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Paediatrics > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

What factors should be included in the birth history for a child?

A

Mothers age Number of pregnancies / deliveries Prenatal care Delivery method Pregnancy illnesses / rashes Maternal ETOH / drug use Meconium staining Gestational age / corrected age

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2
Q

What does a family history of neonatal deaths suggest?

A

Metabolic disorders

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3
Q

Which tissues are of neuroectodermal origin?

A

CNS Hair Skin Teeth Nails

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4
Q

What is a normal head circ. at birth?

A

35 cm. Grows at 2 cm/month for the first 3 months, then 1 cm/monh from 3-6 months

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5
Q

What conditions may lead to an enlarged anterior fontanelle?

A

Hypothyroidism, trisomy syndromes, in utero malnutrition, hypophospatemia, rickets, osteogenesis imperfecta and hydrocephalus.

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6
Q

How do you test CN2 in a neonate?

A

After 30 weeks gestation they have a blink response to bright light and have a reliable pupillary reflex. Fixing and following of gaze suggests normal visual acuity. Check the RAPD.

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7
Q

How do you test CN3/4/6 in a neonate?

A

Eye movements by fixing and following on mother’s face. Dolls eye reflex.

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8
Q

How do you test CN5 in a neonate?

A

Watching the neonate suck / swallow on a dummy / bottle feeding.

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9
Q

How do you test CN7 in a neonate?

A

Facial symmetry at rest and during suckling

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10
Q

How do you test CN8 in a neonate?

A

Spinning the infant to see if the eyes remain static or fixed on an object. Caloric testing can be performed for formal assessment. Hearing can be assessed by blink reflex to loud sounds

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11
Q

How do you test CN10 in a neonate?

A

Infant cry sound
Gag reflex

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12
Q

How do you test CN11 in a neonate?

A

Spontaneous shoulder function / movement

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13
Q

How do you test CN12 in a neonate?

A

Observation of atrophy and / or tongue deviation

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14
Q

How does tone change in neonates?

A

28-40 weeks there is increased flexor tone legs>arms; After 40 weeks the extensor tone becomes balanced.

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15
Q

What does extension of the lower limbs in the premature infant indicate?

A

Hypotonia

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16
Q

How is cervical tone tested?

A

Pulling the infant up to a sitting position. In a term infant there is some head lag. Tone is tested through vertical and horizontal suspension

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17
Q

What is the vertical suspension test?

A

Holding the infant upright under the axilla. If hypotonic will slip through the hands.

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18
Q

What is the horizontal suspension test?

A

Hold the infant in the prone position. The infant should be able to hold their head up

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19
Q

When can reflexes be tested in neonates?

A

After 33 weeks gestation. Note extensor plantars and sustained clonus up to 8 beats is normal.

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20
Q

What is the moro reflex?

A

Dropping the infants head in relation to the body results in extension and abduction of the arms. This is mediated by the vestibulospinal pathway and disappears by 6 months of age.

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21
Q

When should the palmar grasp reflex be lost?

A

4-6 months

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22
Q

How do you test CN3/4/6 in an infant?

A

Visually follow a toy

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23
Q

Where do lesions causing upgaze paralysis localise to?

A

Periaqueductal gray matter (pineal region / tectal tumours).

Vertical gaze center is the rostral interstitial nucleus of the MLF whilst the horizontal gaze center is the PPRF.

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24
Q

What is the difference between phorias and tropias?

A

Phorias are squints that can be overcome by fixating on an object whilst tropias are squints at rest that cannot be overcome by fixating on an object

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25
Q

How can phorias be identified?

A

The cover test - when fixating on an object, both eyes align. When the eye is covered the squint returns.

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26
Q

How are CN9/10/11 tested in infants?

A

Gag reflex Shoulder droop / head tilt

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27
Q

How are spasticity and rigidity differentiated?

A

Spasticity is clasp knife (velocity dependent) whilst rigidity has resistance throughout the full range of movement

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28
Q

At what age do infants crawl?

A

9-12 months

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29
Q

At what age do infants walk?

A

12-15 months

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30
Q

What does limb atrophy suggest?

A

LMN lesion

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31
Q

How can fine motor coordination be tested?

A

Have the child reach for objects and look for tremor or dysmetria

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32
Q

What does the absence of the parachute response predict?

A

A predictor of whether a child will develop the ability to walk. It should be present by 8 months

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33
Q

What is a conduction aphasia?

