) 110 to 130 ml
b) 150 to 170 ml
c) 230 to 250 ml
d) 280 to 300 ml
e) 350 to 370 ml
4 Which of the following is not a recognised
form of treatment for detrusor over activity?
a) Ileocystoplasty
b) Botulinum toxin A
c) Sigmoid colocystoplasty
d) Medication with Alpha adrenergic block-
ing agents
e) Medication with anticholinergic agents
CHAPTER 14: UROLOGIC
ANOMALIES IN ANORECTAL
MALFORMATIONS AND RENAL
ECTOPIA
1 What percentage of children with anorectal
malformations have anomalies of the uri-
nary tract?
a) 25% to 35%
b) 35% to 45%
c) 45% to 55%
d) 55% to 65%
e) 65% to75%
2 A newborn male infant has a low anorectal
anomaly. What imaging modality is cur-
rently recommended for initial imaging of
his spine?
a) MRI
b) CT
c) Spinal x-ray and ultrasound
d) Bone scan
e) Contrast myelography
3 Which of the following is not a recognised
feature of the VACTERL association?
5 What is the most common cause of neuro-
genic bladder in children?
a) Spinal injury
b) Myelomeningocele
c) Transverse myelitis
d) Sacral agenesis
e) Lipomyelomeningocele
a) Limb anomalies
b) Vertebral anomalies
c) Anorectal anomalies
d) Optic anomalies (coloboma)
e) Renal anomalies
4 A healthy newborn female infant is born
with a presumed diagnosis of unilateral
renal agenesis – based on antenatal ultra-
sound. Only one (normal) kidney is seen on
the postnatal ultrasound scan. What investi-
gation (if any) is indicated next?
a) None. e diagnosis of renal agenesis had
been conrmed by ultrasound.
b) CT
c) Laparoscopy d
d) Mag 3 renogram
e) Isotope renography (DMSA)
Self-Assessment Section: Questions 355
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5 Which of the following statements does not
apply to horseshoe kidneys
a) Horseshoe kidneys are well visualised on
DMSA renography
b) Horseshoe kidneys occur more commonly
in children with Turners syndrome
c) e autopsy incidence of horseshoe kidney
is reported to lie between 1:400 and 1:1800
d) 35% to 45% of horseshoe kidneys are asso-
ciated with fusion of the upper pole renal
parenchyma
e) Horseshoe kidney is associated with an
increased incidence of PUJ obstruction
CHAPTER 15: BLADDER
EXSTROPHY AND EPISPADIAS
1 What is the approximate incidence of blad-
der exstrophy?
a) 1 in 5000 live births
b) 1 in 25,000 live births
c) 1 in 50,000 live births
d) 1 in 150,000 live births
e) 1 in 300,000 live births
2 Which of the following is not a common fea-
ture of primary epispadias?
a) Sphincteric weakness
b) Pubic diastasis
c) Normal calibre ureteric orices
d) Urethra sited on the dorsal aspect of the
penis
e) Anterior ectopic anus
3 A 3.5 kg male infant with classic bladder
exstrophy is transferred to a specialist centre
at 6 hours of age. He is otherwise well with
no evidence of cardiac or other anomalies.
What is now regarded as the optimal man-
agement of this infant?
a) Primary closure with pelvic osteotomy in
the rst 48 hours of life
b) Primary closure without pelvic osteotomy
in the rst 2–3 weeks of life
c) Parentral nutrition for one month fol-
lowed by closure with pelvic osteotomies
d) Temporary urinary diversion with ure-
terostomies following by primary closure
and undiversion at 6 months
e) Delayed closure with augmentation cysto-
plasty at 12 months
4 Which of the following statements best
describe the surgical closure of bladder
exstrophy?
