Pediatric GU Flashcards
- A female newborn is found to have a sacral mass. On MR this is lobulated with well-defined margins and heterogeneous SI on T1 with high, intermediate and low SI areas. It is a predominantly eternal lesion, covered by skin with only minimal presacral component. Which is the most likely diagnosis?
A. Myelomeningocele
B. Sacrococcygeal teratoma
C. Rectal duplication cysts
D. Haemangioma
E. Lymphoma
B. Sacrococcygeal teratoma
Fat, soft tissue and calcification cause the heterogeneous SI on T1. This is the most common solid tumour in the
newborn, more common in females.
@# (CNS) 11. Which is the most prevalent intraventricular tumour?
A. Meningioma
B. Choroid plexus paplilloma
C. Astrocytoma
D. Dermoid
E. Subependymoma
C. Astrocytoma
Ependymomas followed by astrocytoma are the most common.
- Regarding neuroblastomas:
A. Produces catecholamines in all cases
B. Are most common in 5-10 years
C. Have a better prognosis in a younger child
D. The most common site following the adrenals is the neck
E. Esthesioneuroblastoma is a very malignant tumour arising from olfactory mucosa
E. Esthesioneuroblastoma is a very malignant tumour arising from olfactory mucosa
Neuroblastomas produce catecholamines in 95% of cases.
The peak age of diagnosis is < 3 and there is a better prognosis in older children.
The most common site is adrenals, followed by chest, followed by neck.
- An 8-month-old has a neuroblastoma in the right adrenal with metastatic spread to the liver, bone marrow and skin. What stage of disease does he have?
A. Stage 2B
B. 3
C. 2A
D. 4
E. 4S
E. 4S
Stage 4S applies to infants who demonstrate a tumor on one side of the body, as seen in Stages 1 and 2, but also have affected liver, skin, and/or bone marrow.
Stage 4 involves distant lymph nodes and bone with or without bone marrow/ liver/other organs.
- Currarinos triad is a rare association of anomalies including anorectal malformations, lumbrosacral anomalies and which of the following?
A. Meconium ileus
B. Presacral mass
C. Ileal atresia
D. Hirshsprungs
E. Cloacal extrophy
B. Presacral mass
This may be teratoma, anterior meningocele or enteric cyst.
- Regarding imaging of duplicated kidneys:
A. Micturating Cystourethroram (MCUG) shows reflux into the upper pole
B. Duplicated kidneys tend to be smaller
C. Duplex kidneys may have more than 1 renal artery and vein
D. US shows dilatation of the lower pole calyx with a normal upper pole
E. A band of medulla crosses the cortex of the kidney onUS
C. Duplex kidneys may have more than 1 renal artery and vein
In cases of duplicated kidneys, MCUG shows reflux into the lower pole.
Duplicated kidneys tend to be larger and US shows dilatation of the upper pole calyx and a normal lower pole.
A band of cortex can be seen to cross the medulla.
- Regarding posterior urethral valves:
A. Are seen equally on males and females
B. Associated with antenatal polyhydramnios
C. Gradual change in calibre occurs in the posterior urethra
D. Are associated with a smooth walled urinary bladder
E. Are associated with vesicoureteric reflux
E. Are associated with vesicoureteric reflux
Occurs in boys. Associated with oligohydramnios, an abrupt change in calibre of the posterior urethra and a
trabeculated urinary bladder wall.
- Neonatal ultrasound shows kidneys whch are enlarged bilaterally hyperechoic with loss of normal corticomedullary differentiation. Cysts are noted which are less than 1cm. There are focal rosettes of radially oriented
dilated collecting tubules. What is the diagnosis?
A. Autosomal Recessive Polycistic Kidney Disease (ARPKD)
B. Autosomal Dominant Polycistic Kidney Disease (ADPKD)
C. Asymmetrical large renal cysts
D. Tuberous sclerosis
E. Multicystic Dysplastic Kidney (MCDK)
A. Autosomal Recessive Polycistic Kidney Disease (ARPKD)
ADPKD have normal renal echogenicity. Small cyst may not be immediately identified on ultrasound.
@# 46. Which of the following are common features in mesoblastic nephroma?
A. Cystic areas of necrosis
B. Areas of haemorrhage
C. Calcification
D. Invasion of vessels
E. Polyhydramnios in pregnancy
E. Polyhydramnios in pregnancy
Cystic areas of necrosis, areas of haemorrhage and calcification are uncommon.
@# 47. Nephroblastomatosis, Beckwith-Wiedemann syndrome, chromosome 11 abnormalities and trisomy 18 are all associations of which of the following?
A. Autosomal recessive polycystic kidney disease
B. Neuroblastoma
C. Nephroblastomatosis
D. Wilms’ tumour
E. Multilocular cystic nephroma
D. Wilms’ tumour
These are all known associations of Wilm’s tumor
- A female infant is found to have a pelvic mass. She is systemically well. US shows a well-defined fluid-filled
cavity between the bladder and rectum with some debris but no increased vascularity. There is uterine distension
and echogenic debris within the vagina. Hydronephrosis is noted. Which is the diagnosis?
A. Pelvic abscess
B. Hydrometocolpos
C. Ovarian tumour
D. Fallopian tube torsion
E. Sacrococcygeal teratoma
B. Hydrometocolpos
MR shows mixed SI on T1 & T2 due to blood products and may be associated with uterine and cervical anomalies.
- Which of the following is a feature of neuroblastoma?