A

Results in lesion of the arcuate fasciculus. They retain the ability to name objects but not to repeat them.

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34
Q

Where does calculation function reside?

A

Dominant angular gyrus - damage to this area results in acalculia which is part of Gerstmann’s syndrome

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35
Q

What levels do the spinal accessory nerve roots arise from?

A

C3/4

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36
Q

What is the GMFCS?

A

Gross motor function classification system: 1 = Normal 2 = Difficulty walking long distances 3 = walks with hand held mobility aid 4 = needs a wheelchair and help transferring 5 = powered wheel chair, limited head and trunk control

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37
Q

What is the pathophysiology of Cerebral palsy?

A

Perinatal infarct involving the peri ventricular WM which compromises descending inhibition from reticular nuclei to the spinal cord. This causes abberant spinal reflex arcs.

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38
Q

What is the management of cerebral palsy?

A
  1. OT and physio for orthotics and mobility aids
  2. Pharmacological intervention with baclofen / Botox injections
  3. Orthopaedic surgery to release contractures and bone deformity.
  4. Intrathecal baclofen pump or SDR
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39
Q

What is the selection criteria for SDR?

A

Based on Peacocks criteria:

  1. Age >3 years
  2. >1 year after ortho surgery
  3. Spastic diplegia - no ataxia and good trunk control
  4. GMFCS 2/3
  5. PVL on mri that spares basal ganglia and cerebellum
  6. No femoral head subluxation
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40
Q

What are the EMG responses to SDR?

A

Based on Fasano’s SDR protocol:

0 = single discharge

1 = sustained discharge in single segment

2 = sustained discharge adjacent segment

3 = discharge in all Ipsilateral segments

4 = contralateral muscle response

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41
Q

What proportion of the dorsal roots are sectioned in SDRs?

A

50-75% of the total

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42
Q

What are the indications for botox in focal spasticity?

A
  1. Improve motor function
  2. Improve care / hygiene
  3. Reduce Pain / sleep disturbance
  4. Improve tolerance of orthoses
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43
Q

What are the contraindications to Botox treatment for focal spasticity

A
  1. Severe muscle weakness
  2. Allergy to botoc
  3. On aminoglycoside treatment
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44
Q

What is microcephaly?

A

A head circ >2SD below the mean Primary microcephaly is present at birth Secondary microcephaly occurs post-natally

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45
Q

What are the causes of microcephaly?

A

Isolated Syndromic - Downs, Trisomy 13, Trisomy 18, fetal alcohol syndrome Development - NTDs, holoprosencephaly, lissencephaly etc Metabolic - aminoacidurias / storage disorders Environmental - Perinatal ischaemia / infection, toxin, malnutrition

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46
Q

What are the causes of macrocephaly?

A

Familial Hydrocephalus BESS Neurophakomatosis Achondroplasia Haemorrhage AVM Cyst Tumour

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47
Q

What is the dose of a fluid bolus in a child?

A

20 ml/Kg (dose of RBCs in 10 ml/kg!)

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48
Q

What is the normal HR and BP for a child?

A

<12 months = <160 & >60 mmHg

12-24 months = <150 & >70 mmHg

2-5 years = <140 & >75 mmHg

6-12 years = <120 & >80 mmHg

>13 years = <100 & >90 mmHg

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49
Q

What is the normal RR for a child?

A

<12 months = <60 12-24 months = <40 2-5 years = <35 6-12 years = <30 >13 years = <30

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50
Q

What volume of blood loss results in bradycardia in a child?

A

>45%

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51
Q

What are Brushfield spots?

A

Speckled iris seen in Down syndrome

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52
Q

What cardiac defects are seen in Down syndrome?

A

ASD / VSD / PDA / Tetralogy of fallot

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53
Q

What is clindodactyly>

A

Curved little finger seen in Downs

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54
Q

After what age are the brainstem criteria in children the same as adults?

A

>2 months

Between 37 weeks and 2 months diagnosis of brainstem death is very difficult.

Two different consultants should perform the first and second examinations >12 hours apar if >30 days old and >24 hours apart if <30 days old

Two apnea tests required with final pCO2 >60

Ancillary tests not needed unless clinical examination and apnea cannot be completed

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55
Q

What is a Galant reflex?

A

Scratching the skin on the back lateral to the spinous process resulting in curvation of the trunk (concave to the stimulated side)

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56
Q

What is a crossed extensor reflex?

