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DMST ABD 3 > Congenital Anomalies And Variants > Flashcards

Congenital Anomalies And Variants Flashcards

1
Q

What are variants?

A

Variation of anatomy

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2
Q

Are variants pathological?

A

No

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3
Q

What are examples of variants? 2

A
  1. Redials lobe
  2. Junctional fold
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4
Q

Can variants affect function?

A

Yes it could interfere

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5
Q

What are anomalies in terms of major and minor impacts?

A

Both

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6
Q

What are some differences with anomalies? 4

A

Abnormalities in
1. Number
2. Size
3. Position
4. Structure

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7
Q

Can anomalies interfere with function?

A

More likely to interfere

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8
Q

What are hypertrophied columns of bertin? 2

A
  1. Double layers of cortex between pyramid
  2. Indents in renal sinus laterally
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9
Q

Where are hypertrophied columns of bertin located?

A

Upper/ middle portions of kidney

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10
Q

How big should HCB be?

A

Should not be >3cm

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11
Q

What does HCB look like sonographically compared to cortex?

A

Isoechoic to continuous with cortex

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12
Q

What is junctional parenchymal defect?

A

Incomplete embryological fusion

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13
Q

Junctional parenchymal defects are identified more often where?

A

On the right

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14
Q

What does junctional folds look like sonographically?

A

Hyperechoic, wedge shaped

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15
Q

What can junctional parenchymal defects be confused with?

A

Scarring

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16
Q

What is an extra renal pelvis?

A

When the renal pelvis is located medial to renal sinus

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17
Q

What might extra renal pelvis mimic?

A

May mimic hydro

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18
Q

What is extra renal pelvis more prone to?

A

Hydronephrosis

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19
Q

Can the extra renal pelvis depress?

A

Yes, when patient is placed in the prone position

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20
Q

Is extra renal pelvis a normal variant?

A

Yes

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21
Q

Where is dromedary humps located?

A

Lateral aspect of the left kidney

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22
Q

What does dromedary hump look like?

A

Thickening or bulging of cortex

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23
Q

What do we visualize with the dromedary hump?

A

Visualize CM junction to rule out mass

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24
Q

What is fetal lobulation?

A

Infolding of cortex but no thinning

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25
Q

What is the contour of fetal lobulation?

A

Scalloped

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26
Q

How often do we see fetal lobulation?

A

51% of adults

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27
Q

What is renal sinus Lipomatosis?

A

When there is excess fatty infiltration in sinus

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28
Q

What is renal sinus lipomatosis related to? 2

A
  1. Aging commonly
  2. Obesity
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29
Q

What does the renal sinus lipomatosis look like sonographically?

A

Enlarged echogenic area

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30
Q

What is the most common congenital anomaly of the UT?

A

Duplicated collecting system

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31
Q

What does a complete duplicated collecting system look like? 3

A
  1. 2 collecting systems
  2. 2 Ureters
  3. Superior ureter has ectopic bladder insertion
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32
Q

What does a incomplete duplicated collecting system look like?

A

Ureters join and then insert into bladder

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33
Q

What is duplicated collecting systems look like or is associated with? 4

A
  1. UPJ obstruction
  2. Uterus didelphys
  3. Reflux
  4. Ureterocele
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34
Q

What are ureteroceles?

A

Cystic dilation of terminal ureter

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35
Q

How does someone get ureterocele?

A

Congenital or acquired

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36
Q

What does ureteroceles have a higher incidence of?

A

UTIs and obstructions

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37
Q

What are horseshoe kidneys?

A

Fusion at lower poles

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38
Q

Malrotation and associated UPJ obstructions lead to what?

A

Increased risk for infection and stone formation

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39
Q

What does horseshoe kidneys look like on u/s? 4

A
  1. Lower pole obscured
  2. Lower in abdomen
  3. Inferior pole more medial
  4. Isthmus anterior to great vessels
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40
Q

What is an ectopic kidney?

A

Failure to ascend in utero

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41
Q

Where is the ectopic kidney usually?

A

Typically in pelvis and unilateral

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42
Q

How big is the ectopic kidney?

A

Small

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43
Q

How is the ectopic kidney typically placed in the pelvis?

A

Abnormally rotated

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44
Q

What is crossed ectopia? 2

A
  1. Both kidneys on same side
  2. Ectopic kidney fused to other
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45
Q

What is compensatory hypertrophy?

A

When Tissue or organs expands in volume?

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46
Q

Is compensatory hypertrophy diffuse or focal?

A

Either or

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47
Q

What does compensatory hypertrophy cause the kidney to do?

A

Enlarge

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48
Q

What are some conditions that cause compensatory hypertrophy? 2

A
  1. Unilateral renal agenesis
  2. Nephrectomy
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49
Q

What are supernumerary kidneys?

A
  1. Small extra kidney
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50
Q

Where does the blood supply of the supernumerary kidney come from?

A

Own supply

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51
Q

How common is supernumary kidneys?

