Peds Flashcards
1
Q
most common location of choroid plexus papilloma
A
lateral ventricle (atrium) 50%
2
Q
most common brain tumor <1 yo
A
choroid plexus papilloma
3
Q
genetic mutation associated with ATRT
A
SMARCB1 (22q11.2)
4
Q
location ATRT (atypical teratoid rhabdoid tumor)
A
47% supra, 41% infra, 12% both
5
Q
features chiari I
A
peglike cerebellar tonsils ≥ 5 mm below foramen magnum
± syringohydromyelia, scoliosis, hydrocephalus
6
Q
features chiari II
A
- small post fossa
- low lying tentorium/torcula
- pulled down brainstem w/ long, low 4th vent
- tectal beaking
- deficienct falx cerebri
- brainstem kinking
- large massa intermedia
- cerebellar tonsils & vermis displaced down
- myelomeningocele
- tethered cord
7
Q
umbilical arterial line location
A
L3-5 or T8-10
8
Q
umbilical venous line location
A
tip at cavoatrial junction at level of hemidiaphragm
9
Q
normal hip alpha angle
A
> 60 degrees
10
Q
double bubble sign
A
Duodenal atresia, stenosis, web
Annular pancreas
11
Q
normal ligament of treitz location
A
Left of spine
Same level as or superior to duodenal bulb
Posterior
12
Q
causes microcolon
A
- ileal atresia
- meconium ileus
13
Q
congenital pulmonary airway malformations (CPAM)
A
Type I: large dominant cyst, 2-10 cm Type II: small cysts <2cm Type III: microcysts Type IV: unlined cyst Type 0: lethal, global arrest lung development
14
Q
ossification centres elbow
A
capitellum radial head medial trochlea olecranon lateral
15
Q
normal end of conus
A
L2
16
Q
normal width filum terminale
A
2 mm
17
Q
most common cause upper intestinal obstruction
A
jejunal atresia
18
Q
most common benign liver lesion of infancy
A
infantile hepatic hemangioma
19
Q
second most common benign pediatric liver lesion
A
mesenchymal hamartoma of liver
20
Q
genetic associations with hepatoblastoma
A
- Beckwith-Wiedemann syndrome
- Gardner syndrome
- familial adenomatous polyposis
- type 1A glycogen storage disease
- trisomy 18
21
Q
liver lesions increasing AFP
A
hepatoblastoma
HCC
22
Q
most common pediatric hepatic malignancy
A
hepatoblastoma
23
Q
genetic syndrome associated with duodenal atresia
A
trisomy 21
24
Q
risk factors functional immaturity of colon
A
- Infant diabetic mother
- Mag sulfate
25
most common location hirschprung
short segment rectosigmoid (73%)
26
chiari III
Low occipital or high cervical meningoencephalocele containing cerebellum ± brainstem, meninges, vessels, CSF
Midline bone defect within supraoccipital bone, opisthion
27
Child with cystic cerebellar hemispheric mass + enhancing mural nodule
pilocytic astrocytoma
28
Most common 4th ventricular neoplasm of childhood
medulloblastoma
29
feature to distinguish schwannoma from meningioma
T2 hyperintensity (meningioma hypo)
30
diffuse or hemispheric cerebellar mass
| thickened folia w/ striated appearance
Dysplastic Cerebellar Gangliocytoma (look for cowden)
31
most common branchial cleft cyst
2nd - angle of mandible
32
1st branchial cleft cyst location
in or near parotid
33
3rd/4th branchial cleft cyst locations
lowest point pyriform sinus, extend to thyroid lobe (4th)
34
genetic syndrome associated with lymphatic malformation neck
turners
35
most common soft tissue sarcoma of childhood
rhabdomyosarcoma
36
swyer james syndrome
Acquired unilateral pulmonary hypoplasia (postinfectious bronchiolitis obliterans)
Small, hyperexpanded lung with relative decrease in vascularity
37
leptomeningeal cyst
aka growing skull fracture in <3 yo
| traumatic tear of the dura allows leptomeninges ± brain parenchyma to herniate into skull # or suture
38
abdominal tumors in Beckwith-Wiedemann
Wilms
hepatoblastoma
nephroblastoma
adrenal cortical carcinoma
39
DiGeorge syndrome
22q11. 2 deletion syndrome
