Pediatrics Flashcards
Age at elbow ossification
CRITOE
Capitellum (1) Radial head (3) Internal (medial) epicondyle (5) Trochlea (7) Olecranon (9) External (lateral) epicondyle (11)
What direction does a transphyseal distal humerus fracture typically displace?
posteromedial
radiocapitellar line is preserved
Tx of transphyseal separation of distal humerus
CRPP
How to differentiate between transphyseal separation distal humerus and elbow dislocation
Arthrogram
In elbow dislocation, the radiocapitellar line is disrupted
Most common femur fracture associated with abuse
transverse
Most common and second most common cause of death in children
1st - accident
2nd - abuse
The greatest skeletal linear growth occurs during…
the first year of life.
Best measurement of LLD if there is joint contracture
CT scanography
Meleneus method
girl reaches skeletal maturity at 14 years, boys at 16 years
Treatment of LLD for projected length
< 2 cm: non-op
2-5 cm: epiphysiodesis
> 5 cm: limb lengthening
Advantage of dome osteotomy
provides most bone contact with the least amount of translation and shortening
Partial physeal arrest results in…
angular deformity while complete results in LLD.
When to treat physeal arrest with bar resection and interposition
physeal bar < 50% and > 2 yrs or > 2 cm growth remaining
otherwise, complete epiphysiodesis and address LLD PRN
Cerebral palsy leads to…
static encephalopathy before 2 years old.
How to treat CP medically
baclofen activates GABA receptors –> inhibited action potentials
Best predictor for ability to ambulate in CP
sitting by age 2
Best predictor for functional improvement after surgery for UE deformity in CP
voluntary control of motion
In CP, NM hip dysplasia is…
with acetabular deficiency located posterosuperior.
***The migration index correlates with GMFCS level.
Treatment of CP pt w/ NM hip dysplasia (generally)
< 4 yrs: adductor and psoas release
> 4 yrs:
- functioning child: dega and proximfal femoral osteotomy
- non-functioning (GMFCS V): femoral head resection (+/- PFO)
Lyme caused by…
borrelia burgdorferi via deer tick (ixodes).
Lyme symptoms
erythema migrans
CN7 palsy
carditis
encephalopathy
Tx of lyme if no neuro or cardiac sx
doxycycline (not in kids < 8 yrs) or amoxicillin for 28 days
***if neuro or cardiac sx, then needs IV ceftriaxone, cefotaxime or PCN G
Timing of prosthesis fitting for congenital UE amp
6 months (sitting)
Timing of prosthesis fitting for congenital LE amp
12 months (walking)
Rett syndrome
- ataxia, hypotonia, chorea, NM scoliosis
- X-linked dominant (male fetuses die in utero)
Treatment of radial neck fx
< 30 degrees angulation: cast w/o reduction
> 30 degrees: closed reduction and cast (if CR fails –> perc vs open reduction and pinning)
Nursemaid’s elbow
radial head subluxation with interposition of annular ligament in the radiocapitellar joint
Treatment of nursemaid’s elbow
reduce by supinating and flexing, no immobilization
To assess rotation of BBFF, check….
AP xray. Radial styloid and biceps tuberosity should be 180 degrees.
Lateral xray. ulnar styloid and coronoid should be 180 degrees.
Distal 1/3 BBFF or DRF in kids can be treated with…
SAC.
BBFF displaced should be treated with…
reduction and immobilization with thumb pointing away from apex of fx.
Apex volar fx (supination injury): pronate forearm
Apex dorsal fx (pronation injury): supinate forearm
Usual reason for pink, pulseless hand after SCH CRPP
brachial artery spasm
Crossed pins for SCH are…
biomechanically more stable***.
What structures prevent reduction in SCH fx?
extension type: brachialis, median nerve, brachial artery
flexion type: ulnar nerve
Best view to evaluate lateral condyle fracture
internal oblique
Treatment of lateral condyle fx
CRPP if > 2 cm of displacement
**Vascular supply comes posteriorly so avoid posterior dissection to prevent lateral condyle AVN
Cubitus valgus can present with…
tardy ulnar nerve palsy.
