Peds Flashcards

Question

Operative options for lateral condyle humerus fracture?

Answer 1

1. CRPP * Weiss Type II (2-4mm displacement, intact articular cartilage) * Technique * Closed reduction performed with forearm supinated, elbow extended, varus stress to elbow followed by fragment manipulation anteromedially * Two parallel or slightly divergent K-wires plus a transverse pin to control rotation 2. Open reduction * Weiss and Jakob Type III (articular cartilage disruption, displaced \>4mm, fragment malrotation) * Technique * Kocher interval (anconeus and ECU) * Avoid posterior and distal dissection (risk of fragment AVN) * Anatomic reduction under direct visualization with fluoro confirmation * Fixation * K-wire OR Partially threaded screw

Answer 2

1. Lateral spur 2. Nonunion (due to synovial fluid, pull of common extensor origin, poor metaphyseal circulation to distal fragment) * More common with nonoperative treatment 3. Cubitus varus (20%) * More common in nondisplaced and minimally displaced fractures 4. Cubitus valgus (10%) 5. Tardy ulnar nerve palsy * Progressive ulnar nerve paralysis developing late (average 22 years post injury) * Manage with anterior ulnar nerve transposition 6. Fishtail deformity * Deepening of the trochlear groove, no clinical significance 7. Growth disturbance * Minimal and involved medial aspect of the condyle (little effect on length or deviation)

Answer 3

Roberts maneuver * Elbow valgus * Forearm supination * Elbow extension * Wrist extension

Answer 4

1. Supine 2. Tourniquet and Esmarch 3. Medial incision just anterior to the medial epicondyle 4. Identify fragment and ulnar nerve * Ulnar nerve does not need to be released – protect throughout with blunt retractor 5. Reduction with “milking” technique * Flex wrist, supinate forearm, flex elbow and apply Esmarch from distal to proximal 6. Place wire through medial epicondyle fragment from inside out 7. Use wire to reduce fracture to distal humerus site and hold with additional small wire or 18 gauge needle 8. Drill and advance a 4.0mm partially threaded cannulated screw with washer up the medial column * Avoid bicortical fixation * Avoid olecranon fossa * Usual length is 35-40mm

Answer 5

1. Loss of motion 2. Cubitus valgus 3. Nonunion * Nonoperative (49.2% union) * Operative (92.5% union) 4. Ulnar nerve symptoms * No difference in operative vs. nonoperative 5. Operative * Septic arthritis * Myositis ossificans * Pin tract infections * Radial nerve injury

Answer 6

1. Nonoperative * 2-3 weeks in cast followed by progressive ROM * Indications * \<30° angulation and \<2mm translation * Full pronation and supination 2. Closed reduction * Indications * Unacceptable angulation * Block to supination or pronation * Techniques * Patterson – hold the elbow in extension and apply distal traction with the forearm supinated and pull the forearm into varus while applying direct pressure over the radial head * Israeli – with elbow flexed 90 and forearm in supination apply thumb pressure to anterolateral radial head while forearm is gradually taken into pronation * Esmarch – wrap forearm distal to proximal while holding elbow in varus 3. Percutaneous reduction * Indications * Failed closed reduction after limited number of attempts * Techniques * K-wire joystick technique * Leverage technique – pin is inserted into the fracture site and levered into position * Push technique – blunt end of large K-wire is pushed against the posterolateral aspect of the radial head * Metaizeau technique * A thin flexible nail or smooth wire with a curved tip is inserted from the distal radius into the intramedullary canal of the radius. The tip of the flexible nail is advanced to the fracture site and into the radial head. The nail is then rotated to rotate the radial head onto the shaft 4. Open reduction * Indications * Failed closed and percutaneous reduction attempts * Gap at fracture site after reduction (indicates soft tissue interposition) * Technique * Lateral approach * 1-2 K-wires from radial head (non-articular zone) to radial metaphysis

Answer 7

1. Proximal ulna fracture OR plastic deformation of the ulna 2. Radial head dislocation

Answer 8

Bado * Type 1: Apex anterior proximal ulna fracture with anterior dislocation of the radial head * Type 2: Apex posterior proximal ulna fracture with posterior dislocation of the radial head * Type 3: Apex lateral proximal ulna fracture with lateral dislocation of the radial head * Type 4: Fractures of both the radius and ulna at the same level with an anterior dislocation of the radial head (1-11% of cases)

Answer 9

1. Nonoperative * Bado I, II and III * Technique * Closed reduction * Successful when ulnar length is restored, pattern is length stable and radial head reduces spontaneously 2. Operative * Bado I, II, and III with failed closed reduction * Length unstable ulnar fracture * Bado IV * Technique * Intramedullary nail for length stable patterns * Plate fixation for length unstable/comminuted patterns * Note – annular ligament reconstruction is rarely indicated with restoration of ulnar length

Answer 10

1. Ulnar osteotomy * Osteotomy at point of maximum angulation * Gains length * Redirects radial head to capitellum (apex of ulnar osteotomy correction should point to the direction you want the radial head to go) * fixed with plate and screw 2. Radial head reduction * initially, attempt to reduce radial head into intact annular ligament * if unsuccessful, pie-crust annular ligament and attempt second reduction * if unsuccessful, incise annular ligament and repair around radial neck following reduction 3. +/- annular ligament repair/reconstruction * indicated after ulnar osteotomy reassessed and determined to be adequate or optimized and radial head still unstable * Bell Tawse technique – lateral triceps tendon

Answer 11

1. Features * Posterior dislocation * Round radial head * Hypoplastic capitellum * Bilateral * No history of trauma 2. Treatment * Nonoperative, consider operative in adulthood if symptomatic or restricts ROM (radial head resection)

Answer 12

1. Start with ulna (easier nail passage) 2. Ulna start point either olecranon or lateral to olecranon through anconeus split 3. Radius start point is just proximal to the distal radial physis between the 1st and 2nd compartments 4. Nails are cut as close to bone to minimize irritation but long enough to allow retrieval 5. Tourniquet is not routinely used

Answer 13

1. Limit gentle closed reduction attempts to 1-2 in ED 2. Limit gentle closed reduction attempts to 1-2 in OR (after failed ED attempt) 3. Do not reattempt closed reduction after 7-10 days following injury

Answer 14

1. Nonoperative * Indications * Minimal growth remaining 2. Operative * Indications * \>2mm growth remaining * Progressive deformity * Ulnar sided wrist pain * Limited ROM * Options * Physeal bar resection and interposition * Epiphysiodesis * Partial arrest = epiphysiodesis of remaining growth plate * Complete arrest = ulnar epiphysiodesis * Ulnar shortening osteotomy * Radial osteotomy * Corrects angular deformity * Distraction osteogenesis

Answer 15

1. \<4 = MFCA, LFCA and artery of ligamentum teres 2. \> 4= MFCA predominately

Answer 16

1. Avoid incising capsule across intertrochanteric line 2. Avoid posterior dissection of the femoral neck

Answer 17

1. Osteomyelitis 2. Simple and aneurysmal bone cysts 3. Fibrous dysplasia 4. Langerhans cell histiocytosis 5. Osteogenesis imperfecta 6. Disuse osteopenia 7. Metabolic bone disease 8. Malignancy

Answer 18

1. Perform urgently within 24 hours 2. Age \<10 perform on radiolucent operating table, ≥10 perform on fracture table 3. Perform with hip in extension, slight abduction and internal rotation, apply longitudinal traction and gentle adjustments in leg position 4. Avoid forceful manipulation 5. Anatomic reduction is desired but some angulation is acceptable 6. Closed reduction is preferred and usually successful

Answer 19

1. Open hip fracture 2. Vascular injury requiring repair 3. Pathological hip fracture requiring bone culture, biopsy, or grafting 4. Failed closed reduction

Answer 20

1. Anterior (Smith-Peterson) * Requires separate incision for fixation 2. Anterolateral (Watson-Jones) 3. Lateral (Hardinge)

Answer 21

1. Fracture stability is more important than preservation of the physis 2. Transphyseal screw fixation is the most stable and is recommended for most fractures 3. Physis-sparing fixation is less stable (recommended only in children \<4 to 6) 4. Fracture fixation is dependent on patient age, patient size and skeletal maturity 5. Capsular decompression should be performed after fracture reduction and fixation 6. Spica cast use is dependent on fracture type, fracture fixation and patient size

Answer 22

1. Children \<8 years 2. Pathological fractures that are not stable after fixation 3. Fractures treated with smooth K-wires 4. Fractures treated with physeal sparing technique

Answer 23

1. Placed no less than 5mm from the subchondral bone 2. Avoid posterior perforation or screw placement in the anterolateral quadrant of the epiphysis (reduce risk of iatrogenic injury to blood vessels) 3. Avoid transphyseal fixation in patients \<10 (however stable fixation should not be compromised to spare the physis) 4. Postoperatively can TTWB with crutches if stable pattern

Answer 24

1.Osteonecrosis (most common) * What are the predictors for osteonecrosis? * Fracture type * Type IB = highest risk (100%) * Type I (38%) \> Type II (28%) \> Type III (18%) \> Type IV (5%) * Reported rates of osteonecrosis according to the Delbet classification are 38% to 50% for type I, 28% for type II, 8% to 18% for type III, and 5% to 10% for type IV. * Older patient * Age \>10 * Displaced fractures * What is the Ratliff classification of osteonecrosis? * Type I = entire femoral head * Type II = segments of femoral head * Type III = femoral neck 2. Coxa vara * Femoral neck shaft angle \<120 3. Premature physeal closure * Can lead to coxa valga or vara (asymmetric arrest) * Can lead to LLD 4. Nonunion * Up to 10% * Most common in Type II * Management is a valgus osteotomy 5. Chondrolysis 6. Infection 7. Posttraumatic SCFE 8. Overgrowth of the femoral shaft

Answer 25

1. ≤6 months a.Pavlik harness * Early spica casting 2. 6 months – 5 years * Stable fracture pattern * Early spica casting * Unstable fracture pattern * Traction with delayed spica casting * External fixator 3. 5-11 years * Length stable fracture and patient \<100lbs * Flexible IM nails * Length unstable fracture OR very proximal/distal fracture OR any weight * Submuscular plating 4. ≥11 years * Patient ≤100lbs * Flexible IM nails * Patient \>100lbs * Antegrade rigid IM nail * Proximal or distal fracture OR comminution * Submuscular plating

Answer 26

1. Patient is supine on a radiolucent flattop table 2. Fracture reduction is achieved with knee flexion, traction and F-tool 3. Use two nails with a combined diameter equal to 80% of the IM canal at its narrowest width 4. Retrograde start point is ~2.5cm from the distal femoral physis 5. Nails are rotated so the concavities face each other and remain symmetric 6. For better rotational control, one nail is advanced to the femoral neck and the other to the greater trochanter 7. Only 1-1.5cm of the nail should remain outside the bone 8. A knee immobilizer is applied at completion of case

Answer 27

1. Largest nail possible * Each nail should be 40% of the minimum diameter of the diaphysis 2. Two nails * Achieving 80% canal fill 3. Prebend nails * 30 degree C-shaped bend at the level of the fracture 4. Opposite bends of the two rods at the fracture site (spread within the diaphysis) * Resists bending 5. Divergence of rods in metaphysis * Torsional control

Answer 28

Lateral trochanteric entry point

Answer 29

1. Salter-Harris I – II * Nonoperative (bivalved long leg cast) * Nondisplaced * \<2mm displacement after closed reduction * Operative * Reducible but unstable * Two crossed transphyseal smooth pins (antegrade or retrograde) 2. SH II with large Thurston-Holland fragment * Percutaneous partially threaded cannulated screw * In metaphysis parallel to physis and perpendicular to fracture 3.SH III – IV * Nonoperative * Nondisplaced * Operative * \>2mm displacement * Closed possible open reduction to achieve reduction * 4.5- to 7.3-mm cannulated screws are placed parallel to the articular surface of the epiphysis and/or metaphysis

Answer 30

1. Type I - junction of the distal and proximal apophysis * Type IA = nondisplaced/minimally displaced * Type IB = displaced and hinged at junction * Type IC = patellar tendon periosteal sleeve avulsion 2. Type II - junction of tibial tubercle and proximal epiphysis * Type IIA = not comminuted b.Type IIB = comminuted 3. Type III - extend intra-articular (most common) * Type IIIA = not comminuted * Type IIIB = comminuted 4. Type IV - fracture of the tibial tuberosity that extends posteriorly along the proximal tibial physis creating an avulsion of the entire proximal epiphysis 5. Type V - extends intra-articular and posteriorly along physis of proximal tibia (‘Y’ pattern) Note: Ogden modification of Watson-Jones classification was from Type I-III with subclassification A/B * Frankl added type IC

Answer 31

1. Nonoperative * Nondisplaced or minimally displaced (\<2-3mm) Type I 2. Operative * Displaced Type I * Type II-V * Midline anterior incision * Parapatellar arthrotomy or arthroscopy if intra-articular extension * Generally, 2-3 4.0mm cannulated screws with washers are inserted through the tubercle into the metaphysis and epiphysis * Consider prophylactic anterior compartment fasciotomy

Answer 32

1. Genu recurvatum * Rare as most injuries occur as growth is slowing down 2. Compartment syndrome * Anterior tibial recurrent artery at risk 3. Hardware irritation

Answer 33

Meyers and McKeever classification * Type I - minimally displaced * Type II - anterior displacement with intact posterior hinge * Type III - complete fracture displacement * Type IV - complete fracture displacement with fragment rotating out of fracture bed OR comminuted

