1
Q
genetic inheritance of pseudoachondroplasia
A
AD
2
Q
genetic inheritance of achondroplasia
A
AD, 80% spontaneous mutation
3
Q
genetic defect of pseudoachondroplasia
A
COMP
4
Q
genetic defect of achondroplasia
A
FGFR3
5
Q
genetic defect of spondyloepiphyseal dysplasia
A
type II collagen
6
Q
champagne pelvis
A
achondroplasia
7
Q
spinal manifestations of pseudoachon
A
lumbar lordosis and cervical instability
8
Q
genetic defect of Jansen metaphyseal chondrodysplasia
A
PTHRP
9
Q
genetic defect of McCune Albright
A
cAMP
10
Q
spinal manifestations of achondroplasia
A
lumbar lordosis and stenosis, short pedicles with decreased interpedicular distance
11
Q
upper limb manifestations of achondroplasia
A
radial head subluxation and trident hands
12
Q
abnormal epiphyseal development with concomitant spine involvement
A
SED
13
Q
abnormal epiphyseal development with no concomitant spine involvement
A
MED
14
Q
non-ortho manifestation of the disease with abnormal epiphyseal development and concomitant spine involvement
A
retinal detachment is common with SED
15
Q
dumbbell shaped bones, especially femur
A
Kniest syndrome
16
Q
genetic defect of Schmid’s metaphyseal chondrodysplasia
A
type X collagen
17
Q
gene defect of disease with flattened femurs, valgus knees
A
COMP, (MED)
18
Q
found in the urine of Morquios
A
keratan sulfate
19
Q
Morquios mnemonic
A
Most common, Odontoid hypOplasia, Keratan sulfate, I is clOudy, Intelligent
20
Q
inheritance of Hurlers
A
AR
21
Q
inheritance of Hunters
A
XLR
22
Q
spinal manifestations of diastrophic dysplasia
A
cervical kyphosis
23
Q
non-ortho manifestations of diastrophic dysplasia
A
caluiflower ears, cleft palate
24
Q
genetic defect in cleidocranial dysplasia
A
CBFA1, RUNX2 which are transcription factors for osteocalcin
25
this is thought to be related to COL6A1 in Down syndrome
type 6 collagen abnormality = joint laxity, hip/patellar dislocation
26
decreased amounts of Factor VIII
hemophilia A
27
abnormal factor VIII
von Willebrands
28
squared patella, femoral condyles
hemophilia
29
when is synovectomy for hemophilia indicated
recurrent hemarthroses despite medical mgmt. Can be done arthroscopically
30
this is a relative contraindication for surgery in hemophilia
presence of IgG inhibitors
31
spinal manifestation of dz that causes abnormal collagen cross-linking
basilar invagination common in severe OI
32
this can occur from bisphosphonate use in OI
iatrogenic osteopetrosis
33
can be used to treat the dz with rugger jersey spine
bone marrow transplant useful in malignant forms of osteopetrosis
34
radiologic findings of marfan's
scoliosis and acetabular protrusio
35
two differences b/t marfans and homocystinuria
superior lens in marfans, and no osteoporosis
36
non-ortho manifestation of JIA
iridocyclitis; slit lamp exam q 6 mos
37
why get a CT instead of MRI for treatment of brachial plexus palsy
with humeral internal rotation contracture, 70% get progressive glenoid hypoplasia due to position of humeral head
38
Two findings following birth plexus injury that carry poor prognosis
lack of biceps function at 6 mos and presence of a horners syndrome are both bad
39
surgical option for birth plexus injury
lat and teres major transfer to the shoulder external rotators. If done before age 2 can also release subscap
40
stretching for torticollis
rotate chin toward ipsilateral shoulder and tilt head toward contralateral
41
imaging for torticollis
ultrasound; predictive of failure of nonop mgmt if severe fibrosis present
42
physiologic classes of CP
spastic, athetotic, ataxic, mixed
43
most common type of CP
spastic, characterized by slow, restricted movements bc of simultaneous agonist/antagonist action
44
