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INRC > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

What is the most common cause of an abdominal pseudocyst in a patient with a shunt?

A

Proprionobacterium acne infection of the shunt

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2
Q

What are the features of Scaphocephaly?

A

Reduced biparietal diameter with frontal and occipital bossing leading to an elongated (boat shaped) head due to premature fusion of the Sagittal suture

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3
Q

Which synostosis case should you do an MRI scan?

A

Brachycephaly and posterior plagiocephaly due to crowding of the posterior fossa resulting in chiari

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4
Q

What are the indications for treatment of single suture synostosis?

A

Cosmetic
Raised ICP
Chiari malformation

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5
Q

What is the management of craniosynostosis?

A

Clinical assessment
USS
X-Ray / CT scan no necessary
MRI if suspecting associated intracranial pathologies

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6
Q

What are the procedure types for craniosynostosis?

A

Destabilisation (passive) if young than 9months - 1 year

Remodelling (active) if older

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7
Q

When is a fronto-orbital advance required?

A

Metallic, unilateral or bilateral coronal suture fusion

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8
Q

What is turricephaly aka oxycephaly?

A

This is fusion of the coronal suture plus any other suture e.g. Coronal with lambdoid suture etc. This is the worse form of synostosis.

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9
Q

Features of Crouzon’s disease?

A

AD FGFR2 mutation
Proptoisis, hyperteloris
Mid face hypoplasia

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10
Q

How can Apert’s and Crouzon’s be differentiated?

A

Syndactylies are seen with Apert’s syndrome so look at the hands!!

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11
Q

What is the difference between diastematomyelia and diplomyelia?

A

Look this up!

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12
Q

What is the causes of spinal lipomas?

A

Separation of neural tube from the surrounding ectoderm causing the neural plate to allow entry of mesenchymal cells

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13
Q

What are the types of spinal lipoma?

A

Dorsal
Transitional
Terminal

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14
Q

What is the rate of shunt infection in the paediatric population?

A

5-15% (majority

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15
Q

What are the important layers in spins bifida closure?

A

Placode
Dura
Fascia
Skin

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16
Q

What is the clinical grading of spasticity?

A

Level 1-5 (look it up)

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17
Q

What are the surgical options for spasticity?

A 
Selective Dorsal Rhizotomy (Level 2/3)
Intrathecal baclofen (level 4/5)
Mainstay of treatment is physio therapy / Botox / tendon release etc
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18
Q

What are the indications for Intrathecal baclofen?

A

GMFCS level 4 and 5
No s/e of oral administration
Positive Intrathecal test dose

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19
Q

How do you do a selective dorsal Rhizotomy?

A

See pictures

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20
Q

How do you manage a Pilocytic astrocytoma in NF-1?

A

More conservatively as they are less aggressive and they develop many of them

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21
Q

What are the features of Diencephalic syndrome?

A

??

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22
Q

What is the surgical management of optic pathway Gliomas in children?

A

Chronic progressive disease that should only be intervened surgically to debulk lesions without causing diencephalic syndrome

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23
Q

Which genetic make up has best outcome in medulloblastoma?

A

Wnt pathway

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24
Q

Why do an LP 2 weeks post-op for medulloblastoma?

A

Has a poor prognosis even after complete resection if the LP is position

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25
Q

What is post-fossa syndrome?

A

Cerebellar mutism
Ataxia
Cerebellar acquired cognitive disorder

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26
Q

What is the cerebellar mutism study?

A

To determine the risk factors for post-fossa syndrome in posterior fossa tumours

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27
Q

What are the future direction of medulloblastoma management?

A

ETV and biopsy
Wait for molecular histology and then decide on how aggressive to be with resection based on this
Don’t be too aggressive to begin with to prevent post fossa syndrome

28
Q

What surgical approaches are used for medulloblastoma?

A

Telovelotonsillar approach

Transvermian approach

29
Q

When would you try for full resection of Craniopharyngioma?

A

Anterior sellar lesions anterior the chiasm without significant hypothalamic / stalk disease

30
Q

What is the most difficult anatomical configuration for craniopharyngioma surgery?

A

Pre-fixed chiasm

31
Q

What are the goals of craniopharyngioma surgery?

A

No attempt at dissecting hypothalamus
No damage to pituitary stalk
Gross total resection

32
Q

What is the management of pineocytoma?

A

If >10mm then:
Resection only
+/- radiosurgery for residual / recurrence

33
Q

What is trilateral retinoblastoma syndrome?

A

Bilateral retinoblastomas with a pineoblastoma

34
Q

What are papillary tumours of the pineal region?

