ORTHOPEDIC REVIEWLearn it, live it, love it!

Dolor, Tumor, Calor, RuborSTICKOR

Cerebral PalsyDefinition: non-progressive neurologic disorder in infants <3 yoNumber 1 risk factor: Pre-maturityTypes: A ARMS Ataxic (5%), Athetoid (10%), Rigid (10%), Mixed (15%), Spastic (60%)

Athetoid is almost unheard of now due to the decreased frequency of Erythroblastosis Fetalis (ABO incompatibility)Blecks Criteria (2=poor, 1=fair, 0=good chance will walk) (The chance the CP patient will ambulate 15m w/wo

forearm walker on level surface before 3yo)F oot Placement (absence of is bad) “steppage”-brush dorsum of foot or anterior aspect of tibia on table

and the normal child will flex the hip and knee, dorsiflex ankle, and place the foot on the table spontaneously.

E xtensor Thrust - apply pressure to sole of foot with hip in flexed position and the child will extend entire leg. (In a normal child, this reflex will disappear at 2 months, persistence is abnormal.)

M oro Reflex (persistence of is bad) startle reflex or quick extension of neck –The child will abduct and extend all 4 extremities, extend the spine, extend and fan the digits except for flexion of the distal phalanges of the index finger and thumb. This reflex normally disappears at 4 –6 months.

S ymmetric Tonic Neck Reflex Place the child in the quadriped position: When you flex the neck the LE ext/UE flex, and

when you extend the neck the LE flex/UE ext. This reflex normally appears by 6 months.N eck Righting Reflex - Place child supine-rotate head to one side and the normal patient will log roll

shoulders/pelvis as one to the same side. A ssymmetric Tonic Neck Reflex (persistence of bad) fencers posture. Place patient supine and rotate

head to one side for 5 seconds then rotate to other side. The “face side” arm and leg will extend and the “occiput side” arm and leg will extend. This reflex normally disappears at 4-6 months.

P arachute Reflex (absence of bad) Suspend patient and suddenly move head toward the floor. The normal patient will extend his arms and wrists so as to break the force of the fall.

Spine/ScoliosisThoracolumbar (45%) C-shaped/paralytic curvesLumbar (25%)Thoracic (16%)Double primary (14%)

Standing Postures: 1. Knees extended, Hips flexed, Excessive lumbar lordosis2. Knees extended, Hips flexed, Forward lean of trunk3. Knees flexed, Hips flexed (Jumpers position)

2 foot problems: equinus, planovalgusCompensation for equinus: 1. Genu recurvatum

2. Midfoot break3. Hindfoot valgus

1

SCFEEpidemiology: 2:100,000, Age 10-15 yo, M:F 2-5:1, 2 years younger in females, 25-40% bilateral, L>R

Ant/lat displacement of the femoral neck on the head-head stays located in acetabulumPresent as pain in knee with limited IR/ER--mom brings kid in because of limpBody types: Tall, thin athletic male (rare)

Hypogonadal, obese, delayed sexual maturityEtiology theories:

1. S ynovial hypervascularity (increased height of growth plate) slip through zone of Hypertrophy2. E piphyseal cartilage weakening (dyschondroplasia)3. T hinning & weakening of the periosteum-lose support for head

4. M etabolic (Radiation, Renal osteodystrophy, Scurvy)5. E ndocrine (Hypothyroidism, GH, Sex hormone)6. T rauma

7. P hysical theory (Shear stress with oblique physis, deep acetabulum)8. E piphysiolysis (through zone of proliferation)9. T raumatic separation (through zone of calcification)

Anatomy: Perichondral ring is last structure to failLoder Classification:

1. Acute: <3 weeks, trauma, disabling pain, unstable Stable(can walk on it) one pin2. Acute on Chronic: >3 weeks, limp Unstable(can’t walk on it) two pins3. Chronic: most common ~47+% AVN rate (Loder et al)

Stages: Preslip-x-ray-widening of physis, +/- Blush signMinimal slip (<1 cm)Moderate slip (1 cm-2/3 head)Severe slip (>2/3 head)

Lovell/Winter Classification: I (0-33%), II (33-50%), III (>50%)X-ray: Shentons line, Kleins line, Blush sign-osseous density in chronic slips in the metaphysis just below

epiphysis (healing bone) aka Steele's sign, pistol-grip deformity, Jones bone--reactive bone on medial side

Need to know: Chronicity-acute vs. chronicOpen/closed growth platesSeveritySystemic diseases

Treatment: 1. cast2. percutaneous pins (Stable--can walk on it--one pin, Unstable--can’t walk on it--two pins)3. bone-peg epiphysiodesis4. osteotomies (Southwick, Sutherland, Sugioka)--valgus and rotation5. sex hormone manipulation--not recommended6. open epiphysiodesis with bone graft 7. Dunn procedure--shortens femoral neck

Objectives:Prevent further slip, and if acute, gently reduce slip but don't tell anyoneCause early closure of physisPrevent AVN/chondrolysis/DJDIncrease function of child

Complications: AVN – from unstable slips (47+% chance of AVN-Loder et al)Chondrolysis (worst complication) from leaving pins proud through femoral head

Mosely approach/withdraw test-ROM under fluoro to check if pins are in joint space"Safe zone" 8mm from subchondral bone

DJD, LLD

2

Perthes DiseaseEpidemiology: 1:1200, M:F 4:1, 15% bilateral (AVN in different stages), Peak age 5-6yo, familial 10-15%

If bilateral think:1. Meyers Dysplasia – AVN in same stage in both hips2. Multiple Epiphyseal Dysplasia 3. Spondyloepiphyseal Dysplasia – spine involved--platyspondyly

If black think HgbSSEtiology: Protein C & S or AT-III deficiencies, high lipoprotein a, hemophilia A/B,

secondary to trauma, other vascular diseases, exposure to cigarette smokingMost commonly presents as PAINLESS limpClassifications:

Goff-Aegeter-Ferguson---WAFRI: Widened joint space--apparent (delayed growth of ossific nucleus, hypertrophied cartilage)II: AVN (subchondral fx, crescent sign)III: Fragmentation-subchondral comp fx with cystic irreg in head, metaphyseal cyst formationIV: Resolution (Normal, Coxa magna/plana/vara/breva, gr. troch overgrowth esp if <5yo

