Pediatrics

Scoliosis- AIS

o Surgical treatment thoracic curves > 50, lumbar >

30o = inc of smaller curves among

male/female, but female ratio on curves > 30 deg 10:1

o rib heads of thor curve should have apical lordosis – if not, then MRI (syringomyelia)

o Brace Milwaukee or Boston with

support for apex T7 and above Boston underarm for apex T8

and below Charleston for TL 25-35 curves

o PSF gold standard for thoracic curves revision as high as 19%

o ASF w/ instrumentation for thoracolumbar curves

o ASF/PSF for skeletal immaturity, < 10 yo, severe curves > 75 deg

ant disc when pt Risser grade 0, large/stiff curves (< 50% flex index)

o SMA syndrome – from hypercorrection of curves in thin people

Tx NGT with IVF, prolongedo Delayed infx – from P. Acnes or Staph

epi (one year later) Tx hdwr removal with 3 wks

IV antibxo Peak velocity 10 cm per year, occurs

just before menses in femaleso MRI for atypical curves, absence thor

lordosis, atypical pain, neuro abn, foot deform, ext rapid curve prog

o Thoracoscopic release/fusion in prone is effective, better tolerated than when pt is lateral

o SSEPs

50% dec in amplitude, inc in latency of 10% concerning

o SRS-22 scores not improved w/ Cobb angle correction

- Infantile 2 mo – 3 yro Left thoracico Male predominanceo 22% had neural axis abn, 80% of these

pt req surgo if rib overlaps vert body at apex of

curve, high risk prog (phase 2 rib) if overlap (phase 1), then must

meas RVADo Rib-vertebral angle difference

Measure of rotation Difference b/w RVA on each

side > 20 deg w/ phase 1 rib, high

risk progressiono < 25 deg curve, RVAD < 20 deg,

observeo 25-35, RVAD > 20 cast/braceo > 35, MRI, surgery

- Skeletal immaturityo Risser 0 o peak height velocity (right afterwards)o menarche (b/w 0-1 is menarche)o Risser 1 = 25% ossification o Risser 2 = 50% ossification of iliac

cresto Risser 3 = 75% ossificationo Risser 4 = 100%

- Juvenile 3-10 yro High incidence of cord abnormalities

Syringomyelia Arnold-Chiari malformation

o Right thoracic MCo 95% have prog curveso Thoracic curves prog more commonlyo All curves > 20 deg should be

evaluated w/ MRI (20% neural axis abn)

Pediatrics

o Asf/psf- Congenital Scoliosis

o Failure of segm MCo Associated anomalies

Gu 25%, cardiac 10%, spinal dysraphism 30%

o Worst prognosis: unilateral bar w/ contralateral hemivertebrae

o Usual treatment ASF/PSF or PSF in situ Hemivertebral resection

Can do in < 5 yo Correct curves < 40 deg

No role for bracingo Obliquity at LS junction caused by

hemivert tx w/ hemivertebrectemy early

before compensatory curve is fixed

o Rib fusions – adverse thor growth known as thor insuff sx Expansion thoracoplasty

indicated – prosth rib distractoro Surgery for any progression

- Congenital kyphosis o highest risk for neurologic

compromiseo Failure of formation MCo May progress to paraplegiao Type I – def of vert body, but post

elements ok surg tx immediate

o Type II – vert bodies fused less progressive, less deform lower risk of paraplegia

o Surg if pt < 3-5 yo, before kyphosis

> 50 deg, PSF ok hypertext cast for 6 mo

if kyphosis > 50 deg, ant release w/ strut graft w/ PSF

NF

- Congenital type curve pattern- Vertebral scalloping, enlarged foramina, short

segmented, sharp curves, kyphoscoliosis, penciling of ribs

o > 3 ribs penciled = significant risk of progression of curve

- Must be ASF/PSF- MRI preop- NF1 has

o Plexiform neurofibromaso Dural ectasia

Spondylolysis- MC L5- Initial: limit activity/brace- Chronic: repair vs. fusion

Spondylolisthesis- Dysplastic – elongation of pars, facet joints

stretched, presents with more back pain (compression of nerve roots)

o more prone to deformity and neuro def- Isthmic – defect, less back pain, facet jt intact- Surgery for grade III, progression of

deformity, fail conservative managemento Psfo Grade I-II in situo III and above may need reduction w/

instrumentation- High slip angle – means more junctional

kyphosis- Pelvic incidence

o obliquity of sacrum to pelvis meas on lateral XX

o fixed angle larger in pt w/ higher-grade deform when compared w/ low-grade slips

- risk of progression greatest during adoles growth spurt, esp w/ dysplastic spondylolysis

