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Flashcards in Paeds Deck (104)
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1

Cyanotic, decreased pulmonary flow, Normal sized heart/mildly enlarged heart

- ToF
- Pulmonary atresia + VSD (variant of ToF)

2

Cyanotic, decreased pulmonary flow, Massive cardiomegaly

- Ebsteins
- Pulmonary atresia without VSD
- Non cardiac
-- Rhabdomyoma
-- Mediastinal mass
-- AV fistula
-- CDH

3

Causes of bowel obstruction in children

AIMs
A - Adhesions, Appendicitis
I - Intussusception, Incarcerated Inguinal Hernia
M - Malrotation volvulus, Meckels diverticulum

4

Most common age and sex for intussusception

3m-1y (almost all <3yrs). - above this age think of pathologic lead point.
Girls, Winter/spring (viral illness time)
Thought to be due to lymphoid hypertrophy in the mesentery.

5

X-ray findings of intussusception

Paucity of gas ascending colon,
mass within ascending/transverse colon,
SBO
(Can image left decubitus or prone)

6

Henoch-Schönlein Purpura

Small vessel vasculitis affecting skin, joints, GI tract, GU tract.
Can cause small bowel intussusception

7

Where do swallowed foreign bodies normally get stuck?

In the upper oesophagus in the thoracic inlet.

8

Causes of jaundice beyond 4 weeks age

Biliary atresia
- Absent gallbladder
- Normal uptake of radio tracer but doesn't enter the bowel (should be seen within the bowel within 15min)
Neonatal hepatitis
- Delayed uptake/excretion of radio tracer

9

Hepatic mass in 3yo with elevated AFP

Hepatoblastoma

10

Causes of hepatic masses in under 5yo

Hepatoblastoma (+ AFP)
Haemangioendothelioma
Mesechymal hamartoma
Metastatic disease (Wilms or neuroblastoma)

11

Causes of paediatric hepatic masses in over 5yo

HCC (elevated AFP)
Embryonal sarcoma, undifferentiated
Hepatic adenoma
Haemangioma
Metastatic disease

12

Multiple liver masses

Abscesses
Metastatic disease
Lymphoproliferative disease
Multiple adenomas (in association with gaucher disease or fanconi anaemia)

13

Haemangioendothelioma (liver mass)

Vascular tumour
May present with high output cardiac failure
Usually involute over weeks to months

14

Mesenchymal hamartoma (liver mass)

Considered a developmental anomaly rather than neoplasm
Predominantly cystic mass
Rare

15

Pseudomambranous colitis

Pancolitis.
Yellow plaque-like growths on the bowel wall (pseudomembranes)
Severe wall thickening with mucosal hyper enhancement.
Not much mesenteric or soft tissue involvement.

16

Neutropenic colitis

Ascending colon and terminal ileum
Adventitial hyperenhancement and pericolonic fluid
Necrosing inflammation with ischaemia and secondary bacterial infection.

17

Graft vs Host (bowel)

Usually in bone marrow patients
Donor T cells attack the mucosa of the host and may replace it with granulation tissue.
Hyperenhancement of the mucosal layer. May or may not have wall thickening throughout the GI tract.

18

Lymphoproliferative Disorder

Typically in post-solid organ transplants.
Proliferation of EBV infected cells (host usually not previously infected with EBV).
Lymphoma like growth, though more likely parenchymal masses than lymphadenopathy.
Usually occurs in the vicinity of the transplant.

19

Vesicoureteric reflux grading

1 - Ureter only
2 - Ureter and calyces (no blunting)
3 - Blunting of the calyces
4 - Tortuous dilation of the collecting system
5 - Very tortuous and dilated ureter.

20

Duplex kidney. What abnormality typically occurs in the upper and lower moieties

Upper pole - Obstruction secondary to ureterocele (both start with vowels). Ectopic ureter insertion medial and inferior to lower pole ureter.
Lower pole - Reflux. (both start with consonants)

21

Drooping lily sign

When there is contrast reflux into the lower moiety and the lower moiety is pushed inferiorly secondary to the obstructed upper pole moiety.

22

Primary megaureter

Hirschprung equivalent in the ureter
Distal aperstaltic part is narrowed and relatively obstructed.
More common on the left and in boys.

