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307 Cards in this Set
- Front
- Back
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What is a meningocele |
Most common form of spina bifida - 90% Cyst without cord/root involvement |
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What is a lipomeningocele |
A form of lumbosacral lipoma Associated with tethering and distematomyelia |
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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 |
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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 |
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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 |
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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 |
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How do you evaluate and treat a tethered cord |
MRI Surgically with a release |
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What is a common allergy associated with spina bifida |
Latex Acute - IGE mediated Delayed - T cell mediated |
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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 |
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In spina bifida, what position is the foot in |
equinus, cavus, valgus |
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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 |
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How do fractures present in spina bifida? How are they treated? |
Red, hot, swollen extremity Non-operative. Well padded splint |
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What is mode of inheritance of Duchenne |
X-linked recessive (almost all boys) |
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What are some hallmarks of Duchenne? |
Delayed ambulation (>18 months) Calf hypertrophy toe walking progressive scoliosis in >90% once non-ambulatory |
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What is the genetic abnormality in Duchenne and Becker? |
Dystrophin ABSENT in Duchenne Dystrophin DEFICIENT in Becker's Causes fragility of myofiber membrance |
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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" |
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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 |
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What surgical complication are Duchenne's patients at increased risk for? How is this treated? |
Malignant hyperthermia
Dantrolene |
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What is mode of inheritance of Spinal Muscular Atrophy? |
Autosomal recessive |
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What is genetic abnormality in Spinal Muscular Atrophy? |
deficiency of survival motor neuron (SMN) protein --> causes loss of ANTERIOR HORN CELLS |
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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 |
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How are hip dislocations and scoliosis treated in Spinal Muscular Atrophy? |
Hip dislocation - no treatment Scoliosis - fuse in SMA III (to pelvis if nonamb) |
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What is the classic triad in Friedreich's Ataxia? |
Ataxia Loss of deep tendon reflexes Extensor babinski response |
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What is associated with Friedreich's Ataxia? What do they die from? |
Hypertrophic cardiomyopathy and DM Die of cardiomyopathy |
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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 |
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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 |
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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 |
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Which GMFCS level is most highly associated with hip dislocation? |
5 |
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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!! |
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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 |
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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 |
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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 |
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What are indications for "selective" dorsal rhizotomy |
For ambulatory, spastic diplegia - GMFCS I/II |
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What brace is best to address crouch gait |
Ground reaction AFO - pushes knee into extension |
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Is bracing indicated for paralytic scoliosis? |
No - does not stop progression |
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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 |
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Where is acetabular deficiency in CP? |
Posterolateral |
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What are VDRO indications in patients >8, <8? |
<8 - uncovering >60% >8 - uncovering of 40% |
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What is most common CP Answer? |
Spastic diplegia |
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A 3 yr old with L3 myelomeningocele has bilateral dislocated hips. Management should consist of |
observation |
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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) |
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What is the muscle pair to the tibialis anterior? Tibialis posterior? |
TA - peroneus longus TP - peroneus brevis |
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What do the ectoderm and endoderm contribute musculoskeletally? |
Ectoderm - spinal cord Endoderm - notochord --> nucleus pulposus |
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When does the notochord disappear |
When somites form vertebrae from mesoderm |
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What does notochord persist as in an adult? |
Nucleus pulposus |
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What is a chordoma a remnant of? |
Notochord remnant |
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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) |
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What does thalidomide inhibit? |
Apical ectodermal ridge --> limb formation |
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What gene is important for longitudinal growth? |
Sonic hedgehog |
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What is important for ulna-radius differentiation? |
ZPA - Zone of Polarizing Activity |
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Osteopetrosis is a dysfunction of |
osteoclasts |
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What is first secondary ossification to form? When?
