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Prometheus Qs > Module 5: Paediatrics > Flashcards

Module 5: Paediatrics Flashcards

1
Q

most common cause of acute respiratory obstruction in young children

A

croup

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2
Q

steeple sign think

is what

A

croup
loss of normal lateral convexities of the subglotic trachea

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3
Q

H Influenza causes

A

epiglottitits

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4
Q

thumb sign is what

A

swelling of epiglottis

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5
Q

epiglottitis asphyxiation is cause by what

A

aryepiglotic folds

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6
Q

exudative infection of the trachea can cause

A

exudative tracheatitis

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7
Q

exudative tracheatitis bug is

A

Staph A

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8
Q

exudative tracheatitis buzzword sign

A

linear soft tissue filling defect within the airway

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9
Q

what is the next step is there is pseudothickening from neck not being truly lateral

in retropharyngeal cellulitiis and abscess

A

repeat with extended neck

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10
Q

Retropharyngeal abscess vs suppurative node?

A

Retropharyngeal abscess is midline

supparative node is medial to the carotid

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11
Q

subglottic hemangioma

can cause what

A

subglottic obstruction.

Like croup but will b e one sided

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12
Q

subglottic haemangiomas are associated with

A

cutaenous haemangiomas (50%0

PHACES syndrome (7%)

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13
Q

what is the PHACEs syndrom e

A

Posterior fossa
Haemgiomas
Arterial anomalies
Coarctation of aorta, cardiac defects
Eye abnormalities
Subglottic haemangiomas

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14
Q

measurements of concern on lateral xr
for retropharyngeal absess

A

C2 >6mm
>22mm at C6

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15
Q

Should adenoids encroach on the airway

A

no

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16
Q

Exudative tracheitis sign on lateral radiograph

A

linear filling defect

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17
Q

meconium aspiraiton more common in what delivery time baby

A

term or post-mature babies

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18
Q

meconium aspiraiton appearance of lungs

A

ropy
hyperinflated

20-40% have pnuemothoraxx

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19
Q

TTN affects which ne wborn (dleivery style)

A

c-section

diabetic mother
maternal sedation

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20
Q

TTN findings start at what time

A

6 horus

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21
Q

TTN is normally done by what time frame

A

3 days

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22
Q

SDD (surfatant deficiency) which babies

A

pre term

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23
Q

Chest radiograph appearance of SDD

A

low lung volums and bilateral granular opacities

NOT pleural effusion as with B haemolytic pnuemonia

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24
Q

surfactant replacement therapy risks

A

pulmonary haemorrhage
PDA

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25
Q

Neonatal pneumoina

look like what on radiograph

A

patchy
asymmetric hilar densitiies
hyper inflation

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26
Q

Beta haemolytic strep pneumonia

neonate

A

dirty birth canal

low lung volums
GRANULAR opacities
pleural effusions

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27
Q

Persistent pulmonary HTN
caused by

A

primary

secondary to hypoxia (infection, aspiraiton)

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28
Q

pulmonary interstitial emphysema

A

linear lucencies

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29
Q

PIE vs bronchopulmonary dysplasia

A

age

PIE less than 2 weeks

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30
Q

Chronic lung disease - who get this?

A

small, pre term kid.
after 2 weeks get hazy lungs.

band like opacities is a buzzword

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31
Q

Pulmonary hypoplasia causes

A

Primary or secondary

secondary
- decreased hemithoracic volume ( decreased vascular supply, decreased fluid

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32
Q

decreased hemithoracic volume what can cause this,

A

(CONGENTIAL DIAPHRAGMATIC hernia

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33
Q

types of bronchopulmonary sequestration

A

intralobar

extralobar

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34
Q

bronchopulmonary sequestration distinction is based on

A

pleural covering

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35
Q

sequestration blood supply

A

intralobar drains to pulmonary veins

extra drain to systemic veins

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36
Q

neonatal lung volumes on radiograph

if low

A

surfactant deficiency

heta haemolyticpneumonia (pleural effusion)

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37
Q

neonatal lung volumes on radiograph

if high

A

meconium aspiration

transient tachypnoea

neonatal pneumonia

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38
Q

more common?

sequestration type?

