Peds Flashcards
in extension SCH frx what denotes more instability
medial commiution leading to varus
WB in titanium elastic nails for femur frx
allowed
what exacerbates sx of a peds spondy
extension
are oblique xr needed to dx a spondy
NO - lateral is enough
highest risk group for spondy slip progression
just before teen growth spurt, general reduced risk with age
OCD imaging of choice
MRI; not arthrogram; never XR
tx of peds discitis with vt osteo
IV Abx - no I&D needed
highest incidence of peds spondy in what population
Native americans
UBC tx in prox hum
generally even with frx first tx is observation - if it remains painful or cortex does NOT thicken after frx healing then C&G&BG
tx of suspected Lyme Septic arthritis
aspiration, lyme serology and oral amoxicillin 30 days
UBC by skeletal maturity
they typically resolve
what percent of peds spondy develop to needing surgery
less than 5%
how to diagnose and tx a neonatal elbow injury
get arthrogram - generally Transphyseal and need to visualize the distal humerus
achondroplasia features
lumbar stenosis, lordosis; short pedicles, post radial head dislocation, frontal bossing, genu varum
achondroplasia genetics
FGFR 3, gly-arg substitute slows prolif zone, Auto dom but 90% sporadic
SED genetics
type 2 collagen COL2A1 gene - auto dom
SED features
short trunk and limbs, abnormal epiphyses INCLUDING spine; odontoid hypoplasia and resultant atlanto axial instability; coxa vara, DDH, hearing loss, retinal detachment
what is Kniest dysplasia
same gene as SED (COL2A1); auto dom. Joint contractures, dumbell femurs, respiratory problems, cleft palate, kyphoscoliosis; retinal detachment and hearing loss.
Cleidocranial dysplasia genetics
CBFA1 - transcription factor that activates osteoblast differentiation auto dominant, affects intramembranous ossification
features of cleidocranial dysplasia
clavicles absent, delayed skull closure, frontal bossing, coxa vara (osteotomy for > 100deg); delayed ossification of pubis; genu valgum; short middle phalanx of 3-5th finger
Nail patella syndrome genetics
lin homeobox gene transcription factor; autosomal DOM. - also in eyes and kidneys
features of nail patella
aplasia or hypoplasia of patella/condyles; iliac horns; post dislocation of radial head; 30% get renal fx and glaucoma as adults
diastrophic dysplasia genetics
sulfate transporter gene - affects proteoglycan sulfate groups in cartilage - auto REC.
features of diastrophic dysplasia
short, rhizomelic, cervical kyphosis and kyphoscoliosis; hitchiker thumb; cauliflower ears; rigid club feet; skew foot; severe OA joint contractures
mucopolysacchridoses genetics
all are auto recessive except Hunters (x-link recessive)
Metaphyseal Dysplasia(MD) genetics
Schmid type defect in collagen X (COL10A1); Jansen type - PTHreceptor defect which regulates chondrocyte differentiation; McKusick type worse type - affects ribosome nucelic acid component of mitochondria
MD features schmid
mild; coxa vara, genu valgum
MD features jansen
short, squat stature, hyper Ca; bulbous metaphyseal expansion of long bones; extremity malalignment
MD McKusick type
C1-2 instability; hypoplastic cartilage; immunocomp; malignancies, thin hair, intestinal malabsorption/megacolon; ankle deformities due to fibular overgrowth; ligamentous laxity; genu varum and pectus abnormalities
pseudoachondroplasia genetics
COMP gene on C19 - extracellular matrix glycoprotein in cartilage - Auto Dom
pseudoachondroplasia features
DDH, early OA, scoliosis; norma face, c1-2 instability due to odontoid dyspasia; metaphyseal flaring
multiple epiphyseal dysplasia (MED) genetics
COMP, COL 9A2, COL9A3, for collagen 9 ; links collagen 2 to cartilage- auto dom.
