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DMST 315 OBGYN 3 > Module 9: Fetal GI Pathology > Flashcards

Module 9: Fetal GI Pathology Flashcards

1
Q

The ureteric buds become what? 4 (Anatomy)

A
  1. Ureters
  2. Renal Pelvis
  3. Calyces
  4. Collecting tubules
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2
Q

The metanephros gives origin to what?

A

Nephrons and the glomeruli and loop of henle

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3
Q

Fetal urine production begins around what week?

A

11 LMP

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4
Q

Early on, the primitive kidney (metanephros) is located where?

A

In the pelvis

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5
Q

The kidneys migrate where later on? What happens if this does not happen? 2

A
  1. To their normal paraspinal position
  2. Ectopic pregnancy
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6
Q

The cloaca is divided into what two parts? 2

A
  1. Urinary
  2. Rectal segments
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7
Q

The caudal end of the cloaca forms what? 2

A
  1. The urethra and bladder neck.
  2. The rest of the bladder is derived from the Allantois which ends at the urachal
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8
Q

The mesonephric ducts (ureters) enters what?

A

Posterior wall of the bladder, at the trigone

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9
Q

Prior to 8 weeks LMP< the fetal external genitals are in what state?

A

Ambiguous state

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10
Q

At 8-10 weeks LMP, with the presence of hormones (testosterone) what are formed?

A

Male genitalia is formed

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11
Q

What happens when male genitalia is formed?

A

Wolffian ducts grow and Müllerian ducts are inhibited

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12
Q

In females, with no Male hormones, the normal female genes develop due to what?

A

Maternal estrogen

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13
Q

What happens when female genitalia develop?

A

Wolffian ducts regress and Müllerian ducts are stimulated

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14
Q

Fetal kidneys are routinely imaged in what view?

A

Trans

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15
Q

If pathology is detected in fetal trans, what happens?

A

Sag views are included

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16
Q

What is the normal kidney length of the fetus?

A

Equal to the gestational age
20 weeks = 20mm

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17
Q

Mild fetal renal pelvis dilation can be up to what? 2

A
  1. 5mm up to 20 weeks gestation
  2. 8mm from 20 weeks to term
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18
Q

Bilateral renal agenesis is associated with what other anomalies? 5

A
  1. Other GU abnormalities
  2. GI abnormalities
  3. Cardiac abnormalities
  4. Sirenomelia (mermaid syndrome)
  5. Teratogens (Warfarin, Cocaine, maternal diabetes type 1)
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19
Q

Unilateral renal agenesis may be what? (Asymptomatic or symptomatic)

A

Asymptomatic and not noticed in utero

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20
Q

What is seen (or not seen) with bilateral renal agenesis? 5

A
  1. Absent kidneys
  2. Absent bladder
  3. Oligiohydramnios- anhydraminos
  4. Small abdominal circumference
  5. IUGR
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21
Q

With bilateral renal agenesis, adrenals assume what kind of shape?

A

Discoid shape and can be mistaken for kidneys

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22
Q

What position does the adrenal glands look like with agenesis of the kidneys?

A

Lying down

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23
Q

What should we do with a sonographic assessment with agenesis of the kidneys? 5

A
  1. Confirm presence or absence of kidneys
  2. Look for anechoic renal pelvises
  3. Look for renal cortex
  4. Use colour doppler to confirm presence of renal arteries
  5. Assess amniotic fluid
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24
Q

What does Potter’s syndrome refer to?

A

Bilateral renal agenesis

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25
Q

Potter’s sequence refers to what? (consequence)

A

The consequence of severe oligiohydramnios

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26
Q

What is the indicators of potter’s syndrome? 3

A
  1. Pulmonary hypoplasia
  2. Abnormal hand and foot positions
  3. Facial anomalies
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27
Q

What are abnormal of Potter’s syndrome? 3 (legs and hips)

A
  1. Bowed legs
  2. Clubbed feet
  3. Hip dislocation
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28
Q

With potter’s syndrome, malformations are caused by what?

