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Paeds > Practise vivas > Flashcards

Practise vivas Flashcards

1
Q

Prune belly

A

Abdominal distension from urinary obstruction causes hypoplasia of musculature
Cryptorchidism -bladder impedes descent
Dilated ureters - from urinary obstruction -either urethral atresia or bladder functionally not emptying

Potter sequence in utero

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2
Q

VUR grading

A

1 ureter
2 pelvis
3 mild dilatation
4 tortuous ureter with moderate dilatation, blunted fornices
5 tortuous, severe dilatation, loss of fornices and papillary impressions

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3
Q

Posterior urtethral valves

A

3 types, type 1 most common, type 2 no longer considered a valve
1 two mucosal folds down from verumonatum (embrylogic vagina, at level of entrance of ejaculatory ducts, has a raised bit of urothelium) fuse lower down
2 from ureteric orifice superiorly to verumonatum
3 abnormal canalisation of urogenital membrane - a circular diaphragm with central opening

Associated with Downs, craniospinal defects, bowel atresia

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4
Q

Potter sequence

A

Pulmonary hypoplasia
IUGR
Abnormal facies
Limb abnormalities - club feet and contractures

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5
Q

Vascular rings

A

Pulmonary sling / aberrant L PA - only one between oesophagus and trachea - anterior indentation on oesophagus.
Aberrant right subclavian most common, usually asymptomatic, not a complete ring
Double arch most common symptomatic ring
Right arch with aberrant left SC and left ligamentum arteriosum

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6
Q

L TGA

A

Meso or levocardia
Abnormally straight vascular pedicle

VSD in 70-80%
Pulmonary stenosis in 40-50%
If have both above, may be cyanosed

Associated with situs inversus

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7
Q

VHL

A 
Chromosome 3
HIPPEL
Clear cell RCCs
Bilateral endolymphatic sac tumours
RCCs and cerebellar haemangioblastomas most common cause of death
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8
Q

Grisel syndrome

A

Torticollis
Subluxation of atlantoaxial joint
Inflammatory ligamentous laxity, usually retropharyngeal abscess

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9
Q

Fanconi anaemia

A

Commonest inherited marrow failure
AR
Associated with horseshoe kidney
Higher incidence in Ashkenazi Jews, Afrikaners
Increased incidence of AML
Radial ray anomalies, including absent thumb

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10
Q

Radial ray anomaly

A

Partial to complete aplasia of radius +/- thumb
Many associations:
Fanconi anaemia
Trisomy 18
Holt Oram (congenital heart defects and upper limb anomalies - ASD, coarctation, radial ray, clavicle hypoplasia)

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11
Q

Wormian bones

A 
PORK CHOPS
Pyknodysostosis
OI
Ricket
Kinky Hair
Cleidocranial dysostosis
Hypothyroid
Otopalatodigital
Primary acroosteolysis (Hajdu Cheney(
Syndrome of Downs

Remember Downs, OI, cleidocranial.

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12
Q

Klippel feil

A

Cervical vertebral fusion
Female prediliction
Short neck, low hairline, restricted neck motion
Associated with sprengel deformity (congenital scapula elevation, associated with omovertebral bar)

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13
Q

Sprengels

A

Congenital scapula elevation
Omovertebral bar often present
May be fibrous, cartilaginous, or osseous
Associated with Klippel Feil, spina bifida, underdevelopment of clavicle and humerus

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14
Q

Calcified discs

A 
Ochronosis - nucleus pulposis
Transient
Juvenile chronic arthritis
Ank spond
Pseudogout
Haemochromatosis
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15
Q

SUFE

A

Posteomedial displacement of femoral head/epiphysis

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16
Q

Paediatric elbow fractures

A

Supracondylar
Lateral condyle (6-10)
Medial epicondyle (older children, adolescents)
Radial head dislocation (infancy, childhood)
Radial neck fracture (8-11)

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17
Q

OI

A

Osteoporosis and fragile bones that fracture easily, blue sclera, dental fragility, hearing loss

