Paeds Flashcards
What are the weak and spastic muscles in equinovarus foot?
Spastic: TP and TA
Weak: PB, PL
Treatment for physeal arrest:
Bar resection with interposition Indications
- Less than 50% growth plate involvement
- >2 years or 2cm growth remaining
Ipsilateral complesion of arrestIndications
- >50% physeal involvement
- Can combined with contralateral epiphysiodesis and/or ipsilateral lengthening
Treamtnet of CP hips
Adductor and psoas release ± abduction bracing
- Kids < 4 & Reimer’s index >40%
Proximal femoral osteotmy & soft tissue release
- kids > 4 OR Reimer’s index > 60%
Abduction osteotomy or girdlestone
- Chronic painful dislocation in GMFCS 5
Salvage acetabular procedure (Chiari, shelf)
- Skeletally mature with subluxation/dislocation
7 indicators of poor prognosis with LCP
Age (bone age) > 8 (bone age 6) years at presentation
Female
Decreased hip ROM (decreased abduction)
Gage sign: radiolucency in the shape of a V in the lateral portion of the epiphysis
Calcification lateral to the epiphysis
Lateral subluxation of the femoral head
Horizontal physis
Metaphyseal cysts
What pulleys need to be released in childresn’ trigger finger?
A2 & A3
Upper extremity contractures in CP
Shoulder IR
Elbow flexion
Forearm pronation
Wrist flexion
Thumb in palm deformity:
- Flexed MCP
- Extended IP
Finger-flexion deformity
Swan neck
When do you IMN a femur in paediatrics (age & weight)
> 11 years
>49 kg
Remember to use lateral start point
Classification for PFFD
Aiken
A:
- Femoral head: Present
- Acetabulum: normal
B:
- Femoral head: Present
- Acetabulum: Dysplastic
C:
- Femoral head: Absent
- Acetabulum: severly dysplastic
D:
- Femoral head: Absent
- Acetabulum: Absent
What Risser stage correspnds to the fastest growth spurt?
Risser 0
Congenital rib anomalies (ie fused ribs) have what association with congenital scoli?
occur on the concave side of the curve
makes sense
They (in and of themselves) have no effect on curve size or rate of progression
(They are not talking about phase 2 ribs here, a la Mehta angle)
Treatment algorithm of femur fractures in paeds:
- pavlik or early spica casting
7m - 5 years:
- >2-3cm shortening: traction with delayed spica casting or ORIF
6-11 years:
- length stable: flexible IM nails
- Length unstable: ORIF (plates) vs. ex-fix
Approaching skeletal maturity (>11 years)
- length stable or
- Length unstable or >100lbs: IM nail with lateral start point
- Length unstable in proximal or distal end: ORIF plate/screws
dDx for anterolateral bowing (2)
NF
tibial deficiency
Indications for anterior approach in addition to posterior approach in scolisis
Large curve (>75 degrees)
Stiff curves
Skeletally immature (Risser grade 0, boys
Indications for hemivertebrectomy in congenital scoli
Progressive curve >40 degrees
Patient <5
lumbosacral vertebra best (but can be done in thoracic)
(JAAOS 2004)
dDx for septic hip? (3)
OM
Psoas abscess
Transient synovitis
How much does a leg grow in a year and where does the growth come from?
23 mm /year
- 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)
What is H and how does it help evaluate DDH?
Hilgenreiners Line
A normal head should be below it.
newborn comes in with congenital dislocation of knee and DDH, which do you treat first and why?
Knee
b/c you can’t get a pavlik on with a dislocated knee
What x-ray is this?
What is the diagnosis?
45 degree oblique
Calcaneonavicular coalition
Should Down syndrome kids avoid contact sports? If yes, what indication?
Avoid sports if progressive radiographic instability or signs of myelopathy
When do children achieve 1/2 of the final leg length?
- girls at age 3
- boys at age 4
What is the weakest zone of the growth plate?
Hypertrophic zone
When do you need to excise a physeal bar in a growth arrest following distal femur fracture?
Indicated when deformity is present with a physeal bar of
<50% and at least 2 years or 2 cm of growth remaining
Risk factors for birth fractures
Vaginal deliveries
breech presentation
prolonged labor
macrosomia (>4.5 kg)
Name the 3 (Mehta) indicators of progression in congenital idiopathic scoliosis
Cobb >20 degrees
Phase 2 rib
Rib-vertebral angle difference (RVAD) >20
Thoracolumbar curve
Kid with septic hip: what position will the hip be held in?
Flexion, abduction, ER
5 associated conditions of external tibial torsion:
Miserable malalignment syndrome
Osgood Schlatter disease
Osteochondritis dessicans
Early degenerative joint disease
Neuromustular conditions
What is is the predictable angular deformity with pediatric tibial fractures treated with LLC?
Varus if fibula intact.
Valgus if fibula also broken.
Flexion type SCHF results in cubitus ____________?
Valgus
It causes varus displacement, leading to cubitus valgus
BLocks to reduction in DDH
Labrum
Inverted limbus
capsule
transverse acetabular ligament
ligamentum teres
pulvinar
Common injuries associated with TL spine trauma in paeds
Same as in adults:
GI: Small bowel most common
Lung contusion/pneumothorax
Head injury
7 donor options for nerve transfer in Brachial plexopathy?
sural
intercostal
spinal accessory
phrenic
cervical plexus
contralateral C7
hypoglossal
Zone of injury for proximal humerus fractures?
