Paeds - Scott Flashcards
(140 cards)
1
Q
RFs for brachial plexus birth palsy
A
Macrosomia
Difficult presentation
Shoulder dystocia
Forceps/instrumented delivery
Breech position
Prolonged labour
2
Q
Blauth classification of thumb hypoplasia
A
1 smaller thumb normal structures
2 some thenar muscles missing (do opponensplasty)
MCP UCL deficiency (reefing)
web space contracture (z plasty)
3A same as 2 with some tendon deficiencies (do transfers)
3B **Absent CMC joint** Do amp/pollicization
4 floating thumb (amp/pollicization)
5 absent thumb (pollicization)
3
Q
Order of ossification of carpal bones
A
Capitate, hamate, triquetrum, lunate, scaphoid, trapezium, trapezoid, pisiform
On PA of wrist, proceed clockwise starting at capitate (except pisiform last. just remember that)
4
Q
Order of ossification of tarsal bones
A
Calcaneus, talus, cuboid, cuneiforms
5
Q
Algorithm for paediatric proximal humerus treatment
A
In general:
Nonop if age 7 or less. Only reduce if >75degrees and polytrauma/skin tenting.
Consider reduction/pinning:
8-11y/o with angulation >60
Operative indications:
Adolescent (12 and over) with fracture >50% displaced and angled >45degrees; open fracture; Vascular injury; intraarticular fracture
6
Q
Supracondylar fractures
Medial displacement - pronation or supination?
Lateral displacement - pronation or supination?
A
Medial - pronate to tighten the intact medial periosteal hinge.
Lateral - supinate to tighten the intact lateral periosteal hinge.
7
Q
Milch classification of lateral condyle?
What salter harris classification of each?
A
type 1 lateral to trochlear groove (SH4)
type 2 into trochlear groove (SH2)
8
Q
Jacob classification of lateral condyle fractures
A
1 <2mm
- 2-4mm (fix)
- >4mm (fix)
9
Q
3 A’s of paediatric compartment syndrome
A
Agitation
Anxiety
increasing Analgesia requirements
10
Q
Criteria for acceptable reduction of BBFF or DR fractures in kids
A
Acceptable alignment (radial/ulnar shaft)
<9 years old: angulation <15, rotation <45
>9 years old: angulation <10, rotation <30
Complete displacement allowed if <10years
Consider ORIF if >13years old
Acceptable alignment (distal radius/ulna)
<9 years old: dorsal angulation <30
>9 years old: dorsal angulation <20
11
Q
Difference between TENs and plate ORIF for paeds BBFF.
A
TENs had shorter OR time and less blood loss
12
Q
Name the procedure to reconstruct annular ligament in missed monteggia fractures
A
Bell-Tawse procedure
Uses a strip of triceps fascia/tendon to reconstruct annular ligament
13
Q
Operative indications for radial neck fracture
A
Residual angulation >30 (some say >45) after closed reduction
Displacement >3-4mm
<45 of pronation/supination
14
Q
Name 3 closed and 2 percutaneous reduction techniques for radial neck
A
Closed
Patterson: Forearm extended and supinated. Varus stress and thumb pressure over radial head
Israeli: Elbow at 90. Pronate the supinated forearm with thumb direction over radial head
Elastic bandage: Wrap from wrist proximally, will often see spontaneous reduction.
Open
Perc pin
Metaizeau technique - retrograde elastic nail up into head, then rotate the pin/nail.
15
Q
Order of distal tibial physeal closure
A
- Central
- Posterior
- Medial
- Lateral (anterolateral)
16
Q
Triplane fracture - describe # pattern
A
Sagittal in the epiphysis
Axial in the physis
Coronal in the metaphysis
17
Q
Possible etiology of cubitus varus deformity post-supracondylar?
A
Malreduction with fragment medialized, internally rotated and extended.
18
Q
Subclassification of Gartland 3 sch#
A
3A: posteromedial
3B: posterolateral
19
Q
Monteggia reduction techniques
A
Bado Classification
- Reduce with flexion and supination. Cast at 110degress of flexion in forearm supination
(Hyperpronation injury in elbow extension)
- Reduce in extension with pronation. Cast in extension. (Hypersupination injury)
- Reduce in extension with supination and valgus stress. Cast in flexion (110) and supination.
- Usually surgery. If nonop, then cast in flexion (110) and supination.
20
Q
Volume of fluid resusc bolus in paeds
A
20cc/kg
21
Q
Reason for MRI in congenital scoliosis
A
Rule out:
Syrinx, tethered cord, intradural lipoma, diastematomyelia, chiari malformation
Recall: chiari type 1 herniation of cerebellar tonsils. See cervical syrinx
Chiari 2: more severe cerebellar herniation, lumbar syrinx.
