Peds 2 Flashcards
AIS:
What Adams forward bend warrants referral
What is most common curve: right vs left, C/T/L
> 7deg
R thoracic = common
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
When does peak growth velocity happen
Before menarche
Before Risser 1
Why do we operate on AIS curves?
T curves > 50deg
L curves > 30 deg
Continue to progress after maturity 1-2deg / yr
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
Who do you do ant post fusion for in AIS
Young to prevent crankshaft
+/- curves > 75deg (controversial)
2 potential causes for persistent pain after AIS fusion + treatment
Pseudarthrosis - revision fusion
Delayed infection - think P.Acnes
- If fused, ROH + abx
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
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
Congenital scoli
Worst prognosis?
Worst
- Unilat bar, CL hemivert
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
Why early surgery for congenital kyphosis
Often progressive and can cause neurologic compromise
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
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
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)
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
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
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
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
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
3 packaging disorders usually grouped together
DDH
Torticollis
Metatarsus adductus
RF DDH
Breech = most impt
1st born, female, +fam history
What is ortolani vs barlow signs
Ortolani = reducible with abduction
Barlow = dislocatable with adduction and posterior translation
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)
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
