Paeds - Scott Flashcards

1
Q

RFs for brachial plexus birth palsy

A

Macrosomia

Difficult presentation

Shoulder dystocia

Forceps/instrumented delivery

Breech position

Prolonged labour

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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)

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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)

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4
Q

Order of ossification of tarsal bones

A

Calcaneus, talus, cuboid, cuneiforms

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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

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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.

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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)

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8
Q

Jacob classification of lateral condyle fractures

A

1 <2mm

  1. 2-4mm (fix)
  2. >4mm (fix)
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9
Q

3 A’s of paediatric compartment syndrome

A

Agitation

Anxiety

increasing Analgesia requirements

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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

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11
Q

Difference between TENs and plate ORIF for paeds BBFF.

A

TENs had shorter OR time and less blood loss

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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

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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

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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.

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15
Q

Order of distal tibial physeal closure

A
  1. Central
  2. Posterior
  3. Medial
  4. Lateral (anterolateral)
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16
Q

Triplane fracture - describe # pattern

A

Sagittal in the epiphysis

Axial in the physis

Coronal in the metaphysis

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17
Q

Possible etiology of cubitus varus deformity post-supracondylar?

A

Malreduction with fragment medialized, internally rotated and extended.

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18
Q

Subclassification of Gartland 3 sch#

A

3A: posteromedial

3B: posterolateral

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19
Q

Monteggia reduction techniques

A

Bado Classification

  1. Reduce with flexion and supination. Cast at 110degress of flexion in forearm supination

(Hyperpronation injury in elbow extension)

  1. Reduce in extension with pronation. Cast in extension. (Hypersupination injury)
  2. Reduce in extension with supination and valgus stress. Cast in flexion (110) and supination.
  3. Usually surgery. If nonop, then cast in flexion (110) and supination.
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20
Q

Volume of fluid resusc bolus in paeds

A

20cc/kg

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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.

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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

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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

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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)

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25
Q

Indications for mehta casting in early-onset scoliosis

A

RVAD >20

Cobb >25 degrees

Phase 2 rib

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26
Q

Indications for surgery in early-onset scoliosis

A

Cobb >50 can do growing rods or VEPTR

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27
Q

Incidence of neuraxis abnormalities in early onset (juvenile) scoliosis

A

15-30%

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28
Q

Risk factors for progression in adolescent idiopathic scoliosis

A

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)

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29
Q

Features of neurofibromatosis spine

A

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.

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30
Q

Diagnostic criteria for NF-1

A

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

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31
Q

5 causes of hemihypertrophy

A

Idiopathic

Klippel Trelawny

NF

Beckwith Wiedemann

Proteus syndrome

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32
Q

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?

A

Failure of formation

Curve <50 PSF alone

More severe curve do ant/post

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33
Q

Algorithm for Scheuermann’s kyphosis

A

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

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34
Q

Definition of Scheuermann’s kyphosis

A

Anterior wedging of >5 degrees across 3 or more consecutive vertebrae

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35
Q

Features of dysplastic spondy

A

Maloriented/hypoplastic facets

Sacral deficiency/Domed sacrum

Hypoplastic pars

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36
Q

RFs for progression in paediatric spondylolisthesis

A

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)

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37
Q

Treatment of paediatric spondylolisthesis?

A

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)

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38
Q

Indications for MRI in idiopathic scoliosis

A

Age <10

Left thoracic curve

Painful

Apical thoracic kyphosis

Rapid curve progression

An associated syndrome

Neurologic abnormality (ie. asymmetric abdo reflex)

Congenital abnormalities

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39
Q
# Define the following:
End vertebrae

Stable vertebrae

Neutral vertebrae

Apical vertebrae

A

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

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40
Q

3 criteria to define skeletal maturity

A

Risser 4

<1cm growth in 2 visits 6 mos apart

2 years post menarche

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41
Q

Define failure of bracing in AIS

A

6degrees or more progression

Absolute curve progression to >45 degrees

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42
Q

Components of Lenke AIS classification

A

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

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43
Q

Things to examine for with early-onset scoliosis

A

Plagiocephaly

Torticollis

DDH

MTA

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44
Q

Which dysplastic does NOT get c spine instability

A

Achondroplasia

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45
Q

Regarding ossification of the C2 vertebrae

A

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!!