Collagen type 1 production disorder
No sex or ethnic link
3 main types
1 - mild
2 - perinatal lethal
3 - progressive deforming
Wormian bones
Pseudoarthroses at fracture sites
Gracile bones
Basilar invagination
Otosclerosis
Associated with congenital cataracts
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18
Q

Kartageners

A

Primary ciliary dyskinesia
Situs inversus, chronic sinusitis, bronchiectasis, infertility in males
No gender prediliction

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19
Q

Meconium plug

A

Higher in maternal diabetes
Transient, functional bowel obstruction
Normal rectal size, unlike Hirschsprungs
Calibre change around splenic flexure

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20
Q

Proximal and distal bowel obstruction differentials

A

.

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21
Q

Hirschsprungs

A

Rectosigmoid ratio <1 (normal is >1)

Term neonates, especially boys (esp short segment disease)
75% present within first few weeks, 90% in first five years
Very small amount may present as adults
Full thickness rectal biopsy for diagnosis

Downs association (10%)
Also neuroblastoma
22
Q

Rickets

A

Rachitic rosary

Flayed, splayed metaphyses

23
Q

In utero bowel perforation / abdominal calcification / scrotal

A 
Atresias
Ischaemia
Gastroschisis
Fetal volvulus
Meconium ileus
Maternal cocaine consumption a risk factor for in utero perforation

Liver calcs from infections in utero

24
Q

Duplex kidney

A

Upper pole has ectopic insertion (medial and inferior, often with a uretrocele)
Upper pole obstructs
Lower pole refluxes

Associated with fanconi anaemia (as is Horseshoe).

25
Q

Cephalohaematoma

A

Traumatic subperiosteal haematoma from birth injury
Bound by suture lines
Uni or bilateral
Increased incidence with ventouse and forceps
May become peripherally calcified
Resolve spontaneously

Cf caput succedaneum which is subcutaneous serosanguinous collection from birth trauma

26
Q

Right congential diaphragmatic hernia

A

13%
Aneuploidy in up to 50% of hernias, either side
Intrathoracic liver has poor prognosis
DH associated with sequestrations

27
Q

Temperature probe on CXR

A

A midline device, in the oesophagus

28
Q

Ewing sarcoma involving sacrum

A 
SPACE MONGREL (sacrum)
Sarcoma(osteo/chondro)/SC teratoma
Plasmacytoma
ABC
Chordoma
Ependymoma
Mets
Osteomyelitis
Neuroblastoma
GCT
Rectal
Ewings (14% of Ewing is sacral)
Lymhoma / leukaemia
29
Q

Horseshoe kidney associations

A

Stones, infection, PUJ obstruction
Trauma susceptibility
Wilms, TCC, carcinoid
HTN

Trisomy, Turners (20% of trisomy 18%)
VACTERL

30
Q

Round pneumonia

A

Mean 5, 90% <12
Underdeveloped pores of Kohn and canals of Lambert in children, so infection cannot spread throughout lobe
No specific bacteria. Strep pneumoniae most common.
Most commonly superior segment of lower
lobe, and solitary

31
Q

RDS

A

First few hours of life
May be cyanosed
Preterm, maternal diabetes
Hazy opacity, often bilateral symmetrical, small lung volumes (may be large as ventilated)
May be complicated by interstitial emphysema, other ventilation complications including subglottic stenosis, pulmonary haemorrhage, bronchopulmonary dysplasia / CLD

CLD: oxygen toxicity and hypoxia
Reticular markings and lucent areas in hyperexpanded lungs
May have pulmonary HTN and cardiomegaly
Mosaic attenuation and bronchial wall thickening on CT

32
Q

Kohlers

A

AVN of navicular

33
Q

ACL avulsion

A

Tibial eminence

More common in children

34
Q

IVH grading /germinal matrix haemorrhage

A 
1 - germinal matrix
2 - into non-dilated ventricles
3 - into dilated ventricles
4 - parenchymal extension
1 and 2 good prognosis, 4 poor
35
Q