Answer 34

1. Type I * Nonoperative, long leg cast in slight flexion 2. Type II * Closed reduction and casting * Aspiration of hematoma and injection of local anaesthetic, extend knee fully or near full, long leg cast * Acceptable reduction is \<3mm displacement on lateral view * Operative * \>3mm displacement on lateral view * Open arthrotomy or arthroscopic * Suture fixation through tibial tunnel OR antegrade cannulated screws in epiphysis OR combination * Perform EUA following fixation

Answer 35

1. Anterior horn of meniscus (medial \> lateral) 2. Intermeniscal ligament 3. Rotated fracture fragment

Answer 36

1. Loss of fixation 2. Prominence of hardware 3. Loss of motion 4. Reoperation * Higher in screw vs. suture fixation 5. ACL laxity 6. Arthrofibrosis 7. Nonunion

Answer 37

1. Pearls * Continually palpate calf compartments. * Address concomitant pathology before managing the avulsion fracture * Disengage any interposed soft tissue 2. Pitfalls * Remove only the necessary portions of the intermeniscal ligament * Leave at least 1 to 2 cm between tunnels * Maintain tension on all sutures while each one is tied 3. Potential Complications * Loss of motion/arthrofibrosis * Residual displacement or laxity * Nonunion or malunion * Growth disturbance

Answer 38

1. Timing * 18 month transitional period prior to complete closure * Complete closure at 14 in girls and 16 in boys 2. Progression of closure * Central \> anteromedial \> posteromedial \> posterolateral \> anterolateral

Answer 39

1. Nonoperative * Closed reduction and casting * Delay greater than a week increases risk of physeal injury with closed reduction Operative * Failure of closed reduction * Open reduction and casting * Unstable fracture * Percutaneous fixation * ORIF * Thurston-Holland fragment allows for screw fixation parallel to physis and perpendicular to fracture

Answer 40

1. Presentation * Medial malleolus fractures * Tillaux fractures – avulsion of anterolateral distal tibia epiphysis (AITFL insertion) 2. Nonoperative * \<2mm displacement 3. Operative * \>2mm displacement * Anterolateral approach for Tillaux fractures * Medial approach for medial malleolus fractures * Screw fixation within the epiphysis parallel to physis

Answer 41

1. Presentation * Triplane * Sagittal plane through epiphysis, axial plane through physis and coronal plane through metaphysis * Medial malleolus (vertical) 2. Nonoperative * \<2mm displacement 3. Operative * \>2mm displacement or \>2mm physeal gap * Medial malleolus fracture * Medial approach * Screw into epiphysis and metaphysis parallel to physis * Triplane fracture - closed reduction and perc screws OR open reduction if reduction not achieve by closed means - epiphyseal fracture is usually amenable to anteroalteral or posteromedial placed screws parallel to the physis - metaphyseal fragment usually gets captured wit direct anterior to posterior based screws

Answer 42

1. Corrective osteotomy for angular deformity 2. Fibular epiphysiodesis to limit overgrowth and lateral impingement 3. Physeal bar resection may be considered if \<50% of the physis is compromised and \>2 years or \>2cm of growth remain * Peripheral bars approached directly * Central bars through metaphyseal window Best indications for bar resection (as per Jarvis) * Younger children * Smaller bar * Shorter time interval (since bar development) * Central is better than peripheral * Traumatic etiology (as opposed to infectious, etc) * Healthy surrounding physis

Answer 43

1. No/vague explanation for significant injury. 2. Denial of trauma in setting of significant boney injury. 3. Mechanism of entry, not consistent with fracture type, energy associated with fracture or severity of injury. 4. Injury inconsistent with a child’s physical and/or developmental capabilities. 5. Inconsistent history across caregivers or changing histories provided by caregivers. 6. Different different witnesses with different explanations. 7. Injuries resulting from a family/domestic violence incident. 8. Previous history of inflicted trauma. 9. Witnessed inappropriate behaviour to a child placing them at risk of NAT 10. Delay in seeking care for injury.

Answer 44

Increasing anxiety, agitation and analgesia requirement

Answer 45

1. Ensure normotensive - hypotension should be avoided (decreases perfusion pressure to tissue) 2. Remove circumferential dressings - bivalving and splitting the cast by 0.5cm has been shown to decrease pressure by 47% in the anterior compartment and 33% in the deep posterior compartment of the leg 3. Maintain limb at heart height - elevation can reduce arteriovenous pressure gradient 4. Provide supplemental oxygen

Answer 46

1. Emergent decompressive fasciotomy 2. Myonecrosis should not be debrided at time of fasciotomy - muscle recovery is more robust than adults - myonecrosis has an 80fold increased risk of functional deficit 3. Wound closure should be delayed - VAC may eliminate need for split thickness skin grafts 4. Delayed diagnosis still warrants decompression - in presence of tissue and nerve damage a fasciotomy is warranted due to children’s potential for recovery

Answer 47

Nonop indications - ≤2mm displacement (weiss type 1) Operative - >2mm displacement (weiss type 2 and 3) - Incongruent articular surface - Progressive displacement on serial radiographs - Failure of nonop treatment (delayed healing or inability to maintain or tolerate casting)

Answer 48

Fibula fracture present - risk of valgus alignment (due to contraction of anterior and lateral musculature) Fibula fracture absent - risk of varus alignment (due to contraction of anterior muscles and tethering of the intact fibula) 60% will develop varus angulation in the first 2 weeks

Answer 49

1. Female 2. Feet first (breech) 3. First born 4. Family history 5. Oligohydramnios 6. Swaddling 7. Caucasian

Answer 50

Left hip (60%)

Answer 51

1. Dislocated – Ortolani positive (early), Galleazzi sign 2. Dislocatable – Barlow positive 3. Subluxable – Barlow suggestive 4. Other – asymmetric gluteal fold, decreased hip abduction (\>3 months), wide perineum (bilateral dislocated hips)

Answer 52

* Iliopsoas tendon (creates hourglass capsule) * Adductor tendon (limits abduction) * Inverted labrum * Contracted inferomedial capsule * Transverse acetabular ligament * Pulvinar * Ligamentum teres * Limbus (ridge of cartilage tissue that divides the acetabulum into a true and a false acetabulum)

Answer 53

1. Ultrasound prior to femoral head ossification (\<6months) 2. Radiographs following femoral head ossification (\>6months)

Answer 54

1. Lines drawn parallel to the iliac wing, roof of acetabulum, labrum 2. Alpha angle * Formed between line parallel to ilium and acetabular roof * Normal = \>60 3. Beta angle * Formed between line parallel to ilium and labrum * .Normal = \<55 4. Femoral head should be bisected by line parallel to ilium * Morin index = percentage of the head covered by the acetabulum (below the ilium line) * Calculated as the width of the femoral head below the line divided by the width of the femoral head * Normal = \>50% * Borderline = 46-50% * Abnormal = \<46%

Answer 55

1. Delayed ossification of femoral head (small) 2. Hilgenreiner’s line = horizontal line through the right and left triradiate cartilage * Normal femoral head should be below line 3. Perkins line = line perpendicular to Hilgenreiner’s line passing through point at the lateral acetabular roof * Normal femoral head lies medial to line 4. Shenton’s line = line along the inferior femoral neck and inferior superior pubic ramus * Normal = smooth and unbroken 5. Acetabular index = line parallel to the acetabular roof forms angle with Hilgenreiner’s line * Normal = \<25 after 6 months of age * (24 at 24)

Answer 56

1. Hip flexion 100+/-10 degrees * Controlled by anterior strap – in line with anterior axillary line 2. Hip abduction in the safe zone (between maximum abduction which places head at risk of AVN and adduction point where hip dislocates/subluxes) * Controlled by the posterior strap – at level of scapula * Straps should not force abduction, rather should prevent adduction beyond neutral 3. Chest halter strap at nipple level

Answer 57

1. Transient femoral nerve palsy (excessive flexion) 2. Femoral head AVN (excessive abduction) * Due to compression of the posterosuperior retinacular branch of the medial femoral circumflex artery 3. Brachial plexus neuropathy (compression by shoulder straps) 4. Pavlik harness disease * Persistent pavlik harness use despite unsuccessful reduction resulting in pathologic changes – damage to femoral head, acetabular cartilage * “Prolonged positioning of the dislocated hip in flexion and abduction that potentiates dysplasia, particularly of the posterolateral acetabulum, and increases the difficulty of obtaining a stable closed reduction.” [Jones et al. J Pediatr Orthop. 2018 Jul; 38(6): 297–304.] 5. Skin breakdown (groin and popliteal fossa) 6. Inferior dislocation (excessive flexion)

Answer 58

1. Major muscle imbalance (eg. myelomeningocele – L2 to L4 functional level) 2. Major stiffness (eg. arthrogryposis) 3. Ligamentous laxity (eg. Ehlers-Danlos syndrome)

Answer 59

1.Neonate – 6 months * First line = Pavlik Harness * 95% resolution of hip instability in Ortolani positive hips maintained in Pavlik for 6 weeks * \>50% failure rate if used in patients \> 6 months * Harness applied with followup in one week to confirm reduction (confirmation by clinical exam and US both acceptable) followed by weekly followup to adjust straps and confirm reduction * Duration of treatment variable * Minimum 6 weeks of full time use (23 hours a day) * Usually followed by period of weaning * One algorithm treats until hip normal by US (Graf classification type I) [J Child Orthop. 2018 Aug 1; 12(4): 308–316.] * If not reduced after 2 weeks – discontinue use and consider closed/open reduction at 4-6 months 2. 6 months – 4 years * First line = Closed reduction +/- adductor tenotomy * Closed reduction performed in OR * Reduction achieved with flexion, abduction, longitudinal traction, slight posterior pressure to GT * Arthrogram used to assess quality of reduction (medial dye pool \<5mm or \<16% of the width of the femoral head indicates concentric reduction) * Adductor tenotomy can be performed to widen the “safe zone” * Consider if narrow “safe zone” of less than 40° * Hip spica cast applied with 100° flexion and abduction in the “safe zone” (\<55°) with molding posterior to GT * 100° of flexion and 40–50° of abduction referred to as the “human position” of the hip * Reduction is confirmed with CT (or MRI) * Spica cast use for 3 months * Cast change at 6 weeks – assess reduction, stability and hygiene purposes * Followed by abduction brace fulltime for 4 weeks * Followed by nighttime brace for 4 weeks * Second line = open reduction * Indicated in cases of failed closed reduction * Technique: * Smith-Peterson approach with modified “bikini” incision, adductor tenotomy, psoas recession, T capsulotomy, remove blocks toreduction, capsulorrhaphy (lateral leaf brought medial) * Ligamentum teres is guide to true acetabulum * Possible femoral shortening osteotomy if \> age 3 or under tension after reduction * Possible acetabular procedure if \>18 months * Spica cast is used for approximately 6 weeks with immobilization in about 30 degrees of abduction, 30 degrees of flexion, and 30 degrees of internal rotation

Answer 60

1. Medial * Advantages * Can be done at \<12months * Directly addresses inferomedial blocks to reduction, less blood loss * Disadvantages * Cannot perform capsulorrhaphy or bony work, risk of AVN * Note – Ludloff described interval between adductor longus and pectineus 2. Anterior * Advantages * Performed at \>12 months * Less AVN risk * Can perform capsulorrhaphy or bony work

Answer 61

Needle directed medial to lateral 45° to the thigh and 45° to the horizon aiming towards the ASIS * Inject 1:1 ratio of saline:contrast

Answer 62

* \<6 months = Pavlik harness * 6-12 months = closed reduction and spica casting * Closed reduction, possible adductor tenotomy, hip arthrogram, spica casting, CT/MRI * 12-18 months = open reduction * Open reduction, adductor tenotomy/psoas recession, capsulorrhaphy, spica casting, CT/MRI * 18 months – 3 years = open reduction and pelvic OR femoral osteotomy * \>3 years = open reduction and pelvic + femoral osteotomy * Open reduction, adductor tenotomy/psoas recession, pelvic osteotomy, femoral shortening and derotation osteotomy, capsulorrhaphy, spica casting, CT/MRI

Answer 63

1. Shortening of the femur reduces contact pressure on the femoral head thereby reducing the risk of osteonecrosis 2. Derotation of the femur reduces the excessive anteversion 3. Technique * Performed through a lateral approach * Subtrochanteric osteotomy just below level of LT * Amount of shortening is determined by amount of overlap after femoral head reduced in the acetabulum * Amount of derotation is determined by matching the opposite limb

Answer 64

Improves the stability of the open reduction, improves the coverage of the femoral head * Type of osteotomy is based largely on surgeon preference

Answer 65

1. Improvement in the acetabular index 2. Sharp (not rounded) lateral border of the acetabulum 3. Narrow teardrop 4. Intact Shenton line

Answer 66

1. Failure of femoral head to ossify (or failure of an already present ossific nucleus to grow) within 1 year of reduction 2. Broadening of the femoral neck 3. Increased density of the femoral head (followed by fragmentation) 4. Residual deformity after ossification is complete

Answer 67

Kalamchi and MacEwan * Type I - alteration in the ossific nucleus * Type II - lateral physeal damage * Type III - central physeal damage * Type IV - total damage to the head and physis

Answer 68

1. Obesity (~50% are above the 95th percentile for weight) 2. Boys 3. Pacific Islanders 4. African american 5. Endocrinopathy (hypothyroidism, panhypopituitarism, growth hormone abnormalities, hypogonadism) 6. Radiation to the proximal femur 7. Renal osteodystrophy

