1
Q
Osteogenesis Imperfecta decrease is amout of?
A
Normal Type I collagen - COLA1a1, - Glycine substitution AR form is more severe SABER Shin, Bisphosphonates
2
Q
Prophylactic slip indiations?
A
- Hypothyroidsm
. Girls Les than 10, boys less 12 - obesity
3
Q
Mutation of Marfan/s syndrome?
A
Fibrillin-1
4
Q
Best Prognostic indicator for good outcome after LCP?
A
<6 years old
- Fragmentation phase is 6months after initial. this is when lateral pillar classification determined
5
Q
Bowed legs at 2 years, knocked knees at 3 yeaers
A
valugs until normal at 7 years
6
Q
Angle of drennan (blounts) line from metaphyseal beaks perpendicular to axis of tibia?
A
> 16d has greater than 95* progression
- Operative is grter than 4 in ALL stages
NEED to correct to 10-15d of VALGUS
7
Q
Distal Clavicle Physeal fractures pathoanatomy?
A
The clavicle displaces away from physis and periosteal sleeve, physis and sleeve stay attached to AC/CC ligaments
8
Q
Clubfoot correction
A
- Cavus
- Adductus
- Varus
- equinus ( Achilles tenotomy)
9
Q
Calcaneonavicular coalition Tx
A
- immobilization/ rest
- resection with EDB interposition
- No supple- arthrodesis
If talocalcaneal >50% arthrodesis/<50% resection
10
Q
C1-C2 fusion for downs syndrome AAI
A
ADI
>5mm and myelopathic
or >10m
11
Q
Neurmofibromatosis phenotype
A
-ANTERIOR LATERAL BOWING tibia (posterior medial- packaging problems) - Café-AuLait- SMOOTH (California) - Short SHarp Dytrophic Scoliosis - AD, Cutaneous neuromas
12
Q
Progression of Thorcacolumbar kyphosis in Achondroplasia?
A
Will improve by 12-18 months when child ambulates
FGFR-3 mutation
13
Q
FGFr2 mutation ?
A
Aperts
14
Q
Klippel-feil syndrome and scapular deformity?
A
Sprengels deformity- difficulty in shoulder abduction
15
Q
What is the cut-off age for AI to be less than 25d?
A
6months
Normal Alpha Angle (>60)
Normal Beta Angle <55
16
Q
What to do if pavlik harness has failed after 3 weeks in child <6months old?
A
Convert to semi-rigid abduction orthosis
17
Q
When do you do closed reduction and spica casting in DDH?
A
6-18months old
18
Q
When do you do open reduction and spica casting of DDH?
A
> 18months old
19
Q
Open reduction with femoral or acetabular osteotomies in children greater than?
A
2years old
20
Q
What does vancomycin bind to?
A
D-Ala-D-Ala moieties in bacterial cell wall
21
Q
What other work-up does Juvenile idiopathic Arthritis need (JIA?
A
Optho for anterior uveitis
22
Q
Infantile tibia var indications for operative treatment?
A
- needs to have osteotomy by age of 4
23
Q
When are barlow and Ortaloni maneuvers useless?
A
After the age of 6mo.
24
Q
Breakdown of femoral shaft fracture treatment in kids?
A
<6months- Pavlick versus spica cast
7m-5 years- <2/3cm shortening- spica
>2cm short: ORIF/Flex Nails
6-11y: length stable- flexible nail
unstable- ORIF, ExFix
>11 year: Flex nails ,10lbs, Troch lateral entry
25
Coxa Vara Surgery angle?
Hilgrenrieners epiphyseal angle
Line thorugh physis adn H line
<45d can observe, >60d Valgus osteotomy
26
30s subunit on ribosome
Aminoglycoside(gentamicin, tobramicin)
| Tetracycline (doxycycline)
27
50s ribosomal subunit acotrs
Macrolides (azithromycin, erythromycine)
| Clindamycin
28
Pseudoachondroplasia gene and differences?
- NO frontal bossing, No spinal stenosis
| - COMP on chromosome 19
29
What is the rash associated with LYME disease?
Erythema Migrans
- Amoxicillin/Doxycycline
- Borrelia Burgdorferi
30
Elbow fracture associated with dislocation?
ME fracture- Plan for dissection? Brachialis (MCTN), Triceps (Rad. N. )
31
Excessive supination during SWING phase of gait after ponsetti castin? What Tendon to transfer and where?
