Pediatrics Flashcards
(485 cards)
1
Q
Does the literature support leg length discrepancies after transphyseal ACL reconstruction in skeletally immature patients?
A
Several studies have not show any leg-length discrepancy. If performing transphyseal fixation should avoid oblique tunnel position, high-speed tunnel reaming, and increasing tunnel diameter (>8mm).
2
Q
What is characteristic of a patient with diplegia cerebral palsy?
A
Right and left side affected equally. Minimal spasticity in upper limbs. Lower limb spasticity predominates.
3
Q
What are thought to be causes of cerebral palsy?
A
perinatal TORCH infections, prematurity (most common), anoxic injuries, head injuries, and meningitis.
4
Q
What is the most common neurapraxia associated with supracondylar humerus fractures?
A
AIN (Branch of median nerve)
Unable to flex IP joint of thumb and DIP joint of index finger.
A-OK sign
5
Q
What is the second most common neurapraxia associated with supracondylar humerus fractures?
A
Radial nerve.
Can’t exten wrist, MCP joint, or IP joint fo thumb.
Rembmer PIP and DIP can still be extended via intrinsic function of ulnar nerve.
6
Q
What neurapraxia is associated with flexion type supracondylar fractures?
A
ulnar nerve.
Intrinsic function. cross fingers over.
7
Q
What are the ossification centers of the elbow and when do they first ossify?
A
Capitellum 1
Radial Head 4
Medial Epicondyle 6
Trochlea 8
Olecranon 10
Lateral epicondyle 12
CRMTOL
8
Q
Which ossification center is the last to fuse in the elbow?
A
Medial Epicondyle at 17.
Capitellum 12
Radial Head 15
Medial Epicondyle 17
Trochlea 12
Olecranon 15
Lateral Epicondyle 12
9
Q
Which supracondylar humerus fracture type is most likely to require an open reduction?
A
Flexion type.
10
Q
What is considered poorly perfused in regards to capillary refill?
A
> 2 seconds.
11
Q
Where should the anterior humeral line fall in children 5 or older? Where should it fall in children less than 5?
A
5 or older is should intersect the middle third of the capitellum.
In children less than 5 it should touch the capitellum.
12
Q
What is Baumanns angle?
A
Line parallel to the longitudinal axis of the humeral shaft and a line along the lateral condylar physis as viewed on the AP image.
Normal is 70-75 degrees.
Deviation of more than 5-10 degrees indicates coronal plane deformity.
13
Q
What is a an indication for pinning SCH fractures other than extension and flexion types?
A
Medial column collapse.
14
Q
What is a concerning risk of floating elbow in pediatric patients?
A
Compartment syndrome.
15
Q
What is the brachialis sign in regards to SCH fractures in pediatric patients?
A
ecchymosis, dimpling/puckering atecubital fossa, and or palpable subcutaneous bone.
Indicates proximal fragment buttonholed thorugh brachialis.
16
Q
How should pins be inserted for flexion type SCH fractures?
A
Pins should be inserted in extension.
17
Q
What difference is there in stability between three lateral pins and crossed pins?
A
No significant difference but corssed pins are strongest to torsional stress.
18
Q
In what cases are three pins required over two for SCH fractures?
A
Comminution and Gartland type III and IV.
19
Q
If having to place a medial pin for SCH fracture how can you reduce ulnar nerve injury?
A
Place medial pin with elbow in extension.
Use small medial incision.
20
Q
What is the most common complication associated with SCH fractures?
A
Pin Migration 2%.
21
Q
What causes cubitus varus in pediatric patients?
What functional limitation does it cause?
A
Fracture varus malunion.
It is not caused by growth disturbance.
Usually only a cosmetic issue and causes little functional limitation.
22
Q
What is a complication of immobilizing a SCH in greater than 90 degrees of elbow flexion?
A
Increase in deep volar forearm compartment pressures. Leading to Volkmann ischemic contractures.
23
Q
What should be done about post-operative stiffness after CRPP of SCH fx?
A
allow patient to work on motion on their own.
Literature doesn’t support physical therapy.
Almost always resolved by 6 months.
24
Q
Olecranon avulsion fracture is highly suspicious of which condition?
A
Osteogensis imperfecta.
25
What is the Milch classification of pediatric elbow fractures?
Type I fracture line is lateral to trochlear groove. Less common. Elbow is stable.
Type II fracture line is medial to trochlear grove. More common. More unstable.
26
What does the lateral ecchymosis imply in a lateral condyle fracture of the distal humerus?
tear in the aponeurosis of the brachioradialis and signals an ustable fracture.
27
What view best shows fracture displacement of pediatric lateral condyle fracture?
Internal oblique view.
This is because fracture is posterolateral.
28
What is an indication for open reduction of lateral condyle fracture of the distal humerus?
Articular incongruity.
Greater than 4mm of displacement.
29
Where is the blood supply located for the lateral condyle?
Posteriorly.
This is why an anterolateral approach is used.
30
What is the most common complication of pediatric lateral condyle fractures?
Stiffness.
31
What is a complication of not aligning the periosteum after fixation of a lateral condyle fracture?
Lateral overgrowth or spurring.
32
What is the most common cause of Cubitus valgus after lateral condyle fracture?
More commonly due to nonunion but it can be due to lateral physeal arrest.
Slow progressive ulnar nerve palsy caused by stretch.
Incidence is 10%. Less common than cubitus varus
33
What is treatment for cubitus valgus?
Supracondylar osteotomy after skeletal maturity and ulnar nerve transposition.
34
What is more common after cubitus varus or cubitus valgus?
cubitus varus.
35
Unsuccesful outcomes with radial head fractures in pediatric patients has been correlated with what age?
Age \> 10 yrs.
36
What complications must you have a high suspicion for after pediatric radial head fracutres?
forearm compartment syndromes.
37
Will you see a fat pad sign with radial neck fractures?
Not always as a portion of the radial neck is extra-articular so fat pad signs and effusion may be absent.
38
What is the criteria for treating a radial neck fracture with immobilization alone.
\<30 degrees of angulation
\<3mm translation
7 days of immobilization followed by early ROM.
39
When is open reduction of a radial neck fractute indicated?
\> 45 degrees of persistent angulation after attempt at closed percutaneous reduction.
Open reductions have been associated with greater loss of motion, increased osteonecrosis, and synostosis. Controversial as it is not known if this is due to worse fractures undergoing open reduction.
40
What should you do to protect the radial nerve during a Kocher approach?
Pronate.
41
What after 20-40% of radial head fractures but usually does not affect function?
Radial head overgrowth.
42
When should a rigid nail be considered in pediatric femur fractures?
Age \>= 11 years
Weight \> 49 kg
Very proximal or distal fractures
Unstable comminuted or long oblique fracture patterns.
43
What is the starting point for a lateral trochanteric entry nail?
At the tip of the greater trochanter.
Angle should be 12 degrees offset to the anatomic axis of the femur.
Piriformis fossa should be avoided to avoid the arterial blood supply.
44
At what age can acetabular index be measured on an AP radiograph?
What is a normal measurement?
2 years
25 degrees or less.
45
When can you attempt closed reduction of tibial eminence fractures?
Less than 5mm otherwise ORIF vs AAIF.
46
What is the pathoanatomy of a nursemaids elbow?
subluxation of annular ligament which then becomes interposed between radial head and capitellum.
Rare after 5 years of age.
47
At what age do pediatric elbow dislocations usually occur?
Older children 10-15 years.
48
What is the most common associated fracture with a pediatric elbow dislocation?
avulsion of the medial epicondyle.
49
Most common neuropraxia seen with pediatric elbow dislocations?
Ulnar Nerve.
50
What should be part of your differential when seeing pediatic elbow dislocations, especially radiocapitellar joint?
Congenital dislocations.
51
What is the anatomic classification of pediatric elbow dislocations?
What is it based on?
Which one is most common?
Posterolateral, posteromedial, anterior (rare), and divergent.
position of the proximal radio-ulnar joint in relation to the distal humerus.
posterolateral.
52
How long should a stable pediatric elbow dislocation be immobilized after reduction?
minimize to 1-2 weeks.
53
What is the most important part to visualize on post reduction radiographs of a pediatric elbow dislocation?
medial epicondyle to ensure it is not within the joint.
54
What is the most common complication following treatment of pediatric elbow dislocations?
loss of terminal extension.
Most often due to prolonged immobilization.
55
What should you look for in an isolated pediatric radial head dislocation?
Need to look for plastic deformation of the ulna.
Rare to have an isolated radial head dislocation.
56
In what position should a Bado I type fracture be immobilized?
Bado 1 is anterior dislocation of radial head.
Axpe anterior ulna.
110 degrees of flexion with full supination to tighten interosseous membrane and relax biceps tendon.
57
In what position should a Bado II type fracture be immobilized?
Bado II is posterior radial head dislocation.
Apex posterior ulna.
Immobilize in full extension.
58
How should a Bado III type fracture be immobilized?
Lateral radial head dislocation.
apex lateral proximal ulna.
Immobilize in full extension and valgus mold.
59
Most common neurapraxia associated with Moteggia fractures?
PIN.
10% of acute injuries.
Almost always spontaneously resolves.
60
Most common nerve injury with pediatric both bone forearm fractures?
How often does it occur?
Median nerve
1% of fractures.
61
Acceptable reduction parameters for pediatric both bone forearm fractures?
62
Is there a difference in outcomes of distal both bone forearm fractures treated in short arm casts vs long arm casts?
No increase in loss of reduction with short arm casts.
63
What difference in in union rates, radial bow, and rotations is there between flexible IMN and ORIF of pediatric both bone forearm fractures?
None.
64
What is the refracture rate following treatment of both bone forearm fractures?
5-10%
Associated with greenstick patterns and plate removal.
65
How much does motion improve after resection of synostosis following pediatric both bone forearm fractures?
Rarely leads to any improvement in motion.
66
When do you consider operative intervention for pediatric proximal humerus fractures?
Open fx.
Neurologic injury.
fractures displaced more than 50% in children greater than 11 years old.
67
What is the most common fracture in children under 16 years old?
Distal radius fracture.
68
What is the growth rate of the distal radius physis?
5.25mm/yr.
40% growth of upper extremity.
75% growth of the radius.
69
What other injuries can be seen with pediatric distal radius fractures and must be ruled out?
DRUJ
Ulnar styloid
Elbow injuries
Scapholunate interval
70
What directional deformity is least likely to remodel?
Rotational deformities.
71
What angulation is acceptable in a pediatric distal radius fracture for ages \< 9 yrs?
\> 9 yrs?
30 degrees
20 degrees
72
How much bayonette apposition is acceptable in pediatric both bone forearm and distal radius/ulna fractures?
\<1cm.
73
Loss of reduction for a distal radius fracture treated with a cast is associated with?
poor cast index.
sagital/coronal widths need to .8 or less.
74
Indications for CRPP of pediatric distal radius/ulna fractures?
Unstable patterns
Unable to reduce initially
Loss of reduction at follow-up
SH1 or 2 fractures in setting of neurovascular compromise
Compartment syndrome
75
Risk factors for thermal injuries with casting?
Water \> 74F
More than 8 layers
Placing cast while setting on pillow
Wrapping fiberglass over plaster.
76
What apophyseal avulsion is seen with hamstrings or adductors?
ischial avulsion.
77
What eccentric muscle contraction can lead to a AIIS avulsion?
Rectus femoris.
78
What apophyseal avulsion involves the sartorius?
ASIS
79
What muscles are involved in pubic symphysis and iliac crest avuslions?
Abdominal muscles
80
What muscles is involved in a lesser trochanter apophyseal avulsion?
iliopsoas.
81
Which pelvic ring injury is most common in pediatric patients?
lateral compression.
82
Do pediatric pelvic ring injuries have a higher or lower rate of hemorrhage when compared to adult pediatric ring injuries?
lower.
83
What injuries are associated with pediatric pelvic ring fractures?
At what rate do these occur?
CNS and abdominal visceral injuries.
\> 50%
84
How are pediatric pelvic ring fractures with instability and \< 2cm of displacement treated?
Bed rest followed by progressive mobilization.
