Peds Flashcards
1
Q
Peds multisystem organ failure vs adult
A
Peds: occurs early after admission, affects ALL ORGANS simultaneously***
Adult: occurs 48 hours after injury and begins with lungs**
2
Q
how many mL of intravascular blood volume per kg in a 5 year old child?
A
75-80 mL/kg**
3
Q
Cast burn risk higher in plaster or fiberglass?
A
fiberglass***
4
Q
What are 5 zones of physical growth plate? Diseases?
A
1- Reserve zone*
Cells store lipids, glycogen, PG for later
Dz: Gaucher’s*, diastrophic dysplasia, psuedoachondroplasia
2- Proliferative zone
Proliferation of chondrocytes w/ longitudinal growth and stacking of chondrocytes
Highest rate of extracellular matrix production
Increased O2 tension inhibits calcification
Dz: Achondroplasia, Gigantism, MHE*
3- Hypertrophic zone
Zone of maturation, chondrocyte hypertrophy and calcification
3 zones w/in hypertrophic
1-Maturation: prep for calcification and growth
2-Degenerative zone: further prep and growth in size (5X)
3-Zone of provisional calcification: chondrocyte death to release Ca2+ for calcification
Type X collagen produced** (important for mineralization)
dz: SCFE*
Rickets*
Fx’s most common through zone of provisional calcification**
4- Primary spongiosa
Vascular invasion, osteoblasts algin on cartilage bars, forms woven bone that later remodels
dz: metaphyseal “corner fx” in NAT**, Scruvy
5 - Secondary spongiosa
Internal remodeling, replacing woven bone w/ lamellar bone
dz: renal SCFE
5
Q
What growth factor regulates chondrocyte maturation?
A
Indian headgehog gene***
6
Q
What is Groove of Ranvier and why important?
dz?
A
During fist year of life, zone spreads over adjacent metaphysic to form a fibrous circumferential ring bridging from epiphysis to diaphysis
- Ring increases mechanical strength of physics and is responsible for APPOSITIONAL bone growth
dz: osteochondroma
7
Q
Distal humerus physeal separation - what to be worried about? findings on XR?
Tx?
A
Worried about NAT**
XR: posteromedial displacement of the radial and ulnar shaft relative to distal humerus***, forearm not aligned with humeral shaft
Tx: generally CRPP, combine w/ arthrogram to determine direction of initial displacement and adequate reduction**
8
Q
complications of distal humeral physeal separtaion?
A
Can be indicative of NAT**
Posteromedial displacement***
May lead to:
Cubitus VARUS**
AVN of medial condyle**
9
Q
When to treat humeral shaft fx operatively in peds?
A
Open
floating elbow
POLYTRAUMA***
10
Q
Age of fusion of peds elbow?
A
CTE-ROI for order, internal/medial epicondyle fuses at 16-18**
11
Q
How to image medial epicondyle fx in peds?
A
Internal oblique** or DISTAL HUMERAL AXIAL VIEW*
Improves accuracy of measuring displacement
Obtain by angling beam 25 degrees anterior to long axis of humerus
12
Q
What cord doe ulnar nerve originate from in plexus? where does it run?
A
Ulnar from MEDIAL cord
splits two heads of FCU in proximal forearm
Runs SUPERFICIAL to transverse ligament at wrist
Runs MEDIAL to associated artery at level of wrist
Through Guyon’s canal
13
Q
Which direction does fx displace for medial epicondyle fx in peds and what is best imaging to see it?
A
fx displaces ANTEROMEDIALLY*** (from flexor pronator mass)
Best view: distal humeral axial XR** (other best view is IR)
14
Q
What happens to LCL with lateral condyle fx for peds?
A
Remains INTACT and attached to the lateral condyle fragment proximally and the radial neck distally***
15
Q
Classification of lateral condyle fx?
Best imaging modality?
Tx?
Complications?
A
Milch classficiation
Type I: fx line is lateral to trochlear groove
Imaging: INTERNAL OBLIQUE view most accurately shows displacement as fx is POSTEROLATERAL***
Tx:
If <2 mm displacement can tx in LAC for 4-6 wks
2-4 mm displacement = CRPP
>4 mm displacement = ORIF***
AVOID POSTERIOR dissection = –> blood supply from posterior***
Complications
Most common: stiffness
NONUNION = higher than other elbow fx*
CUBITUS VALGUS and TARDY ULNAR NERVE PALSY*
Due to physeal arrest or more commonly a nonunion**, 10% of time, tx w/ supracondylar osteotomy after maturity and ulnar n. transposition
LATERAL OVERGROWTH
Up to 50% no matter what - counsel family, disruption of periosteum, lateral periosteum realignment will prevent this**
16
Q
Tx of radial neck fx?
A
Nonop
<30 degrees angulation, immobilize for 7 days
CRPP
>30 degrees of residual angulation after closed reduction***
ORIF
>45 degrees with closed or perc methods
Ass’d with greater decrease ROM, increased AVN** (up to 70% with ORIF***)
17
Q
Who does worse after radial neck fx fixation in peds?
A
Patients OVER 10 YEARS OLD***
18
Q
What position does child keep arm in for nursemaid elbow? Tx?