- malformation of 3rd/4th pharyngeal pouches: thymic & parathyroid aplasia
- CHD: ToF, TA, interrupted aortic arch
- cleft lip ± palate
- facies: elongated face, short philtrum, facial asymmetry, prominent nose
- decreased immunity
- learning disability
40
WAGR syndrome
```
11p13 mutation:
Wilms tumors (greatly incr risk)
aniridia (complete/partial absence of the iris)
genitourinary abN
retardation
```
41
TORCH infections
```
toxoplasmosis
other (syphilis, varicella-zoster, parvovirus B19)
rubella
cytomegalovirus (most common)
herpes simplex virus
```
42
respiratory distress syndrome
```
aka hyaline membrane disease
preterm
bilateral, symmetrical
granular, ground glass
*decreased lung volumes
complications: bronchopulmonary dysplasia
```
43
transient tachypnea of newborn
```
C-section
bilateral, symmetrical
diffuse airspace
fluid in fissure, pleural effusion
hyperinflation
```
44
meconium aspiration
neonatal distress, meconium
bilateral, symmetrical
coarse, ropey, pleural effusion
hyperinflation
45
neonatal pneumonia
O2 requirement, inflam markers
unilateral, asymmetric: airspace opacities, patchy/confluent
perihilar streaky
46
Ebstein anomaly
- at birth
- cyanotic
- very large heart
- right to left shunt (decreased vascularity)
47
hypoplastic left heart
- pulmonary venous congestion (not shunt)
- neonatal
- needs correction to live
48
ASD
- enlarged pulm arts (left to right)
- acyanotic
- can present late
49
tuncus arteriosus
- enlarged pulm arts (left to right)
| - cyanotic (present early)
50
pulmonic stenosis cxr
- post-stenotic dilation of main and left pulm art
| - 2/2 direction post-stenotic jet
51
Weigert Myer Law
in complete ureteral duplication:
- upper moiety inserts more medial and caudal
- lower moiety more lateral and cephalad
52
risk factors for developmental hip dysplasia
mechanical factors that reduce in utero space and movement:
- first-born status
- large fetal size
- oligohydramnios
- breech positioning
also females & infants with FHx of DDH
53
epiphyseal equivalents
bones considered similar to epiphyses and have a similar list of diseases:
- patella
- calcaneus
- carpal bones
- greater and lesser trochanters
- most apophysis
54
asplenia: lung association
bilateral *tri*lobed lungs with bilateral minor fissures
| bilateral eparterial bronchi
55
polysplenia: lung association
bilateral *bi*lobed lungs with no minor fissures
| bilateral hyparterial bronchi
56
achondroplasia: spinal findings
- progressive ↓ in interpedicular distance in L spine
- posterior vertebral body scalloping
- gibbus deformity of T-L junction
- anterior vertebral body beaking or wedging (bullet-shaped)
- ↑ lumbosacral lordosis
57
hypertrophic pyloric stenosis
muscle >3 mm thick
pyloric channel >17 mm long
persistent delayed emptying
58
normal postnatal AP renal pelvic diameter
<10mm
(measure after 48h of birth d/t dehydration)
(prenatal at 16-27 weeks: <4mm
prenatal at >28 weeks: <7mm)
59
bilateral urinary tract dilation in male infant
posterior urethral valve until proven otherwise
need renal ultrasound and VCUG
60
prune belly syndrome - triad
cryptorchidism
urinary tract anomalies
hypoplastic or absent abdominal wall musculature
-almost exclusively males
61
airspace opacities + pneumothorax in a term infant
meconium aspiration
| AIRP
62
screening interval for Wilms tumour in Beckwith-Wiedemann and WAGR syndromes
US every 3 months until age 8-10 years
(NCI 2020)
hypothesis: tumour will be detected at a lower stage
63
bone metastasizing renal tumour of childhood
clear cell carcinoma
| Wilms doesn’t go to bone
64
most common renal tumour in neonates
mesoblastic nephroma
| mimics Wilms which presents 6mo-4yr
65
screen which infants for IVH using US?
