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Millers > Peds 2 > Flashcards

Peds 2 Flashcards

1
Q

AIS:
What Adams forward bend warrants referral
What is most common curve: right vs left, C/T/L

A

> 7deg
R thoracic = common

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2
Q

Name indications for MRI in AIS

A

L thoracic curve
Pain
Kyphosis (AIS hypokyphoitc)
Rapid curve progression
Neuro signs - hyperreflexia, asymm abdominal reflexes
Congenital anomalies / other - cavovarus feet

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3
Q

When does peak growth velocity happen

A

Before menarche
Before Risser 1

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4
Q

Why do we operate on AIS curves?

A

T curves > 50deg
L curves > 30 deg
Continue to progress after maturity 1-2deg / yr

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5
Q

What are criteria for AIS bracing
What is operative criteria AIS

A

25-40deg = brace
Flexible curves aka Risser 0/1/2
Goal to stop progression not correct deformity

> 45deg = operative

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6
Q

Who do you do ant post fusion for in AIS

A

Young to prevent crankshaft
+/- curves > 75deg (controversial)

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7
Q

2 potential causes for persistent pain after AIS fusion + treatment

A

Pseudarthrosis - revision fusion

Delayed infection - think P.Acnes
- If fused, ROH + abx

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8
Q

Early onset scoli
- Age of onset
- Curve location/type
- What rib vertebral angle difference has a high risk for progression

A

EOS
<10yo
L thoracic common
RVAD >20 = high risk of progression

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9
Q

What is rib vertebra angle difference measuring?
What values are benchmarks for obs vs brace

A

Measures rotation

RVAD = (concave - convex) at apical vertebra
<20 = obs
>20 = brace

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10
Q

Congenital scoli
Worst prognosis?

A

Worst
- Unilat bar, CL hemivert

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11
Q

What is congenital scoliosis thoracic insufficiency?

A

Thoracic wall motion limited
- Congenital rib fusions
- Thoracic scoli fusion at a young age

Alveoli continue to form until 8yo so avoid T fusion as long as you can
Osteotomize ribs if needed

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12
Q

Why early surgery for congenital kyphosis

A

Often progressive and can cause neurologic compromise

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13
Q

NF
- Inheritance
- 2 types of scoli
- Trt

A

AD
1. Nondystrophic - idiopathic like
2. Dystrophic
XR: vert scalloping, short seg sharp curves
Trt
- Pre op MRI
- Bracing less effective
- ASF/PSF bc PSF alone high rate of pseudoarthrosis

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14
Q

Define spondylolysis
Associated PE findings
Most sensitive test
Trt

A

Pars frx wo displacement (vs listhesis)
Assoc HS tightness
Sensitive: SPECT (single photon emission CT) - when XR are normal
Trt
1. Rest/brace - stop repetitive ext activity
2. Repair vs fusion

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15
Q

Spondylolisthesis
- Most common level
- Trt

A

L5
Indications: >50% slip, progression, fail non op
Fusion:
Low grade fuse in situ
Higher grade, reduce to improve slip
Likely need anterior col support (TLIF +/- PSIF)

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16
Q

Diagnostic criteria Scheuermanns
Trt

A

3 consecutive vert with >5deg ant wedging
Other:
- Disc narrowing
- End plate irregularity (Schmorl nodes = disc herniating into endplate)

Trt
Surg > nonop - PSF

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17
Q

Klippel Feil
- Cause
- Presenting triad
- Associated conditions (2)
- Trt

A

Failure of segmentation

  1. Low hairline
  2. Web neck
  3. Limited C spine ROM

Assoc: cong scoli, Sprengel (undescended scapula)

Trt - avoid collision sports

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18
Q

2 peds diagnoses that can have AA instability
Indications for fusion

A

Downs - ADI >10mm or sx
JRA - fuse if >5mm motion on flex/ex XR

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19
Q

2 peds dx that can have rotatory AA instability
Painful or painless
How does the head rotate in relation to the subluxed facet
Trt

A

Painful (vs CMT painless)

C1-2 facet sublux/dislocation
- Rotate chin to CL side of the facet sublux

Grisel disease = retropharyngeal irritation then AA instab
Torticollis

Trt:
Sx < 1wk - observe
Sx >1wk - traction (halo) -> bracing

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20
Q

Cong muscular torticollis
- Painful or painless
- Which side does head lean towards
- Associated conditions x2
- Trt

