ORTHOPEDIC REVIEW



ORTHOPEDIC REVIEW

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Dolor, Tumor, Calor, Rubor(STICKOR

Cerebral Palsy

Definition: non-progressive neurologic disorder in infants R

Ant/lat displacement of the femoral neck on the head-head stays located in acetabulum

Present as pain in knee with limited IR/ER--mom brings kid in because of limp

Body types: Tall, thin athletic male (rare)

Hypogonadal, obese, delayed sexual maturity

Etiology theories:

1. S ynovial hypervascularity (increased height of growth plate) slip through zone of Hypertrophy

2. 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 fail

Loder Classification:

1. Acute: 3 weeks, limp Unstable(can’t walk on it) two pins

3. Chronic: most common ~47+% AVN rate (Loder et al)

Stages: Preslip-x-ray-widening of physis, +/- Blush sign

Minimal slip (2/3 head)

Lovell/Winter Classification: I (0-33%), II (33-50%), III (>50%)

X-ray: Shenton’s line, Klein’s 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. chronic

Open/closed growth plates

Severity

Systemic diseases

Treatment: 1. cast

2. percutaneous pins (Stable--can walk on it--one pin, Unstable--can’t walk on it--two pins)

3. bone-peg epiphysiodesis

4. osteotomies (Southwick, Sutherland, Sugioka)--valgus and rotation

5. sex hormone manipulation--not recommended

6. 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 anyone

Cause early closure of physis

Prevent AVN/chondrolysis/DJD

Increase 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

Perthes Disease

Epidemiology: 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 hips

2. Multiple Epiphyseal Dysplasia

3. Spondyloepiphyseal Dysplasia – spine involved--platyspondyly

If black think HgbSS

Etiology: Protein C & S or AT-III deficiencies, high lipoprotein a, hemophilia A/B,

secondary to trauma, other vascular diseases, exposure to cigarette smoking

Most commonly presents as PAINLESS limp

Classifications:

Goff-Aegeter-Ferguson---WAFR

I: 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 formation

IV: Resolution (Normal, Coxa magna/plana/vara/breva, gr. troch overgrowth esp if 50% Lateral pillar height remains => if 6yo=operate

C: operate

Conway (bone scan stages-“biologic”)

I: total lack of uptake

II: lateral column uptake

III: gradual fill of ant/lat epiphysis

IV: base fill in epiphysis adjacent to physis

Sequence of events: (Salter) VCRR TOPS

1. Vascular compromise (see etiologies above) \

2. Cessation of growth of bony epiphysis _____ \ potential Perthes

3. Revascularization (from periphery) /

4. Resumption of ossification which is fragile /

5. Trauma leads to pathologic fracture (onset of True Perthes)

6. Onset of resorption

7. Plastic bone replacement

8. Subluxation....Residual deformity

Head at Risk

1. Gage’s Sign (lateral V-shaped lucency on the ossific center)

2. Lateral calcification (represents fracture through epiphysis extending into physis)

3. Subluxation (worst)

4. 4. Metaphyseal cysts

5. 5. Horizontal physis

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

Evaluation

Center-Edge angle

Center of femoral head ....Vertical line through this point.....Line from center of head to lateral edge of acetabulum

Normal is 20-40(

Pathologic F, 30-40% bilateral

Etiology: PAAAG

1. Packing phenomenon

2. Arrested fetal development

3. Abnormal tendon insertions

4. Abnormal talus rotation

5. Germ plasm defect

Associations: PB PB MAD LSO

1. PFFD

2. Bifurcated femur

3. Pierre-Robin Syndrome – Micrognathia with glossoptosis (X-linked recessive)

4. Bilateral uni-digit foot

5. Myelodysplasia

6. AMC (Arthrogryposis)

7. Diastrophic dwarfism

8. 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 findings

1. Shorter leg 1. Forefoot adduction

2. Smaller calf 2. Parallelism of talo/calc on lat max

3. Tibia internally rotated (? Internal tibial torsion) dorsiflexion

4. 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 lateral

8. Adduction-forefoot (forefoot supination)

9. Varus-hindfoot

10. Equinus-hindfoot

Poor Prognosis: 1. Teratogenic (Myelomeningocele, AMC, Diastrophic dwarfism) (Crawfordism—MAD)