Zone of provisional calcification (part of hypertrophic)
However may go through several
Spares proliferative
JAAOS 2015
2 life-threatening intra-operative complications in DMD:
intraoperative cardiac event
malignant hyperthermia
Consult anesthesia & cardio preop
What condition can be confirmed using fibroblast culturing to analyze type I collagen in equivocal cases?
OI
(best for type 4)
Presence of what is the main finding that differentiates NF2 from NF1?
bilateral vestibular schwannomas
** Also the DONT get scoliosis
What is P and how does it help evaluate DDH?
Perkins Line
Femoral head ossification should be medial to this line
Kid with hemihypertrophy. What must you do?
serial ultrasounds q3 months until age 7,
then physical exam q6 until skeletal maturity
TO RULE OUT WILM’S TUMOUR
Criteria for Septic Hip
Kocher:
Fever >38.5C
WBC >12,000mm^3
ESR >40mm/h
Refusal to weight bear on affected side
3/4 = 93% chance of septic arthritis
Risk factor for AVN of the hip
Trauma
Steroid use
Radiation
How do you differentiate CVT and oblique talus on x-ray
oblique talus:
navicular will reduce on plantarflexion latearl
Meary’s angle <35 degrees
In SCFE, the screw should be placed in what relationship to the intertrochanteric line to avoid what complication?
lateral to the intertrochanteric line
To avoid screw impingement
Criteria/algorithm for septic arthritis vs. transient synovitis
History of fever (T > 38.5C)
Non-weight-bearing
ESR > 40 mm/h
WBC > 12,000 cells/mm3. T
The probability of septic arthritis is
- 0.2% with zero predictors
- 3.0% with one predictor
- 40.0% with two predictors
- 93.1% with three predictors
- 99.6% with four predictors.
7 options for interpositional graft after resection of physeal bar:
Fat
PMMA
Cranioplast (like PMMA: takes longer to set, less exothermic reaction, less chance of heat necrosis)
Bone wax
Cartilage
Muscle
Silicone
3 indications for ORIF in pediatric distal femur fracture?
- open fracture
- failed closed reduction of SH1 or SH2
- perisoteum usually infolded in these case
- SH 3 - 4 in order to get anatomic reduction of joint surface
Reduction Maneuver for displaced medial epicondyle fracture into joint?
Robert’s Technique
- Supination of the forearm - stretches flexor/pronator mass
- Valgus stress on the elbow - opens up ulnohumeral joint medially and stretch FP mass
- Extending the wrist and fingers - causes a pull on FP mass
- Early motion within 3-5 days minimizes risk of stiffness
Treatment for dynamic supination post clubfoot
Tib ant transfer to lateral cuneiform
When can you determine the Herring stage in LCP?
Fragmentation stage (~6 months after symptoms start)
Most common cause of revision following early spica casting of a femur fracture in paeds?
Loss of reduction
Although rare
Classification of tibial spine/eminence fractures
Meyers & McKeevers
I: undisplaced
II: displaced with posterior hinge
III: completely displaced with no bony contact
IV: comminuted
Diagnosis
Bisphosphonates on OI
Results in metaphyseal banding at each dosing
How does TA compare to PL in:
a) Clubfoot
b) Cavo - varus foot
a) TA stronger
b) TA weaker
Name 3 conditions that can present with teratologic hip?
arthrogryposis
myelomeningocele
Larsen’s syndrome
What is the Sofield-Miller procedure?
Realignment osteotomy with rod fixation for OI
Fassier-Duval rods can be used.
Three methods of predicting LLD?
- 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
- Multiplier method
DDH U/S:
What is minimal age?
4-6 weeks.
Treatment options for Delbet 1–4?
- Type 1 b is always ORIF
- Types I-III can be treated with:
0-3yrs of age = smooth wires +/- spica
4-10yrs of age = 4.5-6.5 mm cannulated screws
>10 yrs old = 6.5-7.3mm cannulated screws
- Type IV fractures are treated with pediatric or adult DHS depending on age.
*** Consider Capsular decompression to reduce pressure, usually for type 2s, however this is controversial
How much fill of the femoral canal do you want with nancy nails?
80%
(Therefore each nail should be diameter x 0.8 / 2)
(i.e. canal is 1 cm, then use 2 x 4 mm nails)
X-ray findings of clubfoot
Dorsiflexion lateral (Turco)
- hindfoot parallelism between talus and calcaneus (talocalcaneal angle <35 deg)
AP:
- Kite’s talocalcaneal angle <20 degrees (N = 20-40)
- Talus - 1st MT angle <5 degrees
Most common complication after distal femoral physeal injury
LLD/growth arrest
Name 5 non-ortho manifestations of OI
Blue sclera
Hearing loss
Brownish opalescent teeth (dentiogenesis imperfecta)
Wormian skull bones (puzzle piece intrasutural skull bones)
Increased risk of malignant hyperthermia
Asymptomatic Paediatric Isthmic spondy, soccer player. Do you limit sports?
No
Manage with close observation and no restrictions
Most important factors to rule out septic arthritis (2)
Patient weight bearing on affected limb
CRP
What motion is most deficient in Sprengels?
Abduction
4 technical considerations for fixation of pediatric distal femur fracture?
