Peds Miller Notes



Scoliosis

- AIS

o Surgical treatment

▪ thoracic curves > 50, lumbar > 30

o = 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, prolonged

o Delayed infx – from P. Acnes or Staph epi (one year later)

▪ Tx hdwr removal with 3 wks IV antibx

o Peak velocity 10 cm per year, occurs just before menses in females

o 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 yr

o Left thoracic

o Male predominance

o 22% had neural axis abn, 80% of these pt req surg

o if rib overlaps vert body at apex of curve, high risk prog (phase 2 rib)

▪ if overlap (phase 1), then must meas RVAD

o Rib-vertebral angle difference

▪ Measure of rotation

▪ Difference b/w RVA on each side

▪ > 20 deg w/ phase 1 rib, high risk progression

o < 25 deg curve, RVAD < 20 deg, observe

o 25-35, RVAD > 20 cast/brace

o > 35, MRI, surgery

- Skeletal immaturity

o 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 crest

o Risser 3 = 75% ossification

o Risser 4 = 100%

- Juvenile 3-10 yr

o High incidence of cord abnormalities

▪ Syringomyelia

▪ Arnold-Chiari malformation

o Right thoracic MC

o 95% have prog curves

o Thoracic curves prog more commonly

o All curves > 20 deg should be evaluated w/ MRI (20% neural axis abn)

o Asf/psf

- Congenital Scoliosis

o Failure of segm MC

o 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 bracing

o 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 distractor

o Surgery for any progression

- Congenital kyphosis

o highest risk for neurologic compromise

o Failure of formation MC

o May progress to paraplegia

o 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 neurofibromas

o 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 management

o Psf

o Grade I-II in situ

o 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

- 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 – CTLSO

o 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 nl

o 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 remains

o CT scan to document – Tx w/ brace, then traction

o 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 dislocation

o 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 SC

o 50% have late neuro deterioration

o cause by vasc insult watershed area

o < 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 fx

o kyphosis < 20 deg tx w/ hyperextension casting

- Slipped vert apophysis

o fx of vert endplate

o MC involves caudal endplate of L4

o teenagers

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 contractures

o Correction of 20 deg or more is indicated

- Ober-Yount procedure

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

- Clubfoot

o Rigid, poor response to casting

▪ 30% have at birth

o 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

- scoliosis

o congenital

o 100% incidence in thoracic levels

o most progression before age 15

o average curve prog 5 deg/year for curves > 40 deg

o need curve < 40, pelvic obliquity < 25 to sit properly

o bracing ineffective

o surg: ASF/PSF

o high pseudarthrosis rate

o infx 15-25% (thin skin, then dura underneath)

- congenital kyphosis

o classic in spina bifida

o 10-15% of pt

o causes recurrent skin breakdown

o 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 release

o 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 chromosome

o Frame shift of all mRNA distal to deletion

o Codes nonsense protein

- Elevated CPK

- Scoliosis

o 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-Welander

o Onset of M. weakness b/w 2-15 yrs

Friedrich’s ataxia

- AR

- GAA trinucleotide repeat in both copies of frataxin gene on chrom 9

o Frataxin is mitochondrial protein – involved in iron metabolism

o 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 velocity

o 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 infants

o Triple arthrodesis contraindicated b/c feet may lose prot sensation

- most affected muscles (weakness)

o Anterior tibialis

o Peroneus brevis

o Intrinsics foot

o Intrinsics hand – wasting

- Hip dysplasia

o 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 wedge

o Rigid

▪ Calcaneal osteotomy

o 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

o Presynaptic cholinergic receptor

o Lasts 3-6 mo

- AFO’s

o Solid for spastic diplegia

o Crouch gait – floor reaction AFO’s

▪ b/c of ankle plantar weakness

- Baclofen

o GABA agonist

o Side effect is decreasing M. tone

- Toe walking

o Ankle DF > 5-10 deg, then AFO

o > 10 deg, then TAL

- Crouch gait

o 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 phase

o Consider rectus femoris transfer w/ medial hamstring lengthening

▪ Risks of lateral hamstring lengthening incl weakness leading to knee hypertext during stance phase

- Dorsal rhizotomy

o For pt 4-8 yo

o Spastic diplegia, walking gait abnormality

o DR and PT better than PT alone

o Some pt may need more ortho procedures

- Scoliosis

o Spastic quad at highest risk

o C-shaped curve

o Resistant to bracing

o Surg indications: progressive deformity, sitting imbalance, pelvic obliquity

o Controversial b/w ASF/PSF vs. PSF

o Need segmental fixation

o Must fuse to pelvis

- Hip subluxation

o Early tx: adductor/flexor release (< 4 yo)