22
Q
Indications for hemi-vertebrectomy in congenital scoli
A
Age 5 or less
Curve >40
Progressive curve
spinal imbalance
hemivert in TL junction or lower
23
Q
Indications for hemi-epiphysiodesis in congenital scoli
A
Pt 5 or less
Curve <40
Balanced spine
Growth potential on concave side (intact plates)
Fully segmented hemivertebrae or bar
24
Q
Place the following in order of risk of progression (highes to lowest) of congenital scoli curve:
Wedge
Hemivertebrae
Block
Unilateral bar
Double hemivertebrae
Unilateral bar with contralateral hemivertebrae
A
Unilateral bar with contralateral hemivertebrae (5-10deg per yr)
Unilateral bar (5-9)
Double hemivertebrae (2-5)
Hemivertebrae (1-4)
Wedge (<2)
Block (<2)
25
Indications for mehta casting in early-onset scoliosis
RVAD \>20
Cobb \>25 degrees
Phase 2 rib
26
Indications for surgery in early-onset scoliosis
Cobb \>50 can do growing rods or VEPTR
27
Incidence of neuraxis abnormalities in early onset (juvenile) scoliosis
15-30%
28
Risk factors for progression in adolescent idiopathic scoliosis
Curve \>25 deg before skeletal maturity
Age \<12 at presentation
Tanner \<3 (females)
Risser stage 0-1
Open triradiate cartilage
Thoracic curve (moreso than Lumbar)
Double curve (moreso than single)
Peak growth velocity (Olecranon closure at END of PGV)
29
Features of neurofibromatosis spine
Scoliosis: Nondystrophic & Dystrophic
(Note: Dystrophic has 2 types)
*Type 1: kyphosis \<50 deg; Type 2: kyphosis \>50 deg*
_Features of NF spine:_
Rib pencilling; TP spindling; vertebral scalloping; vertebral wedging; vertebral rotation, dumbbell lesions, widened interpedicular distance, pedicle dysplasia, dural ectasia, paravertebral soft tissue mass.
30
Diagnostic criteria for NF-1
_2 or more of the following:_
6 or more Cafe au lait spots
(\<5mm prepub; \>15mm postpubertal)
2 or more neurofibromas
Axillary freckling
Optic glioma
2 or more Lisch nodules
Primary relative w/ NF
Distinctive osseous lesion
31
5 causes of hemihypertrophy
Idiopathic
Klippel Trelawny
NF
Beckwith Wiedemann
Proteus syndrome
32
Which is worse in congenital kyphosis? Failure of formation (type 1) or failure of segmentation(type 2)?
How to decide PSF vs combined posterior/anterior?
Failure of formation
Curve \<50 PSF alone
More severe curve do ant/post
33
Algorithm for Scheuermann's kyphosis
Note: Normal thoracic kyphosis is 20-45deg
Curve \<60: Observe
Curve 60-75 with \>1 year growth remaining: Brace with CTLSO (milwaukee)
Curve \>75 in skeletally mature: PSF
34
Definition of Scheuermann's kyphosis
Anterior wedging of \>5 degrees across 3 or more consecutive vertebrae
35
Features of dysplastic spondy
Maloriented/hypoplastic facets
Sacral deficiency/Domed sacrum
Hypoplastic pars
36
RFs for progression in paediatric spondylolisthesis
Bilateral pars defect
Dysplastic spondy
Female sex
\>50% slip (Grade 3 or 4 Meyerding)
Increased lumbar lordosis
Slip angle \>50degrees
Increase pelvic incidence (normal is 47degrees)
37
Treatment of paediatric spondylolisthesis?
**_TLSO_**
Acute pars stress rxn (Hot on SPECT)
**_PT/Activity mod_**
Chronic pars stress rxn (Cold on SPECT)
Low grade slip
**_Pars repair_**
L1-L4 single/multiple pars defects
**_Surgery_**
Low grade (failed nonop) L5-S1
High grade (L4-S1 with A/PLIF)
38
Indications for MRI in idiopathic scoliosis
Age \<10
Left thoracic curve
Painful
Apical thoracic kyphosis
Rapid curve progression
An associated syndrome
Neurologic abnormality (ie. asymmetric abdo reflex)
Congenital abnormalities
39
```
# Define the following:
End vertebrae
```
Stable vertebrae
Neutral vertebrae
Apical vertebrae
End vertebrae: Last vertebrae that are maximally tilted in the curve concavity (used to measure Cobb)
Stable vertebrae: Most proximal vertebrae that is most closely bisected by the CSVL
Neutral vertebrae: SP to pedicle distances are equal
Apical vertebrae: Furthest laterally from the CSVL
40
3 criteria to define skeletal maturity
Risser 4
\<1cm growth in 2 visits 6 mos apart
2 years post menarche
41
Define failure of bracing in AIS
6degrees or more progression
Absolute curve progression to \>45 degrees
42
Components of Lenke AIS classification
_1. Curve type_
Measure curves (largest is Major Curve)
Is minor curve structural (bends out less than 25 degrees?)
