Paeds & Congenital Flashcards

1
Q

Causes of bowel obstruction in an older child?

A

AA-II-MM

Appendicitis
Adhesions
Inguinal hernia
Intussusception
Midgut volvulus
Meckel’s diverticulum

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2
Q

On hepatoblastoma:

“the commonest primary liver tumour in children”

  1. Associations?
  2. Presentation?
  3. Appearance on CT/MR?
  4. Two key points to check?
A
  1. BWS, hemihypertrophy, prematurity, and many others…
  2. Abdominal mass/distension + raised AFP
  3. Well-defined, heterogenous, mainly low attenuation mass; often coarse calcification; may be haemorrhage and rim enhancement
  4. Is it multifocal? Is there vascular invasion?
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3
Q

On left-sided isomerism:

“aka polysplenia”

  1. Most common features?
  2. Cardiac defects?
  3. Other features?
A
  1. Abnormal spleen, abnormal IVC - interrupted with continuation of azygous or hemiazygous
  2. Less than in right-sided isomerism. ASD much more common
  3. Bowel malrotation; truncated pancreas; biliary atresia
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4
Q

On right-sided isomerism:

“aka asplenia”

  1. Most common features?
  2. Other features?
A
  1. Asplenia, severe cyanotic heart disease (TAPVR)
  2. Bowel malrotation; horseshoe kidney
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5
Q

Liver mass in <3, with raised AFP and possible precocious puberty, coarse calcifications and vascular invasion?

A

Hepatoblastoma

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6
Q

Liver mass in <3 with raised EGF, low platelets and signs of congestive cardiac failure?

A

Haemangioendothelioma

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7
Q

Liver mass in <3 which is cystic, AFP negative and lacks calcifications?

A

Mesenchymal hamartoma

T1 - variable due to cyst content
T2 - hyperintense
T1 +c - septae and stromal components can enhance

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8
Q

Solid renal mass in <1, hypoechoic on US with involvement of sinus fat but no herniation into renal pelvis and hypoenhancement on CT?

A

Mesoblastic nephroma

“Most common solid renal tumour of infancy”

May have cystic spaces

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9
Q

Cystic renal mass in neonate with no functioning renal tissue?

A

Multicystic dysplastic kidney

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10
Q

Nephroblastomatosis
- on US
- on CT

A
  • small nodules of varying echogenicity, may be enlarged kidneys if diffuse
  • peripheral parenchymal nodules that enhance less than adjacent tissue; if diffuse, homogenous peripheral low attenuation that forms a “rind” around the kidney
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11
Q

Differential diagnosis for lucent metaphyseal bands?

A

Leukaemia
Infection (TORCH)
Neuroblastoma mets
Scurvy
Rickets

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12
Q

What kind of ASD is seen in Down’s syndrome?

A

Ostium primum ASD (part of spectrum of endocardial cushion defects)

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13
Q

Which kind of ASD has the better prognosis?

A

Ostium secundum - may close on its own, less symptomatic

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14
Q

Bone marrow transplant complications of the early pre-engraftment period (15-30 days) and early post-engraftment period (100 days)?

A

Interstitial pneumonitis
- infective CMV
Infection - fungi, Klebsiella
Haemorrhage
Oedema
PE
Calcifications
BOOP/COP

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15
Q

Bone marrow transplant complications of the late post-engraftment period?

A

Chronic GVHD
Bronchiolitis obliterans
Infection
Lymphoid interstitial pneumonia
Fibrosis

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16
Q

Multilocular cystic nephroma
- demographics?
- appearance?
- differentiate from Wilms?
- differentiate from MCDK?

A

Under 5s, M»F

Multilocular cystic mass with thick capsule
- haemorrhage, calcification + necrosis are rare!
- often herniates into renal pelvis
- claw sign may be present

There shouldn’t be solid enhancing nodular components in a cystic nephroma

In MCDK cysts replace the entire kidney, there is no claw of functioning renal tissue; cystic nephroma does not occur in the perinatal period whereas MCDK does

17
Q

Types of tracheo-oesophageal fistula

A

A - isolated atresia, no fistula
B - atresia with a proximal TOF

These two will have no gas in the bowel

C - atresia with distal TOF
D - double fistula with intervening atresia

These two will have lots of gas in the bowel

E - isolated fistula, no atresia

18
Q

Most common associations of corpus callosum dysgenesis/agenesis?

A

Colpocephaly - hydrocephalus of the posterior horns lateral ventricle

Interhemispheric lipoma

19
Q

Joubert syndrome?

Important associations?

A

Cerebellar vermis agenesis leading to “molar tooth” sign of superior cerebellar peduncles

Retinal dysplasia, MCDK, nephropthisis, hepatic fibrosis

20
Q

Direction of formation of corpus callosum?

Direction of cleavage of brain?

Which structure is absent in all forms of holoprosencephaly?

Alobar holoprosencephaly?

Semi-lobar?

A

Genu to splenium (front to back), with rostrum last

Back to front - therefore milder forms will only have fusion at the front

The septum pellucidum

Single large ventricle, fused thalami, single (azygos) ACA, no corpus callosum or falx, facial deformities

Intermediate form - absent septum pellucidum, falx/interhemispheric fissure anteriorly, thalami partly fused, monoventricle with some development of the occipital/temporal horns