A

Passive flexion of one lower limb results in extension of the other lower limb with adduction and internal rotation

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57
Q

What is the Rossolimo response?

A

Tapping of the toes causes flexion

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58
Q

What is the suprapubic extensor response?

A

Pressing the skin over the pubic bone causes extension, adduction and internal rotation of the lower limbs

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59
Q

At what age does a child sit with and without support?

A

6 and 9 months

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60
Q

When should a child walk independently?

A

15 months

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61
Q

When can a child write their own name?

A

6 years (also identifies written letters and numbers)

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62
Q

What are the features of cloverleaf deformity aka kleeblattschadel?

A

Frontal towering

Bitemporal expansion

Bilateral supraorbital recession with proptosis

Hypertelorism

Midface hypoplasia

Caused by premature fusion fo the sagittal, coronal and lambdoid sutures

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63
Q

What synostosis syndrome is associated with a broad big toe or thumb?

A

Pfeiffer syndrome

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64
Q

What synostosis syndrome is associated with syndactyly?

A

Aperts syndrome

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65
Q

What head shape does metopic synostosis cause?

A

Trigonocephaly. Associated with Chiari 1 in 30% due to reduced intracranial volume, callosal dysgenesis and holoprosencephaly. Metopic suture closes normally between 3-9 months.

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66
Q

What causes scaphocephaly?

A

Sagittal suture synostosis

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67
Q

What causes a parallelogram-shaped head?

A

Positional plagiocephaly. Note the ear and forehead go forward, unlike with unilateral lambdoid synostosis where the forehead and ear go backwards.

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68
Q

What is the incidence of craniosynostosis?

A

1 in 2000-2500 live births

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69
Q

What is the most common type of synostosis?

A

Sagittal > Metopic > Unicoronal > Lambdoid

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70
Q

What causes a Harlequin sign?

A

Unicoronal suture synostosis causes restriction of the orbit to advance

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71
Q

What are the causes of copper beating?

A

Chronic hydrocephalus

Craniosynostosis

Hypophosphatasia

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72
Q

What are the features of Saethre-Chotzen syndrome?

A

Caused by a genetic mutation in the TWIST1 gene.

Causes unicoronal or bicoronal (brachycephaly) synostosis.

Associated with ptosis, low hairline, wide-spaced eyes and broad nose and hand/feet abnormalities.

Second and third fingers may be webbed and the big toe may have a duplicate bone.

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73
Q

What bony abnormalities are seen with NF1?

A

Lytic defects in the lambdoid suture

Absent orbital roof or floor causing pulsatile exopthalmos

Elevated lesser sphenoid wing

Enlarged middle cranial fossa

Enlarged CN foramina

J-shaped sella turcica

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74
Q

What do skull epidermoids look like?

A

Sharply demarcated skull lesions with well defined sclerotic borders. They usually disappear within a few years of discovery.

75
Q

What causes multiple destructive foci in the calvaria?

A

Think TB, myeloma and LCH

76
Q

What is the cephalic index?

A

CI = Biparietal distance / orbitofrontal distance.

Normal is 74-83.

77
Q

What is dolichocephaly?

A

When the head is longer than expected. The cephalic index is <74. Sagittal synostosis is a form of dolichocephaly.

78
Q

Which craniosynostosis is associated with low-mid frequency sensorineural hearing loss?

A

Muenke’s sydrome (61%)

Seen more in males

Uni or bicoronal synostosis

*Note the rest of the craniosynostosis syndromes have conductive hearing loss

79
Q

What are the features klippel-feil?

A

Short neck

Low posterior hairline

Limited neck mobility

80
Q

What gene mutation causes achondroplasia?

A

FGFR3 gene

Autosomal dominant but 80% of cases are sporadic

81
Q

What causes hydrocephalus in achondroplasia?

A

Flattened skull base causes foraminal stenosis and venous hypertension or cervicomedullary compression. This results in macrocephaly. Most stabilise so do not shunt.

82
Q

What are probst bundles?

A

Non-crossing callosal axons in cases of callosal agenesis resulting in widely spaced lateral ventricles and racing car sign

83
Q

Where are intracranial lipomas?

A

Most commonly in the interhemispheric fissure (50%) occupying the subarachnoid space so blood vessels and cranial nerves course through them.

Show a chemical shift artefact.

