Pediatrics Flashcards

Question 1

Q

What are associated anomalies with this deformity?

Answer

A

Klipell Fiel

congential scoliosis

UE abnormalities

diastomeomyelia

renal disease

Question 2

Q

What is the classification of spregnel?

Question 3

Q

What are the procedures for spregnel

Answer

A

Done for stage 3/4 to improve cosmesis and abduction

Between ages 3-8, >8 is not a good candidate

Woodward with clavicular osteotomy is the preferred procedure

Procedure is done prone, tunnel subfacia anterior to do the clavicle

Question 4

Q

What are risk factors for brachial plexus palsy

Answer

A

large babies
difficult presentation
shoulder dystocia
forceps delivery
breech position
prolonged labor

Question 5

Q

What are prognostic factors for brachial plexus palsy?

Answer

A

90% will recover without intervention - can occur for up to 2 years

70% of those affected will develop glenoid retroversion

Good - biecps twitch by two months

**Bad **

lack of biceps function
preganglionic injuries; Horner’s syndrome, loss of rhomboid function

avulsion injuries (worst prognosis)

Question 6

Q

What is Erb’s palsy?

Answer

A

Mechanism

results from excessive displacement of head to opposite side and depression of shoulder on same side producing traction on plexus

occurs during difficult delivery in infants or fall onto shoulder in adults

Physical exam

clinically, arm will be adducted, internally rotated, at shoulder; pronated, extended at elbow (“waiter’s tip”)
C5 deficiency

axilllary nerve - weakness in deltoid, teres minor
suprascapular nerve - weakness in supraspinatus, infraspinatus
musculocutaneous nerve - weakness to biceps

C6 deficiency

radial nerve - weakness in brachioradialis, supinator

Prognosis - best prognosis

Question 7

Q

What is klumpke’s palsy?

Answer

A

Mechanism

rare in obstetric palsy
usually avulsion injuries caused by excessive abduction (person falling from height clutching on object to save himself)
other causes may include cervical rib, or lung mets in lower deep cervical lymph nodes

Physical exam

deficit of all of the small muscles of the hand (ulnar and median nerves)
clinically, presents as “claw hand”

wrist held in extreme extension because of the unopposed wrist extensors
hyperextension of MCP due to loss of hand intrinsics
flexion of IP joints due to loss of hand intrinsics

Prognosis

poorer
frequently associated with a preganglion injury and Horner’s Syndrome

Ptosis, myosis, anhydrosis

Question 8

Q

Draw the brachial plexus

Question 9

Q

What are indications for microsurgery associated with bracial plexus palsy

Answer

A

complete flail arm or horner’s at one month

Question 10

Q

Diagnosis? Incidence? Options for treament (general)

Answer

A

Brachial Plexus Palsy - No IR

At time of birth
- PT for ROM and monitor
- 90% will improve
Early micro surgery (graft or repair)
- fail arm at one month
- horner’s syndrome at one month
- root avulsion (only grafting)
contracture release (any age)
- pec, subscap z-lengthening, arthroscopic subscapular release
L’Episcopo lat dorsi/teres major transfer with pec major contracture release
- requires a congruent joint
- usually children < 4yo
Proximal humerus rotational osteotomy
- incongruent joint
- >5yo

Question 11

Q

Deformity associated with madelung’s

Answer

A

Volar carpal subluxation

proximal radial synostosis

increased radial inclination

volar tilt

volar-ulnar tethering of vickers ligament

Question 12

Q

What is vickers ligament

Answer

A

thickening of radiolunate ligament - madelungs

Question 13

Q

What is leri-wiell

Answer

A

rare genetic disorder caused by mutation in the SHOX gene
SHOX stands for short-statute homeobox-containing gene
anatomically at the tip of the sex chromosome - pseudo-sex linked disorder
causes mesomelic dwarfism (short stature)
Normal intellegene
associated Madelung’s defomity of the forearm

Question 14

Q

What is the treatment for this deformity

Answer

A

watch if they are young, deformity is small; assess with serial XR

Consider OR for pain or functional deformity

Physiolysis or Vickers release - open physis with progression

Radial ostotomy, vickers release, +/- ulnar ostoetomy, +/- ulnar epiphyseodesis

Question 15

Q

What is the evidence for screening DDH

Answer

A

No evidence to screen

Imaging prior to 6 months in infants with clinical evidance, fam hx, breech,

Question 16

Q

What factors are associated with increased incidence of LCP?

Answer

A

positive family history
low birth weight
abnormal birth presentation
children exposed to second hand smoke
Asian, Eskimo and Central European decent

Question 17

Q

What are the risk factors of LCP?

Answer

A

family history of disease in 1.6-20% of cases
has been found to be associated with ADHD in 33% of cases
bone age is delayed in 89% of patients

Question 18

Q

What factors are associated with poor prognosis in this patient?

Answer

A

bone > 6 years
female sex
decreased hip range of motion

Lateral pillar, head sphericity

Most patients will do ok until 6th decade

Question 19

Q

What are the caterall risk factors associated with LCP?

Answer

A

Gage sign - V-shaped radiolucency in the lateral portion of the epiphysis and/or adjacent metaphysis

calcification lateral to the epiphysis
lateral subluxation of the femoral head
horizontal proximal femoral physis
metaphyseal cyst

Question 20

Q

What are the lateral pillar and stulberg classfications

Answer

A

Lateral pillar (fragmentation phase)

A- none

B - >50% no height loss

B/C - >50% height loss

C - < 50% lateral pillar

Question 21

Q

What are treatment options for LCP?

Answer

A

Goal : low stulberg classficiation

< 8 = nothing, casting is controversial

> 8, lateral pillar B and B/C in fragmentation phase = femoral osteotomy or pelvic ostetomy containment procedure

C - later treatment

Question 22

Q

Outcomes you should know for LCP

Answer

A

no positive effect has been found for containment surgery performed after initial or early fragmentation stage

children with lateral pillar A and those with B under 8 years did well regardless of treatment

large recent studies show improved outcomes with surgery for lateral pillar B and B/C in children > 8 years (bone age >6 years)

poor outcome for lateral pillar C regardless of treatment

Question 23

Q

Risk factors for developing this pathology

Answer

A

males (male to female ratio is 3:2)
African Americans
Pacific islanders
obese children - single greatest risk factor

during period of rapid growth
bilateral in 20% (20-40% May go on to develop bilateral)

Question 24

Q

When would you consider endocrine WU for SCFE and what are you looking for?

Answer

A

child is < 10 years old
weight is < 50th percentile

hypothyroidism (labs show elevated TSH)

*renal osteodystrophy** (abnormal BUN and creatinine)
*growth hormone treatment**

Question 25

Q

What is the risk of AVN associated with the loder classification?

Answer

A

stable - AVN = 10%

able to bear weight with or without crutches

unstable - AVN = 47%

unable to ambulate (not even with crutches)

Question 26

Q

Chondrolysis after SCFE is associated with

Answer

A

unrecognized implant penetration of the articular surface
spica cast immobilization
Advanced SCFE
Intertrochanteric osteotomy

Question 27

Q

AVN following SCFE is associated with

Answer

A

unstable slip (47%)
hardware placement in posteriosuperior femoral neck
Increased number of pins
Cuneiform osteotomy

Question 28

Q

What are options for late deformity in SCFE

Answer

A

arthroscopic vs mini anterior for osteochondroplasty

surgical dislocation for more severe deformity

Southwick osteotomy

Question 29

Q

Differential? Indications for surgery?

Answer

A

Differential for aquired coxa vara
- AVN: (DDH or Perthes)
- Fibrous Dysplasia
- SCFE
- Rickets
- Osteomyelitis of Hip and Septic Arthritis
- traumatic
- Paget’s Disease
Imaging/Indications for valgus osteotomy
- Hilgenreiners angle < 45 - nonop
- Hilgenreiners angle > 60
- neck shaft angle < 110
Considerations with osteotomy
- might need to tension abductors
- may have LLD

Question 30

Q

thought to be a cause of sacral agenesis

Answer

A

maternal diabetes

Question 31

Q

associations with sacral agenesis

Answer

A

gastrointestinal disorders (imperforate anus)
genitourinary disorders
cardiovascular disorders
lower extremity deformities
progressive kyphosis

Question 32

Q

what are the clinic findings associated with sacral agenesis

Answer

A

prominent lower lumbar spine
extended knees, flexed hips, and equinovarus feet.
classic sign of buttock dimping

Question 33

Q

What are leg abnormalities associated with PFFD

Answer

A

orthopaedic manifestations

fibular hemimelia (50%)
ACL deficiency
PCL deficiency
Hypoplastic lateral condyle = genu valgum
coxa vara
knee contractures
Hypoplastic or dislocated patella

nonorthopaedic manifestations

dysmorphic facies found in rare autosomal dominant type

Question 34

Q

What is the Aitken classficiation for PFFD

Question 35

Q

What are treatment options for PFFD

Answer

A

limb lengthening with or without contralateral epiphysiodesis
- indications
  - predicated limb length discrepancy of <20 cm at maturity
  - stable hip and functional foot
  - femoral length >50% of opposite side
  - femoral head present (Aiken classifications A & B)
- contraindications
  - unaddressed varus, proximal pseudoarthrosis, or acetabular dysplasia
    - can do a containment procedure in A/B
knee arthrodesis with foot ablation
- indications
  - ipsilateral foot is at the level of contralateral knee or more proximal
  - prosthetic knee will not be below the level of the contralateral knee at maturity
  - need for improved prosthetic fit, function, and appearance
femoral-pelvic fusion
- indications
  - femoral head absent (Aiken classifications C & D)
- Fusion
  - fuse the femur to the acetabulum with the femur at 90 degrees
  - knee flexion and extension becomes hip motion
- Brown’s procedure
  - __180 degree rotation so the knee becomes the hip and the ankle becomes the knee
  - function as a BKA
Van Ness rotationplasty
- indications
  - ipsilateral foot at level of contralateral knee
  - ankle with >60% of motion
  - absent femoral head (Aiken classifications C & D)
- surgical technique
  - 180 degree rotational turn through the femur
  - ankle dorsiflexion becomes knee flexion
  - allows the use of a below-knee prosthesis to improve gait and efficiency
amputation
- indications
  - femoral length <50% of opposite side
- surgical technique
  - preserve as much length as possible
  - amputate through the joint, if possible, in order to avoid overgrowth which can lead to difficult prosthesis fitting
- fit for prosthesis at 6 months for UE and 1 yr for LE

Question 36

Q

What are the goals of pediatric amputation?

Answer

A

(1) Preserve length.
(2) Preserve important growth plates.
(3) Per- form disarticulation, rather than transosseous amputation, whenev- er possible.
(4) Preserve the knee joint whenever possible.
(5) Sta- bilize and normalize the proximal portion of the limb.
(6) Be pre- pared to deal with other issues in addition to limb deficiency.

Question 37

Q

What is commonly associated with limb hemihypertrophy and what exam do you need to perform?

Answer

A

Wilm’s tumour

serial US until age 5

Question 38

Q

What are the growth rates of the lower limb?

Answer

A

proximal femur - 3 mm / yr (1/8 in)
distal femur - 9 mm / yr (3/8 in)
proximal tibia - 6 mm / yr (1/4 in)
distal tibia - 5 mm / yr (3/16 in)

Question 39

Q

What are four ways to assess projected limb length

Answer

A

Green-Anderson tables

uses extremity length for a given age

Moseley straight line graph

improves on Green-Anderson method by reformatting data in a graph form
accounts for differences between skeletal and chronologic age
minimizes error
averages serial measurements

Multiplier method

prediction based on multiplying the current discrepancy by a sex and age specific factor
most accurate for congenital LLD

1/2 of final leg length

girls at age 3
boys at age 4

Question 40

Q

What is the most accurate way to predict LLD in congential differences

Answer

A

mutiplier

Question 41

Q

What are the options for LLD

Answer

A

Shoe lift

< 2 cm

shortening of long side - epiphysiodesis

2-5 cm projected LLD

limb lengthening of short side

> 5 cm projected LLD

lengthening often combined with a shortening procedure (epiphysiodesis, ostectomy) on long side

physeal bar excision
indications

bony bridge involves <50% of physis
at least 2 years left of growth

Question 42

Q

What are the principles of lengthening

Answer

A

initiation

perform osteotomy at CORA and place fixator
metaphyseal corticotomy to preserve medullary canal and blood supply (too low = risk of nonunion)

distraction

wait 5-7 days then begin distraction
distract ~ 1 mm/day
following distraction keep fixator on for as many days as you lengthened

concurrent procedures

some ream over a nail so ex-fix can be removed sooner

But can put them at risk of infection

lengthening often combined with a shortening procedure (epiphysiodesis, ostectomy) on long side

Complications

Incomplete arrest/ angular deformity
Pin site infections
Fracture
Delayed union
Premature cessation of lengthening
Joint subluxation/dislocation

Question 43

Q

What are the indications for genetic testing or syndromal work-up in PFFD

Answer

A

usually occurs in isolation

Do work-up if other associated deficiencies

Question 44

Q

What is the most accurate way to measure LLD

Answer

A

CT scanogram - more accurate in the setting of contractures

Question 45

Q

What are the non-operative treatment options for LLD

Answer

A

<2cm no treatment
2-5 - shoe lift
>20cm requires extension prosthesis

Question 46

Q

What are the structural components of congential knee dilocation?

Answer

A

quadricep tendon contracture (short, fibrosis)
anterior subluxation of hamstring tendon
absent suprapatellar pouch
tight collateral ligament

patella laterally dislocated

Question 47

Q

What syndromes and conditions often occur with congenital knee dislocation?

Answer

A

Syndromes

myelomeningocele
arthrogryposis
Larsen’s syndrome

Conditions

DDH

clubfoot

Metatarsus adductus

congential vertical talus

Question 48

Q

What is the treatment of congential knee dislocation?

Answer

A

Close reduction, long leg cast - usually in patients with subluxation and most will respond to serial casting to correct hyperextension

Takes presedence over DDH and foot deformity

Question 49

Q

What are the operative indications for congential knee dislocation?

Answer

A

usually severe dislocation with associated neuromuscular condition

indications

if failure to gain 30 degrees of flexion after 3 months of casting
Or if the knee is not reducible with closed reduction

goal of surgery is to obtain 90 degrees of flexion with

quadriceps tendon lengthening (V-Y quadricepsplasty or Z lengthening) - always

anterior joint capsule release
hamstring tendon posterior transposition
collateral ligaments mobilization

postoperative

cast in 45 to 60 degrees of flexion for 3 to 4 weeks

Question 50

Q

Where is a pediatric popliteal cyst located? How is it different from an adult and what is the treament

Answer

A

semimembranosus
medial head of gastrocnemius

Not associated with medial meniscal tear

Usually resovles without surgery

Question 51

Q

What is the differential for genu valgum

Answer

A

bilateral genu valgum

physiologic
renal osteodystrophy (renal rickets)

Because it usually presents when the child is skeletally in valgus, the weakness tends to accentuate this deformity

skeletal dysplasia
Morquio syndrome
spondyloepiphyseal dysplasia
chondroctodermal dysplasia

unilateral genu valgum

physeal injury from trauma, infection, or vascular insult
proximal metaphyseal tibia fracture (Cozen’s fracture)

Will see evidence of physeal dysplasia or assymetric growth lines

benign tumors
fibrous dysplasia
osteochondromas
Ollier’s disease

Question 52

Q

What are the deformities associated with blounts and what are the radiographic features?

Answer

A

genu varum

flexion, Internal tibial torsion

Radiographic findings

varus focused at proximal tibia
severe deformity
asymmetric bowing
progressing deformity
sharp angular deformity
lateral thrust during gait
metaphyseal beaking

Question 53

Q

What are two pertinant radiographic measurements to help quantify blounts

Answer

A

**metaphyseal-diaphyseal angle (Drennan) **

angle between line connecting metaphyseal beaks and a line perpendicular to the longitudinal axis of the tibia
>16 ° is considered abnormal and has a 95% chance of progression
<10 ° has a 95% chance of natural resolution of the bowing

tibiofemoral angle

angle between the longitudinal axis of the femur and tibia

Question 54

Q

What is your differential for genu varum

Answer

A

blounts

osteogenesis imperfecta
osteochondromas
trauma
various dysplasia

Bilateral

Rickets - osteopenia and widened physes
Skeletal dysplasia

Question 55

Q

What are the options for treatment of blounts

Answer

A

KAFO

indications

Stages I and II in children < 3 years (especially if unilateral)

bracing must continue for approximately 2 years for resolution of bony changes

Outcomes

Improved if unilateral
Poor results - bilateral, obese
Improvement should occur with-in one year

Proximal valgus osteotomy or growth modulation

children > 3 years
risk of recurrence is significantly lessened if performed before 4 years of age
stages V and VI
failure of brace treatment

***Make sure you overcorrect by 10 deg if doing an osteotomy

Question 56

Q

Risk factors for Blounts

Answer

A

A disease with medial physeal interuption, with progression to fibrous tissue and eventually physeal bar

Obesity

Hispanic/Black

Vit D

Early walking

Question 57

Q

What are you considerations in the treatment of infantile blounts?

Answer

A

Valgus/flexion/IR - proximal tibial osteotomy

Progression - will often need further sx

Growth moduation - lateral hemiephyseodesis

LLD - unilateral disease

Joint line - medial plateau elevation

Compartment syndrome - anterior fasciotomy

Bar resection - with PMMA or fat, for more severe disease

Question 58

Q

What are the radiographic findings of adolesenct blount

Answer

A

narrowing of the tibial epiphysis
widening of the medial tibial growth plate
occasional widening of the lateral distal femoral physis

metaphyseal beaking less commonly seen with adolescent Blount’s

Question 59

Q

How is adolesenct blount different from infantile?