A. Large hypoechoic mass arising the suprarenal region
B. Calcification in most cases on CT
C. No increased vascularity
D. Echogenic liver lesions
E. Vascular invasion
A. Large hypoechoic mass arising the suprarenal region
Low attenuation metastatic liver lesion are echogenic on US. May have lytic/sclerotic or mixed bone metastases,
increased vascularity and vascular encasement.
@# 50. A child with Wilms’ tumour has exomphalos, macroglossia, gigantism and hepatomegaly. Which is the diagnosis?
A. Hemihypertrophy
B. Drash syndrome
C. Beckwith-Wiedemann
D. Trisomy 18
E. Trisomy 21
C. Beckwith-Wiedemann
A, B and C are all associations of Wilm’s tumour. 10-20% of patients with Beckwith-Wiedemann develop
Wilm’s tumour.
4) A 3-year-old child with aniridia is found to have a palpable abdominal mass. The mass is shown to arise from
a kidney and to contain cystic elements on ultrasound scan. No calcification is seen in the tumour on CT. Which
of the following diagnoses is the most likely?
a. renal cell carcinoma
b. neuroblastoma
c. angiomyolipoma
d. Wilms’ tumour
e. ossifying renal tumour of infancy
d. Wilms’ tumour
Wilms’ tumour is the commonest renal tumour of childhood. Seventyfive per cent occur in children under 5 years,
5–10% are bilateral and 10% are multifocal. Calcification is seen in less than 15%. Nephroblastomatosis is a
precursor, and the disease is associated with the WT1 and WT2 genes of chromosome 11. The WT1 abnormal
gene is found in the WAGR syndrome of Wilms’ tumour, aniridia, genitourinary abnormalities and learning
disability. It is also found in the DRASH syndrome of male pseudohermaphroditism and progressive
glomerulonephritis. The abnormal WT2 gene is found with the Beckwith–Wiedemann syndrome and
hemihypertrophy.
9) A baby boy is investigated for renal failure. The imaging findings are of bladder distension and a posterior
urethral valve, ureters measuring 10 mm in diameter bilaterally, undescended testes and widely separated
abdominal rectus muscles. What is the most likely diagnosis?
a. prune-belly syndrome
b. primary vesicoureteric reflux
c. developmental aperistalsis of the distal ureter
d. neuropathic bladder
e. ureterocele
a. prune-belly syndrome
(a) to (e) are all causes of a megaureter – that is, a ureter over 7 mm in diameter. The hallmark of prune-belly
syndrome is a distended bladder, and it is associated with posterior urethral valves. Bladder distension causes the
triad of widely spaced abdominal rectus muscles, hydroureteronephrosis and cryptorchidism. The last occurs
because the large bladder interferes with testicular descent.
24) Abdominal ultrasound is performed on a neonate on the highdependency unit to investigate a palpable mass.
A heterogeneous avascular suprarenal mass is identified. Cystic change and a peripheral hyperechoic rim develop
over a series of scans. Which of the following is the most likely cause of the abdominal mass?
a. nephroblastoma
b. neuroblastoma
c. adrenal haematoma
d. phaeochromocytoma
e. myolipoma
c. adrenal haematoma
Adrenal haemorrhage is not only the commonest cause of neonatal adrenal mass, but is also more likely to be seen in neonates in a high-dependency unit because it is associated with perinatal stress, hypoxia, septicaemia and hypotension. It can be unilateral or bilateral, but, even when bilateral, it does not usually cause adrenal insufficiency. Initially, the haematoma appears as an avascular heterogeneous mass on ultrasound scan that becomes cystic and smaller over a period of weeks.
A hyperechoic rim can form, representing peripheral calcification. Haematomas can become infected, resulting
in an abscess.
Neuroblastoma is the main differential diagnosis; on ultrasound scan, it appears as a hyperechoic
mass that can have internal flecks of calcification. Repeat ultrasound scan at 1 week will not show the changes that haematoma undergoes.
28) A 5-year-old boy has recurrent urinary tract infection. A micturating cystourethrogram is performed, during
which contrast from the bladder enters both ureters and reaches the pelvis and calyces bilaterally without any
urinary tract dilatation. Of what grade is this vesicoureteric reflux?
a. I
b. II
c. III
d. IV
e. V
b. II
The international grading system for vesicoureteric reflux divides reflux into five classes. Grade I describes reflux
into the ureter only, with grade II referring to reflux into the pelvicalyceal system without calyceal dilatation or
blunting. Grade III reflux is associated with mild pelvicalyceal and ureteric dilatation, though forniceal angles
remain distinct. Grade IV is associated with a tortuous ureter and moderate dilatation of the pelvicalyceal system,
with blunting of the forniceal angles. Grade V reflux describes grossly dilated tortuous ureters with marked
pelvicalyceal dilatation and obliteration of the forniceal angles.
36) A 1-month-old girl has liquid discharge from the umbilicus. Which of the following provides a suitable
explanation?
a. vesicourachal diverticulum
b. urachal cyst
c. patent urachus
d. bladder exstrophy–epispadias complex
e. cloacal exstrophy
c. patent urachus
Embryologically, the cloaca is divided by the urorectal septum into a dorsal part that develops into the rectum
and a ventral part that gives rise to the allantois, bladder and urogenital sinus. The wolffian and mu¨llerian ducts
drain into the ventral cloaca. The allantois becomes the urachus, which is the umbilical attachment of the bladder.
