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Abd / SS 2 > Module 6 : Congenital > Flashcards

Module 6 : Congenital Flashcards

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
Q

what is renal sinus lipomatosis

A
  • excessive fatty infiltration of the renal sinus
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26
Q

what 2 things is renal sinus lipomatosis associated with

A
  • aging

- obesity

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27
Q

US look of renal sinus lipomatosis

A
  • enlarged echogenic sinus
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28
Q

what is the most common anomaly of the UT

A
  • duplicated collecting system
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29
Q

what is a duplicated collecting system `

A
  • variable duplication of the renal sinus
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30
Q

what are the 2 types of duplicated collecting system

A
  • complete

- incomplete

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31
Q

what is complete duplicated collecting system

A
  • 2 collection systems
  • 2 ureters
  • superior ureter will have ECTOPIC bladder insertion
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32
Q

what is incomplete duplicated collecting system

A
  • duplicated ureters join before inserting into the bladder

- Y shaped ureter

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33
Q

what 4 things is duplicated collecting system associated with

A
  • UPJ obstruction
  • uterus didelphys
  • reflux
  • ureterocele
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34
Q

US look of duplicated collecting system

A
  • kidney longer than normal
  • 2 separate sinus regions
  • cortex through entire centre of the kidney
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35
Q

what is a ureterocele

A
  • cystic dilation of th eternal ureter which protrudes in the urinary bladder
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36
Q

is ureteroceles congenital or acquired

A
  • can be both
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37
Q

what other disorders will patients with ureteroceles commonly have

A
  • UTIs

- obstruction

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38
Q

what is a horseshoe kidney

A
  • fusion of the kidney at lower poles
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39
Q

what leads to an increased risk for infection and stone formation with horseshoe kidney

A
  • malrotation

- associated UPJ obstruction

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40
Q

US look of horseshoe kidney

A
  • difficult to identify lower pole
  • lower position in abdomen
  • inferior pole situated more medially
  • renal tissue drapes ANTERIOR to great vessels (isthmus)
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41
Q

what is an ectopic kidney

A
  • failure of kidney to ascend in utero
42
Q

where is the kidney usually located with ectopic kidney

A
  • pelvis and unilateral
43
Q

what does the ectopic kidney usually look like

A
  • small and abnormally rotated
44
Q

what is crossed ectopia

A
  • both kidneys located on one side

- upper pole of ectopic kidney usually fused to the lower pole of normal kidney

45
Q

what is compensatory hypertrophy

A
  • tissue/organ expands in volume
46
Q

is compensatory hypertrophy diffuse or focal

A
  • DIFFUSE sometimes focal
47
Q

what 2 factors will cause a normal kidney to enlarge

A
  • unilateral kidney agenisis

- nephrectomy

48
Q

what is a supernumerary kidney

A
  • very rare

- small extra kidney with its own blood supply

49
Q

is a true hypoplastic kidney common or rare

A
  • rare
50
Q

what is a hypoplastic kidney

A
  • contains two few nephrons
51
Q

is hypoplastic kidney unilateral or bilateral

A
  • both
52
Q

US look of hypoplastic kidney

A
  • small kidney

- difficult to visualize

53
Q

what are the characteristics of congenital megacalices

A
  • non obstructive
  • unilateral
  • normal function and parenchyma
  • enlarged clubbed calyces
  • increased risk of kidney stones
54
Q

characteristics of congenital megaureter

A
  • functional obstruction of ureter due to distal peristalsis
  • men
  • left
  • may lead to hydro
  • fusiform dilation of the distal third of the ureter
55
Q

characteristics of ureteropelvic obstruction

A
  • results in hydro
  • males
  • left
  • caused by functional or anatomical anomaly
  • ## increased incidence of MCKD and contralateral renal agenisis
56
Q

what is the most common palpable mass of the neonatal abdomen

A
  • UPJ obstruction
57
Q

US look of UPJ obstruction

A
  • ballooning of renal pelvis
  • cortical atrophy
  • ureter normal
58
Q

characteristics of ptosis

A
  • renal fascia tears

- prolapse of kidney

59
Q

characteristics of multiple renal arteries

A
  • developmental anomaly’s

- failure of regression of renal vessels during ascent

60
Q

what are 3 bladder anomalies

A
  • exstrophy
  • hypospadia
  • bladder outlet obstruction
61
Q

is bladder exstrophy common or rare what gender is it more common in

A
  • rare

- men

62
Q

what is bladder exstrophy

A
  • large anterior abdominal wall defect
63
Q

what gender is effected by hypospadias

A
  • males
64
Q

what is hypospadias

A
  • urethra opens posteriorly not centrally

- check for renal anomaly’s

65
Q

what is bladder outlet obstruction and what does it cause

A
  • obstruction of bladder outlet

- hydro

66
Q

what are the 3 potential causes of bladder outlet obstruction

A
  • neurogenic bladder
  • tumors
  • congenital malformations (posterior urethral valves)
67
Q

