peds Flashcards
Question
Answer
genetic inheritance of achondroplasia
AD, 80% spontaneous mutation, FGFR3, champagne pelvis, lumbar lordosis and stenosis, short pedicles with decreased interpedicular distance. Foramen magnum stenosis- central apnea. radial head subluxation and trident hands
genetic defect of pseudoachondroplasia
COMP
genetic defect of spondyloepiphyseal dysplasia
type II collagen
spinal manifestations of pseudoachon
lumbar lordosis and cervical instability
genetic defect of Jansen metaphyseal chondrodysplasia
PTHRP
genetic defect of McCune Albright
cAMP.(cafe au lait spots in coast of Maine pattern)endocrine abnormalities (precocious puberty)\renal phosphate wasting due to FGF-23 (oncogenic osteomalacia) unilateral polyostotic fibrous dysplasia obtain AP spine radiographs to look for scoliosis
abnormal epiphyseal development with concomitant spine involvement
SED
abnormal epiphyseal development with no concomitant spine involvement
MED
non-ortho manifestation of the disease with abnormal epiphyseal development and concomitant spine involvement
retinal detachment is common with SED
dumbbell shaped bones, especially femur
Kniest syndrome - COL2A1 defect. Dwarf, retinal detach,
genetic defect of Schmid’s metaphyseal chondrodysplasia
type X collagen, AD, Severe coxa vara, gene varrem like rickets
gene defect of disease with flattened femurs, valgus knees
COMP, (MED)
found in the urine of Morquios
keratan sulfate
Morquios mnemonic
AR, Most common, Odontoid hypOplasia, Keratan sulfate, I is clOudy, Intelligent. C Spine instability
inheritance of Hurlers
AR
inheritance of Hunters
XLR
diastrophic dysplasia
autosomal recessive, mutation in DTDST gene (SLC26A2) on chromosome 5 , encodes for sulfate transporter protein. cervical kyphosis. Common in Finnish. Cauliflower ears, hitchhiker thumb. Hip/knee contractors
genetic defect in cleidocranial dysplasia
CBFA1, RUNX2 which are transcription factors for osteocalcin
this is thought to be related to COL6A1 in Down syndrome
type 6 collagen abnormality = joint laxity, hip/patellar dislocation
Factors for Hemaphilia A & B
VIII, IX. X linked recessive
abnormal factor VIII
von Willebrands
squared patella, femoral condyles
hemophilia
when is synovectomy for hemophilia indicated
recurrent hemarthroses despite medical mgmt. Can be done arthroscopically. VIII must be 50% normal
this is a relative contraindication for surgery in hemophilia
presence of IgG inhibitors
spinal manifestation of OI
basilar invagination common in severe OI
this can occur from bisphosphonate use in OI
iatrogenic osteopetrosis
radiologic findings of marfan’s
scoliosis and acetabular protrusio
two differences b/t marfans and homocystinuria
superior lens in marfans, and no osteoporosis
non-ortho manifestation of JIA
iridocyclitis; slit lamp exam q 6 mos
Two findings following birth plexus injury that carry poor prognosis
lack of biceps function at 3-6 mos and presence of a horners syndrome are both bad.Preganglionic will have no rhomboid
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
imaging for torticollis
ultrasound; predictive of failure of nonop mgmt if severe fibrosis present
physiologic classes of CP
spastic, athetotic, ataxic, mixed
most common type of CP
spastic, characterized by slow, restricted movements bc of simultaneous agonist/antagonist action
this type of CP shows involvement of the lower extremities more than the uppers
diplegic CP involves the lowers more than the uppers
in this type of CP pts are usually unable to walk
quadriplegic CP, in contrast to hemi or diplegic
surgery for stiff-leg gait in CP
rectus transfer to hamstrings
scissor gait problem
adductor contracture
risk for scoli in CP highest with
spastic quads
general rule for surgery in CP
none before age 3, unless hip at risk
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
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
this foot deformity is most common in spastic diplegia
equinovalgus foot
this foot deformity is most common in spastic hemiplegia
equinovarus foot
cause of equinovalgus foot in spastic CP
overpull of the peroneal muscles
cause of equinovarus foot in spastic CP
overpull of the AT, PT, or both
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
when are split-muscle transfers helpful in spastic CP foot deformities
when the muscle is spastic in both swing and stance phases of gait
no flexion creases
arthrogryposis
multiple joint contractures, decreased anterior horn cell activity, normal intelligence
arthrogryposis
upper extremity mgmt in arthrogryposis
anterior triceps transfer, posterior soft tissue release
surgical mgmt of bilateral hip dislocation in arthrogryposis
usually left unreduced because ambulation is preserved
surgical mgmt of bilateral elbow pathology in arthrogryposis
leave one in extension for hygiene, flex one for feeding
knee contractures, hip dislocation in arthrogryposis
correct the knee first
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.
