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307 Cards in this Set

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What is a meningocele

Most common form of spina bifida - 90%


Cyst without cord/root involvement

What is a lipomeningocele

A form of lumbosacral lipoma


Associated with tethering and distematomyelia

What are some risk factors for spina bifida

Inadequate maternal intake of folic acid (0.4-0.8mg/day)


Pregestational maternal diabetes


In utero exposure to valproate, carbamazepine

How do you differentiate level of spina bifida

L2 - no quad


L3/4 +quad, poor hamstring


L5 +quad/medial hamstr, no gluteus medius (waddling gait) or maximus


S1 +quad/gluteus medius

What is associated with a myelomeningocele

80-90% have hydrocephalus


MOST will need a shunt


Function correlates to presence of a shunt, # of infections, # of revisions


Arnold-Chiari (cerebellar tonsils thru foramen magnum)


Feeding, crying, breathing problems


Diastematomyelia - bony, fibrous, cartilage bar dividing spinal cord longitudinally

How can one detect a tethered cord?

Scarring of cord preventing ascent of cord with vertical growth


Change in neuro status, worsening/progressive scoliosis


Gait change



How do you evaluate and treat a tethered cord

MRI


Surgically with a release

What is a common allergy associated with spina bifida

Latex


Acute - IGE mediated


Delayed - T cell mediated

What are indications for hip reduction in spina bifida

Sacral involvement and walks with or without support


Unilateral dislocation in mid/low lumbar with patient who crutch walks


NO INDICATION with bilateral disloc in low lumbar or with unilateral high lumbar/thor

In spina bifida, what position is the foot in

equinus, cavus, valgus

What is the goal in a spina bifida foot?


What is done for a supple foot? Rigid foot?

Braceable foot


Tenotomy for supple


Osteotomy for rigid

How do fractures present in spina bifida?


How are they treated?

Red, hot, swollen extremity


Non-operative. Well padded splint

What is mode of inheritance of Duchenne

X-linked recessive (almost all boys)

What are some hallmarks of Duchenne?

Delayed ambulation (>18 months)


Calf hypertrophy


toe walking


progressive scoliosis in >90% once non-ambulatory

What is the genetic abnormality in Duchenne and Becker?

Dystrophin ABSENT in Duchenne


Dystrophin DEFICIENT in Becker's


Causes fragility of myofiber membrance

How is Duchenne's diagnosed in lab? Clinically?

CK >10,000


DNA studies


RARE need for muscle biopsy




Clinically - waddling, wide based gait, toe walking, calf pseudohypertrophy, Gower's "push to stand"

How and when is Duchenne's treated? How does this work?

Corticosteroids - improves natural history


Stablizes myofiber membrane


Upregulates dystrophin associated proteins (utrophin)


Start at age 5-7

What surgical complication are Duchenne's patients at increased risk for? How is this treated?

Malignant hyperthermia

Dantrolene


What is mode of inheritance of Spinal Muscular Atrophy?

Autosomal recessive

What is genetic abnormality in Spinal Muscular Atrophy?

deficiency of survival motor neuron (SMN) protein --> causes loss of ANTERIOR HORN CELLS

What is worst type of Spinal Muscular Atrophy? How and when do these patients die?

Type I - Wernig Hoffman (80% of patients)


Die of respiratory failure betw age 2-8




Type III stand independently and can walk till age 5-20, normal life span

How are hip dislocations and scoliosis treated in Spinal Muscular Atrophy?

Hip dislocation - no treatment


Scoliosis - fuse in SMA III (to pelvis if nonamb)

What is the classic triad in Friedreich's Ataxia?

Ataxia


Loss of deep tendon reflexes


Extensor babinski response

What is associated with Friedreich's Ataxia? What do they die from?

Hypertrophic cardiomyopathy and DM


Die of cardiomyopathy

What position are upper and lower extremities in with arthrogryposis?

Upper - shoulders adducted, IR, elbows variable, wrists flexed


Lower - hips flexed, adducted, ER, knees variable, severe clubfoot

When do brain insults occur in cerebral palsy? Are the lesions fixed or progressive?

Developing fetal or immature (<2 yr old) brain


Fixed, non-progressive lesions

What are some associated and possible causative factors of cerebral palsy?

Prematurity


Periventricular Leukomalacia


Intraventricular hemorrhage


Intrauterine infection (ToRCH, chorioamnionitis)


Hypoxic-ischemic injury


Embolus


Hemorrhage


Brain malformation

Which GMFCS level is most highly associated with hip dislocation?

5

What happens if an achilles is overlengthened in CP surgery? Hamstring over lengthened?

Achilles - crouch gait. Don't lengthen without addressing hamstring


Hamstring - stiff-knee gait, xs ant pelvic tilt




Address all levels of tightness at once!!

In CP, how is dynamic supination in swing phase treated?


Hindfoot varus in stance?

Split tibialis anterior


Split tiibialis posterior




Complete transfer of TA and TP not recommended

What are indications for subtalar and 1st MTP fusions in CP?

End stage arthritis or severe joint instability


PPV --> triple


Hallux valgus --> 1 MTP fusion

What is mechanism of botulinum toxin?


Baclofen?

Irreversible pre-synaptic blockof Ach release


Temporary (3-6 month)


Baclofen is a GABA agonist- GABA is an inhibitory transmittor


works centrally to inhibit reflexes, dec tone


intrathecal baclofen is less sedating


be careful of withdrawal with pump malfunc

What are indications for "selective" dorsal rhizotomy

For ambulatory, spastic diplegia - GMFCS I/II



What brace is best to address crouch gait

Ground reaction AFO - pushes knee into extension

Is bracing indicated for paralytic scoliosis?

