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Abd / SS 2 > Module 6 : Congenital > Flashcards

Module 6 : Congenital Flashcards

(102 cards)

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1
Q

what is a variation

A
  • variation of anatomy
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2
Q

is a varient pathological what could it interfere with

A
  • no pathological

- could interfere with function

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3
Q

are variants and anomalies congenital or developmental

A
  • both
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4
Q

can anomalies be major or minor

A
  • yes
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5
Q

what are 4 different areas where there could be anomalies

A
  • number
  • size
  • positon
  • structure
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6
Q

are anomalies or variants more common to interfere with function

A
  • anomalies
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7
Q

what are the 15 anomalies of the urinary tract

A
  • hypertrophied column of Bertin
  • junctional parenchymal defect
  • extra renal pelvis
  • dromedary hump
  • fetal lobulation
  • renal sinus lipomatosis
  • duplicated collecting system
  • ureteroceles
  • horse shoe kidney
  • ectopic kidney
  • compensatory hypertrophy
  • supernumerary kidney
  • hypoplasia
  • congenital megacalices
  • congenital megaureter
  • ureteropelvic obstruction
  • ptosis
  • multiple renal arteries
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8
Q

what is a hypertrophied column of Berlin (HCB)

A
  • double layer of cortex between medullary pyramids
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9
Q

where is a HCB commonly located

A
  • upper or middle portion of the kidney
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10
Q

how does a HCB indent the sinus

A
  • laterally
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11
Q

what is the upper limit of normal for an HCB

A

< 3cm

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12
Q

US appearance of HCB

A
  • isoechoic and continuous with renal cortex
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13
Q

what causes a junctional parenchymal defect

A
  • incomplete embryological fusion
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14
Q

what kidney is junctional parenchymal defect more commonly identified

A
  • right

- liver gives us a good window to see them

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15
Q

US look of junctional parenchymal defects

A
  • hyperechoic wedge shape area

- can be confused with scarring

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16
Q

where is an extra Renal pelvis (ERP) located

A
  • medial to renal sinus
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17
Q

what might an ERP mimic and what can patients be more prone to

A
  • hydro

- hydro

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18
Q

can ERP decompress

A
  • yes when patient in prone
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19
Q

where is a dromedary hump most commonly seen

A
  • lateral aspect of left kidney
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20
Q

what is a dromedary hump

A
  • thickening or bulging of the cortex
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21
Q

what is important to visualize to diagnose a dromedary hump vs a mass

A
  • CM junction

- will have pyramids

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22
Q

what is fetal lobulation

A
  • infolding of the cortex with no thinning
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23
Q