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Distal femur
39 weeks |
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What is primary limiting membrane and mechanical support for the periphery of the physis? |
Perichondral ring of La Croix |
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What are the 3 zones of the normal growth plate? |
Reserve Proliferative Hypertrophic |
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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) |
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What provides latitudinal growth to growth plate? |
Groove of Ranvier |
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What reinforces growth plate and binds it to the periosteum? |
Perichondral ring of La Croix |
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What does thyroxine deficiency cause? |
growth retardation, epiphyseal necrosis, SCFE Essential for cartilage growth, maturation |
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When do boys and girls stop growing?
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Boys - 14
Girls - 16 |
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What ossification centers are present at birth? |
distal femur proximal tibia calcaneus talus cuboid |
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What is the difference between an exon and an intron? |
Exon - part of DNA that encodes RNA Intron - spacer DNA that is removed |
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Does achondroplasia have complete or incomplete penetrance? What is the difference? |
Complete penetrance. Phenotype will always match genotype |
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Match: Structural and Enzyme Autosomal Dominant and Recessive |
AD - structural AR - Enzyme |
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What is mode of inheritance for Familial Hypophosphatemic Rickets? |
X-linked Dominant |
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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 |
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How are patients with PVL S Aureus treated? |
Clindamycin |
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What organism is most common in Acute Hematogenous Osteomyelitis (AHO) in patients age 6-48 months? |
Kingella Kingae (gm negative) |
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How is Kingella Kingae detected? |
PCR |
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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% |
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What is most sensitive test for acute hematogenous osteomyelitis? |
CRP - elevated in 98% of patients within 6 hrs |
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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 |
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In the embryo, cells from the ectodermal layer give rise to which elements of the spine? |
Spinal cord |
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What do the endoderm and mesoderm contribute to the spine? |
Endoderm - notochord ---> nucleus pulposus Mesoderm - muscles and bony elements of spinal column |
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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 |
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When does the medial sternoclavicular joint epiphysis close? |
age 23 |
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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 |
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The proximal epiphysis of the humerus contributes what % to growth? |
80% |
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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 |
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What is frequently interposed in proximal humerus fracture |
Biceps Periostem/joint capsule |
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What is Baumann's angle? What is normal Baumann's angle? |
capetillar epiphysis and joint line Normal is 72 degrees |
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What is most commonly involved nerve of supracondylar fractures? flexion-type? |
AIN Flexion type - Ulnar nerve |
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What construct is recommended for supracondylar humerus fractures? What is strongest construct biomechanically? |
Divergent lateral pins Strongest construct - one medial, one lateral |
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Pulseless, pink hand pre reduction? Post reduction? Avascular? |
CRPP Serial exams Vascular exploration (never angiogram) |
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WHat is the most likely indicator for a pediatric compartment syndrome post supracondylar humerus fracture? |
Increasing pain med requirement |
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What is the most common complication to SCH fx? And why? |
Cubitus varus secondary to malreduction |
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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 |
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What are surgical indications for pediatric elbow lateral condyle fractures? |
2-4mm displaced with hinge - CRPP, +/- arthrogram >2mm with malrotation - ORIF |
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What is important to note in approach to pediatric lateral condyle fractures |
Maintenance of posterior soft tissue attachments |
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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 |
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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 |
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What determines your treatment of monteggia fractures in peds? |
Stability and length of ulna |
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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 |
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WHat is common complication of one inc technique in ORIF bbffx? |
Synostosis |
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How much does distal radial physis contribute to longitudinal growth? |
80% |
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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 |
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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 |