No - does not stop progression

What are spinal fusion indications in CP?

Curve >40-50


Positional problems


Ant release indicated in curves >90, severe stiffness (>70) with bend/traction


Ext to pelvis in curves >90, nonambulators

Where is acetabular deficiency in CP?

Posterolateral

What are VDRO indications in patients >8, <8?

<8 - uncovering >60%


>8 - uncovering of 40%

What is most common CP Answer?

Spastic diplegia

A 3 yr old with L3 myelomeningocele has bilateral dislocated hips. Management should consist of

observation

An 8 yr old with Duchenne's has positive Gower's and is beginning to fall frequently. Gait pattern would indicate what of his lumbar lordosis, hip flexion, plantar flexion

Increased lumbar lordosis, increased hip flexion and increased ankle plantar flexion (to address his hamstring weakness)

What is the muscle pair to the tibialis anterior? Tibialis posterior?

TA - peroneus longus


TP - peroneus brevis

What do the ectoderm and endoderm contribute musculoskeletally?

Ectoderm - spinal cord


Endoderm - notochord --> nucleus pulposus

When does the notochord disappear

When somites form vertebrae from mesoderm

What does notochord persist as in an adult?

Nucleus pulposus



What is a chordoma a remnant of?

Notochord remnant

What are the 3 ossification centers of spine?

Vertebral body center (epiphyseoid - growth continues till age 16-18)


Paired neural arch (growth till age 8 - canal diameter fixed at this point)

What does thalidomide inhibit?

Apical ectodermal ridge --> limb formation

What gene is important for longitudinal growth?

Sonic hedgehog

What is important for ulna-radius differentiation?

ZPA - Zone of Polarizing Activity

Osteopetrosis is a dysfunction of

osteoclasts

What is first secondary ossification to form? When?

Distal femur

39 weeks


What is primary limiting membrane and mechanical support for the periphery of the physis?

Perichondral ring of La Croix

What are the 3 zones of the normal growth plate?

Reserve


Proliferative


Hypertrophic

What are differences in Calcium, collagen and oxygen tension in the reserve, proliferative and hypertrophic zones of growth plate

Reserve - Lowest Ca, Highest type II collagen, low O2 tension


Proliferative - Highest O2


Hypertrophic - Type X collagen, Lowest O2, high Ca (zone of provisional calcification)

What provides latitudinal growth to growth plate?

Groove of Ranvier

What reinforces growth plate and binds it to the periosteum?

Perichondral ring of La Croix

What does thyroxine deficiency cause?

growth retardation, epiphyseal necrosis, SCFE


Essential for cartilage growth, maturation

When do boys and girls stop growing?

Boys - 14

Girls - 16


What ossification centers are present at birth?

distal femur


proximal tibia


calcaneus


talus


cuboid

What is the difference between an exon and an intron?

Exon - part of DNA that encodes RNA


Intron - spacer DNA that is removed

Does achondroplasia have complete or incomplete penetrance? What is the difference?

Complete penetrance.


Phenotype will always match genotype

Match:


Structural and Enzyme


Autosomal Dominant and Recessive

AD - structural


AR - Enzyme

What is mode of inheritance for Familial Hypophosphatemic Rickets?

X-linked Dominant

What is most common organism in Acute Hematogenous Osteomyelitis?

PVL (Panton-Valentine Leukocidin) S Aureus


More complex infection, prolonged fever, DVT, abscesses, multisystem organ failure

How are patients with PVL S Aureus treated?

Clindamycin

What organism is most common in Acute Hematogenous Osteomyelitis (AHO) in patients age 6-48 months?

Kingella Kingae (gm negative)



How is Kingella Kingae detected?

PCR

What are the Kocher criteria?


4/4 = ?% septic arthritis


3/4 = ?% septic arthritis

Nonweightbearing


ESR >40


Fever >38.5


WBAC >12,000


4/4 = 99%


3/4 = 93%

What is most sensitive test for acute hematogenous osteomyelitis?

CRP - elevated in 98% of patients within 6 hrs

What are empiric abx for acute hematogenous osteo for:


birth - 1 month


1-3 months

Birth-1 month: Ampicillin/sulbactam and Gent


1-3 months: Vanco and ceftriaxone


Severe infections: Clinda or Vanco + Rifampin

In the embryo, cells from the ectodermal layer give rise to which elements of the spine?

Spinal cord

What do the endoderm and mesoderm contribute to the spine?

Endoderm - notochord ---> nucleus pulposus


Mesoderm - muscles and bony elements of spinal column

What is ectrodactyly?


This is caused by a defect in which embryonic location?

a central absence of rays - referred to as cleft foot or a "lobster claw"


Defect in Apical Ectodermal Ridge

When does the medial sternoclavicular joint epiphysis close?

age 23

When do you reduce posterior SC joint and what work-up/precautions should be made?

Respiratory or NV compromise


CT Scan


Should be reduced with CT service

The proximal epiphysis of the humerus contributes what % to growth?

80%

What are acceptable pediatric proximal humerus fracture parameters?

<5 yrs - 100% displacement, 70 deg angulated


5-12 yrs - 50-100% displacement, 40-70 deg angulated


>12 yrs - <50% displacement, <40 deg angulated

What is frequently interposed in proximal humerus fracture

Biceps


Periostem/joint capsule

What is Baumann's angle?


What is normal Baumann's angle?



capetillar epiphysis and joint line


Normal is 72 degrees

What is most commonly involved nerve of supracondylar fractures? flexion-type?