Supracondylar osteotomy if needed.
How to prevent lateral spurring with lateral condyle fractures
anatomic reduction
Status of LCL in lateral condyle fractures
intact, attached to lateral condyle fragment proximally and radial neck distally
Best view to determine displacement of a medial humeral epicondyle fracture
distal humeral axial view
Pediatric trigger thumb
Notta’s node (thickened flexor tendon nodule)
Fixed deformity > 1 yr of age: release A1 pulley
**be careful of radial digital nerve
Pediatric trigger finger
in addition to A1 pulley release, may need to release one or more FDS slips
Most common osbstetric brachial plexus birth palsy
upper trunk (Erb’s) palsy C5-C6
**Has a better prognosis because of preserved hand function
In Erb’s palsy, treatment should include…
parents performing passive stretching with emphasis on shoulder elevation, abduction and ER.
Treatment of brachial plexus palsy with elbow flexion contracture
serial casting
Late presentation of a brachial plexus palsy will show…
IR contracture, posterior glenohumeral dysplasia and dislocation
If > 5 yrs old, perform proximal humerus derotation osteotomy to improve ER.
Sprengel deformity is thought to be due to…
an embryonic vascular interruption (like poland syndrome).
Sprengel deformity is associated with…
omovertebral body, klippel-feil syndrome and congential scoliosis.
What motion is most limited in a sprengel deformity?
shoulder abduction
The psoas is intervated by…
the lumbar plexus.
The iliacus is innervated by…
the femoral nerve.
Injury to the greater trochanter apophysis leads to…
coxa valga ( > 135 degrees).
Overgrowth of the greater troch apophysis leads to…
coxa vara (< 120 degrees).
Most common complication after injury to the pediatric proximal femur is….
AVN (injury to MFCA).
What vessels are injured with piriformis entry IMN in a kid?
lateral epiphyseal vessels (deep branches of MFCA).
In a pediatric distal femur fracture, if pins need to cross the physis, use….
smooth k-wires.
Treatment of tibial eminence fracture
Type I (nondisplaced) --> immobilize with knee in extension Type II (intact posterior hinge) --> closed reduction Type III (displaced) --> open vs scope reduction and fixation
Premature closure of the tibial tubercle apophysis can lead to…
recurvatum deformity.
In a cozen phenomenon, the valgus deformity will…
generally resolve but the affected limb will be longer.
Tillaux fracture
SH III ankle fracture with avulsion of AITFL.
*ER injury
**obtain CT to evaluate step off
Triplane fracture
SH IV –> SH II in lateral view and SH III in AP view
*obtain CT scan
Closure of the distal tibial physis
Central physis is first to close while anterolateral is last (which is why tillaux and triplanes affect the anterolateral region).
Can treat pediatric distal tibial fractures with closed reduction if…
there is < 2 mm of articular stepoff. Need to internally rotate foot to reduce typically.
In a SHII distal tibia fracture, what may prevent successful closed reduction?
entrapment of torn anterior periosteum
Cast ankle in…
neutral to resting plantarflexion (37 degrees) to prevent compartment syndrome.
Risk factors for development of DDH (4)
firstborn, female, breech, family history
Barlow and Ortolani
Barlow dislocations, Ortolani reduces
In DDH, the acetabular deficiency is…
anterolateral.
The alpha angle determines…
the degree of horizontalization of acetabular sourcil.
Normal is > 60 degrees.
The beta angle measures…
displacement of the hip abductor muscles.
Normal is < 55 degrees.
If a hip is still dislocated after 3 weeks of Pavlik harness, then…
switch to semirigid abduction orthosis.
Treatment of femoral nerve palsy in harness
discontinue harness and observe
Hyperabduction in pavlik harness leads to…
impingement of posterosuperior retinacular branch fo the MFCA leading to AVN.
