ŠĻą”±į>ž’ ,.ž’’’)*+’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’’ģ„Į @ šæ°–bjbj)) †2KzKz”Ž’’’’’’ˆ¢¢¢¢¢¢¢t¦¦¦8P¦ŒÜ¦ÜtĖēnÄØÄØČØČØČØnŖnŖnŖJēLēLēLēLēLēLē$9éR‹ė„pē¢å­LŖLŖ"å­å­pē¢¢ČØČ؄…ēįįįå­Ö'¢ČØ¢ČØJēįå­Jēįįöć¢¢BäČØøØ 0˜;—C£Ē¦»ÕøävęŌ›ē0Ėēä,ģs֖ ģBä¶4źŠ¢¢¢¢ģ¢Bä4nŖęT«¤įų«„|¬inŖnŖnŖpēpētt¤”¦ įtt¦Scoliosis AIS Surgical treatment thoracic curves > 50, lumbar > 30 = inc of smaller curves among male/female, but female ratio on curves > 30 deg 10:1 rib heads of thor curve should have apical lordosis – if not, then MRI (syringomyelia) Brace Milwaukee or Boston with support for apex T7 and above Boston underarm for apex T8 and below Charleston for TL 25-35 curves PSF gold standard for thoracic curves revision as high as 19% ASF w/ instrumentation for thoracolumbar curves ASF/PSF for skeletal immaturity, < 10 yo, severe curves > 75 deg ant disc when pt Risser grade 0, large/stiff curves (< 50% flex index) SMA syndrome – from hypercorrection of curves in thin people Tx NGT with IVF, prolonged Delayed infx – from P. Acnes or Staph epi (one year later) Tx hdwr removal with 3 wks IV antibx Peak velocity 10 cm per year, occurs just before menses in females MRI for atypical curves, absence thor lordosis, atypical pain, neuro abn, foot deform, ext rapid curve prog Thoracoscopic release/fusion in prone is effective, better tolerated than when pt is lateral SSEPs 50% dec in amplitude, inc in latency of 10% concerning SRS-22 scores not improved w/ Cobb angle correction Infantile 2 mo – 3 yr Left thoracic Male predominance 22% had neural axis abn, 80% of these pt req surg if rib overlaps vert body at apex of curve, high risk prog (phase 2 rib) if overlap (phase 1), then must meas RVAD Rib-vertebral angle difference Measure of rotation Difference b/w RVA on each side > 20 deg w/ phase 1 rib, high risk progression < 25 deg curve, RVAD < 20 deg, observe 25-35, RVAD > 20 cast/brace > 35, MRI, surgery Skeletal immaturity Risser 0 peak height velocity (right afterwards) menarche (b/w 0-1 is menarche) Risser 1 = 25% ossification Risser 2 = 50% ossification of iliac crest Risser 3 = 75% ossification Risser 4 = 100% Juvenile 3-10 yr High incidence of cord abnormalities Syringomyelia Arnold-Chiari malformation Right thoracic MC 95% have prog curves Thoracic curves prog more commonly All curves > 20 deg should be evaluated w/ MRI (20% neural axis abn) Asf/psf Congenital Scoliosis Failure of segm MC Associated anomalies Gu 25%, cardiac 10%, spinal dysraphism 30% Worst prognosis: unilateral bar w/ contralateral hemivertebrae Usual treatment ASF/PSF or PSF in situ Hemivertebral resection Can do in < 5 yo Correct curves < 40 deg No role for bracing Obliquity at LS junction caused by hemivert tx w/ hemivertebrectemy early before compensatory curve is fixed Rib fusions – adverse thor growth known as thor insuff sx Expansion thoracoplasty indicated – prosth rib distractor Surgery for any progression Congenital kyphosis highest risk for neurologic compromise Failure of formation MC May progress to paraplegia Type I – def of vert body, but post elements ok surg tx immediate Type II – vert bodies fused less progressive, less deform lower risk of paraplegia 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 > 3 ribs penciled = significant risk of progression of curve Must be ASF/PSF MRI preop NF1 has Plexiform neurofibromas 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) 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 Psf Grade I-II in situ III and above may need reduction w/ instrumentation