Answer

A

Infantile - bilateral, severe, usually will progress (overcorrect osteotomy by 10-15 deg), < 3 can be treated with KAFO

Adolesenct - unilateral, no progression (do not overcorrect osteotomy), femoral valgus will not resolve,

Question 60

Q

Treatment options for adolescent blounts

Answer

A

lateral tibia and fibular epiphysiodesis
- indications
  - mild to moderate deformity with growth remaining
- outcomes
  - up to 25% may require formal osteotomy due to residual deformity
proximal tibia/fibula osteotomy
- indications
  - more severe cases in the skeletally mature
- No overcorrection
  - There should be no further progression of the deformity
- outcomes
- multiplanar external fixation following osteotomy allows gradual angle and length correction and decreases risk on neurovascular structures
- Will usually appear valgus due to big thighs, patient should be warned that their alignment is normal
distal femoral osteotomy or epiphysiodesis
- indications
  - for distal femoral varus deformity of 8 degrees or greater
- Should be done at the same time at the tibia
- Will not resolve as it will in infantile blounts

Question 61

Q

What is the diganosis of normal genu valgum

Answer

A

between 3-4 years - 20 degrees
after age 7 valgus - 12 degrees
after age 7 the intermalleolar distance should be <8 cm

Question 62

Q

What are the indications for observation of genu valgum

Answer

A

genu valgum <15 degrees in a child <6 years of age

(there is no role for bracing a pathological deformity because it will often progress, a physiological deformity will correct on it’s own; only blount’s is an acception to this)

Question 63

Q

What are your indications and options for surgery for genu valgum

Answer

A

<10 yo
- > 15-20° of valgus
>10 yo
- if line drawn from center of femoral head to center of ankle falls in lateral quadrant of tibial plateau
Options
- Hemiepiphyseodesis/tethering - guided growth
- osteotomy (no growth remaining)
- TSF if need combined angular and lengthening > 10% of bone

Question 64

Q

What is the epidemiology of pseudosubluxation of the spine

Answer

A

demographics

seen in children less than 8 years

location

C2 on C3 is most common
C3 on C4 is second most common

Pathophysiology

caused by the horizontal nature of the facet joints at younger ages

facet joints become more vertical with age

Answer 62

A

Swischucks line

spinolaminar line drawn from spinolaminar point on C1 to C3
spinolaminar point on C2 should be within 1.5 mm of spinolaminar line
helpful to differentiate pseudosubluxation from true injury

Answer 63

A

reduction of subluxation with neck extension
spinolaminar line within 1.5mm of C2
no history or physical findings of significant trauma
absence of anterior soft-tissue swelling

Answer 64

A

**trauma
retropharyngeal irritation (Grisel's disease)**

other less common causes include

Down’s syndrome
rheumatoid arthritis
tumours
congenital anomalies

Answer 65

A

Dynamic CT gold standard - rotational alignment won’t change

Answer 66

A

Nonoperative

soft collar, anti-inflammatory medications, exercise program

indications

subluxation present for < 1 week (traumatic or Grisel’s disease)
many patients probably reduce spontaneously before seeking medical attention

head halter traction and bracing

indications

subluxation persists > 1 week

technique

small amount (5 lbs.) usually enough
either in hospital or at home
muscle relaxants and analgesics may be needed

halo traction and bracing

indications

subluxation persists > 1 mos.

posterior C1-C2 fusion

indications

subluxation persists > 3 mos (or late diagnosis)
neurologic deficits present

Answer 67

A

Approach
- supine, flexed, abducted, ER
- incision along adductor longus 3cm from the pubic tubercle
- gracilis, adductor longus
- adductor brevis, adductor magnus
  - protect the posterior division of the obturator nerve
- Protect the medial circumflex artery, release the psoas
Pros
- directly addresses block to reduction
- can be used in patients under 12 months of age
- less blood loss with less soft tissue dissection
- less scar formation for future surgery
Cons
- unable to perform a capsulorrhaphy
- higher association of AVN due to disruption of the medial circumflex artery

Answer 68

A

No evdience for screening
Should get imaging < 6 months if family history, breech, clinical evidence
No need for U/S , 6 weeks
No need to re-examine a hip that was normal at initial imaging
No support for bracing an unstable hip with normal radiological markers
No evidence to show whether limited or delayed bracing is better
No evidence which brace works better
No evidence to monitor hip

Answer 69

A

Age at diagnosis plus 3 months

Answer 70

A

Extra-articular

iliopsoas contracture - constricts anterior capsule
Adductor contracture

Adductor tenotomy can improve abduction to maintain reduction

capsular constriction - hourglass contracture

Intra-articular

inverted labrum
Pulvinar
hypertrophied ligamentum teres

**Neo-limbus forms around the false acetabulum, it is not recommended to resect this

Answer 71

A

flexion of 100 deg.

abduction of 45 deg

neutral rotation

3 months

‘human position’
change cast at 6 weeks for hygiene

Answer 72

A

Physical
- Overall assessment, assess for torticollis, spina bifida, clubfoot, MA, other dislocations or contractures, LLD
- LLD, thigh assymetry
- barlow, ortelani (cannot use after 3 months)
- ROM - limited abduction
- trendeleburg, prominent GT, line from GT to umbelicus < 45
Ultrasound
- > 6weeks of age, < 6 month
- alpha angle > 60
- > 50% coverage
- assess psoas, capusule, boney acetaulum
- look for subluxation of the femoral head
Radiography
- Hilginreiners line, perkins line, shentons line
- AI - <25 deg over 6 months
- development of teardrop is a good sign for reduction

Answer 73

A

Arthrogram
- <5mm dye pool on AP
- psoas over the capsule will create the hourglass
Safe zone
- > 60 deg = increased risk of AVN
- adductor and psoas release

Answer 74

A

Mechanical axis = 87 deg (knee is in 3 deg of valgus)

LDFA (lateral distal femoral angle) = 84 deg (accounting for 6 deg of valgus of the femur)

Medial proximal tibia angle = 87 deg (accounting for 3 deg of varus)

Answer 75

A

Try to do multi-level surgery at one sitting

Trauma/fixation should allow them to WB immediately or they will never walk again

Answer 76

A

Screws across physis (? Reversisble)
Pheimster bone block
Drilling
Staples - reversible
8 plates - reversible

Answer 77

A

Age, amount of growth left
Projected growth discrepancy
Rotational profile, angular deformity
Multilevel surgery should be done at one sitting

(Especially in kids with syndromes and slow mobility because they won’t be able to rehab several surgeries)

Weight bearing

(Consider that in some kids with poor mobility, you need to use a strong construct because they won’t be able to rehab a long deficit from weight bearing)

**Can address angular deformity with modulated growth and then address LLD at maturity - this is an alternative to doing a TSF - Then decide based on LLD

Especially with a valgus knee you need to consider the peroneal nerve

Answer 78

A

peroneal nerve injury (valgus knee)

Prevention

perform a peroneal nerve release prior to surgery
gradually correct the deformity if there is a large defomity
utilize a closing wedge technique

*Hardware failure** - staples
*Overcorrection** - monitor every 3 months

Answer 79

A

50% are spontaneous
autosomal dominant (AD)
mutation in NF1 gene on chromosome 17q21
- codes for neurofibromin protein
negatively regulates Ras signaling pathway
neurofibromin deficiency leads to increased Ras activity
affects Ras-dependent MAPK activity which is essential for osteoclast function and survival
neurofibromatosis is the most common genetic disorder caused by a new mutation of a single gene

Answer 80

A

extremity deformities

congenital anterolateral bowing and pseudoarthrosis of tibia/ fibula and forearm
hemihypertrophy

spine involvement

scoliosis & kyphosis (dystropic and nondystropic)
atlantoaxial instability

Answer 81

A

screen early

non-dystrophic can become dystrophic

MRI and CT to assess dystrophic featrures, soft tissue mass, neuroma that can make approach difficult

nondystrophic treated as AIS

Answer 82

A

NF1 (von Recklinghaussen disease)

most common

NF2

associated with bilateral vestibular schwannomas

not associated with scoliosis

Segmental NF

features of NF1 but involving a single body segment

Answer 83

A

Developmental delay - most common genetic disease to cause learning disorder
Gross motor, speech, hearing can all be affected
Unidentified bright objects on MRI in brain with unknown clinical significance
Increased risk for
- Stroke
- Hypertension
- Heart disease
- vasculopathy

Answer 84

A

marfan

ehuler danlos

NF1

ank spond

Answer 85

A

Screen early

MRI and CT to rule out dystropic

monitor every 6 months - can progress to dystrophic

non-dystropic can be treated like AIS

Answer 86

A

a dystrophic curve will progress more rapidly when there are > 3 features

indications

dystrophic scoliosis
Monitor < 20 every 6 months, then fuse >40

Type 1 - no kyphosis
Type 2 - kyphosis >50
Indications for anterior (combined anterior and posterior fusion is controversial)

>90 deg curve
Rapid progression
>50 deg kyphosis

perform early in young children (< 7 yrs) with dystrophic curves

3 weeks pre-op traction shown to help

screen for cervical instability

use pedical screws, better even with deformation due to poor bone quality

complications

high rate of pseudoarthrosis with PSF alone (40%)
pseudoarthrosis rate still high with ASF&PSF (10%)
some recommend augmenting the PSF with repeat iliac crest bone grafting 6 months after the primary surgery

Answer 87

A

psuedoarthrosis

Answer 88

A

50% with anterolateral bowing have NF1
10% of NF1 have anterolateral bowing

Answer 89

A

anteromedial

hemimelia
loss of lateral rays of the foot

posteromedial

abnormal intrauterine positioning
- dorsiflexed foot pressed against anterior tibia
- will develop leg length discrepancy
associated with calcaneovalgus deformity

anterolateral

NF1
CPT

Answer 90

A

The pathology for NF1 and CPT are the same - increased fibrous tissue, decreased functional osteoclasts (RAS pathway)
Deficiency of the periosteum to differentiate myofibroblasts and chondrocytes

Answer 91

A

worse prognosis associated with age and presence of fracture

Bracing - if no fracture

bone grafting with surgical fixation

in bowing with pseudoarthrosis or fracture - prognosis depends on correction of bowing and stabilization

aggressive nonunion debridement, shortening and compression across nonunion

IM nail vs ex-fix (no plate on pathological bone)

*osteotomize the fibula and fix with a k-wire as well

plan to never take the fixation out

amputation with prosthesis fitting

indications

three failed surgical attempts (or when patient asks for it)

Syme’s often superior to BKA due to atrophic and scarred calf muscle in these patients

Answer 92

A

X3 attempts or when patient asks for it

>5cm LLD

unacceptable deformity

prolonged hospitalization

Answer 93

A

refracture

LLD

Answer 94

A

** resection of pseudarthrosis to grossly normal bone**
correction of deformity
bone grafting

With BMP-Rh2 (controversial)
Iliac crest graft

stabilization of the remaining segments

IM nail vs ex fix (no plates)
Stabilize the fibula also

if <8 yo or for distal fracutre can use a nail crossing the ankle threw the calc

Answer 95

A

CPT/NF1

Fibrous dysplasia
OM
Fibrosarcoma
Fibular hemimelia

Answer 96

A

Intrauterine positioning

often associated with calcvalgus foot

often get LLD later, need to follow and perform epiphyseodesis at appropriate time

Answer 97

A

75%

lobster-claw hand
preaxial polydactyly
ulnar aplasia

Answer 98

A

fibular hemimelia (linked to sonic hedge hog gene)

Answer 99

A

anteromedial tibial bowing
- most common cause is fibular hemimelia
ankle instability
- secondary to a ball and socket ankle
equinovalgus foot deformity
tarsal coalition (50%)
absent lateral rays
femoral abnormalities (PFFD, coxa vara)
cruciate ligament deficiency
genu valgum
- secondary to lateral femoral condyle hypoplasia
significant leg length discrepancy
- shortening of femur and/or tibia

Answer 100

A

Look
- Shortening of the affected limb
- anterolateral bowing of the tibia
- prominent fibula
Feel
- flexion contracture
- ability to extend knee
- stability of knee
**Foot **
- rigid equinovarus

Answer 101

A

knee disarticulation followed by prosthetic fitting
- indications
  - complete absence of the tibia
  - no active knee extension present (most cases)
tibiofibular synostosis with modified Syme amputation
- indications
  - proximal tibia present with intact extensor mechanism and minimal flexion contracture
Syme/Boyd amputation
- indications
  - ankle diastasis

Answer 102

A

congenital fibular deficiency

Acherman
- Ia - partial deficiency, short fibula
- Ib - 30-50% absenct, does not support ankle
- II - complete absence
Birch
- type 1 - functional foot (>3 rays)
  - A - <6%
    - nothing, orthosis
    - epiphyseodesis
  - B - 6-10%
    - lenghening
    - epiphyseodesis
  - C - 11-30%
    - may require more than one lengthening or orthosis
  - D - >30% difference
    - usually requires amputation
- Type 2- non-funcational foot (<3rays)
  *

Answer 103

A

Necessary Imaging
- AP bilateral lower extremities
  - look for associated deformities
- CT can help assess LLD
- MRI if there is functional instability
- Echo, renal U/S
Invovle multi-disciplinary team
- PT, psych, prosthetist
- have the families visit clinics to see both reconstructive and amputation options
- emphasize amputation is not a failed option
Principles
- Depends on the functionality of the foot and the LLD
- keep in mind ACL def when treating LLD
  - no role for ACL reconstruction
- Not advocated to do tibial osteotomy to correct deformity at the time of amputation
- May need a distal femoral osteotomy later to correct valgus
Lengthening
- contralateral epiphyseodesis
- one at 5cm, one at 7cm
- may need a final one
Genu Valgum
- <15 deg can do epiphyseodesis alone
- >15 deg might need to do an osteotomy
- need to correct for amputations as well to ensure proper prosthesis fitting
Amputation
- Indications
  - >30% LLD
  - >5cm at birth LLD
  - functional UE
- Boyd or Syme
- Done when they start to walk

Answer 104

A

Amputation outcomes
- higher satisfaction
- lower readmission
- fewer complications
Symes amputation
1. Posterior migration of the heel pad
2. Reformation of the calcaneus (ie, painful stump)
3. Wound infection
4. Heel pad slough
5. Fitting adjustment problems
Boyd amputation (maintains more height)
1. Delayed calcaneotibial fusion or nonunion
2. Calcaneal migration and malalignment
3. Wound infection
Limb lengthening
1. Pin tract infections
2. Peroneal nerve injury Tibial/femoral fractures
3. Severe depression
4. Nonunion of the osteotomy site Joint stiffness
5. Joint subluxation
6. Persistent or recurrence of knee and foot deformities
7. Residual limb length inequality

Answer 105

A

Functional foot
LLD <30% at maturity

Answer 106

A

Tibial hemimelia has a known genetic, AD cause where fibular hemimelia is sporatic

Answer 107

A

40 deg fem anteversion at birth, masked by femoral ER at times; internal tibial torsion

tibia externally rotates and becomes normal by age 6

femural anteversion resolves by age 10

Answer 108

A

ER Hip/IR hip
- > 70 IR, <20 ER = fem anteversion
thigh-foot-axis (5 IR)
- -10 = internal torsion
transmalleolar axis (5 IR)
heel bisector angle
foot progression angle (-5-20)

Answer 109

A

0-1 yo - MA

1-3 yo - internal tibial torsion

>3 yo - femoral anteversion

Answer 110

A

*normal** - heel bisector line through 2nd and 3rd toe webspace
*mild** - heel bisector line through 3rd toe
*moderate** - heel bisector through 3rd and 4th toe webspace
*severe** - heel bisector through 4th and 5th toe webspace

Answer 111

A

Hip Rotation
- Infants - 30 IR, 70 ER
- 10yo - 50 IR, 45 ER
- at 10yo - IR > 80 with ER < 10 is indication for surgery
Tibia
- Infant - IR, resolves by 4yo
- 6yo - ER 10 deg
- at 80yo - ER >40 deg is indicatin for surgery

Answer 112

A

Simple - MTA
Complex - MTA, lateral shift of midfoot
Skew foot - MTA, valgus hindfoot
Complex skew foot (serpentine foot) - MTA, lateral shift, valgus hindfoot

Answer 113

A

pain
limb length discrepancy
progressive deformity
family history positive for rickets/skeletal dysplasias/mucopolysaccharidoses
limb rotational profiles 2 standard deviations outside of normal

Answer 114

A

Flexible deformities that can actively be corrected to midline - No treatment required

Flexible deformities that can passively be corrected to midline - Serial stretching by parents at home

Rigid deformity with medial crease - Serial casting with the goal of obtaining a straight lateral border of foot

usually will improve in 6-8 weeks of casting
some will recurr by 2-4 yo

Answer 115

A

Indications:

failed non-op

severe deformity and difficulty with shoe-wear

> 5yo

Techniques:

*Soft tissue procedures alone are no longer recommended due to soft tissue necrosis

*lateral column shortening -** done with cuboid closing wedge ostetomy
*medial column lengthening -** includes a cuneiform opening wedge osteotomy with medial capsular release and abductor hallucis longus recession (for atavistic first toe)

Answer 116

A

a benign condition that resolves spontaneously in 90% of cases by age 4
another 5% resolve in the early walking years (age 1-4 years)

Answer 117

A

late pregnancy
first pregnancies
twin pregnancies
oligohydramnios

Answer 118

A

Adduction deformity of the great toe characterized by
- deformity at metatarsophalangeal joint
- short thick 1st metatarsal
- firm band-like abductor hallucis muscle
Associated conditions
- often associated with polydactyly – duplication of the great toe
Must be differentiated from metatarsus adductus
- Lateral border of the foot is normal
- More rigid than MA
Early casting is used, associated with hallux valgus
Severe deformity is indication for osteotomy

Answer 119

A

Poorly understood why this develops
No good measurement to define skewfoot vs MA
Surgical treatment is only indicated if function is severely limited
opening wedge and closing wedge osteotomies
- indications
  - indicated if serpentine deformity is symptomatic and significantly limits function
  - operative treatment is difficult and often times deformity is accepted and observed
technique
- calcaneal osteotomy for hindfoot valgus
- possible midfoot osteotomies to correct midfoot and forefoot deformities
- multiple metatarsal osteotomies with forefoot pinning and tarsometatarsal capsular release (Hamen procedure)

Answer 120

A

>6yo

>10 deg IR

rarely causes issues

will not get worse with growth

Answer 121

A

Will increase with age and is associated with malalignment and patellofemoral syndrome

“miserable malalignment” syndrome
- a condition defined as external tibial torsion with femoral anteversion
Osgood-Schlatter disease
osteochondritis dessicans
early degenerative joint disease
- an association between external tibial torsion and early degenerative joint disease has been found
neuromuscular conditions
- may be found with neuromuscular conditions such as myelodysplasia and polio

Answer 122

A

indications
- >8yo
- >40deg (3 SD above the mean)
technique
- supramalleolar osteotomy
  - cross pins for young children
  - IM nail for skeletally mature
- Fibular osteotomy for more severe deformity

Answer 123

A

indications
- < 10° of external rotation on exam in an older child (>8-10 yrs)
- rarely needed - only if there is severe comprimised function - tripping, inefficient gait
  - Can lead to inefficient abductor function
Derotational femoral osteotomy
- typically performed at the intertrochanteric level
- consider correction of varus if present
- amount correction needed can be calculated by (IR-ER)/2
- can be done prone or supine depending on what other procedures you will also be performing

Answer 124

A

CNS
- CP
- Frederichs ataxia (often can be first diagnosis)
Spinal Cord
- Myelodysplasia, syrinx/synringomyelia (unilateral), tethered cord (unilateral), tumors, GBS
- Polio (more historical)
Peripheral neuropathy
- CMT/HSMN - most common cause
  - Result of a number of mutations resulting in dymyelination
  - Most common is X-linked; Peripheral-myelin-protein 22 (PMP-22)
Aquired - more common in adults
- Tibial fracture/compartment syndrome, sciatic nerve injection, peroneal neuropathy
Residual clubfoot

Answer 125

A

Plain radiographs
- AP - varus
- Lateral
  - meary’s angle = 0
  - calcaneal pitch = 30 deg
EMG
- concerns of CMT or HSMN
- may also require genetic testing
Spine MRI
- unilateral
- progressive deformity

Answer 126

A

Activity adjustments
- A runner with a cavus foot needs longer time to recover from injury and is more at risk of injury
Accomodative orthotics with high shoe box for claw toes
- Good running shoes for runners
PT
- Quads strengthening
- Can help decrease the load the ankle sees when running
- (2.5X body weight, alleviated by eccentric quads contracure)
Baclofen, diazepam, Botulinum toxin
- For neuromuscular/CP

Answer 127

A

Transfered tendons lose one grade, need at least grade 4
should be in straight line of pull
try to adhere to bone rather than tendon
fixed deformites should be corrected first
iatrogenic problems can happen in neuromuscular (ie; CP tib ant transfer; will get a new contracture; better to do a split tendon transfer)

Answer 128

A

hand anomalies
diastrophic dwarfism
arthrogryposis
tibial hemimelia
Myelomeningocele

Answer 129

A

midfoot Cavus (tight intrinsics, FHL, FDL)
forefoot Adductus (tight tibialis posterior)
hindfoot Varus (tight tendoachilles, tibialis posterior)
hindfoot Equinus (tight tendoachilles)

Answer 130

A

talar neck is medially and plantarly deviated
- Some believe that this is the primary deformity as it is the most consistent
- Talus grows medially toward the navicular
the calcaneus is in varus and rotated medially around talus
- Deltoid, spring ligament and PTT keep calcaneous varus
- Achilles keeps heel in equinus
the navicular and cuboid are displaced medially
- Cuboid is morphologicall normal, just displaced
- Navicular is tethered by the above as well as the knot of henry, capsule

Answer 131

A

talus and calcaneus are parallel; usually talocalcaneal angle is > 35 deg
dorsiflexion view of turco
- flat talar head
- talocalcaneal angle < 35 deg

Answer 132

A

talocalcaneal angle (Kite's) is \< 20° (normal is 20-40°) 
talus-first metatarsal angles is negative (normal is 0-20°)

Answer 133

A

CLUBFOOT

Month 1-4 - PONSETI CASTING
- Weekly Serial Casting (with knee in 90° of flexion )
- First correct cavus by aligning 1st MT with remaining metatarsals
  - This requires supination of the forefoot, it is pronated; usually take one week
- Then correct adduction and heel varus by rotating calcaneus and forefoot around talus (head of talus acts as fulcrum)
  - This needs to be done prior to equinus correction, otherwise the calcaneus will be locked under the talus
  - Usually takes 4 weeks; cast application while feeding can distract the infant
- Achilles tenotomy at week 8 (required in 80%)
  - Then correct equinus with TAL (If you don’t wait until the navicular is reduced then you will get a rockerbottom foot)
  - Perform when foot is 70° abducted and heel is in valgus
  - Ponseti method is complete transverse cut
  - Can be done in clinic for a small child, or in the OR for a larger/older baby for better analgesia and better case moulding
  - No stiches need, just a light dressing, then the cast is applied
  - Cast in maximal dorsiflexion and abduction after TAL, overcorrection will prevent recurrence
- This cast is left on 3-4 weeks, more in a larger child
Month 4-8 - Foot abduction orthosis (FAO)
- full time until ~ 1 year of age
- nights only from age 1 to ~4
- With Denis Browne bar in external rotation (_70° in clubfoot and 40° in normal foo_t)
  - Fit FAO on day of TAL
  - Normal to be irritable when casts come off, important to explain this to parents and emphasize risk of recurrence
2-4 years - Anterior tibial tendon transfer
- at 2 yrs of age (10-20% will require)
- 10-20% will need TA transfer with or without repeat TAL for recurrent supination, varus, and/or equinus
- Occurs due to incomplete reduction of navicular, changes tib ant from a dorsiflexor to a supnator
- 4-6 weeks repeat casting
  - Keep tib ant under the retinaculum to prevent bowstring
  - +/- TAL