- avoid multiple attempts at reduction
- avoid physis with hardware if possible
- if physis must be crossed (SH I and SH II with small Thurston-Holland fragments), use smooth k-wires
- SH II fracture, if possible, should be fixed with lag screws across the metaphyseal segment avoiding the physis
- postoperatively follow closely to monitor for deformity
The patella vascular supply is composed of _____ branches which stem from which 3 larger arteries?
6 small branches ( the geniculates plus anertior tibial recurrant)
Popliteal, Superficial femoral and Anterior Tibial
3 associations with tarsal coalition:
Fibular hemimelia
Apert’s
PFFD
“People with tarsal coalition masturbate a lot: FAP FAP FAP”
Main blood supply to femoral head in kids >4
Medial femoral circumflex artery
via: posterosuperior lateral epiphyseal branch
&
posterior inferior retinacular branch
Specifically for the distal radius, when do you want to operate to excise a distal radius physeal bar?
If >2mm (not cm) of growth remains
progressive deformity
symptomatic
5 risk factors for LCP
Positive family history
Low birth weight
Abnormal birth presentation
Second hand smoke exposure
Asian, Inuit, central european descent
Manifestations of Achondroplasia
Rhizomelic dwarfism
Affects Proliferative Zone of Growth plate
frontal bossing
Foramen magnum stenosis
Kyphosis
Lumbar stenosis
Decreasing interpedicular distance from L1-L5
Champagne glass pelvis
Genu Varum
Trident Hands
V-shaped Physis
What happens if the GT apophysis is prematurely arrested?
hip will go into valgus
(medial side keeps growing)
Best pin configuration for SCHF?
Lateral divergent pins (2 vs 3)
Describe the deformity in congenital vertical talus
Rockerbottom foot:
Hindfoot: equinovalgus (everted & lateralized)
Midfoot: rigid dorsiflexion
Forefoot: abducted & dorsiflexed
What part of the growth plate does a SCFE occur in?
Hypertrophic zone
caused by weakness in the perichondral ring
MPS with least spinal abnormalities?
San Filippo
only has scoliosis, and even that is rare
JAAS 2013
Lateral Pillar Classifiation: (LCP)
Group A: lateral pillar maintains full height
Group B: Lateral pillar maintains >50% height
Group B/C: Lateral pillar narrowed (2-3mm) or poorly ossified with ~50% height
Group C: Lateral pillar maintains
Cause and treamtent of stiff-knee gait in CP?
Cause: rectus femoris firing out of phase
Treatment: transfer of distal rectus femoris tendon
Characteristic lesion in Fredrich’s ataxia
Spinocerebellar degenerative disease, therefore, lesions in:
- Dorsal root ganglia (peripheral)
- Corticospinal tracts (central)
- Dentate nuclei in the cerebellum
- Sensory peripheral nerves
First line treamtent in tarsal coalition?
trial of non-op with immobilization or orthotics - always
What is the best treatment for Sever’s disease?
Calcaneal apophysitis
Best is achilles stretching - may decrease recurrence
No role for operative management
What must you do for workup in patient with congenital scoliosis?
Echo: cardiac defects - 10%
Renal ultrasound and GU workup - genitourinary defects - 25%
MRI - spinal cord malformations
Surgical treatment for CMT claw toes?
Jones procedure
(transfers the extensor tendons of the great and lesser toes through the bone into the metatarsal neck)
What is angle A and how does it help evaluate DDH?
Acetabular Index
varies with age (decreases)
normal is less than 25 deg 2yo kid
(remember 2yo is ~20deg)
4 indications for surgery in infantile Blount’s
Stage I and II in children > 3 years
Stage III, IV, V, VI in children <3 years
failure of brace treatment
metaphyseal-diaphyseal angles > 20 degrees
Can a Klippel Feil patient play contact sports?
No if they have fusion of C3 and above
WHat joints are most commonly invovlved in JIA?
knee > hand/wrist > ankle > hip > C-spine
What are ideal factor levels in a hemophiliac for :
- acute hematomas
- acute hemarthrosis and soft tissue surgery
- skeletal surgery
- acute hematomas
- increase blood factor levels to 30%
- acute hemarthrosis and soft tissue surgery
- increase blood factor levels to 40-50%
- skeletal surgery
- increase blood factor levels to 100% for first week following surgery then maintain at > 50% for second week following surgery
Indications for contralateral pinning in SCFE
High risk patients:
Endocrinopathy
Obese
Young age, indicated by:
- Boys
- Girls
- Open Triradiate cartilage
Adequate reduction parameters for Delbet 2-4?
Type II
- accept <2mm cortical translation
- <5 deg angulation
- no malrotation
Type III and IV
- accept <10 degrees of angulation
4 negative prognostic indicators of pediatirc spetic arthritis?
age
associated osteomyelitis
hip joint (versus knee)
delay >4 days until presentation
Why do you need a pre-op MRI in sprengels?
To identify omovertebral bar
By definition, what three characteristics have to be present for JIA diagnosis?
- Persistant inflammatory arthritis
- > 6 weeks
- Patient < 16 years
Kocher Criteria for Septic Hip
T > 38.5C
WBC > 12
ESR > 40
Refusal to weight bear
2/4: 40% chance
3/4: 93% chance
4/4: 99% chance of septic hip
What are the primary surgical options for the three types of CP gait?
Toe walker - Gastrocs release vs. TAl dependant on Silverskiold test
Crouch Walker - multiple simultaneous soft tissue releases (hip, knee, ankle)
Stiff Knee - transfer of distal rectus femoris tendon
What construct for SCHF pinning has the most biomechanical stability?