MED features
short; does not involv spine; genu vulgum; hip osteoNec; double layer patella
Ellis van crevel EVCgenetics
mutation in EVC gene; auto recessive
Ellis van crevel features
acromesomelic shortening (middle and distal imbs); POST axial polydactyly’ genu valgum; 60% congenital heart disease; medial iliac spikes; fusion of carpals - capitate and hamate
diaphyseal dysplasia also called
camurati-engelemen - symmetric thick cortex of tibia, femur, humerus - tx with NSAIDS - watch for LLD
Leri Weil dyschondrosteosis genetics
SHOX gene; auto dom. On sex chromosomes
Leri Weil features
mild short stature mesomelic shortening; madelung deformity
what does CFBA code for
TF for osteocalcin
MED height
patients are NORMAL height EARLY on but then develop their short stature later
SED types
AD , COL2A; milder version is rarer shows up later 8-10y and is X-recessive; BOTH have C-spine instability
marfan genetics
Fibrillin 1-FBN 1 n C15; auto dom.
marfan features
60 percent have scoliosis hard to brace; need MRI prior to spinal fusion due to high rate of dural ectasia
Ehlers danlos genetics
about half are classic COL5A1 or 2 mutation; auto dom. For collagen 5; type 6 is auto recessive has severe kyphoscoliosis; type 4 is COL3A1 and auto dom - has the GI and artieral ruptures
additional workup is needed for Ehlers Danlos
echo for aortic root dilation
types of JRA
systemic type is stills disease (least common, worst); olig (4 joints or less)is most common; poly (5+ joints)
features of systemic JRA (stills disease)
hepatosplenomegaly, lymphadenopathy, pericarditis, anemia; ages 5-10 equal genders; poor prognosis
features of OLIGO JRA
peak age is 2-3; 4 joints or less; most common and more in girls; generally good long term prognosis ; uveitis is possible. Limp that imrpoves; LLD is possible, check optho exam
hypophosphatemic rickets
x-linked dominant; impaired renal absoprtion of phosphate
hypophosphatemic rickets lab values
LOW Phos; NORMAL Ca, PTH, Vit D. HIGH ALK; tx with high dose phos and vit D
Nutritional rickets values
LOW vit D Ca and Phos; HIGH PTH and ALP; tx with vit D
Vit D Dependent Rickets Type 1
cant make 1,25. LOW ca, phos, Vit D, normal 25 hydroxy D; HIGH ALP and PTH ; tx with 1,25 vit d replacement
vit D dependent rickets 2
bad receptor; LOW ca, phos. VERY HIGH vit D1,25; HIGH ALP; PTH - tx with hi dose vit 1,25 vit D and Ca
hypophosphostasia
cant make ALP; high Ca and phos, low ALP, normal PTH, vit D
patholoy in Gauchers
Auto Rec. beta glucosidase doesn_t work; lipids build up; infantile type is lethal. Can be cured with BMTx early on; earlenmeyer flask deformity
what is def of spastic CP
rigid and increased tone with rapid stretching - most benefit from ortho surgeries
Hemi vs Diplegic CP
Hemi is one half; Di is Lower
normal stance swing ratio
62% stance 38% swing
gmfcs 2,3,4
2 has some trouble with uneven ground; needs railing for stairs but NO assistive device; 3 needs a device, but can get around; 4 needs wheel mobility most of the time
gmfcs1
can do everything including runnig, jumping - BUT balance speed and cooridnation are impaired
AFO types for CP
solid ankle improve walking speed and stride length in most CP patients; hinged help prevent equinus; floor reaction help prevent crouch gait due to excess dorsiflexion
what to check before releaseing eqquinus in CP
is the contracture from the HIP/KNEE? Leading to crouch gait and eqiunus might be compensatory
scoliosis rates in CP
in hemiplegic and dipegic is 20%; quad is 60-80% and bed ridden is 100%
quadriplegic CP scoliosis after skeletal maturity
continues to progress
incidence of hip subluxation in CP
in quads up to 50% - not the same as DDH
management of CP scoli curves
age over10 and > 40-50 degrees interfering with sitting; if 90 deg or more, need to do ant release; QoL, balance and cosmesis improves with sugery
can botox prevent progression of hip migration
nope
tx of hips in CP
if < 8 and < 60% subluxation- soft tissue procedures; if > 60% or > 8y and 40% the femoral and pelvic bony work
distal recturs femoris transfer used for
swvere crouch gait, but can worsen it in GMFCS 3-4
mc cause of crouch gait
spastic hamstring
severe crouch gait fixed with
either guided growth OR closing wedge anterior osteotomy of distal femur - extension
pes planovalgus in CP
best outcoms with calc slide are before age 6; after you need to do mid foot work
fractures in CP
