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MCQ Flashcards

1
Q

Rheumatic fever

A

Follows pharyngeal group A strep infection (pyogenes)
Type 2 hypersensitivity
2 major, or 1 major and 2 minor Jones criteria
Joints (migratory arthritis, can cause Jaccoud)
Heart (pancarditis)
Nodules (subcut)
Erythema marginatum
Sydenham chorea
Aschoff nodules histologically

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2
Q

Corpus callosum agenesis

A

Not associated with Downs or Chiari 1
Associated with interhemispheric lipoma, grey matter heterotopia, cephalocele, non-Downs trisomy, Dandy walker, absence of anterior commisure, septo-optic dysplasia, agyria/pachygyria, Chiari 2, midline arachnoid cyst, hydrocephalus

Genu forms first, so is present in partial. Exception is holoprosencephaly
Elongated formen of monro in dysgenesis

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3
Q

Tricuspid atresia

A

Cyanotic
Obligate ASD or PFO
Variable lung vasculature - depends on presence of TGA or VSD which are associated i.e. can be oligaemic or plethoric
Also associated with asplenia and right sided arch

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4
Q

Neonatal adrenal haemorrhage

A

Bilateral 10%
R>L 7:3
More common with breech, difficult delivery, hypoxia, fetal distress
1st week of life
Increased risk in diabetic mums, and large babies

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5
Q

Hepatoblastoma

A

AFP
Prediliction for right lobe
3rd most common child abdominal mass
43% of liver masses in children and most common primary liver tumour
Slightly more common in males
Heterogeneous, may have haemorrhage, necrosis (cystic), calc.
Predominantly echogenic

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6
Q

Beta angle

A

DDH
Opposite the Alpha angle - ilium to labrum rather than ilium to acetabulum
<77 degrees normal

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7
Q

Cervical spine radiographs

A

SCIWORA 5-65%, may be delayed up to 48 hours
Lateral displacement of C1 on C2 lateral masses up to 6mm normal in 4 year old (sum of both sides) (and may be seen up to 7 years old)

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8
Q

Dense metaphyseal bands

A 
Rickets
Leukaemia
Lead
Infection (Torch), infantile growth arrest
Early hypothyroidism (cretinism)
Scurvy, syphilis

DENSE LINES
Vit D, elemental (heavy metals and arsenic), normal, systemic, estrogen, Leukaemia, infection and idiopathic hypercalc, never forget healed rickets, early hypothyroidism (cretinism), scurvy, syphilis, sickel cell

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9
Q

Toddler fracture

A

Tibial, calcaneal, cuboid, fibula, diaphyseal (talus, metatarsals)
Not navicular

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10
Q

Pathological fracture causes (paeds)

A 
UBC (40%)
Non ossifying fibroma (19%)
Fibrous dysplasia (16%)
Osteosarcoma (15)
ABC (10%)
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11
Q

NEC

A

Not associated with maternal diabetes
Associated with Hirschsprungs, bowel obstruction
Can cause strictures, 9-30%, regardless of severity, multiple common, and most occur in the colon

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12
Q

Supravalvular aortic stenosis

A

Associated with infantile hypercalcaemia, Marfans

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13
Q

Polyhydramnios

A

Duodenal atresia, but not rectal atresia

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14
Q

PUJ obst

A

Bilateral in 10-40%
Accounts for 2/3 of hydronephrosis in utero
And is the most common cause of neonatal hydropnephrosis
Contralateral other renal anomalies may be seen - MCDK, renal agenesis

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15
Q

Primary megaureter

A

Congenital idiopathic alteration at VUJ

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16
Q

SUFE

A

Posteromedial displacement of head
Acute cartilage necrosis in 7-10%
Line of klein doesn’t intersect femoral head (line along superior fermoral neck)
AVN epiphysis in 15%
Bilateral in 20-40%
Subsequent degenerative change related to degree of lsip

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17
Q

Celery stalk metaphysis

A

Metaphyseal longitudinal linear bands of sclerosis

Rubella, syphilis, CMV, osteopathia striata

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18
Q

Sequestration

A

Intra >extra (2:1)
Extra almost always LLL (10% infradiaphragmatic). LLL most common site for intra also (60%, followed by RLL)
Intralobar presents later
Posterior basal segment most common

Extra presents neonatal with respiratory distress, cyanosis, or infection
Intra presents in late childhood or adolescence with recurrent infections

CAN have airbronchogram, as intralobar may have abnormal connection to bronchial tree and both can connect to GI tract. Usually absent though

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19
Q

Rectosigmoid ratio

A

Normally >1 (i.e. rectum bigger)

Reversed in Hirschsprungs

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20
Q

Heart disease approach

A

Acyanotic - heart failure (including hypoplastic left heart, coarctation), or shunt vascularity
Cyanotic, decreased pulmonary flow - TOF, Ebstein (Ebstein usually has secundum ASD - may not be cyanotic if not a lot of shunting)
Cyanotic, increased pulmonary flow - transposition (obligatory shunt), truncus, tricuspid atresia (obligatory shunt/ASD for cyanosis, VSD may give increased pulmonary flow), TAPVR (obligatory shunt), tingle ventricle

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21
Q

Ebstein

A

Depending on degree of shunting, may or may not be cyanotic
Common (nearly always) to have an ASD or PDA

Apically displaced septal and posterior leaflets

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22
Q

Kawasaki disease

A 
Small to medium vessel vasculitis in young children
Preference for coronary vessels - myocarditis and coronary artery aneurysms
Fever that doesn't respond to abs.
Mucocutaneous lymph node syndrome
Lymphadenitis, conjunctivitis, uveitis
Strawberry tongue, perianal erythema
Desquamation of palms and soles
Hydrops of the gallbladder
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23
Q