Medial and lateral crossed pins
But we don’t use them b/c of the risk of injury to the ulnar nerve
WHen i say Olecranon apophysis avulsion fracture, you say:
osteogenesis imperfecta
4 indications for open reduction of pediatric elbow dislocation?
- open dislocation
- inability to acheive closed reduction
- incarcerated fragment - usually medial epicondyle or tip of coronoid
- seves instability following closed reduction
What is the pediatric equivalent of Lauge Hansen classification?
Diaz / Tachdjian
SAD, SER, PER, SPF (Supination plantar flexion)
(compare Lauge-Hansen which is SAD, SER, PER, PAB)
What is normal range for IR and ER of hip?
IR = 20-60
ER = 30-60
4 options for elbow release in arthrogryposis
Triceps to biceps
Steindler flexorplasty
Pec Major to biceps
Triceps V-Y lengthening and posterior capsulectomy
Describe the anatomy of CVT
Dorsal structures are tight (navicular dorsally dislocated)
Rocketbottom foot
- hindfoot equinovalgus
- rigid midfoot dorsiflexion
- forefoot abducted and dorsiflexed
Tib post is tight
What are the 2 cardinal manifestations of Marfan’s syndrome?
Aortic root dilatation
Superior lens dislocation
If both are present, do not need genetic testing - therefore test for both (Echo and optho consult)
Indication for surgery in LCP?
Lateral pillar B, B/C, C in kids >8 (bone age >6)
They do better with pelvic/femoral osteotomy
What did Sillence classify?
OI
How does a patient with SCFE clinically present?
Pain
Obligatory ER
At what level of SPina Bifida is hip dislocation most common and why?
L3 - uopposed hip flexion and adduction
Name 3 syndromes with abnormal ossification of secondary growth centers
Spondyloepiphyseal dysplasia
Multiple epiphyseal dysplasia
diastrophic dysplasia
How does a positive coleman block test effect treatment of cavo-varus foot?
Rigid hindfoot varus.
Do: calcaneal valgus producing osteotomy
What antibiotic should you avoid in paeds?
Cipro (fluorquinolone) b/c of risk of cartilage damage
DMD Scoliosis
1) 1 surgical indication that is unique compared to AIS
2) To pelvis?
1) respiratory function
2) Controversial
In what condition is a full-length semi-rigid insole orthotic with a depression for the first ray and a lateral wedge useful?
Mild cavo-varus foot
LLD Principals:
a)
b) 2-5 cm
c) > 5 cm
a)
b) 2-5 cm = shorten long side
c) > 5 cm = lengthen short side +/- shorten long side
What are the only 2 recommendations with “moderate” or above strength in the AAOS CPG guidelines forDetection of DDH and management up to 6 months?
- No universal screening
- performing an imaging study before 6 months of age in infants with one or more of the following risk factors: breech presentation, family history, or history of clinical instability.
Who gets Gower’s sign? Describe it
Rises by walking hands up legs to compenate for gluteus maximum and quadriceps weakness
Conditions that may cause SCFE?
- obesity (most important)
- hypothyroidism (labs show elevated TSH)
- osteodystrophy of chronic renal failure
- Rickets
In LCP, when do you want to perform surgery (if indicated)
initial or fragmentation phase
no positive effect has been found for containment surgery performed after initial or early fragmentation stage
What are the buzz words you need to say if you are proposing a closed reduction with hip spica for DDH?
- Arthrogram to confirm reduction
- Medial dye pool should be 5 mm or less with no interposed limbus
- Immobilize in 100 flex, 45 abduction and neutral rotation (SAFE ZONE)
- CT to confirm (with SELECTIVE CUTS)
- Change after 6 weeks
- 12 weeks total
- Do adductor tenotomy if unstable safe zone (i.e. if too much abduction required to hold reduction)
Muscle imbalance in equinovalgus foot?
Opposite of equinovarus
TA/TP weak
PB/PL strong
Classification of CP Hips & Treatment
Hip at risk:
- Hip abduction
- Partial uncovering of femoral head
- Remier’s index
- Treatment: Prevent dislocation: Adductor release ± psoas release, Avoid obturator neurectomy
Hip Subluxation
- Reimer’s index >33%
- Disrupted Shenton’s line
- Treatment: Adductor tenotomy if abduction tight, Proximal femur and pelvic osteotomy if significant dysplasia
- Outcome: Reimer’s >60-70%, hips will dislocate
Spastic dislocation
- Frankly dislocated hip
- Reimer’s index >100%
- Treatment: Open reduction with varus derotational osteotomy, ± femoral shortening & pelvic osteotomy
Windswept hips
- Abduction of one hip with adduction of contralateral one
- Treatment: Brace adducted hip ± tenotomy, Release abduction contracture of abducted hip
What is a Charnley WIlliams rod used for?
ORIF of NF tibial pseudoarthrosis
Principals for bladder extrophy repair?
Multidiosciplinary
Gen surg + Urologist
stage I: primary closure of bladder (newborn)
stage II: epispadias repair in males (1-2 y/o)
stage III: bladder neck reconstructions (4 y/o)
pelvic osteotomies may be performed at any stage of process
2 methods of percutaneous reduction of radial head fracture
- K-wire joystick technique
- Metaizeau technique
involves retrograde insertion of a pin/nail across the fracture site
fracture is reduced by rotating the pin/nail
What is a reason why intra-thecal baclofen is preferred over PO for CP?
