Children with Renal Abnormalities

Question 1

The nature of disease:

• Lesions may be:
  
  • ​C..
  • In..
  • Tu..
  • In..
  • T…
  • Others (e.g. autoimmune)

Answer 1

• Lesions may be:
  
  • ​Congenital
  • Infective
  • Tumours
  • Inflammatory
  • Traumatic
  • Others (e.g. autoimmune)

Question 2

Urology Development - Where it all starts

• The … bud (also known as the metanephrogenic diverticulum) is a protrusion of the … duct that appears during the embryological development of urogenital organs.
• It will eventually form the urinary collecting system (i.e. collecting tubes, calyces, renal pelvis, ureter) of the kidney.
• This occurs after the ureteric bud connects with the metanephric ….

Answer 2

• The ureteric bud (also known as the metanephrogenic diverticulum) is a protrusion of the mesonephric duct that appears during the embryological development of urogenital organs.
• It will eventually form the urinary collecting system (i.e. collecting tubes, calyces, renal pelvis, ureter) of the kidney.
• This occurs after the ureteric bud connects with the metanephric blastema.

Question 3

Congenital Problems - Urology

• … Absent kidney
  
  • One good kidney sufficient for a normal life
  • You can’t be a … …
• Mayer-Rokitansky syndrome:
  
  • Abnormalities of the vagina (…), uterus, fallopian tubes associated most commonly with an … … (unilateral)

Answer 3

• Unilateral Absent kidney –
  
  • One good kidney sufficient for a normal life
  • You can’t be a kidney donor !
• Mayer-Rokitansky syndrome:
  
  • Abnormalities of the vagina (agenesis), uterus, fallopian tubes associated most commonly with an absent kidney (unilateral)

Question 4

What is Mayer-Rokitansky syndrome?

Answer 4

• Abnormalities of the vagina (agenesis), uterus, fallopian tubes associated most commonly with an absent kidney (unilateral)

Question 5

What is a horseshoe kidney?

Answer 5

What is a horseshoe kidney?

Question 6

Abnormalities of the kidney can predisose you to get other complications - what is the most common?

Answer 6

UTI

Question 7

What is Vesicoureteral reflux (VUR)?

Answer 7

With vesicoureteral reflux, urine flows backward from the bladder, up the ureter to the kidney.

Question 8

What is the commonest ‘surgical’ cause of UTI?

Answer 8

Vesicoureteral reflux (VUR) (when the flow of urine goes the wrong way)

Question 9

Vesicoureteral reflux (VUR) may be diagnosed after … or … diagnosis

Answer 9

after a UTI, or may be antenatal diagnosis

Question 10

When does Secondary Reflux occur? (Secondary vesicoureteral reflux)

Answer 10

• Secondary VUR occurs when an obstruction in the bladder or urethra causes urine to flow backward into the kidneys.
• Secondary VUR can occur at any age and can be caused by surgery, injury, a pattern of emptying the bladder that’s not normal, or a past infection that puts pressure on the bladder.

Question 11

Anatomy of Vesicoureteral Reflux

• Vesicoureteric reflux is, in the majority of cases, the result of a primary maturation abnormality of the vesicoureteral junction resulting in a short distal … … tunnel.
• If this tunnel is very … - end up with the uteric opening is quite lateral - predisposes to reflux

Answer 11

• Vesicoureteric reflux is, in the majority of cases, the result of a primary maturation abnormality of the vesicoureteral junction resulting in a short distal ureteric submucosal tunnel.
• Normally, ureter approaches bladder - submucosal tunnel
• If this tunnel is very short - end up with the uteric opening is quite lateral - predisposes to reflux

Question 12

Grades of Vesicoureteric Reflux

• How many are there?

Answer 12

• grade 1: reflux limited to the ureter
• grade 2: reflux up to the renal pelvis
• grade 3: mild dilatation of ureter and pelvicalyceal system
• grade 4: tortuous ureter with moderate dilatation
  
  • blunting of fornices but preserved papillary impressions
• grade 5: tortuous ureter with severe dilatation of ureter and pelvicalyceal system
  
  • loss of fornices and papillary impressions 2

Question 13

What is Bilateral Vesicoureteric Reflux?

Answer 13

Vesicoureteral reflux (VUR) is a condition in which urine flows backward from the bladder to one or both ureters and sometimes to the kidneys (bilateral = both kidneys)

Question 14

Damage to kidneys after Vesicoureteric Reflux

Answer 14

• Left kidney - chronic scarring as a result of pyelonephritis (after reflux)
• Right kidney is normal

Question 15

Management of Vesicoureteric Reflux

• Is it operative?
• What age does refluxing usually stop?
• How do we stop the child getting infections?