2. Short, fat, rigid foot

3. Physical findings: Keel-shaped heel, Midfoot crease, Atavistic great toe

4. Delay in treatment

5. Failure to respond to initial surgery

Turco’s Ball and Socket Ankle:

Ball = talus

Socket = calcaneus, cuboid, navicular, y-bifurcate ligament

Pathologic anatomy

1. Medial deviation of head and neck of talus

2. Navicular medially/plantarly subluxed

3. 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 Kite’s angle AP)

Must release lateral ligaments (CFL, PTFL) to correct spin

Kite’s Angle: Talo-calcaneal angle on AP (Normal 20-40, osteotomizing talar neck looking for talo/nav joint)

Tendon lengthening: Heel cord, Post tib, FHL, FDL

Posterior capsulotomy at subtalar, tibiotalar joint

Ligament 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 clubfoot

Soft tissue: Anterior tibialis tendon transfer __\ corrects “pigeon toe” deform, allows foot to Posterior tibialis tendon transfer / dorsiflex into neutral position

Heyman-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 arthrodesis

Talectomy => especially in AMC

Lower Extremity Abnormalities

Congenitally Short Femur & Fibular Hemimelia

Associated findings

1. PFFD

2. Hypoplastic lateral condyle

3. Hypoplastic tibial spines (absent cruciates)

4. Fibular hemimelia

5. Ball and socket ankle

6. Tarsal coalition

7. Absent lateral rays

PFFD Aitken Classification

A: Head present, Coxa vara, +/- Pseudarthrosis-head not articulating with acetabulum

B: Head present, Pseudarthrosis at neck (Most common)

C: No head, spike pointing toward acetabulum

D: No head or acetabulum

Coxa Vara

Autosomal Dominant

Def’n: Shaft-Neck angle 60( => valgus osteotomy-overcorrect to allow for growth

Tibial Bowing

Anterolateral -- >55% have Neurofibromatosis

Malignant - High risk of pseudarthrosis

Anteromedial Bowing--Think O.I.

Posteromedial bowing (“Kyphoscoliosis tibia”)-- Benign

Association: Calcaneovalgus deformity

Residual defect is LLD

Streeter's Dysplasia Congenital constriction band syndrome

Tx: Staged Z-plasties

Dysplasia Epiphysialis Hemimelica (Trevor's Disease)

Prevalence: 1: 1 million

M:F 3:1

Intra-articular osteochondroma which stops growing at maturity. Sx. – dec. ROM, pain

Multiple lesions 70% of the time

Medial side > lateral joint side

Most common in talus

Treatment is aggressive surgery

Knee Disorders

Congenital Knee Dislocation

Classification (Finder)

1. Physiologic: 20 degrees hyperextension, Resolves by age 8

2. Simple: Physiologic that carries into adulthood

3. Subluxation: Fong’s Horns

Blount's Disease

Epidemiology: Blacks, Obese, Short, Early walkers

X-Ray: Fragmentation of medial tibial metaphysis

Failure of development of proximal tibial epiphysis

Drennan: Metaphyseal-diaphyseal angle (>11 or 14 degrees)

Classification (Langenskiold - applies only to infantile)

1. Irregularity 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 located

Type B: Calc-cuboid d/l

Coleman/Stelling Tx:

Stage 1- Stretching, Casting

Stage 2- Release/lengthen anterior dorsiflexors, Triple capsulotomy, ORIF talo/nav and talo/calc with pins

Stage 3- HCL, Posterior capsulotomy, Kidner procedure (Post tib transfer under navicular)

Older kids, may have to excise navicular

Zed Foot/Skew Foot

Navicular is lateral to talus, forefoot adduction, hindfoot valgus

Peroneal Spastic Flatfoot

Sx: Flatfoot, Pain over lateral calf

Etiology: Tarsal coalition, Trauma, JRA, Neoplasms

Tarsal Coalition

Age: 8-12 yo

Sx: Multiple ankle sprains, Decr subtalar ROM

Types: 1. Calcaneonavicular (Most common) pain in sinus tarsi with decr in subtalar ROM

X-Ray: Sloman's view, Anteater nose

Webster/Steward Tx: BKC first => Excision w/ peroneus brevis interposition

2. Talocalcaneal

X-Ray: CT is best, if middle facet not horizontal => coalition

Dorsal beaking of talar neck, narrow subtalar jt, broad lateral talar process

Tx: BKC (walking), if still painful: Excise if M, increase IMA (>100), first MTPA (>200)