Ordinarily, this atrophies to become the umbilical ligament. If it remains patent throughout its entire length, urine
can drain via the umbilicus. A urachal sinus and a vesicourachal diverticulum describe patent portions of the
urachus at the umbilical and bladder ends respectively.
34) Ultrasound scan of the abdomen of a newborn girl reveals an abdominopelvic cyst. It is thin walled and
anechoic, and has a ‘daughter cyst’. Of the following which is the most likely diagnosis?
a. Wilms’ tumour
b. ovarian cyst
c. cystic lymphatic malformation
d. choledochal cyst
e. cystic teratoma
b. ovarian cyst
Ovarian cysts in the newborn are more common than enteric duplication cysts, giant meconium pseudocysts,cystic lymphatic malformations or choledochal cysts. Other rarer causes of intraabdominal cystic structures in the newborn include cystic teratomas, gastric teratomas, cystic granulosa cell tumour of the ovary, ovarian teratomas and cystadenomas. Ovarian cysts may become echogenic due to the haemorrhage that can occur if they tort (twist).
These cysts also have associated normal ovarian tissue, and the daughter cyst represents a follicle along the wall.
Wilms’ tumours are solid and occur later in life.
A giant meconium pseudocyst has a thick echogenic wall, and viscous echogenic contents. It is formed by meconium leak following fetal bowel perforation due to intestinal obstruction in meconium ileus, ileal atresia or volvulus. Twenty-five per cent show peritoneal or cyst calcification,which is pathognomonic for meconium pseudocyst. Bowel obstruction may be present also.
Cystic lymphatic malformations appear as large, well-circumscribed, cystic, thin-walled structures with multiple thin septa. Internally, the fluid can be echo free or echoic because of haemorrhage, debris, chyle or infection. Mesenteric,omental or retroperitoneal cysts are seen.
Choledochal cysts are subhepatic or in the porta hepatis. They are seen separate from the gallbladder and are round, tubular or teardrop shaped, and connected to the biliary tree.
41) At a 20-week, antenatal ultrasound scan, a fetus has a renal pelvis diameter measured at 7 mm unilaterally.
When the scan is repeated at 35 weeks, the renal pelvis measures 12 mm. Which of the following is the most
appropriate follow-up for the neonate?
a. discharge
b. ultrasound scan at 6 weeks
c. ultrasound scan within 24 hours of birth
d. micturating cystourethrogram soon after birth
e. 99mTc-labelled DMSA scan soon after birth
c. ultrasound scan within 24 hours of birth
Pyelectasis can be regarded as a renal pelvis diameter greater than 4– 5 mm at 20 weeks’ gestation or 7 mm at 33
weeks, or above 10 mm at birth. Local protocol varies as to which fetuses to follow up and how. Some centres
perform an ultrasound scan on all neonates who have had a renal pelvis diameter of above 5 mm at any point;
others may only investigate if there is a renal pelvis above 10 mm persisting to birth. Ultrasound scan soon after
birth, however, is the best way of detecting severe obstructive pathology, such as posterior urethral valves, that
may warrant rapid surgical intervention. On this scan, if there is persisting dilatation above 10 mm, antibiotic
prophylaxis and micturating cystourethrogram (MCUG) are appropriate. If reflux is seen on the MCUG, DMSA
and repeat ultrasound scan at 6 weeks are appropriate. DMSA is used to assess parenchyma for scarring. If no
reflux is seen, MAG3 scan within 6 weeks is suggested to look for obstruction. If the renal pelvis diameter is less
than 10 mm within 24 hours of birth, followup ultrasound scan 6 weeks later is suggested.
54) A 12-year-old boy is investigated for abdominal pain. On ultrasound scan, there are large, echo-free, septated,
cystic collections around both kidneys causing scalloping of the renal outline. On CT, these collections are close
to water density. Which of the following is the most likely diagnosis?
a. renal lymphangiectasia
b. bilateral Wilms’ tumours
c. bilateral adrenal neuroblastoma
d. bilateral hydronephrosis
e. medullary sponge kidney
a. renal lymphangiectasia
Renal lymphangiectasia is a very rare developmental malformation probably caused by a failure of the developing
kidney lymphatics to establish communication with the extrarenal lymphatic system. Abnormal lymphatic
channels dilate, resulting in cystic lesions in the parapelvic, perinephric and, less commonly, retroperitoneal
regions. The lesions are of water attenuate
@# 55) A 15-year-old female, whose father had progressive renal failure, presents with anaemia, polyuria and
haematuria. On ultrasound scan, her kidneys are small and smooth. Which associated finding is most likely?
a. pancreatic cysts
b. posterior fossa haemangioblastoma
c. cystocele
d. nerve deafness
e. hypertension
d. nerve deafness
Alport’s syndrome or chronic hereditary nephritis is the unifying diagnosis. It is inherited, probably in an
autosomal dominant fashion. Ocular abnormalities can also occur, including congenital cataracts, nystagmus, myopia and spherophakia. Hypertension is not a feature. The renal impairment is progressive in affected males
but non-progressive in females.
Cerebellar and retinal haemangioblastomas occur in von Hippel–Lindau syndrome, along with renal, pancreatic and adrenal cysts.