- Rf for prog: vert, dome-shaped sacrum, trapezoidal L5, kyphotic slip angle

Pediatrics

- high-risk sports 5x more likely to have unfavorable outcome

- if pars interarticularis is chronic, then brace used until Asx

- direct repair reserved for pt w/ defects at L4 or L3

o L5 tx w/ L5-S1 fusion

Scheuermann’s kyphosis- > 5 deg wedging, 3 consecutive vert- differentiate from post kyphosis w/

hyperextension lat XX – apical region in Sch is inflex

- disc narrowing- end plate irregularity- Schmorl’s nodes- Tx

o 65-80 deg – CTLSOo Ant release/PSF for curve > 80 deg

Klippel Feil- Low hairline- Web neck- Limited cervical ROM- Conservative Tx

AAI- Flex-ex views

o Up to 2.5 mm in adults is nlo Up to 7.5 mm in children is nl

- ADI 4-5 mm is nl- Rotatory subluxation

o Grisel’s dz: retropharyngeal irritation if deformity fixed, less pain,

but torticollis remainso CT scan to document – Tx w/ brace,

then tractiono if sx > 3 mo, then post arthrodesis

Pseudosubluxation- C2/C3 in < 8 yo- Spinal laminar line drawn – Schwischuk’s line

o If intact, then is pseudosubluxation

Spine- post arches fuse by 3-4 yo- neurocentral synchondrosis b/w lat masses and

body fuse @ 7 yo- upper C-spine injuries MC b/w birth and 8 yo

o b/c horizontal facets, large head size, M. weakness, inc neck motion

- Swischuk’s line drawn along post arch of C1-3, should pass w/in 1.5 mm of post arch of C2

- MRI can clear C-spine in intubated, obtunded, uncooperative children

- for dx atlanto-occipital dislocationo Wackenheim line drawn along clivus

and should intersect tangentially the tip of odontoid

o Powers ratio = line from basion to post arch of atlas / line from opisthion to ant arch (> 1.0, or < 0.55 is disruption

o no halo or traction- odontoid fx

o only 50% apposition necessary- rupture of transverse lig is rare (10% of ped C-

spine inj)o avulsion of transv lig to C1 MC

- Halo use: 68% complication rates- SCIWORA cause of paralysis in 20-30%

children w/ inj of SCo 50% have late neuro deteriorationo cause by vasc insult watershed areao < 10 yo more likely to have perm

paralysis (difference in inj type)o tx: immobilization 2-3 mo in C-collar

- disc is stronger than cancellous bone – vert body is 1st structure to fail

- TL fxo kyphosis < 20 deg tx w/

hyperextension casting- Slipped vert apophysis

o fx of vert endplateo MC involves caudal endplate of L4o teenagers

Pediatrics

Myelodysplasia- Risk factors

o Maternal hyperthermia, maternal IDDM, valproic acid, folate def

85% caused by folate def- C-section when dx is known- Level = lowest functioning root- Surgical infx rate 5-25%- Hip dislocations

o Most common L3-L4 levels (unopposed hip flexion, adduction)

o Hip flexion contractures (strong flexors, weak extensors)

- Knee flexion contractureso Correction of 20 deg or more is

indicated- Ober-Yount procedure

o IT band lengthening prox/dist to tx hip abd contracture

- Clubfooto Rigid, poor response to casting

30% have at birtho Req surgery

- vertical talus, calcaneus feet (no GCS complex)

o thigh-foot angle greater than 20 deg needs supramalleolar osteotomy

o valgus foot deformity seen at L45 level deformity

- scoliosiso congenitalo 100% incidence in thoracic levelso most progression before age 15o average curve prog 5 deg/year for

curves > 40 dego need curve < 40, pelvic obliquity < 25

to sit properlyo bracing ineffectiveo surg: ASF/PSFo high pseudarthrosis rateo infx 15-25% (thin skin, then dura

underneath)

- congenital kyphosiso classic in spina bifidao 10-15% of pto causes recurrent skin breakdowno tx: Kyphectemy

Arthrogryposis- multiple congenitally rigid joints- sensation intact- nl intelligence- risk factor: oligohydramnios- limitation of ROM, decreased M. mass- clubfoot: surgery

o initial: soft tissue releaseo later: talectemy, or triple arthrodesis

- teratologic hip dislocation (hip was never in joint)

o pseudoacetabulum at presentation o Pavlik contraindicated (AVN)o Tx open reduction vs. nothing

Open red via medial approach

Larsen’s Syndrome - Multiple joint dislocations- Flattened facies- Scoliosis- Cervical kyphosis

Sacral agenesis- Dimpling over buttocks- Imperforate anus- Kyphosis – lumbar- Knees extended- Hips flexed

Duchenne’s- Sex-linked recessive- Point deletion of dystrophin gene

o Located Xp21 on X chromosomeo Frame shift of all mRNA distal to

deletiono Codes nonsense protein

- Elevated CPK

Pediatrics

- Scoliosiso PSF for curves > 20-25 deg

- Steroids improve M. strength, slow progressive weakening

- Post tib tendon transfer for equinovarus

Becker’s- X-linked transmission- cardiomyopathy

Fascioscapulohumeral MD- Autosomal dominant- nl CPK- scapular winging

Spinal Muscular Atrophy- progressive symmetric M. weakness caused

by loss of ant horn cells- AR- Mutation in survival motor neuron gene on

chrom 5q- Type II – infantile

o M. weakness more advanced in legs than arms

o Hip dislocation occur in 62% Usu painless

- Type III – Kugelberg-Welandero Onset of M. weakness b/w 2-15 yrs

Friedrich’s ataxia- AR- GAA trinucleotide repeat in both copies of

frataxin gene on chrom 9o Frataxin is mitochondrial protein –

involved in iron metabolismo Tx w/ Coenzyme Q

- Spinocerebellar deg dz- Onset < 10 yo- Hypertrophic cardiomyopathy, DM- Death at 25 yrs (cardiac)- Cavus feet- Scoliosis