UAC

A

Low position, L3-5

High position, T6-10

36
Q

UVC

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

Should go umbilical vein, left portal vein, ductus venosus, left or middle hepatic vein, IVC, and sit at IVC-RA junction

37
Q

Coxa valga

A

Often associated with shallow acetabular angles and femoral head subluxation

Bilateral, neuromuscular disorders e.g. cerebral palsy, or skeletal dysplasias e.g. Turners, MPS
Unilateral from trauma with growth arrest

VDRO = varus derotation osteotomy

38
Q

Shunts

A

ASD - right atrial, right ventricle enlargement
VSD - left atrial, may have left and right ventricular
PDA - left atrial and left ventricular

39
Q

Neuroblastoma v Wilms

A

Calcification common in neuro
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

Neuroblastomas along sympathetic chain, differential also of ganglioneuroma and ganglioneuroblastoma

40
Q

Cloverleaf skull

A 
Trilobate shape
Severe craniosynostosis (multiple sutures at once)
Thanotophoric dyplasia (type 2), severe Aperts or Crouzons
41
Q

Thanatophoric dysplasia

A

.

42
Q

Canavan disease

A 
A leukodystrophy
Death before 5, often before 18 months
Ashkenazi Jews
Autosomal recessive
Acumulation of NAA
CaNAAvan
Megalencephaly
Diffuse white matter disease
Involves subcortical U fibres
No enhancement
43
Q

Leukodystrophies

A

dysmyelinating diseases which affect children
Lysome storage (MPS - Hurlers, Morquio, Neimann Pick, metachromatic leukodystrophy, Fabry disease)
Mitochondrial dysfunction (MELAS, Leighs, other)
Peroxisomal (Zellweger - cerebrohepatorenal syndrome, adrenoleukodystrophies - X-linked, involes splenium, and testes and adrenals)
Amino acid metabolism - Canavan
MLC, Alexander disease

44
Q

Metachromatic leukodystrophy

A

The most common leukodystrophy (dysmyelination)
A lysosome storage disorder
Reduced NAA, increased lactate
Tigroid sparing of venules
Subcortical U fibre sparing - butterfly pattern
Autosomal recessive
Infantile, juvenile and adult forms

Motor, sensory, or psychiatric

45
Q

MLC

A

Megalenchephalic leukoencephalopathy (with subcortical cysts)
Van der Knaap disease
AR
White matter cystic degeneration and megalencephagly
T2 increased white matter with sparing of basal ganglia
Subcortical cysts of CSF intensity anterotemporal and frontoparietal
Antiepileptics to manage epilepsy
Mild motor delay, mild mental deterioration

46
Q

Alexander disease

A 
Fibrinoid leukodystrophy
Sporadic
Anterior dominant, extends posteriorly
Basal ganglia become involved
Normal NAA
Fatal
Infantil, juvenile, and adult forms.
Enhancement may be seen
47
Q

Megalencephaly and white matter disease

A

MLC, Alexander, Canavan

(Adrenoleukodystrophy and metachromic lyuekodystrophy both spare subcortical U fibres)

Megalencephaly means large brain cf macrocephaly. In general associated with syndromes.

48
Q

18q dilation

A

.

49
Q

Luckneschadel

A

Craniolucunae, lacunar skull
Associated with Chiari 2 - seen in 80% of such cases
Pits of non-ossified fibrous bone in the inner table

Differential of copper beaten skull - prominent gyral indentations in the skull in raised intracranial pressure - hydrocephalus, craniosynostosis, masses,

50
Q

Pyloric stenosis measurements

A 
Pi
3.1415
Muscle thickness >3mm
Transverse diameter >14mm
Length >15mm
51
Q

Adrenoleukodystrophy

A

Lack of oxidation of very long chain fatty acids
Accumulate in CNS myelin, adrenal cortex, leydig cells of testes
X-linked
Posterior predominant demyelination, involves splenium
Usually symmetric
Leading edge enhancement - associated with disease progression

52
Q

Ewing sarcoma

A

Slight male prediliction

Paeds (4 decks)

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