important level in myelodysplasia and why
L4 gives quad function, important bc allows some community ambulation
change in function, UTI, or new deficit in spina bifida
this can be associated with tethered cord or hydrocephalus
can be confused with infection in myelodysplasia
fractures can mimic infection in ages 3-7 in spina bifida
perioperative concern in myelodysplasia
many myelodysplastics are latex allergic
only instance in which hip containment is not controversial in myelodysplasia
in myelodysplasia pts that have a functioning quadriceps
what has no bearing on the functional outcome of a myelomeningocele
whether the hips are reduced or not
since hip position does not affect myelo outcomes, mgmt consists of
soft tissue releases
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
this is typically avoided in myelodysplastics
triple arthrodeses reserved for severe deformities and sensate feet
surgical mgmt of rigid clubfoot in myelodysplastics
subtalar release, TAL and tib post lengthening, AT transfer to the dorsal midfoot
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*.
scapulothoracic fusion
FSH, facioscapulohumeral muscular dystrophy
Nystagmus, wide gait, cardiomyopathy
Friedrich’s ataxia, cavus foot, scoliosis
CMT aka…
peroneal muscle atrophy. Defect Chr17, PMP22
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)
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
hallmark of polio
motor weakness with normal sensation
site of destruction of poliovirus
anterior horn cells and brainstem motor nuclei
survival motor neuron gene
spinal muscular atrophy
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
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)
hemihypertrophy
assoc with Wilms tumor (serial abd ultrasound) as well as more commonly with neurofibromatosis
threshold for scoli evaluation
7*
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
who gets MRI in peds scoli
painful scoli, left curves, onset before 11 (Juvenile), associated syndromes or neural deficits, congenital abnormalities
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)
stable vertebra
most proximal vertebra that is most closely bisected by the center sacral line
end vertebra
the most tilted vertebra
neutral vertebra
has no rotation in the axial plane
structural curve
either the largest curve or one that does not bend to less than 25*
acute infection following PSF for scoli
D/I, abx suppression, hardware retention until fusion. Typically Staph aureus
delayed infection following PSF for scoli
Typically P acnes or Staph epi. Hardware removal, check for pseudoarthrosis, abx.
treatment for pseudoarthrosis in spine fusion (maybe just scoli)
compression instrumentation and bone grafting
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…)
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*
indication for casting scoliosis
Mehta casting can be done for young pts with flexible curves
this type of scoli routinely has progression of their curves
juvenile idiopathic scoli has high rate of curve progression, ages 3-10
why are MRI’s routinely ordered in JIScoli
there is 25% rate of spinal cord abnormalities in JIS
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
nutritional lab markers for successful scoli surgery
leukocyte counts above 1500, albumin > 3.5
two additional concerns in congenital scoliosis, after MRI
renal ultrasound, (25% GU defect rate) and cardiac workup (echo? 10% cardiac anomalies)
two main types of congenital scoli
failure of segmentation (bar) or failure of formation (hemivertebra)
worst prognosis in congenital scoli
unilateral bar with contralateral fully segmented hemivertebra
this type of hemivertebra has higher risk of progression
if a hemivertebra is fully segmented, rather than unsegmented, its risk for progression is higher
when resection is indicated in congenital scoli
if a hemivertebrae is fully segmented, it has high risk of progression and can be resected
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.
this type of congenital kyphosis is most common
failure of formation
this type of congenital kyphosis has the worst prognosis
type I, or failure of formation
this type of congenital kyphosis has the highest risk for neuro complications
type I, or failure of formation
indications for PSF in congenital kyphosis
Favored in kids
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.