High slip angle – means more junctional kyphosis Pelvic incidence obliquity of sacrum to pelvis meas on lateral XX 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 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 65-80 deg – CTLSO Ant release/PSF for curve > 80 deg Klippel Feil Low hairline Web neck Limited cervical ROM Conservative Tx AAI Flex-ex views Up to 2.5 mm in adults is nl Up to 7.5 mm in children is nl ADI 4-5 mm is nl Rotatory subluxation Grisel’s dz: retropharyngeal irritation if deformity fixed, less pain, but torticollis remains CT scan to document – Tx w/ brace, then traction if sx > 3 mo, then post arthrodesis Pseudosubluxation C2/C3 in < 8 yo Spinal laminar line drawn – Schwischuk’s line 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 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 Wackenheim line drawn along clivus and should intersect tangentially the tip of odontoid Powers ratio = line from basion to post arch of atlas / line from opisthion to ant arch (> 1.0, or < 0.55 is disruption no halo or traction odontoid fx only 50% apposition necessary rupture of transverse lig is rare (10% of ped C-spine inj) avulsion of transv lig to C1 MC Halo use: 68% complication rates SCIWORA cause of paralysis in 20-30% children w/ inj of SC 50% have late neuro deterioration cause by vasc insult watershed area < 10 yo more likely to have perm paralysis (difference in inj type) tx: immobilization 2-3 mo in C-collar disc is stronger than cancellous bone – vert body is 1st structure to fail TL fx kyphosis < 20 deg tx w/ hyperextension casting Slipped vert apophysis fx of vert endplate MC involves caudal endplate of L4 teenagers Myelodysplasia Risk factors 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 Most common L3-L4 levels (unopposed hip flexion, adduction) Hip flexion contractures (strong flexors, weak extensors) Knee flexion contractures Correction of 20 deg or more is indicated Ober-Yount procedure IT band lengthening prox/dist to tx hip abd contracture Clubfoot Rigid, poor response to casting 30% have at birth Req surgery vertical talus, calcaneus feet (no GCS complex) thigh-foot angle greater than 20 deg needs supramalleolar osteotomy valgus foot deformity seen at L45 level deformity scoliosis congenital 100% incidence in thoracic levels most progression before age 15 average curve prog 5 deg/year for curves > 40 deg need curve < 40, pelvic obliquity < 25 to sit properly bracing ineffective surg: ASF/PSF high pseudarthrosis rate infx 15-25% (thin skin, then dura underneath) congenital kyphosis classic in spina bifida 10-15% of pt causes recurrent skin breakdown tx: Kyphectemy Arthrogryposis multiple congenitally rigid joints sensation intact nl intelligence risk factor: oligohydramnios limitation of ROM, decreased M. mass clubfoot: surgery initial: soft tissue release later: talectemy, or triple arthrodesis teratologic hip dislocation (hip was never in joint) pseudoacetabulum at presentation Pavlik contraindicated (AVN) 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 Located Xp21 on X chromosome Frame shift of all mRNA distal to deletion Codes nonsense protein Elevated CPK Scoliosis 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 M. weakness more advanced in legs than arms Hip dislocation occur in 62% Usu painless Type III – Kugelberg-Welander 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 Frataxin is mitochondrial protein – involved in iron metabolism Tx w/ Coenzyme Q Spinocerebellar deg dz Onset < 