Tx: 1. Release adductor hallucis

2. Medial capsulotomy, Attach adductor tendon to 1st metatarsal

3. Resect exostosis, Proximal opening wedge osteotomy of 1st metatarsal (medially)

4. Medial MTP capsulorrhaphy

Most common complication: recurrence

Intoeing

Metatarsus Adductus (Neonate to 18 months)

Etiology: Packing phenomenon

Differences from clubfoot

1. Can correct forefoot and hindfoot

2. Hindfoot often in valgus (not varus)

3. Talocalcaneal divergence on AP X-Ray

4. 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 degrees

Etiology: 1. Hereditary

2. Packing phenomenon

3. Pull of iliopsoas

4. Sleeping position

5. TV squat position, “W” position

Types: A - Compensation: Tibia externally rotated, Valgus feet

B - No compensation

Tx: Osteotomy indications: >8 yo, M, Usually bilateral

2. Traumatic: due to distal radius fx--volar/ulnar physis growth cessation, no triangulation

Scoliosis

Non-Structural

Etiology: LLD, hip flexion contractures, neuromuscular imbalance, postural

Flexible on bending films, correct with supine films

Structural

WAR: Wedging, Angulation, Rotation

Treat WARP: Wedging, Angulation, Rotation, Progression

1. Idiopathic

Epidemiology: 8-11% of children 10-13 yo

0.3% require treatment, 0.03% require surgery

Family Hx (Risk): Mom (10x), Mom + older sister (20x), Older sister (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 curve

4. Double primary (R thoracic, L lumbar) [Best cosmesis]

Milwaukee brace

-Indications: Curve 40-45, Thoracic lordosis, Psych probs, Poor cosmesis

Surgery

>45 degrees, Progressive Spinal Cord Monitoring

Risk of progression: 1. SSEP

1. Lonstein table 2. MEP

Curve 5-19 20-29 3. EMG

Risser 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

T: T more flexible

II. T>L: L more flexible, L must cross midline

III. T-L curve: L doesn’t cross midline, apex usually at T 7/8, fuse L 1/2

IV. Long T curve: L4 points into curve, L5 balanced over sacrum

V. Double thoracic curve (not double major!) T1 tilts into concavity, elevation of left shoulder

Surgery tips:

Fuse primary curve only

Fuse 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. Congenital

Etiology: Failure of Segmentation (bar) vs Formation (hemivertebrae)

Most progressive:

1. Unilateral unsegmented bar (Most malignant)

2. Multiple unilateral hemivertebrae

3. Unsegmented bar with convex hemivertebrae

4. 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 prevention

3. Improve pulmonary function

4. Improve function

5. Improve nursing

-Curve >50 degrees in standers, >30 degrees in sitters

4. Mesenchymal

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

Myelodysplasia

-Fuse to sacrum (high incidence of infection, pseudarthrosis) => may stop walking

-Hydromyelia: Suspect if sudden worsening curve

5. Neurofibromatosis

-Short segments, Sharply angulated

Types: 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. Idiopathic

3. 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 lateral

Scheurman's Kyphosis

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

Fixed deformity at puberty

Treatment:

-Four 6's (Curves < 60)

1. 6 weeks - Risser

2. 6 months - Milwaukee Brace

3. 6 weeks - Wean (8 hours out per day)

4. 6 months - Nighttime brace

-Surgery

1. Kyphosis >70 deg

2. Wedging >10 deg

3. Pain

4. Neurologic findings

5. Progressive deformity

Juvenile Roundback

Flexible, treatment is postural exercises

Congenital Kyphosis

Types: I. Failure of formation

II. Failure of segmentation

III. Mixed

Treatment: Early PSF ( high risk of paralysis

Spondylolysis/Spondolisthesis

Population: gymnasts, football players, weight-lifters, divers, eskimo’s (Never in non-ambulators)

