peds Flashcards

1
Q

schizencephaly

A

CSF fluid filled clefts in the grey matter leading from the ventricles to the surface of the cortex; lined by GM.
may be bilateral in up to 50%
- open or closed.
- polymicrogyria

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2
Q

relationship between SNR and voxel size?

A

linear -> SNR increases with voxel volume.
SNR increases by sqrt of measurements (# phase encoding steps, # excitations) and decreases with sqrt of receive bandwidth

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3
Q

effect of decreasing matrix size and increasing slice thickness on voxel size and SNR?

A

increase voxel volume and SNR

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4
Q

Imaging time for a single slice is ?

A

TR x number of phase encoding steps x averages or number of excitations.

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5
Q

decreasing matrix size in phase encoding direction will also affect imaging time for a single slice how?

A

decrease time

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6
Q

pediatric UTI - % with duplication?

A

quoted as 25-40%

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7
Q

Hutch diverticulum

A

congenital bladder diverticulum at the VUJ, in absence of posterior valves

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8
Q

VUR demographics

A

retrograde flow of urine from bladder ot ureter, 1-3% of kids, 40-50% of kids with UTI
more common in caucasians, M>F in newborns, then 5-6x F>M after age 1

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9
Q

VUR etiologies

A

primary (ectopic ureters, lack/short intravesicular ureteric course, detrusor muscle abnormality)
secondary: cystitis/UTI, bladder diverticulum, calculi)

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10
Q

grading VUR

A

1: ureter only
2: reflux to renal pelvis, no dilation/blunting
3: mild dilation of ureters/calyces
4: moderate dilation, tortuosity, blunting of calcyces
5: severe dilation and tortuosity

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11
Q

spinning top urethra

A

seen in young children/girls
- dilated posterior urethra resulting from increased bladder pressure against closed sphincter
- bladder retraining needed

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12
Q

ureterocele

A

congenital abnormal dilation of the distal ureter. intra (at UVJ) or extra-vesciular( below/medial to expected UVJ)
appears as cystic intravesciular mass near UVJ/early filling defect posteriorly
- 5-7X F>M, up to 10% bilateral
Think: duplication

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13
Q

Weigert-Meyer law

A

describes the relationship of renal moieties to drainage in a completely duplicated system
- upper: ectopic ureter (medial inferior), often ending in ureterocele, and usually obstructed
- lower: normal ureter insertion, reflux

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14
Q

mesoblastic nephroma

A

aka fetal renal hamartoma
- most common congenital renal mass
- solid unencapsulated mass, usually near the renal hilum, tends to invade renal parenchyma and surrounding structures
- classic and cellular subtypes (and mixed)

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15
Q

DDx for infant retroperitoneal mass

A

mesoblastic nephroma
MCDK
Wilms
Rhabdoid tumor
retroperitoneal sarcoma
teratoma
neuroblastoma
extrapulmonary sequestration

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16
Q

MCDK

A

multiple renal cysts of variable size, with thinning and fibrotic changes of the renal parenchyma
due to oibstruction of the UPJ
- usually prenatal Dx
- unilateral, non-functional kidney

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17
Q

Wilm’s tumor

A

aka nephroblastoma
most common pediatric renal malignancy and abdominal CA
- slight F>M, usually <5yo

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18
Q

what are nephrogenic rests?

A

persistent metanephric tissue, usually regress during childhood
- found in up to 100% of bilateral Wilm’s

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19
Q

nephroblastomatosis

A
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20
Q

single renal mass <1yo

A

mesopblastic nephroma
wilms
rhabdoid

21
Q

single renal mass in 1-10yo

A

wilms
rhabdoid (rare, 2% peds malignancies)
clear cell sarcoma (rare younger than 6 mo)

22
Q

omphalocele vs gastroschisis

A

OM: midline abdominal wall defect with herniation covered by peritoneum/membrane with the umbilical cord inserting on it. 50-70% have associated anomalies (genetic syndromes),. prognosis determined by other anomalies.
GS: abdominal wall defect (right of midline) without membrane covering herniated bowel. usually isolated. prognosis determined by bowel condition

23
Q

pentalogy of Cantrell

A

classic 5 malformations:
1. midline abdo defect
2. lower sternal defect/cleft
3. anterior diaphragmatic defect
4. defect of the diaphragmatic pericardium
5. intracardiac defect

Other
- omphalocele
- cardiac position outside the chest, or rotated with apex pointing towards the chin,
other cardiac anomalies (septum, tetralogy, ebstein, LV diverticulum, effusions)

24
Q

best clue for pentalogy of Cantrell?