PO associated with cognitive impairment.
Treatment of dislocated hip in myelomeningocele?
Surgical reduction of hips in patients with spina bifida is associated with a high failure rate and therefore treatment indications are controversial.
Reduction for patients with L4 level is most controversial and may be considered if unilateral.
Dislocated hips in patients with L3 level and above are typically left alone.
dDx for cavovarus foot (5)
Charcot-Marie-Tooth
Freidreich’s ataxia
Cerebral palsy
Polio
spinal cord lesions
Anterolateral bowing:
what’s the chance this patient has NF?
What is the chance an NF patient has anterolateral bowing?
what’s the chance this patient has NF?
50%
What is the chance an NF patient has anterolateral bowing?
10%
3 INDICATIONS for nerve repair/grafting in Brachial plexopathy?
complete flail arm at 1 month of age
Horner’s syndrome at 1 month of age
lack of antigravity biceps function between 3-6 months of age
Orthopaedic Manifestations of Charcot Marie Tooth (HMSN)? (4)
pes cavus
hammer toes
hip dysplasia
scoliosis
5 conditions associated with Cavovarus foot?
- Charcot-Marie-Tooth
- Freidreich’s ataxia
- Cerebral palsy
- Polio
- spinal cord lesions
4 dDx for leg bowing in kids?
Physiologic
Blount’s disease
Osteogenesis imperfecta
Rickets/osteomalacia
Syndromic
Describe Birch Classification for fibular hemimelia
Type I: Functional foot
try to save foot
Ia: 0-5% inequality
Ib: 6-10%
Ic: 11-30%
Id: >30%
Type II: Nonfunctional
IIa: functional UE: amputation
IIb: nonfunctional UE: consider salvage
Differential for unilateral valgus (3)
Proximal metaphyseal tibial fracture (Cozen’s)
UnilateralPhyseal injury
- Trauma
- Infection
- Vascular insult
Benign tumour
- Fibrous dysplasia
- Osteochondroma
- Ollier’s disease
2 optioins for treating elbow flexion contractures
Clarke’s pectoral transfer
Steindler’s flexorplasty
Treatment of hallux varus
Conservative:
- most resolve with time
Can do abductor hallucis release
excsision of central epiphyseal bracket
Defect in diastrophic dysplasia?
DTDST gene (SLC26A2)
codes for diastrophic dysplasia sulfate transporter gene on chromosome 5
How do you estimate blood volume in a kid?
75 - 80 mL/kg
Principals of Proximal tibia/fibula valgus osteotomy for Blounts?
- overcorrect into 10-15° of valgus because medial physeal growth abnormalities persist
- distal segment is fixed in valgus, external rotation and lateral translation
- temporary lateral physeal growth arrest with staples or plates can be used
- include a bar resection (epiphysiolysis) when a physeal bar is present (Langenskiold V and VI)
- consider hemiepiphysiodesis if bar > 50%
- medial tibial plateau elevation is required at time of osteotomy if significant depression is present
- consider prophylactic anterior compartment fasciotomy
How do you tell if the tib post or tib ant is the driving force in an equinovarus foot?
Confusion test
in a seated position, get patient to perform resisted hip flexion
If toes dorsiflex and supinate, then likely TA is the driving force
Most common 2 complications after fixation of femoral neck fracture in paeds:
- AVN
- coxa vara
4 conditions associated with CVT?
- myelodysplasia (common)
- arthrogryposis
- diastematomyelia
- chromosomal abnormalities
High association with genetic or neuromuscular disorder (50%)
3 nonorthopaedic conditions of marfans?
- cardiac abnormalities
- aortic root dilatation
- possible aortic dissection in future
- mitral valve prolapse
- superior lens dislocations (60%)
- spontaneous pneumonthoraces
NIH Consensus Development Conference Statement diagnosis criteria for NF1?
Two or more of the following:
- six or more café-au-lait macules over 5 mm in greatest diameter in prepubertal individuals and over 15 mm in postpubertal individuals.
- two or more neurofibromas of any type or one plexiform neurofibroma.
- freckling in the axillary or inguinal region.
- optic glioma.
- two or more Lisch nodules (iris hamartomas).
- a distinctive osseous lesion such as sphenoid dysplasia or thinning of long bone cortex with or without pseudarthrosis.
- a first-degree relative (parent, sibling, or offspring) with NF-1 by the above criteria.is based on presence of both
Bado Classification
Monteggia Fractures
Dislocation goes where the apex is (THINK the apex pushes the radial head out)
I: apex anterior proximal ulnar fracutre with anterior dislocation of radial head
II: Apex posterior ulna fracture with posterior dislocation of the radial head
III: Apex lateral ulna fracture with lateral dislocatio nof the radial head
IV: BBFF with anterior dislocation of radial head
Contraindications to Pavlik harness (4)?
- Abnormal muscle function (i.e. spina bifida, spasticity)
- Age over 6 months
- Teratologic hip dislocation
- Failure of Pavlik treatment for 3 weeks.
When evaluating function of CP kid, what are 6 areas to ask about?
- nutritional status
- respiratory function
- sitting/standing posture
- upper and lower extremities function
- communication skills
- acuity of hearing and vision
What are the upper limits of normal for valgum and IM distance in a patient over the age of 7?
- valgum < 12 degrees
- IM distance < 8cm
in SCHF with median sensory symptoms, what is the major complication that is now at increased risk/most commonly to be missed?