most common site of skeletal involvement in neurofibromatosis
spinal involvement common in NF
two types of NF related scoli
nondystrophic (which is treated like AIS) and dystrophic (tight sharp curves, vertebral scalloping, rib penciling)
why is rib penciling important in NF dystrophic scoli
Penciling of 3 or more ribs prognostic of rapid deformity
thresholds for surgical treatment of dystrophic NF scoli
surgery for curve progression, and when above 40*. Also kyphosis.
diastematomyelia
tissue bar that causes cleft in the spinal cord
treatment of diastematomyelia
unless spinal deformity present (has to be resected first) or a neuro deficit it can be observed
earliest radiographic finding of discitis
loss of the normal lumbar lordosis
indication for repair of a pars defect
pars defects at L4 and above
this spondylolisthesis has the highest risk for progression
dysplastic type of spondy has highest risk of progression
spondy slip of less than 50% treated with
after failure of non-op (bracing, PT, activity mod), arthrodesis +/- instrumentation
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
additional imaging study besides AP and Lat lumbar spine films when pediatric pars defect suspected
SPECT scanning done for equivocal cases of peds spondy
definition of kyphosis
45* curve, 3 sequential vertebra with >5* of wedge
frequency of MRI in Scheuermann’s kyphosis
neurologic changes are rare, so are MRI’s. But if present get MRI.
Non-op treatment for scheuerman’s kyphosis
bracing done for progressive curves in pts that have at least one year of growth (
Indications for surgery in scheuerman’s kyphosis
Pain after PT, skeletal maturity, curve >75*. Cosmesis is relative indication
klippel-feil etiology
failure of formation or segmentation in multiple cervical segments during 3rd-8th weeks of gestation
conditions associated with klippel-feil
congenital scoliosis, renal aplasia, sprengel deformity, congenital heart deformities
although not often seen, classic triad of klippel-feil
low posterior hairline, webbed neck, decreased cervical motion
atlantoaxial instability assoc with these
down syndrome, JRA, various osteochondrodystrophies
grisel dz
retropharyngeal inflammation, leads to rotatory atlantoaxial instability
indications for surgical mgmt of os odontoideum
Instability (>10mm ADI,
distinguishing pseudosubluxation from true subluxation
in pseudo, the posterior spinolaminar line is not disrupted. This is a normal finding in kids younger than 8
cervical spine, disc calcification, elevated ESR, pain, decreased ROM
disc calcification syndrome, conservative mgmt, self-limiting
sprengel’s deformity
undescended scapula, winging. Most common congenital shoulder deformity of children.
common causes for in-toeing in peds
metatarsus adductus in infants, tibial torsion in toddlers, and femoral anteversion up to 10 yrs
common causes for out-toeing in peds
external hip contractures in infants, from the tibia or femur in older children or adolescents
normal foot progression angle and its significance
-5 to 20. Abnormal is non-specific rotation problem
normal prone hip rotation angle and its significance
>70* internal rotation, or
normal thigh foot angle and its significance
normal 0-20.
when surgery is done for tibial torsion
7-10 years old, supramalleolar osteotomy
Congenital DDH can be associated with
other uterine packaging defects, such as torticollis and metatarsus adductus
risk factors for DDH
breech first-born females with a family history. Most often the leFt.
Ortolani/Barlow
Can’t both be positive. Ortolani, out and you can get it in. Barlow, back in but you can get it out
anterior straps of pavlik
flexion
posterior straps of pavlik
abduction
when does ossific nucleus of femoral head appear
~ 6 mos
radiographic position of femoral ossific nucleus in DDH
superior to hilgengreiners, lateral to perkins
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
From 6-18 months, DDH treatment consists of
perform hip arthrography, percutaneous adductor tenotomy, closed reduction, and spica casting.
After walking age up to age 6-8, DDH treatment consists of
open reduction
Unilateral DDH after age 8
leave it alone.
after age 6, DDH unilateral
consider osteotomy (until age 8…)
after age 4 this isn’t an option for treatment of DDH anymore
femoral osteotomy not successful that late
bilateral DDH after age 6
leave it alone
filling defect on arthrogram of unsuccessful DDH reduction attempt
superior limbus
this position can result in femoral nerve palsy in pavlik treatment of DDH
excessive flexion
this position can result in femoral head osteonecrosis in pavlik treatment of DDH
excessive abduction
approach for open reduction of DDH
anterior (less risk of damage to MFCA)
these pelvic osteotomies require periacetabular metaplasia for success
Chiari, shelf
this pelvic osteotomy leaves the posterior column intact
PAO
triangular ossification defect inferomedial femoral neck
congenital coxa vara
treatment of congenital coxa vara
depends on the Hilgengreiner-Epiphyseal angle: 60*.