75) Which of the following features on ultrasound scan is most suggestive of a horseshoe kidney?
a. unilateral upper pole calyx dilatation
b. unilateral lower pole calyx dilatation
c. bilateral upper pole calyx dilatation
d. laterally oriented renal long axis
e. medially oriented renal long axis
e. medially oriented renal long axis
Ninety per cent of horseshoe kidneys are joined at the lower poles by a parenchymal or fibrous isthmus. The
isthmus lies at the L4–5 level, where renal ascent is arrested by the inferior mesenteric artery. The pelves and
ureters are anterior, and pelviureteric junction obstruction is more common. Incidence is 1–4/1000 births, making
it the commonest renal fusion abnormality. Cardiovascular, skeletal, central nervous system, anorectal and
genitourinary malformations are associated. There are associations with trisomy 18 and Turner’s syndrome.
Associated genitourinary anomalies include hypospadias, undescended testes, bicornuate uterus and ureteral
duplication. The incidence of infection and stones increases in a horseshoe kidney.
(GU) 77) On an antenatal ultrasound scan, unilateral fetal ureteric dilatation is identified. Follow-up imaging
after birth reveals persistent dilatation of a non-tortuous ureter that is aperistaltic in its distal segment. There are
no other associated urogenital anomalies. Which of the following is the most appropriate diagnosis?
a. prune-belly syndrome
b. primary vesicoureteric reflux
c. primary megaureter
d. neuropathic bladder
e. ureterocele
c. primary megaureter
The primary megaureter occurs because of developmental aperistalsis of the distal ureter. The ureter tends to be
dilated but straight in a congenital primary megaureter. Primary vesicoureteric reflux and obstructive secondary
megaureter tend to cause tortuous ureteric dilatation
79) On antenatal ultrasound scan, oligohydramnios, non-visualization of the urinary bladder and bilateral enlarged hyperechoic kidneys are recorded. Renal failure ensues within the first month of life. An older sibling aged 5 years has established portal hypertension. The parents are phenotypically normal. What are the odds of further
offspring of the same parents having the same disease?
a. zero
b. 1 in 5
c. 1 in 4
d. 1 in 2
e. unity
c. 1 in 4
Infantile polycystic kidney disease is autosomal recessive. Both parents and half the children will be carriers. The
sonographic hallmark is enlarged echogenic kidneys. The individual cysts are small, measuring 1–2 mm, and can
be defined only with a high-resolution probe. Autosomal kidney disease affects the liver also. The less severe the
renal disease, the more severe the hepatic periportal fibrosis.
(GU) 79) An IVU is performed on a teenager. On a 15-minute, full-length radiograph, the right ureter and
collecting system appear normal, while the left collecting system is displaced laterally and inferiorly, giving a
‘drooping flower’ appearance. What is the most likely congenital anomaly?
a. bilateral ureteral duplication
b. left ureteral duplication
c. left ureteral diverticulum
d. recently passed left ureteric stone
e. crossed fused ectopia
b. left ureteral duplication
The ‘drooping flower’ appearance on IVU occurs with a duplicated system when the obstructed dilated upper
pole collecting system displaces the contrast-opacified, lower collecting system laterally and inferiorly. The
Weigert–Meyer rule describes the commonest relationship of insertion of the duplicated ureters into the bladder.
The upper collecting system ureter inserts ectopically into the bladder inferior and medial to the orthotopic ureter,
which enters the bladder near the trigone. The ectopic ureter may be stenotic or obstructed with or without
ureterocele
87) Which of the following would be regarded as abnormal during ultrasound scan of the kidneys in a neonate?
a. echogenicity of the renal cortex similar to that of liver
b. large hyperechoic medullary pyramids
c. paucity of renal sinus fat
d. lobulated renal contour
e. echogenic septum at anterosuperior margin of the kidney
b. large hyperechoic medullary pyramids
The average length of the normal neonatal kidney is 4.5 cm. Ultrasound appearances of the kidney in the neonate
and infant show a number of differences from those of the older child and adult. Cortical echogenicity is increased,
as the glomeruli form 20% of the cortex in the neonate but only 9% in the adult, and may be comparable to that
of the liver or the spleen. The medullary pyramids are larger and more hypoechoic than in older children and
adults, and there may be little or no fat in the renal sinus. Persistent fetal lobulation may give the kidneys a
lobulated contour. In addition, echogenic septa may be normally seen at the anterosuperior or posteroinferior
margins of the kidney, representing the sites of fusion of metanephric elements. Most kidneys attain an adult
pattern by 6 months of age
96) A 16-year-old female with a history of imperforate anus corrected soon after birth is investigated for primary
amenorrhoea. Ultrasound scan shows the uterus and upper vagina distended by moderately echogenic material.
Which of the following is the most likely cause of the amenorrhoea?
a. longitudinal vaginal septum
b. transverse vaginal septum
c. cervical dysgenesis
d. obstructed uterine horn
e. endometritis
b. transverse vaginal septum
Haematometrocolpos is described, which at this age can be due to a transverse vaginal septum or imperforate
hymen. There is an association with imperforate anus, hydronephrosis, renal agenesis and dysplasia, polycystic
kidneys, duplication of the vagina and uterus, sacral hypoplasia and oesophageal atresia. Cervical dysgenesis and
obstructed uterine horn would produce haematometra.
3 An antenatal US performed at 20 weeks demonstrated a right renal pelvis with an AP diameter of 5 mm. A 32
week scan shows the diameter to be 10 mm. The post-natal US at 4 days confirms unilateral right-sided neonatal
hydronephrosis. What is the commonest cause of these findings?