Answer 134

A

Re-start casting
Indications for surgery
- resistant feet
- rockerbottom from casting
Surgical options
- ST release and tendon transfers
  - Posteromedial release - plantar fascia, PTT lengthening
    - Deltoid ligament, spring ligament, knot of henry
    - Abductor Hallucis is released to get access to these structures
    - Whether to release subtalar or ankle capsule depends on the surgeon
  - Tibialis anterior transfer
    - To medial cuneiform (only if ossified)
    - For neuromuscular kids should use a split transfer
  - Repeat TAL
    - Posterior talo-fib and tib-fib ligaments
    - Make sure to do your silverskiold
    - dosiflexion osteotomy
- Medial opening or lateral closing
  - 3-10yo
  - first ray or cuniform
- Triple arthrodesis
  - 8-10 yo with refractory
- Talectomy
  - 8-10 with insensate foot

Answer 135

A

Complications with nonoperative treatment
- deformity relapse
  - relapse in child < 2 years
    - noncompliance with FAO
    - Inappropraite technique, missed neurological or paralytic foot
    - treat with repeat casting
  - relapse in child > 2 years
    - treat initially with casting
      - consider TA tendon transfer
      - consider repeat TAL
- dynamic supination
  - treat with whole anterior tibial tendon transfer
Complications with Surgical Treatment
- residual cavus
  - result of placement of navicular in dorsally subluxated position
- pes planus
  - results from overcorrection
- undercorrection
- intoeing gait
- osteonecrosis of talus
  - results from vascular insult to talus resulting in osteonecrosis and collapse
- dorsal bunion
  - caused by dorsiflexed first metatarsal and overactivity of anterior tibialis

Answer 136

A

Associated syndromes
- cerebral palsy (usually spastic hemiplegia)
- Duchenne muscular dystrophy
- residual clubfoot deformity
- tibial deficiency (hemimelia)
Muscle imbalance include spasticity of
- tibialis posterior and/or tibialis anterior
- gastroc-soleus complex
- abscense of ligamentous laxity

Answer 137

A

gait analysis
- diagnosis made with gait analysis showing
- overactivity of tibialis posterior and/or
- overactivity of tibialis anterior
**confusion test **
- helps to distinguish AT vs PT as the primarily involved muscle
- patient performs active hip flexion against resistance while seated
  - in a positive confusion test, the tibialis anterior fires with active hip flexion and is typical in patients with CP
  - if the foot supinates with dorsiflexion, tibialis anterior is most likely contributing to the equinovarus deformity

Answer 138

A

TAL with tibialis posterior split transfer to peroneus brevis
- indications
  - spastic hemiplegia in patient ages 4 to 7
  - flexible equinovarus hindfoot
  - tibialis posterior spastic in both stance and swing phase (continous activity)
- technique
  - reroute half of tendon dorsally and insert into peroneus brevis
Rancho procedure
- indications
  - overactive anterior tibialis
  - flexible equinovarus deformity
- technique
  - split anterior tibialis transfer to cuboid with TAL and intramuscular lengthening of the posterior tibial tendon
**Calcaneal osteotomy **
- indications
  - is required in a rigid hindfoot varus deformity
- technique
  - lateral closing wedge osteotomy to incur valgus to the heel

Answer 139

A

Common foot deformity seen with
- spastic diplegic and quadriplegic cerebral palsy
- fibular hemimelia
Deformity consists of
- midfoot abduction
- heel valgus
- equinus contracture
- typically bilateral
Muscle imbalance includes (see overview of foot deformity muscle imbalances)
- spasticity of
  - peroneals
  - gastoc-soleus complex
- weakness of
  - ligamentous laxity
  - posterior tibialis
  - anterior tibialis

Answer 140

A

bracing and physical therapy
- indications
  - flexible deformities
- technique
  - ankle foot orthosis or supramalleolar orthosis
botox injections

Answer 141

A

soft tissue procedures
- TAL
- peroneus brevis lengthening
**calcaneal osteotomy **
- medial slide ostetomy or calcaneal lengthening osteotomy
lateral column lengthening procedure
- performed through calcaneus or cuboid
Grice procedure
- extra-articular subtalar arthrodesis
- place ICBG in lateral subtalar joint to block valgus
- does not interfer with tarsal bone growth
subtalar arthrodesis
- stabalizes subtalar joint in correct alignment without fusion
- implant or spacer is placed laterally in the subtalar joint to prop open

Answer 142

A

Pressure sores
Compartment syndrome
- Univalve plaster
  - 50% release
  - Then webril is 20% release
- Bivalve fiberglass
Atrophy, cartilage degredation, ligament weakness
- Don’t immobilize for too long
- Functional casting when appropraite
- Patients with neuromuscular disease are at increased risk of ostetopenia and atrophy
  - Should immobilize them for less time

Cast saw burn

Answer 143

A

Complication prevention
- Plaster application
  - Don’t apply fiberglass over curing plaster
  - Don’t put plaster on a pillow
  - Use recommended water temperature
- fiberglass
  - Stretch and relax for fiberglass
  - Don’t leave sharp fiberglass edges
General
- Don’t go past the MC heads, limits fingers
- Don’t end at the fibula = peroneal nerve
- Use safe position for the hand
- Hip spica
  - Don’t put lower limb first and use to traction
  - Can cause compartment syndrome and popliteal occulsion
Cast application
- Appropriate padding
  - Too much padding can cause loose cast, skin irritation and loss of reduction
- Cast should look like and oval
  - Optimal width:height = 0.7
- Don’t change position of the cast after applied
  - Weakens the cast
  - Puts pressure in the flexion crease

Answer 144

A

WB AP/lateral, oblique, harris (TC)
Lateral
- Meary’s angle - 0 deg
- Calcaneal pitch - 20 deg
- rule out tarsal coalition, verticle talus
- assess where break in foot is
AP
- Ist MT-talus angle - 20deg
- rule out accessory navicular
oblique
- best image to see a Cal-nav coalition

Answer 145

A

Navicular, spring ligament, anterior facet calcaneus

Answer 146

A

Non-op
- always do this first - counsel parents should resolve as child grows and will not affection function**
- supportive shoewear
- rigid orthoses
- Achilles stretching - if tolerated
Operative - only indicated with prolonged resistance to non-op and pain
- Address achilles as per silverskiold
- Calcaneal lengthening osteotomy
  - +/- brevis lengthening (not longus, lengthing longus helps to pronate the foot and encourge arch formation)
  - If very severe can consider also a calcaneal sliding osteotomy
- +/- medial cuneiform osteotomy to address the forefoot supnation

Answer 147

A

**calcaneal lengthening osteotomy (Evans) **
- with or without a cuneiform osteotomy and peroneal tendon lengthening
Calcaneal lateral opening wedge osteotomy (Dwyer)
**Sliding calcaneal osteotomy **
- corrects the hindfoot valgus
plantar base closing wedge osteotomy of the first cuneiform
- used to correct the supination deformity
- Can be combined with an opening wedge osteotomy of the cuboid
“Triple C”
- Sliding calcaneus
- Opening cuboid
- Closing cuneiform

Answer 148

A

Differntial
- Calcaneovalgus
- posteromedial bowing
- oblique talus
- verticle talus
- paralytic foot deformity
  - L5 myelomeningocele
imaging
- AP/lateral
  - CVT will have valgus midfoot and increased Talo-calc angle (>40 deg)
  - CVT = meary’s angle = 20 deg
- forced plantarflexion
  - the navicular will reduce in CV foot (MT will line up with talus)
- Dorsiflexion
  - in CVT the talus will remain in equinus

Answer 149

A

Will resovle on it’s own
May develop LLD if associated with posteriomedial bowing

Answer 150

A

Accessory Navicular
Convervative
- activity restriction, shoe wear modification, NSAIDS, short perior immobilization
- most will become asymptomatic at skeletal maturity
Resection of accessory navicular
- will not correct flatfoot
- MRI to assess PTT - if needs to be transfered beform Kindler procedure (transfer PTT to inferior navicular)
- otherwise straight incision, incise PTT longitudinal, resect with osteotome, then close PTT)

Answer 151

A

CVT or “Persian slipper” - 50% will have associated neuromusular conditions

myelodysplasia (common)
arthrogryposis
diastematomyelia
chromosomal abnormalities

Answer 152

A

similar to vertical talus but reduces with plantarflexion
usually improves with conservative treatment, very few will require CRPP for residual subluxation

Answer 153

A

Deformity
- fixed hindfoot equinovalgus
  - due to contracture of the Achilles and peroneal tendons
- rigid midfoot dorsiflexion
  - secondary to the dislocated navicular
- forefoot abducted and dorsiflexed
  - due to contractures of the EDL, EHL and tibialis anterior tendons
Casting to stretch lateral tissues - reverse of clubfoot
- supnated forefoot, abducted midfoot, heel valgus
- equinus is the same
Closed or open reduction with pinning
- once the foot is stretched, the navicular is reduced to the talus, pinned and casted
- usually require TAL
- +/- tib ant lengthening (limited plantarflexion)
- +/- tib ant transfer to talus (>2yo)
- Cast for 8 weeks, remove pin, into boots and bars
Talectomy for resistent cases

Answer 154

A

demographics
- incidence varies from 1%-6%
- most coalitions found are incidental and asymptomatic
Genetics
- AD, with partial penetrance
age of onset (when pain develops)
- Calcaneonavicular - 8-12 years old
- Talocalcaneal - 12-15 years old

Answer 155

A

Tarsal coalition
Associated conditions
- fibular deficiency
- Apert syndrome
Differential Diagnosis for Rigid flatfoot
- Verticle talus
- Peroneal spastic flatfoot - secondary to another condition such as JRA
- Trauma or infection

Answer 156

A

Lateral
- C sign
- anteater sign
Internal Oblique (45deg)
- best view for CN coalition
Harris view
- TC coalition
Saltzman standing view
- hindfoot varus
CT
- best way to assess coalition
- assess for other coalition
- rule out bilateral
MRI only necessary if fibrous lesion

Answer 157

A

bilateral CT to assess for other coalition and extent of coalition
Trial conservative management
- rest, orthotics
- many will improve
Associated Deformities
- Lateral column lengthening (the group that says this - for > 16deg valgus)
  - If the patient is old enough they will have a more developed flat foot
  - Some groups won’t take down the coalition
- Achilles or gastro lengthening
  - Can do a silverskiold intra-op once you’ve taken down the coalition
surgical resection of coalition with interposition of fat graft (TC) or extensor digitorum brevis (CN)
- indications
  - resistant cases when non-operative management fails to relieve symptoms
- outcomes
  - 80% will experience pain relief
  - High rates of recurrence, but are lower with tendon interposition (and lower with fat graft than nothing)
  - Predictors of poor outcome with resection
    - Coalition > 50% of the posterior facet (not middle facet
    - Hindfoot valgus 16 deg
    - Narrowing of the posterior TC facet
subtalar arthrodesis
- indications
  - role has not been well established
  - Used to be done more often, but now we have good results with surgical resection
triple arthrodesis (subtalar, calcaneocuboid, and talonavicular)
- indications
  - advanced coalitions that fail resection

Answer 158

A

Poor prognosis
- >16 deg valgus
- >50% posterior facet
Supine with tourniquette
medial incision underneath FHL centered over the sustentaculum
identify the sheath of FHL and identify the NV structures
Incise the FDL sheath and retract it inferiorly
identify the anterior and posterior facets
use a burr to debride the posterior facet ensuring to not drift superiorly or inferiorly
- assess the mobility of the subtalar joint
put bone wax on the bleeding bone and use fat interposition
- can use FHL if you are doing a repeat debridement
Repair the FDL sheath with closure
Reasons for failure
- >16 deg valgus
- >50% posterior facet
- incomplete resection
- lack of interposition
- lack of identification of other coalitions

Answer 159

A

Incision over the lateral side of the foot between the EDC and the peroneals
Fatty tissue and EDB are elevated to expose the anterior process of the calcaneus
Use small osteotomes to remove a trapezoidal peice of bone
- make sure to remove the entire coalition
Fat interposition
- taken from a 2cm transverse incision in the buttock crease
Release the tourniquette, get appropriate hemostasis and sew EDB over the interposition

Answer 160

A

Poor prognosis
- Coalition > 50% of the posterior facet (not middle facet)
- Hindfoot valgus 16 deg
- Narrowing of the posterior TC facet
Complications
- incomplete resection
- recurrence
- wound complications
- injury to adjacent cartilage

Answer 161

A

Kholer’s disease

usually 4-7 yo
M>F
centeral 1/3 is watershed zone prone to stress # and AVN
intermittent symptoms for 1-3 years, treat with NSAIDS, self-limiting
often have deformity but no clinical effect
Can treat with SLC when painful

Answer 162

A

Sever’s disease

sclerosis can be normal as well, as seen below
overuse with running, jumping
often seen during peak growth velocity
MRI
- stress fracture
- lytic lesion
- infection
Treatment
- activity modificaiton
- Achilles tendon stretches (can help decrease recurrence)
- ice application before and after athletic endeavors
- use of heel cups or heel pads
- NSAIDs
- short leg cast immobolization of persistent pain

Answer 163

A

Iselin’s

traction apophysis base 5th MT
8-13yo, girls and boys
self-limited

Answer 164

A

Atavastic great toe (Congential Hallux valgus)

Adduction deformity
- deformity at metatarsophalangeal joint
- short thick 1st metatarsal
- firm band-like abductor hallucis muscle
Associated conditions
- polydactyly
- differentiate from metatarsus adductus
Adductor can be released in rare cases

Answer 165

A

Polydactyly of the foot
- AD
- usually the lateral ray
Treatment
- ablation of lateral digit
- 9-12 months

Answer 166

A

Overlapping 5th toe

congential
bilateral
problems with shoewear
Anatomy
- smaller
- adducted, flexed (EDL is contracted)
- rotated
- MTP dislocated dorsally
Butler Procedure
- Racket incision
- Tenotomy of extensor
- dorsal capsulotomy - dorsal medially
- Allows toe to flex, reduce and rotate
- No tension, no pinning
McFarland Procedure
- syndactelization of 4th toe

Answer 167

A

Congenital Curly Toe

flexion and varus deformity of the interphalangeal joints
- flexor digitorum longus (FDL)
- flexor digitorum brevis (FDB) is the most common cause
True incidence and natural history unknown
- usually involves lateral three toes
- usually bilateral
__Usually requires no treatment
Operative - releases FDL
- nailbed deformity
- children > 3yo

Answer 168

A

Local Gigantism

form of gigantism usually isolated to fingers or toes “macrodactyly” but may affect an entire limb
congenital causes:
- neurofibromatosis
- proteus syndrome
- klippel-Trenaunay-Weber
acquired causes:
- amyloidosis
- elephantiasis (filariasis)
- av malformation
- tumor
- acromegaly
Treatment
- dependant on case
- epiphyseodesis vs reconstruction vs amputation

Answer 169

A

Brachymetatarsia

Usually 4th
- can lead to hallux valgus if 2nd ray
Usually bilateral
Treatment
- Acute lengthening < 15mm +/- shortening of other MT (see below)
  - extensor tenotomy, capsulotomy
  - k-wire to appropraite MT parabola
  - plate and iliac crest graft
- Gradual distraction > 15mm
  - extensor tenotomy, capsulotomy
  - two mini pins proximal and distal
  - can use cuneiform or cuboid

Answer 170

A

General rules in the treatment of brachymetatasalgia

Answer 171

A

larger head size relative to the trunk
- folcrum is at C2-3
**increased physiologic motion **
- horizontally oriented facet joints
- shallow facet joints
- elevated ligamentous laxity
- weak ligaments and capsule
- weaker muscles
Need a backboard with a head cut-out
Injury >C3 more common (87%)
- higher incidence of neuro injury (35-60%) and mortality
- injuries at C1 lead to a mortality rate of 17%
- injuries at C4 lead to a mortality rate of ~4%
The spinal column (but not the cord) can stretch 5cm without rupture

Answer 172

A

Ossify - 3yo

Fuse - 6yo

Answer 173

A

prevertebral swelling < 2/3 of adjacent vertabral width
smooth contour lines of
- anterior vertebral bodies
- posterior vertebral bodies
- spinolaminar line (inside lamina)
- tips of spinous process
parallel facet joints
- normal retropharyngeal space
  - < 6 mm at C2
  - < 22 mm at C6
retrotracheal space < 14 mm
atlanto-dens interval < 5 mm in children and < 3 mm adolescents
absent vertebral body wedging
- 7% of normal children have a wedge shaped C3 vertebral body
absence of of cervical lordosis
- loss of cervical lordosis may be found in 14% of normal children
C2-3 or C3-4 pseudosubluxation < 4mm
- considered normal as long as the posterior laminar line is contiguous

Answer 174

A

SCIWORA

20-30% of pediatric c-spine injuries
Can present in delayed fashion - often more severe (50%)
Soft tissue injury = 40-70% of pediatric injury
Column can stretch 5cm, cord can stretch < 1cm
Management
- MRI to assess cord
  - Complete cord disruption
  - Major cord hemorrhage
  - Minor cord hemorrhage
  - Edema
  - nothing
Treatment - no consensus
- Rest, monitor
- Pressure support
- Immobilization with external collar 12 weeks
- Progressive mobilization 12 weeks
Prognosis
- Complete injury worse prognosis (worse at presentation = worse prognosis)
- Incomplete injury has good recovery

Answer 175

A

BDI < 12.5mm is normal (best one and easiest to remember)

Answer 176

A

Atlano-occipital dissociation

High mortaility rate with neuro injury
MRI or CT to assess boney anatomy
Stabilize initially with halo or minerva
Posterior Occiput to C2 fusion
- Maintain C-spine precautions
  - head board cutout < 8yo
  - fiberoptic intubation
  - sandwich to flip
  - neuromonitoring
- Be aware of where you are fusing head
- Post-op minerva for docile or halo for active kid

Answer 177

A

Atlantoaxial instability

Anatomy
- C1-2 transvere ligament (runs between lateral mass) is the primary stabilizer
- Alar ligaments are secondary
Imaging
- ADI > 5mm = TL rupture (or >3mm in adolescents)
  - Alar ligaments can prevent cord compression
- ADI > 10mm = alar ligament rupture
Treatment (with or without fracture)
- Closed reduction in extension
- External immobilization
  - ADI <10mm
  - No neurological compromise
- C1-C2 fusion
  - Gallie/brooks in young children (not big enough for screws)
  - If old enough can treat as adult
  - Halo to augmnet

Answer 178

A

Downs
Larsens
Morquio

plus many many other dysmorphic syndromes or ligamentous laxity

Answer 179

A

Atlantoaxial Rotatory Displasment (AARD)

trauma
retropharyngeal irritation (Grisel’s disease)
- caused by upper respiratory infection or retropharyngeal abscess
- thought to be linked to lymphatic edema in area of cervical spine
other less common causes include
- Down’s syndrome
- rheumatoid arthritis
- tumours
- congenital anomalies

Answer 180

A

Atlantoaxial rotatory displacement

Type 1
- Unilateral facet subluxation with intact transverse ligament.
- Odontoid acts as a pivot point and there is no anterior subluxation.
- Most common and benign type.
Type II
- Unilateral facet subluxation with 3 to 5 mm of anterior displacement.
- Injured Transverse ligament
- One facet acts as pivot point and one lateral mass is displaced
Type III
- Bilateral anterior facet displacement of > 5 mm.
  - Rare with higher risk of neurologic involvement or instantaneous death.
- Both lateral masses are displaced
Type IV
- Posterior displacement of atlas (C1).
- Rare with higher risk of neurologic involvement or instantaneous death

