Pediatrics Neuro Flashcards

1
Q

Baby brain signal differences

A

Opposite adult pattern because myelin high water content (adult=cholesterol, glycolipids). T1 at age 1, T2 at age 2.

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2
Q

Most idea signal to assess myelination in kids?

A

T1 before age 1, T2 after.

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3
Q

Order and timing of brain myelination

A
  • brainstem, pst limb internal capsule myelinated at birth.
  • Back to front, inf to sup (OC at 12 mo –> FL at 18mo)
  • subcortical FTP at 40 mo
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4
Q

Changes in signal of pituitary, globes pallidus, and bone marrow

A
  • Pit = T1 & T2 bright (ant –> iso at 2 mo, pst T2 hypo)
  • GP=darker (Fe incr)
  • Bmarr (active T1 Hypo –> fatty T1 hyper)
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5
Q

order of sinus development

A
  • MESF, complete by 15 yrs
  • ME present at birth, visible by 5mo/1yr (respectively)
  • FS (~5 yrs on CT)

*I (ME) can Smell Finally!

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6
Q

colpocephaly-what is it, ass?

A

asymmetric dilation of occipital horns

  • Colpocephaly is a congenital brain abnormality in which the occipital horns - the posterior or rear portion of the lateral ventricles (cavities) of the brain – are larger than normal because white matter in the posterior cerebrum has failed to develop or thicken.
  • cc genesis, colocephaly, pericallosal lipoma
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7
Q

order of corpus callous formation

A

Front to back –> rostrum

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8
Q

CC parts (Good Bodies In utero Start Right)

A

Rostrum under. Genu, body, isthmus, splenium.

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9
Q

Probst bundles

A

WM tracts destined to cross the CC

Probst bundles or longitudinal callosal fascicles denote white matter fibers normally destined to cross the corpus callosum that instead parallel the interhemispheric fissure in patients with agenesis of the corpus callosum. These lead to widely spaced lateral ventricles forming the characteristic racing car sign.

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10
Q

CC agenesis findings

A
  • abscent cc
  • widely spaced lateral ventricles (steer horn, vertical ventricles)
  • many other associations (MC anomaly seen with other cos malformation?), MC= lipoma.
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11
Q

MC locs intracranial lipomas ass with cc agenesis

A

inter hemispheric (50%), quad cistern (25%)

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12
Q

Iniencephaly

A
  • deficient occipital bone with defect in the cervical region. (Inien=back of head/neck)
  • star gazing
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13
Q

Inien

A

Back of head/neck

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14
Q

True or false-hindbrain preserved in anencephaly

A

True

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15
Q

what’s the most common location encephalocele and most classic association

A

occipital. Chiari III

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16
Q

Rhombencephalosynapsis-what is it, features, classic imaging, associations

A
  • Absent Vermis –> abN fusion cerebellum.
  • Rounded fastigial point, absent primary fissure, small 4th Vt, aqueduct stenosis–> hydrocephalus
  • classic image: transversely oriented/continuity of single-lobed cerebellum
  • association=holoprosencephaly, aqueductal stenosis
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17
Q

Joubert Syndrome-path, features, classic imaging, associations

A
  • AR ciliopathy –> hindbrain malformation –> dysgen rotated vermis/small cerebellum –> MOLAR TOOTH
  • large 4th vt, absent fastigial point and primary fissure, absent pyramidal decussation
  • Ass- retinal dysplasia (50%), MCDK (30%), liver fibrosis (COACH)
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18
Q

molar tooth

A

dysgenesis of bs isthmus –> elongation pontomesencephalic junction –> deepening of interpeduncular fossa –> thick, straight, Long superior cerebellar peduncles

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19
Q

3 consistent findings of “Classic Dandy Walker”

A

Triad:

1) Vermis hypoplasia (usually inf pt). Cerebellar hemispheres displaced anterolateral (shape/volume maintained). Vermis upward rotation
2) torcular lamboid inversion (2/2 elevated tentorium.)
3) dilated 4th Vt

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20
Q

buzzword “torcular lamboid inversion”

what is the lambdoid and torcula?

A

Classic DW

  • torcula-confluence of sinuses
  • lamboid- BL sutures connecting parietal and occipital bones
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21
Q

What defines and comprises the Dandy-Walker continuum?