85
How are apophyseal avulsions of the pelvis and proximal femur treated when they have 2cm of displacement.
Non-operative.
Need \>2-3 cm for operative treatment.
PWB for 2-4 weeks
Stretching and strengthening at 4-8 weeks
Return to sport at 8 weeks if asymptomatic
86
Rate of premature closure of triradiate cartilage with acetabular fractures?
\<5%
Higher risk in children \< 10yrs old at time of injury.
87
What is the most common orthopaedic reason for hospitalization in pediatric patients?
Femur fractures.
88
Is early surgical intervention recommended in children with closed head injureis and femur fractures?
Yes, children to not have the increased pulmonary complications that are seen in Adults.
Decreased length of hospital stays.
89
What femur fracture patterns and up to what age can be treated in a pavlik harness?
Any pattern
\< 6 months of age
90
What children can undergo spica casting for femoral fractures?
What are relative contraindications?
Up to 5 years of age.
polytrauma, open fractures, and shortening \>2-3cm
91
Indications for flexible intramedullary nailing for femur fractures?
5-11 years.
Less than 49kg
Length stable.
92
How would you treat a distal femoral buckle fracture in a 5 year old?
Long leg cast. Spica not needed for distal buckle fractures.
Can also treat non-displaced SH 1&2 distal femur fractures in LLC.
93
What is the most common complication seen with flexible intramedullary nailing of femur fractures?
pain near knee at insertion site of nails.
94
What secondary deformities of the proximal femur can occur after IMN via greater trochanteric insertions?
Narrowing of the femoral neck
Premature fusion of greater trochanter apophysis
Coxa valga
Hip subluxation
95
What is the most common complication in patients \<10 years old with femur fractures?
Overgrowth leading to leg length discrepancy.
.7-2 cm that typically occurs within 2 years of injury.
This is why shortening in treatment is acceptable
96
How is a Thurston-Holland fragment created?
Salter-Harris II fracture
Physis fails on tension side.
Metphysis fails on compression side
97
What is the rate of physeal arrest after distal femoral physeal fracture?
30-50%
Increased incidence with increased fracture displacement and SH type.
SH1 36%
SH2 58%
SH3 49%
SH4 64%
98
What is the first epiphysis in the body to ossify?
Distal femoral.
99
Growth rates of lower extremity physes?
Proximal femur 3mm
Distal femur 9mm
Proximal tibia 6mm
Distal tibia 5mm
100
Most common cause of irreducible SH1 and SH2 fractures of the distal femur?
interposed periosteum on the tension side of the fracture.
101
Indiction for physeal bridge excision that occurs after distal femur fracture?
physeal bar of \<50%
2 years or more and 2.5cm of growth remaining.
102
What is more common in pediatric patients hip fracture of hip dislocations.
hip dislocations.
80% are traumatic posterior dislocations
Can occur due to low injury sports injuries in children less than 10 years of age
103
Study of choice for post hip reduction imaging of a child?
MRI
Better to evaluate soft tissues and cartilaginous hip
Less radiation
104
When open reducing a pediatric hip dislocation which approach should be used?
Should be perfromed in the direction of the dislocation
So either kocher-langenbeck or Smith-Peterson
105
What is current thoughts on the most common cause of osteonecrosis in pediatric hip dislocations?
Delayed time to reduction of more than 6 hours.
Rates of AVN 3-15%
Decreased incidence in children under age of 5.
106
What does injury to greater trochanteric apophysis lead to?
Overgrowth?
Shortening of GT and coxa valga
Overgrowth leads to coxa vara
107
Describe the Delbet Classification?
108
Treatment for proximal femur fractures in pediatric patients if non-displaced and pt is \< 4yrs old?
Closed reduction and spica abduction casting.
109
What is the difference in how displaced intertrochanteric fractures and proximal are treated in pediatric patients less than 4 yrs and older than 4 yrs?
Older than 4 yrs Cannulated screws
Less than 4 yrs smooth or threaded pins/K wires
110
Should you cross the femoral head physis in proximal femur fractures?
Consider stoppin short of physis in 4-6 year olds.
Cross physis when there is little metaphyseal bone and patients are \> 12 years old.
Otherwise controversial.
If not crossing physis should place in post-op spica.
111
What is the most common complication of pediatric hip fractures?
AVN
112
What is the 2nd most common complication of proximal femur fractures?
Coxa vara
Defined as a neck-shaft angle \<120 degrees
More common if fracture is treated non-operatively
113
Treatment of Coxa Vara that occurs after proximal femoral fractures?
Pts 0-3 with neck-shaft angle \>110 degrees will remodel
Mild coxa vara in 6-8 year olds can perfrom surgical arrest of trochanteric apophysis
Subtrochanteric or intertrochanteric valgus osteotomy. For older patients with non-union or severe trendelenburg limp of FAI.
114
What mechanism typically caused a pediatric tibial eminence fracture?
rapid deceleration or hyperextension/rotation of the knee.
Same mechanism that would cause ACL tear in adults.
115
What is the most common reason for failed closed reduction of a tibial eminence fracture?
meniscal tear with entrapment of the naterior horn of the medial meniscus being the most common.
116
Most common complication with tibial eminence fractures?
Loss of motion, especially loss of extension. May be due to impingment due to incomplete reduction.
Arthrofibrosis is most common with surgical reconstruction.
117
What tibial eminence fracture con be treated non-operatively?
What is the recommended protocol?
Type I (less than 3mm displacement and Type II (minimally displaced with intact posterior hinge).
+/- aspiration with injection of lidocaine. closed reduction with extension. Then casting in full extension for 3-4 weeks. Gradual rehab program.
118
How do patellar sleeve fractures usually occur?
In what population do the most commonly occur?
Indirect injury from quadriceps contraction applied to a flexed knee.
Males 5:1. Age 8-12. \<1% of pediatric fracrues but \>50% of pediatirc patellar fracutres.
119
What are Kocher's Criteria?
fever \>= 38.5 C
Refusal to bear weight on the affected extremity
ESR \> 40
WBC \> 12k
120
Does tibial tubercle fractures occur by concentric contraction during jumping or eccentric contraction during forced knee flexion?
Trick question, it occurs from both mechanisms.
121
What are the two ossification centers of the tibial tubercle?
proximal tibial physis is the primary ossification center.
tibial tubercle physis is the secondary ossification center.
phsis closes from posterior to anterior and proximal to distal this is why the tubercle physis is at greater risk of injury.
122
What artery has been implicated in compartment syndrome after tibial tubercle fracture?
Recurrent anterior tibial artery.
123
What is different when treating a periosteal sleeve avulsion tibial tubercle fractures as oppossed to a displaced fracture extending through the tubercle?
Need to immobilize the sleeve avulsion for 8-10 weeks as oppossed to 4-6 weeks becuase soft tissue healing vs bone healing.
124
What is the most common complication following surgical repair of a tibial tubercle fracture?
What deformity can occure after a tubercle fracture?
Bursitis from harware irritation.
Recurvatum because anterior arrests while posterior continues to grow.
Uncommon to see leg length discrepancies because of the age at which these occur 12-15 yrs.
125
Describe the predictable closure of the proximal tibia physis.
Sagittal plane- posterior to anterior
Coronal plane-medial to lateral
Axial plane- posteromedial to anterolateral
126
Does the medial collateral ligament insert proximal or distal to the proximal tibial physis?
Distal.
127
What is Cozen's phenomenon?
tendency of a proximal tibial metaphyseal fracture to develop a late valgus deformity.
Should be casted in extension with a varus mold.
Valgus deformity usually resolves spontaneously
Develops 5-15 months after injury with maximum deformity at 12-18 months.
Incidence as high as 50-90%
128
Treatment for a cozen fracture?
Observe for 12-24 months. Most spontaneously resolve.
Average deformity at its worst 18 degrees.
Average final deformity of 6 degrees.
guided growth or osteotomy rarely indicated for deformities \> 15-20 degrees near skeletal maturity.
Usually have a limb length discrepancy on average of 9mm with the affected limb being longer.
129
Acceptable reduction of a pediatric tibia diaphyseal fracture?
\<50% of translation
\< 1cm of shortening
\<5-10 degrees of angulation in the sagittal and coronal planes
130
Tibial shaft fracture with intact fibula will fall into?
Tibial shaft fracture with associated fibula fracture will fall into?
Varus
Valgus and recurvatum
131
What is a tillaux fracture?
Salter-Harris III fracture of the anterolateral distal tibia.
Cased by avulsion of the anterior inferior tibiofibular ligament.
More common in girls.
Seen in kids nearing skeletal maturity. Older than triplane fracture age group.
132
What is the mechanism of injury that leads to a tillaux fracture?
What distinguishes it from a triplane fracture?
Supination-external rotation injury.
Lack of coronal plane fracture in the posterior distal tibial metaphysis.
133
What is the pattern of distal tibial physeal closure?
Central -\> anteromedial -\> posteromedial -\> lateral
134
Non-operative treatment of a Tillaux Fracture
Reduction by dorsiflexing and then internally rotating foot.
Long leg cast (to control rotation) for 3-4 weeks.
Short leg cast or CAM boot 2-4 weeks.
135
What is a type V Saltar-Harris fracture?
Is there a type VI?
Crush injury to the physis. Dificult to identify initially, usally diagnosis made on follow-up.
Yes. Rare. Perichondral ring injury that results from an open injury such as a lawnmower injury or iatrogenic.
136
What amount of physeal widening is acceptable to treat non-operatively?
\< 3mm.
137
Which Saltar-Harris ankle fracture has the highest rate of growth distrubance?
SH IV medial malleolus fracture.
138
Risk factors for growth arrest after saltar-harris fractures?
Degree of initial displacement. 15% incresed risk for every 1mm of displacement.
Residual physeal displacement \> 3mm.
High-energy injury mechanism.
SHIII and IV fractures
139
When is physeal bar resection indicated after pediatric ankle fractures?
\< 20 degrees of angulation with \< 50% of physeal involvement and \> 2 years of growth remaining.
140
When is an ipsilateral fibular epiphysiodesis indicated after a pediatric ankle fracture?
Bar of \> 50% and \> 2 years of growth remaining.
141
What deformity can occur after triplane, SH1, and SH2 ankle fracutres?
Rotational deformity.
External foot rotation angle.
Treatment is derotational osteotomy.
142
Which fracture typically happens in younger patients Tillaux or Triplane?
Triplane fractures (average age is 13 years old.)
143
What are the three components of a triplane fracture?
Fracture through epiphysis, physis, and metaphysis.
The orientation can differ depending on the type of triplane fracture. Lateral vs medial.
Lateral: Epiphysis sagittal with metaphyseal coronal
Medial: Epiphysis coronal with metpahyseal sagittal
144
What are the common cutaneous and musculoskeletal manifestations of Neurofribromatosis?
cage au lait spots, axillary freckling, lisch nodules in the eye, scoliosis, long bone bowing, and pseudoarthrosis.
anterolateral tibial bowing.
145
What pediatric patient may be a candidate to undergo a selective dorsal rhizotomy?
4-8 year old with spastic diplegia who is ambulatory and has no evidence of athetosis.
Involves selective resection of the L2-S1 nerve roots that do not show a myographic or clinical response to stimulation.
146
What is athetosis?
slow, involuntary, convoluted, writhing moevements of the fingers, hands, toes, and feet.
147
What is the most common infectious organism in neonates?
Group B Strep
148
True or false children with mycobacteria tuberculosis are more likely to have extrapulmonary involvement?
True
149
Risk factor for development of a DVT in children with osteomyelitis?
CRP\>6
surgical treatment
age \>8 years old
MRSA
infrequent complication in children.
150
Position of the hip that has the least amount of intracapsular pressure?
flexion, abduction, and internal rotation.
How a child with transiet synovitis or septic arthrits may present.
151
What are some lab values that can be used to distinguish transient synovitis from septic arthrits?
CRP 20mg/L or 2mg/dl. Less than that more likely synovitis. More than that more likely septic arthritis
If aspiration is perfromed Synovial WBC cutoff of 50,000WBC
Other things are fever is mild or absent in synovitis
152
What age is pediatric septic hip arthritis most common?
first few years of life.