Treatment of DDH at 6-18 months if non-op fails
closed reduction and spica casting
Blocks to closed reduction in DDH
inverted labrum, inverted limbus, transverse acetabular ligament, capsule, pulvinar, ligamentum teres
Treatment of DDH > 18 months old
open reduction +/- osteotomy and spica casting
DDH is associated with…
femoral antersion and coxa valga (needs VDRO)
Open approach to DDH is typically…
anterior (Smith-Pete) to minimize risk to MFCA.
Treatment of residual acetabular dysplasia with open triradiate
Pemberton osteotomy or Salter
Angles indicating adult hip dysplasia
LCEA < 20 or Tonnis angle > 10
Treatment of Legg-Calve-Perthes in a young child
activity modification and symptomatic treatment
Best prognostic factor for Legg-Calve-Perthes
age less than 6
Treatment of Legg-Calve-Perthes in 8-11 year old
containment surgery (femoral or pelvic osteotomy) for AVN
If there appears to be bilateral legg-calve-perthes, think…
multiple eiphyseal dysplasia (MED)
Tx of congenital knee dislocation
if knee can be passively flexed to neutral, treat with serial casting followed by bracing at bedside
if not, open reduction (quad lengthening, soft tissue release).
Congenital knee dislocation is associated with…
myelomeningecele, arthrogryposis, DDH, clubfoot
If have congenital hip and knee dislcations, which to treat first
knee
Congenital longitudinal deficiency includes…
proximal femoral focal deficiency, fibular hemimelia, ACL deficiency, and tarsal coalition
Which gene regulates limb bud formation
sonic hedge hog
Fibular hemimelia is associated with…
absent lateral rays and equinovalugs foot
Treatment of fibular hemimelia
w/ functional, plantigrade foot –> sx to address LLD
w/ non-functional foot –> syme amp
In SCFE, what is the direction of displacement?
metaphysis slips anterior and superior relative to epiphysis
What will MRI of SCFE show?
metaphyseal edema
What zone of the growth plate does a SCFE involve?
hypertrophic zone
What does SCFE have referred knee pain?
medial obturator nerve
Acute vs chronic SCFE
less or more than 3 weeks
In situ fusion of a SCFE should be…
proximal and anterior (compared to CRPP for adults).
Contralateral fixation of contralateral hip in SCFE if…
< 10 years old or has endocrine disorder.
Placement of screw to prevent impingement in SCFE
should be lateral to the intertrochanteric line to prevent screw impingement w/ acetabulum/labrum during hip flexion
Maximum physiologic genu valgum occurs between…
ages 2-4.
When to consider distal femoral medial plate hemiepiphysiodesis for genu valgum
> 7 years old w/ valgus > 12 degrees
Hueter-Volkmann principle
compression across growth plate slows growth
If a patient with genu valgum has a large deformity with inadequate growth remaining for hemiepiphysiodesis, treat with…
distal femoral varus osteotomy.
Posteromedial tibial bowing is…
physiologic. Treat with observation.
* May have LLD.
**associated with calcaneovalgus.
Anterolateral tibial bowing is associated with…
NF1.
Tx of anterolateral tibial bowing
no fx or pseudarthrosis: total contact orthosis
bowing w/ acute fx: LLC
pseudarthrosis: surgical fixation w/ bone graft
anteromedial tibial bowing is associated with…
fibular hemimelia
Normal progression of knee alignment
physiologic genu varum until <2 years old –> maximum genu valgum at 3 years old –> physiologic genu valgum at 7 years old
Infantile Blounts is seen in…
kids 2-5 years old.
Mild infantile blount’s treatment (stage I&II) for kids less than 3 years old
valgus-inducing KAFO brace (unload medial compartment)
Treatment of moderate to severe infantile blounts or in kids > 3 years old
proximal tibia/fibula valgus osteotomy.
**Overcorrect 10-15 degrees of valgus
Adolescent blounts is seen in…
kids over 10 years old and is usually unilateral.