A

intralobar

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39
Q

how do people with intralobar sequestration present

A

pneumonia in their late teens / adult

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40
Q

intralobar is commonly which segment

A

left lower lobe posterior segments

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41
Q

no pleural cover for sequestration ascribed to

A

intralobar

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42
Q

Extralobar sequestration present in

A

infancy

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43
Q

what are the associations to extralobar sequstration

A

Congenital Cystic Adenomatoid malformation (CCAM)

Congenital diaphragmatic hernia

vertebral anomalies

congenital heart disease

pulmonary hypoplasia

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44
Q

why does extralobar sequestration rarely get infected?

A

has its ow pleural covering

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45
Q

bronchogenic cysts - do they connect to the airway?

if they have gas in them?

A

no

if gas in them consider infection

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46
Q

CCAM / CPAM different types

A

1 - 3
cystic
solid
3 is middle ground

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47
Q

if theres systemic arterial feeding a CCAM?

A

not a CCAM but a sequestration

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48
Q

congital lobar emphysema has preference for which lobe?

A

left upper lobe

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49
Q

how to treat congenital lobar emphysema

A

lobectomy

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50
Q

congenital Bochdalek hernia is found where?

A

back left of the diaphragm

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51
Q

associations of Congenital Diaphragmatic Hernia

A

malrotated
congenital heart disease

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52
Q

mortality of the congenital diaphragmatic hernia

A

is associated to the hypoplasia

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53
Q

what is round pneumonia

A

less than 8 years old
looks like a mass

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54
Q

Common bug for round pneumonia

A

S Pneumoniae

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55
Q

What is Swyer James

A

unilateral lucent lung

post viral lung infection in childhood post infectious obliterative bronchiolitis

the size of the affected lobe is smaller! Of note for a lucent lung

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56
Q

what causes papillomatosis ?

A

perinatal HPV

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57
Q

sickle cell kids get what in bones

A

infarcts
H shaped vertebra

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58
Q

cystic fibrosis vs primary ciliary dyskinesia

A

sodium pump broken therefore thick secretions in CF. Men are missing vas deferens.

PCD - cilia motile doesn’t work. Therefore lower lobe predominant. Men sperm not motile

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59
Q

PCK and Kartaganers relationship

A

50% will have Kartageneres (situs inversus)

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60
Q

Thmic rebound on PET

A

can be bright
and large

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61
Q

Germ cell tumour in anterior mediastinal masses - three types

A

Teratoma - calc and fat

Seminoma - bulky lobulated

NSGCT - necrotic

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62
Q

Middle mediastinal masses

A

Lymphadenopathy (TB or lymphoma)
Duplication cysts (bronchogenic or enteric)
Bronchogenic - water attenuation.
Enteric- water attenuation close the oesophagus.

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63
Q

Posterior mediasintal mass

A

Neuoblastoma

Ewing sarcoma

Neuroenteric cyst

Extramedllary hematopoiesis

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64
Q

posterio med mass - neuroblastoma

found in age -

A

less than 2

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65
Q

Askin (PNET) is now considered to be

A

past of the Ewing sarcoma .

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66
Q

Neuroenteric cyst - associated with

A

vertebral anomalised

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67
Q

What is extramedullar hematopoiesis

A

myloproliferative disorder patients, manifests with big liver and spleen.

