Paediatrics Flashcards
1
Q
What is distal femoral cortical irregularity
A
Distal femoral cortical irregularity has also been referred to as “cortical desmoid”, “cortical irregularity syndrome” and “distal femoral defect”. The typical radiological appearance is a radiolucent cortical defect with adjacent sclerosis. It is often asymptomatic and is almost an incidental finding.
2
Q
What is choanal atresia?
A
Congenital occlusion of the nasal cavity from the nasopharynx - the ‘choanae’
Maybe due to bone or soft tissue
3
Q
What is CHARGE syndrome ?
A
Colombo - gap in retina
Heart defects
Atersia of the choanae
Retardation of development
Genitourinary anomalies
Ear anomalies
4
Q
What are the key features of a pyriform aperture stenosis
A
Piriform aperture < 11mm
Hypoplasia of the hard palate
Medial displacement of the maxillary nasal process - i.e. it moving inwards to make the piriform aperture smaller
5
Q
What virus causes croup
A
Parainfluenza virus
6
Q
What virus causes epiglottitis ?
A
H.influenza virus
7
Q
What is Lemierre syndrome ?
A
Post oral infection - such as tonsillitis. There is extension into the internal jugular vein. This then causes septic pulmonary emboli or mediastinitis - mediastinal extension via the danger space
8
Q
What bug is responsible for exudative bacterial tracheitis ?
A
S.aureus
9
Q
What is the most common congenital diaphragmatic hernia ?
A
Bochdalek - back on the left
Causing bowel to herniate
Can result in pulmonary hypoplasia
10
Q
What is Congenital diaphragmatic hernia associated with ?
A
Bowel malrotation
Neural tube defects
Tuners syndrome
Trisomy 21
11
Q
What is sequestration?
A
Aberrant lung tissue with systemic blood supply- usually arising from the thoracic aorta
Most common in the left lower lobe
12
Q
What virus causes bronchiolitis
A
RSV
13
Q
What typically causes round pneumonia ? what does it look like?
A
S. Pneumoniae - looks like a round pulmonary opacity with well-defined margins, mimicking a mass
14
Q
What are the causes of bronchiolitis obliterans syndrome ?
A
Final common pathway of small airway obstruction:
Causes include allogenic lung and bone marrow transplant - post lung transplant
Post infectious
Related to toxins / drug exposures
15
Q
Which infections are associated with swyer-James Macleod syndrome ?
A
Adnovirus and mycoplasma pneumoniae
16
Q
What is crpytogenic organising pneumonia
A
Filling of the bronchioles and alveoli with granulation tissue polyps -
It is organising pneumonia of unknown cause
17
Q
What is the atoll sign meant to be associated with ?
A
Cryptogenic organising pneumonia.
18
Q
What is holt-oram syndrome
A
ASD + upper extremity bone deformities , including abscence or hypoplasia of the thumb
19
Q
What is the most common ASD malformation ?
A
Ostium secundum - failure of closure of the Ostium secundum with the septum
20
Q
What are the 4 components of Tetrology of fallot?
A
- Overriding Aorta
- VSD
- RV hypertrophy
- Pulmonary atresia
21
Q
Which syndromes is TOF associated with ?
A
DiGeorge
VACERAL
Trisomy 21
22
Q
What is the classic CXR finding of TOF and why?
A
Boot shaped - due to RV hypertrophy and reduced pulmonary vascularture.
No cardiomegaly
23
Q
What is Ebstine anomaly ?
A
Where the right atrium is too low - causing a bigger RA and a smaller RV. This causes reduced pulmonary vasculatture and cyanosis and not enough blood can get too the lungs.
24
Q
What is the classic CXR finding of Ebstine anomoly ?
A
HUGH box shaped Herat with MASSIVE right atrial enlargement.
25
What is the most common type of ASD ?
Ostium secundum - Caused by incomplete covering of the secundum by the septum.
This one might close without treatment
26
What are the two most common extracadial shunt lesions resulting in acyanotic congenital heart disease ?
Vein of Galen malformation and hepatic hemangioendothelioma.
27
In paediatric congenital heart disease what is aortic coarctation associated with ?
Biscupid aortic valve.
Turners syndrome
Shone syndrome
Intracerebral berry aneurysms
28
When to patients with Patent ductus arteriosus classically present ?
Infants develop CHF around day 7-10 due to vascular resistance falling.