50% of cases occur in children younger than 2 years of age.
153
Rsik factors for neonatal septic arthritis?
prematurity
Cesarean section
NICU
Invasive procedures, even venous catheterization and heel puncture
154
What is a provocative test to evaluate a patient for a psoas abscess?
Psoas sign which is pain caused by extension and internal rotation of the limb.
155
What is the treatment of choice for a large psoas abscess?
Percutaneous ultrasound or CT guided drainage.
Open drainage is indicated for a secondary psoas abscess that has spread from the bowel as both can be addressed at the same time.
156
Is there a surgical correction for Sprengel deformity?
Yes
Indicated for children with severe cosmetic concerns or functional deformities (abduction \< 110-120 degrees)
Best to perform surgery from 3 to 8 years of age. Greater risk of nerve impairment after the age of 8.
Woodward of Greeen procedure. Can imporve abduction by 40-50 degrees. Basically detatch and move medial parascapular muscles to allow scapular to migrate inferiorly.
157
Where and what side does congenital pseudoarthrosis of the clavicle most commonly occur?
Right side.
Middle 1/3
two ossification centers. One medial and one latera.
158
What is the pathophysiology of coxa vara?
proximal femoral cartilaginous physis or ossification center defect in the inferior femoral neck.
No clear inheritance pattern.
159
What are the different etiologies of coxa vara?
developmental
Congenital (such as PFFD)
Acquired (SCFE, Perthes, infection)
Dysplasia ( OI, Jansen, Schmid, SED)
cretinism
160
What measurement is considered a varus neck shaft angle in pediatric proximal femurs?
\< 120 degrees
161
What is Hilgenreiner-iphyseal angle used for?
How is it measured and what is a normal and abnormal measurement?
Assessment of coxa vara
angle between Hilgenreiner's line and a line through proximal femoral epiphysis.
Normal \< 25 degrees
\<45 degrees unlikely to progress
54-60 degrees requires close follow-up even if asymptomatic.
162
Goals of corrective valgus derotation osteotomy?
Over-correct varus neck shaft angle. Reduce Hilgenreiner physeal angle to \< 38 degrees.
Correct leg length discrepancy
Correct hip anteversion/retroversion
re-establish abductor msucle tensioning
163
What occurs in 70% of infants with obstetric brachial plexopathy?
Glenohumeral dysplasia
See increased glenoid retroversion, humeral head flattening, posterior humeral head subluxation
Caused by internal rotation contracture.
164
What is the prognosis for obstetric brachial plexopathy?
90% of cases will resolve spontaneously without intervention.
Recovery may occur for up to 2 years.
165
What are favorable variables for spontaneous recovery from obstetric brachail plexopathy?
Poor prognostic variables?
GOOD: Erb's Palsy, Complete recovery if biceps and deltoid are anti-gravity by 3 months, and early twitch biceps activity.
POOR: Lack of biceps function by 3 months. Preganglionic injuries(worst prognosis as they are avulsions from the cord. Seen with Loss of rhomboid fuction, and elevated hemidiaphragm). Horner's syndrome. C7 involvement. Klumpke palsy.
166
What percent of obstetric brachial plexopathies with Horner's Syndrome will recover?
10% recover spontaneous motor function.
167
Loss of rhomboid funtion and elevated hemidiaphragm signify what kind of plexopathy?
preganglionic injury
avulsion from the cord
will not recover spontaneously
168
When should EMG be used for obstetric brachial plexopathies?
rarely
poor reliability
Often underestimate the severity of injury.
169
What is the most common type of obstetric brachial plexopathy?
Erb's Palsy (C5,6)
170
What is Erb's Palsy
Brachial plexopathy C5,6
Best prognosis for spontaneous recovery
adducted, internally rotated shoulder
Pronated forearm, extended elbow
C5: axillary, suprascapular, and musculocutaneous nerve deficiency.
C6: radial nerve deficiency
171
What is Klumpke's Palsy?
C8,T1 Brachial plexopathy
Rare
deficit of all the small muscles of hand (ulnar and median nerves
Claw Hand: wrist in extreme extension, hyperextension of MCP due to loss of hand intrinsics, and flexion of IP joints fue to loss of hand intrinsics.
Poor prognosis
Frequently associated with a preganglionic injury and Horner's Syndrome
172
What is treatment for a preganglionic obstetric brachial plexus palsy?
When should it be done?
Neurotization using expendable motor fascicles from the median and ulnar nerves to biceps and brachialis branches of the musculocutaneous nerves.
Before 3 months of age.
173
What is the difference between neurotization and nerve transfer?
**nerve transfer** refers to fasciciles from one nerve transferred into another nerve that supplies a muscle.
**Neurotization** refers to placing nerve fascicles directly into a neuromuscular junction of a muscle.
174
What is a Hoffer procedure?
Latissimus dorsi and teres major transfer.
Indicated for persistent internal roation contracture or external rotation weakness without glenohumeral dysplasia in children with a history of brachial plexopathy.
175
What is a Wickstrom procedure?
proximal humeral derotation osteotomy.
Indicated for persistent internal roation conractures or external rotation weakness **with glenohumeral dysplasia.**
176
What is the treatment for elbow flexion contractures after obstetric brachial plexopathy?
\< 40 degrees: serial nighttime elbow extension splinting. Prevent progression but does not correct contracture.
\> 40 degrees: serial elbow extension casting
Operative for severe persistent contrctures. Perform anterior capsular release with biceps/brachialis tendon lengthening. There is a high recruurence rate with this.
177
What is done for phrenic nerve palsys from obstetric plexopathies?
If persistent may require diaphragm plication.
178
What is the treatment for residual forearm supination contracture after obstetric brachial plexopathies?
Intact passive pronation -\> biceps rerouting transfer
Limited passive forearm pronation -\> forearm osteotomy with biceps rerouting tendon transfer
179
What is a teratologic hip?
dislocated in utero and irreducible on neonatal exam.
pseudoacetabulum.
associated with neuromuscular conditions and genetic disorders.
Commonly seen with arthrogryposis, myelomeningocele, Larsen's syndrome, Ehlers-Danlos
180
What is the most common orthopaedic disorder in newborns?
Hip dyslplasia.
181
What are the risk factors for hip dysplasia?
Firstborn
Female
Breech
Family history
Oligohydramnios
More common in left hip due to left occipur anterior being the most common intrauterine position of the fetus.
182
Where is acetabular deficiency in spastic cerebral palsy?
posterosuperior
typical deficiency for hip dysplasia is anterior or anterolatera.
183
What are the associated conditions with hip dysplasia?
Known as "packaging deformities"
Congenital muscular torticollis
Metatarsus adductus
Congenital knee dislocation
184
What physical exam maneuvers can be performed at \>3months to 1 year to screen for hip dysplasia?
Ortolani and Barlow rarely positive after three months
Limitation in hip abduction
Leg length discrepancy
Klisic test (Line from long finger placed over GT and ASIS should point to umbilicus if the hip is normal. If it is dislocated it will point half way between umbilicus nad pubis.
185
What physical exam findings will you find in a child greater than one who is walking who has hip dysplasia?
Pelvic obliquity
Lumbar lordosis
Trendelenburg gait
toe walking
186
When does the femoral head begin to ossify?
By 6 months.
187
Desbribe the following ragiographic lines:
Hilgenreiner's Line
Perkin's Line
Shenton's Line
Acetabular Index
CEA of Wiberg
Femoral head ossification should be inferior to this line.
Femoral head ossification should be medial to this line.
Should be a continuous arc between inferior border of the femoral neck and the superior margin of the obturator foramen.
Angle formed by H and a line from a point on the lateral triradiate cartilage to a point on lateral margin of acetabulum. Should be \< 25 deg in patients \> 6 months
Angle from P line and a line from the center of the femoral head to the lateral edge of the acetabulum. \< 20 deg is considered abnormal. Only reliable in patients \> 5 years old.
188
What are possible blocks to reduction of a dysplastic hip?
What should be done after to confrim reduction?
Inverted labrum
Inverted limbus (fibrous tissue)
transverse acetabular ligament
Hip Capsule (contracted by the iliopsoas tendon causing an hourglass deformity)
Pulvinar
LIgamentum teres
ARTHROGRAM
189
Treatment recommended for a 4 month old found to have hip dysplasia with a dislocated hip that is reducible?
Pavlik harness for those \<6 months old.
Contraindicated in teratologic hip dislocations, spina bifida, and spasticity.
190
Treatment for hip dysplasia in 6-18 month olds?
closed reduction and spica casting
191
Treatment for a 20 month old child who has failed closed reduction for right hip dislocation?
open reduction and spica casting for 18-24 month old children.
192
Treatment for a 3 year old with residual hip dysplasia on x-ray with coxa valga?
Open reduction with a femoral osteotomy
Pelvic osteotomies are more commonly used in childre \> 4 years old and those with an increased acetabular index.
193
What are complications related to Pavlik harnesses?
AVN (seen with extreme abduction \> 60 degrees, due to impingement of the posterosuperior retinacular branch of the medial femoral circumflex artery)
Transient femoral nerve palsy seen with hyperflexion.
Pavlik disease, which is erosion of the pelvis superior to the acetabulum. IMPORTANT TO DISCONTINUE THE HARNESS IF THE HIP IS NOT REDUCED BY 3-4 WEEKS.
194
What should you do with a infant who is 4 weeks old and has been treated in a Pavlik harness for over three weeks but remains Ortolani positive?
Convert to a semi-rigid abduction brace with weekly ultrasounds for another 3-4 weeks.
195
How much medial dye pooling is concerning on an arthrogram performed after a closed reduction of a dysplastic hip?
\>7mm is associated with poor outcomes and AVN
Want \< 5mm
196
What can be done if an unstable safe zone is required to maintian reduction of a dysplastic hip?
adductor tenotomy if \> 60 degrees is required to maintian reduction.
197
What position should the limb be immobilized in a spica cast for a child with hip dysplasia?
100 degrees of flexion
45-55 degrees of abduction
Neutral rotation
Confrim after with imaging.
Change cast at 6 weeks. 3 months total of treatment.
198
Which approach should be used for open reduction of a hip with dysplasia in a child? Medial or Anterior?
Anterior in children \> 12 months. Advantages: capsulorrhaphy can be performed. decreased risk of AVN
Medial in children \< 12 months. PROS: directly addresses blocks to reduction, decreased blood loss CONS: unable to perform capsulorrhaphy and higher risk of AVN
199
What are the two salvage pelvic osteotomies used for hip dysplasia?
Shelf
Chiari
200
What pelvic osteotomies require an open triradiate cartilage?
Salter, Steele(Triple), and Pemberton.
Dega as well but this is only for severe cases. Favored in neuromuscular dislocations and patients with posterior acetabular deficiency.
201
When are children with delayed diagnosis of hip dysplasia and a dislocated hip better off treated non-operatively?
Bilateral dislocations function better if 6 years of age or older.
Unilateral dislcoation better if patient is \> 8 years old.
202
What is cloacal exstrophy?
Where bladder exstrophy ivolved wht intestingal track as well.
1/200,000 infants
203
What MSK issue is seen in patients with bladder exstrophy?
Acetabuli are 12 degrees retroverted.
Without pubis to tether the anterior ring the posterior elements retrovert
Waddling gait with external foot progression.
PELVIC OSTEOTOMY IS PERFORMED IN ORDER TO DECREASE TENSION ON THE BLADDER AND ABDOMINAL WALL REPAIR. Not required in newborns, skin traction and hip flexed 90 degrees is enough. Plate fixation \> 8 yrs. Otherwise ex-fix.
204
What is the single greatest risk factor for SCFE?
Obesity.
Other risk factors include males, acetabular retroversion and femoral retroversion, males, and specific ethnicities(african americans, pacific islanders, and latinos.
205
Which zone of the physis does the SCFE occur through?
Hypertrophic zone.
206
What conditions are associated with SCFE?
What are the indications for an endocrine work-up?
Hypothyroidism (most common, will see an elevated TSH)
Renal osteodystrophy
Growth Hormone deficiency
Panhypopituitarism
Down Syndrome
Child is \< 10 years old
Weight is \< 50th percentile
207
Would the inability to ambulate unless using crutches indicated a stable or unstable SCFE?