o Distinguishes it from CMT

CMT- AD, AR, and X-linked- Demyelinating forms that slow nerve cond

velocityo CMT I, III, IV

- Axonal forms that dec compound muscle act pot

o CMT II- Disease of peripheral nervous system- Peripheral myelin protein 22 abnormal

o On chrom 17- Connexin 32 abn in X-linked- Dejerine-Sottas syndrome = HSMN III

o Occurs in infantso Triple arthrodesis contraindicated b/c

feet may lose prot sensation- most affected muscles (weakness)

o Anterior tibialiso Peroneus breviso Intrinsics footo Intrinsics hand – wasting

- Hip dysplasiao Pt more prone to sciatic nerve palsy

after pelvic surg- Scoliosis

o Left thoracic, kyphotic curves- Cavovarus foot

o Mild, flexible Plantar fascia release, 1st MT

dorsal closing wedgeo Rigid

Calcaneal osteotomyo Severe

Triple arthrodesis

CP- Non-progressive, UMN dz- Spastic, dystonic, ataxic, hypotonic- Onset < 2 yo- Botox (botulinum-A toxin)

o Competitive inhibitor at motor endplate – irreversible binder

Pediatrics

o Presynaptic cholinergic receptoro Lasts 3-6 mo

- AFO’so Solid for spastic diplegiao Crouch gait – floor reaction AFO’s

b/c of ankle plantar weakness- Baclofen

o GABA agonisto Side effect is decreasing M. tone

- Toe walkingo Ankle DF > 5-10 deg, then AFOo > 10 deg, then TAL

- Crouch gaito Multiple release (not just heel cord –

would worsen gait)- Stiff Knee gait

o Limited knee flexion in swing phase b/c of rectus spasticity

o EMG w/ rectus firing out of phaseo Consider rectus femoris transfer w/

medial hamstring lengthening Risks of lateral hamstring

lengthening incl weakness leading to knee hypertext during stance phase

- Dorsal rhizotomyo For pt 4-8 yoo Spastic diplegia, walking gait

abnormalityo DR and PT better than PT aloneo Some pt may need more ortho

procedures- Scoliosis

o Spastic quad at highest risko C-shaped curveo Resistant to bracingo Surg indications: progressive

deformity, sitting imbalance, pelvic obliquity

o Controversial b/w ASF/PSF vs. PSFo Need segmental fixationo Must fuse to pelvis

- Hip subluxationo Early tx: adductor/flexor release (< 4

yo)o Later tx: VDRO, pelvic osteotomieso Late complications – adduction

contracture, scissoring gait, dislocationo For a chronic painful dislocation

Abduction osteotomy (tip head away from pelvis)

Proximal femoral resection But can get superior

migration- Equinovalgus foot

o Cause: spastic peroneals, heel cord contracture (Achilles), ligamentous laxity

o Hindfoot valgus (from loose ligaments)

o Tx TAL/calcaneal osteotomy Grice procedure (lateral

column lengthening), w/ TAL- Equinovarus foot

o Cause: over pull of tibialis anterior or post tibialis, tight Achilles

o Tx: TAL + post tib split transfer, or Rancho (split tib ant and TAL)

o SPLATT If post tib tendon out-of-phase

then complete transfer If PTT continuous activity,

then split transfero Try to delay surgeries until at least 6

yoo Surgery before age 8 – 75% of them

fail- Foot

o Hallux valgus deformities – MTP fusion

- Hando Aggressive tx to stretch agonistic

muscle, and strengthen antagonistso FA hyperpronation limits hand fx

Pediatrics

Tx w/ rerouting of pronator teres through interosseous membrane

o Wrist/finger flexion deformity Tx w/ t-x FCU to wrist

extensorso More severely affected pt – muscle

lengtheningso More functional pt – dynamic tendon

transferso Thumb-in-palm contracture

Tx w/ Z-plasty syndactyly release

Care not to overrelease adductor

Fibular Hemimelia- Equinovalgus foot deformities (no ankle

support)- Usu distal deficiency- LLD- Tx:

o Good foot (at least 4 rays), then limb lengthening (5-8 cm) or epiphysiodesis (2-5 cm)

o Bad foot (3 rays or less) Amputation

9-12 months of age

Tibia Hemimelia- Equinovarus foot- AD- Tx, depends on magnitude of deficiency

(BKA vs. AKA)o If quad fx, then save distal parto If no quad, then knee disarticulation

Congential Pseudarthrosis of Tibia- Associated with NF (50%)- Anterolateral bowing- Tx: bracing (w/ no fracture)

o w/ fracture, then IM nail with bone graft

o vasc fibula (but only heals at one end)- NEVER osteotomy!!