Treatment of adolescent blounts
small deformity w/ adequate remaining growth: lateral tibia/fibula hemiepiphysiodesis.
large deformity, skeletally mature: proximal tibia/fibula osteotomy
Congenital vertical talus
rigid rocker bottom/flatfoot deformity due to dorsal dislocation of the navicular on the talus
Plantarflexion lateral xray will reveal…
oblique talus: talonavicular joint is reduced
CVT: persistent dorsal dislocation of the navicular
CVT will have a meary angle….
> 20.
Treatment of CVT
reverse ponseti casting followed by surgery at 6-12 months (soft tissue releases, reduction of TN joint, pinning)
*refractory cases –> talectomy, triple arthrodesis
Foot progression angle determines…
in-toeing (negative value) or out-toeing (positive value)
3 common causes of in-toeing
- metatarsus adductus (infant)
- internal tibial torsion (toddler)
- femoral anteversion (childhood)
Dx of internal tibial torsion
internal thigh foot progression angle (negative value)
Dx of external tibial torsion
external thigh foot progression angle (positive value)
Femoral anteversion is associated with…
packaging disorders (DDH, metatarsus adductus, congenital muscular torticollis).
**Increased hip IR
2 common causes of out-toeing
- external rotation contracture of hips
2. external tibial torsion
Treatment for recurrent clubfoot after casting is…
recasting.
Extensive soft tissue release for clubfoot is associated with…
poor long term foot function.
Treatment of dynamic supination following ponseti casting
anterior tibialis transfer to lateral cuneiform
Treatment of dorsal bunion following ponseti casting
(due to dorsiflexed first MT)
FHL lengthening and FHB flexor to extensor transfer
Charcot marie tooth genetics
autosomal dominant usually
duplication of peripheral myelin protein 22 (PMP22) on chromosome 17
Charcot Marie Tooth Characteristics
cavovarus foot, claw toes/hammertoes
**intrinsic foot muscles (longest axons) are first to be weakened
OI genetics
AD and AR forms, COL1A1/COL1A2 –> glycine substitution in procollagen –> impaired cross-linking of colalgen
OI traits
- congenital anterolateral radial head dislocations
- olecranon avulsion fx
- blue sclera
- basilar invagination –> myelopathy
- dentinogenesis imperfecta
Long term use of bisphosphonates in OI will show…
parallel metaphyseal bands on xrays
Fix fractures in OI with…
intramedullary (load-sharing) constructs
Achondroplasia genetics
AD
gain of function mutation in FGF3R –> inhibits chondrocytes in the zone of proliferation thus impairing endochondral bone formation
Risk factor for achondroplasia
advanced parental age
Thoracolumbar kyphosis in achondroplasia will…
likely resolve at walking age.
Achondroplasia traits
- lumbar spinal stenosis (2/2 short pedicles adn decreased interpedicular distance)
- normal intelligence
- trident hands
- stenosis of foramen magnum –> central apnea
- champagne glass pelvis
- genu varum
What type of dwarfism is achondroplasia?
rhizomelic dwarfism (disproportionate limbs, arms shorter than forearms, thighs shorter than legs)
Osteopetrosis
marble bone disease due to osteoclast dysfunction
Osteopetrosis is linked to…
a defect in TCIRG1 gene which encodes a subunit of the proton pump in osteoclasts.
Impaired osteoclast carbonic anhydrase activity –> cannot acidify Howship lacunae for bone resorption.
Traits of osteopetrosis
- cranial nerve palsies (esp. optic nerve)
- pancytopenia 2/2 encroachment of bone marrow
- erlenmeyer flask femur
- rugger jersey spine
Neurofibromatosis I genetics
AD NF1 gene (neurofibromin)
Traits of NF1
- smooth cafe au lait spots
- optic glioma
- axillary freckling
- Lisch nodules (iris hamartomas)
- dystrophic scoliosis
- hemihypertrophy
- anterolateral tibial bowing
- pseudarthrosis of tibia/radius/ulna
NF2 has…
vestibular schwannomas.