23

Urachal anomalies

Patent urachus
Urachal diverticulum
Urachal cyst
Urachal sinus

24

Prune belly syndrome

Hypoplasia abdominal walls
Urinary tract abnormality
Cryptorchidism
(females can't have cryptorchidism so can only have pseudo-prune belly)

25

Infantile autosomal recessive polycystic kidney disease

Small renal cysts predominate. (little hepatic fibrosis)
Enlarged kidneys. Echogenic.

26

Juvenile autosomal recessive polycystic kidney disease

Hepatic fibrosis dominates
Hepatosplenomegaly. Portal hypertension.
Kidneys may have visible cysts or appear normal.

27

Autosomal dominant polycystic kidney disease

Usually first identified in adults.
Cysts increase in size and number with age and may compress/destroy to normal parenchyma.
Cysts can be found in liver and pancreas. Associated with berry aneurisms (10%).

28

Things to assess with wilms tumour

Where in kidney
Renal vein/IVC involvement
Ureter position (anterior or posterior)
Contralateral kidney involvement
LN and lung/liver mets

29

Associations with Wilms Tumour

Usually presents around age 3
associated with overgrowth syndromes (hemihypertrophy, Beckwith-Wiedemann syndrome

30

Wims tumour appearances c.f neuroblastoma

Grows as a ball from within kidney
Pushes vascular structures aside, goes anterior to aorta
Invades renal vein/IVC
Calcification rare (curvilinear)
20% lung mets

31

Neuroblastoma appearances c.f Wilms Tumour

Surrounds and engulfs blood vessels
Calcifications common, stippled (85%)
No vascular invasion
Pushes kidney inferiorly.
Not commonly causing lung mets.

32

What is nephroblastomatosis

Plaque-like nephrogenic rests which may act as precursors for Wilms Tumour.

33

Multilocular Cystic Nephroma

Cystic mass with multiple septations, benign. Cannot be differentiated from Wilms Tumour on imaging.

34

Differentials on imaging for Wilms Tumour

Multilocular cystic nephroma
Mesoblastic nephroma (fetal renal hamartoma)

If older child more likely to be RCC.

35

Mesoblastic nephroma

Fetal renal hamartoma.
Most common renal mass in neonates.
Solid infrarenal mass with variable enhancement.
Indistinguishable from Wilms.

36

Angiomyolipoma

Fatty infiltrating mass of the kidneys
Associated with tuberous sclerosis
Haemorrhage into AML most common cause of death in patients with tuberous sclerosis (larger lesions >4cm associated with dysplastic arteries and aneurisms).

37

3 most common malignancies of childhood

Leukaemia
Primary brain tumour
Neuroblastoma

38

Neuroblastoma stage 4s

Age less than 1yr
Primary tumour with mets to skin, liver or bone marrow

Often require no treatment and has 100% survival.

39

Neuroblastoma staging

1 - Confined to organ of origin (90%)
2 - Outside of organ of origin but not crossing centre line (75%)
3 - Crosses centre line (30%
4 - Disseminated disease (10%
4s - under 1yr, Primary tumour and mets to skin, liver bone marrow (100%)

40

Testicular germ cell tumours

Seminoma (40%)
Non-seminomatous (60%)
- Yolk sac
- Embryonal
- Teratoma
- Choriocarcinoma
- Mixed (seminomatous and non-seminomatous)

10% of testicular neoplasms are not germ-cell
- Lymphoma, leukaemia, Rhabdomyosarcoma**

41

What is Sinding-Larsen-Johansson syndrome?

Chronic avulsion of the patellar tendon at it's attachment to the inferior patellar.

42

TORCH infections?

T - toxoplasmosis
O - Other (syphilis)
R - Rubella
C - Cytomegalovirus
H - Herpes

43

Features of congenital Rubella infection

Sensorineural deafness
Cataracts
Cardiac (VSD, ToF)
--- These three make up Gregg syndrome---
Intellectual disability
Microcephaly
IUGR
Bony changes
- Frayed metaphases, celery stalking

44

Wimberger corner sign

Erosion of medial metaphysis of the proximal tibia
Quite specific for congenital syphilis.