Answer 181

A

Atlantoaxial Rotatory Displacement

Radiographs
- recommended views
  - AP, open-mouth odontoid
    - look for variation in size and distance from midline of C2 lateral masses (reflects rotation)
  - lateral
    - facet joint appears anterior and wedge shaped instead of normal oval shape
  - cervical flexion & extension views
    - may be useful to exclude instability
    - may be difficult due to position of head and resisted neck motion
Dynamic CT
- is diagnostic gold standard
- take CT with head straight forward, and then in maximal rotation to right and left
- will see fixed rotation of C1 on C2 which does not change with dynamic rotation
- look for anterior displacement, this is more concerning
Nonoperative
- soft collar, anti-inflammatory medications, exercise program
  - subluxation present for < 1 week (traumatic or Grisel’s disease)
  - follow-up closely and ensure reduction
    - can discontinue bracing if reduced
  - Then one more check to make sure they stay reduced
- halter traction and bracing 4-6 weeks
  - subluxation persists > 1 week, or if they don’t reduced with soft collar
  - technique - usually admitted
    - head halter
    - small amount (5 lbs.) usually enough
    - muscle relaxants (diazepam) and analgesics may be needed
  - immobilize for 6 weeks
    - ensure the reduction is maintained once you take the collar off
    - anterior displacement more concerning
- halo reduction
  - subluxation persists > 1 mos (or late diagnosis)
  - neurologic deficits present
  - apply halo
    - start light, will require sedation
    - can continue traction for up to 3 weeks
  - prognosis is gaurded, often will not reduce or will re-subluxate following discotinuation of immobilization
- posterior C1-C2 fusion - gallie/brooks
  - unable to reduce at any stage
  - continued anterior displacement despite reduction
  - >3 months

Answer 182

A

unstable cervical spine with spinal cord injury
atlantoaxial instability
chronic atlantoaxial rotatory displacement (AARD)

Answer 183

A

Odontoid fracture

Acutely
- Usally seen on lateral
- Can get CT to assess
- Flex-ex views if appropriate
- MRI - static if chronic to assess cord
  - Then can do dynamic MRI to assess stability of the fragment
Treatment
- Reduction with Halo or Minerva for 3 months
  - Acute
  - Confirm reduction with XR
  - Confirm healing with fle-ex views
Posterior C1/2 fusion
- If no decompression necessary
- Brooks/Gallie - if young
- C1/2 lateral mass screws if older
Posterior Occiput-C2 fusion
- For chornic injuries that you need to decompress posteriorly
- Otherwise hard to get your bone graft in

Answer 184

A

AVN: (DDH or Perthes)
Fibrous Dysplasia
SCFE
Rickets
Osteomyelitis of Hip and Septic Arthritis
traumatic
Paget’s Disease

Answer 185

A

Cleidocranial Dysplasia
Chondrodysplasia Punctata
Metaphyseal Chondrodysplasia
Gaucher’s Disease
Multiple Epiphyseal Dysplasia
Proximal Focal Femoral Deficiency
Spondyloepiphyseal Dysplasia Congenita

Answer 186

A

Pseudosubluxation

Up to 40%/4mm subuluxation
- C2/3 most common
- then C3/4
Children <8yo
- due to physiological changes
Imaging
- Swischuk’s line
  - spinolaminar line drawn from spinolaminar point on C1 to C3
  - spinolaminar point on C2 should be within 1.5 mm of spinolaminar line
Confirmation
- reduction of subluxation with neck extension
- spinolaminar line within 1.5mm of C2
- no history or physical findings of significant trauma
- _absence of anterior soft-tissue swel_ling

Answer 187

A

orthopaedic
- generalized ligamentous laxity and hypotonia
- C1-2 instability
- hip subluxation and dislocation
- patellofemoral instability and dislocation
- scoliosis & spondylolithesis
- pes planus
- primus varus
- SCFE
Phenotypic presentation
- Facial features
  - Flat nasal bridge Epicanthal folds Upward-slanting palpebral
  - fissures Open mouth
- Hand abnormalities
  - Small finger hypoplasia Small finger clinodactyly Single, deep palmar crease (simian crease)
- Characteristic pelvis with lateral flare of iliac wings
- Joint hypermobility Ligamentous laxity Hypotonia
- Short stature
- Mental impairment
Medical Issues
- Cardiac abnormalities Congenital heart disease (50)
  - Atrioventricular septal defect (45)
  - Ventricular septal defect (35)
  - Patent ductus arteriosis (7)
  - Tetralogy of Fallot (4)
- Leukemia
  - Leukemia (1)
  - Acute lymphoblastic leukemia (0.33)
  - Acute myeloid leukemia (0.33)
- Otolaryngologic abnormalities
  - Hearing loss (75)
  - Otitis media (50 to 75)
- Obstructive sleep apnea (50 to 75)
- Ophthalmologic disorders
  - Refractive errors (35 to 75)
  - Strabismus (27 to 57)
  - Congenital cataracts (NA)
- Gastrointestinal disorders
  - Gastrointestinal atresias (12)
  - Celiac disease (7)
  - Hirschsprung disease (1)
- Skin disorders (87)
- Neurologic and psychiatric disorders
  - Mental impairment (NA)
  - Seizures (8)
  - Alzheimer’s disease (in adulthood) (75)
  - Disruptive behavior disorders (17)
  - Depression (in adulthood) (6)
- Endocrine disorders
  - Hypothyroidism (15)
  - Diabetes mellitus (1)

Answer 188

A

C-spine instability in down syndrome

Take a complete history; common symptoms
- neck pain
- sensory deficiet
- bladder control
Always get supervised flex-ex views, static MRI, get dynamic MRI if difficulty with visualizaiton or concerns of cord
- especially if young and not yet ossified spine
- ADI > 10mm = C1/2 pathology
- Powers > 1.0 = occiput pathology
- In multi-level, need to consider more the SAC and cord encroachment (MRI)
  - SAC < 14mm
Screening - highly controversial
- prior to high risk activity (olympics)
- surgery
Asymptomatic, ADI < 10mm on flex-ex
- family education on what to expect if it develops
- full activity
Asymptomatic, ADI > 10mm on flex-ex
- MRI to confirm no cord pathology
- family education
- restrict activities
- annual imaging
Symptomatic, ADI > 10mm
- posterior fusion depending on pathology
- decompression as necessary (occ-C2 fusion required for graft)
- Halo 3 months post-op
Complications
- pin site infection
- 60% pseudoarthrosis
- Wound dehisence
- Junctional instability
- Loss of reduction
- Neurological deterioration
- Resorption of bone graft

Answer 189

A

Higher than sex match, healthy children

C1-2 Pathology (assessed with observed flex-ex and ADI measurement)
- Atlantoaxial instability
- Os odontoideum
- persistent dentocentral synchondrosis of C2
- spina bifida occulta of C1
- ossiculum terminale
Atlano-occipitial instability (assessed with powers ratio)

Answer 190

A

usually have normal, function hips at birth and develop issues later at mobilization
Multiple presentations
- Reducible and stable
  - Some will reduce spontaneously, might resolve with age
- Reducible and unstable
- fixed
Subluxation or habitual painful dislocation can be progressive and lead to degenerative arthritis and pain, so it is advocated to do surgery in childhood if boney pathology is present
Treatment
- Bracing or prolonged immobilization
  - indicated in younger child without bony changes
- capsulorrhaphy and pelvic and femoral osteotomies
  - indicated in the symptomatic older child
  - surgery associated with high complication rate

Answer 191

A

Klipell-Fiel

Rare disease with congenital fusion of C2-C7
Classic Triad
- low posterior hair line
- short webbed neck
- limited cervical ROM
Associated conditions
- congenital scoliosis
- Sprengel’s deformity (33%)
- renal disease (aplasia in 33%)
- synkinesis (mirror motions)
- congenital heart disease
- brainstem abnormalities
- congenital cervical stenosis
- basilar invagination
- atlantoaxial instability (~50%)
- adjacent level disease (100%)

Answer 192

A

Torticollis

Pathophysiology
- some studies suggest association with intrauterine compartment syndrome of SCM
- non-SCM causes include
  - ophthalmologic
  - vestibular
  - congenital
  - traumatic
  - tumors
Associated conditions
- Packing disorders
  - DDH (5 - 20% association)
  - metatarsus adductus
- traumatic delivery
- plagiocephaly (head assymetry)
- congenital atlanto-occipital abnormalities
Differential
- Rotatory atlanto-axial instability / Grisel’s disease
- Klippel-Feil syndrome
Imaging
- XR/CT
  - if no palpable mass
  - to rule out AARD/klippel-fiel
- US can show lesion in SCM
Treatment
- passive stretching
  - condition present for less than 1 year
  - limitation less than 30°
  - should include lateral head tilt away from the affected side and chin rotation toward the affected side (opposite of the deformity)
  - 90% response with-in one year
- Z plasty lengthening or distal bipolar release of SCM
  - > 1 year
  - > 30°

Answer 193

A

Sponylolisthesis

Myerding
- Grade I - < 25%
- Grade II - 25-50%
- Grade III - 50-75%
- Grade IV - 75-100%
- Grade V - Spondyloptosis
Wiltse-Newman
- I. Dysplastic
- II. Isthmic
  - IIA, Disruption of pars as a result of stress fracture
  - IIB, Elongation of pars without disruption related to repeated, healed microfractures
  - IIC, Acute fracture through pars
- III. Degenerative
- IV. Traumatic
- V. Pathologic

Answer 194

A

Spondylolisthesis

epidemiology
- 47% incidence in hyperextension athletes
- 15% of pars lesion progress to spondylolisthesis
- most common at L5-S1 (90%) in pediatric population (L4-5 most common in adult population)
risks of progression
- >50%
- dysplasic slips (Wiltse Type I)
History
- neurologic symptoms include
  - hamstring tightness (most common) and knee contracture
  - radicular pain (L5 nerve root)
  - bowel and bladder symptoms
  - cauda equina syndrome (rare)
- listhetic crisis
  - severe back pain aggravated by extension and relieved by rest
  - neurologic deficit
  - hamstring spasm - walk with a crouched gait
Physical Exam
- Look
  - flattened lumbar lordosis
  - Phalen-dixon gait
  - Scoliosis - Sciatic, olisthetic
- Feel
  - palpable step off of spinous process
  - popliteal angle > 50 = hamstring tightness
- Move
  - limitation of lumbar flexion and extension
  - pain with single-limb standing lumbar extension
- neurologic exam
  - ASIA
  - straight leg raise may be positive
  - rectal exam if bowel and bladder symptoms present

Answer 195

A

AP
- Napolenons hat sign
- Spina bifida
Lateral
- dysplastic vs isthmic
- Myerding slip grade
sacral slope - >50 deg = progression
- line along the sacrum and then perpendicular along the superior endplate
- line along superior endplate L5
Pelvic indidence
- doesn’t change but correlates to severity of disease
- center of S1 endplate to center of femoral head
Sacral slope = pelvic incidence - pelvic tilt
Other Imaging
- Bone Scan - may be hot
- CT - assess boney morphology
- MRI - if neuro symptoms

Answer 196

A

Best indication for non-op
- Low grade isthmic slips
- Less likely that dysplastic will respond to non-op, but can always try it with close follow-up
observation with no activity limitations
- asymptomatic patients with low-grade spondylolithesis or spondylolysis
- may participate in contact sports
physical therapy and activity restriction
- symptomatic isthmic spondylolysis
- symptomatic low grade spondylolithesis
- technique
  - physical therapy should be done for 6 months and include
    - hamstring stretching
    - pelvic tilts
    - abdominal strengthening
- watch low grade dysplastic carefully as there is a higher chance of progression
TLSO bracing for 6 to 12 weeks
- acute pars stress reaction spondylolysis
- isthmic spondylolysis that has failed to improve with physical therapy
- low grade spondylolithesis that has failed to improve with physical therapy
- outcomes
  - brace immobilization is superior to activity restriction alone for acute stress reaction spondylolysis
Operative
- for low grade slips requires minimum 6 months trial non-op
par interarticularis repair
- L1 to L4 isthmic defect that has failed nonoperative management
- multiple pars defects
L5-S1 in-situ posterolateral fusion with bone grafting
- L5, low grade spondylolithesis (Myerding Grade I and II) that
  - has failed nonoperative treatment
  - is progressive
  - has neurologic deficits
  - is dysplastic due to high propensity for progression
L4-S1 posterolateral fusion, +/- reduction, (+/- ALIF)
- high grade isthmic spondlylithesis (Meyerding Grade III, IV, V)
- reduction is extremely controversial with no accepted guidelines

Answer 197

A

Benefits of reduction
- Improves saggital balance
- Reduces stenosis (better in patients with cauda eqina, radiulopathy)
- Reduces risk of pseudoarthrosis
  - Sacral slope > 45 deg
  - Hypermobile L5/S1
  - Decompression
  - Sacral dysplasia
  - Spina bifida
  - Secondary changes of S1 from slip
- Cons of reduction
  - risk of significant complications (8-30%) including
  - L5 and S1 nerve root injuries
  - sexual dysfunction
- catastrophic neurologic injury
Options for reduction
- Pre-op halo
- Pantaloon cast in hyperextension with uninstrumented fusion
- Anterior fusion with ALIF
- Intra-op reduction with posterior fusion
Your plan for treatment - Wide decompression with instrumented partial reduction and posterolateral fusion
- Prone positioning, neuromonitoring
- Posterior approach from L4-S2
- L5 laminectomy with wide decompression of L5/S1 nerve roots
- Partial excision of the disc with posterosuperior aspect of S1
- Pedicle screws L4, L5, S1 with iliac wing screws
- Gradual reduction with cantilever flexion of the pelvis with downward force on the sacrum
  - Over 1 hour
  - Goal to reduce to grade 2
- Visualize neural elements, neuro check
  - If concerns, reduce correction
  - Improve decompression
Post-op TLSO for 3 months
Complications
- Always rule out infection
- Pseudoarthrosis (most common)
  - if asx with mild slip progression then no treatment
  - Pain, gait disurbance or neurological injury = revision instrumentation, or decompression
- Neurological injury
  - L5 weakness, bowel/bladder
  - best is prevention
    - neuromonitoring
    - decompression before reduction
    - gradual, partial reduction
  - Post-op cauda equina = exploration with wide decompression of sacral roots

Answer 198

A

demographics
- most common in children < 3 years old
social risk factors
- low income, unemployed
- single parent homes
- parents were abused
- drug abuse
- recent job loss of parent
- children with disabilities (cerebral palsy, premature)
- step children
Prognosis
- If unreported, 30-50% chance of repeat abuse and 5-10% chance of death from abuse
Differential Diagnosis
- true accidental injury
- osteogenesis imperfecta
- metabolic bone disease

Answer 199

A

Common injuries associated with abuse
- long bone fxs in infant that is not yet walking
- multiple bruises
  - retinal hemorrhage
- multiple fxs in various stages of healing
- corner fxs
  - primary spongiosa (metaphyseal)
  - high specificity for child abuse
- posterior rib fractures
- bucket handle fractures
  - same as corner fractures
  - avulsed bone fragment is seen en face as a bucket handle
- transphyseal separation of the distal humerus
Symptoms
- pain related to fractures
- fractures are the second most common lesion in abused children (skin is the first)
- frequency of fractures
  - humerus > tibia > femur
  - diaphyseal fractures 4 times more common than metaphyseal
Physical exam
- skin lesions
  - most common presenting lesion

Answer 200

A

Corner fracture associated with abuse

Differential
- abuse
- osteogenesis imperfecta
  - keep in mind that kids with OI are also at risk of abuse
- metabolic bone disease
Radiographs
- AP and lateral of bone or joint of suspicion
- initial evaluation should include skeletal survey
Bone scan
- used as an alternative or adjunctive study for patients 5 years of age or older as skeletal surveys not as reliable in older children
Head CT - shaken baby
- subdural hematoma
- retinal hemorrhage
- skull fracture
Report abuse to appropriate agency
- failure to report = 50% risk of repeat abuse and 15% mortality
- early involvement of social workers and pediatricians is essential
- reporting suspected child abuse is mandatory
Hospital admission
- admit infants with fractures to the hospital and consult child protective services
Obtain social service consult
Best to involve pediatric medical team or any other teams necessary
- neuro
- optho
- derm
- gyne

Answer 201

A

Neglect (52% of victims in 1995)
physical abuse (25%)
sexual abuse (13%)
emotional maltreatment (5%)
medical neglect (3%)

Answer 202

A

overview
- complete arrest leads to shortening
- see Leg Length Discrepancy
- partial arrest leads to angulation
CT to assess location of bar
- Peterson classification
treatment
- bar resection with interposition
  - < 50% physeal involvement
  - > 2 years or 2cm growth remaining
  - approach: straight lateral vs metaphyseal
    - drill out area with burr, ensure no residual bar
    - fat or PMMA
- ipsilateral completion of arrest
  - > 50% physeal involvement
  - can combine with contralateral epiphysiodesis and/or ipsilateral lengthening
- osteotomy +/- lengthening
  - skeletally mature

Answer 203

A

Salter-Harris classification
- SH-I is most frequent in <5 year olds
- SH-II is most frequent in >12 year olds
Neer-Horowitz classification
- type I is minimally displaced (< 5mm)
- type II is displaced less than 1/3 of the shaft width
- type III is displaced greater than 1/3 and less than 2/3 the shaft width
- type IV is displaced greater than 2/3 the shaft width
Non-operative Indications
- < 6yo with any displacement
- proximal humerus has 80% growth of humerus with lots of ability to remodel
Indiations for reduction and pinnning
- severly displaced fractures in adolescents
- <50% apposition or >45° angulation
- Neer-Horowitz III-IV
- open fractures in any age
- fractures associated with vascular injuries
- intra-articular displacement
closed reduction
- performed with the arm in 90° abduction and 90° external rotation
- stab incision, be aware of axillary nerve 5cm below acromion
- blocks to reduction can include
  - long head the biceps tendon
  - joint capsule
  - Periosteum
open reduction
- required if block is present or acceptable reduction is unable to be obtained
- deltopec appraoch
fracture stabilization
- stabilized with immobilization
- threaded percutaneous pins
  - non-threaded pins migrate
- screw fixation or retrograde flexible nail fixation are other options
Complications
- Nerve injuries
  - occur in less than 1% of cases
  - typically are neuropraxias
  - associated with a medially displaced shaft
  - close proximity to brachial plexus
  - functional recovery by 9 months without treatment
- Malunion
  - deformities are well tolerated due to range of shoulder motion
- Growth arrest
  - rare

Answer 204

A

transphyseal distal humerus fracture/distal humerus physeal seperation

Typically seen in children under the age of 3
Associated with abuse or battered child syndrome (up to 50%)
Imaging
- difficult to diagnosis - humerus is posterior and medial
- can confirm with MRI or arthrography
Nonoperative
- posterior molded splint then long arm casting
- indicated in nondisplaced fractures
Operative
- CRPP vs ORIF
  - indicated in displaced fractures
Complications
- Cubitus varus
- Medial condyle AVN

Answer 205

A

Common nerve injuries
- anterior interosseous
  - the most common
  - extension
- radial nerve palsy
  - second most common neurapraxia
- ulnar nerve palsy
  - seen with flexion-type injury patterns
- usually resolve with time, median nerve injury is a reason for urgent fixation with open exploration
Gartland Classification
- Type I Nondisplaced, beware of subtle medial comminution leading to cubitus varus
- Type II Displaced, posterior cortex intact
  - IIa - no rotation, translation
  - IIb - rotation and translated
- Type III Completely displaced
- *Type IV Complete periosteal disruption with instability in flexion and extension
Imaging
- Anterior humeral line
  - bisects middle 1/3 capitellum
- Bauman angle
  - line along diaphysis, line

Answer 206

A

hypoplastic facets

maloriented facets

sacral deficieny

abnormal pars development (leads to elongation and slippage)

Answer 207

A

posterior molded splint then long arm casting at at 90° or less
- indications
  - Type I (non-displaced) fractures which are very rare
  - Type II fractures that meet the following criteria
    - anterior humeral line intersects capitellum
    - minimal swelling present
    - no medial comminution
- typically used for 3 weeks
Treatment in emerg
- Splint in 20-40 deg of flexion
- Admit to monitor compartment Q2H by nursing
- Non-sedating anaglesia
closed reduction and percutanous pinning
Flexion - still do closed technique
closed reduction (extension-type)
- patient supine
- gentle traction, milk the fracture out of the brachialis
- Corrent translation and varus/valgus
  - posteromedial fragments: forearm pronated with hyperflexion
  - posterolateral fragments: forearm supinated with hyperflexion
- Flex the arm with pressure on the olecranon to translate the fracture anterior
- Hyperflex the elbow to lock the reduction
two lateral pins
- one in each column
- add a third if concerned about stability
- flex to 90, confirm stability
crossed pins
- biomechanically strongest to torsional stress
- higher risk of ulnar nerve injury (3-8%)
- highest risk if placed with elbow in hyperflexion
Post-op
- Cast at 60-80 deg flexion in posterior splint in neutral rotation, montior for compartment syndrome
- Follow-up with XR one week, re-inforce cast with fiberglass
- Cast off, pins out at 3 weeks with start ROM and splint for protection
Other considerations
open reduction with percutaneous pinning
- reduction cannot be obtained closed
- more frequently required with flexion type fractures
- technique
  - anterior approach typically utilized
immediate closed reduction and percutanous pinning
- vascular compromise is present (e.g, pale, cool hand)
- “floating elbow” - most commonly radius
  - increased risk of compartment syn
- Patient who cannot be monitored for compartment syndrome (young, disability)
- TIII is NOT an indication