Answer 69

1. 13.5 for boys 2. 12 for girls

Answer 70

1. Temporal classification * Pre-slip * Symptoms, no radiographic evidence of slip (may have physis widening/irregularity) * Acute slip * \<3 weeks of symptoms * Chronic slip * \>3 weeks of symptoms * Most common type (85%) * Acute-on-chronic slip 2. Loder classification * Stable – patient can walk and bear weight, with or without crutches * Nearly 0% incidence of osteonecrosis * Unstable – patient unable to walk even with crutches * Up to 50% incidence of osteonecrosis * 10-60% (30%)

Answer 71

1. Displacement in relation to the width of the metaphysis * \<33% = mild * 33-50% = moderate * \>50% = severe 2. Southwick angle * Epiphyseal-shaft angle is measured on the frog-leg lateral * Degree of slip is calculated by subtracting the epiphyseal-shaft angle on the uninvolved side from that on the side with SCFE * \<30 degree = mild * 30-50 degree = moderate * \>50 degree = severe * If both hips are involved 12° is considered normal

Answer 72

1. Widening and irregularity of the physis 2. Metaphyseal blanch sign of Steel * Radiographic double density created by the posteriorly displaced epiphysis overlapping the medial metaphysis 3. Kleins line – epiphysis is flush with or below the line (AP view)

Answer 73

1. Patient placed supine on fracture table with involved extremity in traction 2. Triangulate with fluoro to confirm skin start point * On AP and lateral use a wire placed on the skin so that it projects over the centre of the epiphysis and perpendicular to the physis * Draw a line on the skin parallel to these lines, the point where they intersect a stab incision is made 3. Advance the guidewire free hand through the incision and fascia down to start point on the anterior femoral neck 4. Advance the wire with drill so that the wire is in the centre of the epiphysis and perpendicular to the physis on both the AP and lateral views 5. Measure the screw length with depth gauge 6. Ream over the wire 7. Advance a 7.3mm cannulated screw over the wire such that 4-5 threads engage the epiphysis and the tip is no closer than 5mm within subchondral bone * If \<5 threads across physis, 41% progress \>10° * If 5 threads across physis, no progression 8. Confirm screw does not penetrate joint with the “near-far” technique by taking hip from max internal to external rotation * Screw tip should move closer to subchondral bone (approach), then move away (withdraw) * True position of screw is found at moment approach changes to withdraw 9. Consider pinning contralateral hip based on risk factors

Answer 74

1. Obesity 2. Endocrinopathy (hypothyroidism, GH treatment, etc.) 3. Young age of first SCFE (\<9y) * Skeletally immature/open triradiate cartilage 4. Children with adiposogenital dystrophy (low GnRH, hypogonadism, increased caloric intake/obesity) 5. Unable to comply with close clinical and radiologic observation due to geographic or social reasons 6. High posterior sloping angle (\>14°)

Answer 75

1. Sagittal plane = posterior displacement of epiphysis on metaphysis 2. Coronal plane = displacement of epiphysis into varus 3. Axial plane = external rotation of the femur on the epiphysis

Answer 76

1. Slip angle \<15 (southwick angle) * Arthroscopic femoral neck osteochondroplasty 2. Slip angle 15-30 * Limited open anterior arthrotomy and femoral neck osteochondroplasty 3. Slip angle 30-45 * Surgical hip dislocation and femoral neck osteochondroplasty 4. Slip angle \>30 * Flexion intertrochanteric osteotomy * Achieves flexion, valgus and IR * Modified Imhauser technique * Lateral approach to the proximal femur * Chisel for blade plate is inserted into the femoral neck at the appropriate flexion angle * Transverse osteotomy is made just proximal to the LT * Blade plate is inserted and stabilized with a screw in the proximal fragment * The distal fragment is internally rotated the desired amount and flexed to reduce to the plate 5. Slip angle \>60 * Combined flexion intertrochanteric osteotomy and surgical hip dislocation (possibly staged)

Answer 77

1. Age = 5-8 2. Males (5:1) 3. Bilateral 10-15% 4. Delayed bone age compared to chronological age

Answer 78

* Males * Family history * Delayed bone age * Low birth weight * Second hand smoke * Low socioeconomic status

Answer 79

1. Other causes of AVN * Sickle cell disease * Other hemoglobinopathies (eg. thalassaemia) * Chronic myelogenous leukemia * Steroids * Traumatic hip dislocation * Treatment of DDH * Septic arthritis 2. Epiphyseal dysplasias * MED, SED, mucopolysaccharidoses, hypothyroidism 3. Other syndromes * Osteochondromoatosis, metachondromatosis, Schwartz-Jampel syndrome, others

Answer 80

Waldenstrom * Initial * Fragmentation * Reossification * Healed (residual)

Answer 81

1. Stulberg * Class I - Normal hip joint * Good outcomes * Class II - Spherical head with enlargement, short neck or steep acetabulum * Good outcomes * Class III - Nonspherical head (ovoid, mushroom-shaped, umbrella-shaped) * Poor outcomes, FAI and instability * Class IV - Flat head, flat acetabulum * Poor outcomes, very early OA/FAI * Class V - Flat head with incongruent hip joint * Poor outcomes, very early OA/FAI 2. Three types of congruency are described: * Spherical congruency (classes I and II) * Aspherical congruency (classes III and IV) * Aspherical incongruency (class V) 3. Note: increasing class # correlates with onset of osteoarthritis

Answer 82

Based on the extent of head involvement during fragmentation stage * Group I - anterior head involvement (25%) * Group II - anterior and central head involvement (50%) * Group III - only a small part of the epiphysis is not involved (usually posteromedial) (75%) * Group IV - total head involvement (100%) Note: poor interobserver reliability

Answer 83

1. Lateral subluxation 2. Lateral calcification 3. Diffuse metaphyseal reaction (i.e. metaphyseal cysts) 4. Horizontal growth plate 5. Gage sign * V-shaped radiolucency in the lateral portion of the epiphysis and/or adjacent metaphysis NOTE – indicate a more severe disease course

Answer 84

Based on the height of the lateral 15-30% of the femoral epiphysis (aka. Lateral pillar) during fragmentation phase * Group A - no loss of height * Group B - \<50% loss of height * B/C Border * B/C 1 - lateral pillar is narrow (2-3mm wide) * B/C 2 - poorly ossified * B/C 3 - exactly 50% height loss without central depression * Group C - \>50% loss of height Note: better interobserver reliability compared to Catterall and is prognostic

Answer 85

Based on radiographic crescent sign in fragmentation stage * Class A – crescent involves \<1/2 femoral head involved * Class B – crescent involves \>1/2 femoral head involved Note: Allows for early classificaiton

Answer 86

1. Extent of femoral head deformity and loss of hip joint congruity at maturity (Stulberg classification) 2. Age at onset * \>8y do worse, \<6y do best 3. Extent of subchondral fracture (Salter-Thompson classification) 4. Extent of head involvement at the fragmentation stage (Catterall classification) 5. Two or more Catterall head-at-risk signs (lateral subluxation, lateral calcification, diffuse metaphyseal reaction, horizontal growth plate, Gage sign) 6. Lateral pillar height at the fragmentation stage (lateral pillar classification) 7. Premature physeal closure

Answer 87

1. Lateral extrusion of the femoral head results from synovitis and articular hypertrophy 2. Results in abnormal contact with acetabular rim and predisposes to femoral head deformation 3. Defined as the percentage of the femoral ossific nucleus that lies outside the bony acetabulum (extrusion = A/Bx100)

Answer 88

1. Dynamic hip arthrogram * What 3 findings on hip arthrogram indicate hip abduction as determined from hip adduction to abduction? * The extruded lateral portion of the head contacts the acetabulum and does not move under the lateral acetabulum with further abduction * The center of rotation moves from the epiphysis (or center physis) to the lateral acetabulum * The medial dye pool increases in size 2. Alternative – dynamic radiography * Defined as widening of the medial joint space \>2mm and decreased superolateral joint space with hip abduction

Answer 89

Containment of the femoral head in the acetabulum with the aim to achieve a spherical femoral head and congruent joint ultimately to minimize risk of OA

Answer 90

1. Age \<6 * Nonsurgical * Activity modification and PT * Abduction bracing/casting (i.e. Petrie cast) 2. Age 6-8 * Evidence is not clear * Primary treatment is non-surgical * ROM and XR check q3 months in fragmentation stage * If hip abduction \<30°or lateral subluxation on XR: * Do arthrogram for hinge abduction * If concentric motion: * Brace/tenotomy to maintain motion * If losing motion, do VDRO * If hinge abduction: * Do shelf acetabuloplasty 3. Age 8-11 * Primary treatment is surgical containment * Treat early (before significant femoral head deformity) * Lateral Pillar B and B/C have greatest benefit * VDRO * Pelvic osteotomy * Salter/triple/shelf * Can do staged if irritable hip * Adductor tenotomy/casting x 6 weeks, then VDRO 4. Age \> 11 * Poor prognosis * Effectiveness of surgery unclear

Answer 91

1. Nonoperative containment * Petrie casting * A-frame brace (abduction orthosis) * NOTE: rarely used now given results of operative containment [Lovell and Winter] 2. Protected WB 3. Physiotherapy

Answer 92

1. Chiari or shelf acetabuloplasty 2. Valgus femoral osteotomy

Answer 93

1. Redirectional (volume stable) * Salter * Cuts both columns (requires internal fixation) * Improves anterior and lateral coverage * Rotates through pubic symphysis * Correction = 15° of lateral coverage, 25° of anterior coverage * Triple * Same as Salter but adds superior and inferior pubic rami osteotomies * No hinge, acetabulum free to rotate * Ganz (PAO) * Posterior column intact 2. Reshaping (volume reducing) * Dega * No internal fixation required * Improves lateral coverage * Rotates through the triradiate cartilage * Pemberton * No internal fixation required * Improves anterior coverage * Rotates through the triradiate 3. Salvage * Shelf * Chiari * Medial displacement osteotomy * Hinges on the pubic symphysis

Answer 94

1.75% are autosomal dominant mutations in: * 66% COMP (collagen oligomeric matrix protein) * 24% matrillin-3 (MATN3) * 10% collagen IX (COL9A)

Answer 95

1. Childhood presentation 2. Early fatigue with walking/playing 3. Limited motion 4. Limp 5. Periarticular hip, knee, shoulder pain 6. Contractures hip, knee, shoulder 7. Brachydactyly 8. Mild short stature, normal trunk height

Answer 96

1. Bilateral symmetric involvement 2. Hip * Loss of height, irregular, underdeveloped femoral epiphysis * Short, wide, varus femoral neck * Acetabular irregularities 3. Knee * Genu valgum * Double layered patella (anterior/posterior) - pathognomonic 4. Hands * a. Brachydactyly 5. Spine * Endplate irregularities or Schmorl nodes

Answer 97

Skeletal survey

Answer 98

1. Accurate diagnosis 2 .Family planning (educate on pattern of inheritance and chance offspring will be affected) Note – should be offered to all patients

Answer 99

1. LCP * MED is bilateral, symmetric – LCP is rarely bilateral (13%) * MED can involve joints other than the hips (importance of skeletal survey) * MED is usually associated with acetabular changes – initially acetabulum are normal in LCP * MED does not have metaphyseal cysts 2. Spondyloephiphyseal dysplasia * SED has vertebral body abnormalities and significant scoliosis – MED has minimal or no spine involvement * Mutation in COL2A1 * Spine manifestations [Orthobullets] * Atlantoaxial instability, odontoid hypoplasia or os odontodium, kyphoscoliosis, increased lumbar lordosis, platyspondyly (flattened VB) 3. Congenital hypothyroidism 4. Mucopolysaccharidoses 5. Pseudoachondroplasia 6. Diastrophic dysplasia

Answer 100

1. Static encephalopathy due to injury of the immature brain 2. The resulting nonprogressive upper motor neuron disease results in muscle imbalances that can lead to progressive musculoskeletal dysfunction

Answer 101

1. Physiologic classification * Spastic * Athetoid * Ataxic * Mixed * Hypotonic 2. Anatomic * Quadriplegic * Diplegic * Hemiplegic 3. GMFCS * Level I-V

Answer 102

1. Prematurity 2. Low birth weight 3. Anoxic brain injuries * Meconium aspiration, birth asphyxia, respiratory distress syndrome 4. Perinatal infections (ToRCH) * Toxoplasmosis, Other (syphilis, varicella-zoster, parvovirus B19), Rubella, Cytomegalovirus (CMV), and Herpes 5. Meningitis 6. Brain trauma (NAT) 7. Prenatal intrauterine problems * Placental abnormalities

Answer 103

MRI brain * Periventricular leukomalacia

Answer 104

1. Spasticity and contractures 2. Scoliosis 3. Hip instability and dislocations 4. Foot deformity * Planovalgus, equinovarus 5. Gait abnormalities

Answer 105

1. Multidisciplinary consultations * Pediatrics, anaesthesia, ICU, PT/OT, dietician, APS (pain management team) 2. Investigations * Echocardiogram, ECG, CXR 3. Medications * Continue anti-spastic and anti-epileptic medication 4. Optimize nutrition 5. Difficult airway * Restricted mouth opening, poor dentition, difficult positioning, excess salivation 6. Difficult positioning (contractures) 7. GERD/aspiration risk 8. Prone to hypothermia 9. ICU post op 10. Consider chest physio

Answer 106

* Physical exam * Kinetic analysis * Kinematic analysis * Force plate (pedobarography) * Dynamic EMG * Video

