ORTHOPEDIC REVIEW Learn it, live it, love it! Dolor, Tumor, Calor, RuborSTICKOR Cerebral Palsy Definition: non-progressive neurologic disorder in infants <3 yo Number 1 risk factor: Pre-maturity Types: 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” 1
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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.
2 foot problems: equinus, planovalgusCompensation for equinus: 1. Genu recurvatum
2. Midfoot break3. Hindfoot valgus
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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)
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
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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)
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
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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
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
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"
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)
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
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)
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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
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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
Tarsal CoalitionAge: 8-12 yoSx: Multiple ankle sprains, Decr subtalar ROMTypes: 1. Calcaneonavicular (Most common) pain in sinus tarsi with decr in subtalar ROM
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
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)
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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:
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
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)
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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
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)
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
< 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
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
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
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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
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
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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
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
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P yknodysostosis – form of osteopetrosis showing short stature, separated cranial sutures, delayed closure of fontanelles and hypoplasia of the terminal phalanges.
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
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)
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
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
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
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
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
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