What is important about the unstable vs stable designation?
Stable.
Unstable would be if the child cannot ambulated even with the help of assistive devices.
Its prognostic. Unstable have a higher risk of osteonecrosis 47% vs \<10%.
Unstable should be treated urgently.
208
What can symptom can present with a SCFE and lead to misdiagnosis.
What is this symptom due to?
Knee pain
pain activation of the medial obturator nerve.
209
What is the Drehmann sign?
Obligatory external rotation during passive flexion of the hip found in children with a SCFE.
Due to a combination of synovitis and impingement of the displaced anterior-lateral femoral metaphysis on the acetabular rim.
210
What is Klein's line?
Line drawn along the superior border of the femoral neck.
Will intersect lateral femoral head in a normal hip.
211
When should you considere contralateral hip prophylactic fixation of a SCFE?
What about capsulotomy and open reduction?
\<10 years of age, open triradiate cartilage, endocrine disorders, and obese males.
Contoversial, if done only in unstable SCFES where intracapsular pressur eis double.
Really never, maybe severe slips that are unstable.
212
Describe how a screw should be positioned for percutaneous in situ fixation of a SCFE
One cannulated partially threaded screw is usually sufficient.
Starts on anterior aspect of femur in order to cross perpendicular to physis.
Should be center center in epiphysis
5 threads accorss physis
Screw should be less than 5mm from subchondral bone.
213
What is the modified Dunn procedure?
Surgical hip dislocation with open capital realignment and fixation to correct the acute proximal femoral deformity in SCFEs.
214
What osteotomy is used to treat SCFE patients who go on to have restricted hip flexion motion, and external rotation deformity?
Imhauser osteotomy.
Flexion, internal roation, and valgus-producing proximal femoral osteotomy.
215
What is the cause of PFFD?
What are the associated conditions?
primarily sporadic
autosomal dominant form associated with sondic hedge-hog gene. WILL HAVE DYSMORPHIC FACIES
Fibular hemimelia (50%), ACL deficiency, coxa vara, and knee contractures
216
When can PFFD be treated with limb lengtheing with or without contralateral epiphysiodesis instead of some sort of amputation, rotationplasty, or arthrodesis and ablation?
Predicted limb length \< 20cm at maturity
Stable hip and functional foot
Femoral lenght \> 50% of opposite side
Femoral head present (Aitken classifications A&B)
Contraindictaions inculde unaddressed coxa vara, proximal femoral neck pseudoarthrosis, or acetabular dysplasia.
217
When can you consider performing a Van Ness rotationplasty vs Amputation and knee fusion in a patient with PFFD?
If foot is predicted to be at knee level can do a Van Ness rotationplasty with or without hip stabilization depending on codition of hip joint. Should be do early once child has reached one year of age. Do not perform if child \> 12 yrs.
If foot is predicted to be proximal to contralateral knee joint then syme amputation with knee fusion.
218
What is Legg-Calve-Perthes?
Idiopathic avascular necrosis of the proximal femoral epiphysis in children.
Treatment is generally observation when \< 8 yrs and femoral and/or pelvic osteotomy when \> 8 yrs.
219
What does symmetrical involvement of bilateral hips with perthes suggest?
Multiple epiphyseal dysplasia.
typically Perthes is asymmetrical asynchronous involvement.
220
What do up to 75% of patients with Legg-Calve-Perthes have some form of?
coagulopathy
thrombophilia present in up to 50% of patients
Possible association with Protein S and Protein C deficiencies.
ADHD associated in 33% of cases.
Bone age is delayed in 89% of patients.
221
What is the most important good prognositc indicator for children with Legg-Calve-Perthes?
Age \< 6 yrs at presentation.
Approximately half of patients develop premature ostoarthritis secondary to an aspherical head.
222
Describe the stages of Legg-Calves-Perthes
Waldenstrom
223
Describe the lateral Pillar (Herring) Classification of Legg-Calves-Perthes.
224
What is the Stulberg classification used for in Legg-Calves-Perthes?
Gold standard for rating residual femoral head deformity and joint congruence.
Recent studies show poor interobserver and introbserver reliability.
225
What are the classic physical exam signs of Legg-Calves-Perthes?
Painless limp
Insidious onset
Loss of internal rotation and abduction
Tendelenburg giat
226
What are the main goals of non-operative treatment of Legg-Calves-Perthes?
Keep the femoral head contianed and maintaing good motion.
Activity restricition, protected weight bearing, and physical therapy exercises.
60% do not require operative intervention.
Bracing and casting for containement have not been found to be helpful.
227
What two osteotomies may be indicated for Children \> 8 yrs of age with Lateral Pillar B or B/C Perthes hips?
Shelf osteotomy of the pelvis to prevent lateral subluxation and resultant lateral epiphyseal overgorwth
Abduction-extension osteotomy to reposition the hinge sement away from the acetabular margin, correct shortening from fixed adduction, and improve abductor mechanism by adding contractile length.
228
Children with what conditions can have congenital knee dislocations?
What are associated conditions?
myelomeningocele, arthrogryposis, and Larsen's syndrome
50% will have hip dysplasia
Clubfoot.
Metatarsus adductus
229
What is the treatment algorithim for a child with both a congential knee dislocation and a dislocated hip?
Treat knee first.
Then Pavlik harness for hip.
Cannot get patient into Pavlik harness until knee is no longer dislocated.
230
When do you consider surgical treatment for a congenital knee dislocation?
Knee that cannot be passively reduced beyond 30 degrees of flexion in a child that is \> 6months of age.
Failure to gain 30 degrees of flexion after 3 months of casting.
Goal of surgery is to obtain 90 degrees of flexion.
231
Treatment for a predicted leg length discrepancy of 2-5 CM
epiphysiodesis or other from of shortening of affected limb.
232
Treatment for LLD projected to be \>5cm but \< 20cm?
Lengthening of affected side with shortening of long side.
233
What are the principles of distraction osteogenesis?
Metaphyseal corticotomy to preserve medullary canal and blood supply
Wait 5-7 days then begin distraction
Distract 1mm/day
Following distraction keef fixator on for as many days as you lengthened. Lengthening over a nail has the advangtage that the ex-fix can be removed sooner.
234
Where is a popliteal cyst normally located in a child?
Betwen the semimembranosus and medial head of the gastrocnemius.
From herniated posterior knee joint capsule synovium.
Mass will transilluminate
Majority resolve spontaneously.
235
What are the risk factors for infantile Blount's disease?
Overweight children
Early walkers \<1 year
Hispanic and black
Best outcomes with early diagnosis and unloading of the medial joint.
236
Describe the process of physiologic genu varum.
Genu varum is normal in children less than 2 years.
Migrates to neutral around 14 months.
Peak genu valgum at 3 years of age.
Genu valgum migrates back to normal physiologic valgus at 7 years of age.
237
What conditions can be associated with pathologic genu varum?
Rickets
OI
MED, SED
Metaphyseal dysostosis (Schmidt, Jansen).
Focal fibrocartilaginous defect.
Thrombocytopenia absent radius.
Proximal tibial physeal injury.
GENU VARUM COMMONLY ASSOCIATED WITH INTERNAL TIBAIL TORSION.
238
What is the metaphyseal-diaphyseal angle of Drennan used for?
Used to assess Blounts
Angle formed by line connectedin metaphyseal beaks and a line perpendicular to the longitudinal axis of the tibia.
\> 16 degrees is considered abnormal. 95% Chance of progression.
\< 10 degrees has a 95% chance of natural resolution of the bowing.
239
When is brace treatment with a KAFO indicated for blounts?
Only in infantile blounts.
Stage I and II children \< 3 yrs
Bracing must continue for approximately 2 years for resolution of bony changes
Improved outcomes if unilateral. Poor results with obesity and bilaterality.
If working should see improvement within 1 year.
240
When is a proximal tibia/fibula vlagus osteotomy indicated?
Stage I and II children \> 3 years
Any child \>= 4 years
Stage III, IV, V, and VI
Failure of brace treatment
Metaphyseal-diaphyseal angle \> 20 degrees.
Perfrom osteotomy below tibia tubercle. Overcorrect into 10-15 degrees of valgus
Consider prophylactic anterior compartment release.
241
What orthopaedic conditions are associated with Neurofibromatosis?
Scoliosis.
Anterolateral bowing of the tibia.
Bowing of forearm leading to ulnar and radial pseudoarthrosis.
242
Which type of Neurofibromatosis is most common?
Which kind is associated with bilateral vestibular schwannomas?
NF1 most common. Known as Von Recklinghaussen disease.
NF2 is associated with bilateral vestibular schwannomas.
243
What physical exam findings are consistent with Neurofibromatosis?
Hemihypertrophy
Cafe-au-lait spots
Axillary freckling
Scoliosis
Anterolateral bowing and pseudoarthrosis of the tibia.
Dermal Plexiofrom (bag of worms, associated with multiple nerves instead of one) may be seen.
Lisch nodules- benign pigmented hamartomas of the iris.
Verrucous hyperplasia. Oral mucosa lesion.
244
What tumors are associated with neurofibroma?
Wilms tumor(nephroblastoma)
Neurofibroma (plexiform type): pathognomonic for NF1, only in 4% of patients. Can undergo malingnant transformation to neurofibrosarcoma.
245
What is dystrophic scoliosis?
When do you see it?
Typically thoracic kyphoscoliosis with a short segmented sharp curve involving 4-6 vertebrae?
Distorted ribs and vertebrae
Neurofibromatosis Type 1.
246
Anterolateral bowing is associated with what disease?
Neurofibromatosis
247
What is anteromedial tibial bowing associated with?
Fibular hemimelia
248
What is thought to be the cause of posteromedial bowing?
Abnormal intrauterine positioning.
Dorsiflexed foot pressed against anterior tibia.
Will develop leg length discrepancy and calcaneovalgus deformity.
249
Treatment for tibial pesudoarthrosis in a patient with neurofibromatosis.
Bone grafting with surgical fixation.
Total contact casting if anterolateral tibial bowing without pseudoarthrosis or fracture.
\< 4 yrs extend fixation to clacaneus
5-10 years extend fixation to Talus.
250
What is the most common congenital long bone deficiency?
Fibular deficiency.
Leads to anteromedial tibial bowing.
No known inheritance pattern.
Linked to sonic hedge-hog gene.
251
What conditions are associated with fibular deficiency(anteromedial tibial bowing)?
Ball and socked ankle.
Talipes equinovalgus
trasal coalition(50%)
Absent lateral rays.
PFFD and Coxa Vara
DDH
Genu valgum (secondary to lateral femoral condyle hypoplasia)
LLD
252
What is a Farmer's Procedure?
Use of a free vascularized fibular graft from the contralateral leg to treat tibial pseudarthrosis.
Placment of Lizarov or Taylor Spatial frame for bone lengthening.
253
If it comes to amputation for a patient with anterlateral tibial bowing and a pseudoarthrosis what amputation should be performed?
Sympe amputation.
Pseudoarthrosis is managed by the prosthetic socket.
Calf muscles are not as robust making BKA less desirable for coverage.
254
On average when does fragmentation of the proximal femoral epiphysis occur in patients with LCP?
6 months.
255
What is the most common associated condition with posteromedial tibial bowing?
Leg length discrepancy on average of 3-4 cm.
Posteromedial tibial bowing usually corrects over 5-7 years.
need to monitor, may require age appropriate epiphysiodesis.
256
What is the inheritance pattern of tibial deficiency?
What are the associated conditions.
autosomal dominant inheritance pattern.
ectrodactyly (cleft hand), preaxial polydactyly, and ulnar aplasia.
Treatment is determined by stability of knee joint.
257
What is a cause of anterolateral tibal bowing besides neurofibromatosis?
Tibial deficiency.
258
What are the three main causes of intoeing?
Femoral anteversion.
Metatarsus adducts (infants)
Internal tibial torsion (toddlers)
259
What is the demographics for femoral anteversion?
Pathophysiology?
Associated conditions?
Prognosis?
2:1 girls, 3-6 yrs of age, can be hereditary.