A

Neuroectodermal tumours of the pineal region in adults

35
Q

Where can germinomas arise?

A 
Suprasellar region (so do pituitary function!)
Pineal region
36
Q

What are poor prognostic markers for non-germinomatous germ cell pineal region tumours?

A

Post-op AFP>1000

Residual malignant tumour

37
Q

What is the management of pineal region teratomas?

A

Gross surgical resection

Chemo / Rad are unhelpful

38
Q

Which pineal tumour has high AFP?

A

Yolks sac tumours

39
Q

Which pineal region tumours have high bHCG?

A

Choriocarcinomas (comes from Syncitiotrophoblasts)

40
Q

What is the landmark for measuring DBS targets?

A

3rd ventricle and AC-PC line

41
Q

4 month with a cystic lesion in the nose. What should you think of?

A

Encephalocoele

42
Q

What is the management algorithm for pineal region tumours?

A 
MRI brain and spine
Send serum and CSF markers
Treat hydrocephalus (with ETV)
If markers negative then need a biopsy
If markers positive then chemo + Rad
Resect any residual contrast enhancing areas
Usually need resection for TERATOMA!
43
Q

Who are the high risk group for pineal region tumours?

A

> 6 years

AFP >1000

44
Q

Classification of brainstem tumours?

A 
Anatomical location:
Choux (1999)
1 = Intrinsic diffuse
2 = Intrinsic focal
3 = Exophytic (dorsal / lateral)
4 = **
45
Q

What is diencephalic syndrome?

A

Look it up:
Emaciation
etc

46
Q

Management algorithm for brainstem tumours in paediatrics?

A

Treat hydrocephalus (ETV)
If diffuse - no biopsy
If focal biopsy +/- resection

47
Q

What are the potential mechanisms of arachnoid cyst pathology?

A

One way valve
Osmotic gradient
Active CSF secretion
Distention by CSF pulsations

48
Q

What is the classification system for middle fossa arachnoid cysts?

A

Galassi:
1 - Biconvex (don’t need tx)
2 - Square type in the sylvian fissure (may need tx)
3 - Large causing brain shift (may need surgery)
4 - Temporal lobe hypoplasia (risk of SDH)

49
Q

What is the PCB2 line?

A

In cases of Chiari malformation with peg retroflexion. Draw a line from basion on posterior corner of C2 body. If the peg retroflexion is >9mm then likely will need OC fusion

50
Q

What is the clival spinal angle (CXA)?

A

Where a line draw along the clivus intersects a line along the back of C2. The angle between these lines should be >125 degress.

51
Q

Which Chiari patients need an OCF?

A

If the pBC2 >9 mm and CXA <125 deg

52
Q

Nice memento for neurosurgery?

A

‘Cure sometimes
Comfort most of the time
Care always’

53
Q

What is a Chiari 0?

A

Tight posterior fossa with tonsillar decent <5 mm and have a syrinx

54
Q

What trajectory do you use for ETV?

A

Precoronal burr hole (2cm ant)

Use a trajectory that simulates the clival angle

55
Q

What is the most consistent landmark during ETV?

A

Infundibular recess

56
Q

During ETV what catheter do you use to open the stoma?

A

3F embolic catheter

57
Q

What forms the lateral walls of the 3rd ventricle?

A

Thalamus superior, sulcus limitans and then hypothalamus below

58
Q

What sight for ETV can you do when the floor of the 3rd ventricle is distorted / not safe?

A

Translamina terminalis ETV (need a 30 deg scope) - sight is above the chiasmatic recess

59
Q

What are the main causes of complex craniosynostosis?

A 
Muenke
Crouzon / Pfeiffer
Sathre-Chotzen
Aperts syndrome (syndactyly)
All have FGF2 mutations
60
Q

Which condition has turribrachycephaly?

A

Cloverleaf skull seen with **look it up

61
Q

What is the differential of a coccygeal pit?

A

Spina bifida occulta (this is over the sacrum!) and has a hairy skin patch

62
Q

What are subpial filum terminale lipomas?

A

The dura is intact around the lipoma. If the fat extends outside the dura then it is a lipomyelomeningocoele

63
Q

What is the Chapman classification for spinal lipomas?

A

Dorsal
Caudal
Transitional

64
Q

What are the goals of surgery for spinal lipomas?

A

Untether the cord
Prevent progressive neurological deficit
…

65
Q

How do you grade concussion?

A

Mild - Transient confusion <15 mins
Moderate - Confusion >15 mins
Severe - LOC

INRC (7 decks)

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