AIN


Flexion type - Ulnar nerve

What construct is recommended for supracondylar humerus fractures? What is strongest construct biomechanically?

Divergent lateral pins


Strongest construct - one medial, one lateral

Pulseless, pink hand pre reduction? Post reduction? Avascular?

CRPP


Serial exams


Vascular exploration (never angiogram)

WHat is the most likely indicator for a pediatric compartment syndrome post supracondylar humerus fracture?

Increasing pain med requirement

What is the most common complication to SCH fx? And why?

Cubitus varus secondary to malreduction

What injury is often initially misdiagnosed as a dislocate elbow in a 3 yr old?


What should you be concerned for?

Distal humerus epiphyseal fracture


A/w abuse

What are surgical indications for pediatric elbow lateral condyle fractures?

2-4mm displaced with hinge - CRPP, +/- arthrogram


>2mm with malrotation - ORIF

What is important to note in approach to pediatric lateral condyle fractures

Maintenance of posterior soft tissue attachments

WHat is one of the most devastating complications of lateral condyle fractures? And how does it occur?

cubitus valgus with tardy ulnar n palsy


Secondary to malreduction and subsequent growth

In prediatric radial neck fractures, what are the 3 rules of 3's?

Non op if


<30 degrees angulation


<3mm translation


<1/3 radial head

What determines your treatment of monteggia fractures in peds?

Stability and length of ulna

What are non-operative indications of peds both bone forearm fractures?

<8 yr old: 20 deg shaft angulation, bayonet appostion, NO rotation


>8 yr old: 10 deg shaft agulation, No rotation

WHat is common complication of one inc technique in ORIF bbffx?

Synostosis

How much does distal radial physis contribute to longitudinal growth?

80%

What is acceptable displacement for pediatric distal radius fractures in patients >12, <12 yr old?

<12 yr old: 25 deg angulation in sagittal plane, 50% translation, up to 10 yr old - 100% bayonet


>12 yr old: 10 deg angulation in sagittal plane, 10 degrees radioulnar deviation


NO ROTATION

What are surgical options for growth disturbance in physeal distal radius fractures?

Physeal bar resection if it comprises <50% physis, >2 yrs of growth, fat graft interposition

How much angulation is tolerated in peds base of 1st metacarpal fractures?

30 degrees

WHat is a Seymour's fracture?

Physeal fracture of distal phalanx with nailbed laceration and incarceration of germinal matrix

How are pediatric phalangeal neck fractures treated?

Closed vs open reduction, pin fixation

How are pediatric phalangeal physeal fractures usually treated?

Closed reduction, buddy taping


*must check post reduction xrays

What is blood volume/kg in children

75 ml/kg

What are three A's to pediatric compartment syndrome?

Anxiety, agitation, Analgelsic requirement

What muscles attach to the following bones causing pelvic avulsion fractures?


Iliac crest


ASIS


AIIS


Ischium

Iliac crest - abdominal muscles


ASIS - Sartorius


AIIS - Rectus femoris


Ischium - hamstring

What nerve injury is a/w pediatric posterior hip dislocation?

Peroneal branch of sciatic nerve

What is often interposed/preventing reduction of pediatric hip dislocations?

Interposed labrum, capsule, ligamentum teres, osteochondral fragment

What increases risk of AVN in pediatric hip fractures?

Increased displacement


Lower Delbet type


I - transepiphyseal


II - transcervical (most common)


III - basicervical


IV - intertrochanteric

How does blood supply change at age 4 to femoral head?

<4 yr old - both medial and lateral fem circumflex


>4 yr old -mostly MFCA via posterosup and posteroinf retinacular branches

What occurs with trochanteric apophyseal arrest? overgrowth?

Arrest --> coxa valga


Overgrowth --> coxa vara

How are pediatric femoral shaft fractures treated?

<3 yr - eval for child abuse


0-6 months - pavlik vs spica


6 mon-5 yr - <2cm shortening - spica


5-11 yr - unstable - bridge plate


stable, <110 lbs - flexible nails (2 retrogr with 80% canal fill)


Length unstable or >110 lbs - bridge plate


>11 yr old - trochanteric entry locked nail



Distal femur physeal fractures operative indication? Fixation technique?

Displacement


Smooth crossed pin fixation or transverse lag screw if metaphyseal fragment is large enough

Most common post operative complication of distal femura physeal fractures?

Growth arrest (60%)

What is most likely incarcerated in an irreducible tibial spine fracture?

anterior horn of medial meniscus

What is most common post operative complication of tibial spine fractures?

Arthrofibrosis

What is non-operative indication for tibial tubercle fracture?

<2mm displacement


Tx with LLC

What is most devastating complication of tibial tubercle fractures? Why does it happen?

Compartment syndrome


Recurrent anterior tibial artery

What is a Cozen's fracture?

Proximal tibial metaphyseal fracture

What is the most common complication of Cozen's fracture?

Valgus deformity (50%)


Increases over 18 months, then corrects


Initial tx - observation

What is accepted in pediatric tibial shaft fractures?

<1cm shortening, 5-10 deg angulation


RISK OF VARUS WITH INTACT FIBULA

What is a toddler's fracture? Treatment?

Spiral distal tibial shaft fx in walking toddler


NOT a/w abuse


Tx (even with negative xrays) - LLC x3 weeks


Initial xrays negative in 25%

What are indications for bar excision in physeal arrests?

Bar excision if <50% of physis involved, >2 yrs left of growth

In what direction do distal tibial physis close?

Central --> Posterior --> Medial --> Lateral

What is a tillaux fracture?

anterolateral distal tibial epiphyseal fracture


caused by avulsion of anterior inferior tib-fib ligament

What is displacement pattern of a triplane fracture?

