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CNIH > UT abnormalities > Flashcards

UT abnormalities Flashcards

1
Q

the kidney is made from the?

A

Metanephros

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2
Q

at 7th week GA the metanephros develops from the?

A 
metanephric diverticulum (ureteric bud)
metanephric mass of the intermediate mesoderm
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3
Q

What is the ureteric bud?

  • gives rise to?
  • interacts with?
A
  • is an outgrowth of the mesonephric duct
  • Gives rise to the ureter, renal pelvis, calyces and collecting tubules
  • Interacts with the metanephric mesoderm to induce formation of the nephrons
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4
Q

3 phases of nephrogenesis?

A
  1. Pronephros
  2. Mesonephros
  3. Metanephros
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5
Q

In embryo the kidneys are located in the pelvis. When do they ascent to the adult position?

A

by 11th week and they start to produce urine

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6
Q

When is the cloaca divided?

A

9th week

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7
Q

9th week, the cloaca is divided by the urorectal septum into?

A 
Rectum posteriorly
Urogenital sinus anteriorly:
- Urinary bladder
- Female urethra
- Most of male urethra
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8
Q

The bladder is continuous with what?

A

allantois

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9
Q

what does the allantois become?

A

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

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10
Q

at birth what apperance do the kidneys have?

A
  • multilobular appearance
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11
Q

when does completion of smoothing of the kidneys occur?

A

during childhood

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12
Q

when is renal architecture finalized?

A

betwene 5-15 weeks

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13
Q

bladder develops from what?

A

theupper part of the urogenital sinus - connected with the allantois

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14
Q

what does the allantois form?

A

forms a fibrous cord, the urachus, which following birth, becomes themedian umbilical ligament

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15
Q

When does male and female genitalia become apperent?

A

11 weeks

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16
Q

When does male testicular descent occur?

A

25 weeks gestaiton

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17
Q

after what week does fetal urine production become a source of amniotic fluid?

A

after 16 weeks

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18
Q

Normal AFV in the second half of a pregnancy implies what?

A

at least one functioning kidney and a patent urinary conduit to the amniotic cavity

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19
Q

What is fetal lung development affected by? (3)

A
  • amniotic fluid
  • available thoracic space
  • neuromescular functions
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20
Q

during fetal development the kidney is a major source of?

A

proline

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21
Q

what is proline?

A

aids in the formation of collagen and mesenchyme in the lung

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22
Q

What is one of the most frequent sites of congenital anomalies detected with fetal sonography?

A

Urinary tract

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23
Q

What to evaluate in fetal urogenital system? (5)

A 
1. Kidneys
2, collecting system 
3. bladder
4. gender
5. amniotic fluid volume
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24
Q

Renal anomalies may be associated with what other anomalies? (2)

A
  1. VATER

2. VACTERAL

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25
Q

VATER stands for?

A

V- vertebral defects
A- anal atresia
T & E- tracheo-esophageal fistula
R- radial defects and renal anomalies

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26
Q

VACTERL stands for?

A 
Vertebral defects
Anal atresia
Cardiac defects
Tracheo-esophageal fistula
Radial defects and Renal anomalies
Limb abnormalities
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27
Q

Bilateral renal agenesis is what typer of anomaly?

A

lethal congenital anomaly

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28
Q

Bilateral renal agenesis?

A 
Male predominance
Ureteric bud fails to develop
Nephrons do not form
No urine is produced
Severe oligohydramnios results
Pulmonary hypoplasia is the major cause of neonatal death
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29
Q

What is Potter Sequence or Syndrome

A

refers to any case that presents with oligohydramnios or anhydramnios regardless of the source of the loss of amniotic fluid

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30
Q

What is Potter Sequence or Syndrome

A

refers to any case that presents with oligohydramnios or anhydramnios regardless of the source of the loss of amniotic fluid

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31
Q

Potter sequence is characterized by? (5)

A 
Renal failure
Severe oligohydramnios
Face – beaked nose, low set ears, prominent epicanthic folds, hypertelorism
Limb deformities
IUGR
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32
Q

Potter sequence is characterized by? (5)

A 
Renal failure
Severe oligohydramnios
Face – beaked nose, low set ears, prominent epicanthic folds, hypertelorism
Limb deformities
IUGR
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33
Q

Epicanthal Fold?