A
  • Group of pst fossa malformations. In order of severity:
    1) classic DWM
    2) Vermian hypoplasia (VH)
    3) Blake pouch (BP) remnant/cyst
    4) Mega Cisterna magna (MCM)
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22
Q

manifestation classic DW

A
  • OB screen

- intracranial P, macrocephaly (1mo)

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23
Q

classic DW associations

A
  • hydrocephalus (90%)

- CNS malformations (~40%)- cc agen, encephalocele, heterotopia, polymicrogyria, etc.

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24
Q

vermis hypoplasia/variant DWM

A
  • small/hypoplastic vermis with dilation of 4th Vt.
  • vermis superiorly rotated, no torcular lamboid inversion
  • Hydro (+) (25%)
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25
Q

Blake pouch

A
  • sac like cystic protrusion through foramen of magendie into infra/retro cerebellar region
  • cyst communicates with 4th Vt –> upward rotation of vermis, displacement 4th Vt choroid plexus in its superolateral margin (choroid swinging into pouch is classic.)
  • Cyst communicate with 4th Vt, not cistern magna.
  • dilated 4th vt, hydro (+)
  • enlarged tegmentovermian angle (usually 0˚)–> “open 4th vt”
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26
Q

mega cisterna magna

A
  • focal enlargement retrocerebellar css space

- no hydro

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27
Q

Holoprosencephaly (HPE). Forms.

A
  • failure of midline cleavage
  • back –> front
  • forms (increasing sev):
    4) Arhinencephaly
    5) Septo Optic Dysplasia/de Morsier Syndrome
    1) lobar
    2) semi-lobar
    3) alobar
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28
Q

lobar holoprosencephaly

A

anterior aspect variably not cleaved. Absent septum pellucid and partial/normal cc. Normal thalamus

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29
Q

semi-lobar holoprocencephaly

A
  • > 50% frontal lobes fused
  • thalamus fused (partial/complete). occ/temporal horns partially dev.
  • cleft lip/palat
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30
Q

alobar holoncephaly

A
  • No midline cleavage
  • single Vt.
  • cyclops monster face
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31
Q
  • monster cyclops eyes
  • clef lips/palates
  • pyriform aperture stenosis (forum nasal process overgrowth)
  • solitary median maxillary incisor (mega-incisor)
A

buzzwords for HPE spectrum

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32
Q

Arhinencephaly. What is it. Buzzword. Ass.

A
  • minor HPE expression
  • midline olfactory bulbs/tracts abscent
  • “can’t smell”
  • Kallmann syndrome (+ hypogonadism, mental retardation.)
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33
Q

buzzword: can’t smell

A

arhinencephaly

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34
Q

Meckel-Gruber Syndrome triad. Strong ass?

A
  • triad: 1) occipital encephaloceles 2) multiple renal cysts 3) polydactyly
  • strong ass= holoprosencephaly
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35
Q

Septo Optic Dysplasia-AKA. What is it? Strong ass?

A

-AKA De Morsier syndrome

  • minor HPE expression.
  • Absent septum pellucidum + hypoplastic optic structure (optic chiasm, optic nerves are tiny)
  • hypothalamic-pituitary axis abN

-strong ass=schizencephaly

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36
Q

Azygos anterior cerebral artery

A

common trunk of ACA ass w/ SOD and lobar HPE

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37
Q

proliferative neuroepithelium

A

-neuronal-glial stem cells org in periVt region. Proliferate symmetrically –> asymm.

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38
Q

Brain proliferation

A
  • symm (1 sc –> 2 sc’s) –> asymm (1 sc –> 1 sc + 1 differentiated cell)
  • con’t for several cycles –> sc’s apoptose
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39
Q

neuroglial migration

A
  • 6 layers. younger cells move past older ones (inside out)
  • 1st = preplate
  • 2nd = cortical plate (permanent)
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40
Q

neuroglial organization

A

“gyrification”=cortical folding (probably from speed of cortex expansion relative to WM)
-require right #cells (proliferation) & right order (migration)

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41
Q

problems with brain proliferation, migration or organization

A
  • proliferation-too many or not enough cells
  • migration-abN located cells
  • organization- too many or few folds
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42
Q

Hemimegalencephaly

A
  • “hamartomatous overgrowth” of part vs total cerebral hemisphere 2/2 differentiation/migration failure.
  • big side & Vt
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43
Q

Rasmussen’s encephalitis

A
  • atrophic cerebral hemisphere –> ex vacuo dilation Vt.

