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Flashcards in Paediatric Deck (205):
1

What is the success rate for ETV with tectal glioma?

90%

2

What score is used to calculate success of ETV?

Kulkarni et al 2010 ETV success score based on :

Age - Older the better
Etiology - bad if post infectious and high if tumour
Previous shunt - better if no previous shunt

3

What else can be done in addition to ETV for obstructive hydrocephalus?

Choroid plexus coagulation

4

What is better ETV or shunt?

Recent meta-analysis has shown that outcomes are the same due to side-effects of both procedures.

5

What is the rate of shunt failure in one year for children

40%

6

What are the reasons for shunt failure in young children?

Obstruction, infection, over-drainage and loculation

7

Level 1 evidence for neonatal hydrocephalus?

Don't give acetazolamide / serial LPs

8

What is the risk of VPS in neonates?

Risk of necrotising enterocolitis (NEC) as preterm so may get infected
Child may be too small to shunt

9

How is neonatal hydrocephalus best treated?

Ventriculosubgaleal shunt +/- reservoir until the child is old enough to shunt

10

What is the differential diagnosis of an intracranial cyst?

See image

11

What is the classification for middle cranial fossa arachnoid cysts?

Galassi classification
1 - free communication with cistern
2 - little communication
3 - no communication

12

What is the pathogenesis of arachnoid cyst?

Ball valve mechanism
Fluid produced from cyst wall
Osmotic gradient

13

Does operating on an arachnoid cyst alter seizure frequency?

NO

14

What is a hydatid cyst?

The larval stage of tape worm echinococcus granulosus

15

How is a hydatid cyst resected?

Dowling hydrodissection technique to deliver the cyst

16

What is the difference between cavum septum, cavum vergae and cavum velum interpositum?

**

17

What is the origin of a colloid cyst?

Neuroepithelial remnant of the paraphysis / ependyma
OR
Midline inclusion cyst

18

What are the contents of a colloid cyst?

Mucin, blood, cholesterol

19

What is the natural history of colloid cyst?

Over 50 y

20

What is the differential diagnosis of a colloid cyst?

Subependymoma
Neurocysticercosis
CSF flow artifact
RARE - aneurysm / glioma / meningioma / pineal region tumour / germinoma

21

What are the causes of non-obstructive hydrocephalus?

Excess production - choroid plexus papilloma / carcinoma
Reduced absorption - immaturity of arachnoid villi, venous hypertension, IVH

22

What is the incidence of craniosynostosis?

1 in 2500

23

Where is the metopic suture?

Forehead

24

What deformity does metopic suture craniosynostosis cause?

Trigonocephaly

25

What does coronal suture craniosynostosis cause?

Brachycephaly if bilateral or anterior plagiocephaly if unilateral

26

What is the differential diagnosis of posterior plagiocephaly?

Positional vs unilateral lambdoid suture closure

27

What are the causes of secondary synostosis?

Microcephaly - not due to genetic suture abn but due to lack of brain growth
Prematurity
Shunting
Trauma
Positional

28

How can lambdoidal plagiocephaly be differentiated from positional plagiocephaly?

Position of the ear is moved forward with positional and posterior with lambdoid suture fusion

29

What age do sutures ossify (not close!)

8 years

30

What is the classical correction of scaphocephaly

Barrel stave osteotomies to widen the skull
Performed age 6 - 8 months

Followed by helmeting for 6-12 months if they are young

31

What does 'copper beating' of the skull suggest in craniosynostosis?

Raised ICP

32

What are the important parts of treatment for brachiocephaly?

Frontal craniotomies followed by fronto-orbital advancements

33

What syndromes cause craniosynostosis?

These all have FGF mutations:
Apert - syndactyly and flat midface
Crouzon - orbital hypertelorism
Pfeiffer - syndactyly, web neck
Moncke

34

How do you perform a classical repair of metopic suture synostosis?