Catteral (Prognosis: I, II good/ III, IV poor)I: Anterolateral head involvementII: Central collapse (medial, lateral columns intact)III: Central sequestrum (medial column intact, head within head) IV: Whole head involvement (III and IV physis at risk due to minimal support)

Salter-Thompson (True Perthes only with subchondral fracture-starts ant/lat, prognostic classification in predicting eventual involvement of the femoral head - apply only after crescent sign)

A: less than half of the head (intact lateral margin)B: more than half the head (absence of lateral margin)

Herring (Lateral pillar classification)-central/medial pillars are irrelevantA: Minimal involvement lateral pillarB: >50% Lateral pillar height remains => if <6yo=observe, >6yo=operateC: <50% Lateral pillar height remains =>operate

Conway (bone scan stages-“biologic”)I: total lack of uptakeII: lateral column uptakeIII: gradual fill of ant/lat epiphysisIV: base fill in epiphysis adjacent to physis

Sequence of events: (Salter) VCRR TOPS1. Vascular compromise (see etiologies above) \2. Cessation of growth of bony epiphysis _____ \ potential Perthes3. Revascularization (from periphery) /4. Resumption of ossification which is fragile /5. Trauma leads to pathologic fracture (onset of True Perthes)6. Onset of resorption7. Plastic bone replacement8. Subluxation....Residual deformity

Head at Risk1. Gages Sign (lateral V-shaped lucency on the ossific center)2. Lateral calcification (represents fracture through epiphysis extending into physis)3. Subluxation (worst)

4. Metaphyseal cysts5. Horizontal physis

Good prognosis: Less than half the head involved and less than a half dozen years of age

Evaluation

3

Center-Edge angleCenter of femoral head ....Vertical line through this point.....Line from center of head to lateral

edge of acetabulumNormal is 20-40Pathologic <20, if <15 need to augment Salter with a shelf

Method of Mose: Radius= Good (w/in 2mm), Fair (2-4mm), Poor (>4mm)Treatment Goals – essentially ROM and containment

1. Decrease initial irritability2. Maintain ROM3. Prevent subluxation 4. Obtain spherical head

Surgical Treatment:Soft tissue releases – adductor longus, ileopsoas lengthening, medial capsulotomySalter: Age <6, Total head involvement, Congruity, Good ROM, Poor compliance w/ orthosis

Lengthens leg VDRO: Procedure of choice w/ bilateral or dislocated hips

Complications: Short leg limpResidual deformity – applied at skeletal maturity (Stulberg classification)

1. Round head, round acetabulum (congruent)2. Sperical head with coxa magna/breva/vara, round/dysplastic acetabulum (congruent)3. Mushroom head, dysplastic acetabulum (congruent)4. Square head, square acetabulum (congruent)5. Square head, round acetabulum (non-congruent)

Developmental Dysplasia of the Hip (DDH)

4

Incidence: 1/1,000---AVN, the most devastating complicationRisks: First born, Female, Frank breech, Fair complexion, Family history, Facial twist (torticollis)Diagnosis: Barlow/Ortolani initially, Galeazzi/Limitation of abduction (best after 2 mos of age)

Also on physical exam:Nelaton's line: Line b/w ASIS & ischium on the lateral....greater troch should be lowerKlisic's line: Line from greater troch to ASIS....should extend cephalad to umbilicusAsymetric thigh, gluteal folds

X-Ray: Shenton's lineLateral displacement –Reimer Migration Index (Asymmetry b/w medial femoral metaphysis and midline)Quadrant: Horizontal = Hilg., Vertical =Perkins (Normal: Medial prox femoral metaphysis

in medial/lower quadrant)Pathologic: Acetabular index >30, CE angle <20

Van Rosens View: Adduct......Push......Abduct.......Internal rotationHourglass Deficiency: (Arthrogram) caused by IliopsoasGage's Position: -1 = Above Hilgenreiners line

0 = Below Hilg. line, Above nl position+1 = At normal level+2 = Below normal level

Graf Classification (ultrasound)Classification & Treatment (per AHC):

I: (<6 weeks) Pavlik harness - 3 mo treatment/ 1 mo of ageII: (6wk - 1 year)

Ortolani +: PavlicOrtolani -: TAAT (Traction, Adductor tenotomy, Arthrography, Trumpet cast)

III: (1-3 years): TAAT, +/- VDRO, Pelvic osteotomyIV: (>3 years): Open reduction, femoral shortening, +/- pelvic osteotomyIn newborn with ultrasound +, but physical exam -, observation and repeat physical exam

Blocks to closed reduction (LT CLIP)Hypertrophied Ligamentum teresTransverse acetabular ligamentMedial CapsuleInverted LabrumIliopsoasPulvinar

Signs of AVN (Salter): AVN increases with age and d/l1. No ossific nucleus by 1 year following tx 25% at 1yo2. Failure of growth of ossific nucleus following tx 50% 3yo3. Broadened femoral neck 67% with no tx.4. Sclerosis, Fragmentation5. Residual deformity (Coxa magna/plana/vara)

AVN resulting in a mushroom head will produce the xray finding of "the sagging rope sign"

Clubfoot: Talipes EquinovarusIncidence: 1/1,000, M(65%) > F, 30-40% bilateral

5

Etiology: PAAAG1. Packing phenomenon2. Arrested fetal development3. Abnormal tendon insertions4. Abnormal talus rotation5. Germ plasm defect

Associations: PB PB MAD LSO1. PFFD2. Bifurcated femur3. Pierre-Robin Syndrome – Micrognathia with glossoptosis (X-linked recessive)4. Bilateral uni-digit foot5. Myelodysplasia6. AMC (Arthrogryposis)7. Diastrophic dwarfism8. Larsen’s Syndrome – Multiple congenital dislocations including ant. Dislocation of the tibia on the

femur, frontal bossing, flat facies, hypertelorism, depressed nasal bridge.9. Amniotic Band Syndrome (Streeters Dysplasia)10. Other Packing Phenomena (DDH, Torticollis)

Physical Findings: X-Ray findings1. Shorter leg 1. Forefoot adduction2. Smaller calf 2. Parallelism of talo/calc on lat max 3. Tibia internally rotated (? Internal tibial torsion) dorsiflexion4. Talus internal/medial rotation 3. Decr. Kite’s angle on AP of foot 5. Lateral dimple over ant/lat talus 4. Plantar/medial subluxation of 6. Small/wider foot navicular (ossifies @ 5yrs) 7. Cavus-midfoot 5. Stacking of metatarsals on lateral8. Adduction-forefoot (forefoot supination)9. Varus-hindfoot10. Equinus-hindfoot