a) e complete primary repair described by
Mitchell is the simplest procedure and the
one best suited to non-specialists
b) e Kelly operation is performed without
osteotomy in the rst 2–3 weeks of life
c) e Kelly operation is suitable for use in
girls as well as boys
d) e Modern Staged Reconstruction was
devised by Ransley and Gearhart
e) Use of the international standardised con-
tinence grading system (ISCGS) ensures
consistent reporting of continence rates
5 Which of the following statements best
describes the features of primary epispadias
in females?
a) Normal continence occurs spontaneously
in 85% of cases by 7 years of age
b) Sphincter weakness incontinence is
invariably present
c) e external genitalia are usually normal
d) e clitoris is usually absent
e) e overall chances of achieving preg-
nancy are less that 10%
356 Appendix I
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CHAPTER 16: HYPOSPADIAS
1 Which of the following statements is most
applicable to chordee?
a) It is present in 75% of cases of distal
hypospadias
b) Successful correction in childhood may
not prevent recurrence aer puberty
c) A 2 stage repair is always required
d) Chordee can usually be successfully cor-
rected with a ventral tunica plication
e) e tubularized incised plate (Snodgrass)
technique has the highest success rate for
the correction of moderately severe chordee
2 What is the overall incidence of co-existing
upper urinary tract anomalies in boys with
distal hypospadias?
a) 1% to 5%
b) 5% to 10%
c) 10% to 15%
d) 15% to 20%
e) 20% to 25%
3 Which of the following gra materials is
most widely used in the rst stage of a two
stage repair of proximal hypospadias?
a) Post auricular skin
b) Buccal skin
c) Bladder mucosa
d) Porcine dermis
e) Inner preputial skin
4 Which if the following statements best
describes the aetiology and epidemiology of
hypospadias?
a) Environmental factors (endocrine disrup-
tors) have been demonstrated to account
for >20% of cases
b) e incidence of hypospadias in rst
degree relatives is approximately 10%
c) Genetic factors are more likely be impli-
cated in distal hypospadias
d) Co existing abnormalities of the upper uri-
nary tract are present in 15–20% of cases
e) e incidence is lower in the ospring of
older mothers
5 Which of the following statements is most
applicable to the urethrocutaneous hypospa-
dias stulae?
a) e overall incidence for all types of hypo-
spadias repair is 5–7%
b) e optimal time for stula repair is
within 6 weeks of the original surgery
c) Success rates for ap based techniques are
no higher than simple closure
d) e reported recurrence rate in some pub-
lished series is 50%
e) Suprapubic catheter drainage is manda-
tory to reduce the risk of recurrence
CHAPTER 17: THE PREPUCE
1 Which of the following statements best
describes the aetiology and management
of balanitis xerotica obliterans (BXO) in
children
a) BXO carries a 1% to 3% risk of penile car-
cinoma before the age of 20 years
b) Treatment with topical steroids avoids the
need for surgery in 80% to 85% of cases
c) Preputioplasty is preferable to circumci-
sion for the treatment of BXO in boys over
10 years of age.
d) Glans involvement is present in 5 to 7% of
cases
e) BXO is rare in children under 5 years of age
2 e parents of a 4-year-old boy are con-
cerned because they have observed obvious
ballooning of his prepuce during micturi-
tion. Which of the following is most appli-
cable in this case?
a) e ballooning probably denotes the pres-
ence of BXO
b) Measurement of his urine ow rate is
likely to show partial obstruction.
c) e most likely diagnosis is congenital
mega prepuce
d) e parents can be reassured that balloon-
ing is a self-limiting phenomenon of no
pathological signicance
e) e presence of ballooning is a strong
indication for circumcision
Self-Assessment Section: Questions 357
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3 Which of the following statements best
describes the features of congenital mega
prepuce?
a) e condition is characterised by an exces-
sively long, redundant prepuce
b) e overall appearances may resemble a
‘buried’ penis
c) A standard circumcision usually gives sat-
isfactory cosmetic results
d) Any male siblings will also have a 1:10
chance of being born with this condition
e) Voiding is usually normal despite the
megaprepuce
4 Which of the following statements is most
applicable to preputioplasty?