Answer 208

A

Managment in emerg
- full NV exam
- Best way to assess vascular status
  - colour
  - cap refill
  - temp
- splint in position of comfort
- call vascular and make them aware
- plan for urgent closed, possible open CRPP
- angiography is not used, slows time to assessment
Urgent reduction and pinning
- Sterile doppler, drap the leg
- Attempt a closed reduction as outlined
- don’t have very good parameters for closed reduction
- If acceptable
  - reassess pulse
  - if no pulse but pink
  - splint at 60-80 deg, admit and monitor for 48 hours
- If not acceptable - open
  - anterior approach
  - if vessel requires repair call vascular

Answer 209

A

Open fracture
Dysvascular limb
Skin puckering
Floating elbow
Median nerve palsy
Evolving compartment syndrome
Young age
Cognitive disability

Answer 210

A

Cubitus Varus/Gunstock demformity/malunion

Cubitus varus (gunstock deformity)
- caused by fracture malunion
- usually a cosmetic issue with little functional limitations - this is changing
- Can lead to attenuation of the LUCL and eventually PLRI
  - Changes the force of the triceps (assymetric)
- Corrective osteotomy is recommended if substantial deformity is present
  - Explain to parent risk of PLRI
  - Opening lateral wedge
- Timing
  - >1 year after no improvement in ROM
  - When old enough to comply with PT
Compartment syndrome
- TIII, admit and monitor pre-op and post-op with Q2H compartment checks
- Increased risk with worse deformity and vascular injury (even after repair)
- Treatment - forearm fasciotomy
Pin migration
- most common complication (~2%)
Infection
- occurs in 1-2.4%
- typically superficial and treated with oral antibiotics
Recurvatum
- common with non-operative treatement of Type II and Type III fractures
Nerve palsy
- usually resolve with no need for treatment or investigation
- median nerve injury is the only one that is an indication for urgent treatment and open exploration
Vascular Injury (as discussed)
- Volkmann ischemic contracture
- rare, but dreaded complication associated with supracondylar humerus fractures
more often as a result of brachial artery compression with treatment utilizing elbow hyperflexion and casting than true arterial injury
increase in forearm compartment pressures and loss of radial pulse with elbow flexed greater than 90°
rarely seen with CRPP and postoperative immobilization in less than 90°
Stiffness
Rare after casting or pinning
Will resolve by 6 months
Literature does not support use of PT

Answer 211

A

pin migration

Answer 212

A

C - captiellum (2)

R - radial head (4)

T - trochlea

I - internal/medial epicondyle (6)

O - olecranon (8)

L - lateral epiconyle (10)

Answer 213

A

Medial Epicondyle fracture

9-14 yo
chronic apophysitis can occur in pitchers
Injury mechanism
- Elbow dislocation - often incacerated
- Direct injury
Physical
- block to extension can indicate incarceration
Imaging
- AP, lateral, internal oblique
- Evidence of incarceration
  - incongruent joint line
  - irregular physeal line
  - fragment at the level of the joint should be consider encarcerated
Treatment
- brief immobilization (1 to 2 weeks) in a long arm cast or splint
  - < 5mm displacement
  - 5-15 mm remains controversial
- open reduction internal fixation
  - indications
    - displaced fx with entrapment of medial epicondyle fragment in joint
    - Open injury
      - relative
      - ulnar nerve dysfunction
      - > 5-15mm displacement
      - high level athletes
  - posteromedial approach to elbow
    - incision is made directly anterior to medial epicondyle
    - Identify and protect the ulnar nerve
    - patient supine on table with arm abducted to 90 degrees and externally rotate
  - reduce fracture
    - Flex the wrist, supinate the arm, flex the elbow
    - Apply eshmarch distally to proximally
    - Preliminary fixation with k-wire superolaterally, avoid olecranon fossa
    - Use c-arm to confirm position
  - Fracture fixation
    - Unicortical cannulated 4-0 screw for fixation
    - Bicortical associated with complications
    - K-wires indicated for smaller fragments or in younger children
- Bulky dressing, sling for comfort
  - ROM to start in 2 weeks
  - Splint for a non-compliant patient

Answer 214

A

Complications are low
Infection, cubitus varus
Nerve injury
- ulnar nerve can become entrapped
- neuropathy with dislocation which usually resolves
Missed incarceration
- missed incarceration of fragment in elbow joint
Elbow stiffness
- loss of elbow extension, avoid prolonged immobilization
Non-union

Answer 215

A

Lateral Condyle Fracture

Milch Classification
- Type I - Fracture line is lateral to trochlear groove (can be very hard to tell on XR)
  - SH4
- Type II- Fracture line into trochlear groove
  - SH2
Fracture Displacement Classification
- Type 1 - Displacement <2mm, indicating intact cartilaginous hinge
  - non-operative if cartilagenous hinge is intact
- Type 2 - Displacement 2-4mm, displaced joint surface
- Type 3 - Displacement >4mm, joint displaced and rotated
Radiographs
- ap, lateral, and internal oblique views
  - internal oblique view most accurately shows maximum displacement and fracture pattern
Ultrasound
- can identify fracture as well, and can accurately assess whether the cartilage hinge is intact
- needs an experienced technician
MRI - good, but expensive and need to sedate child
- Good to assess integrity of cartialge
Arthrogram
- Usually done in OR so often pinned at the same time
- can assess if cartilage hinge is intact; if intact can do CRPP
- Good to differentiate SCH from lateral condyle in young children

Answer 216

A

Displaced lateral condyle fracture (type 2)

Nonoperative - long arm casting
- only indicated if < 2 mm of displacement, which indicates the cartilaginous hinge is most likely intact
- Keep in mind that the fragment is mostly cartilaginous and that the amount of displacement may be more than perceived on the XR
- If concerns get an US or MRI
- technique
  - weekly follow up
  - radiographs out of cast may be useful
  - 5-10% risk of late displacement
  - 6 weeks of immobilization
  - You can get stress views to access for fracture stability, evidence of instability of the fracture with delayed union may lead you to ORIF
CRPP
- indications
  - no evidence of intra-articular incongruity
- technique
  - athrogram to confirm intact articular surface
    - lateral soft spot
    - straight posterior if too much swelling
  - divergent pin configuration most stable
open reduction and percutaneous pinning
- indications
  - if > 2mm of displacement
  - any joint incongruity
- technique
  - direct lateral approach
  - Between brachioradialis and triceps (or can use kocher interval)
  - Extend the wrist to relax the extensors, reduce the fragment
  - a_void dissection of posterior_ aspect of lateral condyle (source of vascularization)
- Two lateral pins versus screw fixation, may need a third for stability
  - Screw fixation may be more stable and prevent overgrowth
  - If there is a large metaphyseal segment, try to pin the metaphysis and the epiphysis sepearately
  - If there is just a small metaphyseal segment, you may have to pin the physis to the metaphysis
- Pins are left in place until evidence of fracture healing (6 weeks)
Open reduction with Grafting
- Can be used for delayed union of > 12 weeks
- A small wedge of bone is placed in the fracture site, trying to avoid displacing the fibrous union of the articular surface - this could result in growth disturbance
- no good awnswer of when to take down the fibrous union and it is up to the descrepancy of the surgeron to some degree

Answer 217

A

AVN
- posterior dissection can result in lateral condyle osteonecrosis
- may also occur in the trochlea
Nonunion
- fracture is intra-articular and bathed in synovial fluid
- caused from delay in diagnosis and improper treatment
- If a nonunion develops, the fragment will migrate proximally and this is where the valgus deformity comes from
- Fix this only if they are apprehensive - bone graft and screw, don’t reduce the fibrous union
- Rarely results following surgery
  - 15-30% with nonop treatment
Malunion
- More common get cubitus varus (20%), poorly understood why
- may result in cubitus valgus (10%)and tardy ulnar nerve palsy
- Treatment
  - Osteosynthesis with ulnar nerve transposition
Lateral overgrowth/prominence (spurring)
- in up to 50% of cases regardless of treatment, families should be counselled in advance, usually resovled with time and remodelling
- lateral periosteal alignment will prevent this from occurring
Growth arrest with or without angular deformity
- Several osteotomies are available to correct this deformity if necessary
- Care must be taken not to malalign the forearm
Unsatisfactory appearance of surgical scar

Answer 218

A

Radial head fracture with 90 deg angulation

Discuss the reduction ladder with the family
Physical
- assess joint above and below, NV exam
- compartments
- assess ROM - specifically looking at pro/sup
- elbow stability
More imaging
- Greenspan view - can be helpful
  - 45 deg oblique with elbow in neutral rotation
immobilization +/- closed reduction
- most fractures can be treated closed
- if < 30° angulation immobilize without closed reduction
- if >30° angulation perform closed reduction and immobilize if angulation reduced to < 30°
- followup
  - begin early ROM at 3-7 days to prevent stiffness
operative percutaneous reduction
- indications
  - > 30° of residual angulation
  - 3-4 mm of translation
  - < 45° of pronation and supination
- reduction techniques
  - Patterson maneuver
    - hold the elbow in extension and apply distal traction with the forearm supinated and pull the forearm into varus while applying direct pressure over the radial head
  - Israeli technique
    - pronate the supinated forearm while the elbow is flexed to 90° and direct pressure stabilizes the radial head
  - elastic bandage technique
    - tight application of an elastic bandage beginning at the wrist continuing over the forearm and elbow may lead to spontaneous reduction
  - K-wire joystick technique
    - __be careful of the PIN
    - pronate the forearm
    - use a posterolateral pin placement, if it remains unstable after reduction pin the fracture
  - Metaizeau technique
    - involves retrograde insertion of a pin/nail across the fracture site
    - fracture is reduced by rotating the pin/nail
  - Check the stability of your fracture and ROM once you have your reduction
  - Posterior splint, change at 1 week
  - Assess healnig at 3 weeks, if not healed then 2 more weeks
  - ROM starts when healing and pins are out
open reduction
- fracture that cannot be adequately reduced with closed or percutaneous methods
- outcomes
  - open reduction has been associated with a greater loss of motion, increased rates of osteonecrosis and synostosis compared with closed reduction.
- lateral approach - anconeus/ECU
  - be mindful of the pin 2.5cm distal to radial head
  - repair the annular ligament if it’s disrupted
- fixation
  - avoid transcapitellar pins
  - internal fixation only used for fractures that are grossly unstable

Answer 219

A

Decreased range of motion
- loss of pronation more common than supination
Radial head overgrowth
- 20-40% of fractures
- usually does not affect function
Osteonecrosis
- 10-20% of fractures
- up to 70% of cases occur with open reduction
Synostosis
- most serious complication
- occurs in cases of open reduction with extensive dissection or delayed treatment

Answer 220

A

relationship of bicipital tuberosity and radial styloid should be 180 degrees from each other on the AP radiograph
relationship of coronoid process and ulnar styloid should be 180 degrees from each other on the lateral radiograph

Answer 221

A

complete neurovascular assessment, check for open wounds and compartment syndrome
closed reduction and immobilization
- most pediatric forearm fractures can be treated without surgery
- Concious sedation
- Reduction with flouro__
  - apex volar fractures (supination injuries)
    - forearm pronation
  - apex dorsal fractures (pronation injuries)
    - forearm supination
- Long arm cast in stable position
- 3 ways of remodelling
  - Bone will straighten as adjacent physes grow
  - Physeal growth will adjust as per heuter-volkman law
  - True shaft remodelling via wolf’s law
- Followup
  - close monitoring in cast with weekly radiographs for first 3-4 weeks following reduction
  - change to munster at 4 weeks
  - casting for 6-12 weeks total typical
percutaneous vs open reduction and nancy nailing
- absolute indications
  - > 10 yo = >15 degrees, rotation >45
  - <10 = >10 degrees, rotation >30 degrees
  - bayonet apposition in children older than 10 years
  - both bone forearm fractures in children > 13
- relative indications
  - highly displaced fractures
- allows smaller dissection and advantage of a load-sharing device allowing rapid healing
- fixation of one bone often sufficient stability
- considerations
  - shorter surgical time than ORIF
  - less blood loss than ORIF
  - equal union rates, radial bow and rotation as ORIF
- techniques
  - radius is done first, retrograde
  - ulna is second
  - one may suffice
- Post-op care
  - Splint that allows and and elbow motion
  - See at 4-6 weeks to assess
  - Pins are removed at 6 moths
open reduction and internal fixation
absolute indications
- unacceptable alignment following closed reduction (as above)
- open fractures
- refractures
same technique as an adult

Answer 222

A

Refracture
- occurs in 5-10% following both bone fractures
- is an indication for ORIF
Malunion
- loss of pronation and supination is common but mild
Compartment syndrome
- may occur due to high energy injuries
- may occur due to multiple attempts at reduction and rod passage

Answer 223

A

Tibial Tuberosity Fracture

Mechanism
- active quads, flexed knee (basketball)
- extensor mechanism pulls off secondary ossification center
Compartment syndrome
- due to recurrent anterior tibial artery
Ogden Classification
- Type I - fracture of the secondary ossification center near the insertion of the patellar tendon
- Type II - fracture propagates to proximal to the junction with the primary ossification center
- Type III - fracture extend posteriorly to cross the primary ossification center
- Modifier: A (nondisplaced), B (displaced)
Newer descriptions have been added to the original system
Type 4 - is a fracture through the entire proximal tibial physis
Type 5 - is a periosteal avulsion of the extensor mechanism from the secondary ossification center
- can get an US to discen patellar sleeve vs tibial tubercle

Answer 224

A

Displaced, Comminuted, Tibial Tuberosity Fracutre

Nonoperative - long leg cast in extension for 4-6 weeks
- usually Type 1 and 2 injuries
- minimal displacement (< 2 mm)
- acceptable displacement after closed reduction
Closed reduction and percutaneous fixation
- approach
  - closed reduction often done under anesthesia
- percutaneous clamping
  - internal fixation with 4.0 cancellous partially threaded screws
  - larger screws can be used but may cause soft tissue irritation in the long-term
- pros
  - no open reduction
  - excellent healing potential
- cons
  - inability to clean fracture site or removal soft tissue interposition
  - hardware irritation can necessitate implant removal
Open reduction and internal fixation
- approach
  - midline incision to the fracture site
- technique
  - evaluate and clean fracture site
  - remove any soft tissue interposition (periosteum)
  - anatomic reduction of fracture fragments
  - internal fixation with 4.0 cancellous partially threaded screws
- pros
  - anatomic reduction and stable fixation
  - excellent healing potential
  - may allow for earlier range of motion
- cons
  - hardware irritation can necessitate implant removal
Open reduction with arthrotomy and internal fixation
- approach
  - midline approach or median parapatellar arthrotomy
  - joint surface must be visualized to assure anatomic reduction
  - alternatively, arthroscopy can be used to directly assess the articular reduction
- technique
  - same as above
  - evaluate for meniscal tears and repair or debride as appropriate
  - evacuate intraarticular hematoma
  - visualize joint surface to achieve anatomic reduction
- pros
  - addresses intraarticular extension and soft tissue injuries
- cons
  - arthrotomy may require longer immobilization and/or rehabilitation
Open reduction and soft tissue repair
- approach
  - midline incision to the soft tissue injury site
- technique
  - evaluate soft tissue injury
  - remove any soft tissue interposition (periosteum)
  - heavy suture repair of periosteum back to the secondary ossification center
- postoperative care
  - immobilization
  - long leg cast for 8-10 weeks
- cons
  - prolonged healing time due to soft tissue healing
post-op care
- immobilization
- long leg cast for 4-6 weeks
  - prolonged immobilization needed in Type 2 and 3 injuries
- non-weight bearing
- rehabilitation
  - progressive extensor mechanism strengthening
  - return to sports no sooner than 3 months

Answer 225

A

Recurvatum deformity
- more common than leg length discrecancy
- growth arrest anteriorly as posterior growth continues leading to decrease in tibial slope
Compartment syndrome
- related to injury of anterior tibial recurrent artery
Loss of range of motion
Bursitis
- due to prominence of screws and hardware about the knee

Answer 226

A

Marfans

Ankylosing Spondylitis

Euler’s Danlos

OI

Neurofibromatosis

Answer 227

A

Paraspinal mass (helps to distinguish from AIS)
Dumbell lesions
Dural ectasia
Vertebral body scalloping

Answer 228

A

Marfans

Orthopaedic conditions
- Arachnodactyly
- scoliosis (50%)
- protrusio acetabuli (15-25%)
- ligamentous laxity
  - recurrent dislocations (patella, shoulder, fingers)
- pes planovalgus
Nonorthopaedic conditions
- cardiac abnormalities
  - aortic root dilatation
  - possible aortic dissection in future
    - Most lethal consequence
    - May require prophylatic surgery to repair if > 5cm
  - mitral valve prolapse
- superior lens dislocations (60%) (ectopia lensitis)
  - Mypoia
  - Glaucoma
  - cataracts
- pectus excavatum
- spontaneous pneumonthoraces
- dural ectasia (>60%)
- Meningocele

Answer 229

A

autosomal dominant
mutation in fibrillin-1 (FBN1) gene
- located on chromosome 15 (locus CH 15q21)
- multiple mutations identified
Recommendations for sports
- Low energy activity allowed
- Contact sports, rapid accel/deccl contra-indicated (weight lifting, martial arts)
  - Lens dissection
  - Aortic dislocation
- Maintain aerobic level < 50%

Answer 230

A

History
- scoliosis is usually the first to be diagnosed
- may be a history of ankle sprains secondary to ligamentous laxity
- possible history of dislocations
- Chest pain, family history
Symptoms
- asymptomatic in most cases
Physical exam
- dolichostenomelia (arm span greater than height (>1.05 ratio)
- arachnodactyly (long, thin toes and fingers)
  - Wrist sign
- ligamentous hyperlaxity
  - pes planus
- scoliosis, pectus excavum
- listen to lungs, heart
Radiographs
- scoliosis series of spine
- CXR - pneumothorax, heart
- findings
  - scoliosis
  - kyphosis
CT
- Narrow pedicles
- Wide TP
- Vertebral scalloping
- Thin laminae
MRI
- MRI of spine prior to surgery
- look for dural ectasia
  - Commonly between L5-S2
  - Thinning of the pedicel cortices
  - Widening of the foramen
  - Anterior myelomeningocele
Cardiac studies
- cardiac evaluation prior to surgery
- cardiac consult
- echocardiogram

Answer 231

A

Often progresses more rapidly than AIS
Often have double and triple major curves
40% have kyphosis > 50deg
bracing
- Most are small and will not require operative treament
- Indications
  - early treatment of mild curve
  - <45 deg
  - Risser 0-2
- outcomes
  - ineffective in most cases - need to discuss this with family
ASF +/- PSF with instrumentation
- indications
  - >45 deg
  - rapidly progressing curve in a skeletally immature patient
  - large curve in a skeletally mature patient
- Technique
  - May need to use growing rods if young
  - Often need to go to iliac
  - Selective thoracic fusion is less successfu
  - Avoid short fusion
- Outcomes - higher complication rate than idiopathic scoliosis surgery
  - Blood loss
  - infection
  - pseudoarthrosis
  - Failure of fixation is common due to thin bones
  - May develop deformity around instrumentation = ‘adding on’

Answer 232

A

Diagnosis
- center edge angle of Wiberg >40°
- crossing of the ilioischial line by the acetabular line medially by
  - >3 mm in men
  - >1 mm in boys
  - >6 mm in women
  - >3 mm in girls
  - crossing of the iliopectineal line by the acetabular line
Nonoperative
- observation unless severe symptoms develop
If <8yo, progressive can consier epiphyseodesis
- Will be stable after 20 yo and do not adversly affect function

Answer 233

A

MED is bilateral at the same stage
MED has no metaphyseal cysts
acetabular changes associated with MED
MED will have changes in other joints

Answer 234

A

capillary refill
skin temperature
skin colour

Answer 235

A

bilateral
irreducible hip
> 7 weeks old (controversial and debated)