Poor Prognosis: 1. Teratogenic (Myelomeningocele, AMC, Diastrophic dwarfism) (Crawfordism—MAD)2. Short, fat, rigid foot3. Physical findings: Keel-shaped heel, Midfoot crease, Atavistic great toe4. Delay in treatment5. Failure to respond to initial surgery

Turco’s Ball and Socket Ankle:Ball = talusSocket = calcaneus, cuboid, navicular, y-bifurcate ligament

Pathologic anatomy1. Medial deviation of head and neck of talus2. Navicular medially/plantarly subluxed3. Calcaneus in varus (to articulate with abnormal talus position)4. Medial subluxation of forefoot (navicular medial and plantar)5. Calcaneus -cuboid articulation is medial to tib-fib interosseus space (medial spin)

Medial spin: Abnormal talus position causes calcaneus to spin medially (decreased Kites angle AP) Must release lateral ligaments (CFL, PTFL) to correct spin

Kites Angle: Talo-calcaneal angle on AP (Normal 20-40, <30 infers varus)

Treatment:Manipulation (70 forefoot abduction, 20 heel valgus, 10 dorsiflexion)Casting (1-1-2-2-1: 1 week, 1 week, 2 weeks, 2 weeks, 1 month)

6

Order of correction: CAVE Cavus, Adductus, Varus, EquinusMaximum lateral dorsiflexion film

If parallelism b/w calcaneus and talus then percutaneous HCLStraight or reverse last shoesPosterior medial release (most dangerous comp. =>osteotomizing talar neck looking for talo/nav joint)

Tendon lengthening: Heel cord, Post tib, FHL, FDLPosterior capsulotomy at subtalar, tibiotalar jointLigament release: Talofibular, Calcaneofibular, Talocalcaneal, Y-bifurcate (calc-cuboid, calc-nav), Spring (med plantar calc-nav)Reduction and pinning Talonavicular joint

Further treatment for partially corrected, over corrected, or recurrent clubfootSoft tissue: Anterior tibialis tendon transfer __\ corrects “pigeon toe” deform, allows foot to

Posterior tibialis tendon transfer / dorsiflex into neutral positionHeyman-Herndon-Strong tarsometatarsal capsulorrhaphy

Bony: Metatarsal osteotomies (for residual forefoot adduction)Evans calcaneal osteotomy (calcaneal-cuboid osteotomy / fusion)Dwyer calcaneal osteotomy (medial opening wedge osteotomy)Triple arthrodesisTalectomy => especially in AMC

Lower Extremity Abnormalities

Congenitally Short Femur & Fibular Hemimelia

7

Associated findings1. PFFD2. Hypoplastic lateral condyle3. Hypoplastic tibial spines (absent cruciates)4. Fibular hemimelia5. Ball and socket ankle6. Tarsal coalition7. Absent lateral rays

PFFD Aitken ClassificationA: Head present, Coxa vara, +/- Pseudarthrosis-head not articulating with acetabulumB: Head present, Pseudarthrosis at neck (Most common)C: No head, spike pointing toward acetabulumD: No head or acetabulum

Coxa VaraAutosomal DominantDefn: Shaft-Neck angle <125 degrees, metaphyseal triangleAssociated with Congenitally Short FemurHilgenreiner-Epiphyseal Angle-angle subtended through physis from Hilg line

Normal is 20. Observe from 20-60.>60 => valgus osteotomy-overcorrect to allow for growth

Tibial BowingAnterolateral -- >55% have Neurofibromatosis

Malignant - High risk of pseudarthrosisAnteromedial Bowing--Think O.I.Posteromedial bowing (Kyphoscoliosis tibia)-- Benign

Association: Calcaneovalgus deformityResidual defect is LLD

Streeter's Dysplasia Congenital constriction band syndromeTx: Staged Z-plasties

Dysplasia Epiphysialis Hemimelica (Trevor's Disease)Prevalence: 1: 1 millionM:F 3:1 Intra-articular osteochondroma which stops growing at maturity. Sx. – dec. ROM, painMultiple lesions 70% of the timeMedial side > lateral joint sideMost common in talusTreatment is aggressive surgery

Knee DisordersCongenital Knee Dislocation

Classification (Finder)

8

1. Physiologic: 20 degrees hyperextension, Resolves by age 82. Simple: Physiologic that carries into adulthood3. Subluxation: <90 degrees hyperextension, resisted flexion beyond neutral4. Dislocation, tibia ant/prox to femur5. Complex variants associated with AMC, MM

Physiologic Genu VarumNormal under age 2Association: Internal tibial torsion

Nail-Patella Syndrome-OnchoosteodysplasiaAutosomal Dominant Absence of patellae with abnormal nail bedsElbow flexion contracturesShort statureIliac wings => Fong’s Horns

Blount's DiseaseEpidemiology: Blacks, Obese, Short, Early walkersX-Ray: Fragmentation of medial tibial metaphysis

Failure of development of proximal tibial epiphysisDrennan: Metaphyseal-diaphyseal angle (>11 or 14 degrees)

Classification (Langenskiold - applies only to infantile)1. Irregularity <3yo Ignorant2. Beaking 2-4yo Butt-heads3. Depression of physis 4-6yo Don’t4. Step-off 5-10yo Study5. Two epiphyses Their6. Bony bar Books

Common Foot ProblemsCalcaneovalgus

Common (1/1000)Findings: Forefoot dorsiflexed (against tibia), Everted, Abducted

9

Tx: Stretching exercises (Casting if rigid)

FlatfeetTypes: 1. Flexible: Ligamentous laxity, No treatment

2. Rigid: Insole supports if symptomatic (R/O CVT, Tarsal coalition)

Congenital Vertical Talus (Rocker Bottom foot, Serpentine foot, Persian slipper foot)0.5:1000, associated with AMC, MMFindings: Fixed equinus of hindfoot, Fixed dorsiflexion of forefoot (Post tib/Per longus=dorsiflexors)Sine qua non=navicular is dorsally dislocated and sits on anterior head/neck of talus*X-Ray: No reduction of talonavicular joint on maximum plantarflexion view, Meary’s Angle >0

