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5. Tiffany's Cards > Tiffany's Peds Cards > Flashcards

Tiffany's Peds Cards Flashcards

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1
Q

Achondroplasia gene

A

FGFR-3, autosomal dominant

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2
Q

Pseudoachondroplasia gene

A

COMP, autosomal dominant

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3
Q

Multiple epiphyseal dysplasia (MED) genes

A

COMP, COL9A, autosomal dominant

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4
Q

Spondyloepiphyseal dysplasia (SED) gene

A

COL2A1,
Congenita - autosomal dominant
Tarda - X-linked recessive

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5
Q

Kniest dysplasia gene

A

COL2A, autosomal dominant

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6
Q

Apert syndrome gene

A

FGFR-2, autosomal dominant

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7
Q

Charcot Marie Tooth gene

A

PMP22, autosomal dominant

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8
Q

Marfan gene

A

Fibrillin-1 (FBN1), autosomal dominant

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9
Q

Multiple hereditary exostosis (MHE) gene

A

EXT, autosomal dominant

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10
Q

Neurofibromatosis gene

A

NF1, autosomal dominant

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11
Q

Osteogenesis imperfecta type gene

A

COL1A1 and COL1A2
Types 1+4 - autosomal dominant
Types 2+3 - autosomal recessive

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12
Q

Ehlers-Danlos gene

A

COL5A - found in 40%, autosomal dominant
Other gene involved, but COL5A is for the classic type

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13
Q

Diastrophic dysplasia gene

A

DTD, sulfate transport gene, autosomal recessive

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14
Q

Friedreich’s ataxia gene

A

Frataxin, autosomal recessive

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15
Q

Gaucher disease gene

A

B-glucocerebrosidase deficiency, autosomal recessive

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16
Q

Spinal muscular atrophy gene

A

SMN, autosomal recessive

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17
Q

Vitamin D resistant (familial hypophosphatemic) rickets gene

A

PHEX gene, X-linked dominant

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18
Q

Becker’s muscular dystrophy gene

A

dystrophin, X-linked recessive

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19
Q

Duchenne muscular dystrophy gene

A

dystrophin, X-linked recessive

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20
Q

Hunter syndrome gene

A

Sulpho-iduronate-sulphatase, autosomal recessive

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21
Q

Hurler syndrome

A

Alpha-L iduronidase, autosomal recessive

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22
Q

At what age does puberty start, reach peak growth velocity + finish? What physeal closure marks peak velocity?

A

Girls 10 - 12 - 14
Boys 12 - 14 - 16
Peak growth velocity marked by olecranon closure*
*RC question

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23
Q

When does the triradiate cartilage close?

A

Midway during ascending phase of puberty

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24
Q

RC: What corresponds to peak growth velocity?
A. Closure of olecranon apophysis
B. Risser 1
C. Closure of triradiate
D. Menarche