Answer 236

A

Developmental dysplasia of the hip
Transient synovitis of the hip
Osteomyelitis of the proximal femur/ pelvis
Henoch-Schönlein purpura
Pyomyositis of the surrounding musculature
Traumatic synovitis
Fracture
Intra-abdominal pathology
Sacral agenesis
Superficial cellulitis
Superficial abscess
Psoas abscess
Pyogenic sacroiliitis
Acute leukemia
Various rheumatologic disorders
Nonspecific arthritides
Proximal focal femoral deficiency
Acute rheumatic fever

Answer 237

A

vertical physis
coxa varum
femoral retroversion
obesity

Answer 238

A

synovial fluid
pull extensor mechanism
poor blood supply

Answer 239

A

radiation
inexpensive
dynamic
no need for sedation or fasting
can be done before ossification

Answer 240

A

DDH - diagnose and follow treatment
- can assess acetabulum, femoral head and dynamic stability
SCFE - can’t see pre-slip
FAI - only to assess CAM lesion
Psoas - dynamic evaluation of snapping hip
- guided injection can rule out pathology
Clubfoot - can monitor realignment of the TN joint
- assess the adequacy of complete reduction; predict failure
Spine
- Tethered cord - assess for location of conus
  - conus below L2/3 with limited motion = tether cord
- Spinal dysraphysm
  - closed - lipomyelocele, lipomyelomeningocele, posterior meningocele, and myelocystocele
Peripheral nerves
- subluxation
- extent of damage (endo/epi/perineruium vs scar)
Neonatal brachial plexus palsy - assess glenoid, position of head, stability
Infection
- periosteal abcess from OM
- hard to reach joint aspiration
- abcess diagnosis and culture
Biposy (obviously) or assessment of cyst/tumor

Answer 241

A

subcutanoeus mass
bowel/bladder
anorectal or urogenitary abormalities
foot abormalities from neuro dysfunction
VATER syndrome

Answer 242

A

U/S guided nerve blocks - last longer, less medication, fewer complicaitons
U/S guided botulism injection - imporved needle placement, less time, improved results
Removal of foreign body

Answer 243

A

Note that these are not mutally exclusive and you should always screen for all of them; see baby below with thumb hypoplasia, fanconi’s anemia; also has several features of VACTERL
- CBC
- echo
- Renal U/S
- full head-toe exam
These patients should be treated with a team based approach - invovle genetics, pediatrics and all necessary specialties
TAR
- autosomal recessive condition with thrombocytopenia and absent radius
- thrombocytopenia fluctuants over time
  - exaccerbated by infection, stress
- presents in first week of life
  - purpura, petechiae
  - intracranial hemmorhage - fatigues, lethary, difficulty feeding
- low platlets, normal chormosomes
- Basic first aid, platelet transfusion
- different in that thumb is typically present
Fanconi’s anemia
- autosomal recessive condition with aplastic anemia
- can get hemmorhage, infection, neoplasia
- Work-up
  - CBC - pancytopenia or complete aplasia
  - Diagnosis - Fanconi screen and chromosomal breakage test to screen
- treatment is bone marrow transplant
Holt-Oram syndrome
- autosomal dominant condition characterized by cardiac defects
  - ASD most common
- diagnoses with blood test and genetics
VACTERL Syndrome
- vertebral anomalies
- anal atresia
- cardiac abnormalities
- tracheoesophageal fistula
- renal agenesis
- limb defects
VATER Syndrome
- vertebral anomalies, anal atresia, tracheoesophageal fistula, esophageal atresia, renal agenesis)

Answer 244

A

Bayne and Klung

Type I
- Deficient distal radial epiphysis
Type II
- Deficient distal and proximal radial epiphyses
Type III
- Present proximally (partial aplasia)
Type IV
- Completely absent (total aplasia - most common)

Answer 245

A

Rule out associated disorders and life threatening conditions
Team based approach with peds/genetics
Assess function, estabilish goals; invovled senior colleague
Nonoperative
- Indications
  - (1) functional compensation established
  - 2) mild deformities with good function and cosmesis
  - (3) associated medical anomalies
  - (4) severe, bilateral elbow extension contractures in patients who rely on wrist flexion and radial deviation of the hand for movement of the hand to the face.
- passive stretching
  - target tight radial-sided structures
  - should also be done pre-op
hand centralization
- indications
  - good elbow motion and biceps function intact
  - done at 6-12 months of age
    - Stage the forearm first, and then the thumb at 18 month
    - followed by tendon transfers
- technique - centralization of the wrist with overcorrection
  - involves resection of varying amount of carpus, shortening of ECU, and, if needed, an angular osteotomy of the ulna (> 30 deg bowing; be sure to spare ulnar distal physis)
- options
  - may do as two stage procedure in combination with a distration external fixator
  - vascularized free fibular with lateral rays are experimental

Answer 246

A

Congenital radial head dislocation

can bedifferentiated from a traumatic dislocation by
- bilateral involvement
- hypoplastic capitellum
- convex radial head
- other congenital anomalies
- lack of history of trauma
- difficult to reduce
Pathoanatomy
- almost always posterior dislocation of radial head
- often combined with bowing and shortening of radius

Answer 247

A

Bluth Classification Thumb Hypoplasia

Type I
- Minor hypoplasia
- All musculoskeletal and neurovascular components of the digit are present, just small in size
- No surgical treatment required
Type II
- All of the osseous structures are present (may be small)
- MCP joint ulnar collateral ligament instability
- Thenar hypoplasia
- Treatment
  - Stabilization of MCP joint
  - Release of first web space
  - Opponensplasty
Type IIIA
- Musculotendinous and osseous deficiencies
- CMC joint intact
- Absence of active motion at the MCP or IP joint
Type IIIB
- Musculotendinous and osseous deficiencies.
- Basal metacarpal aplasia with deficient CMC joint
- Absence of active motion at the MCP or IP joint.
- Thumb amputation & pollicization
Type IV
- Floating thumb
- Attachment to the hand by the skin and digital neurovascular structures
Type V
- Complete absence of the thumb

Answer 248

A

First web space deepening
- usually performed with Z-plasty
- Excision of redudant FPL if causing contracture
Opponensplasty (opposition transfer)
- flexor digitorum superficialis - 2
- abductor digiti minimi (subsidized with palmaris) - 3b
Stabilization of MCP joint
- fusion
- reconstruction of UCL with FDS
- reconstruction of UCL with free tendon graft

Answer 249

A

Complete thumb ablation if still present
Other option is 2nd toe transfer
Pollicization
- plan skin incision to avoid skin grafts
- isolate index finger on its neurovascular bundles
- detach first dorsal and palmar interosseous muscles
- shorten digit by removing index finger metacarpal and epiphyseal plate
- stabilize index MCP joint
- reattach and balance musculotendinous units
- reconstruct long extensor tendons
- rebalance flexor tendons

Answer 250

A

get an increase FPL thickening with triggering at A1
- 25% are bilateral
- presents with a fixed deformity
- > 2yo = resolution unlikley
- Usually not painful
- Notta’s nodule = flexor tendon prominence
passive extension exercises and observation
- indications
  - usually considered first line of treatment
  - not recommended for fixed deformities in older children
- technique
  - passive thumb extension exercises
  - duration based on clinical response
- outcomes
  - 30-60% will resolve spontaneously before the age of 2 years old
  - <10% will resolve spontaneously after 2 years old
extension splinting
- indications
  - consider alongside stretching regime
  - not recommended with fixed deformities in older children
- technique
  - splints maintain IP joint hyperextension and prevent MCP joint hyperextension
  - duration for 6-12 weeks
- outcomes
  - 50-60% resolution in all age groups
  - high drop out rate from therapy
A1 pulley release
- indications
  - fixed deformity beyond age of 12 months of age
  - failed conservative treatment
- outcomes
  - 65-95% resolution in all age groups

Answer 251

A

Radial Digital nerve injury
- caution must be performed during release as digital nerves at high risk due to proximity to flexor tendon and A1 pulley
Wound complications
- scar contracture
abscess
infection
IP flexion deficit
Bow-stringing of flexor tendon
- usually related to release of the oblique pulley

Answer 252

A

Congenital flexion-adduction deformity of the thumb that persists beyond the 3rd or 4th month of life
male-to-female ratio is approx 2.5:1
risk factors
- consanguinity
- family history
Pathophysiology
- autosomal dominance inheritance of variable expressivity
- may be sporadic
- attenuation or deficiency of extensor pollicis longus or brevis, or both
- contracture of the 1st web space
- contracture of adductor pollicis or first dorsal interosseous muscle
- global instability of first MP joint
- abnormal articular cartilage of first MP joint
Orthopedic considerations
- lower limb anomalies
  - congenital vertical talus
  - congenital talipes equinovarus (bilateral)
- upper limb anomalies
  - flexion deformities of the four fingers
Associated conditions
- arthrogryphosis (congenital joint contractures affect tow or more areas in the body)
- digitotalar dysmorphisms
- Freeman-Sheldon syndrome
- X-linked MASA syndrome

Answer 253

A

attenuation or deficiency of extensor pollicis longus or brevis, or both
contracture of the 1st web space
contracture of adductor pollicis or first dorsal interosseous muscle
global instability of first MP joint
abnormal articular cartilage of first MP joint

Answer 254

A

serial splinting and stretching for 3-6 months
- first-line treatment over a trial period of 3-6 months for all types
- begin treatment around the age of 6 month old
- outcome
  - good definitive results with Type I congenital deformities when one of the EPL or EPB tendons are present
  - poor results with Type I deformities when both EPL/EPB tendons are absent
  - poor results with Type II or III deformities
tendon transfer to EPL
- indications
  - Type I or II with residual deficiency in active extension
- technique
  - extensor indicis transfer to remnant of extensor tendon
thumb reconstruction
- indications
  - failed conservative treatment
  - soft-tissue deficiency in the thumb-index finger webspace (Type III)
  - Type II or III deformity with significant MCP joint contractures
- technique
  - usually delayed until the age of 3 to 5 years old
  - widening the first webspace
    - transposition flap of skin (dorsal rotational advancement flap)
    - four-flap or five-flap Z plasty of skin
  - deepening the first webspace by releasing soft-tissue
    - releasing origins of thenar musculature from transverse carpal ligmant
    - releasing joint capsule of first MP joint
  - tendon transfer
    - FPL Z-lengthening in the forearm
    - isolated EPB tendon will not usually require tendon transfer
    - EPB and EPL absence is best reconstructed with tendon transfer
arthrodesis
- severe deformities when skin release and tendon trasnfer cannot overcome joint deformity.

Answer 255

A

Type I (Supple clasped thumb)
- Thumb able to be passively abducted and extended against resistance of thumb flexors. No other digital anomly present.
Type II (Clasped thumb with contracture)
- Thumb cannot be passively extended and abducted. This may occur with or without other digital anomaly.
Type III (Rigid clasped thumb)
- Clasped thumb that is associated with arthrogryposis and marked soft-tissue deficits.

Answer 256

A

acrosyndactyly
- digits fuse distally and proximal digit has fenestrations (e.g., constriction ring syndrome)
Poland Syndrome
Apert Syndrome
Carpenter syndrome
acrocephalopolysyndactyly

Answer 257

A

Simple
- Only soft tissue involvement, no bony connections
Complex
- Side to side fusion of adjacent phalanges
Complicated
- Accessory phalanges or abnormal bones involved in fusion
Complete vs. Incomplete
- Complete: syndactyly the skin extends to finger tips
- incomplete: skin does not extend to fingertips

Answer 258

A

Goal is to have as many independant and functional digits as possible
- deep and wide web
- cover deficits with soft tissue
Indications
- skin bridge that hinders use
Contraindications
- super digit
- complex digit that functions in unison
- no active muscle control with functional digits

Answer 259

A

Timing
- syndactyly
  - perform at ~ 1 year of age
  - controversial
- acrosyndactyly
  - perform in neonatal period
- if multiple digits are involved perform procedure in two stages to avoid compromising vasculature
  - early surgery allows this
  - thumb and index at < 6months
- release digits with significant length differences first to avoid growth disturbances
technique
- zigzag flaps are created during release to avoid longitudinal scarring
- a nailbed can be re-created in complete syndactyly
complications
- Web creep
  - most common complication of surgical treatment
  - reconstruct web space with local skin flaps
- Nail deformities
- Scar
- Avascular digit
  - __never operate threw both sides of the digit

Answer 260

A

unilateral chest wall hypoplasia
- due to absence of sternocostal head of pectoralis major
hypoplasia of the hand and forearm
symbrachydactyly and shortening of middle fingers
- result of absence or shortening of the middle phalanx
simple complete syndactyly of the short digits
hypoplasia or absence of the pectoralis major, pectoralis minor, deltoid, serratus anterior, external oblique, and latissimus dorsi
Sprengel’s deformity
scoliosis
dextrocardia
absence or underdevelopment of the breast
carpal coalition or hypoplasia
radioulnar synostosis
nail agenesis

Answer 261

A

FGFR2 gene
Rosebud Hands
- bilateral complex syndactyly of hands and feet
  - index, middle, and ring fingers most affected
- symphalangism
Dysmorphic Facies
- premature fusion of cranial sutures (craniosynostosis) results in flattened skull and broad forehead (acrocephaly)
- hypertelorism (increased distance between paired body parts, as in wide set eyes)
normal to moderately disabled cognitive function
glenoid hypoplasia
radioulnar synostosis

Answer 262

A

preaxial polydactlyly
- thumb duplication
postaxial polydactlyly
- small finger duplication
central polydactlyly

Answer 263

A

Type I Bifid distal phalanx
Type II Duplicated distal phalanx
Type III Bifid proximal phalanx
Type IV Duplicated proximal phalanx (most common)
Type V Bifid metacarpal
Type VI Duplicated metacarpal
Type VII Triphalangia

Answer 264

A

goals of treatment
- to construct a thumb that is 80% of the size of the contralateral thumb
- resect smaller thumb (usually radial component)
- preserve / reconstruct medial collateral structures in order to preserve pinch function
- reconstruction of all components typically done in one procedure
type 1 combination procedure (Bilhaut-Cloquet)
- indications
  - type I, II, or III
- technique
  - involves removing central tissue and combining both digits into one
- outcomes
  - approximately 20% have late deformity
  - problems include stiffness, angular and size deformity, growth arrest, and nail deformities
type 2 combination procedure
- indications
  - usually favored approach for type III and IV
  - type V and VI usually require more complex transfer of intrinsics and collateral ligaments
- technique
  - preserve skeleton and nail of one component and augment with soft tissue from other digit and ablation of lesser digit (radial digit most commonly)
type 3 combination procedure
- indications
  - when one digit has superior proximal component and one digit has superior distal fragment (type V, VI, and VII)
- technique
  - a segmental distal transfer (on-top plasty)

Answer 265

A

Classification
- Type A - well formed digit
- Type B - rudimentary skin tag (vestigial digits)
formal reconstruction with a Type 2 combination
- indications
  - Type A
- technique
  - preserve radial digit
  - preserve or reconstruct collateral ligaments from ulnar digit remnant
  - preserve muscles
tie off in nursery or amputate before 1 year of age
- Type B

Answer 266

A

natural history
- increased incidence of acute and chronic pain in adults if left untreated
- curves > 90° are associated with cardiopulmonary dysfunction, early death, pain, and decreased self image
risk factors for progression (at presentation)
- curve magnitude
  - before skeletal maturity
    - > 25° before skeletal maturity will continue to progress
  - after skeletal maturity
    - > 50° thoracic curve will progress 1-2° / year
    - > 40° lumbar curve will progress 1-2° / year
- remaining skeletal growth
  - < 12 years at presentation
  - Tanner stage (< 3 for females)
  - Risser Stage (0-1)
  - open triradiate cartilage
  - peak growth velocity is the best predictor of curve progression
    - in females it occurs just before menarche and before Risser 1 (girls usually reach skeletal maturity 1.5 yrs after menarche)
    - most closely correlates with the Tanner-Whitehouse III RUS method of skeletal maturity determination
    - if curve is >30° before peak height velocity there is a strong likelihood of the need for surgery
- curve type
  - thoracic more likely to progress than lumber
  - double curves more likely to progress than single curves

Answer 267

A

Side bending cobb > 25 deg
Kyphosis > 20 deg
Largest curve is always structural

Answer 268

A

Anatomic definition
- thoracic T2-T11
- thoracolumbar T2-L1
- Lumbar L1-L4
Identifiy the primary structural curve (1-6)
- proximal thoracic
- main thoracic
- thoacolumbar/lumbar
Lumbar modifier
- relationship of central sacral vertical line to apical lumbar vertebrae
  - A - CSVL between pedicles
  - B - CSVL hits pedicles
  - C - CSVL medial to pedicles
Saggital thoracic modifier
- Measure sagital Cobb from T5 to T12
- Assign modifier
  - hypokyphotic (-) if < 10°
  - normal if 10-40°
  - hyperkyphotic (+) if >40°
    *

Answer 269

A

prevent progression
maintain/improve coronal & sagittal alignment
level shoulders
correct deformity
save motion segments

Answer 270

A

End vertebra:
- vertebra that define ends of curve, are tilted maximally toward the concavity
Neutral vertebra:
- the most cephalad & caudal vertebrae in a curve without axial rotation
Stable vertebra:
- the thoracic or lumbar vertebra cephalad to a lumbar scoli that is most closely bisected by the CSVL assuming that the pelvis is level (SRS)
Selecting upper instrumented vertebra (UIV):
- Goals
  - prevent shoulder imbalance
  - prevent prox curve progression
  - prevent prox junctional kyphosis
- Levels
  - T2 for pre-op L shoulder elevation
  - T3 for pre-op level shoulders
  - T4 for pre-op L shoulder depression
Selecting lower instrumented vertebra (LIV):
- Try to preserve motions and levels
- Goals for inclusion
  - all structural curves
  - lumbar non-structural curves >45° on PA XR
  - significant rotation/wedging
- Levels
  - LIV Lenke 1, 2: modifier A → vert touching CSVL; mod B/C → prox stable vert
  - LIV Lenke 3-6: distal end vertebra [many exceptions; no scoli surgeons agree..]