** can’t see nav until approx 5yo, so talus doesn’t line up with meta-tarsals** if talus does line up with the meta-tarsals it is called an OBLIQUE VERTICAL TALUS

Type A: Calc-cuboid locatedType B: Calc-cuboid d/lColeman/Stelling Tx:

Stage 1- Stretching, CastingStage 2- Release/lengthen anterior dorsiflexors, Triple capsulotomy, ORIF talo/nav and talo/calc with pinsStage 3- HCL, Posterior capsulotomy, Kidner procedure (Post tib transfer under navicular)

Older kids, may have to excise navicular

Zed Foot/Skew FootNavicular is lateral to talus, forefoot adduction, hindfoot valgus

Peroneal Spastic FlatfootSx: Flatfoot, Pain over lateral calfEtiology: Tarsal coalition, Trauma, JRA, Neoplasms

Tarsal CoalitionAge: 8-12 yoSx: Multiple ankle sprains, Decr subtalar ROMTypes: 1. Calcaneonavicular (Most common) pain in sinus tarsi with decr in subtalar ROM

X-Ray: Sloman's view, Anteater noseWebster/Steward Tx: BKC first => Excision w/ peroneus brevis interposition

2. TalocalcanealX-Ray: CT is best, if middle facet not horizontal => coalition

Dorsal beaking of talar neck, narrow subtalar jt, broad lateral talar processTx: BKC (walking), if still painful: Excise if <50% joint, o/w fuse (triple is last resort)

Hallux ValgusF>M, increase IMA (>100), first MTPA (>200)Tx: 1. Release adductor hallucis

2. Medial capsulotomy, Attach adductor tendon to 1st metatarsal3. Resect exostosis, Proximal opening wedge osteotomy of 1st metatarsal (medially)4. Medial MTP capsulorrhaphy

Most common complication: recurrence

IntoeingMetatarsus Adductus (Neonate to 18 months)

Etiology: Packing phenomenonDifferences from clubfoot

1. Can correct forefoot and hindfoot

10

2. Hindfoot often in valgus (not varus)3. Talocalcaneal divergence on AP X-Ray4. Talocalcaneal convergence on Lateral X-Ray

Types: I. Corrects spontaneously (Tx: Observation)II. Passively corrects (Tx: Stretching)III. Rigid (Tx: Casting, surgery)

Internal Tibial Torsion (18-36 months)Normal: Transmalleolar axis = 20 degrees of external rotation

Femoral Anteversion (3-8 years)Newborn ~ 40 degrees, Adult ~ 15-20 degreesEtiology: 1. Hereditary

2. Packing phenomenon3. Pull of iliopsoas4. Sleeping position5. TV squat position, “W” position

Types: A - Compensation: Tibia externally rotated, Valgus feetB - No compensation

Tx: Osteotomy indications: >8 yo, <10 degrees external rotation (Stehali)

Osteochondroses and Pseudoosteochondroses

Koehler's Disease (AVN of navicular)Sever's Disease (Calcaneal apophysitis)Iselin’s Disease ( Prox. 5th metatarsal apophysitis)Diaz’s Disease (Talus AVN)Pump Bump (Bursitis at tendoachilles attachment)Freiburg's Infraction (AVN of 2nd MT head)Van Neck's Disease (normal appearance in ischiopubic fusion)

11

Upper Extremity DisordersKlippel Feil Syndrome

Failure of segmentation of upper cervical spineTriad (seen <50%): Low hairline, Decreased ROM, Short neckX-Rays: look for omovertebral bone connecting spinous process to scapulaAssociated findings:

1. Scoliosis-congenital (60%) 4. Hearing (30%)2. Sprengel's (40%) 5. Synkinesia (18%) mirror-like movements3. Renal (35%) 6. Heart (14%)

TorticollisAssociations: DDH (20%)Tx: Release before 5-6 yo (Ocular righting reflex)Acquired must r/o;

AA rotatory subluxationPosterior fossa tumorsSpinal canal tumorsRetropharyngeal abscess

Sprengel's DeformityFailure of scapular descent (normal C4 to T7)Sx: Cosmesis, Decreased ROMAssociations: Scoliosis (Klippel-Feil), Torticollis, Hairlip, Renal disordersTx: most common complication is brachial plexopathy => clavicle ostectomy/morcelization to avoid

Woodward: Muscular release at spinous processesGreen: Muscular release at scapula.....Brought down.....Secured

Clavicle PseudarthrosisLocation: Right side (Think dextrocardia if on left side)

Congenital Fusion of Elbow (Boomerang Elbow)Association: Idiopathic scoliosis

Congenital Radioulnar SynostosisNo limits in function

Radial ClubhandAutosomal dominant (~1/100,000)Classification (Bayne) -- (SHIT)

I. Short radius---ShortII. Hypoplastic radius---HypoplasticIII. Partial absence---Isn't all thereIV. Total absence (most common)---Totally not there

Absence of radial artery and nerve, absent distal biceps tendonAssociations: TAR (Thrombocytopenia, Absent Radius)

Holt-Oram (cardiac septal defects)Fanconi syndrome (aplastic anemia)VATER (Vertebral, Anal atresia, Tracheo-Esophogeal, Radial)VACTERLS (Vertebral, Anal, Cardiac, Tracheo-Esoph., Renal, Limb def, Single umbilical artery)

Treatment: Bora- soft tissue release with realignment over ulna Delorme centralization procedure- metallic rod to stabilize hand over ulna

Madelung's DeformityProminence of distal ulna, abnormal ulnar aspect of distal radial physisTypes: 1. Congenital: Triangulation of proximal carpals

F>M, Usually bilateral2. Traumatic: due to distal radius fx--volar/ulnar physis growth cessation, no triangulation

Scoliosis

12

Non-StructuralEtiology: LLD, hip flexion contractures, neuromuscular imbalance, posturalFlexible on bending films, correct with supine films

Structural

WAR: Wedging, Angulation, RotationTreat WARP: Wedging, Angulation, Rotation, Progression

1. IdiopathicEpidemiology: 8-11% of children 10-13 yo

0.3% require treatment, 0.03% require surgeryFamily Hx (Risk): Mom (10x), Mom + older sister (20x), Older sister (<50x), Both parents (80x)

Complications: cosmesis, pain, neurologic, cardiopulmonary (if apex above diaphragm)Cardiopulmonary: >100-110