A
slightly flexed and pronated*
Reduce in supination and flexion***
19
Q
Most common nerve injury after supracondylar humerus fx in kids? second? Flexion type?
A
Most common: AIN (ok sign), median nerve
2nd: radial
flexion: Ulnar***
20
Q
Benefit of crossed pin in SCH fx?
A
Better torsional stability***
21
Q
When to remove pins in SCH fx? How to deal with postop stiffness?
A
3 weeks*** in clinic
stiffness: rare, but will normalize by 6 months***, no PT necessary
22
Q
What type of SCH fx is more likely to require open reduction?
A
Flexion type***
Also ulnar nerve deficits
23
Q
What causes cubits varus after SCH fx?
Reason to fix?
A
Malreduction of fx***
NOT overgrowth of lateral physics or growth arrest of medial physis***
Reason to fix generally COSMESIS***
24
Q
Most common fx ass’d with peds elbow dislocation”
A
Medial epicondyle fx***
25
When to no longer reduce peds DRF?
After one week after injury***
26
Risk factors for cast burn?
hot dipping water
More than 8 layers of plaster
Placing arm on pillow while casting
Fiberglass over plaster
27
how to immobilize Monteggia fx in kids...type I? Type II? Type III?
Type I: anterior displacement of radial head, immobilize in 110 degrees of flexion and FULL SUPINATION to tighten interosseous membrane and relax biceps***
Type II: posterior displacement of radial head, immobilize in full extension**
type III: lateral displacement of radial head, full extension with valgus mold
28
What does splinting in supination and 110 deg of flexion accomplish for peds type I Monteggia fx?
Tightens interosseous membrane***
Also relaxes biceps*
29
Highest chance of requiring re-maninpulation after BBFFx in peds?
initial TRANSLATION of fx, not angulation***
30
Most predictive femoral fx for child abuse?
Transverse pattern***
not spiral or oblique*
31
When can do operation in polytrauma peds patient w/ closed head injury?
Can do early (<24 hours)***
Decreased LoS, femoral shaft fx NOT ass'd w/ pulmonary complications*
32
How to tx peds femur fx?
<6 mo?
6 mo - 5 years?
when to tx operatively?
<6 months: Pavlik***
6 months - 5 years: Spica casting*** (don't use w/ open or shortening w/ >2-3 cm or polytrauma
Operative: kids >5
Weighing <100 lbs/49kg: flexi nails*** (if fx stable)
Unstable and >5 years and >100 lbs: sub muscular plate
>11 yo and >100 lbs: IMN
Polytrauma, open, vac injury, segmental/sig comminution: ex fix
33
Most common complication in younger patients (peds) after femur fx?
Leg length discrepancy***, generally overgrowth
34
What deformity from peds proximal tibial traction pin?
Proximal tibial recurvatum from tibial tubercle growth arrest***
35
Most common complication from peds spica cast tx for femoral shaft fx?
Loss of reduction*** (malunion 3x more likely than LLD)**
36
Peds distal femoral fx...what zone of physis?
Which side is Thurston-Holland fragment?
Physeal arrest?
Zone: zone of hypertrophy***
Thurston-Holland in metaphysic fails on the compression side and physics fails on tension side*
Physeal arrest 30-50%***
Increased with SH type, fx displacement, surgical hardware invading physis
37
What is indication to fix AIIS avulsion?
Direct head of rectus femoris bony avulsion with >2 cm of displacement**
38
Peds hip dislocation...if non concentric what is next step?
If requires open reduction, what approach?
If non concentric: get MRI***
If requires open reduction (non concentric), approach in direction hip was dislocated (posterior approach for posterior dislocation)**
39
highest risk of AVN after hip fx in peds patient?
Dislocation of epiphysis > transphyseal fx w/o dislocation > transcervical fx > basicervical > IT fx ***
40
Classification of tibial tubercle fx in peds?
Type I: fx of secondary ossification center near insertion of patellar tendon
Type II: fx propagates proximal between primary and secondary ossification centers
Type III: fx extending into both ossification centers (most common)
Type IV: through entire proximal tibial physis
Type V: periosteal sleeve of extensor mech
41
Reason for possible compartment syndrome w/ tibial tubercle fx in peds?
Most common complication?
Injury to anterior tibial recurrent artery***
Most common: recurvatum deformity (growth arrest anteriorly and posterior growth continues to decrease tibial slope)***
42
What is deformity that happens after proximal tibia fx in peds?
late VALGUS deformity (Cozen's phenomenon)***
Can occur regardless of Tx*
Observe for 12-24 months***, will likely remain, but mild
43
Tibial eminence fx in peds - where does ACL attach vs intermeniscal ligament?
Ass'd injuries?
ACL attaches 9 mm posterior to the intermeniscal ligament**
PCL does NOT attach to spines*
Ass'd injuries: occur in 40%
Meniscal injury
Collateral ligament injury
Osteochondral fx
44
What can block reduction in tibial spine fx in peds?
Medial meniscus*** (anterior horn)
45
Common complication of surgically treated tibial spine fx in peds?
Arthrofibrosis***
46
Is ACL laxity common after tibial spine fx in peds? Symptomatic?
Common, but rarely symptomatic***
47
How does peds ankle close physis (order)?