Posteromedial bowing- Associated calcaneovalgus foot- Tx: nothing (it spontaneously corrects),

stretching- 2-5 cm LLD at maturity

Blount’s- Infantile

o Prox med tib physiso Onset < 3 yoo Genu varumo Metaphyseal diaphyseal angle > 13

deg If > 20 deg, then worse

outcomeo Ass w/ medial tibial physis growth

arresto Tx: Brace w/ KAFOo Surgery for pt with bar, bracing

failures Best results early < 5 yo Need to over-correct into

valgus- Adolescent’s

o Overweight, African-americano Tx: lateral hemiepiphysiodesis, prox

tib osteotomy

Clubfoot- Hindfoot – equinovarus- Forefoot – supination, adductus- Midfoot – cavus

o Plantar flexion of foreft on hindft- Talus smaller than nl- Talar neck deviated in plant med direction- Can be identified on u/s @ 12 wks gest- Tx: LLC, serial casting w/ 50% success rate

o Surg tx @ 9-10 monthso Ponseti method

Pediatrics

Rotation of foot around fixed talus

Calcaneus rotated laterally/superiorly (not translated)

Order 1. correct cavus

o DF 1st MT against talus

2. correct adductus/varus

3. correct equines final LLC for 3 wks 40% req lat t-x of tib ant b/w

ages 2-5 TAL @ 6-8 wks Bracing

Night-time until 2-4 yo 80-90% success rate

- XX: hindfoot parallelism

Vertical talus- Congenital convex pes valgus- Dorsolateral dislocation of TN jt- DF contracture of foreft on hindft- Rocker bottom foot- Talipes equinovalgus- Ass w/ spina bifida, arthrogryposis, sacral

agenesis- Diff dx: calcaneovalgus deformity

o Spont resolveso Differentiated by flexible midft but DF

contracture of ankle- XX: lateral plantar flexion view: talus not in

line w/ metatarsalso Navicular is dorsally dislocated

- Tx: surg release post med @ 6-9 mo of age, pin talonavicular jt,

o Also, dorsal approach to TN jt, Achilles lengthening – better correct

o Naviculectemy – older childo NEVER casting

Calcaneovalgus foot- Positional deformity- No dislocation of talonavicular joint- Corrects w/ stretching exercises- Ass w/ posteromedial bowing

Metatarsus adductus- Ass w/ DDH- Trapezoid shape of med cuneiform, med

orientation of 1st MT-medial cun jt- Correct w/ stretching when supple beyond

midlineo When deformity is supple, no tx

required- Casting, brace for bad deformities

o Casting < 1yoo Lateral press at calc-cub jt

- Surg for residual deformity (osteotomy)o Opening wedge of med cuneiform

Tarsal Coalition- AD, full penetrance- TC & CN coalitions =- Valgus hindft, dec subtalar motion

o More severe in TC coalition- 8-12 yrs symptomatic CN- 12-16 yrs sx TC, when coalition ossifies- interposition of EDB- > 50%, valgus of hindft, narrowing of post

facet, then excision is bad

Adolescent bunion- Wait until epiphyses closed- 50% recurrent rate- RF for recurrence: pes planus, tight Achilles,

long great toe- Metatarsal osteotomy

Cavus foot- May be neurogenic – check for hairy patches,

XX- Coleman block test, assesses flexibility

Pediatrics

o If hindfoot fixed, then must include this w/ surg tx

- Tx: plantar release, metatarsal osteotomy, tendon transfer, calc osteotomy (when hindft fixed)

o Dorsiflexion osteotomy of 1st MT when hindft flexible

- Avoid triple arth

Kohler’s Dz- AVN of tarsal navicular

o From repetitive trauma to midft w/ weight-bearing

- MC in boys, bilateral in 20%- Tx: casting SLWC, 1 mo- XX delayed by 8 mo- Often resolves spontaneously

Freiberg’s Infarction- adolescent female athletes- longest MT (2nd) typically affected

Peroneal spastic flatfoot- Calcaneonavicular coalition – most common

causeo Bar seen on oblique viewo CT scan to r/o second coalitiono Resect < 14 yo

- Can be caused by trauma, JRA

Osteomyelitis- Metaphyseal, hematogenous- MC org: Staph aureus- Elevated WBC, ESR, CRP

o CRP better than ESR in monitoring response

o More rapid decline in response to tx- XX: nl early- Aspirate w/ large-bore needle- Tx: IV, then oral antibx (if no pus)

o Surgery if pus

Septic arthritis

- Hematogenous- Hip, shoulder, elbow, ankle (jt w/ intra-art

metaphyseal)- SI jt

o Bedresto Bld cxo IV antibx

- Aspirate!- Gonorrhea MC for adolescents

Discitis- Need MRI- Tx: IV antibx for staph, limit activity (no

biopsy)

Perthes Dz- AVN prox femoral epiphysis- Protein S/Protein C implicated as potential

etiologyo 75% children have abn clotting factors

- tend to be shorter, delay in bone maturation 2 yr, high rate of ADHD, exposed to 2° smoke

- Boys affected 4:1o outcomes are =

- 4-8 yo age range MC- bilateral 12% (never symmetric)- poor prognosis > 6 yo, total head involvement- 50% of pt w/ Perthes will dev degen OA of

hip- diff dx: Meyer’s dysplasia, SED, MED, sickle

cell, Gaucher’s, hypothyroidism- Hinge abduction poor prog sign- MRI can correlate w/ bone scan for early

Pertheso % physeal involvement may correlate

better w/ long-term prog than fem head involv

- Tx is supportiveo Goal is to maintain motiono Femoral/pelvic osteotomy reserved for

late sx

SCFE

Pediatrics

- Obese, rapid growth, males- increased shear stress across growth plate

weakened by rapid growth (puberty) or endocrine

- atypicalo < 10 yo, > 16 yo, weight < 50th perc

- male:female 3:2- Neck displaced anteriorly, externally rotated- On XX: Klein’s line, widened physis- Fracture through hypertrophic zone

o Increased height of zone of hypertrophy

- Stable hip (can bear weight)o Incidence of AVN is 3-5%

- Unstable hip (cannot bear weight)o Incidence of AVN is 50%, regardless

of tx- Tx: Pin in situ, or where reduced by

positioning, bilateral if endocrine etiologyo If can insert 4 threads into epiphysis,

then don’t need 2 pins- PWB for 6 wks- 60% of opposite hip slips are Asx

o should prophy pin most- Endocrine w/u < 10 yo

Sprengel’s deformity- Cong failure of descent of scapula opposite 5th

cervical vert to final nl position- MC in girls- Bilateral in 10-30% pt- Cavendish classification

o Grade I mild – level GH jto Grade II visible lump in necko Grade III 2-5 cm of shoulder elevationo Grade IV shoulder near occiput