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46
Q

List 6 facts about C spine pseudosubluxation in kids

A
  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
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47
Q

Fielding classification of Atlantoaxial rotatory displacement

Treatment algorithm?

A
  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

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48
Q

Klippel Feil

Classic triad?

Other associations?

A

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

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49
Q

Congenital Coxa Vara

Definition?

When to observe? When to operate?

A

CCV

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.)

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50
Q

Goals of corrections in congenital coxa vara (3)

Complications?

A

Over correct NSA to about 150deg

HEA to <30deg

Anteversion of 10deg

Complications

Premature physeal closure

GT overgrowth

Loss of correction

Dysplasia

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51
Q

Poor prognostic factors for LCP

A

Age >6 at presentation

Herring lateral pillar C

Decreased abduction

Female gender

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52
Q

Herring Lateral Pillar classification?

A

A - full height

B lateral pillar >50%

B/C border - 50% with lateral ossifciation & narrowed about 2-3 mm

C - <50% lateral pillar height

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53
Q

List the 5 Catterall head at risk signs

A
  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)
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54
Q

Waldenstrom stages of LCP

A

Initial - smaller sclerotic epiphysis

Fragmentation - “dissolving” femoral head

Reossification - new bone appearing, irregular

Remodeling - until skeletal maturity

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55
Q

Stulberg classification (helps to pronosticate re OA risk)

A
  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

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56
Q

Algorithm for Perthes treatment

A

<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

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57
Q

Zone of physis involved in SCFE

A

Hypertrophic zone

**Weakness of perichondrial ring**

58
Q

Reasons to consider prophylatic pinning of SCFE in contralateral hip

A

Obese patient

Male patient

Endocrinopathy

Young age at initial slip (<10 or open triradiates)

59
Q

Southwick angle?

A

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
Q

Does pinning SCFE cause early physeal closure?

A

Yes, on average about 10 mos sooner than non pinned side.

Pinned 12 mos

non pinned 22 mos

61
Q

Complications of SCFE treatment

A

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
Q

Components of Southwick osteotomy

A

Valgus, flexion, IR

63
Q

Which law states that compression across the physis slows longitudinal growth?

Which law states that epiphyseal distraction stimulates growth?

A

Heuter-Volkmann law

Delpech’s law

64
Q

Langenskold class’n of Blounts - describe it

A
  1. Metaphyseal beaking
  2. Saucerization of beaking
  3. Step
  4. Epiphysis bends down in to metaphysis
  5. Bar
  6. Severe bar
65
Q

Infantile Blount’s treatment

A

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
Q

Differences with adolescent blounts?

A

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
Q

DDx for valgus knee in kids

A

Bilateral

Physiologic

Renal Osteodystrophy

Dysplasia (Morquio, pseudoachon, SED)

Unilateral

Post-traumatic (physeal arrest or Cozen’s)

Infection

Tumour

Ollier’s Disease

68
Q

Ddx for varus knees in kids

A

Physiologic

Blount’s (could be unilateral too)

Osteogenesis Imperfecta

Dysplasia (MED, SED, achon)

Vit D deficient Rickets

Vit D resistant Rickets (XLhypophosphatemic)

69
Q

For Sprengel’s deformity name two procedures and describe?

A

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
Q

Tibial deficiency
List 7 associated conditions
Principles of management

A

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
Q

Fibular deficiency (hemimelia)

List 8 associated MSK findings

A

“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
Q

Treatment principles of fibular hemimelia

A

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
Q

Differential for LLD

A

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
Q

Amount of growth per year by site of lower extremity

A

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
Q

Methods of predicting final LLD at maturity

A

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
Q

PFFD

Classification

A

Aitken Classification

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
Q

PFFD

Treatment approach

A

Treatment depends on:

  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
Q

List 4 things consistent across all PFFD classes

A

Short femur

ER of thigh

Valgus of distal femur

Lax knee ligaments

79
Q

Complications of club foot treatment

List 2 from nonop tx

List 6 from op tx

A

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
Q

What is hindfoot parallelism?