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68
Q

mediastinal mass by age

?lymphoma

A

if over 10

if under think thymus

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69
Q

how to distinguish between a pleuropulmonary blastoma and an askin tumour

A

PPB doesn’t invade the ribs

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70
Q

Course of the umbilical venous cathter

A

umbilical vein

left portal vein

ductus venosus
hepatic vein

IVC

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71
Q

mbilica lartery cathter should sit

A

T8-10

or

L3 - L5

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72
Q

Esophageal atresia subtypes

number
ax
common type
air in stomach means

A

5

VACTERAL

N - blind oesophagus, oesophagus to trachea

H type

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73
Q

VACTERAL is WHAT

most common affected

A

Vertebral anomalies
Anal
Cardiac
TE fistula
Renal
Limb

heart and kidneys

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74
Q

Vascular ring that goes between the oesophagus and the trachea

A

Pulmonary sling

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75
Q

most common symptomatic ring

A

doulbe aortic arch

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76
Q

neonatal obstruction - high causes

A

Midgut volvulus or malrotation

duodenal atresia

duodenal web

annular pancreas

jejuna atresia

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77
Q

neonatal obstruction - low causes

A

Hirschsprung

meconium plug syndrome

ileal atresia

meconium ileus

anal atresai / colonc atresia

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78
Q

radiograph

triple bubble dx

A

jejunal atresia - caused by vascular insult during development

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79
Q

doulbe bubble with gas

A

distal gas excludes atresia.

ddx - duodenal web, stenosis or volvulus midgut

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80
Q

radiograph

multiple diffuse dilated loops

differential and management plan

A

low obstruction

enema contrast
then upper GI if that is normal

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81
Q

malrotation puts which ligament on the right

A

Trietz

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82
Q

malrotation blood vessel orientation

A

SMA to the right of the SMV

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83
Q

Corkscrew duodenum is diagnositc of

A

midgut volulus

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84
Q

pyloric stenosis age range

A

2 - 12 weeks

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85
Q

pyloric stenosis length criteria

A

4mm and 14mm

single wall and legnth

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86
Q

two types of gastric volvulus

A

organoaxial

mesenteroaxial

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87
Q

duodenal web is posiootined

A

distal to ampulla vater (bilious vomitting )

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88
Q

duodenal web is ax with what

A

downs and malrotation

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89
Q

annular pancreases causes waht in adults vs kids

A

adults pancreatitis

kids - duodenal obstruction

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90
Q

differentials for long microcolon

A

meconium ileus (CF patients)

Distal ileal atresia (fetus gets a vascular insult to cause this)

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91
Q

large bowel contrast enemea

caliber change

ddx

A

small left colon syndrome or Hirschsprungs

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92
Q

what is small left colon syndrome

A

transient functional colonic obstruction

relieved by contrast enems

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93
Q

who gets small left colonic syndrome

A

infants of diabetic mothers

(NOT ax to CF)

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94
Q

what is Hirschsprungs

A

failure of ganglion cells to migrate and innervate the distal colon

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95
Q

appearance of hirschsprungs

A

rectum is smaller than sigmoid or sawtooth pattern (spasm)

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96
Q

what is total colonic aganglionosis

A

super rare version of Hirschprungs
affects the Terminal ileus
mimic microcolon.

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97
Q

What is meconium periotonitis ?

A

calcified mass in mid abdo

sterile periotneal reaction to an intra utero bowel perforaiton

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98
Q

Imperforate anus / ectopic anus

associated to

A

tethered cord
- need US to review for this

VACTERL

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99
Q

Tender abdomen in an oler child

AIM diagnosis

A

Appendicitis or adhesion
inguinal hernia or Itussusception
Midgut volvulus / meckels

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100
Q

Appendicitis size

A

> 6mm

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101
Q

intusseption target sign size

how does it affect the tratment

A

> 2.5cm - likely to be ileocolic and so air enema

<2.5 small bowel small bowe, not getting an air enema

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102
Q

Meckels diverticulum rule of 2s

A

Persistent omphalomesenteric duct

2% of population
2 hetertopic mucosa (gastric and pancreatic)
2 ft from IC valve
2 inhes long and 2cm in diameter
present symtpoms pre-2yo

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103
Q

Which nuclear medicine scan in meckels

A

pertechnetate

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104
Q

WHAT IS an enteric duplication cyst

A

failure to canalize.
Can communicate with GI lumen.

common in ileal region.

can cause IN UTERO obstruction and perforation

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105
Q

distal intestinal obstruction syndrome

affects which patient group

A

CF

aren’t compliant with their pancreatic enzymes

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106
Q

NEC

who gets

A

prem
low birth weighty
cardiac
perinatal asphyxia
Hirschsprung

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107
Q

what does NEC look like?