- Associated with Klippel-Feil, cong scoliosis- High small scapula- Tx: observation vs. Woodward procedure

(midline incision, reflect off rhomboids, pulling scapula down – just for cosmetic reasons)

o Surg @ at least 3 yr of age

o Brachial plexus injury greatest in child > 8 yo

Congenital Pseudarthrosis of clavicle- 100% is Right side (failure fusion of

medial/lateral anlage), unilateral- Tx: grafting/internal fixation vs. observation

Birth Brachial Plexus Palsy- MC involves C5 and C6- Adduction internal rotation deformity- Retroversion of glenoid, post sublux/medial

flattening of hum head, prominent acromion- Exploration if biceps has not returned by 6 mo

age- If lose passive ER in adduction, then likely

GH dysplasia- 1 in 11 newborns w/ clavicle fx had BP palsy- Prox hum does not ossify until 6 mo age- Lat dorsi/teres major transfer

o @ age 4 yrs

Congenital dislocation of radial head- Post dislocation w/ bowing/shortening of

radius- Radial head is mushroom shaped- Tx: excision of radial head at maturity – helps

motion

Radioulnar Synostosis - Arm is fixed in varying degrees of pronation- Tx is based on functional deficit

o One arm in pronation, other in supination

Madelung’s deformity- Blount’s of radius - Associated w/ Leri-Weill syndrome

(dyschondrosteosis)o ADo Deletions in SHOX gene

- Radial/volar hand displacement due to partial def of growth of distal radial physis

Pediatrics

- Most do not require osteotomy- Can be compared to infection, tumor on XX

Congenital amputations- UE prosthesis @ 6 months

Radial clubhand- Longitudinal deficiency, variable lengths- Associated w/ TAR, VATER- Tx dependent on elbow function (biceps)

o If have biceps, surgical centralization is option

o If not, leave where it is

Duplicate thumb- Tx: Take out the ulnar-most thumb typically

(ligamentous stability)- Leave the most stable and dominant

Congenital dislocation of hip- RF: first-born, female, breech, pos fam hx- Associated w/ torticollis, metatarsus adductus- Ortolani sign – dislocation (can put it back in)- Barlow’s sign – dislocatable (in adduction)

o See these signs early < 6 mo- Adduction contracture, asymmetrical limb

signso + Galeazzi sign

- Tx: if reducibleo 0-6 mo: Pavlik harness – tx in

abduction & flexiono 6 mo – 2 yrs, or irreducible –

arthrogram, closed reduction spica cast for 3 mo

o > 2 yrs – open reduction, +/- femoral osteotomy w/ shortening

femoral osteotomy sufficient up to age 4 yrs

pelvic osteotomy usu necessary later

Pemberton – volume depleting

Shelf – salvage

Salter – rotational o Bilateral > 6 yo

Tx: leave aloneo Unilateral > 8 yo

Tx: leave alone

Developmental dysplasia of hip- Failure of acetabular development- Jt well-developed by 11th fetal week- RF: female gender, family hx, breech

o 20% of pt in breech w/ DDH- Ass w/ oligohydramnios, cong recurvatum of

knee, torticollis- Index > 30-35 deg is significant (> 30 deg by

age of 2 yo)o Tx: abduction splinting (early)

- Pt walk w/ glut medius insuff b/c of high position of GT

- Alpha angle > 60 deg, beta angle < 35 dego u/s can be used at week 3 or 4

- Femoral head ossification by age 6 mo- Ischemic necrosis

o dx based on XX – failure of growth of ossific nucleus 1 yr p reduction

o or broadening of fem neck 1 yr po or inc density, then frag of ossified

fem head- Pavlik harness

o If hip hyperflexed, then fem nerve palsy, inf hip dislocation

o If hip posterior sublux – lead to posterior wall def (Pavlik dz)

- Tx: surgicalo Closed red

Preferred to age 2 Good w/ adductor tenotomy

o Open red Older than age 2 Medial approach

inverted labrum cannot be seen

Pediatrics

medial fem circumflex is in deep field of dissection

anterior approach all impediments can be

addressedo Femoral osteotomyo Pelvic osteotomy

Salter 25deg lateral coverage 10deg ant coverage

Pemberton, Dega reshaping osteotomies req open triradiate

Triple innominate PAO – extension, adduction,

anteversion Shelf, Chiari (requires

metaplasia – does not use native cartilage like upper ones)