A

Answer: A

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25
List methods of estimating bone age (3)
1. L hand XR - radiographic atlas of hand + wrist by Greulich + Pyle 2. Sauvegrain method - elbow ossification 3. Risser
26
What is the definition of skeletal maturity? (3)
1. Risser 4 2. <1cm height growth over 6mo 3. 2yrs post menarche
27
What is the Risser classification?
Describes stages of iliac apophysis ossification. Useful for assessing spine bone age as the spine matures later than limbs. Ossifies from lateral to medial 1. 25%. Early spine puberty 2. 50%. Spine max velocity 3. 75%. Slowing spine growth 4. 100%. Slowing spine growth 5. Iliac apophysis fused to iliac crest. Growth finished
28
Describe how knee varus/valgus changes during childhood
Max varum at birth, becomes peak valgum by 3yo. Normalizes at 5yo to 5deg valgum
29
List the zones in the physis
Epiphysis Reserve zone Proliferative Hypertrophic zone - subdivided into maturation, degenerative, provisional calcification Metaphysis RC question
30
Describe the percentage growth contributed by each physis to each bone
31
What pathologies are associated w the zone of provisional calcification?
Rickets/osteomalacia, hypophosphatasia
32
What pathologies are associated w the reserve zone of the physis?
Gaucher, diastrophic dysplasia, Kneist
33
What pathologies are associated w the proliferative zone of the physis?
Achondroplasia, MHE, gigantism
34
What pathologies are associated w the hypertrophic zone of the physis
SCFE, rickets, hypophosphatasia, enchondromas, mucopolysaccharide, fractures
35
What is the Heuter-Volkman law?
Compression across physis slows growth. Tension accelerates growth
36
Name some pediatric milestones
4mo - good head control 8mo - sits independently 10 - crawls 12 - walks independently. Must walk by 18mo!
37
% of #s due to NAT?
% of #s due to NAT 90% occur <5yo 50% <1yo 30% <3yo
38
Most common presentation of NAT?
Skin lesions
39
Most common #s in NAT?
Humerus > tibia > femur
40
Risk factors of NAT?
Child-related: 1st born, unplanned pregnancy, premature, disability, step-child Parent-related: single parent, recent stressor, unemployed, drug use, personal hx of abuse as child, low socioeconomic status, lack of social support
41
DDx of NAT?
True accident, OI, osteopenia of prematurity, disuse osteopenia, kidney disease, liver disease
42
Red flags when suspecting NAT (4)
1. Delay in seeking care 2. Inconsistent hx 3. Long bone # in nonambulatory kid 4. Specific # types (later slide)
43
What are some # types associated with NAT?
#s at metaphysis/physis junction Corner #s Bucket handle #s Epiphyseal separations - esp distal humerus
44
What imaging do you need to order for NAT W/U?
Suspected bone XR Skeletal survey or bone scan
45
What is the difference between skeletal survey XR for NAT and dysplasia?
Dysplasia - skull, spine, chest, pelvis, one side of arm, one side of legs NAT - all of above, but need both sides of arms + legs
46
When suspect NAT, what must you do?
Admit for multidisciplinary evaluation Appropriate imaging Consult CPS + social service Treat injury
47
What is congenital pseudarthrosis of the clavicle? Treatment?
Congenital failure of fusion between medial + lateral ossification centers. Due to subclavian artery compression. Sometimes mistaken for NAT Nonop - if asymptomatic ORIF w iliac crest bone graft if pain, impaired fxn or cosmesis
48
50% of pediatric septic hip is before what age?
2yo
49
Risk factors for neonatal septic hip? (2)
C-section Prematurity
50
Routes of inoculation for septic hip? (3)
Direct trauma Hematogenous Extension from tissue
51
Describe the blood supply to the hip before 4yo (3)
1. Ligamentum teres - posterior branch of obturator art 2. Circumflexes 3. Transphyseal metaphyseal branches (disappear between 4-17yo, then reappear)
52
What joints have intracapsular metaphyses? (4)
1. Shoulder 2. Elbow 3. Hip 4. Ankle NOT KNEE
53
DDx of hip pain in kids of all ages? (6)
OM/septic hip DDH SCFE Perthes Psoas abscess Transient synovitis
54
RC: 7yo presents w 5wk history of painless limp. On exam, he has decreased abduction and internal rotation. There is a trendelenburg gait. What is the diagnosis? A. DDH B. JRA C. Transient synovitis D. Perthes
Answer: D (Perthes) DDH would not be acute JRA and transient synovitis would be painful
55
Indicators of poor prognosis in pediatric septic joints? (4)
Age <6mo Associated OM Hip (instead of knee) Delayed presentation >4d
56
What are the longterm sequelae of pediatric septic hip?
AVN Physeal arrest - LLD, angular deformity
57
Physical exam findings in pediatric septic hip?
Refusal to WB FABER position Pseudoparalysis
58
Most common organisms in pediatric septic hip? (5)
GBS, GAS, S. aureus, N. gonorrhea, K. kingae
59
Most common organisms in neonatal septic hip? (2)
GBS - community acquired S. aureus - nosocomial
60
Most common organism in pediatric septic hip >2yo?
S. aureus
61
Most common organisms in adolescent septic hip? (2)
S. aureus, gonorrhea
62
What are the signs and treatment of gonorrhea septic hip?
Migratory polyarthritis, red papules Tx - penicillin alone. Don't need I+D
63
What is the Kocher criteria? (4)
Fever >38.5 ESR >40 WBC >12 Unable to WB All criteria met - 99% likely septic hip 3/4 - 93% 2/4 - 40% CRP >2 is not apart of Kocher criteria. Independent risk factor of 74% probability
64
Pediatric septic hip - what is the order of sensitivity of the following factors - fever, WB status, ESR, CRP, WBC?
Fever > CRP > ESR > NWB > WBC
65
Findings on native joint aspiration that suggest infection?
>50k cells w >75% PMNs
66
Approach to I+D native septic hip?
Bikini incision - Smith Pete approach
67
MRSA infections are associated w what complications?
DVT + septic emboli
68
What part of the bone is most susceptible to osteomyelitis?
Metaphysis. Slow flow in metaphyseal vessels due to sharp turns. Time for bacteria to lodge
69
Which joints are at risk of septic infection from OM? (4)
Shoulder, elbow, hip, ankle. NOT knee
70
What are the bone findings in chronic OM?
Periosteal elevation from abscess Sequestrum - necrotic bone Involucrum - outer layer of new bone
71
What is the most sensitive image modality to detect OM?
MRI w gadolinium
72
Which biochemical marker is best for monitoring treatment response in OM?
CRP Elevated within 6hrs of infection. Decreases rapidly w treatment. Normal in a week If doesn't decrease after 48h, reconsider treatment (ESRP peaks in 3d but decreases too slowly to guide tx)
73
RC: What is true regarding osteomyelitis? A. High CRP >20 has a LOW sensitivity B. High WBC has LOW sensitivity C. Radiographs are appropriate for diagnosis D. Bone scan is useful to follow long term
Answer: B CRP is the most sensitive test. WBC is the least. XR is not the best for diagnosing OM because it takes weeks for changes to be seen. Secondly, in patients w neuro-arthropathy or Charcot, XR findings look similar.
74
RC: In the setting of chronic osteomyelitis that has malignant transformation, what is most true? A. Wide excision reconstruction is preferred to amputation B. Most commonly metastasize to lungs C. Clinical presentation often involves worsening purulence, foul smell and drainage D. Marjolin's tumor does not commonly transform in squamous cell
Answer: C Marjolin's tumor classically results from aggressive squamous cell. Think of this whenever there is a chronic ulcer. Need a biopsy to confirm malignancy. Most common site of mets are lymph nodes. Most common clinical features are pain, foul smell and drainage. Best treatment is amputation. Can consider reconstruction if distal and non-metastatic. No evidence that reconstruction does better than amputation.
75
Indications for antibiotics alone for OM treatment?
Early disease No subperiosteal or bone abscess Improvement w antibiotics within 48H - follow CRP
76
Indications for I+D in OM?
Bone or subperiosteal abscess Failure to respond to abx Chronic infection Technique - debride devitalized tissue, drill into intraosseous collections + remove sequestrum. Send for cx + bx
77
RC: In pediatric patients undergoing biopsy, when should samples also be sent for anaerobes, acid-fast bacilli and fungus? A. All patients B. Osteomyelitis w associated septic arthritis C. Osteomyelitis following a penetrating injury
Answer: C Always send for pathology and aerobic culture. Anaerobe, acid-fast and fungus should also be sent for - Failed primary treatment (Failed initial abx tx?) - immunocompromised - Penetrating wounds
78
RC: A previously healthy kid presents w pain to the mid tibia w a fever a elevated ESR. Found to have MRSA tibial osteomyelitis. The child comes from an area w a high prevalence of MRSA. What is the best antibiotic? A. Cefazolin B. Rifampin C. TMP-SMX D. Clindamycin
Answer: D (clindamycin) Antibiotics for serious MRSA: vancomycin, daptomycin, linezolid Antibiotics for stable MRSA: clindamycin 2016
79
What is chronic recurrent multifocal osteomyelitis? Diagnostic criteria? (3)
Idiopathic inflammatory disease 1. Multiple sites of apparent OM 2. Pathology + culture neg 3. No response to antibiotics
80
What is the prognosis and treatment of chronic recurrent multifocal osteomyelitis?
Comes + goes, resolves in 3yrs. Treatment - NSAIDs + pamidronate, as per Rheum. Rarely causes growth disturbance
81
RC: You are seeing a patient that has a diagnosis of chronic recurrent multifocal osteomyelitis. What is the ideal plan regarding treatment? A. Biopsy and cultures are negative in this condition and should be treated according to rheumatology B. Arrange for percutaneous or open biopsy followed by antibiotics C. Admit to hospital for 6wks for broad spectrum IV antibiotics D. Can become a Brodie's abscess
Answer: A
82
RC: What is true regarding chronic recurrent multifocal osteomyelitis? A. After core biopsy, it is best treated w I+D, followed by antibiotics B. Bone scan is needed in the workup C. It is an inflammatory condition that behaves like osteomyelitis but has sterile cultures
Answer: C Inflammatory process, don't treat w antibiotics. Treat w NSAIDs and pamidronate. A bone scan can determine other sites but is not necessary
83
What are poor prognostic factors for physeal injuries?
Higher SH grade (3+4) Higher energy trauma Numerous reduction attempts Poor final reduction Distal femur + tibia #s Greater initial displacement
84
What is the F/U protocol for physeal injuries?
Repeat XR q3mo until normal growth for 6mo
85
What are Harris lines?
Radiographic finding after physeal injury. Represents growth arrest + resumption. Represents physis position at time of injury
86
List management options for growth disturbance after physeal injury (4)
1. Physeal bar resection 2. Epiphysiodesis 3. Chondrodiastasis 4. Limb lengthening + deformity correction
87
What are the indications for physeal bar resection?
Existing or developing deformity w >2yrs growth left <50% physis affected >1yr without drainage if physeal bar secondary to infection
88
Describe the technique for physeal bar resection
Map physeal bar w MRI Peripheral bar - approach direct + excise overlying periosteum Central bar - make metaphyseal window or osteotomy Remove bar completely w bar. Prevent thermal damage w irritation + suction Dry scope to check complete resection Interpositional graft Insert metallic markers on either side of physis for F/U - to differentiate growth of affected vs other side
89
Interpositional graft options for physeal bar resection?
Fat graft - autologous but no support Artificial dura PMMA - don't use. Generates heat + damages physis
90
Describes the types of epiphysiodesis
Permanent - drilling + curettage Reversible - physeal staple, 8 plate, transphyseal screws
91
Indications for epiphysiodesis for physeal injury management?
Physeal bar >50% Central physeal arrest - projected 2-5cm LLD at maturity. Do contralateral limb Peripheral physeal arrest - do ipsilateral limb
92
List methods of predicting limb length at maturity (3)
1. Arithmetic method 2. Multiplier method 3. Moseley straight line method
93
What is the arithmetic method of predicting leg length?
Girls stop growing at 14. Boys stop growing at 16. Growth remaining at each physis = (yrs growth remaining) x (inches of growth from that physis/yr)
94
What is the multiplier method of predicting leg length?
Theory - leg growth is constant, based on age + sex Current limb length x multiplier = predicted length at maturity Multiplier based on current bone age
95
Describe the indication + technique of chondrodiastasis for physeal injury management
Indication - adolescents approaching maturity Ex-fix applied above + below physis Distract physis to lengthen + correct angular deformity (Heuter Volkman law) Intraop - can over distract + twist to break physeal bar
96
RC: 12yo has a 4.5cm LLD. Bone age is 14.5. Scanogram shows equal deficit from tibia and femur. What should be done? A. Delayed epiphysiodesis of femur and tibia B. Immediate epiphysiodesis of femur and tibia C. Epiphysiodesis of femur D. Lengthening w Ilizarov
Answer: B Question stem didn't specify which physes would be treated. Assume it's distal femur and proximal tibia Use the arithmetic method: growth of femur and tibial physes are 3/9/6/5 If boys stop growing at age 16, this child has 1.5yrs of growth left. If you close the distal femur and proximal tibia physes of the long leg now, the 4.5cm discrepancy + 1.2cm growth from the remaining physes = 5.7cm LLD at maturity Remaining growth of the short leg is 1.5 x (3+9+6+5) = 3.45 Final LLD would be 2.25, which can be treated w a heel lift Technically, if his growth rate remained the same and the end deficit was 4.5, could also treat this w a shoe lift. But this wasn't an option 2017
97
Acceptable alignment for peds trauma
98
What structures are at risk w posterior displacement of medial clavicle physeal fractures?
Brachiocephalic art + v Internal jugular v Phrenic n Vagus n Trachea Esophagus
99
Describe the serendipity XR view for medial clavicle physeal #
40deg cephalic tilt Anterior displaced - clavicle above other side Posterior displaced - clavicle below other side
100
Describe the management options and indications for medial clavicle physeal #s
1. Observation - anterior displacement. Posterior displacement w/o mediastinal injury 2. Closed reduction under anesth - posterior displacement w mediastinal injury 3. ORIF - failed closed reduction or chronic symptomatic posterior displacement
101
What is the contraindication to closed reduction of posteriorly displaced medial clavicle physeal #s?
Late presentation. May be adherent to mediastinal structures
102
Describe the closed reduction technique for anterior displaced medial clavicle physeal #
Longitudinal traction to both arms Posterior pressure to medial fragment Can grasp medial fragment w towel clip
103
Describe reduction + fixation technique for posterior displaced medial clavicle physeal #
Longitudinal traction to affected arm w shoulder adducted CT after to confirm stability Grasp medial fragment w towel clip + pull anterior If unsuccessful, do open. Fix w sutures. Don't fix w pins - will migrate
104
Regarding % of physeal growth contribution, which bone has the largest proximal : distal growth difference? Which bone is 2nd largest?
Humerus - proximal physis contributes 80% growth + distal contributes 20%. 2nd is femur - proximal is 30%, distal is 70%
105
What is the Neer-Horwitz classification for pediatric proximal humerus #s?
Type 1 - minimally displaced 2 - displaced <1/3 shaft width 3 - displaced 1/3 - 2/3 width 4 - displaced >2/3 width
106
What are the acceptable alignment parameters for pediatric proximal humerus #s?
<5yo: <70deg angulation, 100% displaced 5-12yo: <45-70deg >12yo: <45deg angulation or <2/3 displaced *RC question
107
RC: Proximal humerus # in a 14yo girl. 45deg angulation and 60% translation. What is the best treatment? A. ORIF B. Hanging cast C. CRPP
Answer: C
108
Describe the deformity in proximal humerus #s. Describe the reduction maneuver for proximal humerus #s.
Deformity: proximal fragment is varus + apex anterior from rotator cuff. Distal fragment is anterior, adducted + shortened from pec major + deltoid Reduction maneuver: - Traction - Shoulder flexion + abduction to 90deg - ER
109
If unable to reduce a proximal humerus # closed, what structures may be blocking reduction?
Long head of biceps - most common Joint capsule Periosteum Deltoid
110
For pediatric proximal humerus #s, what are fixation options? (4)
1. Perc pinning - avoid axillary n, musculocutaneous n + posterior circumflex (ER arm to avoid). Smooth pins can be removed in clinic. Threaded need OR to remove 2. Retrograde flexinails. Avoid physis 3. Cannulated screws 4. Plate
111
Proximal humerus #s may fall into varus malunion. How would this affect function?
Glenohumeral impingement
112
In pediatric humeral shaft #s, what are acceptable alignment parameters?
Young (?8yo): <45deg angulation Older: 20deg varus/valgus, 20deg procurvatum, 15deg rotation, 2cm shortening
113
RC: All are true regarding the use of flexible nails for humeral shaft #s in pediatrics, except: A. Low risk of radial n injury B. Low risk of delayed union C. Should not be used in OI D. Shortening is well tolerated in the upper extremity
Answer: C 2cm shortening is acceptable Surgical management in OI should include intramedullary fixation
114
What are common mechanisms of injury for distal humerus physeal separations?
Birth trauma - shoulder dystocia, traumatic delivery, excessive traction during c-section NAT FOOSH
115
What are the treatment options for distal humerus physeal separation?
Casting - limited role. Most are displaced. Only for late presentation for comfort. Osteotomy later for deformity CRPP - most cases, to maintain reduction
116
Describe the technique for CRPP of distal humerus physeal separation
1. Elbow arthrogram: 50/50 saline + contrast 2. Reduction. Flex elbow for pinning 3. 2-3 k-wires, divergent at #. Bicortical 4. Check ROM on fluoro
117
What complications are associated w distal humerus physeal separation? (3)
1. cubitus varus. 70% incidence. Due to medial condyle AVN, malunion or growth arrest 2. Fish tail deformity. Medial or lateral condyle AVN 3. Growth disturbance, causing length discrepancy or angular deformity
118
At what age do the pediatric elbow ossification centers ossify and fuse?
Ossification: CRITOE Fusion: CTE-ROI
119
When viewing a pediatric elbow XR, what are you looking for? (7)
1. Anterior humeral line - should pass through middle third of capitellum 2. Humerocapitellar angle: normal is 65-80deg. Lateral XR. Angle between humeral shaft + line along capitellum physis. Important for supracondylar #s. Sometimes called lateral Baumann's angle 3. Baumann's angle: normal is 70-75deg. AP XR. Angle between humeral shaft + capitellum physis. 4. Radiocapitellar line - radial shaft should align w center of capitellum. Important for Monteggia 5. PUDA 6. Anterior sail sign 7. Posterior fat pad - occult #
120
What is the most common type of pediatric supracondylar #?
Extension type
121
What are common nerve injuries associated w pediatric supracondylar #s?
Extension type: AIN most common (posterolateral displacement), radial n 2nd most common (posteromedial displacement) Flexion type: ulnar n
122
What is the Gartland classification?
Pediatric supracondylar #s 1: nondisplaced 2: displaced but posterior cortex intact 2A: posterior tilt, no rotation 2B: there is rotation or medial impaction 3: complete displaced 4: complete periosteal disruption. Unstable in flexion + extension. Diagnosed intraop w EUA Also: medial comminution type + flexion type
123
What complications are associated with supracondylar #s? (7)
1. Operative related - pin migration, infection, stiffness 2. Cubitus valgus malunion - tardy ulnar n palsy 3. Cubitus varus malunion - gunstock deformity. Causes elbow instability 4. Recurvatum - due to nonop tx of extension types 5. Nerve palsies - AIN, radial, ulnar 6. Vascular injury 7. Compartment syndrome, Volkmann contracture
124
In what elbow position do you cast/splint a supracondylar #?
Nonop or CRPP w only lateral pins: neutral rotation, don't flex beyond 90deg to decrease risk of compartment syndrome If cross pinning: splint in extension to prevent ulnar n subluxing over medial pin
125
Kid presents w type 3 supracondylar #. Well perfused, good pulses, neuro intact. What is your plan?
Splint for elective surgery same day OR. Type 3 can wait 21h without increased complications as long as NVI
126
Kid presents w supracondylar # that is pulseless but well perfused. What is your plan? What is your DDx?
Pink + pulseless. Urgent same day OR. DDx - vascular injury, thrombosis, spasm Do not reduce bedside, only reduce in OR. Reduction may lacerate brachial artery + causing expanding hematoma (causing compartment syndrome) If continues to be pink + pulseless after reduction - monitor vascular + neuro status x24h Pulses can return in 3wks
127
In a kid w a supracondylar # and a pink/pulseless hand, when would you open the #?
- Reduction attempt (in OR!) causes expanding hematoma - AIN palsy preop (sometimes) - Unable to reduce closed
128
Kid presents w supracondylar # that is pulseless + poorly perfused. What is your plan?
White + pulseless. Emergent OR now. - Ask when injury happened. May need prophylactic fasciotomy - Bring to OR - Call vascular (or plastics if hospital doesn't have vascular) - Closed reduction + R/A - Open if unable to reduce closed, continues to be poorly perfused, or expanding hematoma
129
Describe the incision used to open a supracondylar when suspecting a vascular injury?
Anterior, S shaped incision. Proximal arm starts medial on humerus, cross antebrachial fossa + bring distal arm lateral on forearm
130
Describe the closed reduction technique for an extension-type supracondylar #
- Longitudinal traction - Correct varus/valgus. Thumb points towards direction of displacement. Pronate if fragment displaced medially. Supinated if fragment displaced laterally - Flex elbow + place pressure on fragment to correct extension - Assess w fluoro + pin RC question
131
Describe how to use crossed pins when fixing pediatric supracondylar #s
- Mini open incision medially - Place pin w elbow extended, after placing lateral pins - Don't cross pins at # site (very unstable) - Splint arm in extension
132
What is the danger of using a medial pin when fixing pediatric supracondylar #s?
Ulnar n Highest risk when pin placed w elbow in flexion. Ulnar n may sublux anterior over the medial epicondyle in some kids.
133
In pediatric supracondylar #s, what is most stable: 3 lateral pins vs crossed pins?
No difference in stability
134
RC: Type 3 supracondylar #, which of the following is true? A. Splint in 120deg flexion to maintain reduction B. Equivalent outcomes w lateral and crossed pinning C. Require emergent surgical management D. There is limited remodeling for translational deformity
Answer: B Do not splint beyond 90deg flexion, otherwise increases compartment syndrome risk. Type 3 can wait as long as NVI Translational deformity remodels 2017, 2019
135
In kids, what type of # is most commonly associated with elbow dislocations?
Medial epicondylar #
136
In what direction are pediatric medial epicondylar #s usually displaced?
Anteriorly. Avulsion injury from flexor-pronator mass
137
What XR views is most helpful in assessing medial epicondyle #s?
Internal oblique + axial view. AP + lateral may not be accurate. The medial epicondyle is a posteromedial structure. Fragment is displaced anterior
138
RC: Which accurate describes the anatomy of the distal humerus? A. 30deg anterior angulation, 6deg valgus, IR B. 30deg anterior angulation, 6 varus, IR C. 10deg anterior angulation, IR D. 10deg anterior angulation, 6deg varus, ER
Answer: A Distal humerus has 30 anterior angulation, 6deg valgus and 5deg IR
139
What are the indications for nonoperative treatment of pediatric medial epicondyle #s? What are the outcomes compared to surgery?
Indication: <5mm displacement. Controversial: 5-15mm displacement Outcomes: 9x more osseous union with surgery. Nonop leads to more fibrous union, which doesn't affect function + is asymptomatic
140
What are the indications for ORIF for pediatric medial epicondyle #s?
Absolute indications: - Open # - Fragment incarcerated in joint - Post reduction ulnar neuropathy when assoc w elbow dislocation - Medial condyle involved (articular surface) Relative indications: - Ulnar n neuropathy - >5-15mm displacement - High level athlete (thrower, gymnast) - Associated w elbow dislocation
141
Describe the surgical technique for ORIF of pediatric medial epicondyle #s
- Medial approach between brachialis + triceps - Isolate ulnar n - Screw or k-wire fixation
142
RC: 12yo girl presents 6mo after being treated nonoperatively for a minimally displaced medial epicondyle fracture. There is a non-union, but she has full ROM and no pain. What now? A. ORIF w pins B. Place in above elbow cast C. Followup in 6mo D. Get MRI to investigate for fibrous nonunion
Answer: C Nonoperative management of medial epicondyle #s lead to more fibrous unions. But this doesn't affect function and is asymptomatic. 2017
143
In peds, why do lateral condyle #s have worse outcomes than supracondylar #s?
- Articular - Often missed - Higher malunion/nonunion risk
144
What is the Milch classification?
Pediatric lateral condyle #s Type 1: # is lateral to trochlear groove Type 2: # extends into trochlear groove Not as useful as Weiss classification