84
Q

What is a chemical shift artefact?

A

Where fat is mismapped onto the image

85
Q

What is this condition?

A

Note the pituitary bright spot in the floor of the third ventricle as the posterior pituitary has not formed

86
Q

How can you differentiate a persistent blake’s pouch cyst from a dandy-walker malformation?

A

Blake’s pouch cysts have choroid plexus displaced into the cyst. These are due to an imperforate foramen of magendie.

87
Q

What are the features of a mega-cisterna magna?

A

Enlarged cisterna magna

No hydrocephalus

Intact vermis

Most likely due to cerebellar volume loss

Asymptomatic patients with an incidental finding,

**IF there is hydrocephalus then think Persistent Blakes pouch cyst.

88
Q

What are the types of lissencephaly?

A

Type 1 = classical with lissencephaly and band heterotopia. Cortex has 4 layers.

Type 2 = Lissencephaly with cobblestone cortex. No layers.

LIS1 gene mutation

89
Q

What lines schizencephaly?

A

Abnormal gray matter (polymicrogyria)

Cortical development phases are proliferation, migration and organisation. Schizencephaly can be caused by disruption of any of these

90
Q

What is the most common location for polymicrogyria?

A

Sylvian cortex.

MRI shows irregular appearance of the inner and outer cortex with diffuse cortical thickening.

91
Q

What are the considerations in a patient with a spinal dysraphism?

A

Cutaneous features

Neurological status (lower limb power and sphincter function)

Bowel / bladder function (neurogenic bladder) **Must do urodynamic testing for preoperative evaluation**

Orthopaedic (scoliosis, kyphosis, lordosis, leg length discrepancy and foot deformity)

92
Q

What are the presenting features of a tethered cord?

A

Back pain

Leg pain

Regression of sphincter control

Regression of mobility

Scoliosis

93
Q

Why do an MRI in a patient with a myelomeningocele?

A

Assess associated pathologies such as split cord malformation, lipoma, dermoid, epidermoid and chiari 2.

94
Q

What did the MOMS trial show the benefits of fetal myelomeningocele repair were?

A

Lower risk of death and need for VP shunt insertion during the 1st year of life (68% vs 98%) with fetal closure between 18-25 weeks. There was improved motor and mental development at 30 months.

95
Q

What is the acute management of a myelomeningocele?

A

Keep prone

Assess lesion then cover with sterile gauze and cling film

Prophylactic antibiotics

Surgical closure within 72 hours

96
Q

How do you treat a neurogenic bladder in a child with spinal dysraphism?

A

Clean intermittent catheterisation

Antibiotic prophylaxis

Anticholinergic medications (reduce the reflex parasympathetic contraction against a closed sphincter which causes vesicoureteric reflex).

97
Q

What causes a new or rapid progression of a scoliosis in a patient with a myelomeningocele?

A

Shunt block

Syrinx

Tethering

Most children with an MMC above L2 will develop a scoliosis

98
Q

What is the difference between a lipomyelocele or a lipomyelomeningocele?

A

In lipomyelocele the lipoma:placode interface is within the canal whilst in lipomyelomeningocele the interface is outside the canal.

99
Q

What substance is T1 bright but dark on STIR?

A

FAT

100
Q

When does the L5 lamina fuse posteriorly in normal cases?

A

5-6 years

101
Q

What is a dermal sinus tract?

A

An epithelium lined cavity between the skin and the CNS. Patients have a midline dimple. Caused by a failure of gastrulation due to defective notochord development.

102
Q

What are the types of split cord malformation?

A

Type 1 (25%) = 2 dural sacs with a midline fibrous septum

Type 2 (75%) = 1 dural sac and no midline septum

103
Q

What is caudal regression syndrome?

A

Failure of secondary neurulation resulting in spinal anomalies, imperforate anus, cloacal/genital anomalies, pulmonary hypoplasia, limb dysplasias

104
Q

What is a dorsal enteric cyst?

A

Where the is a continuation of bowel through the vertebral body, spinal canal and cord to the skin

105
Q

How do you test mothers for fetal NTDs?

A

Serum maternal AFP at 15-20/40

106
Q

What fetal USS findings are suggestive of a Chiari 2?

A

Lemon - narrow frontally due to low pressure

Banana - posterior convex cerebellum

Microcephaly

Obliteration / full foramen magnum

107
Q

What stage of embryological development is affected in Currarino triad?