o Later tx: VDRO, pelvic osteotomies

o Late complications – adduction contracture, scissoring gait, dislocation

o 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 transfer

o Try to delay surgeries until at least 6 yo

o Surgery before age 8 – 75% of them fail

- Foot

o Hallux valgus deformities – MTP fusion

- Hand

o Aggressive tx to stretch agonistic muscle, and strengthen antagonists

o FA hyperpronation limits hand fx

▪ Tx w/ rerouting of pronator teres through interosseous membrane

o Wrist/finger flexion deformity

▪ Tx w/ t-x FCU to wrist extensors

o More severely affected pt – muscle lengthenings

o More functional pt – dynamic tendon transfers

o 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 part

o 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 physis

o Onset < 3 yo

o Genu varum

o Metaphyseal diaphyseal angle > 13 deg

▪ If > 20 deg, then worse outcome

o Ass w/ medial tibial physis growth arrest

o Tx: Brace w/ KAFO

o Surgery for pt with bar, bracing failures

▪ Best results early < 5 yo

▪ Need to over-correct into valgus

- Adolescent’s

o Overweight, African-american

o 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 months

o Ponseti method

▪ 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 resolves

o Differentiated by flexible midft but DF contracture of ankle

- XX: lateral plantar flexion view: talus not in line w/ metatarsals

o 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 child

o 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 midline

o When deformity is supple, no tx required

- Casting, brace for bad deformities

o Casting < 1yo

o 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

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 cause

o Bar seen on oblique view

o CT scan to r/o second coalition

o 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 Bedrest

o Bld cx

o 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 etiology

o 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 4:1

o 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 Perthes

o % physeal involvement may correlate better w/ long-term prog than fem head involv

- Tx is supportive

o Goal is to maintain motion

o Femoral/pelvic osteotomy reserved for late sx

SCFE

- Obese, rapid growth, males

- increased shear stress across growth plate weakened by rapid growth (puberty) or endocrine

- atypical

o < 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 etiology

o 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 jt

o Grade II visible lump in neck

o Grade III 2-5 cm of shoulder elevation

o 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 AD

o Deletions in SHOX gene

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

- 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 signs

o + Galeazzi sign

- Tx: if reducible

o 0-6 mo: Pavlik harness – tx in abduction & flexion

o 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 alone

o 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 deg

o 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 p

o 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: surgical

o Closed red

▪ Preferred to age 2

▪ Good w/ adductor tenotomy

o Open red

▪ Older than age 2

▪ Medial approach

• inverted labrum cannot be seen

• medial fem circumflex is in deep field of dissection

▪ anterior approach

• all impediments can be addressed

o Femoral osteotomy

o 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, or

o progression or

o Trend gait or

o If neck-shaft angle 90 deg or less, or progression – then valgus osteotomy

o 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 less

o Epiphysiodesis for discrepancy of 2.5- 4.0 cm

o Lengthening 4 cm or greater

o 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

- 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 4

o Affects skull, long bone development

o 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 affected

o greatest endochondral growth

- function and self-image as good as average adults

- Hypochondroplasia

o Not apparent before age 2

o Spinal stenosis, genu varum, short

o Management same as achondro

- Thanatophoric dysplasia

o Severe, usually lethal

o FGFR-3

o 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 COL2A1

o 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 MC

o AD

o Type X collagen

▪ Mutation in COL110A1

o Short stature, waddling gait, genu varum

o Looks like rickets

- Jansen type

o AD

o Short

o Hypercalcemia, hyperphosphaturia

- McKusick type

o AR

o Mutation RMRP, nuclear gene

o 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

▪ 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 idiopathic

o 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 glycosyltransferases

o localized to Golgi apparatus

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 hyperlax

o No surgery

- Tx cervical spine

o 10% w/ Asx AAI

o > 10 mm instability or neuro sx, then PSF

▪ if < 10 mm, asx, then can do sports

- Tx hips

o Should operate

o common to have dislocation with mild dysplastic hips

Turner’s

- phenotypically females

- XO

- 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

- Complications

o 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 extension

o Ulnar MC in flexion

o Posteromed fx – radial nerve at risk

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 palsy

o Vascular issues

▪ If still out after reduction, then arteriogram vs. exploration

- Compartments

o Deep volar sign more elevated than others – reduction did not improve

o 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 pt

o short splint immob – neg effect on outcome

o 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

- 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 abn

o 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 |AR |Lens dislocation—Inferior |

|(looks like Marfan’s | |Mental retardation |

|but with inferior | |Tall, thin |

|dislocation, stiff | |No family history |

|joints, and | |Dx: UA for |

|osteoporosis). | |homocysteine—defect in |

| | |cystathionine B-synthase |

| | |leads to too much |

| | |homocysteine |

| | |Rx: Vit B6, no methionine |

| | |in diet |

|NF-I |AD/neurofibrillin |Scoliosis |

| |Variable penetrance |Pseudoarthrosis tibia (AL |

| |High rate new mut. |bowing) |

| | |Multiple NOF |

| | |Multiple neurofibromas |

|Gaucher’s Dz |AR/glucocerebrosidase |Most common lysosomal |

| | |storage dz |

| | |AVN of femoral head |

| | |Pathologic fractures |

| | |Thrombocytopenia |

| | |(Splenectomy) |

| | |Enlarged distal metaphyseal|

| | |area (Erlenmeyer flask |

| | |deformity) |

| | |No MR |

|Marfan’s |AD/fibrillin (Ch 15) |Lens dislocation—superior |

| | |(60%) |

| | |MVP, aortic dissection |

| | |Laxity, scoliosis (60%) |

| | |Long thin limbs |

| | |Pectus deformity |

|Larsen’s |AD&R |Multiple joint dislocations|

|(type of | |Scoliosis, clubfeet, cerv. |

|arthrogryposis) | |kyphosis |

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