Answer 107

1. Stance phase patterns * Normal * Jump Gait * Characteristics = loss of heel strike at initial contact and toe contact pattern for duration of stance phase * Subdivisions: * *True equinus* = plantarflexion relative to the tibia * Subdivisions: * Normal knee/hip * Extended knee/hip * Flexed knee/hip * *Apparent equinus* = normal alignment relative to the tibia with flexed knee and hip * Crouch Gait * Characteristics = flat-foot or calcaneal contact for the duration of stance phase due to ankle plantarflexion muscle group insufficiency + knee flexion * Subdivision: * *Compensated* = knee is offloaded in midstance by hip flexion, anterior pelvic tilt, anterior trunk tilt * *Uncompensated* = knee is not offloaded in midstance 2. Swing phase patterns * Normal * Stiff Gait * Characteristics = limited knee flexion during swing phase * Subdivisions * *Knee source* = limited knee flexion due to spasticity of the rectus femoris * *Hip source* = due to deviations at the hip (decreased flexion and internal rotation)

Answer 108

1. Jump gait, true equinus, normal knee/hip * Single level management * Botox or TAL or gastroc recession 2. Jump gait, true equinus, hyperextended knee/hip * Same as above (spontaneous resolution of knee/hip extension) 3. Jump gait, true equinus, flexed knee/hip * Tone management (Botox or intrathecal Baclofen) and single event multilevel surgery (SEMLS) * Management summary [European Journal of Neurology 2001;8 (Suppl. 5), 98-108]: * Spasticity management * Botox injections to calf, hamstrings, (hip) * Selective dorsal rhizotomy * Contracture management * SEMLS – gastroc, hamstring, psoas lengthening 4. Jump gait, apparent equinus * Direct management of knee/hip deviations (do not address the ankle) * Management summary [European Journal of Neurology 2001;8 (Suppl. 5), 98-108]: * Spasticity management * Botox injections to hamstrings, iliopsoas * Contracture management * SEMLS – hamstring, psoas lengthening * Inappropriate TAL or gastroc recession will result in crouch gait 5. Crouch gait, compensated * Often tolerated in younger, smaller, lighter and stronger patient 6. Crouch gait, uncompensated * SEMLS, orthotics, physical therapy * Management summary [European Journal of Neurology 2001;8 (Suppl. 5), 98-108]: * Spasticity management * Botox injections to hamstrings, hip * Contracture management * SEMLS – hamstring, psoas lengthening, osteotomies for torsional abnormalities or distal femur extension osteotomy 7. Stiff gait, knee source * Single level surgical management (rectus femoris to medial hamstring) 8. Stiff gait, hip source * Do not address the knee

Answer 109

1. Internal, single level * Single level surgical management (eg. tibial rotation osteotomy) 2. Internal multilevel * SEMLS 3. External, single level * Single level surgical management 4. External, multilevel * Rarely surgery (often due to obesity that cannot be corrected) 5. Neutral, off-setting (miserable malalignment) * SEMLS

Answer 110

1. Hip at risk 2. Subluxation 3. Dislocation 4. Dislocation with degeneration and pain

Answer 111

Difficulties with hygiene, sitting, gait and pain

Answer 112

Severity of neurological involvement (increasing GMFCS level)

Answer 113

1. Femoral anteversion 2. Coxa valga 3. Focal deformation of femoral head (erosion from acetabular margin) 4. Epiphysis becomes wedge shaped and displaces superolaterally

Answer 114

1. Increased acetabular index 2. Posterosuperior acetabular deficiency

Answer 115

1. Reimer’s Migration Index * Vertical drawn from the lateral acetabular margin * Width of the uncovered head (lateral to the vertical) divided by the total width of the femoral head * Normal = \<25% at age 4 2. Acetabular index * Angle formed between Hilgenreiner’s line and line along Sourcil * Normal = \<25° in child \<5, \<20° in adult 3. Sourcil shape * Type 1 = lateral corner is sharp and below the level of the weightbearing dome * Type 2 = lateral corner is blunted and above the level of the weightbearing dome

Answer 116

Between ages 2 and 8 have two orthopedic examinations per year including: * AP pelvis radiographs * Assess AI and MI * Hip abduction ROM

Answer 117

1. \<45° abduction 2. MI \>25%

Answer 118

50% * Femoral epiphysis begins to lose the support of the bony pelvis * Will not spontaneously reduce

Answer 119

1. Soft tissue lengthening * ‘Preventative’ * Can also consider nonop including Botox, bracing, therapy 2. Reconstruction * Soft tissue lengthening, shortening VDRO, acetabuloplasty, +/- capsulotomy 3. Salvage * Castle procedure * McHale procedure * Arthrodesis * Arthroplasty

Answer 120

1. Soft tissue lengthening * Indications: * \<8 years with hip abduction \<30° and MI 25-60% * Contraindications: * No contractures or spasticity * \>4 years with MI \>60% 2. Reconstruction * Indications: * \>4 years with MI \>60% and no degeneration * \<8 years with failed soft tissue lengthening (MI \>40% 1 year postoperative) * \>8 years with MI \>40% and no degeneration 3. Salvage * Indications: * Painful dislocated hips with degeneration \*\*Simplified 1 [Curr Rev Musculoskelet Med (2012) 5:126–134] * Preventative (Soft tissue lengthening) * MI \>40% * Increase in MI \>10% in the last year * Abduction \<30° * Reconstruction * MI \>50% * Evidence of hip subluxation /early dislocation * No evidence of degenerative changes in the femoral head * Salvage * Painful degenerative dislocated hips * Previously failed reconstructions \*\*\*Simplified 2 [Orthopaedics & Traumatology: Surgery & Research 105 (2019) S133–S141] * MI 10-30% * Botox and positioning * MI 30-40% * Soft tissue release * MI \>40% * Painless = reconstruction * Painful * Triradiate open = salvage * Triradiate closed = salvage or THA

Answer 121

1. Soft tissue lengthening * Transverse incision 1-3cm distal to inguinal crease * Adductor longus tenotomy * Gracilis myotomy * +/- adductor brevis lengthening * Iliopsoas tenotomy in nonambulators * Psoas tenotomy in ambulators 2. Reconstruction * Soft tissue lengthening (as above to achieve \>45° abduction) * Shortening VDRO * Shortening is the most important component (functionally lengthens the muscles) * Subtrochanteric osteotomy is performed * Femoral head is reduced into acetabulum (capsulotomy performed if reduction not achieved closed – typically not needed) * Amount of femoral shortening is then judged based on overlap between proximal and distal segments (usually 1-3cm) * Acetabuloplasty * Triradiate open * Dega is preferred as the deficiency is posterosuperior * Salter is contraindicated as it does not alter posterior coverage * Triradiate closed * PAO, triple, (shelf/chiari) 3. Salvage * Castle procedure * Femoral head is resected distal to LT * Rectus and vastus lateralis are sewn over the femur * Abductors, psoas and capsule are sewn over the acetabulum * McHale procedure (valgus support osteotomy) * Femoral head is resected and a subtrochanteric valgus osteotomy is used to direct the lesser trochanter into the acetabulum and the remaining femoral shaft away from the pelvis

Answer 122

1. Family history 2. Boys 3. Race (highest in Hawaiians and Maoris) 4. Early amniocentesis (\<13 weeks) 5. Oligohydramnios 6. Exposure to cigarette smoke inutero

Answer 123

1. Six signs are scored from 0 (no abnormality), 0.5 (moderate abnormality), 1 (severe abnormality) * 3 signs related to the hindfoot * Severity of the posterior crease * Emptiness of the heel * Rigidity of the equinus * 3 signs related to the midfoot * Curvature of the lateral border of the foot * Severity of the medial crease * Position of the lateral part of the head of the talus 2. Predicts need for tenotomy * 85% of feet with a score above 5 required tenotomy

Answer 124

Hindfoot parallelism * Talus and calcaneus are parallel/less divergent on AP and lateral

Answer 125

CAVE * Midfoot cavus * Forefoot adductus * Hindfoot varus * Hindfoot equinus

Answer 126

1. Navicular displaces medially (articulates with the medial head of talus) 2. Cuboid is adducted infront of the calcaneus 3. Metatarsals are adducted on the midfoot 4. Calcaneus is adducted and inverted around the talus medially 5. Forefoot is pronated relative to the hindfoot (causing cavus) 6. Tight muscles (gastroc/soleus, tib post, FHL, FDL) 7. Tight posteromedial capsule and ligaments

Answer 127

1. Serial foot manipulation followed by casting to maintain the correction with foot abduction orthosis as the final stage * Manipulations are held for 1-3 minutes followed by above knee plaster cast with knee at 90° flexion * Weekly cast change and manipulation * ~6 cast changes required to correct most clubfeet 2. Ponsetti method started ideally within the first month of life 3. The order of foot deformity correction is cavus, adductus, varus, equinus (CAVE) * Cavus correction * Usually achieved with the first cast * Technique – pressure under first metatarsal head to elevate it in line with other metatarsals * Forefoot adduction and hindfoot varus corrected simultaneously * With foot in slight supination and equinus the forefoot is abducted while stabilizing counterpressure is applied to the lateral head of the talus * This will simultaneous correct adduction and hindfoot varus as the calcaneus abducts freely under the talus (important to avoid max dorsiflexion) * Equinus correction * Perform when hindfoot is neutral or slight valgus and forefoot is abducted 70° relative to the leg * Technique – progressive dorsiflexion applied with broad pressure over sole of foot 4. Heel cord tenotomy * Required in ~75% of cases * Performed after 4-6 weeks of casting * Percutaneous tenotomy performed in clinic or OR followed by cast immobilization for 3-4 weeks 5. Foot abduction orthoses (‘boots and bars’, Denis-Browne bar) * Required to prevent relapse * 15° dorsiflexion needed for proper fit * Clubfoot placed in 70° abduction, unaffected foot in 40° abduction with feet shoulder width apart * Worn full time (23 hours/day) for 3 months followed by bed and naptime use until age 4 (range 2-5) * Patient should be followed every 3 months after bracing starts until 2 years of age

Answer 128

Failure to comply with foot abduction orthoses

Answer 129

Most frequently by age 5 * Rare after age 5 and extremely rare after 7

Answer 130

1. Loss of dorsiflexion is the earliest sign 2. Older infants – mild forefoot adductus, cavus, heel varus and limited abduction 3. Walking child – increased lateral contact during stance phase, heel varus, inward deviation of toes, and dynamic supination during swing phase

Answer 131

1. Mild dorsiflexion loss * If early, home exercise program and increased foot abduction orthosis use 2. If <10° dorsiflexion * Repeat manipulation and casting as per Ponsetti (2-3 casts usually required changed weekly) * Repeat tenotomy if 15° dorsiflexion not achieved * Resume foot abduction orthosis 3. If >2.5 years of age: * Consider anterior tibial tendon transfer to 3rd cuneiform (now sufficiently ossified) * First requires obtaining original correction with manipulation and casting (2-3 casts) and heel cord tenotomy if <10° dorsiflexion * Anterior tendon should never be split (split weakens eversion power) * Technique for anterior tibial tendon transfer: * 3-4cm incision starting just distal to the navicular and extending proximal inline with tibialis anterior tendon is made * The tibialis anterior tendon is released from the base of the 1st MT and a whip stitch is placed in the tendon * A 2nd incision is made over the lateral cuneiform and localized with fluoro guidance, once confirmed a drill hole is made dorsal to plantar * The tendon is tunneled subcutaneously from the medial to lateral incision * Keith needles are threaded on to the sutures and passed through the drill hole and out through the plantar aspect of the foot * The ankle is dorsiflexed and everted and the sutures are tied over a button * The patient is casted for 6 weeks 4. If age 4-9 with well-formed medial cuneiform ossific nucleus: * Consider closing wedge osteotomy through the cuboid and medial opening wedge osteotomy through the cuneiforms (flip-flop technique) 5. Patients whose parents are unwilling to allow repeated cast and brace treatment and patients with feet that are otherwise refractory to the Ponseti method: * Consider posteromedial release (required in less than 5%) * Highly associated with development of pain, stiffness and weakness in late adolescence and early adulthood * Technique for posteromedial release [Lovell and Winter]: * Cincinnati incision * Extends medially from navicular, posteriorly just below medial malleolus and 1cm proximal to the posterior heel crease, laterally just below lateral malleolus ending at the sinus tarsi * Releases include: * Heel cord lengthening * Posterior release of ankle and subtalar joint * Plantar fascia * Abductor hallucis * +/- Tib post tendon lengthening * +/- Talonavicular joint capsule release * +/- FHL and FDL release

Answer 132

1. Ipsilateral DDH (70-100% of cases) 2. Clubfoot 3. Arthrogryposis 4. Myelodysplasia 5. Larsen syndrome

Answer 133

1. Grade 1 = recurvatum 2. Grade 2 = subluxation 3. Grade 3 = complete dislocation

Answer 134

1. Knee hyperextension 2. Inability to flex knee in complete dislocation

Answer 135

1. Nonoperative * Closed reduction and serial casting * Closed reduction achieved by traction followed by knee flexion * Serial casting in progressive knee flexion 2. Operative * Failure of nonoperative * <30° of flexion after 3 months of casting * Performed at ~6 months of age * Involves open reduction and quadriceps lengthening * Percutaneous quadriceps release * Open V-Y quadriceps advancement * Possible femoral shortening osteotomy (relative lengthening of extensor mechanism) [POSNA]