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How much angulation is tolerated in peds base of 1st metacarpal fractures? |
30 degrees |
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WHat is a Seymour's fracture? |
Physeal fracture of distal phalanx with nailbed laceration and incarceration of germinal matrix |
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How are pediatric phalangeal neck fractures treated? |
Closed vs open reduction, pin fixation |
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How are pediatric phalangeal physeal fractures usually treated? |
Closed reduction, buddy taping *must check post reduction xrays |
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What is blood volume/kg in children |
75 ml/kg |
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What are three A's to pediatric compartment syndrome? |
Anxiety, agitation, Analgelsic requirement |
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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 |
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What nerve injury is a/w pediatric posterior hip dislocation? |
Peroneal branch of sciatic nerve |
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What is often interposed/preventing reduction of pediatric hip dislocations? |
Interposed labrum, capsule, ligamentum teres, osteochondral fragment |
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What increases risk of AVN in pediatric hip fractures? |
Increased displacement Lower Delbet type I - transepiphyseal II - transcervical (most common) III - basicervical IV - intertrochanteric |
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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 |
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What occurs with trochanteric apophyseal arrest? overgrowth? |
Arrest --> coxa valga Overgrowth --> coxa vara |
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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 |
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Distal femur physeal fractures operative indication? Fixation technique? |
Displacement Smooth crossed pin fixation or transverse lag screw if metaphyseal fragment is large enough |
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Most common post operative complication of distal femura physeal fractures? |
Growth arrest (60%) |
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What is most likely incarcerated in an irreducible tibial spine fracture? |
anterior horn of medial meniscus |
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What is most common post operative complication of tibial spine fractures? |
Arthrofibrosis |
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What is non-operative indication for tibial tubercle fracture? |
<2mm displacement Tx with LLC |
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What is most devastating complication of tibial tubercle fractures? Why does it happen? |
Compartment syndrome Recurrent anterior tibial artery |
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What is a Cozen's fracture? |
Proximal tibial metaphyseal fracture |
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What is the most common complication of Cozen's fracture? |
Valgus deformity (50%) Increases over 18 months, then corrects Initial tx - observation |
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What is accepted in pediatric tibial shaft fractures? |
<1cm shortening, 5-10 deg angulation RISK OF VARUS WITH INTACT FIBULA |
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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% |
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What are indications for bar excision in physeal arrests? |
Bar excision if <50% of physis involved, >2 yrs left of growth |
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In what direction do distal tibial physis close? |
Central --> Posterior --> Medial --> Lateral |
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What is a tillaux fracture? |
anterolateral distal tibial epiphyseal fracture caused by avulsion of anterior inferior tib-fib ligament |
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What is displacement pattern of a triplane fracture? |
Epiphyseal sagittal plane fracture Metaphyseal coronal plane fracture Physeal displacement in axial plane |
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SH I/II fractures of great toe should be treated with? |
I&D, antibiotics, nail bed repair, +/- K wire Treat similar to seymour fracture |
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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 |
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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 |
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What action do AER/FGF promote? ZPA/Sonic hedgehog? Wnt? |
AER/FGF - prox/distal, interdigital necrosis ZPA/Sonic Hedgehog - Radioulnar Wnt - Dorsovolar |
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Transverse Failure of formation congenital anomalies are due to dysfunction of what signaling pathway? |
AER --> FGF |
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Longitudinal failure of formation congenital anomalies are due to dysfunction of what signaling pathway? |
ZPA --> Sonic Hedgehog |
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How should you further work up longitudinal failures of formation? |
CBC, renal ultrasound, echo |
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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 |
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WHat is a contraindication to treatment of radial longitudinal deficiency? |
Elbow contracture/fusion |
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With radial longitudinal deficiency, centralization procedures aim to centralize to what? |
3rd metacarpal |
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Ulnar and radial longitudinal deficiency are associated with wrist and elbow stability/instability? |
RLD - stable elbow, unstable wrist ULD - unstable elbow, stable wrist |
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Cleft hand is a failure of differentiation or formation? |
Formation |
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Radioulnar synostosis is a failure of differentiation or formation? what position is forearm in? |
Differentiation Pronated |
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Syndactyly is associated with what other syndromes? |
