Urinary Systems

Question 1

What are the 2 renal anomalies that are associated with other abnormalities:

Answer 1

VATER

VACTERL

Question 2

VATER:

Answer 2

vertebral defect
anal atresia
tracheo-esophageal fistula
radial defects and renal anomalies

Question 3

VACTERL:

Answer 3

vertebral defect
anal atresia
cardiac defects
tracheo-esophageal fistula
radial defects and renal anomalies
limb abnormalities

Question 4

Bilateral renal agenesis is a lethal cogenital anomaly. What happens?

Answer 4

failure of the ureteric bud to develop, so then no nephrons do not form.
no urine is produced
oligohydramnios
pulomnary hypoplasia is cause of death
*one diagnosed, parents are at risk for having another child with renal agenesis (4%)

Question 5

Potter Syndrome:

Answer 5

bilateral renal agenesis and oligohydramnios and anhydramnios
also has facial abnormalities (low set ears, beaked nose, epicanthic folds and downward slanted eyes
IUGR
limb deformities

Question 6

“lying down” adrenal sign is associated with

Answer 6

renal agenesis

Question 7

What excludes the diagnosis of bilateral renal agenesis?

Answer 7

visualization of normal fetal bladder (be careful it’s not urachal diverticulum)

Question 8

Unilateral renal agenesis:

Answer 8

more common than bilateral

associated w/ normal AFV and visualization of the bladder

Question 9

If kidney is not found in the renal fossa, what could it be?

Answer 9

absence/ectopic
contralateral kidney may be enlarged and vesicoureteral reflux may occur
Associated abnormalities: genital. cardiac, skeletal, GI
*risk increases when parent also has unilateral agenesis

Question 10

Renal Ectopia:

Answer 10

pelvic, crossed, crossed fused, horseshoe

Question 11

The most common form of renal ectopia is

Answer 11

pelvic kidney (seen adjacent to the bladder)
can be hypoplastic or dysplastic

Question 12

Horseshoe kidney tissue attaches where?

Answer 12

lower poles

Question 13

Horseshoe kidney:

Answer 13

relatively benign but it has a higher incidence with vesicoureteral reflux, renal calculi, UTI’s and hydropnephrosis

Question 14

Horseshoe kidney can be associated with:

Answer 14

Urogenital
Cardiac
Skeletal
CNS
Chromosomal
Turner syndrome, trisomy 18, trisomy 9

Question 15

What is the most common neoplasm in the fetus and newborn?

Answer 15

congenital mesoblastic nephroma (benign hamartoma composed of mesochymal tissue)

Question 16

What two renal neoplasms are indistinguishable from each other?

Answer 16

Wilm’s tumor
congenital mesoblastic nephroma

Wilm’s is a malignant lesion that is extremely rare and composed of epithelial tissue

Question 17

What are the 3 classifications of renal cystic disease?

Answer 17

dysplastic (severe obstruction most likely during embryogenesis)
hereditary (PKD)
non-dysplastic (simple)

Question 18

Multi Cystic Dysplastic Kidney:

Answer 18

most common form of cystic disease in childhood
one of the most common abdo masses in the neonate
a/w atretic ureter and pelvoinfundibular atresia (passage is closed)

Question 19

How do cysts form from obstruction

Answer 19

complete obstruction or atresia impairs ureteral branching and results in decreased division of collecting tubules and inhibition of induction and maturation of nephrons
the collecting tubules enlarge and develop cysts in their terminal portions

Question 20

Multi Cystic Dysplastic Kidney appearance:

Answer 20

multiple cysts of varying size
no normal renal tissue present
kidney not functional
not usually transmitted and is not a/w with liver or panc disease
contralateral kidney, bladder and AFI is normal
normally unilateral

Question 21

MCDK can be mistaken for? What can be used to tell the difference?

Answer 21

hydronephrosis

hydronephrosis communicates with itself
kidney cysts do not communicate

Question 22

Multicystic renal dysplasia is bilateral in _____ ______ of cases

Answer 22

19-24%

Question 23

Unilateral obstructive cystic renal dysplasia is caused by:

Answer 23

ureteropelvic or vesicouteral junction obstruction

Question 24

Bilateral obstructive cystic renal dysplasia is caused by:

Answer 24

severe bladder outlet obstruction, usually urethral atresia or posterior urethral valves

Question 25

Infantile autosomal recessive polycystic kidney disease:

Answer 25

2 sets of abnormal genes in order for disease to develop
involves kidneys and liver
lots of appearances (perinatal or juvenile)

Question 26

Difference between perinatal and juvenile infantile autosomal recessive PKD:

Answer 26

perinatal-severe renal disease, minimal hepatic fibrosis and early death from pulmonary hypoplasia
juvenile – minimal renal disease, marked hepatic fibrosis and longer survival

Question 27

Can see infantile autosomal recessive PKD at what GA?

Answer 27

as early as 16W (oligohydramnios + renal changes)

Question 28

IPKD can be associated with:

Answer 28

Meckel-Gruber syndrome (IPKD, encephalocele and polydactyl)

Question 29

What’s commonly associated with IPKD?

Answer 29

an elevated KC/AC ratio (40%) is an indication of renal enlargement and commonly associated with infantile polycystic kidney disease

Question 30

What is often seen with adult autosomal dominant PKD?

Answer 30

nephromegaly w/ or w/o an increase in echogenicity

sometimes normal appearance so people may not be aware

Question 31

Simple renal cysts:

Answer 31

seen as early as 14-16W, most resolve by 20-24W

will do f/u so they make sure it doesn’t become multicystic

Question 32

Mild pyelectasis:

Answer 32

mild dilatation of renal pelvis

can be transient, physiological phlegmon,, or pathologic

Question 33

Hydronephrosis:

Answer 33

dilation of renal pelvis and calyces
most common fetal abnormality detected with prenatal U/S
most often from obstruction

Question 34

Renal pelvic diameter is abnormal when

Answer 34

RPD >5mm at 18-23W or RPD >7mm in the 3rd trimester

Question 35

When measuring for hydronephrosis, where do we measure

Answer 35

measure the fluid and also the kidney itself

Question 36

What are the grades of hydroneprhosi:

Answer 36

grade 0: no hydroneprhosis, intact central renal complex
grade 1: only dilated renal pelvis, some fluid in renal pelvis
grade 2: dilated renal pelvis and few calices are visible
grade 3: calices are dilated
grade 4: dilation of renal pelvis and calices and with thin parenchyma

Question 37

What’s the most common cause of neonatal hydronephrosis?

Answer 37

UPJ obstruction (muscular abnormality)

UVJ is second most common

Question 38

Metanephros develop from the what at what week?

Answer 38

metanephric diverticulum (ureteric bud)
7th week GA

Question 39

What is the ureteric bud:

Answer 39

outgrowth of mesonephric duct
gives rise to the ureter, renal pelvis, calcyes and collecting tubules
interacts with metanephric mesoderm to induce formation of the nephrons

Question 40

Kidneys ascend to adult position by week:

Answer 40

11

Question 41

The allantois constricts to become a :

Answer 41

fibrous cord, the urachus, which extends from apex of bladder to the umbilicus

Question 42

Bladder develops from the:

Answer 42

upper part of urogenital sinus and connected to the allantois

Question 43

What does the allantois become after birth?

Answer 43

it becomes obliterated and forms the urachus (fibrous cord), which becomes the median umbilical ligament

Question 44

The urine becomes a major source of amniotic fluid when?

Answer 44

16wks