Answer 271

A

atypical curve pattern (left thoracic curve, short angular curve, apical kyphosis)
rapid progression
excessive kyphosis
structural abnormalities
neurologic symptoms or pain
foot deformities
asymmetric abdominal reflexes
a syrinx is associated with abnormal abdominal reflexes and a curve without significant rotation

Answer 272

A

Standing AP, lateral, traction films, bending films
Cobb angle - >10deg = scoliosis
coronal balance
- C7 plumb line to central sacral vertical line
sagittal balance
- C7 plumb from center of C7 to the posterior-superior corner of S1
Stable vertebrae, end vertebrae, apical vertebrae, neutral vertebrea
clavicle angle
- best predictor of postoperative shoulder balance
- AC-AC

Answer 273

A

observation alone
- cobb angle < 25°
- obtain serial radiographs to monitor for progression
bracing
- cobb angle from 25° to 45°
- only effective for flexible deformity in skeletally immature patient (Risser 0, 1, 2)
- goal is to stop progression, not to correct deformity
posterior spinal fusion
- cobb angle > 45°
- can be used for all types of idiopathic scoliosis
- remains gold standard for thoracic and double major curves (most cases)
anterior spinal fusion
- best for thoracolumbar and lumbar cases with a normal sagittal profile
anterior / posterior spinal fusion
- larges curves (> 75°)
- stiff curves
- young age
  - Risser grade 0
  - girls <10 yrs, boys < 13 yrs)
- in order to prevent crankshaft phenomenon

Answer 274

A

Indications
- Risser 0-2
- flexible curve
- 25-45
recommended for 16-23 hours/day until skeletal maturity or surgical intervention deemed necessary
brace types
- curves with apex above T7
  - Milwaukee brace (cervicothoracolumbosacral orthosis)
  - extends to neck for apex above T7
- apex at T7 or below
  - TLSO
  - Boston-style brace (under arm)
  - Charleston Bending brace is a curved night brace

Answer 275

A

poor in-brace correction
hypokyphosis (relative contraindication)
male
obese
noncompliant (effectiveness is dose related)

Answer 276

A

Monitoring
- somatosensory-evoked potentials (SSEPs)
  - upper exteremity peripheral nerve from positioning (most common reversible neuro injury)
- motor-evoked potentials (MEPs)
  - impending spinal cord dysfunction
  - need to use propofol for intubation
- Ankle clonus
- Stagnaras wake-up test
Causes
- direct injury - screws; wires
- expanding hematoma
- distraction of the cord
- prolonged hypotension
- decreased HbB
- ligation of segmental vessels
Algorythm for neurological injury (>50% amp, >10% latency)
- check hemoglobin and transfuse as necessary
- elevate blood pressure (MAP >80)
- improve oxygenation and temperature
- reverse correction
- remove instrumentation if the spine is stable
- administer Stagnaras wake up test
- Steroids and admit to ICU if still no movement
  - MRI to assess cord

Answer 277

A

Assess HbB
Assess MAP > 80
- fluids
- transfuse
- pressors
Temperature
MRI to assess hematoma
Go to OR
- if dealy to get MRI
- decompress hematoma
- release correction

Answer 278

A

Neurologic injury
- paraplegia is 1:1000
- increased risk with kyphosis, excessive correction, and sublaminar wires
Pseudoarthrosis (1-2%)
- presents as late pain, deformity progression, and hardware failure
- an asymptomatic pseudarthrosis with no pain and no loss of correction should be observed
Infection (1-2%)
- presents as late pain
- incision often looks clean
- Propionibacterium acnes most common organism for delayed infection (requires 2 weeks for culture incubation)
- attempt I&D with maintainence of hardware if not loose and withing 6 months
Flat back syndrome
- early fatigability and back pain due to loss of lumbar lordosis
- rare now that segmental instrumentation addresses sagittal plane deformities
- decreased incidence with rod contouring in the sagittal plane and compression/distraction techniques
- treat with revision surgery utilizing posterior closing wedge osteotomies
- anterior releases prior to osteotomies aid in maintenance of correction
Crankshaft phenomenon
- rotational deformity of the spine created by continued anterior spinal growth in the setting of a posterior spinal fusion
- can occur in very young patients when PSF is performed alone and the anterior column is allowed continued growth
- avoided by performing anterior diskectomy and fusion with posterior fusion in very young patients
SMA syndrome (superior mesenteric artery syndrome)
- presents with symptoms of bowel obstruction
- treat with NG tube and IV fluids
Hardware failure
- late rod breakage can signify a pseudarthrosis

Answer 279

A

Risk factors
- BMI < 25th percentile for age
- Stiffer thoracic curve, less than 60% correction on lateral bending films.
- Laterally displaced lumbar curve (Lenke B or C)
- Two stage procedure
- Anterior surgery
- Thoracotomy
Presentation
- bowel obstruction at 3rd part of duodenum
- nausea, vomiting, bilious vomiting, hyperactive bowel sounds, post prandial fullness, epigastric pain or discomfort, abdominal distension
Treatment
- NPO
- NG tube
- fluids

Answer 280

A

Drowsiness, irritability, muscle cramps - hyponatremia

Answer 281

A

high incidence of neural axis abnormalities (18-25%)
syringomyelia
- cyst or tubular cavity within spinal cord
- can be seen in a scoliotic curve without rotation
- can manifest as an asymmetric umbilicus reflex
Arnold-Chiari syndrome
- cerebellar tonsil are elongated and protruding through the opening of the base of the skull and blocking CSF flow)
tethered cord
dysraphism
spinal cord tumor

Answer 282

A

Cobb < 25 degrees
RVAD < 20 degrees (rib-vert angle diff; mheta)
Phase 1 ribs

Answer 283

A

observation alone
- indications
  - Cobb < 25 degrees
  - RVAD < 20 degrees
  - Phase 1 ribs
- 90% will resolve spontaneously
casting and bracing
- Cobb < 35
- RVAD > 20 degrees
surgical deformity correction (growing rod construct vs ASF&PSF)
- Cobb > 35 to 40 degrees

Answer 284

A

most cases occur spontaneously
maternal exposures
- diabetes
- alcohol
- valproic acid
- hyperthermia
genetic
knowlege evolving
notch gene

Answer 285

A

VItamin supplementation (e.g. Iron, folate, B12)
Erythropoietin
bipolar sealant ( no clear benefit)
Argon-bean coagulation ( no clear benefit)
Topical hemostatic agents ( eg. collagen agents, fibrin sealants)
Antifibrinolytics (TXA - topical or IV)
Reinfusion systems (cell-saver)
Transfusion protocols ( lower transfusion trigger - HGb 80)

Answer 286

A

with associated systemic anomalies, up to 61%
- cardiac defects - 10%
- genitourinary defects - 25%
- spinal cord malformations
- Most commonly associated with other MSK issues
  - Vertical talus
  - clubfoot
  - spregnels
  - limb hypoplasia
with underlying syndrome or chromosomal abnormality
- VACTERL syndrome
  - in 38% to 55%
- Goldenhar/OculoAuricularVertebral Syndrome
  - hemifacial microsomia and epibulbar dermoids
- Jarcho-Levin Syndrome/Spondylocostal dysostosis
  - short trunk dwarfism, multiple vertebral and rib defects and fusion
  - most commonly autosommal recessive
  - often associated with thoracic insufficiency syndrome (TIS)
    - caused by shortening of the thorax and rib fusions
    - result is thorax is unable to support lung growth and respiratory decompensation
- Klippel-Feil syndrome
  - short neck, low posterior hairline, and fusion of cervical vertebrae
- Alagille syndrome
  - peripheral pulmonic stenosis, cholestasis, facial dysmorphism

Answer 287

A

most rapid in the first 3 years of life
- Also with growth spurts, namely puberty
Thoracolumbar > upper thoracic
presence of fused ribs increases risk of progression
Rate of progression from greatest to least is:
- unilateral unsegmented bar with contralateral hemivertebra
  - greatest potential for rapid progression (5 to10 degrees/year)
- unilateral unsegmented bar >
- fully segmented hemivertebra >
- unsegmented hemivertebra >
- incarcerated hemivertebra >
- unincarcerated hemivertebra >
- block vertebrae
  - little chance for progression (<2 degrees/year)

Answer 288

A

observation and bracing
- some partially segmented hemivertebrae
- nonsegmental hemivertebrae
- incarcerated hemivertebrae
- Block vertebrae
- absence of documented progression
bracing
- not indicated in primary treatment of congenital scoliosis (no effectiveness shown)
- may be used to control supple compensatory curves
in situ posterior fusion
- indications
  - older patients
    - girls > 10 yrs
    - boys > 12 yrs
  - significant progression
  - neurologic deficits
  - declining respiratory function
- drawback is stunted growth of the thorax and abdomen
anterior/posterior spinal fusion +/- vertebrectomy
- indications
  - In young patients anterior fusion needed to prevent progression or crankshaft
    - young patients with significant progression, neurologic deficits, or declining respiratory function
      - girls < 10 yrs
      - boys < 12 yrs
  - patients with failure of formation with contralateral failure of segmentation at any age
- technique
  - nutritional status of patient must be optimized prior to surgery
growing rod construct
- indications
  - may be used in an attempt to control deformity during spinal growth and delay arthrodesis
- outcomes
  - need to be lengthened approximately every 6 months for best results
VEPTR (vertical expanding prosthetic titanium rib)
- indications
  - thoracic insufficiency syndrome
- outcomes
  - long-term follow up is needed to determine efficacy

Answer 289

A

in situ arthrodesis, anterior/posterior or posterior alone
- indications
  - unlateral unsegmented bars with minimal deformity
- As risk for crackshaft and may not prevent progression in children <10
hemiepiphysiodesis
- indications
  - intact growth plates on the concave side of the deformity
  - Cannot use in a patient with unsegmented bars
  - patients less than 5 yrs. with < 40-50 degree curve
- mixed results
osteotomy
- osteotomy of bar
- for more severe; rigid deformities
hemivertebrectomy
- hemivertebrae with progressive curve causing truncal imbalance and oblique takeoff
  - often caused by a lumbosacral hemivertebrae
- patients < 6 yrs
- flexible curve > 40 degrees
- Difficulty procedure requiring both anterior and posterior exposure
spinal column shortening resection
- indications
  - deformities that present late and have severe decompensation
  - rigid, severe deformities
  - pelvic obliquity, fixed

Answer 290

A

in situ arthrodesis, anterior/posterior or posterior alone
- unlateral unsegmented bars with minimal deformity
hemiepiphysiodesis
- intact growth plates on the concave side
- patients less than 5 yrs
- < 40-50 degree curve
- mixed results
osteotomy
- osteotomy of bar
hemivertebrectomy
- hemivertebrae with progressive curve
  - truncal imbalance
  - oblique take-off
- often caused by a lumbosacral hemivertebrae
- Best candidate
  - patients < 6 yrs
  - flexible curve
  - < 40 degrees
spinal column shortening resection
- deformities that present late and have severe decompensation
- rigid, severe deformities
- pelvic obliquity, fixed

Answer 291

A

Distraction Based
- growing rods
  - significant remaining axial growth
  - progressive deformity >50°
  - flexible spinal deformity
- Verticle Expandable Prosthetic Titanium Rib
__Guided growth implants
- Luque trolley - historical
Compression based
- tethers the concave side
- Heuter-volkaman law

Answer 292

A

Definition
- > 45 degrees
- anterior wedging of >5 degrees across three consecutive vertebrae
>75 will cause thoracic pain
throacic > thoracolumbar

differentiated from postural kyphosis (20-45 deg) by rigidity of curve

Answer 293

A

findings
- anterior wedging across three consecutive vertebrae
- disc narrowing
- endplate irregularities
- Schmorl’s nodes (herniation of disc into vertebral endplate)
- scoliosis
- compensatory hyperlordosis
  - important to look for spondylolysis on lumbar films
hyperextension lateral xrays
- can help differentiate from postural kyphosis
- Scheuermann’s kyphosis remains inflexible on bending xray

Answer 294

A

observation alone
- indications
  - kyphosis < 60°
  - asymptomatic (or pain is mild)
- most patients fall in this group and can be treated with observation alone
bracing with extension-type orthosis (CTLSO - modified Milwaukee brace)
- kyphosis 60°-80°
- < Risser stage 3
- asymptomatic
- outcomes
  - patient compliance is often an issue
  - usually does not lead to correction but can stop progression
PSF with dual rod instrumentation +/- anterior release and interbody fusion
- kyphosis > 75 degrees
- rigid
- skeletally mature patient
- neurologic deficit
- spinal cord compression
- severe pain in adults
anterior release
- improved pedicle screw posterior constructs are often sufficient and use of anterior procedures is decreasing
- indicated in larger curves that are more rigid in nature
  - outcomes
    - studies show 60-90% improvement of pain with surgery (no correlation with amount of correction)

Answer 295

A

approach
- posterior midline to thoracic spine
arthrodesis
- current recommendation is to include entire kyphotic Cobb angle and stop distally to include the first lordotic disc
- in thoracolumbar Scheuermanns’s spine fusion usually extends to L3
- posterior bone grafting from iliac crest is recommended to enhance fusion
fixation technique
- usually a combination of pedicle screws and hooks
- hooks may be dangerous at apex of curve as they can potentially compress spinal cord
- do not always have to instrument at apex
correction technique
- in situ bending
  - may be used to obtain final correction
  - goal is to obtain correction to _final kyphosis from 40-50°_f
- posterior spine shortening technique of Ponte
  - indicated in stiff curves where correction is needed
  - done by removing spinous processes at apex, ligamentum flavum, and performing complete superior and inferior facet resection
it is important not to overcorrect as it may lead to junctional kyphosis
- do not exceed 50% of original correction

Answer 296

A

Neurologic complications
- higher than idiopathic scoliosis corrective surgeries
- must monitor with motor and somatosensory evoked potentials
Junctional kyphosis
- occurs in 20-30% of patient
- avoid by
  - making proper selection of fusion levels
  - avoid overcorrection (correction should not exceed 50% of original curve)

Answer 297

A

natural history of disease
- usually benign
- marked improvements in 24-48 hours
- complete resolution < 1 week
Kocher Criteria
- WBC >12
- unable to WB
- Fever > 38.5
- ESR > 40
- CRP >20

Answer 298

A

History
- Pregnancy History
  - diabetes, valproic acid
  - infection
  - screening
- Birth
  - presentation, delivery
- Trauma
- Family history
Physical Exam
- Skin - nevi/café au lait
- Neck ROM - klippel-feil, torticollis
- Clavicles
- Hips - Ortalani/Barlow/Galleazi
- Foot and lower limb alignment
- ROM - arthrgryposis
- Spine - gluteal cleft, hairy patch, dimples
- Reflexes
  - Grasp reflex
    - Present until 2-4 months
  - Plantar response
    - Assess for assymetry (CP)
  - Moro Reflex
    - Should be gone by 6 months
    - Consider developmental delay (CP)
  - Walking reflex
    - Should be gone by 6 months

Answer 299

A

Brachial Plexus Injury
- No pain
- See topic
Fracture
- Crepitus
- Can be difficult due to large ossification centers
- US can be very useful
- Clavicle # common, femur less common
  - After discharge must consider child abuse
  - If multiple fractures need to consider rickets or OI
- Treatment
  - Upper extremity - pin the arm to the shirt
  - Femur - Hip spica
- Prognosis
  - Only need to immobilize for one week
  - LLD or deformity is very rare
Infection
- Difficult because of their immature immune system
- Joint may or may not be red
- 76% of OM in infants will become septic arthritis
- Leukopenia may be more suggestive of infection
- Staph Aureus > GBS
- Blood cultures positive 30-40% of the time

Answer 300

A

Some reaction is normal
- Between 1-4 months and bilaterally is likely normal
- > 2mm is likely pathological
Differential
- Rickets (multiple)
- Osetogenesis imperfecta (multiple)
- Caffey disease
- Congential syphilis
- Infection
- Malignancy
- Child Abuse
- Hypervitamniosis A
- Prostaglandin infusion
- Extracorporeal membrane oxygenation
- Scurvy

Answer 301

A

Mild Contractures
- Some contractures are normal and will usually resolve with-in a few months
Early DDH
- See topic
- Klisic Test
  - Place a middle finger over the GT and an index finger over the ASIS
  - This should point to the umblicus
  - In a dislocation the line will be below the umbelicus because the hip is sitting superiorly
Congenital Knee Dislocation
- Likley due to contracture of the quads femoris
- Associated with clubfoot, arthrogryposis, larsen syndrome and myelodysplasia
- Treatment
  - Traction and closed reduction followed by progressive increased flexion with casting
  - Need to treat this before the DDH so you can get them in a pavlik
Congenital Muscular Torticollis
- Etiology is controversial - possible intrauterine compartment syndrome and fibrosis of the sternocleidomastoid muscle
- Often seen with MA and DDH
- Can often feel an olive sized mass in the SCM muscle that is replaced by fibrous tissue
- Risk Factors
  - Breech
  - Difficult delivery
- Treatment
  - Massage and stretching
  - Rule out klippel-Feil syndrome or C-spine anomalies in non-responders
SCM muscle release at 5 yo for sever cases

Answer 302

A

Exposure
- GBS - transvaginal delivery
- NICU - MRSA
Risk Factors
- invasive procedures
  - catheterization
  - heel puncture
- NICU treatment
  - thin skin
  - access lines
- prematurity/immunocompromise
- breech
Presentation
- limited movement
- edema of buttock or leg
- different resting position of hip
- anorexia irritability, lethargy
often afebrile, labs often unreliable

Answer 303

A

Dislocated septic hip

DDH
Transient synovitis of the hip
Osteomyelitis of the proximal femur/ pelvis
Henoch-Schönlein purpura
Pyomyositis
Traumatic synovitis
Fracture
Intra-abdominal pathology
Sacral agenesis
Superficial cellulitis
Superficial abscess
Psoas abscess
Pyogenic sacroiliitis
Acute leukemia
Various rheumatologic disorders
Nonspecific arthritides
Proximal focal femoral deficiency
Acute rheumatic fever

Answer 304

A

aspiration & inspection of jt fluid
- cell ct, gm stain, Cx (an/aero, fungal)
- - gm stain or Cx, WCC >30,000, PMNs >75% = septic arthritis [Cx often negative]
- Orgs: s. aureus, GBS, strep pneumo, Klebsiella, and Proteus
Imaging: XR
- capsular swelling
- widening of the jt space
- subluxation or dislocation
- radiolucency in the proximal femoral metaphysis, or periosteal elevation
Imaging: US
- effusion; difference >5 mm compared with the contralateral side is considered an indication for aspiration because the larger measurement represents an additional 5 mL of fluid

Answer 305

A

Type 1: almost nml
- 1B is coxa magna
- treatment
  - address like LCP as needed
  - may require abduction bracing and NWB for extended period of time (until reossification)
  - +/- pelvic osteotomy
Type 2: coxa breva, vara or valga
- GT overgrowth
- LLD
- treatment
  - Pavlik/brace
  - prox fem redirectional osteotomy
Type 3: fem neck involved
- angular deformity
- ante/retroversion
- pseudarthrosis
- treatment
  - varus or valgus DRO
  - ± bone graft
  - (& address LLD)
Type 4: severe; fem neck stump
- (4A) or absent neck / no hip artic
- (4B), LLD, acetab dysplasia, prox migration of femur
- treatment
  - GT arthroplasty w/ abductor advancement (+FVO & pelvic ost)
  - pelvic support procedures to prevent pistoning
  - arthrodesis
  - do nothing and allow to migrate superiorly (also has had ok reported outcomes)

Answer 306

A

hip
- more at risk in neonates because of epiphyseal artery, resolves by 18 months
shoulder
elbow
ankle
(not the knee)

Answer 307

A

age < 6 months
associated osteomyelitis
hip joint (versus knee)
delay >4 days until presentation

MRSA

Answer 308

A

Femoral head destruction
- complete destruction of the femoral head and neck, easily visible on x-ray
- salvage operations exist including varus/valgus proximal femoral osteotomies
Deformity
- physeal damage leads to late angular deformity and leg length discrepancy
Joint contracture
Hip dislocation
Growth disturbance
Gait abnormalities
Osteonecrosis
Ankylosis or fibrous union of the joint

Answer 309

A

ID consult
Emperic antibiotics - IV because often become septic
- vancomycin
- gentamycin
Urgent open irrigation through an anterior approach
- some studies will suggest that repeat aspiration can also lead to good outcomes without the need for anesthetic and scarring

Answer 310

A

CRP > 6
surgical treatment
age > 8-years-old
MRSA
Meningitis

Answer 311

A

involucrum
- a layer of new bone growth outside existing bone seen in osteomyelitis
sequestrum
- necrotic bone which has become walled off from its blood supply and can present as a nidus for chronic osteomyelitis

Answer 312

A

Staph aureus is the most common organism in all children
- recent strains of community acquired (CA) MRSA have genes encoding for Panton-Valentine leukocidin (PVL)
- PVL-positive strains are more associated with complex infections
- MRSA is associated with increased risk of DVT and septic emboli
Group B strep
- neonates
Kingella kingae
- younger age groups
Pseudomonas
- direct puncture wounds to the foot
H. influenza has become much less common with the advent of the haemophilus influenzae vaccine
Mycobacteria tuberculosis
- children are more likely to have extrapulmonary involvement
- biopsy with stains and culture for acid-fast bacilli is diagnostic
Salmonella
- sickle cell patients

Answer 313

A

types IA and IB show lucency
type II is a metaphyseal lesion with cortical bone loss
type III is a diaphyseal lesion
type IV shows onion skinning
type V is an epiphyseal lesion
type VI is a spinal lesion

Answer 314

A

Criteria
- multiple sites of apparent osteomyelitis
- pathology and cx’s are negative
- no response to antibiotics
responds well to NSAIDS
- traditionally it has been through of as having relatively benign sequelae.
- however, several case reports of growth disturbance have been reported.