Centeral > anteromedial > poseteromedial > lateral ***
Fibular physis closes 12-24 moths after tibia
48
What is Chaput fragment? Wagstaffe? Volkmanns?
Chaput tubercle: from AITFL insertion onto anterior lateral distal tibial epiphysis
Wagstaffe: from AITFL onto distal fibula
Volkmann: from posterior aspect of lateral distal tibial epiphysis to posterior aspect of distal fibula (PITFL)
49
What are risk factors for growth arrest in peds ankle fx?
Medial malleolus SH IV fx = highest risk****
Degree of displacement: 15% increased risk of physeal injury for every 1 mm of displacement
Residual physeal displacement*** (more important than initial displacement)***
50
Which side in peds ankle fx will periosteum block reduction?
Periosteum interposed on tension side of injury***
51
What is a Tillaux fx?
SH III fx of anterolateral distal tibia epiphysis***
Avulsion of the AITFL***
Tx closed if < 2mm displacement, ORIF if > 2mm
Seen w/in one year of skeletal maturity, OLDER than triplane fx
Reduce with INTERNAL rotation***
52
Triplane fx in peds?
SH IV fx in multiple planes
Eiphysis fx on lateral aspect in SAGITTAL plane (same ass tillaux fx), seen on AP film*** (SH III)
Physis separated in AXIAL plane***
Metaphysic fx'd on posterior aspect in CORONAL plane, seen on lateral*** (SH II)
Reduce lateral triplane w/ internal rot, and medial triplane in eversion**
53
Most concerning long term consequence of triplane fx/reason to proceed to OR if not reduced acceptably?
Post traumatic arthritis***
Due to patient being close to skeletal maturity and fx occurs due to closure of physis/near closure, so growth disturbance is less of an issue**
54
Which joints in peds develop septic arthritis after metaphyseal breakthrough of infection?
Ankle
Hip
Elbow
Shoulder
NOT KNEE***
55
Sequestrum vs involucrum?
Sequestrum: necrotic bone walled off from blood supply that is nidus for chronic osteomyelitis ***
Involucrum: outer layer of new bone formed by periosteum***
56
Risk factors for DVT after osteomyelitis in peds pt?
CRP >6
Pt >8 y/o***
MRSA***
Surgical tx of osteomyelitis***
NOT TEMPERATURE**
57
Most common bug for osteomyelitis following varicella infxn?
Group A beta hemolytic strep***
NOT MRSA**
58
Risk factor for neonatal septic arthritis?
Risk factors for poor prognosis?
Neonatal septic arthritis:
Prematurity (relatively immunocompromised)***
C-section***
Pts in NICU
```
Poor prognostics:
Age <6 months***
Ass'd osteomyelitis**
Delay >4 days until presentation***
hip (vs knee)*
```
59
Kocher criteria for septic arthritis in peds?
WBC >12k
Inability to bear weight
Temp >38.5 C
ESR >40
If all 4 --> 99.6%
Fever most sensitive > CRP > ESR > refusal to bear weight > WBC**
60
What is most likely aspirate from 17 y/o with likely septic hip arthritis?
Normal joint fluid***
Neiserria gonorrhoeae presents with negative synovial fluid aspiration in >50%***
61
Abx for Lyme's? How does it work?
Doxycycline (if over 8 y/o, if under then give amoxicillin)
Doxy works by inhibiting protein synthesis in 30s ribosome****
62
Prognosis of Obstetric brachial plexopathy?
90% improve w/o intervention***
Favorable:
Erb's palsy**
Complete recovery possible if biceps and deltoid are anti-gravity at 3 months**
Early which biceps activity suggest favorable outcome***
```
Poor:
lack of biceps at 3 months**
Preganglionic injuries (loss rhomboid from dorsal scapular n. or elevated hemidiaphragm (phrenic)
Horner's --> <10% recover**
C7 involvement
Klumpke's
```
63
What motion is most limited in Sprengel's deformity?
Shoulder ABduction***
64
What is Legg-Calve-Perthes? Tx?
Prognosis?
How long until changes show up on XR and what stage?
Idiopathic AVN of proximal femoral epiphysis in peds
Tx is observation in patients <8 and femoral and/or pelvic osteotomy in >8 y/o***
Prognosis:
Bone age <6 is most important***
Sphericity of femoral head
Lateral pillar classification
XR changes at 6 MONTHS in the early fragmentation stage of dz***
65
What to do if peds pt has bilateral hip LCP at same time?
Get imaging of spine and knees to look for multiple epiphyseal dysplasia***
66
DDH tests: Barlow, Ortolani, Galeazzi?
Most sensitive test after 3 months?
Barlow: Dislocates hip by adduction and depressing of flexed femur**
Ortolani: Reduces dislocated hip by elevation and abduction of flexed femur**
Galeazzi: apparent LLD due to unilateral dislocated hip w/ hips flexed to 90 and feet on table.
Most sensitive after 3 months = lack of hip abduction*** No longer Barlow or Ortolani positive due to soft tissue contractors around hip**
67
When to start getting XR for DDH?
Normal acetabular index?