Omphalocele

A

Trisomies - 20-50%. T18 most common
Associated with Beckwith-Weidemann
Pentalogy of Cantrell
Bladder exstrophy

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24
Q

Nasal pyriform aperture stenosis

A

Associated with central megaincisor, holoprosencephaly, clinodactyly, pituitary dyfunction

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25
Q

NF1

A 
Cafe au lait spots >6
Axillary or inguinal freckling
1st degree relative
Lisch nodules (iris hamartoma)
Optic pathway gliomas (20% have some sort of glioma)
Neurofibromas x2 or plexiform NF
Distinctive bone lesion e.g. sphenoid wing dysplasia, gracile long bones.
2 of the above
Chromosome 17

UBO (unidentifed bright object) / FASI (focal area of signal intensity) on T2

Increased risk of Wilms, Phaeo, neuroblastoma, astrocytoma as above, rhabdomyosarc, CML, neurofibrosarc /MPNST

Lower zone interstitial fibrosis in 20%

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26
Q

NF2

A 
MISME
Ependymomas are spinal
Bilateral acoustic schwannomas. May have spinal schwannomas.
Cranial and spinal meningiomas
Few external signs - can have cafe au lait spots, but no lisch nodules
Syringohydromyelia
Cataracts
22 AD
Not associated with neurofibromas
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27
Q

Dandy Walker malformation

A

Hypoplastic vermis, cephalic rotation of remnant
Cystic dilatation of 4th ventricle
Enlarged posterior fossa with torcula lambdoid inversion

In 70%, other CNS abnormality present
Cortical dysplasia, polymicrogyria, heterotopia
Corpus callosum dysgenesis
Holoprosencephaly
Schizencephaly
Lipoma of corpus

Trisomy 13 and 18,
Non syndromic non CNS associations

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28
Q

Cystic fibrosis lung and cancers

A 
Central and upper lobe predominant (and apical lower lobe)
Bronchiectasis progresses to cystic
Lymphadeonpathy
Pulmonary artery hypertension
Hyperinlfation
Consolidation
Pneumothoraces
Mucous plugging
Bronchial artery hypertrophy, haemoptysis

In CF there is an increased risk of GI tract, pancreatic, and biliary tree cancer, and lymphoma.
Risk is more pronounced in those with organ transplants

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29
Q

Sturge-Weber

A

Facial port wine stain and pial angiomas (ipsilateral)
Trigeminal nerve (V1) port wine stain
Occipital pia > parietal >frontal>temporal>mibrain>cerebellum
Not hereditary
Associated with coarctation, paragangliomas
Gyriform / tram-track calcification
Isipliateral skull and sinus hypertrophy
Enlargement of ipsilateral choroid plexus

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30
Q

TAPVR

A 
All venous blood enters right atrium
A R to L shunt required survival
Supracardiac >50% - left vertical vein
Cardiac 30%, to coronary sinus
Infracardiac
Mixed
1/3 have other cardiac lesion
Associated with heterotaxy, particularly asplenia

Snowman in supracardiac: Enlargerd right atrium forms body of snowman, while dilated vertical vein, SVC and brachiocephalic vein form head

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31
Q

Snowman

A

TAPVR, supracardiac

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32
Q

Egg on a string

A

Transposition
Egg-shaped heart from LA enlargement and abnormally convex right atrium
Narrow superior mediastinum from stress thymic atrophy and hyperinflated lungs

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33
Q

Box-shaped

A

Ebstein

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34
Q

Boot-shaped

A

Upturned apex from RVH

Typically seen in TOF

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35
Q

CMV

A

The most common cause of congenital infective and brain damage
May get symptoms after 6-9 months
Microcephaly, sensorineural deafness, mental retardation, seizures
Periventricular calcs (as opposed to toxoplasmosis which is more basal ganglia), hydrocephalus
White matter lesions on MR

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36
Q

Umbilical venous catheter

A

May cause intrahepatic haematoma if perforates a vessel wall. These may calcify. Thrombus can also calcify.
Injecting hyperosmolar solutions into the portal vein contributes to fibrosis and thrombosis

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37
Q

VSD

A

Left to right shunting increases over initial period of life as pulmonary vascular resistance decreases
Can cause RV hypertrophy

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38
Q

Prune Belly

A

Megaureter - non-obstructed, reflux. Dilater posterior urethra without obstruction. (large distended bladder with thickened wall without trabeculation)
Cryptorchidism
Ando wall underdevelopment
Potter sequence and oligo

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39
Q

MCDK

A

Pelvi-infundibular type and hydronephrotic-obstructive type
First type most common - non-communicating cysts
Second type may have a dominant pelvic cyst
Fatal if bilateral
No increased risk of malignancy
Atretic ureter - obstruction or atresia may be the cause of the kidney problem
40% have abnormal contralateral kidney, mostly PUJ obstruction (although Dahnert suggests it may be reflux)
Also assocaited with ipsilateral reflux - 25%, or ectopic ureter

(ARPKD can’t see cysts antenatally - just enlarged echogenic kidneys)
(not associated with Caroli, unlike AD and ARPKD and MSK)

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40
Q

Sacrococcygeal teratoma

A

Most common germ cell tumour of childhood
75% benign
Calcs in 60%, more frequent in benign (seldom observed in malignant)
Predominantly external 47%
Predominantly external with significant presacral portion 35%
Predominantly sacral, and predominantly presacral both 10%

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41
Q

Femoral head AVN

A

Anterior weight bearing portion affected first
Cartilage remains intact until late
Subchondral fracture intermediate time
MR better than bone scan

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42
Q

FICAT

A

1 almost normal
2 looking abnormal
3 subchondral fracture
4 secondary degeneration