A

ectopia cordis + omphalocele

25
Best clue for body stalk abnormality/limb-body-wall-complex
abdo wall defect + scoliosis
26
Body stalk abnormality
rare malformation, major abdo wall defect with organs (liver) develop outside the abdominal cavity/attached to placenta, severe kyphoscoliosis, and short/absent UC. +/- - craniofacial and vertebral anomalies - renal abnormalities - limb abnormalities - bladder exstrophy
27
cloacal exstrophy
absence of normal bladder, anal atresia (lack normal anal dimple), low abdo wall defect may have bifid scrotum/penis/clitoris - look for renal abnormalities, spine anomalies, spinal bifida
28
when should soft markers be applied?
18-22 wks. Not before 16 and not after 24
29
strong 'soft' marker
thick NF absent nasal bone in caucasians
30
moderate 'soft' markers
absent nasal bone in non-caucasians mild lateral ventriculomegaly bowel echogenicity > bone
31
weak 'soft' markers
intracardiac echogenic focus (brighter than bone) renal pelvis >4mm short femur <2.5th centile short nasal bone incomplete chorion and amnion membrane separation hypercoiled umbilical cord
32
isolated single umbilical artery is a marker for?
other structural anomalies - recommend level 2 and follow up growth scans advised *not aneuploidy marker
33
choroid plexus cysts are RF for?
Edward syndrome - increased LR by factor of 7X however, isolated CPCs with no other structural abnormality very rare to have Tri18
34
holoprosencephaly is classic for ? trisomy
13 (seen in 75%) if isolated abnormality though, only 4% incidence of chromosome abnormality
35
fetal skeletal dysplasia definition
heterogenous group of disorders related to the abN development/growth/maintenance of bone and cartilagenous tissues - some are idiopathic, others genetic - some are lethal
36
top 4 common skeletal dysplasias
achondrogenesis Osteogenesis Imperfecta thanatophoric dysplasia achrondroplasia
37
features suspicious for skel dysplasia
growth deficiency bowing/short long bones vertebral defects rib defects multiple # abnormal cranial shape
38
role of U/S for skel dysplasias
identification and narrowing of DDx assessment of the involved bones/organs predict lethality
39
U/S markers predictive of lethality in skel dysplasia
Most linked to the chest, including: thoracic circumference <5th %ile at the level of 4chamber view thorax:abdo circumference ratio <0.6 short thorax ribs <70% of thoracic circumference narrow AP on sag, concave/bell shaped thorax
40
Ectrodactyly
condition characterized by absence or malformation of one or more of the fingers or toes
41
clinodactyly
fingers curved/bent to one side
42
micromelia
shortening of the entire limb
43
rhizomelia
shortening of proximal segment of limb
44
mesomelia
shortening of the intermediate aspect of the limb
45
acromelia
shortening of the extremities involving the distal limb
46
thanatophoric dysplasia
lethal skel dysplasia often abnormal skull shape & macrocranium, small thorax normal bone mineralization, no fractures
47
hypomineralization in skeletal dysplasia
OI T2, achondrogenesis, hypophosphatasia
48
another name for Sturge Weber
encephalotrigeminal angiomatosis
49
sturge-weber signs
2/2 failure of cortical vein development -> congestion and infarcts. - tram-track gyral calcs, volume loss, enlarged choroid plexi (ipsilateral) - facial nevus flammus/port-wine - choroidal angioma, buphthalmos, retinal telangiectatic vessels, scleral angioma, and iris heterochromia