Packaging disorder. Often bilateral.
DDH, Metatarsus adducts, and congenital muscular torticollis.
260
What should you be concerned for in a 4 year old child that sits in the W position and has an awkward running style?
Femoral anterversion.
261
What tests should be performed to evaluate a child with intoeing.
Look at foot for any deformity (metatarsus adductus). Evaluate hindfoot and subtalar motion.
Tbial torsion: Thigh-foot angle in prone position. Infants 5 deg internal with range -30 to 20. Age 8 mean 10 deg ext. Range -5 to 30.
Femoral anteversion: hip motion in prone position. IR- Normal is 20-60. \>70 deg is abnormal. ER- Normal is 30-60. \< 20 deg is abnormal.
Trochanteric prominence angle test estimates IR when greater trochanter is most prominent.
262
What is the treatment for a 9 yo child with 5 degrees of external rotation?
Derotational femoral osteotomy.
Performed at the intertrochanteric level.
Amount of correction needed can be calculated by (IR-ER)/2
263
What is the prognostic difference between external tibial torsion and internal tibial torsion?
External tibial torsion may actually cause disability and degrade physical performance.
264
What is miserable malalignment syndrome?
Combination of external tibial torsion with femoral anteversion.
265
What coditions are associated with external tibial torsion?
Miserable malaignment syndrome?
Osgood-Schlatter disease
Osteochondritis dessicans
Early degenerative joint disease
Neuromuscular conditions such as myelodysplasia and polio.
266
What is the operative indication for external tibial torsion?
When is it indicated?
Supramalleolar rotational osteotomy.
Proximal tibial osteotomies are avoided secondary to higher risk factors associated with this procedure.
Children older than 8 years of age with \> 40 degrees of external rotation.
267
What is the difference between metatarsus adductus and skew foot?
Metatarsus adductus is adduction of forefoot with normal hindfoot alignment.
Skew foot (also known as serpentine foot) has tarsometatarsal adductus with abnormal hindfoot alignment: talonavicular lateral subluxation and hindfoot valgus.
Nonoperative treatment for skew foot is usually ineffective.
268
What is the prognosis for metatarsus adductus?
Most resolve spontaneoulsy.
Require parent manipulation and at most casting. Only exceptsion is if it is a rigid deformity.
No longterm pain or decreased function.
269
What is an atavistic great toe?
Congenital hallux varus.
270
Treatment for metatarsus adducts?
Operative treatment indicated in \> 5 yr olds who fail non-op reatement, have difficult with shoe wear, and pain.
Perform lateral column shortening with cuboid closing wedge osteotomy and medial column lengthening with cuneiform opening wedge osteotomy, medial capsular release, and abductor hallucis longus recession for atavistic first toe.
271
What is the most common type of hereditary motor-sensory neuropathy?
Charcot-Marie-Tooth
Characterized by genetic mutations in myeling proteins that lead to leg muscle atrophy and loss of sensation and proprioception in early adulthood.
Most commonly inherited autosomal dominant but can be recessive or X-linked.
272
What muscles are affected in Charcot-Marie-Tooth?
**Peroneus brevis**-usually first affected and most profound. Leads to imbalance and varus deformity.
**Tibialis anterior**-weakness leading to drop foot.
**Intrinsic muscles of the hand and foot-** check for wasting of 1st dorsal interossei in hands
273
What are the orthopaedic manifestations of Charcot-Marie-Tooth?
Pes cavovarus
Hammer toes
Hip dysplasia
Scoliosis
Hand muscle atrophy and weakness
274
What is the difference between Type 1 and Type 2 Charcot-Marie-Tooth?
275
What causes the cavovarus foot in Charcot-Marie-Tooth?
Weak tibialis anterior is overpowered by unaffected peroneus longus.
Leads to plantar flexion of the first ray.
276
What will be found on EMG of a patient with Charcot-Marie-Tooth?
Low nerve conduction velocities
Prolonged distal latencies
found in peroneal, ulnar, and median nerves
Can also see low amplitude nerve potentials due to axonal loss.
277
What causes the varus positioning of the foot in Charcot-Marie-Tooth disease?
A normal tibialis posterior overpowering weak peroneus brevis.
278
What soft tissue reconstruction procedures are indicated in Charcot-Marie-Tooth disease for patients with:
Cavus
Plantar Flexed first ray
Dorsifelxion weakness leading to drop foot
Ankle equinous
Toe clawing
Plantar release.
Peroneus longus to brevis- decreases plantarflexion force on the first ray without weakening eversion.
Posterior tibial tendon transfer to dorsum of foot.
Gaxtrocnemius recession vs TAL. Gastroc recession preferred.
Jones transfer(s) EHL to neck of 1st MT and lesser toe extensors to 2nd-5th MT necks
279
How often does scoliosis occur in children with CMT?
What is the characteristic curve?
Treatment?
10-20% of the time.
Left thoracic and kyphotic curve.
Bracing not effective. Progressive deformity \> 50 degrees = fusion and isnstrumentation.
280
If you have a unilateral cavovarus foot in a child what must be ruled out?
Tethered spinal cord or spinal cord tumor.
281
What conditions may present with a cavovarus foot?
CMT Disease
Cerebral Palsy
Freidreich's Ataxia
Spinal cord lesions
Polio
282
What will be some of the presenting history and complaints for a patient with a cavovarus foot?
Lateral ankle pain and recurrent ankle sprains.
Lateral foot pain, can lead to fifth metatarsal stress fractures.
Plantar calluses under 1st and 5th metatarsal heads.
Plantar fasciitis
283
What is signified by a Meary's Angle \> 4 degrees?
Meary's angle is the talo-first metatarsal angle on a lateral foot x-ray.
When it is \> 4 degrees apex dorsal it is consistent with a cavovarus foot.
Apex plantar associated with pes plano valgus.
284
What age do you expect calcaneonavicular coalition?
Talonavicular coalition?
8-12 years.
12-15 years.
Calcaneonavicular is most common.
285
What deformities can you see with a coalition?
Flattening of longitudianl arch
Abduction of forefoot
Valgus hindfoot
Peroneal spasticity
286
How is nonsyndromic tarsal coalition inherited?
Autosomal dominant
287
Associated conditions with tarsal coalition?
Fibular hemimelia
carpal coalition
FGFR-associated cronaiosynostosis (FGFR-1, 2, and 3)
Apert Syndrome
Pfeiffer Syndrome
Crouzon Syndrome
Jackson-Weiss Syndrome
Muenke Syndrome
288
What is first line treatment for tarsal coalitions?
Immobilization with casting and analgesics.
6 weeks
30% of symptomatic patients will become pain free.
Shoe inserts may actually cause more discomfort because the defomrity is rigid.
289
What is a major indication for or against coalition resection that is also used as a prognostic indicator?
The amount of joint that the coalition involves.
\>50% found to have poor outcomes. Considere arthrodesis in this case
Resection better if \< 50%
290
When is a triple arthrodesis indicated for a pediatric patient with tarsal coalition?
Failed resection.
Degenerative changes in other joints.
Multiple coalitions.
Greater than 50% involvement of posterior facet of subtalar joint.
Child needs to be older than 12 to prevent limitatin on foot growth.
291
Describe a flexible pes plano valgus?
collapse of medial longitudinal arch
Hindfoot valgus
Forefoot abduction
Corrects when rising onto toes.
292
Treatment for a flexible pes planovalgus.
Observation. Almost always resolves spontaneously, especially in asymptomatic patients. Arch develops with age.
Strethcing, shoewear modifications, and orthotics.
Important to understand that orthotics do not change natural history of the diesease.
293
Surgical treatment for flexible pes planovalgus in pediatric patient.
Calcaneal Lengthening Osteotomy(Evans procedure)
With or without cuneiform osteotomy.
Possible peroneal tendon lengthening.
Sliding calcaneal osteotomy can correct the hindfoot valgus.
Plantar based closing wedge osteotomy of the first cuneiform corrects the supination deformity.
294
What percent of congenital vertical talus is associated with a neuromuscular disease or chromosomal aberration?
50%
Myelomeningocele
Arthrogyrposis
Diastematomyelia
Congenital dislocation of the hip
Cerebral palsy
Spinal Muscular Artrophy
Hip dysplasia
50% Bilateral. 2:1 M:F
295
What is the pathoanatomy of congenital vertical talus?
**Rigid foot deformity**: irreducible dorsolateral navicular dislocation, vertically oriented talus, and calcaneal eversion with attenuated spring ligament.
**Soft tissue contractures**: Displacement of peroneal longus and posterior tibialis tendon so they function as dorsiflexors rather than plantar flexors. Contracture of achilles tendon.
296
What is Kohler's Disease?
Who does it commonly occur in?
Avascular necrosis of the navicular bone.
Children 4-7 yrs.
4:1 Boys to Girls.
Central 1/3 of the navicular is a watershed zone. Makes it susceptbile to injury.
Last bone in the foot to ossify.
Self-limited after 1-3 years.
297
What is the recommended treatment for Kohler disease?
Always non-operative management.
Immobilization with short leg walking cast.
298
What is Sever's disease?
In what population is it seen?
Overuse injury of the calcaneal apophysis.
Immature athletes participating in running and jumping sports.
Just before or during peak growth.
self-limiting entity.
299
What is the recommended treatment for Sever's Disease?
Activity modification.
Achilles tendon stretches.
Ice.
Heel cups or heel pads.
NSAIDs.
Short leg cast immobilization if persistent.
**Recurrence is common but surgery is never the answer on the test.**
300
What is the most common musculoskeletal birth defect?
Clubfoot
Congenital talipes equinovarus
1:1000
Highest prevalence in Hawaiians and Maoris
80% clubfoot is an isolated deformity.
301
What contractures contribute to the characteristic deformity in clubfoot?
CAVE
Cavus: tight intrinsics, FHL, and FDL
Adductus of forefoot: tight tibialis posterior
Varus: tight tendoachilles, tibialis posterior, tibialis anterior
Equinus: tight tendoachilles
302
What is the boney deformity of clubfoot?
Talar neck is medially and plantarly deviated.
Calcaneus is in varus and rotated medially around talus.
Navicular and cuboid are displaced medially.
303
What abnormality of the anterior tibial artery is common regardless of etiology of clubfoot?
Hypoplasia or absence.
304
What conditions can be associated with clubfoot?
Arthrogryposis
Diastrophic dysplasia
Myelodysplasia
Tibial hemimelia
Amniotic band syndome (Streeter dysplasia)
Pierre Robin Syndrome, Opitz syndrome, Larsen syndrome, and prune-belly syndrome
305
What do you see on physical exam with a clubfoot besides the foot deformities?
Small calf
Shortened tibia
Medial and posterior foot skin creases.
306
What relationship is seen between the talus and the calcaneus in clubfoot?
Hindfoot parallelism.
Talus and calcaneus are less divergent than normal.
Talocalcaneal angle \<25 degrees on a dorsiflexion lateral (Turco view).
307
What is the success rate of the Ponseti method?
What should you counsel parents about the childs lifetime limitations?
90% success in avoiding comprehensive surgical rlease.
Children can be expected to walk, run, and be fully active in the absence of other comorbidities.
308
When is surgical intervention necessary in club feet?
80-90% will need a heel cord tenotomy.
Resistant or recurrent club feet who have failed Ponseti casting and bracing.
Posteromedial soft tissue release and tendon lengthening. Stiffness and pain is common.
Extent of soft tissue release correlates with long-term function.
Should be done at 9-10 months of age in non-syndromic feet so walking is not delayed.
309
How should a FAO (foot abduction orthosis be used in club feet?
Full time for 3 months then at night (+/-) naps for 2-4 years.
FAO noncompliance is biggest risk factor for deformity recurrence.
310
How should dynamic supination be treated in a child who has been treated for club foot?
Can do whole or split tibialis tendon transfer.
Whole tendon transfer seems to be the preferred answer choice for OITE.
Tib Ant should be transferred to lateral cuneiform.
Required 30-50% of the time
Should be perfomred at 2-5 yrs.
311
Describe the Ponseti method.
Correct in order of CAVE deformity.