10 yo Hypertrophic cardiomyopathy, DM Death at 25 yrs (cardiac) Cavus feet Scoliosis Distinguishes it from CMT CMT AD, AR, and X-linked Demyelinating forms that slow nerve cond velocity CMT I, III, IV Axonal forms that dec compound muscle act pot CMT II Disease of peripheral nervous system Peripheral myelin protein 22 abnormal On chrom 17 Connexin 32 abn in X-linked Dejerine-Sottas syndrome = HSMN III Occurs in infants Triple arthrodesis contraindicated b/c feet may lose prot sensation most affected muscles (weakness) Anterior tibialis Peroneus brevis Intrinsics foot Intrinsics hand – wasting Hip dysplasia Pt more prone to sciatic nerve palsy after pelvic surg Scoliosis Left thoracic, kyphotic curves Cavovarus foot Mild, flexible Plantar fascia release, 1st MT dorsal closing wedge Rigid Calcaneal osteotomy Severe Triple arthrodesis CP Non-progressive, UMN dz Spastic, dystonic, ataxic, hypotonic Onset < 2 yo Botox (botulinum-A toxin) Competitive inhibitor at motor endplate – irreversible binder Presynaptic cholinergic receptor Lasts 3-6 mo AFO’s Solid for spastic diplegia Crouch gait – floor reaction AFO’s b/c of ankle plantar weakness Baclofen GABA agonist Side effect is decreasing M. tone Toe walking Ankle DF > 5-10 deg, then AFO > 10 deg, then TAL Crouch gait Multiple release (not just heel cord – would worsen gait) Stiff Knee gait Limited knee flexion in swing phase b/c of rectus spasticity EMG w/ rectus firing out of phase Consider rectus femoris transfer w/ medial hamstring lengthening Risks of lateral hamstring lengthening incl weakness leading to knee hypertext during stance phase Dorsal rhizotomy For pt 4-8 yo Spastic diplegia, walking gait abnormality DR and PT better than PT alone Some pt may need more ortho procedures Scoliosis Spastic quad at highest risk C-shaped curve Resistant to bracing Surg indications: progressive deformity, sitting imbalance, pelvic obliquity Controversial b/w ASF/PSF vs. PSF Need segmental fixation Must fuse to pelvis Hip subluxation Early tx: adductor/flexor release (< 4 yo) Later tx: VDRO, pelvic osteotomies Late complications – adduction contracture, scissoring gait, dislocation For a chronic painful dislocation Abduction osteotomy (tip head away from pelvis) Proximal femoral resection But can get superior migration Equinovalgus foot Cause: spastic peroneals, heel cord contracture (Achilles), ligamentous laxity Hindfoot valgus (from loose ligaments) Tx TAL/calcaneal osteotomy Grice procedure (lateral column lengthening), w/ TAL Equinovarus foot Cause: over pull of tibialis anterior or post tibialis, tight Achilles Tx: TAL + post tib split transfer, or Rancho (split tib ant and TAL) SPLATT If post tib tendon out-of-phase then complete transfer If PTT continuous activity, then split transfer Try to delay surgeries until at least 6 yo Surgery before age 8 – 75% of them fail Foot Hallux valgus deformities – MTP fusion Hand Aggressive tx to stretch agonistic muscle, and strengthen antagonists FA hyperpronation limits hand fx Tx w/ rerouting of pronator teres through interosseous membrane Wrist/finger flexion deformity Tx w/ t-x FCU to wrist extensors More severely affected pt – muscle lengthenings More functional pt – dynamic tendon transfers 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: Good foot (at least 4 rays), then limb lengthening (5-8 cm) or epiphysiodesis (2-5 cm) 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) If quad fx, then save distal part If no quad, then knee disarticulation Congential Pseudarthrosis