45

Caffey disease

Infantile cortical hyperostosis
Irritability, fever, tender over periostitis.
Usually involves mandible, clavicle, ribs, scapula, arms, femur

46

Causes of permeative bone lesions in child YOUNGER than 5yrs

Langerhans cell histiocytosis
Osteomyelitis
Metastatic neuroblastoma

47

Causes of permeative bone lesions in child OLDER than 5yrs

Lanherhans cell histiocytosis
Lymphoma/leukaemia/osteomyelitis
Ewing sarcoma

48

Types of LCH

**Multiple organs, multiple sites
- Letterer-Siwe disease (acute disseminated LCH). Poor prognosis

**Single organ, Multiple sites
- Hand-schüller-Christian disease (usually skeletal involvement, may also have hepatosplenomegaly, exophthalmos,dermatitis, growth restriction

**Single organ, single site
- Eosinophilic granuloma

49

Most common sites for Ewing Sarcoma

Femur > Pelvis (2/3 in these locations) > Tibia > Humerus > Ribs.
Metadyaphyseal > Diaphyseal > Metaphyseal

50

Gene changes with ewing sarcoma

T(11;22)(q12;q24) translocation. (90%)
Most have EWS/ETS gene fusion (85%)

51

Most common sites for stress fractures

Tiba > Fibula > Metatarsals > Calcaneus

52

Most common site of metastatic spread osteosarcoma

Lungs and bone.
More likely to metastasise if in axial flat bones (which is more likely in an older age group.

53

Rate of malignant degeneration in osteochondromatosis

5%

54

Rate of malignant degeneration in enchondromatosis (Olliers disease)

30%.
Lesions tend to be in the metaphyses of long bones rather than in the hands/feet like solitary lesions.

55

What is McCune-Albright syndrome?

Polyostotic fibrous dysplasia, skin pigmentation, endocrine abnormalities (eg precocious puberty in girls).

56

Radiographic changes in Mucopolysaccharidoses (defects in lysosomal enzymes). AKA dysostosis multiplex.

- Anterior beaking of vertebral bodies
- Focal lumbar kyphosis (gibbous deformity)
- Tall and flared iliac wings + steep acetabular angles
- Gracile and dysplastic hips
- Squared and proximally tapered metacarpals

57

Some problems associated with osteopetrosis

- Defective osteoclasts
- High body calcium but low serum calcium (superimposed rickets)
- Thickened skull base causes tightness around cranial nerves
- Small marrow causes pancytopaenia

Looks like sclerotic bone with bone in bone appearance.

58

Most common bacteria for septic arthritis in children

Staph aureus, Grp A strep.

59

Osteosarcoma is associated with what condition?

Congenital retinoblastoma.

60

In idiopathic scoliosis, what direction does the thoracic curvature normally go?

Convex to the right.

61

What is the index for bone maturity (over the iliac wings...)

Risser index.
Grade 1 - lateral 25%
Grade 2 - Lateral 50% etc.
(3 - 75%. 4 - 100%. 5 - Apophysis fused to iliac wing)

62

Skeletal findings in severe anaemia (sickle cell, thalassaemia)

- Widening of the marrow and thinning of the cortex
- Widening of the diploid space of the skull with 'hair on end' appearance (particularly thalassaemia)
- Indented or flattened vertebral body endplates.

Also look for cardiomegaly, enlarged spleen (small and calcified in sickle cell from auto infarction), gallstones.

63

Tibial changes with neurofibromatosis type 1

Anterior bowing
Sclerosis
Cortical defects
Pseudoarthrosis
Twisted (ribbon-like) ribs

64

Some features of dermatomyositis

Autoimmune inflammation of muscles + skin rash
Typically affects anterior/lateral thigh muscles
Subcutaneous oedema and calcifications are hallmarks of chronic transformation.

65

Differentials for soft tissue malignancy in children

Infants - Fibrosarcoma
Children - Rhabdomyosarcoma

pNET
Synovial sarcoma
Aggressive fibromatosis (locally aggressive, high T2 signal).

Also consider :
Myositis ossificans
Chronic foreign body
Fat necrosis

66

Likely diagnosis of child with torticollis and asymmetric appearances of sternocleidomastoid.

Fibromatosis Colli.

67

MR spectroscopy. What does Choline, Creatine and Lactate represent?