29
What are the two sub-types of transposition of the great arteries ?
Dextrose - TGA
Levo TGA
30
What is the most common cause of cyanotic heart disease in newborns ?
TGA
31
What are the different subtypes of total anomalous pulmonary venous return ?
Supracardiac - pulmonary veins go to left subclavian vein
Cardiac - coronary sinus
Infracardiac - hepatic veins/ portal vein/ hepatic IVC
Mixed
32
What are the anterior mediastinal masses ?
The 4 T’s
- Terrible lymphoma
- Teratoma
- Thymus / Thymoma
- Germ cell tumor
33
What are the middle mediastinal masses ?
Foregut duplication cysts
Neuroenteic cysts
Lymphadenopathy - lymphoma or granulomatous disease.
34
What are the posterior mediastinum masses?
Neurogenic tumours :
neurobloastoma (malignant ) - > ganglioneurobloastoma ->
ganglioneuroma (benign)
35
What is dose the spinnaker sail sign represent ?
Pneumomediastinum
36
Where should the Umbilical artery catheter be positioned?
Terminates ideally at T6-T10 or L3-L5.
37
What syndrome is rhabdomyoma associated with ?
Tuberous sclerosis
38
Where do cardiac fibroma usually arise ? What is the pathognomic sign?
Intraventriclar septum
Central calcification - differentiating them from rhabdomyomas
39
What is the most common paediatric tumour arising from the cerebral hemisphere ?
Astrocymtoma
40
Where does NEC tend to occur ?
Distal ileum and right colon
41
WHO is at risk of NEC ?
Pre-term babies, congenital heart disease, hirschsprung disease and umbilical venous catheter or immunosupression
42
What is the most common delayed complication of NEC ?
Bowel stricture
43
What are the imaging criteria of pyloric stenosis ?
>3mm thick
Longer than 15mm
No gastric contents passing through the pylorus
44
What age should you be concerned about a intersusspection ?
< 3 months or > 3 years
Need to look for a lead point
45
What pressure do you inflate to for trying to reduce an intersusseption ?
3x 80
3x 100
3 x 120mmHg
46
How many types of tracheoesophagel fistulas are there ? Which is the most common ?
5 types A - E
The most common is oesophageal atresia with distal trachooesophgeal fistula (87%)
Atresia - the oesophagus is just cut in half
B -proximal fistula
C - distal fistula ( most common)
D- Double fistulation ]
E- Just a joining between the two
47
What are the 3 causes of proximal bowel obstruction - neonate
1. Malrotation mid gut volvulus - surgical emergency
2. Duodenal artesia
3. Jejunal atresia
48
What are the surgical causes of low bowel obstruction ?
Hirschsprung disease and distal atresias
49
What is a micro colon?
An abnormally small colon - < 1cm
50
What is secreted by the small bowel to prevent micro colon?
Sulcus entericus
51
What is one of the earliest manifestations of cystic fibrosis>
Meconium ileus - almost 100% of babies with meconium ileus have CF
52
Group A ependymoma tumours occur in which age group ?
Under 5 - group A epdnymoma
Over 5 - group B ependymoma
53
What is small left colon caused by ?
Temporary immaturity of the colonic ganglion cells - causing the distal colon to have abnormal motility right after birth
54
What are the most common causes of bowel obstruction in children ?
AAIIMM
Appendicitis
Adhesions
Inguinal/internal hernia
Meckles diverticulum
Malrotation
55
What percentage of meckles contains gastric mucosa?
25 %
56
What scan can be done to look for meckles ?
Tc-99m Pertechnetate scan
57
What sign on US might you see in biiliary atresia?
Triangular cord sign - echogenic fibrous tissue adjancent tot he portal vein representing abnormal bile duct
58
What is bilary atresia associated with ?
Polysplenia and trisomy 18
59
How does hepatoblastoma usually present ?
Painless abdominal mass with raised AFP
60
What is usually elevated in hepatblastoma ?
AFP > 1000ng/ml
61
What does a hepatoblastoma look like ?
Large, heterogenous, lobulated mass, that takes over the whole liver parenchyma, often cause IVC thrombosis
62
What age does HCC commonly occur in ?
Over 5s
63
What are the causes of VUR
Primary - Abnoramlly short intravesicular portion of the distal ureter. Or secondary due to distal obstruction - posterior urethral valves or neurogenic bladder.