of Tibia Associated with NF (50%) Anterolateral bowing Tx: bracing (w/ no fracture) w/ fracture, then IM nail with bone graft 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 Prox med tib physis Onset < 3 yo Genu varum Metaphyseal diaphyseal angle > 13 deg If > 20 deg, then worse outcome Ass w/ medial tibial physis growth arrest Tx: Brace w/ KAFO Surgery for pt with bar, bracing failures Best results early < 5 yo Need to over-correct into valgus Adolescent’s Overweight, African-american Tx: lateral hemiepiphysiodesis, prox tib osteotomy Clubfoot Hindfoot – equinovarus Forefoot – supination, adductus Midfoot – cavus 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 Surg tx @ 9-10 months Ponseti method Rotation of foot around fixed talus Calcaneus rotated laterally/superiorly (not translated) Order 1. correct cavus 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 Spont resolves Differentiated by flexible midft but DF contracture of ankle XX: lateral plantar flexion view: talus not in line w/ metatarsals Navicular is dorsally dislocated Tx: surg release post med @ 6-9 mo of age, pin talonavicular jt, Also, dorsal approach to TN jt, Achilles lengthening – better correct Naviculectemy – older child 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 When deformity is supple, no tx required Casting, brace for bad deformities Casting < 1yo Lateral press at calc-cub jt Surg for residual deformity (osteotomy) Opening wedge of med cuneiform Tarsal Coalition AD, full penetrance TC & CN coalitions = Valgus hindft, dec subtalar motion 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 If hindfoot fixed, then must include this w/ surg tx Tx: plantar release, metatarsal osteotomy, tendon transfer, calc osteotomy (when hindft fixed) Dorsiflexion osteotomy of 1st MT when hindft flexible Avoid triple arth Kohler’s Dz AVN of tarsal navicular 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 Bar seen on oblique view CT scan to r/o second coalition Resect < 14 yo Can be caused by trauma, JRA Osteomyelitis Metaphyseal, hematogenous MC org: Staph aureus Elevated WBC, ESR, CRP CRP better than ESR in monitoring response More rapid decline in response to tx XX: nl early Aspirate w/ large-bore needle Tx: IV, then oral antibx (if no pus) Surgery if pus Septic arthritis Hematogenous Hip, shoulder, elbow, ankle (jt w/ intra-art metaphyseal) SI jt Bedrest Bld cx 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 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 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 % physeal involvement may correlate better w/ long-term prog than fem head involv Tx is supportive Goal is to maintain motion 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 < 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 Increased height of zone of hypertrophy Stable hip (can bear weight) Incidence of AVN is 3-5% Unstable hip (cannot bear weight) Incidence of AVN is 50%, regardless of tx Tx: Pin in situ, or where reduced by positioning, bilateral if endocrine etiology If can insert 4 threads into epiphysis, then don’t need 2 pins PWB for 6 wks 60% of opposite hip slips are Asx 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 Grade I mild – level GH jt Grade II visible lump in neck Grade III 2-5 cm of shoulder elevation 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) Surg @ at least 3 yr of age 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 @ 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 One arm in pronation, other