Answer 136

1. Treat congenital knee dislocation first 2. Once adequate knee flexion achieved patient can be placed in Pavlik harness * Pavlik harness helps to hold knee in flexion and maintain hip reduced

Answer 137

Congenital, irreducible lateral patellar dislocation present at birth

Answer 138

1. Anatomical * Tight lateral structures (capsule, ITB, etc) * Quadriceps contracture * Lateralized patellar tendon insertion on tibia * Hypoplastic patella * Shallow trochlear groove 2. Clinical * Knee flexion contracture * Genu valgum * External tibial torsion * Prominent femoral condyles

Answer 139

Operative * Principles: * Extensive lateral release * ITB, capsule, biceps femoris * VY lengthening of quadriceps * Medial capsule imbrication OR MPFL reconstruction * Lateral patellar tendon insertion addressed via: * Roux-Goldthwait procedure: * Lateral half of patellar tendon detached from tibial tubercle, passed deep to remaining patellar tendon and attached medial to the medial half of the patellar tendon * Patellar tendon periosteal sleeve medialization (complete medialization of patellar tendon)

Answer 140

Decreased femoral neck shaft angle believed to be a result of a primary defect in endochondral ossification of the medial part of the femoral neck

Answer 141

Painless limp (unilateral) or waddling gait (bilateral) * Due to abductor weakness and minor LLD in unilateral cases

Answer 142

1. Decreased femoral neck-shaft angle (\<120^o) 2. Vertical position of physeal plate 3. Triangular metaphyseal fragment in inferior femoral neck with associated inverted Y appearance 4. Shortened femoral neck 5. Decrease in normal anteversion

Answer 143

Hilgenreiner epiphyseal angle (H-E) * Angle between the physeal plate and Hilgenreiner line * Normal = \<25° (average 16°) * Coxa vara = 40-70°

Answer 144

1. Nonoperative * H-E angle \<45 * H-E angle 45-59 and asymptomatic 2.Operative * H-E angle \>60 * H-E angle 45-59 and symptomatic * Symptomatic limp, Trendelenburg gait, or progressive deformity * Neck shaft angle \<100 * Technique: * Valgus-producing proximal femoral osteotomy

Answer 145

1. <38° H-E angle [Orthobullets] 2. 16° H-E angle [Lovell and Winter]

Answer 146

1. PFFD is associated with fibular deficiency in 70% to 80% of cases 2. Associated conditions are similar to that of fibular hemimelia

Answer 147

1. Aitken * Class A * Femoral head ossification delayed * Acetabulum well formed * Femur is short * Proximal femur is at or above level of acetabulum * Class B * Femoral head ossification delayed * Mild acetabular dysplasia * Proximal femur is above level of acetabulum * Subtrochanter region will not ossify and forms pseudoarthrosis * Class C * Femoral head does not form * Severe acetabular dysplasia * Femur is shorter than B * Entire proximal femur does not form * Class D * Femoral head does not form * Acetabulum does not form * Distal femoral condyles are at level of acetabulum

Answer 148

1. Nonoperative * ‘bridge treatment’ from time patient begins to walk to surgical treatment (~3 years of age) * Bilateral PFFD 2. Operative * 3 main procedures (stable hip) * Knee fusion with foot ablation * Indications: * \>20cm of LLD at maturity * Foot above level of contralateral knee * Ankle has \<60° arc of motion * Stable hip * Foot ablation (amputation) through Boyd or Syme amputation * Van Nes rotationplasty * Indications: * \>20cm of LLD at maturity * Foot at level of contralateral knee * Ankle has \>60° arc of motion * Stable hip * Technique: * Leg is rotated 180° through the knee arthrodesis * Limb lengthening * Indications: * \<20cm of LLD at maturity * Stable hip * Good knee, ankle, foot function * Unstable hip (Aitken C/D) * Possible iliofemoral fusion (knee then functions as hip and ankle as knee)

Answer 149

1. True (structural) LLD = anatomic difference in length of one of the segments of the lower extremity (femur, tibia, foot) 2. Apparent (postural) LLD = discrepancies that are not true differences in anatomic segment lengths (eg. knee flexion contracture) 3. Functional LLD = the sum of the true and apparent leg-length discrepancy (most important in treatment decisions)

Answer 150

1. Congenital * PFFD, fibular hemimelia, tibia hemimelia, posteromedial tibial bowing, clubfoot, hemihypertrophy 2. Acquired * Trauma (growth arrest, overgrowth, malunion), radiation, tumor, infection

Answer 151

1. True leg length * Measure from ASIS to medial malleolus 2. Apparent leg length * Measure from umbilicus to medial malleolus (affected by pelvic obliquity) 3. Galeazzi sign * Difference in knee heights suggests difference in femoral lengths 4. Heel pad height * With patient prone assess difference in heights of the heel pads, suggests difference in tibia/fibula length 5. Block method * Place blocks under foot of short leg until pelvis level, height of block indicates LLD

Answer 152

1. Teleoroentgenogram (C) * Standing alignment film (35cm × 90cm) taken with a single exposure at a distance of 2 m centered on the knee joint * Advantages – angular deformity assessment * Disadvantage – magnification error 2. Orthoroentgenogram (A) * Three separate exposures at the hip, knee, and ankle all placed on the same longstanding film with ruler centered over each joint * Advantages – no magnification error * Disadvantages – cannot assess angular deformity 3. Scanogram (B) * All three joints are placed on one smaller film; obtained having the film and x-ray source move * Advantages – no magnification error * Disadvantages – cannot assess angular deformity 4. CT scanogram * CT scan through hip, knee, and ankle to assess length * Advantages – accurate length measurements in setting of joint contractures * Disadvantages – cannot assess angular deformity

Answer 153

Greulich and Pyle * Bone age x-ray (left hand) * The clinician or radiologist can compare this child’s x-ray with those in the atlas and develop a bone age with a given standard deviation

Answer 154

1. Moseley Straight line graph [www.pedipod.com] 2. Multiplier method 3. Estimation method

Answer 155

1. Proximal femur = 3mm/year 2. Distal femur = 9mm/year 3. Proximal tibia = 6mm/year 4. Distal tibia = 5mm/year

Answer 156

1. \<2cm * Observation * Shoe lift 2. 2-5cm * Shoe lift * Contralateral epiphysiodesis * Contralateral skeletal shortening (usually for patients with inadequate growth remaining for epiphysiodesis) 3. \>5-20cm * Limb lengthening +/- contralateral epiphysiodesis 4. \>20cm * Amputation and prosthetic fitting

Answer 157

1. Initiation * Ensure stable joint above and below prior to lengthening * If joint subluxes during lengthening extend the frame across the joint * Perform corticotomy and place fixator * Metaphyseal corticotomy is preferred due to good blood supply * Percutaneous corticotomy that minimizes trauma to the periosteum and preserves the blood supply of the marrow and periosteum 2. Distraction * Wait 5-7 days then begin distraction (allows for neovascularization of corticotomy site) * Distract ~ 1 mm/day in four 0.25mm increments daily * Do not distract more than 20% of the bones original length * Following distraction keep fixator on for as many days as you lengthened 3. Concurrent procedures * May lengthen over a nail so ex-fix can be removed sooner * Lengthening often combined with a shortening procedure (epiphysiodesis, ostectomy) on long side 4. Note: * Type of bone formation during distraction osteogenesis = intramembranous ossification

Answer 158

1. Bilateral genu valgum * Physiologic * Renal osteodystrophy (renal rickets) * Skeletal dysplasia (Morquio syndrome, Spondyloepiphyseal dysplasia, Chondroctodermal dysplasia) 2. Unilateral genu valgum * Physeal injury from trauma, infection, or vascular insult * Proximal metaphyseal tibia fracture (Cozen fracture) * Benign tumors * Fibrous dysplasia * Osteochondromas * Ollier’s disease

Answer 159

3-4 years of age (8-10 degrees) * Corrects to stable adult valgus by age 6-7 (5-7 degrees)

Answer 160

1. Nonoperative * Observation (bracing not indicated) 2. Operative * Indications: * Mechanical axis passes through zone 3 (beyond the lateral tibial plateau) * .Mechanical axis passes through zone 2 (outer half of the lateral plateau) in presence of pain * Timing : * Usually deferred to 10-11 years of age * Options * Hemiepiphysiodesis * Requires 1-2 years of growth remaining * Eight-plate (guided growth plate) or staple placed extraperiosteally, parallel to physis, central on lateral view * Placed medially in femur +/- tibia depending on location of deformity * Normally the mLDFA and MPTA = 87° * Monitor every 3 months with radiographs, remove once mechanical axis passes through central 1/3 knee joint * Distal femur varus osteotomy * Performed when inadequate growth remaining, severe deformity or immediate correction desired

Answer 161

~50% (may not be symmetric)

Answer 162

* Obesity * Hispanic and black children * ?early walkers * ?Vit D deficiency * ?zinc deficiency

Answer 163

Spontaneous deceleration of growth occurs at the posteromedial proximal tibial physis resulting in: * Varus/flexion/internal rotational deformity * Medial and posterior “sloping” of the proximal tibial epiphysis * In unilateral cases, variable relative tibial shortening

Answer 164

Disruption of the normal columnar architecture of the physis, replacement of physeal cartilage by fibrous tissue and, in the most severe form, osseous bridging (physeal arrest) between the epiphysis and metaphysis

Answer 165

1. Deformity: * Proximal tibia varus * Increased internal tibial torsion * In unilateral cases, leg length inequality 2. Palpable prominence or “beaking” of the proximal medial tibial epiphysis and metaphysis 3. No tenderness, knee effusion, or restriction of joint motion 4. Dynamic test: * Lateral thrust may be noted in the child’s gait * Single limb stance, the varus deformity acutely accentuates as if the knee were unstable

Answer 166

1. Sharp varus angulation of the tibia metaphysis 2. Widening and irregularity of the medial aspect of the growth plate 3. Medial sloping and irregular ossification of the epiphysis 4. Beaking of the medial part of the epiphysis 5. The distal femur is usually normal (if abnormal it is a valgus deformity

Answer 167

Langenskiold’s Classification * Stage I: medio-distal beaking of the upper proximal tibial metaphysis. * Stage II: wedging of the medial part of the upper tibial epiphyseal secondary ossification center plus a saucer shaped defect of the upper surface of the metaphyseal beak due to its dissolution, fragmentation & collapse. * Stage III: stepping of the infero-medial border of the secondary ossification center but without extending distal to the physeal plate level plus deeping of the metaphyseal saucer into a step in the medial metaphysis. * Stage IV: the epiphyseal secondary ossification center passes more distally and cross distal to the physeal level to fill the metaphyseal step. * Stage V: separation of the most medial part of the ossification center from the bulk of the secondary ossification center and resides now in the depth of the metaphysiseal step below the physis. * This is radiologically expressed as either a horizontal cleft (double epiphysis) or complete absence of the medial secondary ossification center as it will be overshadowed by the upper medial tibial metaphysis. * Stage VI: medial epiphyseal plate closure with a bony bridge

Answer 168

Metaphyseal-Diaphyseal Angle (Drennan) * ≤9° suggest physiologic varus * 95% chance of natural resolution of bowing * ≥16° likely indicate infantile Blount disease * 95% chance of progression * \>°9 and \<16° are considered indeterminant and merit careful observation

Answer 169

1. Persistent physiologic varus 2. Vitamin D-deficiency rickets 3. Renal osteodystrophy 4. Vitamin D-resistant (hypophosphatemic) rickets 5. Metaphyseal dysostosis 6. SED or MED 7. Thrombocytopenia-absent radius syndrome 8. Focal fibrocartilaginous defect 9. Proximal tibial physeal injury (eg. infection, fracture, irradiation)

Answer 170

Nonsurgical * Anti-varus long leg bracing during ambulation * Indications: * Patients aged ≤3 years with unilateral involvement at stage 2, clear radiographic evidence of infantile Blount disease, or lateral thrust with ambulation Surgical * Indications: * Patient is age ≥4 years * Langenskiöld stage III or greater * Demonstrates progressive radiographic deformity * Options: * High tibial and fibula osteotomy\* * Treatment of choice for progressive varus or brace failure * Goal = Full to overcorrection of varus, flexion, and internal rotational deformities of the tibia * Tibial osteotomy is performed below the tibial tubercle * Closing wedge, opening wedge, dome, serrated, and inclined acceptable * Stabilization with 1-2 pins plus long leg cast * Lateral translation of the distal fragment to lateralize the mechanical axis of the limb is advisable * Growth modulation * Alternative to HTO * Consider in young patients with less than Langenskiold stage IV * Extraperiosteal tension band plate or staple across the lateral tibial physis * Slight overcorrection such that the mechanical axis is lateral to the centre of the knee * Note – does not correct the internal tibial torsion * Physeal arrest resection * Hemiplateau elevation * Angular deformity correction and lengthening

Answer 171

Rare, usually unilateral

Answer 172

1. Progressive varus deformity 2. With or without knee pain 3. Leg length discrepancy with unilateral or asymmetric bilateral 4. Variable internal tibial torsion and proximal tibial flexion (procurvatum) deformities 5. Limp or lateral thrust

Answer 173

1. Tibia findings * Proximal varus deformity * Widening or lucency of the medial tibial physis * Proximal procurvatum * Distal valgus 2. Femur findings * Distal femoral physeal growth disturbance * Varus deformity

Answer 174

1. Nonsurgical management not indicated 2. Surgical management * Proximal tibial osteotomy with internal or external fixation * Correction of the deformity, rather than overcorrection, is the goal of surgery because the proximal tibial physis typically grows symmetrically postoperatively * Distal femoral varus deformity and distal tibia valgus deformity may need to be addressed