Congenital coxa vara- Decreased neck-shaft angle- Vertical position of physeal plate- Triangular fragment in inferior femoral neck- Inverted-Y lucency along femoral epiphysis- nl Hilgenreiner’s angle 25 deg

o > 60 deg, progression of deformity, no healing

- hip abductors weak – high-riding GT- Tx: surgical

o Hilg physeal angle > 60 deg, oro progression oro Trend gait oro If neck-shaft angle 90 deg or less, or

progression – then valgus osteotomyo goals: neck-shaft angle to 160 deg,

Hilg angle 25 deg, norm fem rotation- premature closure of prox fem physis occurs

by 24 mo

PFFD

- Can present as severe shortening, absent hip, or cervical pseudarthrosis

- 50% have fibular hemimelia- Tx depends on magnitude of deficiency

o If bilateral, then limbs should be left alone

o If pseudarthrosis cervical, then Repair operatively

Congenital short femur- 15-20% short femur- externally rotated femur, bulky thigh- absent ACL, bowing of femur- Tx: can be lengthened if 30% or less

shortening

LLD- Distal femur 9 mm, prox tibia 6 mm per

year – can use as rule of thumb- Tx

o Shoe lift for inch or lesso Epiphysiodesis for discrepancy of 2.5-

4.0 cmo Lengthening 4 cm or greatero Prosthesis w/ or w/o conversion for

30% or more shortening- Does not cause back pain

Amputations- Diaphyseal level tend to overgrow by

appositional growth- UE prosthesis @ 6 mo, LE prosthesis @ 1 yr

Congenital dislocation of knee- Ass w/ dislocation of hip- Tx knee dislocation first if both present

o Progressive casting/stretching in flexion

o Once 90 deg of flexion, then tx hip dislocation

Congenital patellar dislocation

Pediatrics

- Comprehensive Lateral release, and realignment, need a checkrein (semitendinosus wrapped around to keep patella medial)

Traumatic patellar dislocation- Closed tx successful in about 75%- For recurrent, proximal realignment, (lat

release, med augmentation)o No bony proc – will shut down physis

OCD- If growth plate open, 50-75% will heal w/o

fragmentation- Young children w/ activity modification- Surgery for a loose body, impending

fragmentation

Osgood-Schlatter’s- Microfx of immature apophysis due to

overuse- Tx: rest, act modification

Discoid meniscus- Mechanical sx, lack full extension- Usu lateral meniscus- Tx: partial vs. complete excision- 75% end up having total meniscectemy

eventually

popliteal cyst- Tx: observation

FGFR- 4 identified- FGFR 3

o Chromosome 4o Affects skull, long bone developmento Achondroplasia, hypochondroplasia,

thanatophoric dysplasia (lethal)

Neurofibromin- NF 1 gene- Chromosome 17

- NF

Fibrillin- Chromosome 15- Marfan’s sx

Dwarfism- Acromelic – hand shortening- Mesomelic – trunk shortening- Rhizomelic – root or proximal shortening

Achondroplasia- AD, rhizomelic

o shortening prox limb- FGFR 3- single nucleotide substitution

o converts either G to A or G to C position 380 in domain of

protein results in Glycine to arginine

subst- Frontal bossing, bowed legs- Genu varum 90%

o also internal tibial torsion- Spinal malalignment (70%)

o Kyphosis (typically TL junction) MC in infants resolves when child walks

later than others with dwarfism

o Spinal stenosis (pedicles short) cerv stenosis is life-threatening cerv instability is rare

- humerus, femur most affectedo greatest endochondral growth

- function and self-image as good as average adults

- Hypochondroplasiao Not apparent before age 2o Spinal stenosis, genu varum, shorto Management same as achondro

- Thanatophoric dysplasia

Pediatrics

o Severe, usually lethalo FGFR-3o Severe restrictive lung dz

Psuedoachondroplasia- AD- Mutation in COMP gene

o Extracellular calcium-binding glycoprotein in thrombospondin family

o Involved in chondrocyte migration/prolif

o Mutations in type III repeat region result in decreased calcium binding

o 30% w/ in-frame deletion mutation- Along w/ MED, part of same dz spectrum- Need early THA

Diastrophic Dwarfism (twisted dwarf)- AR- Mutation in sulfate transporter gene

o Affects cartilage b/c of negatively charged sulfate groups in PG molecules

- Short stature- Tracheomalacia- Cervical kyphosis – resolves spont

o Kyphoscoliosis in TL spine may need OR

- Hitchhiker thumb (short metacarpals)- Cauliflower ears- Scoliosis- Bad clubfeet- Flexion contractures of knees

Spondyloepiphyseal Dysplasia- AD- Congenital form MC- Short stature, short trunk, short limbs- Type II collagen

o Mutation in COL2A1o Affects cartilage and vitreous humor

- Platyspondyly (flat), shortening of vert height, odontoid hypoplasia

o Kyphoscoliosis managed w/ brace- Coxa vara, genu valgum, planovalgus foot- Retinal detachment, myopia, hearing loss- Epiphyseal irregularities- Less common X-linked form

o Mutation in SEDL gene Involved in vesicle transport

from ER to Golgi o Milder dz

Kneist’s Dysplasia- AD- Mutation in COL2A1- Short trunk- Flat face- Myopia, retinal detachment, deafness

Metaphyseal Chondrodysplasia- Schmid type

o MCo ADo Type X collagen

Mutation in COL110A1o Short stature, waddling gait, genu

varumo Looks like rickets

- Jansen typeo ADo Shorto Hypercalcemia, hyperphosphaturia

- McKusick typeo ARo Mutation RMRP, nuclear geneo Impaired cellular immunity, anemia,