_2. Lumbar modifier_
A B or C depending relationship of apical lumbar vertebrae to the CSVL
_3. Sagittal modifier_
Between T5-T12, hypo, normo, or hyperkyphotic
43
Things to examine for with early-onset scoliosis
Plagiocephaly
Torticollis
DDH
MTA
44
Which dysplastic does NOT get c spine instability
Achondroplasia
45
Regarding ossification of the C2 vertebrae
Neurocentral synchondrosis fuses around age 3-6
Basilar (dens-body) synchondrosis fuses around age 3-6
Tip fuses around age 12
Should be no synchondrosis in the lower dens in a kid older than about 7!!
46
List 6 facts about C spine pseudosubluxation in kids
1. Occurs between C2 and C3
2. Swischuk's Line (from anterior aspect of C1 and C3 SPs) should be within 1.5mm of posterior arch of C2
3. Up to 4mm of listhesis of c2 on c3 may be seen
4. Posterior spinolaminar line should remain intact
5. Should correct with extension
6. Should not worsen with flexion
47
Fielding classification of Atlantoaxial rotatory displacement
Treatment algorithm?
1. Unilateral facet subluxation. No anterior translation. (TL intact)
2. Unilateral with anterior subluxation. TL not intact
3. Bilateral with anterior sublux. TL not intact
4. Posterior subluxation of C1 on C2
Soft collar 1wk
Halter 1month
HALO traction 3 months
If all fails: C1 C2 fusion
48
Klippel Feil
Classic triad?
Other associations?
Failure of normal segmentation/formation of cervical vertebra.
_Triad_
Limited C-spine ROM, Short webbed neck, Low posterior hairline.
Other associations?
Sprengel's, scoliosis, Renal agenesis, cardiac defects, AA instability, basilar invagination
SYNKINESIS: smile causes blinking
49
Congenital Coxa Vara
Definition?
When to observe? When to operate?
_CCV_
## Footnote
Defined as Neck-shaft angle \<125 and Hilgenreiner's epiphyseal angle of \>25 (normal is \<25)
Observe if HEA \<45 (will resolve)
HEA 45-60 - operate if pain/limp. Can also observe.
HEA \>60 or NSA \<110 = operate. Will get worse!
(Do Valgus ITO with derotation to generate normal anterversion.)
50
Goals of corrections in congenital coxa vara (3)
Complications?
Over correct NSA to about 150deg
HEA to \<30deg
Anteversion of 10deg
_Complications_
Premature physeal closure
GT overgrowth
Loss of correction
Dysplasia
51
Poor prognostic factors for LCP
Age \>6 at presentation
Herring lateral pillar C
Decreased abduction
Female gender
52
Herring Lateral Pillar classification?
A - full height
B lateral pillar \>50%
B/C border - 50% with lateral ossifciation & narrowed about 2-3 mm
C - \<50% lateral pillar height
53
List the 5 Catterall head at risk signs
1. Gage sign (V-shaped lucency at lateral physis)
2. Calcification lateral to epiphysis
3. Lateralization of head
4. Horizontal physis
5. Metaphyseal cyst(s)
54
Waldenstrom stages of LCP
Initial - smaller sclerotic epiphysis
Fragmentation - "dissolving" femoral head
Reossification - new bone appearing, irregular
Remodeling - until skeletal maturity
55
Stulberg classification (helps to pronosticate re OA risk)
1. Normal hip
2. Enlarged femoral head, shortened neck
3. Non spherical head (ovoid, mushroom)
4. Flat head
5. Flat head with incongruent joint
\*\*Stulberg 1 - 0% ranging up to Stulberg 5 with 80% chance of OA at age 40
56
Algorithm for Perthes treatment
_\<6_
NONOP. Physio, Scottish-Rite orthosis (SRO abduction)
Goal is to maintain ROM. Can consider restricted WB until reossification
_6-8_
Controversial. The PSG (Perthes study group) found no diff b/w op & nonop but Wiig study showed varus osteotomies did better than SRO/Physio.