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43
Q

CPAM

A

25% of cogenital lung disease
95% of cystic lung disease
1 50%
2 40%
3 10%
2 associated with renal agenesis/dys, pulmonary sequestration (hybrid), cardiac disease. No well documented lobar prediliction
Overall 25% have associated anomalies - karyotype indicated in such cases

Contralatera mediastinal shift in 90%

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44
Q

Congenital diaphragmatic hernia

A

Cyanosis due to persistent fetal circulation in hypoplastic lung.
Associated with malrotation, renal anomalies
84% left, 2% bilateral
1/2-4000

Morgagni associated with heart disease, bowel malrotation, chromosomal, mental retardation, pericardial deficiency
(morgagni are R>L, but central)
(majority of Morgagni herniae are asymptomatic unless they strangulate)

20% have an associated abnormality (Morgagni)
Cardiac 25%
Chromosomal 30%
Pulmonary hypoplasia is commonest association

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45
Q

Intusussception

A

90% idiopathic
Contraindication to air enema: peritonitis, free air
Reduce chance of success: >24 hours, poor clinical condition, small bowel obstruction
50% <1
Ileocolic 75-90^, then ileoileal, then colicocolic
25% have no radiographic abnormality

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46
Q

Vascular malformations

A

Infantile haemangioma are most common head and neck tumour of infancy. Proliferate in 1st year then involute.
High velocity, low resistance, distinct mass
AVMs don’t have an identifiable mass

Haemangiomas are more prevalent in premature, low birth weight
Grow then start involuting in 1st year - don’t need treatment, 50% completely resolved by 5 years
Can cause Kasabach-Meritt syndrome

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47
Q

Posterior fossa mass

A

Pilocytic astrocytoma most common
Then medulloblastoma
Then ependymoma
Medulloblastomas tend to be older than ependymomas
Ependymomas calcify most (40-50%), then medullo (20%) then astro (10%) (not good distinguisher)

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48
Q

Medulloblastoma

A

Adults with medulloblastoma may have desmoplastic subtype
Sclerotic bone mets most common extraneural met (65%, 35% lytic) [extraneural mets in 5%)
Posterior spinal drop mets 50% - normal flow of CSF from cisterna magna (these may trigger a desmoplastic reaction)
Radiosensitive
Grade 4
Poor prognosis if <3, CSF mets at presentation, or incomplet resection

In adults, often desmoplastic subtype, prone to recurrence
Better prognosis than in childen
Adult variety demonstrates cystic change in about 80%

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49
Q

Ependymoma

A

Grade 2
Anaplastic subtype grade 3 - 15%
Myxopapillary grade 1
5 year survival 14% for children with intracranial lesions, 76% for adults
Subarachnoid dissemination in 7% of infratentorial, 1.6% of supratentorial

Heterogeneous enhancement. Calcs in children, haemorrhage in adults.

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50
Q

Normal myelination

A

Posterior portion of posterior limb of internal capsule myelinated at birth. Anterior portion by 1 month
Anterior limb by 3 months
Optic nerves 1 month
Optic radiations 3 months
Splenium of corpus callosum. Dorsal to ventral, caudal to cephalad, central to peripheral.
Splenium T1 bright at 4 months, genu 6 months
T1 bright precedes T2 low
Adult pattern by 18 months

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51
Q

Nasolacrimal dacrocytocele

A

Round ovoid mass from medial canthus to inferior meatus along course of duct

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52
Q

Holoprosencephaly - cause, associations, facial features

A

Environmental and genetic factors implicated
Early gestational insult
Associated with trisomy 13 and 18
Sonic hedgehog gene
Endocrine abnormalities - diabetes insipidus in 70%, hypothyroidism 11%, hypocortisolism 7%, GH def 5%
Proboscis, cyclopia, cleft lip, hypotelorism, solitary central incisor
Olfactory tracts absent

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53
Q

Eosinophilic granuloma

A

Tends to spare posterior elements
Can cause anterior wedging and vertebra plana
Thoracic>lumbar>cervical
A soft tissue component is common

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54
Q

Holoprosencephaly - types

A

Alobar, semilobar, lobar
Alobar most severe
Alobar - Fused thalami, single ventricle
Semilobar - anterio fusion and thalamic fusion. Corpus callosal agenesis or hypoplasia
Lobar - Subtle fusion e.g. cingulate gyri, frontal horns of lateral ventricles, fornices, absent septum pellucidum. Thalami separate

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55
Q

Septo-optic dysplasia

A

Thought of as part of holoprosencephaly spectrum
Absence of septum pellucidum and optic nerve hypoplasia
Associated with schizencephaly
May not be able to distinguish from lobar holoprosencephaly

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56
Q

Spondyloepipthyseal dysplasia

A

Dysplasia, spine and proximal epiphyseal centres
Abnormal collagen synthesis
Congenital and tarda forms
Short proximal limbs, normal size hands and feet
Coxa vara, flattened femoral head
Atlantoaxial instability
Craniovertebral junction stenosis
Scoliosis
Platyspodyly
Short neck and short trunk with protruding abdomen
Normal IQ and life span

Associations: myopia, retinal haemorrhage, hearing loss, nephrotic syndrome

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57
Q

Cortical desmoid

A

Misnomer – not true desmoid.
AKA distal femoral metaphyseal irregularity.
Asymptomatic, do not touch lesion, adolescents, slight male predominance
Posteromedial aspect of distal femoral metaphysis at insertion of adductor magnus or medial head of gastrocneumius,
High T2 / STIR, enhances, with normal or slight increased bone scan uptake
Lamellated periosteal reaction, may simulate aggressive lesion.