higher in NWB patients; consider giving IV pamidronate to those with Z score < 2
myelomingocele pathogenesis
failure to close neural tube elements at 3-4 weeks
meningocele vs myelomeningocele
meningocelee has little to no impariment bc neural elements stay in side canal ; MM-cele has spinal elements sticking out
prenatal dx of Meningocele/MM-cele
AFP in serum 60-90% accurate or via US or via Amniocentesis
RF for spina bifida
low folic acide, previous birth with SB; maternal DM; in utero exposure to valproic acid and carbamazepime
mm-cele at L3 vs 4
significant difference in ambulation; need quads and hamstrings for ambulation if these are present then 80% are comm amb with K/AFO
issues with MM-cele in the spine
tethering leading to scoliosis; syrinx; shunt problems (for treating hydrocephalus)
high vs low sacral mm-cele
low has intact GSC; high does NOT have gsc and has a gluteal lurch or pelvic obliquity
mmcele with dislocated hip
general do NOT reduce
tx of mmcele foot deformities
tenotomy over tendon transfers - better to have a flail braceable foot
sx of duchenne
decreased ambulation by 8-9; wheelchair by 12; and progressive solciosis/respiratory disease and death by second decade
CPK level of duchenne
usually diagnostic and 100x normal
EMG of duchenne
myopathic, decr amplitude, short duration of polyphasic motor
biopsy of DMD
fibrofatty replacement of muscle
weakness in duchenee
proximal muscles first
Duchenne and anesthesia
risk for malignant hyperthermia -pre-tx with dantrolene
when to treat DMD spine
after 20 deg
SMA type 1 acute
severe, death at age2
SMA type 1 chronic
delayed onset at 6-18months; weaker in LOWER extremities; hip dislocations at 60% and joint contractures common
SMA 3 - kugelberg
onset after 18 months; wheelchair by late adulthood, normal lifespan; weaker proximal muscles
SMA 4-
adult onset; moderate proximal weakness
genetics of SMA
ALL SMA types are missing SMA 1 gene (on c5; severity based on how much SMA2 gene is available; progressive loss of alpha motor neurons in anterior horn of cord
when to fuse SMA spine
generally after age 10 o greater than 40 deg; can use VEPTER rods
when to fix SMA scoliosis to pelvis
if spin is > 40 deg and FVC > 40%
SMA joint contractures
common but generally not treaated even if hip and knee ones exceed 30-40
charcot marie tooth type 1
type 1 most common; peripheral mylein degen; leads to decre motor NCV in upper limb onset in first-second decade; PMP 22 gene on c17;
charcot marie tooth type 2
second decade; auto dom. Myelin is in tact but wallerian degen occurs decreased motor AND sensory; EMG has normal or long action potential
charcot marie tooth type 3
severe; peripheral nerve demyelination; Auto recessive; presents in infacncy; enlarged peripheral nerves; ataxia; MPZ gene;
cavus foot in CMT tx
typicall p longus is over powering so transfer to p brevis and move EHL over to 1st MT to help tib ant
friedrichs ataxia timeline
onset by age 25; death from cardiac complications by 40-50s; affects UE NCV
genetic repeat in friedrichs ataxia
GAA on c-9; frataxin gene; used for cell iron homeostasis
tx of Freidrich ataxia foot
pes cavovarus and is progressive and rigid - bracing does not work - arthrodesis is the main choice
tx of Freidrich ataxia spine
when > 50 deg and DO NOT need to fuse to pelvis; cant use SSEP monitoring
RETT disease
xlinked dominant condition of gray matter of brain - kids start out normal 6-18 months then regress; females die; men get klinefelters - they get scoliosis and need to fuse spine
Klippel Feli c-spine shows
congenital vertebral abnormalities; may see a sprengels asociated as well due to thoracic spinous process tether
what to screen for in Klippel Feli
c-spine films; GU and hearing loss and cardiac
Klippel Feli and sports participation
no contact if massive c-spine fusion or C2 involvement; if below C3 there is 1 or 2 levels involved and noraml neck motion then ok to play
normal age of appearance for femoral head
4-7 months
osteomyelitis after 7 days xray appearance
erosive metaphyseal lesion with loss of cortical integrity is seen generally after 7 days
motion in LCP
restricted passive motion; can present with trendelenberg gait too
renal disease and pediatric hip pain is a scenarior for
renal osteodystrophy affecting proximal femur with physeal widening due to secondary hyper PTH - can lead to SCFE
rate of AVN afer open mod Dunn for SCFE
26-30%