what is a posterior urethral valve and who is it most common in

A
  • flap of mucosa obstructs the bladder outlet

- males

68
Q

what do patients with posterior urethral valves present with

A
  • palpable flank mass and failure to thrive
69
Q

US look of PUV

A
  • thick walled bladder
  • key hole appearance
  • dilated ureters/hydro
70
Q

what are the 4 urachal anomalies

A
  • patent
  • urachal cyst
  • urachal sinus
  • diverticulum
71
Q

what is the most common urachal anomaly

A
  • patent

- associated with urethral obstruction

72
Q

which gender is affected more by urachal anomalies

A
  • men
73
Q

US look of urachal anomalies

A
  • anechoic mass between the bladder and umbilicus
74
Q

characteristics of situs inversus

A
  • organ of abdominal cavity reversed
  • liver located on the left
  • spleen right
  • orientation of heart reversed
75
Q

what are the 5 vascular variations of the abdomen

A
  • accessory hepatic vein
  • variations in hepatic branching
  • hepatic artery branching from (LGS SMA)
  • extra renal arteries
76
Q

what are the 3 types of organ agenisis

A
  • complete
  • partial
  • biliary atresia
77
Q

what three complete organ agenisis is incompatible with life

A
  • liver
  • adrenal (bilateral)
  • kidney (bilateral)
78
Q

is GB agenisis common or rare or life threatening

A
  • rare

- not life threatening

79
Q

is bladder agenisis rare and what is it associated with

A
  • rare

- still born or multiple other abnormalities

80
Q

with what organs partial agencies would compensatory hypertrophy show

A
  • liver
  • pancrease
  • unilateral kidney or adrenal
81
Q

what is biliary atresia

A
  • bile ducts from hilum of liver enter to the duodenum are obliterated
  • rare
  • treated surgically
82
Q

what 6 structures may be duplicated

A
  • multiple hepatic ducts
  • duplicated renal collecting system
  • bladder
  • gallbladder
  • panc tail
  • accessory adrenal glands
83
Q

what 5 organs can be ectopic

A
  • kidneys
  • ureteral insertions
  • GB
  • panc tissue
  • accessory spleens
84
Q

what are the 2 pancreatic anomalies

A
  • annular

- pancreatic divisum

85
Q

characteristics of annular panc

A
  • rare
  • panc head surrounds second part of duodenum
  • males
86
Q

what is the most common panc variant

A

pancreatic divisum

87
Q

characteristics of panc divisum

A
  • dorsal and ventral buds do not fuse
  • 2 separate ducts
  • prone to pancreatitis
88
Q

what are 2 adrenal gland abnormalities

A
  • congenital hypoplasia

- congenital hyperplasia (adrenogenital syndrome)

89
Q

characteristics of adrenal hypoplasia

A
  • hormone production altered
  • males
  • hypogonadism
90
Q

characteristics of adrenal hyperplasia

A
  • autosomal recessive
  • interferes with production of cortisol and aldosterone
  • females results in vitalization (more male)
  • precocious puberty
  • adrenals diffusely enlarged
91
Q

what are 3 splenic variants

A
  • accessory spleen
  • born again spleen
  • wandering spleen
92
Q

what is an accessory spleen

A
  • splenunculi
  • located at splenic hilum
  • same texture and echogenicty as spleen
93
Q

what is a born again spleen

A
  • hypertrophy of accessory spleen post splenectomy
94
Q

what is a wandering spleen

A
  • long mesentery (point of attachment)

- prone to torsion

95
Q

what are congenital splenic abnormalities a part of and what are the 2 types

A
  • spectrum of anomalies known as visceral heterotaxy

- asplenia, polysplenia

96
Q

what is asplenia

A
  • dominant right side organs
  • midline liver
  • GU/GI tract anomalies
  • compeer cardiac malformations
97
Q

what is polysplenia

A
  • dominant left side organs
  • biliary atresia
  • absence of GB
  • GI abnormalities
98
Q

what is hypertrophic pyloric stenosis

A
  • congenital narrowing of pylorus
  • hypertrophy of pyloric muscle
  • new born males
99
Q

clinical presentation of HPS

A
  • projectile vomitting
  • palpable abdominal mass
  • dehydration
  • weight loss, failure to thrive
100
Q

US technique of HPS

A
  • patient in supine or RLD
  • obtain long and short of pylorus
  • evaluate real time motion
101
Q

US look of HPS

A
  • doughnut sign
    + hypo echoic muscle mass with central hyperechoic lumen
  • thickness > 3mm HPS
  • pyloric canal length > 15mm consistent with HPS
102
Q

what is bezoars

A
  • mass of foreign material in the stomach usually hair

Abd / SS 2 (7 decks)

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