PEDIATRIC Section 9: Congenital GU (Ureter/Urethra) Flashcards
his is a “wastebasket” term for an enlarged ureter which is intrinsic to the ureter (NOT the resuh of a distal obstruction)
Congenital (primary) MEGAureter
Give the causes of Congenital (Primary) Megaureter
(1) distal adynamic segment (analogous to achalasia, or colonic Hirschsprungs),
(2) reflux at the UVJ,
(3) it just wants to be big (totally idiopathic).
What type of Congenital Megaureter causes hydronephrosis?
Distal adynamic type
Retrocaval Ureter (circumcaval)- This is actually a problem with the development of the IVC, which grows in a
manner that pins the ureter.
Retrocaval Ureter (circumcaval)-
Retrocaval Ureter (circumcaval) in IVP
“Reverse J” or “fishhook”
When you say Duplicated collecting system, you say
“Weigert- Meyer Rule”
What is the “Weigert- Meyer Rule”?
the upper pole inserts INFERIOR and MEDIALLY.
The upper pole in a duplicated collecting system is prone to what?
The lower pole is prone to?
Upper pole: ureterocele formation and obstruction
Lower pole: Reflux
In girls, a duplicated system can lead to
Incontincee (Ureter may instert BELOW the sphincter - sometimes VAGINA)
A cystic dilation of the intravesicular ureter, secondary to obstruction at the ureteral orifice.
Ureterocele
Ureterocele in IVP will show?
“Cobra head” sign with contrast surrounded by a lucent rim, protruding from the contrast filled bladder.
This is associated with a duplicated system (specifically the upper pole).
Ureterocele
The ureter inserts distal to the external sphincter in the vestibule.
Ectopic Ureter
Ectopic Ureter is common in what patients?
Female
+ incontinence
(not associated with incontinence in men)
Where are ureteroceles best demonstrated
Early filling of the VCUG
This is a fold in the posterior urethra that leads to outflow obstruction and eventual renal failure (if it’s not fixed).
Posterior Urethral Valves
It is the most common cause of urethral obstruction in male infants.
Posterior Urethral Valves
What is the fold in the posterior urethra?
The fold is a Wolfian Duct tissue remnant
Diagnosis?
Describe
Posterior urethrel valves
an abrupt caliber change between the dilated posterior urethra and normal caliber anterior urethra.
Diagnsosis.
Describe
Posterior Urethral Valves:
The MRI would have to show hydro in the kidney and a “key-hole” bladder appearance.
Triad of Posterior Urethral Valves:
- Hydronephrosis
- Bladder Dilation
- Oligiohydramnios
Buzzword of posterior urethral valves
Peri renal fluid collection. - non specific
When you see hydronephrosis on routine prenatal ultrasound - What would be the next step if its persistent once born?
Repeat ultrasound
Gold standard diagnostic procedure for hydronephrosis in the newborn
VCUG
Non-Obstructive Causes of Hydro in Baby Boys
-Vesicoureteral Reflux (VUR)
-Primary Megaureter
-Prune Belly = Zebra
Obstructive Causes of Hydro in Baby Boys
-PUV (posterior urethral valves)
-UPJ Obstruction
- Ureteral Ectopia
Horizontal angle of the urethra =
reflux can develop (should be oblique)
What is the most common cause of a palpable renal “mass” in childhood?
Hydronephrosis
Describe the grading system for VUR 1-5
- One is reflux halfway up the ureter,
- Two is reflux into a non-dilated collecting system, (calyces still pointy),
- Three you have dilation of the collecting system, and calyces get blunted 4.
Four the system gets mildly tortuous, - Five the system is very tortuous.
Chronic reflux can lead to? and eventually result in?
Chronic reflux can lead to scarring. This scarring can result in hypertension and/or chronic renal failure.
Describe “Hutch Diverticula”
Persistent canalization of the umbilical attachment to the bladder
Urachus
the urachus atrophies into what?
This usually atrophies into the umbilical ligament.
Most common complication of urachal remnant
INFECTION
Urachal anomalies are twice as common in?
Boys
When guys with with urachus gets cancer, it’s this type
AdenoCA (90%)
“Midlune bladder structure”
Urachus
Describe thes urachal spectrum
Diagnosis?
Urachal cancer
This is a herniation of the urinary bladder through a hole in the anterior infra-umbilical abdominal wall.
Bladder Exstrophy
The extruded bladder has an increased incidence of what malignancy?
AdenoCA
Diagnosis?
“Manta Ray Sign” - with unfused pubic bones.
This looks like a monster wide pubic symphysis on an
AP pelvic radiograph.
GU and GI both drain into a common opening (like a bird). This only happens in females.
Cloacal Malformation
Causes of Neurgenic bladder?
spinal dysraphism (tethered cord, sacral agenesis, and all the other fucked up spine stuff).