Neurofibromas may transform into…
malignant peripheral nerve sheath tumors.
**malignant transformation is associated with increased uptake on PET-CT.
Larsen syndrome is…
bilateral congenital hip, knee and elbow dislocations (specifically radial head); clubfoot, cervical kyphosis (*Need C-spine imaging).
Larsen syndrome is due to…
a mutation in filamin B.
Freidreich’s ataxia genetics
AR
GAA repeat mutation in frataxin gene –> absence of frataxin leads to spinocerebellar degeneration (primarily posterior columns of the spinal cord)
Traits of Freidreich’s ataxia
- cavovarus foot
- ataxia
- areflexia
- scoliosis
Marfan syndrome genetics
AD
fibrillin-1 gene (FBN-1)
Traits of Marfan
- dolichoostenomelia
- arachnodactyly
- scoliosis
- protrusio acetabuli
- dural ectasia
- pectus excavatum
- aortic root dilation
- mitral valve prolapse
- superior lens dislocation
Muscular dystrophy genetics
X-linked recessive
Duchenne: absent dystrophin
Becker: decreased dystrophin
Muscular dystrophy facts
- Gower’s sign
- elevated CPK
- corticosteroid slows progression
Treatment of scoliosis in muscular dystrophy
early posterior spinal fusion (20-30 degrees curve) to prevent restrictive lung disease.
Multiple epiphyseal dysplasia (MED) genetics
usually AD
Cartilage oligometric matrix protein (COMP) mutation –> type II collagen dysfunction
MED facts
- impaired endochondral ossification of epiphyses
- disproportionate dwarfism
Spondyloepiphyseal dysplasia (SED) genetics
COL2A1 mutation
AD (congenita)
X-linked recessive (tarda)
SED is associated with…
atlantoaxial instability –> myelopathy
Diastrophic dysplasia genetics
AR DTDST gene (sulfate transport protein)
Diastrophic dysplasia traits
- rhizomelic dwarfism
- cauliflower ears
- hitchhiker’s thumb
- clubfeet
- cervical kyphosis
- cleft palate
Metaphyseal chondrodysplasia
metaphysis is dysplastic while epiphysis is normal
Jansen’s is…
a type of metaphyseal chondrodysplasia. AD. activating mutation of PTHrP leads to delayed maturation in the zone of hypertrophy
Cleidocranial dysplasia genetics
AD
Runx2/Cbfa1 mutation –> impaired intramembranous ossification
What structures are affected in cleidocranial dysplasia?
midline
clavicles, wide pubic symphysis, open fontanels
Campomelic dysplasia
SOX9 mutation
-bowing of long bones with overlying cutaneous dimpling, hypoplastic scapulae
In general, mucopolysaccharidosis (lysosomal storage disorders) show…
proportionate dwarfism and carpal tunnel syndrome.
Morquio disease
AR
galactosamine-6-sulfate sulphatase, beta-galactosidase
keratan sulfate accumulation
odontoid hypoplasia –> atlantoaxial instability, normal intelligence
Hurler disease
AR
alpha-L-iduronidase
dermatan sulfate accumulation
San filippo disease
AR
heparan sulfate accumulation
Hunter disease
X-linked recessive
sulpho-iduronate-suphatase
dermatan/heparan sulfate accumulation
clear corneas
Down syndrome genetics
trisomy 21: type 6 collagen leads to ligamentous laxity y
down syndrome traits
- atlantoaxial and occipitocervical instability
- hip dysplasia due to hypotonia, patellofemoral instability
Beckwith-Wiedemann syndrome
- hemihypertrophy
- macroglossia
- Wilms tumor (requires frequent abdominal/pelvis US and AFP levels)
Ehler-Danlos
- COL5A1/COL5A2 mutation
- get echo to evaluate aortic root dilation
Gaucher disease
sphingollipid lysosomal storage disorder
- AR
- beta-glucocerebrosidase
Congenital muscular torticollis presents with…
palpable mass (SCM), usually painless. Head tilts toward while chin rotates away from affected side.