A

skin fold of the upper eyelid that covers the inner corner (medial canthus) of the eye

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34
Q

Bilateral renal agenesis sono apperance?

A

No visualization of fetal kidneys
No visualization of fetal bladder (over 1 hour)
Before 16 weeks gestation, AFV is not dependent upon fetal renal function and fetal urine may be normal
After 16 weeks, severe oligohydramnios
‘Lying down’ adrenal sign (the adrenal is not forced into its normal shape by the kidney)*
Absent renal arteries using Colour Doppler

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35
Q

Bilateral renal agenesis common pitfalls?

A
  • Bowel or adrenal gland in the renal fossae may be mistaken for kidneys’
  • Visualization of a normal fetal bladder excludes the diagnosis
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36
Q

Unilateral renal agenesis occurs in about?

A

1 in 1000

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37
Q

unilater renal agenesis is associated with? (2)

A
  1. normal AFV

2. normal visualization of bladder

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38
Q

unilater renal agenesis pittfall?

A
  • failure to image in renal fossae in the far field because of acoustic shadowing from the spine
  • especially in TRV place
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39
Q

unilateral renal agenesis- If a kidney is not found in the renal fossa what should you assume?

A
  • absent or ectopic kidney
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40
Q

what occurs to contralateral kidney if unilateral renal agenesis occurs?

A
  • may be enlarged because of compensatory hypertrophy

- Vesicoureteral reflux may occur

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41
Q

Unilateral renal agenesis-associated abnormalities? (4)

A

Genital
Cardiac
Skeletal
GI

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42
Q

Renal ectopia?

A 
One or both kidneys may be in an abnormal position:
Pelvic
Crossed
Crossed fused
Horseshoe
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43
Q

Most common form of renal ectopia?

A

pelvic kidney

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44
Q

Horseshoe kidney?

A

1/500 births
Abnormal longitudinal axis of both kidneys
Bridge of renal tissue connecting the lower poles
Findings are subtle and surrounding bowel can obscure the fused isthmus

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45
Q

In horseshoe kidneys, there is a higher incidence of? (4)

A
  • vesicoureteral reflux
  • renal calculi
  • urinary tract infections
  • hydronephrosis
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46
Q

Horseshoe kidney is associated with? (5)

A
  1. Urogenital
  2. Cardiac
  3. Skeletal
  4. CNS
  5. Chromosomal
    - Turner syndrome, trisomy 18, trisomy 9
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47
Q

MOst common neoplasm in the fetus and newborn?

A

Congenital mesoblasic nephroma

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48
Q

what is congenital mesoblastic nephrons?

A
  • benign hamartoma composed of mesochymal tissue
49
Q

wilms tumor?

  • what is it?
  • what is it composed of?
A

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

50
Q

Dysplastic cysts?

A
  • Multicystic dyspastic kidney and dysplastic kidney resulting from early severe obstruction
  • Most forms or renal dysplasia are likely due to renal obstruction during embryogenesis.
  • Dysplasia = abnormality of development or an epithelial anomaly of growth and differentiation
51
Q

Hereditary cysts?

A

Polycystic kidney disease and the inherited syndromes

52
Q

Non-dysplastic non-hereditary cysts?

A

simple cysts

53
Q

Multi-Cystic Dysplastic Kidney (MCDK)

  • what is it?
  • what is associated with? (2)
A

Is most common form of cystic disease in childhood
Represents one of the most common abdominal masses in the neonate
Associated with atretic ureter and pelvoinfundibular atresia

54
Q

atresia?

A

a condition in which a body orifice or passage in the body is abnormally closed or absent

55
Q

What is MCDK?

A
  • Kidney is replaced by multiple cysts of varying size and no normal renal tissue present (kidney not functional)
  • Typically unilateral
56
Q

MCDK- Observations most consistent with a multicystic dysplastic kidney?