145
What is the Weiss classification?
Pediatric lateral condyle #s. Describes displacement to guide treatment - Type 1: <2mm, intact cartilage hinge. Cast - Type 2: 2-4mm. Intact articular hinge on arthrogram (thus, more stable). CRPP - Type 3: >4mm. Articular hinge not intact. Likely need to open to reduce before pinning
146
Describe the pin or screw configuration when fixing pediatric lateral condyle #s
1 pin in lateral column, 1 pin in transverse column parallel to joint. Divergent. This is a different configuration than supracondylar #s. Direction should be from posterolateral to anteromedial
147
List complications associated w pediatric lateral condyle #s
1. Stiffness: self resolving 2: Nonunion 3. Capitellum AVN: assoc w posterior dissection 4. Trochlear AVN: fishtail deformity from growth arrest. Causes pain, limited ROM, proximal ulnar migration (overloads radiocapitellar joint) and arthritis. Does not cause humeral length deficiency 5. Lateral overgrowth/prominence 6. Cubitus valgus: tardy ulnar n 7. Growth arrest
148
RC: All of the following are true regarding a fishtail deformity of the distal humerus, except A. Associated w supracondylar humerus # B. Results from central physeal growth arrest C. Predisposes to early ulnohumeral degenerative changes D. Results in significant humeral length deficiency
Answer: D Fishtail deformity associated with all distal humerus #s (almost usually mentioned w lateral condyle or medial epicondyle #s). Results from central physeal arrest and can cause arthritis. Only 20% physeal growth from distal physis.
149
Definition of Monteggia injury?
Radial head dislocation + proximal ulna # (or plastic deformity in kids)
150
True or false: in Monteggia injuries, the ulnar # apex and radial head dislocation are always in the same direction
True! RC question gave you the direction of the ulnar # apex. You had to indirectly know the direction that the radial head was dislocated. They asked if you had to increase or decrease the PUDA to reduce the head.
151
What is the Bado classification? In what position are each splinted?
Monteggia # 1: anterior radial head dislocation. Splint in flexion + supination 2: posterior radial head dislocation. Full extension 3: lateral radial head dislocation. Full extension + valgus mold 4: anterior radial head dislocation, radial head # + ulna #
152
What are signs associated w congenital radial head dislocation? (6)
1. Bilateral 2. Posterior dislocation 3. Convex head (usually radial head is concave) 4. Enlarged head 5. Atraumatic 6. Associated w syndromes
153
What are the indications for nonoperative management of pediatric Monteggia #s?
Types 1-3, as long as ulnar # + radial head dislocation stable after reduction
154
What are the indications for ORIF of pediatric Monteggia #s?
- Types 1-3 w unstable ulnar # or radial head - Type 4 - Open # - Older kids may be treated like adults? Technique: plate ulna, ORIF radial head w screws like in adults
155
RC: 16yo is assaulted and presented w a type 1 Monteggia #. What is the most appropriate treatment? A. Closed reduction and cast B. Flexible nail of the ulna and reduction of radial head C. Resect radial head D. ORIF ulna and closed reduction of radial head
Answer: D Apparently we are to treat a 16yo like an adult. Adult Monteggia are treated w ulna ORIF and radial head closed reduction Should not treat w flexible nails. Not rigid enough to keep ulna aligned Don't resect the radial head in acute cases
156
Describe the management options of chronic Monteggia #s
Radial head reduction vs resection - Radial head reduction, order of procedure: closed reduction > open reduction via Boyd approach for ulnar osteotomy + open radiocapitellar joint reduction - Radial head resection: after skeletal maturity
157
Describe the Boyd approach
No internervous plane: approach is between anconeus - Posterolateral incision along lateral epicondyle + extend along ulnar shaft - Incise anconeus tendon along its attachment on the ulna - Elevate anconeus flap anteriorly - Detach supinator from ulnar origin - Pronate forearm to protect PIN (within substance of supinator) - Retract anconeus + supinator anteriorly to expose capsule
158
In both bone forearm #s, describe how to determine rotational alignment radiographically. (4)
1. AP view: bicipital tuberosity + radial styloid should be 180deg apart 2. Lateral view: coronoid + ulnar styloid should be 180deg apart 3. Diameters of proximal + distal fragments should match 4. Cortical thickness of proximal + distal fragments should match
159
What are acceptable alignment parameters for pediatric both bone forearm #s?
Controversial Younger than 10yo - <15deg angulation - <30deg malrotation - <100% translation - bayonet is ok, <1cm short Older than 10yo - <10deg angulation - <30deg malrotation - <50% translation - No bayonet, no shortening Despite all this, rotation does not remodel and some say no malrotation is acceptable
160
True or false: in both bone forearm #s, using an above elbow cast (as opposed to below elbow) decreases the risk of loss of reduction
False. No difference in loss of reduction w short vs long arm cast. (I would still use a long arm cast in practice, but this is the new literature)
161
Define "cast index" What sort of fractures is this term most often associated with?
Cast index = lateral width / AP width Goal is cast index <0.8 You want the AP width to be > than lateral width This helps you maintain reduction Cast index is often used when discussing both bone forearm and distal radius #s RC question
162
RC: What is true of the cast index? A. Measured from cast to skin and versus soft tissue shadow B. Width of sagittal plane compared to width of coronal plane of the inner cast at the fracture site C. Can predict functional outcome
Answer: B
163
In pediatric both bone forearm #s, what are the indications for flexinail vs plate fixation?
Flexinails: - Unacceptable alignment - >13yo (thus, near maturity) Plate fixation: same as flexinail indications but w additional indications - Open # - Refracture - Highly comminuted
164
Describe the technique for flexinailing pediatric both bone forearm #s
Can start w either radius or ulna, whichever is easiest 1. Ulnar nail inserted through olecranon. Avoid ulnar n 2. Radial nail can be inserted through either: - Radial styloid between compartments 1 and 2. Risk superficial radial n - Lister's tubercle. Risk EPL attritional rupture 3. Open starting points w awl 4. Reduce bone. Up to 3 unsuccessful passage attempts allowed before opening #. Otherwise risk compartment syndrome
165
How do the outcomes for pediatric both bone forearm flexinailing compare to ORIF?
Flexinailing has - Shorter OR time - Less blood loss - Equal union rates, ability in restoring radial bow, ability in restoring rotation
166
When flexinailing pediatric both bone forearm #s, what is the risk of using a radial styloid start point?
Superficial radial nerve neuroma
167
When flexinailing pediatric both bone forearm #s, what is the risk of using a Lister's tubercle start point? When does this complication usually occur?
EPL attritional rupture. 6-12wks postop
168
What is the treatment for EPL attritional ruptures?
EIP to EPL tendon transfer
169
What are the risk factors for compartment syndrome in pediatric both bone forearm #s? (2)
1. High energy trauma 2. Multiple attempts (>3) at reduction + passing flexinail
170
What are some complications associated w pediatric both bone forearm #s?
- Refracture: assoc w plate removal or green stick patterns - Malunion leading to loss of prosupination. Treat w corrective osteotomy - Compartment syndrome - Synostosis: assoc w high energy trauma and head injury
171
RC: Which of the following is true: both bone forearm # w 10 deg rotational malunion A. A midshaft malunion will result in more decreased pronation B. A midshaft malunion will result in a more decreased supination C. A distal malunion will result in more decreased pronation D. A distal malunion will result in a more decreased supination
Answer: B Pronation losses were similar for both distal and middle forearm deformities. However, supination loss is more affected w midshaft deformities than distal ones. 2017
172
What percentage of growth do the proximal and distal radial physis contribute?
Proximal: 25% Distal: 75%
173
What is the most common site of pediatric distal radius #s?
Metaphysis
174
What are the acceptable alignment parameters for pediatric distal radius #s?
Controversial <9yo - <20deg angulation - <50% translation >9yo - >10deg angulation Rotation does not remodel. No malrotation acceptable for any age RC question
175
RC: 8yo presents to your clinic 10d after a distal radius # (no XR, no mention of physeal injury). He has been splinted and has 20deg apex volar angulation. He has a well molded cast. What do you do next? A. Followup in 4-5wks B. Hematoma block and repeat closed reduction C. Drill osteoclasis and fixation D. Open reduction and plate
Answer: A
176
True or false: there is no difference in loss of reduction w short arm vs long arm cast for distal radius #s
True. Can use either. Goal is for cast index <0.8
177
What is the risk of re-displacement w pediatric distal radius #s?
30% incidence Most important risk factor: initial complete displacement. 7x more likely to redisplace Anatomic reduction reduces risk for re-displacement RC question
178
RC: Regarding entirely metaphyseal distal radius fractures in children treated w closed reduction and casting, which of the following is true about re-displacement? A. 30% incidence B. More common if younger than 10yo C. An above elbow cast prevents re-displacement D. Transverse patterns are more unstable
Answer: A No difference in stability in above elbow vs below elbow cast. Transverse patterns are more stable. (JPO 2015: Distal radius fractures in children: risk factors for redisplacement following closed reduction)
179
What is the indication for CRPP in pediatric distal radius #s? Describe the technique
Unstable pattern - unable to maintain reduction in cast Radial styloid pins, just proximal to physis If need transphyseal pin, use smooth wires If intra-articular #, pin transversely across epiphysis Dorsal pin may restore volar tilt Dangers: superficial radial n
180
For physeal injuries to the distal radius, what are the management principles for acute vs late presentations?
Acute presentation: gentle reduction, NO re-manipulation Chronic presentation: NO late reductions. Do not attempt reduction after 3ds even if unacceptable alignment. May cause growth arrest. Just well moulded cast to prevent further displacement. Hopefully remodels RC question
181
RC: 12yo comes in 10days after a SH2 distal radius # with 15deg radial inclination on AP XR and 50deg apex volar on lateral XR. What is the best treatment option? A. ORIF w plate B. ORIF and perc pinning C. Repeat closed reduction and cast D. Cast with appropriate molding and observe
Answer: D SH2 and 10days after injury. Therefore, physeal injury w delayed presentation. Don't reduce as it could cause growth arrest. Just cast with a mold to prevent further displacement.
182
What is a Galeazzi fracture? In pediatrics, what is a Galeazzi equivalent?
Distal third radius # and DRUJ disruption Galeazzi equivalent: distal third radius # and displaced ulnar physeal injury
183
What is the Walsh classification?
Galeazzi fractures Type 1: Radius displaced dorsally, due to supination force Type 2: Radius displaced volarly, due to pronation force
184
What is the closed reduction technique for Galeazzi injuries?
- Supinate if ulna displaced dorsally - Pronate if ulna displaced volarly
185
In an irreducible DRUJ injury, what is tissue is most likely interposed?
ECU
186
How would you surgically manage a pediatric Galeazzi injury?
Radius: fix w volar plate or flexinail DRUJ pinning: - Dorsal approach if unable to reduce DRUJ due to interposed tissue - PIN ulna to radius in supination - If large ulnar styloid fragment, pin that too Splint in supination
187
What are the entry points for radius flexinails? (2)
1. Radial styloid between compartments 1 and 2 2. Lister's tubercle
188
True or False: in pediatric traumatic hip dislocations, associated acetabular #s are common
False. Less common than in adults due to cartilaginous acetab. Most common type: posterior wall #
189
In pediatric traumatic hip dislocations, what increases the risk of AVN?
Not reduced within 6hrs
190
Describe the leg position upon presentation in patients w anterior or posterior hips dislocations
Anterior: FABER Posterior: FADIR
191
What is the management of pediatric traumatic hip dislocations?
Reduction within 6hrs of injury. Options after reduction include - Spica cast - <10yo: bedrest + abduction splint x4wks - >10yo: bracing + protected WB x 6wks
192
Regarding the greater trochanteric apophysis, what deformity results from growth arrest? What deformity results from overgrowth?
Growth arrest causes a short GT and coxa valga Overgrowth causes coxa vara
193
Describe the blood supply to the proximal femur in a pediatric patient
- Ligamentum teres: main blood supply at birth. Decreases after 4yo - Metaphyseal vessels: contributes at birth but disappears ages 4-17 because blocked by physis. Reappears after 17yo - Lateral femoral circumflex: contributes at birth but regresses later in childhood - Medial femoral circumflex: posterosuperior and posteroinferior branches. Become main blood supply at 4yo
194
What is the Delbet classification?
Proximal femur fracture classification Type 1: transphyseal. 100% AVN risk. 2: transcervical. 30% risk. 3: basicervical. 20% risk 4: intertrochanteric. 10% All displaced #s or kids >4yo need surgery. Types 1-3 are treated w pin/screws if displaced or in kids >4yo. Type 4 is treated w DHS if displaced or kids >4yo Any type can be treated w spica if undisplaced in kids <4yo
195
What are the treatment options for proximal femur #s?
- Closed reduction + spica - Emergent ORIF + capsulotomy - Closed vs open reduction internal fixation - ORIF w sliding hip screw
196
What are the indications for emergent ORIF and capsulotomy in pediatric proximal femur #s? Why is capsulotomy or hip aspiration suggested?
Indications include - Open # - Vascular injury requiring repair - Associated hip dislocation - Type 1 w epiphyseal dislocation - Fractures w significant displacement (may increased AVN risk) Capsulotomy or aspiration may decrease AVN risk
197
What are the indications for closed reduction + spica in pediatric proximal femur #s?
Only if acceptable alignment in kids <4yo, any type Kids w displaced #s or >4yo, need surgery that is dependent on # type
198
What are the indications for pin/screw fixation vs DHS for pediatric proximal femur #s?
DHS is for type 4 that are either displaced or kids >4yo Pin/screw fixation are for types 1-3 that are either displaced or kids >4yo
199
What complications are associated w pediatric proximal femur #s? (6)
1. AVN 2. Coxa vara 3. Coxa valga 4. Nonunion 5. Physeal arrest 6. Chondrolysis
200
What are the risk factors for AVN in pediatric proximal femur #s? (4)
1. Increasing age 2. Fracture type (Delbet classification) 3. Delayed reduction >24h 4. Unstable reduction
201
In pediatric proximal femur fractures, which # patterns are associated w coxa vara? Coxa valga?
Coxa vara - types 1-3 Coxa valga - type 4
202
What are the treatment options for coxa vara after pediatric proximal femur #s? What are the indications? (3)
1. Observation: kids <3yo will remodel 2. Trochanteric apophysis surgical arrest. Mild coxa vara in kids <8yo 3. Subtroch or intertroch valgus osteotomy: associated nonunion, severe Trendelenburg gait, FAI or older patients
203
RC: What is true regarding femoral neck #s? A. Rate of osteonecrosis is 5% B. It is ok to cross the physis w fixation C. Capsular decompression (?) D. Age <11yo is a risk factor for osteonecrosis
Answer: B Rate of osteonecrosis is at least 10%, depending on Delbet classification Increasing age is a risk factor Question stem didn't mention anything further regarding capsular decompression.
204
In pediatric femoral shaft #s, what are the treatment options and indications for each? (6)
205
List 5 implant-related principles of femur shaft flexinails
1. Nail size: 2 nails together must fill 80% canal 2. Pre-bend the nails to contact the canal walls at the # site. Bend 30deg. Nails should bend in opposite directions 3. End caps stabilize the nails 4. Titanium nails are better than stainless steel: better strength. Steel nails are stiffer and have higher reoperation rates 5. Insert 2 nails retrograde. Starting points is 2cm above distal physis
206
What are the advantages and disadvantages of using flexinails for pediatric femoral shaft #s?
Advantages: - Small incision, minimal soft tissue damage - Don't need to immobilize - Early WB at 6wks - Decreased hospital stay Disadvantages: - Irritation at tip - Risk shortening/angulation if length unstable - Risk malunion if very proximal or distal #s
207
What are the advantages and disadvantages of using anterograde IM nailing in pediatric femoral shaft #s?
Advantages - Immediate stability + WB - Known technique for many surgeons Disadvantages - AVN associated w piriformis entry. Use GT or lateral entry to avoid. Medial femoral circumflex lies near piriformis fossa
208
What are the indications for anterograde IM nail in the management of pediatric femoral shaft #s?
- Near skeletal maturity (>11yo) - Length unstable
209
RC: 54kg 10yo w a midshaft femur #. What is the treatment? A. Ex-fix B. Rigid IM nail C. Flexible IM nail D. Submuscular plate
Answer: D With that body weight, flexible nails are not appropriate. Rigid IM nail or plate would be possible treatments, except the stem specifies that the pt is 10yo Rigid IM nail is appropriate if the pt is >11yo Plating is okay for pretty much all ages 2015
210
What SH type # is most common in pediatric distal femur physeal injuries?
SH2
211
What is a Thurston Holland Fragment?
Distal femur physeal injury, SH2. Triangular portion of metaphysis remaining w epiphysis
212
What is the incidence of physeal injury causing LLD or angular deformity in distal femur physeal injuries? What are the risk factors?
30-50% incidence. Risk factors: - SH type - # displacement - Surgical HW invading physis
213
What are the treatment options and indications for pediatric distal femur physeal injuries? (3)
1. Long leg cast for nondisplaced #s 2. CRPP - SH 1 +2. Some SH 3 + 4 if anatomic reduction possible 3. ORIF - Most SH 3 + 4. Irreducible SH 1 + 2 due to interposed periosteum on tension (lateral) side Smooth k-wires if crossing physis. Otherwise, use screws
214
True or False: in pediatric ACL injuries, they are classically due to tibial eminence #s
True. There is increasing incidence of ACL ruptures now but classically associated w eminence #s
215
Risk factors for pediatric ACL tears? (5)
1. Increased posterior tibial slope 2. Increased Q angle 3. Decreased intercondylar notch width 4. Stronger quads then hamstrings 5. Landing position: hip IR + knee valgus
216
Indications for nonoperative management of pediatric ACL tears? (3)
1. Younger (<11yo) 2. <50% tear 3. Anteromedial bundle
217
Why is it better to fix pediatric ACL tears acutely, rather than delay until skeletal maturity?
Delaying found to increase incidence of: - Irreparable meniscal tears - Chondral damage This is due to repeated episodes of instability
218
In pediatric ACL reconstruction, what are the general types of reconstruction techniques? (4)
- Extra articular - Physeal sparing - Partial transphyseal (in tibia) - Complete transphyseal
219
Describe the concept of extra-articular ACL reconstruction in kids
No tunnels made. Autograft IT band fixed to femur and brought intra articular, then fixed to tibia. Good stability but may be over constrained
220
Describe the concept of physeal sparing (all epiphyseal) ACL reconstruction in kids
Indicated when >2yo growth left. Tunnels made in femur and tibia epiphyses. Hamstring autograft used. Best restores normal knee kinematics
221
Describe the concept of partial transphyseal ACL reconstruction in kids
Indicated when modest growth left. Spares femur physis and transphyseal tibial tunnel
222
List risk factors during ACL reconstruction in kids that may cause growth disturbance? (3)
1. HW across physis 2. Bone across physis (BPTB graft) 3. Large tunnels in physis
223
True or False: autograft is best in pediatric ACL reconstruction
True. Autograft is best. Usually quadrupled hamstring used. Bone blocks increase risk of growth arrest. Allograft associated w 4x revision rate.
224
In patella dislocations, list what you should examine or workup to determine the cause of recurrent instability
- Standing alignment - Axial alignment: TT-TG - Rotational alignment: rotational profile - Trochlear groove morphology: patellar tilt, lateral femoral condyle dysplasia - Generalized ligamentous laxity - Soft tissue: MPFL tears, tight lateral retinaculum
225
Which is better in recurrent pediatric patella dislocations, MPFL reconstruction or repair?
Reconstruction is better (5% recurrence). Repair alone does poorly (60%) recurrence.
226
In pediatric MPFL reconstruction, where is the femoral origin?
Between medial epicondyle and adductor tubercle. About 5mm distal to physis
227
In pediatric patella dislocations, what are risk factors for recurrence? (6)
1. Age <18 2. Trochlear dysplasia 3. Patella alta 4. Increased TT-TG 5. Miserable malalignment: femur anteversion, genu valgum and external tibial torsion 6. Female
228
True or False: autograft is best for pediatric MPFL reconstruction
False. Equal results with autograft or allograft for MPFL reconstruction. This is unlike with ACL, where autograft is always best for kids
229
RC: In a 10year old boy w recurrent patella instability and a TTTG measuring 26mm, what of the following is the least recommended: A. Medial patellofemoral soft tissue reconstruction B. Supra-patellar soft tissue realignment procedure C. Tibial tubercle osteotomy
Answer: C Do not do tibial tubercle osteotomy in skeletally immature
230
In patellar sleeve fractures, what is most common: superior pole or inferior pole fractures?
Inferior pole is most common
231
What are some clinical findings in kids w patellar sleeve #s? (4)
1. Unable to WB 2. High-riding patella (if inferior pole #, which is most common) 3. Palpable gap at either proximal or distal end of patella due to disrupted extensor mechanism (depends on location of #) 4. Difficulty w active knee extension
232
What are XR findings w patellar sleeve #s?
Flecks of bone near superior or inferior poles Patella baja or alta depending on where the # is
233
What is the treatment of patella sleeve #s?
2 options 1. Cast x6wks: rare. Only if undisplaced w intact extensor mechanism 2. ORIF: displaced, articular stepoff, disrupted extensor mechanism Medial parapatellar approach. Repair retinaculum and extensor mechanism with sutures, tension band, suture anchors, screws, etc Cast in extension after
234
What structure attaches to the medial tibial spine?
ACL
235
Why are kids more prone to tibial eminence #s?
Not completely ossified. More prone to eminence #s than cruciate ligament tears
236
What is the Meyers and McKeever classification?
Pediatric tibial eminence fractures Type 1: nondisplaced Type 2: minimally displaced w intact posterior hinge Type 3: completely displaced. Type 3+ is completely displaced and rotated Type 4: completely displaced, rotated and comminuted
237
What are the clinical findings in kids w tibial eminence #s?
Knee flexed Positive anterior drawer
238
What are the treatment options and indications for pediatric tibial eminence #s? (2)
1. Closed reduction and full extension cast: type 1 and type 2 (if reducible) 2. Fixation: Types 3 + 4, and irreducible type 2
239
What are common blocks to reduction in tibial eminence #s?
Meniscus and intermeniscal ligament Most common: medial meniscus
240
Describe the fixation technique for pediatric tibial eminence #s
Arthroscopic vs open Debride # and disengage entrapped soft tissue Reduce and fix with either suture or screws
241
What are the advantages and disadvantages of suture or screw fixation in treating pediatric tibial eminence #s?
Suture fixation - Advantage: use for smaller fragments. Minimal physeal damage - Disadvantage: more technically demanding arthroscopically Screw fixation - Advantage: less technically demanding, possibly earlier mobilization - Disadvantage: for larger fragment, HW irritation, may impinge ROM, iatrogenic comminution, physeal injury
242
True or False: surgically treated pediatric tibial eminence #s are associated with ACL laxity
True. ACL laxity after tibial eminence # is more common after surgical treatment. Laxity is noted on Lachman. However, usually functionally stable ACL reconstruction later in 15-25%
243
RC: What is the most important factor in causing arthrofibrosis in tibial eminence injuries in kids? A. Open fixation, instead of arthroscopic B. Length of immobilization C. Screw fixation, rather than suture D. Injury to meniscus
Answer: B Early ROM decreases the risk of arthrofibrosis
244
What are risk factors associated w tibial tubercle #s?
Jumping shorts: basketball, football, sprinting, high jump
245
Describe how the ossification centers of the proximal tibia close
2 ossification centers: physis and tubercle apophysis Closes from posterior to anterior, proximal to distal
246