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58
Q

VHL

A

Clear cell renal cancers

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59
Q

Rickets

A

Genu varum
Delayed closure of anterior fontanelle
Scoliosis

Growth plate widening
Frayed metaphyses

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60
Q

Pyloric stenosis

A

Single muscular wall >3mm
Length >15mm
Diameter >14mm
3.1415 to remember (pi)

Associations: Turners, T18, TOF

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61
Q

Toxoplasmosis

A

Microcephaly, or macro from hydrocephalus

Basal ganglia calcs, (if periventricular more likely CMV, or TS)

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62
Q

Pentalogy of Cantrell

A 
Omphalocele
Ectopia cordis
Diaphragmatic defect
Pericardial defect 
Cardiac malformations (ASD, VSD, TOF, LV diverticulum)
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63
Q

Scurvy

A

Ground glass osteoporosis
Periosteal reaction from subperiosteal haemorrhage

Wimberger ring - circular epiphyseal shadow from haemorrhage
Frankel line - dense metaphyseal band

Trummerfield zone - lucent band under Frankel line
Pelken spurs - spurred metaphyses, results in cupping

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64
Q

Lucent metaphyseal bands

A 
Normal TLC
Normal
TORCH
Leukaemia
Chronic illness

Scurvy (Trummerfield zone under Frankel line), Rickets

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65
Q

Neuroblastoma

A

Mostly sporadic, but associated with: Beckwith weideman, DiGeorge, Hirschsprung, NF1
Homer Wright rosettes (also seen in medulloblastoma, PNET, pineoblastoma)

Can cause cerebellar ataxia
May have proptosis from orbital mets

2/3 in abdomen, with 2/3 of these in the adrenals

4s - <1, mets to skin, liver, bone marrow

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66
Q

Beckwith Weidemann

A

Congenital overgrowth disorder
Macroglossia, omphalocele, cardiac anomalies, localised gigantism
AD chromosome 11, but most sporadic
Wilms, neuroblastoma, hepatoblastoma, pancreatoblastoma, rhabdomyosarcoma, gonadoblastoma, ardenocortical carcinoma, renal stones

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67
Q

DiGeorge

A 
22q deletion
Velocardiofacial
CATCH22
Cardiac anomalies (conotruncal)
Abnormal facies
Thymic abscence
Cleft palate
Hypocalcaemia
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68
Q

Neuroblastoma v Wilms

A

Calcification common in neuro (only 20% Wilms)
Neuro younger
Neuro poorly marginated v wilms well circumscribed
Neuro encases vasculature, Wilms invades IVC / renal vein
Neuro extends into chest, elevates the aorta, and crosses midline behind aorta

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69
Q

Blakes pouch cyst

A

Infravermian cyst, communicates with 4th ventricle but not cisterna magna
Causes hydrocephalus
Non-perforation of Foramen of Magendie
Differential of Dandy Walker, mega cisterna magna.

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70
Q

Meckel Gruber syndrome

A

Renal cystic dysplasia, holoprosencephaly, and polydactyly (post axial i.e. 5th digit)
Pseudo trisomy 13
Usually fatal at birth (from pulmonary hypoplasia or neonatal renal failure)

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71
Q

Zellweger syndrome

A

Cerebrohepatorenal syndrome
Death within first year
Hepatomegaly and hyperechoic kidneys (can have MCDK), abnormal brain

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72
Q

Congenital lobar emphysema

A

LUL > RML > RUL
Right hemithorax most common
LLL infrequent

Can be diagnosed in utero
Can present as opacified mass with midline shift initially (retained fetal fluid), then a hyperlucent segment
More common in males 3x

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73
Q

VUR grading

A

1 ureter
2 renal pelvis
3 dilated ureter and pelvis mild
4 tortuous dilated ureter with preserved papillary impressions
5 tortuous dilated ureter with blunted papillae

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74
Q

Biliary atresia

A

Pretreatment with phenobarbital 5mg/kg/day for 5 days to increase biliary excretion and minimise false posiives on HIDA scan

75
Q

CF abdominal manifestations

A

In CF there is an increased risk of GI tract, pancreatic, and biliary tree cancer, and lymphoma.
Risk is more pronounced in those with organ transplants

Focal biliary cirrhosis. Fatty hepatic infiltration.
Gallstones, ductal strictures, sclerosing cholangitis
Pancreatic fatty replacement, pancreatitis, duct strictures
Renal stones, nephrotic syndrome from secondary amyloidosis
DIOS, reflux, intussusception, pneumatosis

76
Q

Renal tumour children

A

Mesoblastic nephroma (neonates - commonest neonatal mass)
Wilms
Clear cell sarcoma (bone mets may suggest this over Wilms)
RCC
Rhabdoid tumour (calcifies more than Wilms, subcapsular collections characteristic but also seen in Wilms)
ORTI (benign)

77
Q

Mesoblastic nephroma

A

Commonest renal tumour in neonates, 90% <1
Generally benign but can be aggressive
Removed - can be aggressive and can have sarcomatous degeneration
Generally solid with no calcification

78
Q

Alport syndrome

A

.

79
Q

Carpenter syndrome

A

.

80
Q

Thanatophoric dysplasia

A

.