64
How many grades are there when assessing VUR.
5
65
What are the two radionuclide scans that can be performed for VUR ?
1. Radionuclide cystogram: inserting Tc99m into the bladder, assessing for reflex.
2. Tc-99m DMSA renal scintigraphy scan - gold standard for detection of renal cortical scarring (seen as areas of relative photopenia
66
What does the dropping Lilly sign indicate ?
Duplex kidney - reduced number of calycs
- duplicated system with a functioning lower pole calycs which is displaced inferiorly by the hydronephrotic upper pole.
Renal Ptosis - normal number of calcyes
67
What are the common causes of uteropelvic junction obstruction?
Abnormal development of the upper segment of the ureter
Aberrant renal artery
Aperistaltic segment of the ureter
68
How would prune belly syndrome and posterior urethral valves differ on imaging?
Prune belly - the entire urethral system is dilatated
Posterior urethral valves - only from the prostatic urethra upwards is dilated.
69
What are the three major findings in prune belly syndrome ?
Lack of anterior abdominal walls
Dilataiton of the renal system
Cryptorchidism (bilateral undescended testes)
70
What are patients with horeshoe kidney at risk of ?
Damage from the vertebral body - so can’t play certain sports
Increased risk for stones, infection and cancer - Wilms, TCC and renal carcinoid.
71
What syndrome is associated with horseshoe kidney?
Turner’s syndrome
72
What gene mutation is seen in multilocular cystic nephroma ?
DICER 1 gene mutation ( this is not seen in the adult version )
73
SImple renal cysts are found in which syndromes ? ?
Tuberous sclerosis
Von Hippel-lindau
74
ADPKD is associated with extra-renal cysts where ?
The liver
75
What is APRKD associated with ?
Hepatic fibrosis - it is inversely related to the severity of renal disease
76
Causes of cystic renal lesions?
multilocular cystic nephroma
Multicystic dysplastic kidney
Simple renal cyst
ADPKD
ARPKD
77
What does Wilms tumours arise from ?
Persistent Metaneprhic blastema (one of the 2 embryolical tissues that form the kidney )
78
Which patients are screened for Wilms tumour
Beckwith-wiedemann syndrome
WAGR syndrome - Wilms tumour, aniridia, genitourinary anomliaise, mental retardatation
Horseshoe kidney
Trisomy 18
79
What subtype of Wilms tumour would you expect with bone metastasis ?
Clear cell Wilms tumour
80
What subtype of RCC affects sickle cell disease patients ?
Renal medullary carcinoma
81
What age group do rhabdoid tumours of the kidney occur ?
< 1
Maybe < 2
82
What is the cause of rhabdoid tumours of the kidney
Deletion of hte long arm Ch 22 - which is a tumour suppressor gene
83
What are the most common primary malignancies that metastases to the kidneys ?
Neuroblastoma
Leukaemia
Lymphoma
84
What age does Wilms tumour occur in ?
3-5 years
85
How can you usually differentiate between Wilms tumour and mesoblastic nephroma
Age of patient
Mesoblastic nephroma - occurs in children < 1 yrs
Wilms tumour 3-5
However both are removed surgically as cannot determine between them
86
Over what size are angiomyolipomas at risk of haemorrhage ?
Greater than 4cm
87
What condition are Angiomyolipomas associated with ?
Tuberous sclerosis
88
Are nephroblastomatosis usually uni or bilateral?
Bilateral, multi focal and confluent
89
Whats the most common site for mets in Wilms tumour ?
Lung
90
What is a differential list for multiple solid renal masses ?
Nephroblastomatosis
Lymphoma
Multi focal pyelonephritis
Multiple infarcts
91
What is neuroblastoma
A malignancy that arises from primitive neural crest cells of the sympathetic nervous system.
92
What is the most common extracranial malignancy in children ?
Neuroblastoma
93
Which syndromes are neuroblastoma associated with
NF-1
DiGeorge
Hirschsprungs
Beckwith-Wiedemann
94
What type of bone lesions are seen in neuroblastoma ?
Lytic and permeative
95
If a neuroblastoma crosses beyond the contra lateral vertebral body what stage is it ?
Stage 3 - as it has crossed the midline
96
What are the features of hand-Schiller-Cristian disease?
