Peds Flashcards
most common location of choroid plexus papilloma
lateral ventricle (atrium) 50%
most common brain tumor <1 yo
choroid plexus papilloma
genetic mutation associated with ATRT
SMARCB1 (22q11.2)
location ATRT (atypical teratoid rhabdoid tumor)
47% supra, 41% infra, 12% both
features chiari I
peglike cerebellar tonsils ≥ 5 mm below foramen magnum
± syringohydromyelia, scoliosis, hydrocephalus
features chiari II
- 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
umbilical arterial line location
L3-5 or T8-10
umbilical venous line location
tip at cavoatrial junction at level of hemidiaphragm
normal hip alpha angle
> 60 degrees
double bubble sign
Duodenal atresia, stenosis, web
Annular pancreas
normal ligament of treitz location
Left of spine
Same level as or superior to duodenal bulb
Posterior
causes microcolon
- ileal atresia
- meconium ileus
congenital pulmonary airway malformations (CPAM)
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
ossification centres elbow
capitellum radial head medial trochlea olecranon lateral
normal end of conus
L2
normal width filum terminale
2 mm
most common cause upper intestinal obstruction
jejunal atresia
most common benign liver lesion of infancy
infantile hepatic hemangioma
second most common benign pediatric liver lesion
mesenchymal hamartoma of liver
genetic associations with hepatoblastoma
- Beckwith-Wiedemann syndrome
- Gardner syndrome
- familial adenomatous polyposis
- type 1A glycogen storage disease
- trisomy 18
liver lesions increasing AFP
hepatoblastoma
HCC
most common pediatric hepatic malignancy
hepatoblastoma
genetic syndrome associated with duodenal atresia
trisomy 21
risk factors functional immaturity of colon
- Infant diabetic mother
- Mag sulfate
most common location hirschprung
short segment rectosigmoid (73%)
chiari III
Low occipital or high cervical meningoencephalocele containing cerebellum ± brainstem, meninges, vessels, CSF
Midline bone defect within supraoccipital bone, opisthion
Child with cystic cerebellar hemispheric mass + enhancing mural nodule
pilocytic astrocytoma
Most common 4th ventricular neoplasm of childhood
medulloblastoma
feature to distinguish schwannoma from meningioma
T2 hyperintensity (meningioma hypo)
diffuse or hemispheric cerebellar mass
thickened folia w/ striated appearance
Dysplastic Cerebellar Gangliocytoma (look for cowden)
most common branchial cleft cyst
2nd - angle of mandible
1st branchial cleft cyst location
in or near parotid
3rd/4th branchial cleft cyst locations
lowest point pyriform sinus, extend to thyroid lobe (4th)
genetic syndrome associated with lymphatic malformation neck
turners
most common soft tissue sarcoma of childhood
rhabdomyosarcoma
swyer james syndrome
Acquired unilateral pulmonary hypoplasia (postinfectious bronchiolitis obliterans)
Small, hyperexpanded lung with relative decrease in vascularity
leptomeningeal cyst
aka growing skull fracture in <3 yo
traumatic tear of the dura allows leptomeninges ± brain parenchyma to herniate into skull # or suture
abdominal tumors in Beckwith-Wiedemann
Wilms
hepatoblastoma
nephroblastoma
adrenal cortical carcinoma
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
WAGR syndrome
11p13 mutation: Wilms tumors (greatly incr risk) aniridia (complete/partial absence of the iris) genitourinary abN retardation
TORCH infections
toxoplasmosis other (syphilis, varicella-zoster, parvovirus B19) rubella cytomegalovirus (most common) herpes simplex virus
respiratory distress syndrome
aka hyaline membrane disease preterm bilateral, symmetrical granular, ground glass *decreased lung volumes complications: bronchopulmonary dysplasia
transient tachypnea of newborn
C-section bilateral, symmetrical diffuse airspace fluid in fissure, pleural effusion hyperinflation
meconium aspiration
neonatal distress, meconium
bilateral, symmetrical
coarse, ropey, pleural effusion
hyperinflation
neonatal pneumonia
O2 requirement, inflam markers
unilateral, asymmetric: airspace opacities, patchy/confluent
perihilar streaky
Ebstein anomaly
- at birth
- cyanotic
- very large heart
- right to left shunt (decreased vascularity)
hypoplastic left heart
- pulmonary venous congestion (not shunt)
- neonatal
- needs correction to live
ASD
- enlarged pulm arts (left to right)
- acyanotic
- can present late
tuncus arteriosus
- enlarged pulm arts (left to right)
- cyanotic (present early)
pulmonic stenosis cxr
- post-stenotic dilation of main and left pulm art
- 2/2 direction post-stenotic jet
Weigert Myer Law
in complete ureteral duplication:
- upper moiety inserts more medial and caudal
- lower moiety more lateral and cephalad
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
epiphyseal equivalents
bones considered similar to epiphyses and have a similar list of diseases:
- patella
- calcaneus
- carpal bones
- greater and lesser trochanters
- most apophysis
asplenia: lung association
bilateral trilobed lungs with bilateral minor fissures
bilateral eparterial bronchi
polysplenia: lung association
bilateral bilobed lungs with no minor fissures
bilateral hyparterial bronchi
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
hypertrophic pyloric stenosis
muscle >3 mm thick
pyloric channel >17 mm long
persistent delayed emptying
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)
bilateral urinary tract dilation in male infant
posterior urethral valve until proven otherwise
need renal ultrasound and VCUG
prune belly syndrome - triad
cryptorchidism
urinary tract anomalies
hypoplastic or absent abdominal wall musculature
-almost exclusively males
airspace opacities + pneumothorax in a term infant
meconium aspiration
AIRP
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
bone metastasizing renal tumour of childhood
clear cell carcinoma
Wilms doesn’t go to bone
most common renal tumour in neonates
mesoblastic nephroma
mimics Wilms which presents 6mo-4yr
screen which infants for IVH using US?