Sx: Low back/buttock/thigh pain, Tight hams, Waddling gait, Neuro findings

Etiology: 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 sign

Face: Transverse process Body: lamina

Eye: Pedicle Front leg: Inferior facet

Ear: Superior facet Hind leg: Transverse process

Neck: Pars interarticularis Tail: Spinous process

Nerve root involvement: Isthmic type – L5, Dysplastic type – S1

Diastematomyelia

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

Suspicion: 1. Congenital scoliosis

2. Neuromuscular Def (Neuro. bladder, Gait abnorm., Uni. Clubfoot / Cavus / Flatfoot)

3. Cutaneous abnormality – hairy patch

4. X-Ray: Widened inter-pedicular distance

Midline spike

Narrow disk space

Myelomeningocele

Level: Lowest root level of active muscle

Increased incidence of latex allergy and UTI

Ambulatory status (Hoffer): Community

Household

Non-functional (In gym/PT only)

Non-walker

Factors affecting ambulation

1. Extent of paralysis 5. Hydrocephalus

2. Spinal deformity 6. Intelligence

3. Hip instability 7. Home environment

4. 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 stability

Mustard: 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

< 2 yo: Stretching

No surgery until able to sit independently

>2 yo: Put in standing braces when able to sit independently

Spine: 80% have scoliosis

consider ant/post

fuse to sacrum

Galveston: rods between inner/outer tables

Warner: through S1 foramen

Dunn/McCarthy: place bars over sacrum anteriorly

Sacral Agenesis

Mother always has insulin dependent diabetes mellitus

Types: Renshaw classification (Type 2 most common)

1. Partial or total unilateral absence of sacral elements

2. Complete loss of lower sacral/coccygeal elements, stable articulation between ilia

3. L5/S1 non-union, no sacrum, ilia articulate with the sides of the lowest vertebra present

4. No L5/S1 junction, no sacrum, caudal end plate of the lowest vertebra fused to ilia or an iliac amphiarthrosis

Muscular Dystrophy

Duchenne’s

Features:

1. Age 2-7, stop walking avg. 8-10yo

2. Sex-linked recessive

3. Clumsy gait (Gower’s sign - use hands to stand up from floor)

4. Pseudohypertrophy of calves – 80% of patients

5. 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 size

2. Central nuclei

3. 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 hips

2. 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

Sx: Chest protuberance, Scapular winging, Calf hypertrophy

Spinal Muscular Atrophy (Auto Recessive)

Anterior horn cell degeneration

Classification:

I. Infantile (4 yo

III. Juvenile (>2 yo), “Kugleburg-Weidlander”, can sit w/ support, may walk, live to adult

Fascioscapulohumeral Dystrophy (Landouze-Dejerine MD)

Autosomal Dominant

Findings: 1. Upper extremity weakness

2. Scapular winging

3. Increased trapezius mass

4. Popeye forearms

5. Can't blow up balloon, puff cheeks, purse lips, or whistle

5. deltoids usually spared

Dwarfism

Achondroplastic Dwarfism

Characteristics:

1. Short stature 7. Genu varum

2. Paddle-shaped ribs 8. Coxa valga

3. Alphabet block vertebrae 9. Trident hands

4. Narrowed inter-pedicular space 10. Frontal bossing

5. Lumbar kyphosis 11. Saddle shaped nose

6. Horizontal sacrum 12. Short mid-face

Pseudo-achondroplasia

Characteristics:

1. Short limb 5. Odontoid hyperplasia

2. Normal head 6. Epiphysis and Metaphysis affected

3. Flame vertebra (platyspondyly) 7. Joint hyperlaxity

4. Normal interpedicular distance

Ellis van Crevald

Characteristics:

1. Short stature

2. Poor dentition

3. 6 digit hands

4. Immunodeficiencies

Diastrophic Dwarfism

Characteristics

1. Short limbs

2. Scoliosis

3. Hitchiker thumb – short 1st metacarpal

4. Cauliflower ears

5. Equinovarus

6. Flexion contractures

7. Usually distal femoral epiphysis not ossified at birth

Metabolic Disorders

Osteopetrosis (Marble-bone Disease, Marie-Alber-Schoenberg Dz)

Osteoclast disorder, don’t respond to PTH tx.