-Correction: No change in tidal volumes; V-Q ratio, oxygenation improved-Thoracic lordosis: Early decompensation, Mitral valve prolapse

Types: Infantile (0-3 yrs), Juvenile (3-10 yrs), Adolescent (10-maturity)Infantile: Great Britain, Left thoracic curves, RVAD < 20 good prognosis

Vertebral maturity: Fusing of vertebral apophysis (best indicator)Curve patterns: 1. Right thoracic (most common) [Worst cosmesis]

2. Thoracolumbar (second most common)3. Lumbar curve4. Double primary (R thoracic, L lumbar) [Best cosmesis]

Milwaukee brace-Indications: Curve <33 degrees, Progressive, Skeletal immaturity-Contraindications: Curves >40-45, Thoracic lordosis, Psych probs, Poor cosmesis

Surgery>45 degrees, Progressive Spinal Cord Monitoring

Risk of progression: 1. SSEP1. Lonstein table 2. MEP

Curve 5-19 20-29 3. EMGRisser 0,1 22% 68% 4. Stagnara wake-up test 2,3,4 1.6% 23%2. Weinstein table

10-12 yo 13-15 yo >16 yo<19 degrees 25% 10% 0%20-29 deg 60% 40% 10%30-59 deg 90% 70% 30%60 deg 100% 90% 70%

Kings Classification: 5 distinct idiopathic thoracic curve patternsI. L>T: T more flexibleII. T>L: L more flexible, L must cross midlineIII. T-L curve: L doesnt cross midline, apex usually at T 7/8, fuse L 1/2IV. Long T curve: L4 points into curve, L5 balanced over sacrumV. Double thoracic curve (not double major!) T1 tilts into concavity, elevation of left shoulder

Surgery tips:Fuse primary curve onlyFuse neutral (not rotated) to neutral vertebrae (Moe)Lowest vertebrae fused must also be stable (centered over lumbosacral junction)Avoid L4 and L5 when possible

2. CongenitalEtiology: Failure of Segmentation (bar) vs Formation (hemivertebrae)

13

Most progressive:1. Unilateral unsegmented bar (Most malignant)2. Multiple unilateral hemivertebrae3. Unsegmented bar with convex hemivertebrae4. Hemivertebrae w/ kyphosis

Anomalous takeoff: congenital element at LS junction with compensatory curve above 3. Neuromuscular

Treatment: Fuse to sacrum (Bracing not effective)Indications: 1. Cosmesis

2. Pain prevention3. Improve pulmonary function4. Improve function5. Improve nursing

-Curve >50 degrees in standers, >30 degrees in sitters 4. Mesenchymal

Morquio’s, Marfan’s, AMC, Dwarfs, OI, Scheuermann’sMyelodysplasia

-Fuse to sacrum (high incidence of infection, pseudarthrosis) => may stop walking-Hydromyelia: Suspect if sudden worsening curve

5. Neurofibromatosis-Short segments, Sharply angulatedTypes: 1. Dysplastic (Pseudarthrosis common after fusion-17%)

-Wedging, Rotation of apical vertebrae, Scalloping of vert bodies, Spindling of transverse processes, Enlarged foramina, Pencil ribs

2. Idiopathic3. Kyphosis: >50 degrees.....Fuse anterior and posterior

-Pseudarthrosis common 6. Trauma 7. Miscellaneous

post radiation

Kyphosis: normal is 20-40, measure from T3-T12 on lateralScheurman's Kyphosis

Def: >3 successive vertebrae with >5 degrees wedging (Sorenson's rule)Fixed deformity at pubertyTreatment:

-Four 6's (Curves < 60)1. 6 weeks - Risser2. 6 months - Milwaukee Brace3. 6 weeks - Wean (8 hours out per day)4. 6 months - Nighttime brace

-Surgery1. Kyphosis >70 deg2. Wedging >10 deg3. Pain4. Neurologic findings5. Progressive deformity

Juvenile RoundbackFlexible, treatment is postural exercises

14

Congenital KyphosisTypes: I. Failure of formation

II. Failure of segmentationIII. Mixed

Treatment: Early PSF (<3 yo if rapidly progressive) Halo-femoral traction contraindicated => high risk of paralysis

Spondylolysis/SpondolisthesisPopulation: gymnasts, football players, weight-lifters, divers, eskimo’s (Never in non-ambulators)Sx: Low back/buttock/thigh pain, Tight hams, Waddling gait, Neuro findingsEtiology: Hammer (Inf. facet of L4), Anvil (Sup. sacral facet) creates fx/defect in pars interarticularis (usually L5)Classification (Wiltse-Newman-Macnab 1976) Classification of degree of slip (Meyerding):

I. Dysplastic (Congenital, 94% Spina bifida occulta) Grade I: 0-25%II. Isthmic (elongated pars) Grade II: 25-50%III. Traumatic Grade III: 50-75%IV. Degenerative Grade IV: 75-100%V. Pathologic Grade V: >100% => spondyloptosis

Anatomy (Scotty Dog): x-ray = Napolean’s hat signFace: Transverse process Body: laminaEye: Pedicle Front leg: Inferior facetEar: Superior facet Hind leg: Transverse processNeck: Pars interarticularis Tail: Spinous process

Nerve root involvement: Isthmic type – L5, Dysplastic type – S1Diastematomyelia

Epidemiology: F:M 4:1, lesion of spinal column which protrudes from posterior midline dividing cord (usually L1-L3)

Suspicion: 1. Congenital scoliosis2. Neuromuscular Def (Neuro. bladder, Gait abnorm., Uni. Clubfoot / Cavus / Flatfoot)3. Cutaneous abnormality – hairy patch4. X-Ray: Widened inter-pedicular distance

Midline spike Narrow disk space

MyelomeningoceleLevel: Lowest root level of active muscleIncreased incidence of latex allergy and UTIAmbulatory status (Hoffer): Community

Household Non-functional (In gym/PT only) Non-walker

Factors affecting ambulation1. Extent of paralysis 5. Hydrocephalus2. Spinal deformity 6. Intelligence3. Hip instability 7. Home environment4. Obesity

Foot: Valgus deformity often truly ankle valgus (get AP of ankle if fib physis above mortise = ankle valgus)Hip: Must have strong quads (lesion L3 or lower) before surgically address hip dislocation

Surgery: External oblique transfer to greater trochanter => improves sitting balance, trunk stabilityMustard: Iliopsoas transfer through anterior ilium notch to greater troch