4-6 months, after femoral heads begin to ossify***
Normal acetabular index: <25 degrees when older than 6 months***
Center edge angle: <20 degrees, only reliable when > 5 years old*
68
Ultrasound of DDH: what ages? Alpha angle? Beta angle?
timing: 6 wks to 4-6 months***
Prior to 4-6 weeks can give wrong results
Alpha angle: angle from line from bony acetabulum to ilium, normal is > 60 ***
Beta: Angle from labor and ilium
Normal <55
69
Blocks to reduction of hip in DDH?
```
Blocks:
1- Inverted labrum**
2- Inverted limbus**
3- Hypertrophied TAL**
4- Hip capsule constricted by iliopsoas tendon causing hourglass deformity of capsule**
5- Pulvinar**
6 - Ligamentum teres**
```
70
Treatment for DDH..ages?
<6 months with reducible hip: Pavlik*** (if fails at 3-4 weeks then try semi rigid abduction brace before moving to further intervetion**)
6 mo - 18 months with failure of pavlik: Closed reduction and spica casting***
>18 months: open reduction and spica casting*** (or failure of closed reduction)
>2 years with residual hip dysplasia and anatomic changes on femoral side: open reduction wand femoral osteotomy*** (after 4 y/o then pelvic osteotomy)
> 2 years with residual hip dysplasia and anatomic changes on acetabular side: open reduction and pelvic osteotomy***, used more in kids >4 y/o
71
Medial approaches to hip for DDH - Weinstein, Ludloff and Ferguson?
Weinstein: Between sartorius and adductor longus then next to NV bundle, then between Iliopsoas and pectineus (anteromedial)
Ludloff: Sartorius and adductor longus, then lateral to pectins between pectineus and adductor brevis (medial approach)
Ferguson: between adductor longus and gracilis (posteromedial), then between adductor brevis and magnus
72
Tx failure predictor with closed reduction of DDH?
Dye pool 7+ mm --> tx failure and AVN***
73
What makes up the teardrop in pelvis on XR?
Quadrilateral surface and cotyloid fossa***
Shows up by 18 months in normal kids***
74
What osteotomies can be used in DDH with non concentric hips?
Chiari**
| Shelf**
75
Which zone of physis does SCFE occur through?
How does shaft and head change position?
Hypertrophic zone***
Histo shows granulation tissue between columns in hypertrophic zone***
Metaphysic translates ANTERIOR and EXTERNALLY ROTATES***
Epiphysis remains in acetabulum posterior to translated metaphysic**
76
Who to get endocrine w/u for in SCFE?
Child is <10 y/o***
Weight is <50th percentile***
Hypothyroidism*** (TSH)
Renal osteodystrophy (BUN and Cr)***
GH deficiency
Panhypopituitarism
77
Southwick slip angle for SCFE - how to measure and grades?
Epiphyseal-diaphyseal angle differences (can be measured on AP or frog leg lateral)***
Mild <30 deg
Moderate 30-50 deg
Severe 50+ difference
78
What motion is lost in SCFE?
Flexion, IR, abduction ***
79
Who to do a prophylactic SCFE fixation on?
Where to start screw?
How many threads should cross physis w/ screw?
Where to avoid with screw?
High risk for contra slip
Initial slip at young age <10 y/o***
open irradiate cartilage ***
Obese males***
Endocrine disorders (hypothyroidism)***
Start ANTERIORLY on proximal femur to cross perpendicular to physis***
Get 5 threads across, decreases risk of progression of slip***
Avoid posterosuperior femoral neck --> can disrupt vascular supply*** (anterior superior has more risk of being intra-articular)
80
What to do if both hip and knee are dislocated in newborn?
Tx knee first*** --> can't get Pavlik on hip if knee dislocated**
81
When to intervene surgically for congenital dislocation of knee?
6 months - 1 year if does not respond to conservative tx**
Open VY quadricepsplasty
82
Infantile Blount's vs Adolescent Blount's?
Infantile
Pathologic genu varum in children 2-5 y/o***
male > female***
More common, bilateral in 50%***
Risks: early walking/large stature/obesity***
May be self-limited
Bone involvement: Proximal medial tibia physis, producing genu varus/flexion/IR*** AND may have compensatory distal femoral VALGUS**
Tx: bracing and surgery
Adolescent Blount's
Pathologic genu varum in children >10 y/o
Less comm and less severe
More likely to be unilateral**
Risks: obesity***
Bone involvement: Proximal tibia physis AND may have distal femoral VARUS and distal tibia VALGUS**
Progressive --> NEVER spontaneously resolves
Tx: surgery only
83
Describe normal physiologic pattern of genu varum in children (changes through ages)
Genu varum/bowed legs normal in children LESS THAN 2 y/o***
Genu varum migrates to neural at approx 14 months**
Continues to peak genu valium/knocked knees at 3 years of age***
Genu valium then migrates back to normal physiologic valgus at 7 y/o***
84
PE of infantile Blount's?
XR angle?