in supination Madelung’s deformity Blount’s of radius Associated w/ Leri-Weill syndrome (dyschondrosteosis) AD 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) If have biceps, surgical centralization is option 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) See these signs early < 6 mo Adduction contracture, asymmetrical limb signs + Galeazzi sign Tx: if reducible 0-6 mo: Pavlik harness – tx in abduction & flexion 6 mo – 2 yrs, or irreducible – arthrogram, closed reduction spica cast for 3 mo > 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 Bilateral > 6 yo Tx: leave alone 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 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) 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 u/s can be used at week 3 or 4 Femoral head ossification by age 6 mo Ischemic necrosis dx based on XX – failure of growth of ossific nucleus 1 yr p reduction or broadening of fem neck 1 yr p or inc density, then frag of ossified fem head Pavlik harness If hip hyperflexed, then fem nerve palsy, inf hip dislocation If hip posterior sublux – lead to posterior wall def (Pavlik dz) Tx: surgical Closed red Preferred to age 2 Good w/ adductor tenotomy 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 Femoral osteotomy 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 > 60 deg, progression of deformity, no healing hip abductors weak – high-riding GT Tx: surgical Hilg physeal angle > 60 deg, or progression or Trend gait or If neck-shaft angle 90 deg or less, or progression – then valgus osteotomy 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 If bilateral, then limbs should be left alone 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 Shoe lift for inch or less Epiphysiodesis for discrepancy of 2.5- 4.0 cm Lengthening 4 cm or greater 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 Progressive casting/stretching in flexion 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) 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 Chromosome 4 Affects skull, long bone development 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 shortening prox limb FGFR 3 single nucleotide substitution 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% also internal tibial torsion Spinal malalignment (70%) Kyphosis (typically TL junction) MC in infants resolves when child walks later than others with dwarfism Spinal stenosis (pedicles short) cerv stenosis is life-threatening cerv instability is rare humerus, femur most affected greatest endochondral growth function and self-image as good as average adults Hypochondroplasia Not apparent before age 2 Spinal stenosis, genu varum, short Management same as achondro Thanatophoric dysplasia Severe, usually lethal FGFR-3 Severe restrictive lung dz Psuedoachondroplasia AD Mutation in COMP gene Extracellular calcium-binding glycoprotein in thrombospondin family Involved in chondrocyte migration/prolif Mutations in type III repeat region result in decreased calcium binding 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 Affects cartilage b/c of negatively charged sulfate groups in PG molecules Short stature Tracheomalacia Cervical kyphosis – resolves spont 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 Mutation in COL2A1 Affects cartilage and vitreous humor Platyspondyly (flat), shortening of vert height, odontoid hypoplasia Kyphoscoliosis managed w/ brace Coxa vara, genu valgum, planovalgus foot Retinal detachment, myopia, hearing loss Epiphyseal