_\>8_
Containment surgery for B and B/C border
C - nonop. Will do poorly regardless of treatment
57
Zone of physis involved in SCFE
Hypertrophic zone
\*\*Weakness of perichondrial ring\*\*
58
Reasons to consider prophylatic pinning of SCFE in contralateral hip
Obese patient
Male patient
Endocrinopathy
Young age at initial slip (\<10 or open triradiates)
59
Southwick angle?
Measure on lateral xray angle b/w tangent to epiphysis and axis of femoral neck.
Difference between other side
Mild \<30
Mod 30-50
Severe \>50
60
Does pinning SCFE cause early physeal closure?
Yes, on average about 10 mos sooner than non pinned side.
Pinned 12 mos
non pinned 22 mos
61
Complications of SCFE treatment
AVN (4-6% but higher with high grade slip)
(screw placement in posterosuperior head)
Chondrolysis (2%)
Contralateral hip (20-80% range) SCFE
Deformity of proximal femur
Fracture
Infection
Progression of slip
Arthritis
62
Components of Southwick osteotomy
Valgus, flexion, IR
63
Which law states that compression across the physis slows longitudinal growth?
Which law states that epiphyseal distraction stimulates growth?
Heuter-Volkmann law
Delpech's law
64
Langenskold class'n of Blounts - describe it
1. Metaphyseal beaking
2. Saucerization of beaking
3. Step
4. Epiphysis bends down in to metaphysis
5. Bar
6. Severe bar
65
Infantile Blount's treatment
_Observe_
Physiologic varus (varus \<18mos of age expected)
_KAFO_
3yrs old or younger with MDA \>16 and Langenskold 1 or 2
_Proximal tib-fib osteotomy_
Progression despite bracing
4yrs old or older at presentation
Any age with Langenskold 3 or higher
Add in bar resection in Langenskold 5 or 6
PRN medial plateau elevation
66
Differences with adolescent blounts?
No potential for correction
Always surgical
Can treat mild deformity with _growth modulation_
Severe @ skeletal maturity requires _osteotomy_.
DON'T need to overcorrect the valgus the way you would in infantile Blount's
67
DDx for valgus knee in kids
**_Bilateral_**
Physiologic
Renal Osteodystrophy
Dysplasia (Morquio, pseudoachon, SED)
**_Unilateral_**
Post-traumatic (physeal arrest or Cozen's)
Infection
Tumour
Ollier's Disease
68
Ddx for varus knees in kids
Physiologic
Blount's (could be unilateral too)
Osteogenesis Imperfecta
Dysplasia (MED, SED, achon)
Vit D deficient Rickets
Vit D resistant Rickets (XLhypophosphatemic)
69
For Sprengel's deformity name two procedures and describe?
Woodward - parascapular muscles removed off spinal origin and moved distally
Green - parascapular muscles taken off scapular insertion and moved proximall on scapula (scapula moved distally)
70
_Tibial deficiency_
List 7 associated conditions
Principles of management
Ectrodactyly (lobster claw hand)
Preaxial polydactyly
Ulnar aplasia
Scoliosis
Hip Dysplasia
Bifurcation of femur
Equinovarus foot (usually rigid)
**_Management_**
_Principles: Assess for knee stability and extensor mechanism integrity._
Type 1a: no proximal tibia: knee disartic
Type 1b: Unossified prox tibia: tx like 2
Type 2: Present prox tibia (extensor intact). Fuse fibula to prox tibia and do Syme's
Type 3: Present distal tibia only. Knee disartic.
Type 4: Tibfib diastasis: Syme's.
71
Fibular deficiency (hemimelia)
List 8 associated MSK findings
"4 foot, 2 hip, 2 knee"
_4 foot_: Ball&Socket ankle, equinovalgus foot, absent lateral rays, tarsal coalition
_2 hip:_ Coxa vara, PFFD
_2 knee:_ Genu valgum, cruciate deficiency
72
Treatment principles of fibular hemimelia
Classification doesn't help with treatment here! Think in terms of principles.
