UROLOGY - BLADDER AND URETHRA CONGENITAL DISORDERS

Question 1

What is posterior urethral valves?

Answer 1

A Congenital disorder in boys where posterior urethra is obstructed by membranous folds.

Question 2

What are the possible manifestations of posterior urethral valves?

Answer 2

Bilateral Hydronephrosis (increase in intravesical pressure causes bladder wall hypertrophy and collagen deposition which makes it less compliant so urine backs up into kidneys) = kidney problems -> CKD or ESRD
Oligohydramnios can cause lung hypoplasia and potter syndrome
Increased risk of reflux of urine if dysfunction of uterovesical junction
Urine stasis can cause increase UTI risk

Question 3

How is posterior urethral valves diagnosed?

Answer 3

Prenatal ultrasound
Or post nasally with voiding cystourethrogram which shows a dilated and elongated posterior urethra

Question 4

What causes posterior urethral valves?

Answer 4

disruption of normal urethral development in weeks 9-14 - abnormal integration of wolffian duct which results in large plicae colliculi that fuse anteriorly = less urine can leave

Question 5

How are posterior urethral valves treated?

Answer 5

Surgery and ablation

Question 6

What is hypospadias?

Answer 6

an abnormal opening at bottom of urethra

Question 7

What is epispadias?

Answer 7

an abnormal opening on the top of the urethra.

Question 8

Who is hypospadias and epispadias more common in?

Answer 8

Boys
Hypospadias is associated with chordee, inguinal hernias and cryptochoridism
Epispadias is associated with bladder extrophy and bifid clitoris in females

Question 9

Outline the pathology of hypospadias?

Answer 9

in a boy the urethral folds along the penile urethra don’t meet up and close properly which causes the opening to be at the bottom of the penile shaft.
In girls the urethra opens into the vaginal wall

Question 10

Outline the pathology of epispadias?

Answer 10

In boys the problem starts in the 6th week of gestation and the genital tubercle grows in the posterior direction towards the rectal area. This results in an opening on the upper surface.
In girls the urethra develops too far anteriorly

Question 11

What are the 3 types of hypo/epispadias?

Answer 11

Glanular - urethra opens near the head of the penis
Midshaft
Penoscrotal - urethra opens near the scrotum

Question 12

Whats the cause of hypo/epispadias?

Answer 12

not fully understood but seems to be related to exposure to abnormal levels of androgens and oestrogens.

Question 13

What are the symptoms of hypo/epispadias?

Answer 13

In boys it can cause difficult with targeting and incontinence
In females it often occurs later in life and can present with frequent, painful urination and recurrent UTIs.
Later on it can present with sexual dysfunction, infertility and psychosocial problems

Question 14

How is hypo/epispadias treated?

Answer 14

Surgery to reconstruct the urethra within the first 2 years of life
Hormone therapy can be used if additional problem e.g. boys with micro penis
Shouldn’t have circumcision as foreskin may be useful for reconstruction

Question 15

What is vesicoureteral reflux?

Answer 15

When urine moves back up from the bladder into the kidneys

Question 16

What is primary vesicoureteral reflux?

Answer 16

When a child is born with a defect of the uterovesical junction

Question 17

What is secondary vesicoureteral reflux?

Answer 17

when there is an obstruction at some point in the urinary tract
- recurrent UTIs causing ureter inflammation
- posterior urethral valve disorder
- flaccid neurogenic bladder

Question 18

Outline the staging for vesicoureteral reflux?

Answer 18

• Grade 1 - urine backs up into ureters
• Grade 2 - urine fills entire ureter and renal pelvis
• Grade 3 - urine begins to stretch ureters and renal pelvis
• Grade 4 - ureters swell so much that it curves. Renal pelvis and calyxes become moderately swollen and distorted
• Grade 5 - urine fills ureters, pelvis and calyxes - this amount of pressure can lead to kidney failure

Question 19

What are the symptoms of vesicoureteral reflux?

Answer 19

In mild cases there are no symptoms
In severe cases the urinary stasis can cause pyelonephritis, renal fibrosis. This damage can reduce blood flow to the kidneys - they can respond by raising bp which can cause hypertension

Question 20

How is vesicoureteral reflux diagnosed?

Answer 20

Abdominal ultrasound, voiding cystourethrogram, radionuclide cystogram

Question 21

How is vesicoureteral reflux treated?

Answer 21

primary can improve over time on its own. Some individuals may need surgery to remove a blockage or repair the valve

Question 22

What is bladder exstrophy?

Answer 22

Congenital abnormality that results in a bladder that protrudes out of the abdomen and into the outside environment.

Question 23

What are the implications of bladder exstrophy?

Answer 23

This prevents the normal development of the abdominal wall and the fusion of the pelvis - this leaves a wide split in the symphysis pubis.
Most cases involve epispadias also.
It also causes a more anteriorly located anus, the anterior part of penis is shorter and the vagina is often wider, shorter and more vertically orinetated.

Question 24

Whats the proposed pathology of bladder exstrophy?

Answer 24

Theory is that the mesenchymal cells fail to migrate from the endoderm of the cloaca to the anterior abdominal wall. Without mesenchymal support, the cloacal membrane becomes unstable amd opens up to the outside prematurely. This creates a hole in the lower abdominal wall which exposes the bladder to the outside.

Question 25

What are the complications of bladder exstrophy?

Answer 25

incontinence, increased risk of UTI

Question 26

How is bladder exstrophy diagnosed?

Answer 26

It’s mostly clinically obvious at birth but can be diagnosed on a prenatal ultrasound

Question 27

How is bladder exstrophy managed?

Answer 27

Surgical closure in first few weeks of life