Poland's (absent pec major, hypoplasia) Apert's (Acrocephalosyndactyly) Carpenter's (acrocephalopolysyndactyly) |
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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 |
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Camptodactyly is a failure of formation or differentiation? How is it treated? |
Camptodactyly - digital flexion contracture Differentiation Splinting Surgery for contracture >40 deg |
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What is clinodactyly? How is treated? |
digital angulation in radioulnar plane surgical options include physiolysis, osteotomy |
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What determines whether a hypoplastic thumb is saved/reconstructed vs pollicization? |
IIIA - absent extrinsics, STABLE CMC joint IIIB - absent extrinsics, UNSTABLE CMC joint |
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What is difference between pre and post axial duplication? |
Pre - thumb Post - other digitf |
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What is most common Wassel pre axial duplication? |
Wassel type IV - duplication of both prox and distal phalanges, one metacarpal |
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How are pre-axial duplications treated? |
Ablation of radial thumb Preservation of EPL/FPL Collateral ligament reconstruction (often RCL) |
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Post axial duplication mode of inheritance? Demographic? |
AD African-americans>white Caucasians should get genetic eval |
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Macrodactyly is a/w what syndromes? |
NF Proteus Klippel-Trenaunay-Weber |
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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 |
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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) |
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What are indications for brachial plexus microsurgery? |
Horner's syndrome+flail limb+absent biceps at 3 month Absent biceps at 6-9 months |
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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 |
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How is congenital torticollis treated? |
Stretching Hip xray to r/o DDH 5% who fail may need release of SCM |
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How does idiopathic atlantoaxial subluxation present? |
SCM is tight on SAME side of rotation may follow minor trauma or URI (Grisel's) |
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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 |
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How should infantile scoliosis be evaluated? |
MRI to r/o syrinx, chiari, tether |
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What factors predict progression of infantile scoliosis? |
Present over age 1 Rib vertebral angle (RVA)>20 Curve >40 |
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How is infantile scoliosis treated? |
Cast then brace for progressive type <4, curve <50 Operate if curve >60, failure of cast |
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Juvenile scoliosis should be worked up with what? |
MRI |
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Pulmonary compromise in AIS occurs at what curve? |
>70 degree thoracic curve |
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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 |
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When is peak height velocity? |
Risser 0 Between 11-13 for girls Between 13-15 for boys |
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What are indications for MRI in AIS? |
Significant back/neck pain Neuro abnormality Left thoracic curve <10 yr old with curve >20 |
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When should an anterior release be performed in AIS? and why? |
Curves >70 degrees Open triradiate cartilages --> causes crankshaft phenomenon |
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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 |
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How is Scheurmann Kyphosis treated? |
MOST - non op Brace - curve <70, risser <3, flexible deformity |
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Failure of formation/differentiation --> Vertebral bar/hemivertebrae |
Bar - failure of segmentation Hemivertebra - Failure of formation |
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What evaluation should be done in congenital scoliosis? |
Echo and renal ultrasound, spine MRI |
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What are specific indications for convex hemiepiphyseodesis? |
Concave growth potential (NO BAR) <5 involved vertebrae <70 deg curve <6 yr old |
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Halo immobilization in young children require how many pins and torque? |
8-12 pins LOW torque 4 ft/lbs |
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How is SCIWORA evaluated and treated? |
MRI - most sensitive at 48 hrs 12 weeks immobilization |
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What are Ortolani and Barlow maneuvers? |
Ortolani - hip is OUT and reduces with ABD Barlow - hip is IN, BACKS out with ADD |
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What are 3 conditions associated with DDH? |
Metatarsus adductus Torticollis Congenital knee dislocation |
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How is DDH clinically diagnosed in patients >3 months? |
Limitation of abduction Galeazzi sign LLD |
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What is an abnormal alpha angle in infant hip ultrasound? Abnormal beta (femoral head angle covered)? |
Alpha angle <60 degrees Beta >55 degrees |
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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 |
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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 |
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What are approaches to open reduction of infant/toddler hip? |
Medial - > AVN risk? (<1 yr old) Anterolateral - capsule plication and osteotomies |
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What are common blocks to reduction of dislocated hip? |
psoas capsule Inverted labrum/limbus Ligamentum teres Pulvinar TAL |
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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 |