(a) Ectopic ureterocoele
(b) Pelvi-ureteric junction obstruction
(c) Posterior urethral valve
(d) Prune-belly syndrome
(e) Vesico-ureteric junction obstruction
(b) Pelvi-ureteric junction obstruction
The most common causes of antenatal hydronephrosis (regardless of gender), in order of decreasing frequency
are: PUJ obstruction (22%), posterior urethral valve (18%), ectopic ureterocoele (14%), Prune-belly syndrome
(12%), and VUJ obstruction (8%)
6 A 3 year old girl presents with increasing abdominal girth. US demonstrates a mass within the region of the left
kidney. The patient proceeds to CT examination. Which of the following findings favours Wilms’ tumour over
neuroblastoma?
(a) Calcification within the mass
(b) Mass crosses the mid line
(c) Mass encases and lifts the aorta
(d) Mass invades into the IVC
(e) Presence of bone metastases
(d) Mass invades into the IVC
Classically neuroblastoma encases vessels, whereas Wilm’s tumours may invade vessels. Calcification is more
common in neuroblastoma (85-95%) than Wilm’s (15%), neuroblastoma commonly crosses the midline, only
10% of Wilm’s tumours are bilateral. Neuroblastoma typically metastasises to bone and lymph nodes; Wilm’s to
the liver, lungs and lymph nodes.
A 3 year old boy presents with abdominal swelling. US and subsequent CT examination show a large 12 cm mass
inferior to the liver. There is distortion of the renal parenchyma and apparent exophytic growth. There is poor
enhancement and the aorta and IVC are displaced. Which of the following features is not a known association?
(a) Aniridia
(b) Cerebellar ataxia
(c) Horseshoe kidney
(d) Macroglossia
(e) Male pseudohermaphroditism
(b) Cerebellar ataxia
Cerebellar ataxia is associated with neuroblastoma. Wilm’s is associated with WAGR (includes duplex/
horseshoe/ fused kidneys), Drash syndrome (male pseudo-hermaphroditism), and BeckwithWiedemann
syndrome (macroglossia). It is also associated with spontaneous aniridia and hemihypertrophy
9 A neonatal male patient failed to pass meconium by 48 hours and was found to have an imperforate anus of the
high malformation subtype. A colostomy was formed in the neonatal period and surgical repair completed in
infancy. At the age of 5 the patient presents with a UTI. What is the likely underlying aetiology of the UTI?
(a) Colovesical fistula
(b) Neurogenic bladder
(c) Pelvi-ureteric junction obstruction
(d) Rectourethral fistula
(e) Urethrocoele
(b) Neurogenic bladder
Anorectal malformation may be low (commonest), intermediate, or high, depending on the relation to the levator
sling. High malformations are associated with fistulas and a high association with GU (50%), and VACTERL
anomalies. History of surgical repair makes fistulas unlikely; post-op patients often suffer urinary incontinence;
the additional association of spinal anomalies make neurogenic bladder (which predisposes to UTI) a likelihood.
There is no association with PUJ obstruction or urethrocoele.
14 An 18 month old boy is found to have a neuroblastoma. A subsequent CT is performed for staging purposes.
This reveals the primary tumour and evidence of metastases in the skin, liver, lung and bone marrow.
What is the appropriate stage?
(a) Stage I
(b) Stage II
(c) Stage Ill
(d) Stage IV
(e) Stage IV-S
(d) Stage IV
Stage I limited to organ of origin.
Stage II regional spread, but does not cross midline.
Stage III tumour extends across midline, or is a unilateral tumour with contralateral LNs.
Stage IV: the tumour has spread to distant lymph nodes, bone, bone marrow, liver, skin or other organs.
Stage 1IV-S is a subset of neuroblastoma in infants < 1 year with a good prognosis
20 Which of the following conditions has the highest frequency of non-genitourinary anomalies?
(a) Ask-Upmark kidney
(b) Bilateral ectopia
(c) Crossed-fused ectopia
(d) Duplex kidney
(e) Horseshoe kidney
(e) Horseshoe kidney
Horseshoe kidneys are associated with other renal anomalies in 50%, and non-GU system anomalies in 30% (GI,
cardiac, skeletal). In 90% of crossed ectopia there will be fusion (crossed-fused ectopia); associated anomalies
are rare. Ask-Upmark kidney shows segmental hypoplasia; there is controversy over whether it is a congenital
anomaly or a sequelae of severe pyelonephritis. In the cited reference, no patients with duplex kidneys were found
to have non-GU related anomalies.
(GU) 24 A 10 year old boy, recently arrived from the Indian subcontinent, presents with vague abdominal
distension and discomfort. US shows bilateral multiple, non-communicating, well defined cystic lesions in the peripelvic region, renal parenchyma and in the perirenal spaces. CT shows the lesions to be homogeneous with no significant contrast enhancement. At MRI the lesions were of low signal intensity on T1W and high signal intensity on T2W, again without enhancement. Needle biopsy showed areas of connective tissue with an`endothelial lining. What is the likeliest diagnosis?
(a) Multicystic dysplastic kidney
(b) Multilocular cystic nephroma
(c) Tuberculosis
(d) Wilm’s tumour
(e) Renal lymphangiectasia
(e) Renal lymphangiectasia
Although rare, renal lymphangiectasia has a characteristic imaging appearance resulting from the failure of the
renal lymphatic tissue.
25 A 4 year old girl has a proven first UTI. E. coli is cultured and there is a good response to antibiotics. Her GP phones to ask your advice on the most appropriate way to proceed with imaging. What should you recommend?