312
What foot deformity is associated with a dorsal bunion of the 1st MTP that alos has a muscular strength imbalance between anterior tibialis and peroneus longus?
Congenital talipes equinovarus.
Weak peroneus longus with stronger flexor hallucis and anterior tibial tendon lead to a dorsiflexed first metatarsal.
313
What is the inheritance pattern of polydactyly of the foot?
Autosomal dominant
1:500 births.
314
When is operative treatment for a congenital curly toe indicated?
Rarely
Severe cases with nail deformity.
Child should be \> 3 yrs old.
Tenotomy as effective as transfer.
315
What is another name for an epiphyseal bracket?
Delta phalanx
Aberrant cartilage extending along the diaphysis interfers with normal longitudinal growth.
Found in short tubular bones. 11% found in the great toe.
Leads to a short wide triangular or trapezoidal phalanx.
316
How is a Delta phalanx best treated?
Excision of the bracketed epiphysis with fat graft interposition.
Early intervention leads to more chance for angular correction and longitudinal growth.
317
What is cerebral palsy?
Nonprogressive motor neuron disease (static encephalopathy) due to injury to the immature brain.
By definition should be diagnosed before 2 years of age.
Most common cause of chronic childhood disability.
2-3:1000 live births.
318
What are the orthopaedic manifestations of cerebral palsy?
Fractures
Contractures
Upper extremity deformities
Hip subluxation and dislocation
Spinal deformity
Foot deformities
Gait disorders
319
When do you consider using pamidronate in children with cerebral palsy?
\> or = 3 fractures with a DEXA Z-score \< 2 SD
320
True or False a pre-operative MRI is performed before Scoliosis surgery in all patients with Cerebral Palsy?
False, the indications for MRI are the same as other scoliosis patients.
321
Describe the three classifications systems for cerebral palsy.
Physiologic
Anatomic
Gross Motor Function Classification Scale (GMFCS)
322
What percentage of children with CP get progressive hip subluxation?
50% off patients with spastic quadripalegic CP.
323
Which way does dislocation occur in a child with CP?
posterior and superior \>95% of the time.
324
What is Reimers migration index?
Percent of femoral head with no acetabular coverage.
**Most accurate method to identify and monitor hip stability.**
\<33% = at risk
\>33% = subluxated hip.
325
When should a girdlestone procedure be performed for a child with CP who has a chronically dislocated hip?
Never.
No longer performed because it uniformly causes pain.
326
What are the salvage technique(s) for symptomatic and chronically dislocated hips in cerebral palsy?
**Vagus support osteotomy**- femoral head resection + valgus subtrochanteric femoral osteotomy. Called the McHale technique.
**Castle resection-interposition arthroplasty-** Proximal femur is ressected at the level of the lesser trochanter so there are no remaining muscle attatchement to lead to further deformity. Oversew abductors, psoas, and hip capsule over acetabulum.
**Total hip arthroplasty** is an option in ambulatory patients.
327
What are the operative soft tissue and reconstructive procedures and associated indications for a subluxated hip in a child with Cerebral Palsy?
328
When performing a hip adductor tenotomy what nerve must you watch out for?
What complication does neurectomy lead to?
Obturator nerve.
Hip Abduction contracture.
329
What is the point of Single-Event Multi-Level Surgery (SEMLS) in children with cerebral palsy?
Addresses the multiple planes and levels of deformity during a single surgery to avoid annula suergeries and prolonged bouts of recovery required after each surgical session.
330
What is the difference between primary and secondary deviations in children with CP and a gait deformity?
**Primary deviations-** Those caused by the primary CNS insult: spasticity, weakness, compromised proprioceptive pathways
**Secondary deviations-** Growth related deviations that arise due to abnormal loading in the setting of primary gait deviations: Muscle contractures, bony deformities, and joint subluxations or deviations.
331
What is the difference between qualitative and quantitative gait analysis?
Which is better?
Qualitative is descriptive, tries to simplify and classify. Often unsuccessful.
Quantitative- Uses technology to characterize the gait in all three planes of the deformity.
**Quantitative is more accurate.**
332
What is pedobarography?
Special force plate that shows contact pressures through the stance phase.
333
What role does chemodenervation play in cerebral palsy with regards to gait?
Use of botulinum neruotoxin A may be used to temporize certain muscle groups in order to delay surgical management.
Can also be used as a primary treatment modality.
**Doesn't work for fixed deformity.**
334
When can you consider percutaneous heel cord lengthening in children with CP and equinous contracture?
Should be delayed until the patient is at least 6 years old to prevent recurrence.
335
What is a stiff knee gait?
What is the recommended treatment?
Common in spastic diplegic CP.
Characterized by limited knee flexion in swing phase due to rectus femoris firing out of phase (seen on EMG)
**Rectus transfer**- tranfer it posterior to the center of rotation of the knee so that rectus activation creates a knee flexion vector.
336
In children with CP what procedure would you recommend for the following:
4 degree knee flexion deformity?
20 degree knee flexion deformity in a 11 y/o girl?
30 degree knee flexion deformity in a 15 y/o boy?
**Medial hamstring lengthening**- Fractional lengthening at the myotendinous junction. Not uncommon for contractures to recur.
**Guided growth surgery**- two years of growth remaining 10-25 degree deformity.
**Supracondylar femur extension osteotomy +/- patellar tendon advancement or shortening**- 10-30 degree deformity with severe quadriceps lag in patients close to skeletal maturity
337
What is a normal popliteal angle?
25 degrees.
338
What is the most common foot deformity in cerebral palsy?
Equinous contracture.
339
For what pediatric foot deformity would you use a Evan's lateral calcaneal lengthening osteotomy?
Flexible Pes Planovalgus.
340
What is the most common associated congenital abnormality with spina bifida?
Type II Arnold-Chiari Malformation.
70% also have hydrocephalus
341
What potential allergic complication should you be concerned about in a patient with myelomeningocele?
Latex Allergy
Type 1 IgE mediated hypersenitivity.
Should be distinguished from other latex allergies which is a Type 4 hypersensitivity or contact dermatitis that is T cell mediated.
342
What is the difference between spinal bifida oculta, meningocele, and myelomeningocele?
Defect in vertegral arch with confined cord and meninges
Protruding sac without neural elements
Protruding sac with neural elements.
343
What lab study can be performed to screen for spina bifida?
Alpha-fetoprotein
Elevated in 75% of children with open spina bifida.
Obtain during second trimester.
344
What is often confused with an infectious process in children with cerebral palsy?
What is the recommended treatment?
Fractures of long bones due to osteopenia.
Higher frequency the higher the level of the defect. Common in the hip and the knee in children 3-7 years of age.
Short perior of immobilization in a well padded cast for fracture in acceptable alignment. Avoid long term immobilication which can lead to osteopenia and repeat fractures.
345
How should a child's hip with spina bifida and a dislocated hip be treated?
OBservation.
Surgical treatment is highly controversial due to a high rate of failure.
346
When is a hip flexion contracture considered operatvie in a child with spina bifida?
What procedure is recommended?
\> 40 degrees.
Anterior release and tenotomy of iliopsoas, sartorius, rectus femoris, and tensor fascia lata.
347
What is the most common foot deformity seen with spina bifida?
How is it different from the idiopathic version?
talipes equinovarus 30% of the time.
Very rigid and insensate. Serial casting still treatment of choice but there is a high complication rate.
348
What is sacral agenesis highly associated with?
How does it differ from myelodysplasia?
Maternal diabetes
Protective sensation is usually intact so there is a lesser rate of decubitus ulcers. Even though this is the case they still can have sensory deficits.
349
What is an expected outcome of untreated complete sacral ageneiss?
Progressive kyphosis.
Child is unable to support the trunk without using his hands for support.
Should be treated with a spinal stabilization procedure.
350
What disease is charaterized by lesions in dorsal root ganglia, corticospinal tracts, dentate nuclei in the cerebellum and sensory peripheral nerves?
Friedreich's Ataxia
Most common form of spinocerebellar degenerative disease.
Onset between 7-15 yrs. Age of onset related to number of GAA repeats.
351
What conditions are associated with Friedrich's Ataxia?
Pes cavovarus foot
Scoliosis. Guaranteed if onset of disease is before 10. Progression of scoliosis if onset is before 15.
Cardiomyopathy. Coenzyme Q and other antioxidants have been shown to decrease rate of cardiac deterioration but they do not effect ataxia.
352
How would you describe the gait of someone with Friedrich's Ataxia?
staggering wide base gait.
353
What physical exam findings can you find with a patient with Friedrich's Ataxia?
Classic triad: Ataxia, areflexia, and positive plantar response
Weakness
Nystagmus
Scoliosis
Cavovarus food: rigid and associated claw toes.
354
What will and EMG show in a patient with Friedrich's Ataxia?
defects in motor and sensory.
increase in polyphasic potentials.
conduction velocities are decreased in upper extremities.
355
What trearment is recommended for patients with Friedrich's Ataxia and a cavovarus foot?
Observation if non-ambulatory. Bracing and stretching not helpful because it is rigid.
Early dieseas in ambulatory patient: plantar release, tranfers, +/- metatarsal and calcaneal osteotomy.
Late disease in nonambulatory who have issues with tranfers can consider triple arthrodesis.
356
What disease is consistent with a child that has adducted and internally rotated shoulders. No flexion creases in the elbows. Hips flexed, abducted, and externally rotated. Normal intelligence, facies, sensation, and viscera?
Arthrogryposis
357
What orthopaedic manifestations are seen in Arthrogryposis?
Upper extremity deformity: absence of shoulder muscles, thin limbs, elbows extended, wrists flexed and ulnarly deviated, intrinsic plus deformity, adducted no thumbs, and no flexion crease at the elbow.
Teratologic hip subluxation/dislocation.
Knee contractures
Clubfoot or vertical talus
C-Shaped scoliosis(33%)
Higher rate of fractures.
358
What studies should be performed at three months of age in a baby diagnoses with arthrogryposis?
Neurologic studies
Enzyme tests
Muscle biopsies
359
What treatment is recommended for upper extremity deformity in arthrogryposis?
First line of treatment is passive manipulation and serial casting.
Operative should be considered after the age of 4. Includes soft tissue releases, tendond transfers, and osteotomies.
360
How should a child with a irreducible teratalogic hip dislocation and a 40 degree knee flexion contracture be treated?
Hamstring release of the knee, best performed early at 6-9 months. Should be done before the knee.
Only way to reduce teratologic hip dislocations is with an open reduction and likely a femoral shortening osteotomy.
361
At what ages do you d a medial approach for an open hip reduction?
Anterior approach?
up to 12 months.
After 12 months.
362
What is dolichostenomelia?
Long narrow limbs.
Arm span is greater than height.
363
What orthopaedic conditions are associated with Marfan Syndrome?
Arachnodactyly(long slender digits)
Scoliosis (50%)
Protrusio acetabuli (15-25%)
Ligamentous laxity -\> recurrent dislocations
Pes planovalugs
Dural ectasia (60%)
Meningocele
Pectus excavatum or carinatum
364
What non-orthopaedic conditions are associated with Marfan Syndrome?
Cardiac: aortic root dilation, aortic dissection, and mitral valve proplapse.
Superior lens dislocations (60%)
Spontaneous pneumothorax
Skin striae and recurrent hernias.
365
What is the difference between and AFO and a PLSO?
AFO controls the foot in both stance and swing phase.
PLSOControls excessive ankle plantar flexion in the sing phase but doesn't control anything in stance phase and allows for ankle forsiflexion.
366
What is Larsen's Syndrome?
rare genetic disorder
characterized by findings of:
ligamentous hyperlaxity
Abnormal facial features (flattened nasal bridge, hypertelorism, and prominent forehead).
Multiple joint dislocations.
367
What are the orthopaedic manifestations of Larsen's Syndrome?
Hand deformities
Scoliosis
Clubfeet
Cervical kyphosis: May present with extremity weakness secondary to myelopathy. Caused by hypoplasia of the cervical vertebrae. **Need to obtain screening radiographs to avoid catastrophic complications.**
Joint locations often include knees and radial heads that are bilateral.
368
How should cervical kyphosis but no neurologic defects be treated in a 14 month old with Larsen's Syndrome?