Principles:
1. Assess _LLD, ankle stability and quality of foot_
2. If foot not plantigrade can it be MADE plantigrade? Does it have 3 or more rays?
Treatment
_Ankle stable, and foot OK?_
Lengthen, or shorten other side (if expected LLD \<5cm at maturity)
_Unstable ankle but foot ok?_
Can lengthen and fuse tibiotalar joint
_Unstable ankle and stiff bad foot? (less than 3 rays and non-plantigrade?_
Syme
_Severe projected LLD? (\>10cm)_
Syme
\*\*Remember, may need tibial osteotomy if severe bowing of tibia\*\*
Consider foot salvage when there is a nonfunctional upper extremity
73
Differential for LLD
Hip: DDH, coxa vara, PFFD
Neuromuscular: CP, polio
Acquired: Trauma/tumour/infxn
Other: Hemimelias, dysplasias, hemihypertrophy
_Recall ddx for hemihypertrophy_
Idiopathic, Klippel Trelawney, Proteus, Beckwith Wiedemann, Neurofibromatosis
74
Amount of growth per year by site of lower extremity
Proximal femur: 3mm/yr
Distal femur: 9mm/yr
Prox tibia: 6mm/yr
Distal tibia: 5mm/yr
By the time Trav gets a job, he'll be 39. Then he'll retire at 65.
75
Methods of predicting final LLD at maturity
Anderson & Green
Arithmetic method of White
Mosely straight line graph (requires follow up for 3 data points!)
Paley multiplier method
\*\* For Paley, need chronologic age, leg length and multiplier\*\*
LLD at maturity = Present LLD \* Multiplier
76
PFFD
Classification
Aitken Classification
## Footnote
A. Head present, normal acetab. Very short femoral shaft. At maturity, *head will be attached to shaft*.
B. Head present, mild acetab dysplasia.
At maturity, *head will NOT communicate with the femoral shaft.*
C. No head, severe acetab dysplasia. Tapered proximal femur.
D. No head, Absent acetab. Most of femur absent save for a small remnant distally in most cases.
77
PFFD
Treatment approach
_Treatment depends on:_
## Footnote
1. Is the hip/knee/ankle stable/salvageable?
2. What is the current and expected LLD?
3. Is femur currently \>50% or \<50% contralateral side.
_Considerations:_
Aitken A: Good. Treat with lengthening.
Aitken B: May need acetabular osteotomy to achieve stability of hip. May also need to correct proximal pseudarthrosis and do valgus osteotomy.
Aitken C & D (no head) should have Brown procedure (femoropelvic fusion)
_If stable hip, femur \>50% other side and expected LLD \<20cm_: Salvage leg and lengthen. (Gillespie A)
_Short femur and expected LLD \>20cm?_ (Gillespie B&C) Treat with prosthesis. Two options for this and it depends on ROM/stability of ankle. If ankle sucks, do a symes and fuse the knee. This will require an _AKA prosthesis_. If the ankle ROM is \>60, can do a Van Ness and use a _BKA prosthesis_.
78
List 4 things consistent across all PFFD classes
Short femur
ER of thigh
Valgus of distal femur
Lax knee ligaments
79
Complications of club foot treatment
List 2 from nonop tx
List 6 from op tx
Nonop
Relapse of the deformity (mgmt with repeat casting, repeat TAL prn)
Dynamic supination (mgmt with full tib ant transfer to lateral cuneiform)
Op
Residual cavus
Pes planus (overcorrection)
Undercorrection
Intoeing gait
AVN of talus
Dorsal bunion (Tib ant, FHB and abd hall overpull)
80
What is hindfoot parallelism?
See parallel axes of talus and calcaneus on lateral xray of a CLUB FOOT
Important distinguisher from a congenital vertical talus
81
Normal Kite's angle?
What is the kite's angle in club foot?
CVT?
Normal is around 25-40
Read on the AP foot
In club foot, calcaneus is inverted and medially rotated under the talus which decreases Kite's angle \<25.
CVT (aka "congenital pes valgus") has a high Kite's angle \>40
82
When is achilles tenotomy performed during ponseti technique?
Prior to last cast
Forefoot must be abducted 70 degrees (hypercorrected) and hindfoot must be in valgus
83
Write a prescription for Denis Brown boots/bar
Bonus: what % will need a tib ant transfer?
To be worn after casting for 23hrs/day for 3 mos.
Treated foot in 70 ER, contralat foot in 40ER
Then wear for naps/nighttime until age 4
10-20% need Tib ant transfer to lateral cuneiform.
84
What must you do as part of the workup of congenital vertical talus
(hint, it's not get a plantarflexion lateral xray of the foot)
Image the spine
50% of CVT associated with a neurologic or chromosomal disorder
(myelodysplasia, diastematomyelia, chromosomal abnormality, arthrogryposis)
85
Difference between CVT and oblique talus?
Oblique talus reduces in plantarflexion
86
Position of the calcaneus in congenital vertical talus
Equinus
Valgus/Eversion
ER
Lateral displacement
87
Tarsal coalition
What % are bilateral?
What % of pts have more than 1 coalition?
50% bilateral
10-20% more than 1
88
Child with dorsiflexed calcaneus and plantarflexed forefoot.