In tibial tubercle fractures with associated compartment syndrome, what else may be injured?
Anterior tibial recurrent artery
247
True or False: tibial tubercle #s may be associated w popliteal artery injury
True. Passes posterior over metaphyseal fragment. CTA if suspect injury
248
What is the Ogden classification?
Tibial tubercle # 1: through apophysis 2: between physis and apophysis 3: through apophysis and extends posterior to cross physis 4: through entire physis 5: proximal sleeve avulsion
249
What are the treatment option for tibial tubercle #s?
- Cast in extension: type 1, minimally displaced or acceptable displacement - ORIF: types 2-5 displaced. Fix with smooth K-wires, screws and sutures
250
What deformity may result from tibial tubercle #s?
Recurvatum deformity. More common than LLD Due to anterior growth arrest but posterior growth continues. Results in decreased tibial slope
251
RC: What is a complication of tibial tubercle #s? A. Compartment syndrome B. Something C. Something D. Something
Just know that compartment syndrome is a complication. Due to tear of recurrent anterior tibial artery 2017
252
What are acceptable alignment parameters for pediatric tibial shaft #s?
<5-10deg angulation. Accept 10deg angulation if <8yo <1cm shortening 50% translation
253
What are treatment options for pediatric tibial shaft fractures?
- Long leg cast: acceptable reduction - Ex-fix: DCO, extensive soft tissue injury - Flexinails: unacceptable alignment, near maturity
254
Describe the order of closure of the distal tibial physis
Central > anteromedial > posteromedial > latera.
255
What is a triplane #?
Transitional # due to distal tibial physeal closure. Typically metaphyseal # on coronal plane, physeal # on axial plane, epiphyseal # on sagittal plane Can be different # patterns but this is the classic
256
With triplane #s, which components of the # would you see on AP and lateral XR views?
AP: epiphyseal # as it is a sagittal plane #. SH3 type Lateral: metaphyseal # as it is a coronal plane #. SH2
257
With triplanar #s, what radiologic sign may be seen on CT?
Mercedes-Benz sign
258
Describe the technique for closed reduction and casting of triplanar #s
Indication: <2mm displaced Reduce the fibula first Lateral triplanar #s: most common type. Reduce w IR Medial triplanar #s: reduce w ER Long leg cast to control rotation x4wks, then short leg cast x2wks
259
Describe the technique for operative treatment of triplanar #s
Indication: >2mm displaced CRPP vs ORIF Approach for ORIF depends if lateral or medial triplanar # Lateral triplanar: anterolateral approach Medial triplanar: anteromedial approach Fixation: smooth k-wires or cannulated screws
260
RC: What is true of a triplanar # on XR? A. SH3 # seen on AP view, SH2 # seen on lateral view B. SH2 seen on AP, SH3 seen on lateral C. SH4 on AP view D. SH4 seen on mortise view
Answer: A SH3 # line seen on AP view. # is in the sagittal plane SH2 # line seen on lateral view. # is in the coronal plane
261
What is a tillaux fracture?
SH3 # of anterolateral tibial epiphysis due to AITFL avulsion. Occurs within 1yr of physis closure, older then triplanar group
262
What does the AITFL connect to?
Chaput tubercle on tibia and Wagstaffe tubercle on fibula
263
What is the best XR view to visualize a tillaux #?
Mortise view
264
Describe the technique for closed reduction and casting in tillaux #s?
Indication: <2mm displaced Reduction technique: IR foot Long leg cast x4wks then short leg x2wks
265
Describe the operative technique for tillaux #s
Indication: >2mm displaced CRPP: k wire to joystick reduction then fix w k wire or cannulated screws ORIF: anterolateral approach. Visualize joint for reduction. Transphyseal fixation is OK as physis almost closed Postop: long leg cast x4wks for rotational stability, then short leg cast x2wks
266
What is juvenile idiopathic arthritis?
Autoimmune inflammatory arthritis lasting >6wks in kids <16yo Must R/O infection Diagnostic criteria: must have one of the following - Rash - RF positive - Iridocyclitis - C-spine involved - Pericarditis - Tenosynovitis - Intermittent fever - Morning stiffness
267
True or False: juvenile idiopathic arthritis is often RF positive
False. <15% are RF-positive RF positive is included in the diagnostic criteria though
268
What is iridocyclitis and why is it important to R/O in JIA?
Type of anterior uveitis. Needs immediate ophtho consult for slit lamp exam. Can cause rapid vision loss. Higher risk if ANA positive ANA+: requires slitlamp exam q4mo ANA-: slitlamp exam q6mo
269
What is Still's disease?
Acute onset JIA presenting with - Multiple joint involvement - Fever - Rash - Splenomegaly Usually 5-10yo
270
What are the 3 types of JIA? Which is most common and which has the worse prognosis?
Pauciarticular: most common (50%) Polyarticular Systemic: worse prognosis
271
What is the difference between pauciarticular and polyarticular JIA?
Pauciarticular - <5 joints involved - Large joints - Asymmetric: causes LLD. Involved limb is longer - Early onset is more common in girls x4 and associated w iridocyclitis Polyarticular - >5 joints involved - Small joints (hand/wrist)
272
What is the prognosis of JIA?
Pauciarticular: 70% remission Polyarticular: 60% remission Systemic: worst. Most likely to become adult RA
273
What systemic findings are associated w systemic JIA?
- Rash - Fever - Multiple joints - Hepatosplenomegaly - Lymphadenopathy - Pericarditis - Heme: anemia, high plts/WBC - Stills disease
274
What must be done preop for JIA?
Flex/ex XR for atlantoaxial instability
275
RC: What are radiographic findings in the c-spine in JIA?
1. Atlantoaxial instability 2. Basilar invagination 3. Odontoid erosion 4. Facet (apophyseal) joint ankylosis 5. Subaxial instability 6. Small cervical vertebrae (hypotrophy): most common at C4/5
276
What is the treatment for JIA?
DMARDS and slitlamp exams Ortho-wise: treat LLD and deformities. May need arthrodesis or arthroplasty
277
What is the O'Brien classification of pediatric radial head #s?
Type 1: <30deg angulation Type 2: 30-60 Type 3: >60 deg
278
What XR view should you order to better assess radial head #s?
Radiocapitellar view. Oblique later XR performed by placing arm on table w elbow flexed 90deg. Thumb pointing upwards
279
What are the indications for nonoperative management of pediatric radial head #s?
Able to achieve acceptable alignment w closed reduction. - <30deg angulation - <3mm translation
280
Describe closed reduction techniques for pediatric radial head #s (3)
Patterson maneuver: elbow in extension/supination. Traction + varus force. Direct pressure over head Israeli: elbow flexed to 90 and pronation. Direct pressure over head Esmarch: tightly apply Esmarch from wrist to above elbow. May spontaneously reduce
281
Describe CRPP techniques for pediatric radial head #s (3)
1. K-wire push technique: use blunt head to push against fragment 2. K-wire lever technique: kwire into fracture site to lever 3. Metaizeau technique: pin/nail retrograde across # site. Rotate pin/nail to reduce # Pin # if unstable
282
Describe the open reduction technique for pediatric radial head #s
Kocher approach. Internal fixation only if grossly unstable
283
True or False: in pediatric radial head #s, loss of pronation is a much more common complication than loss of supination
TRUE
284
True or false: in pediatric radial head #s, the incidence of osteonecrosis is increased w open reduction
True. 70% incidence w open reduction. Radial head is mostly cartilage in kids. Blood supply from metaphysis
285
RC: What are poor prognostic factors in radial neck #s in kids?
1. Older (>10yo) 2. Associated injuries, comminution 3. Requires open reduction 4. Delayed treatment 5. Poor reduction (>30deg angulation, 3mm translation)
286
What is a Nursemaid's elbow and what age group is it found in?
Annular ligament subluxation after sudden traction on extended and pronated elbow Found in kids 1-4yo
287
When suspected Nursemaid's elbow, what position is the patient's arm usually held? What is the physical exam like?
Arm held in slight flexion and pronation Pain to lateral aspect of elbow. Full flexion and extension Pain w supination
288
Describe the closed reduction techniques for Nursemaid's elbow (2)
-Supination technique: supinate forearm and flex elbow maximally. Put pressure on radial head to reduce - Hyperpronation: elbow flexed to 90deg and hyperpronate forearm
289
In pediatric elbow dislocations, what is the most commonly associated #?
Medial epicondyle #
290
True or False: elbow dislocations are common in kids <3yo
False. Most common in 10-15yo. "Elbow dislocation" in kids <3yo, suspect physeal separation from NAT
291
What is Sprengel's? Describe the associated deformity
Congenitally small + undescended scapula High-riding Medially rotated scapula (glenoid faces inferior) Triangle shape
292
What motion is typically limited in Sprengel's?
Shoulder abduction. Due to loss of scapulothoracic motion
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True or False: Sprengel's is the same as scapular winging
False. Sprengel's is a congenitally small and undescended scapula. It may be associated w winging but not necessarily. Winging is from injury to the long thoracic n or serratus anterior
294
What is an omovertebral bone?
Present in 50% of Sprengel's deformity. Forms a connection between the scapula and C-spine. Restricts shoulder motion
295
What conditions are associated w Sprengel's? (3)
1. Congenital scoliosis: most common 2. Klippel-Feil 3. Spinal dysraphism: spina bifida, split cord, etc
296
For operative management of Sprengel's, name the 2 procedures that can be performed and general principles of each. What are your options in regards to protecting the brachial plexus?
1. Green procedure 2. Woodward procedure - more popular Both procedures are similar. Position prone. Incision over Cspine spinous processes. In Green procedure, detach muscles from scapula for exposure. In Woodward, detach muscles from spinous processes for exposure. Excise omovertebral bone. Externally rotate scapula and move inferior. Reattach muscles Options to protect the brachial plexus - Clavicle resection - Clavicle osteotomy
297
What are the outcomes of operative management for Sprengel's?
Both Green and Woodward procedures improve abduction by 45deg
298
What nerves innervate pec major? Where do they branch off of on the brachial plexus?
Medial and lateral pectoral n. From medial and lateral cords, respectively
299
What are the risk factors for obstetrical brachial plexopathy? (6)
1. Large for gestational age 2. Multiparous pregnancy 3. Difficult presentation (breech) 4. Shoulder dystocia 5. Forceps delivery 6. Prolonged labor
300
Define preganglionic brachial plexus injury vs postganglionic
Preganglionic: lesion is proximal to sensory (dorsal root) ganglion
301
What are the implications of preganglionic brachial plexus injuries?
Preganglionic injuries involve root avulsions and will not spontaneously recover. Require nerve transfer
302
In obstetrical brachial plexopathy, certain nerve injuries suggest preganglionic injury. List them (5)
1. Horner's syndrome: sympathetic chain 2. Phrenic n: elevated hemidiaphragm on XR 3. Long thoracic n: winged scapula from serratus anterior 4. Dorsal scapular n: rhomboids 5. Suprascapular n: supra and infraspinatus
303
What nerve roots are involved in Erb's, Klumpke's, and total obstetrical brachial plexopathy? Which is most common
Erbs: C5-6 (sometimes C7) Klumpke: C8-T1 Complete: with or without Horner's Erbs is most common > complete > Klumpke Note: these terms are outdated. Now, we use the Narakas classification
304
What is the Narakas classification?
Obstetrical brachial plexopathy. Eponym classification (Erb's, Klumpke) is outdated Group 1 (Duchenne-Erb's): C5-6 Group 2 (Intermediate): C5-7 Group 3 (Total): C5-T1 without Horner's Group 4 (Total w Horner's)
305
What major nerves are involved in Erb's palsy? What is the typical deformity?
C5-6. C7 involved in 50% Suprascapula, musculocutaneous, axillary, and radial n Waiter's tip deformity - Adducted and IR shoulder - Extended and pronated elbow - If C7 involved, no wrist extension. Will be fixed in flexion
306
What neonatal reflexes should be examined in obstetrical brachial plexopathy?
1. Moro: sudden loss of support. Spread out arms (abduction) and then pull back in (adduction) 2. Tonic neck reflex: aka fencing reflex. When face turned to one side, arm/leg on that side become extended. Opposite arm/leg flex
307
Describe the general principles of managing obstetrical brachial plexopathy
1. R/O pseudoparalysis from trauma, meaning is this true plexopathy? 2. Neonatal reflexes 3. Determine roots involved. R/O Horner's. Don't need EMG/NCS (unlike adults) 4. PT to maintain ROM. Don't need splinting (unlike in adults) 5. Serial exams over first 3-6mo of life. Monitor biceps recovery w cookie test 6. If no biceps recovery by 3-6mo, send to plastics for microsurgery. If preganglionic, can send at 3mo. If not preganglionic, can wait until 6mo to refer 7. After recovered from nerve surgery (wait at least 2yrs), assess remaining functional deficits for tendon transfers, etc
308
List 2 motor grading systems developed to assess obstetrical brachial plexopathy
1. Active Movement Scale. Use to track neuro recovery. Motor graded from 0-7. Grades 0-4 if gravity eliminated, grades 5-7 if movement possible against gravity 2. Mallet classification. Assesses 5 functions: shoulder abduction, shoulder ER, hand to neck, hand to mouth, hand to sacrum
309
What is the cookie test?
Assess biceps recovery in obstetrical brachial plexopathy. Place cookie in hand of affected side. Hold elbow adducted. If able to bring cookie to mouth, then has biceps function
310
What are good prognostic factors for obstetrical brachial plexopathy? (2)
1. Erb's palsy 2. Biceps activity by 2mo. Specifically, antigravity activity
311
What are poor prognostic factors for obstetrical brachial plexopathy? (4)
1. Klumpke's or complete palsy 2. No biceps activity by 3mo 3. Preganglionic injury 4. Horner's syndrome
312
What is Horner's syndrome?
Ptosis, miosis, anhidrosis
313
How do you workup phrenic n involvement in obstetrical brachial plexopathy?
Chest fluoro or US
314
What are the indications for nerve transfer in obstetrical brachial plexopathy? Indications for nerve grafting?
Nerve transfer for preganglionic Nerve grafting for postganglionic
315
How long should you wait after microsurgery for obstetrical brachial plexopathy before proceeding to tendon transfer?
Wait at least 2yo to ensure no more recovery after nerve surgery
316
What is the overall prognosis for obstetrical brachial plexopathy without intervention?
90% will resolve without intervention
317
What is the most common nerve graft used for obstetrical brachial plexopathy?
Sural n
318
Describe the principles of nerve grafting for Erb's palsy
Indications - postganglionic. No biceps return after 6mo - Cable graft w sural n - Resect neuroma - Graft C5 to suprascapular and posterior division of superior trunk (axillary n) - Graft C6 to the anterior division of superior trunk (musculocutaneous)
319
Describe the principles of nerve transfer for Erb's palsy
- Spinal accessory n (CN 11) transferred to suprascapular n - Thoracic intercostals (T2-4) transferred to lateral and posterior cords
320
Describe the principles of nerve surgery for total obstetrical brachial plexopathy
Priority is hand function Graft C5 and C6 nerve roots to medial and ulnar n Transfer spinal accessory n (CN 11) to suprascapular n Transfer thoracic intercostals (T2-4) to lateral and posterior cord
321
What shoulder deformities result from Erb's palsy? (2)
1. IR contraction 2. Dislocation from posterior subluxation and glenoid retroversion
322
In Erb's palsy, what are the treatment options for shoulder subluxation and glenoid deformity? (2)
- Minimal glenoid deformity: open reduction, subscap release. Possible lat dorsi transfer - Retroverted glenoid deformity: humeral derotational osteotomy
323
In Erb's palsy, what are the treatment options for shoulder IR contracture? What are the indications? (4)
1. Subscap release: failed PT (<30deg ER) by 1yo, no glenoid dysplasia 2. Pec major release +/- lat dorsi transfer: failed PT by 2yo, no glenoid dysplasia 3. Humeral derotational osteotomy: persistent contracture + glenoid dysplasia 4. Arthrodesis: nonfunctional deltoid but good hand/wrist fxn
324
Describe the principles of humeral derotational osteotomy
Indication: persistent IR contracture with glenoid dysplasia Deltopectoral approach Transverse osteotomy just proximal to deltoid insertion on humerus. Protect radial n just posterior Position distal humerus in 30deg ER Plate fixation
325
Shoulder arthrodesis: what is the position of fusion?
30-30-30 Abduction 30 deg Forward flexion 30deg IR 30deg Goal: hand to mouth
326
In Klumpke's palsy, what are the treatment options for elbow flexion contracture? (2)
1. Splinting - Night time extension splint to prevent progression - Elbow extension casting when contracture >40deg 2. Biceps and brachialis lengthening
327
In Klumpke's palsy, what are the treatment options for forearm supination contracture? What is the goal? (2)
Goal: 20deg supination. Allows gravity assisted wrist flexion, which aids in digit extension via tenodesis 1. Biceps tendon rerouting transfer: distal insertion rerouted around radial neck and sutured to itself, to act as pronator instead of supinator. Indications: intact passive pronation. 2. Forearm osteotomy and biceps rerouting: when passive pronation is limited
328
RC: Which of the following is true of obstetric brachioplexopathy? A. Phrenic n injury is indicative of root avulsion B. Horner's is usually associated w upper plexus injury C. Limited biceps flexion at 3mo is indicative of neurotmesis D. Neurotmesis is usually repairable
Answer: A (phrenic n is indicative of root avulsion) Root avulsion is preganglionic Horner's is associated w lower trunk injuries 2017
329
What are the risk factors for DHH? (5)
1. First born 2. Female 3. Frank breech 4. Family hx 5. Oligohydramnios
330
What is a teratologic hip? What is the treatment?
Related to DDH. Dislocated in utero and irreducible. Have pseudoacetabulum Treatment: open reduction. Don't use Pavlik or closed reduction
331
What conditions are associated w a teratologic hip?
1. Arthrogryposis 2. Lumbosacral agenesis 3. Diastrophic Dwarfism 4. Larsen's 5. Chromosomal abnormalities
332
What is the typical acetabular deficiency in DDH? In CP?
DDH: anterolateral CP: Posterosuperior
333
List the soft tissue blocks to reduction in DDH (7)
LLLPPCT 1. Labrum 2. Limbus 3. Ligamentum teres 4. Pulvinar 5. Psoas tendon 6. Capsule 7. Transverse acetab ligament RC question
334
List common packaging disorders (7)
1. Congenital torticollis 2. DDH 3. Congenital knee dislocation 4. Posteromedial tibial bowing 5. Calcaneovalgus foot 6. Club foot 7. Metatarsus adductus
335
After what age are Barlow and Ortolani tests no longer useful?
3mo After this, soft tissue tighten. These tests will not be useful
336
After 3mo, what is the most sensitive physical exam test for DDH?
Decreased hip abduction. Cannot use Ortolani or Barlow tests after 3mo due to soft tissue tightening
337
Regarding imaging modalities for DHH, at what age range should you use US? When should you start using XR instead?
US useful at 4wks to 4mo >4mo, use XR. Head ossifies. Can't see through ossified head on US
338
What are the treatment options for DDH when the child is <6mo?
Pavlik harness is the only option
339
What are 3 parameters you should measure on US for DDH?
1. Alpha angle: between acetab and ilium. Should be >60deg 2. Beta angle: between labrum and ilium. Should be <55deg 3. Head coverage %
340
RC question: List 6 XR findings of DDH before 6mo old
Acetabulum 1. Acetabular Index >25deg 2. Widened teardrop 3. Rounded acetabular corners Head 4. Delayed femoral head ossification 5. Head not inferomedial to Hilgenreiner and Perkin's lines 6. Disrupted Shenton's line Bonus - coxa valga
341
What is the Tonnis classification in DDH?
XR classification based on femoral head displacement relative to Perkin's line and superior acetab rim In DDH, head displaces laterally and superiorly Grade 1: Head is reduced. Medial to Perkin's Grade 2: Lateral to Perkin's but below acetab rim Grade 3: Level w acetab rim Grade 4: above acetab rim
342
In DDH, what are the implications of increasing Tonnis grade?
Each increase in Tonnis grade doubles the likelihood of failing nonop
343
What is a Pavlik Harness and what movements does it restrict?
Dynamic abduction brace. Prevents extension, adduction and IR
344
What are the indications for using a Pavlik Harness in DDH?
<6mo and reducible head
345
How often should the Pavlik Harness be worn for DDH and what is the duration of treatment?
Controversial. Some say wear for 23h/d for 6wks. Then wean out for another 6wks. During treatment, F/U q2mo w repeat exam and US. Continue F/U until maturity for recurrent instability or acetab dysplasia
346
Describes the straps involved in a Pavlik Harness and how to use them
Chest strap: at nipple line Anterior strap: flexion Posterior strap: abduction
347
Describe the ideal positioning of the legs in a Pavlik Harness. What complications are associated w its use?
90-100deg flexion, 45deg abduction Complications include - Transient femoral n palsy: from hyperflexion. Won't see kid kicking (knee extension) - AVN: from hyperabduction >60deg due to impinging on medial femoral circumflex - Brachial plexus palsy: from shoulder straps
348
When using a Pavlik Harness, what is the stable zone? What is the safe zone? What can you do to increase them?
Stable zone: degrees of abduction between dislocation and max abduction possible Safe zone: degrees of abduction between dislocation and safe amount of abduction Can increase your zone w adductor tenotomy to increase abduction
349
After starting a Pavlik Harness of DDH, when should you followup next and why?
Should followup after a week to make sure family is using the harness correctly and see if hip reduced. If hip is not reduced after 3wks, need to stop the harness. Otherwise, risk Pavlik Harness disease
350
What is Pavlik Harness disease?
Develops if harness is worn but hip is not reduced. Dislocated hip causes posterosuperior acetab wear.
351
RC: A child w DDH is treated in a Pavlik harness and develops femoral n palsy. What is not true? A. Can present bilaterally B. Leads to increased need for open procedure C. 60% resolve spontaneously D. 2.5% incidence
Answer: C 100% resolve spontaneously Most occur within 1wk. Indeed 2.5% incidence Risk factors: older kid, larger kid, more severe dysplasia
352
In DDH, what should you do if the hip remains dislocated with Pavlik Harness?
Discontinue if not in hip after 3wks to prevent Pavlik Harness disease. Plan for closed reduction when kid is 4mo (safe for anesthesia)
353
If the Pavlik Harness is successful in keeping DDH hip reduced, what are some radiographic signs suggesting acetab remodeling? (3)
1. Acetab Index decreases 2. Smooth horizontal sourcil 3. U-shaped tear drop
354
Describe the bony abnormalities in DDH hips (4)
1. Shallow anteverted acetab 2. Excessive femoral anteversion 3. Delayed head ossification 4. Coxa valga
355
What gait patterns are associated with DDH? (2)
1. Trendelenburg: abductor insufficiency from dislocated hip 2. Toe walking: compensate for shortened affected side
356
What are Hilgenreiner's and Perkin's lines? Where should the head normally sit in relation?
Hilgenreiner: horizontal line through triradiate Perkin's line: perpendicular to floor, along lateral acetab edge Head should be inferomedial to these lines
357
In the hip, what is Shenton's line?
Arc along femoral neck and obturator foramen. It is discontinuous if hip is dislocated
358
What is the acetabular index? What does it mean?
Angle formed between - Hilgenreiner's line - Line from triradiate to lateral acetab margin Normal is <25deg Measures inclination of acetab roof
359
What is the center edge angle? What does it mean?
Angle between - Vertical line through head - Line from head center to lateral acetab margin Normal >20deg Measures acetab coverage
360
How does the teardrop sign change in DDH?
If subluxed/dislocated, widened. Becomes U-shaped when hip is reduced
361
What are the treatment options for DDH in kids after failing Pavlik or late presentation?
1. Closed reduction + spica 2. Open reduction +/- femoral shortening derotational osteotomy +/- pelvic osteotomy
362
What are the indications and contraindications of closed reduction/spica for DDH?
Indications - Failed Pavlik (Do closed reduction >4mo when safe for anesthesia) - Presented too late for Pavlik Contraindication: teratologic hip (needs open reduction)
363
Describe the principles of closed reduction and spica cast for DDH
- Reduction technique: Ortolani - Arthrogram - Adductor tenotomy if too tight - Spica - MRI postop
364
Describe how to do a hip arthrogram for DDH closed reduction?
50/50 mix of radio-opaque dye and NS. Inject 2cc w 18gauge needle Abduct hip and palpate adductor longus. Place needle inferior to adductor longus at head/neck junction. Aim for ipsilateral shoulder Assess medial dye pool. If <5mm, likely reduced. If >7mm, not reduced
365
How is the spica cast positioned for DDH?
"Human position" 90deg flexion and 45 abduction. Neutral rotation (Bonus fact: hip only rotated in spica for proximal femur #. Needs IR)
366