Fronto-orbital advancement and resection of the synostosed suture

35

What is dolichocephaly?

Long narrow head. Scaphocephaly is a type of dolichocephaly and signifies a pointed suture.

36

Do you need a CT scan to treat scaphocephaly?

No

37

What is spinal dysraphism?

**

38

What are the lemon and banana signs?

On 20 week USS: Anteriorly the skull flattens and look like a lemon; Posteriorly there looks to be a banana over where the ambient cisterns are; These are classical findings of a child with a myelomeningocoele.

39

What embryological abnormality causes spinal dysraphism?

Abnormal closure of the neural tube during primary neurulation
(note secondary neurulation is from S2 down)

40

What conditions are caused by abnormalities of secondary neurulation?

Thickened filum with tethered cord
** something else

41

What is MOMS?

Management of myelomeningocoele study - RTC

42

What are the outcomes of MOMS?

Decreased shunts and chiari 2 in infants with IU repair

43

What is the prognosis of IU repair of myelomeningocoele?

Sacral will walk
Lumbar 50:50% normal walking
Thoracic will not walk

44

When should a myelomeningocoele be repaired in a term baby?

Closure within 48 hours; Wrap it in plastic

45

How does shunt malfunction present in a patient with previous myelomeningocoele repair?

Shunt malfunction can result in leg pain / weakness then think about chiari 2, syrinx, dermoid, arachnoid and tethered cord.

46

Patient with hairy patch on lower back but otherwise normally. Diagnosis?

Split cord malformation (normal walking and bowel etc)

47

Patient with spinal cord malformation presenting with recurrent meningitis

Dermal sinus

48

What is a sacrococcygeal sinus / pit?

These are low between the gluteal fold and are not of clinical significance as do not connect with the cord

49

Where is the conus in the newborn?

L3 - returns to L1/2 by 3 months!

50

When should you operate on a dermal sinus?

As soon as safe due to risk of infection

51

Where do paediatric tumours occur?

1 year - 75% infratentorial

52

What is infantile desmoplastic astrocytoma?

WHO grade 1 - can have neuronal differentiation and is called desmoplastic infantile ganglioglioma

53

Supratentorial mass lesion with cartilage, respiratory epithelial and neural cells. Diagnosis?

Teratoma

54

What are the histological features of medulloblastoma?

Homer-Wright Rosettes, small blue cells high density

55

What does the term desmoplastic mean?

Lots of collagen

56

Which medulloblastoma molecular subtype has the best prognosis?

Wnt (wingless) abnormality


Group 3 have the worse prognosis - have MYC amplification

57

What are the cerebellar embryonal tumours?

ATRT, medulloblastomas and ETANTR

58

What are ATRT?

Atypical rhabdoid/teratoid tumour

59

What are ETANTR?

These are part of a family of Embryonal tumours with multilayered rosettes (ETMRs)

60

What do ependymal rosettes look like?

Here the cells coalesce around an ependymal lined cavity

61

What are the features of a desmoplastic infantile glioma?

No restriction, large cyst, no calcification and durally based!

62

4 year old with hyperdense midline posterior fossa tumour. Diagnosis?

Medulloblastoma

63

What are the factors for risk stratification of medulloblastomas?

High risk is any of: Age 1.5 cc

64

What is the management of high risk medulloblastomas?

Radio and chemotherapy gives 20-65% 5 year survival if >3 years
Chemotherapy only and bone marrow rescue if

65

DD of suprasellar tumours in children?

Craniopharyngiomas
Chiasmatic hypothalamic glioma - solid or cystic, no calcification and increased restricted diffusion
Suprasellar germinoma

66

What is the management of hypothalamic low grade gliomas?

Chemotherapy

67

Pineal region tumour with calcification, heterogenous with calcifications and CSF tumour marker negative. Diagnosis?

Mature teratoma - needs surgical resection!

68

Approaches to the pineal region?