Hirschsprung’s dz

Multiple epiphyseal dyplasia- AD- Short stature- Type II collagen and Type IX

o Mutation in COL9A2 for type IX collagen

Pediatrics

Milder than those w/ COMP- no hip involvement

- COMP inhibitor – associated with- Epiphyseal irregularities (NO SPINE)

o Genu valgum Tx w/ hemiepiphysiodesis

- Hip problems managed similar to Perthes

Ellis-van Creveld Syndrome- AR- Polydactyly, short stature, mesoacromelic

shortening, sparse hair, dysplatic nails/teeth- Congenital heart dz in 60%- Genu valgum- Linked to gene EVC

o Expressed in higher levels in distal limb, vert bodies

Dyschondrosteosis- AD- Short stature, mesomelic shortening,

Madelung’s- Mutation in SHOX

o Located at tip of both sex chromosomes

o Encodes homeobox-containing DNA transcription factor

o Also ass w/ growth failure in Turner’s

Spondylometaphyseal dysplasia- AD- Short stature- Platyspondyly- Scoliosis - Coxa vara

Diaphyseal dysplasia- Lack of metaphyseal remodeling

Osteopetrosis- Dense bones, brittle- Obliteration of medullary canal (looks like

chalk)

- Rugger jersey spine- Anemia- Nl life expectancy- Macrocephaly- Deafness- Blindness- HSM- Tx: BM transplant

o Malignant form: 1,25 dihydroxy vitamin D

o Tx fractures via casting

Neurofibromatosis- Neurofibromin

o Ras signaling pathway- Chrom 17- Café au lait spots- Hemihypertrophy- Scoliosis (ASF/PSF)

o nondystrophic – like idiopathico dystrophic

short, sharp 4-6 spinal levels MC scalloping of vert end plates,

foraminal enlargement, pecilling of ribs

resistant to brace- Pseudarthrosis

o prophylactic total contact bracing to prevent

- Plexiform neurofibroma- Dural ectasia- neurofibrosarcoma transformation results if

somatic mutation in nl copy of gene

Hereditary Multiple Exostosis- AD, 1 in 50,000- transf to malign CHSA/OSA 3%- mutations in EXT1 or EXT2

o encode glycosyltransferaseso localized to Golgi apparatus

Pediatrics

o responsible for synthesis of heparin sulfate GAGs

o EXT1 necessary for diffusion of hedgehog protein

- Knudsen “two-hit” model of tumor suppressor gene inactivation

o both copies of gene must be inactivated to abolish nl tumor supp act

Morquio’s- Mucopolysaccharodosis- AR- Short stature (proportional)- Anterior beak at TL spine- 2nd-5th MC narrow- Excrete keratan sulfate in urine- C1-2 instability common- Genu valgum- Severe hip deformity- Physeal involvement (like Perthes’)

Hurler’s- corneal clouding, deafness HSM, CV abn- BM transplantation

Gaucher’s Disease- AR- HSM- Glucocerebrosidase def- AVN hips, mimics Perthes- Tx: enzyme replacement can be successful

Marfan’s - Fibrillin- Chromosome 15- Lens dislocation – superior and lateral- Arm span > height- arachnodactyly- Mitral valve prolapse- Scoliosis

o Mortality for surgery very high

Cleidocranial dysplasia- Coxa vara- AD- Broad forehead, phalanx shortening, short

stature- Wormian bones in skull- Clavicle malformation (looks like

pseudarthrosis of clavicle)- Dislocation of hips, knees

o Coxa vara- CBFA1 abnormality

o Important in osteoblast differentiation

Nail-Patella Syndrome- AD- Dysplatic nails, hypoplastic patella- Mutation in LMX1B

o Determines nl dorsoventral pattern in limb bud

- 30% renal failure- post dislocation of radial head

Down’s- Trisomy 21- Ligamentous hyperlaxity- Patellofemoral instability

o Surg difficult b/c ligam hyperlaxo No surgery

- Tx cervical spineo 10% w/ Asx AAIo > 10 mm instability or neuro sx, then

PSF if < 10 mm, asx, then can do

sports- Tx hips

o Should operateo common to have dislocation with mild

dysplastic hips

Turner’s- phenotypically females- XO

Pediatrics

- Mutation in SHOX gene- short stature, webbed neck, low hairline- scoliosis similar to idiopathic curves

JRA- Ass w/ uveitis

OI- AD for the most part, AR are lethal- Type I collagen defect- Familial- Blue sclerae- Multiple fx- Long bone deformities- Tx: bracing, multiple osteotomies, IM nails

Ollier’s Dz- Multiple enchondromatosis- LE deformity- Genu valgum- Mafucci’s syndrome

o w/ hemangiomas

Child abuse- Mult fx in various stages of healing- Corner fx (distal femur, prox tibia)

o Torsional mechanism- Multiple bruises- Unreasonable stories- Any fx in infants

Physeal fx- Zone of provisional calcification

o Pliable area of growth plate- CR and cast- SH III, IV, may require ORIF- For open injuries

o Joint articulation preferable to prevent overgrowth

- Complicationso Physeal arrest (completed vs. partial)o Angular deformity tx

bar resection w/ interposition if

> 2cm growth remaining

< 50% physeal involvement (MRI)

ipsilateral epiphysiodesis if > 50% physeal

involvement contralateral epiphysiodesis vs.