(Increased abductor power)Sharrard: Iliopsoas transfer through post-medial ilium to greater troch

(Increased abductor & extensor power)Lower extremity management

15

< 2 yo: StretchingNo surgery until able to sit independently

>2 yo: Put in standing braces when able to sit independentlySpine: 80% have scoliosis

consider ant/postfuse to sacrum

Galveston: rods between inner/outer tablesWarner: through S1 foramenDunn/McCarthy: place bars over sacrum anteriorly

Sacral AgenesisMother always has insulin dependent diabetes mellitusTypes: Renshaw classification (Type 2 most common)

1. Partial or total unilateral absence of sacral elements2. Complete loss of lower sacral/coccygeal elements, stable articulation between ilia3. L5/S1 non-union, no sacrum, ilia articulate with the sides of the lowest vertebra present4. No L5/S1 junction, no sacrum, caudal end plate of the lowest vertebra fused to ilia or an iliac

amphiarthrosis

Muscular DystrophyDuchenne s

Features:1. Age 2-7, stop walking avg. 8-10yo2. Sex-linked recessive3. Clumsy gait (Gowers sign - use hands to stand up from floor)4. Pseudohypertrophy of calves – 80% of patients5. Weak proximal muscle masses (Shoulder and Pelvic girdle muscles affected first)6. Hip flexion, Iliotibial band/Tensor fascia, knee flexion, foot plantar flexion contractures

[Hip and knee extension lag test: >90 deg contracture then release]7. Lumbar lordosis (Protuberant abdomen)8. Posterior tib. tendon tends to retain power over long period of time

Histology: (Do not biopsy at previous EMG site)1. Muscle fibers vary in size2. Central nuclei3. Signet ring (infiltration of fat), Chicken-wire fence [End stage disease]

Functional Assessment Scale (10 parts, based on walking, stair climbing...)Scoliosis

Types: 1. J-type: Most common, Windswept hips2. Z-type: Pelvis OK, Translation at L-S junction

Treatment: Surgery if curve >30 deg in sitter (May cease walking)Becker's

Tend to live longer

16

Sx: Chest protuberance, Scapular winging, Calf hypertrophySpinal Muscular Atrophy (Auto Recessive)

Anterior horn cell degenerationClassification:

I. Infantile (<12 mos), Oppenheimers Dz, Floppy Baby, no sit or walk, die ~4 yoII. Intermediate (3-18 mos), Werding-Hoffmans, sit OK, no walk, live >4 yoIII. Juvenile (>2 yo), “Kugleburg-Weidlander”, can sit w/ support, may walk, live to adult

Fascioscapulohumeral Dystrophy (Landouze-Dejerine MD)Autosomal DominantFindings: 1. Upper extremity weakness

2. Scapular winging3. Increased trapezius mass4. Popeye forearms5. Can't blow up balloon, puff cheeks, purse lips, or whistle

5. deltoids usually spared

DwarfismAchondroplastic Dwarfism

Characteristics:1. Short stature 7. Genu varum2. Paddle-shaped ribs 8. Coxa valga3. Alphabet block vertebrae 9. Trident hands4. Narrowed inter-pedicular space 10. Frontal bossing5. Lumbar kyphosis 11. Saddle shaped nose6. Horizontal sacrum 12. Short mid-face

Pseudo-achondroplasiaCharacteristics:

1. Short limb 5. Odontoid hyperplasia2. Normal head 6. Epiphysis and Metaphysis affected3. Flame vertebra (platyspondyly) 7. Joint hyperlaxity4. Normal interpedicular distance

Ellis van CrevaldCharacteristics:

1. Short stature2. Poor dentition3. 6 digit hands

17

4. Immunodeficiencies

Diastrophic DwarfismCharacteristics

1. Short limbs2. Scoliosis3. Hitchiker thumb – short 1st metacarpal4. Cauliflower ears5. Equinovarus6. Flexion contractures7. Usually distal femoral epiphysis not ossified at birth

Metabolic DisordersOsteopetrosis (Marble-bone Disease, Marie-Alber-Schoenberg Dz)

Osteoclast disorder, don’t respond to PTH tx.X-ray: Erlenmeyer flask, Rugger jersey spineAssociations: Anemia, Immunodeficiency => BMT to cure

RicketsTypes:

1. Vitamin D Deficient (Dietary rickets)-Rare-Age 6 mos - 3 yrs-Low Vit D > Decr Ca, PO4 absorption > Incr PTH (Low-nl Ca, Low PO4)-Associations: Prematurity, Hyperalimentation, Anti-convulsants (Compete

with Vit D uptake)-Sx: Weakness, Lethargy, Bowlegs, Protuberant abdomen, Cranio tabes (large

forehead), Rachitic rosary (large osteochondral rib articulation)-Tx: Vit D, Calcium

2. Vitamin D Resistant (Renal tubular insufficiency, Familial hypophosphatemic rickets)-X-linked dominant (F:M.....2:1)-Impaired renal reabsorption of PO4 (GFR normal) > Low PO4, Nl Ca, incr alkphos-Impaired response to Vitamin D-Sx: Bowing, Frontal bossing, Incr lumbar lordosis, Poor teeth, Short-Tx: Phospate, Vitamin D3 (High dose, Stop before surgery)

3. Renal Insufficiency (Chronic renal failure)-tx = high dose Vit D, dialysis => kid txp

18

X-Ray: Widened physisMetaphyseal flaring (Trumpeting)Physeal cupping, Serrated metaphyseal edgeLoosers lines (Fracture on compression side of bone)Milkmans fracture (Pseudofracture)

Osteogenesis ImperfectaDefect in collagen synthesis – Type 1 collagen-(alpha 2)Classification (Sillence):

I. AD, Blue sclera, hearing loss, most common, most mild (Familial)IA. Dentinogenesis absentIB. Dentinogenesis presentII. AR or AD, Blue sclera (perinatal fatality)III. AR, Normal sclera, fractures at birth, progressive and deforming (Auto Recessive)IV. AD, Normal sclera, bowing of long bones, improves at puberty, normal hearing (Sporadic)IVA: nl teethIVB: Dentinogenesis imperfecta

Surgery: Sofield-Miller (Shish-kebob)Correct scoliosis if curve > 45, use PMMA to augment and segmental fusion techniquesIncreased risk for malignant hyperthermia