- AI, <10 yrs

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44
Q

What to consider in setting of 1) mismatched hemisphere size + 2) dilated Vt.

A
  • Hemimegalencephaly, Rasmussen’s encephalitis & Dyke- Davidoff Masson (cerebral heme-atrophy).
  • ass w/ polymicrogyria, pachygyria and heterotopia
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45
Q

Dyke-Davidoff Massons (cerebral hemi-atrophy)

A

V similar to Rasmussen encephalitis + UL skull thickening & expanded sinuses

  • super sag sinus & fissure moved across midline
  • 2/2 in utero/childrehood stroke
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46
Q

Lissencephaly spectrum

A
  • abN migration
    1) Classic type 1
    2) double cortex band heterotopia
    3) lissencephaly “cobblestone” type 2
    4) periVt nodular heterotypic
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47
Q

Lissencephaly Classic Type 1-findings, ass, genetics/epidemiology

A
  • failure to migrate both in # & order –> 4 layers (reverse order) diffuse depot btw Vt & pial surf –> no folding
  • smooth surface, thick cortex, figure 8 brain via shallow vertical Sylvian fissures
  • colpocephaly
  • Autosomal, M=F, ?ass w/ CMV
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48
Q

Double cortex band heterotopia- Findings, ass, genetics/epidemiology

A
  • mildest form lissencephaly
  • disorgz/undermigration –> 2nd layer of cortical neurons deep to more superficial cortex
  • “double cortex”
  • ass with seizures
  • X linked, F>M
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49
Q

Lissencephaly “cobblestone” type 2-findings, MC loc, ass

A
  • overmigration neurons –> additional layer of cortex composed of GM nodules (var size/shape)
  • MC loc adj to sylvian fissure
  • ass w/ congenital musular dystrophy and retinal detachment
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50
Q

Periventricular nodular heterotopia

A
  • failed neuron migration –> GM nodular depo along Vt

- ass w/ seizure do’s

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51
Q

When can you detect DW and DW variant?

A

OB US >18 wks gestation

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52
Q

periVt nodular heteroptopia vs subependymal tubers TS

A

tubers T2 brighter, +/- Ca

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53
Q

Heterotypic grey matter

A

-Heterotopia (HTP): Abnormally located gray matter (GM) due to foreshortened or prolonged neuronal migration
Anywhere from periventricular germinal zone to pia
-Ectopic nodular or ribbon-like foci following GM signal intensity on every MR sequence
-Locations: Periventricular, subcortical, pial
Periventricular nodular HTP (most common)
Band HTP ~ laminar HTP, double cortex
Subcortical nodular HTP (focal or multinodular)
Pial: “Cobblestone” cortex

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54
Q

polymicrogyria- findings, ass

A
  • abN orgz: insult –> obliteration layer 5 –> superficial layers overfold & fuse –> excessive/disorgz gyral convolutions with shallow sulci –> nodular
  • Perisylvian. UL or BL
  • Zika = mcc in Brazil, SA
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55
Q

Schizencephaly- what/findings, types, ass

A

-abN org: cleft of brain lined by GM extending from cortical surface to Vt.

  • closed lip (20%, less severe)-cleft wall closely apposed/fused. +/- nipple of GM at LV
  • open lip (80%, more severe)- cleft walls separated by CSF

-ass- absent SP (70%), focally thinned cc, optic n hypoplasia (30%), epilepsy

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56
Q

hydrancephaly & porencephalic cyst

A
  • vascular insult –> encephalomalacia (necrosis of GM & WM–> cystic degeneration)
  • not lined by GM
  • scoop of ice cream (porencephalic cyst) vs whole pint (hydrancephalic)
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57
Q

Hydrancephaly: Causes, what’s preserved, ass

A

BL ICA occlusion –> massive destruction BL cerebral hemispheres

  • cerebellum, mb and falx preserved
  • *Herpes (classic), toxo, CMV
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58
Q

classic cephalocele location

A

occipital

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59
Q
  • Cephalocele-what is it?