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What is a reasonable indication for observation of DDH? |
Children >5 yr old with bilateral dislocations SMA Spina bifida |
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What are the major complications to treating DDH? |
Residual dysplasia (20%) AVN (10-20%) hyperabd, forced abd, medial approach Redislocation (5%) NV damage (1%) |
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What disorders are a/w Legg Calve Perthes? |
Protein C/S deficiency (Hypercoag disorders) |
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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 |
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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 |
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What is on differential for bilateral perthes? |
Spondyloepiphyseal dysplasia tarda Multiple epiphyseal dysplasia Meyer's dysplasia Hypothyroidism Steroids |
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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 |
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What stage and age do you consider treatment for Perthes? |
Fragmentation stage Stage B/C Age >8 |
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What are some treatment options for Perthes? |
Petrie casting Salter pelvic osteotomy VDRO |
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What factors are protective in Perthes? |
Age <8 <50% head involvement |
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For SCFE: Avg age Gender Demographic |
9-16 M>F Obese Pacific Islander/African American |
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What work up should be done for bilateral SCFE? |
Endocrine - hypothyroid - hypogonadism - panhypopituitaryism - renal osteodystrophy |
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What differentiates acute vs chronic? |
< or > 3 weeks |
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What zone of epiphysis is fractured in SCFE |
Hypertrophic |
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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) |
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What is present on clinical exam of SCFE? |
Painful IR Decreased IR/ABD/Flex Obligate ER with flexion Ext FPA |
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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 |
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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 |
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How is SCFE treated? |
gentle operative positioning reduction (if any reduction at all) In situ perc pin |
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What are parameters of screw fixation in SCFE? |
Center-center of head 3-5 mm short of chondral surface 4 threads past physis |
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When should a contralateral hip be pinned in SCFE? |
Bilateral (symptoms or abnormal xray) Endocrinopathy/metabolic disorder Age <10, obese |
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What are the risk factors for chondrolysis in SCFE? |
Hardware <3mm from subchondral surface Persistent hardware penetration Prolonged pre-treatment symptoms Cast immobilization |
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What is major complication to SCFE? What are risk factors? |
AVN - acute, unstable slip (50% AVN risk) - overreduction - superolateral pin placement - femoral neck osteotomy |
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What are the 4 deformities of clubfoot? |
CAVE Cavus (mismatch betw foreft pronation, hindft sup) Forefoot ADDuction Hindfoot Varus Ankle Equinus |
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What is the pathophysiology of clubfoot? |
Rotation of the navicular causing uncovering of the talus |
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What syndromes and associations are there with clubfoot? |
Diastrophic or streeter dysplasia Prune-belly syndrome Tibial hemimelia Arthrogryposis Spina bifida |
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What radiographic findings are found in clubfoot? |
Adductus of talar-1st metatarsal on AP Parallelism of talus and calcaneus on both AP and lateral |
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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 |
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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 |
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What deformities typically recur in clubfoot? |
Equinus and varus |
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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 |
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What position is the ankle/foot in congenital vertical talus? |
Ankle equinus, midfoot dorsiflexion Hindfoot eversion, forefoot abductionWI |
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With congenital vertical talus, where does the navicular sit in relation to the talus? |
Dorsally dislocated |
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What is congenital vertical talus associated with? |
50% due to genetic or neuromusc cause Spina bifida sacral agenesis diastematomyelia arthrogryposisWhat |
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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 |
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How is congenital vertical talus treated? |
serial casting Surgery at 6-12 months - Lengthen EHL, EDL, TA, peroneals, achilles - reduce and pin talonavicular joint |
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What are the most common tarsal coalitions? |
Calcaneonavicular (age 8-12) Talocalcaneal (12-15) |
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How do tarsal coalitions commonly present? |
Rigid flatfooot (hindft valgus persists with heel rise) Frequent ankle sprains, peroneal spasm |
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Calcaneonavicular coalitions are best evaluated with what xray? Finding? |
45 degree oblique Anteater |
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Talocalcaneal coalitions demonstrate what radiographic signs? |
C sign of lefleur - outline of talar dome and sustentaculum (on lateral) Talar beaking Decreased subtalar motion |
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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 |
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IF a patient has bilateral cavus feet, what eval should be undertaken? |