Genu varus, FLEXION and INTERNAL ROTATION***
+ cover up test**
Lateral thrust on walking
XR: Metaphyseal-diaphyseal angle of Drennan
Angle between line connecting metaphyseal beak and line perpendicular to long axis of tibia
>16 deg abnormal and has 95% chance of progression
11-16 degrees = close observation
<10 degrees has 95% chance of natural resolution
85
Tx of infantile Blount's...nonop vs op
Nonop
Brace w/ KAFO --> stage I and II in children LESS THAN 3 y/o
Must maintain braces for 2 years to resolve bone changes
Outcomes:
Improved if unilateral
Poor if ass'd w/ obesity and bilateral**
If successful, improvement w/in 1 year
```
Operative
Proximal tiba/fibula valgus osteotomy**
Stage II and II in kids > 3 y/o***
Stages III, IV, V, VI***
Age 4+ in all stages***
Failure of braces w/ progressive deformity
Metaphyseal-diaphyseal angle >20**
```
Osteotomy below tibial tubercle
Less risk of re urgency if done prior to age 4
OVERCORRECT by 10-15 deg valgus because medial physeal growth abnormalities persist***
86
Where to place osteotomy to decrease translation and minimize shortening?
Dome osteotomy with center of dome located at CORA/center of rotational angulation***
87
What is normal intermalleolar distance in kid?
8 cm***
| Greater = valgus, may need intervention
88
Neurofibromatosis
Inheritance pattern?
Mutation? chromosome?
Extremity deformities?
Spine involvement?
Inheritance: AD***
Mutation: NF1 gene on 17q21***
Negatively regulates Ras pathway (deficiency of NF leads to increased Ras) --> affects Ras dependent MAPK activity which is essential for osteoclast function and survival***
Extremity deformities
Congenital ANTEROLATERAL bowing and pseudoarthosis of tibia/fibula AND forearm***
HEMIHYPERTROPHY***
Spine
Scoliosis and kyphosis***
Atlantoaxial instability***
89
NF diagnosis
```
Has 2 of any of the following:
6+ cafe au lait spots
2+ neurofibromas or 1 plexiform
Freckling of inguinal or axillary region
Optic gliomas
2+ lisch nodules (iris hamartomas)
1st degree relative with NFI1
```
90
What dz often presents with hemihypertrophy?
NF1***
91
Scoli in NF1
XR?
MRI?
Tx?
2 forms --> non dystrophic and dystrophic
Non: longer curve and treated like AIS
dystrophic: THORACIC KYPHOSCOLIOSIS with short segmented and sharp curve with distorted ribs
XR: vertebral scalloping**
Penciling of ribs --> 3+ ribs is poor prognostic factor for rapid progression)***
Enlarged foramina**
MRI: always get preop ***
Dural ectasia***
Dumbbell lesion of neurofibromas on nerve root**
Tx: nondystrophic: like AIS
Dystrophic: ASF + PSF, do early, but high rate of pseudo***
92
What non-ortho condition should NF1 patients be worked up for as adults?
Aortic stenosis***
93
lateral XR shows abrupt dysplastic kyphosis at C2/3 in peds pt...what condition is this pathognomic for?
NF1***
94
Most important sign of impending rapid progression of spinal deformity in NF1 patient?
Pencilling of 3+ ribs***
95
What is ass'd with anteromedial tibial bowing?
Inheritance?
Ass'd conditions?
Fibular deficiency***/fibular hemimelia
Most common long bone deficiency
No known inheritance...linked to SONIC HEDGEHOG gene
```
Ass'd conditions:
Ankle instability 2/2 ball and socket joint****
Clubfoot
Tarsal coalition (50%)***
DDH
LLD
Knee cruciate deficiency***
Lateral ray deficiency***
Fermoral shortening
Coxa vara**
```
96
Most common sequelae of posteromedial bowing?
Ass'd with?
LLD*** --> average is 3-4 cm***
Ass'd with calcaneovalgus***
97
Muscle imbalances in cavovarus foot?
Tibialis anterior weaker than peroneus longus***
Peroneus brevus wekker than posterior tibial*** (PT-PB)**
98
Coleman block test - how to perform? What does it show?
Place 1" block under lateral foot (eliminates contribution of plantar flexed 1st ray and forefoot pronation to the hind foot deformity)
Evaluates flexibility of hind foot deformity***
Flexible hind foot will correct to neutral or valgus when block placed under lateral foot**
Rigid foot will not correct to neutral**
99
Tx of cavovarus foot - nonop? operative?
Nonop
Full length insole with depression for fist ray and lateral wedge (mild deformity in adult)
AFO - best for dynamic deformity
Lace up brace
Operative
Peroneus longus to brevis transfer*** (for plantar flexed 1st ray) --> decreases plantaflexion force on 1st ray w/o weakening eversion
PT tendon transfer
PT is stronger than evertors and maintains strength for long time in cavovarus foot. --> can't transfer PT to dorsal of foot if server weakness of AT**
TAL
1st MT dorsiflexion osteotomy *** --> for flexible hind foot varus (normal Coleman block) --> corrects forefoot pronation during the hindfoot deformity
Jones transfer of EHL to neck of 1st MT and laser toe extensors to 2-5 MT necks*** --> toe clawing combined with caves foot --> also can do IP fusion of hallux ***
If rigid:
Lateralizing calcaneal valgus producing osteotomy***
100
Steps to diagnose CMT?
1 - PE
2- electrodiagnostic studies***
3- Genetic test to confirm
101
CMT inheritance? chromosome?
Orthopedic manifestations?