- What are the contents of “meningocele”, “meningoencephalocele”, “encephalocele”, “cystocele”, “myelocele”

A

Cephalocele=umbrella term for herniation of cranial contents through a defect in the skull

  • meningocele= CSF, meninges
  • meningoencephalocele=encephalocele- CSF, meninges, brain
  • cystocele-CSF, meninges, Vt
  • myelocele- SC
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60
Q

Chiari Malformation types

A
  • I-tonsillar herniation +/- cervical syrinx
  • 1.5-1 +2, no nt defects
  • II- (less tonsillar herniation.) Vermian displacement.
  • III- (Chiari II) + occipital encephalocele
  • IV-forget it exists
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61
Q

Classic features chiari II

A

low lying torcula, tectal beaking, hydrocephalus, clival hypoplasia

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62
Q

Chiari I-MOA, clinical syx, ass

A
  • MOA: underdevelopment pst fossa –> overcrowding, downward displacement. (or less commonly s/p trauma)
  • > 5 mm tonsillar herniation below basion/opisthion level
  • ass- cervical syrinx, klippel-feil syndrome. NO nt defect.
  • syx’s- 1) occ ha (worse w/ sneezing) 2) weakness/spasticity/loss of proprioception via P on cord
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63
Q

Klippel-Feil Syndrome

A

congenital spinal fusion

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64
Q

Chiari II-classic findings & associations

A
  • cerebellar herniation (more vermian than tonsillar)
  • low lying torcula
  • tectal beaking
  • clival hypoplasia
  • banana and lemon signs
  • obliteration cisterna magna
  • elongated, effaced 4th Vt
  • only seen in pst w/ nt defect
  • classic associations=lumbar myelomeningocele, cc hypo genesis, spina bifida
  • neurumuscular defects, ex: clubfoot
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65
Q

Chiari III

A
  • Chiari II + occipital encephalocele containing cerebellum and/or bs, occipital lobe, +/- 4th Vt
  • ass: cervical syrinx, tether cord, hydrocephalus, agenesis of cc
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66
Q

medial temporal sclerosis

A

hippocampal volume loss (BL 10%) + gliosis/scar (T2 bright)–> loss of normal morphology/interdigitations

  • MCC partial complex epilepsy
  • add findings (less commonly described)-IL fornix & max body atrophy), CL amygdala enlargement
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67
Q

Craniosynostosis

A
  • Premature fusion of one or several sutures
  • people with Premature fusion have trouble getting dates to the Prom
  • sporadic (85%), syndromic (15%)
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68
Q

Order of fusion of sutures

A

metopic (frontal) (front –> back) –> coronal (lat –> med) –> lamboid (lat –> med) –> sagittal (back –> front)

Me Can Love Smelling!

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69
Q

Which craniosynostosis are idiopathic?

A

sagittal & UL coronal

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70
Q

Names ass w/: sagittal, lamboid (BL), coronal and metopic synostosis

A
  • sagittal= scaphocephaly or dolichocephalic
  • lamboid (BL)=turricephaly
  • coronal =bradycephaly
  • metopic= trigonocephaly
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71
Q

Trigonocephaly

A
  • triangular shape of skull 2/2 metopic (frontal) synostosis preventing transverse growth
  • hypotelorism (eyes close together)
  • medial orbit slanted up
  • single suture ass w/ cognitive disorders (growth restriction of FL)
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72
Q

Brachycephaly

A
  • coronal synostosis (UL > BL)
  • UL-IL flattening, superolateral elevation orbit (harlequin eye), CL protrusion (frontal bossing)
  • BL ass w/ syndromes
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73
Q

Harlequin eye deformity

A

orbital superolateral elevation

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74
Q

anterior plagiocephaly

A

UL coronal synostosis

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75
Q

posterior plagiocephaly

A

UL lambdoid synostosis

-+/-posterior ear movement

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76
Q

deformational plagiocephaly

A

benign positional molding

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77
Q

turricephaly. Ass?

A

BL lambdoid synostosis

  • tall cranium (oxycephaly, acrocephaly)
  • rhomboencephalosynapsis
78
Q

scaphocephaly or dolichocephalic- ass with which dx?

A
  • sagittal synostosis
  • long, narrow head (like an upside down boat)
  • “beaking” of fused suture
  • no hydrocephalus, normal IQ
  • ass w/ Marfans (both tall and skinny!)
79
Q

MC & LC synostosis

A

sag=mc, lamboid=LC

80
Q

MCC abnormal skull shape in infant

A

positional plagiocephaly

81
Q

Additional neuro defect to think of in setting of “BL lamboid craniosynostosis”