What is it and how treated?
Cause?
Calcaneocavus foot
Transfer tib ant to the calcaneus
Neuromuscular cause.
ie Polio with paralysis of gastrosoleus.
89
Deformities of windblown hand?
Thumb-in-palm
MCP contracture
MCP ulnar drift
90
What happens in windblown hips?
One side flexes, adducts and IR
Other side abducts, ER, and flex OR extend
91
Most common level (and highest level) for dislocated hips in myelodysplasia
L3
Hip flexors/adductors working
No abduction
92
Lowest level that can cause hyperextension deformity at the knee
L4 (because quads are intact)
If L5 intact, hamstrings are working
93
Myotomes for hip:
Flexion
Adduction
Abduction
Extension
Flexion L1-2
Adduction L2-3
Abduction L4-5
Extension L5-S1
94
Myotomes for knee
Extension
Flexion
Extension L3-4
Flexion L5-S1
95
Myotomes for ankle
DF
PF
Inv
Ev
EHL
FHL
DF L4
PF S1
Inv L4-5
Ev L5-S1
EHL L5
FHL S1-2
96
Indications for surgical intervention in Down's patient with C1-2 instability
Progressive or acute onset of neurologic symptoms
SAC \<14mm
MRI evidence of cord compression
97
Deformities in arthrogryposis
_Upper limb_
Shoulders adducted & IR
Elbows extended
Forearm pronated
Wrists flexed/ulnarly deviated
Thumb adducted
Intrinsic plus hand
_Lower Limb_
Hips flexed, abducted & ER
Knees flexed
Club feet
98
Features of Larsen's Syndrome
Dislocations (hip, knees, shoulders, elbows)
Club feet
Cervical kyphosis (fuse early to prevent neuro deficits)
99
Down Syndrome
List orthopaedic manifestations
Ligamentous laxity!
_Spine_: Spondy, AA instability, OC instability, Scoliosis
_Hip_: Dysplasia, SCFE
_Knee_: PF instability, Valgus
_Feet_: Metatarsus primus varus, Pes planus
_Hand_: Small finger hypoplasia/clinodactyly; single palmar crease
100
Down Syndrome
List non orthopaedic manifestations
_Cardiac_: ASD/VSD/PDA/Fallot
_Heme_: Leukemia (1%)
_Endocrine_: Hypothyroid, Diabetes Mellitus
_ENT_: Hearing loss, Sleep apnea
_Ophtho_: Refractive errors/strabismus/cataracts
_Neuro_: Mental retardation, early Alzheimers
101
Considerations for perioperative mgmt of sickle cell patients
Administration of hydroxyurea
Supplemental oxygen
Exchange transfusion
Warming to prevent hypothermia
102
List 7 causes of Congenital Coxa Vara
Cleidocranial Dysplasia
Chondrodysplasia Punctata
Metaphyseal Chondrodysplasia
Gaucher's Disease
Mutiple Epiphyseal Dysplasia
PFFD
Spondyloepiphyseal Dysplasia Congenita
103
List 8 causes of acquired coxa vara
OI
Fibrous dysplasia
SCFE
Perthes
Rickets
Osteomyelitis
Paget's Dz
Posttraumatic
104
Extraskeletal manifestations of Osteogenesis Imperfecta (7)
Blue sclera (in types 1&2)
Dentinogenesis imperfecta
Hearing loss
Thin skin
Heart valve pathology
Ligamentous laxity
Risk of malignant hyperthermia
105
Skeletal manifestations of Osteogenesis Imperfecta (8)
Bowed long bones
Thinned cortices
Sabre shins
Scoliosis
Compression #s ("Codfish vertebrae")
Coxa vara
Protrusio acetabuli
Basilar invagination
106
Marfan syndrome
Mutation of what?
List 5 affected organ systems
Fibrillin 1 protein
(affects 1: elastic matrix microfibrils and 2: TGFbeta signalling pathway)
1. Skeletal (scoliosis, protrusio, lig laxity)
2. Ocular (ectopia lentis - superior dislocation)
3. Cardiac (aortic dilation, dissection, regurge)
4. Dural ectasia
5. Resp (spontaneous pneumo)
107
Issues with Marfan's scoliosis perioperatively
FYI: Marfan's have higher prevalence of double thoracic and triple major curves. 40% have kyphosis \>50 degrees
Issues:
Fusion indicated for curves \>45
High rate of complications (blood loss, dural tear, infection, pseudarthrosis)
High rate of failed fixation. Maximize points of fixation!