81
Q

ARPKD

A

Small cysts, <2mm, not appreciable on USS
Large, echogenic centrally with lucent compressed cortex peripherally
(Medulla may become hypoechoic compared with cortex, giving a halo)

82
Q

Hutch diverticulum

A

Congenital bladder diverticula
At the VUJ
Almost exclusively in boys
Associated with reflux as normal insertion is disrupted

83
Q

Retinoblastoma

A

Radiosensitive
90% risk in familial RB - also osteosarcs (AD)
55% of cases have a germline mutation, most (so about 1/3 overall) bilateral
Flexner Wintersteiner rosettes (relatively specific)
Also Homer-Wright rosettes (also in other PNETs)

84
Q

Rhabdomyosarc

A

Peak 2-5, but can be any age of childhood

Head and neck most common, then extremites the GU tract

85
Q

Adrenoleukodystrophy

A 
X-linked dysmyelinating disorder
Posterior predominant
Involves splenium, peritrigonal white matter
Leading edge enhancement
Usually symmetric
Involves adrenals and testes also
Accumulation of VLCFAs
86
Q

Chiari 2

A 
Lemon head
Banana cerebellum
May cause hydrocephalus
Scalloping of petrous temporal bone
Fenestrated or agenic falx with interdigitated gyri
Tectal beaking - fusion of midbrain colliculi into single posterior beak protruding into cerebellum
Septum pellucidum may be absent
May have corpus callosum dysgenesis
87
Q

Chiari 3

A

Low occipital and high cervical encephalocele

Herniation of posterior fossa contents

88
Q

Pilocytic astrocytoma

A

Most common posterior fossa mass children
Calcifies less than epen and med
Association with NF1 (20% have a glioma)
Most cyst with enhancing nodule - 60% (half of these non-enhancing nodule). 20% heterogeneous 20% solid.
WHO1
Drop mets possible but not common (WHO1)

89
Q

Tarsal coalition

A

= calcaneonavicular and talocalcaneal

Middle facet most commonly involved in talocalcaneal

90
Q

Blalock-Taussig shunt

A

Palliative operation for cyanotic heart disease with reduced pulmonary flow, prior to definitive corrective surgery
End to side systemic (SCA) to pulmonary artery.
Distal SCA flow sacrificed, so no mammary flow, so get unilateral rib notching, upper 3 or 4 ribs on operation side

91
Q

Caudal regression

A

Associated with maternal diabetes - 200-400x risk if insulin dependent
VACTERL and Currarino triad associations (anorectal malformation and presacral mass)
Associated with neurogenic bladder, motor deficits > sensory

92
Q

Diastematomyelia

A

Scoliosis in 50-75%. 5% of congenital scoliosis have diaste
Tethered cord syndrome
May have one or 2 dural sacs
Vertebral anomalies common

93
Q

Hydrocephalus

A

70-90% of myelomeningocele have hydrocephalus
25% of hydrocephalus have spina bifida
Other causes - aqueductal stenosis, other Chiaris, Dandy Walker, mass
Obstructive, dysgenesis, destructive (infection, infarct or haemorrhage

Aqueductal stenosis most common cause of fetal hydrocephalus (40%) but also states spina bifida most common cause of ventriculomegaly (working on difference between ventricularmegaly and hydrocephalus)
Aqueductal stenosis can rarely be X-linked recessive

94
Q

McCune Albright

A

Polyostotic fibrous dysplasia
Precocious puberty
Coast of Maine cafe au lait
(mazabraud is polyostotic fibrous dysplasia and multiple intramuscular myxomas)

95
Q

Turners

A

Hypoplasia of odontoid and C1
Horseshoe kidney - renal anomlies in 30-50%
Normal skeletal maturation with 15 growth arrest, delayed fusion 20
7x risk colon cancer
Risk gonadoblastoma
60% complete, 30% mosaic
Not associated with increased maternal age
Associated with HTN, glucose intolerance, IBD
Hydrops - lymphatic failure
Cystic hygroma
Bicuspid aortic valve
Aortic coarctation
Madelung, short 4th, scoliosis, short, increased carrying angle
Positive carpal sign (carpal angle of 117 degrees or less - too much angulation in the proximal row)
Pyloric stenosis
Reduce maternal AFP (along with trisomies)

Not associated with advanced maternal age

96
Q

Fragile X

A 
Most common inherited mental retardation
Trinucleotide repeat
Autism, seizures
Big forehead, prognathism, macrocephaly
Large testes
Lax joints
Flat feet
Mitral valve prolapse
Slightly short
97
Q

Achondroplasia

A 
Homozygous lethal, respiratory failure
Most common non-lethal skeletal dysplasia
Decreased acetabular angle
Narrowed foramen magnum
Posterior vertebral body scalloping
Metaphyseal flaring
Tombstone iliac wings
Champagne pelvis
Narrowed interpedicular distance
Short pedicle canal stenosis
Trident hand - short stubby fingers with separation between middle and ring

Epiphyseal maturation and ossification unaffected
Quantitavely defective endochondral bone formation (not membranous bone formation which is skull, middle third of clavicle, mandible and maxilla)

80-90% sporadic

98
Q

Periosteal reaction in children

A 
Caffeys
JIA, reactive arthritis
Syphilis. Other osteomyelitis - TORCH
Scurvy (subperiosteal haemorrhage)
Hypervitaminosis A
Hypervitaminosis D (only listed as children on radiopaedia)
Renal osteodystrophy
Venous stasis
Prostaglandins for PDA closure in premature
Healing fractures
99
Q

Craniosynostosis

A

Haematologic - sickle cell, thalassaemia
Metabolic - Rickets, HyperD, Hypercalcaemia, hyperthyroid
Dysplasias - hypophosphatasia, achondroplasia, metaphyseal dysplasia, Hurlers
Syndromes - Crouzons, Aperts,
Microcephaly
After shunting

100
Q

Ochronosis

A

=alkaptonuria
Various MSK and renal manifestations
Calculi and nephrocalcinosis
Osteoporosis, disc calcs, syndesmophytes, early OA, chondral calcinosis