A

PAINLESS

Head leans towards the side where SCM is contracted (duh..) - chin rotates away
Should feel palpable mass = SCM

Associations:
DDH - packaging disorders
CL skull flattening (plagiocephaly)

Trt = stretching, PT

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21
Q

3 packaging disorders usually grouped together

A

DDH
Torticollis
Metatarsus adductus

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22
Q

RF DDH

A

Breech = most impt
1st born, female, +fam history

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23
Q

What is ortolani vs barlow signs

A

Ortolani = reducible with abduction

Barlow = dislocatable with adduction and posterior translation

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24
Q

When does the prox fem oss nucleus appear?
XR findings for DDH

A

6mo
UP + OUT
Above horizontal Hilgenreiner (through triradiates)
Lateral to Perkins (vertical at edge of tab)

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25
Q

Trt DDH <6mo
When/how do you assess for reduction?

A

<6mo = Pavlik
Put in Pavlik, US at 3wks, if not reduced get arthrogram + CR -> abduction brace

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26
Q

What is Pavlik disease

A

Posterior tab def 2/2 wearing a Pavlik w/o reduction

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27
Q

Treat DDH > 6mo - walking age

A

Arthrogram, CR, bracing

28
Q

What does a sup-lat filling defect on hip arthrogram indicate?

A

Inverted limbus aka labrum precursor

29
Q

Considerations for treating DDH after kid is walking

A

Usually need open reduction since been out for so long
Possible femoral osteotomy +/- shortening to keep reduced
+/- pevlic osteotomy

Consider leaving out
Bilateral > 6yo
Unilat > 8yo

30
Q

What is Legg Calve Perthes
2 factors for poor prognosis
2 factors that characterize a good outcome

A

AVN prox fem epiphysis

Poor prognosis:
1. >6yo
2. Total head involvement

Good outcome: more spherical fem head, good ROM

31
Q

SCFE
Where in the growth plate does the slip happen
What direction does the neck displace
What hormone is elevated

A

Hypertrophic zone
Neck goes ant + ER
Leptin

32
Q

What is dominant BS to femoral head?

A

Lat epiphyseal art -> MFCa
Br off profunda femoris

33
Q

What is a stable vs unstable SCFE? What does this correlate with?

A

Stable: weight bearing, AVN risk <10%
Unstable: can’t walk (even w/ crutches), AVN risk > 25%

34
Q

Indications for bilateral pinning SCFE

A

Endocrine
Open tri-radiate (young)

35
Q

What gene is implicated in femoral deficiency

A

SHH

36
Q

Algorithm for treatment of congenital knee dislocation

A

If ipsi hip dislocation, treat the knee first so can flex the knee for the Pavlik

37
Q

Name 3 diseases that affect 1ary center of ossification (aka present at birth)

A

PFD
Cleidocranial dysplasia
Apert syndrome

38
Q

Cleidocranial dysplasia:
Inheritance
Type of dwarfism
Gene defect
Bone problem

A

AD
Proportionate dwarfism
CBFA1 gene = chromosome 6
Bad TF for osteoclastin
Osteoblasts don’t differentiate properly
Bad intramembranous ossification

39
Q

Presentation cleidocranial dysplasia

A

No clavicles!
Coxa vara, +/- valgus osteotomy

40
Q

Apert Syndrome:
Inheritance
Gene mutation
Presentation

A

AD
FGFR2 gene
Rosebud hand - syndactyly hand/feet

41
Q

Multiple epiphyseal dysplasia
Inheritance
Dwarfism type
Gene mutation

A

AD
Disproportionate dwarfism = short limbs, normal trunk
- Problem is at the epiphysis!
- Normal spine
COMP gene - reduces type 2 col

42
Q

MED presentation

A

Angular knee deformities - valgus
Premature OA - early arthroplasty
- Fucked up epiphysis

43
Q

Differentiate spondyloepiphyseal dysplasia from MED
- Gene
- Presentation

A

COL21 mutation - chr 12
- Also produced abnormal type 2 col
Proportionate dwarfism - spine involved
- Beware odontoid hypoplasia