A

See parallel axes of talus and calcaneus on lateral xray of a CLUB FOOT

Important distinguisher from a congenital vertical talus

81
Q

Normal Kite’s angle?

What is the kite’s angle in club foot?

CVT?

A

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
Q

When is achilles tenotomy performed during ponseti technique?

A

Prior to last cast

Forefoot must be abducted 70 degrees (hypercorrected) and hindfoot must be in valgus

83
Q

Write a prescription for Denis Brown boots/bar

Bonus: what % will need a tib ant transfer?

A

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
Q

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)

A

Image the spine

50% of CVT associated with a neurologic or chromosomal disorder

(myelodysplasia, diastematomyelia, chromosomal abnormality, arthrogryposis)

85
Q

Difference between CVT and oblique talus?

A

Oblique talus reduces in plantarflexion

86
Q

Position of the calcaneus in congenital vertical talus

A

Equinus

Valgus/Eversion

ER

Lateral displacement

87
Q

Tarsal coalition

What % are bilateral?

What % of pts have more than 1 coalition?

A

50% bilateral

10-20% more than 1

88
Q

Child with dorsiflexed calcaneus and plantarflexed forefoot.

What is it and how treated?

Cause?

A

Calcaneocavus foot

Transfer tib ant to the calcaneus

Neuromuscular cause.
ie Polio with paralysis of gastrosoleus.

89
Q

Deformities of windblown hand?

A

Thumb-in-palm

MCP contracture

MCP ulnar drift

90
Q

What happens in windblown hips?

A

One side flexes, adducts and IR

Other side abducts, ER, and flex OR extend

91
Q

Most common level (and highest level) for dislocated hips in myelodysplasia

A

L3

Hip flexors/adductors working

No abduction

92
Q

Lowest level that can cause hyperextension deformity at the knee

A

L4 (because quads are intact)

If L5 intact, hamstrings are working

93
Q

Myotomes for hip:

Flexion

Adduction

Abduction

Extension

A

Flexion L1-2

Adduction L2-3

Abduction L4-5

Extension L5-S1

94
Q

Myotomes for knee

Extension

Flexion

A

Extension L3-4

Flexion L5-S1

95
Q

Myotomes for ankle

DF

PF

Inv

Ev

EHL

FHL

A

DF L4

PF S1

Inv L4-5

Ev L5-S1

EHL L5

FHL S1-2

96
Q

Indications for surgical intervention in Down’s patient with C1-2 instability

A

Progressive or acute onset of neurologic symptoms

SAC <14mm

MRI evidence of cord compression

97
Q

Deformities in arthrogryposis

A

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
Q

Features of Larsen’s Syndrome

A

Dislocations (hip, knees, shoulders, elbows)

Club feet

Cervical kyphosis (fuse early to prevent neuro deficits)

99
Q

Down Syndrome

List orthopaedic manifestations

A

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
Q

Down Syndrome

List non orthopaedic manifestations

A

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
Q

Considerations for perioperative mgmt of sickle cell patients

A

Administration of hydroxyurea

Supplemental oxygen

Exchange transfusion

Warming to prevent hypothermia

102
Q

List 7 causes of Congenital Coxa Vara

A

Cleidocranial Dysplasia

Chondrodysplasia Punctata

Metaphyseal Chondrodysplasia

Gaucher’s Disease

Mutiple Epiphyseal Dysplasia

PFFD

Spondyloepiphyseal Dysplasia Congenita

103
Q

List 8 causes of acquired coxa vara

A

OI

Fibrous dysplasia

SCFE

Perthes

Rickets

Osteomyelitis

Paget’s Dz

Posttraumatic

104
Q

Extraskeletal manifestations of Osteogenesis Imperfecta (7)

A

Blue sclera (in types 1&2)

Dentinogenesis imperfecta

Hearing loss

Thin skin

Heart valve pathology

Ligamentous laxity

Risk of malignant hyperthermia

105
Q

Skeletal manifestations of Osteogenesis Imperfecta (8)

A

Bowed long bones

Thinned cortices

Sabre shins

Scoliosis

Compression #s (“Codfish vertebrae”)

Coxa vara

Protrusio acetabuli

Basilar invagination

106
Q

Marfan syndrome

Mutation of what?