After successful closed reduction and spica cast application for DDH, what is the followup? How long should the spica be on for? What is the management after?
Change spica after 6wks Should have spica cast on for 3mo Then abduction brace for 2mo (1mo full time, 1mo night time)
367
On MRI, how do you determine if the hip is reduced in DDH?
Line from pubic rami should intersect w proximal femur metaphysis
368
Describe the principles of open reduction for DDH
1. Bikini incision, Smith-Pete approach 2. Identify the true acetabulum: iliopsoas will constrict capsule into hourglass shape. Cut iliopsoas. T capsulotomy 3. Remove soft tissue blocks to reduction: limbus, lig teres, pulvinar, transverse acetab lig 4. Reduce head. Should be deep and concentric - If all good, do capsulorrhaphy - If can't unable to reduce or tight reduction: do femoral osteotomy - If reduced but dysplastic acetab (so reduction is not deep and concentric), do pelvic osteotomy if kid >18mo
369
Describes the principles for femoral derotational shortening osteotomy in DDH. What are the indications?
Indications: - Unable to reduce hip during open reduction - Tight fit once reduced. Risk of AVN - Also corrects excessive femoral anteversion Technique - Transverse intertrochanteric osteotomy - Reduce head + remove overlapping bone - Derotate anteversion by ER femur - Fix w 4 hole plate
370
True or False: femoral shortening osteotomy in DDH reduces AVN rate
True. Decreases to 0-10% rate
371
What are the indications for a pelvic osteotomy in DDH? (2)
1. >18mo. Unlikely to remodel 2. Severe dysplasia. High AI and deficient anterolateral coverage
372
Which pelvic osteotomies are commonly used for DDH? What are your considerations for each? (3)
1. Salter: redirectional, for mild dysplasia. Provides anterolateral coverage but uncovers posteriorly. Don't do for CP or shallow pelvis 2. Dega: acetabuloplasty, so for shallow and large acetab. Global coverage, so can use for CP 3. Pemberton: acetabuloplasty, so for shallow and large acetab. Anterolateral coverage
373
What are the 3 categories of pelvic osteotomies. Define each
1. Redirectional. Shifts acetabulum but preserves its shape/volume 2. Acetabuloplasty. Changes acetab shape/volume. Incomplete osteotomies, coverage is w own articular cartilage 3. Salvage. Changes acetab shape/volume. Uses extra-articular bone to augment existing acetab, forms fibrocartilage
374
What are the contraindications for redirectional pelvic osteotomies? (2)
1. Shallow acetab 2. Non-concentric reduction (If shallow acetab, better to do acetabuloplasty or salvage)
375
List the 3 pelvic osteotomies that rely on a cartilaginous hinge
1. Salter: pubic symphysis 2. Dega: triradiate 3. Pemberton: triradiate
376
List the 3 types of redirectional pelvic osteotomies. At what age rage are each typically done and why?
1. Salter: done before 8yo, because requires pubic symphysis as hinge. After 8yo, becomes too stiff 2. Triple: done in adolescents. Pubic symphysis is too stiff for Salter. Triradiate is still open so can't do PAO (Ganz) 3. PAO: done after triradiate fusion because osteotomy goes through triradiate. This is why its a common non-salvage osteotomy done in adults
377
Which non-salvage pelvic osteotomy can be performed in adults and why?
Triple and Ganz Don't require mobile pubic symphysis (Salter) or open triradiate (Dega, Pemberton)
378
What are the indications and contraindications for a Salter pelvic osteotomy? What are the general techniques?
Indication: mild dysplasia in kids <8yo (mobile pubic symphysis) CI: CP DDH due to deficient posterior coverage, shallow acetab Osteotomy from sciatic notch to above AIIS. Iliac crest bone graft wedge into osteotomy site
379
What are the indications and contraindications for a Triple pelvic osteotomy? What are the general techniques?
Indications: adolescents and older CI: shallow acetab Technique: Salter osteotomy and cuts in superior and inferior rami
380
Which redirectional pelvic osteotomy does not violate the sciatic notch?
PAO
381
What are the indications and contraindications for PAO? What are the general techniques?
Indications: closed triradiate CI: open triradiate. Shallow acetab Technique: 4 cuts in superior and inferior rami, incomplete cut in ilium between ASIS/AIIS, and vertical in ischium. Doesn't violate sciatic notch
382
What are the 2 types of acetabuloplasty?
Pemberton and Dega
383
What are the indications and contraindications for acetabuloplasty? What are the general techniques?
Difference between Pemberton and Dega: Pemberton involves only anterolateral roof, Dega involves whole acetab roof (so longer cut) Indications: shallow acetab requiring shape/volume change CI: closed triradiate (hinge) Technique: Incomplete cut from just above AIIS towards sciatic notch, without violating. Wedges placed. No fixation needed
384
What are the 2 types of salvage pelvic osteotomies?
Shelf and Chiari
385
What are the general principles of a Shelf osteotomy?
Provides anterolateral coverage Strips of bone graft taken from outer ilium. Length determined by desired CEA Strips are wedged above acetab. Held together by repairing reflected rectus head Complication: graft may resorb
386
What are the general principles of a Chiari osteotomy?
Provides anterolateral coverage Osteotomy from sciatic notch to AIIS. Medialize acetabulum. Internal fixation.
387
What is the definition of coxa vara? What are possible causes?
Neck-shaft angle <120deg 3 main causes - Congenital: associated w PFFD - Acquired: trauma, infection, SCFE, Perthes - Developmental: ossification defect in medial femoral neck
388
What is the cause of developmental coxa vara?
Due to ossification defect in medial femoral neck. Unknown etiology - WB causes progressive varus deformity, which further increases compression at medial neck - Deformity causes vertical proximal physis. Increases shear force across physis Presents in early childhood after starting to WB
389
How do patients present w developmental coxa vara?
- Presenting after walking has begun - Painless. Waddling, Trendelenburg gait (coxa vara decreases abductor tension and causes weakness) - LLD - Prominent (high riding) GT - Restricted ROM
390
What are key XR findings for developmental coxa vara? (5)
1. Neck shaft angle <120 2. Short femoral neck 3. Vertical physis 4. Inverted Y sign - triangular metaphyseal fragment at inferior neck. Pathognomonic 5. Hilgenreiner-epiphyseal angle. Normal <25. Coxa vara >25
391
What is the Inverted Y sign on hip XR?
AP view. Triangular metaphyseal fragment at the inferior femoral neck. Pathognomonic for developmental coxa vara
392
What is the Hilgenreiner-epiphyseal angle?
For developmental coxa vara On AP XR. Angle between - Hilgenreiner's line - Line through proximal femoral epiphysis Normal <25deg Coxa vara >25deg
393
How is the Hilgenreiner-epiphyseal angle used to prognosticate developmental coxa vara?
Correlates w risk of progression <45deg: unlikely to progress 45-60: indeterminate >60: likely to progress
394
What are the treatment options for developmental coxa vara? What are the indications for each? (2)
1. Observation. Indications: H-E angle <60deg. Should closely follow H-E angles between 45-60deg as risk of progression is indeterminate 2. VDRO (valgus derotation osteotomy). Indications - H-E angle >60deg - He angle 45-60 but with Trendelenburg gait, hip abductor fatigue or angle progression
395
Describe the general principles of valgus derotation osteotomy for developmental coxa vara
- Direct lateral approach - Likely need adductor tenotomy - Osteotomy: intertroch or subtroch. Do not do at neck (poor outcomes) - Overcorrect varus to minimize recurrence - Correct version - Blade plate - Can also do GT epiphysiodesis to prevent recurrence - Hip spica x6wks
396
What is the rate of recurrence after VDRO for developmental coxa vara?
Up to 50%. Decrease w overcorrection
397
What is the rate of premature physeal closure after VDRO for developmental coxa vara?
Up to 90% within 2yrs
398
What is Legg-Calve-Perthes disease?
Idiopathic osteonecrosis of the femoral head. Mostly in boys, 5-8yo
399
Perthes is a diagnosis of exclusion. What is the DDx? (5)
1. Septic hip 2. Sickle cell 3. Corticosteroid therapy 4. Skeletal dysplasia - suspect of both hips at same stage of disease 5. Mucopolysaccharidoses
400
What are risk factors and associated conditions of Perthes?
Risk factors 1. Famhx 2. Low birth weight 3. Abnormal birth presentation 4. 2nd hand smoke Associated conditions 1. ADHD (30%) 2. Delayed bone age (90%)
401
What is the clinical presentation of Perthes?
- Insidious groin pain and limp - Limited abduction and IR - Mild LLD
402
What are key XR findings in Perthes? (9)
1. Widened joint space: cartilage grows but no ossification 2. Crescent sign 3. Smaller head 4. Head collapse (loss of height) Head at risk signs 5. Gage sign: V-shaped lucency 6. Lateral epiphysis calcification 7. Lateral femoral head subluxation 8. Horizontal physis 9. Metaphyseal cyst
403
What is the Waldenström classification?
Radiographic stages of Perthes 1. Initial: sclerotic epiphysis. Widened joint space. Cartilage grows but no ossification 2. Fragmentation: lasts 1yr. Crescent sign from subchondral #. Collapse. Bisphosphonates may help here 3. Reossification 4. Remodeling
404
RC: What are prognostic factors for Perthes? (10)
1. Age of onset: worse if >8 2. Gender: worse if female 3. Extent of head involved. Catterall classification. Extensive = bad 4. Extend of deformity and joint incongruity. Stulberg classification. Aspherical, noncongruous = bad 5. Extent of subchondral resorption. Salter-Thompson classification. Crescent sign >50% of head = bad 6. >2 Catterall head at risk signs 7. Lateral pillar height (aka Herring classification). Herring C = bad 8. Premature physeal closure 9. Stiff ROM 10. >20% hip extruded
405
What is the Stulberg classification?
For Perthes disease. There is a relationship between femoral head shape and early OA risk. 1: normal 2: minor head change 3: aspherical head 4: Flattened head, congruous acetab 5: Collapsed head, noncongruous acetab Poor interobserver reliability. Can't determine until skeletal maturity
406
What is the Salter-Thompson classification?
Based on radiographic crescent sign Class A: crescent sign involves <50% femoral head Class B: crescent sign involves >50%
407
What is the Catterall classification?
Extent of head involvement at fragmentation stage. Classified as groups 1: anterior epiphysis only 2: anterior and central epiphysis 3: most of epiphysis, sparing posteromedial corner 4: total head
408
What are the Caterall head at risk signs? (5)
1. Gage sign: v-shaped lucency at lateral epiphyseal/metaphyseal junction 2. Lateral epiphysis calcification 3. Lateral femoral head subluxation 4. Horizontal physis 5. Metaphyseal cyst
409
What is the lateral pillar (Herring) classification?
Height of lateral third of epiphysis. Has best interobserver agreement A: full height. Good outcome B: 50 - 100% height. Poor outcome if >8yo B/C: 50% C: <50% height. Poor outcome in all
410
Which classification systems are to be used for the fragmentation stage of Perthes? (3)
1. Salter-Thompson: crescent sign 2. Caterall: head involvement 3. Lateral pillar (Herring): height
411
The treatment for Perthes disease is for which stage?
Fragmentation. Reossification and remodeling stages don't need treatment
412
What are the indications for nonoperative management of Perthes? General principles?
Indications: <6yo or lateral pillar A Activity restriction Protected WB until reossification Bracing/orthotics are not helpful
413
What are the outcomes for kids <8yo with Perthes?
Kids <6yo do well without treatment if lateral pillar A, B or B/C Most end up w Stulberg 1 or 2 at maturity. 80% good outcome Did poorly if pillar C
414
Generally, what are the indications for operative management in Perthes? (3)
1. >8yo 2. Herring B 3. Herring B/C Kids <8yo all do well w nonop except if Herring C All kids w Herring C do poorly regardless of treatment
415
What are the operative options for Perthes? What are the indications? (2)
1. Femoral varus osteotomy +/- pelvic osteotomy Indications: head extrusion in kids >8yo or B, B/C Make varus to reposition in acetab. Pelvic osteotomies: salter, triple, Dega or Pemberton 2. Femoral valgus osteotomy +/- salvage pelvic osteotomy Indications: head extrusion + painful hinging during abduction Salvage osteotomies: shelf or Chiari
416
What femoral head deformities are associated with Perthes? (2)
1. Coxa magna: wider head and bigger acetab. Due to overexaggerated healing 2. Coxa plana: flat head
417
What is the epiphyseal slip-in index? How do you measure it?
Measure w arthrography. Used to determine if VDRO would be beneficial Place hip in 40deg abduction to mimic hip position after varus osteotomy Index is ratio between - Horizontal distance from lateral acetab rim to tip of epiphysis - Horizontal distance from tear drop to lateral acetab rim ESI <20% = head won't be well contained after VDRO. Poor outcomes w VDRO ESI >20% = head will be contained. Good outcomes w VDRO Well contained head after VDRO is important for remodelling into spherical head
418
RC: Which of these patients is least likely to benefit from a VDRO? A. 8yo w Herring B B. 7yo w lateralized hip C. Epiphyseal slip index >20% D. Performing the osteotomy during initial or fragmentation phase
Answer: B This is the best answer but it depends on the situation. If it is a lateralized hip that causes hinge abduction, then the pt would benefit from a valgus osteotomy. If it is a lateralized hip that can be contained w a VDRO (check w arthrogram and hip in 40deg abduction), then VDRO would still be useful The other choices would benefit more though 2018, 2016
419
What are the complications associated w Perthes? (6)
1. Femoral head deformity: coxa magna and plana 2. Lateral hip subluxation from extrusion. May cause hinge abduction 3. Premature physeal arrest 4. OA 5. Acetab dysplasia 6. FAI and labral injury
420
What is SCFE and in what direction is the slip?
Femoral neck metaphysis displaced anterior and superiorly compared to epiphysis
421
What is the leg position in SCFE? What motions are limited?
Leg is in extension, varus (adduction), ER Limited FABIR (flexion, abduction, IR)
422
List risk factors for SCFE (11)
1. Obesity: single greatest risk 2. Male 3. Ethnicity: Blacks, Pacific Islanders 4. Peak growth velocity (12 in girls, 14 in boys) 5. Younger age: risk factor for bilateral slip Anatomic variants that increase shear force 6. Femoral anteversion 7. Decreased neck-shaft angle 8. Physeal obliquity. More vertical = bad Other conditions 9. Previous radiation 10. Endo disorders 11. Down syndrome
423
What endocrine disorders are associated with SCFE? (6)
1. Hypothyroidism 2. Growth hormone 3. Renal osteodystrophy 4. Panhypopituitarism 5. Hypo or hyperparathyroidism 6. Hypogonadism
424
Who should have an endocrine workup for SCFE? (2)
1. <10yo 2. Weight <50 percentile
425
What zone of the physis does SCFE affect?
Hypertrophic zone
426
What are the classification systems for SCFE? (4)
1. Traditional: based on duration. Pre-slip, acute, chronic, acute on chronic 2. Loder: ability to WB 3. % Displacement 4. Southwick angle
427
What is the Loder classification?
For SCFE. Based on ability to WB and correlates w AVN risk - Stable: able to WB, even if need crutches. 10% AVN risk - Unstable. Unable to WB. 50% AVN risk
428
What is the % displacement classification for SCFE?
Displacement of epiphysis relative to metaphysis Mild: <33% Moderate: 33-50% Severe: >50%
429
What is the Southwick Angle Classification for SCFE?
Difference in epiphyseal-shaft angle between both sides, on frog-leg latera XR Normal: 12deg Mild: <30deg Moderate: 30-50deg Severe: >50deg
430
What is the clinical presentation of SCFE?
1. Pain: groin, knee 2. Limited ROM in FABIR: flexion, abduction, IR 3. Prefer sitting w affected leg crossed over the other 4. Drehman sign: obligatory hip ER w passive flexion 5. Foot is ER on foot progression 6. Trendelenburg gait 7. LLD
431
What is the best XR view to detect subtle SCFE slips?
Frogleg lateral
432
What are key XR findings in SCFE? (3)
1. Klein's line 2. Physeal widening 3. Blurred metaphysis: aka blanch sign of Steel. Due to overlapping metaphysis and epiphysis
433
What is Klein's line?
For SCFE. Line along superior femoral neck should intersect the epiphysis. In SCFE, the epiphysis is flush or below line
434
What is the primary goal of SCFE treatment?
Prevent progression Longterm followup shows that remodeling occurs after insitu fixation For very severe slips, some may correct the slip, but still controversial
435
What are the treatment options for SCFE? Treatment for residual deformity later?
- In situ perc pinning - Modified Dunn - Contralateral prophylactic pinning Residual deformity: proximal femur osteotomy
436
Describe the reduction maneuver for SCFE in situ pinning
None. Trick question. There is no closed reduction. There is serendipitous reduction when positioning on table
437
For in situ pinning of SCFE, 1 screw is sufficient. Why are the advantages and disadvantages of using 2 screws?
Advantage: slightly more stable Disadvantages 1. Violates physis more 2. Difficult to assess joint violation 3. Increased AVN risk, as more likely to violate lateral epiphyseal vessels 4. Labral tear, seen when 2nd screw used and penetrates anterosuperior quadrant
438
Describe the technique for SCFE in situ pinning
- Serendipitous reduction while positioning on radiolucent table - Start at anterior neck. Aim posterosuperior into epiphysis - 6.5 or 7.3 cannulated screw - Approach-withdraw on fluoro to assess joint penetration - Postop: WB if stable, NWB if unstable
439
Describe ideal screw positioning w SCFE in situ pinning
Center of epiphysis Perpendicular to physis Cross physis w 5 screws 6.5 or 7.3 cannulated screws. Fully threaded easer to remove later
440
What are the goals and indications for the modified Dunn for SCFE management?
Goal: surgical hip dislocation for capital realignment. Severe slips may not remodel adequately. May lead to limited ROM and FAI Indication: severe slips
441
Describe the technique for the modified Dunn procedure for SCFE
- Gibson approach - Surgical hip dislocation w z-capsulotomy - Develop retinacular flaps. Epiphysis will remain attached to posterior flap (blood supply) - Starting anteriorly, free the epiphysis from the metaphysis - Debride callus from along posterior metaphysis to allow reduction and prevent retinaculum from kinking - Reduce epiphysis + fix
442
What is the main complications w the modified Dunn procedure for SCFE?
AVN
443
Indications for contralateral prophylactic pinning in SCFE? (6)
High risk patients or risk of loss to followup 1. Obese male 2. Endocrine disorder 3. Down syndrome 4. Initial slip at younger age (<10yo). Literature: younger kids more likely to have bilateral SCFE 5. Open triradiate: indicates younger 6. Unreliable followup (lives in remote area, etc)
444
Describe the management of residual deformity for SCFE. Indications? Procedure?
Need corrective osteotomy. Can be intertrochanteric or subtrochanteric. Avoid neck osteotomy as risk of AVN Indications: painful or function-limiting deformity Fix w blade plate Correction needed (FABIR) - Flexion - Valgus (abduction) - IR
445
What are the risk factors for AVN for SCFE? (3)
1. High grade slips 2. Attempts at reduction 3. Hardware in posterosuperior neck
446
What complications are associated with SCFE? (5)
1. Femoral head osteonecrosis 2. Contralateral SCFE 3. Residual hip deformity 4. Slip progression 5. Labral tear from additional 2nd screw during in situ fixation
447
What other conditions are associated w congenital knee dislocation? (4)
1. Myelomeningocele 2. Arthrogryposis 3. Larsen's 4. Packaging disorders
448
What is the clinical presentation of congenital knee dislocation? What key physical examinations must be done?
Hyperextended knee at birth Assess passive flexion as it determines treatment R/O DDH
449
What is the incidence of ipsilateral hip dislocation in congenital knee dislocation?
70 to 100%
450
If a baby has both DDH and congenital knee dislocation, which should be treated first?
Treat congenital knee dislocation first. Can't place in Pavlik if knee doesn't bend
451
What is the Tarek classification?
For congenital knee dislocation Grade 1: >90deg passive flexion. On XR, appears as recurvatum Grade 2: 30-90deg passive flexion. On XR: subluxated or dislocated Grade 3: <30deg passive flexion. On XR: dislocated
452
What are the treatment options for congenital knee dislocation? What are the indications?
1. Reduction and casting for Tarek grade 1 2. Soft tissue release: Tarek grade 2 or 3, recurrence after casting
453
Describe the goals and technique for soft tissue release in congenital knee dislocation
Goal: 90deg flexion Quads: recession or V-Y lengthening Anterior capsule release Hamstring posterior transposition Postop: cast in 60 flexion x4wks
454
In congenital patella dislocation, what direction is the dislocation?
Lateral initially. Later, w worsening genu valgum, will dislocate posteriorly
455
At what age does the patella ossify?
3-5yo
456
What osseous abnormalities are associated w congenital patella dislocation? (3)
1. Small/absent patella 2. Hypoplastic trochlea 3. External tibial torsion
457
What soft tissue abnormalities are associated w congenital patella dislocation?
1. Tight lateral structures (IT band, lateral retinaculum) 2. Tight quads: causes superior subluxation
458
What conditions are associated w congenital patella dislocation? (5)
1. Larsen 2. Arthrogryposis 3. Diastrophic dysplasia 4. Nail-patella syndrome 5. Down syndrome
459
What is the clinical presentation of congenital patella dislocation? (5)
1. Delayed walking 2. Genu valgum 3. Posterior patella dislocation: with enough genu valgum. Then quads act as knee flexors 4. Knee flexion contracture 5. Small patella
460
What is the treatment of congenital patella dislocation?
Need OR. Cannot treat nonop Andrish technique: maybe look into this? I didn't
461
What is a bipartite patella? What is the most common location for the accessory fragment?
Congenital failure of fusion. Most common is superolateral fragment Usually asymptomatic
462
A bipartite patella is asymptomatic. In what situation may it cause persistent pain?
Trauma. Either direct, indirect or from jumping activities. Disrupts fibrocartilaginous zone between the accessory fragment and main patella. Zone can't heal by bony union, so causes persistent pain
463
How do you differentiate a bipartite patella from a patella #?
Bipartite patella is: - Located superolaterally usually - Smooth, rounded borders - Bilateral in 50%
464
What is the best XR view to visualize a bipartite patella?
AP view
465
How do you an a weight-bearing and non weight-bearing skyline view for a bipartite patella? What are you looking for?
NWB view: do prone WB view: do while squatting Look for fragment displacement on WB view
466
Why may you MRI a bipartite patella?
Assess if it is the source of pain Will see edema around the fragment if it's the cause
467
What is the nonoperative treatment for bipartite patella?
Immobilize in brace at 30deg flexion. NSAIDs. PT for isometric quads strengthening
468
What are the operative treatment options for bipartite patella? (5)
1. Open excision accessory fragment. For failed nonop >6mo or significant displacement after trauma 2. Arthroscopic excision: case reports on this. May disrupt less quads tendon 3. Lateral retinacular release: for superolateral fragment to remove traction force from vastus lat 4. Vastus lateralis release: for superolateral fragment. To avoid long retinacular release, which may cause maltracking 5. ORIF: for large fragments. Controversial. Limited reports
469
What complications are associated w bipartite patella? (5)
1. Patellofemoral maltracking: due to excising large fragment or retinacular release 2. Degenerative changes of patellofemoral joint if maltracking 3. Persistent knee pain 4. Quads weakness 5. Osteonecrosis
470
Pediatric proximal tibia metaphyseal #s are common in what age group? What is the mechanism of injury?
Ages 3-6yo Low energy valgus force. Usually going down slide in lap of adult. Knee extended and gets caught
471
What is the treatment for pediatric proximal tibia metaphyseal #s? (2)
1. Long leg cast w varus mold. May need closed reduction if displaced 2. Open reduction. Rare. Indicated if unable to reduce due to interposed tissue. May supplement w K-wires, then long leg cast
472
What complications are associated w pediatric proximal tibial metaphyseal #s? (2)
1. Cozen phenomenon 2. LLD: affected tibia is longer, usually <1cm
473
What is the incidence of Cozen phenomenon? When does it develop? When is the deformity the worst? What is the prognosis?
50-90% incidence Deformity develops 5-15mo after injury Deformity is worst at 18mo Most self resolve
474
What are the risk factors for Cozen phenomenon? (4)
1. Incomplete reduction 2. Concomitant injury to proximal tibial physis 3. Infolded periosteum 4. Injury to pes insertion. Causes loss of medial physeal tether (?) and results in asymmetric growth
475
What are the treatment options for Cozen phenomenon? (2)
1. Observation. Most will resolve by 3yrs 2. Operative: guided growth or proximal tibial osteotomy. Indicated if >15deg deformity and near maturity
476
What is Blount's disease? What age group is typical for infantile vs adolescent Blount's