Compartment syndrome
They cannot give the regular symptoms (pain) of compartment syndrome so the risk goes up
Give general principles of operative treatment for PFFD
Limb Lengthening
- if predicted LLD <20cm
- If femoral length >50% of opposite side
Amputation ±prosthesis
- If femoral length <50% contralateral side or LLD >20cm
- If foot is proximal to level of contralateral knee
- If prosthetic knee will not be below the level of the contralateral knee
*based on level of knee - must have it normal to walk normal
Hip Fusion
- If absent acetabulum (Aitken D), fuse residual limb to pelvis and make knee into a hip
4 indications for osteotomy in Infantile Blounts?
What osteotomy would you do?
- Stage I and II in children > 3 years
- Stage III, IV, V, VI in children
- failure of brace treatment
- metaphyseal-diaphyseal angles > 20 degrees
proximal tibia/fibula valgus osteotomy
Surgical treatment of hip dislocation in myelodysplasia is controversial b/c of high failure rates. What is the anatomic cause of failure? Be specific
Paralytic hip dislocation (not teratologic)
B/c of paralysis of the hip abductors and extensors and unopposed pull of the hip adductors and flexors
This leads to high relapse rates
Non-ortho associations of hemihypertrophy (2 major types)
Malignant intra-abdominal tumours
- Wilm’s - most common
- adrenal carcinoma
- hepatoblastoma
GU abnormalities
- medullary sponge kidney
- polycystic kidney
- inguinal hernia
What is von Recklinghaussen disease?
NF1
Best x-ray view for lateral condyle fracture?
internal oblique
What disease causes progressive loss of alpha-motor neurons in the anterior horn of the spinal cord?
SMA
“A” is for alpha motor neurons and anterior horn
Management of equinovarus foot
Flexible:
AFO, casting, botox
tendon transfer, either:
TA/TP split transfer
TA to cuboid
TP to brevis
depending on what’s tight
Rigid:
lateral closing wedge calc ostetomy
Name the physeal zone associated with:
SCFE (not renal)
Rickets (provisional calcification zone)
Enchondromas
Mucopolysacharide disease
SED
MED
hypertropic
What are the 5 physeal zones?
- Reserve
- Proliferative
- Hypertrophic
- Primary Spongiosa
- Secondary Spongiosa
What is the best indicator of peak growth?
Risser 0 or closure of triradiate (occurs at same time)
WHere does a Salter osteotomy hinge on?
Symphysis Pubis
1 cut from AIIS to sciatic notch
What dysplasia has metaphyseal changes of the tubular bones with normal epiphysis?
metaphyseal chondrodysplasia
Kid with MPS comes in with burning in radial 3 digits, worse at night, some clumsiness of the hands. Top 2 dDx
Carpal tunnel syndrome: MPS is one of the most common causes of CTS in kids
cervical myelopathy (unless its San Fillipo - no C-spine issues)
What is the only lower limb deficiency with a defined inheritance pattern?
What is the inheritance pattern and what must you do once diagnosis is made?
Tibial deficiency
AD
Must counsel parents of risk with further children
What 2 actions may help reduce AVN rates in femoral head/neck fractures in paediatric patients?
early reduction
Joint decompression (hematoma aspiration or core decompression)
Treatment for lateral pillar A/B in kid less than 8 (bone age less than 6)
Nonoperative
They do well regardless
Treatment for calcaneovalgus foot?
observation and parental stretching
3 exam findings consistant with tarsal coalition?
- flattening of arch
- valgus hindfoot
- peroneal spasticity
In clubfoot release, what has the greatest influence on functional outcomes?
Extent of soft tissue release
4 complications of radial head fractures
Which 2 are related to open reduction?
- Decreased range of motion
- loss of pronation more common than supination
- Radial head overgrowth
- Osteonecrosis
- up to 70% of cases occur with open reduction
- Synostosis
- occurs in cases of open reduction with extensive dissection or delayed treatment
Classification of Sprengel’s:
see chart
How long do you wear a Pavlik?
23 h/day for 6 weeks then ween over 6 weeks.
8 orthopedic manifestations of Downs?
- generalized ligamentous laxity and hypotonia
- C1-2 instability
- hip subluxation and dislocation
- patellofemoral instability and dislocation
- scoliosis & spondylolithesis
- pes planus
- primus varus
- SCFE
Surgical option in resistant vertical talus?
talectomy
Preferred fixation of SHII distal femoral physeal fracture
Lag screw through the metaphyseal flare piece
Valproic acid has what detrimental effect on surgery?
Increases bleeding time
Name the physeal zone associated with:
- Gaucher’s
- diastrophic dysplasia
Reserve
Spine changes in NF1
Vertebral scalloping
Rib penciling
TP spindling
Vertebral wedging
Paravertebral soft-tissue mass
Short curve with severe apical rotation
intervertebral foraminal enlargement
Wideened interpediculate distance
Dysplastic pedicles
What fracture type has the highest rate of growth arrest in the body?
SH4 of medial malleolus
How is CP gait desribed/classificed
Sagittal plane
- Jump
- Crouch
- Stiff knee
Transverse plane
- IR
- ER
- Neutral
Coronal plane:
- Genu varum
- Genu valgum
- hip adduction
4 indications for OR in pediatric pelvis fracture?
