Paediatric Urology

Question 1

From where do the urinary and genital systems develop

Answer 1

Intermediate mesoderm (common mesodermal ridge running along the posterior wall of the abdominal cavity)

Question 2

What is the origin of the definitive kidney

Answer 2

Metanephros

Question 3

Where is the ureter/renal pelvis derived

Answer 3

Mesonephric duct

Question 4

Where is the collecting system of the kidney derived

Answer 4

Ureteric bud from the mesonephric duct

Question 5

Where is the excretory system (e.g. glomeruli, tubules) of the kidney derived

Answer 5

Metanephric blastema

Question 6

From where are the bladder (apart from the trigone) and urethra derived

Answer 6

Urogenital sinus (anterior part of the cloaca)

Question 7

Where is the trigone of the bladder derived

Answer 7

Mesoderm

Question 8

What sex is the default

Answer 8

Female

Question 9

When do the gonads acquire male/female morphology

Answer 9

Week 7

Question 10

Where in the testes is testosterone produced

Answer 10

Leydig cells

Question 11

What happens to the medullary cords at puberty

Answer 11

Acquire a lumen to form the seminiferous tubules

Question 12

What structure guides the descent of the testis

Answer 12

Gubernaculum

Question 13

When do the testes reach A) the inguinal canal, and B) the external inguinal ring

Answer 13

A) 7 months

B) 8 months

Question 14

When does the Processus Vaginalis close and what are the consequences of this not happening

Answer 14

• 1 year

- Hydrocele or congenital inguinal hernia

Question 15

What gives rise to the ovarian ligament

Answer 15

Gubernaculum

Question 16

What gives rise to the round ligament

Answer 16

Uterovaginal canal (Gubernaculum)

Question 17

What does the mesonephric duct contribute to the male genitalia

Answer 17

• Epididymis
• Vas deferens
• Seminal vesicles
• Ejaculatory ducts

Question 18

From where are the Fallopian tubes derived

Answer 18

Paramesonephric ducts

Question 19

Describe the development of the male external genitalia

Answer 19

• Genital tubercle becomes the phallus
• Urethral folds (of cloaca) become the penile urethra
• Genital swellings form the scrotum

Question 20

What is the result of failure of fusion of the urethral folds

Answer 20

Hypospadias

Question 21

Describe the development of the female external genitalia

Answer 21

• Genital tubercle forms the clitoris
• Urethral folds do not fuse and become the labia minora
• Genital swellings become labia majora

Question 22

What is Potter Syndrome

Answer 22

• Bilateral renal agenesis
• Characteristic features of oligohydramnios and characteristic facial appearance
• Pulmonary hypoplasia leads to stillbirth

Question 23

In whom are lower pole renal vessels common

Answer 23

Children with pelviureteric obstruction

Question 24

What is the inheritance pattern of adult polycystic kidney disease

Answer 24

Autosomal dominant

Question 25

What is the inheritance patterns of infant polycystic kidneys

Answer 25

Recessive

Question 26

What are the associations of APKD

Answer 26

- Cystic change in other organs e.g. liver, spleen, pancreas - Berry Aneurysms of the circle of Willis - Mitral valve prolapse

Question 27

What urological conditions is tuberous sclerosis associated with

Answer 27

- Angiomyolipomata | - Polycystic kidneys

Question 28

Describe horseshoe kidney

Answer 28

- Fusion of the inferior pole occurs before the kidneys have rotated along the long axis - The IMA prevents ascent - Ureter enters the bladder ectopically

Question 29

Describe uretrocele

Answer 29

- Cystic dilatation of the intramural part of the ureter | - Commonly occurs in association with the upper-pole ureter of a duplex system

Question 30

Describe a retrocaval ureter

Answer 30

- Abnormality in the development of the posterior cardinal vein - Ureter lies BEHIND vena cava - Compression of the ureter can occur between IVC and vertebrae

Question 31

What is the investigation of choice for assessing PUJ obstruction

Answer 31

MAG-3 scan will show delayed drainage of the kidneys

Question 32

When is surgery indicated in PUJ obstruction

Answer 32

- Symptomatic - Increasing hydronephrosis on serial USS - Declining renal function

Question 33

When would nephrectomy be considered in PUJ obstruction

Answer 33

If the kidney is functioning poorly (<15% differential function)

Question 34

How is PUJ obstruction treated

Answer 34

Dismembered retroperitoneal (Anderson-Hynes) pyeloplasty

Question 35

Define vesicoureteric reflux

Answer 35

Abnormal backflow of urine from the bladder into the ureter and kidney

Question 36

Describe the pathophysiology of VUR

Answer 36

- Shortened intramural course of ureter due to lateral displacement - Diminished function of VU junction

Question 37

Outline the 5 grades of VUR

Answer 37

``` 1 = reflux into ureter only, no dilatation 2 = reflux into renal pelvis on micturition, no dilatation 3 = mild/moderate dilatation of the ureter, pelvis, calyces 4 = dilatation of the renal pelvis and calyces with moderate ureteral tortuosity 5 = gross dilation with ureteral tortuosity ```

Question 38

What does VUR predispose to

Answer 38

Recurrent UTI

Question 39

How is VUR managed

Answer 39

- Prophylactic antibiotics | - Surgery if progressive renal damage or breakthrough UTI

Question 40

What is the most common cause of bladder outlflow obstruction in boys

Answer 40

Posterior urethral valves

Question 41

Describe hypospadias

Answer 41

Urethral meatus opens onto the ventral surface of the penis (75% distally located)

Question 42

What is associated with hypospadias

Answer 42

- Undescended testis - Inguinal hernia - Bifid scrotum - Chordae and renal and ureteric abnormalities

Question 43

When is surgery performed for hypospadias

Answer 43

Between 9 and 18 months

Question 44

How is hypospadias treated

Answer 44

- Urethroplasty | - Penile reconstruction

Question 45

Define a retractile testis

Answer 45

Sits high in the scrotum or inguinal canal but can be milked into the scrotum and stays in the scrotum with minimal tension

Question 46

Define an undescended testis

Answer 46

During development the testicle has been arrested somewhere along the course of normal descent and does not reach the bottom of the scrotum by 3 months of age

Question 47

Define a maldescended/ectopic testis

Answer 47

Situated somewhere other than the usual course of descent

Question 48

What is the increase in risk of malignancy associated with Cryptochidism

Answer 48

10x

Question 49

When should orchidopexy be performed for undescended testis

Answer 49

Before 1 year (6-18 months)

Question 50

What structural abnormality are undescended testes typically associated with

Answer 50

Patent processus vaginalis

Question 51

What is indicated if the testis are impalpable

Answer 51

Laparoscopy

Question 52

Inheritance pattern of congenital adrenal hyperplasia

Answer 52

Autosomal recessive

Question 53

Features of Klinefelter syndrome (47 XXY)

Answer 53

- Increased height - Mild learning disability - Gynaecomastia - Infertility

Question 54

Describe testicular feminisation

Answer 54

- Chromosomally XY | - BUT present as females due to complete androgen insensitivity of the genitalia

Question 55

What gene codes for Wilm's tumour

Answer 55

WT1 gene

Question 56

How are children <6 months of age investigated following UTI

Answer 56

USS 6 weeks post-UTI

Question 57

How are children aged 6 months to 3 years investigated following atypical UTI

Answer 57

- USS | - DMSA scan