a) Preputioplasy can be used as an eective
alternative to circumcision in 70% to 80%
of boys with BXO
b) Retraction of the prepuce should be
avoided for three to six months aer the
procedure
c) Regular retraction of the prepuce should
be advised in the early post operative
period
d) Preputioplasty is equally successful in all
age groups
e) None of the above
5 A 5-year-old boy is referred with a non
retractile prepuce. e prepuce is healthy
but cannot be retracted beyond the tip of the
glans because of preputial adhesions. What
management should be advised?
a) Reassurance, see again only if symptoms
arise
b) Circumcision
c) Preputioplasty
d) Surgical release of adhesions under gen-
eral anaesthesia
e) Surgical release of adhesions under gen-
eral anaesthesia if the prepuce is still
partially adherent to the glans at 6 years
of age
CHAPTER 18: TESTIS,
HYDROCOELE AND
VARICOCOELE
1 Which of the following statements best apply
to the aetiology and pathophysiology of the
undescended testis?
a) e incidence of undescended testis is 0.5–
1% in term infants
b) Transformation from fetal gonocytes to
Ad dark spermatogonia occurs at around
36–38 weeks of gestation
c) Following meiotic divisions primary sper-
matocyte gives rise to 8 spermatozoa
d) Gonocyte transformation is impaired in
undescended testes
e) e histological appearances of ascending
testes are normal
2 A 3-year-old boy is referred with a unilateral
impalpable testis. Which of the following is
the most reliable means of conrming the
presence or absence of the testis?
a) Abdominal ultrasonography
b) MRI
c) CT scan
d) Retrograde venography
e) Laparoscopy
3 Which of the following statements best apply
to the management of the undescended and
retractile testes?
a) Orchidopexy should be performed for
ascending testes at the time of presentation
b) Double blind studies have demonstrated
that hormonal treatment for congenitally
undescended testes has a success rate of
25–30%
c) e Fowler-Stephens procedure is opera-
tion of a choice for high inguinal testes
d) e optimal age for orchidopexy for a con-
genitally undescended testis is currently
thought to be 2 years of age
e) e parents of boys with retractile testes
can be reassured that no treatment or follow up are required
358 Appendix I
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4 An 11-year-old boy presents with a painless
grade III le sided varicocele. What advice
should be given to his parents?
a) Infertility is inevitable unless some form
of intervention is undertaken
b) Treatment of the varicocele is likely to
carry a 50% failure rate regardless of the
mode of treatment
c) A CT renal scan should be performed in
view of the 2% risk of Wilms tumour
d) An ultrasound scan of the testes and kid-
neys is all that is required at this stage
e) A semen sample should be analysed before
determining further management
5 Testicular ultrasound in a 14-year-old boy
demonstrates bilateral microcalcication.
is is an incidental nding following a
sporting injury. What action should be
advised in the light of these ndings?
a) Reassurance, no further follow up unless
symptomatic
b) Urgent testicular biopsy
c) Testicular biopsy around the age of 19 or
20 years
d) Bilateral prophylactic testicular xation in
view of the high risk of testicular torsion
e) Six monthly ultrasound review for 5 years
with annual ultrasound examination
thereaer
CHAPTER 19: THE ACUTE
SCROTUM
1 Scrotal exploration is being performed in a
14-year-old boy for a presumed diagnosis of
right testicular torsion. He has a 10-hour his-
tory of symptoms. e diagnosis is conrmed
but despite detorsion, the testis remains dark
blue in colour with no evidence of perfusion.
What action should the surgeon take?
a) Right orchidectomy. Defer xation of
the le testis for at least 3 months second
because of the risk of infection
b) Leave the right testis in situ because it
might contribute to endocrine function.