A
  • Multiple cysts of varying sizes are seen in the right paraspinous location.
  • No normal appearing kidney identified
  • The contralateral kidney, bladder and amniotic fluid are all normal.
57
Q

The main distinguishing feature is the pattern of renal cysts with MCDK?

A

cysts are randomly distributed, non communicating, and of variable size.

58
Q

Severe hydronephrosis?

A

In contrast, with hydronephrosis: renal cysts are similar in size, communicating with each other

59
Q

Main D/D MSDK?

A

Severe hydronephrosis

60
Q

Multicystic renal dysplasia is bilateral in what % of cases?

A

19-24%

61
Q

Unilateral Obstructive Cystic Renal Dysplasia can be caused by?

A

ureteropelvic or vesicoureteral junction obstruction=

62
Q

Bilateral Obstructive Cystic Renal Dysplasiacan be caused by?

A

can be caused by severe bladder outlet obstruction, usually urethral atresia or posterior urethral valves

63
Q

Obstructive Cystic Renal Dysplasia severity is related to?

A

timing and severity of the obstruction to urine flow

64
Q

Sono apperance of cystic renal dysplasia?

A
  • Variable

Most typical finding: small echogenic kidney with one or several small subcapsular, cortical cysts + evidence of urinary obstruction.

  • Renal cysts may change in size over time as well as the size of the kidney and parenchymal echogenicity.
65
Q

Infantile Autosomal Recessive Polycystic Kidney Disease involves?

A
  • An autosomal recessive disorder = two copies of an abnormal gene must be present in order for the disease or trait to develop.
  • Infantile Autosomal Recessive Polycystic Kidney Disease involves the kidneys and the liver
66
Q

perinatal Infantile Autosomal Recessive Polycystic Kidney Disease?

A

severe renal disease, minimal hepatic fibrosis and early death from pulmonary hypoplasia

67
Q

juvenile Infantile Autosomal Recessive Polycystic Kidney Disease?

A

minimal renal disease, marked hepatic fibrosis and longer survival

68
Q

Infantile Autosomal Recessive Polycystic Kidney Disease ultrasound?

A
  • Both kidneys are symmetrically enlarged (3-10X larger than normal) and show increased echogenicity.
  • Distinct parenchymal cysts are usually too small to be resolved.
  • Most cases: renal function is severely impaired
  • There is severe oligohydramnios and bladder may not be seen.
  • The size of the kidneys and cysts do not correlate with renal function
69
Q

Infantile Autosomal Recessive Polycystic Kidney Disease can be diagnosed at what week?

A

16 week

- oligohyramnios + characteristic renal changes

70
Q

an elevation of what is associated with infantile polycystic kidney disease?

A

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

71
Q

autosomal dominant means?

A

A single abnormal gene on one of the first 22 non-sex (autosomal) chromosomes from either parent can cause an autosomal disorder

72
Q

adult autocomal dominant polycystic kidney disease ?

A

autosomal, dominant polycystic disease, which only rarely manifests in the fetus or prior to adulthood

73
Q

adult autocomal dominant polycystic kidney disease ultrasound?

A

bilateral, however, the sonographic appearance in the fetus is variable

74
Q

mose common finding of adult autosomal dominant polycystic kindey disease?

A

Nephromegaly with or without an increase in renal echogenicity is the most common finding; cortical cysts may or my not be seen

75
Q

Simple Renal Cysts on ultrasound?

A

Ultrasound shows a small solitary, unilocular cyst near the periphery of the kidney

76
Q

simple renal cysts seen as early as? resolve by?

A

Seen as early as 14 – 16 weeks

Most resolve by 20 – 24 weeks gestation

77
Q

Mild Pyelectasis?

A
  • mild dialation of the renal pelvis
78
Q

mild pyelectasis is a normal sono finding wich may be seen in?