List 5 affected organ systems

A

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
Q

Issues with Marfan’s scoliosis perioperatively

A

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
Q

Diagnostic criteria for dural ectasia

A

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
Q

Definition of “hip at risk” in CP

Definition of “subluxed hip” in CP

A

Hip at risk

Abduction <45deg and Migration index >25deg

Subluxed hip

Abduction <30 and MI 25-50 (some sources say 25-60deg)

110
Q

Cause of Jump Gait in CP

A

Equinus foot

111
Q

Cause of crouch gait in CP

A

Gastrosoleus weakness

112
Q

Cause of stiff gait in CP

A

Either knee or hip

Knee: diminished flexion due to spastic quads

Hip: Weak hip flexors

113
Q

Describe the Rancho procedure

A

For equinovarus foot in CP

If TIB ANT is the principle deforming force

Tib post and achilles lengthening
Split tib ant transfer to cuboid

114
Q

Most common foot deformity in:

Hemiplegia

Diplegia

Quadriplegia

A

Hemiplegia: Equinovarus

Diplegia: Equinovalgus

Quadriplegia: Equinovalgus

115
Q

Foot deformities by level in myelodysplasia

A

L1, L2, L3 - Equinovarus

L4 - Cavovarus

L5 - Calcaneovalgus

116
Q

Indications for fusion of scoliosis in Duchenne muscular dystrophy

A

Curve >20degrees in nonambulatory patient

Rapid progression

FVC <35%

117
Q

Outline surgical mgmt of foot deformity in CP

A

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
118
Q

What else do you need to check in Charcot Marie Tooth other than the cavovarus feet?

A

Hips and spine

15% have hip dysplasia

Also R/O scoliosis

119
Q

Risk factors for myelodysplasia

A

Folate deficiency

Maternal hyperthermia

Maternal diabetes

Use of valproate

120
Q

Achondroplasia

Features on xray

A

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

121
Q

Which zone of physis affected in achondroplasia?

A

Proliferative zone

122
Q

Spinal Considerations in Achondroplasia

A

**No Upper C-Spine instability!**

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

123
Q

How to manage myelodysplasia:

Spine

Hips

Knees

Feet

GO!

A

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!

124
Q

Duchenne stuff

Physical findings

Treatment goals

A

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.

125
Q

CMT

Exam findings

Don’t forget to look for…

A

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.

126
Q

Friedreich’s ataxia

  1. 3 classic associated conditions
  2. 3 classic physical findings
A
  1. Cavovarus feet, Scoliosis, Cardiomyopathy
  2. Ataxia, areflexia, positive Babinski
127
Q

List 2 predictors of progression of scoliosis in Friedreich’s ataxia

A

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

128
Q

What do you need to think of when faced with a child who has multiple congenital dislocations?

A

Cervical kyphosis

It’s Larsen’s syndrome and they should have cervical fusion done early in life.

129
Q

Upper limb CP.

How to determine if wrist contracture is due to finger flexor or wrist flexor tightness?

A

See how much wrist extension they achieve with fingers fully extended vs fingers flexed.

130
Q

CP hip treatment algorithm approach
for migration index <50

A

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***

131
Q

CP hip treatment for migration index >50%

A

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.

132
Q

Outline the GMFCS

A
  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
133
Q

RFs for CP

A

Prematurity

Anoxic injury

Intrauterine factors

Infections

(Toxo, Rubella, CMV, Herpes)

Meningitis

134
Q

Outline radiographic findings of SCFE

A

Klein’s line

Widening of physis

Metaphyseal “Blanch” sign of Steele (overlap of metaphysis and epiphysis)

Southwich angle

135
Q

Technical pearls/goals for SCFE pinning

A

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

136
Q

Diagnosis to think of in a toddler with decreased ambulation, abdo pain, fever, decreased appetite.

A

Discitis

137
Q

2 risk factors for paediatric septic arthritis of the hip

A

Prematurity

C - section

138
Q

Algorith for atlantoaxial rotatory subluxation

A

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

139
Q

List associated conditions with CVT (4)

A

Myelomeningocele

Arthrogryposis

Diastematomyelia

Chromosomal abnormalities

140
Q
A