Congenital Flashcards

1
Q

What is a variant?

A

Non-pathological variation of anatomy

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2
Q

What is an anomaly?

A

Major or minor abnormality of anatomy that is more probable to interfere with function

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3
Q

What is an HCB?

A

Hypertrophied Column of Bertin

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4
Q

Which urinary tract variant consists of a double layer of cortex between medullary pyramids?

A

HCB

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5
Q

Where does a hypertrophied column of bertin indent the renal sinus and where is it located?

A

Indents Laterally

Located in the upper or middle portion of kidney

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6
Q

An HCB should not be greater than what measurement in any dimension?

A

> 3cm

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7
Q

A junctional parenchymal defect is:

  1. Due to what?
  2. How does it appear?
  3. Where is it seen more often?
A

Incomplete embryological fusion

Hyperechoic wedge that can be confused with scarring

Seen more on right only because we see the RT KID better

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8
Q

Where is an extra renal pelvis located?

A

Medial to the renal sinus

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9
Q

Patients with an extra renal pelvis may be more prone to what?

A

Hydronephrosis (that can decompress when put in prone position)

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10
Q

A dromedary hump involves which aspect of the left kidney?

A

Lateral aspect

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11
Q

What is important to visualize in order to differentiate a dromedary hump from a mass?

A

CM junction

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12
Q

What is fetal lobulation?

A

Infolding of the cortex with no thinning, scalloped contour

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13
Q

What percentage of adult kidneys have some persistent lobulation?

A

51%

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14
Q

What is renal sinus lipomatosis?

A

Excessive fatty infiltration of the renal sinus

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15
Q

What is the most common congenital anomaly of the UT?

A

Duplicated collecting system

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16
Q

What are the two kinds of duplicated collecting systems?

A

Complete and incomplete

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17
Q

With complete duplicated collecting systems the superior ureter will have what kind of insertion?

A

Ectopic bladder insertion

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18
Q

What happens with an incomplete duplicated collecting system?

A

Duplicated ureters join before inserting into the bladder

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19
Q

What is a duplicated collecting system associated with? (4)

A

UPJ obstruction, uterus didelphys, reflux, and ureterocele

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20
Q

What is a ureterocele?

A

Cystic dilatation of the terminal ureter which protrudes into the urinary bladder

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21
Q

What do patients with ureteroceles have a high incidence of?

A

UTI’s and obstruction

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22
Q

What is a horseshoe kidney?

A

Fusion of the kidneys at the lower poles

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23
Q

Horseshoe kidneys can lead to an increased risk for what?

A

Infection and stone formation

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24
Q

What is important to visualize in order to confirm the presence of a horseshoe kidney?

A

Renal tissue that connects the lower poles (Isthmus)

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25
Q

Why does an ectopic kidney occur?

A

Kidney fails to ascend in utero, typically located in the pelvis unilaterally

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26
Q

Is diffuse or focal compensatory hypertrophy typically seen?

A

Diffuse

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27
Q

What may an enlarged normal kidney be caused by?

A

Unilateral renal agenesis or nephrectomy

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28
Q

What is supernumerary kidney?

A

Very rare small extra kidney with its own blood supply

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29
Q

How is hypoplasia different than atrophy of the kidney?

A

Hypoplasia occurs from Day 1, whereas with atrophy the kidney begins normal and then ceases to function, thus atrophying

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30
Q

A true hypoplastic kidney is very rare and may be unilateral or bilateral. True or false?

A

TRUE

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31
Q

What are enlarged clubbed calyces of the kidney known as?

A

Congenital megacalices

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32
Q

What is a functional obstruction of the ureter due to distal aperistalsis?

A

Congenital megaureter

33
Q

Who does congenital megaureter affect more and on what side?

And how does it appear on US?

A

Males, left

Fusiform dilation of distal third of ureter

34
Q

What is a UPJ obstruction?

A

Ureteropelvic junction obstruction

35
Q

What is the most common palpable mass of the neonatal abdomen?

A

UPJ obstruction

36
Q

With UPJ obstruction there is increased incidence of which two things?

A

Contralateral MCKD and contralateral renal agenesis

37
Q

What would you see sonographically with a UPJ obstruction?

A

Ballooning of renal pelvis

38
Q

What can appear the same as an ectopic kidney although it isn’t congential? (And what is it due to?)

