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Case review > peds > Flashcards

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
Q

Best clue for body stalk abnormality/limb-body-wall-complex

A

abdo wall defect + scoliosis

26
Q

Body stalk abnormality

A

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
Q

cloacal exstrophy

A

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
Q

when should soft markers be applied?

A

18-22 wks. Not before 16 and not after 24

29
Q

strong ‘soft’ marker

A

thick NF
absent nasal bone in caucasians

30
Q

moderate ‘soft’ markers

A

absent nasal bone in non-caucasians
mild lateral ventriculomegaly
bowel echogenicity > bone

31
Q

weak ‘soft’ markers

A

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
Q

isolated single umbilical artery is a marker for?

A

other structural anomalies - recommend level 2 and follow up growth scans advised
*not aneuploidy marker

33
Q

choroid plexus cysts are RF for?

A

Edward syndrome - increased LR by factor of 7X
however, isolated CPCs with no other structural abnormality very rare to have Tri18

34
Q

holoprosencephaly is classic for ? trisomy

A

13 (seen in 75%)
if isolated abnormality though, only 4% incidence of chromosome abnormality

35
Q

fetal skeletal dysplasia definition

A

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
Q

top 4 common skeletal dysplasias

A

achondrogenesis
Osteogenesis Imperfecta
thanatophoric dysplasia
achrondroplasia

37
Q

features suspicious for skel dysplasia

A

growth deficiency
bowing/short long bones
vertebral defects
rib defects
multiple #
abnormal cranial shape

38
Q

role of U/S for skel dysplasias

A

identification and narrowing of DDx
assessment of the involved bones/organs
predict lethality

39
Q

U/S markers predictive of lethality in skel dysplasia

A

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
Q

Ectrodactyly

A

condition characterized by absence or malformation of one or more of the fingers or toes

41
Q

clinodactyly

A

fingers curved/bent to one side

42
Q

micromelia

A

shortening of the entire limb

43
Q

rhizomelia

A

shortening of proximal segment of limb

44
Q

mesomelia

A

shortening of the intermediate aspect of the limb

45
Q

acromelia

A

shortening of the extremities involving the distal limb

46
Q

thanatophoric dysplasia

A

lethal skel dysplasia
often abnormal skull shape & macrocranium, small thorax
normal bone mineralization, no fractures

47
Q

hypomineralization in skeletal dysplasia

A

OI T2, achondrogenesis, hypophosphatasia

48
Q

another name for Sturge Weber

A

encephalotrigeminal angiomatosis

49
Q

sturge-weber signs

A

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

Case review (8 decks)

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