ipsilateral lengthening

Physeal Zone Abnormalities- Reserve Zone –

o Gaucher’s, Diastrophic dwarfism, Kneist syndrome, Pseudoachondroplasia

- Proliferative Zone o Achondroplasia, gigantism

- Hypertrophic Zone o Rickets, osteomalacia –Enchondromas

–Mucopolysaccharidoses (Morquio’s, Hurler’s) –SCFE

o Physeal fractures (Zone of Provisional Calcification)

Tillaux Fx- SER mechanism- Dist tib physis closes centrally, then medially,

then laterally

Monteggia Fx- Tx: CR, casting- Ulnar osteotomy to correct malunion essential

in tx missed Monteggia fx

Galeazzi fx- Tx: CR, casting, but more often will need

ORIF

SCH fx- Nerve injury

o AIN MC in extensiono Ulnar MC in flexiono Posteromed fx – radial nerve at risk

Pediatrics

o Posterolat fx – medial n, brach A at risk

- Divergent lateral pins similar stability to cross pins on extension, varus and valgus (not for axial stress)

- Extension type MC- Complications

o Varus malunion Inc inc of ulnar neuropathy,

late lateral condyle fx, PL rotatory instability

o Nerve palsyo Vascular issues

If still out after reduction, then arteriogram vs. exploration

- Compartmentso Deep volar sign more elevated than

others – reduction did not improveo Rose w/ elbow flexion > 90 deg

Lateral condyle fx- Splint, cast, but FOLLOW closely!- Leads to cubitus valgus- Displaced, then ORIF- Blood supply is posterior

Medial condyle fx- 5-15 mm displacement heal well- High rate of complications (ON of trochlea,

nonunion, fixation failure, stiffness)

Forearm fx- sign decrease in bone mass found in children

w/ FA fx age 9-16 compared to controls- postural balance scores poorer in fx group- in snowboarding, wrist guards lowered

incidence of wrist injuries

Distal Radius fx- pt w/ median N. sx better tx w/ CRPP than

with cast- 4% of DRF, 50% of distal ulna fx w/ growth

discrepancies > 1 cm

Scaphoid fx- MRI can detect occult fx- Waist fx in children same risks of ON as adult- Compl rates in cast vs. surgery are equal

Tendon Lacerations- no differences in total active motion b/w pt tx

w/ early mobilization and casting for 4 wks- in FPL repairs, long-term limited motion of IP

jt occurred in 1/3 of pto short splint immob – neg effect on

outcomeo zone of injury, early mob, digital nerve

injury – no effect on outcome

Trigger Finger- trigger fingers have higher rate of spont

resolution and earlier age of onset than trigger thumb

- 50% of pt undergoing simple A1 pulley release will have residual triggering (more to release)

Hemophilia- Hemorrhage into iliacus muscle- Compresses femoral nerve- Parasthesias in saphenous N. branch- 1st dose of clotting factor elevates level to 80%

of nl- If jt bleeds 3x in 6 mo, then chronic synovitis- Synovectemy if bleeding despite 3 mo of

prophylaxis, or resumption of bleeding w/in 1 yr of successful prophylaxis

o Can inject P32 chromic phosphate into jt – radionuclide synovectemy

Reduces freq of bleeds by 50% Best for pt w/ inhibitor (IgG

response to clotting factor)

Sickle Cell Dz- single subst of valine for glutamic acid at 6th

AA position- hydroxyurea can decrease painful episodes

Pediatrics

- bone infarcts 50x MC when compared to others

- serum procalcitonin concentration is negative predictor of MS infx

Thalessemia- SCFE and early OA @ increased rates

Thrombocytopenia w/ Absent Radii- moratlity in early infancy 40%- Muscle starts on humeral shaft and inserts into

hand (brachiocarpalis) – excision recommended to prevent radial deviation of hand

- Knee involvement – genu varum, patella abno Should be called TARK

Sports- MRI can be as accurate in children as in adults- < 12 yo, MRI lower in sens/spec- teenager w/ skel age 14 yr or older, Tanner

stage 4/5 can be considered skel mature- > 2 cm growth remaining, consider hamstring

recon- tears < 50%, nonsurgical- meniscus w/ high false + rate b/c inc

vascularity of meniscus in children- ACL tears have 50% ass meniscal tear

Dx Gene/Inheritance Defining Characteristics

Homocystinuria(looks like Marfan’s but with inferior dislocation, stiff joints, and osteoporosis).

AR Lens dislocation—InferiorMental retardationTall, thinNo family historyDx: UA for homocysteine—defect in cystathionine B-synthase leads to too much homocysteineRx: Vit B6, no methionine in diet

NF-I AD/neurofibrillinVariable penetranceHigh rate new mut.

ScoliosisPseudoarthrosis tibia (AL bowing)Multiple NOFMultiple neurofibromas

Gaucher’s Dz AR/glucocerebrosidase Most common lysosomal storage dzAVN of femoral headPathologic fracturesThrombocytopenia (Splenectomy)Enlarged distal metaphyseal area (Erlenmeyer flask deformity)No MR

Marfan’s AD/fibrillin (Ch 15) Lens dislocation—superior (60%)MVP, aortic dissectionLaxity, scoliosis (60%)Long thin limbsPectus deformity

Larsen’s(type of arthrogryposis)

AD&R Multiple joint dislocationsScoliosis, clubfeet, cerv. kyphosis