(Thin lamina, thin pedicles, osteopenia)
108
Diagnostic criteria for dural ectasia
2 Major
Dural sac width below L5 is greater than above L4
Anterior sacral meningocele
2 Minor
Scalloping at S1 \>3.5mm
L5 nerve root sleeve diameter \>6.5mm
_Need 1 Major, or 2 minor to make dx of dural ectasia_
109
Definition of "hip at risk" in CP
Definition of "subluxed hip" in CP
Hip at risk
Abduction \<45deg and Migration index \>25deg
Subluxed hip
Abduction \<30 and MI 25-50 (some sources say 25-60deg)
110
Cause of Jump Gait in CP
Equinus foot
111
Cause of crouch gait in CP
Gastrosoleus weakness
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Cause of stiff gait in CP
Either knee or hip
Knee: diminished flexion due to spastic quads
Hip: Weak hip flexors
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Describe the Rancho procedure
For equinovarus foot in CP
If TIB ANT is the principle deforming force
Tib post and achilles lengthening
Split tib ant transfer to cuboid
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Most common foot deformity in:
Hemiplegia
Diplegia
Quadriplegia
Hemiplegia: Equino**_varus_**
Diplegia: Equino**_valgus_**
Quadriplegia: Equino**_valgus_**
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Foot deformities by level in myelodysplasia
L1, L2, L3 - Equinovarus
L4 - Cavovarus
L5 - Calcaneovalgus
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Indications for fusion of scoliosis in Duchenne muscular dystrophy
Curve \>20degrees in nonambulatory patient
Rapid progression
FVC \<35%
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Outline surgical mgmt of foot deformity in CP
_Equinovarus_
If tib ant dominant: Rancho (Tib post and achilles lengthening with split tib ant transfer to cuboid)
If tib post dominant: Split tib post transfer to peroneus brevis
_Equinovalgus_
(3 options)
1. Lengthen achilles and peroneals & do calc lengthening osteotomy.
2. If Severe? can do medial calc osteotomy and/or cuboidal opening wedge with medial cuneiform closing wedge
3. Triple arthrodesis
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What else do you need to check in Charcot Marie Tooth other than the cavovarus feet?
Hips and spine
15% have hip dysplasia
Also R/O scoliosis
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Risk factors for myelodysplasia
Folate deficiency
Maternal hyperthermia
Maternal diabetes
Use of valproate
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Achondroplasia
Features on xray
_Spine_
TL kyphosis, L lordosis, Short & wide pedicles, scalloped vertebrae
_Pelvis_
Champagne pelvis (wider than deep), deep set hips
_Knee_
Inverted V shape physis, Genu varum
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Which zone of physis affected in achondroplasia?
Proliferative zone
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Spinal Considerations in Achondroplasia
\*\*No Upper C-Spine instability!\*\*
## Footnote
_Foramen magnum stenosis_
_T-L kyphosis_
- must prevent unsupported sitting in young kids
- TLSO if kyphosis \>30 develops
- Fuse if \>50
_Spinal Stenosis_
-Need WIDE decompression and do it 3 levels above and down to S2. Need robust instrumentation
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How to manage myelodysplasia:
Spine
Hips
Knees
Feet
GO!
_Spine_
Dysplastic posterior elements. Requires anterior and posterior fusion with extension to the pelvis. Bracing doesn't generally work. Indicated if progressive (most cases are). High risk of pseudarthrosis and infection!
_Hips_
Highest risk of dislocation at L3 level (unopposed flexion and adduction). Surgical mgmt is controversial. Don't reduce unless they have functional quads. Flexion contractures should do release of TFL, sartorius & rectus, and add in iliopsoas if non ambulator. Abduction contracture can do Ober Yount IT band release.
_Knees_
Weak quads: KAFO
Flexion contracture: Hamstring release or supracondylar osteotomy
Extension contracture: Serial casting
_Feet_
Club feet: Serial casting or posteromedial release if older kid
Calcaneovalgus: consider transfer of Tib Ant posteriorly to calcaneus.
Note: may see alpha fetoprotein in amniocentesis. Often have latex allergy!
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Duchenne stuff
Physical findings
Treatment goals
_Physical_
Gower's sign, positive DTRs, calf pseudohypertrophy, proximal muscle weakness, scoliosis, equinovarus foot, joint contractures.
Labs: elevated CK
_Treatment_
Feet: passive stretching, nighttime AFO splinting, Rancho procedure if bad enough (split tib ant transfer with TAL and tib post lengthening)
Knees: stretching. KAFO if quads weakness. May need hamstring releases.
Spine: Needs instrumented PSF if curve \>20, FVC \<35%
NOTE: Risk of malignant hyperthermia. MH causes massive rise in intracellular calcium which overcapacitates the use of ATP to sequester calcium, overwhelming the cell's oxidative metabolism and contributing to hyperthermia.