They should increase from left to right, like a plane taking off (cho-cr-naa)
- Choline - Synthesis and breakdown of cell membranes (cell turnover)
- Creatine - Stable for many disorders. Increased in hypometabolism, decreased in hypermetabolism.
- NAA - Neuronal marker (associated with myelin). Decreased when there is less or absence of brain tissue
- Lactate - elevated in ischaemia, infarct, high grade tumours, abscess.

68

Posterior fossa tumour with restricted diffusion?

Medulloblastoma (hypercellular)
Ependymoma and astrocytoma do not

69

Grading of germinal matrix haemorrhage

1 - Confined to germinal matrix
2 - Intraventricular extension
3 - Ventricular distension
4 - Intraparenchymal haemorrhage

70

Periventricular leukomalacia

Infarct of the watershed white matter in infant
USS show heterogeneous appearance followed by cystic degeneration and eventually volume loss. T2 hyperintensity later in life.

71

Features of chiari 2 malformation

- Cerebellum, medulla and 4th ventricle within the cervical canal.
- Increased massa intermedia (just inferior to 3rd ventricle)
- Tectal beaking
- Interdigitation of cerebral gyri through fenestrated falx.
Inferior pointing of lateral ventricles.
- Hydrocephalus.

72

Three types of holoprosenncephaly

Alobar
Semilobar
Lobar

73

Alobar holoprosencephaly

No separation of brain, ventricles. No falx. No corpus callosum.
Usually stillborn or neonatal death.

74

Semilobar holoprosencephaly

Posterior partial cleavage
- Temporal horns formed
- Single anterior ventricle
- Partial separation of thalami.
- Midline structures may be present posteriorly (falx, corpus callosum).
- Single ACA.

75

Lobar holoprosencephaly

Failure of cleavage of frontal horns
Occipital and temporal horns well formed
Absent septum pellucidum.
Corpus callosum absent or dysplastic.

76

Posterior fossa cyst - differentials

Dandy walker malformation
Dandy walker variant
Arachnoid cyst
Mega cisterna magna

77

Dandy walker continuum

DW malformation:
- Complete or partial vermis agenesis
- Posterior fossa cyst communicating with 4th ventricle
- Elevated torcula
- Absent falx cerebelli
- Often associated with holoprosencephaly, schizencephaly, agenesis corpus callosum, heterotopias, polymicrogyria.
DW variant:
- Hypoplastic vermis

78

What is schizencephaly?

Cleft from ependyma (ventricles) to pia which is lined by grey matter.
Can be open lipped or closed lipped.
Thought to be migrational rather than infarct (porencephaly).

79

What is Lissencephaly?

Arrest of migration of neutrons
Results in agyria or pachygyria (small gyri).
Polymicrogyria is possibly similar but controversial.

80

Possible appearances with vein of galen malformation

High output heart failure on CXR
- Enlarged heart
- Widened mediastinum
- Pulmonary oedema
Increased vascularity/vascular mass at the posterior aspect of 3rd ventricle.

81

What is the difference between porencephaly and encephalomalacia?

Porencephaly
- infarct before end of 2nd trimester. No glial scar. Cyst forms, thin-walled, communicates with ventricles. No septations.
Encephalomalacia
- infarct in third trimester. Glial scar formation. High T2 signal with multiple separations.

82

What is hydranencephaly?

Complete destruction of the parts of the brain supplied by anterior circulation, secondary to occlusion of bilateral ICA.
Usually remaining = thalami (not fused), falx (therefore not holoprosencephaly), occipital and inferior temporal lobes, brainstem.

83

Diagnostic features of NF1

CAFE SPOT
C - Cafe au lait lesions
A - Axillary/inguinal freckling
F - Fibromas (neurofibromas x 2, or plexiform neurofibroma)
E - Eye hamartoma (lisch nodules)
S - Skeletal abnormalities (gracile bowing, sphenoid wing)... not part of diagnostic criteria...
P - Positive family Hx
OT - Optic Tumours (Optic Glioma)

84

Features of NF2

MISME
Multiple Inherited Shwannomas, Meningiomas, Ependymomas.

85

Sturge Weber

Port wine stain, usually in V1 (ophthalmic division, trigeminal nerve) distribution.
Pial angiomas (result in altered flow to the brain and resultant ischaemic changes.
- Subcortical calcification, increased veins (intra and extra axial), ipsilateral atrophy, increased size choroid plexus.