Blue-black discolouration of ear cartilage and ocular tissue

101
Q

Hirschsprung

A

80% short segment, 15% long, 5% entire colon
Rectosigmoid <1
Associated with Downs
Rare in the premature
Typically failure to pass meconium is presentation, but can be intermittent constipation and paradoxical diarrhoea

102
Q

Wormian bones

A 
OI
Downs
Rickets
PORKCHOPS
Pyknodystostosis
Kinky hair
Cleidocranial dysostosis
Hypothyroid, hypophosphatasia
Various Ps - progeria, pachydermoperiostosis, HajduCheney

Note Downs, cleidocranial dysostosis and cretinism, which are all on this list, are the causes of delayed suture closure (although cleidocranial also causes craniosynostosis)
While Ricket and hypophosphatasia are on the differential for premature closure/craniosynostosis

103
Q

Down MSK manifestations

A 
11 ribs
Flared iliac wings with decreased acetabular angles / horizontal acetabulae
Hypersegmented manubrium
Persistent metopic suture
Atlanto-axial subluxation
Wormian bones
Sandle gap
Clinodactyly

(also have small cerebellum)

104
Q

Cretinism

A 
Short stature, large head with delayed suture closure, mental retardation
Delayed skeletal development
Stippled epiphyses
(wormian bones)
A cause of gibbus deformity

Delayed dentition
Hypoplastic 5th digit
Hypertelorism

(no skeletal changes with adult hypothyroid)

(hypothyroid is on the list of causes of basal ganglia calcs)

105
Q

Cleft lip / palate

A 
Roughly 1:1000
Associated with holoprosencephaly, trisomy 13 and 18, Dandy Walker
1 Lip
2 Lip and palate unilateral
3 Bilateral lip and palate
4 midline lip and palate
106
Q

Newborn CXR

A

Right hemidiaphragm posterior ribs 7-9

57% CTR or greater enlarged, so 0.6

107
Q

MPS

A

Hurlers (1), Morquoi (4)
Hurlers most severe, Morquio slightly more common
Hurlers death 10-15. Morquiou 20-40
Lysosome storage disease

J shaped sella
Widened (and shortened) long bones and ribs (thick ribs)
Cysts in the corpus callosum are pathognomonic. Also found in peritrigonal white matter and can occur in other lobes
Vertebral body beaking
Vertebra plana universalis (morquio. Hurlers have normal height)
Atlantoaxial subluxation
Long slender pedicles
Cardiomegaly (hypertrophic then dilated)
Prominent perivascular spaces (corpus callosum cysts pathognomonic, also in peritrigonal white matter as above)
Madelung
Pointing of proximal metacarpals
Gibbus deformity
Hepatosplenomegaly
Herniae
Dysplastic odontoid

108
Q

Non-accidental injury

A

Question with which is not typical
Answer probably fractures with dislocation
Can have diaphyseal spiral fractures in non-ambulatory patient

109
Q

Ribbon ribs

A

NF1 most common

OI
Trisomy 18

110
Q

Chondroblastoma

A

Commonest epiphyseal tumour in children
Chicken wire calc
Proximal humerus common, then femur and tibia, with 10% in hands and feet
Lots of oedema

111
Q

Duodenal atresia

A

3% of Downs have
30% of DA cases have Downs
Most common high intestinal obstruction in newborns

95% of intestinal obstructions with 2 weeks of birth are from stenosis or atresia
Ileal atresia is 50%

112
Q

Brodies abscess

A

Subacute pyogenic osteomyelitis
Tibial metaphysis common
Penumbra sign - rim has higher T1 than abscess contents

113
Q

Human bites

A

Strep most common, then staph

114
Q

Diaphyseal periosteal reaction

A

In large long bones in 50% of infants <6months

115
Q

Supravalvular aortic stenosis

A

Associated with infantile hypercalcaemia syndrome, and Marfans, Williams syndrome

116
Q

Tracheo-oesophageal fistula

A

If distal fistula, 90% distended with gas

117
Q

Klippel Trenaunay

A

Port wine stain
Hemihypertrophy
Vascular malformations (venous)

118
Q

Hemihypertrophy

A

Beckwith Weideman
Klippel Trenaunay
NF1 (previous question asked “hemihypertrophy associated with gliomas - correct if referring to NF1)
McCune Albright

Proteus syndome

119
Q

Adrenal cortical carcinoma

A

60% secretory
Non-secretory are more common in adults
Children usually present with precocious puberty - cushings is rare compared with adults where this is common

120
Q

Enlarged vestibular aqueduct

A

> 1.5mm
Bilateral in 50-66%
Commonly associated with other anomalies e.g. the Mondini malformation (Cochlear malformation)

121
Q

Radial ray anomaly

A 
Radial hypoplasia or aplasia, with or without thumb abnormality
Fanconi anaemia
Holt Oram
Trisomy 18 (possibly 13)
Vacterl
Valproate, thalidomide
Amniotic band
122
Q

Hypoplastic clavicle

A

Cleidocranial dysostosis, Holt Oram

123
Q

Fanconi anaemia

A

Commonest inherited bone marrow failure syndrome
Associated with horseshoe kidney
Radial ray anomaly
AR
AML predisposition
Triphalangeal thumb (pre-axial polydactyly)

Hyperpigmetnation and microcephaly

Ashkenazi jews and afrikaners

124
Q

Holt Oram

A 
Clavicle hypoplasia
Radial ray anomaly
Heart disease - ASD, or VSD
Coarctation
Autosomal dominant
125
Q

Cleidocranial dysostosis

A 
Clavicle hypoplasia
May have Sprengels (high scapula)
Wormian bones
Supernumerary teeth
Brachycephaly (premature coronal suture closure)
Basilar invagination
126
Q