Osteochondrosis at proximal medial tibial physis that may progress to physeal bar. Due to overloading. Results in decreased growth at posteromedial corner of proximal physis. Causes varus deformity Infantile: 2-5yo Adolescent: >10yo
477
True or false: infantile Blount's is more severe than adolescent
TRUE
478
What is the tibia deformity associated w Blount's?
Affects posteromedial proximal physis. Results in: - Varus - Procurvatum - Internal rotation - Posteromedial sloping at proximal epiphysis
479
What are risk factors for infantile Blount's disease? (3)
1. Early walking 2. Obesity 3. Ethnicity: Hispanic, black
480
What is the DDx of genu varum? (6)
1. Persistent physiologic varus 2. Proximal tibial physeal injury: infection, trauma, radiation 3. Metabolic bone disease (rickets, renal osteodystrophy, hypophosphatasia) 4. OI 5. Skeletal dysplasia (MED, SED) 6. Focal fibrocartilaginous defect
481
What are the clinical findings in infantile Blount's? (7)
1. Varus 2. Procurvatum 3. Internal rotation 4. Compensatory distal femur valgus if advanced 5. Palpable medial prominence - from beaking 6. LLD if unilateral Blount's 7. Gait: lateral thrust
482
What is the Langenskiold Classification?
Stages of infantile Blount's disease, based on XR Stages 1-4: worsening medial metaphyseal beaking and sloping Stages 5-6: physeal bar formation
483
True or False: infantile Blount's disease can spontaneously resolve
True. Common in Langenskiold stage 2 Possible in stage 4
484
What are XR findings in infantile Blount's? (6)
1. Asymmetric bowing: if symmetric, suspect skeletal dysplasia 2. Narrowed medial epiphysis 3. Medial joint depression 4. Medial metaphyseal beaking 5. Medial and posterior sloping 6. Metaphyseal-diaphyseal (Drennan) angle
485
What is the metaphyseal-diaphyseal (Drennan) angle?
Differentiate between physiologic varus vs infantile Blount's Angle between: - Line perpendicular to longitudinal axis - Line connecting metaphyseal beak <10deg: physiologic. 95% resolve 10-16deg: indeterminate. Follow closely >16deg: Blount. 95% progression
486
What is the nonoperative treatment for infantile Blount's? What are the indications?
KAFO until bony changes resolve (usually in 2yrs) Indications, must be all of the following: - <3yo - Unilateral - Langenskiold 1/2
487
What are the indications for operative management in infantile Blount's? (3)
1. Age >4yo: no longer physiologic varus, no matter the Langenskiold stage 2. Langenskiold stage 3+, no matter the age 3. Metaphyseal-epiphyseal (Drennan) angle >16
488
What are the operative options for infantile Blount's disease? (2)
1. High tibial osteotomy 2. Physeal bar resection
489
What are the operative options for adolescent Blount's? (4)
1. High tibial osteotomy 2. Physeal bar resection 3. Epiphysiodesis 4. Medial plateau elevation
490
Why do most kids require high tibial osteotomy in infantile Blount's?
Need to unload the medial physis or it won't grow. As such, guided growth or physeal bar resection won't be very effective. As per Heuter-Volkman law, physis under compression won't grow Can do guided growth, physeal bar resection and hemiplateau elevation in addition to osteotomy
491
Describe technique for high tibial osteotomy for infantile Blount's
- Osteotomy inferior to tubercle. Dome osteotomy, w apex of dome inferior like a smiley face - Overcorrect to 10deg valgus to offload medial side - Laterally translate to lateralize mechanical axis - ER to correct internal rotation - Pin fixation w Steinman pins - Do physeal bar resection if stage 5/6 - Long leg cast
492
What complications are associated w tibial osteotomy for infantile Blount's? (4)
1. Compartment syndrome 2. Peroneal n injury 3. Delayed union or malunion 4. Recurrence. Increased risk if osteotomy done <4yo
493
What are the indications for hemiplateau elevation in Blount's?
For older kids only after proximal tibial physis has fused. Requires intra-articular osteotomy. Place graft under to elevate.
494
What are the risk factors for adolescent Blount's?
Obesity
495
What is the distal femur deformity in infantile and adolescent Blount's?
Infantile - compensatory distal femur valgus Adolescent - varus
496
True or False: adolescent Blount's can resolve spontaneously?
False. Will progress. Thus, bracing not considered. Only infantile can resolve spontaneously in stages 2 and 4
497
True or False: the treatment for adolescent Blount's is always surgery
True. Bracing is not effective.
498
What is the rate of bilaterality in infantile and adolescent Blount's?
Infantile: 50% Adolescent: rare
499
RC: A 10yo has stage 5 Blount's disease. Which of the following is least likely to correct axial alignment? A. Tibial hemiplateau elevation B. Proximal tibial osteotomy C. Proximal tibial epiphysiolysis D. Tibial plateau varus producing osteotomy
Answer: A. Hemiplateau elevation
500
RC: A 10yo w stage 5 Blount's disease and has depression of the medial tibial plateau. Which of the following options would be least helpful? A. Distal femoral osteotomy for compensatory valgus deformity B. Proximal tibial epiphysiodesis C. Varus correcting proximal tibial osteotomy w medial plateau elevation
Answer: A (distal femoral)
501
RC: There is no debate that surgical treatment is required for adolescent Blount's disease. What is true about guided growth? A. Treatment has poor results in children over 12yo B. Poor outcomes if BMI >35 C. It is associated w a high rate of hardware failure D. A limited amount of growth remaining limits the utility of this treatment
Answer: D Guided growth requires growth remaining and no physeal bar 2016
502
True or False: in idiopathic pediatric genu valgum, the deformity is often at the distal femur?
TRUE
503
What should you observe in 7yo kids in regards in genu valgum? (3)
1. Valgus should not increase 2. Should be <12deg valgus 3. Intermalleolar distance should be <8cm
504
What are the indications for observation in pediatric genu valgum?
Valgus <15deg and age <6yo
505
What are the indications for operative treatment of pediatric genu valgum? (2)
1. In <10yo, valgus >15deg 2. In >10yo, mechanical axis falls in lateral quadrant of tib plateau
506
What are the operative options for pediatric genu valgum? (2)
1. Hemiepiphysiodesis. Femur and/or tibia depending on situation 2. Distal femur varus osteotomy Decision depends on whether there is sufficient growth left
507
When performing a hemiepiphysiodesis, how do you avoid injuring the physis?
Place the staple/8 plate/whatever extra-periosteally
508
How do you prevent peroneal n injury when performing a distal femur varus osteotomy for genu valgum? (3)
1. Pre-emptive peroneal n release 2. Gradual correction 3. Closing-wedge technique
509
What is rickets?
Qualitative defect in bone mineralization due to inadequate PO4 and Ca. Occurs before maturity Osteomalacia is the same disease but after maturity
510
What zone of the physis is affected in rickets?
Zone of provisional calcification, in the hypertrophic zone
511
What is the clinical presentation of rickets? (7)
1. Hypotonia 2. Waddling gait 3. Genu varum 4. Tibial bowing 5. Rachitic rosary 6. Abnormal dentition 7. Pathologic #
512
What are XR findings in rickets? (8)
1. Physeal widening 2. Metaphyseal cupping 3. Poor bone density 4. Genu varum 5. Rachitic rosary: at osteochondral junction in ribs 6. Codfish vertebra: biconcave vertebral body 7. Cat back: kyphosis 8. Looser's zones: insufficiency # seen on compression side of bone. Common at proximal femur, ribs, ulna, rami
513
What is the function of vitamin D?
Kidneys: Ca resorption, PO4 excretion Bone: differentiate osteoclasts. osteoblasts mineralize bone GI: Ca and PO4 absorption Net effect: Increased Ca and PO4
514
What are the types of rickets?
1. Vitamin D resistant: familial hypophosphatemic 2. VitD deficient: nutritional 3. VitD dependent
515
What is vitamin D resistant rickets? At what age does it present? What is the treatment?
AKA familial hypophosphatemic Most common heritable rickets Presents at ~1yo X-linked dominant: PHEX gene Net effect is decreased PO4 absorption and decreased VitD activation Treatment: calcitriol and PO4 replacement
516
What is the active form of vitamin D? What enzyme activates vitD?
Calcitriol (1,25-OH2-VitD3) Enzyme: 25-OH-1α- hydroxylase
517
What lab abnormality is seen in vitamin D resistant rickets?
Decreased PO4
518
What is the function of phosphate?
Key bone mineral (as hydroxyapatite) Homeostasis: resorbed and excreted by kidneys Low PO4 causes vitD activation in kidneys High PO4 results in: - Increased PTH - Bone resorption to increase Ca - VitD inactivation
519
What are the sources of calcitriol?
- Dietary - Conversion from liver and kidney Note: sunlight will convert vitamin D to D3, which still needs to be converted to calcitriol in the liver or kidney
520
At what age does VitD deficient rickets present? What are the risk factors? What is the treatment?
Nutritional deficiency. Rare now that vitD is added to milk Presents at 6mo-3yo Risk factors - Premature - >6mo breastfed without supplementation - Malabsorption (like celiac) - Chronic parenteral nutrition - Vegetarian Treatment: vitamin D (not necessarily calcitriol)
521
What lab abnormality is seen in VitD deficient rickets?
Low Vitamin D, low Ca, Low PO4 Rationale: low vitD in take. The net effect of vitD is to increase Ca and PO4 absorption from the gut, but this does not happen
522
What is vitamin D dependent rickets?
Autosomal recessive. 2 types. Both present w hypotonia, growth failure and hypocalcemic seizures Type 1: joint deformity and fractures in infancy - Lack 25-OH-1α- hydroxylase to convert to calcitriol - Treatment: calcitriol Type 2: bone pain and dental caries/hypoplasia - Mutated calcitriol receptor - Treatment: vitamin D
523
What lab abnormality is seen in type 1 vitamin D dependent rickets?
Low calcitriol, low Ca, Low PO4 Rationale: lack enzyme to convert vitD to calcitriol The net effect of vitD is to increase Ca and PO4 absorption from the gut, but this does not happen
524
What lab abnormality is seen in type 2 vitamin D dependent rickets?
High calcitriol, low Ca, low PO4 Defective calcitriol receptor so the body makes more to have an effect. But no effect so gut doesn't absorb Ca and PO4
525
RC: What is true regarding the inheritance of x-linked hypophosphatemic rickets? A. If the father has the disease, 50% of his children will get it B. If the mother has the disease, 100% of sons will get it C. If the father has the disease, 100% of daughters will get it D. If the mother has the disease, 75% of the daughters will get it
Answer: C You need to know that vitamin D resistant (hypophosphatemic) rickets is x-linked dominant. PHEX gene 2016
526
What is hypophosphatasia?
Metabolic bone disease due to mutation in TNSALP. Autosomal recessive. Decreased alkaline phosphatase activity results in poor bone mineralization. Ca and PO4 accumulate
527
What lab abnormality is seen in hypophosphatasia?
Low ALP, high Ca, high PO4 Poor ALP activity. So Ca and PO4 don't form hydroxyapatite in bone
528
What is the clinical presentation of hypophosphatasia?
Genu varum Abnormal dentition
529
What zone of the physis is affected by hypophosphatasia?
Hypertrophic zone. Zone of provisional calcification doesn't form
530
What are XR findings in hypophosphatasia? (3)
1. Genu varum 2. Physeal widening 3. Deossification of bone next to physis
531
What is renal osteodystrophy? Describe the pathophysiology
Metabolic bone disease due to chronic renal disease. Causes poor bone mineralization. Main pathophysiology: - Hyperphosphatemia: damaged kidney doesn't excrete PO4, accumulates - Hypocalcemia: High PO4 inactivates calcitriol so Ca not absorbed in GI or kidney - Secondary hyperparathyroidism: due to low Ca and high PO4. Initially a good adaptation. Increases bone resorption to increase Ca. Increases PO4 excretion from kidneys. Eventually, kidney function becomes so poor that not enough PO4 excreted. Net effect of PTH becomes just bone resorption
532
What lab abnormality is seen in renal osteodystrophy?
Low Ca High PO4 and PTH
533
What is a Brown tumor?
AKA osteitis fibrosa cystica. Lytic bone lesion due to hyperparathyroidism Seen in renal osteodystrophy due to secondary hyperparathyroidism. Not actually neoplastic
534
What are the 3 types of tibial bowing? What is the differential Dx of each?
1. Anterolateral - NF - Congenital tibial pseudarthrosis - Tibial deficiency 2. Anteromedial: fibular deficiency 3. Posteromedial: physiologic
535
What are the types of neurofibromatosis?
NF-1: most common NF-2: assoc w bilateral vestibular schwannomas
536
What is the inheritance of NF-1?
Autosomal dominant 50% inherited 50% spontaneous mutation Mutation on chromosome 17
537
What are typical extremity manifestations of neurofibromatosis? (5)
1. Anterolateral bowing 2. Tibial pseudarthrosis 3. Hemihypertrophy 4. Forearm: bowing and obliteration of medullary cavity 5. Ulnar or radial pseudarthrosis
538
What are common spine manifestations of neurofibromatosis? (3)
1. Scoliosis: key word is dystrophic 2. Kyphosis 3. Atlantoaxial instability
539
What is the most common skeletal manifestation of NF?
Scoliosis
540
What tumors are associated w neurofibromatosis? (3)
1. Neurofibroma, plexiform type. Pathognomonic. May transform into neurofibrosarcoma 2. Wilms tumor 3. Verrucous hyperplasia: oral mucosa. May transform into carcinoma
541
What is the NIH diagnostic criteria for neurofibromatosis? (7)
Requires 2 of the following: 1. Café-au-lait spots: 6+ - Prepuberty: >5mm - Post puberty: >15mm 2. Lisch nodules: 2+. These are iris hamartomas 3. Optic glioma 4. Axillary or inguinal freckling 5. Neurofibromas: 2 of any type or 1 plexiform type 6. Osseous involvement 7. First degree relative w NF1
542
What is the incidence of tibial bowing in neurofibromatosis? What is the rate of NF in those w tibial bowing?
5-10% NF have bowing (anterolateral) 50% w bowing have NF
543
In kids w anterolateral tibial bowing, what factor determines if treatment will be operative vs nonoperative?
Presence of pseudarthrosis or # No pseudarthrosis = bracing is ok
544
What is the nonoperative treatment for anterolateral bowing. What are the indications? What is the goal?
Bracing. Indicated when there is bowing without pseudarthrosis or # Options: clamshell orthosis or patellar tendon bearing orthosis Goal: prevent pseudarthrosis
545
True or False: osteotomy of anterolateral bowing is indicated to correct deformity?
False. Osteotomy is contraindicated. High risk of not healing and coming pseudarthrosis
546
What are the surgical options for managing anterolateral bowing? What are the indications?
Surgical management indicated for pseudarthrosis. Options: 1. Surgical fixation w IM device 2. Amputation
547
Describe the principles of surgical fixation in tibial pseudarthrosis
IM fixation is key - Resect pseudarthrosis and bone graft - IM rod for tibia, either fixed length or telescoping - Fibula often has pseudarthrosis too. IM fixation w flexinail - Synostosis of tibia and fibula to increase healing. Place screws to connect them, such as across the syndesmosis - Take periosteum graft and wrap around pseudarthrosis site to increase healing
548
What are common bone graft options for tibial pseudarthrosis? (2)
1. Iliac crest 2. Vascularized fibular graft from contralateral leg (Farmer's procedure)
549
What is are common complications after surgical fixation of tibial pseudarthrosis? (3)
1. Recurrent #: in 50% even after initial union 2. Valgus deformity 3. LLD at maturity: average 5cm
550
What are the amputation options for tibial pseudarthrosis?
1. Syme amputation. Indicated in a very short leg. Fix pseudarthrosis for prosthesis fitting 2. Amputation at pseudarthrosis. Indicated when persistent motion after multiple failed attempts at fixation
551
What are common XR findings in a spine w neurofibromatosis? (3)
1. Vertebral scalloping: erosion due to dural ectasia or intraspinal neurofibroma 2. Rib pencilling. Poor prognosis for scoliosis. Rapid curve progression if >3 ribs affected 3. Enlarged foramina: due to neurofibroma
552
Why is a spine MRI always needed preop in neurofibromatosis? What are typical findings?
R/O tumors. Neurofibromas are vascular and present a bleeding risk Findings: - Dural ectasia: dural sac dilation that erodes bone. Vertebral scalloping - Dumbbell lesion: neurofibroma that expands through foramina
553
What are the types of scoliosis in NF?
1. Dystrophic 2. Nondystrophic
554
What is dystrophic scoliosis? What are the types?
In NF spine - Presents earlier - Rapid progression - Short segment, involving 4-6 vers - Sharp curve - Thoracic kyphosis 2 types of dystrophic scoli based on degree of kyphosis Type 1: kyphosis <50deg Type 2: kyphosis >50deg
555
What are risk factors for the progression of dystrophic scoliosis in NF-1?
Dystrophic features increase the risk of progression - Rib pencilling - Vertebral wedging - Para or intraspinal masses - Vertebral scalloping - Enlarged foramina w defective pedicles However, the only independent risk factor is rib pencilling Degree of kyphosis possibly is a risk factor Curve severity is not a risk factor
556
What is the treatment for nondystrophic scoliosis in NF?
Treat as if idiopathic scoli based on age (infantile vs adolescent, etc)
557
True or False: bracing is effective for dystrophic scoliosis in NF?
False. Not effective
558
What are the treatment options for dystrophic scoliosis in NF? What are the indications?
1. Observation if <20deg curve. F/U Q6mo 2. Fusion if >20deg
559
What are the principles of spinal fusion for dystrophic scoliosis in NF?
Perform early, before 7yo Anterior and posterior fusion for: - Greater correction - Decreases pseudarthrosis rate from 40% w posterior alone to 10% w A + P
560
RC: All of the following about neurofibromatosis scoliosis are true except: A. In a dystrophic curve, the Cobb ankle is not predictive of progression B. Dystrophic curves are most common C. If scoliosis is present younger than age 8, 70% will become dystrophic D. Associated w dural ectasia
Answer: B Dystrophic curves are not more common, but they are more severe Dystrophic changes (rib penciling, etc) are risk factors. Curve severity is not. It is indeed associated w dural ectasia, which causes some of the dystrophic features (vertebral scalloping, pedicle thinning) Nondystrophic scoli may become dystrophic. Biggest risk factor is scoliosis before age 7yo 2018, 2017
561
Why must nondystrophic scoliosis associated w NF-1 be monitored closely?
May become dystrophic (modulation). Overall, 65% risk of modulation in all nondystrophic scoliosis in NF-1. Age is the biggest predictor. If present w scoliosis <7yo, 80% risk of modulation into dystrophic scoli
562
RC: In Neurofibromatosis 1, which of the following is not true? A. 50% of cases are from sporadic mutation B. Mutation is on chromosome 17 C. Individuals w affected fathers are more significantly affected than individuals w affected mothers D. 6% of NF-1 have pseudarthrosis
Answer: C Mutation is on chromosome 17. 50% of cases are inherited (autosomal dominant) and 50% are sporadic mutations. Patients w NF-1 have a 5-10% risk of pseudarthrosis 2016
563
What is tibial deficiency? What is its inheritance? What are associated conditions?
Preaxial deficiency. Autosomal dominant Associated with - "Clubfoot": rigid equinovarus and supination - Ectrodactyly (cleft hand) - Preaxial polydactyly
564
What is the Jones classification?
For tibial deficiency 1A: no tibia. No extensor mechanism, hypoplastic distal femoral epiphysis 1B: tibial anlage present, delayed ossification. Intact extensor mechanism. Normal femoral epiphysis 2: proximal tibia only 3: diaphysis and distal tibia only 4: ankle diastasis at distal tib/fib joint. Short tibia
565
What is the clinical presentation of tibial deficiency? What should you examine that will guide treatment?
1. Short leg 2. Anterolateral bowing 3. Prominent fibular head 4. Knee flexion contracture 5. Assess extensor mechanism: guides treatment 6. Assess knee stability: guides treatment 7. "Clubfoot"
566
What are key XR findings for tibial deficiency? (4)
1. Deficient tibia 2. Hypoplastic distal femur epiphysis in type 1A 3: proximally migrated fibula. This is key. Helps you detect subtle tibia deficiency 4: ankle diastasis
567
What are the treatment options for tibial deficiency? (4)
1. Knee disarticulation 2. Tib/fib synostosis and BKA 3. Syme/Boyd amputation 4. Supramalleolar osteotomy and lengthening
568
What are the indications for knee disarticulation for tibial deficiency?
- Complete tibia absence - No extensor mechanism
569
What are the indications for tib/fib synostosis and BKA for tibial deficiency?
Proximal tibia only with intact knee extension
570
What are the indications for Syme/Boyd amputation for tibial deficiency?
Jones type 4: most of tibia intact but ankle diastasis with unstable foot
571
What are the indications for supramalleolar osteotomy and lengthening for tibial deficiency?
Jones type 4: most of tibia intact but ankle diastasis w unstable foot Instead of doing Boyd/Syme amputation. Can do supramalleolar osteotomy to position foot plantigrade
572
True or False: acceptable treatment for tibia deficiency includes fibula centralization under the femur
False. Called the Brown procedure. No longer performed due to high failure rate
573
What is the only lower limb deficiency w an inheritance pattern?
Tibial deficiency. Autosomal dominant
574
RC: A 12month old boy presents to your clinic w LLD. XR shows absent tibia. He is unable to actively extend his knee. What is the next step in management? A. Through knee amputation B. MRI to look for tibial anlage C. Syme amputation D. Fibular transfer to create a single bone lower limb
Answer: A (through knee amputation) The indications of amputation are: complete absent tibia or no extensor mechanism It's true that the pt may have a tibial anlage that has delayed ossification. However, without an extensor mechanism, the treatment would still be amputation so no point in MRI Syme amputation would be for an intact tibia and intact extensor mechanism, but unstable foot Fibularization is the Brown procedure. It is historical and no longer acceptable 2016
575
What conditions are associated w fibular deficiency? (10)
1. Anteromedial bowing 2. Tarsal coalition 3. Ball and socket ankle 4. Absent lateral rays 5. Foot deformity: equinovalgus or planovalgus if tarsal coalition 5. PFFD 6. Coxa vara 7. Lateral femoral condyle hypoplasia 8. Genu valgum 9. Cruciate ligament deficiency 10. LLD
576
What is the Achterman and Kalamchi Classification?
Types of fibular deficiency 1A: nearly normal. Proximal fibula is distal to tibial physis. Distal fibula is proximal to talus 1B: partial absence. Unable to support ankle joint 2: complete absence
577
What are key XR findings in fibular deficiency?
1. Fibula: short or absent 2. Small tibial spines 3. Shallow intercondylar notch 4. Femur shortening 5. Coxa vara 6. Tarsal coalition 7. Ball and socket ankle
578
What are the goals of treatment for fibular deficiency? (2)
1. Foot and ankle stability 2. Equal limb lengths
579
List the treatment options for fibular deficiency (4)
1. Shoe lifts and orthotics 2. Contralateral epiphysiodesis 3. Limb lengthening 4. Syme amputation or more proximal amputation Other operations may be needed such as - Supramalleolar osteotomy to correct ankle valgus - Foot procedures for valgus foot - Proximal tibial osteotomy for genu valgus
580
What are the indications for shoe lifts or orthotics in the management of fibular deficiency?
LLD at maturity >2cm Otherwise stable ankle and plantigrade foot. Only for type 1A
581
What are the indications for contralateral limb epiphysiodesis alone in the management of fibular deficiency?
Mild LLD <5cm or <10% at maturity Requires the affected side to have a stable ankle and plantigrade foot
582
What are the indications for limb lengthening alone in the management of fibular deficiency?
Mild LLD <5cm or <10% at maturity Requires a stable ankle and plantigrade foot
583
What are the indications for treatment of fibular deficiency with a combination of contralateral limb epiphysiodesis and ipsilateral limb lengthening?
Moderate LLD at maturity: 10-30% length Affected side must have stable ankle and plantigrade foot
584
What are indications for Syme amputation in the management of fibular deficiency?
- Nonfunctional or unstable foot/ankle - LLD >30% Perform at 1yo for early prosthesis and better psychosocial acceptance
585
What is a Syme amputation? What is a common complication?
Ankle disarticulation. Remove talus and everything distal. Keep heel pad Common complication: heel pad migration
586
What are the outcomes of Syme amputation for fibular deficiency?
Better satisfaction than limb lengthening (88 vs 55%) Similar function to peers athletically and psychologically
587
What is a Boyd amputation?
Talus excised. Fuse calcaneus to tibia. Amputate rest of foot Advantage: unlike in Syme amputation, avoids heel pad migration and heel pad grows w patient Disadvantage: bulbous compared to Syme
588
Syme vs Boyd amputation: which is generally preferred?
Syme. Boyd is bulbous and difficult to fit into prosthesis. But be aware that Syme is assoc w heel pad migration
589
RC: All of the following are true regarding Syme amputations in kids except A. Heel pad migration is a common problem B. If you leave the calcaneus attached, the heel pad will grow as the child grows C. Kids function at the same level as their peers D. Poor outcomes if multiple failed surgeries before syme