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)
treatment regimen for Perthes
NSAIDs, traction, protected weightbearing, maintain ROM
Herring proposed surgery for these Perthes pts
B/C and C pts older than 8
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
SCFE occurs here
weakness of the perichondrial ring and slippage through the hypertrophic zone of the growth plate
ROM exam finding in SCFE
obligate ER with flexion
Difference in stable vs unstable SCFE with respect to AVN rates
No pts with stable develop AVN; Half of those with unstable slips do
Indications for prophylactic SCFE pinning
Endocrinopathies,
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
threshold for amputation in femoral deficiency
if the femur is less than 50% of the contralateral side
Indication for CT in LLD
knee flexion contracture
rough guide for length per year in mm in the leg
5-9-6-3, from prox femur to distal tibia
LLD less than 2 cm
lifts, non-op
LLD more than 5 cm
lengthening
ESR value in transient synovitis
typically less than 20mm/hr
Utility of CRP in peds infections
should decline within 48-72 hrs of treatment; if not change the treatment
indications for operative mgmt of peds osteomyelitis
failure to respond to abx, frank pus (MRI or aspiration), or sequestered abscess
when does periosteal new bone form in peds osteomyelitis
5-7 days
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
This pathogen does not need surgical drainage if it causes septic arthritis
Neisseria, can be treated with penicillin (need to verify…)
metaphyseal beaking
infantile blounts
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
this amount of genu valgum is gon’ be foine
up to 15* from 2-6 yrs
indications for surgery in genu valgum
more than 10cm between medial mals, more than 15* valgus, and only if older than 10 yrs
posteromedial tibial bowing
physiologic, intrauterine packaging. Commonly associated with calcaneovalgus feet and LLD
anteromedial tibial bowing
associated with fibular hemimelia, equinovarus, tarsal coalition, and LLD
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.
initial treatment for tibial bowing associated with NF
anterolateral = total contact brace
only long bone deficiency with a known inheritance pattern
tibial hemimelia, autosomal dominant
other deformity often present with tibial hemimelia
lobster claw hand
most frequent location OCD
lateral aspect of MFC
indications for OCD lesions
loose or little growth remaining
indications for discoid meniscus
only if symptomatic and torn: saucerization
clinical features of clubfoot
forefoot adductus and supination, combined with hindfoot varus and equinus
radiographic appearance of talus / calcaneus in clubfoot
parallel
CAVE in clubfoot Ponseti method
order of correction, cavus, adductus, varus, then equinus.
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
possible gene cause for clubfoot
PITX-1
treatments for clubfoot after ponseti casting
achille’s tenotomy at 6-8 weeks, braces for 2-4 years
clinical appearance of metatarsus adductus
heel bisector line that hits lateral to the 2nd webspace
treatment for metatarsus adductus
supple feet can be stretched, stiff feet can be casted
treatment for skewfoot
non-operative treatment does not work
test in pes cavus
besides full neuro workup, a Coleman block test tells you whether hindfoot corrects or not
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.
this is not the answer in pes cavus that does not correct with Coleman block testing
Avoid triple arthrodesis; use calcaneal slide instead
irreducible dorsal dislocation of the navicular on the talus, and fixed equinus hindfoot
CVT
radiographic findings in CVT
long axis of the talus hits posterior to the metatarsal axis
irregular middle facet on Harris heel view
talocalcaneal coalition
most common tarsal coalition in children 10-12
calcaneonavicular
most common tarsal coalition in children 12-14
subtalar
surgical treatment for subtalar coalition
only after failing non-op, if there is 50%, subtalar fusion (verify this with foot talk)
juvenile bunions
don’t operate unless skeletally mature
radiographs in this condition mimic CVT
flexible pes planus, except a plantar-flexed view shows talar axis passing above the metatarsal cuneiform axis
this view is helpful in accessory navicular
external oblique
threshold for spine surgery in OI
treated at 35* if crappy bone, with PSF.