A

Gastrulation (notochordal abnormality)

108
Q

What rate do neonated produce CSF?

A

25 ml/day. This increases to adult capacity as a toddler.

109
Q

What is the most common intrauterine infection that causes hydrocephalus?

A

Toxoplasmosis > Mumps > CMV

110
Q

What is the risk of a shunt tap causing an infection?

A

2 in 1000

111
Q

What is the ETV success score?

A

Age: <1 month = 0, 1-6 months = 10, 6-12 moths = 30, 1-10 years = 40 and >10 years = 50

Etiology: Infection = 0, MMC/IVH/tumour = 20, Aqueductal stenosis = 30

Shunt previously: No = 10

Provides the 6 month ETV success rate.

112
Q

What are Lundberg waves?

A

Describe mean ICP patterns in patients

A = mean wave ICP >50 mmHg lasting between 5-20 minutes. Suggests ICP exceeding cerebral compliance

B = mean wave ICP 20-50 lasting < 5 minutes. Seen in sleep.

C = mean wave ICP < 20 occuring every 10 seconds. Due to oscillations in baroR and chemoR control.

113
Q

What is thought to be the underlying etiology of BESS?

A

Immaturity of the arachnoid villi

114
Q

What is the triad of shaken baby syndrome?

A

Subdural haemorrhages

Retinal haemorrhages

Encephalopathy

115
Q
A
116
Q

What organism causes intracerebral abscesses in neonates?

A

Citrobacter Koseri

117
Q

What is the LCAM1 mutation associated with?

A

X-linked hydrocephalus

118
Q

What is a birds nest appearance of distal shunt tubing associated with?

A

Migration of distal catheter out of the peritoneum into the subcutaneous tissue

119
Q

What is the incidence of haemangioblastoma in children?

A

1% of posterior fossa tumours in children

120
Q

What is the peak age for pilocytic astrocytoma?

A

5-13 years

121
Q

What is the treatment of an OPG?

A

Resect nerve of the blind eye to save contralateral vision if threatening the chiasm

Chemotherapy is given if < 9 years

Radiation is contraindicated in NF-1 due to risk of moya moya and other brain tumours

122
Q

What are DIPGs histologically?

A

High-grade fibrillary astrocytomas

Median survival 12 months

Treatment is with radiotherapy (54Gy)

123
Q

Which germ cell tumours have high AFP and HCG?

A

Embryonal carcinomas

(AFP rise alone = Yolk sac > Teratoma and HCG rise alone = choriocarcinoma)

124
Q

What does a pineal tumour with a rim of exploded calcium suggest?

A

Pineal parenchyma tumour (pineocytoma / pineoblastoma)

125
Q

What is the treatment of a non-germinomatous germ cell tumour?

A

Neoadjuvant chemotherapy, followed by resection and post-op irradiation (craniospinal radiation may be need if evidence of cs seeding)

126
Q

What is the difference on MR Spect between a choroid papilloma and carcinoma?

A

Papillomas have a myoinositol peak where as carcinomas have an elevated choline peak

127
Q

What is the treatment for medulloblastoma?

A

Maximal safe resection

If Standard risk > craniospinal irradiation with post fossa boost and concurrent chemotherapy.

If high risk > craniospinal irradiation if >3 years, myeloablative chemotherapy followed by stem cell transplantation.

128
Q

What does an INI1 mutation in posterior fossa tumour signify?

A

ATRT.

Treat with max safe resection, chemotherapy and radiotherapy

129
Q

What is aside effect of platinum-based chemotherapy?

A

High freq hearing loss

130
Q

What are the most common paediatric intramedullary tumours?

A

Astrocytoma 46% > Ganglioglioma 35% > Ependymoma 12% > Haemangioblastoma 2%

131
Q

What is the paediatric GCS?

A

E (1- no response, 2- pain, 3- voice, 4- spontaneous)

V ( 1- no response, 2- inconsolable, 3- intermittently inconsolable, 4- consolable, 5- orientated/ smiles)

M (1- no movement, 2- extension to pain, 3- flexion to pain, 4- withdrawal to pain, 5- withdraws from touch, 6- spontaneous and purposeful movement)

132
Q

What is Reye syndrome?

A

A reaction to aspirin seen in children that have a viral illness characterised by vomiting, encephalopathy and hepatic dysfunction

133
Q

What is the PECARN algorithm?