this type of CP shows involvement of the lower extremities more than the uppers
diplegic CP involves the lowers more than the uppers
45
in this type of CP pts are usually unable to walk
quadriplegic CP, in contrast to hemi or diplegic
46
surgery for stiff-leg gait in CP
rectus transfer to hamstrings
47
scissor gait problem
adductor contracture
48
risk for scoli in CP highest with
spastic quads
49
general rule for surgery in CP
none before age 3, unless hip at risk
50
CP hip at risk
abduction angle
51
this can be done if there is a loss of knee flexion during the swing phase of gait
out-of-phase rectus femoris transfer to the semi-T or gracilis muscle
52
indication for an out-of-phase rectus femoris transfer to the semi-T or gracilis muscle
loss of knee flexion in swing phase of gait in CP
53
this foot deformity is most common in spastic diplegia
equinovalgus foot
54
this foot deformity is most common in spastic hemiplegia
equinovarus foot
55
cause of equinovalgus foot in spastic CP
overpull of the peroneal muscles
56
cause of equinovarus foot in spastic CP
overpull of the AT, PT, or both
57
two basic rules about surgical mgmt of most common foot deformity in spastic hemiplegia
this is equinovarus. You can't just lengthen, and you can't just transfer a whole muscle. You have to split transfer
58
when are split-muscle transfers helpful in spastic CP foot deformities
when the muscle is spastic in both swing and stance phases of gait
59
no flexion creases
arthrogryposis
60
multiple joint contractures, decreased anterior horn cell activity, normal intelligence
arthrogryposis
61
upper extremity mgmt in arthrogryposis
anterior triceps transfer, posterior soft tissue release
62
surgical mgmt of bilateral hip dislocation in arthrogryposis
usually left unreduced because ambulation is preserved
63
surgical mgmt of bilateral elbow pathology in arthrogryposis
leave one in extension for hygiene, flex one for feeding
64
knee contractures, hip dislocation in arthrogryposis
correct the knee first
65
initial and recurrent treatment for foot deformities in arthrogryposis
soft tissue release first, but recurrence may need bony procedure (talectomy). Goal is stiff, plantigrade foot.
66
important level in myelodysplasia and why
L4 gives quad function, important bc allows some community ambulation
67
change in function, UTI, or new deficit in spina bifida
this can be associated with tethered cord or hydrocephalus
68
can be confused with infection in myelodysplasia
fractures can mimic infection in ages 3-7 in spina bifida
69
perioperative concern in myelodysplasia
many myelodysplastics are latex allergic
70
only instance in which hip containment is not controversial in myelodysplasia
in myelodysplasia pts that have a functioning quadriceps
71
what has no bearing on the functional outcome of a myelomeningocele
whether the hips are reduced or not
72
since hip position does not affect myelo outcomes, mgmt consists of
soft tissue releases
73
how are the surgical mgmts of valgus deformity in mature and immature pts different
in skeletally immature pts you do a distal tibial hemiarrest or Achilles tendodesis to the fibula. In mature pts you do a distal tibial osteotomy
74
this is typically avoided in myelodysplastics
triple arthrodeses reserved for severe deformities and sensate feet
75
surgical mgmt of rigid clubfoot in myelodysplastics
subtalar release, TAL and tib post lengthening, AT transfer to the dorsal midfoot
76
mgmt of scoliosis in dz with high serum levels of creatine phosphokinase
in muscular dystrophy, scoliosis can progress rapidly due to lack of muscle support. This can have significant effect on respiratory function, and therefore is treated earlier. Curves of 25-30*.