A
  • Normal fetuses as transient, physiologic phenomenon, or
  • It may be pathologic and represent the earliest manifestation of a urinary tract abnormality (mild fetal renal pyelectasis before 21 W = 1.1%)
79
Q

Hydronephrosis

A
  • It refers to abnormal dilation of renal pelvis and calyces

- can be bilateral or unilateral

80
Q

What is the most common fetal urinary tract abnormality detected with prenatal US?

A

hydronephrosis

81
Q

what represents the most common cause of fetal hydronephrosis?

A
  • obstruction

- non - obstructive vesicoureteral reflux (VUR)

82
Q

How to measure hydronephrosis?

A

Measurement of the antero-posterior renal pelvic diameter (RPD) on a transverse scan of fetal abdomen

83
Q

the Measurement of the antero-posterior renal pelvic diameter (RPD) on a transverse scan of fetal abdomen evaluates? (3)

A
  1. Renal pelvic dilation (mild- moderate- marked)
  2. Calyceal dilation
  3. Parenchymal atrophy
84
Q

Renal pelvic diameter measurement hydronephrosis?

A

If RPD ≥ 5 mm at 18 - 23 W, or

If RPD ≥ 7 mm in the 3rd trimester

85
Q

Hydronephrosis grading 1-4?

A

Grade 0: no hydronephrosis; intact central renal complex.
Grade 1: only dilated renal pelvis; there is some fluid in the renal pelvis.
Grade 2: dilated renal pelvis and a few calices are visible.
Grade 3: all the calices are dilated.
Grade 4: further dilation of renal pelvis and calices, with thin renal parenchyma.

86
Q

what is the most common cause of neonatal hydronephrosis?

A

UPJ obstruction

87
Q

Uretero-Pelvic Junction Obstruction are due to?

A

muscular abnormality in the ureteral wall

88
Q

are uretero-pelvic junction obstructions typically bulateral or unilateral?

A

unilateral (10% to 30% bilateral)

89
Q

Sonographic findings of unilateral UPJ Obstruction include?

A
  • Dilation of the renal pelvis with or without dilation of the calyces.
  • The ureter, bladder, and AFV should appear normal.
  • Bilateral UPJ is associated with variable degrees of oligohydramnios and a variable prognosis depending on the severity and duration of renal obstruction
90
Q

Uretero-Vesical Junction Obstruction?

A

UVJ obstruction is the second most common cause of fetal and neonatal hydronephrosis

91
Q

Sonographic findings of unilateral UVJ obstruction

A
  • Variable degrees of ureteral dilation (hydroureter) and variable degrees of hyronephrosis.
  • AFV and bladder are normal.
  • With bilateral UVJ obstruction variable severity of oligohydramnios depending on the obstruction
92
Q

A secondary cause of UVJ obstruction with a specific sonographic appearance is a?

A

uterocele

93
Q

what is a uterocele?

A

is a prolapse of the distal ureteric mucosa into the urinary bladder causing a cystic dilation of the prolapsed segment in the bladder.

94
Q

Simple ureterocele ?

A

if the ureter is normally located in the bladder wall.

95
Q

Ectopic ureterocle?

A

if the ureter inserts into the bladder in an ectopic location

96
Q

Ectopic ureterocele associated with?

A

renal duplication (duplex kidney). It is usually associated with focal or segmented hydronephrosis involving the upper pole of the duplex kidney

97
Q

Diagnosis of ureterocele?

A
  • It may be made when a thin-walled cystic structure can seen in the bladder in association with hydroureter and/ or hydronephrosis.
  • Hydroureter is located posteriorly and should be seen to communicate with the renal pelvis and/ or bladder.
  • Variable prognosis depending on the obstruction
98
Q

POSTERIOR URETHRAL VALVE

A

obstructive developmental anomaly in the urethra and genitourinary system of male newborns

It is the most common cause of bladder outlet obstruction in male newborns

99
Q

most common cause of urinary obstruction at the urethral level is?

A

PUV- osterior urethral valves

100
Q

What is PUV?

A

valve like tissue flaps in the proximal or prostatic portion of the male urethra.

101
Q

The most specific sonographic finding of PUV?