irregularities Less common X-linked form Mutation in SEDL gene Involved in vesicle transport from ER to Golgi Milder dz Kneist’s Dysplasia AD Mutation in COL2A1 Short trunk Flat face Myopia, retinal detachment, deafness Metaphyseal Chondrodysplasia Schmid type MC AD Type X collagen Mutation in COL110A1 Short stature, waddling gait, genu varum Looks like rickets Jansen type AD Short Hypercalcemia, hyperphosphaturia McKusick type AR Mutation RMRP, nuclear gene Impaired cellular immunity, anemia, Hirschsprung’s dz Multiple epiphyseal dyplasia AD Short stature Type II collagen and Type IX Mutation in COL9A2 for type IX collagen Milder than those w/ COMP- no hip involvement COMP inhibitor – associated with Epiphyseal irregularities (NO SPINE) 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 Expressed in higher levels in distal limb, vert bodies Dyschondrosteosis AD Short stature, mesomelic shortening, Madelung’s Mutation in SHOX Located at tip of both sex chromosomes Encodes homeobox-containing DNA transcription factor 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 Malignant form: 1,25 dihydroxy vitamin D Tx fractures via casting Neurofibromatosis Neurofibromin Ras signaling pathway Chrom 17 Café au lait spots Hemihypertrophy Scoliosis (ASF/PSF) nondystrophic – like idiopathic dystrophic short, sharp 4-6 spinal levels MC scalloping of vert end plates, foraminal enlargement, pecilling of ribs resistant to brace Pseudarthrosis 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 encode glycosyltransferases localized to Golgi apparatus responsible for synthesis of heparin sulfate GAGs EXT1 necessary for diffusion of hedgehog protein Knudsen “two-hit” model of tumor suppressor gene inactivation 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 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 Coxa vara CBFA1 abnormality Important in osteoblast differentiation Nail-Patella Syndrome AD Dysplatic nails, hypoplastic patella Mutation in LMX1B Determines nl dorsoventral pattern in limb bud 30% renal failure post dislocation of radial head Down’s Trisomy 21 Ligamentous hyperlaxity Patellofemoral instability Surg difficult b/c ligam hyperlax No surgery Tx cervical spine 10% w/ Asx AAI > 10 mm instability or neuro sx, then PSF if < 10 mm, asx, then can do sports Tx hips Should operate 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 w/ hemangiomas Child abuse Mult fx in various stages of healing Corner fx (distal femur, prox tibia) Torsional mechanism Multiple bruises Unreasonable stories Any fx in infants Physeal fx Zone of provisional calcification Pliable area of growth plate CR and cast SH III, IV, may require ORIF For open injuries Joint articulation preferable to prevent overgrowth Complications Physeal arrest (completed vs. partial) 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 – Gaucher’s, Diastrophic dwarfism, Kneist syndrome, Pseudoachondroplasia Proliferative Zone Achondroplasia, gigantism Hypertrophic Zone Rickets, osteomalacia –Enchondromas –Mucopolysaccharidoses (Morquio’s, Hurler’s) –SCFE 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 AIN MC in extension Ulnar MC in flexion Posteromed fx – radial nerve at risk 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 Varus malunion Inc inc of ulnar neuropathy, late lateral condyle fx, PL rotatory instability Nerve palsy Vascular issues If still out after reduction, then arteriogram vs. exploration Compartments Deep volar sign more elevated than others – reduction did not improve 