Answer 15

• Is it operative? - Mostly non-operative
• What age does refluxing usually stop? - about age 5
• How do we stop the child getting infections? - Small dose of Antibiotics as a prophylactic measure

Question 16

Vesicoureteric reflux (VUR) can be corrected endoscopically by a procedure known as …

Answer 16

• STING
  
  • Submucosal injection - was tefflon - now Deflux
  • Introduced - submucosal needle - where the ureter and bladder meet

Question 17

What is Hydronephrosis?

Answer 17

Hydronephrosis (plural: hydronephroses) is defined as dilatation of the urinary collecting system of the kidney (the calyces, the infundibula, and the pelvis)

Question 18

Is infection common in Hydronephrosis?

Answer 18

No

Question 19

Hydronephrosis may be diagnosed … and can be confirmed using a … scan

Answer 19

Hydronephrosis may be diagnosed antenatally and can be confirmed using a MAG3 scan

Question 20

Hydronephrosis + UTI may result in …

Answer 20

Pyonephrosis (a term given to an infection of the kidney with pus in the upper collecting system which can progress to obstruction.)

Question 21

Acute pyelonephritis is a sudden and severe …

Answer 21

• kidney infection
  
  • It causes the kidneys to swell and may permanently damage them.

Question 22

What is a Mega-Ureter?

Answer 22

Megaureter (dilated ureter) is an abnormality of one or both of the ureters of a child.

Question 23

Mega-ureter has to be differentiated from …

Answer 23

Vesicoureteric reflux

Question 24

Mega-ureter

• Not as common as …
• May be associated with other problems like … system
• Associated Urteroceles
• Rarely simple …
• Has to be differentiated from …reflux

Answer 24

• Not as common as PUJ (Pelviureteric junction obstruction)
• May be associated with other problems like duplex system
• Associated Urteroceles
• Rarely simple stenosis
• Has to be differentiated from V-U-reflux

Question 25

MAG3 scan showing ...

Answer 25

obstruction

Question 26

What is PUV?

Answer 26

Posterior urethral valves (PUV) is a condition found only in boys that affects the urethra (the tube which runs from the bladder to the outside)

Question 27

_PUV (Posterior urethral valves)_ * What gender? * Presents in the ... period (usually) * How ill? * Diagnosed and managed when? * Outcome depends on initial ... damage

Answer 27

* **Boys** only * Presents in the **neonatal** period (usually) * Infant may be **very ill** * **Antenatally** diagnosed and some managed **antenatally** * Outcome depends on initial **renal** damage

Question 28

Some boys are born with a ... penis

Answer 28

* Buried penis * Can be corrected surgically

Question 29

What is Hypospadias?

Answer 29

Hypospadias is a condition in which the opening of the urethra is on the underside of the penis instead of at the tip

Question 30

A child with Cryptorchidism and Hypospadias may actually be a ...

Answer 30

female with congenital adrenal hyperplasia - important to check actual gender at birth to determine sex of child (USS of pelvis and checking for chromosomes)

Question 31

What is a Ureterocele?

Answer 31

A ureterocele is a cystic out-pouching of the distal ureter into the urinary bladder (congenital)

Question 32

_Testicular descent_​ * Usually, the testes descend from abdomen through inguinal canal into scrotum * As it does this - there are outpouchings - becomes processus vaginalis and tunica vaginalis testes * If this outpouching doesnt obliterate itself and remains patent, it can cause inguinal ... and ...

Answer 32

* Usually, the testes descend from abdomen through inguinal canal into scrotum * As it does this - there are outpouchings - becomes processus vaginalis and tunica vaginalis testes * If this outpouching doesnt obliterate itself and remains patent, it can cause inguinal **hernia** and **hydrocele**

Question 33

What is an Undescended Testis?

Answer 33

Is a testis that cannot be manipulated to the bottom of the scrotum, without undue tension of the spermatic cord

Question 34

_Incidence of Undescended Testis_ * ... % newborn boys * Preterm infants incidence ...% or more * 2/3 descend spontaneously * full term \< 6 weeks * preterm \< 3 months * ...% at the age of one * ... predisposition * Bilateral 10-25% * Right=left 55%=45%

Answer 34

* **3.4** % newborn boys * Preterm infants incidence **20**% or more * 2/3 descend spontaneously * full term \< 6 weeks * preterm \< 3 months * **1**% at the age of one * **Familial** predisposition * Bilateral 10-25% * Right=left 55%=45%

Question 35

_Undescended Testis_ * Impalpable testis: a..., intra-... or intra-... * Undescended testis: in the ... * Ectopic testis: abdominal wall, thigh, base of penis or contra-lateral side * ... testis - used when testis has normally descended at birth but over the years gets pulled back - quite rare

Answer 35

* Impalpable testis: **absent, intra-abdominal or intra-canicular** * Undescended testis: in the **groin** * Ectopic testis: abdominal wall, thigh, base of penis or contra-lateral side * **Ascending** testis - used when testis has normally descended at birth but over the years gets pulled back - quite rare