A

Ptosis of the kidney

Prolapse of kidney due to trauma that causes tearing

39
Q

What is a developmental anomaly that is failure of regression of the renal vessels during ascent?

A

Multiple renal arteries

40
Q

What bladder anomaly is more common in males and presents as a large anterior abdominal wall defect?

A

Exstrophy

41
Q

What is it called when the urethra opens posteriorly, not centrally on the penis?

A

Hypospadia

42
Q

What should you check for if your male patient has hypospadia?

A

Renal anomalies

43
Q

What does bladder outlet obstruction cause?

A

Bilateral hydronephrosis

44
Q

What are potential causes of bladder outlet obstruction? (3)

A

Neurogenic bladder, tumors, and congenital malformations

45
Q

A flap of mucosa that obstructs the bladder outlet is a congenital malformation causing bladder outlet obstruction known as what? (And how does it appear on US?)

A

Posterior urethral valves

US = Dilated posterior urethra (keyhole sign) with dilated ureters, hydro and thick bladder wall

46
Q

What are the 4 types of urachal anomalies?

A

Patent, urachal cyst, urachal sinus, and diverticulum

47
Q

What is the most common type of urachal anomaly?

A

Patent

48
Q

Who does urachal anomalies affect more?

A

Men

49
Q

What is situs inversus?

A

Organs of the abdominal cavity are reversed

50
Q

What would be an example of a replaced hepatic artery?

A

Hepatic artery branching from the left gastric artery or SMA instead of the celiac axis

51
Q

The complete agenesis of which organs are not compatible with life?

A

Liver, bilateral adrenals and bilateral kidneys

52
Q

If a fetus has agenesis of the bladder what may happen?

A

Stillborn or have multiple associated abnormalities

53
Q

What will you often see with partial agenesis?

A

Compensatory hypertrophy

54
Q

Bile ducts from the hilum of the liver to the duodenum being obliterated refers to what rare condition?

A

Biliary atresia

55
Q

If the pancreatic tail seems longer than normal, what may the patient have?

A

Duplication of the pancreas

56
Q

Where will an ectopic GB be seen?

A

Embedded in the liver tissue

57
Q

What pancreatic anomaly is consistent with the pancreatic head surrounding the second part of the duodenum?

A

Annular

58
Q

Who does annular pancreas affect more?

A

Males

59
Q

What is the most common pancreatic variant?

A

Pancreatic divisum

60
Q

Which pancreatic anomaly is prone to pancreatitis?

A

Pancreatic divisum

61
Q

Which adrenal gland abnormality is associated with hypogonadism?

A

Congenital hypoplasia

62
Q

Adrenogenital syndrome is also known as what?

A

Congenital hyperplasia

63
Q

What is an autosomal recessive disorder that interferes with the production of cortisol and aldosterone?

A

Adrenogenital syndrome

64
Q

What is the sonographic appearance of congenital hyperplasia?

A

Adrenals may appear normal or diffusely enlarged

65
Q

What are three splenic variants?

A

Accessory spleen, born again spleen, and wandering spleen

66
Q

Congenital splenic abnormalities are part of a spectrum of anomalies known as what?

A

Visceral heterotaxy

67
Q

Asplenia refers to what?

A

Dominant right side organs

68
Q

Polysplenia refers to what?

A

Dominant left side organs

69
Q

What may also be present with asplenia?

A

Midline liver, GU/GI tract anomalies, complex cardiac malformations

70
Q

What may also be present with polysplenia?

A

Biliary atresia, absence of the gallbladder, and GI abnormalities

71
Q

What does HPS stand for?

A

Hypertrophic Pyloric Stenosis

72
Q

What is the classic sonographic appearance of HPS?

A

Doughnut sign

73
Q

Muscle thickness of what measurement is consistent with HPS?

A

3mm or greater

74
Q

Pyloric canal length of what measurement is consistent with HPS?

A

15mm or greater

75
Q

What are bezoars?

A

Mass of foreign material in the stomach, most commonly hair

76
Q

What is another name for accessory spleen?

A

Splenunculi

77
Q

What is a born again spleen?

A

Hypertrophy of an accessory spleen or other splenic tissue post splenectomy

78
Q

What is a wandering spleen?

A

Spleen with long mesentery that is prone to torsion