A

key hole sign

  • describes a dilated urinary bladder with a dilated proximal urethra
102
Q

PUV sono apperance?

A
  • The bladder wall may appear abnormally thickened and there may be bilateral hydroureters and hydronephrosis.
  • Oligohydramnios may be mild to severe depending on the degree of obstruction
103
Q

Non visualization of the bladder is mostly due to?

A

specific fetal renal abnormalities.

104
Q

Absence of the bladder due to renal abnormalities must be?

A

bilateral

105
Q

Failure to Produce Urine abnormalities?

A 
Bilateral Renal Agenesis
 Bilateral multicystic kidneys
 Bilateral severe renal dysplasia
 Bilateral severe UPJ obstruction
 Autosomal recessive polycystic disease
 Severe intrauterine growth restriction
106
Q

Failure to store urine abnormalities?

A

Bladder exstrophy
Cloacal exstrophy
Bilateral single system ectopic ureters

107
Q

Failure to store urine abnormalities?

A

Bladder exstrophy
Cloacal exstrophy
Bilateral single system ectopic ureters

108
Q

BLADDER MEGACYSTIS

A

An abnormal, large fetal bladder is a sign of bladder outlet obstruction and may be associated with oligohydramnios, hydroureters (obstructive uropathy) and/ or hydronephrosis.

109
Q

The most common cause of megacystis is?

A

posterior urethral valves

110
Q

Bladder megacystis measurements?

A

bladder diameter >7 mm in the 1st trimester
bladder diameter >30 mm in the 2nd trimester
bladder diameter >60 mm in the 3rd trimester

111
Q

main causes of bladder megacystis? (2)

A
  • distal stenosis

- reflux

112
Q

associated anomalies with bladder megacystis?

A 
Posterior urethral valves
 Chromosomal anomalies
 Oligohydramnios
 Megacystis megaureter syndrome
 Prune belly syndrome
113
Q

Causes of Megacystis

A 
Posterior urethral valves
 Urethral atresia/stricture
 Prune Belly Syndrome
 Megalourethra
 Cloacal malformation
 Megacystis-microcolon-intestinal hypoperistalsis syndrome
114
Q

Cloacal Malformation?

A

A persistent cloaca = a confluence of the rectum, vagina, and urethra into a single common channel.
Occurs exclusively in girls and comprise the most complex defect in the spectrum of anorectal malformations.
Cloacal anomalies occur in 1 per 20,000 live births.
This defect is one of the most formidable technical challenges in pediatric surgery.
Goals of treatment: anatomic reconstruction, achieving bowel and urinary control, as well as normal sexual function.

115
Q

Bladder Extrophy

A

Ventral defect in the infraumbilical abdominal wall.
urinary bladder is exposed and opens anteriorly (the mucosal surface of the bladder is everted through the abdominal defect
Absent fetal bladder in its normal position within the pelvis
Normal kidneys.
Normal amniotic fluid volume.

116
Q

BLADDER EXSTROPHY sono?

A

Fluid-filled bladder not identified
Mucosa may be seen as irregular mass on the anterior abdominal wall, inferior to the umbilicus
It is mostly isolated and rarely associated with OEIS complex (Omphalocele, Exstrophy of Bladder, Imperforate anus, and Spinal defects)

117
Q

Urethral Atresia

A

It is the second common cause of urethral level obstruction and causes the most severe form of urinary obstruction, since fetal urine is unable to pass into the amniotic fluid.
Sonographic findings: megacystis associated with anhydramnios

118
Q

PRUNE BELLY SYNDROME

A

This is also known as Eagle-Barrett Syndrome:
Absent anterior abdominal musculature
Undescended testes (cryptorchidism)
Urinary tract abnormalities (megacystis, hydroureters)
Characteristics:
Bladder distention interferes with the descent of the testes and is responsible for cryptorchidism).
Prognosis:
Variable and depends of the severity of the obstruction and the degree of olioghydramnios.

119
Q

prune belly syndrom classic triad?

A

Absent abdominal musculature
Undescended testis
Urinary tract abnormalities

CNIH (69 decks)

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