PEDIATRIC Section 9: Congenital GU

Question 1

Two flavors of Renal Agenesis?

Answer 1

Both are absent
One is absent

Question 2

Renal agenesis is associated with Potter sequence if its?

Answer 2

bilateral

Question 3

One kidney absent is associated with what conditions?

Answer 3

Reproductive

Question 4

How many % of women with unilateral renal agenesis have associated genital anomalies. What are they?

Answer 4

70% of women with unilateral renal agenesis have associated genital anomalies

Usually - unicornuate uterus or a rudimentary horn)

Question 5

_% of men with unilateral renal agenesis have these genital anomalies:

Answer 5

20% of men are missing the epididymis, and vas deferens on the same side they are missing the kidney + Seminal vesicle cyst on that side.

Question 6

Potter Sequence

Answer 6

Insult = maybe ACE inhibitors = kidneys don’t form = no piss = can’t develop lungs (pulmonary hypoplasia

Question 7

Lying Down Adrenal is also called?

Describe

Answer 7

“Pancake adrenal sign”

Question 8

Diagnosis

Answer 8

Lying Down Adrenal

elongated appearance of the adrenal not normally molded by the adjacent kidney. It can be used to differentiate surgical absent vs congenitally absent.

Question 9

This is the most common fusion anomaly

Answer 9

Horseshoe Kidney

Question 10

Horseshoe Kidney basically means

Answer 10

he kidney gets hung up on the IMA

Question 11

What are the complications of horshoe kidney?

Answer 11

1. Complicationsfrom Position - Easy to get smashed against vertebral body - kid shouldn’t play football or wrestle.
2. Complicationsfrom Drainage Problems: Stones, Infection, and Increased risk of Cancer (from chronic inflammation) - big ones are Wilms, TCC, and the Zebra Renal Carcinoid.

Question 12

Syndrome associated with Horseshoe kidney

Answer 12

Turner’s Syndrome

Question 13

One kidney comes across the midline and fuses with the other.

Answer 13

Crossed Fused Renal Ectopia

Question 14

In Crossed Fused Renal Ectopia, the ectopic kidney is located whre?

Answer 14

Inferior

Question 15

Crossed Fused Renal Ectopia:
What kidney more commonly crosses?

Answer 15

Left over to the right fusing in the lower pole

Question 16

Complications of Crossed Fused Renal Ectopia

Answer 16

stones, infection, and hydronephrosis (50%).

Question 17

Crossed Fused Renal Ectopia classic CT picture?

Answer 17

The first at the level of the kidneys hinting that one kidney may be absent.

The second through the bladder (on a delayed phase) showing two opacified ureters.

Question 18

Crossed Fused Renal Ectopia is most common in what gender? and is associated in what condition?

Answer 18

Male

VACTERL

Question 19

This is the most common congenital anomaly of the GU tract in neonates.

Answer 19

Congenital UPJ Obstruction

Question 20

Congenital UPJ Obstruction

About 20% o f the time, these are A. Bilateral or unilateral?

Most (b. __ %) of these are thought to be caused by c. _____?

Answer 20

A. bilateral

B. Most (80%) of these are thought to be caused by

C. Intrinsic defects in the circular muscle bundle of the renal pelvis.

Question 21

Treatment fo Congenital UPJ Obstruction

Answer 21

Pyeloplasty

Question 22

How to tell the difference between a prominent extrarenalpelvis vs a congenital UPJ obstruction?

Answer 22

“Whitaker Test”, which is a urodynamics study combined with an antegradepyelogram.

Question 23

Scenario
Teenager with flank pain after drinking “lots of fluids.” + non dilated uterters =

Answer 23

Think of Congenital UPJ Obstruction

Question 24

When you have AR Polycystic Kidney Disease, you have this.

Answer 24

congenital hepatic fibrosis is ALWAYS present in ARPKD.

Question 25

Kids with ARPKD have these conditions

Answer 25

HTN + Renal Failure + Congenital Hepatic Fibrois

Question 26

Common cause of death in ARPKD

Answer 26

Portal hypertension

Question 27

diagnsosis?

describe

Answer 27

ARPKD

smoothly enlarged and diffusely echogenic, with a loss of corticomedullary differentiation.

Cysts tend to be tubular and spare the cortex.

Question 28

Neonatal Renal Vein Thrombosis associations

Answer 28

Maternal DM + Sepsis + Dehydration

Question 29

Neonatal Renal Vein Thrombosis Acute vs Chronic

Answer 29

When acute, will cause renal enlargement. When chronic, will result in renal atrophy.

Question 30

Neonatal Renal artery Thrombosis occurs secondary to what?

What is the presentation?

Answer 30

umbilical artery catheters

severe hvpertension.

Question 31

Diagnosis?

describe

Answer 31

Prune Belly (Eagle Barrett Syndrome)

shown on a babygram with a kid shaped like a pear (big wide belly).

Question 32

Triad Eagle Barret Syndrome

Answer 32

or Prune belly

• Deficiency of abdominal musculature
• Hydroureteronephrosis
• Cryptorchidism (Bladder distension interferes with descent of testis)