44
Q

Diastrophic dysplasia
- Gene mutation + chromosome
- Inheritance

A

AR
DTD gene, chr 5
- Encodes a sulfate transporter gene
- Undersulfate PGs

45
Q

Presentation of diastrophic dysplasia

A

Hitch hiker thumb
Cauliflower ear
C spine kyphosis

46
Q

Achondroplasia:
Inheritance
Mutated gene
What part of bone growth is affected

A

AD
FGFR3 - activating mutation
Endochondral growth affected
Failure in the proliferative zone
Quantitative defect

47
Q

Hurlers syndrome:
Inheritance
What enzyme deficiency - what accumulates as a result

A

AR
Def alpha-L-iduronidase
Dermatan sulfate accumulates in CNS = mental retardation

48
Q

San Filippos syndrome:
Inheritance
What accumulates

A

AS
Heparan sulfate

49
Q

Morquio’s syndrome
Inheritance
What accumulates
Presentation

A

AR
Keratan sulfate
Cloudy cornea
Normal intellect (different than other muccopolysacc)
Coxa vara
Bullet shaped metacarpals
Odontoid hypoplasia - C spine instab -> decompress/fuse

50
Q

2 types of osteochondromas
Gene mutation

A

Sessile
Pedunculated
EXT gene

51
Q

Rickets:
Pathophys
Part of physis affected
XR findings

A

Can’t mineralize chondroid - build up chondrobasts/chondroid in the hypertrophic zone (enlarges)
Can’t mineralize so poorly defined zone of Ca2+
XRs: physeal cupping + widening

52
Q

Vit D resistant rickets
Inheritance
Pathophys
Labs
Trt

A

XL dom
Can’t reabsorb PO4 in kidneys - renal wasting
Ca normal, PO4 low, alk phos high
Trt LE deformities with osteotomies

53
Q

Pathophys renal rickets

A

Poor kidney fxn = can’t convert vit D
PO4 accumulates - chelates Ca
Look Ca low = stim parathyroid - 2ary hyperPTH

54
Q

OI:
Gene defect
Amino acid substitution
Resulting error

A

COL1A2 gene
Glycine substitution
Abnormal type 1 col
Blasts can’t make enough osteoid

55
Q

Medical treatment OI

A

IV pamidronate = clast apoptosis

56
Q

Scurvy:
Cause
XR finding

A

Poor vit C intake
Can’t convert proline to hydroxyproline
XR: lucent metaphyseal line

57
Q

Osteopetrosis:
Inheritance
Chromosome
Protein affected
Presentation
Trt: medical vs frx

A

AR
Ch 11
Carbonic anhydrase def - don’t remodel bone
Lose medullary canal = anemia
Blindness = optic n encroachment
Trt
- Medical = bone marrow transplant
- Frx = can’t to IM fixation bc canal is gone

58
Q

NF1:
- Chr
- Presentations

A

Chr 17, NF1 gene
Cafe au lait spots
Hemihypertrophy
Lische nodules (iris hamartomas)
AL tibial bowing
Scoli

59
Q

Marfans:
Chromosome
Inheritance
Cardiac issue
Eye issue

A

Ch 15 - fibrillin
AD
Mitral valve prolapse
Sup lens dislocation

60
Q

Larsen:
Protein mutation
Spine presentation

A

Filamin B mutation
C spine kyphosis - watch for progression/myelopathy

61
Q

3 diseases that had radial deficiency

A

Holt Oram
TAR
Fanconi anemia

62
Q

Holt Oram
Inheritance
Presentation

A

AD
Cardiac + radial deficiency

63
Q

TAR
Inheritance
Presentation

A

AR
TCP/anemia
Radius absent, thumb present

64
Q

Fanconi anemia
Inheritance
Presentation

A

AR
Pancytopenia
Radial deficiency

65
Q

What determines the treatment algorithm for thumb hypoplasia?

A

CMC stability
Def CMC joint - think pollicization

66
Q

Peds trigger thumb
- Presentation
- Trt

A

FIXED flexion deformity
<1yo - 30% can resolve spont
Otherwise A1 release

67
Q

2 poor prognostic factors for birth plexopathy

A

No biceps after 6mo
Horner syndrome

Millers (29 decks)

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