Genetic testing for Charcot Marie Tooth (hereditary motor sensory neuropathy) |
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What is weak/strong with cavus foot? |
Weak - TA, peroneus brevis Strong - peroneus longus |
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What orthotic should be prescribed for cavus foot? |
Full length, semi-rigid insole, lateral hindfoot positing, first ray depression |
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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) |
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How are accessory naviculars approached operatively? |
Splitting of posterior tibialis Most common cause of persistent pain in inadequate resection |
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What are some benign pediatric foot conditions? |
Metatarsus adductus Calcaneovalgus foot Flexible flatfoot Osteochondroses Kohler's |
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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 |
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What is Sever's disease? |
Calcaneal apophysitis |
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What is Iselin disease? |
5th metatarsal apophysitis - often confused as pseudojones/fx Often athletes age 8-13 |
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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 |
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How are curly toes treated? |
Usually observation Flexor tenotomy if symptoms and >3 yr old |
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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 |
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What further workup should be done in a patient with hemihypertrophy? |
Abdominal u/s and AFP to eval for Wilm's tumor |
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What is the most accurate way to eval limb length discrepancy? |
CT scanogram |
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How much growth is expected in distal femur/prox tibia per year? |
Distal femur - 9mm Proximal tibia - 6mm |
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How much LLD is acceptable at maturity? |
2cm |
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How much acute shortening can be achieved in femur? Tib-fib? |
Femur 8-10cm Tib-Fib 3 cm |
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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 |
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What is normal knee angulation as growth proceeds? |
18-24 months - physiologic varus 5-6 year - physiologic valgus >7 - normal adult alignment |
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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 |
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What are the components of miserable malignment? |
Femoral anteversion External tibial torsion flat feet |
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What are surgical indications for tibial torsion? |
Persistence >5 yr old >20 deg IR Rarely indicated unless clubfoot, CP, MM, Blounts |
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What is mode of inheritance of tibial hemimelia? |
AD |
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What determines treatment of tibial deficiency? |
No extensor mechanism - knee disarticulation +extensor mechanism - reconstruction - tib-fib synostosis, lengthening Ankle stability/length determine foot retention |
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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 |
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What determines treatment of fibular hemimelia? |
Predicted LLD Status of knee/ankle (cruciate def, LFC hypoplasia) |
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When is a Syme amputation indicated in fibular hemimelia? |
Unstable ankle <3 ray foot (relative) |
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What is best radiographic eval of knee OCD? |
Tunnel (notch) view to eval lateral aspect of MFC MRI to determine extent of lesion |
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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 |
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How are discoid menisci diagnosed radiographically? |
Lateral joint space widening MRI - loss of bow tie in 3 consecutive cuts |
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How are discoid menisci treated? |
ONLY IF SYMPTOMATIC - saucerize |
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Congenital knee dislocations are associated with what syndromes? |
Arthrogryposis Myelomeningocele Larsen's HIP DYSPLASIA (MUST RULE OUT) |
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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 |
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What is the mode of inheritance for Neurofibromatosis? |
AD |
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What is gene mutation, gene product and pathway affected in Neurofibromatosis? |
NF-1 affectes neurofibromin - regulation of Ras pathway |
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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 |
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What is mutation, abnormal protein and mode of inheritance for OI? |
COLIA1 or IA2 genes Decrease type I collagen AD or AR inheritance |
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What are clinical features of OI? |
blue sclera hearing loss increased risk of malignant hyperthermia Basilar invagination (HA, neck pain, neuro signs) |
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What is mode of inheritance, gene mutation in Marfan's? |
AD Fibrillin I mutation |
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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) |
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What is important for pre op planning with Marfan's? |
Pre op cardiac eval and MRI for dural ectasia |
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Most Ehler's Danlos mutations occur in what type of collagen? |
V |
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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 |
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What are ortho manifestations of Down's syndrome? |
atlantoaxial instability scoliosis spondylolisthesis SCFE Hip and patellar instability |