what will the outer contour of the kidney look like with fetal lobulation

A
  • scalloped cortex
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24
Q

is fetal lobulation common or uncommon

A
  • more common than not
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25
what is renal sinus lipomatosis
- excessive fatty infiltration of the renal sinus
26
what 2 things is renal sinus lipomatosis associated with
- aging | - obesity
27
US look of renal sinus lipomatosis
- enlarged echogenic sinus
28
what is the most common anomaly of the UT
- duplicated collecting system
29
what is a duplicated collecting system `
- variable duplication of the renal sinus
30
what are the 2 types of duplicated collecting system
- complete | - incomplete
31
what is complete duplicated collecting system
- 2 collection systems - 2 ureters - superior ureter will have ECTOPIC bladder insertion
32
what is incomplete duplicated collecting system
- duplicated ureters join before inserting into the bladder | - Y shaped ureter
33
what 4 things is duplicated collecting system associated with
- UPJ obstruction - uterus didelphys - reflux - ureterocele
34
US look of duplicated collecting system
- kidney longer than normal - 2 separate sinus regions - cortex through entire centre of the kidney
35
what is a ureterocele
- cystic dilation of th eternal ureter which protrudes in the urinary bladder
36
is ureteroceles congenital or acquired
- can be both
37
what other disorders will patients with ureteroceles commonly have
- UTIs | - obstruction
38
what is a horseshoe kidney
- fusion of the kidney at lower poles
39
what leads to an increased risk for infection and stone formation with horseshoe kidney
- malrotation | - associated UPJ obstruction
40
US look of horseshoe kidney
- difficult to identify lower pole - lower position in abdomen - inferior pole situated more medially - renal tissue drapes ANTERIOR to great vessels (isthmus)
41
what is an ectopic kidney
- failure of kidney to ascend in utero
42
where is the kidney usually located with ectopic kidney
- pelvis and unilateral
43
what does the ectopic kidney usually look like
- small and abnormally rotated
44
what is crossed ectopia
- both kidneys located on one side | - upper pole of ectopic kidney usually fused to the lower pole of normal kidney
45
what is compensatory hypertrophy
- tissue/organ expands in volume
46
is compensatory hypertrophy diffuse or focal
- DIFFUSE sometimes focal
47
what 2 factors will cause a normal kidney to enlarge
- unilateral kidney agenisis | - nephrectomy
48
what is a supernumerary kidney
- very rare | - small extra kidney with its own blood supply
49
is a true hypoplastic kidney common or rare
- rare
50
what is a hypoplastic kidney
- contains two few nephrons
51
is hypoplastic kidney unilateral or bilateral
- both
52
US look of hypoplastic kidney
- small kidney | - difficult to visualize
53
what are the characteristics of congenital megacalices
- non obstructive - unilateral - normal function and parenchyma - enlarged clubbed calyces - increased risk of kidney stones
54
characteristics of congenital megaureter
- functional obstruction of ureter due to distal peristalsis - men - left - may lead to hydro - fusiform dilation of the distal third of the ureter
55
characteristics of ureteropelvic obstruction
- results in hydro - males - left - caused by functional or anatomical anomaly - increased incidence of MCKD and contralateral renal agenisis -
56
what is the most common palpable mass of the neonatal abdomen
- UPJ obstruction
57
US look of UPJ obstruction
- ballooning of renal pelvis - cortical atrophy - ureter normal
58
characteristics of ptosis
- renal fascia tears | - prolapse of kidney
59
characteristics of multiple renal arteries
- developmental anomaly's | - failure of regression of renal vessels during ascent
60
what are 3 bladder anomalies
- exstrophy - hypospadia - bladder outlet obstruction
61
is bladder exstrophy common or rare what gender is it more common in
- rare | - men
62
what is bladder exstrophy
- large anterior abdominal wall defect
63
what gender is effected by hypospadias
- males
64
what is hypospadias
- urethra opens posteriorly not centrally | - check for renal anomaly's
65
what is bladder outlet obstruction and what does it cause
- obstruction of bladder outlet | - hydro
66
what are the 3 potential causes of bladder outlet obstruction
- neurogenic bladder - tumors - congenital malformations (posterior urethral valves)
67
what is a posterior urethral valve and who is it most common in
- flap of mucosa obstructs the bladder outlet | - males
68
what do patients with posterior urethral valves present with
- palpable flank mass and failure to thrive
69
US look of PUV
- thick walled bladder - key hole appearance - dilated ureters/hydro
70
what are the 4 urachal anomalies
- patent - urachal cyst - urachal sinus - diverticulum
71
what is the most common urachal anomaly
- patent | - associated with urethral obstruction
72
which gender is affected more by urachal anomalies
- men
73
US look of urachal anomalies
- anechoic mass between the bladder and umbilicus
74
characteristics of situs inversus
- organ of abdominal cavity reversed - liver located on the left - spleen right - orientation of heart reversed
75
what are the 5 vascular variations of the abdomen
- accessory hepatic vein - variations in hepatic branching - hepatic artery branching from (LGS SMA) - extra renal arteries
76
what are the 3 types of organ agenisis
- complete - partial - biliary atresia
77
what three complete organ agenisis is incompatible with life
- liver - adrenal (bilateral) - kidney (bilateral)
78
is GB agenisis common or rare or life threatening
- rare | - not life threatening
79
is bladder agenisis rare and what is it associated with
- rare | - still born or multiple other abnormalities
80
with what organs partial agencies would compensatory hypertrophy show
- liver - pancrease - unilateral kidney or adrenal
81
what is biliary atresia
- bile ducts from hilum of liver enter to the duodenum are obliterated - rare - treated surgically
82
what 6 structures may be duplicated
- multiple hepatic ducts - duplicated renal collecting system - bladder - gallbladder - panc tail - accessory adrenal glands
83
what 5 organs can be ectopic
- kidneys - ureteral insertions - GB - panc tissue - accessory spleens
84
what are the 2 pancreatic anomalies
- annular | - pancreatic divisum
85
characteristics of annular panc
- rare - panc head surrounds second part of duodenum - males
86
what is the most common panc variant
pancreatic divisum
87
characteristics of panc divisum
- dorsal and ventral buds do not fuse - 2 separate ducts - prone to pancreatitis
88
what are 2 adrenal gland abnormalities
- congenital hypoplasia | - congenital hyperplasia (adrenogenital syndrome)
89
characteristics of adrenal hypoplasia
- hormone production altered - males - hypogonadism
90
characteristics of adrenal hyperplasia
- autosomal recessive - interferes with production of cortisol and aldosterone - females results in vitalization (more male) - precocious puberty - adrenals diffusely enlarged
91
what are 3 splenic variants
- accessory spleen - born again spleen - wandering spleen
92
what is an accessory spleen
- splenunculi - located at splenic hilum - same texture and echogenicty as spleen
93
what is a born again spleen
- hypertrophy of accessory spleen post splenectomy
94
what is a wandering spleen
- long mesentery (point of attachment) | - prone to torsion
95
what are congenital splenic abnormalities a part of and what are the 2 types
- spectrum of anomalies known as visceral heterotaxy | - asplenia, polysplenia
96
what is asplenia
- dominant right side organs - midline liver - GU/GI tract anomalies - compeer cardiac malformations
97
what is polysplenia
- dominant left side organs - biliary atresia - absence of GB - GI abnormalities
98
what is hypertrophic pyloric stenosis
- congenital narrowing of pylorus - hypertrophy of pyloric muscle - new born males
99
clinical presentation of HPS
- projectile vomitting - palpable abdominal mass - dehydration - weight loss, failure to thrive
100
US technique of HPS
- patient in supine or RLD - obtain long and short of pylorus - evaluate real time motion
101
US look of HPS
- doughnut sign + hypo echoic muscle mass with central hyperechoic lumen - thickness > 3mm HPS - pyloric canal length > 15mm consistent with HPS
102
what is bezoars
- mass of foreign material in the stomach usually hair

Abd / SS 2 (7 decks)

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