Answer 175

Jones Classification * Type 1a * No proximal tibia visible on radiograph * Extensor mechanism absent * Hypoplastic distal femoral epiphysis * Type 1b * Proximal tibia eventually ossifies and extensor mechanism will often function * Distal femoral epiphysis appears normal * Type 2 * Proximal tibia present at birth but short tibia * Distal tibia fails to ossify * Type 3 * Diaphyseal and distal tibia present but proximal tibia absent * Type 4 * Short tibia * Fibula migrated proximal * Diastasis of distal tib-fib joint

Answer 176

1. Shortened tibia 2. Rigid equinovarus-supinated foot pointing toward the perineum 3. Prominent fibular head

Answer 177

Nonoperative * Indications: * Bilateral tibial deficiency with active knee extension and acceptable foot position Operative * Type 1A and other types with no extensor mechanism * Knee disarticulation with prosthetic fitting * Type 1B and 2 with intact extensor mechanism * Tibiofibular synostosis with modified Syme amputation * Type 3 * Rare (limited data) * Ankle disarticulation and prosthetic fitting * Type 4 * Projected LLD \<5cm = soft tissue correction of foot deformity + later contralateral epiphysiodesis * Projected LLD \>5cm = Syme amputation and prosthetic fitting\*

Answer 178

Fibular hemimelia

Answer 179

1. Foot and ankle * Absent lateral rays * Tarsal coalition * Ball and socket ankle * Ankle instability * Equinovalgus (equinovarus less frequently) 2. Lower extremity * Fibular hemimelia/amelia * Anteromedial tibial bowing * Hypoplastic lateral femoral condyle * Genu valgum * ACL deficiency * PCL deficiency * Patella alta * Hypoplastic patella * PFFD * Varus and valgus femoral neck * Femoral retroversion * Acetabular dysplasia 3. Upper extremity * Ulnar hemimelia/amelia * Syndactyly 4. Other * Renal anomalies * Cardiac anomalies

Answer 180

1. Achterman and Kalamchi * Type IA * Fibula is present * Proximal fibular epiphysis is distal to the level of the tibial growth plate * The distal fibular growth plate is proximal to the dome of the talus * Type IB * Partial absence of the fibula * The fibula is absent for 30% to 50% of its length proximally * Distally, the fibula is present but does not support the ankle. * Type II * Complete absence of the fibula 2. Birch classification * Type 1 = foot preservable (3 or more rays present) * Subdivided based on overall percentage limb-length inequality compared with the contralateral side * Type 1A = \<6% (correlates to a projected expected inequality at maturity of ≤5 cm) * Type 1B = 6-10% * Type 1C = 11 to \<30% * Type 1D = ≥30% * Type 2 = foot is not preservable * Subdivided based on presence or absence of upper extremity deficiency requiring the use of the foot to substitute for upper extremity prehension * Type 2A = functional upper extremity * Type 2B = nonfunctional upper extremity

Answer 181

Type 1A * No treatment OR orthosis OR epiphysiodesis Type 1B * Epiphysiodesis ± lengthening Type 1C * 1 or 2 lengthenings ± epiphysiodesis or extension orthosis Type 1D * \>2 lengthenings OR amputation OR extension orthosis Type 2A * Amputation Type 2B * Consider salvage

Answer 182

1. Primary problems are LLI, foot deformity, and ankle instability * Goal of achieving normal WB, normal gait and equal limb length 2. Orthoses and/or epiphysiodesis candidates: * Mild LLI (\<6%) and functional plantigrade foot 3. Limb lengthening +/- epiphysiodesis candidates: * Less severe foot deformities (eg. 3 or more present rays) * Predicted LLI \<30% 4. Amputation candidates: * Severe foot deformity (eg. 3 or more absent rays) * Predicted LLI ≥30% at the age of skeletal maturity * \>5cm discrepancy at birth

Answer 183

Syme or boyd * Performed at the time the child attempts to walk

Answer 184

1. Neurofibromatosis Type I * Of patients with anterolateral tibial bowing – 50% have NF * Of patient with NF – 5-10% have anterolateral tibial bowing 2. Fibrous dysplasia (15% of cases)

Answer 185

The diagnostic criteria for NF-1 are met when two or more of the following are found: * ≥6 café-au-lait macules \>5 mm in greatest diameter in prepubertal persons and \>15 mm in greatest diameter in postpubertal persons * ≥2 neurofibromas of any type or one plexiform neurofibroma * Freckling in the axillary or inguinal region * Optic glioma * ≥2 Lisch nodules (dome-shaped gelatinous masses developing on the surface of the iris) * A distinctive osseous lesion, such as sphenoid dysplasia or thinning of long bone cortex, with or without pseudarthrosis * A first-degree relative (parent, sibling, or offspring) with NF-1 as diagnosed using the listed criteria **CAFÉ SPOT (mnemonic for diagnostic criteria)** * Café au lait spots (coast of california, smooth) * Axillary and inguinal freckling * neuroFibromas * Eye (lisch nodules) * Skeletal abnormality (bowing/thinning of long bone, pseudoarthrosis of tibia) * Positive Family History * Optic Tumor (optic glioma)

Answer 186

1. Anterolateral bowing of the tibia may be apparent at birth or may progress with weight bearing 2. Spontaneous resolution is uncommon 3. Fracture with resultant pseudarthrosis typically occurs in the first 4 to 5 years of life 4. Fracture risk decreases at skeletal maturity 5. Once established, the natural history of a pseudarthrosis is that of persistent instability and progressive deformity

Answer 187

Nonoperative * Indicated for anterolateral bowing in absence of fracture * Involves bracing when weightbearing until skeletal maturity * Goal is to prevent progressive deformity and fracture/pseudoarthrosis Operative * Osteotomies to correct bowing is contraindicated * Surgery is indicated once pseudoarthrosis develops * No surgical technique has proven superior * Principles include: * Resection of the pseudoarthrosis * Stable fixation * Correction of angular deformity * Surgical options include: * IM rod and bone grafting * Circular fixator with bone transport * Vascularized fibular graft * Adjunctive use of bone morphogenetic protein * Amputation * Consider after persistent pseudoarthrosis after 2-3 failed surgeries * Syme amputation preferred

Answer 188

Valgus deformity (57.8%) * Others: * Malalignment (anterior bowing) * LLD * Refracture

Answer 189

Calcaneovalgus foot

Answer 190

1. Progressive correction of the deformity 2. Posterior bow typically remodels completely 3. Medial bow less likely to remodel completely – residual valgus remains 4. Not associated with pathologic fracture or pseudarthrosis of the tibia 5. Considerable leg length discrepancy typically develops

Answer 191

Nonoperative * Observation (correction usually occurs over 5-7 years) * Passive stretching of foot/ankle * AFO * Shoe lift Operative * Contralateral Epiphysiodesis (proximal tibia) +/- lengthening to address LLI

Answer 192

1. Rotational * Femoral anteversion * Internal tibial rotation * Metatarsus adductus * Miserable malalignment syndrome – femoral anteversion, external tibial torsion, pes planovalgus 2. Foot * Clubfoot * Skewfoot (metatarsus adductus and hindfoot valgus) * Metatarsus primus varus * Hallux varus 3. Others * Cerebral palsy * Spastic hemiplegia with overactive posterior tibial tendon (only evident in swing phase) * DDH

Answer 193

1. Gait 2. Observation 3. Condition specific testing * Femoral anteversion * Prone hip ROM (abnormal is \>70° IR, \<20° ER) * Internal tibial torsion * Thigh-foot angle (abnormal \>10° IR) * Bimalleolar angle * Metatarsus adductus * Heel bisector line lateral to 2nd webspace

Answer 194

1. Femoral anteversion * Usually nonop * Surgery is a proximal femoral derotation osteotomy (subtroch fixed with locking plate) 2. Internal tibial torsion * Usually nonop * Surgery is a proximal or supramalleolar derotation osteotomy 3. Metatarsus adductus * Usually nonop (spontaneous resolution, manipulation and serial casting) * Surgery can include medial opening wedge osteotomy of the medial cuneiform +/- lateral closing wedge osteotomy or osteotomies of the bases of metatarsals two through four

Answer 195

1. Neurological (2/3) * Central – CP, Friedrich’s ataxia, CVA * Cord – SMA, myelomeningocele (L4), tethered cord, diastematomyelia * Peripheral – CMT\*, polio 2. Clubfoot/recurrent clubfoot 3. Traumatic

Answer 196

1. Forefoot * Pronation, adduction, first ray plantarflexion 2. Midfoot * Cavus 3. Hindfoot * Varus, calcaneus hyperdorsiflexion

Answer 197

1. WEAK * Tibialis anterior * Peroneus brevis * Intrinsics 2. STRONG * Peroneus longus * Tibialis posterior * Long toe extensors and flexors 3. Result of muscle imbalances: * Claw toes * Weak intrinsics vs. strong long toe extensors and flexors * First ray plantarflexion and forefoot pronation * Weak tibialis anterior vs. strong peroneus longus * Hindfoot varus * Weak peroneus brevis vs. strong tibialias posterior

Answer 198

Tripod effect * Pronated forefoot causes the medial forefoot to strike the ground first, as the lateral forefoot is brought to the ground the subtalar joint is forced into supination which causes the hindfoot varus

Answer 199

1. Coleman block test 2. Silfverskiold test 3. Neurological exam 4. Muscle strength testing (tendon transfer consideration)

Answer 200

1. Calcaneal pitch \>30 2. Meary angle \>4 (apex dorsal usually centered at the medial cuneiform) 3. Fibula overlies the posterior 1/3 of the tibia on lateral view 4. Vertically oriented midfoot with a “stacked” conformation of the talonavicular joint and calcaneocuboid joint (see picture with sinus tarsi see-through sign)

Answer 201

* Evidence of a progressive deformity * Painful callosities under the metatarsal heads or base of the fifth metatarsal * And hindfoot/ankle instability despite nonoperative treatment

Answer 202

1. Correct the deformity AND balance the deforming muscle forces 2. SOFT TISSUE RELEASES * Medial plantar release which can include: * Proximal release of the abductor hallucis * Tibialis posterior tendon lengthening * Talonavicular joint capsulotomy * Plantar fasciotomy * Possible TAL or gastroc recession 3. OSTEOTOMIES * 1st metatarsal dorsiflexion osteotomy * Alternative – plantar medial cuneiform opening wedge osteotomy (may be more desirable as it is at the apex of deformity and will not affect the 1st MT growth plate) * Lateral calcaneus closing wedge osteotomy (Dwyer) or lateral calcaneal slide 4. TENDON TRANSFERS * Peroneus longus to peroneus brevis transfer * Tibialis posterior to tibialis anterior - 4 incision technique * Jones transfer of EHL to neck of 1st MT (relieves 1st toe clawing)

Answer 203

Triple fusion

Answer 204

1. Obesity 2. Delayed motor development 3. Connective tissue disorders

Answer 205

Pes Planovalgus * Forefoot – supination, abduction * Midfoot – flattening of medial longitudinal arch, prominent talar head * Hindfoot – valgus

Answer 206

1. Observation * Flattened medial longitudinal arch * Hindfoot valgus * Too many toes sign 2. Toe stance * Flexible = arch reconstitutes * Rigid = arch does not reconstitute 3. Hallux extension * Arch reconstitutes due to windlass effect 4. Decreased dorsiflexion * Correct the hindfoot valgus prior to testing dorsiflexion * Reduced dorsiflexion indicates tight heel cord

Answer 207

1. Lateral * Meary angle – apex plantar * Calcaneal pitch (normal = 10-30°; \<10° = pes planus) * Talocalcaneal angle (normal = 25-55°; \>55° = hindfoot valgus) 2. AP * Talonavicular uncoverage * Talocalcaneal (Kite) angle (normal = 15-30°; \>30° = hindfoot valgus) 3. Oblique * Assess for calcaneonavicular coalition

Answer 208

1. Asymptomatic * No treatment 2. Symptomatic * Nonoperative * Orthotics * Achilles stretching * Operative * Indications: * Failure of nonoperative * Modified Evans\* * Sinus tarsi type approach * Inferior extensor retinaculum and EDB are elevated off calcaneus * Pin the CC joint to prevent subluxation * Osteotomy is made 2cm from CC joint between anterior and middle facets * Trapezoidal bone allograft is placed in the opening wedge and pinned * +/- gastroc recession or TAL * +/- 1st MT plantarflexion osteotomy if forefoot supinated * Calcaneo-cuboid-cuneiform osteotomy (‘Triple C’) * Medial calcaneal slide osteotomy * Cuboid lateral opening wedge osteotomy * Medial cuneiform plantar closing wedge osteotomy (corrects supination) * +/- gastroc recession or TAL * +/- medial reefing of TN joint capsule

Answer 209

1. Tarsal coalition 2. Congenital vertical talus 3. Peroneal spastic flatfoot without coalition 4. Iatrogenic or posttraumatic deformity

Answer 210

1. Prevalence ~ 1 in 10,000 live births 2. 50% of cases are isolated (idiopathic) 3. 20% have a positive family history 4. 50% of cases are non-isolated (occur in patients with neurologic disorders, genetic defects or syndromes) * Neurologic disorders = arthrogryposis, myelomeningocele, diastematomyelia * Genetic defects = aneuploidy of chromosomes 13, 15, and 18 * Syndromes = De Barsy, Costello, and Rasmussen syndromes and split hand and split foot limb malformation disorders

Answer 211

1. Hindfoot equinus and valgus * Caused by contracture of the Achilles tendon and the posterolateral ankle and subtalar joint capsules 2. Midfoot and forefoot dorsiflexion and abduction * Secondary to contractures of the tibialis anterior, extensor digitorum longus, extensor hallucis brevis, peroneus tertius, and extensor hallucis longus tendons and the dorsal aspect of the talonavicular capsule 3. Navicular dislocated dorsolaterally on the head of the talus 4. Vertical talus 5. Cuboid dislocated dorsolaterally on the calcaneus

Answer 212

1. Convex plantar surface 2. Deep creases on the dorsum of the foot 3. Rigid deformity 4. Distinct palpable gap dorsally where the navicular and talar head would articulate in a normal foot * If the gap reduces with plantar flexion of the forefoot, then the deformity has a degree of flexibility (good prognosis) 5. Motor function of plantarflexion and dorsiflexion of the toes * Elicit by stimulating the dorsum and plantar aspects of the foot * Record as absent, slight, definitive (prognostic value) 6. Assess for associated conditions * Facial dysmorphic features, sacral dimple, etc.