(a) OMSA scan at 6 months
(b) MCUG at 6 weeks
(c) No imaging
(d) US acutely
(e) US at 6 weeks
(c) No imaging
Recent NICE guidelines recommend no imaging for simple (nonatypical) first UTls in infants 6 months - 3 years
and children over 3 years which respond to antibiotics within 48 hours. In children over 3 years with an atypical
UTI, an US should be urgently arranged, in those with recurrent UTls a 6 week US and 6 month DMSA scan
should be arranged; there is no role in this age group for MCUG. An US is always performed in those < 6 months
old (routinely at 6 wks), if this is abnormal, or there is an atypical organism, or recurrent UTls, MCUG is
performed. MCUG may be considered in those 6 months – 3 years if there is pelvic dilation on US, or a family
history of VUR
26 Which of he following types of polycystic kidney disease is associated with greatest degree of liver fibrosis?
(a) Autosomal dominant PKO
(b) Antenatal autosomal recessive PKO
(c) Infantile autosomal recessive PKO
(d) Juvenile autosomal recessive PKO
(e) Neonatal autosomal recessive PKO
(d) Juvenile autosomal recessive PKO
ARPKD is associated with liver fibrosis and portal hypertension. The degree of hepatic fibrosis is inversely related
to the degree of renal disease. The relative involvement is: antenatal (90%; renal disease; minimal hepatic
fibrosis), neonatal (60%; mild fibrosis) infantile (20%; moderate fibrosis) and juvenile (10%; gross liver fibrosis).
Juvenile type is postulated to be a primary hepatic fibrosis rather than an ARPKD.
33 A 30 year old man presents with recurrent loin pain. Ultrasound shows a horseshoe kidney. Which of the following statements is incorrect?
(a) The incidence at autopsy is 1:2,000
(b) Fusion of the kidneys occurs at the lower pole in 90%
(c) It is more common in males
(d) Ascent of the kidney is arrested at the L3 level by the inferior mesenteric artery
(e) It is seen in 60% of patients with Turners syndrome
(a) The incidence at autopsy is 1:2,000
It is the commonest fusion abnormality and is seen in 1:400 patients at autopsy. It can be complicated by
obstruction, reflux or stone formation. The isthmus can contain functioning renal tissue.
39 An antenatal US shows oligohydramnios, a protruberant abdomen, bilateral hydronephrosis and markedly dilated and tortuous ureter. The posterior urethra is dilated and there is a large distended thin-walled bladder. At birth the testicles are noted to be undescended. What is the most likely diagnosis?
(a) Cloacal abnormality
(b) Megacystis-microcolon-hypoperistalsis (MMH) syndrome
(c) Posterior urethral valves
(d) Prostatic rhabdomyosarcoma
(e) Prune-belly syndrome
(e) Prune-belly syndrome
Prune-belly syndrome is a triad of absent/ deficient abdominal muscules, renal dysplasia (bilateral hydroureters/
hydronephrosis), and cryptorchidism. Due to the associated absence of the prostate gland, the posterior urethra is
often dilated proximally - the entire urethra may be dilated. PUVs are a key differential, but the implied abdominal
wall defect, thin-walled bladder, and cryptorchidism go against this. MMH causes insufficiency of the abdominal
viscera, but occurs in females. Rhabdomyosarcoma would be unusual antenatally.
(GU) 42 A neonate is found to have an abdominal wall defect and bilateral cryptorchidism. Which of the following features would not support a syndromic diagnosis?
(a) Bilateral ureteromegaly
(b) Trabeculated low capacity bladder
(c) Widened prostatic urethra
(d) Urethral dilatation
(e) Asymmetric renal size
(b) Trabeculated low capacity bladder
The classical triad of prune-belly syndrome is abdominal wall defect/ weakness, bilateral cryptorchidism and
genito-urinary abnormalities. Typically, a thickened, non-trabeculated, large volume bladder is seen. Non GU
abnormalities affecting the lungs (CCAM, lung hypoplasia), musculoskeletal system (scoliosis, pectus
excavatum), cardiovascular (VSD, PDA, Fallots) and GI system (Hirschprung’s disease, malrotation) are also
seen
(GU) 46 A 3 year old undergoes CT of the abdomen which shows an 8 cm heterogeneous enhancing mass which does not extend across the midline, but which displaces major vessels. Which of the following conditions is not associated?
(a) Cryptorchidism
(b) Hypoplasia of the iris
(c) Hemihypertrophy
(d) Drash syndrome
(e) Proptosis
(e) Proptosis
The features described would suggest a diagnosis of Wilm’s tumour which has a number of associations including
Beckwith-Wiedemann syndrome, sporadic aniridia, hemihypertrophy and other genitourinary abnormalities
including Drash syndrome. Proptosis can be seen in neuroblastoma.
8 A 3 year old boy with a Wilm’s tumour undergoes CT examination to aid staging. The CT shows a large mass
arising from the right kidney, with liver lesions, vertebral lesions, and enlarged hilar lymph nodes. Based on these
findings, the stage is?
(a) Stage I
(b) Stage II
(c) Stage Ill
(d) Stage IV
(e) Stage V
(d) Stage IV
Stage I: tumour limited to the kidney.
Stage II: extension into the perinephric space +/-vessel invasion.
Stage III: LNs in the abdomen or pelvis, or peritoneal invasion.
Stage IV: haematogeneous spread to lung/ liver/ brain/ bone, or to LNs outside the abdomen or pelvis.