(radiographic)
Type 1 Avulsion Injuries with > 2-3 cm displacement
Type II Iliac Wing Fractures with > 2-3 cm displacement
Type III pelvic ring with displaced acetabular fractures > 2mm
Type IV pelvic ring with instability and > 2 cm pelvic ring displacement
Risk of contralateral SCFE in otherwise healthy kid?
50%
(10-60%)
this is asking the risk of contralateral SCFE, not bilateral SCFE
Bilateral is 20% in normal, 80% in endorinopathy
Acceptable Alignment BBFF?
15 degrees, rotation >45 degrees. bayonet apposition ok
>10: >10 degrees, rotation >30 degrees
New idea says NO rotation can be tolerated
both bone forearm fractures in children> 13 is an indication for surgery
Who gets Duchenne muscular dystrophy?
Males only
X-linked recessive
4 signs of residual DDH in kid > 3 months
Limited hip abduction
Pelvic obliquity
+ galeazzi
Trendelenburg gait
4 joints with intra-articular metaphyseal cortex
hip, shoulder, elbow, and ankle
What are Woodward and Green procedures used for and what is the difference?
Sprengels
Woodward involves detachement of medial scapular muscles from their origin on the spinous process and re-attachemnt after inferior migration of the scapula.
Green is similar except you detach the muscular insertions off of the medial border instead.
*** Remember to consider clavicel osteotomy to reduce the chance of nerv einjury.
Spinal manifestations of Achondroplasia:
Foramen magnum stenosis (NOT c-spine instability)
Kyphosis
Lumbar stenosis
Ulnohumeral dislocation in paeds: What fracture pattern are you worried about?
Medial epicondyle fracture
make sure it’s not incarcerated in the ulnohumeral joint. If it is, it’s an indication for surgery
What is Nail-Patella syndrome?
Hypoplastic Nails and Petallae
AD inheritance
Also includes:
Laxity
scoliosis
scapular hypoplasia
presence of cervical ribs
amongst other things
2 techniques to reduce a radial head fracture
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
AAOS guidelines for paediatric femoral shaft fractures
6 months - 5 years: spica
5 years - 11 years: TENS, IMN or submuscular plating
>11 years: submuscular plating or TENS
They make NO reommendations on weight in the official criteria
JAAOS 2011 states:
We believe that regardless whether a patient has an unstable comminuted or oblique fracture, rigid nails are appropriate for patients aged >11 years who weigh >49 kg.
We recommend the lateral trochanteric approach to avoid the risks associated with starting at or near the piriformis and near the tip of the trochanter
Indications for selective thoracic fusion:
Non-structural lumbar curve (Lenke 3 or above)
Lower end vertebra touches CSVL
Lenke 1C, 2C, 3C, 4C
No significant sagittal imbalance
Major Thoracic Curve
Double thoracic Curve
What does the VilleFranche classification describe?
Ehlors Danlos
Villefranche Classification (1998)
Classical - Type I (gravis) and Type II (mitis)
COL5A1 or COL5A2 mutation
There are several other sub types
Best surgical appraoch to CP HV?
1st MTP fusion +/- Akin
Best test for looking at cross-sectional imaging of tarsal coalition
Best test to look for fibrous coalition
CT best to look at cross-sectional imging
MRI best to look at fibrous coalitions
7 reasons that in-toeing requires further investigations?
Developmental delay
prematurity
Pain
LLD
Progressive deformity
Family history of rickets, skeletal dysplasias, mucopolysaccharidoses
Limb rotational profiles 2 standard deviations outside the normal
Abnormal physical exam (Dwarf, syndromic, abnormal neuro exam (ie reflexes)
1.
What is the risk of AVN for Delbet Type 1B?
Nearly 100%
(transphyseal proximal femur fecture with displacement of epiphysis out of acetabulum)
2 complications of transphyseal distal humerus fracture?
Cubitus varus
Medial condyle AVN
Complications seen with too much abduction in Pavlik?
AVN
What is the muscle imbalance in dynamic supination post Ponsetti Casting?
Tib ant overpull in relation to peroneal muscles
tarsal coalition
Chance of it being bilateral?
Patient with 1 coalition has what percentage of having another?
50% chance of being bilateral
20% chance of a second coalition if they have 1
What is the most common cause of early death in Morquio?
Spinal cord stenosis
JAAOS 2013
Name 4 featuers of Acnohdroplasia (there are about 15)
classic rhizomelic dwarfism
- adult height ~ 50 inches
- humerus shorter than forearm and femur shorter than tibia
- normal trunk
facial features
- frontal bossing
- button noses
- small nasal bridges
extremities
- trident hands (inability to approximate extended middle and ring finger)
- bowed legs (genu varum)
- radial head subluxation
- muscular hypotonia
spine
- thoracolumbar kyphosis (often resolves at weight-bearing age)
- excessive lordosis (due to short pedicles)
Othopaedic Manifestations of CMT?
Scoliosis
Pes Cavus
Hammertoes
Hip dysplasia
What are the 4 deformities of clubfoot?
- midfoot Cavus (tight intrinsics, FHL, FDL)
- forefoot Adductus (tight tibialis posterior)
- hindfoot Varus (tight tendoachilles, tibialis posterior, tibialis anterior)
- hindfoot Equinus (tight tendoachilles)
In a patient with Sprengel’s, what is the most likely associated abnormality to expect?