77
scapulothoracic fusion
FSH, facioscapulohumeral muscular dystrophy
78
Nystagmus, wide gait, cardiomyopathy
Friedrich's ataxia, cavus foot, scoliosis
79
CMT aka...
peroneal muscle atrophy. Defect Chr17, PMP22
80
first foot deformity of dz caused by defective PMP22 and its etiology
Plantarflexed first ray occurs first in CMT, due to the weakened anterior tib (as well as PL/PB)
81
algorithm for surgical mgmt of CMT foot
if the hindfoot varus is flexible, you can get away with post tib transfer. If not you have to do a calcaneal osteotomy at a minimum, possibly a triple arthrodesis
82
hallmark of polio
motor weakness with normal sensation
83
site of destruction of poliovirus
anterior horn cells and brainstem motor nuclei
84
survival motor neuron gene
spinal muscular atrophy
85
mgmt of hip instability in dz with autosomal recessive loss of the anterior horn cells in the spinal cord
hip dislocation or subluxation in SMA is treated nonoperatively
86
what to tell parents of a child with SMA considering spinal fusion about the mgmt of the extremities
there may be a transient decrease in the function of the uppers, and contractures in the lowers should be addressed prior to spine surgery (sitting balance)
87
hemihypertrophy
assoc with Wilms tumor (serial abd ultrasound) as well as more commonly with neurofibromatosis
88
threshold for scoli evaluation
7*
89
risk factors for AIS progression
Curve more than 20 in young, more than 45 if mature. Thoracic curves progress faster than lumbar, as do double curves in comparison to single curves
90
who gets MRI in peds scoli
painful scoli, left curves, onset before 11 (Juvenile), associated syndromes or neural deficits, congenital abnormalities
91
2 indications for anterior + PSF in scoli
very large curves (75*+) that may need anterior releases to improve flexibility, and very young pts that still have spinal growth remaining (crankshaft)
92
stable vertebra
most proximal vertebra that is most closely bisected by the center sacral line
93
end vertebra
the most tilted vertebra
94
neutral vertebra
has no rotation in the axial plane
95
structural curve
either the largest curve or one that does not bend to less than 25*
96
acute infection following PSF for scoli
D/I, abx suppression, hardware retention until fusion. Typically Staph aureus
97
delayed infection following PSF for scoli
Typically P acnes or Staph epi. Hardware removal, check for pseudoarthrosis, abx.
98
treatment for pseudoarthrosis in spine fusion (maybe just scoli)
compression instrumentation and bone grafting
99
differences between infantile and adolescent idiopathic scolis
Infantile is more common in boys. More commonly has a left curve. Associated more with congenital defects. And most curves resolve spontaneously, although there are exceptions (RVAD scale...)
100
these radiographic parameters are indicative of high risk for progression of infantile scoli
if there is overlap of the medial rib relative to the apical vertebra, or Rib Vertebral Angle greater than 20*
101
indication for casting scoliosis
Mehta casting can be done for young pts with flexible curves
102
this type of scoli routinely has progression of their curves
juvenile idiopathic scoli has high rate of curve progression, ages 3-10
103
why are MRI's routinely ordered in JIScoli
there is 25% rate of spinal cord abnormalities in JIS
104
who commonly gets a cardiac workup for scoli surgery
Pts with duchennes muscular dystrophy get cardiomyopathy, and commonly have scoli due to loss of muscle support
105
nutritional lab markers for successful scoli surgery
leukocyte counts above 1500, albumin > 3.5
106
two additional concerns in congenital scoliosis, after MRI
renal ultrasound, (25% GU defect rate) and cardiac workup (echo? 10% cardiac anomalies)
107
two main types of congenital scoli
failure of segmentation (bar) or failure of formation (hemivertebra)
108
worst prognosis in congenital scoli
unilateral bar with contralateral fully segmented hemivertebra
109
this type of hemivertebra has higher risk of progression
if a hemivertebra is fully segmented, rather than unsegmented, its risk for progression is higher
110
when resection is indicated in congenital scoli
if a hemivertebrae is fully segmented, it has high risk of progression and can be resected
111
sole (?) indication for fusion anteriorly only, in pediatric scoli
as part of surgical mgmt of fully segmented hemivertebral congenital scoli that has begun progressing. Combined with hemivert excision.