A

Very rare

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52
Q

What is an intrathoracic kidney?

A

When the kidney ascends to high in the body

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53
Q

How common is renal hypoplasia?

A

True form very rare

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54
Q

What is renal hypoplasia?

A

To few nephrons

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55
Q

Is renal hypoplasia is unilateral or bilateral?

A

Can be both

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56
Q

What does renal hypoplasia look like on u/s? 2

A
  1. Small kidney
  2. May be difficult to visualize
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57
Q

Are congenital megacalices obstructive?

A

No

58
Q

Congenital megacalices are unilateral or bilateral?

A

Typically unilateral

59
Q

How does congenital megacalices function?

A

Normally

60
Q

What does congenital megacalices look like? 2

A
  1. Normal parenchyma
  2. Enlarged clubbed calyces
61
Q

Individuals with congenital megacalices are at risk of what?

A

Kidney stone

62
Q

What is a congenital megaureter?

A

Functional ureter obstruction

63
Q

Where is the location of congenital megaureter?

A

Distal aperistalsis

64
Q

Which demographic does congenital megaureters affect?

A

Men>women

65
Q

Which side does congenital megaureters affect?

A

Lt > RT

66
Q

What can congenital megaureters lead to?

A

Hydronephrosis

67
Q

What does congenital megaureter look like on U/S?

A

Fusiform dilation of the distal third of the ureter

68
Q

What does UPJ obstructions appear with?

A

Hydronephrosis

69
Q

Which demographic is affected typically with UPJ obstruction?

A

Males>Females

70
Q

In terms of UPJ obstructions, which side does it usually occur on?

A

Left > right

71
Q

What is the most common neonatal palpable abdominal mass? (Where does it appear)

A

UPJ obstruction

72
Q

UPJ obstructions can be functional or anatomical?

A

Both

73
Q

UPJ obstruction can have an increased incidence in which two disorders?

A
  1. MCDK
  2. Contralateral renal agenesis
74
Q

What are some s/s of UPJ obstruction? 4

A
  1. Chronic, vague back pain
  2. Ballooning of the renal pelvis (Pelvicaliectasis)
  3. Cortical atrophy
  4. Ureter normal
75
Q

When does multiple renal artery’s occur?

A

Developmental

76
Q

What is multiple renal arteries a result of?

A

Failure of regression of renal vessels during ascent

77
Q

What is bladder extrophy?

A

Large anterior abdominal wall defect where the bladder protrudes out of the abdomen.

78
Q

Who’s is affected by exstrophy?

A

Males

79
Q

How common is bladder exstrophy?

A

Rare

80
Q

Who is affected by hypospadia?

A

Males

81
Q

In terms of hypospaid, what do we need to do?

A

Check kidneys for anomalies

82
Q

What is bladder hypospadia?

A

Urethra opens posteriorly, not centrally

83
Q

What is bladder outlet obstruction? What does it cause?

A
  1. Obstruction
  2. Hydronephrosis
84
Q

Besides hydroneprhosis, What are some potential causes of bladder outlet obstruction? 3

A
  1. Neurogenic
  2. Tumours
  3. Congenital malformations?
85
Q

Posterior urethral valves are most common in which demographic?

A

Males

86
Q

What are posterior urethral valves?

A

Flap of mucosa obstruction outlet

87
Q

What does posterior urethral valves look like? 4

A
  1. Palpable flam mass/ failure to thrive
  2. Thick walled bladder, dilated posterior urethra (keyhole sign)
  3. Dilated ureters
  4. Hydroneprhosis
88
Q

What are urachal anomalies?

A

When the bladder descends into the true pelvis a urachal formed

89
Q

Where is urachal anomalies located in a fetus?

A

Bladder located at umbilicus

90
Q

How many types of urachal anomalies are there?

A

Four types

91
Q

Urachal Anomalies usually do what?

A

Obliterate

92
Q

What are the four types of urachal anomalies?

A
  1. Patent (Most common)
  2. Urachal cyst
  3. Urachal sinus
  4. Diverticulum
93
Q

What happens to the urachal normally?

A

Obliterates

94
Q

What is situs inversus?

A

Organs in the abdominal cavity reversed

95
Q

What does the organs look like with situs inversus? 3

A
  1. Liver in left hypochondrium
  2. Spleen on right
  3. Orientation of heart reversed
96
Q

What are vascular variations?

A

Variations in hepatic vein branching

97
Q

What is something we see with vascular variations? 3

A
  1. Accessory hepatic veins
  2. Replaced hepatic artery
  3. Extra renal arteries
98
Q

What does the hepatic artery branch from in terms of vascular variation? 2

A
  1. Left gastric artery
  2. SMA
99
Q

What is the prognosis of complete organ agenesis?

A

Incompatible with life

100
Q

What are some organs typically affected by complete organ agenesis? 3

A
  1. Liver
  2. Adrenal (bilateral)
  3. Kidneys (bilateral)
101
Q

How often does complete organ agensis affect GB? What is the prognosis?