TA
- LATERAL Cuneiform 1
- Cuboid 2
32
Rugger Jersey Spine pathonogmonic?
| Erlemeyer Flask of distal femur
Osteopetrosis
| - Defect in Carbonic Anhydrse (hearing los, anemia
33
Duchene's Muscular dystrophy
- Inheritance pattern
- gene/protien
- XLR
- Gowers sign
- Scoliosis in 90-95%
- Dystrophin
- Bewckers is less severe form (some dystrophin)
34
Tibial Torsion test ?
Thigh-Foot angle (normal between -5-->20
35
Increase femoral anteversion signs?
- In towing gate
- Knee IN LINE with foot
Increase interrotaiton
36
Normal Heel bisector line?
Between the 2nd/3rd
37
When should physiologic genu Varum resolve?
2 years, especiialy b3
38
Jump gait conctractures?- Hip flexion, Knee Flexion, Plantar flexion
Crouch Gain Contractures?
Hip Flexion, kne Flexion, Ankle Dorsiflexxion (calcaneous on floor during entire stance phse)
39
Iselin's Disease is ?
traction apophysitis of base of 5th metatarsal
40
Which artery is HYPOPLASTIC in clubfoot?
Anterior tibial artery
41
LDFA Normal?
MPTA Normal
F-T Angle Normal
85-90
85-90
5-10
42
hallmarks of marfans?
```
AD
Fibrillin-1
Dolichostenomelia
Scoliosis
Dural ectasia
Cardiac problems
acetabular protrussio
```
43
Uper Cervical problem in achondroplasia
Foramen Magnum stenosis
44
What iw gene for Spinal Muscular Atrophy?
Survival Motor Neuron II (SMN2)
45
Arthrogyrposis hip reduction? Open or closed?
Probably not going to be able to do it with CLosed, will be open and with MEDIAL approach
- No elbow flexion, wrist flexed , internally rotated shoulder
46
What is common etiologic bacterial agent following varicella injection/infection?
Group A- beta hemolytic strep
47
What muccopolysaccaraidosis is odontoid hypoplasia associated with?
Moroqiuo Syndrome
| Accumulation of Keratan sulfate
48
Bilateral Flattening of the femoral epiphsysis?
| Unilateral Flattending of Femoral Epiphysis?
- Bilateral - Multiple Epiphyseal Dysplsia- Survey
| - Unilateral- Legg-Cathe Perthes
49
?Which Zone does SCFE usually happen in
Zone of Hypertrophy
50
Growth plate zone associated with achondroplasia?
Proliferative Zone
| - also gigantism
51
Hypertrophic Zone pathology?
SCFE
SH Fractures
Rickets
52
Gauchers and Diastrophic Dysplsia Chondral zone?
Resting Zone
53
Most Likely strucutre that is trapped under tibial eminience fractures?
Medial meniscus
54
Erbs Palsy with Favorable recovery if what is seen early?
Biceps function/ elbow flexion
55
Tibial Bowin
- Anterior Lateral?
- Anterior Medial?
- AL- NF-1
| - AM- Fibular Hemimelia (no ACL, Absent Lateral Ray
56
Fibrilin-1 is on what chromosome?
15
57
Packing problems in utero
DDH, MT adductus, Torticollis
58
Calcaneal Valgus AND and PM bowing? What should you expect?
LLD 4-6 cm
59
Salter and Triple (Steele)
- rotational osteotomies (think if them like PAOs with pubis/triradiates)
- Salter is ONE cut
- Triple is obviously 3
- Rotate through the pubis
60
Pemberton and DEGA Osteotomy are what type of osteotomies?
Volumetric reducing osteotomies
61
Where is the hip volume loss in CP kids?
Posterior superior- need to graft in the back
| - DDH is most anterior lateral
62
What is the most common position of the chile in utero?
Head own, LEFT hip towards sacrum
| - Left hip more common in DDH
63
What is the grey Zone in infantile blounts angle of Drennen?
11-16d
64
When does the the proximal femoral epiphysis form or show up
- 6 months
65
When does closed reduction of hip probably not work?
Definitely over 18 months WONT work
66
What does fibrous dysplasia on histology
Scattered calcification- Chinese Letters
67
Fibular Hemimelia things ?