Ehlers-Danlos SyndromeDefect in collagen metabolismSx: Skin hyperextensibility (Cigarette paper skin), Joint hypermobility, Bruisability, Soft tissue

calcification, Bone fragility, Bursa formation, OsteopeniaMany types

MucopolysaccharidosisFeatures:

1. Proportionate short stature2. Anterior beaking of vertebrae (platyspondyly)3. Bullet-shaped metacarpals4. Wide pelvis

Types: 1. Hunter 2. Hurler 3. Morquio 4. San-Filippo 5. Scheie 6. Maroteaux-Lamy 7. Sly

HypothyroidismCongenital (Cretinism)

F>MDwarfism, Mental retardation, Osteopatia cretinoidia (~Perthes)

AcquiredSCFE

ScurvyAge 6-12 mosSx: Decr appetite, Slow wt gain, Bleeding gums, Subperiosteal hemorrhageX-Ray: Wimbergers ring (density around cartilaginous ossific nucleus)[Subperiosteal hemorrhage Differential: Scurvy, Menkes Kinky Hair Syndrome, Abuse, Neglect]

Wormian bonesC leidocranial dysostosis – retarded ossification of membranous as well as cartilaginous precursors of bone. Congenital absence of clavicles combined with softness of the calvarium.H ypothyroidismO steogenesis ImperfectaM einkes Kinky Hair Syndrome

19

P yknodysostosis – form of osteopetrosis showing short stature, separated cranial sutures, delayed closure of fontanelles and hypoplasia of the terminal phalanges.

Helmet HeadsO.I.Frontometaphyseal DysplasiaDentoagenesis

Neurofibromatosis: Tumors of neural crest/mesodermal originTypes: 1. NF-1 (von Recklinghausen, Peripheral NF) => chromosome 17- (long arm)

2. NF-2 (Central NF, Acoustic neuroma) => chromosome 22Diagnosis for NF1: (2 out of 7)

1. Cafe-au-lait spots: Five >5mm (child), Six > 1.5cm (adult)2. Neurofibroma (2) or Plexiform (1)3. Freckles (axillary, groin)-Crowe Sign4. Osseous lesions- sphenoid dysplasia or thinning of cortex of a long bone (+/- pseudarthrosis)5. Optic glioma6. Lisch nodules7. Primary relative with NF

Findings: 1. Spinal deformity (Scoliosis, Kyphosis, or both) 23.6% of patients (Most common bone abnormality) Short segmented, Sharply angulated

2. Tibial pseudarthrosis (congenital tibial dysplasia): Antero-lateral bow-5.7%Types I: Dense cortex, Canal OK [Observe]

IIA: Failure of tubulation [Brace]IIB: Cystic lesion or pre-fracture [Bone graft]IIC: Frank Fracture- “sucked candy” appearance of bone[Bone graft +/- Amputate]

Other Classifications of congenital pseudarthrosis of the tibia: Incidence 1/190,000 M>FBoyd AndersonI. Fx present at birthII. Hourglass constriction DysplasticIII. Bone cysts CysticIV. Sclerotic segment ScleroticV. Dysplastic fibula FibularVI. Intra-osseous NF Clubfoot or congenital band type

3. Hemihypertrophy - 1.4%(Subperiosteal bone proliferation) 4. Bone erosions: Secondary to pressure from neurofibroma (Dumbell lesion in vertebrae)

20

5. Neoplasias (Leukemia, Wilms tumor, Rhabdomyosarcoma (UG tract)) 6. Pectus deformity – 4.3%

7. Plexiform neurofibroma 25% 8. LLD – 7.1%

Diagnosis for NF2: (need 1)1. Bilateral CN VIII neuromas2. 1 relative with NF2

GaitDeterminants of Gait:

1. Pelvic rotation2. Pelvic tilt3. Knee flexion after heel strike in stance4. Foot and ankle motion5. Knee motion6. Lateral Displacement of Pelvis

Limb LengtheningIndication: 8-12 yo, Discrepancy > 4cmWagner: open corticotomy, distract, plate, bring back later and bone graftIlizarov: perc corticotomy (don’t violate medullary canal), ring fixator, wait 5-7 days, distract 1mm/dayDe Bastiani; same as Ilizarov, except use unilateral ex-fix (OrthoFix)

TumorEpidemiology: Benign - 3 most common -> UBC, Osteochondroma, NOF

Malignant - 3 most common -> Osteosarcoma > Ewings > RhabdomyosarcomaWork-up: H&P, X-Ray, Bone scan, CT, MRI, Angiogram, Lung CTEnneking Staging 5 yr Survival

IA Low grade Intra-comp No mets 97%IB Low grade Extra-comp No mets 89%IIA High grade Intra-comp No mets 73%IIB High grade Extra-comp No mets 45%III Mets 8%

Osteochondroma: Most common tumor--failure of differentiation/tubulation at prox metaphysisMultiple hereditary exostosis (Ehrenfried’s Dz)

Autosomal dominantShort statureX-ray: trabeculae flow into lesion, lesions point away from physis

Tx: Excise if - Limit ROM, Pain, Neurovascular compromiseMalignant degeneration: 5-15%

EnchondromaUnilateral, MonomelicOlliers Dz (Multiple enchondroma)--Maffucis Syndrome (Ollier's+Hemangioma->risk of malignancy)

Unicameral Bone Cyst--usually in proximal humerus (fallen leaf sign)Type: Active (w/in 1 cm of physis), Latent (>1cm from physis)Tx: Aspiration, Steroid injection (Scaglietti), possible Nancy nail

Aneurysmal Bone CystMultiple loculations with septaX-Ray: Cyst is wider than metaphysisHisto: vascular lakes, with giant cell laden epithelized septa

Osteoid Osteoma--common in prox fem metaphysis (calcar), and posterior elements of the spinePea in a pod” sclerotic nidus <1cmSymptoms: night pain relieved by ASA, exacerbated by EtOH

21

Histiocytosis (EG)EG of spine - Vertebrae plana (platyspondyly) aka Calves vertebraeHans-Schuller-Christian Dz: Histiocytosis, Skin & Bone...., Pituitary (DI, Exophthalmos)Letterer-Siwe Dz: HSC Dz w/ MR, Cystic manifestations

Fibrous DysplasiaFailure of complete remodeling of immature bone, Shepherd’s Crook in prox femurCafe-au-lait spots: Coast of Maine [Neurofibromatosis: Coast of California]Albrights Syndrome: 1. Fibrous dysplasia (“Shepherds crook” deformity if in hip) + 2.