A

Rare and not life threatening

102
Q

How common is complete organ agenesis of the bladder? What is the prognosis? 2

A
  1. Rare
  2. Stillborn or anomalies
103
Q

Where are some common areas for partial agenesis? 3

A
  1. Liver
  2. Pancreas
  3. One kidney or Adrenal gland
104
Q

What is seen with partial organ agensis?

A

Compensatory hypertrophy

105
Q

How common is biliary atresia?

A

Rare

106
Q

What is biliary atresia?

A

Ducts from liver hilum to duodenum obliterated

107
Q

How is biliary atresia treated?

A

Surgically

108
Q

What might patients with biliary atresia have when born?

A

Jaundice 2-3 weeks after birth

109
Q

What is seen with biliary atresia in terms of the GB and the CBD? 2

A
  1. Small or absent GB
  2. Non visualization of the CBD
110
Q

What are common duplication of organs? 5

A
  1. Multiple hepatic ducts
  2. Duplicated renal collection system
  3. Gallbladder (only one functions)
  4. Pancreatic tail (Larger than normal)
  5. Accessory adrenal glands
111
Q

If there is accessory duplication of the adrenal glands, where are they located? Is it easily found on u/s?

A
  1. Located anywhere in the body
  2. Undectable u/s
112
Q

What are 5 examples of ectopic organs?

A
  1. Kidneys
  2. Urethral insertion
  3. Gallbladder
  4. Pancreatic tissue
  5. Accessory spleens
113
Q

What are pancreatic anomalies? 2

A
  1. Annular
  2. Pancreatic division
114
Q

How common is annular pancreatic anomalies?

A

Rare

115
Q

Who is most commonly affected by annular pancreatic anomalies ?

A

Males> females

116
Q

What does annular pancreatic anomalies look like?

A

Heads surrounds the 2nd part duodenum

117
Q

What is pancreatic division?

A

Dorsal and ventral buds do not fuse therefore we have two separate ducts

118
Q

Pancreatic divisum usually results in what?

A

Pancreatitis

119
Q

What are two adrenal gland anomalies?

A
  1. Congenital hypoplasia
  2. Congenital hyperplasia
120
Q

What is Congential hypoplasia of adrenal glands?

A

When hormone production altered

121
Q

Who is most likely affected by adrenal congenital hypoplasia?

A

Mostly Male

122
Q

What do we see with congenital hypoplasia of the adrenal glands?

A

Hypogonadism

123
Q

What is congenital hyperplasia of adrenal glands?

A

Interferes with cortisol and aldosterone production

124
Q

Congenital hyperplasia of adrenal gland are autosomal what?

A

Recessive

125
Q

Who is most likely affected by congenital hyperplasia of the adrenal glands?

A

Females

126
Q

What is a s/s of congenital hyperplasia?

A

Precocious puberty

127
Q

What does congenital hyperplasia of the adrenal gland looks like on u/s?

A

Normal or diffusely enlarged

128
Q

What are examples of splenic variants? 2

A
  1. Accessory spleen
  2. Splenunculi
129
Q

Where are splenic variants located?

A

At splenic hilum

130
Q

What splenic variants texture and echogenicity?

A

Normal

131
Q

What is a born again spleen?

A

Hypertrophy of a accessory spleen or other splenic tissue post splenectomy

132
Q

What is a wandering spleen? What is it prone to? 2

A
  1. Long mesentery
  2. Prone to torsion
133
Q

What is congenital splenic abnormality part of?

A

Part of spectrum of anomalies known as visceral heterotaxy

134
Q

What happens with asplenia? 3

A

Dominant right side organs
1. Midline liver
2. GU/GI tract anomalies
3. Complex cardiac malformation

135
Q

What happens with polyspenia? 3

A

Dominant left sided organs
1. Biliary atresia
2. Absense of GB
3. GI abnormalities

136
Q

What is hypertrophic pyloric stenosis? 2

A
  1. Congenital narrowing of the pylorus
  2. Hypertrophy of the pyloric muscle
137
Q

Who is usually affected by hypertrophy pyloric stenosis?

A

Males > females

138
Q

When does hypertrophy pyloric stenosis present?

A

First 2 months of life

139
Q

What are hypertrophic pyloric stenosis s/s? 4

A
  1. Projective vomiting
  2. Palpable, olive shaped mass
  3. Dehydration
  4. Weight loss, failure to thrive
140
Q

What is the technique for finding HPS? 3

A
  1. Supine and RLD
  2. Long and short axis
  3. Evaluate real time motion
141
Q

What does HPS look like on u/s? 3

A
  1. Donut sign
  2. Muscle wall thickness 4mm or greater
  3. Pylorus canal length >16mm
142
Q

What is Bezoars? 3

A
  1. Masses of foreign material in the stomach
  2. Hair
  3. Inspissated milk in an infant

DMST ABD 3 (11 decks)

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