X-ray: Erlenmeyer flask, Rugger jersey spine

Associations: Anemia, Immunodeficiency => BMT to cure

Rickets

Types:

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

X-Ray: Widened physis

Metaphyseal flaring (Trumpeting)

Physeal cupping, Serrated metaphyseal edge

Looser’s lines (Fracture on compression side of bone)

Milkman’s fracture (Pseudofracture)

Osteogenesis Imperfecta

Defect in collagen synthesis – Type 1 collagen-(alpha 2)

Classification (Sillence):

I. AD, Blue sclera, hearing loss, most common, most mild (Familial)

IA. Dentinogenesis absent

IB. Dentinogenesis present

II. 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 teeth

IVB: Dentinogenesis imperfecta

Surgery: Sofield-Miller (“Shish-kebob”)

Correct scoliosis if curve > 45(, use PMMA to augment and segmental fusion techniques

Increased risk for malignant hyperthermia

Ehlers-Danlos Syndrome

Defect in collagen metabolism

Sx: Skin hyperextensibility (“Cigarette paper skin”), Joint hypermobility, Bruisability, Soft tissue

calcification, Bone fragility, Bursa formation, Osteopenia

Many types

Mucopolysaccharidosis

Features:

1. Proportionate short stature

2. Anterior beaking of vertebrae (platyspondyly)

3. Bullet-shaped metacarpals

4. Wide pelvis

Types: 1. Hunter 2. Hurler 3. Morquio 4. San-Filippo

5. Scheie 6. Maroteaux-Lamy 7. Sly

Hypothyroidism

Congenital (Cretinism)

F>M

Dwarfism, Mental retardation, “Osteopatia cretinoidia” (~Perthes)

Acquired

SCFE

Scurvy

Age 6-12 mos

Sx: Decr appetite, Slow wt gain, Bleeding gums, Subperiosteal hemorrhage

X-Ray: Wimberger’s ring (density around cartilaginous ossific nucleus)

[Subperiosteal hemorrhage Differential: Scurvy, Menkes Kinky Hair Syndrome, Abuse, Neglect]

Wormian bones

C leidocranial dysostosis – retarded ossification of membranous as well as cartilaginous precursors of bone. Congenital absence of clavicles combined with softness of the calvarium.

H ypothyroidism

O steogenesis Imperfecta

M einke’s Kinky Hair Syndrome

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

Helmet Heads

O.I.

Frontometaphyseal Dysplasia

Dentoagenesis

Neurofibromatosis: Tumors of neural crest/mesodermal origin

Types: 1. NF-1 (von Recklinghausen, Peripheral NF) => chromosome 17- (long arm)

2. NF-2 (Central NF, Acoustic neuroma) => chromosome 22

Diagnosis 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 Sign

4. Osseous lesions- sphenoid dysplasia or thinning of cortex of a long bone (+/- pseudarthrosis)

5. Optic glioma

6. Lisch nodules

7. 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>F

Boyd Anderson

I. Fx present at birth

II. Hourglass constriction Dysplastic

III. Bone cysts Cystic

IV. Sclerotic segment Sclerotic

V. Dysplastic fibula Fibular

VI. 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)

5. Neoplasias (Leukemia, Wilm’s 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 neuromas

2. 1( relative with NF2

Gait

Determinants of Gait:

1. Pelvic rotation

2. Pelvic tilt

3. Knee flexion after heel strike in stance

4. Foot and ankle motion

5. Knee motion

6. Lateral Displacement of Pelvis

Limb Lengthening

Indication: 8-12 yo, Discrepancy > 4cm

Wagner: open corticotomy, distract, plate, bring back later and bone graft

Ilizarov: perc corticotomy (don’t violate medullary canal), ring fixator, wait 5-7 days, distract 1mm/day

De Bastiani; same as Ilizarov, except use unilateral ex-fix (OrthoFix)

Tumor

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

Malignant - 3 most common -> Osteosarcoma > Ewing’s > Rhabdomyosarcoma

Work-up: H&P, X-Ray, Bone scan, CT, MRI, Angiogram, Lung CT

Enneking 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 metaphysis

Multiple hereditary exostosis (Ehrenfried’s Dz)

Autosomal dominant

Short stature

X-ray: trabeculae flow into lesion, lesions point away from physis

Tx: Excise if - Limit ROM, Pain, Neurovascular compromise

Malignant degeneration: 5-15%

Enchondroma

Unilateral, Monomelic

Ollier’s Dz (Multiple enchondroma)--Maffuci’s 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 Cyst

Multiple loculations with septa

X-Ray: Cyst is wider than metaphysis

Histo: vascular lakes, with giant cell laden epithelized septa

Osteoid Osteoma--common in prox fem metaphysis (calcar), and posterior elements of the spine