Inheritance: AD DUPLICATION of chromosome 17***
Codes for peripheral myelin protein 22 (PMP22)*** expressed in Schwann cells***
May also be X linked or AR
```
Ortho manifestations:
Cavovarus***
Hammer toes
Hip dysplasia***
Scoliosis***
Hand muscle atrophy and weakness
```
102
What does EMG show for CMT patients?
Low nerve velocities with PROLONGED DISTAL LATENCIES in peroneal, ulnar, median nerves
Can Laos see low amplitude nerve potentials 2/2 axonal loss***
103
CMT scoliosis
10-20% patients w/ CMT
LEFT thoracic and kyphotic curves distinguish from AIS
Tx:
Bracing not used --> not effective***
Tx w/ fusion when >50 degrees***
104
```
Correcting clubfoot - order?
Month 1-4?
First position?
When TAL if necessary?
How often TAL necessary?
When to start FAO and for how long?
When to consider TA transfer?
```
Order: CAVE (cavus, adductus, varus, equinus)
First position: correct caves w/ forefoot SUPINATED (not pronated) --> pronation would worsen cavus
2nd correct adduction and heel varus by rotating calc and forefoot around talus into FOREFOOT ABDUCTION
TAL: with last cast, do when foot at least 60 abducted and hell in values --<> generally at 8 weeks, cast in max dorsiflexion for 3 weeks after TAL
TAL needed in 80%***
FAO: foot abduction orthosis, 23 hrs/day for 3 months, then night/naps until ge 4
TA transfer: at 2-5 y/o (30-50% require) --> for patients that have SUPINATION during gait***
105
Who gets dorsal bunion?
Clubfoot/talipes equinovarus patients***
due to strong AT overpowering weak PL*** (important)
106
How to treat dynamic supination in peds pt after clubfoot?
When to do procedure?
Transfer AT to lateral cuneiform*** (from medial and under surface of first cuneiform)
Perform after age 3*** (when lateral cuneiform has ossified)**
107
Vertical talus imaging?
Forced plantar flexed lateral XR = diagnostic ***
Persistent dorsal dislocation of talonavicular joint (oblique talus reduces on this view which can then tx with cast)
tx:
serial manipulation and casting prior to surgical release --> stretch dorsolateral soft tissues***
Then at 6-12 months surgical release and TN joint reduction and pinning***
108
Most common tarsal coalition?
Inheritance?
Subtalar changes?
Sx?
Tx?
Most common: calcaneonavicular***, then Talocalc
Inheritance: AD***
Causes lack of normal IR of subtalar joint***
Most are ASYMPTOMATIC (75%) but present with recurrent ankle sprains***
Sx: calcaneonavicular: sinus tarsi and inferior fibula pain
Talocalcaneal: distal to medial mall or medial foot pain
Tx:
Initial tx = immobilizing w/ casting for 6 wks with SLC
Operative: coalition resection w/ interposition graft --> 80-85% of patients get pain relief***
109
What is Kohler's disease?
How long for sx?
Pathophys?
Tx?
Rare AVN of navicular, 80% in males
sx: Dorsomedial mid foot pain, intermittent sx for 1-3 years after dx****
Pathophys: Blood supply of the central 1/3 of navicular = watershed, last bone to ossify so increases vulnerability to mechanical compression***
Tx
NSAID and immobilization in SLC (weight bearing cast) if pain with activities*** (decreases length of Sx)
110
Definition of vertical talus?
Ass'd with?
Irreducible DORSAL DISLOCATION of NAVICULAR on talus*** --> rigid flatfoot deformity at birth
```
Ass'd w/ neuromuscular dz or chromosomal abnormalities 50% of time***
Myelomeningocele
Arthrogryposis
Diastematomyelia
DDH
CP
SMA
```
111
Definition of CP?
| Risk factors?
Definition: onset of nonoprogressive UMN/STATIC ENCEPHALOPATHY due to injury of immature brain dx'd prior to age 2 yo
```
Risks:
Prematurity - most common
Anoxic injuries
Perinatal infxn (Toxo, CMV, herpes, ToRCH)
NAT
Brain malformations etc
```
112
Types of CP
GMFCS levels?
Types:
Quadriplegic - total body and nonambulatory
Diplegic: Legs more than arms but still ambulatory --> IQ may be normal (injury to brain is MIDLINE***)
Hemiplegic: Arms and legs on one side of body affected, will eventually be able to walk regardless of tx**
Gross Motor Function Classification Scale (GMFCS)
Level I: near normal, independent ambulator
Lvl II: Walks independently but difficulty w/ uneven, minimal ability to jump
Lvl III: Walks w/ assistive devices
Lvl IV: Severely limited walking, primarily in wheelchair
Lvl V: Nonambulator w/ global involvement, dependent in all aspects of care***
113
How does botox work?
Botox: Competitive inhibitor of presynaptic cholinergic receptors w/ finite lifetime (2-3 months)
114
Most prognostic for the ability of CP child to walk?
Ability to sit at 2 years old***
115
What does Thomas test evaluate?
hip flexion contracture***
If positive, consider Psoas lengthening***
116
What to do with gastroc contracture not responding to Botox injxn in 3 yo?
Serial casting in patients <6 y/o***
117
Allergic consideration for patient with spina bifida?