Posterio cervical fusion.
With neurolgic defects should be treated with anterior/posterior cervical decompression and fusion.
369
How are hip dislocations in Larsen's syndrome treated?
Can attempt closed reduction but only once. Rarely succesful.
Usually need open reduction but even this is controversial in bilateral dislocations. If being performed should be performed early and only once.
370
How are knee dislocations in Larsen's syndrome treated?
Closed reduction and casting can be attempted. Often not succesful
Open reduction with femoral shortening and collateral ligament excision when they remain unstable after closed reduction.
371
What is the most common genetic disease resulting in death during childhood?
spinal muscular atrophy
Progressive weakness starts proximally and moves distally.
372
What is the pathophysiology of spinal muscular atrophy?
Progressive loss of alpha-motor neurons in anterior horn of spinal cord.
373
What is the inheritance of spinal muscular atrophy?
autosomal recessive
374
What is the classification for spinal muscular atrophy?
375
What is one way you can distinguish Duchenne's Muscual Dystrophy from Spinal Muscular Atrophy on physical exam?
Presence or absence of deep tendon reflexes.
Duchenne's they are present while they absent in SMA.
Tongue fasciculations present in SMA.
376
How is SMA diagnosed?
What medication has been FDA approved for treatment?
DNA analysis and muscle biopsy.
**Nusinersen** administered intra-thecally.
377
What are the orthopaedic manifestations of SMA?
Hip dislocations- treated with observation alone. Typically painless and there is a high rate of recurrence.
Scoliosis
Lower extremity contractures.
378
What is the rate of scoliosis in SMA?
When does it occur?
Does it typically progress?
almost everyone
By 2-3 years
Often progressive.
379
What is the treatment a patient with Scoliosis and SMA?
Bracing can be used will delay but not prevent surgery in children younger than 10 years.
Operative is PSF with fusion to pelvis.
Need to address any hip contractures first.
Keep a laminectomy area free of fusion for administration of NUsinersen.
If curve \> 100 degrees. PSF with anterior release and fusion.
Curves are typically very flexible.
380
How should hip, knee, and ankle contracture typically be treated in children with SMA.
Physical therapy.
Surgical release usually not ideal given function is not improved in non-walkers and recurrence is common.
381
What disease affects young males only and begins between 2-6years of age?
Duchenne Muscular Dystorphy
X-linked recessive.
382
What is the treatment for Duchenne Muscular Dystrophy?
Prednisone .75mg/kg/day for a 5-7 year-old child with progressive disease.
Significant positive effect on disease progression: Delays deterioration of pulmonary function. Acutely imporves strength, slows progressive weakening, prevents scoliosis, and prolongs ambulation.
Pulmonary care with nightly ventilation.
383
What are the side effects of medical treatment for Duchenne's?
Osteonecrosis
Weight gain
Cushingoid appearance
GI symptoms
Mood lability
Headaches
Short stature
Cataracts
384
What is the common foot deformity seen with Duchenne?
What is the treatment?
Equinovarus
Stretching, PT, and night time AFO.
If not helping operative: tendinoachilles lengthening with posterior tibialis tendon transfer and toe flexor tenotomies.
385
What are the associated medical conditions with Achondroplasia?
Weight control problems
Hearing loss
Tonsillar hypertrophy
Frequent otitis media
386
What features can you see on physical exam for a patient with achondroplasia?
Rhizomelic dwarfism ( Shorter Humerus and Femur)
Frontal bossin
Trident hands
Genu varum
Radial head subluxation
Muscular hypotonia in infancy
Thoracolumbar kyphosis and excessive lordosis.
387
An imaging a patient has a champagne glass pelvis, squared iliac wings, and inverted V in distal femur physis. What disease do they have?
Achondroplasia
388
When might you consider upper extremity lengtheing in a child with Achondroplasia?
If it is necessary to maintian ADL's.
389
What is the treatment for genu varum in a patient with Achondroplasia?
If having pain, fibular thrust, and progressive deformity then tibia +/- femoral osteotomy.
Based on CORA
390
What is Multiple Epiphyseal Dysplasia?
Form of dwarfism characterized by irregular delayed ossification at multiple epiphyses.
Caused by failure of formation of secondary ossification center (epiphysis).
Spectrum of disorders with a spectrum of phenotypes.
Presents between age 5-14 years.
391
What is most commonly affected in MED?
Proximal humerus
Proximal femur
392
What is a form of short limbed disproportionate dwarfism?
MED
393
A patient has the following physical exam fingings, what is the disease process?
Normal facies, spine, intelligence, and neurologic exam.
Valgus knee, short stubb fingers and toes, and some joint contractures.
MED
394
What will radiographs of a patient with MED show?
Irregular delayed ossification at multiple epiphyses
Bilateral proximal femoral epiphyseal defects
Valgus knee with flattened femoral condyles and double layer patella.
Short stunted metacarpals
Short metatarsals.
395
How can you differentiate MED from spondyloepiphyseal dysplasia?
Spondyloepiphyseal dysplasia involves the following:
Involves the spine.
Has a sharp curve.
Atlantoaxial instability and cervical myelopathy.
This is a mutation in type II collagen.
396
How do you differenitate Perthes from MED?
MED will have symmetric bilatera presentation with early acetabular changes and lack of metaphyseal cysts.
Can perform skeletal survery.
397
What is usually the treatment for MED?
Physical therapy.
NSAIDS for early OA
**Childhood hip deformities usually resolve by skeletal maturity.**
Occassionally with do realigning osteotomy or hemiepiphysiodesis at the knee for progressive genu valgum.
398
Where is diastrophic dysplasia most common?
Finland.
399
What phsical exam findings and features are consistent with diastrophic dysplasia?
Rhizomelic shortening
Cleft palate 60%
Cauliflower ears 80%
Hitchhikers thumb
Thoracolumbar scoliosis
Severe cervical kyphosis
Genu Valgum
Skewfoot
Rigid clubfeet
400
What disease should be expected in a patient with hypoplastic or absent clavicles?
Cleidocranial dysplasia (dysostosis)
Rare 1 in 1,000,000
Failure of intramembranous ossification
401
What are the orthopaedic manifestations of Cleidocranial dysplasia?
**propoertionate dwarfism**
clavicle dysplasia/aplasia
wormian bones
Frontal bossing
Delayed fontanelle ossification
coxa vara
shortened middle phalanges of 3-5 fingers
delayed ossification of pubis
delayed eruption of permanent teeth
402
What is usually the only orthopaedic operative procedure performed on patients with cleidocranial dysplasia?
Coxa vara with a neck shaft angle of less than 100 degrees.
403
What are the orthopaedic manifestations of mucopolysaccharidoses?
Porportionate dwarfism
Increased rate of carpal tunnel syndrome
C1-C2 instability
Delayed hip dysplasia
Abnormal epiphyses
Bullet shaped phalanges
Genu Valgum
404
Nonorthopaedic conditions associated with Mucopolysaccharidoses?
Complex sugars in the urine
visceromegaly
Corneal clouding
Cardiac disease
Deafness
mental retardation (**Except Morquio syndrome**)
Enlarged skull
405
What role does bone marrow transplant play in mucopolysaccharidoses?
Improved life expectancy.
Doesn't alter orthopaedic manifestations.
406
How do you differentiate Morquio, Hurler, San Filippo, and Hunter syndrome?
**Morquio**: Odontoid hypoplasia that leads to cervical instability. Obtain flexion-etension x-rays.
**Hurler:** Gargolylism, finger triggering
Morquio and Hurler have corneal clouding the other two have clear corneas.
407
What are the orthopaedic manifestations of OI?
ligamentous laxity
short stature
scoliosis
**codfish vertebrae**
basilar invagination
Olecranon apophyseal avulsion fx
Coxa Vara
**Congenital anterolateral radial head dislocations.**
408
What are the non-orthopaedic manifestations of OI?
Blue sclera
Dysmorphic triangle shaped facies
Hearing loss 50%
Dentinogenesis imperfecta
Vormian skull bones
Hypermetabolism: **Increased risk of hyperthermia,** hyperhidrosis, tachycardia, tachypnea, and heat intolerance
Think skin prone to subcutaneous hemorrhage
**Mitral valve prolapse and aortic regurgitation.**
409
What leads to saber shin?
Multiple fractures of the tibia in children with Osteogenesis imperfecta.
410
What can lead to apnea, altered consciousness, ataxia, and myelopathy.
Basilar invagination.
Usually in the third or fourth decade of life. However, it can present as early as the teenage years.
411
What are some important considerations regrading bisphosphonate use and osteogenesis imperfecta?
Indicated in most cases of OI to reduce fractrue rate, pain, and improve amublation.
Does not affect development of scoliosis
**Need to maintain bisphosphonate free period around the time if IM rodding**
412
How would you treat the following fractures in a child with OI?
18 month old with displaced both bone forearm fracture?
6 year old with displaced both bone forearm fracture?
16 year old with displaced both bone forearm fracture?
Reduction and casting: Fractures in children under 2 should be treated the same as children without OI.
Telescoping IM rods
IM rods, even in teenages who are no longer growing. Too high a risk of refracture with plates.
413
Macrocephaly, hepatosplenomegaly, and osteomyelitis of the mandible or dental abscesses would be characteristic of what disease process?
Osteopetrosis. Autosomal recessive form. This form has more frequent fractures and overall worse prognosis.
Autosomal dominant form is usually asymptomatic. Patients usually first learn about the disease after fracture.
414
How should fractures in a patient with osteopetrosis be managed?
For all but proximal femur fractures usually prolonged casting and non-weight bearing. There is increased risk of malunion and refracture. Exhibit delayed healing.
Proximal femur fracture. Use plate and screws. Avoid IMN. Slow steady drilling with constant cooling and changing of the drillbit.
415
What is the medical management for osteopetrosis?
Autosomal recessive: Bone marrow transplant and high dose calcitriol (1,25 dihydroxy vitamin D).
Autosomal Dominant: Interferon gamma-1beta
416
What medical conditions are associated with Down Syndrome?
Mental Retardation
Cardiac disease (50%)
Endocrine disorders(hypothyroidism)
Premature aging
Duodenal atresia
Hypothyroidism
Alzheimer's disease
417
What are the orthopaedic manifestations of down syndrome?
Generalized ligamentous laxity and hypotonia
Short stature
C1-C2 instability
Occipitocervical instability
Delayed motor milestones (Walk at 2-3 years of age)
Hip subluxation and dislocation
Patellofemoral instability and dislocation
Scoliosis and psondylolisthesis
Pes planus, metatarsus primus varus
SCFE
418
What physical exam findings are often found in down syndrome?
Flattened facies
Upward slanting eyes
Epicanthal folds
Single palmar crease (simian crease)
Ligamentous laxity
Scoliosis
419
How often and when does hip instability occur for children with down syndrome?
5%
occurs between age 2-10 years
Young with no bony changes or dislocation -\> Abduction bracing
Older or symptomatic -\> Capsulorrhaphy and pelvic and fremoral varus osteotomies.
420
What foot deformity is seen in 50% of patients with down syndrome?
How should it be treated?
Pes planus or Planovalgus
Orthotics if symptomatic. Rarely but if refractory to orthotics then surgical correction.
Can also see Metatarsus primus varus and Hallux valgus (25%)
421
What is the diagnostic criteria for JIA?
**Must rule out infection.**
Must include one of the following: Rash, presence of RF, iridocyclitis, c-spine involvement, pericarditis, tenosynovitis, intermittent fever, or morning stiffness.
Inflammatory arthritis lasting \> 6 weeks in a patient younger than 16 years of age.
422
What is the prognosis of JIA?
50% patients symptoms resolve without sequelae
25% are slightly disabled
25% have crippling arthritis or blindness
Best prognosis pauciarticular \> polyarticular \> systemic
423
What radiographs should be obtained in patients with JIA?
Flexion-extension neck radiographs to rule out atlantoaxial instability.
424
Which leg is typically longer in JIA?
The affected leg is typically longer.
Seen in oligoarticular disease
Can consider epiphysiodesis
425
What treatment is recommended for JIA?
DMARDs and frequent opthalmologic exams.