“Pea in a pod” sclerotic nidus 20yo, flat bones more common

X-Ray: Onion skinning due to periosteum trying to wall off soft tissue expansion

Histo: + PAS, monotonous sheets of round cells

Osteosarcoma

Sx: Swelling, Tenderness, Decr ROM, Warmth, Pathologic fx

Differential Dx: Infection, Ewing’s, 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, ABC

Growth 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

Maturation

Degeneration

Provisional Calcification

Most common fractures:

SH I Distal fibula

SH II Distal radius

SH III Distal tibia

SH IV Distal femur

Growth:

Lower Extremity: Proximal femur 10

Distal femur 40

Proximal tibia 30

Distal tibia 20

Individual Bones: Prox. Humerus 80% Prox. Femur 30%

Dist. Humerus 20 Dist. Femur 70

Prox. Radius 25 Prox. Tibia 57

Dist. Radius 75 Dist. Tibia 43

Prox. Ulna 20 Prox. Fibula 60

Dist. Ulna 75 Dist. Fibula 40

Ossification sequences:

Ankle: Middle (at Poland’s hump), Medial, Lateral

Elbow:

Appearance: Closure:

Come Rub My Tool Of Love TLC OR ME

Capitellum 6 months Trochlea

Radial head 2 yrs Lateral epicondyle 13 yrs

Medial epicondyle 4 Capitellum

Trochlea 6

Olecranon 8 Olecranon 15 yrs

Lateral epicondyle 10 Radial head

Medial Epicondyle 17 yrs

Epiphyseal Centers present at birth: Full Term Children Have These Centers

1. Distal femur

2. Proximal tibia

3. Calcaneous

4. Proximal humerus

5. Talus

6. Cuboid

Hemoglobinopathies (Sickle Cell Disease)

Sx: Small size (due to persistent anemia, or decr O2 at growth plate)

Osseous manifestations:

1. Marrow hyperplasia

2. Infarction

3. Growth disturbances

4. Osteomyelitis

5. Pathologic fracture

X-Ray: Vertebrae - Most common involved bone, Cup-like biconcavity in central endplate -“Step-deformity”

Skull - Thick cortex, Widened diploe, “Porcupine quill”, “Hair-on-end”

Joints - Bone crisis, Bone infarcts

Hip: AVN, Osteochondritis, Coxa vara

Differential Dx: Perthes (SS = Older [age > 10yo], Black)

Chung/Ralston classification

Group 1: Total head involvement ~ Perthes

Group 2: Localized involvement ~ OCD

Group 3: Late changes

Hand-Foot Syndrome

Age < 2yo

Sx: Painful soft tissue swelling of hands/feet

X-Ray: Periosteal new bone, Lytic lesions

Osteomyelitis

Sx: ~ Bone crisis

Organism: Salmonella

Juvenile Rheumatoid Arthritis (Still’s Disease)

Sx: Fever, Rash, Anemia, Splenomegaly, Multiple joint involvement

Age: < 6yo, 10-15 yo

Dx: Synovitis of joint (Knee, ankle, elbow, wrist) > 6 weeks

Types: Monoarticular, Pauciarticular (2-4 joints), Polyarticular (>4 joints)

Septic Arthritis

Epidemiology: Age 1-3yo

Organisms: Staph A., Streptococcus, Pneumococcus, H. flu (#1 35,000], Inc protein, Dec glucose[40 < blood Glc])

Diff Dx (Septic hip):

1. Osteomyelitis 4. Iliopsoas abscess 7. Perthes

2. Toxic synovitis 5. JRA

3. Diskitis 6. Rheumatic fever

Sequelae (Septic hip):

1. Dislocation of hip 4. Coxa magna

2. Osteomyelitis of fem neck 5. Coxa vara

3. AVN 6. Leg length discrepancy

Poor Prognosis:

1. Delay in diagnosis (>5 days)

2. Hip joint

3. Associated osteomyelitis

4. Younger age (diagnosis difficult in infants)

Osteomyelitis

Seasonal (Spring, Fall)

Etiology: 1. Hematogenous

2. Direct extension

3. 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 dyscrasias

2. Cellulitis 6. Tumor

3. Syphilis 7. Hypervitaminosis A

4. Sickle cell crisis 8. Caffe’s 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)

Complications:

1. Bone overgrowth 2. Growth Arrest 3. Pathologic Fracture

4. 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)

Diskitis

Sx: Refusal to walk (9 yo)

X-Ray: Crosses disk space

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Last revised 7/02

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