Answer: B If you do a syme amputation, the calcaneus is resected Heel pad migration is a problem. Kids function athletically and psychologically similar to their peers
590
What is posteromedial tibial bowing?
Physiologic, due to intrauterine positioning
591
What is the differential Dx for posteromedial tibial bowing?
1. Calcaneovalgus foot 2. Vertical talus
592
Where is the apex of deformity in posteromedial tibial bowing vs calcaneovalgus foot?
Tibia vs ankle
593
What is the treatment for posteromedial tibial bowing?
Observation. Most resolve by 7yo
594
Why should posteromedial tibial bowing be followed if they resolve without treatment?
Average 3cm at maturity 50% end up requiring epiphysiodesis of the longer limb
595
RC: You are called to the nursery to see a newborn w a foot deformity. You are given clinical pictures and XRs that show posteromedial bowing. What should you advise the parents? A. Will need serial casting B. Should have an amputation C. Osteotomy for re-alignment D. Will need to be followed for leg length discrepancy
Answer: D Average 3cm LLD at maturity 2017
596
What is proximal femur focal deficiency (PFFD)? How many this present (what is the spectrum of disease?)
Congenital defect in proximal femur ossification center Spectrum of disease: 1. Absent hip 2. Femoral neck pseudarthrosis 3. Short femur 4. Absent proximal femur
597
What conditions are associated w PFFD? (6)
1. Fibular deficiency in 50% 2. Acetab dysplasia 3. Coxa vara 4. ACL deficiency 5. Genu valgum 6. Knee flexion contracture
598
What is the Aitken classification?
For PFFD Class A: femoral head present, normal acetab. Short femur Class B: femoral head present but dysplastic acetab. No bony connection between head and shaft Class C: no head, dysplastic acetab. No articulation between femur and acetab Class D: no head, absent acetab
599
Before addressing longitudinal deficiency in PFFD, what should be addressed first? (3)
1. Coxa vara 2. Femoral neck pseudarthrosis 3. Acetab dysplasia
600
What are the treatment options for PFFD? (5)
1. Limb lengthening and contralateral epiphysiodesis 2. Rotationplasty 3. Knee arthrodesis and foot ablation 4. Amputation 5. Femoral-pelvic fusion .
601
What are the indications for limb lengthening and contralateral epiphysiodesis in PFFD?
- LLD at maturity <20cm, >50% of contralateral limb - Stable hip and foot - Coxa vara, femoral neck pseudarthrosis, acetab dysplasia addressed
602
What is the maximum length that can be obtained w each limb lengthening procedure? Ideally, what is the maximum amount of lengthening procedures that should be performed?
5cm Correct LLD in 3 or less procedures
603
What is the indication for knee arthrodesis and foot ablation in the management of PFFD?
Foot is proximal to contralateral knee Syme amputation
604
What are the indications for rotationplasty in the management of PFFD?
Foot at level of contralateral knee Ankle has motion
605
What is the indication for femoral-pelvic fusion in the management of PFFD?
Absent femoral head
606
What is the indication for amputation in the management of PFFD? Where should the amputation take place?
Femoral length <50% of contralateral limb Amputate through joint to - Preserve length - Avoid overgrowth, which makes prosthesis fitting difficult
607
What is hemihypertrophy?
>5% abnormal asymmetry between L and R sides. Includes head, trunk and internal organs. Larger in length of circumference. Note: this is different from pseudo hemihypertrophy
608
What is the etiology of hemihypertrophy? (5)
1. Idiopathic 2. Beckwith-Wiedemann syndrome: Wilm's tumor 3. Proteus syndrome: asymmetric growth 4. Klippel-Trenauney syndrome: vascular malformation
609
What is Beckwith-Wiedemann syndrome?
Autosomal dominant overgrowth syndrome Pancreatic islet cell hypertrophy causes repeated infantile hypoglycemia Major criteria: overgrowth, abdo wall defects, macroglossia Minor criteria: hemihypertrophy, ear anomalies, nephromegaly
610
What orthopaedic conditions are associated w hemihypertrophy? (2)
1. LLD 2. Scoliosis: compensatory 3. Peripheral n entrapment
611
What medical conditions are associated w hemihypertrophy? (2)
1. Malignant abdo tumors, especially Wilm's tumor 2. Genitourinary abnormalities
612
What type of tumor is most common in hemihypertrophy? What is the followup for this?
Wilm's tumor (kidney tumor) Serial abdo US q3mo until 7yo. Then physical exam q6mo until skeletal maturity
613
In a child presenting w intoeing, what are 3 anatomic reasons why. Which is associated w packaging disorders? What is the natural hx of these deformities if untreated?
1. Femoral anteversion: resolve by 10yo 2. Internal tibial torsion: resolve by 4yo 3. Metatarsus adductus: resolve by 4yo Femoral anteversion and metatarsus adductus assoc w packaging disorders. Internal tibial torsion is "probably" as well
614
What is femoral anteversion at birth and how does it change over time?
At birth, 35deg Decreases over time to adult anteversion by 8yo: 15deg
615
What is the clinical presentation of femoral anteversion? How do you assess w physical exam?
- Intoeing gait - Sits in W position - Trips while ambulating Physical exam - Trochanteric prominence angle - Rotational profile
616
What is the trochanteric prominence angle test?
Physical exam. Use when assessing excessive femoral anteversion Degree of IR when GT is most prominent
617
List all the components of assessing rotational profile
1. Foot progression 2. Hip IR and ER 3. Thigh-foot angle 4. Lateral foot border 5. Heel bisector
618
How do you assess the rotational profile of the hip? What are normal values?
Test ROM in prone - Increased IR if >70deg. Normal is 20-60deg - Decreased ER if <20deg. Normal is 30-60deg
619
How do you assess the rotational profile of the tibia? What are normal values?
Thigh-foot angle in prone position Abnormal >10deg IR Normal in infants: 5deg IR Normal in 8yo: 10deg ER
620
How do you assess the rotational profile of the foot?
- Lateral border of foot should be straight - Heel bisector. Normally through 2nd/3rd webspace
621
What are the treatment options for excessive femoral anteversion in a child? What are the indications?
1. Observation: most resolve by 10yo 2. Derotational femoral osteotomy. Indicated if <10deg ER in older child. Rarely needed
622
True or False: Bracing, PT and sitting restrictions are useful for managing excessive anteversion in kids?
False. Doesn't change natural history. Most resolve spontaneously by 10yo
623
What is the normal foot progression angle?
Normal <20deg ER
624
What are the treatment options for internal tibial torsion?
1. Observation: most resolve by 4yo 2. Derotational tibial osteotomy: indicated if doesn't resolve and causes functional problem. Can do supramalleolar or proximal osteotomy. Supramalleolar is preferred as less complications
625
What are the components of miserable malalignment? (2)
1. External tibial torsion 2. Femoral anteversion
626
What are the treatment options for external tibial torsion?
1. Activity modification. Does not resolve on its own, unlike internal tibial torsion 2. Tibial osteotomy: supramalleolar preferred over proximal tibial due to decreased complications
627
What is the deformity in clubfoot? What muscles are responsible for each component of the deformity?
CAVE - Cavus: intrinsics, FHL, FDL - Forefoot adductor: tib post - Hindfoot varus: tib post - Hindfoot equinus: Achilles
628
Which bone acts as a fulcrum in clubfoot? (6)
Talus. When correcting clubfoot, need to rotate forefoot and calcaneus around the talus
629
What conditions are associated with clubfoot? (7)
1. Myelodysplasia 2. Arthrogryposis 3. Tibia deficiency 4. Diastrophic dysplasia 5. Larsen syndrome 6. Amniotic band syndrome 7. Other packaging disorders
630
Clubfoot can be diagnosed in utero via US. Why does it matter in which trimester it was diagnosed?
1st trimester: likely associated w other anomalies 2nd trimester: typically true clubfoot if first diagnosed here 3rd trimester: possible false positive if first diagnosed here. Due to intrauterine crowding
631
When assessing clubfoot, what are typical findings on physical exam? What conditions do you need to R/O as part of your exam?
- Intrinsic hypoplasia of affected leg: smaller foot and calf - Shorter tibia - Medial and posterior foot creases - R/O packaging disorders: DDH, torticollis, etc - R/O spinal dysraphism
632
What is the relationship between the talus and calcaneus on XR in a clubfoot compared to a normal foot?
Hindfoot parallelism. Talus and calc are parallel on AP and dorsiflexion lateral view - AP view: talus and calc are normally divergent - Dorsiflexion lateral: talus and calc normally convergent
633
What are the principles of the Ponseti Method?
For clubfoot management - Weekly serial casting with likely TAL - FAO (boots and bars) x23h for 3mo - FAO while sleeping until 4yo
634
Describe the Ponseti casting technique
Weekly serial casting. Cast w knee at 90deg flexion - Cavus: SUPINATE forefoot by elevating 1st ray. Aligns plantar-flexed 1st ray w other rays - Adductus: rotate forefoot around talus - Varus: rotate calcaneus around talus - Equinus: aim for 15deg dorsiflexion. Address after forefoot can be abducted to 70deg and hindfoot corrected. 90% need TAL Cast in max dorsiflexion and forefoot abduction x3wks
635
What correction must be achieved first before correcting the equinus deformity in clubfoot? What happens if you attempt to correct equinus before other deformities corrected?
Correct forefoot adductus to 70deg abduction Correct hindfoot varus to valgus If hindfoot still in varus, correcting equinus leads to rockerbottom deformity
636
How often should boots and bars be worn for after serial casting for clubfoot?
23h/d for 3mo, then during sleep until 4yo
637
What are the indications for tib ant transfer in clubfoot? What is the technique?
Indications: dynamic supination during gait. This is due to overactive tib ant However, make sure this is not a relapse. If it is a relapse, re-cast first. Transfer to ossified lateral cuneiform (ossifies at 3yo) Can do whole or split tendon. OITE preferred whole tendon. Comparable results
638
What is the best indicator of recurrence in clubfoot? What are risk factors? (2)
Best indicator: loss of dorsiflexion (normal: >10deg) Risk factors for recurrence in kids <2yo - FAO noncompliance - Lower parental education
639
What is 1st line management for recurrent clubfoot?
Re-cast. Then consider repeat TAL or tib ant transfer
640
What are the surgical options and indications in managing recurrent clubfoot? (5)
1. Posteromedial soft tissue release and TAL. Indicated in recurrence in young kids. Aim to do before 9mo to avoid delaying walking 2. Medial column lengthening or lateral column shortening. Indicated in rigid recurrence in older kids (3-10yo) 3. Talectomy: recurrent rigid clubfoot in arthrogryposis 4. Multiplanar supramalleolar osteotomy: salvage in older kids 5. Triple arthrodesis: almost never indicated. Don't do in insensate feet
641
What are complications associated with clubfoot management?
1. Recurrence 2. Rockerbottom foot: correcting equinus before hindfoot varus corrected 3. Undercorrection 4. Talus AVN 5. Dorsal bunion (?)
642
RC: You are seeing a 2.5yo who was previously treated successfully for unilateral idiopathic clubfoot w Ponseti casting and percutaneous TAL. During gait, he supinates his forefoot during swing phase and seems to be walking on the lateral border of his foot. His heel was in varus. What would be your initial recommended treatment at this time? A. Tib ant tendon transfer B. Tib post tendon transfer C. Posteromedial release D. Repeat Ponseti casting
Answer: D This is not just dynamic supination during swing. He has recurrence: walking on lateral border, heel in varus. 2018
643
RC: 2yo presents for previously treated idiopathic clubfoot w Ponseti casting. He now presents w intoeing gait and has dynamic supination w gait. What is the best treatment? A. Tib ant transfer B. Tib post transfer C. Re-casting D. Medial calcaneal osteotomy
Answer: C Patient has recurrence (intoeing gait). He doesn't just have dynamic supination Tib ant transfer would be for only dynamic supination. Would be performed at 3yo when when lateral cuneiform ossified. 2011
644
What are the risk factors for metatarsus adductus? (4)
1. Late pregnancy 2. First pregnancy 3. Twin pregnancy 4. Oligohydramnios
645
What conditions are associated w metatarsus adductus?
Packaging disorders
646
What should you look for on physical exam for metatarsus adductus? (5)
1. Gait: intoeing 2. Flexible or rigid deformity? 3. If flexible, actively or passively correctable? 4. Medial foot crease 5. Rotational profile: including lateral foot border and heel bisector
647
What physical exam maneuver actively corrects metatarsus adductus
Tickling the foot
648
What is the Bleck classification?
For metatarsus adductus. Severity based on heel bisector line Normal: bisector through 2nd/3rd webspace Mild: through 3rd toe Moderate: 3rd/4th webspace Severe: 4th/5th webspace
649
What is a serpentine (skew) foot?
Metatarsus adductus Midfoot lateral shift: talonavicular lateral subluxation Hindfoot valgus
650
What is the Berg classification?
For metatarsus adductus - Simple metatarsus adductus (MTA) - Complex: MTA and midfoot lateral shift - Skew foot: MTA and valgus hindfoot - Complex skew foot (serpentine): MTA, lateral shift and hindfoot valgus
651
What are the nonoperative treatment options for metatarsus adductus? What are the indications?
Usually resolves by 4yo - Observation: if flexible and actively corrected - Serial stretching at home: if flexible and passively corrected - Serial casting: if rigid deformity w medial crease. Goal is straight lateral foot border
652
What are the operative treatment options for metatarsus adductus? What are the indications?
1. TMT capsulotomies. For failed nonop in kids <4yo 2. Lateral column shortening, medial column lengthening and MT osteotomy. Kids >5yo and interfering w shoes - Lateral column shortening: cuboid osteotomy - Medial column lengthening: cuneiform - MT osteotomies and pinning
653
What is the operative treatment for serpentine foot?
Similar to metatarsus adductus correction - Lateral column shortening through cuboid - Medial column lengthening through cuneiform - MT osteotomies and pinning - Calcaneus osteotomy to correct valgus
654
What are the deformities in a cavovarus foot?
1. Cavus: elevated longitudinal arch from 1st ray plantar flexion and forefoot pronation 2. Forefoot adduction 3. Hindfoot varus
655
In unilateral cavovarus foot, what must be ruled out?
Neural axis abnormality, such as tethered cord or tumor
656
What is the most common cause of bilateral cavovarus feet?
Charcot Marie Tooth
657
What common causes of cavovarus foot? (10)
Neuro 1. Neuroaxial abnormality: tethered cord, tumor 2. CMT 3. Friedreich ataxia 4. CP 5. Stroke Traumatic 6. Talus # malunion 7. Compartment syndrome 8. Crush injury Other 9. Idiopathic: subtle and bilateral 10. Residual clubfoot
658
What is the clinical presentation of cavovarus foot like? (5) (Physical exam on next card)
1. Recurrent lateral ankle sprain, from peroneal tendon pathology 2. Lateral foot pain from excessive WB 3. 5th MT stress # from excessive lateral WB 4. Plantar calluses on heads and lateral border 5. Plantar fasciitis from elevated arch
659
What should be included in your physical exam of a child w cavovarus foot? Key findings? (7)
1. Coleman block test 2. Peek-a-boo heel 3. Silfverskiold test 4. Gait: unstable. Increased time on both limbs 5. Hand: interosseous wasting 6. Scoliosis: suspect CMT 7. Spinal dysraphism
660
Describe the normal alignment of the talus and calcaneus on an AP XR
- Axis of talus parallel to 1st MT axis - Axis of calcaneus falls on 4th MT axis - Talus and calcaneus are divergent. Angle between (talocalcaneal angle) is 20-40
661
Is the talocalcaneal angle increased or decreased in clubfoot?
Decreased. <20deg. Talus and calcaneus display hindfoot parallelism in clubfoot
662
Is the talocalcaneal angle increased or decreased in cavovarus feet?
Decreased. <20deg in hindfoot varus
663
Is the talocalcaneal angle increased or decreased in planovalgus feet?
Increased. >40deg in hindfoot valgus
664
What are the types of flatfoot? (3)
1. Flexible flatfoot: ligamentous laxity 2. Flexible flatfoot w short achilles 3. Rigid flatfoot: CP, neuroaxial abnormality, tarsal coalition (most common), accessory navicular
665
True or False: toe standing reconstitutes the arch when pes planus is due to tarsal coalition
False. Tarsal coalition causes rigid pes planus
666
What is the clinical presentation and physical exam like in pediatric pes planus?
1. Assess if flexible vs rigid: toe rise reconstitutes arch if flexible 2. Silfverskiold 3. Palpable navicular due to uncoverage or accessory navicular 4. Hindfoot and ankle ROM
667
RC: 8yo has a flexible flatfoot. What is the true? A. Most can be managed w orthotics B. Surgical plan would consist of soft tissue procedures C. 30% have short achilles
Answer: A Surgery would include bony procedures as well (lateral column lengthening vs calc osteotomy and 1st cuneiform osteotomy). Soft tissue alone is insufficient 30% have short gastroc-soleus
668
RC: A child presents w pes planus, which improves w heel rise. The hindfoot corrects to neutral. W the knee extended, the ankle is 20deg plantarflexed. W the knee flexed, the ankle can dorsiflex to 10deg. What do these clinical findings indicate? A. Tight gastroc B. Tight achilles C. Rigid pes planus D. Tarsal coalition
Answer: A (tight gastroc) This is the silfverskiold test Child appears to have flexible pes planus. In rigid pes planus and tarsal coalition, the arch would not reconstitute w heel rise and the hindfoot would not correct 2016
669
What is the clinical presentation of accessory navicular? (4)
1. Flexible flatfoot 2. Pain on medial midfoot 3. Medial plantar enlargement 4. Pain w resisted inversion
670
What is the treatment for painful accessory navicular? (2)
1. Donut pad around navicular 2. Excision
671
For pediatric pes planus, what XR findings are you assessing? (6)
AP 1. Talar-1st MT angle for forefoot abduction 2. Talonavicular angle for navicular uncoverage 3. Talocalcaneal angle for valgus hindfoot Lateral 4. Talar-1st MT (Meary's) angle: decreased 5. Calcaneal pitch: decreased (<20deg) Oblique 6. R/O tarsal coalition
672
Describe the calcaneal lengthening osteotomy for the management of pediatric pes planus
Calcaneal lengthening osteotomy (CLO) aka Evans procedure 1. Z-lengthen peroneus brevis 2. Release abductor digiti minimi 3. Stabilize calcaneocuboid joint w pin to keep from subluxating 4. Osteotomy between anterior and middle facets. 1cm proximal to CC joint 5. Place structural bone graft 6. Medial soft tissue plication 7. If forefoot supinated: medial cuneiform closing wedge osteotomy to plantarflex and correct supination 8. TAL
673
Describe the triple C osteotomy for the management of pediatric pes planus
1. Posterior calcaneus: sliding and medial closing wedge 2. Medial cuneiform: closing wedge 3. Cuboid: opening wedge
674
What are the outcomes in the management of pediatric pes planus between the calcaneal lengthening osteotomy vs triple C?
1. CLO better at correcting TN coverage 2. Equal functional scores 3. CLO has higher complications: - CC joint subluxation: avoid by pinning - 5th MT stress # from lateral foot overload - CC joint OA
675
RC: What is part of the Evans procedure for a pediatric idiopathic flexible flatfoot? A. Temporarily stabilize the CC and TN joints B. 50% increase of fusion rate w autogenous bone graft C. Can correct forefoot supination w opening wedge osteotomy of medial cuneiform D. Osteotomy of the anterior process should be 4mm proximal to the cc joint
Answer: C is the most correct You stabilize the CC joint, not the TN joint No issues w nonunions in kids Classically, the Evans procedure used a closing wedge osteotomy but opening wedge works too Osteotomy should be 10-15mm proximal to cc joint C is the most correct out of all 2015
676
RC: What is true when comparing the triple C osteotomy and an Evans CLO for pediatric flatfoot? A. The Evans procedure is better at correcting talonavicular head coverage B. Triple C is associated w higher complications than Evans procedure C. A complication of Triple C is calcaneocuboid joint subluxation
Answer: A Evans has better correction but higher complications than Triple C. Most of the complications due to CC joint subluxation. Literally from JAAOS 2014 flatfoot deformity in children and adolescents 2018
677
What foot deformity is associated w tarsal coalition?
Rigid pes planus. Loss of arch. Hindfoot valgus. Does not correct w toe rise. Peroneal spasm
678
What conditions are associated w tarsal coalition? (3)
1. Fibular deficiency and ball and socket ankle 2. Apert syndrome 3. Muenke syndrome
679
In patients w tarsal coalition, what is the incidence of multiple coalitions? What is the incidence of bilateral coalitions?
20% have multiple. 50% have bilateral
680
What are the most common locations for tarsal coalitions? At what age do they ossify?
Most common: calcaneonavicular. Ossifies at 8-12yo 2nd most common: talocalcaneal. Ossifies at 12-15yo
681
What are radiographic findings in tarsal coalition? (3)
All found on lateral view 1. Talar beaking: nonspecific sign in tarsal coalition 2. Anteater sign: CN coalition 3. C-sign: talocalcaneal coalition
682
What is it important to obtain a CT or MRI prior to OR for tarsal coalition?
For multiple coalitions
683
What are the management options for tarsal coalition?
1. Observation: asymptomatic 2. Walking boot or casting: initial tx. 30% improve 3. Coalition resection 4. Subtalar arthrodesis
684
Describe the technique for calcaneonavicular coalition resection
- Sinus tarsi incision - Interposition w fat or EDB - Combine w calc slide if significant hindfoot valgus
685
Describe the technique for talocalcaneal coalition resection
-Medial incision between FDL and neurovasc bundle - Interposition w fat - Combine w calc slide if significant hindfoot valgus
686
RC: You are seeing a 12yo w ankle pain. Given the XR (they showed a ball in socket ankle), what is the underlying pathology? A. Ehlers-Danlos B. Post-traumatic C. Lateral ligament instability D. Tarsal coalition
Answer: D Tarsal coalitions may present w a ball in socket ankle joint, especially in limb deficiencies (like fibular deficiencies). The coalitions prevent inversion/eversion. Ball and socket joint allows inversion/eversion though the ankle joint
687
What is vertical talus?
Rigid flatfoot. Navicular dislocated dorsolateral to talus Forefoot abduction and dorsiflexion Convex plantar surface from prominent talar head (rockerbottom) Hindfoot equinovalgus
688
What gait pattern do patients w vertical talus have?
Calcaneal gait (peg leg)
689
What are the soft tissue contractures in vertical talus?
Hindfoot equinovalgus: achilles and peroneals Forefoot abduction and dorsiflexion: EHL, EDL, tib ant
690
What is the DDx of vertical talus?
Oblique talus
691
What is oblique talus?
Talonavicular subluxation. Reduces on plantarflexion XR Treatment: observation
692
Describe the clinical findings in vertical talus (5)
1. Rockerbottom foot deformity 2. Poor ankle dorsi and plantarflexion 3. Prominent talar head medially 4. Contracture: achilles, peroneals, extensors 5. Calcaneal (peg leg) gait
693
At what age does the navicular ossify?
3yo
694
What are you looking for on lateral XRs when assessing vertical talus?
1. Meary's angle (1st MT-talus angle). Normally axis of 1st MT and talus should be in line. 1st MT acts as a surrogate for navicular as not yet ossified. Talus axis will be inferior to 1st MT axis in vertical talus 2. Maximum plantar flexion lateral: persistent talonavicular dislocation. Talus axis will still be inferior to 1st MT axis. Oblique talus would be reduced in this view 3. Maximum dorsiflexion lateral: determine if dorsiflexion if through ankle or dislocation. Determines if equinus contracture needs OR
695
What percentage of vertical talus cases are associated w other conditions? List common associated conditions
50% have neuromuscular or genetic issues 1. Neural axis abnormalities: myelomeningocele, diastematomyelia 2. Arthrogryposis 3. CP 4. Spinal muscular atrophy
696
True or False: vertical talus can be managed nonoperatively
True Historically, vertical talus was treated w preoperative casting to loosen tissues before OR The Dobbs method does only reverse Ponseti casting. No surgery. After casting, placed in boots and bars
697
Describe the principles of operative vertical talus management
- Serial manipulation and casting. Reverse Ponseti. - Percutaneously pin navicular to talus. Open if can't do closed - TAL for equinus - Transfer tib ant to talar neck if reduced open - Lengthening dorsal structures if tight: EDL, EHL, peroneus brevis - Boots and bars in plantigrade and neutral rotation
698
For vertical talus, what position are the feet held in boots and bars? How often should they be worn?
Plantigrade and neutral rotation Wear 23h/d x3mo, then while sleeping until 3yo
699
What are the outcomes after serial casting only vs extensive soft tissue release for the management of congenital vertical talus?