112
this type of congenital kyphosis is most common
failure of formation
113
this type of congenital kyphosis has the worst prognosis
type I, or failure of formation
114
this type of congenital kyphosis has the highest risk for neuro complications
type I, or failure of formation
115
indications for PSF in congenital kyphosis
Favored in kids
116
indications for PSF plus anterior procedure in congenital kyphosis
Older than 5, or with curves > 50*. If there are neuro deficits: vertebrectomy/decompression PLUS ASF then PSF.
117
most common site of skeletal involvement in neurofibromatosis
spinal involvement common in NF
118
two types of NF related scoli
nondystrophic (which is treated like AIS) and dystrophic (tight sharp curves, vertebral scalloping, rib penciling)
119
why is rib penciling important in NF dystrophic scoli
Penciling of 3 or more ribs prognostic of rapid deformity
120
thresholds for surgical treatment of dystrophic NF scoli
surgery for curve progression, and when above 40*. Also kyphosis.
121
diastematomyelia
tissue bar that causes cleft in the spinal cord
122
treatment of diastematomyelia
unless spinal deformity present (has to be resected first) or a neuro deficit it can be observed
123
earliest radiographic finding of discitis
loss of the normal lumbar lordosis
124
indication for repair of a pars defect
pars defects at L4 and above
125
this spondylolisthesis has the highest risk for progression
dysplastic type of spondy has highest risk of progression
126
spondy slip of less than 50% treated with
after failure of non-op (bracing, PT, activity mod), arthrodesis +/- instrumentation
127
spondy slip of more than 50% treated with
usually surgery as there is pain and risk of progression; L4-S1 fusion. Decompression performed if there is high-grade slip or neurologic symptoms
128
additional imaging study besides AP and Lat lumbar spine films when pediatric pars defect suspected
SPECT scanning done for equivocal cases of peds spondy
129
definition of kyphosis
45* curve, 3 sequential vertebra with >5* of wedge
130
frequency of MRI in Scheuermann's kyphosis
neurologic changes are rare, so are MRI's. But if present get MRI.
131
Non-op treatment for scheuerman's kyphosis
bracing done for progressive curves in pts that have at least one year of growth (
132
Indications for surgery in scheuerman's kyphosis
Pain after PT, skeletal maturity, curve >75*. Cosmesis is relative indication
133
klippel-feil etiology
failure of formation or segmentation in multiple cervical segments during 3rd-8th weeks of gestation
134
conditions associated with klippel-feil
congenital scoliosis, renal aplasia, sprengel deformity, congenital heart deformities
135
although not often seen, classic triad of klippel-feil
low posterior hairline, webbed neck, decreased cervical motion
136
atlantoaxial instability assoc with these
down syndrome, JRA, various osteochondrodystrophies
137
grisel dz
retropharyngeal inflammation, leads to rotatory atlantoaxial instability
138
indications for surgical mgmt of os odontoideum
Instability (>10mm ADI,
139
distinguishing pseudosubluxation from true subluxation
in pseudo, the posterior spinolaminar line is not disrupted. This is a normal finding in kids younger than 8
140
cervical spine, disc calcification, elevated ESR, pain, decreased ROM
disc calcification syndrome, conservative mgmt, self-limiting
141
sprengel's deformity
undescended scapula, winging. Most common congenital shoulder deformity of children.
142
common causes for in-toeing in peds
metatarsus adductus in infants, tibial torsion in toddlers, and femoral anteversion up to 10 yrs
143
common causes for out-toeing in peds
external hip contractures in infants, from the tibia or femur in older children or adolescents
144
normal foot progression angle and its significance
-5 to 20. Abnormal is non-specific rotation problem
145
normal prone hip rotation angle and its significance
>70* internal rotation, or
146
normal thigh foot angle and its significance
normal 0-20.