Epiphyseal sagittal plane fracture


Metaphyseal coronal plane fracture


Physeal displacement in axial plane

SH I/II fractures of great toe should be treated with?

I&D, antibiotics, nail bed repair, +/- K wire


Treat similar to seymour fracture

What fractures are suspicious for child abuse?

Posterior rib, distal humeral physis, femur in nonambulator, <1 yr old with multiple fx in different stages of healing, metaphyseal corner

The following signaling centers create what gene products?


AER (apical ectodermal ridge)


ZPA (zone of polarizing activity)


Wnt

AER - FGF's


ZPA - Sonic hedgehog


Wnt - Wnt

What action do AER/FGF promote?


ZPA/Sonic hedgehog?


Wnt?

AER/FGF - prox/distal, interdigital necrosis


ZPA/Sonic Hedgehog - Radioulnar


Wnt - Dorsovolar

Transverse Failure of formation congenital anomalies are due to dysfunction of what signaling pathway?

AER --> FGF

Longitudinal failure of formation congenital anomalies are due to dysfunction of what signaling pathway?

ZPA --> Sonic Hedgehog

How should you further work up longitudinal failures of formation?

CBC, renal ultrasound, echo

Radial longitudinal deficiency is associated with what other congenital syndromes?

Fanconi's anemia (aplastic anemia tx with BMT) TAR (low plt)


Holt-Oram (AD, cardiac septal defects)


VACTERRLY

WHat is a contraindication to treatment of radial longitudinal deficiency?

Elbow contracture/fusion

With radial longitudinal deficiency, centralization procedures aim to centralize to what?

3rd metacarpal

Ulnar and radial longitudinal deficiency are associated with wrist and elbow stability/instability?

RLD - stable elbow, unstable wrist


ULD - unstable elbow, stable wrist

Cleft hand is a failure of differentiation or formation?

Formation

Radioulnar synostosis is a failure of differentiation or formation? what position is forearm in?

Differentiation


Pronated

Syndactyly is associated with what other syndromes?

Poland's (absent pec major, hypoplasia)


Apert's (Acrocephalosyndactyly)


Carpenter's (acrocephalopolysyndactyly)

Syndactyly - border or central digits 1st? Staged or bilateral simultaneous? Most common complication?

Border digits first


One hand at a time


Release after 18 months to minimize web creep

Camptodactyly is a failure of formation or differentiation? How is it treated?

Camptodactyly - digital flexion contracture


Differentiation


Splinting


Surgery for contracture >40 deg



What is clinodactyly? How is treated?

digital angulation in radioulnar plane


surgical options include physiolysis, osteotomy

What determines whether a hypoplastic thumb is saved/reconstructed vs pollicization?

IIIA - absent extrinsics, STABLE CMC joint


IIIB - absent extrinsics, UNSTABLE CMC joint

What is difference between pre and post axial duplication?

Pre - thumb


Post - other digitf

What is most common Wassel pre axial duplication?

Wassel type IV - duplication of both prox and distal phalanges, one metacarpal

How are pre-axial duplications treated?

Ablation of radial thumb


Preservation of EPL/FPL


Collateral ligament reconstruction (often RCL)

Post axial duplication mode of inheritance? Demographic?

AD


African-americans>white


Caucasians should get genetic eval

Macrodactyly is a/w what syndromes?

NF


Proteus


Klippel-Trenaunay-Weber

What are some risk factors for brachial plexus birth palsy?


What roots commonly affected?



Macrosomia


Shoulder dystocia


multiparous pregnancy


History of prior BPBP


C5-6 +/-7

What are poor prognostic factors for brachial plexus birth palsy?

Biceps take longer than 2 months to recover


Horner's syndrome (associated with root avulsion/preganglionic lesion)

What are indications for brachial plexus microsurgery?

Horner's syndrome+flail limb+absent biceps at 3 month


Absent biceps at 6-9 months

What is congenital torticollis?


How is it evaluated?

Head tilted toward affected side, rotated to OPPOSITE side


eval for Klippel Feil (congenital c-spine fusion) - low hairline, Sprengel's--> MRI brainstem/spinal cord to eval for intradural abn



How is congenital torticollis treated?

Stretching


Hip xray to r/o DDH


5% who fail may need release of SCM



How does idiopathic atlantoaxial subluxation present?

SCM is tight on SAME side of rotation


may follow minor trauma or URI (Grisel's)

Os Odontoideum is usually the result of what?


How is it evaluated?


Treated?

Unrecognized C2 fracture in early childhood


Flexion/extension radiographs


If unstable - C1-2 posterior arthrodesis

How should infantile scoliosis be evaluated?

MRI to r/o syrinx, chiari, tether

What factors predict progression of infantile scoliosis?

Present over age 1


Rib vertebral angle (RVA)>20


Curve >40

How is infantile scoliosis treated?

Cast then brace for progressive type <4, curve <50


Operate if curve >60, failure of cast

Juvenile scoliosis should be worked up with what?

MRI

Pulmonary compromise in AIS occurs at what curve?

>70 degree thoracic curve

What are indications and treatment options for AIS?

Risser 0-2 with >20 would brace


Risser 3-5 with <50 would observe


Risser 0-5 with curve >50 - surgery

When is peak height velocity?

Risser 0


Between 11-13 for girls


Between 13-15 for boys

What are indications for MRI in AIS?

Significant back/neck pain


Neuro abnormality


Left thoracic curve


<10 yr old with curve >20

When should an anterior release be performed in AIS? and why?