when should you see femoral ossificaiton center after reduction of hip
if not seen by 12 months post reduction it_s a sign of prox femoral growth disturbance and AVN
what deformity is seen with SCFE
varus, flexion; ext rot. Causes a Lost of hip flexion and internal rotation
nerve injuries with SCH frx
AIN with extension is most common; second most common is radial; Flexion type most common Is ulnar nn
order of ossification at elbow
capitellum (1), radial head(4), medial epi (6), trochlea (8), olecranon(10), lateral epi (12)
gene involed in club foot
PITX1-TBX4 it is a SNP
what amonunt of coalition is allowed before resection in foot
generally less than 50% - requires a CT
suspected peds fem neck stress frx - what is first step
MRI - not just activity modification
what is a/w fibular hemimelia
absent ACL and absent rays of the foot
what conditions have MYH3 gene mutation
codes for myosin heavy chain 3; seen in distal arthrogyropsis; sheldon hall and freeman sheldon
sx of Beckwith Weidman
C-11; neonatal hypoglycemia, macroglossia, large organs, hemi hypertrophy and embryonal tumors like Wilms Tumor
tx of mid shaft clavcile in peds with 100 % displacement
sling
dystrophic scoliosis features seen with
NF-1- look for penciled ribs, scalloped vertebrate, severe rotation and short sharp kyphoscoliosis
clubfoot associated with what vascualr anamoly
absent anterior tibial artery
first step in treating a RECURRENT clubfoot
recasting
kingella knee infection rule out
generally need a PCR for RTX protein - common scenario for day care patient
toddler or baby with band on digit
called hair tourniquet syndrome
inheritance of tarsla coalition
auto dom.
which group of genetic conditions has more tarsal coalitions
FGFR related conditions Apert,
sprengels caused by
vascular interruption at subclavian artery
what perecnt of CBC is elevated in osteo and septic OA
25% for osteo; 30-60% for septic joint
CRP and ESR in peds septic joint timeline
generally rises to abnormal by 6 hours; >2 is predictive; ESR takes 3-5 days for peak.
XR Timeline of osteo
4-7 days new periosteal bone; 10-14d, osteolysis; 1-2 weeks metaphyseal rarefaction and intraosseous abscess
kocher criteria
ESR>40; temp over 38.5; inability to bear weight, CRP > 2; and WBC > 12
how to choose MRI for septic joint vs going to OR
if > 3 of these 5 criteria; ag >4; sx > 3d; CRP > 13.8; platelets < 314k, and ANC > 8600
PMN and Leukocyte difference in Transient synovitis vs Septic Hip
TS - 25% PMN; 5-10K cellcount; vs 75% PMN and 50K cell count
peds osteo can mimic
LEUKEMIA! - CBC will show leukopenia or anemia!! But all other labs may look like infection (ESR, CRp)
elevated urine or serum homovanillic acid HVA or vanillylmandelic VMA
metastatic neuroblastoma
diagnosis of CRMO
whole body bone scan or MRI - lab markers can be normal
demographics of CRMO
girls more than boys generally age of onset is around 10
tx of CRMO
scheduled NSAIDS works for upto 80%
Kingella features
Gr-; needs PCR; kids < 4y; 50% of Septic joint under age 2; might present as subacute infection; can use oropharynx swab
most common spot for TB in kids
spine; typically ant 2/3 of vt body; usually in TL jxn, joint invovlement tends to be hip or knee
diagnosis of septic TB in kids
acid fast bacilli on stain
lab values in lyme OA
typically ESR and CRP are elevated but WBC can be normal
tx of lyme
oral abx including doxy, amox, and cefuroxime
medium for growing gonorrhea culture
chocolate blood agar
how soon does septic oA damage cartilage
8 hours
cause of septic joint in peds 2w to 4wks
Group B Strep
shoe puncture infections
stilll more common to get s aureus but pseudomonas is a concern; only 1% become deep infection
tx of shoe puncture deep infection
if deep then most likely pseudomonas
Discitis diagnosis and tx
xray is normal for 2-3 weeks but MAY show loss of sagittal contour; MRI confirms; abx is mainstay - do NOT need biopsy ; typically kids under 5
vertebral osteo
older children more painful on exam than diskitis; MRI again for diagnosi; tx with anti-staph abx
in sickle cell how to rule out infection vs crisi
labs and MRI are simillar and not helpful - need aspirate or Gram stain
risk factors for DDH
12-33% have affected family member; 6% risk if sibling; 12% parents; 36% if sibling AND parent; female, breech, first born, and ANY intrauterine packing condition
packing conditions a/w DDH
metatarsus adductus, muscular torticolis