86

Tuberous sclerosis

Multiple hamartomas affecting brain, kidneys, eyes.
Vogt Triad - Seizures, Intellectual disability, adenoma sebaceum.
See subcortical tubers, subependymal tubers and giant cell astrocytomas (large tuber near foramen of monroe).
Renal cysts and AML
Cardiac rhabdomyomas

87

Radiologic findings of NF1

"NF1 spots" - high T2 lesions in basal ganglia, brainstem, cerebellum.
Optic tract glioma
Cerebral astrocytoma
Hydrocephalus
Vascular dysplasia (aneurisms, moyamoya)
Sphenoid wing dysplasia
Nerve sheath tumours
Scoliosis
Lateral meningocele.

88

Classic finding (diagnostic) of NF2

Bilateral acoustic schwannomas.

89

Neurological findings of cytomegalovirus (most common TORCH infection)

Periventricular calcifications and dilation of the ventricles.

90

Toxoplasmosis neurological findings (2nd most common TORCH infection)

More scattered cerebral calcifications compared with cytomegalovirus.

91

Cause and findings of subacute sclerosing panencephalitis

Cause - reactivation of latent measles.
Findings - Increased T2 signal throughout the periventricular white matter, cerebral atrophy, ventricular dilation.

92

Cause and findings of ADEM

Cause - post viral or post immunisations
Findings - Patchy areas of increased T2 signal of the white matter

93

Brain appearances of HSV-1 infection

Can lead to necrotising meningoencephalitis.
If secondary to reactivation and migration along the trigeminal nerve, can cause oedema/necrosis in the temporal lobe(s).
If systemic infection, any part of the brain can be affected

94

Posterior fossa tumours

Cerebellar astrocytoma (most common)
Ependymoma
Brainstem glioma
Medulloblastoma

95

Imaging features of cerebellar astrocytoma

Best prognosis - often only require resection.
From within cerebellar vermis (pushes the 4th ventricle anteriorly).
If cystic has an enhancing nodule.
If solid has heterogeneous enhancement.
Well-defined.
No calcification or haemorrhage.

96

Imaging features Medulloblastoma

Most malignant tumour. Considered a pNET.
From inferior medullary velum
Fills the 4th ventricle from the roof with poorly defined margins into the cerebellum
Hyperdense and diffusely enhancing CT
Mild hypo/hyperintesity T2.
Typically hypercellular (diffusion restricting).
Seed easily into the subarachnoid space and central canal.

97

Imaging features of brainstem glioma

Moderately aggressive version of astrocytoma
Typically from within the pons (can be exophytic).
Pushes 4th ventricle posteriorly.
Non-enhancing.
Enlargement of the brainstem.
Doesn't cause hydrocephalus.
Presents with cranial nerve symptoms.

98

Imaging features of ependymoma

Benign lesion of the ciliated ependymal lining of the 4th ventricle.
Typically arises from the floor of the 4th ventricle. (60%)
May extend through foramina into the spinal canal or cisterna magna.
Hetergeneous appearance and enhancement on both CT and MRI.
Often calified (70%) and have well-defined margins.

99

Cerebral tumours

Glial origin neoplasm
- Glioblastoma, astrocytoma, oligodendroglioma, ganglioglioma
Choroid plexus tumours
- Papilloma or sarcoma
pNET
Ependymoma

100

Suprasellar tumours

Craniopharyngioma( epithelial cell within rathkes cleft or pituitary stalk. Can be more cystic + calcium (adamantinomatous) or solid without calcium (papillary))
Pineal tumour (pineoblastoma, pineocytoma)
Optic glioma
Germ cell tumour

101

Things to consider with orbital cellulitis

Pre or post septal involvement (line drawn across the bony confines of the orbit approximates the border)
Post septal is normally associated with ethmoid sinus disease.
Drainable collection or not
Usually extraconal and subperiosteal.

102

Cyst along the anterior border of SCM is most likely....?

Branchial cleft cyst.
(2nd branchial cyst most common, usually at the angle of the jaw)

103

Cyst centrally at the base of the tongue

Thyroglossal duct cyst.

104

Klippel-Feil syndrome

2 or more segmental fusion of vertebra
Also may have sprengle deformity and omovertebral bone
Short neck, low hairline.
Associated with GU deformities, congenital heart disease.