Endocrinopathies (precocious puberty) + 3. Cafe-au-lait spotsEwing s

Age 10-30 yoSx: Pain, Mass, Tenderness, Systemic sx (fever)Location: Diaphyseal, Femur #1, in pts >20yo, flat bones more commonX-Ray: Onion skinning due to periosteum trying to wall off soft tissue expansionHisto: + PAS, monotonous sheets of round cells

OsteosarcomaSx: Swelling, Tenderness, Decr ROM, Warmth, Pathologic fxDifferential Dx: Infection, Ewings, Metabolic disease, Traumatic, Congenital lesion

Chemo: MAP: methotrexate, adriamycin, platinum) OR COMPAdra: cytoxin, O(vincristine), methotrexate, prednisone, adriamycin

COMMON IN THE SPINE--ant elements: EG, Hemangioma post elements:Osteoid Osteoma, Osteoblastoma, ABCGrowth Plate

Zones: Resting - Lipid/glycogen/proteoglycan production, Low oxygen tension- Lysosomal storage diseases

Proliferative -Align longitudinally, Chondrocytes multiply, Linear growth-Achondroplasia

Hypertrophic -Cells increase 5x, Matrix preparation, Calcification-Rickets, Enchondromas, Mucopolysaccharidosis, Fractures

MaturationDegenerationProvisional Calcification

Most common fractures:SH I Distal fibulaSH II Distal radiusSH III Distal tibiaSH IV Distal femur

Growth:Lower Extremity: Proximal femur 10

Distal femur 40Proximal tibia 30Distal tibia 20

Individual Bones: Prox. Humerus 80% Prox. Femur 30%Dist. Humerus 20 Dist. Femur 70Prox. Radius 25 Prox. Tibia 57Dist. Radius 75 Dist. Tibia 43Prox. Ulna 20 Prox. Fibula 60Dist. Ulna 75 Dist. Fibula 40

Ossification sequences:Ankle: Middle (at Poland’s hump), Medial, LateralElbow:

Appearance: Closure:Come Rub My Tool Of Love TLC OR ME

22

Capitellum 6 months TrochleaRadial head 2 yrs Lateral epicondyle 13 yrsMedial epicondyle 4 CapitellumTrochlea 6Olecranon 8 Olecranon 15 yrsLateral epicondyle 10 Radial head

Medial Epicondyle 17 yrsEpiphyseal Centers present at birth: Full Term Children Have These Centers

1. Distal femur 2. Proximal tibia3. Calcaneous4. Proximal humerus5. Talus6. Cuboid

Hemoglobinopathies (Sickle Cell Disease)Sx: Small size (due to persistent anemia, or decr O2 at growth plate)Osseous manifestations:

1. Marrow hyperplasia2. Infarction3. Growth disturbances4. Osteomyelitis5. Pathologic fracture

X-Ray: Vertebrae - Most common involved bone, Cup-like biconcavity in central endplate -Step-deformitySkull - Thick cortex, Widened diploe, Porcupine quill, Hair-on-endJoints - Bone crisis, Bone infarcts

Hip: AVN, Osteochondritis, Coxa varaDifferential Dx: Perthes (SS = Older [age > 10yo], Black)Chung/Ralston classification

Group 1: Total head involvement ~ PerthesGroup 2: Localized involvement ~ OCDGroup 3: Late changes

Hand-Foot SyndromeAge < 2yoSx: Painful soft tissue swelling of hands/feetX-Ray: Periosteal new bone, Lytic lesions

OsteomyelitisSx: ~ Bone crisisOrganism: Salmonella

Juvenile Rheumatoid Arthritis (Stills Disease)Sx: Fever, Rash, Anemia, Splenomegaly, Multiple joint involvementAge: < 6yo, 10-15 yoDx: Synovitis of joint (Knee, ankle, elbow, wrist) > 6 weeksTypes: Monoarticular, Pauciarticular (2-4 joints), Polyarticular (>4 joints)

23

Septic ArthritisEpidemiology: Age 1-3yo

Organisms: Staph A., Streptococcus, Pneumococcus, H. flu (#1 <6 mo)Diagnosis: Joint fluid (Incr WBC [>35,000], Inc protein, Dec glucose[40 < blood Glc])Diff Dx (Septic hip):

1. Osteomyelitis 4. Iliopsoas abscess 7. Perthes2. Toxic synovitis 5. JRA3. Diskitis 6. Rheumatic fever

Sequelae (Septic hip):1. Dislocation of hip 4. Coxa magna2. Osteomyelitis of fem neck 5. Coxa vara3. AVN 6. Leg length discrepancy

Poor Prognosis:1. Delay in diagnosis (>5 days)2. Hip joint3. Associated osteomyelitis4. Younger age (diagnosis difficult in infants)

OsteomyelitisSeasonal (Spring, Fall)Etiology: 1. Hematogenous

2. Direct extension3. External

Organisms: Staph A., H. Flu (infants), Salmonella (Sickle cell)Location: Femur > Tibia > Humerus

Metaphysis (Sludging of blood flow in sinusoidal vessels)Differential Diagnosis:

1. Septic arthritis 5. Blood dyscrasias2. Cellulitis 6. Tumor3. Syphilis 7. Hypervitaminosis A4. Sickle cell crisis 8. Caffes Dz (Infantile cortical hyperostosis)

X-Ray: 7-10 days: Soft tissue swelling> 10 days: Lucency or Opacity

Sequestrum (localized area of dead bone) & Involucrum (periosteal new bone formation)

24

Complications:1. Bone overgrowth 2. Growth Arrest 3. Pathologic Fracture4. Amyloidosis 5. Epidermoid Carcinoma

Treatment: 3 weeks IV ABX, then PO ABX until ESR returns to normal; Immobilization-Sickle cell pts: Serial aspirations (No I&D due to risk of Staph wound infxn)

DiskitisSx: Refusal to walk (<3 yo), Abdominal pain (3-9 yo), Back pain (>9 yo)X-Ray: Crosses disk space

THIS FILE SAVED IN MICROSOFT WORD UNDER PEDIATRIC ORTHOPAEDIC REVIEWPlease continue to add and update this document.Last revised 7/02

25