how do toddlers with dislocated hips present
LORDOSIS
DDH and dislocation rates
1% and .1% for dislocation
when can you use xray for DDH
after 6 months
normal ultrasound angles
alpha should be GREATER than 60; Beta shouild be < 55 - alpha goes DOWN
how much coverage should you see on normal hip ultrasound
at least 50% acetab coverage
normal actab index
less than 25deg by age 12; less than 20 by 2 years
normal latearl center edge angle (seen on AP) for PEDS
at least 20 deg
who gets US at birth for DDH
ONLY if risk factors; otherwise wait to 4-6 weeks
tx od DDH
< 6month - pavlik, 6-18 month open vs closed reduction and spica; >18months
pavlik for DDH
tx for 6-8 weeks with weekly US, if still dislocated by 3-4 weeks discontinue - generally 90% success
risks of pavlik
excess abduction > 60 leads to osteonecrosis or pavlik disease which is deformation of posterosup acetab rim; excess flexion beyond 100 can cause fem nn palsy
how to confirm hip reduction in 6-18 month old
arthrogram with < 5mm of medial dye pooling
max age for surgery for dislocation hip
unliteral 10years; bilatearl 6-8 years
after age 2 dislocated hip what must be done
shorteing femoral osteotomy
is IV abx with clinical infection but neg. MRI
yes, start IV abx in setting of suspected infection and cover for MRSA
peds abx for osteo
always cover for MRSA
what does Harris view show for Talocalc coalition
can show an oblique medial facet, suggestive of fibrous or cartilaginous connection
popliteal cysts are between what two structures
gastroc and semi-M bursa
early infant with hip instability what is next step
apply brace and repeat exam in 2 weeks with ultrasound
TL kyphosis in achondroplasia follows what course
resolves with independent walking
which children get overgrowth after femur frx
ages 2-10
gymnasts wrist
distal radius epiphysiolysese - look for wide distal radius physis - tx with SAC for 6 weeks
flexion SCH requires what extra step
are inhertyl unstable and may require open reduction if medial gapping -due to ulnar nerve entrapment.
what scoliosis curve is ATYPICAL
LEFT THORACIC
Uveitis in JIA symptoms
chronic and asymptomatic can cause permanent vision loss in 20-30%
growing rod vs PSF for scoliosis
usually the cutt off for growing rods is < 10
tx for infantile idiopathic scoliosis
ages 0-3 and if RVAD (rib vertebral angle difference ) is < 20 then observe; afte 20deg then begin serial casting
What type of fusion for SMA patients scoliosis
generally PSF is enough unless curve is > 100 deg.
congen vert talus tx
reverse ponsetii casting followed by achiles tenotomy and bracing
DVT with osteo in peds population is seen with
genrally more common with MRSA and those expressing the panton-valentine leukocidin
sprengle is most common in which condition
Klippel Feli
tx of infantile blounts
tib fib osteotomy before age 4
risk of MRSA infection includes risk of
DVT or PE ; needs chemical DVT prophylaxis
torticolis after virus tx
often requires CT or radiographs to asses if rotatory subluxation of c1-2 - tx would involve traction and then halo vest; if unable to reduce then C1-2 fusion
indicator of NF scoliosis progression
rib pencilling;
dystrophic vs non dystrophic scoliosis
dystrophic is more severe, has higher rates of dural ectasia, faster curve progression; nerve injury etc and cannot be treated like idiopathic scoliosis
features of dystrophic scoliosis
faster curve progression, more kyphosis, more neuro injury, dural extasia, dumbell foramina from NF, scallping, rib pencilling, spindling of the TP, severe apical rotation, severe vertebral wedging,
what determines modulatio of non-dystrophic scolosis to dystrophic scoloisis
age, generally if presenting before age 7 up to 80% can modulate.
best xray for peds medial epicondyle displacement
AXIAL view; 45 deg abducted arm with elbow at 90. xray tube is 25 deg from axis of of hum shaft
what injury is associated with peds tib tubercle frx
ogden 3 is a/w Meniscus tear
persistent drainage after I&D of septic hip -what next
check MRI for prox femur osteo
most common complication of transphyseal distal hum frx
cubital varus due to either AVN of medial epicondyle, malunion, or growth arrest - higher than with SCH
what SH type is seen with distal humerus frx
if < 3 SH 1; if > 3 SH 2
when to switch from pavlik to rigid abduction brace
if hip is unstable at 3-4 weeks
camptodactylly
contractures of the fingers