Paediatrics

Question 1

Mesial temporal sclerosis - MRI features?

Answer 1

Small volume hippocampus, high T2 signal

Question 2

Leigh disease - 3 symptoms?

Answer 2

Ataxia, dystonia, ophthalmoplegia

Question 3

Leigh disease - MRI feature, blood feature?

Answer 3

High T2 signal in putamen
Elevated lactate: pyruvate ratio

Question 4

What is adrenoleukodystrophy?

Answer 4

X-linked demyelinating disease with hyperintense T2 signal affecting the occipital lobe.

Question 5

What does a neuroblastoma do that a nephroblastoma doesn’t?

Answer 5

Encasement of the aorta

Question 6

What is Kartagener’s syndrome?

Answer 6

Triad of situs inversus, sinusitis and bronchiectasis

Question 7

What is claw sign?

Answer 7

Rim of renal tissue around a mass - mostly in Nephroblastoma

Question 8

What sign is associated with neuroblastoma?

Answer 8

Dropping lily sign - kidney is displaced downwards

Question 9

Nephroblastoma vs Neuroblastoma - effect on IVC?

Answer 9

Neuroblastoma encases
Nephroblastoma displaces or invades

Question 10

Nephroblastoma vs Neuroblastoma - calcification commonality and pattern?

Answer 10

More common in Neuroblastoma - regular & stippled
Nephroblastoma - less common. Curvilinear & phleboliths

Question 11

Nephroblastoma vs Neuroblastoma - crossing over the midline?

Answer 11

Nephroblastoma - less common
Neuroblastoma - more common

Question 12

Age at onset of: hepatoblastoma vs infantile haemangoendothelioma vs HCC?

Answer 12

IHE - <6 months
Hepatoblastoma - 6m - 3yrs
HCC - >5 years

Question 13

Which paediatric liver lesions have a raised aFP?

Answer 13

Infantile haemangioendothelioma
Hepatoma, Hepatoblastoma

Question 14

Features of Hepatoblastoma? (2)

Answer 14

Coarse calcifications, high T2 signal

Question 15

HCC Feature?

Answer 15

Central scar which can calcify

Question 16

Infantile haemangioendothelioma features? (3)

Answer 16

heart failure, vascularity in lesion, fine granular calcifications

Question 17

Beckwith-Wiedemann syndrome predisposes to…?

Answer 17

Wilms tumour

Question 18

Tuberous sclerosis predisposes to…?

Answer 18

Angiomyolipoma

Question 19

vHL syndrome predisposes to…?

Answer 19

RCC

Question 20

WAGR syndrome predisposes to…?

Answer 20

Wilms tumour

Question 21

Omphalocele definition?

Answer 21

Congenital midline defect with a surrounding membrane

Question 22

Gastroschisis - difference to omphalocele? (3)

Answer 22

No surrounding membrane
Always on the right side
Raised maternal serum aFP

Question 23

What syndrome does not predispose to Wilms tumour?

Answer 23

Birt-Hogg-Dube syndrome
- predisposes to chromophobic RCC

Question 24

Tuberous sclerosis triad of symptoms

Answer 24

Epilepsy, mental retardation and facial angiofibroma

Question 25

Tuberous sclerosis GI manifestations? (3)

Answer 25

Splenic hamartomas Renal cysts & AMLs Hamartomatous Rectal polyps

Question 26

GI manifestations of Neurofibromatosis Type 1

Answer 26

Hirschsprung disease, Carcinoid, renal artery stenosis

Question 27

GI manifestations of Neurofibromatosis Type 2

Answer 27

None!

Question 28

GI manifestations of Sturge-Weber syndrome

Answer 28

Paraganglioma

Question 29

GI manifestations of vHL Syndrome? (4)

Answer 29

Renal cysts, RCC, phaeochromocytoma, pancreatic cysts

Question 30

HIDA scan features in biliary atresia? (2)

Answer 30

Hepatic activity after 5 minutes No bowel activity after 24 hours

Question 31

T/F: Asplenia has a poorer prognosis than polysplenia

Answer 31

True

Question 32

T/F: Asplenia is associated with horseshoe kidney

Answer 32

True

Question 33

T/F: Most patients with asplenia have TAPVR

Answer 33

True

Question 34

T/F: Most patients with asplenia have an azygous continuation of the IVC

Answer 34

False

Question 35

T/F: The pancreas can be truncated in patients with polysplenia

Answer 35

True

Question 36

Which heterotaxy has a higher association with congenital heart disease?

Answer 36

Right sided isomerism (asplenia)

Question 37

Left sided isomerism features - spleen, IVC, lungs, liver, bowel, pancreas and heart features?