preterm <32 weeks
birth weight <1.5 kg
1st screening US: 4-7 days after birth
66
caudal regression syndrome - 2 groups
group 1: more severe caudal dysgenesis with high-lying (above L1), blunt/club-shaped cord terminus
group 2: less severe dysgenesis with low-lying, tapered, distal cord tethered by tight filum, lipoma, lipomyelomeningocele, or terminal myelocystocele
67
associations with chiari II
- neural tube defect (myelomeningocele)
- dysgenesis corpus callosum
- grey matter heterotopia
- absent septum pellucidum
- syringohydromyelia
- vertebral anomalies
68
pediatric moyamoya presentation (vs adults)
- stroke
- adults = bleed
- early childhood and 5th decade bimodal peak
- east asians
- vasculopathy (radiation, trisomy 21, NF1, sickle)
69
copper beaten skull association
increased ICP
- craniosynostosis
- obstructive hydrocephalus
70
luckenschadel skull associations
chiari II malformation/neural tube defects
71
Ddx wormian bones skull
```
PORK CHOP
Pyknodysostosis
Osteogenesis imperfecta
Rickets
Kinky hair syndrome
```
Cleidocranial dysostosis
Hypothyroidism/Hypophosphatemia
One too many 21 chromosome
Primary acro-osteolysis (Hajdu Cheney)
72
piriform aperture stenosis associations
- choanal atresia
- mega incisor
- midline brain defects --> face predicts brain (CC genesis, holoprosencephaly)
- hypothalamic-pituitary-adrenal axis dysfunction
73
timeline pulmonary interstitial emphysema & chronic lung disease of prematurity
PIE: first week of life
CLD: after 3-4 weeks postnatal
74
congenital diaphragmatic hernia associations
- congenital heart disease
- malrotation
MC is bochdalek, but right side associated w/ GBS pan
75
associations of intralobar and extra lobar sequestration
intralobar: recurrent infections (older kids)
extra lobar: congenital anomalies (younger kids)
CPAM, diaphragm hernia, vertebral anomalies, congenital heart disease, pulmonary hypoplasia
76
classic predilection of RSV pneumonia
segmental or lobar atelectasis, particularly RUL
77
#1 & 2 GI duplication cysts
```
#1 distal ileum
#2 esophageal
```
78
more common in kids? Hodgkins or NHL
Hodgkins 4x
| involves thymus 90% of time
79
klinefelters at risk for which cancers?
300x risk germ cell tumor
| male breast cancer
80
VACTERL
```
Vertebral anomalies 37%
Anal, imperforate 63%
Cardiac 77%
TE fistula or esophageal atresia 40%
Renal 72%
Limb 58%
```
81
associations of malrotation
heterotaxy syndromes
| omphaloceles
82
max pressure reducing intussusception
120 mm Hg
83
contraindications to reducing intussusception
free air
| peritonitis
84
associations with omphalocele
```
trisomy 18 (MC)
cardiac (50%)
GI, CNS, GU
Turners
Klinefelters
Beckwith-Wiedenmann
Pentalogy of Cantrell
Umbilical cord cysts (allantoic cysts)
```
85
Pentalogy of Cantrell
1. omphalocele
2. ectopia cordis (abN location heart)
3. diaphragmatic defect
4. pericardial defect or sternal cleft
5. cardiovascular malf'ns
86
Associations with biliary atresia
polysplenia
| trisomy 18
87
Right sided heterotaxy
- two fissures left lung
- asplenia
- increased cardiac malformations
- reversed aorta/IVC
88
Left sided heterotaxy
- one fissure right lung
- polysplenia
- less cardiac malformations
- azygos continuation of IVC
- 10% biliary atresia
89
age pancreatic mass
1 yo - pancreatoblastoma
6 yo - adenocarcinoma
15 - SPEN
90
management of nephroblastomatosis
USS screening for Wilm's q3month until 7-8 yo
91
wilms
never biopsy
never before age 2 months
5-10% bilateral
92
locations % neuroblastoma
adrenal 35%
retroperitoneum 30%
posterior mediastinum 20% (better outcome than abdominal)
neck 5%
93
stage 4S neuroblastoma
<1 yo
distal mets to skin, liver, bone marrow
excellent prognosis
NOT cortical bone
94
wimberger sign - syphilis
destruction medial portion proximal metaphysis of tibia
95
normal alpha angle hip
| normal acetabular angle hip
>60 degrees
| <30 degrees
96
Kocher criteria
septic arthritis vs transient synovitis
1. fever
2. inability to walk
3. elevated ESR/CRP
4. WBC >12K
- 3/4 = septic
- -ve CRP and weightbear ≠ septic
- CRP strongest indicator septic
97
Bugs:
Epiglottitis
Croup
Exudative tracheitis
H flu
Parainfluenza
Staph A
98
Normal pre vertebral soft tissues
< 6 mm C2
| >22 mm at C6
99
% contra lateral abnormalities in px with MCDK
40%
100
vein of galen malformation types
choroidal (worse): complex networks of AV connections, ++ shunting, high output heart failure, hydrocephalus, present at birth
mural: fewer connections, less shunting, present in infancy/childhood, hydro or developmental delay
101
canavan's disease
NAA peak
- deficiency of aspartoacylase
- NAA accumulates in mitochondria, impairing myelin synthesis