Supracerebellar infratentorial or Occipital Transtentorial

69

Which vein attaches to the vein of galen from inferiorly?

Precentral cerebellar vein

70

Differential diagnosis of a pineal region tumour?

Germ cell tumours - germinomatous or non-germinomatous germ cell tumours
Pineal tumours (pineoblastoma / cytoma)
Gliomas - tectal plate
Others - choroid plexus tumour / cavernoma

71

What does raised CSF bHCG suggest?

Choriocarcinoma

72

What does raised CSF AFP suggest?

Immature teratoma?

73

When should a pineal region tumour be biopsied?

If the tumour markers are absent. If germinoma then no resection; If teratoma, glioma or pineal origin then resect the tumour;

74

What is the diagnostic yield of stereotactic biopsy for pineal region tumours?

90% (1% mortality and 1% major morbidity)

75

Pineal region tumour - ETV or biopsy first?

ETV first as this is main goal. No risk of dissemination of tumour cells by doing ETV before biopsy.

76

Is endoscopic or stereotactic biopsy of pineal region tumours better?

Stereotactic yield 90%; Endoscopic biopsy 80%;

77

What are the types of Chiari malformation?

1 - >5mm tonsillar herniation below the FM; Assoc with syrinx
2 - Herniation of the vermis; Assoc with MMC
3 - Occipital or high cervical encephalocoele
4 - Cerebellar aplasia or hypoplasia

(Chiari 1.5 is tonsilllar herniation with elongated brainstem and 0 is where there is no tonsillar herniation but CSF block at the FM)

78

What are the features of Chiari 1?

Headache - cough related
Syrinx related features:
Sleep apnoea
Swallowing difficulty
Scoliosis
Syrinx motor / sensory abn

79

What is the incidence of Chiari 1 in asymptomatic population?

2.5% are asymptomatic and 1% are symptomatic

80

Why image the entire spine in a child with a chiari malformation?

Tethered cord / MMC / Syrinx

81

What is the natural history of a child with a Chiari 1 (asymptomatic)?

50% stable, 38% improved and 12% worsened;
No neurological deficit occured
Strahle et al 2011

82

What is the first neurological sign with a syrinx?

Loss of reflexes

83

Should a duroplasty be performed with Chiari FMD?

Durham et al 2008 showed no difference in outcome but the re-operation rate was less, however CSF complication rates were higher with opening the dura!!

84

Features of Chiari 2

Vermian descent
CervicoMedullary kinking
Up running nerve roots
Myelomeningocoele
Beaking of the tectum
Enlarged massa intermedia
Hydrocephalus
Cerebral heteratopia / polymicrogyria
Skull craniolacunia (dysplasia of the skull)
Low transverse sinus
Small posterior fossa

85

Should prophylactic chiari decompression be done with MMC?

No as syrinx will only become symptomatic in 15-30%

86

What are the symptomatic complications of Chiari 2?

Medullary compression - poor feeding, prolonged apnoea and stridor (vocal cord palsy)
Hydrocephalus
Syrinx
Upper cervical spine root injury

87

What operation should be performed for Chiari 2?

Basically a cervical laminectomy NOT an FMD;
Usually don't need to remove the suboccipital bone
Probably don't need to open the dura in the majority of these patients.

88

Preterm infant; always stiff and MRI shows reduced white matter in one hemisphere? Diagnosis?

Periventricular leukomalacia

89

What is the most common cause of intractable epilepsy in children that undergo surgery?

42% is due to FCD

90

What is FCD?

Focal cortical dysplasia is a disruption of normal cortical lamination

91

What is the Longterm outcome with VNS for epilepsy?

Up to 50% have >50% reduction in seizure frequency

92

What is the distribution of tumours in an infant?

<1y = 75% supratentorial
>1y = 75% infratentorial

93

What is the histology of a desmoplastic .... paediatric tumour?

Look up

94

What is the rosette feature of medulloblastoma?