Acetabular angle

A

Increased in DDH, neuromuscular disease

Decreased in achondoplasia, Down

127
Q

Delayed suture closure

A

Downs
Cretinism
Cleidocranial dysostosis (also causes premature coronal suture closure / brachycephaly)

128
Q

Sprengel

A

Congenital scapula elevation
May have an omovertebral bar - fibrous, cartilaginous or ossesus - 35%
Associated with Klippel Feil, Spina Bifida, cleidocranial dystostosis

129
Q

Gracile long bones

A 
NIMROD
NF1
Immobility
Muscular dystrophy
RA (JRA)
OI
Dysplasia - marfans and homocysteinuria
130
Q

Grisel syndrome

A

Torticollis from inflammatory ligamentous laxity from retropharyngeal abscess

131
Q

Craniometaphyseal dysplasia

A

Dense skull base, facial bone, mandible
Undertubulated long bones / Erlenmyer Flask (C in CHONG)
Obliterated sinuses
AR

132
Q

Fragmented epiphyses

A

Multiple epiphyseal dysplasia

Stippled epiphyses in cretinism and maternal warfarin use, cranio, chondodysplasia punctata

133
Q

Homocystinuria

A

Eyes - ectopia lentis
CNS - seizures, dystonia, developmental delay
Skeletal - scoliosis, pectus excavatum, long limbs, osteoporosis, ligamentous laxity
Vascular - thromboembolic disease, CAD

Gracile bones (D for dysplasia, along with marfans, in NIMROD)

134
Q

Hyperphosphatasia

A 
Juvenile Pagets
Elevated ALP
Esp Puerto Rican descent
AR
Bowing of all bones
Severe deformity
May be epiphyseal sparing
135
Q

Hypophosphatasia

A 
May resemble rickets
Defective ALP synthesis by osteoblasts
Variable expression - perinatal lethal, mild, infantile, childhood, adult
Hypercalcaemic
Irregular metaphyses
Craniosynostosis
136
Q

Horseshoe kidney

A 
Associated with Trisomies and Turners (18 has 20% rate, Turners 7%)
Fanconi anaemia and VACTERL
Susceptible to trauma
Wilms and TCC
Infection, calculi, HTN, PUJ obstruction
137
Q

Conditions resembling rickets

A

Hypophosphatasia
Metaphyseal chondrodysplasia (type Shmidt)
Biliary atresia
Metabolic bone disease of prematurity

138
Q

Foot ossification

A

Birth:
Calcaneus, talus, cuboid
Metatarsals, phalanges

1 year: lateral cuneiform
3 years: medial cuneiform
4 years: infermediate cuneiform, navicular

Secondary centres:
Should all be present at 3, except calcaneus at 5

139
Q

Acromion ossification

A

Puberty

140
Q

Gibbus deformity

A 
Congenital or acquired
Con:
MPS
Aperts
Cretinism 
Achondroplasia

Acquired:
Infection, TB or pyo
Compression fracture, may be pathological
Scheurmanns
Vertebra plana IMELT - langerhans most common in children

141
Q

Canavan

A

.

142
Q

Alexander

A

.

143
Q

Metachromatic leukodystrophy

A

Lysosome storage disease
The most common hereditary leukodystrophy
AR
Tigroid pattern on MR (perivenular sparing)
Peri atrial and to lesser extent frontal horns
Typically present between 12-18 months
1/3 present in adolescence - may be psychosis

144
Q

Megalencepahlic leukoencephalopathy with subcortical cysts / Van der Knaap

A

.

145
Q

Double aortic arch

A

Most common symptomatic aortic arch variant
80% right dominant
Reverse S indentation on frontal barium swallow

146
Q

Normal sternal ossification centres

A

1 for manubrium
4 for body
1 for xiphoid

147
Q

PVL

A

Periventricular leukomalacia
White matter injury of prematurity
28-36 weeks
Before this get anencephaly, porencephaly
In later parts get more subcortical change also
Then at term involves deep grey and greywhite watersheds

Subacute - develop cysts
Late - parenchymal loss with ventricular enlargement.

Can be graded from 1-4, mostly on the extent of cysts

148
Q

Maternal diabetes

A

Associated with:
Heart - VSD, truncus transposition
Lung - RDS, TTN
GI - situs, meconium plug
CNS - lots - neural tube, holoprosencephaly, caudal regression, sirenomelia
Renal - agenesis, hydronephrosis, ureteric duplication
Skeletal - polydactyly, syndactyly
Other - poly, macrosomia, IUGR, single UA

149
Q

Meconium plug

A

Associated with
Maternal diabetes
Hirschsprungs

150
Q

Head ultrasound v CT

A

Apparently calcs may be seen earlier on USS according to answers

151
Q

ADEM and Hurst

A

.

152
Q

Round pneumonia

A

Suspect other pathology if over 8

153
Q

Bronchial atresia

A

LUL most common
Distal hyperinflation
Distal airway may have mucocele / mucous impaction

154
Q

Pseudokidney sign

A

A sign on longitudinal ultrasound of intussuception
Mesentery and vessels which has herniated into bowel appears as the hilum, while the oedematous bowel wall appears as the renal parenchyma

155
Q

Phaeo associations

A 
MEN2
VHL
NF1
Sturge Weber
TS
156
Q

Congenital syphilis

A 
Wimbergery sign (not wimberger ring of scurvy) - erosion of medial proximal tibial metaphysis, bilateral, pathognomonic
Bone lesions can heal without treatment

1/3 of babies to syphilitic mothers will have, 1/3 won’t, 1/3 miscarry
Early or late presentation (before or after 2 years)
May have bent long bones in utero