A

Pneumatosis

Focal dilated bowel (especially in right lower wquadrant)

featureless small bowel (oedema)

unchanging bowel gas pattern

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108
Q

what is Gastroschisis

A

extra-abdominal evisceration of neonatal bowel through paraumbilical defect

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109
Q

Gastroschisis

membrane
sided
ax anomalies
AFP level
OUtcome
complications post repair

A

NO membrane
right sided
ax anomalies are RARE
Maternal serum AFP will be elevated.
Outcome is usually good

bad reflux

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110
Q

What is omphalocele

A

congenital midline defect with herniation of gut at the base of the umbilical cord

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111
Q

omphalocele

membrane
ax anomalies
outcomes

A

DOES have a membrane
ax tto Trisomy 18 and others
Outcomes not good

umbilical cord cysts

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112
Q

What is physiological gut herniation

A

normal around 6- 8 weeks.

does not cintain any liver

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113
Q

what to see on mesenteric adenitis

A

cluster of right lower quadrant lymph nodes

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114
Q

in heterotaxia syndromes cardiac malformation s are more comon with with side/

A

right sided

115
Q

reverse aorta /IVC more common in which heterotaxy

A

Right sided

116
Q

Liver tumours can be categorised based on

A

age

117
Q

Hemangioendothelioma

hepatoblastoma

mesenchymal hamartoma

found in which age group

A

0 - 3

118
Q

What is a hemangioendothelioma

A

Ax with kasaback - Merritt Syndrome (platelet pooling - highly vascular)

get high output HF.

119
Q

hepatoblastoma is associated with what syndromes

A

hemi-hypertrophy
wilms
beckwith weidemann

120
Q

what marker in blood is elevated in hepatoblstoma

A

AFP

but also bHcG which can cause precocious puberty

121
Q

mesenchymal hamartoma has what appearance?

A

cystic
no calc

but large portal vein feeding

122
Q

liver tumours by age over

5

A

HCC - afp also high

Fibromellar subtype - central scar

Undifferentiated embryonal sarcoma

123
Q

what is an undifferentaited embryonal sarcoma

A

ax to mesenchymal hamartoma.

cystic, more aggressive. septations and fibrous pseudocapsule.

aggressive paeds tumour.

124
Q

choledochal cysts are classified into

A

5 types

125
Q

type 1 choledochal cyst is

A

focal dilation of the CBD and is by far the most common

126
Q

Carolis

type of disease
ax
features

what is the central dot sign

A

AR disease
ax to PCKD and medullary sponge kidney

intrahepatic duct dilatation

central dot sign is portal vein surrounded by cysts

127
Q

hereditory hemorrhagic telangiectasia aslo called

A

osler weber rendu

128
Q

causes of prolonged nenatal jaundice

A

Biliary atresia

hepatittis

129
Q

paeds patient with gallstones think

A

sickle cell

130
Q

Absent gallbladder think

A

biliary atresia

131
Q

features of pancreas in CF patients

A

fibrosis - low T1 and T2
fatty change - high T1

cysts are common

132
Q

Cystic fibrosis
compication of enzyme replacement therapy is what

A

Fibrosing colonopathy

133
Q

what is schwachwan-Diamond syndomrome

A

other cause of shit pacnreas

loose stool, short and have eczema

134
Q

what is dorsal pancreatic agenesis?