Diabetes insipidus
Exopthalmos
Skull lytic lesions
97
What are the bad features of Ewing’s sarcoma ?
> 15
> 8cm
Metastases
No response to chemo
Raised LDH
98
Where does Eosinophillic granulomas occur >
Long bones
Mandible
Spine - vertebral body collapse
Skull - punched out and sharply defined
99
What type of peritosteal reaction does Eosinophilic granulomas have ?
Lamellated
100
Why are bone scans not good for eosinophilic granulomas ?
Usually photopenic - so best reviewed on a skeletal survey
101
How do people with langerhans cells histocytosis commonly present ?
Fever, pain and a mass
102
What is langerhans cell histocytotiss ?
Uncontrolled monoclonal proliferation of langerhans cells and should be considered a malignancy
103
What is a Brodies abscess?
A lytic, metaphyseal lesion seen in cases of infection. It usually extends to the growth plate.
104
What is a sequestum
Dead bone
105
What is the best test for investigating neuroblastoma ?
MIBG scan - 85-90% of patients will have upatake
106
What type of bone lesion would you see in non-Hodgkin lymphoma ?
Osteolytic
107
How many phases are used in a paediatric bone scan. ?
3 phases:
1) vascular phase 0-20seconds
2) blood pool phase
3) delayed phase
108
What is the most common cause of osteomyeltisis in patients with sickle cell disease ?
Salmonella
109
What radiographs would you obtain for suspected dysplasia ?
AP and lateral skull
Chest
DP of the LEFT hand (due to bone age)
AP leg - just one
AP arm - just one
AP abdo and pelvis
Lateral thoracolumbar spine
110
When describing the areas involved for limb shortening :
Where is
1 . Acromelic
2. Mesomelic
3. Rhizomelic
Acro - distal hands or feet
Meso - middle (ulna/tibia )
Rhizo (proximal )
111
What is Brucks syndrome ?
Osteogenesis imperfector with contractures
112
What is osteopoikilosis ?
Sclerosing bony dysplasia with lots of bony islands
113
What is classic shape of bones in achondroplasia ?
Trident hands
114
What feature on a CXR would you seen in osteogenic imperfects ?
Ribbon ribs
115
What is a gynmastic wrists ?
Widening of the physis secondary to excess stress
116
What is the most common salter Harris fracture ?
Salter Harris 2 - 75%
117
What is a tillaux fracture ?
A fracture through the anterolateral aspect of the distal tibia, salter Harris 3.
Various displacement.
118
What is a toddlers fracture?
Undisplaced oblique fracture through the tibial metadiaphysis caused by rotational forces to the leg
119
What views does a skeletal survey include ?
Frontal views of all long bones, rib views with obliques, skull, pelvis, hands/feet and entire spine.
120
Which fractures are highly suspicious for child abuse ?
Posterior rib fracture
Scapula fracture
Sternum
Spinousus process
Classic metaphyseal lesion - bucket handle fracture
121
What is a normal alpha angle ?
> 60
122
When does femoral head ossification occur ?
6 months
123
What is the most common cause of septic arthritis ?
S.aureus
124
What is a systemic cause of bilateral perthes?
Avascular necrosis
125
What would perthes show on a bone scan ?
Reduced uptake
126
What would late perthes show on a bone scan ?
Increased uptake due to repair mechanisms
127
Where is kleins line drawn and what does it show ?
Kleins line is drawn along the lateral margin of the femoral neck - if it doesn’t intersect the femoral head or if there is assymetry, it can indicate SUFE
128
What age does SUFE affect ?
Boys 10-16 years
129
How is SUFE treated ?
Screw fixation
130
How many stages are there in a bone scan?
3 phases
1) early blood phase - vascularity
2) Blood pool phase - looking at soft tissue involvement
3) Delayed images - reflect the osteoblastic response to underlying disease
131
What bug commonly causes osteomyeltisis in sickle cell patients ?
Salmonella
132
Where does discitis commonly occur in children ?
Lumbar spine
133
TORCH infections
Toxoplasmosis
Other - syphillis
Rubella
CMV
Herpes Symplex
134
What is the Wimerger sign
Destructive erosion of the medial aspect of the proximal tibial metaphysics
135
What is the wimberger ring sign ?
Scurvy - showing increased density of the ossification centres
136
What infection can cause symmetric periosteal reaction in a child ?