IgE mediated latex allergy***
20-70% of patients --> anaphylaxis***
118
What is mutation in Marfan's?
Ortho manifestations?
Non-ortho issues?
mutation: Fibrillin-1 gene --> autosomal DOMINANT
Causes MISFOLDED GLYCOPROTEIN which leads to accumulation of TGF beta in lungs, heart valves and aorta*** (component of elastin microfibrils)
```
Ortho manifestations:
Scoliosis (50%)***
Acetabular protrusion***
Dural ectasia***
Pectus***
```
non ortho
Cardiac abnormalities (aortic root dilation, aortic dissection, MVP)***
SUPERIOR lens dislocation (vs homocystinuria with INFERIOR)***
Spontaneous pneumothorax***
119
Is bracing effective for Marfan's scoliosis?
Complications after scoliosis surgery?
Generally NOT effective
Higher complications
Fixation failure, infxn, pseudarthrosis, dural tears (dural ectasia)***
120
What to send pt with Marfan's for when pregnant?
Cards testing --> have have aortic dissection or MVP which can be fatal during pregnancy
NOT genetic testing (as first test)***
121
What to work up patient for who presented at birth with bilateral knee dislocations and has flattened nasal bridge and prominent forehead?
Larsen's syndrome*** (present with multiple dislocated joints***)
Work up for cervical kyphosis***
Can be unstable with potential to cause iatrogenic spinal cored injury with ETT placement***
122
Spinal Muscular Atrophy pathophys?
Inheritance? Mutation?
Ass'd ortho conditions?
Fusion required for scoli?
Pathophys: progressive loss of Alpha motor neurons in anterior horn of spinal cord***
Inheritance: autosomal RECESSIVE
Mutation: survival motor neuron (SMN) gene mutation**** (90% of cases)
Telomeric gene deletion
Ortho conditions:
Hip dislocation and sublaxation
Scoliosis
LE contracture
TONGUE FASCICULATIONS***
Scoli fusion: PSF only (dont require ASF)***
123
SMN1 vs SMN2
For Spinal muscular atrophy***
All SMA patients have loss of SMN1 copies
Number of SMNII copies determine how severe dz is (more copies = less severe)***
124
Duchenne muscular dystrophy cause?
Who?
PE?
Ortho manifestations?
Non ortho?
Cause: Absent DYSTROPHIN gene*** (Vs Beckers with decreased dystrophin)
Who: young males only --> X linked recessive***
PE: Gower sign, calf psuedohypertrophy, affects PROXIMAL muscles first***
Ortho:
Scoliosis
Calf hypertrophy***
Equinovarus foot
Non ortho
Cardiomegaly***
125
Scoli with Duchenne muscular dystrophy - when does progress?
Tests preop?
When to fuse?
Worry w/ anesthesia?
Scoli progresses rapidly from 13-14 (1-2 degrees/month)
Tests: must do cards and pulm function tests*** can be too dangerous to do surgery in some cases
PSF: 20-30 degrees in NONAMBULATORY***
40+ if responding to steroids
FVC <35% ****
Worry w/ anesthesia: Malignant hypertrophy common --> pretreat with dantrolene***
126
Achondroplasia genetics?
Ortho issues?
Genetics: Autosomal dominant*** (risk increases with increasing paternal age)
G380 mutation of FGFR3****
GAIN of function mutation --> increases inhibition of chondrocyte proliferation in PROLIFERATIVE zone of physis****
Ortho
Lumbar stenosis (short pedicles/decreased interpedicular distance, thick facets and LF)
Thoracolumbar kyphosis
Foramen magnum stenosis --> apnea or sudden death in infants***
Champagne glass pelvis (wider than deep)
127
What to do about achondrplast with thoracolumbar kyphosis at 6 months old?
Observe, spontaneously improves at 12-18 months***
128
Diastrophic dysplasia
Cause?
Genetics?
PE?
Form of short limbed dwarfism caused by failure of formation of secondary ossification center (epiphysis)**
Genetics: Autosomal RECESSIVE***
Mutation in DTDST gene on CHROMOSOME 5
Encodes for SULFATE TRASNPORTER PROTEIN***
```
PE:
Rhizomelic shortening (proximal > distal)
CAULIFLOWER EAR***
HITCH HIKER THUMB***
Severe cervical kyphosis
Skewfoot
```
129
What condition has odontoid hypoplasia?
Morquio syndrome***
130
Cleidocranial dysplasia: cause?
Genetics?
Ortho?
Cause: skeletal dysplasia affecting bones formed by INTRAMEMBRANOUS ossification***
Genetics:
Autosomal DOMINANT***
Runx2/CBFA1 mutation****
```
Ortho
Clavicle dysplasia/aplasia***
proportionate dwarfism
Wormian bones
Coxa vara**
Frontal bossing
DELAY IN CLOSURE OF SKULL SUTURES***
```
131
Osteopetrosis cause?
Pathophys?
Ass'd conditions?
Metabolic bone dz caused by DEFECTIVE OSTEOCLAST RESPORTION of immature bone***
Pathophys
Osteoclast dysfunction --> dense bone and obliterated medullary canals --> due to inability to acidify Howship's lacuna***
Predispostion to fx***
Ass'd conditions:
Head --> CN palsies (optic > auditory > trigeminal > facial)
Osteomyelitis --> lack of marrow vascularity and impaired WBC fun
Spine: back pain and spondylolysis
Pelvis: coxa vara --> Fem neck nonunion
132
Classification of Ostepetrosis types?