Homer-wright rossettes

95

What stains for connective tissue?

Reticulin

96

Medulloblastoma with strong reticulin staining?

Desmoplastic / nodular type

97

What are the types of SHH medulloblastoma?

P53 mutant and non-P53 mutant

98

Which molecular medulloblastomas are histologically desmoplastic?

SHH group if non P53 mutant
(Anaplastic if SSH p53 mutant)

99

What are ATRTs?

Tumour of children <5 y
Supra or infratentorial
Rhabdoid cells
INI1 protein (SMARCB1 gene)

100

Which ependymal tumours have the worst prognosis?

Supratentorial RELA positive

101

What mutation is associated with diffuse midline Gliomas

H3 K27M
Very poor prognosis!!

102

What mutation occurs with Pilocytic astrocytomas?

BRAF

103

What are the urinary biomarkers of Pilocytic astrocytomas?

Look up paper

104

What are the radiological features of a desmoplastic infantile glioma?

No restricted diffusion
Can be durally based
Look up the rest

105

What paediatric tumour is hyperdense on CT?

Medulloblastoma

106

Which children have ATRF?

Infant

107

What are the factors that make a medulloblastoma high risk?

High risk =
Age <3
Mets
Tumour residual >1.5cc

108

What is the medullo high risk treatment protocol >3years?

Rt to craniospinal axis with vincrinstine
Post fossa Rt boost
CCNU chemotherapy

109

What is the medullo high risk treatment protocol <3years?

No Rt
Chemotherapy
Stem cell transplant

110

How do manage a DIPG?

RT+Chemo
No need for biopsy (unless abnormal radiological features)
No role for surgery

111

What is the prognosis?

Median survival 9 months

112

Suprasellar lesions with solid or cystic features, bright enhancement, no calcification and increased diffusivity?

Chiasmatic / hypothalamic glioma
(pilocytic or pilomyxoid astrocytoma)

113

What are the treatment for deep / optic pilocytic astrocytomas?

Chemotherapy!! Only 50% respond;
If no response then Surgical resection

114

What factor favours teratoma in the pineal region?

Calcification and marker negative
Needs surgical resection

115

What is a homogeneous tumour in the pineal region?

Germinioma

116

What vein is encountered in the midline during a supracerebellar infratentorial approach?

Precentral cerebellar vein

117

What marker are sent for pineal region tumours?

bHCG (germinoma if <50; NGGCT if >50)
AFP (teratoma)
PLAP (Germinoma)
C-Kit (Germinoma)

118

What do you do if the tumour markers are negative with a pineal region tumour?

If suspected teratoma then resect
Otherwise biopsy (stereotactic if small ventricles or endoscopic with ETV - needs 2 burr holes if rigid scope)

119

With pineal region tumour - do you do ETV or biopsy first?

ETV first and biopsy second as the main goal is to treat hydrocephalus. If the biopsy bleeds ++ you might not be able to do the ETV

120

Does ETV cause tumour CSF spread?

No evidence of increased risk

121

Which condition has narrowing at the jugular foramen?

Achondroplasia

122

What is the treatment for tectal glioma?

ETV

123

How do you calculate the chance of ETV success?

Kulkarni et al 2010:
Age (max 50)
Etiology (max 30)
Previous shunt (max 10)

124

What procedure can you do to improve the success of ETV?

Choroid plexus coagulation (need more than 90% to be coagulated so need a flexible scope!)

125

What is the shunt failure rate?

40% in the first year and 10% the year after

126

What are the types of germinal matrix haemorrhage?

**

127

What interventions are done for hydro secondary to IVH in perinatal period

Subgaleal reservior to buy some time until pt is older and then shunt the patient
EVD - risk of infection
VP shunt - risk of NEC and infection
ETV - likely unsuccessful, no information

128

See the systemic review in JNS:paeds 2014 for hydrocephalus management in paeds

**

129

What is craniosynostosis?