High dose apsirin and NSAIDs are used less frequently.
intra-articular steroid injections.
Slit-lamp examination twice yearly if ANA (-), every 4 months if ANA (+)
**Eye involvement can be indolent and lead to blindness.**
426
What work-up should all patients with Ehlers Danlos get?
Cardiac evaluation with Echo.
1/3 of patients have aortic root dilatation.
427
What can be considered in a patient with joint pain and instability who has Ehler's Danlos Syndrome?
Joint arthrodesis.
Soft tissue procedures are unlikely to be succesful in hypermobile joints.
428
What constitutes hypermobility on the Beighton-Horan Scale?
5 or more points. Out of a 9 point scale.
Passive hyperextension of each small finger \>90 deg 1 point each
Passive abduction of each thumb to the surface of the forearm 1 point each
Hyperextenion of each knee \> 10 deg 1 point each
Hyperextension of each elbow \> 10 deg 1 pint each
Forward flexion of trunk with palms on floor and knees fully extended 1 point.
429
What are the orthopaedic manifestations of hemophilia?
Arthropathy- synovitis, cartilage destruction, joint deformity, and pseudotumor.
Pseudotumor is a intramuscular hematoma.
Iliacus hematoma may compress femoral nerve. Presents with paresthesias in the L4 distribution.
Leg length discrepancy, fractures, and compartment syndrome.
430
What is Jordan's sign?
squaring of patella and femoral condyles in hemophiliacs.
431
How is a synovectomy performed in a patient with hemophilia?
First radioactive synoviorthesis (destruction of synovial tissue with intra-articular injection of radioactive agent)
Followed by surgical synovectomy.
Show to reduce recurrence.
432
What is the most common cause of femur fracture in an infant?
Nonaccidental trauma.
433
What percent of fractures due to non-accidental trauma occur in children younger than 5 years of age?
90%
434
What is the most common cause of death in children?
What is the second most common cause of death?
Accidental injury.
Abuse.
435
What are high specificity fractures for pediatric abuse?
Metaphyseal corner fracture.
Bucket handle fracture
Posteromedial rib fracture
Scapula fracture
Sternal fractures
Spinous process fractures.
436
Cervical spine fractures are more common after trauma in what age group of children?
\< 8 years-old
This is due to the fact that restraints do not fit young children.
437
What is a Broselow tape used for?
Used with pediatric patients to help estimate medication doses, size of equipment, shock voltage for defibrillator etc.
438
What is different about the airway in a child compared to an adult.
Smaller, larger tongue, floppy epiglottis, and large occiput.
Larynx is higher and more anterior. Sits at the level of the C2-C3 vertebral body compared with C6-C7 in the adult.
**Makes visualization more difficult.**
439
What is the most common cause of cause of cardiorespiratory arrest in pediatric trauma patient?
Hypoventilation
440
How much blood volume must be lost before seeing hypotension in a pediatric patient?
25-30%
Hypotension is a late sign.
441
What is recommended for resucitation in the pediatric patient regarding fluids and blood?
Initial bolus of NS 20ml/kg
After two boluses give PRBC 10ml/kg
442
What is the pediatric equivalent to an adult AC joint separation?
Distal clavicle physeal separation.
The periosteal sleeve and physis remain attached to the AC and CC ligaments.
443
At what age do pediatric proximal humeral fractures peak?
15 years.
More common in adolescents.
444
When does the following secondary ossifications centers appear in the proximal humerus?
Epiphysis
GT
LT
6 months
1-3 years
4-5 years
Closes at 14-17 in girls and 16-18 in boys.
445
What are the acceptable parameters for non-operative management of a pediatric proximal humerus fracture?
\<10 years old = Any degree of angulation
10-12 years old = 60-75 degrees of angulation
\>12 years old = up to 45 degrees of angulation or 2/3 displacement.
446
What structure most commonly prevents closed reduction of a proximal humerus fracture?
Long head of the biceps.
447
What percent of pediatric shaft fractures are associated with a radial nerve palsy?
5%
448
What degee of angulation can be accepted in a pediatric humeral shaft fracture and still have an excellent outcome from remodeling?
20 degrees for older children. 35-45 degrees in young children.
Should watch proximal third fractures in children over 10 years of age. These are the ones that go on to deformity.
**Should also examine any shaft fracture for pathologic lesions or abuse as these injuries are relatively rare.**
Malunion of up to 20-30 degrees is well tolerated.
449
True or false. Transphyseal fracture of the distal humerus is associated wth:
Tardy ulnar nerve plasy
Cubitus varus deformity
Avascular necrosis of the medial condyle
False, this is associated with lateral condyle fractures.
True
True
450
What should be performed at the time of CRPP for a distal humeral physeal fracture?
Arthrogram.
Posterior approach often easiest and avoids damage to articular cartilage.
Helps visualize starting points on capitellum and assessment of the quality of reduction.
Mix equal parts saline and contrast.
451
What is the radiographic difference between pediatric elbow dislocation and and a transphyseal separation?
Posteromedial for transphyseal separation where elbow dislocations are typically posterolateral.
452
How long should you follow a child after a distal humerus physeal separation?
2-4 years to insure there is no growth arrest, deformity, or osteonecorsis.
70% will have cubitus varus. This is cause by AVN, malunion, or growth arrest. Tx is a lateral closing wedge osteotomy.
453
What demographic do you usually see medial epicondyle fractures in?
boys age 9-14. (75%)
454
What is a medial condyle fracture associated with in 50-60% of cases?
Elbow dislocation.
Most spontaneously reduce but fragment remains incarcerated in joint in 15% of cases.
455
What radiographs and why should be obtained in pediatric medial epicondyle fractures?
Besides standard elbow radiographs should obtain a internal oblique view.
This is done to evaluate displacement.
Can also obtain a distal humeral axial view. Obtained by angling beam 25 degrees anterior to long axis of humerus. **This is the most accurate radiograph.**
456
Are valgus instability and ulnar nerve dissfuction absolute or relative indications for operative fixation of a medial epicondyle fracture?
relative.
Absolute are open fractures and incarcerated medial epicondyle fractures.
457
What degree of displacement is typically indicated for non-operative treatment of a medial epicondyle fracture?
\<5mm displacement
Elbow should be immobilized 3 weeks in a long arm cast with the elbow flexed to 90 degrees.
458
True or false radiographic union is 9 times more likely with operative treatment of medial epicondyle fractures?
True
459
What is the most common complication after medial epicondyle fractures?
Elbow stiffness.
Most common whether treated non-operatively or operatively.
460
What is the recommended treatment for a asymptomatic lateral condyle fracture non-union?
Observation
Fixation only recommended if there is pain, instability, or cubitus valgus with ulnar nerve palsy.
461
What is Dejerine Sottas Syndrome?
Hereditary motor-sensory neuropathy that is less common than CMT.
Has an early onset demyelinating motor and sensory neuropathy.
462
What is dolichostenomelia?
Unusually long limbs.
Seen in patients with Marfan Syndrome.
463
Which pediatric elbow fracture has the highest risk of non-union when treated non-operatively?
lateral condyle fractures.
464
What is normal acetabular index in an infant?
27.5 degrees.
\> 35 degrees suggests acetabular dysplasia.
465
If performing saucerization of a discoid meniscus how much peripheral rim should be left?
6-8mm
466
What fixation strategy of a SCFE increases risk of AVN?
Hardware placement into the posterosuperior femoral neck.
There has not been shown to be any increased risk with 1 vs 2 screws.
467
What is the most common metatarsal fracture in toddlers?
1st metatarsal shaft
468
This image is an example of what disease?
Spondyloepiphyseal dysplasia (SED)
disproportionate dwarfism, spinal involvement, and a barrel chest.
From a COL2A1 mutation
469
This image is an example of what disease?
Diastrophic dysplasia
Image shows "hitch hikers" thumb and "cauliflower ear"
Also will see cleft palate and short-limbed dwarfism
Due to a sulfate transport mutation.
470
What disease is depicted in the attached image?
Cleidocranial dysplasia
Due to a defect in **core-binding factor alpha 1 (CBFA-1)**
Causing dwarfism and absent clavicles.
471
What disease is depicted in the attached image?
Multiple epiphyseal dysplasia (MED)
Due to a COMP mutation.
Causes disproportionate dwarfism with multiple epiphyses involved, shortened metacarpals, valgus knees, but no spinal involvement.
472
What is an aneurysmal bone cyst?
Benign, non-neoplastic reactive bone lesion filled with multiple blood filled cavities.
Primary form: now known to be a neoplasm as it is driven up upregulation of the ubiquitin-specific protease USP6 (tre2) gene on 17p13 when combined by translocation with a promoter pairing. **Most common translocation t16;17**
Secondary form: No translocation, not neoplastic.
473
What is this an example of?
Developental coxa vara
Due to an ossification defect of the inferior femoral neck.
Results in a morphologically short femoral neck with a relatively vertical physis and decreased femoral anteverion.
The **Hilgenreiner's-epiphyseal angle** is used to grade the severity and likelihood of progression as well as to assess the adequacy of deformity correction following osteotomy.
Vaglus overcorrection is recommended.
474
Rate of physeal arrest after distal femoral fractures?
According to Basener et al.
36% of SH 1 fractures
58% SH 2
49% SH 3
64% in SH 4
475
What is Drehmanns Sign?
Obligate external rotation of the hip with hip flexion.
Seen in SCFEs.
476
How should a congenital knee dislocation in a 7 month old that can be passively flexed to 25 degrees be treated?
Should be treated with open reduction typically at 6 months of age but before the age of 1.
Exception is if it can passively flex past 30 degrees.
477
What growth arrest can be seen in pediatric midshaft femur fractures?
Tibial tubercle growth arrest -\> recurvatum
478
What may be the significance of the neurocentral synchondrosis?
It is a cartilaginous gorwth place that has been implicated as a potential cause of adolescent idiopathic scoliosis.
Develops between the centrum and posterior neural arches.
Closes cervical 5-6 years, lumbar 11-12 years, and thoracic 14-17 years.
479
What are the two most significant predictors of long term outcome in patients with Perthes Disease?
Age at presentation.
Range of motion of the hip.
480
What does this picture depict if this is a 6 year old boy who has difficulty with abduction and external rotation with history of a difficult birth?
Putti sign- copnensatory scapulothoracic motion to adduct the arm resulting in elevation of the superomeidal border of the scapula.
Brachial Plexus Birth Palsy leads to varign degrees of gleno humeral dysplasia with different recommended treatment.
TYPE II and III latissimus dorsi/ teres major tendon transfers for rotatoru cuff weakness and minimal GH joint deformity
Type III and IV: Open/arthroscopic GH reduction , anterior soft tissue contracture releases, posterior tendon transfers for weakness and moderate GH joint deformity
Type IV and V: Humeral derotational osteotomy for persistent internal rotation contractures and external rotation weakness
481
Neurofibromatosis can be associated with what forearm disorder in pediatric patinets?
While much less common than congenital pseudoarthrosis of the tibia, congenital pseudoarthrosis of the forearm is associated with neurofibromatosis in about 50% of cases.
482
Posttraumatic physeal arrest is most common at which physis?
Distal tibia, specifically distal medial tibia.
#2 is distal femur
#3 is proximal tibia
483
What is the recommended treatment for elbow flexion contractures that are the result of brachial plexus birth palsies?
contractures \<35 degrees treat with serial nightime extension splinting.
contractures \>35 degrees treat with serial casting.
Failure on non-operative treatment can be surgically managed with anterior capsular release and concomitant lengthening of the biceps and brachialis tendons.
It is thought that these contractures develop because of persistent triceps weakness from C7 root injuries.
484
what is depicted in the image?
Gage sign- radiolucency in the shape of a V in the lateral portion of the epiphysis.
Found in LCP and indicates a more severe disease course.
The other 4 "at risk signs from Catterall are:
1 Calcification lateral to the epiphysis
2 Lateral subluxation of the femoral head
3 a horizontal physis
4 metaphyseal cysts
485
Repetitive overuse injuries of the physis in gymnansts occur through what zone of the physis?
Zone of provisional calcification
Metaphyseal perfusion is disrupted, which inhibits calcification