preterm <32 weeks
birth weight <1.5 kg
1st screening US: 4-7 days after birth
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
associations with chiari II
- neural tube defect (myelomeningocele)
- dysgenesis corpus callosum
- grey matter heterotopia
- absent septum pellucidum
- syringohydromyelia
- vertebral anomalies
pediatric moyamoya presentation (vs adults)
- stroke
- adults = bleed
- early childhood and 5th decade bimodal peak
- east asians
- vasculopathy (radiation, trisomy 21, NF1, sickle)
copper beaten skull association
increased ICP
- craniosynostosis
- obstructive hydrocephalus
luckenschadel skull associations
chiari II malformation/neural tube defects
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)
piriform aperture stenosis associations
- choanal atresia
- mega incisor
- midline brain defects –> face predicts brain (CC genesis, holoprosencephaly)
- hypothalamic-pituitary-adrenal axis dysfunction
timeline pulmonary interstitial emphysema & chronic lung disease of prematurity
PIE: first week of life
CLD: after 3-4 weeks postnatal
congenital diaphragmatic hernia associations
- congenital heart disease
- malrotation
MC is bochdalek, but right side associated w/ GBS pan
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
classic predilection of RSV pneumonia
segmental or lobar atelectasis, particularly RUL
1 & 2 GI duplication cysts
#1 distal ileum #2 esophageal
more common in kids? Hodgkins or NHL
Hodgkins 4x
involves thymus 90% of time
klinefelters at risk for which cancers?
300x risk germ cell tumor
male breast cancer
VACTERL
Vertebral anomalies 37% Anal, imperforate 63% Cardiac 77% TE fistula or esophageal atresia 40% Renal 72% Limb 58%
associations of malrotation
heterotaxy syndromes
omphaloceles
max pressure reducing intussusception
120 mm Hg
contraindications to reducing intussusception
free air
peritonitis
associations with omphalocele
trisomy 18 (MC) cardiac (50%) GI, CNS, GU Turners Klinefelters Beckwith-Wiedenmann Pentalogy of Cantrell Umbilical cord cysts (allantoic cysts)
Pentalogy of Cantrell
- omphalocele
- ectopia cordis (abN location heart)
- diaphragmatic defect
- pericardial defect or sternal cleft
- cardiovascular malf’ns
Associations with biliary atresia
polysplenia
trisomy 18
Right sided heterotaxy
- two fissures left lung
- asplenia
- increased cardiac malformations
- reversed aorta/IVC
Left sided heterotaxy
- one fissure right lung
- polysplenia
- less cardiac malformations
- azygos continuation of IVC
- 10% biliary atresia
age pancreatic mass
1 yo - pancreatoblastoma
6 yo - adenocarcinoma
15 - SPEN
management of nephroblastomatosis
USS screening for Wilm’s q3month until 7-8 yo
wilms
never biopsy
never before age 2 months
5-10% bilateral
locations % neuroblastoma
adrenal 35%
retroperitoneum 30%
posterior mediastinum 20% (better outcome than abdominal)
neck 5%
stage 4S neuroblastoma
<1 yo
distal mets to skin, liver, bone marrow
excellent prognosis
NOT cortical bone
wimberger sign - syphilis
destruction medial portion proximal metaphysis of tibia
normal alpha angle hip
normal acetabular angle hip
> 60 degrees
<30 degrees
Kocher criteria
septic arthritis vs transient synovitis
- fever
- inability to walk
- elevated ESR/CRP
- WBC >12K
- 3/4 = septic
- -ve CRP and weightbear ≠ septic
- CRP strongest indicator septic
Bugs:
Epiglottitis
Croup
Exudative tracheitis
H flu
Parainfluenza
Staph A
Normal pre vertebral soft tissues
< 6 mm C2
>22 mm at C6
% contra lateral abnormalities in px with MCDK
40%
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
canavan’s disease
NAA peak
- deficiency of aspartoacylase
- NAA accumulates in mitochondria, impairing myelin synthesis