Answer 315

A

Definition
- a rare chronic relapsing condition causing red patches and pustules on the soles of the feet and palms of the hands
- considered a rheumatologic condition and no infectious agents have been identified
- following the outbreak o the lesions, desquamation occurs several days later
- associated with CRMO
Treatment
- responds well to NSAIDS
- oral steroids may be indicated if NSAIDS are not effective

Answer 316

A

Up to 99% of kids are not belted appropraitely

Rear-facing = 10Kg (22lbs)
Forward-facing = 10-22kg (22-48lbs)
Booster seats = 18-27Kg (40-60lbs)
Seatbelts = 27Kg (>60lbs)

Answer 317

A

hip & abd contusions

intra-abd injuries

pelvic & L-spine fxs during deceleration

Answer 318

A

shoulder strap mid-clavicle & sternum; strap low on pelvis; knees bent 90°

Answer 319

A

Type I
- Single localized deformity (e.g., forearm pronation)
Type II
- Full expression (absence of shoulder muscles, thin limbs, elbows extended, wrists flexed and ulnarly deviated, intrinsic plus deformity of hands, adducted thumbs, no flexion creases)
Type III
- Full expression (type II) with polydactyly and involvement of non-neuromuscular systems

Answer 320

A

shoulders adducted and internally rotated (absense of shoulder muscles)
elbows extended (no flexion creases)
wrists flexed and ulnarly deviated
hands with intrinsic plus defomity topic
thumb adducted
hips flexed, abducted, and externally rotated
- subluxation or teratologic dislocation common
knees extended (classical), most of the time flexed
clubfeet
normal intelligence, facies, sensation, and viscera

Answer 321

A

allow optimal functioning in wheelchair or at computer
Elbow extension
- Triceps V-Y lengthening and posterior capsulectomy at 1.5 to 3 years (4 yrs and older?)
Wrist palmar flexion and ulnar deviation
- Flexor carpi ulnaris release, lengthening and/or transfer to wrist extensors
- dorsal carpal closing wedge osteotomy
Thumb in palm contracture and syndactyly
- Z-plasty
- syndactly release
Finger defomity
- PIP arthrodesis

Answer 322

A

nonoperative
- indications
  - bilateral dislocations (controversial)
  - unilateral dislocation in older child (controversial)
- Pavlik harness is contraindicated
closed reduction
- indications
  - rarely successful
medial open reduction with possible femoral shortening
- indications
  - unilateral dislocation
  - may lead to worse function if it leads to a hip flexion contracture because flexion deformities worsen the patient’s gait

Answer 323

A

soft tissue releases (especially hamstrings)
- indications
  - flexion contracture >30 degrees
- best performed early (6-9 months of age)
- perform before hip reduction to assist in maintenance of reduction
femoral shortening through guided growth (epiphysiodesis)
- indications
  - useful in conjunction with osteotomies
- outcomes
  - may not effectively correct chronic poor quadriceps function
supracondylar femoral osteotomy
- indications
  - may be needed to correct residual deformity at skeletal maturity

Answer 324

A

Clubfoot
- Ponseti casting
  - useful in many patients
- soft tissue release
  - first line of treatment in rigid clubfoot
  - failed Ponseti casting in more flexible types
- talectomy vs. triple arthorodesis
  - failed soft tissue releases
  - triple arthrodesis in adolescence
Vertical Talus [topic]
- soft tissue releases
  - first line of treatment
- talectomy
  - if deformities recur despite soft tissue releases

Answer 325

A

GAGs accumulate in lysosomes of chondrocytes, synovium → disorganized physeal architecture → 2° inflammatory response → irregularly shaped metaphyses → skel dysplasia

Treatment
- IV enzyme replacement
- bone marrow transplant
Improved cardiac/resp, improved hearing and resolution of hydrocephalus
no effect on orthopedic manifestations

Answer 326

A

typically none at birth. After a period of normal growth
coarse facial features
fullness of skin/brows
enlarged tongue
retained epicanthal folds
short neck
hydrocephalus
corneal clouding
inguinal/umbilical hernias
hepatosplenomegaly
otitis media, conductive hearing loss
chronic resp tract infections
cardiac d/o, carpal tunnel syn.

Answer 327

A

hip dysplasia
coarse long bones
Madelung deform, ulnar shortening
short metacarpals w/prox tapering
platyspondyly (vert body flattening)
broad clavicles, oar-shaped ribs, skull enlargement
gibbus
L1-2 beaking
Progression of skeletal abnormalities is monitored with regularly scheduled cervical spine MRI, AP and lateral scoliosis radiographs (preferably standing), AP pelvic XR, NCS of hands
lower ext XRs as clinical deformity demands (genu valgum)

Answer 328

A

C-spine
- indications
  - myelopathy
  - instability (>8mm)
  - cord signal change
- isolated occipitocerv stenosis → decompression
- MPS IV (Morquio) instability → decompression w/fusion O-C2
  - (augment w/halo if tenuous fixation)
Gibbus
- Indications
  - progression >70° or myelopathy
- Ant + Post fusion w/instrumentation (augment with brace x3 months)
Hip Dysplasia/Osteonecrosis
- indications
  - prevent progressive subluxation & arthrosis
- San Diego / Dega + FVO
- THA for young adult pts with MPS IV (Morquio) and disabling hip arthrosis
Genu Valgum
- growth modulation (8-plates) can be used
  - growth slows after age 5 & may end after age 8 in this population (NOT A NORMAL PHYSIS)
- Prox tib or distal fem osteotomy
- TKA for disabling arthrosis
Carpal Tunnel Syndrome
- carpal tunnel release (TCL)

Answer 329

A

Hurler
- most severe
- α-L-iduronidase
- Dermatan sulfate, heparan sulfate
Hunter
- Iduronate-2-sulfatase
- Dermatan sulfate, heparan sulfate
San Filipino
- most common
- Heparan sulfate, chondroitin sulfate
Morqouio
- Keratan sulfate, chondroitin sulfate

Answer 330

A

Overall - supnation is the most stable position for the DRUJ

dorsal - supnation

volar - pronation

LAC - 6 weeks

Answer 331

A

Closed reduction in LAC for 6 weeks
ORIF with immoblization
- loss of reduction at follow-up
- unable to get a reduction at time of injury

Answer 332

A

Type I:
- mildest form
- lifelong, blue sclera
- AD
Type II: lethal
- blue slcera, AR
Type III: severe
- normal sclera, AR
Type IV: intermediate
- normal sclera, AD

Answer 333

A

blue sclera
hearing loss
brownish opalescent teeth (dentinogenesis imperfecta)
wormian skull bones (puzzle piece intrasutural skull bones)
Thin skin, prone to bruising
Cardiac manifestations
increased risk of malignant hyperthermia

Answer 334

A

Defect in Type 1 Collagen
- 90% have genetic mutations
- COL 1A1, COL 1A2
- causes abnormal collagen cross-linking via a glycine substitution in the procollagen molecule
bone fragility and fractures
- fractures heal in normal fashion initially but the bone is does not remodel
- Usually decrease with age and stop after puberty
- can lead to progressive bowing
ligamentous laxity
short stature
scoliosis
codfish vertebrae (compression fx)
basilar invagination
olecranon apophyseal avulsion fx
On imaging
- __saber shins (bowing)
- generalized osteopenia
- thin corices

Answer 335

A

Fracture prevention
- early bracing
  - indicated to decrease deformity and lessen fractures
- bisphosphonates
  - indicated in most cases of OI to reduces fracture rate and pain
  - improves amulation, vertebral height improves
  - functions to increase cortical thickness by inhibiting osteoclasts
  - does not affect development of scoliosis
  - chronic use causes horizontal metaphyseal bands seen on radiographs
- growth hormone
- bone marrow transplantation
  - has been used with some success
Fracture treatment
- observation
  - indicated if child is <2 years (treat as child without OI)
- fixation with telescoping rods
  - consider in patients > 2 years
  - allow continued growth

Answer 336

A

increased diameter of haversion canals and osteocyte lacunae
replicated cement lines
increased number of osteoblasts and osteoclasts
decreased number of trabeculae
decreased cortical thickness

Answer 337

A

Delay in healing after osteotomy
transient hypocalcemia/hypophosphatemia
GI upset
NO growth inhibition
NO delay in fracture healing
NO AVN jaw
- associated with mets/chemo

Answer 338

A

Epiemiology
- prevalence is 2-3/10,000
- affects young males only
- age of onset is between 2-6 years of age
Pathophysiology
- dystrophin absence leads to
  - poor muscle fiber regeneration
  - progressive replacement of muscle tissue with fibrous and fatty tissue
  - skeletal and cardiac muscle lose elasticity and strength
Genetics
- X-linked recessive
- Xp21.2 dystrophin gene defect due to point deletion and nonsense mutation
- 30% result from spontaneous mutations
Presentation
- typically presents with waddling gait and toe walking

Answer 339

A

most are unable to ambulate independently by age 10
most are wheelchair dependent by age 15
most die of cardiorespiratory problems by age 20

Answer 340

A

outcomes
- significant positive effect on disease progression
- acutely improves strength
- slows progressive weakening
- prevents scoliosis formation
- prolongs ambulation
- delays deterioration of pulmonary function
side effects
- osteonecrosis
- weight gain
- cushingoid appearance
- GI symptoms
- mood lability
- headaches
- short stature
- cataracts

Answer 341

A

hip abductor
hamstring releases
Achilles tendon
posterior tibialis lengthenings
postoperative care
- early mobilization and ambulation to prevent deconditioning

Answer 342

A

Symptoms
- progressive weakness affecting proximal muscles first (begins with gluteal muscle weakness)
- gait abnormalities
- delayed walking
- toe walking
- clumsy, waddling gait
- difficulty climbing stairs, hopping, or jumping
- decreased motor skills
Physical exam
- calf pseudohypertrophy (infiltration of normal muscle with connective tissue)
- deep tendon reflexes present (unlike spinal muscular atrophy)
- lumbar lordosis
  - compensates for gluteal weakness
- Gower’s sign
  - rises by walking hands up legs to compensate for gluteus maximus and quadriceps weakness
- Trendelenburg sign
Labs
- markedly elevated CPK levels (10-200x normal)
  - CPK leaks across defective cell membrane
- Muscle biopsy
  - will show connective tissue infiltration and foci of necrosis
  - will show absent dystrophin with staining
- DNA testing
  - shows absent dystrophin protein
- EMG
  - myopathic
  - decreased amplitude, short duration, polyphasic motor

Answer 343

A

early PSF with instrumentation
- indications
  - curve > 20° in nonambulatory patient (treat early, < 30° curve, before pulmonary function declines)
  - can wait slightly longer (Cobb ~ 40°) if patient is responding well to corticosteroids
  - FVC drops below 35%
  - rapidly progressive curve
techniques
- extension to pelvis is controversial
Complications
- malignant hyperthermia is common intraoperatively
- pretreat with dantrolene
- intraoperative cardiac events

Answer 344

A

Equinovarus

Muscle imbalance includes
- tibialis posterior persistent function or overactivity
- gastrocnemius-soleus complex contracture
- absence of ligamentous laxity
Diagnosis
- made with gait analysis showing overactivity of tibialis posterior
stretching, physical therapy, AFO use, and botulinum toxin injections
TAL with tibialis posterior split transfer
- reroute half of tendon dorsally and insert into peroneus brevis
- indicated when affected muscle is spastic in both stance and swing phases
Rancho procedure
- TAL
- tibialis posterior lengthening
- reroute half of tibialis anterior laterally and insert into cuboid

Answer 345

A

Cerebral palsy
Congenital muscular dystrophy
Tethered cord syndrome
Diastematomyelia
Autism
Schizophrenia
Global developmental delay
Charcot-Marie-Tooth disease
Spina bifida
Transient dystonic reaction
Venous malformation of the posterior calf muscle
Ankylosing spondylitis
Congenital or posttraumatic limb-length discrepancy

Answer 346

A

Level I
- Near normal gross motor function, independent ambulator
Level II
- Walks independently, but with limitations
Level III
- Dependent ambulator
Level IV
- Minimal walking ability, uses adult assisted or powered devices for community ambulation
Level V
- Nonambulator with global involvment, dependent in all aspects of care

Answer 347

A

Shoulder internal rotation contracture
forearm-pronation / elbow flexion deformity
wrist-flexion deformity
thumb-in-palm deformity
finger-flexion deformity

Answer 348

A

< 6 months
- Pavlik
- Early Spica if needs more control
7m-5 years
- < 2-3 cm short
  - spica
- > 3 cm short or polytrauma
  - Traction with spica
  - ORIF with bridge plate
  - Flexible nails
  - Ex-fix (open or polytrauma)
6-11 years
- Length stable
  - Flexible IM Nail
- Length unstable (comminuted) or Proximal or distal
  - ORIF with plate
  - Ex-fix (damage control)
> 11 years
- Length stable and < 100 lbs
  - Flexible IM Nail
- Length unstable or > 100 lbs
  - IM nail with lateral start point
- Length unstable or Proximal or distal
  - ORIF
- Ex-fix in open/polytrauma/damage control

Answer 349

A

< 10° of coronal plane
< 20° of sagittal plane deformity
no more than 2cm of shortening
< 10° of rotational malalignment

Answer 350

A

most common complication is pain at insertion site near the knee
- reported in up to 40% of patients
- recommended that less than 25mm of nail protrusion and minimal bend of the nail outside the femur are present
increased rate of complications in patients >11 - 12 years of age or > 45 kg
increased rates of malunion and shortening in very proximal and distal fractures, as well as significantly comminuted fractures

Answer 351

A

ON risk is 1-2% with piriformis start in a patient with open proximal physes
- exact risk of ON with greater trochanter and lateral entry nails is unknown
secondary deformities of the proximal femur can occur after greater trochanteric insertions
- narrowing of the femoral neck
- premature fusion of greater trochanter apophysis
- coxa valga
- hip subluxation

Answer 352

A

Leg-Length Discrepancy
- most common complication
- overgrowth
  - 0.7 - 2 cm is common in patients between the ages of 2 - 10 years at time of fracture
    - typically presents within 2 years of injury
- shortening
  - is acceptable if less than 2 - 3 cm because of anticipated overgrowth
  - can be symptomatic if greater than 2 - 3 cm
  - temporary traction or internal fixation used to prevent persistent shortening
Osteonecrosis (ON) of femoral head
- has been reported with piriformis and greater trochanter entry nails
Nonunion
- higher risk with load bearing devices
  - external fixator or submuscular plates
- can occur after flexible intramedullary nailing in patients
  - aged over 11 years old
  - who weigh >49 kg (>108 lb)
Malunion
- typical deformity is varus and flexion of the distal fragment
- remodeling is greatest in sagittal plane (ie flexion/extension deformity)
- remodeling does not occur with rotational malalignment and therefore must be corrected at the initial surgery
- rarely symptomatic
Refracture
- most commonly seen after external fixator removal
- highest risk in transverse and short oblique fractures
- less likelihood of secondary callus formation

Answer 353

A

Benzodiazepine
- works systemically to reduce spasms and anxeity
- used with caution due to effects of sedation
Bacolfen
- works centrally to decrease GABA receptors
- works to inhibit Ca influx from pre-synapic vessels
- drug of choice for spinal cord pathology in adults
Dantrolene
- works peripherally on the muscles to decrease the release of Ca from the sarcoplasmic reticulum
Botox - not given centrally
- injected into the muscle
- works locally to block release of Ach from pre-synaptic vessels

Answer 354

A

Indications
- Congenital
  - Proximal femoral focal deficiency
- Acquired
  - Sarcomas of the hip, femur, knee, and proximal tibia
  - Failed limb salvage following tumor resection
  - Traumatic bone loss
  - Severe burns with an intact distal limb
  - Failed hip or knee arthroplasty
Contraindications
- Absolute
  - clinical sciatic nerve dysfunction
  - en-casement of the sciatic nerve
  - ankle stiffness
  - anticipated motor loss due to expected muscle resection
  - (insensate foot, unstable pelvis)
- Relative
  - __bilateral
  - psychosocial issues

Answer 355

A

Discussion of all possible complications, risks, outcomes
Expected concerns of parents/patient
Meet with physiotherapist and prothetist
- maybe other patients with ampuations
psychologist
Pre-op planning
- bone age, tanner stage
- expected growth of the child
Plastic surgery
- vascular anatstomosis - if vessels are invovled
- skin incision and closure

Answer 356

A

Careful skin incision, incorperate biopsy tract
protect sural nerves and vessles inervating gastroc, disect muscle at the pre-planned level of the femoral ostotomy ensureing adequate normal soft tissue margins, the femur is cut and remove
Soleus is elevated off the tibia, anterior and lateral compartments are disected at the osteotomy site
The tibia is turned 180deg, ensuring the NV structures move medially without tension
Fix with 4.5 hole plate, 4 above, 4 below
- can use a nail
- can also fuse the knee and rotate through the knee
anterior structures and posterior structures are tenodesed with the ankle in neutral dorsiflexion
the vessels are coiled medially and protected in soft tissue
should be able to close with tension-free closure

Answer 357

A

protect sciatic nerve
femoral vessels may need to be cut and re-anastomized
cut at least 5 cm of fibula
have plastics available for help with closure and anastomosis
children <10 can remodel a tibial plateau into a femoral head
If >10 can use a prosthesis for hip stability

Answer 358

A

Rehabilitation
- hip spica in young patients
- mobilize with crutches POD1, gentle ROM of the ankle
- start chemo 2-3 weeks after surgery
- 3 months fitted for prosthesis
  - use ishial bearing prosthesis if inadequate callus
Outcomes
- better function than endoprosthesis or other amputations
- no increase in recurrence
- minimal amounts of depression compared to healthy peers
Early Complications
- ischemia secondary to kinking
- reperfusion injury
- compartment syndrome
- wound or skin necrosis
- deep or superficial infection
- transient peroneal nerve palsy
Late Complications
- nonunion, malunion
- infection
- nerve plasy
- PFFD - derotation
  - may require further rotation procedures
  - not recommended in patients less than 12 (patients less than 6 often need more than 2 procedures)
  - doesn’t happen in patients with tumors

Answer 359

A

DDH
- hip dysplastic at birth
- recognized < 1yo
- physical examination is diagnosis
- mechanical factors
- pain in fourth/fifth decade
- no progressive subluxation
- anterior
- pavlik harness
- Salvage - THA
Neuromusulcar
- not present at birth
- progressive subuluxation with later diagnosis
- posterior-superior
- closed reduction not adequate if diagnosed later
- Salvage - castle procedure, interpositional osteotomy

Answer 360

A

Principles
- identify goals of treatment with parents
- Prevention
  - monitor
  - botox
  - antispasmatics - diazepam, baclofen, dantrolene, tziandine
  - PT
- If surgery is performed, should be bilateral, multi-level surgery
Hip at risk
- Hip abduction of <45° with partial uncovering of the femoral head on radiographs
- Reimers index <33%
- Treatment
  - Attempt to prevent dislocation with adductor release + psoas release, avoid obturator neurectomy
Hip subluxation
- Reimers index >33%
- Disrupted Shenton’s line
- Treatment
  - Treat with adductor tenotomy if abduction is restricted.
  - Consider proximal femur and pelvic osteotomies if significant dysplasia is present
Spastic dislocation
- Frankly dislocated hip
- Reimers index >100%
- Treatment
  - Open reduction with varus derotational osteotomy, + femoral shortening, and pelvic osteotomies
  - can do releases but femoral shortening is better
Salvage for failed surgery
- chiari
- Interposition arthroplasty - Castle procedure
  - femoral head resection distal to LT
  - oversew rectus and vastus lateralis
  - Interpose gluteal muscles into acetabulum
- arthrodesis
Windswept hips
- Abduction of one hip with adduction of the contralateral hip
- Brace adducted hip with or without tenotomy and release abduction contracture of abducted hip

Answer 361

A

Bilateral, multi-level, single stage surgery
Need abduction to 45deg or VDRO will result in abduction
Shortening and varus angulation has the most effect on function
in nonambulators can perform ostotomy at the LT and resect it as your psoas release
open soft tissue lengthening of psoas and adductors and distal hamstrings
lateral approach for femoral osteotomy
- osteotomize the femur and assess if it will reduce
  - 100 deg nonambulators
  - 120 deg in ambulators
- if not then will need to perform a capsulotomy
Anterior approach
- to address the capsule if you need to
- do the periacetabular dega osteotomy
  - extends from the ASIS to the triradiate and hinges through the intact triradiate
Then decide how much femoral shortening to do
- needs to be rigid fixation so ambulation can start right away
- assess with hips at 90 deg, usually 1-3 cm
- can address any LLD
- 0-15 deg derotation, don’t be overzealous
Rehab
- sitting POD1
- walking with-in one week
  *

Answer 362

A

posterolateral more common
bowed radius does not point at capitellum
abnormal radial head
dysplastic capitellum
bilateral
other skeletal dysmorphism
family history

Answer 363

A

Supine on a radiolucent table with c-arm
fixation of one bone often sufficient stability
Radial pin
- Usually do this first, because passage of the first nail is easier
- The ulna is easiest to access if you need to open to pass the nail
- Small incision over first compartment
  - Protect the superficial radial nerve, retract the tendons
- Use an awl to get your start point with flouro
- Precontour the nail, feel it hit the cortex and scrape as it is advanced, don’t use a power tool
- Reduce the fracture and rotate the nail to pass it
- If you are malreduced and having trouble might need to switch to a smaller nail
Ulnar pin
- Start on the lateral subcutaneous border of the ulna
  - Avoids the olecranon physis
- Get your point with an awl, make a small hole and then advance the nail
Assess the whole construct, ensure reduction, cut the pins
Post-op care
- Splint that allows and and elbow motion
- See at 4-6 weeks to assess
- Pins are removed at 6 moths

Answer 364

A

L3

+adductors, absent abductors (L4/5)

= increased risk of dislocation

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