A

only a ventral bud.

therefore no tail and likely to get diabetes as most beta cells are in the pancreatic tail

135
Q

paediatric pancreatic mass by age

A

1 - pancreatoblastoma

6 - adenocarcinoma

15 - solid pseudopapillary tumour of the pancreas

136
Q

renal agenesis comes in what two flavours

A

both kidneys absent (potter)

one kidney absent - reproductions associations

137
Q

what are the associations of unilateral absence of kidney

A

70% of women with unilateral agenesis
genital anomalies
- unicornuate uterus, rudimentary horn

20% men missing epidydimis and vas deferens on the same side. Seminal vesicle cyst on that side

138
Q

panckae adrenal helps discern

A

surgically absent or congenitally absent kidney

139
Q

risks of a horsehwoe kidney

A

can get mashed against the spine
drianage issues - stones, infeciton -risk of cancer . Wilms, TCC. renal carcinoid.

140
Q

Horseshoe assocaited syndromes

A

Turners syndrome

141
Q

prune belly / eagle barrett syndrome

malformiaotn complex in med

tria

A

crap abdo muscles
hydroureteronephrosis
cryptochidism

142
Q

what is congenital UPJ obstruction

A

most common congenital anomaly of the GU tract in neonates.

143
Q

Auto Rec PKD get what

A

HTn and renal failure

fibrosis of liver and abnormal bile ducts

144
Q

US features of Auto Rec PKD

A

kidneys are smoothly enlarged and diffusely echogneic.
loss of corticomedullary diferntiation

145
Q

neonatal renal vein thrombosis

associate to

A

diabetic mothers

146
Q

neonatal renal artery thrombis

cuases
preents as

A

umbilical catheter damage

present with severe hypertension

147
Q

Congenital primary MEGureter

A

dildated ureter

NOT from obstructin

148
Q

cuases of congenital mega ureter

A

distal adynamic segment (like Hirschsprungs)

reflux to the VUJ

idopathic

149
Q

retrocaval ureter can cause

A

partial obstruction and recurrent UTI

150
Q

what is the weigert - meyer rule

A

upper pole inserts inferiorly and medially.

up obstructs
low flow

151
Q

what is a ureterocele?

A

cystic dilation of the intravesicular ureter xsecondary to obstruction at the ureteral orifice.

152
Q

cobra head sign

A

ureterocele

153
Q

posterior urethral valves affect swho?

A

male infants

154
Q

vesicoureteral reflux (VUR)

A

horizontal insertion of ureters and get reflux.

155
Q

what is the bladder urachus

A

attaches bladder to the abdo wall

can get infected
can become malignant (adeno)

156
Q

renal masses by age

for SOLIDS

A

neonate
- nephrobnlastoma (Wilms)
- mesoblastic nephroma

around 4
- wilms
-lymphoma
- multilocular cystic nephroma

teenager
- rcc
- lymphoma

157
Q

what are nephroblastomatosis

A

left over rest cells beyond 36 weeks. can be normal can turn to Wilms.

won’t be necrotic

158
Q

what is a mesoblastic nephroma

A

fetal hamartoma. benign.
involves renal sinus.

can look like wilms but THINK OF AGE

159
Q

cystic renal tumours for age

0 - 3

A

MDK
- no functioning renal tissues

160
Q

can you be born with a Wilms tumour?

A

NO NO NO

161
Q

wilms ax syndromes

A

bekwith-weidmen - macroglossia, omphalocele, hemihypertrophy, cardiac, big organs
sotos - macrocephaly, retarded (CNS stuff, ugly face

WAGR- wilms, anirirdia, genital, growth retarded
Drash - Wilms, Pseudohemaphroditism, prog glomerulonephritis

162
Q

bekwith-weidmen -

A

macroglossia, omphalocele, hemihypertrophy, cardiac, big organs
sotos - macrocephaly, retarded (CNS stuff, ugly face

amnd WIlms

163
Q

how to investigate Wilms

A

never biopsy

164
Q

cystic renal mass in a 4 year old

A

Multilocular cystic nephroma
- non communicating fluid filled locules, surrounded by thick fibrous capsule

165
Q

Neuroblastoma

occur in what age group

A

very young kids

166
Q

Neuroblastoma

classically occur

A

abdo

but also thoracic and even eyes

167
Q

Neuroblastoma

staging - what upstages it ?