Defer xation of the le testis until the fate
of the right testis is known
c) Right orchidectomy combined with simul-
taneous xation of the le testis
d) Leave the right testis in situ because it
might contribute to endocrine function.
Simultaneous suture xation of the le
testis
e) Right orchidectomy and implantation
of silicone gel prosthesis, simultaneous
suture xation of the le testis
2 A 5-year-old boy presents with diuse swell-
ing involving both sides of the scrotum He
is afebrile and has only minimal discomfort.
What action should be taken next?
a) Urgent surgical exploration to exclude an
atypical presentation of bilateral synchronous testicular torsion.
b) Commence intravenous antibiotics
c) Take blood for alpha feto protein and other
tumour markers
d) Oral Prednisilone
e) No specic treatment
Self-Assessment Section: Questions 359
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3 A 13-year-old boy presents with severe right
iliac fossa pain of sudden onset 4-hours
earlier. No abnormality is detected on
abdominal palpation. His right testis is
tender but there is no erythema of the overlying scrotal skin. How should he be investigated and managed?
a) Scrotal ultrasound
b) Admit for observation, re-assess clinical
ndings aer 4 hours
c) Doppler ultrasound
d) Radionuclide scan
e) No investigation, advise patient and par-
ents of risk of torsion and proceed directly
to surgical exploration.
4 A 17-year-old patient with testicular tor-
sion has undergone unilateral orchidectomy
for a non-viable testis. What advice should
be given in response to questions regarding
the prognosis for endocrine function and
fertility?
a) Testosterone levels should be checked
annually since endocrine replacement
treatment may be required
b) ere is a risk that semen quality will be
reduced but his prospects of achieving
paternity will probably be normal
c) He has a 20% chance of being infertile
but could probably achieve paternity with
assisted conception techniques
d) He may experience erectile dysfunction
due to low testosterone levels but this
could be treated with sildenal.
e) None of the above
5 Which of the following statements does not
apply to torsion of the Hydatid of Morgagni?
a) e ‘blue dot’ sign is present in 50% to 60%
of cases
b) e peak incidence is between 10 to 12
years of age
c) It can usually be diagnosed on clinical
grounds
d) e pain is usually more gradual in onset
than testicular torsion
e) Conservative management can be safely
considered if symptoms are mild or resolving
CHAPTER 20: DISORDERS
OF SEX DEVELOPMENT
1 Which is the commonest disorder of sex
development encountered in North America
and Europe?
a) Ovotesticular DSD
b) 5 Alpha reductase deciency
c) 46XX DSD due to congenital adrenal
hyperplasia
d) Mixed gonadal dysgenesis
e) 46XY DSD (male pseudo
hermaphroditism)
2 Which of the following statements best
describes the features of children with
Turner syndrome?
a) Clitoral hypertrophy is present in 80–90%
of patients
b) Normal ovarian tissue is present in 55% to
75% of patients
c) e lifetime risk of gonadal malignancy is
signicantly increased
d) e lifetime risk of gonadal malignancy is
not signicantly increased
e) e diagnosis is most commonly made
during investigation of ambiguous genitalia in infancy
360 Appendix I
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3 Which of the following statements best
describes the aetiology and epidemiology of
congenital adrenal hyperplasia (CAH)?
a) CAH accounts for 20 to 25% of all infants
with ambiguous genitalia
b) e most common underlying endocrine
defect is 11 Beta hydroxylase deciency
c) CAH is an autosomal dominant disorder
with variable expression
d) CAH is an X linked recessive disorder
e) CAH is an autosomal recessive disorder
4 As a consequence of the ndings during a
hernia operation, Sarah, aged 5, is diagnosed
with complete androgen insensitivity syndrome. Which of the following statements
best describes the other features and the
karyotype?
a) e most likely karyotype is 47XXY
b) e most likely karyotype is 46XY
c) e most likely karyotype is 47XYY
d) e gonads identied in the hernial sacs
are most like to be dysgenetic streak gonads
e) e internal genitalia are most likely to
comprise a vestigial uterus and fallopian
tubes.