Curves >70 degrees


Open triradiate cartilages


--> causes crankshaft phenomenon

How are pediatric spondylolistheses treated?

Low grade (<50%) - observation. Usually L5/S1


High grade (>50%) - L4-S1 in situ fusion


reduction w/o decomp --> L5 n root traction injuries

How is Scheurmann Kyphosis treated?

MOST - non op


Brace - curve <70, risser <3, flexible deformity

Failure of formation/differentiation --> Vertebral bar/hemivertebrae

Bar - failure of segmentation


Hemivertebra - Failure of formation

What evaluation should be done in congenital scoliosis?

Echo and renal ultrasound, spine MRI

What are specific indications for convex hemiepiphyseodesis?

Concave growth potential (NO BAR)


<5 involved vertebrae


<70 deg curve


<6 yr old

Halo immobilization in young children require how many pins and torque?

8-12 pins


LOW torque 4 ft/lbs

How is SCIWORA evaluated and treated?

MRI - most sensitive at 48 hrs


12 weeks immobilization

What are Ortolani and Barlow maneuvers?

Ortolani - hip is OUT and reduces with ABD


Barlow - hip is IN, BACKS out with ADD

What are 3 conditions associated with DDH?

Metatarsus adductus


Torticollis


Congenital knee dislocation

How is DDH clinically diagnosed in patients >3 months?

Limitation of abduction


Galeazzi sign


LLD

What is an abnormal alpha angle in infant hip ultrasound? Abnormal beta (femoral head angle covered)?

Alpha angle <60 degrees


Beta >55 degrees

What is normal AI and CEA hip xrays of patients >6 months?

Normal is:


Acetabular Index <30 by 18 months (24 by 24)


CEA >25 by age 6 yr

How is dislocated hip treated in patient 0-3 weeks? >3 weeks? 6-18 months? >18 months?

<3 wks - observe


>3 wks - pavlik


6-18 months - closed reduction with arthrogram (<5mm of medial dye pool = reduced), +/- adductor tenotomy, spica cast (100 deg flex, 45 abd, neutral rotation) x3 months


>18 months - open reduction + spica


>5 yr old w/acetab dysplasia - pelvic osteotomy

What are approaches to open reduction of infant/toddler hip?

Medial - > AVN risk? (<1 yr old)


Anterolateral - capsule plication and osteotomies

What are common blocks to reduction of dislocated hip?

psoas


capsule


Inverted labrum/limbus


Ligamentum teres


Pulvinar


TAL

What are the three main types of pelvic osteotomies for acetabular dysplasia?

Redirectional - Salter/Steele/Ganz


Recontouring - hinge on triradiate


Dega/Pemberton/San Diego


Salvage - fibrous metaplasia


Chiari/Shelf



What is a reasonable indication for observation of DDH?

Children >5 yr old with bilateral dislocations


SMA


Spina bifida

What are the major complications to treating DDH?

Residual dysplasia (20%)


AVN (10-20%)


hyperabd, forced abd, medial approach


Redislocation (5%)


NV damage (1%)

What disorders are a/w Legg Calve Perthes?

Protein C/S deficiency (Hypercoag disorders)

What are 3 radiographic signs of Perthes?

Waldenstrom's - inc'd distance betw fem head and teardrop


Crescent - subchondral fracture


Gages - small osteopenic V at lateral aspect of epiphysis

What are the 4 stages of Perthes

Initial - increased med space


Fragmentation - when you classify, increased head density


Reossification - New subchondral bone


Remodeling - Femoral head reconstituted

What is on differential for bilateral perthes?

Spondyloepiphyseal dysplasia tarda


Multiple epiphyseal dysplasia


Meyer's dysplasia


Hypothyroidism


Steroids

What is the A/B/C classification of Perthes?

Lateral 1/3 pillar of epiphysis


A - no ht loss or density change


B - <50% loss of lateral height


B/C - 50% height loss


C - >50% height loss

What stage and age do you consider treatment for Perthes?

Fragmentation stage


Stage B/C


Age >8

What are some treatment options for Perthes?

Petrie casting


Salter pelvic osteotomy


VDRO

What factors are protective in Perthes?

Age <8


<50% head involvement

For SCFE:


Avg age


Gender


Demographic

9-16


M>F


Obese


Pacific Islander/African American

What work up should be done for bilateral SCFE?

Endocrine


- hypothyroid


- hypogonadism


- panhypopituitaryism


- renal osteodystrophy



What differentiates acute vs chronic?

< or > 3 weeks

What zone of epiphysis is fractured in SCFE

Hypertrophic

In SCFE, what is deformity of femoral neck in relationship to head?

Metaphysis/neck ext rot and slips prox to epiphysis producing a varus deformity (only on occasion valgus)

What is present on clinical exam of SCFE?

Painful IR


Decreased IR/ABD/Flex


Obligate ER with flexion


Ext FPA

What radiographic signs are present in SCFE?

Klein's line lateral to epiphysis (line drawn along sup fem neck in AP)


Blanche sign - hyperdensity of meta/epi overlap

How are SCFE's classified on lateral xray?

Mild - <1/3 neck width displ


Moderate - 1/3 to 1/2 neck width displ


Severe - >1/2 neck width displ

How is SCFE treated?

gentle operative positioning reduction (if any reduction at all)


In situ perc pin

What are parameters of screw fixation in SCFE?

Center-center of head


3-5 mm short of chondral surface


4 threads past physis

When should a contralateral hip be pinned in SCFE?

Bilateral (symptoms or abnormal xray)


Endocrinopathy/metabolic disorder


Age <10, obese

What are the risk factors for chondrolysis in SCFE?

