Peds 2 Flashcards by Lo Kola

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

> 7deg
R thoracic = common

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Name indications for MRI in AIS

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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When does peak growth velocity happen

Before menarche
Before Risser 1

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Why do we operate on AIS curves?

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

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What are criteria for AIS bracing
What is operative criteria AIS

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

> 45deg = operative

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Who do you do ant post fusion for in AIS

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

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2 potential causes for persistent pain after AIS fusion + treatment

Pseudarthrosis - revision fusion

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

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

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

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

Measures rotation

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

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Congenital scoli
Worst prognosis?

Worst
- Unilat bar, CL hemivert

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What is congenital scoliosis thoracic insufficiency?

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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Why early surgery for congenital kyphosis

Often progressive and can cause neurologic compromise

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NF
- Inheritance
- 2 types of scoli
- Trt

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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Define spondylolysis
Associated PE findings
Most sensitive test
Trt

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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Spondylolisthesis
- Most common level
- Trt

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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Diagnostic criteria Scheuermanns
Trt

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

Failure of segmentation

Low hairline
Web neck
Limited C spine ROM

Assoc: cong scoli, Sprengel (undescended scapula)

Trt - avoid collision sports

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2 peds diagnoses that can have AA instability
Indications for fusion

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

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

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

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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3 packaging disorders usually grouped together

DDH
Torticollis
Metatarsus adductus

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RF DDH

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

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What is ortolani vs barlow signs

Ortolani = reducible with abduction

Barlow = dislocatable with adduction and posterior translation

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

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

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

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

What is Pavlik disease

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

Treat DDH > 6mo - walking age

Arthrogram, CR, bracing

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

Inverted limbus aka labrum precursor

Considerations for treating DDH after kid is walking

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

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

AVN prox fem epiphysis Poor prognosis: 1. >6yo 2. Total head involvement Good outcome: more spherical fem head, good ROM

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

Hypertrophic zone Neck goes ant + ER Leptin

What is dominant BS to femoral head?

Lat epiphyseal art -> MFCa Br off profunda femoris

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

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

Indications for bilateral pinning SCFE

Endocrine Open tri-radiate (young)

What gene is implicated in femoral deficiency

SHH

Algorithm for treatment of congenital knee dislocation

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

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

PFD Cleidocranial dysplasia Apert syndrome

Cleidocranial dysplasia: Inheritance Type of dwarfism Gene defect Bone problem

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

Presentation cleidocranial dysplasia

No clavicles! Coxa vara, +/- valgus osteotomy

Apert Syndrome: Inheritance Gene mutation Presentation

AD FGFR2 gene Rosebud hand - syndactyly hand/feet

Multiple epiphyseal dysplasia Inheritance Dwarfism type Gene mutation

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

MED presentation

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

Differentiate spondyloepiphyseal dysplasia from MED - Gene - Presentation

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

Diastrophic dysplasia - Gene mutation + chromosome - Inheritance

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

Presentation of diastrophic dysplasia

Hitch hiker thumb Cauliflower ear C spine kyphosis

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

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

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

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

San Filippos syndrome: Inheritance What accumulates

AS Heparan sulfate

Morquio's syndrome Inheritance What accumulates Presentation

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

2 types of osteochondromas Gene mutation

Sessile Pedunculated EXT gene

Rickets: Pathophys Part of physis affected XR findings

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

Vit D resistant rickets Inheritance Pathophys Labs Trt

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

Pathophys renal rickets

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

OI: Gene defect Amino acid substitution Resulting error

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

Medical treatment OI

IV pamidronate = clast apoptosis

Scurvy: Cause XR finding

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

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

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

NF1: - Chr - Presentations

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

Marfans: Chromosome Inheritance Cardiac issue Eye issue

Ch 15 - fibrillin AD Mitral valve prolapse Sup lens dislocation

Larsen: Protein mutation Spine presentation

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

3 diseases that had radial deficiency

Holt Oram TAR Fanconi anemia

Holt Oram Inheritance Presentation

AD Cardiac + radial deficiency

TAR Inheritance Presentation

AR TCP/anemia Radius absent, thumb present

Fanconi anemia Inheritance Presentation

AR Pancytopenia Radial deficiency

What determines the treatment algorithm for thumb hypoplasia?

CMC stability Def CMC joint - think pollicization

Peds trigger thumb - Presentation - Trt

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

2 poor prognostic factors for birth plexopathy

No biceps after 6mo Horner syndrome

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Peds 2 Flashcards

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