US of congenital muscular torticollis shows…
fibrosis.
Treatment of congenital muscular torticollis
stretching of SCM. stretch in the direction opposite of the contracture (tilt head away while rotate chin towards affected side).
**SCM lengthening/release if non op fails
Benign paroxysmal torticollis
episodic torticollis associated with pallor, agitation adn ataxia.
**typically resolves by 3 years
Klippel-Feil syndrome
short webbed neck, low hairline, limited neck ROM
Klippel-Feil syndrome is due to…
failure of segmentation or resegmentation of somites
Klippel-Feil syndrome is associated with…
congenital scoliosis and Sprengel deformity.
Atlantoaxial rotatory subluxation is usually associated with…
URI (Grisel’s disease), trauma or head/neck surgery.
Gold standard for diagnosis of atlantoaxial rotatory subluxation is…
dynamic CT.
Treatment of Atlantoaxial rotatory subluxation
< 1 week: soft collar, NSAIDs
> 1 week: head halter traction, muscle relaxant –> hard collar for 3 months
> 4 weeks: halo traction –> halo-vest immobilization for 3 months
**If all this fails –> posterior C1-2 fusion
Pseudosubluxation of cervical spine is usually..
C2 on C3 and often reduces with extension radiograph.
Treatment of acute, symptomatic pediatric spondylolysis/listhesis
TLSO
Exam of pediatric spondylolysis
loss of normal lumbar lordosis, tight hamstrings, pain with single limb standing lumbar extension
if xrays are normal and you suspect spondylolysis, obtain…
SPECT (most sensitive)
Spondylolysis is most prevalent in…
native americans
AIS is seen in…
10-18 year olds. Most commonly female with right thoracic curves.
What risser stage has highest risk of curve progression?
stage 0 (peak growth velocity)
Brace AIS for curves…
less than 25 degrees in skeletally immature.
Wear for > 12.9 hours per day.
How to improve pedicle screw pullout strength
- tap 1 mm smaller than screw diameter
2. place screw via straightforward trajectory (vs anatomic)
smallest pedicles in T spine
T4-T6
smallest pedicle in L spine
L1
Infantile idiopathic scoliosis
less than 3 years old
typically male and left thoracic curve
treat with serial casting
What is a predictor of curve progression in infantile idiopathic scoliosis?
rib vertebral angle difference (RVAD) > 20 degrees
Congenital scoliosis can be associated with…
VACTERL. Remember to obtain renal US and echo.
Treatment of congenital scoliosis 2/2 hemivertebra
vertebral excision and short segment fusion
What type of congenital scoliosis finding has the lowest risk for curve progression?
block vertebra
Neuromuscular scoliosis is associated with…
cerebral palsy, SMA, muscular dystrophy, spina bifida
**Treat with early surgery (Posterior instrumented fusion). Extend fusion to include pelvis.
Myelodysplasia
folate deficiency, AFP
In patients with myelomeningecele, fracture presents like…
infxn w/ swelling, redness and elevated ESR/CRP.
Latex hypersensitivity is…
a type I hypersensitivity (IgE mediated).
The neurocentral synchondrosis (NCS) closes first in the….
cervical spine followed by lumbar followed by thoracic.
Risk factor for caudal regression syndrome (sacral agenesis)
maternal diabetes
Spinal muscular atrophy is also known as….
Werdnig-Hoffman disease.
Etiology of SMA
death of alpha motor neurons in anterior horn –> progressive motor weakness (proximal to distal)
Genetics of SMA
autosomal recessive
mutation of the survival motor neuron 1 (SMN1) gene
*Type I is more severe than Type II.
Traits of SMA
- NM scoliosis
- NM hip dislocation
- tongue fasciculations
- absent DTR