147
when surgery is done for tibial torsion
7-10 years old, supramalleolar osteotomy
148
Congenital DDH can be associated with
other uterine packaging defects, such as torticollis and metatarsus adductus
149
risk factors for DDH
breech first-born females with a family history. Most often the leFt.
150
Ortolani/Barlow
Can't both be positive. Ortolani, out and you can get it in. Barlow, back in but you can get it out
151
anterior straps of pavlik
flexion
152
posterior straps of pavlik
abduction
153
when does ossific nucleus of femoral head appear
~ 6 mos
154
radiographic position of femoral ossific nucleus in DDH
superior to hilgengreiners, lateral to perkins
155
Up to 6 months, DDH treatment consists of
Pavlik. Recheck reduction in 3 weeks by ultrasound. If reduced, continue harness until exam and U/S are normal. If it is out, reduce it, get arthrogram, and spica cast
156
From 6-18 months, DDH treatment consists of
perform hip arthrography, percutaneous adductor tenotomy, closed reduction, and spica casting.
157
After walking age up to age 6-8, DDH treatment consists of
open reduction
158
Unilateral DDH after age 8
leave it alone.
159
after age 6, DDH unilateral
consider osteotomy (until age 8...)
160
after age 4 this isn't an option for treatment of DDH anymore
femoral osteotomy not successful that late
161
bilateral DDH after age 6
leave it alone
162
filling defect on arthrogram of unsuccessful DDH reduction attempt
superior limbus
163
this position can result in femoral nerve palsy in pavlik treatment of DDH
excessive flexion
164
this position can result in femoral head osteonecrosis in pavlik treatment of DDH
excessive abduction
165
approach for open reduction of DDH
anterior (less risk of damage to MFCA)
166
these pelvic osteotomies require periacetabular metaplasia for success
Chiari, shelf
167
this pelvic osteotomy leaves the posterior column intact
PAO
168
triangular ossification defect inferomedial femoral neck
congenital coxa vara
169
treatment of congenital coxa vara
depends on the Hilgengreiner-Epiphyseal angle: 60*.
170
predictors of poor outcome in noninflammatory dz causing deformity of the proximal femur secondary to vascular insult
These Perthes pts do worst: females with bone age older than 6, decreased ROM, and lateral column classification (C)
171
treatment regimen for Perthes
NSAIDs, traction, protected weightbearing, maintain ROM
172
Herring proposed surgery for these Perthes pts
B/C and C pts older than 8
173
radiographic signs of poor prognosis in noninflammatory dz causing deformity of the proximal femur secondary to vascular insult
Perthes: lateral calcification, Gage sign (v-shaped defect at the lateral physis, lateral subluxation, metaphyseal cysts, horizontal growth plate
174
SCFE occurs here
weakness of the perichondrial ring and slippage through the hypertrophic zone of the growth plate
175
ROM exam finding in SCFE
obligate ER with flexion
176
Difference in stable vs unstable SCFE with respect to AVN rates
No pts with stable develop AVN; Half of those with unstable slips do
177
Indications for prophylactic SCFE pinning
Endocrinopathies,
178
SCFE pin placement in this location has the highest rate of joint penetration
placing screws in the anterior superior part of the head = highest joint penetration
179
threshold for amputation in femoral deficiency
if the femur is less than 50% of the contralateral side
180
Indication for CT in LLD
knee flexion contracture
181
rough guide for length per year in mm in the leg
5-9-6-3, from prox femur to distal tibia
182
LLD less than 2 cm
lifts, non-op
183
LLD more than 5 cm
lengthening
184
ESR value in transient synovitis
typically less than 20mm/hr
185
Utility of CRP in peds infections
should decline within 48-72 hrs of treatment; if not change the treatment
186
indications for operative mgmt of peds osteomyelitis
failure to respond to abx, frank pus (MRI or aspiration), or sequestered abscess
187
when does periosteal new bone form in peds osteomyelitis
5-7 days
188
these help distinguish septic arthritis from transient synovitis
refusal to bear weight, WBC > 12,000, Fever >101.5, ESR > 40 are not found in transient synovitis. These are Kocher criteria
189
This pathogen does not need surgical drainage if it causes septic arthritis
Neisseria, can be treated with penicillin (need to verify...)