A

Oligiohydramnios

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29
Q

Causes of oligiohydramnios are from what? 3

A
  1. Renal abnormalities
  2. Amniotic fluid leakage
  3. Placental abnormalities
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30
Q

What are some renal abnormalities that cause oligiohydramnios? 3

A
  1. Renal agenesis
  2. Multi cystic dysplastic kidneys
  3. Genitourinary obstruction
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31
Q

Oligiohydramnios impairs normal development in what? 3

A
  1. Extremities
  2. Facial features
  3. Lungs (pulmonary hypoplasia)
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32
Q

What are some facial features/ anomalies that appear in Potter’s sequence? 4

A
  1. Beaked nose
  2. Low set ears
  3. Prominent epicanthic folds
  4. Hypertelorism
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33
Q

What is hydronephrosis?

A

Distension of the renal pelvis and calyces with urine due to an obstruction

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34
Q

What percentage of renal abnormalities are hydronephrosis?

A

75%

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35
Q

What percentage of hydronephrosis is associated with syndromes?

A

20%

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36
Q

Hydronephrosis is typically bilateral or unilateral?

A

Typically unilateral

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37
Q

What are causes of hydronephrosis? 3

A
  1. Obstruction
  2. Reflux
  3. Ureterocele
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38
Q

What are causes of hydronephrosis at 3 possible levels?

A
  1. At the level of the renal pelvis (or along the ureter)
  2. At the level of the ureter insertion in to the bladder
  3. At the level of the bladder outlet/ urethra
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39
Q

What is reflux in terms of hydronephrosis?

A

Ureterovesical valves are not working properly

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40
Q

Ureterocole in terms of hydronephrosis is what?

A

Ectopic placement of the valves

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41
Q

What do we need to do to interrogate hydronephrosis? 4

A
  1. Determine if bilateral or unilateral
  2. Determine degree of hydronephrosis
  3. Determine level of obstruction
  4. Evaluate for other abnormalities
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42
Q

What are some things we see when determining level of obstruction in terms of hydronephrosis? 3

A
  1. Hydronephrosis only/ Only kidney are dilated
  2. Dilated ureter with hydronephrosis
  3. Dilated bladder and keyboard appearance
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43
Q

If we see hydronephrosis only/ only kidneys are dilated where do we see the obstruction?

A
  1. Ureteropelvic junction
  2. UPJ obstruction
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44
Q

If we see dilated ureters with hydronephrosis where do we see the obstructions?

A
  1. Ureterovesical junction
  2. UVJ
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45
Q

If we see dilated bladder and keyhole appearance, where is the obstruction?

A
  1. Urethral junction obstruction
  2. PUV - posterior urethra valve obstruction
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46
Q

What is the most common obstruction?

A

UPJ

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47
Q

Where are UPJ obstructions, most common seen??

A

At the level of the renal pelvis

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48
Q

If a ureteropelvic junction obstruction is unilateral what would we see?

A

Normal bladder and amniotic fluid

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49
Q

If bilateral what do we see with ureteropelvic junction obstruction? 2

A
  1. Oligiohydramnios
  2. Hydronephrosis of varying degrees
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50
Q

What is seen with severe UPJ obstruction?

A

Renal parenchyma may be destroyed

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51
Q

What are some pitfalls of determining UPJ obstructions? 3

A
  1. Be aware of the extra renal pelvis
  2. Prominent renal veins
  3. Reflux
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52
Q

Why should we be aware of the extra renal pelvis? 3

A
  1. Renal pelvis outside
  2. May mimic a small hydronephrosis
  3. Dilated calyces indicated hydronephrosis over extra renal pelvis
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53
Q

In terms of UPC, What do we do with the prominent renal viens?

A

Check with colour doppler

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54
Q

What do we see with reflux? 2

A
  1. Urine is moveing from the bladder up the ureter into the kidney
  2. This is transient meaning the kidney dilation
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55
Q

Where do UVJ obstructions insert. What is Ureterovesical obstruction due to?

A
  1. At the level of the ureter insertion into bladder
  2. Usually due to duplicated collection
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56
Q

What is a duplex kidney?

A

Upper pole ureter inserts Inferiorly in the bladder and will have a ureterocele protrued’

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57
Q

What is a ureterocele?

A

Cystic dilation of the intravesicular portion of the ureter

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58
Q

Ureteroceles are associated with dilation of which side of the kidney in a duplicated collecting system?

A

Upper pole

59
Q

What does UVJ obstruction look like on u/s?

A

Dilated upper pole of the kidney with a dilate tourtous

60
Q

With the UVJ, the ureter may mimic what? 2 why?

A

Bowel
1. Bowel is medial in the abdomen
2. Ureter would be lateral in the abdomen

61
Q

The ectopic ureter is associated with what?