Premature closure of 1 or mor calvarial suture

130

What is craniostenosis?

The skull is too small for the brain

131

What is an elongated boat shaped head called?

Scaphocephaly (sagittal sinus)

132

What is the most common type of craniosynostosis?

Sagittal

133

How do you classify craniosynostosis?

Isolated vs syndromic
Single vs multiple
Primary vs secondary

134

What are the secondary causes of craniosynostosis?

Metabolic disorders
Mucopolysaccharidosis
Haematological disorders
Teratogens
Post-shunts

135

Which type of craniosynostosis is increasing in frequency?

Metopic

136

What is the mercedes benz syndrome?

Bilateral lambdoid and posterior sagittal suture synostosis?

137

What tests can you do for craniosynostosis?

Xray
USS of the sutures
CT (high radiation!)

138

What is the difference between dolichocephaly and scaphocephaly?

Dolichocephaly is positional whilst scaphocephaly is craniosynostosis

139

What happens the eyes with anterior plagiocephaly?

Orbital dystopia (eye is pulled up on the synostosis side!) Needs an orbital advancement

140

How can you differente positional from craniosynostosis plagiocephaly?

The eyes are not affected with positional - So called Harlequin eye!

141

What is metopic suture?

Between the two frontal bones

142

What is caused by metopic suture synostosis?

Trigonocephaly - do a CT scan if you think they have other midline fusion abnormalities such as cleft lip etc

143

How you differential lambdoid synostosis from posterior positional plagiocephaly?

The ear goes back with and the mastoid is lower with lambdoid fusion

144

Do you do a CT scan for lambdoid synostosis?

Yes as the diagnosis is difficult

145

How do you prevent blood loss in paediatrics?

Tranexamic acid

146

Can you take a craniotome across an open suture?

No - It will tear the sinus!!

147

Do you put the bone back after craniosynostosis surgery?

<6 months - don't put bone back
>6 months - put bone back

148

What technique do you know for fixing scaphocephaly?

Clam shell procedure with barrel stave cuts
Put helmet on after to prevent AP growth and promote medial-lateral growth

149

What is moved forward with fronto-orbital advancement?

Orbital bar

150

What are the common syndromes with craniosynostosis?

Mueken
Cruzon
Apert
Pfeiffer
Saethre-Chotzen

151

Which has polysyndactyly

Apert's

152

What is clover leaf skull?

Tree-foil appearance seen with Pfeiffer's syndrome

153

Which syndromic craniosynostosis has hydrocephalus?

Pfeiffer > Crouzon > Apert

154

What is the genetic condition behind craniosynostosis?

Autosomal dominance
FGFR mutations

155

Which patients with craniosynostosis should you do genetic testing?

All coronal
Other abnormality
Multiple suture

156

What is a Chiari 0

Syrinx with CSF block at the FM but no tonsillar decent

157

What is platybasia?

Flattening of the skull base - clivus is shallow with angle >145 with the anterior skull base

158

What proportion of children have an asymptomatic chiari 1?

2.5%!

159

Which Chiari patients need a sleep study?

History of snoring

160

Do you operate on an asymptomatic chiari?

No

161

What is the natural history of an asymptomatic Chiari malformation?

Stable 50%
Improved 40%
Worse 10%

162

Would you operate on an asymptomatic Chiari with syrinx?

Most do not deteriorate and there is no evidence to support surgery. Reasonable to observe or do surgery.

163

What can be mistaken for the tonsils during Chiari 2 operations?

Cervicomedullary kinking

164

Is agenesis of the corpus callosum associated with Chiari 2?

NO

165

What is craniolacunia?

Think areas in the skull

166

Is enlarged mass intermedia associated with Chiari 2?

YES! Also beaking of the tectum

167

Which patients with Chiari 2 have the worst prognosis?

Stridor with bilateral VC palsy at birth!