190
metaphyseal beaking
infantile blounts
191
non-op treatment in blounts dz from 0-4 yrs
Stage I and II: KAFO for pts under 3. Surgery for higher stages and stage II older than 3. Overcorrect into a little valgus. In stage III the epiphysis looks like it is melting into the metaphysis
192
this amount of genu valgum is gon' be foine
up to 15* from 2-6 yrs
193
indications for surgery in genu valgum
more than 10cm between medial mals, more than 15* valgus, and only if older than 10 yrs
194
posteromedial tibial bowing
physiologic, intrauterine packaging. Commonly associated with calcaneovalgus feet and LLD
195
anteromedial tibial bowing
associated with fibular hemimelia, equinovarus, tarsal coalition, and LLD
196
anterolateral tibial bowing
most common cause is congenital pseudoarthrosis of the tibia. 50% of pts with anterolateral bow have NF. Only 10% of pts with anterolateral bow have NF.
197
initial treatment for tibial bowing associated with NF
anterolateral = total contact brace
198
only long bone deficiency with a known inheritance pattern
tibial hemimelia, autosomal dominant
199
other deformity often present with tibial hemimelia
lobster claw hand
200
most frequent location OCD
lateral aspect of MFC
201
indications for OCD lesions
loose or little growth remaining
202
indications for discoid meniscus
only if symptomatic and torn: saucerization
203
clinical features of clubfoot
forefoot adductus and supination, combined with hindfoot varus and equinus
204
radiographic appearance of talus / calcaneus in clubfoot
parallel
205
CAVE in clubfoot Ponseti method
order of correction, cavus, adductus, varus, then equinus.
206
common deformity after clubfoot treatment, its cause, and treatment
persistent supination of the forefoot, which is believed to be from overpull of the anterior tibialis and a weak peroneus, or undercorrection of forefoot supination. This often requires transfer of the AT laterally
207
possible gene cause for clubfoot
PITX-1
208
treatments for clubfoot after ponseti casting
achille's tenotomy at 6-8 weeks, braces for 2-4 years
209
clinical appearance of metatarsus adductus
heel bisector line that hits lateral to the 2nd webspace
210
treatment for metatarsus adductus
supple feet can be stretched, stiff feet can be casted
211
treatment for skewfoot
non-operative treatment does not work
212
test in pes cavus
besides full neuro workup, a Coleman block test tells you whether hindfoot corrects or not
213
treatment for pes cavus
after neuro workup, assess whether supple or not (Coleman block) If flexible, plantar release, metatarsal osteotomy, posterior tib transfer. If hindfoot varus is RIGID, add a calcaneal osteotomy as well.
214
this is not the answer in pes cavus that does not correct with Coleman block testing
Avoid triple arthrodesis; use calcaneal slide instead
215
irreducible dorsal dislocation of the navicular on the talus, and fixed equinus hindfoot
CVT
216
radiographic findings in CVT
long axis of the talus hits posterior to the metatarsal axis
217
irregular middle facet on Harris heel view
talocalcaneal coalition
218
most common tarsal coalition in children 10-12
calcaneonavicular
219
most common tarsal coalition in children 12-14
subtalar
220
surgical treatment for subtalar coalition
only after failing non-op, if there is 50%, subtalar fusion (verify this with foot talk)
221
juvenile bunions
don't operate unless skeletally mature
222
radiographs in this condition mimic CVT
flexible pes planus, except a plantar-flexed view shows talar axis passing above the metatarsal cuneiform axis
223
this view is helpful in accessory navicular
external oblique
224
threshold for spine surgery in OI
treated at 35* if crappy bone, with PSF.
ABOS PART ONE
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