Malignant: Autosomal recessive
Prton pump or Cl channel dysfxn
Fatal at early age
Intermediate: Autosomal RECESSIVE*** --> carbonic anhydrase II dysnfxn***
Live into adulthood
Benign: Autosomal DOMINANT***
Chloride channel dysfunction
Type I: no increased fx risk
Type II: Albers Schonberg dx*** (anemia, path fx, premature OA)
Normal lifespan
Most common form to be managed by ortho**
133
What is Albers-Shonberg dz?
Autosomal dominant type of osteopetrosis***
Inability to acidify Hoship's lacuna**
134
Osteogenesis imperfecta
Cause?
Pathophys?
Genetics?
Ortho manifestations?
OI: Hereditary condition resulting from a decrease in the amount of TYPE I COLLAGEN***
Pathophys: decreased collagen OR production of abnormal collagen
Physeal osteoblasts cannot form sufficient osteoid and periosteal osteoblasts cannot remodel normally***
Genetics
COL1A1 or COL1A2***
Abnormal collagen cross linking via a GLY sub in the pro collagen molecule***
AD and AR forms
AD: more mild (type I and type V)
AR: more severe (Types II and III)
```
Ortho
Fragility fx w/o remodeling --> can get bowing
Scoliosis***
Compression fx
Basilar invagination***
Olecranon apophyseal avulsion fx***
Coxa vara
Congenital anterolateral radial head dislocations (Type V)***
```
135
How to tx DRF in child near skeletal maturity if pt has OI?
Open reduction and intramedullary rod fixation with casting*** --> load sharing device***
Plat fixation = higher risk for fx 2/2 stress shielding**
136
What to think about if patient presents with olecranon apophyseal fx?
Osteogenesis imperfecta***
137
JRA/JIA --> ass'd conditions?
Sx?
Genetics?
Prog?
Imaging?
Ass'd conditions with Juvenile RA or Juvenile Idiopathic Arthritis
C- spine: can lead to AA subluxation***
Ocular involvement: ANTERIOR UVEITIS***, get slit lamp exam***
Sx: often oligoarthritis or polyarthritis that improves during day*** most seronegative***
Genetics: RF positive in only 15%***
Prog: 50% w/o sequelae, 25% slightly disabled, 25% crippling arthritis and/or blindness***
Imaging: Get flex-ex of neck --> r/o atlantoaxial instability***
138
How to tx sickle cell crisis?
Hydroxyurea***
139
Sickle cell anemia vs trait?
Anemia: two abnormal copies of hemoglobin S allele***
Trait: single copy of abnormal Hb gene**
140
Osteomyelitis in Sickle cell...organism?
imaging test?
Organism:
Increased incidence of salmonella*** (may spread from gallbladder infxn***)
Staph still most common**
Imaging: bone scan
Osteomyelitis: normal marrow uptake but abnormal bone scan**
Infarct: decreased marrow uptake, abnormal bone scan***
141
What patients do best with osteonecrosis of humeral head?
Sickle cell patients***
142
Ehlers-Danlos sx?
Genetics?
workup imaging/test?
```
sx/signs:
Hyperelastic/fragile skin***
Joint hyper mobility/dislcoation***
Ligamentous laxity***
DDH
MVP
Scoliosis
```
Genetics: COL5A1 or COL5A2 in 40-50%***
Gene for TYPE V COLLAGEN*** (skin matrix collagen fibrils and basement membrane)
Generally AUTOSOMAL DOMINANT***
Can also have AR form with mutation in Lysol hydroxylase deficiency*** (important in collagen cross-linking)
w/u: get Echo (aortic root dilation***)
143
Where does pseudoarthrosis of clavicle occur most commonly?
Most common in then middle 1/3 of RIGHT side***
Thought to be related to subclavian artery pulsations
144
Most common acetabular position in patients with bladder exstrophy?
Retroversion***
Pubic diastasic, shortened pelvic rami and acetabular RETROVERSION***
145
What dz is caused by a mutation in cartilage oligomeric matrix protein (COMP)?
What other test to get?
Multiple epiphyseal dysplasia (MED)
Delayed and irregular ossification of the epiphyses of long bones
Other test: skeletal survey*** to look for involvement of other epiphyses with issues
If see BOTH femoral heads flattened, think MED NOT LCP***
146
what condition causes Erlenmeyer flask femurs?
Osteopetrosis***
Also "rugged jersey spine"***
147
When to restrict child with Down syndrome from sports for spine reason?
When ADI 6+ mm on flex/ext imaging***
148
how much will peds (young, <9 y/o) DRF correct over time with observation?
1 degree / month***
12 deg volar angulation will correct in 1 year**
149
COL1A1 and COL1A2 vs COL5A1 and COL5A2?
COL1A1 and A2 = OI***
COL5A1 and A2 = Ehler Danlos***
150
Deformities noted with peds ACL (transphyseal)
Tibial recurvatum*** resulting from tibial tubercle apophyseal arrest
Limb length inequality***
Valgus deformity***