5 Which disorder of sex development is char-
acterised by an elevated plasma level of 17
OH progesterone?
a) Mixed gonadal dysgenesis
b) Ovotesticular DSD (true
hermaphroditism)
c) 46XY DSD (male
pseudohermaphroditism)
d) Müllerian inhibitory substance receptor
insensitivity
e) 46XX DSD due to congenital adrenal
hyperplasia
CHAPTER 21: GENITOURINARY
MALIGNANCIES
1 Which of the following statements most
accurately refers to the epidemiology and
aetiology of rhabdomyosarcoma?
a) e prognosis in not inuenced by the site
of origin within the genitourinary tract
b) Tumours with embryonal histology have a
higher survival rate
c) Rhabdomyosarcomas of the bladder and
prostate most commonly present as Stage 1
tumours
d) Approximately 50% of all rhabdomyosar-
comas in children arise within the genitourinary tract
e) Paratesticular rhabdomyosarcoma is usu-
ally treated with chemotherapy prior to
surgery
2 Which of the following statements best
describes evaluation of primary testicular
tumours in prepubertal boys?
a) A CT scan should be performed routinely
because of the high incidence of occult
metastatic disease
b) CT scans can be performed selectively
because of the low incidence of metastatic
disease
c) Serum alpha fetoprotein is the most
reliable marker of malignancy in the rst
6 months of life
d) Painful swellings are of greatest concern
e) e presence of retroperitoneal lymph
nodes is classied as Stage IV
3 What is the approximate current overall
5-year survival rate for children with Stage
I–II nephroblastoma (Wilms tumour)?
a) 20%–30%
b) 40%–50%
c) 50%–60%
d) 80%–90%
e) >95%
Self-Assessment Section: Questions 361
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4 Which of the following statements best apply
to the histology and staging of nephroblastoma (Wilms tumour)?
a) e histology is classied as unfavourable
in 20%–25% % of cases
b) Bilateral tumours are classied as Stage 4b
c) Metastatic spread is present in 25% of
cases at presentation
d) Wilms tumours classically have a biphasic
histological appearance comprised of blastemal and epithelial components
e) Abnormalities of chromosome 16 are
associated with poorer survival
5 Which of the following statements is most
applicable to Congenital Mesoblastic
Nephroma?
a) Complications of treatment are the com-
monest cause of death
b) It accounts for 15% of all pediatric renal
tumours
c) Stage III tumours with mixed histologic
subtype a higher risk of local recurrence
d) e mortality rate is lower when it pres-
ents in the rst month of life
e) It is a benign tumour which never
metastasizes
CHAPTER 22: PEDIATRIC
GENITOURINARY TRAUMA
1 A 7-year-old boy was admitted to hospital
4 hours previously following a fall from a
climbing frame. On admission he was hae-
modynamically stable but is tender in the
le upper quadrant. An ultrasound scan
on admission demonstrated a small haema-
toma in the region of the upper pole. He is
complaining of increasing pain. He now has
tachycardia and his systolic blood pressure
blood is 105 mm. Which of the following
would be most appropriate?
a) Urgent DMSA scan
b) Increased dose of analgesia and repeat
ultrasound scan if no improvement aer
12 hours.
c) Intravenous urogram
d) CT scan
e) Proceed directly to urgent surgical
exploration
2 Which of the following statements is most
applicable to bladder injuries in children?
a) ey are most commonly caused by falls
and sporting injuries
b) CT Cystography should be routinely per-
formed at the time of presentation to
assess the severity of the injury
c) Localized peri vesical extravasation can
usually be managed by catheter drainage
d) Any degree of peri vesical extravasa-
tion is an indication for urgent surgical
exploration
e) e bladder is less prone to injury in chil-
dren than adults because of its smaller size
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