Stage V: bilateral renal tumors at diagnosis.
9 A neonate presents with a visible mass within the left flank. US confirms a solid intra-renal lesion. What is the
likeliest tumour?
(a) Mesoblastic nephroma
(b) Multi cystic dysplastic kidney
(c) Nephroblastomatosis
(d) Neuroblastoma
(e) Wilm’s tumour
(a) Mesoblastic nephroma
Mesoblastic nephroma is the most common solid intrarenal mass in the neonate. 90% present within the 1st year
(most by 3 months). It is benign, but there is a risk of sarcomatous degeneration·. Wilm’s tumour is commoner in
older infants; neuroblastoma is the commonest abdominal malignancy in neonates.
12 What is the commonest presenting symptom of Wilm’s tumour?
(a) Abdominal pain
(b) Haematuria
(c) Hypertension
(d) Palpable abdominal mass
(e) Weight loss
(d) Palpable abdominal mass
The commonest presenting features of Wilm’s tumour are: palpable mass (90%; 12 cm is the mean diameter at
diagnosis), hypertension (50%), pain (35%), anorexia (15%), fever (15%) and haematuria (5%).
19 Antenatal US shows bilateral renal pelvis dilation. A micturating cysto-urethrogram is performed. On the right
side there is reflux of contrast into the ureter, renal pelvis and calyces but no dilatation. On left side there is
mild/moderate dilatation of the ureter, renal pelvis and calyces with minimal blunting of the fornices.
What is the correct grading of the patient’s vesico-ureteric reflux?
(a) Right grade 0, left grade I
(b) Right grade I, left grade II
(c) Right grade II, left grade III
(d) Right grade Ill, left grade IV
(e) Right grade IV, left grade V
(b) Right grade I, left grade II
Grade I - reflux into ureter.
Grade II - reflux into the ureter/ pelvis/ calyces; no dilatation.
Grade III - moderate dilatation with minimal blunting of the fornices.
Grade IV - moderate ureteral tortuosity and obliteration of the fornices.
Grade V - gross dilatation with tortuous ureter.
25 An 18 month old boy presents with fever and intractable watery diarrhoea and is found to be hypertensive. On examination there is a palpable mass in the left side of the abdomen. US and subsequent CT examination reveal a mass superior to the left kidney which is noted to contain hypoechoic areas on US and low attenuation areas on CT. What is the most likely diagnosis?
(a) Adrenocortical carcinoma
(b) Adrenal haemorrhage
(c) Neuroblastoma
(d) Tuberculosis
(e) Wilm’s tumour
(c) Neuroblastoma
Although some of the imaging features may overlap between these conditions, the history is highly suggestive of
neuroblastoma. Intractable diarrhoea is secondary to elevated levels of VIP.’· Hypertension suggests the presence
of increased catecholamine (VMAand HVA) production.
(GU) 34 An otherwise normal baby is found to have an impalpable right testis at birth. At 6 months he undergoes an US to confirm the presence of an undescended testis. What is the most likely location for such a testis?
(a) Inguinal canal
(b) Pubic tubercle
(c) Immediately inferior to the right kidney
(d) Contralateral hemiscrotum
(e) Perineum
(a) Inguinal canal
The inguinal canal is the commonest site for an undescended testis, although it can lie anywhere along its
embryological passage from kidney to scrotum. Ectopic testes can be found in the femoral canal, perineum,
contralateral hemiscrotum or prepubic space. Localisation is important as, if present, there is a greatly increased
risk of malignancy
36 A neonate is found to have a left kidney measuring 5.4 cm, right kidney 4.3 cm. A subsequent IVU demonstrates a complete duplication of the left kidney. The patient returns for follow-up IVU late in childhood. Which of the following signs would be unexpected?
(a) Concave upper border of the left kidney
(b) Increased number of calyces in the left kidney compared to the right
(c) Obstruction of the ectopic ureter
(d) Upper Pole (ectopic) ureter inserting more inferior and media II
(e) Filling defect inferiorly within the bladder
(b) Increased number of calyces in the left kidney compared to the right
Asymmetrical renal size may be the only US finding with a duplex system. The ‘Weigert-Meyer’ rule states that
the lower pole ureter inserts in the normal position, the upper pole ureter inserts inferiomedially, tends to be
stenotic and obstructed and is often associated with a ureterocoele. The lower ureter is prone to VUR. The renal
pelvis will have a concave upper border due to inferior displacement by the · obstructed upper pole, there is lateral
displacement of the kidney and ureters and there are decreased calyces in comparison to the normal side,
producing the ‘drooping lily’ sign.
38 A 20 week antenatal US shows multiple cysts within the left kidney. These vary in size and are randomly
arranged. The parenchyma is difficult to demonstrate and the left renal pelvis cannot be identified. No other
anomalies are identified. What is the most likely diagnosis?
(a) Autosomal dominant polycystic kidney disease
(b) Autosomal recessive polycystic kidney disease
(c) Meckel-Gruber syndrome
(d) Multicystic dysplastic kidney
(e) Multilocular cystic nephroma
(d) Multicystic dysplastic kidney
MCKD is normally unilateral (bilateral in 5-20%) and is thought to be caused by pelvi-infundibular atresia. US
shows multiple cysts of variable size that are randomly arranged and are separated by little or no parenchyma; a
renal pelvis cannot be seen. It is the 2nd most common cause of a neonatal abdominal mass (after hydronephrosis)
and is the most common renal cystic disease in infants. Meckel-Gruber syndrome produces enlarged polycystic
(2-10 mm cysts) kidneys in utero, and typically causes oligohydramnios and pulmonary hypoplasia. MLCN is
characterised by large(> 10 cm) cystic spaces, which will compress or displace the parenchyma.
