PEDIATRIC Section 9: Congenital GU (Ureter/Urethra)

Question 1

his is a “wastebasket” term for an enlarged ureter which is intrinsic to the ureter (NOT the resuh of a distal obstruction)

Answer 1

Congenital (primary) MEGAureter

Question 2

Give the causes of Congenital (Primary) Megaureter

Answer 2

(1) distal adynamic segment (analogous to achalasia, or colonic Hirschsprungs),
(2) reflux at the UVJ,
(3) it just wants to be big (totally idiopathic).

Question 3

What type of Congenital Megaureter causes hydronephrosis?

Answer 3

Distal adynamic type

Question 4

Retrocaval Ureter (circumcaval)- This is actually a problem with the development of the IVC, which grows in a
manner that pins the ureter.

Answer 4

Retrocaval Ureter (circumcaval)-

Question 5

Retrocaval Ureter (circumcaval) in IVP

Answer 5

“Reverse J” or “fishhook”

Question 6

When you say Duplicated collecting system, you say

Answer 6

“Weigert- Meyer Rule”

Question 7

What is the “Weigert- Meyer Rule”?

Answer 7

the upper pole inserts INFERIOR and MEDIALLY.

Question 8

The upper pole in a duplicated collecting system is prone to what?

The lower pole is prone to?

Answer 8

Upper pole: ureterocele formation and obstruction

Lower pole: Reflux

Question 9

In girls, a duplicated system can lead to

Answer 9

Incontincee (Ureter may instert BELOW the sphincter - sometimes VAGINA)

Question 10

A cystic dilation of the intravesicular ureter, secondary to obstruction at the ureteral orifice.

Answer 10

Ureterocele

Question 11

Ureterocele in IVP will show?

Answer 11

“Cobra head” sign with contrast surrounded by a lucent rim, protruding from the contrast filled bladder.

Question 12

This is associated with a duplicated system (specifically the upper pole).

Answer 12

Ureterocele

Question 13

The ureter inserts distal to the external sphincter in the vestibule.

Answer 13

Ectopic Ureter

Question 14

Ectopic Ureter is common in what patients?

Answer 14

Female

+ incontinence

(not associated with incontinence in men)

Question 15

Where are ureteroceles best demonstrated

Answer 15

Early filling of the VCUG

Question 16

This is a fold in the posterior urethra that leads to outflow obstruction and eventual renal failure (if it’s not fixed).

Answer 16

Posterior Urethral Valves

Question 17

It is the most common cause of urethral obstruction in male infants.

Answer 17

Posterior Urethral Valves

Question 18

What is the fold in the posterior urethra?

Answer 18

The fold is a Wolfian Duct tissue remnant

Question 19

Diagnosis?

Describe

Answer 19

Posterior urethrel valves

an abrupt caliber change between the dilated posterior urethra and normal caliber anterior urethra.

Question 20

Diagnsosis.

Describe

Answer 20

Posterior Urethral Valves:

The MRI would have to show hydro in the kidney and a “key-hole” bladder appearance.

Question 21

Triad of Posterior Urethral Valves:

Answer 21

• Hydronephrosis
• Bladder Dilation
• Oligiohydramnios

Question 22

Buzzword of posterior urethral valves

Answer 22

Peri renal fluid collection. - non specific

Question 23

When you see hydronephrosis on routine prenatal ultrasound - What would be the next step if its persistent once born?

Answer 23

Repeat ultrasound

Question 24

Gold standard diagnostic procedure for hydronephrosis in the newborn

Answer 24

VCUG

Question 25

Non-Obstructive Causes of Hydro in Baby Boys

Answer 25

-Vesicoureteral Reflux (VUR) -Primary Megaureter -Prune Belly = Zebra

Question 26

Obstructive Causes of Hydro in Baby Boys

Answer 26

-PUV (posterior urethral valves) -UPJ Obstruction - Ureteral Ectopia

Question 27

Horizontal angle of the urethra =

Answer 27

reflux can develop (should be oblique)

Question 28

What is the most common cause of a palpable renal “mass" in childhood?

Answer 28

Hydronephrosis

Question 29

Describe the grading system for VUR 1-5

Answer 29

1. One is reflux halfway up the ureter, 2. Two is reflux into a non-dilated collecting system, (calyces still pointy), 3. Three you have dilation of the collecting system, and calyces get blunted 4. Four the system gets mildly tortuous, 5. Five the system is very tortuous.

Question 30

Chronic reflux can lead to? and eventually result in?

Answer 30

Chronic reflux can lead to scarring. This scarring can result in hypertension and/or chronic renal failure.

Question 31

Describe "Hutch Diverticula"

Answer 31

Question 32

Persistent canalization of the umbilical attachment to the bladder

Answer 32

Urachus

Question 33

the urachus atrophies into what?

Answer 33

This usually atrophies into the umbilical ligament.

Question 34

Most common complication of urachal remnant

Answer 34

INFECTION

Question 35

Urachal anomalies are twice as common in?

Answer 35

Boys

Question 36

When guys with with urachus gets cancer, it's this type

Answer 36

AdenoCA (90%)

Question 37

"Midlune bladder structure"

Answer 37

Urachus

Question 38

Describe thes urachal spectrum

Answer 38

Question 39

Diagnosis?

Answer 39

Urachal cancer

Question 40

This is a herniation of the urinary bladder through a hole in the anterior infra-umbilical abdominal wall.

Answer 40

Bladder Exstrophy

Question 41

The extruded bladder has an increased incidence of what malignancy?

Answer 41

AdenoCA

Question 42

Diagnosis?

Answer 42

“Manta Ray Sign” - with unfused pubic bones. This looks like a monster wide pubic symphysis on an AP pelvic radiograph.

Question 43

GU and GI both drain into a common opening (like a bird). This only happens in females.

Answer 43

Cloacal Malformation

Question 44

Causes of Neurgenic bladder?

Answer 44

spinal dysraphism (tethered cord, sacral agenesis, and all the other fucked up spine stuff).