A

The likelihood of developing a clinically important TBI in a child GCS 14/15 and hence the need for CT.

All children GCS14 or with palpable skull fracture need a CT.

134
Q

What is the upper limited of normal ICP in a ventilated infant and older child?

A

<5 mmHg in an infant and <10 mmHg in a child.

**In the context on an LP in the lateral decubitus position then 13 mmHg is the limit in a child**

135
Q

What is the management of a growing skull fracture?

A

Duroplasty and autologous cranioplasty

136
Q

What is the CPP range for children with TBI?

A

40-50 mmHg to keep a brain oxygen tension >10 mmHg

137
Q

What is the max rate of rewarming after induced hypothermia in a child?

A

0.5 C/hour

138
Q

What metabolic condition should be ruled out in patients with suspected NAI?

A

Glutaric aciduria type 1

139
Q

How do you treat SCIWORA?

A

12 weeks in a brace

140
Q

How do you confirm atlanto-axial rotatory subluxation?

A

Dynamic CT with 3 positions (neutral, left and right)

If does not resolve within 4 weeks attempt reduction/manipulation and then immobilize. Surgery is needed if irreducible.

141
Q

What is odontoid epiphysiolysis?

A

Fracture through the C2 dentocentral synchondrosis. This fuses at 7 years and is vulnerable in young children.

Fractures through the epiphysis will heal with reduction and immobilisation using a halo for 10 weeks.

142
Q

How many pins are used with a Halo?

A

<2 years = 12

2-7 years = 8

>8 years = 8

Torque in lb/inch is the same as the age.

143
Q

What is the first-line management of a child with an unstable subaxial injury and no neurology?

A

HALO for 3 months

144
Q

What is more likely to heal, a bony fracture or purely ligamentous injury?

A

Purely ligamentous has a lower rate of of healing

145
Q

What is the difference between a cephalhematoma and a caput succedaneum?

A

Cephalhematomas are subperiosteal haematomas and no to cross sutures

Caput succedaneum is a soft tissue scalp swelling after delivery

146
Q

What is pseudosubluxation?

A

Normal translation < 4mm at C2/3 that occurs in children < 8 years due to ligament laxity. Look at the spinolaminar line between C1-3 to differentiate from a true injury.

147
Q

What causes a vein of galen malformation?

A

Persistence of a single median prosencephalic vein of markowski (which is the primitive internal cerebral vein). As a result normal beep veins are absent.

148
Q

What is the fetal function of the median prosencephalic vein of Markowski?

A

It drains the choroid plexus between the 5-10th week of fetal life. With the development of the thalamus, the median prosencephalic vein regresses and the internal cerebral veins take over.

149
Q

What is the difference in presentation between children and adults with Moya Moya?

A

Children present with TIAs and Strokes.

Adults present with bleeds.

150
Q

What are some risk factors for Moya Moya?

A

Asian origin

Radiotherapy/Proton beam

NF-1

Down’s syndrome

151
Q

Where do moya moya related bleed tend to occur?

A

Basal ganglia

Thalamus

Mesial temporal lobe

Intraventricular

(as this where prominent collaterals form from the posterior circulation)

152
Q

Why do you need to assess the collateral supply pre-operatively in Moya moya patients?

A

So that surgery does not disturb them!

153
Q

What is the classification system for Vein of Galen malformations?

A

Lasjaunicas classsififcation:

Choroidal vs Mural

Choroidal type has feeders from the chorodal A, thalamo-perforators and pericallosal A that enter the anterior part of the median prosencephalic vein in the velum interpositum. These present in young children with cardiac failure.

Mural type has feeders from the collicular and posterior choroidal A that enter the inferior lateral part of the median prosencephalic vein and present with failure to thrive and macrocephaly due to hydrocephalus from impaired venous outflow.

154
Q

What is the treatment for vein of galen malformations?

A

Transarterial embolization when the arterial feeders are large enough to hold a microcatheter.

Transvenous when the arteries are of small calibre and in very high flow shunts

155
Q

What are the characteristic features of Wyburn-Mason?

A

Retinal angiomatosis, facial haemangioma and midbrain AVM

156
Q

What are the common approaches to brainstem cavernomas?

A

OZ, pterional and subtemporal for ventrolateral and lateral

Transpetrous, retrosigmoid and combined approaches for dorsolateral

Suboccipital for dorsal

157
Q

Why does crying and hyperventilation cause strokes in Moya Moya?