- Scoliosis
- Klippel-Feil
(JAAOS)
see chart
WHat is the spine deformity in multiple epiphyseal dysplasia
None
dDx of knee or thigh pain paediatric patient:
Knee pathology
Hip pathology
- SCFE
- LCP
- Septic hip
- Transient synovitis hip
*knee/thigh pain mandates a workup for SCFE
How can you be fooled into thinking someone doesnt have an achilles contracture?
(I.e a false negative)
- the hindfoot valgus deformity must be manually corrected first before testing for achilles contracture
- a valgus heel can mask an equinus contracture by allowing a shortened path for the achilles
What are three ways to assess coronal deformity of the L/E on AP xrays?
1) mechanical axis
2) mLDFA 88 (range 85°-90°) and mMPTA (range 85°-90°)
3) Tibial Femoral Angle
Describe the Beighton-Horan scale.
5 or more on 9-point Beighton-Horan scale defines joint hypermobility
- passive hyperextension of each small finger >90° (1 point each)
- passive abduction of each thumb to the surface of forearm (1 point each)
- hyperextension of each knee >10° (1 point each)
- hyperextension of each elbow >10° (1 point each)
- forward flexion of trunk with palms on floor and knees fully extended (1 point)
List 5 features associated with in-toeing that necessitate further workup:
Pain
LLD
Progressive deformity
Family history of rickets, skeletal dysplasias, mucopolysaccharidoses
Limb rotational profiles 2 standard deviations outside the normal
5 things affecting Hypertrohpic Zone of growth plate
SCFE
MED
SED
Schmids
Fractures (SH1)
Ricekts
Enchondromas
What is the natural history of OI fractures with age?
The symptoms improve with age
What are two options to treat AVN
following proximal femur fracture?
● Vascularised free fibula graft
● Core decompression
Preferred treatment of congenital vertical talus
Reverse ponsetti casting +
surgial reduction & pinning of talonavicular joint +
TAL
Paediatric olecranon fractures are highly suspicious for:
Osteogenesis imperfecta
3 indications for operative percutaneous reduction of radial head fractures in peads?
> 30° of residual angulation
3-4 mm of translation
Order of correction in ponsetti method
Cavus first (midfoot)
Then adduction & Varus (hindfoot)
Equinus last
(CAVE)
5 causes of hemihypertrophy
idiopathic
neurofibromatosis
beckwith-weidemann syndrome
Klippel-trenauney syndrome
proteus syndrome
What part of the physis does a SCFE occur in?
Hypertrophic
What percentage of LCP patients will eventually need THA?
50%
What single finding dictates how often slit lamp exams are necessary in JIA?
Presence of ANA
If negative: Every 6 months
If positive: Every 4 months
Name 4 non-ortho manifestations of myelodysplasia
Neurosurgical manifestations
- Arnold-chiari manifestation (Type II); Most common associated congenital abnormality
- Hydrocephalus
- Tethered cord
Urological manifestations
- Neurologic bladder
IgE mediated latex allergy
- Results in profound anaphylaxis
- Present in 20-70% of patients with this disorder
3 complications of radial head/neck fractures
AVN
synostosis
loss of ROM (pronation > supination)
radial head overgrowth
2 spinal deformities with OI
Scoliosis
basilar invagination
(NOT AAI)
4 ortho and 2 nonortho associations with Friedrich’s ataxia
Ortho
- cavovarus foot: often rigid
- scoliosis
- ataxia
- areflexia (but with positive plantar response)
Non-ortho
- Cardiomyopathy
- nystagmus
Never do what when ORIFing a lateral condyle fracture?
Never dissect posteriorly
Blood supply comes from there and will cause AVN if you disrupt it
Difference between bone infarct and osteomyelitis on imaging?
osteomyelitis: normal marrow uptake, abnormal bone scan
infarct: decreased marrow uptake, abnormal bone scan
What 3 spinal deformity conditions are bracing CONTRAindicated in?
Spina Bifida
SCI
Muscular dystrophy
Associated ortho conditions for PFFD (4)
Fibular hemimelia (50%)
ACL deficiency
Coxa vara
knee contractures
4 manifestations of CMT?
- pes cavus
- hammer toes
- hip dysplasia
- scoliosis
SMA treatment:
- Hip Dislocations
- Scoliosis
- Hip Contractures
- Leave dislocated - recurrance is high and usually asymptomatic
- PSF to pelvis
- Deal with hip contractures before scoiliosis correction in order to ensure they can sit in wheel chair. Otherwise leave them alone.
4 radiographic findings in OI
thin cortices
generalized osteopenia
saber shins
skull radiographs reveal wormian bones
Metaphyseal bands (bisphosphonate use)
What part of acetabulum is deficient in neuromuscular hip dysplasia?
posterior superior
DDH U/S:
What is alpha angle and what is normal?
- angle created by lines along the bony acetabulum and the ilium
- normal is greater than 60°
Longitudinal traction in young kid. Arm held in elbow extension and forarm pronation. What is the injury pathomechanism?
Inteprosition of annular ligament in radiocapitellar joint (nursemaid’s elbow)
6 Blocks to reduction in DDH:
ligamentum teres
capsule
transverse acetabular ligament
inverted labrum
Psoas
Pulvinar
2 factors prognostic of long term neurologic sequelae from paediatric trauma:
O2 sat at presentation
GCS 72 hours post injury
Radial Longitudinal Deficiency/Radial CLubhand associated with what conditions (5)
TAR: thrombocytopenia absent radius - check plt
Fanconi Anemia - check Hb
VACTERL
VATER
Holt-Oram - congenital cardiac abnormalities