- ACL out
- Lack of lateral rays of foot
- Ball and Socket joint
- Anterior Medial Tibial bowing
- Sonic Hedgehog
68
What are the muccopolysaccharidosis?
| All are AR except HUNTER ( Xlinked)
- Moroqio- Keratan Sulfate
- Hunters
- Hurlers- Dermatan Sulfate
- San Fellipo
69
Larsens Syndrome ?
Fixed dislocations that are STIFF
Wide set eyes
Club feet
70
What mother factor is associated with sacral agenesis?
Maternal Diabetes
71
NF-1
- Lisch nodules - needs six
- Coast of California- smooth
- dural extasia
- PENCILLING of ribs (three)
- Short, sharp, diastropic curves
72
NF-1
| What chromosome ?
-Lisch nodules - needs six
-Coast of California- smooth
- dural extasia
- PENCILLING of ribs (three)
- Short, sharp, diastropic curves
CHromosome 17
73
What is the LLD asssocaited with Posterior medial tibial bowing?
BOWINR RESOLVES
| 4CM Tibial descrepancy
74
What limb length decrepancy is ok at maturation?
2cm
75
Lengthening with fixitor and osteotomy?
1 month/CM of needed length to remain on
100% complication rate
95% efficacy rate
76
Varus normal Until When 2 years
Valgus Normal 2-6 years
Nomrmal Alignment 7 years
See other
77
Blounts Treatment? <4yo , >4yo
<4yo: Brace as Toddler
>4yo: Surgery
If >4yo and Langenskoild classification you used Surgical OVER CORRECTION
OVER CORRECTION
78
Blunts Disease Angle?
<11d normal
111-15 Monitor
>16 d- treat with braces or surgeryBrce if less thn 4yo, Surgery if Class IV or over 4
79
Tibial Deficiencies
Extensor mechanism; NONE?
Extensor MEchanism intact?
NONE: Knee disarticularion
| Present- BKA, Syme
80
Bowing
Anteriorlateral Bowing?
Posterior Medial?
Anterior Medial
AL: " A-L Bad", Neurofibromatosis, Ch 17, pseuodarthrosis
PM: Calcaneoolavugs foot association, Bowing improves, residual limb length deficiency is 4CM
AM: Fibular hemelia (
81
Anterior lateral tibia bowing without fracture Tx?
AL Bad, But you treat the bowing with orthosis UNLESS there is actual fracture
82
Fibular hemilelia associations:
```
Criciate defieciency
Coxa Vara
Lateral rays absent
Lateral femoral condyle hypoplasia
Ball and socket ankle ( unstable ankle- Syme amputation)
```
83
What side is the discoid meniscus ?
Lateral side
| 3 consecutive cuts on the saggitals show menisceal body
84
Congenital dislocations of hte knee asssociation
Larsens Syndrome, arthrogryposis, myelomenigoceole
| Rule out Hip dysplasia ( need U?S or exrays of the hip)
85
AER ( apical ectodermal ridge)?
Proximal to distal
Inter-digital apoptosis
THALIDOMIDE NHIBITS
86
Zone of Polarizing activity ( ZPA)
Radial to ulnar development
| Sonic Hedghog
87
Wnt
Dorsal to volar developement
88
Ectoderm
Responsible for neural crest, skin, spinal cord
89
Endoderm
Notochord---nucleus pulposis
90
THALIDOMIDE INHIBITS WHAT?
AER ( proximal to distal)
91
Notochord (endoderm) induces the ectooderm to form the spinal cord. when does notchod go away
After somoites form vertebra from mesoderm ( nulceus pulposis is remnant of notochord)
Chordoma is Notochord remnant
92
Vertebral Body Growth ends:
| Neural Arch growth ends:
Body Growth ends at 16-18
| Arch growth at 8 years- canald diameter fixed at 8 yeras
93
Where does the epiphyseal vessels STOP in the growth plate?
Goes through reserve zone and proliferative zone ( high o2)but NOT hypertrophic ZONE (low o2 tension)
94
Groove on Ranvier does what?
Anchors to pericondrium and BONE formation wthout latitudnal growth
95
Perichondral ring of LaCroix
Give mechanical support to the epiphysis and growth plate
96
C-R_I_T_O_E
6months, 3, 5, 7, 9, 11
97
Autosomal Dominant?
1. Achodonroplastia
2. SED
3. Jansen and Smid Metaphyseal Dysplasia
98
Autosomal Recessive
Morquios
Distrphic Dysplasia
Often SINGLE enzyme disorder
99
Partial deletions in Chromosome 15
Prader Willi- Paternal deletion
| Angelmann- Materal deletion
100
Intraarticular metaphysis?