A

midline
contralateral positive nodes all make it stage 3.

168
Q

Neuroblastoma

ax

A

NF1
Hirschsprungs
DiGeorge
Beckwith wiedemann

169
Q

Does wilms calcify

A

no - rarely (<10%)

170
Q

neonatal adrenal haemorrhage can be caused by

A

bith truama / stress

171
Q

adrenal haeorrhage on US

A

anechoic and avscular

172
Q

most common cause of hydrometrocolpos

A

imperforate hymen

173
Q

hydrometrocolpos is associated with

A

uterus didelphys

174
Q

SACROCOCCYGEAL TERATOMA

A

most comon tumour of the fetus

175
Q

ovarian masses are typically what kind

A

dermoids / teratomas (2/3)

176
Q

what is a hydrocele?

A

collection of serous fluid.

painless scrotal swelling.

patent processus vaginalis

177
Q

complicated hydrocele (one with septations)

A

hematocele or pyocele

clinical distinction

178
Q

child

scrotal pain acutely

ddx

A

Testicular appendage torision
testicular torision
epididymo-orchitis

179
Q

part of the epididymus is affected with epididymitis

A

head

180
Q

isolated orchitis the

dx is

A

mumps

181
Q

torsion of the testicular appendages

most common cause of acute scortal pain in which age group

A

7- 14 years

182
Q

torsion of the testicular appendages

what sign is there on clinical

A

blue dot sign

183
Q

testicle torsion can be caused by

A

failure of tunica vaginalis and testis to connect

184
Q

extra testicular mass

A

will be rhabdomyosarcoma

185
Q

testicular microlithiasis - what to do?

A

6 month follow up then yearly

highly depbated

186
Q

Testicular cancer categories

A

Germ Cell
-seminoma
- non seminoma (teratoma, yolk sacs, mixed germ cells)

Non germ cell
–sertoli and leydig

187
Q

which germ cell tumours are seen in first dcade of life

A

yolk sac tumour and teratoma

188
Q

Marker in Yolk sac tumour

A

AFP super elevated

189
Q

Sertoli cell tumours have what appearance

A

burnt out tumour with scar and calc.

190
Q

sertoli cell tumour assocaited to..

A

Peutz Jeghers

191
Q

Why can lymphoma hide in tests

A

blood testes barrier

192
Q

testicular lymphoma appearance

A

multiple hypoechoic masses

193
Q

in kids, fractures, get periosteal reaction in how many days s

A

7- 10 day

194
Q

list Salter Harris classifications 1 - 5

A

SALTR
slipped.
Away form the joint
Lower.
Through.
Ruined, compression

195
Q

what is a toddlers fracutre

A

oblique fracture of the midshaft of the tibia in a child learning to wlak

196
Q

where do kids get stressed fracture

A

calcaneus - after a cast is removed
tibia - proximal posterior aspect

197
Q

common paeds elbow fractures

A

Lateral condylar fracture
medial epicondyle fracture

198
Q

uncommon paeds elbow fracture

A

medial condyle and lateral epicondyle

199
Q

Nursemaids elbow is what

A

arm is pulled on, radial head can sublux ino the annular ligament

200
Q

what is sinding-larsen-johansson

A

chronic traction injury on

insetion of the patellar tendon on the patella

in contrast to osgood schlatter

201
Q

what is a cortical desmoid?

A

lucency at the back of the feumur condyles.

scoop like defect

provs a chronic tug lesion from the adductor magnus

202
Q

what is Blounts disease

A

tibia vara

eg. Rickets.

203
Q

Congenital rubella can affect neonatal bones how?