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What other conditions are a/w Juvenile Idiopathic Arthritis? |
Iridocyclitis (SHOULD SEE OPTHO) pericarditis tenosynovitis |
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Where is defect in Rickets? |
Zone of provisional calcification |
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What are radiographic features of Rickets? |
Increased physeal width Metaphyseal cupping Bowing |
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What are the 3 types of Rickets? |
Familial Hypophosphotemic Hypophosphatasia Nutritional/Vitamin D deficient |
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What is mode of inheritance of Familial Hypophosphotemic Rickets |
X linked dominant |
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What is defect in familial hypophosphotemic rickets? How is it treated? |
Impaired renal tubular phosphate absorption Treated with Vitamin D and phosphate |
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What is mode of inheritance of hypophosphotasia rickets? Where is defect? |
AR Defect in alkaline phosphatase |
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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) |
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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 |
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What is associated with Streeter Dysplasia? |
Congenital constriction bands Clubfeet Syndactyly Anterolat bowing/pseudoarthrosis Cleft lip/palate cardiac/renal issues |
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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) |
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What is mode of inheritance of Achondroplasia? |
AD |
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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 |
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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 |
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What is mode of inheritance of Diastrophic Dysplasia? Where is gene mutation? |
AR Sulfate transporter DTDST gene |
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What are several clinical signs of Diastrophic Dysplasia? |
Cauliflower ear Cleft palate Hitchhiker thumb |
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How is early arthritis treated in Diastrophic Dysplasia? Rigid clubfoot? |
Early arthroplasty Ponsetti casting |
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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 |
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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 |
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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 |
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What is mode of inheritance of Spondyloepiphyseal Dysplasia (SED) Congenita? Where is defect? |
AD Collagen type II |
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What is mode of inheritance of Spondyloepiphyseal Dysplasia (SED) Tarda? |
X linked recessive Less severe than SED Congenita (AD) |
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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 |
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Where is gene mutation in Multiple Epiphyseal Dysplasia? Where is abnormality? |
COMP IX collagen --> collagen II dysfunction Abnormal ossification of epiphyses with normal spine |
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What are some common features of Mucopolysaccharidoses? |
C1-2 instability Increased rate of CTS Hip dysplasia, abn epiphyses Limb malignment |
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What is mode of inheritance of Mucopolysaccharidoses? What is exception? |
AR - Hurler, Morquio HUNTER - X-Linked Recessive |
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What are features of Morquio? |
Corneal clouding Poor dentition Odontoid hypoplasia (C1/2 instab) |
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What are 3 skeletal dysplasias and 1 syndrome associated with C1/2 instability? |
Mucopolysaccharidoses (Morquio) Pseudoachondroplasia SED Congenita and Tarda Down Syndrome |
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Disorders requiring pre op spine MRI's to eval for dural ectasias |
Neurofibromatosis Marfan's |
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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 |
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What disorders are autosomal dominant? |
Marfan Achondroplasia Pseudoachondroplasia SED Congenita Cleidocranial dysplasia |
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What disorders are autosomal recessive? |
Diastrophic dysplasia Hypophosphatemic Rickets Mucopolysacchroidoses (Hurler, Morquio) |
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What disorders are X linked recessive? |
SED Tarda Hunter syndrome (ONLY muccopoly) |
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What disorder is X linked dominant? |
Familial hypophosphotemic Rickets |
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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 |
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What cervical spinal condition is important to eval for in OI patients? |
Basilar invagination |
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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 |
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The majority of cases of OI are caused by mutations in genes that encode: |
Type I collagen |
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Marfan syndrome is caused by a defect in the gene that encodes for what protein? |
Fibrillin-1 |
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What syndrome is associated with acetabular protrusio? |
Marfan's |
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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 |
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A child of short stature, cauliflower ears, hitchhiker's thumb, kyphoscoliosis typically has a defect in what gene? |
DTDST (diatrophic dysplasia) |
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6 yr old with Morquio is scheduled for LE surgery. Pre surgical testing should include |
C-spine radiographs |
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What two conditions are associated with increased incidence of malignant hyperthermia? |
Duchenne and Osteogeneis imperfecta? |