Hip
Shoulder
Elbow
Ankle
NOT KNEE
101
Most common acute hematogenous infections in Kids?
S. Aureus
PVL Strains: more complex, multisystem organ failure
CLINDAMYCIN
102
Kocher Criteria
Fever
Inability to bear weight
WBC >12
ESR>40
4/4 99% septic hip
CRP >2 independent risk factor for septic hip
103
XLD condition
XL Hypophosphatemic ricketts
104
Parellelism of the Talus and calcaneus on AP AND LATERAL?
Pediatric Clubfoot
105
What is needed after Ponsetti Casting for Clubfoot
Foot External rotation orthosis (70d)
| Full Time for 3 months then night tim until 3-4 years
106
Most common reason for clubfoot recurrance?
lack of bracing compliance
| Need for early recasting
107
Last Portion of Ponsetti Casting?
```
Equinus release (tenotomy)
CAVE
```
108
Long term seqeula of Clubfoot?
Lateral forefoot overload
Have to transfer Anterior TIb. tendon to Lateral cunieform
They have dynamic supination with gait
109
Metatarsus Adductus
Packaging Disorder
85% resolve
Medial devaited forefoot with convex lateral border
110
Type of foot does CVT have?
Dorsal dislocation of navicular on talus
Rocker botttom foot
50% due to neuromuscular or genetic causes
111
What view do you want to get with CVT suspected?
Lateral XR of foot in PLANTAR FLEXION
Axis of first metarsal is ABOVE the talus
(navicular does not ossify until 3 yers
112
What is most common Tarsal Colalition?
1. Calcaneonavicular (45d oblique view) 8-12yo
| 2. Talo Navicular 12-15 yo,
113
What is weak/strong in CMT?
Posterior Tib Strong
Peroneus Longus strong
Peroneus Brevis Wekan,
Anteriro Tib Weak
114
COL1A1 or COL1A2 disease and anethesia risk?
Osteogensis imperfecta
BLue sclera
Malignant Hyperthermia Risk
olecranon apophysis avulsion fractures
115
Bisphosphonates for OI?
Decrease fracture incidence and
| PARK HARISS lines on Xray
116
Fibrillin 1 defect?
Marfans Syndrome
Aortic Roo dilation, SUPERIOR Lends dislocation
AUTOSOMAL DOMINANT
Acetbular Proturssio
117
Achondroplastia Spinal Findings ?
```
FGFR3
Decreasing interpedicular Length from L1-L5
autsomal Dominant ( but much are random and with advnaced paternal age)
```
PROLIFERATIVE ZONE OF PHYSIS
118
Spinal Pathology in Achondroplasia
1. Foramen magnum stenosos ( Sleep apnea and death)
2. Thoracolumbar Kyphosis ( most resolve when walking begings)
3. Lumbar Spinal Stenosis ( usually later)
119
Keratin Sulfate Accumulation Disorder?
Moroquio Syndrome
Abnormal Beta Galatosidase enzyme
ODONTOID HYPOLASIA- C1-C2 instabiliity
120
What do you have to due prior to surgery for
1. Mucopolysaccaradosis
2. Pseudochondroplastia
3. SED
Get flexion extension veiws of C-Spine
121
DDH Timeline Treatment
0-6mo- Pavlik Harness
6-18mo- Spica casting, closed reduction ( placed for 3months- adductor tenotomy
>18monts_ OPEN reduction +/- Pelvic and femoral osteotomies
122
What are looking for on medial die pool on arthrogram in DDH
<5mm for reduction
123
Approaches for Open reduction in DDH
Medial- Risk of AVN
| Anterior lateral- can do capsuloraphy and pelvic osteotomies through same approach
124
What pelvic osteotomies hinge on Triradiates?
Dega
Pemberton
San Diego
125
Types of pelvic Osteotomies ?
Redirectional( Salter, Ganz, Triple)
ReContourning ( Dega, Pemberton, Sandiego)
Salvage; Chiari and Shelf
126
Herring Classification of LCP
a: no loss of laterl 1/3 height
B: <50% loss
B/C: 50% loss of hiehg
C: >50% loss of height
127
Gage Sign?
Osteopenic "V" lateral aspect of physis
128
Bracket Phalanx or Metatarsal in kid- what is treatment ?
PHysiolysis - break it up
OITE Prep Fredericks
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