Answer 213

1. AP foot 2. 3 laterals (max dorsiflexion, max plantarflexion, neutral)

Answer 214

1. AP talocalcaneal angle 2. AP TAMBA (Talar axis-first metatarsal base angle) 3. Lateral TAMBA 4. Lateral talocalcaneal 5. Lateral tibiocalcaneal

Answer 215

1. Neutral lateral view * Talus vertically oriented * Calcaneus in equinus (high tibiocalcaneal angle) * TAMBA \>35° diagnostic for CVT 2. Plantarflexed lateral view * Persistent vertical talus 3. Dorsiflexed lateral view * Persistent hindfoot equinus 4. AP view * Talocalcaneal angle increased (no angle pathognomonic)

Answer 216

Talonavicular subluxation that reduces with forced plantarflexion of the foot

Answer 217

1. Traditional * One or two-stage extensive soft tissue release * Involves release of contracted tendons plus capsulotomy * Complications - wound necrosis, osteonecrosis, undercorrection/overcorrection of deformities, long-term stiffness and degenerative arthritis 2. Minimally invasive * ‘Reverse Ponseti technique’ * Characterized by: * Serial manipulations and casting * Temporary stabilization of the talonavicular joint with K-wire * Achilles tenotomy * Recommended for all CVT regardless of age or associated conditions

Answer 218

1. Manipulation * The thumb of one hand is placed on the head of the talus at the plantar-medial aspect of the midfoot for counterpressure * The other hand plantarflexes and adducts the forefoot * The calcaneus is not touched to allow it to move from valgus to varus * Manipulation is held for 1-2 minutes 2. Casting * Long leg cast applied to hold the foot in position achieved by manipulation * Short leg cast applied first with careful molding * Extended above the knee with knee in 90° flexion * Average of 5 casts required changed weekly * The final cast positions the foot in maximal plantarflexion and inversion to ensure adequate stretching of tendons, capsule and skin 3. K-wire fixation of the talonavicular joint * A single K-wire is placed retrograde from the navicular into the talus with the foot held in maximum plantar flexion * The wire is cut and buried underneath the skin for later removal in the operating room * If the talonavicular joint cannot be reduced by closed means, then a small, 2-cm medial incision is made over the talonavicular joint * An elevator is used to gently lift the talus to a horizontal and reduced position * For patients who require this open procedure, transfer the tibialis anterior tendon from its insertion on the navicular to the dorsal aspect of the talar neck with use of suture fixation of the tendon directly into the talar neck * Dynamic correction of the talonavicular joint 4. Percutaneous Tenotomy of the Achilles Tendon * Once the talonavicular joint is reduced and stabilized with the K-wire, a percutaneous tenotomy of the Achilles tendon is used to correct the equinus deformity * A long leg cast is applied with the ankle and forefoot in a neutral position * The cast is changed 2 weeks postoperatively to manipulate the ankle to 10° of dorsiflexion * The K-wire is removed in the operating room 6 weeks after the index procedure. 5. Boots and bars * The patient then uses a shoe and bar brace system * Worn full time for 2 months and then only at night for 2 years, to prevent relapse * The shoes on the brace are set pointing straight ahead to stretch the peroneal tendons * Parents are also taught foot stretching exercises that emphasize ankle plantar flexion and foot adduction

Answer 219

Geist classification * Type 1 = “sesamoid” * Sesamoid in the tibialis posterior tendon * Type 2 = “synchondrosis” * Accessory bone attached to native navicular by synchondrosis * Type 3 = “synostosis” * Complete bony enlargement

Answer 220

1. Nonoperative – first line 2. Operative – failed nonop * Excision of accessory navicular * Medial approach from talar head to medial cuneiform * Elevate the tibialis posterior tendon * Excise the accessory navicular * Repair tibialis posterior to navicular (suture/suture anchor) * \*\*Do not advance tibialis posterior (increases recovery time with no benefit)

Answer 221

1. Hyperdorsiflexion of the ankle 2. Eversion of the subtalar joint 3. Dorsal surface may rest on anterior tibia 4. Dorsal soft tissue contracture limiting plantarflexion and inversion

Answer 222

Excellent * Severity of deformity does not predict a worse outcome

Answer 223

1. Mild (plantar flexion beyond neutral) – observation 2. Moderate (plantarflexion to neutral or less) – observation plus parental stretching 3. Severe – consider serial casting (rarely needed)

Answer 224

1. Short, thick first metatarsal 2. Fibrous medial band 3. Longitudinal epiphyseal bracket * The condition is characterized by a shortened and angulated first metatarsal. The medial diaphysis and metaphyses of the bone are bracketed by a continuous epiphysis * Suggested by the D-shape of the metatarsal with no cortical differentiation along the convex medial border of the diaphysis 4. Accessory metatarsals/phalanges, duplication of the hallux

Answer 225

Operative * Surgery is mandatory * Technique depends on the associated findings: * Medial soft tissue release – fibrous medial band * With or without syndactylization (creates syndactyly between the 2nd toe and hallux) * Farmer technique (see photo) * Resection (of central portion) and interposition grafting of a longitudinal epiphyseal bracket

Answer 226

Sprengel deformity

Answer 227

1. Elevated scapula 2. Hypoplasia of the scapula 3. Hypoplasia of the periscapular muscles 4. Malrotated scapula * Downfacing glenoid, inferior angle rotated medially 5. +/- omovertebral bone * Present in ~50% * Characterized by fibrous, cartilaginous or mature bone connecting the superomedial aspect of the scapula to the cervical spine (usually C4-C7 spinous process, TVP or lamina)

Answer 228

1. Scoliosis 35–55% 2. Klippel-Feil syndrome 16–27% 3. Rib anomalies 16–48% 4. Omovertebral bone 20–50% 5. Spina bifida 20–28% 6. Torticollis 4% 7. Clavicular abnormalities 1–16% 8. Humeral shortening 6–13% 9. Femoral shortening 1% 10. Talipes equinovarus 1–3% 11. DDH 1–4% 12. Pes planus 1–3% 13. Other 1–3%

Answer 229

1. Cosmetic * Suprascapular region fullness * Neck fullness 2. Functional impairment * Due to decreased ROM * Shoulder abduction mostly affected (usually limited to \<90°)

Answer 230

Cavendish Classification of Sprengel Deformity * Grade 1 (very mild) * Shoulders are level * Deformity is not visible when the patient is dressed * Grade 2 (mild) * Shoulders are almost level * Deformity is visible as a lump in the web of the neck when the patient is dressed * Grade 3 (moderate) * Shoulder is elevated 2–5 cm * Deformity is easily seen * Grade 4 (severe) * Shoulder is elevated * The superior angle of the scapula lies near the occiput

Answer 231

Nonoperative * Cavendish grade 1 and 2 Operative * Cavendish grade 3 and 4 * Timing - ~ age 4-6 * Before age 8 (Increased risk of nerve impairment)

Answer 232

1. Resection of the elevated portion of the scapula 2. Removal of the omovertebral bone 3. Inferior mobilization of the scapula 4. Clavicular resection osteotomy (to avoid brachial plexus injury)

Answer 233

1. Green (classical) * Characterized by detachment of scapular muscles from medial border * Technical points: * Extraperiosteal release of all muscles at their scapular insertions * Resection of the supraspinatus fossa and omovertebral bone (if present) * Reattachment of muscles after caudad mobilization of the scapula * Muscle lengthening as needed 2. Woodward (preferred) * Characterized by detachment of scapular muscles from spinal insertion * Technical points * Resection of the superomedial portion of the scapula and omovertebral bone (if present) * Osteotomy of the clavicle * Detachment of the trapezius and rhomboid muscles * Mobilization of the scapula caudad * Reattachment of muscles back to vertebrae with the scapula in its new position \*\*Can improve abduction by 40-50^o

Answer 234

1. Hypertrophic scar (26–64%) 2. Brachial plexus injury (6–11%) 3. Regrowth of the superior pole of the scapula (30%) 4. Scapular winging (4–17%)

Answer 235

Traction or compression injury to the brachial plexus sustained during birth

Answer 236

Pseudoparalysis secondary to humerus fracture

Answer 237

1. Macrosomia (\>4,500g) 2. Difficult or prolonged labour 3. Shoulder dystocia 4. Multiparous pregnancy 5. Vacuum or forceps delivery 6. Prior BPBP

Answer 238

1. Character of the neurological injury * Root avulsion (preganglionic) * Root rupture (postganglionic) * Neuropraxia 2. Anatomic levels of involvement * C5-6 = Erb’s palsy (‘waiter’s tip’) * C8-T1 = Klumpke palsy (‘claw hand’) * C5-T1 = Total plexus palsy 3. Narakas classification * Group I = C5-6 palsy (~46%) * Group II = C5-7 palsy (~29%) * Group III = flail extremity (total plexus) without Horner syndrome * Group IV = flail extremity with Horner syndrome

Answer 239

1. Good prognostic factors * Erb’s palsy * Antigravity biceps function by 2-3 months 2. Poor prognostic factors * Lack of antigravity biceps function by 3-6 months * Root avulsion (preganglionic) * Suggested by: * Horner’s syndrome * Elevated hemidiaphragm (phrenic n.) * Scapular winging (dorsal scapular n.) * Lack of trapezius function * Klumpke’s palsy * C7 involvement

Answer 240

1. Absence of antigravity biceps function between age 3-9 months 2. Flail extremity and Horner’s syndrome at age 3 months

Answer 241

1. Nonoperative * Physiotherapy/parental stretching while awaiting return of function 2. Operative * Nerve grafting = root rupture (postganglionic) * Nerve transfer = root avulsion (preganglionic)

Answer 242

1. Shoulder internal rotation contracture * Latissimus dorsi and teres major transfer * Pectoralis major and subscapularis lengthening 2. Glenohumeral dysplasia * Proximal humeral derotation osteotomy 3. Elbow flexion contracture * Serial splinting or casting

Answer 243

1. Growth disturbance at the ulnar and volar aspect of the distal radial physis 2. Vicker’s ligament may be a contributor to the growth disturbance due to the increased pressure on the growth plate

Answer 244

Abnormal volar ligament that tethers the lunate to the volar distal radius

Answer 245

1. Ulnar and volar curvature (increased radial inclination and volar tilt) 2. Positive ulnar variance 3. Proximal subsidence of the lunate

Answer 246

The hand appears to be translated volarly and ulnarly relative to the wrist, and dorsal prominence of the ulnar head is a distinguishing feature

Answer 247

1. Leri-Weill Dyschondrosteosis * rare genetic disorder caused by mutation in the SHOX (short-statute homeobox-containing) gene * anatomically at the tip of the sex chromosome * causes mesomelic dwarfism (short stature) 2. Turner Syndrome 3. Nail-Patella Syndrome

Answer 248

* Pain * Limited motion * Cosmesis/deformity

Answer 249

1. Physiolysis and Vicker’s ligament release * Consider in early, mild deformity in the skeletally immature patient * Involves resection of ulnar and volar physis with fat interposition and Vicker’s ligament excision * Often combined with ulna shortening osteotomy or distal ulnar epiphysiodesis 2. Radial dome osteotomy +/- ulnar shortening osteotomy * Consider in more severe deformity * Involves Henry approach, dome osteotomy of distal radius, correction achieved by radial deviation and pronation of the hand and dorsal displacement of the distal fragment, Vicker’s ligament resection

Answer 250

1. Calcaneonavicular * Between the anterior process of the calcaneous and the navicular 2. Talocalcaneal * Between the middle facet of the talocalcaneal joint \*\*NOTE: these two types occur with equal frequency 3. Others * Talonavicular, calcaneocuboid, naviculocuneiform, cuneiform-metatarsal coalitions

Answer 251

25% (75% asymptomatic)

Answer 252

1. Rigid flat foot with peroneal spasticity 2. Hindfoot valgus, forefoot abduction, pes planus (arch does not reconstitute with toe standing)

Answer 253

1. Activity related pain in the sinus tarsi or medial hindfoot 2. Recurrent ankle sprains

Answer 254

8-12 years of age

Answer 255

1. Calcaneonavicular coalition * Anteater sign (elongated anterior process) on oblique foot view 2. Talocalcaneal coaltion * C-sign (lateral) * Dorsal talar beaking 3.Ball and socket ankle

Answer 256

Activity modification, NSAIDs, OTC soft shoe inserts, casting or walking boot

Answer 257

1. Indications for resection: * \<50% posterior facet involvement 2. Indications for subtalar fusion * \>50% posterior facet involvement * Posterior facet joint degeneration * Hindfoot valgus \>16-21 degrees