A

Ureterocele in bladder

62
Q

Duplex kidney with hydronephrosis affects what pole?

A

Upper pole

63
Q

What is UVJ obstruction associated with ?

A

Ureterocele

64
Q

What is the abbreaviation of posterior urethra valves?

A

PUV

65
Q

What is a PUV?

A

Obstructive membranes in the posterior urethra that obstruct the flow of urine at the level of the bladder outlet/ Urethra

66
Q

What does a keyhole bladder look like? 3

A
  1. Large, distended bladder
  2. Dilated proximal urethra
  3. Bladder wall thickness
67
Q

What symptoms do we see with PUV? 2

A
  1. Oligiohydramnios
  2. +/- Hydroneprhosis and hydroureter
68
Q

Who is most likely affected by PUVs?

A

Males typically

69
Q

PUV can be a precursor to what?

A

Prune belly syndrome (eagle- Barrett syndrome)

70
Q

PUVs have a risk of what? What does this lead to?

A
  1. Bladder or renal forniceal rupture
  2. Ascites
71
Q

What do we see with prune belly syndrome? 5 (3 main)

A
  1. Undescended testicles
  2. Very Large bladder, with abdominal distension
  3. Lack of abdominal musculature
  4. Ureters may be tortuous and dilated
  5. Kidneys can be normal, hydronephrotic, dysplastic
72
Q

What are three types of renal cystic disease?

A
  1. Infantile Polycystic disease (ARPKD)
  2. Adult Polycystic disease (ADPKD)
  3. Multi cystic dysplastic kidney
73
Q

Infantile Polycystic kidney disease is what kind of disease?

A

Autosomal recessive disorder (ARPKD)

74
Q

What is the primary defect of Infantile Polycystic kidney disease? (what is primarily affected)

A

Collecting ducts (cortex)

75
Q

What is seen with IPKD ARPKD? 5

A
  1. Multiple small cysts under 1-2 mm
  2. Grossly enlarged echogenic kidneys, but retain form
  3. Always bilateral
  4. Severe oligiohydramnios
  5. Absent bladder
76
Q

What is the diagnostic criteria for IPKD- ARPKD? 3

A
  1. Bilateral enlarged echogenic kidneys
  2. Oligiohydramnios
  3. Absent bladder
77
Q

What are four types of ARPKD based on the onset of the disease?

A
  1. Perinatal
  2. Neonatal
  3. Infantile
  4. Juvenile
78
Q

IPKD is associated with disorders? 3

A
  1. Meckal- Bruner syndrome
  2. Robert’s syndrome
  3. T13
79
Q

What is the DDX for IPKD? 3 (Infantile polycystic kidney disease)

A

Benign glomeruloscelerosis
1. Large
2. Echogenic kidneys but echopenic pyramids
3. Normal fluid

80
Q

What kind of disorder is Adult Polycystic kidney disease?

A

Autosomal dominant disorder (ADPKD)

81
Q

How common is ADPKD seen?

A

Rarely seen in antenatal sonography, however fetus assessed if a parent has the disease and vice versa if a parent has the disease

82
Q

What is seen with APKD? 4

A
  1. Enlarged kidneys with cysts
  2. May be hyperechoic
  3. Bilateral - but ma be more prominent in one kidney
  4. Fetal bladder is present
83
Q

What is the amniotic fluid like in APKD?

A

Normal

84
Q

What are multicystic dysplastic kidneys?

A

Multiple cystic lesions which due not communicate

85
Q

With MCDK, kidneys are shaped how?

A

Kidneys lose their reniform shape

86
Q

In terms of MCDK, unilateral has what kind of prognosis? Why?

A
  1. Good prognosis
  2. Compensatory hypertrophy is often seen in unaffected kidney, and affected kidney is left alone
87
Q

MCDK are rarely what?

A

Bilateral, however if so fatal due to lack of amniotic fluid.

88
Q

Bilateral MCDK is associated with what anomaly?

A

Potter’s sequence

89
Q

Is there a genetic predisposition of MCDK?

A

No

90
Q

What would poor prognosis look like for GU? 6

A
  1. Bilateral renal agenesis
  2. Increased renal echogenicity
  3. Bilateral hydronephrosis
  4. Renal cysts (MCDK)
  5. Bladder obstruction
  6. Oligiohydramnios
91
Q

What is the hydroneprhosis prognosis? 3

A

Depends on the timing of diagnosis
1. first trimester: severe pathology, poor outcome
2. 2nd trimester: most improve or resolve completely
3. 3rd trimester: higher incidence of post natal pathology requiring surgical intervention

92
Q

What is the most common congenital renal tumor in utero?