A

celery stalk appearance.
lucency of the metaphysis.

appear in first week of life

204
Q

Syphilis neonatal bone changes

specific sign is

A

Wimberg sign
- periosteal reaction along medial portion of the proximal metaphysis of the tibia

205
Q

Caffey disease is a disorder comprising of

A

soft tissue swelling
periosteal reaction
irritability

206
Q

Caffey and hot bone scan

classically affecting

A

the mandible

clavicle and ulna are other sites

207
Q

How does protaglandin therpay affect neonatal bones

A

causes periosteal reaction (shoulders)

often in kids who have had PDA kept open from prostaglandin therapy E1/E2

208
Q

what is physiologic periostitis?

A

happends around 3 months, resolve by 6

Femur is involved then the sital tibia.l

209
Q

Langerhans cell histiocytosis is also called what

A

EG

eosinophilic granuloma

210
Q

skull classic appearance in LCH

A

bevelled edges

211
Q

LCH in the spine get

A

vertebra plana

212
Q

osteomyelitis in the bone end changes with age

why

A

to do with the epiphyseal plate.

very young has vessels across the plate, then these regress so there is no blood transport of bacteri across.

but once the palte fuses the infection can get across again

213
Q

brachydactylu means

A

short fingers

214
Q

syndactyly means

A

fused fingers

215
Q

camptodactyly means

A

contractures of fingers

216
Q

Clinodactyly meanss

A

inclinded fingers

217
Q

amelia means

A

limb is absent

218
Q

meromelia means

A

limb is mostly absent

219
Q

acromelic means

A

hands and feet are short

220
Q

mesomelic means

A

forearm and lower leg are short

221
Q

Rhizomelic means

A

femur and humerus are short

222
Q

micromelic means

A

short all over

223
Q

which type of melic is achondroplasia

A

rhizomelic

224
Q

vertebral bodies in thantophoric dwarfism are

A

flat

225
Q

telhpone receiver femur

A

thanotrophic dwarfism

226
Q

blue eyes, hearing impairement and lucent skull / multiple fractures

A

osteogenesis imperfecta

227
Q

describe some of the bone features in osteogenesis imperfecta

A

fibula longer than tibia

hyperplastic callus around multiple fractures

lucent skull

wormian bones

flat/beaked vertebral bodies

228
Q

bone in bone appearanve of vertebra relate to

A

osteopetrosis

229
Q

what is pycnodysostosis?

A

osteopetrosis
wormian bones
acro-osteolysis

wide obtuse mandible

230
Q

what is Klippel FeiL?

A

congenital fusion of the cervical spine

high riding scapula

231
Q

list three mucopolysaccharidoses

A

Hunters / Hurlers / Morquio

232
Q

vertebrla body shape in the muopolysaccharidoses

hands

A

oval shaped with anterior beak

hands - wide metacarpal bone with proximal tapering

233
Q

Gauchers is what kind of disease

A

Lysosomal storage disease

234
Q

Signs of gauchers

A

big spleen
big liver

avn of femoral heads
H shaped vertebra
bone infarcts

Erlenmeyer flask shaped femurs

235
Q

what is a caudal regression syndrome

A

spectrum of disease

sacral / coccyx agenesis.

VACTERL and currarino traids syndromaes assocaited

236
Q

what does talipes mean

A

congenital

237
Q

what does Pes mean

A

foot

or

acquired

238
Q

what does equines mean in context of foot

A

plantar flexed ankle

239
Q

cavus foot

A

high arch

240
Q

hindfoot valgus get what

A

lose the longitudinal arch as the talus has slid forward

241
Q

hindfoot valgus causes

A

flat foot

242
Q

dindfoot varus causes

A

club foot

243
Q

Talocalcaneal coalition happens where

calcaneonaviuclar coaliton happens where

A

Middle facet

Anterior facet

244
Q

what sign exists for the talocalcaneal coalition?

