Paediatrics/Congenital

Question 1

What is a type I posterior urethral valve?

A. A fold of tissue running between the bladder neck and verumontanum
B. A membrane of tissue from the verumontanum to the membarnous urethra
C. A diaphragm of tissue with a central lumen at the level of the membranous urethra
D. A membrane of tissue from the proximal verumontanum dorsally to the bladder neck

Answer 1

B. A membrane of tissue from the verumontanum to the membarnous urethra

Question 2

What is most commonly affected by a ureteral ectopia in a duplicated system?

A. The ureters of both poles of the kidneys
B. The ureter of the upper pole of the kidney
C. The ureter of the lower pole of the kidney
D. There is no rule in this type of malformation

Answer 2

B. The ureter of the upper pole of the kidney

Question 3

When does urine production begin in the human fetus?

A. At 2 to 3 weeks
B. At 4 to 6 weeks
C. At 10 to 12 weeks
D. At 14 to 16 weeks

Answer 3

C. At 10 to 12 weeks

Question 4

What is the approximate incidence of hypospadias?

A. 1 to 125
B. 1 to 250
C. 1 to 500
D. 1 to 1000

Answer 4

B. 1 to 250

Question 5

The SIOP guidelines for the treatment of a Wilms’ tumour are chronologically as follows:

A. First radiation treatment, then surgery and then chemotherapy
B. First surgery, then chemotherapy and then radiation treatment
C. First chemotherapy, then surgery and then radiation treatment
D. First chemotherapy, then surgery and then chemotherapy again

Answer 5

D. First chemotherapy, then surgery and then chemotherapy again

Question 6

A 14-year-old wheelchair-bound girl with a myelo-meningocele has urinary and faecl incontinence. She is on clean intermittent catheterisation and anticholinergics. Bladder capacity is 150 mL, leak point pressure is 60 cm H2O, no reflux. What is the most appropriate management?

A. Bladder augmentation and cathererisable stoma and eppendicostomy (ACE)
B. Bladder augmentation
C. Bladder augmentation and bladder neck reconstruction and catheterisable stoma
D. Appendicostomy (ACE)

Answer 6

A. Bladder augmentation and cathererisable stoma and eppendicostomy (ACE)

Question 7

Which is the most likely diagnosis in a dehydrated neonate with cyanotic heart disease, a hight red blood count, hematuria and a palpable renal mass?

A. Wilms’ tumour
B. Polycystic kidney
C. Renal vein thrombisis
D. Congenital hydronephrosis

Answer 7

C. Renal vein thrombisis

Question 8

A newborn infant presents with episodes of vomiting, dehydration, cyanosis. He shows severe electrolyte and water imbalances. A small penis with chordee, proximal hypospadias and bilateral impalpable testicles are noted. The most probable diagnosis is:

A. Adrenogenital syndrome
B. Hypospadias with renal dysplasia
C. Hypospadias and polycystic kidneys
D. Obstructive uropathy associated with hypospadias

Answer 8

A. Adrenogenital syndrome

Question 9

Distal hypospadias in a boy is usually:

A. Associated with renal abnormalities
B. Treated at the age of 6-18months
C. Treated by multiple stage operations
D. Diagnosed before birth by ultrasonography

Answer 9

B. Treated at the age of 6-18months

Question 10

By which triad of findings is the prune-belly syndrome in boys characterized?

A. Reflux, hypspadias, undescended testicles
B. Vesical extrophy, undescended testicles, reflux
C. Vesical extrophy, reflux, hypoplasia of the abdominal wall
D. Reflux, undescended testicles, hypoplasia of the abdominal wall

Answer 10

D. Reflux, undescended testicles, hypoplasia of the abdominal wall

Question 11

When should the urinary tract be screened in a boy with hypospadias?

A. In every case
B. In case of an esophageal atresia
C. With unilateral undescended testis
D. In case of midshaft hypospadias

Answer 11

B. In case of an esophageal atresia

Question 12

What is needed for the development of the Wolffian duct deriviatives in the 46XY fetus?

A. Testosterone
B. 5-α-reductase
C. Dihydrotestosterone
D. Müllerian inhibiting factor

Answer 12

A. Testosterone

Question 13

Which condition has been reported to occur in association with posterior urethral valves?

A. Exomphalos
B. Hypospadiass
C. Cryptorchidism
D. Orthotopic ureterocele

Answer 13

C. Cryptorchidism

Question 14

In a young boy with acute scrotum which is more suggestive for testicular torsion?

A. Presence of hydrocele
B. Elevation of the scrotum may reduce complaints
C. Presence of fever
D. Loss of cremaster reflex

Answer 14

D. Loss of cremaster reflex

Question 15

Which is the most successful method of varicocele treatment in adolescents?

A. Antegrade sclerosation
B. Microsurgical inguinal ligation
C. Laparoscopic ligation
D. Lymphatic sparing suprainguinal ligation

Answer 15

B. Microsurgical inguinal ligation

Question 16

In an 8-month-old severely ill girl suffering from a proven first febrile urinary tract infection, what is the first advisable diagnostic modality?

A. Renal and bladder ultrasound
B. Voiding cystourethrography (VCUG)
C. Mercaptoacetytriglycin (MAG3) scan
D. Dimercaptosuccinic acid (DMSA) scan

Answer 16

A. Renal and bladder ultrasound

Question 17

When should intermittent catheterization and antimuscarinic treatment be started in children with neurogenic bladder and bowel emptying problems?

A. Soon after birth
B. In infancy
C. Before puberty
D. If upper tract deterioration is present

Answer 17

A. Soon after birth

Question 18

According to the EAU guidelines congenital penile curvature is:

A. Commonly associated with urethra malformation
B. Most commonly dorsally deviated
C. Always ventrally deviated
D. Sometimes laterally deviated

Answer 18

D. Sometimes laterally deviated

Question 19

What is the incidence of posterior urethral valves?

A. 1 in 3 000
B. 1 in 7 000
C. 1 in 10 000
D. 1 in 50 000

Answer 19

B. 1 in 7 000

Question 20

Which is an indication to recommend adolescent varicocelectomy?

A. Grade 2 right-sided varicocele
B. Varicocele associated with hydrocele
C. Ipsilateral testicular hypoplasia
D. Grade 3 varicocele with normal sperm parameters

Answer 20

C. Ipsilateral testicular hypoplasia

Question 21

Which of the following DSD conditions has the highest risk for the development of a malignant germ cell tumour?

A. 45X0 Turners syndrome
B. 47XXY Klinefelters syndrome
C. 46XY complete gonadal dysgenesis
D. 46XX congenital adrenal hyperplasia

Answer 21

C. 46XY complete gonadal dysgenesis

Question 22

Vesico-amniotic shunting during pregnancy:

A. Has no effect on lung function
B. Is associated with significant morbidity
C. Is offered routinely in cases of posterior urethral valves
D. Has improved long-term outcomes in boys with posterior urethral valves

Answer 22

B. Is associated with significant morbidity

Question 23

During pregnancy an US at 20 weeks gestation shows normal amniotic fluid and a significant hydronephrosis on the left side. At 32 seeks the US shows the same hydronephrosis:

A. The parents have to be counseled and offered drainage of the left kidney
B. If the pulmonary situation allows, early delivery should be promoted around week 36-38
C. No specific measures are necessary and the baby should be delivered at term
D. After delivery an US study of the kidneys should be done on day 1 or 2

Answer 23

C. No specific measures are necessary and the baby should be delivered at term