Answer 258

CT and/or MRI * CT – better assessment of coalition size and associated arthritis * MRI – better assessment of associated soft tissue pathology and fibrous/cartilaginous coalitions

Answer 259

1. Incision = Ollier (dorsolateral oblique incision) * Starts 1-2cm distal to tip of fibula and extends towards to talonavicular joint * Can extend as far as the extensor and peroneal tendons 2. EDB proximally is identified and retracted distally to identify the calcaneonavicular bar 3. Visualize the sinus tarsi, calcaneocuboid joint and talonavicular joint 4. Using a ¼ or ½ osteotome a trapezoidal (not triangular) piece of bone is excised 5. Visualize a sufficient gap and ensure motion occurs between the navicular and calcaneus 6. Interpose fat, bone wax or EDB * Free fat graft from buttock with EDB covering * EDB passed into defect with straight Keith needles tied over button on medial foot

Answer 260

1. Medial incision distal to the medial malleolus extending the length of the subtalar joint 2. Identify and protect the NV bundle and FHL 3. Tendon sheath is opened and FDL is taken inferior and tib posterior taken superior 4. Bar resection of the middle facet is completed with osteotome, rongeur and burr until normal cartilage of the posterior facet is visualized 5. Visualize gap and ensure adequate hindfoot motion 6. Bone wax applied to bony ends and fat graft interposed NOTE: can also be done all arthroscopic

Answer 261

1. WBC \> 12,000 cells/µl of serum 2. Inability to bear weight 3. Fever \> 101.3° F (38.5° C) 4. ESR \> 40 mm/h

Answer 262

0 = \<0.2% 1 = 3.0% 2 = 40.0% 3 = 93.1% 4 = 99.6%

Answer 263

1. Rickets is failure or delay of calcification of newly formed bone at long bone physes 2. Due to inadequate calcium or phosphate 3. Occurs at zone of provisional calcification [Orthobullets]

Answer 264

1. Decreased longitudinal bone growth 2. Angular deformities * Genu varum * Genu valgum * Coxa vara 3. Osteomalacia 4. Costochondral enlargement (rachitic rosary) 5. Kyphoscoliosis 6. Skull * Delayed anterior fontanelle closure * Parietal and frontal bossing * Plagiocephaly 7. Delayed primary dentition

Answer 265

1. Wide and indistinct growth plates (HALLMARK) 2. Lateral expansion of growth plates 3. Cupped and splayed metaphysis 4. Short long bones for age 5. Angular deformity (coxa vara, genu varum/valgum) 6. Looser zones * Transverse bands of unmineralised osteoid (‘Pseudofracture’) * Typically appear in the medial aspect of the proximal femur and at the posterior aspect of the ribs 7. Acetabular protrusio 8. Pathological fracture

Answer 266

1. Nutritional * Causes: * Vit D deficiency (most common) * Profound calcium deficiency (rare) * Combined Vit D and calcium deficiency * Presents at 6m - 3y * Pathophysiology [Orthobullets] * Low Vitamin D levels lead to decreased intestinal absorption of calcium * Low calcium levels leads to a compensatory increase in PTH and bone resorption * Bone resorption leads to increased alkaline phosphatase levels * Treatment = Vit D and calcium 2. X-linked hypophosphatemic rickets * AKA Familial hypophosphatemic rickets (x-linked dominant) * Cause = renal phosphate wasting AND low or normal kidney production of 1,25-dihydroxyvitamin D3 * Inability of renal tubules to absorb phosphate * Presents at 1 - 2y * Treatment = phosphate and Vit D (calcitriol) 3. Renal osteodystrophy * Causes = renal failure * Insufficient 1,25-dihydroxyvitamin D3 activation * Reduced phosphate excretion * Hyperphosphatemia causes hypocalcemia (reduced renal uptake) which causes secondary hyperparathyroidism * Treatment = dietary phosphate restriction, phosphate binding agent, Vit D3 4. Hypophosphatasia * Causes = ALP deficiency (autosomal recessive) * Treatment = no medical treatment 5. 1-Alpha-Hydroxylase deficiency (‘Vitamin D dependent’-Type 1) * Causes = unable to convert 25-hydroxyvitamin D3 to its biologically active form of 1,25-dihydroxyvitamin D3 * Treatment = 1,25 Vit D3 6. End organ insensitivity (‘Vitamin D dependent’-Type 2) * Causes = lack receptor for 1,25 Vit D3 * Treatment = high dose 1,25 Vit D3 and Calcium

Answer 267

Mutation in genes coding for Type I collagen resulting in quantitative and qualitative defects * Type I Collagen is a triple-helix molecule is composed of: * Two alpha-1 chains – COL1A1 gene * One alpha-2 chain – COL1A2 gene

Answer 268

1. Ocular * Blue sclera (present in ∼50% OI types) * Other – Glaucoma, Cataracts, Ectopia lentis, lens subluxation/dislocation, Presbyopia (farsightedness) 2. Dentinogenesis imperfecta * Discoloration of the teeth (yellowing and apparent transparency) * Abnormal formation of the teeth such as bulbous crowns and short roots * Teeth wear and break prematurely 3. Hearing loss * Occur in up to 50% of individuals by 50 years of age * Hearing loss may be conductive, sensorineural or mixed * Due to otosclerosis, fracture of the ossicles, as well as neural degeneration 4. Joint hypermobility 5. Easy bruisability 6. Cardiac * Mitral and aortic valve insufficiency * Aortic root dilation 7. Hypercalcuria * Affects 1/3 of OI patients * Increased kidney stone risk 8. Dysmorphic, triangle-shaped facies

Answer 269

1. Fracture * Multiple childhood fractures * Fractures tend to decrease after adolescents (can increase later in life) * Most commonly long bones and vertebral bodies (codfish vertebrae) * Apophyseal avulsion fractures of the olecranon are characteristic of OI 2. Long bone deformity 3. Short stature 4. Wormian skull bones 5. Scoliosis 6. Pectus excavatum/carinatum 7. ↓ Bone mineral density 8. Basilar invagination * Present in ∼8–25% * May present with symptoms including sleep apnea, headache, nystagmus, cranial nerve palsies, ataxia, and quadriparesis *

Answer 270

Silence Classification * TYPE I (non-deforming) * Prevalance = ∼50% (most common) * Severity = most mild (minimally deforming) * Inheritance = AD * Features: * Blue sclera – YES * DI – variable * Ambulatory, normal/slightly short stature, minimal kyphoscoliosis, variable hearing loss * Hallmark is multiple childhood fractures * Likely underdiagnosed * TYPE II (lethal) * Prevalance = N/A (rare) * Severity = lethal * Inheritance = AR * Features: * Fatal in the perinatal period secondary to thoracic bony insufficiency and respiratory complications * Type III (severe deforming) * Prevalance = ∼20% * Severity = most severe form compatible with life * Inheritance = AR * Features: * Blue sclera = NO * DI = YES * Wheelchair-bound or assistive devices, short stature, severe kyphoscoliosis, frequent hearing loss, shortened and bowed limbs, triangular facies, chronic pain * Type IV (intermediate) * Prevalance = ∼20% * Severity = moderate * Inheritance = AD * Features: * Blue sclera = NO * DI = variable * Moderately short stature, moderate kyphoscoliosis, variable hearing loss Modification to Sillence classification * Type V * Prevalance = ∼5-10% * Severity = moderate * Inheritance = AD * Features: * Blue sclera = NO * DI = NO * Normal hearing, mild to moderate short stature, variable kyphoscoliosis * Congenital bilateral anterolateral radial head dislocation with synostosis, hyperplastic callus formation in long bones following fracture * Type VI-XIII * Variable

Answer 271

1. General recommendations * Optimize Vitamin D and calcium intake * Encourage regular (low-risk) weightbearing activities * PT and aquatherapy for moderate-severe forms to maintain function and independence 2. Medical therapy * Bisphosphonates * Often started in childhood * Increases the bone volume but no effect on bone quality * Controlled trials are equivocal about reduction of long bone fractures and have not supported improved mobility or pain status with bisphosphonates * Still standard of care for moderate to severe OI

Answer 272

1. Fracture healing is normal – no need for prolonged immobilization 2. Nonsurgical treatment preferred in equivocal situations 3. 20% will experience nonunion over lifetime 4. Always get full length films to assess for bowing, deformity, limb 5. IM devices preferred over plate constructs * Issues with plates * Prone to fracture at ends of plates (stress riser) * Locking plates – promote stress shielding and bone resporption * Nonlocking plates – prone to failure via screw pullout * Issues with IM devices * Risk of iatrogenic fracture * Abnormal anatomy (supraphysiologic bowing, non-linear/imperforate canals) * Telescoping IM Rods * “Bailey-Dubow” “Sheffield” “Fassier-Duval” * Lengthens with growing bone * Consider after age 2 (prior to age 2 treat as if patient does not have OI)

Answer 273

Sofield osteotomy * “shish kebab” multiple long-bone osteotomies with IM device to correct deformity

Answer 274

1. Bracing not recommended due to fragility of the ribs * Continued progression and chest wall deformity secondary to the brace 2. Spinal fusion is considered when curve is \>45° * In severe OI consider fusion when curve \>35° 3. Consider preoperative bisphosphonate therapy * Strengthens cortical bone and improves pullout strength of pedicle screw * Hold postoperatively for ~4 months to allow remodelling of the fusion mass 4. Consider pedicle screws with cement augmentation * Used at the distal and proximal foundations

Answer 275

1. Surgical treatment for basilar invagination reserved for patients with clinical symptoms * Most commonly includes headaches, cranial nerve palsy, dysphagia, and symptoms of myelopathy, such as hyperreflexia, quadriparesis, and gait abnormality 2. Evidence lacking to support orthotic braces for asymptomatic basilar invagination or to delay independent upright posture until 18 months of age 3. Hydrocephalus addressed with ventriculoperitoneal shunt first 4. Craniocervical fusion with or without traction

Answer 276

Describes a group of nonprogressive disorders that result in fetal akinesia (decreased movement), multiple joint contractures and varying degrees of muscle weakness * Amyoplasia * Refers to the most common type with multiple joint contractures (classical disease) * Usually involves all 4 limbs * Distal arthrogryposis * Refers to involvement of the hands or feet

Answer 277

Independent function * Do not compromise function for cosmesis

Answer 278

1. Midline cutaneous hemangioma (nevus flammeus) on the forehead 2. Limbs appear thin, atrophic and are without normal flexion creases 3. Active motion is limited (weakness and contractures) 4. Characteristic contractures: * Upper extremity * Waiter’s tip – shoulders adducted and internally rotated, elbows extended, forearms pronated, wrist flexed and ulnarly deviated, thumb opposed * Hip * Hip flexion, abduction and external rotation contractures * Hip dislocation (~30%) * Knee * Flexion or extension contractures * Congenital dislocation of the knee * Foot * Rigid equinovarus * Congenital vertical talus * Scoliosis * C-shaped neuropathic curve

Answer 279

1. In general: * Initial treatment should start at birth and involves gentle stretching, range of motion and taping of any contractures * Once the position of a joint is acceptable, lightweight splinting may slow recurrence of contractures * If the joints cannot be placed into an acceptable position, serial casting or soft tissue releases followed by casting may be undertaken * In the lower extremity proceed from distal to proximal (feet – knees – hips) * Surgery may be avoided if deficits can be overcome with adaptive equipment 2. Hips * Hip flexion, abduction and external rotation contractures * Mild contractures – stretching * Soft tissue release * Hip dislocation * Teratologic hip dislocation – not responsive to Pavlik or closed reduction * Unilateral hip dislocation * Open reduction ~6-12 months of age * Observation (consider given high risk of failure and osteonecrosis) * Bilateral hip dislocations * Controversial – often observation * Dislocated supple hips are often better than reduced stiff hips * Open reduction of both will often not result in both being supple and reduced 3. Knee * Stretching and splinting first line * Flexion contracture * Quadricepsplasty * Distal femoral extension and shortening osteotomy * Extension contracture * Hamstring, posterior capsule, PCL release 4. Feet * Equinovarus * Ponsetti method * Posteromedial release – rigid recurrence, incomplete correction, older age at presentation 5. Upper extremity * Extension contracture * Stretching/splinting * Triceps lengthening and posterior capsular release * Internal rotation contracture * External humeral rotation osteotomy * Wrist flexion contracture * Stretching/splinting * FCU transfer to dorsum of hand (FCU is often only functioning wrist flexor/extensor) * Carpal wedge osteotomy * Thumb in palm deformity * Soft tissue release 6. Scoliosis * Bracing ineffective * Anterior and posterior spinal fusion

Answer 280

* Typically on right except in patients with dextrocardia * Treatment if symptomatic = take down pseudoarthrosis, restore length, tricortical ICBG, plate

Answer 281

\<50% physeal involvement and \>2 years or \>2cm growth remaining

Answer 282

Perform serial abdominal ultrasounds (every 3 months) until age 7 to rule out Wilm’s tumor

Answer 283

A 20-70% incidence of IgE mediated latex allergy

Answer 284

Occurs most commonly at L3 level due to unopposed hip flexion and adduction

Answer 285

Spinal muscular atrophy

Answer 286

Deceleration of growth of the posteromedial proximal tibial physis Varus, flexion, internal rotation deformity Medial and posterior sloping off proximal tibial epiphysis Variable relative tibial shortening (in unilateral cases)