Hardware <3mm from subchondral surface


Persistent hardware penetration


Prolonged pre-treatment symptoms


Cast immobilization

What is major complication to SCFE? What are risk factors?

AVN


- acute, unstable slip (50% AVN risk)


- overreduction


- superolateral pin placement


- femoral neck osteotomy

What are the 4 deformities of clubfoot?

CAVE


Cavus (mismatch betw foreft pronation, hindft sup)


Forefoot ADDuction


Hindfoot Varus


Ankle Equinus

What is the pathophysiology of clubfoot?

Rotation of the navicular causing uncovering of the talus

What syndromes and associations are there with clubfoot?

Diastrophic or streeter dysplasia


Prune-belly syndrome


Tibial hemimelia


Arthrogryposis


Spina bifida

What radiographic findings are found in clubfoot?

Adductus of talar-1st metatarsal on AP


Parallelism of talus and calcaneus on both AP and lateral

What order are the deformities in clubfoot treated and how?

CAVE


Cavus - elevate 1st met to supinate forefoot


Adductus/Varus - rotate forefoot around talus until heel in valgus and foot ABD 70 deg


Equinus - achilles tenotomy

How is clubfoot treated post casting? What is the most common cause of relapse?

Foot abduction orthosis with affected foot/feet in 70 ER


3 months full time, then night time till age 4


Noncompliance is most common cause of failureW

What deformities typically recur in clubfoot?

Equinus and varus

How is recurrent clubfoot treated?

0-2 yr usually equine/varus - repeat casting, TAL


3-5 yr dynamic supination during swing phase/lateral WB. tx with TA transfer to lat cuneiform


>5 yr foreft adductus persists. tx with medial opening/lateral closing midft osteotomy in older patients (>4)


Dorsal bunion (strong FHL, TA; weak achilles, peroneus longus) - tx with MTP jt plantar release, FHL transfer, plantar osteotomy of base of 1st

What position is the ankle/foot in congenital vertical talus?

Ankle equinus, midfoot dorsiflexion


Hindfoot eversion, forefoot abductionWI

With congenital vertical talus, where does the navicular sit in relation to the talus?

Dorsally dislocated

What is congenital vertical talus associated with?

50% due to genetic or neuromusc cause


Spina bifida


sacral agenesis


diastematomyelia


arthrogryposisWhat

What imaging is necessary to evaluate congenital vertical talus?

Lateral in plantarflexion


Axis of 1st met will be above talus


Oblique talus will reduce with plantarflexion

How is congenital vertical talus treated?

serial casting


Surgery at 6-12 months


- Lengthen EHL, EDL, TA, peroneals, achilles


- reduce and pin talonavicular joint

What are the most common tarsal coalitions?

Calcaneonavicular (age 8-12)


Talocalcaneal (12-15)

How do tarsal coalitions commonly present?

Rigid flatfooot (hindft valgus persists with heel rise)


Frequent ankle sprains, peroneal spasm

Calcaneonavicular coalitions are best evaluated with what xray? Finding?

45 degree oblique


Anteater

Talocalcaneal coalitions demonstrate what radiographic signs?

C sign of lefleur - outline of talar dome and sustentaculum (on lateral)


Talar beaking


Decreased subtalar motion

How are tarsal coalitions treated?

Nonop initially


Resection with interposition of fat or muscle (EDB for CN coalitions)


Subtalar fusion for TC coalition >50%


Calc osteotomy if hindft valgus >15 deg

IF a patient has bilateral cavus feet, what eval should be undertaken?

Genetic testing for Charcot Marie Tooth (hereditary motor sensory neuropathy)

What is weak/strong with cavus foot?

Weak - TA, peroneus brevis


Strong - peroneus longus

What orthotic should be prescribed for cavus foot?

Full length, semi-rigid insole, lateral hindfoot positing, first ray depression

What surgical options are there for cavus foot <12 yr old? Older patient?

<12 - peroneus longus to brevis transfer


Older - 1st met or medial cuneiform osteotomy, calc osteotomy with +coleman blk (fixed hindft valgus)

How are accessory naviculars approached operatively?

Splitting of posterior tibialis


Most common cause of persistent pain in inadequate resection

What are some benign pediatric foot conditions?

Metatarsus adductus


Calcaneovalgus foot


Flexible flatfoot


Osteochondroses


Kohler's

What is calcaneovalgus foot? What is important to rule out? Treatment?

Extreme dorsiflexion of ankle, subtalar eversion, correctable to neutral


R/o posteromedial bowing, L5 spina bifida


Tx with observation, stretching

What is Sever's disease?

Calcaneal apophysitis

What is Iselin disease?

5th metatarsal apophysitis - often confused as pseudojones/fx


Often athletes age 8-13

What is Kohler's? How is it diagnosed radiographically? Treatment?

AVN of navicular


Sclerosis, fragmentation or flattening of navicular


No biopsy or MRI needed!!


SLC, NSAID's, navicular reconstitutes in 1-3 years

How are curly toes treated?

Usually observation


Flexor tenotomy if symptoms and >3 yr old

How are congenital and adolescent hallux valgus treated?

Congenital - if bracket - bracket excision, soft tis release (fibrous band from hallux to base of 1st met)


- if no bracket - soft tis release


Adolescent - no surgery in skeletally immature due to high likelihood of recurrence

What further workup should be done in a patient with hemihypertrophy?

Abdominal u/s and AFP to eval for Wilm's tumor

What is the most accurate way to eval limb length discrepancy?

CT scanogram

How much growth is expected in distal femur/prox tibia per year?

Distal femur - 9mm


Proximal tibia - 6mm

How much LLD is acceptable at maturity?