A

rhomboencephalosynapsis

82
Q

positional plagiocephaly vs posterior plagiocephaly

A
  • Positional: After birth. IL ant ear mvmt & frontal bossing. Conservative mx.
  • Pst: at birth. IL pst ear mvmt, CL frontal bossing. Surgical.
83
Q

buzzwords associated with NF-1

A
  • abscent/dysplasia of greater sphenoid wing
  • tibial pseudoarthrosis
  • scoliosis
  • lateral thoracic meningocele
  • asterion defect (bone defect adj to lamboid suture)
84
Q

Clover Leaf Skull Syndrome aka Kleeblattschadel

A
  • synostosis of coronal & lamboid sutures (+/- sagittal) –> trilobar skull configuration
  • hydro common
  • ass-thanatophoric dysplasia, apert syndrome, crouzon syndrome
85
Q

Apert’s syndrome

A

brachycephaly + syndactyly (sock hand. typically symmetrically fused hands & feet)

86
Q

Syndromes ass w/ craniosynostoses

A

Apert’s & Crouzon’s

87
Q

Crouzon’s syndrome

A

Brachycephaly, 1st arch structures (maxilla, mandible hypoplasia), hydrocephalus (more than Apert’s), Chiari I (70%!), PDA, ACoA, rhizomelia
*Crouzon’s C’s= coronal sutures, can’t chew, chiari I, CoA, hydroCephalus, Central bone shortening, Crazy eyes (exophthalmos)

88
Q

Skull markings: convolutional, copper, luckenschadel (lacunar)

A
  • convolutional= N pst gyral markings, rapid brain growth (3-7 yo)
  • copper beaten= increased # ant & pst gyral markings, (+) ICP (ex: craniosynostosis, obstructive hydrocephalus)
  • luckenschadel (lacunar)-oval/round/finger shaped defects/craters via defective bone matrix. (chiari II, nt defects.)
89
Q

classic associations of luckedschadel (lacunar) skull markings

A
  • chiari II

- nt defects

90
Q

buzzword: button sequestrum

A

LCH (sequestrum of intact bone)

91
Q

lytic skull lesions in kids

A
  • LCH
  • infection
  • mets (ie: NB)
  • epidermoid cysts
  • leptomeningeal cysts
  • etc
92
Q

parietal foramina

A
  • benign congenital delayed/incomplete ossification parietal bones –> paired, mostly round defects
  • > 5mm-ass with cortical & venous anomalies
  • can get big & cross ml
93
Q

wormian bones: MC suture. Sign of <10, >10, >10 + abscent clavicle

A

-lamboid
<10-idiopathic
>10- osteogenesis imperfecta
-cleidocranial

94
Q

Ddx wormian bones: PORK CHOP

A

PORK CHOP:

  • Pyknodysostosis
  • OI
  • Rickets
  • Kinky Hair Syndrome (Menke’s/abN Cu metabolism)
  • Cleidocranial Dysostosis
  • HypoTh, HypoPh
  • One too many 21st chromosomes (Down’s)
  • Primary Acro-osteolysis (Hajdu-Cheney)
95
Q

congenital misplacement cells from scalp into bony calvarium

A
  • Dermoid & epidermoid of the skull.
  • Bone defect (containing skin (+hair, sweat glands, if dermoid) + sclerotic borders
  • epidermoid: 20-40 yo, parietal (behind ear)
  • dermoid- younger, ML (eyes), ass w/ encephalocele
96
Q

Layers of head

A

-scalp, aponeurosis, periosteum, skull, dura, etc

97
Q

subgaleal hemorrhage

A
  • btw aponeurosis & periosteum
  • cause: vacuum extraction
  • 12-72 hrs; rapid, life threatening blood loss
98
Q

cephalohematoma

A
  • under periosteum (limited by sutures-smaller than smbgaleal hemorrhage)
  • resolves conservatively ~ wks
  • myositis ossificans, superinfection (E. coli)–> OM
  • cause: instrument or vacuum extraction
99
Q

caput succedaneum

A
  • subq hemorrhage above aponeurosis
  • cause: prolonged deliver
  • resolves conservatively ~ d’s
100
Q

mcc morbidity/mortality in children

A

head trauma

101
Q

3 types of skull fractures in children

A

1) diastatic 2) depressed 3) ping-pong

102
Q

Diastatic skull fx

A
  • involves sutures (lamboid)

- >1mm asymmetry

103
Q

Depressed skull fx

A
  • inward displacement (equal to or greater than thickness of skull). High morbidity
  • compound= scalp laceration
  • penetrating=dural tear
104
Q

ping-pong skull fx

A
  • greenstick/buckle fracture (smooth inward deformity)