Answer 37

Spleen - multiple little spleens IVC - interrupted IVC with azygos or hemiazygos continuation Lung lobes - two on either side Liver - situated centrally in the abdomen Bowel - malrotation Pancreas - truncated Heart - ASD, VSD, PAPVR (less severe congenital heart disease)

Question 38

Right sided isomerism features - spleen, IVC, lungs, liver, bowel, pancreas and heart features?

Answer 38

Heart - TAPVR and other anomalies (Almost 100% have severe cyanotic congenital heart disease) Spleen - absent Lung lobes - three on either side (specific finding) Liver - normal position (can be midline or malpositioned) Bowel - malrotation IVC - on same side as aorta (usually the right) Kidneys - associated with horseshoe kidney

Question 39

Which eponymous syndrome is most likely to cause Hypertension in kids?

Answer 39

Takayasu arteritis. Can cause occlusion of aorta or renal arteries -> HTN.

Question 40

What is CPAM? Presentation?

Answer 40

congenital Pulm. airway malformation cystic mass, maybe with an air fluid level.

Question 41

What is PIE?

Answer 41

PIE happens when barotrauma from ventilation causes rupture and air in the interstitium. There is overinflation with black streaks from the hilum.

Question 42

When does PIE become BPD? what does it look like?

Answer 42

more gradual, after a few weeks of ventilation.

Question 43

What does BPD look like on CXR?

Answer 43

Shows up as hyperinflation, opacification and air bronchograms. coarse linear lucencies eventually.

Question 44

When does TTN resolve by?

Answer 44

Peaks at 24hrs. About 3 days after birth.

Question 45

What is Swyer-James syndrome and what does it look like on CXR.

Answer 45

normal development of the infant lung is impeded by bronchiolitis (viral or mycoplasma) at an early age, with superadded acute airspace destruction. Consequently the affected lobe is small and lucent on the CXR due to air trapping.

Question 46

What is infradiaphragmatic TAPVR? and how does it present?

Answer 46

the venous return from the lungs is via the portal vein or IVC. As the anomalous pulmonary vein passes through the diaphragm (at the oesophageal hiatus) it becomes compressed, leading to an acute presentation in the early neonatal period with pulmonary oedema.

Question 47

What causes the "gloved finger sign" + LUL hyperexpansion?

Answer 47

Bronchial atresia

Question 48

cerebral palsy + lower lobe bronchiectasis?

Answer 48

Aspiration pneumonia

Question 49

What soft tissue mass of the heart is associated with tuberous sclerosis?

Answer 49

rhabdomyomas

Question 50

What are the five Ts of cyanotic and plethoric congenital heart disease?

Answer 50

Tingle - i.e. Single - ventricle. Tricuspid atresia Transposition of the great vessels TAPVC Truncus arteriosus

Question 51

What valve abnormality is commonly associated with aortic coarctation?

Answer 51

Bicuspid aortic valve.

Question 51

Ring enhancing lesions mnemonic?

Answer 51

MAGIC DR Mets, abscess glioma, infarction, contusion, demyelination and radiation necrosis

Question 52

Where are the following neck masses located? Cystic hygroma, second branchial cleft cyst

Answer 52

Cystic Hyg - posterior triangle 2nd Branch - between subM gland and SCM muscle

Question 53

Best MRI sequence for Tuberous sclerosis?

Answer 53

Coronal FLAIR MRI

Question 54

DNET tumour characteristics? (3)

Answer 54

temporal lobe lesion epilepsy bubbly/cystic lesion

Question 55

commonest type of craniosynostosis?

Answer 55

Scaphocephaly

Question 56

ring enhancing lesion - how to differentiate between mets vs abscess? (2)

Answer 56

thick nodular enhancing wall = mets restricted diffusion = abscess

Question 57

The best MRI sequence for Optic atrophy?

Answer 57

Coronal CISS (t2)

Question 57

CP angle mass with T2 and FLAIR hyper enhancement?

Answer 57

Epidermoid cyst

Question 58

CP angle mass with no calcification?

Answer 58

schwannoma

Question 59

CP angle mass with frequent calcification in the dura mater?

Answer 59

meningioma

Question 60

Sinus pathology - Mucus retention cyst vs mucocele?

Answer 60

Cyst - football in the sinus, due to mucus gland blockage mucocele - blocked ostium, mucus-filled sinus

Question 61

Sinus pathology - atrochoanal polyp vs inverted papilloma?

Answer 61

polyp - expands through the ostium upwards into the nose papilloma - vascular tumour that erodes the bone beside the sinus, confirmed on MRI

Question 62

Which neck lump is a painless, compressible lump lateral to the jugular vein?

Answer 62

Branchial cleft cyst

Question 63

Neck lump with enlargement of the SCM muscle?

Answer 63

fibromatosis coli