A

continuous C sign

245
Q

vertical talus (equinus hindfoot valgus) is associated with what

A

myelomeningocele

246
Q

sex dominance in DDH

A

female

247
Q

DDH occurs due to

A

abnormal position in utero

more common in oligohydramniotic pregnancies

248
Q

what are the risk factors for DDH

A

Female
irstborn baby
family histroy
breech
oligohydramnios
metatarsus adductus
spina bifida

249
Q

normal alpha angle in Graf measurement s

A

60 degrees

250
Q

Slipped Upper Femoral epiphysis

what is it and what imaging needsd to be done

A

Salter Harris 1 through the femoral physis - needs to be fixed .

Frog leg view is the radiographs needed

251
Q

Male / Female preference of Perthes

A

boys

ages are 5 - 8

252
Q

what sign tolook for on perthes frog leg view

A

subchondral lucency / crescent sign

253
Q

age difference of Perthes vs SUFE

A

Perthes are younger 5 - 8

SuFE is teenager

254
Q

fraying , cupping and irregularity along the physeal margin

whats the diagnosis

A

rickets

255
Q

what is the anterior rib ends appearance in rickets

A

rachititc rosary

256
Q

why does scurvy not occur before 6 months

A

maternal buffer

257
Q

scorbutic rosary is assocaited to

A

scurvy

258
Q

lead poisoning can cause what in the leg

A

wide sclerotic metaphyseal line

(doesn’t spare the fibula as might be expected in a normal vairant line)

259
Q

what are the differentials for lucent metaphyseal bands

A

leukaemia
infection (TORCH)
neuroblastoma mets
endocrine (rickets and scurvy)

260
Q

high supispicion fracturefor NAT

A

metaphyseal corner fracture/bucket handle (non ambulatory child)
posterior rib fracutres

non ambulatory todler fracture

261
Q

which kind of skull fracture might be llegit accidnetal

A

parietal

262
Q

Dating a fracture

A

Periosteal reaction
- less than a week

complete healing
- 12 weeks

263
Q

which fractures heal without a periosteal reaction

A

metaphyseal
skull
costochondral junction

264
Q

Classic NAI mimics

A

Rickets and OI - multiple fractures

265
Q

what is choanal atresia

imaging

ax

A

oronasal membrane seperates the nasal cavity from the oral cavity

Thickened vomer on imaging

ax with charge

266
Q

Congential piriform aperture stenosis

causes what to not form

further imaging

A

primary palate doesn’t form

have to image the brain as midlinedefects are associated

267
Q

most common type of branchial cleft cyst is

A

2nd

268
Q

why does fibromatosis coli look like there are two sometimes

A

two heads to SCM

269
Q

Retinoblastoma

what is the significance of unilateral, bilateral and ?trilateral disease

A

unilateral - sporadic

bilateral and tri (pineal) - related to Chrom 13 auto dom.

270
Q

RB suppressor gene on chrom 13 links to what tumours

A

melanoma
fibrosarcoma
osteosarcoma
retinoblastoma

271
Q

what will retinoblatoma have on imaging

A

calcification

272
Q

what causes ischaemia in a tethered cord

A

growth of spine quicker than cord, and pulling on the cord.

273
Q

Secondary causes of tethered cord

A

myelomeningocele
filum terminale lipoma
trauma

274
Q

what should get screened for tethered cord

A

anal atresia

275
Q

which kind of dimples around the gluteal crease need screening

A

high dimples above the level of the crease

276
Q

PERIVENTRICULAR LEUKOMALACIA IS CAUSED BY

A

`ischaemia to watershed areas.

after 1 -3 weeks get cystic change

277
Q

Germinal matrix haemorrhage seen in

A

prem babies.

278
Q

What age is germinal matrix only present in the caudothalamaic groove

A

32 weeks

nowhere by 36 weeks.

279
Q

Premature suture closure

dolichocephaly

A

sagittal

280
Q

Premature suture closure

trigonocephaly

A

metopic

pointed forehead

281
Q

Premature suture closure

coronal

A

brachycephaly

282
Q

Premature suture closure

plagiocephaly

A

unilateral lamboid

283
Q

Premature suture closure

bilateral lamboid

A

turricephaly

284
Q
A

Prometheus Qs (9 decks)

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