41 Which of the following is not a risk factor for neonatal renal vein thrombosis?
(a) Birth trauma
(b) Dehydration
(c) Diabetic mother
(d) Right adrenal haemorrhage
(e) Sepsis
(d) Right adrenal haemorrhage
Neonatal causes include dehydration (vomiting, diarrhoea), sepsis, birth trauma and prematurity. Glycosuria in
infants of diabetic mothers is another cause, ~md there is an association with increased maternal age. Left adrenal
haemorrhage is a cause of RVT; the lt adrenal vein drains to left RV, the right adrenal drains directly to IVC.
(GU) 49 A 32 year old male is found to have a complete duplex ureteric system on the left side at IVU. Which of the following statements is incorrect?
(a) The upper moiety ureter is more prone to obstruction
(b) Enuresis is less likely in female patients
(c) The lower moiety ureter is more prone to reflux
(d) The upper moiety ureter inserts inferior and medial to the lower moiety ureter
(e) The drorooping lily’ Sign may be seen on his IVU
(b) Enuresis is less likely in female patients
In men the upper moiety ureter always inserts proximal to the external urethral sphincter, thus enuresis is less
likely. By way of contrast, in females the upper moiety ureter commonly inserts distal to the sphincter, making
enuresis more likely. Drooping lily sign refers to IVU obstructed upper moiety ureter displacing lower ur inf.
(GU) 51 A 2 year old presents with pain and abdominal swelling. US shows an 8 cm hyperechoic poorly defined mass in the retroperitoneum, crossing the midline, with areas of acoustics shadowing within it. CT demonstrates
the presence of retroperitoneal adenopathy and encasement of the IVC. What is the likely diagnosis?
(a) Wilm’s tumour
(b) Rhabdomyosarcoma
(c) Renal cell carcinoma
(d) Neuroblastoma
(e) Polycystic kidney disease
(d) Neuroblastoma
Neuroblastoma is the commonest solid abdominal mass in infants. Itis characterised by difficulty in defining it
separately from the kidney, the presence of coarse stippled calcification and the fact it crosses the midline. It may
arise from any site within the sympathetic neural chain.
(GU) 62 A male infant is found to have multiple bladder diverticulae along with other congenital abnormalities.
Which of the following conditions is least likely?
(a) William’s syndrome
(b) Menke’s kinky hair syndrome
(c) Eagl Barrett syndrome
(d) Diamond-Blackfan syndrome
(e) Meckel-Gruber syndrome
(e) Meckel-Gruber syndrome
Meckel-Gruber syndrome is a cause of multiple renal cortical cysts but is invariably fatal at birth due to a
combination of lung hypoplasia and renal failure.
(GU) 64 A 50 year old man undergoes an IVU for suspected uretericcolic. No abnormality is found other than a
right ureter which swings medially at the level of L3/L4. Which of the following is the likeliest diagnosis?
(a) Para-aortic lymphadenopathy
(b) Neurofibroma
(c) Retrocaval ureter
(d) Pelvic lipomatosis
(e) Aortic aneurysm
(c) Retrocaval ureter
Retrocaval ureter is due to a rare embryological failure of IVC formation with persistence of the right posterior
cardinal vein and failure of the right supracardinal system to develop. It may be asymptomatic or cause varying
degrees of proximal hydroureter.
- A neonate has an abdominal ultrasound following the finding of a palpable abdominal mass. The right kidney
is normal. The left kidney is replaced by multiple cysts of varying size and shape. There is no communication
between the cysts, which are separated by septae. Which of the following is the most likely diagnosis?
a. Multilocular cystic renal tumour
b. Autosomal recessive polycystic kidney disease
c. Autosomal dominant polycystic kidney disease
d. Multicystic dysplastic kidney
e. Medullary sponge kidney
- d. Multicystic dysplastic kidney
These features are highly suggestive of multicystic dysplastic kidney. The condition is invariably fatal if bilateral,
but often asymptomatic if unilateral. It is the second commonest cause of a palpable abdominal mass in the
neonate, second only to hydronephrosis. The key features are that no normal renal tissue is present, the cysts do
not communicate with each other and they are separated by septae.
- A three year old boy has an ultrasound and an abdominal CT scan following the discovery of a palpable
abdominal mass. No other symptoms are present. A large abdominal mass measuring 9 cm in maximum crosssectional dimensions is seen. Which one of the following features would favour a diagnosis of Wilms tumour
rather than neuroblastoma?
a. Calcification
b. Bone metastases
c. Lung metastases
d. Encasement of the major vessels
e. Displacement of the kidney
- c. Lung metastases
Lung metastases are rare in neuroblastoma and are seen in only 10% of cases, whereas they are seen in 85% of
metastatic Wilms tumours. Wilms tumours tend to displace rather than encase the vessels, and intrinsic mass
effect, rather than displacement of the kidney, is seen. Calcification is seen in 90%of neuroblastomas but only
10% ofWilms tumours. Wilms tumours are the most common abdominal neoplasmin children between one and
eight years of age. It typically presents with an asymptomatic abdominal mass but pain, haematuria and fever
may be found.