Early and late signs…

157
Q

Medullary cystic disease

A

3 variants – infantile, juvenile, adolescent, and has cysts at corticomedullary junction and medulla. Salt wasting, anaemia, uraemia. Renal failure, lethargy, growth retardation
Normal sized, or small kidneys (ARPKD typically enlarged, echogenic)
Juvenile form known as nephronophthisis
Adult form AD
Juvenile form AR
Cysts are epithelial lined and usually surrounded by inflammatory cells

158
Q

Germinal matrix haemmorhage

A 
67% incidence in babies born 28-32 weeks
(answers suggested 40% <32 weeks)
90% of bleeds occur in first 4 days, with 40% in the first 5 hours
Dex to the mother, and indomethacin to the child, can reduce risk
Risk factors:
Low birthweight
Cyanotic heart disease
Prolonged labour
Multiple gestation
159
Q

Rhabdoid tumour

A

Aggressive renal tumour
60% <1, 80% <2
Can have hypercalcaemia from elevated PTH
66% calcify i.e. more commonly calcify than Wilms
Subcapsular fluid collection also characteristic, but as the tumour is rare, still more likely to be Wilms
If brain tumour also, diagnostic.

160
Q

Clear cell sarcoma, kidney

A

Non-specific, can look like Wilms

Skeletal mets if present, may suggests clear cell

161
Q

Medullary sponge kidney

A

Can be seen in association with Ehlers Danlos, hemihypertrophy or Beckwith Weidemann, and Caroli disease
Causes medullary nephrocalcinosis

162
Q

Cystic renal dysplasia

A

A catch term encompassing MCDK and ARPKD and ADPKD, and obstructive cystic renal dysplasia

163
Q

Antenatal choroid plexus cysts

A

Tri 18 in particular, but also Tri 21, Klinefelter
Incidence 0.2-2.5%
Can be normal
Characteristics of cyst don’t help (e.g. size, bilaterality)

164
Q

Gaucher disease

A

Higher incidence in Ashkenazi jews

165
Q

Transposition of the great arteries

A

35% have a stable shunt - VSD
65% have an unstable shunt - PFO or PDA
Get RVH as it functions as the systemic ventricle

166
Q

Congenital adrenal hyperplasia

A

Autosomal recessive disorders of steroidogenesis, resulting in low steroids (cortisol and aldosterone), and high androgens
Virilisation and genital ambiguiity in females
Low sodium (no aldosterone)
Testicular masses (adrenal rests)
Ovaries may be normal
Dehydration

167
Q

Chronic recurrent multifocal osteomyelitis

A

Idiopathic
Female predominant
Uncertain aetiology
Diagnosis of exclusion
Fever and lethargy uncommon but can occur
Metaphysis and equivalent, most commonly tibia
Clavicle involvement characteristic but not pathognomonic - it is uncommon to have haematogenous osteomyelitis here
Early phase lytic, later sclerotic. Sclerosis is the dominant feature (so answers suggested resembles “healing osteomyelitis”
No abscesses
Antibiotics don’t help
Uncommon

Dahnert suggests may be paeds version of SAPHO

168
Q

Sclerosing ostemyelitis of Garre

A

Children and young adults
Mandible
Often associated with caries, dental infection
Supracortical bony outgrowth, with cortical onion skinning
Dahnert suggests sterile, and may be identical to chrnoic recurrent multifocal osteomyelitis

169
Q

Multilocular cystic nephroma

A

Bimodal
In kids, 3 months to 4 years, 75% male. Do not occur perinatally (to distinguish from MCDK). In addition will still see some normal kidney in this conditions unlike MCDK
In adults, female, 40-60
Multilocular cystic mass
In kids, can’t distinguish from cystic Wilms, so resected
In adults, can’t distinguish from multilocular clear cell RCC so resected

170
Q

Flared ribs

A

Achondroplasia flared anteriorly
Rickets
MPS - paddle ribs / spatulate

171
Q

Vertebra plana universalis

A 
/Platyspondyly
Morquio
Juvenile Pagets / hereditary hyperphosphatasia
Severe Gauchers
OI
Thanotophoric dysplasia
172
Q

Vertebral beaking

A 
Morquio is middle
Rest are inferior
Hurlers
Achondoplasia
Cretinism
Downs

(note MPS achondo and cretinism are on differential for Gibbus also, as well as Aperts and aquired gibbus)

173
Q

Posterior vertebral body scalloping

A 
Achondroplasia
MPS
Mass
Dural ectasia - includes NF1, but also Marfans, OI tarda
Acromegaly
174
Q

Dural mets in children

A

CNS, neuroblastoma, leukaemia - may also met to sutures

175
Q

Right aortic arch

A

About 50/50 with mirror image and aberrent left patterns
With aberrant left - 10% have congenital hear disease, mostly TOF
Mirror image branching usually associated with cyanotic disease (98% according to case review) - TOF, Truncus, Transposition, tricuspid atresia

176
Q

Left and duplicate SVC

A 
Duplication more common
Left or left of duplication usually drains to coronary sinus
5% of left associated with congenital heart disease - even more with duplication
ASD most common association
VSD
TOF
Coarctation
Anomalous PVR
Arrhythmia
177
Q

JNA staging

A

.

178
Q

78-80,141,142,144,151,173

A

.

179
Q

Osteosarcoma staging

A

.

180
Q

Proximal focal femoral deficiency

A

Congenital absence of proximal femur
More common in boys
R predominant
May be part of caudal regression syndrome

May occur with fibular hemimelia (fibular deficiency, also more common in boys)

181
Q

Ewing sarcoma grading

A

.

182
Q

Ewing sarcoma

A

Dark on T1, Bright on T2

183
Q

Osteosarc and Ewing, Mets

A

Metastasize much more commonly to lung than bone

Paeds (4 decks)

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