2cm

How much acute shortening can be achieved in femur? Tib-fib?

Femur 8-10cm


Tib-Fib 3 cm

If expected LLD is 2-5cm, how is this treated? >5cm? >15cm?

2-5: Lift, shorten (physiodesis), lengthen


5-15: Lengthen


>15: Lengthen+shorten, prosthesis +/- amp

What is normal knee angulation as growth proceeds?

18-24 months - physiologic varus


5-6 year - physiologic valgus


>7 - normal adult alignment

When should Blount's disease be treated?

When metaphyseal-diaphyseal angle 11-15 -->q6 month xrays


Angle >16 - treat with braces or osteotomy (age >4 or type IV Langenskiold)


Type IV - bony bridge across medial physis

What are the components of miserable malignment?

Femoral anteversion


External tibial torsion


flat feet

What are surgical indications for tibial torsion?

Persistence >5 yr old


>20 deg IR


Rarely indicated unless clubfoot, CP, MM, Blounts

What is mode of inheritance of tibial hemimelia?

AD

What determines treatment of tibial deficiency?

No extensor mechanism - knee disarticulation


+extensor mechanism - reconstruction


- tib-fib synostosis, lengthening


Ankle stability/length determine foot retention

What are three types of tibial bowing?

Anterolateral (AL BAD) - a/w congenital pseudoarthrosis of tibia (-->surgery), NF


Posteromedial - a/w calcaneovalg foot, improves spontaneously, residual LLD (4cm)


Anteromedial - a/w fibular hemimelia

What determines treatment of fibular hemimelia?

Predicted LLD


Status of knee/ankle (cruciate def, LFC hypoplasia)

When is a Syme amputation indicated in fibular hemimelia?

Unstable ankle


<3 ray foot (relative)

What is best radiographic eval of knee OCD?

Tunnel (notch) view to eval lateral aspect of MFC


MRI to determine extent of lesion

How is OCD treated in skeletally mature? Immature?

Immature, stable lesion - limit activities, WB, will likely heal on own.


Mature and >2cm diameter - surgery


Unstable or displaced OCD (regardless of maturity) - offer surgery

How are discoid menisci diagnosed radiographically?

Lateral joint space widening


MRI - loss of bow tie in 3 consecutive cuts

How are discoid menisci treated?

ONLY IF SYMPTOMATIC - saucerize

Congenital knee dislocations are associated with what syndromes?

Arthrogryposis


Myelomeningocele


Larsen's


HIP DYSPLASIA (MUST RULE OUT)

A 14 yr old skeletally mature girl presents for treatment of LLD 4cm secondary to femur fracture at age 8 treated with spica. Best treated with ?

Lengthening same side femur

What is the mode of inheritance for Neurofibromatosis?

AD

What is gene mutation, gene product and pathway affected in Neurofibromatosis?

NF-1 affectes neurofibromin - regulation of Ras pathway

What are clinical features of Neurofibromatosis?

Pseudoarthrosis of tib-fib (anterolat tib bowing)


Hemihypertrophy


Scoliosis - often short, sharp curve with rib penciling. NEED PRE OP MRI TO R/O DURAL ECTASIAS


neurofibromas


cafe au lait macules


Lisch nodules

What is mutation, abnormal protein and mode of inheritance for OI?

COLIA1 or IA2 genes


Decrease type I collagen


AD or AR inheritance

What are clinical features of OI?

blue sclera


hearing loss


increased risk of malignant hyperthermia


Basilar invagination (HA, neck pain, neuro signs)

What is mode of inheritance, gene mutation in Marfan's?

AD


Fibrillin I mutation

What are clinical features of Marfan's?

Aortic root dilitation


Superior lens dislocation


Pectus excavatum, spontaneous pneumothorax


protrusio acetabulum


Dolichostenomelia (long arm span)


Arachnodactyly (long digits - +thumb, wrist signs)

What is important for pre op planning with Marfan's?

Pre op cardiac eval and MRI for dural ectasia

Most Ehler's Danlos mutations occur in what type of collagen?

V

What is Beighton scale?

Score of 5=hypermobility


2 - small finger passive hyperextension


2 - thumb passive abd to forearm


2 - elbow hyperextension


2 - knee hyperextension


1 - forward flexion of trunk with palms on floor

What are ortho manifestations of Down's syndrome?

atlantoaxial instability


scoliosis


spondylolisthesis


SCFE


Hip and patellar instability

What other conditions are a/w Juvenile Idiopathic Arthritis?

Iridocyclitis (SHOULD SEE OPTHO)


pericarditis


tenosynovitis

Where is defect in Rickets?

Zone of provisional calcification

What are radiographic features of Rickets?

Increased physeal width


Metaphyseal cupping


Bowing

What are the 3 types of Rickets?

Familial Hypophosphotemic


Hypophosphatasia


Nutritional/Vitamin D deficient

What is mode of inheritance of Familial Hypophosphotemic Rickets

X linked dominant

What is defect in familial hypophosphotemic rickets? How is it treated?

Impaired renal tubular phosphate absorption


Treated with Vitamin D and phosphate

What is mode of inheritance of hypophosphotasia rickets? Where is defect?

AR


Defect in alkaline phosphatase

Who is at risk for nutritional rickets?


How is it treated?

Premies, exclusively breast fed, poor intestinal absorption


Treated with Vitamin D (5000 IU daily)



Comparing Familial, hypophosphatasia, nutritional rickets - where are abnormalities?


Calcium


Phos


Alk Phos


PTH


Vit D

Familial - low phos, high alk phos


Hypophos - High Ca and Phos, low alk phos


Nutritional - low phos and Vit D, High alk phos and PTH

What is associated with Streeter Dysplasia?