A

Mesobalstic nephroma

93
Q

IS mesoblastic nephroma benign or malignant?

A

Benign

94
Q

What kind of mass is mesoblastic nephroma?

A

Solitary, solid mass arising from kidney area.

95
Q

Mesoblastic nephroma may cause what to happen in terms of fluid?

A

Polyhydraminos

96
Q

What kind of tumor is a neprhoblastoma? 3 (solid/ cystic, malignancy, occurance in utero)

A
  1. Solid mass
  2. Malignant
  3. Not commonly identified in utero
97
Q

What is the most common abdominal tumor found in newborns?

A

Neuroblastoma s

98
Q

What kind of mass is a neuroblastoma? 2 (location and misc)

A
  1. Mixed cystic/ solid mass near upper pole of kidney
  2. Calcifications may be present
99
Q

What is the malignancy of neuroblastoma? 50% of individuals develop what at birth? Mortality depends on what?

A
  1. Malignant
  2. 50% have METS at birth
  3. Mortality depends on age at diagnosis and stage
100
Q

Ambiguous genitalia may be associated with what? 2 (usually are what?)

A
  1. Adrenal hyperplasia or maternal ingestion of male hormones early in pregnancy
  2. Usually XX fetuses
101
Q

Ambiguous genitalia may not be discovered until when? What happens then?

A
  1. The 16th week LMP
  2. By which time masculinization of affected female has already occurred
102
Q

What is prenatal treatment of ambiguous genitalia?

A

Prenatal treatment includes steroid administration to the mother from 10 weeks LMP to delivery

103
Q

What are X linked syndromes? 2

A
  1. Turners or Noonans’
  2. Hemophilia
104
Q

What are hydrometrocolops?

A

Secretions in uterus and vagina

105
Q

What is the occurrence rate of ovarian cysts?

A
  1. Rare
106
Q

What is the prognosis of ovarian cysts?

A

Benign

107
Q

What does ovarian cysts look like? How do they appear?

A
  1. Unilateral more common that bilateral
  2. Range in size from small cysts to structure filling the entire abdomen
  3. Cystic mass separate from urinary tract
108
Q
A
109
Q

What is this?

A

Fetal kidneys

110
Q

Label

A

Urine filled renal pelvises

111
Q

What is this?

A

Fetal kidneys

112
Q

What is this?

A

Fetal kidneys in sag

113
Q

Label

A

Kidneys and adrenals

114
Q

What is the significance of this image?

A

Fetal bladder

115
Q

What is going on here?

A

Sag Rt adrenals with agenesis NOT kidneys

116
Q

Significance?

A

Missing kidneys

117
Q

What does this mean?

A

Normal Renal Arteries

118
Q

What’s going on here?

A

Normal and abnormal renal arteries

119
Q

What’s happening here?

A

Unilateral Agenesis of the kidney

120
Q

What does this image represent?

A

Unilateral agenesis of the kidney

121
Q

What does this image represent?

A

Bilateral renal agenesis

122
Q

What does this image represent?

A

Potter’s sequence

123
Q

What does this image represent?

A
124
Q
A
125
Q

What’s going on here?

A

Unilateral hydronephrosis

126
Q
A
127
Q

What’s going on here?

A

Bilateral UPJ obstruction

128
Q
A
129
Q
A
130
Q

What is going on here?

A

UVJ obstruction

131
Q

What is this?

A

UVJ obstruction

132
Q

What’s going on here?

A

Urethral obstruction

133
Q

What’s going on here?

A

PUV obstruction

134
Q

What’s going on here?

A

PUV obstruction

135
Q
A
136
Q

What’s going on here?

A

Prune belly syndrome

137
Q

What’s going on here?

A

Prune belly syndrome

138
Q

What’s going on here?

A

Infantile polycystic kidney disease

139
Q

What’s going on here?

A

APKD

140
Q
A
141
Q

What’s going on here?

A

MCDK

142
Q

What’s going on here?

A

Right multicystic dysplastic kidney

143
Q

What does this image represent?

A

Mesoblastic Nephroma

144
Q

What does this image represent?

A

Hydrocele and ovarian cyst

DMST 315 OBGYN 3 (21 decks)

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