Syphilis
137
what are the common sites of ankylosis in juvenile rheumatoid arthritis
wrists, carpometacarpal joines and cervcal spine
138
what is Klippel-Feil syndrome
Fusion of one or more cervical spine - associated with vertebral segmentation anomalise
139
What is stills disease ?
Acute systemic subtype of JIA affecting children under the age of 5. They present with fever, anaemia, leukocytosis, hepatosplenomeagaly and polyarthiris
140
is JIA usually RF positive or negative ?
Negative
141
what is rhizomelia ?
proximal limb shortening - femur or humerus
142
what is mesomelia ?
Middle limb shortening - radius/ular or tibia/fibular
143
What is acromelia
Distal limb shortening - hand or foot,
144
what is Amelia ?
Limb is absent
145
what are the lumbosacral imaging features of achondroplasia ?
Narrowing of the interpedcuncular distances of the lower spine.
Flatenning of the acetabulum
Trident hand
Mickey Mouse ears / tombstone ileum
146
what is the most common leathal skeletal dysplasia ?
Thanatophoric dysplasia
147
what is the radiographic finding of cleidocranial dystosis ?
Lack of one or both of the clavicles.
148
what is wormian bone ?
Lots of bones in the skull due to extrostosis
149
which type of osteogenesis is lethal ?
type 2
150
what are the classic clinical feature of osteogenesis imperfect ?
Blue sclera, bowing of the long bones. hearing impairment due to osteosclerosis. Wormain bones
151
Causes of stippled epiphyses
Chondrodysplasia punctata
Multiple epiphyseal dysplasia
Hypothyroidism
Complication of mother warfarin use
152
what's the difference between mafucci syndrome and Ollier disease ?
mafucci syndrome is characterised with venous malformations which cause phleboliths . Maffucci has a high risk of malignant transformation
153
what's the difference between extosis or osteochondromatosis
osteochondromatosis have a cartilaginous cap
154
What radiology sign might you seen in lead poisoning ?
Lead lines in the fibula
155
What radiology sign might you seen in lead poisoning ?
Lead lines in the fibula
156
What is hurlers syndrome ?
A mucopolysaccharide disease - lysosomal storage disease. This builds up and causes cell death
157
What vertebral body abnormality might you see in hurlers syndrome
Anterior breaking of the vertebral bodies - inferiority
158
What is rickets caused by.
Inadequate VItamin D
159
What is rachitic rosary ?
Anterior cupping of the ribs with widening of the rib epiphyseal cartilage
160
What MRI finding might you see in rickets ?
Loss of the black-white-black line corresponding to an in distinct ZPC
161
What is oncogenic rickets ?
Tumour inducing phosphate depletion resulting in osteomalcia or rickets
162
What might cause oncogenic rickets ?
Nonossifying fibroma or hemangiopericytoma ( rare tumour that grows in the soft tissues and contains all the different types of body structures e.g. fat, muscle, soft tissue, tendons etc)
163
What is the most common primary bone tumour ?
Osteosarcoma
164
Where is conventional osteosarcoma most commonly found ?
Around the knee, , distal femur or proximal tibia
165
What is the second most common primary bone tumour ?
Ewings sarcoma
166
What is ewings sarcoma ?
Aggressive tumour of small round blue cells - of the neuroectodermal differentiation
167
What is the most common site of mets for ewings sarcoma ?
Lungs
168
What is the most common paediatric malignancy ?
Leukaemia
169
How often are bony changes seen in leukaemia ?
50% of cases
170
What radiographic feature might you seen in leukaemia
Lucent metaphyseal bands
171
What is Blount disease ?
Osteochondrosis of the proximal tibial metaphysis causing tibia virus and internal rotation eventually leading to progressive deformity, gait deviations and leg-length discrepancy
172
Where does madelung deformity affect ?
Radius
173
Where does panner disease affect ?
Capitellum
174
What does prostaglandins cause to bones in newborns ?
Thought to slow osteoclast bone resorption leading to a periostea’s reaction
175
what syndrome is associated with annual pancreas ?
downs synrome
176
what is adrenoleukodystrophy?
a inherited disorder x-linked resulting in the build up of VLCFA (Very long chain fatty acids) which results in demylination. mainly affecting the optic and auditory pathway
177
What is Carney's triad ?
pulmonary hamartomas, gastric leiomyosarcomas and extra-adrenal paragangliomas