- favorable outcome

105
Q

indications for surgery in setting of depressed fx

A
  • > 5 mm (ass w/ dural tear)
  • epidural bleed
  • superinfection
  • cosmetic
  • functional (frontal sinus involvement –> mucocele)
106
Q

Fracture vs suture

A
  • fracture >3mm (suture <2mm)
  • wide center (suture equal width)
  • straight line, angular turn (suture squiggly w/ curves)
  • “darker” (suture “lighter”)
107
Q

leptomeningeal cyst

A

fracture + torn dura –> herniation leptomeninges –> mo’s: pulsations wide site) –> epilepsy from manipulation

108
Q

sinus pericranii

A

skull defect 2/2 low flow vascular malformation (superior sagittal - extra cranial venous structure conn via emissary veins)
-US or MRI to show vascular comp. CT for skull defect

109
Q

extra-axial fluid collection most associated with NAT

A

subdural hematoma

110
Q

retroclival hematoma-epidural vs SDH

A
  • Epidural-below tectorial membrane

- SDH=above tectorial membrane

111
Q

BESSI

A

Benign Enlargement of Subarachnoid Space in Infancy (>5 mm, 2/2 immature villi). Mainly ant asp.

  • MCC macrocephaly. (+) SDH
  • 2/3 mo –> resolves 2 yrs (conservative)
  • M > F
  • positive cortical v sign
112
Q

Positive cortical v sign

A

BESSI or cortical atrophy

113
Q

How long does it take PVL cysts to develop?

A

~2 wks

114
Q

PVL associated with…

A

50% develop cerebral palsy

115
Q

PVL severity MC in full or preterm?

A

Full

116
Q

Who gets screened for GMH?

A
  • premature (<32 wks)
  • <1500 g
  • premature + leathery, seizures, decreased hematocrit, not looking well
117
Q

Pyriform aperture & posterior choanae

A
  • Pyriform aperture-ant aspect nasal cae, btw maxillary spines
  • pst choanae- pst asp nasal cavity
118
Q

Pyriform aperture stenosis

A
  • aperture <8mm,
  • too big or fused. Maxillary spines inward bow. crossed by ST
  • ass with ML developmental problem: holoprosencephaly, pituitary dysfunction, central megaincisior (75%, next step: image brain)
119
Q

Choanal atresia

A
  • MCC nasal obstruction
  • UL > BL
  • cyclical cyanosis improves w/ crying. Can’t pass NG tube, rest sdistress while feeding, rhinorrhea
  • EM (aw compromise)
  • syndromic associations: CHARGE, DiGeorge, Treater Collins, Fetal EtOH
120
Q

CHARGE

A

Colomboma, Heart Defect, Atresia (Choanal), Retarded Growth, GU, Ear

121
Q

Dermal tract sinus

A

Failure of dural tract to close–> sinus or cyst (dermoid/epidermoid)
*Prenasal dural communication requires communication through foramen cecum

122
Q

Nasal glioma

A

Brain in wrong place. Not neoplastic, don’t enhance.

123
Q

variations in size if baby cry/compress jugular: dermal sinus dermoid/epidermoid, nasal glioma, encephalocele

A
  • Cysts, glioma-no

- Encephalocele-yes

124
Q

nasal encephalocele

A
  • via dural diverticulum
  • NG displaced laterally
  • ML ass: facial cleft, callosal issues, inter hemispheric lipoma.
  • don’t biopsy (csf leak, meningitis)
125
Q

NG tube displacement lat vs med

A

lat=encephalocele

med=dacrocystocele

126
Q

Dacryocystocole

A
  • obstructed nasolacrimal duct

- 2nd MCC neonatal obstruction (after choanal atresia)

127
Q

treacher collins syndrome

A
  • absent zygomatic arches/malar bones, narrow/overprojected maxilla, mandible hypoplasia (concave curve of horz ramus)–> retruded chin
  • MO-1st & 2nd brachial arch problem
  • problem to intubate
128
Q

pyramidal thyroid lobe

A

-persistent inf TGD (variable on imaging)

129
Q

Guthrie test

A

screening for low TH at birth

130
Q

purpose of Neck US in midline mass

A

confirm N thyroid

131
Q

next step: midline cyst in neck of kid

A

US +/- Tc MIBI or I-123

132
Q

cancer ass w/ thyroglossal duct cyst

A

papillary (enhancing nodule in cyst)