168

Where is the transverse sinus in Chiari 2?

Very low near the FM

169

What is the treatment for Chiari 2?

Cervical laminectomy not FM decompression

170

Should you decompress a medullary cyst below the tonsils in a patient with Chiari 2?

NOPE

171

Do you need to open the dura with Chiari 2?

NOPE

172

What is the lemon and banana sign associated with?

On in utero USS is associated with Myelomeningocoele (Chiari 2 seen on MRI)

173

Myelomeningocoele is associated with which developmental process?

Primary neurulation

174

How can you differentiate a problem with primary or secondary neurulation?

Secondary is below S1/2; Associated with sacral agenesis and rectal malformation

175

Which conditions are associated with failure of gastrulation?

Split cord malformation
Neurenteric cyst

176

What conditions are associated with secondary neurulation failure?

Caudal agenesis
Thick filum
Filum lipoma
Sacral agenesis

177

What is the MOMS study?

RCT into the prenatal repair of myelomeningocele:
Reduces shunt requirement
Reduces Chiari 2
Improved lower limb neurology

178

Do you need to do a C-section for myelomeningoceole?

No evidence either way

179

What should you rule out in patient with back pain and myelomeningocele?

Shunt malfunction!!

180

What is the most likely malformation associated with a hairy patch on the lower back of a child?

Split cord malformation = diastomatomyelia (gastrulation problem)

181

What are the types of split cord malformation?

type 1 = 2 dural sacs with 2 cords separated by spur
type 2 = 1 dural sac with 2 cords

182

What do you do with a ventral dural defect with a split cord malformation?

NO

183

What causes a skin abnormality and a low lying cord?

Limited dorsal myeloschisis

184

What is Limited dorsal myeloschisis

Disorder of primary neurulation resulting in incomplete fusion of the neuropore. Associated with a skin pit / crater (look like cigarette burns). A band of fibrofatty tissue from the skin attaches to the spinal cord and tethers it!

185

What are the problems with dermal sinuses?

Meningitis!

186

What is the saccular form of limited doral myeloschisis?

Cyst at the skin with connection to the cord that needs to be disconnected intradurally. Cysts can be incontinuity with the syrinx (myelocystocoele).

187

What is it called when there is a large lipoma tethering the cord?

Dorsal, transitional or transitional conus lipoma - they slowly loose neurological function so cord needs untethering and aggressive removal of all the fat!

188

What are occult dysraphism?

Closed myelomeningocele - the neurologicla deficit is usually due to tethering

189

At what age does the conus reach the same position as a child?

3 months!

190

Why operate on spinal dyraphism

Cord untethering
Decompression
Remove portal for infection

191

Cephalhaematoma

**

192

Eosinophilia granulomatosis in bone

Irregular edges
No sclerosis
Observe (or curettage / steroids to lesion)

193

Bone punched out lesion, thickened at edges. Midline near ant fontanelle.

Dermoid cyst
Resect it. No need for craniotomy.

194

Growing skull fracture complication

Leptomeningeal cyst

195

Types of hypertonia

Spasticity
Dystonia
Rigidity

196

What is spasticity?

Velocity dependent hypertonia

197

Causes of abnormal gait in a child with CP

Weakness
Incoordination
Contractures
Spasticity

198

Scales for spasticity

Ashworth
Tardieu

199

Pros and cons of selective dorsal rhizotomy vs baclofen

**

200

In paediatric thalamic tumours can grade be estimated from imaging?

NO -- pilocytic and anaplastic all look the same. Need to fully resect the lesion.

201

Causes of paediatric infarction?

**

202

What conditions are associated with Moyà Moyà

NF1
Sickle cell

203

Atretic parietal encephalocoele

Look it up!

204

What are the scalp masses in a baby?

Make a list...

Sinus pericranii
Dermoid
Infantile haemangioma
Encephalocoele

205

How does hydralazine affect ICP?

Vasodilator that increases ICP