Congenital constriction bands


Clubfeet


Syndactyly


Anterolat bowing/pseudoarthrosis


Cleft lip/palate


cardiac/renal issues

What is associated with Achondroplasia?

Rhizomelic limbs (proximal shortness)


Genu varum


Spinal pathology (dec interpediculate distance from L1-->L5)


Horizontal acetabular margins


Squared iliac wings


Foramen magnum stenosis


Trident hands (divergent ring and middle fingers)

What is mode of inheritance of Achondroplasia?

AD

Where is mutation in Achondroplasia?


Where is defect? What zone?

Fibroblast growth factor 3 (FGFR3)


Causes a defect in endochondral bone formation


Affects proliferative zone of physis

What screening study should be done in Achondroplastic infants?

Sleep study


Axial spine imaging


Important to eval foramen magnum stenosis


Presents with central sleep apnea, SID

What is mode of inheritance of Diastrophic Dysplasia?


Where is gene mutation?

AR


Sulfate transporter DTDST gene

What are several clinical signs of Diastrophic Dysplasia?

Cauliflower ear


Cleft palate


Hitchhiker thumb

How is early arthritis treated in Diastrophic Dysplasia?


Rigid clubfoot?

Early arthroplasty


Ponsetti casting

What is mode of inheritance of Pseudoachondroplasia?


Where is mutation? Which is also found in what other disorder?

AD


Mutation in Cartilage Oligometric Proteni (COMP)


Multiple Epiphyseal Dysplasia

Why can Perthes resemble pseudoachondroplasia?


What other disorders resemble Perthes?

With delayed epiphyseal ossification looks like Perthes, but bilateral and simulataneous


SED Tarda, MED can also look like Perthes

What eval should be done pre-op for basically any dysplasia?

Flex-ext c spine or axial imaging to eval C1-2 instability


Fuse if neuro sx or ADI<8mm

What is mode of inheritance of Spondyloepiphyseal Dysplasia (SED) Congenita?


Where is defect?

AD


Collagen type II

What is mode of inheritance of Spondyloepiphyseal Dysplasia (SED) Tarda?

X linked recessive


Less severe than SED Congenita (AD)

What is mode of inheritance of Cleidocranial Dysplasia?


Where is gene mutation?


Where are abnormalities found?

AD


Defect in RUNX2


Abn bones formed by intramembranous ossification

Where is gene mutation in Multiple Epiphyseal Dysplasia?


Where is abnormality?

COMP


IX collagen --> collagen II dysfunction


Abnormal ossification of epiphyses with normal spine

What are some common features of Mucopolysaccharidoses?

C1-2 instability


Increased rate of CTS


Hip dysplasia, abn epiphyses


Limb malignment

What is mode of inheritance of Mucopolysaccharidoses? What is exception?

AR - Hurler, Morquio


HUNTER - X-Linked Recessive

What are features of Morquio?

Corneal clouding


Poor dentition


Odontoid hypoplasia (C1/2 instab)

What are 3 skeletal dysplasias and 1 syndrome associated with C1/2 instability?

Mucopolysaccharidoses (Morquio)


Pseudoachondroplasia


SED Congenita and Tarda


Down Syndrome

Disorders requiring pre op spine MRI's to eval for dural ectasias



Neurofibromatosis


Marfan's

Mutations in:


Achondroplasia


Diastrophic Dysplasia


Marfan


Neurofibromatosis


Pseudoachondroplasia


Multiple Epiphyseal Dysplasia


Cleidocranial Dysplasia

Achondroplasia - FGFR3


Diastrophic - DTDST


Marfan - Fibrillin I


Neurofibromatosis - NF-1


Pseudoachondroplasia - COMP


Multiple Epiphyseal Dysplasia - COMP


Cleidocranial - RUNX2

What disorders are autosomal dominant?

Marfan


Achondroplasia


Pseudoachondroplasia


SED Congenita


Cleidocranial dysplasia



What disorders are autosomal recessive?

Diastrophic dysplasia


Hypophosphatemic Rickets


Mucopolysacchroidoses (Hurler, Morquio)

What disorders are X linked recessive?

SED Tarda


Hunter syndrome (ONLY muccopoly)

What disorder is X linked dominant?

Familial hypophosphotemic Rickets

Examination of a patient reveals cafe au lait spots, dysostosis, congenital pseudoarthosis of the tibia and scoliosis but no acoustic neuromas. The patient has a mutant gene that codes for what?

neurofibromin

What cervical spinal condition is important to eval for in OI patients?

Basilar invagination

What treatment has been shown to improve bone density, decrease fracture incidence and improve function when administered to young children with type III OI?

Bisphosphonates

The majority of cases of OI are caused by mutations in genes that encode:

Type I collagen

Marfan syndrome is caused by a defect in the gene that encodes for what protein?

Fibrillin-1

What syndrome is associated with acetabular protrusio?

Marfan's

5 yr old girl walking with limp x5 weeks, now reports Right knee pain. No trauma. Warmth, effusion, no erythema of R knee. Lab studies show elevated ANA, neg RF, normal WBC. What is most likely diagnosis?

Junvenile idiopathic arthritis

A child of short stature, cauliflower ears, hitchhiker's thumb, kyphoscoliosis typically has a defect in what gene?

DTDST (diatrophic dysplasia)

6 yr old with Morquio is scheduled for LE surgery. Pre surgical testing should include

C-spine radiographs

What two conditions are associated with increased incidence of malignant hyperthermia?

Duchenne and Osteogeneis imperfecta?