A

Lowers the pCO2 and the resulting vasoconstriction causes ischaemia

158
Q

What are the common features of Marfan’s syndrome?

A

Arachnodacyly, high arched palate, lens dislocation, pneumothorax, mitral valve prolapse and intracranial aneurysms

159
Q

How is spasticity graded?

A

Ashworth scale

Note, score of 3 are candidates for intrathecal baclofen. If a test dose of 25-50 micrograms improves by 1 point then it is successful

160
Q

What are the main risk factors with intrathecal baclofen pumps?

A

Headache

CSF leak (5-15%)

161
Q

Where do you place the catheter tip of an intrathecal baclofen pump?

A

T6-10 for diplegia

T1-2 for quadriplegia

Midcervical region for dystonia

162
Q

What are the different parts of a baclofen pump?

A

Central port for drug filling (yellow arrow)

The red circle is the pump

Catheter access port for troubleshooting (blue arrow)

Catheter connector (pink arrow)

163
Q

What level rhizotomy reduces spasticity in the hip flexors?

A

L1!

164
Q

What is the mechanism of action of phenytoin and carbamazepine?

A

Na channel blockers!

165
Q

What is the DBS target for dystonia?

A

Bilateral GPi

166
Q

What are the features of baclofen withdrawal?

A

Fevers, seizures and rebound spasticity

167
Q

What alpha-2-blocker is used in spasticity management?

A

Tizanidine

168
Q

What is a characteristic feature of dystonia?

A

The body parts affected get worse when they move other parts of the body (twisting / patterned movements)

169
Q

What dystonia mutation results in an abnormal TorsinA protein?

A

DYT1

170
Q

How does trihexyphenidyl work?

A

Antimuscarinic used in the treatment of dystonia

171
Q

What is the risk of SAH during pregnancy?

A

Not increased during pregancy, labor or puerperium

172
Q

How do you treat a pregnant patient with a brain tumour?

A

Same treatment as non-pregnant. Try to delay surgery until the second trimester.

Fetal exposure to chemotherapy and radiotherapy to the mother has not shown adverse effects and the fetus should be shielded!

173
Q

How do you treat SAH in pregnancy?

A

If <26 weeks then do what is best for the mother and then allow pregnancy to progress to test

If >34 weeks then C-section and aneurysm treatment.

Between 26-34 weeks treat the aneurysm first and then allow pregnancy to progress to test

174
Q

When ar AVM ruptures most likely to occur during pregnancy?

A

1st and 2nd trimester. Note that the risk of rupture is no higher during pregnancy compared to the rest of life. Elective C-section should be performed if untreated or partially treated AVMs

175
Q

Which hormone causes ligamentous laxity in pregnancy?

A

Relaxin

176
Q

How do you treat Cauda equina syndrome in pregnancy?

A

If >34 weeks then C-section and discectomy

If <34 weekness then discectomy can be prone using a 4-poster frame or in the lateral decubitus position.

177
Q

What is the risk of childhood cancer in a fetus whose mother had a Ct head?

A

1 in a million

178
Q

How do you treat eclamptic seizures?

A

Magnesium via a 6g loading dose IV followed by 2g/hour IV infusion - goal is to achieve a serum level of 4-8 mg/dl

179
Q

What is the risk of coiling to a fetus?

A

Fetal radiation exposure is very small - no additional risk to the fetus

180
Q

How should you manage delivery in a patient with a VP shunt?

A

Prophylactic antibiotics during labour and delivery.

If the patient has no symptoms of raised ICP then vaginal delivery is prefered. If raised ICP then GA and C-section. Note epidural anaesthesia is contraindicated with elevated intracranial pressure.

181
Q

If a pregnant patient has a seizure during pregnancy what should you do?

A

Rule eclampsia (high BP and protein in urine)

Then tap the shunt measuring the opening pressure and MC&S

182
Q

What genes are involved with VoGM?

A

RASA1 EPHB4 HHT

183
Q

Why do VOGM form?

A

Do due presence (failure of regression) of the median prosencephalic vein of markowski

184
Q

How do VoGMs present?

A

Early with high output cardiac failure (right to left shunt with reverse flow - Eisenmenger’s) causing systemic ischaemia and multi-organ failure Late with hydrocephalus from venous hypertension

Paediatrics (13 decks)

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