133
Q

MC location ectopic thyroid tissue

A

base of tongue

134
Q

sac of marbles

A

midline sublingual/submandibular space dermoid (lobules of fat w/i fluid)

135
Q

MC branchial cleft cyst

A

2nd (95%)

  • locs: angle of mandible, pst lat to SMG, ant to SCM, ext btw ICA, ECA (notch sign, pathognomonic)
  • vs necrotic level II LN: thyroid (radiation exposure), NPP (HPV) young/adol
136
Q

notch sign

A

extension of 2nd branchial cleft cyst btw ICA & ECA, pathognomonic

137
Q

mcc orgm septic emboli from IJV

A

fusobacterium necrophorum

138
Q

phlebectasia

A

idiopathic dilated jugular vein, worse with Valsalva maneuver

139
Q

enhancement patterns venous vs lymphatic malformations

A

venous-cystic spaces

lymphatic-septae

140
Q

MC congenital lesions in H/N

A

hemangioma (~6 mo–> grow/peak –> involution 6/10 yrs)

141
Q

Mx hemangioma

A

propranolol (if syx, large/rapidly growing)

142
Q

infantile vs congenital hemangioma

A

cong present at birth and may not involute

143
Q

hemangioma associations

A

PHACES Syndrome

144
Q

cystic hygroma aka

A

lymphangioma

145
Q

Ass lymphangioma

A

-in order: turners (baby can’t TURN it’s head), DS, ACoA (mc CV), fetal hydrops

146
Q

lymphangioma vs hemangioma

A

both T2 bright, hemangioma enhance

147
Q

significance of septations in lymphangioma

A

worse outcome with separations

148
Q

fibromatosis coli

A
  • rare form of infantile fibromatosis that occurs within the sternocleidomastoid muscle.
  • path: birth trauma leading to hemorrhage as a predisposing factor eventually results in fibrosis or malposition (e.g. breech) in the womb.
  • On clinical examination, there may be a growing, hard neck mass 7.
  • enlarged SCM
  • mcc neck mass in infancy. 4 wk old
  • mx=PT, botox
149
Q

mc extra-ocular orbital malignancy in children

A

rhabdomyosarcoma (dermoid= mc benign orbital mass)

-painless proptosis

150
Q

PHACES

A
  • Posterior fossa (DW)
  • Hemangiomas
  • Arterial anomalies
  • Coarctation of aorta, Cardiac defects
  • Eye abN
  • Subglottic hemangioma/Sternal cleft/Supraumbilical raphe
151
Q

central echo complex

A

interface btw ant median tissue and myelinated ventral WM commissure. Interfaces btw things w/ large impedance diff = echogenic

152
Q

What is central canal filled with in newborn?

A

glial fibrils

153
Q

what grows faster: canal or cord?

A

Canal

154
Q

low lying cord/tethered cord-what, types, img

A
  • fixed attachment –> cord stretching via canal growing faster than cord –> ischemia
  • 1˚ vs 2˚ (ass mass)
  • conus 2mm
155
Q

tethered cord associations

A
  • anal atresia

- spina bifida

156
Q

who gets screened for tethered cord?

A
  • anal atresia
  • spina bifida high dimples (Above gluteal crease) via risk intraspinal extension
  • low dimples below creased may bc pilonidal sinuses but never extend intra-spinally, ie: no screening required
157
Q

which is ass w/ chiari malformation?: meningomyelocele or lipomyelomeningocele?

A

meningomyelocele

158
Q

terminal ventricle (ventriculus terminalis)

A
  • distal cord doesn’t involute –> small cyst (~4mm)

- syx if large

159
Q

spondylolysis (pars interarticularis defect)-what, pres, where

A
  • fatigue or stress in childhood
  • back pain in adolescent athlete=classic hx
  • syx=25%
  • 90% L5 –> L4 (+degen change at L4/L5 vs L5/S1)
  • CL sclerotic pedicle (wiggle stress)
160
Q

spondylolisthesis

A

forward slippage

161
Q

canal vs NF foramina in pars defect + anterolisthesis vs purely degenerative spondylolisthesis

A

PD: NF stenosis + spinal canal widening (unless severe)

-purely degen spondylolisthese (not much slippage)-canal stenosis with less severe effects on NF

162
Q

spinal dysraphism

A

group of malformations affecting spine and/or surrounding structures in dorsal of embryo