May see rise in end-tidal CO2, masseter muscle tension, tachycardia, generalized muscle rigidity. Rise in temperature is a later finding.
Need to stop the agent (inhaled, or succinylcholine) and give dantrolene (1mg/kg) to a max of 10mg/kg until symptoms subside. Dantrolene stops calcium release.
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CMT
Exam findings
Don't forget to look for...
CMT affects the peroneal, median and ulnar nerves primarily.
Chr 17 (PMP22) peripheral myelin protein
Intrinsic wasting, clawing, cavus, weak DF and eversion, areflexic. Loss of vibration sense.
Don't miss hip dysplasia and scoliosis.
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Friedreich's ataxia
1. 3 classic associated conditions
2. 3 classic physical findings
1. Cavovarus feet, Scoliosis, Cardiomyopathy
2. Ataxia, areflexia, positive Babinski
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List 2 predictors of progression of scoliosis in Friedreich's ataxia
Onset of disease age \<10
Onset of scoliosis age \<15
Treat scoliosis with instrumented fusion if Cobb \>60
Normal disease onset between age 7-25
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What do you need to think of when faced with a child who has multiple congenital dislocations?
Cervical kyphosis
It's Larsen's syndrome and they should have cervical fusion done early in life.
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Upper limb CP.
How to determine if wrist contracture is due to finger flexor or wrist flexor tightness?
See how much wrist extension they achieve with fingers fully extended vs fingers flexed.
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CP hip treatment algorithm approach
for migration index \<50
_Hip at Risk_
Migration index \>25-30 and Abduction \<45
Monitor twice yearly
_Subluxed_ Hip
MI 25-50
Release adductor longus and gracilis
Release adductor brevis if abduction \<45
Walker? Release psoas at brim
Nonwalker? Release iliopsoas off LT
Proximal hamstring lengthening of popliteal angle is \>45
\*\*\*Do this if age 3 or younger\*\*\*
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CP hip treatment for migration index \>50%
_Surgical indications:_
Age 4 or older, MI\>50% (some say 60%) or 1 year post soft tissue procedure with progression
_Approach_
Soft tissue releases first
Lateral incision: Insert blade chisel and try to reduce hip (do capsulotomy if it doesn't reduce)
Cut femur (below lesser) and apply blade plate.
Anterior approach: Dega osteotomy
With hip reduced, remove overlap of femur.
Fix femur. Can spica, but early physio may be better.
_Goals_: 100degrees for nonambulator
120 degrees for ambulator
Version of 0-15
_Salvage options:_
Chiari; Shelf
Castle procedure: Resect everything above the LT, sew vastus/rectus over the cut end. Interposition of abductor muscles into hip joint.
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Outline the GMFCS
1. No limits but impairments in speed, balance, coordination
2. Can't ambulate on uneven ground
3. Need wheelchair for distance
4. Mostly in wheelchair, may walk at home.
5. Full time wheelchair. Difficulty with antigravity head/trunk posture
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RFs for CP
Prematurity
Anoxic injury
Intrauterine factors
Infections
(Toxo, Rubella, CMV, Herpes)
Meningitis
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Outline radiographic findings of SCFE
Klein's line
Widening of physis
Metaphyseal "Blanch" sign of Steele (overlap of metaphysis and epiphysis)
Southwich angle
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Technical pearls/goals for SCFE pinning
Fracture table, no reduction
Can use 1 vs 2 screws (1 probably enough)
Start in anterior neck proximal to LT (avoid stress riser in subtroch region)
Aim for centre of epiphysis
3 threads into epiphysis, leave 5mm of bone between screw tip and joint
Multiplanar fluoro to ensure screw not in joint
Leave screw proud to find later
Screw head should be lateral to intertrochanteric line
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Diagnosis to think of in a toddler with decreased ambulation, abdo pain, fever, decreased appetite.
Discitis
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2 risk factors for paediatric septic arthritis of the hip
Prematurity
C - section
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Algorith for atlantoaxial rotatory subluxation
_Acute (\<2weeks)_
Collar for 2 weeks, if reduced, monitor. If not, Halter traction.
_Chronic (\>2weeks)_
Halter for 2 weeks. If reduced, collar x3mos
If not, halo traction for 2 weeks. If reduced, vest x3mos
If not, C1C2 fusion. Protect with halo vest for 2-3 mos postop
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List associated conditions with CVT (4)
Myelomeningocele
Arthrogryposis
Diastematomyelia
Chromosomal abnormalities
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