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 short splint immob – neg effect on outcome 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 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 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 DxGene/InheritanceDefining CharacteristicsHomocystinuria (looks like Marfan’s but with inferior dislocation, stiff joints, and osteoporosis).ARLens dislocation—Inferior Mental retardation Tall, thin No family history Dx: UA for homocysteine—defect in cystathionine B-synthase leads to too much homocysteine Rx: Vit B6, no methionine in dietNF-IAD/neurofibrillin Variable penetrance High rate new mut.Scoliosis Pseudoarthrosis tibia (AL bowing) Multiple NOF Multiple neurofibromasGaucher’s DzAR/glucocerebrosidaseMost common lysosomal storage dz AVN of femoral head Pathologic fractures Thrombocytopenia (Splenectomy) Enlarged distal metaphyseal area (Erlenmeyer flask deformity) No MRMarfan’sAD/fibrillin (Ch 15)Lens dislocation—superior (60%) MVP, aortic dissection Laxity, scoliosis (60%) Long thin limbs Pectus deformityLarsen’s (type of arthrogryposis)AD&RMultiple joint dislocations Scoliosis, clubfeet, cerv. kyphosis     Pediatrics   CīQ p • – ® Ž å  f Ś ē  › Ń v Ń ą ;Nbl”³'8]†Šž'12Ep„ÅÕŁŻäģASz’­³ÜŻļö  ČÉĢT‘«ŁöņīņīņźīņźęźņźņźņźāźāźęźŚęÖęĖęźĒęāęāęĒĆĒĆĒĆĒęæĒæĒęƻƻęĆęĆęĆęĆ·­»ę»ęh^(h©@¾56h6k_h©@¾h/ Ah×R½h¹ihęY h+`mHsHh+`hŚźhĘ„5hé ķhŚźhĘ„hEuhÓAņh6k_hEu56D !C—īō+ Q p – ® Ž  f £ ¾ ł  a Ķ * 0 g › żõd¬ķd¬åd¬ķd¬ķd¬ķd¬åd¬åd¬åd¬ķd¬åd¬ķd¬ķd¬åd¬ķd¬åd¬ķd¬åd¬ķd¬ķd¬ķd¬ķd¬åd¬ķd¬ & FgdÓAņ & FgdÓAņ & FgdEu”–Æ–žž› ± æ Ń  L v • © É ų 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Fgd6k_ų<=9=`=Œ=¢=±=Õ= >>$><>V>i>}>„>³>»>Ó>ē>???÷d¬÷d¬ļd¬ļd¬ēd¬ēd¬ßd¬ßd¬ßd¬×dH×Ļd¬×’dՃ×dH×Ēd¬ßd¬ßd¬ßd¬×dH×ßd¬ļd¬Ād¬Ād¬gd6k_ & Fgdi.4 & Fgd×%z & Fgd6k_ & Fgd6k_ & Fgd6k_ & Fgd6k_ & Fgd×%z?-?O?r?…?š?Ļ?ń?@=@€@”@ć@)AEASATAhA}A£AÄAąAįAõA÷d¬÷d¬÷d¬÷d¬÷d¬÷d¬÷d¬ļd¬ļd¬÷d¬ēd¬÷d¬ßd¬ßd¬ēd¬Śd¬Śd¬÷d¬÷d¬÷d¬÷d¬Õd¬Õd¬gdõ ¹gd6k_ & FgdŃ & Fgd6k_ & Fgd” & Fgd6k_ą@ā@ć@EASATAhAÄAąAįAõAB6B8BJB{B¤BĒBņBC9C:CKC^C_C DODPDQDcDDŠDåDęDńDŽEłEūEF&F'F2F3FKF}FšF„FŖF°F±FÄFĖFįFāFüųüųōźųęāźųŽÖŽųŅųĪųĪāĒĆüĆæøĒźų“ųāŖ¦ęžę¦ā”ŖŒæŅęŒŅŒŅˆh/ŗh59*hKLh^(hKL56haGihaGiH*hóh^(hó56hip hŃhnx^hnx^hq* hŃ56hÖšh‹«hi.4hi.4H*hi.4hõ ¹haGih^(h'D56h6k_h'DhŃ5õABJB{B¤BĒBÕBņBC9C:CKC_CtC—C³CĖCōC DPDQDcDD’DŠD÷d¬÷d¬÷d¬ļd¬÷d¬ēd¬ēd¬÷d¬ēd¬ād¬ād¬Śd¬Śd¬Śd¬Ņd¬Śd¬Śd¬Śd¬Śd¬ād¬ād¬÷d¬÷d¬÷d¬ & Fgdq* & Fgdq*gdõ ¹ & FgdÖš & Fgdõ ¹ & Fgdõ ¹ŠDåDęDńD!EJEEŽEF&F'F3FKF}FšF±FÄFįFāFųFG7G8GRG÷d¬ņd¬ņd¬÷d¬÷d¬źd¬÷d¬źd¬÷d¬ņd¬ņd¬ād¬Śd¬ād¬ād¬ād¬ād¬Õd¬Õd¬Ķd¬Ķd¬Õd¬Õd¬ & Fgd59*gd/ŗ & 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ppCpˆpØpŃpśpq.q÷d¬÷d¬÷d¬ļd¬÷d¬÷d¬÷d¬÷d¬÷d¬źd¬źd¬÷d¬÷d¬÷d¬÷d¬ād¬Śd¬÷d¬Śd¬Ņd¬÷d¬÷d¬÷d¬ & FgdŠ`¤ & FgdÅs & Fgdxægd/ŗ & FgdąKõ & Fgd/ŗWo€ožoŸo¼oæoŅoßołośo p ppBpCprp‡pˆpØpŃpśpq~qq’qāqćqäqr×r s ss+suvvv$vNvXvYvnvovrvŒvvŽvœv’v6wHwqwŠw‹ww«wĮwx©xøxęxżx y\yzy^zįzāzģz{"{={?{A{C{N{{üųńķęńßŪŌßŹĘ¾ųŹŗĘŗĘų°ŗ¬ŗ¬¤ų°ŗ ŗ ŗ ŗ¬ – ’ų°ŽŗŽ†Ž†Ž¤hąKõhąKõH*hąKõhw+Šhw+Šhƒ F56hƒ FhfIh„5hfIhƒ Fh„56h„hfIh|š6h|šhƒ Fh|š56 hhT)hhT)hhT) hhT)56 h¶e”h¶e”h¶e” h¶e”56h/ŗhzI¾2Õtįtōt+u,u>uAuqu‚u©uŽuvv$v'v5vCvNvXvYvnvvŽvœv±v÷d¬÷d¬ļd¬źd¬źd¬ād¬ād¬ād¬Śd¬Śd¬Śd¬źd¬źd¬Ņd¬Ņd¬Ņd¬Ņd¬Ņd¬źd¬źd¬Ņd¬źd¬źd¬Ņd¬ & Fgd/ŗ & FgdhT) & FgdhT)gd/ŗ & Fgd¶e” & Fgd¶e”±vävųv’vww(w2w6wHwqwŠw‹ww«wĮwŹwŻwķwx!x,x9xKxNx–x÷d¬÷d¬÷d¬÷d¬÷d¬÷d¬÷d¬÷d¬÷d¬ļd¬ļd¬źd¬źd¬÷d¬ād¬÷d¬÷d¬÷d¬÷d¬ād¬ād¬Śd¬Śd¬Śd¬Śd¬ & Fgdƒ F & Fgdƒ Fgd/ŗ & Fgd/ŗ & Fgd/ŗ–x©xøxęxżx y[y\yzyŠy§yĮyŻyūy-z^zœzįzāzģz{{"{÷d¬ļd¬ēd¬ļd¬ļd¬ļd¬ād¬ād¬ļd¬ļd¬ļd¬ēd¬ēd¬ēd¬ēd¬Śd¬Ņd¬Ķd¬Ķd¬Åd¬ļd¬ļd¬ & 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