163
Q

Spinal dysraphism types

A

open
closed w/ subq mass
close w/o subq mass

164
Q

Open spinal dysraphism/”spina bifida aperta”-what, types (MC), ass, mx

A
  • failed closure 1˚ neural tube –> neural placode exposed through midline defect of skin.
  • myelocele, myelomeningocele (98%), hemimyelomeningocele
  • TETHERED CORD
  • surgical EM (placode exposed)
165
Q

myelocele

A

cord protrudes, no expansion SAS,

-skin/subq discon’t but defect covered w/ flushed with skin

166
Q

meningomyelocele

A

pst defect w/ cord & SAS, above skin

-chiaria II

167
Q

hemimyelomeningocele

A
  • v rare

- splitting sc at same level of dysraphism

168
Q

closed spinal dysraphism w/ subq mass

A
  • meningocele
  • lipomyelocele/lipomyelomeningocele
  • terminal myelocystocele
169
Q

closed spinal dysraphism/ spina bifida occulta

A

pst defect covered by skin

170
Q

meningocele-where

A

csf herniation (sac) the pst defect. NO neural tissue

  • lumbosacral > cervical
  • ant (pre sacral)
171
Q

lipomyelocele/lipomyelomeningocele-what, ass

A
  • lipoma with defect
  • 100% tethered cord
  • lipomyeloschisis=interface in spinal canal
172
Q

terminal myelocystocele-what, where, key component

A

herniation terminal syrinx (“hydrosyringomyelic cavity”) into meningocele

  • no comm w/ rostral sac
  • LS
  • can be non terminal (cervical, thoracic)
173
Q

closed spinal dysraphisms w/o subq mass

A
  • intradural lipoma
  • fibrolipoma of FT
  • tight filum terminale
  • dermal sinus
174
Q

intradural lipoma-mc location

A
  • T spine MC

- +/- pst element defect

175
Q

fibrolipoma of FT

A

fatty filum

-linear T1+ in filum. no other changes to FT or cord.

176
Q

tight filum terminale

A
  • FT >2mm, low lying conus
  • +/-terminal lipoma
  • +”tethered cord syndrome” (via stretching)
177
Q

tethered cord syndrome

A

clinical syx’s: LBP, leg par, neurogenic bladder

178
Q

dermal sinus

A
  • epithelial tract from skin –> deep ST (sometimes spinal canal, dermoid or lipoma)
  • T1(-)-next to bright stuff!
179
Q

diastematomyelia-what, where, classification

A

Cord split sagittally from T9-S1.

  • N above and below
  • classification: 1) fibrous or osseous split 2) +/- 2 thecal sacs –> each has own canal w/ dorsal/ventral horns
180
Q

caudal regression-what, ass

A

blunted high terminating cord, agenesis LS, coccyx

  • VACTERL, currarino triad
  • maternal DM
181
Q

currarino triad

A

1) ANTERIOR sacral meningocele
2) anorectal malformation
3) sacrococcygeal osseous defect (scimitar sacrum)

182
Q

most common euro manifestation of nf-1? How/when do they app? Where?

A
  • NF-1 spots

- high T2 in GP, cerebellum, bs, int capsule, selenium and thalami

183
Q

mc neuro manifestation of tuberous sclerosis

A

tuber: subependymal hamartomatous lesion, MC in LVs
- signal chars variable/rel to age
- older children: iso to GM, Ca
- usually don’t enhance

184
Q

giant cell tumor

A

Tuber near foramen of Monro that rapidly enlarges

185
Q

neuro manifestations of tuberous sclerosis

A
  • tubers
  • GC tumor
  • T2+ in WM 2/2 abN glial cells
186
Q

Triad TS

A

1) seizure
2) MR
3) adenoma sebecum

187
Q

Where does sturge weber manifest?

A

Skin (port wine nevus)
Leptomeninges
Brain

188
Q

pathophys of brain injury in sturge weber

A

vascular malform –> altered bf to brain –> chronic ischemic injury –> atrophy, serpiginous Ca, abN enh
*cranium often thickened adj to abN brain

189
Q

Pathophy of subacute sclerosis pancencephalitis

A

reactivation of latent measles infection

*dx of childhood

190
Q

pathophys of herpes simplex-1 virus

A

necrotizing meningoencephalitis

1) 2˚ activation of latent infection –> migration via branches of trigeminal n –> UL or BL TLs –> hemorrhage
- 2) systemic inf during birth –> any pt of brain