5yr followup as shown that minimally invasive management (Dobbs method uses reverse ponseti casting) results in better ankle ROM and pain scores. It can be used to treat both syndromic and congenital CVT
700
RC: What is true about congenital vertical talus? A. Doesn't require TAL B. Pinning of the TN joint is rarely indicated C. Idiopathic and syndromic can both be treated w casting alone D. The calcaneus is in dorsiflexion
Answer: C Dobbs method uses reverse ponseti manipulation and serial casting. It can treat both idiopathic and syndromic cases (Aslani 2012) When doing operative management of CVT, TAL is often done. TN joint is often pinned The calcaneus is in plantarflexion 2016
701
RC: The position of the calcaneus in vertical talus is A. Equinus, everted, laterally displaced B. Equinus, everted, medially displaced C. Equinus, inverted, laterally displaced D. Equinus, inverted, medially displaced
Answer: A Calcaneus is equinus, everted and laterally displaced
702
What is a calcaneovalgus foot?
Packaging deformity w hindfoot valgus and dorsiflexion (Calcaneus is fancy term for dorsiflexion)
703
What are risk factors for calcaneovalgus foot? (2)
1. Female 2. First born
704
What is the DDx for calcaneovalgus foot? (3)
1. Posteromedial tibial bowing 2. Congenital vertical talus 3. Paralytic foot deformity: L5 spina bifida causing spastic foot dorsiflexion and eversion
705
Where is the deformity apex in calcaneovalgus foot? Posteromedial tibial bowing? Congenital vertical talus?
Calcaneovalgus foot: ankle Posteromedial bowing: tibia Vertical talus: midfoot
706
What are the treatment options and indications for the management of calcaneovalgus foot?
1. Passive stretching. Indicated if can plantarflex past neutral 2. Casting. Indicated if can't plantarflex past neutral
707
What complications are associated w calcaneovalgus foot? (2)
1. LLD if also associated w posteromedial tibial bowing 2. Flexible pes planus
708
What is the definition of cerebral palsy?
Nonprogressive upper motor neuron disease from insult to premature brain, w onset before 2yo
709
What are risk factors for CP?
Premature - Intrauterine infection: SToRCH - Thyroid disease - Congenital brain malformation - Drugs/EtOH Perinatal - Prematurity - Anoxia: nuchal cord, placental abruption, uterine rupture - Kernicterus: due to Rh incompatibility Postnatal - Meningitis/encephalitis - Head injury/Trauma - Cardiac surgery
710
What are the 3 classification systems for CP?
1. Gross motor function classification (GMFCS) 2. Movement dysfunction 3. Anatomic
711
What is the gross motor function classification?
1. Normal, independent ambulator 2. Independent ambulatory, but need rails for stairs/uneven surfaces 3. Assisted walking, WC for long distances 4. WC but can transfer 5. WC bound, no head control
712
RC: 9yo w CP is a community ambulator w forearm crutches at home and at school, but uses a wheelchair for long distances. What is his GMFCS? A. 1 B. 2 C. 3 D. 4
Answer: C (GMFCS 3) 2017
713
What is the movement dysfunction classification for CP? (6)
1. Spasticity 2. Dystonic 3. Athetoid 4. Ataxia 5. Mixed 6. Hypotonic
714
Define spasticity
Velocity dependent increased tone Hyperreflexia w slow movement Due to simultaneous agonist + antagonist contraction
715
Define dystonia
Non-velocity dependent increased tone Involuntary muscle contraction (posturing) Intermittent vs sustained Focal or global
716
Define athetoid
Constant slow, writhing involuntary movement
717
What is the anatomic classification of CP? (3)
1. Quadriplegic: total body involved 2. Diplegic: most common. Upper and lower extremities involved but lower extremity is more affected 3. Hemiplegic: arm/leg on one side
718
How do you examine the lower extremity in CP? (8)
1. Thomas test: hip flexion contracture 2. Hip abduction w hip flexed (Add longus and brevis) and without hip flexed (gracilis) 3. Hip adduction 4. Galeazzi: assess if hip dislocation 5. Ely-Duncan test: assess rectus contracture 6. Popliteal angle: hamstring contracture 7. Silfverskiold: gastroc vs achilles contracture 8. Rotational profile
719
Which XRs needed to be performed at every followup for CP? (2)
1. Hips for dislocation 2. Scoliosis
720
How do you perform the Thomas test?
For hip flexion contracture - Patient supine. Do at end of table so knee flexion contracture doesn't interfere - Fully flex contralateral hip and knee to flatten lumbar lordosis. Affixes pelvis to table - Angle between femur and table
721
How do you assess hip abduction tightness?
- Hip flexed: assesses adductor longus and brevis - Without hip flexed: assesses gracilis
722
What is the Ely-Duncan test?
Assesses rectus femoris tightness Patient lays prone If pelvis pops off table, then tight rectus
723
How do you perform a popliteal angle test?
For hamstring tightness Supine. Flex hip to 90deg Extend knee. Angle between leg and vertical Normal is <50deg
724
What are preoperative considerations for CP?
- Neuro: seizures are common. Valproic acid increases bleeding time. Use TXA, cell saver. Other AEDs cause osteopenia - Osteopenia: from disuse. Use locking plates - Resp: Pulmonary fxn test if possible - GI: Reflux is common. May need swallowing study to prevent aspiration - Nutrition: G-tube if unable to feed and optimize nutrition. Poor nutrition assoc w more complications
725
What is botox and what is its role in the management of CP?
Inhibits Ach release. For spasticity and dynamic contractures. Lasts 3-6mo Helps maintain joint motion during rapid growth and when too young for OR OR under 8yo has high recurrence
726
What are the limitations for botox use for CP? (4)
1. Toxic dose. Limited number of muscles treated at a time 2. New axons sprout by 6mo 3. Develop resistance via antibodies 4. Causes fibrosis over time so counterproductive
727
RC: 6yo w diplegic spastic CP and toe walking. 3mo ago the patient had botox injection and serial casting, which improved symptoms. However, the patient now presents w the same problem. He has ankle 10deg dorsiflexion w knee flexed and 0 dorsiflexion w knee extended. What treatment would you recommend? A. Repeat botox B. Bilateral TAL C. Bilateral gastroc recession D. AFO
Answer: A. Continue botox as it's still effective and CP kids with surgical management before 8yo have high recurrence rates Do NOT do TAL for bilateral spastic diplegia. Creates crouch gait
728
What is R1 and R2 when discussing botox use for spasticity?
R1: limit of ROM due to spasticity R2: limit of ROM due to contracture Large R1:R2 means botox will have large effect since it only affects spasticity
729
What is baclofen and how is it used in the management of CP?
GABA agonist. Given for severe spasticity. Can be given PO but significant cognitive impairments. Giving intra-thecal avoids these side effects. Sudden withdrawal causes seizures
730
At what age do kids develop their adult gait pattern?
7yo
731
What are the 3 common gait patterns seen in CP? Do they affect the stance or swing phase of gait?
Stance 1. Jump gait: true vs apparent equinus 2. Crouch Swing 3. Stiff knee
732
What is jump gait?
Toe walking. No heel strike at initial contact. 2 types: true vs apparent equinus
733
What is true equinus jump gait?
Due to gastroc-soleus spasticity or contracture Hips and knees may be - Hyperextended: will resolve after equinus treated - Hyperflexion knee/hip: due to spasticity or contracture
734
What is apparent equinus jump gait?
Foot appears in plantarflexion relative to the floor, but actually isn't Due to hyperflexion of knee/hip. Do not correct at the ankle!
735
What is crouch gait?
Flat foot at initial contact due to weak ankle plantar flexors. Develops other deformities as a consequence: - Knee hyperflexion contracture - initially as compensation then becomes fixed - Patella alta: quads tendon shortens, patella tendon lengthens due to knee hyperflexion
736
What is stiff knee gait?
Occurs during swing phase. Limited knee flexion . Difficult to clear foot
737
In a CP patient w jump gait due to true ankle equinus, what are the treatment options and indications for the equinus deformity? (3)
1. AFO w lift: for flexible deformity 2. Botox: for spasticity until OR at 8yo 3. Gastroc recession. Goal: 10deg dorsiflexion
738
Why is gastroc recession preferred over TAL in CP?
TAL may cause too much weakness and result in crouch gait
739
What are the consequences of doing a gastroc recession or TAL in a CP patient w jump gait due to apparent equinus?
Causes crouch gait. In apparent equinus jump gait, problem is at the knee or hip
740
What are the treatment options and indications for knee flexion deformity in CP? (3)
1. Medial hamstring lengthening: for <10deg flexion 2. Guided growth: for 10-30deg flexion w growth remaining 3. Distal femur extension osteotomy: for 10-30deg flexion and near maturity
741
In CP, which hamstring tendons are lengthened?
Medial hamstrings: gracilis, semiT, semi M Don't lengthen lateral hamstrings (biceps femoris) in addition. Causes too much hip extension weakness
742
Describe the technique for medial hamstring lengthening in patients w CP
Posteromedial thigh incision, proximal to popliteal fossa Order of lengthening: gracilis, semiT, semi M Gracilis and Semi T: Z lengthening SemiM: fractional lengthening. Cut across fascia like recession Assess popliteal angle after every tendon Goal: 45deg popliteal angle. More than 45deg risks sciatic n palsy
743
True or False: after medial hamstring release, patients w CP lose swing phase knee flexion
TRUE If rectus femoris is also spastic, can transfer rectus to gracilis or semiT. Restores flexion (You may wonder: then what was the point of the hamstring release? The hamstrings were contractured. If rectus is firing too much but not contractured, a transfer would still allow passive extension.. i think)
744
Describe the indications and technique for guided growth in CP patients w knee flexion deformity
Indications: 10-30deg knee flexion contracture w 2yrs growth remaining - Do medial hamstring release - 8 plate on anterior distal femur physis for more growth posteriorly
745
Describe the indications and technique for distal femur extension osteotomy in CP patients w knee flexion deformity
Indications: 10-30deg knee flexion contracture, near maturity - Do medial hamstring release first - Lateral approach through vastus. Distal extent of incision is physis - Trapezoidal osteotomy. Larger anteriorly and smaller posteriorly. This functionally lengthens hamstrings and shortens bone to avoid neurovasc compromise - Patella tendon plication
746
What is the purpose of doing patellar tendon plication after correcting knee flexion deformity in CP kids?
Some may have patella alta due shortened quads tendon and lengthened patella tendon. This is a result of longterm knee flexion contracture. Usually have to do after distal femur extension osteotomy
747
RC: In a CP child w jump gait and 35deg flexion contracture of the knee, treatment includes A. Medial hamstrings lengthening only B. Medial hamstrings lengthening and femur extension osteotomy C. Medial and lateral hamstring lengthening only
Answer: B Don't do both medial and lateral hamstring lengthening in CP. Causes weak hip extensors Medial hamstrings alone indicated for contractures <10deg Extension osteotomy if >10deg flexion contracture and near skeletal maturity
748
In CP kids w stiff gait, what muscle is pathologic? What is the treatment?
Spastic rectus femoris Treatment: transfer rectus so it becomes a knee flexor instead of an extensor Transfer site options: gracilis, semiT, sartorius, IT band No difference in outcome between the transfer sites Gracilis and SemiT preferred if concomitant hamstring lengthening
749
In a CP child with suspected hip contracture, what physical exam test do you perform to prove it’s a truly from the hip? What is the treatment in an ambulatory child?
Thomas test Ambulatory child: psoas tenotomy at pelvic brim. Leave ilacus intact
750
What is the consequence of releasing the entire iliopsoas in a ambulatory CP child who has hip flexion contracture?
Weakens hip flexor too much. Unable to do stairs Releasing both iliacus and psoas reserved for nonambulatory kids only
751
In a child w hip adduction contracture, what is the goal of adductor release? Describe how to do the release
Goal: 45deg abduction Landmark: adductor longus Incision: parallel to groin crease and 1cm distal Order of release: - Adductor longus. Avoid anterior branch of obturator n - Gracilis - Assess if abduction >45deg - If not enough abduction, release adductor brevis
752
What nerve is at risk w adductor release?
Anterior branch of the obturator n. Runs just deep to adductor longus
753
What are the zones of the gastroc-soleus complex?
1. Gastroc soleus muscle bellies 2. Gastroc soleus tendons. Gastroc recession here 3. Achilles tendon. TAL here
754
TAL is generally contraindicated in CP. In what situation is it acceptable? What are the consequences of TAL in CP kids generally?
Only acceptable for spastic hemiplegia. Otherwise should address zone 1 or 2 only in CP. Otherwise risk causing crouch gait
755
What are the most common foot deformities in CP (3)?
1. Hallux valgus 2. Equinoplanovalgus 3. Equinovarus (note: not cavovarus. Cavus is rare in CP)
756
What is the pathophysiology of hallux valgus in CP?
Overactive adductus hallucis If concomitant equinoplanovalgus, also forces toe into valgus to clear the floor
757
What is the surgical management of hallux valgus in CP kids?
1st MTP fusion Least recurrence compared to other techniques
758
What is the surgical management for equinoplanovalgus in kids w CP?
Same as in kids without CP 1. Lateral (Evans) column lengthening: at calcaneal neck (anterior to middle facet) w bone graft 2. Gastroc recession or TAL 3. Plantarflexion closing wedge osteotomy at medial cuneiform
759
What is the pathophysiology of equinovarus in CP?
Inverters overpower evertors Inverters: post tib + tib ant Evertors: peroneals
760
How do you determine if tib post or tib ant are spastic in CP patients w equinovarus foot?
Tib ant: causes forefoot supination - During swing phase, foot supinates (dynamic supination) - Confusion test: pt sits at edge of table and flexes hip. Tib ant will activate as foot dorsiflexes. If foot supinates, then tib ant is spastic Tib post: causes hindfoot varus. Becomes tight when hindfoot brought into valgus
761
List the treatment options and indications for the management of equinovarus foot in CP patients (4)
1. Gastroc recession 2. SPOTT: split posterior tibial tendon transfer. For passively correctable deformity due to tib post 3. SPLATT: split anterior tib tendon transfer. For passively correctable deformity due to tib ant 4. Calcaneal (slide) osteotomy. For fixed hindfoot varus. Must combine w soft tissue procedure
762
Describe the indications and principles of the SPOTT procedure for the management of equinovarus in CP patients
Indication: passively correctable equinovarus deformity due to spastic tib post Transfer to peroneus brevis - Medial incision over tib post - Lateral incision over peroneals - Split tib post. Reroute posterior to tib/fib to suture to peroneus brevis
763
Describe the indications and principles for the SPLATT procedure for the management of equinovarus in CP patients
Indication: passively correctable equinovarus deformity due to spastic tib ant - Medial incision over tib ant - Split tendon and release from 1st MT. Reroute laterally under extensor retinaculum - Transosseous tunnel through cuboid. Tie sutures over button while foot dorsiflexed
764
Describe the pathophysiology of hip dysplasia in CP
Progressive hip deformity that is initially normal at birth (unlike DDH). Progressive adductor and iliopsoas tone causes hip to wear away the posterosuperior acetab wall. Hip subluxes posterosuperiorly
765
Describe the typical proximal femur morphology in CP hips
1. Anteverted (40deg). Normal at birth but doesn't decreases normally 2. Coxa valga 3. Caput valga 4. Medial head becomes flattened: driven into acetab 5. Lateral head becomes flattened: rubs against spastic abductors
766
Describe the typical acetabular deficiency in CP hips
Posterosuperior wall deficiency
767
How does the ability to stand affect prognosis in CP hips
Improves. NWB status (WB bound) worsens hip status
768
Describe the physical exam for assessing CP hips
1. Spine for scoliosis 2. Pelvis for pelvic obliquity 3. Thomas test for hip flexion contracture 4. Hip abduction: w hip flexed (adductors) and without hip flexed (gracilis) 5. Popliteal angle: hamstring contracture
769
How does limited hip abduction affect the risk of dislocation in CP hips?
At risk of dislocation when hip abduction <45deg
770
What are typical radiographic findings for CP hip?
1. Reimer's migration index (MI) 2. Acetabular index: increases as MI increases 3. Neck shaft angle: coxa valga 4. Head deformity: medial head flattening, lateral head flattening
771
What is Reimer's migration index?
Width of uncovered head / total width Hip at risk >25% Subluxation >30% Dislocation >100% Most accurate method of identifying and monitoring hip stability
772
What is the role of hip abduction brace in the management of CP hip?
No role. Doesn't prevent dislocation May cause windblown hips or hyperabduction deformity
773
What parameters suggest a CP hip is at risk? (3)
1. Abduction <45deg 2. Migration index >25% 3. AI >25deg Hips at risk require exam and XR twice a year
774
Describe the treatment algorithm for CP hip
Kids <8yo. Depends on migration index - MI <60%: soft tissue release only - MI >60%: VDRO +/- pelvic osteotomy Kids >8yo: soft tissue release + VDRO +/- pelvic osteotomy
775
At what age should soft tissue releases be done for CP hip?
ASAP once hip is determined to be at risk (based on limited abduction, MI, and AI) This will prevent progressive subluxation and acetab dysplasia
776
At what age should bony procedures be done for CP hip?
Ideally between 4-8yo Before 4yo, remodelling may cause loss of correction After 8yo, can still do if no degeneration. But often too advanced degeneration that salvage is needed
777
In a CP patient w a hip at risk of dislocation, what tendons are addressed as part of the soft tissue release?
Adductors. Goal for abduction >45deg. Adductor longus, gracilis, adductor brevis Hip flexor: iliopsoas. Depends if pt is ambulatory or nonambulatory
778
Describe the technique for hip flexor release in a child w CP. How does it differ in ambulator vs nonambulatory patients?
Ambulatory: release only psoas tendon from pelvic brim. Leave ilacus intact for some flexion Nonambulatory: release both iliopsoas from LT
779
When performing a VDRO for CP patients, what is the goal neck-shaft angle for ambulators vs nonambulators?
Ambulator: 120deg Nonambulatory: 100deg
780
What are common options for pelvic osteotomy in CP hips?
Dega and PAO are good for global coverage Dega if triradiate is open PAO if triradiate is closed
781
What are salvage options for CP hip? (4)
1. Resection of hip 2. Redirectional femur osteotomy 3. Interposition replacement 4. Arthrodesis
782
What is the purpose of the redirectional femur osteotomy as a salvage procedure for CP hip?
Indicated for severe hip adduction that prevents perineal care Valgus producing osteotomy results in a more abducted position
783
What is the most reliable radiographic measure when following a CP hip? A. Migration index B. CEA C. Acetabular index D. Tonnis angle
Answer: A 2016
784
List the upper extremity deformities seen in CP
1. Shoulder IR 2. Elbow flexion and pronation 3. Wrist flexion 4. Thumb in palm 5. Finger flexion
785
What are the treatment options for shoulder IR contracture in CP? When is it indicated?
1. Subscap and pectoralis lengthening 2. Shoulder derotational osteotomy: rarely needed. Usually lengthening is sufficient Indication: 30deg contracture as it interferes w hand function Goal: >30deg ER
786
Describe the technique of shoulder derotational osteotomy for shoulder IR contracture in kids w CP
Deltopectoral approach Transverse osteotomy proximal to deltoid insertion Position in 30deg ER Plate fixation Goal: 30deg ER (Note: same technique used in kids w brachioplexopathy)
787
What is the treatment for elbow flexion deformity in kids w CP?
Release and lengthening 1. Resect lacertus fibrosis 2. Release brachioradialis from origin 3. Lengthen biceps and brachialis 4. Musculocutaneous neurectomy of biceps and brachialis: only if spastic w full passive ROM
788
RC: A 12yo w spastic CP presents w 45deg elbow flexion contracture. Excellent function and ambulates independently. What is not a viable procedure? A. Fractional biceps lengthening B. Musculocutaneous neurectomy C. Biceps tendon z-lengthening D. Flexor-pronator slide
Answer: D? Flexor-pronator slide may refer to a Steindler flexorplasty. This is to restore elbow flexion like in Erb palsy 2017 However, some believe they are referring to a distal flexor-pronator slide. Also, if the child has a fixed flexion contracture, then a neurectomy would not be a viable procedure
789
What are the treatment options for forearm pronation deformity in kids w CP?
1. Release pronator teres 2. Transfer FCU to ECRB
790
What is the danger of transferring pronator teres for the management of forearm pronation deformity in CP kids?
Some transfer pronator teres to an anterolateral position so it supinates Can develop a supination deformity, which is worse than a pronation deformity
791
How does GMFC score affect the incidence of scoliosis in CP?
Incidence increases w GMFC score
792
Describe the pattern of scoliosis seen in CP
Long, C-shaped Kyphosis Left sided curve Associated w pelvic obliquity Begins at younger age and more likely to progress
793
What is the relationship between lumbar scoliosis, pelvic obliquity and hip dislocation in kids w CP?
Lumbar scoliosis may cause pelvic obliquity Pelvic obliquity causes windswept hip deformity. One hip is adducted, other is abducted. Adducted side at risk of dislocation Correcting scoliosis may fix pelvic obliquity Hip surgery does not affect pelvic obliquity
794
What are nonoperative management options for scoliosis in kids w CP?
- Custom seat orthosis - TLSO Neither change natural history. Both for better seating balance
795
What are the indications for scoliosis correction in kids w CP?
Curves >50deg that are progressive and interfere w seating Age >10yo
796
True or False: scoliosis correction in CP improves quality of life for the patients
Maybe? Leaning towards false Typically cited that it's better for caregiver quality of life.
797
How many levels are included in scoliosis correction for CP spine?
Typically from T1-pelvis Multilevel needed due to osteoporotic bone Not including thorax leads to thoracic kyphosis Typically can do all posterior constructs now
798
What is Duchenne muscular dystrophy?
X-linked recessive mutation in dystrophin gene, causing absent protein. Progressive muscle loss, replaced by fibrofatty tissue. Affects proximal muscles first
799
What is the function of the dystrophin protein?
Stabilizes muscle cell membrane so cell leaks intracellular stuff like CK Inflammatory response causes fibrosis Affects skeletal and cardiac muscles
800
What conditions are associated w Duchenne muscular dystrophy? (7)
1. Scoliosis in 90% 2. Equinovarus foot 3. Joint contractures 4. Fractures 5. Cardiomyopathy 6. Static encephalopathy 7. Anesthesia induced rhabdomyolysis
801
What should be done to minimize preoperative risks to patients w Duchenne muscular dystrophy? (3)
1. Anesthesia induced rhabdomyolysis: don't use inhaled anesthetics 2. Intraoperative cardiac events: assess cardiomyopathy w echo 3. Resp status: need PFTs
802
What is Becker's muscular dystrophy? How is it similar or different from Duchenne's?
X-linked recessive mutation in dystrophin gene Similar to Duchenne: calf pseudohypertrophy, high CK levels Different: dystrophin is decreased but not absent. Later onset, slower progression, longer life expectancy
803
Describe the typical natural history of Duchenne's
- Normal at birth and degeneration starts - Diagnosis at 5yo - Needs walking aids at 10 - WC bound at 15 - Cardioresp death at 20
804
What are the first muscles affected in Duchenne?
Gluteals
805
What is the clinical presentation of Duchenne muscular dystrophy?
1. Male 2. Weakness of proximal muscles first 3. Delayed milestones, can't keep up w peers 4. Clumsy, wide-based gait and abductor lurch. Due to tight hip flexors and abductors from prolonged sitting 5. Toe walking: keeps knees extended. Keeps them upright without firing weak quads 6. Calf pseudohypertrophy 7. Gower's sign 8. Scoliosis in 90%. Begins after WC bound 9. Lumbar lordosis: compensate for weak gluts 10. Intact reflexes
806
What studies can you order for patients suspected of having Duchenne's? (3)
1. CK levels: should be elevated 2. DNA testing: dystrophin gene deleted on X chromosome 3. Muscle biopsy: absent dystrophin
807
What are the management options for Duchenne?
1. Corticosteroids 2. Scoliosis correction 3. AFOs at night 4. PT
808
What are the benefits of corticosteroid therapy for Duchenne's? What are the side effects?
Supresses inflammation and thus fibrosis. Benefits: 1. Prolongs ambulation by improving strength 2. Prevents scoliosis 3. Delays pulmonary deterioration Side effects: 1. Osteonecrosis 2. Weight gain 3. Cushingoid appearance 4. Short stature
809
True or False: In patient w Duchenne's, equinus contracture should always be treated surgically or w AFO during ambulation
False. Want to maintain toe walking as it's their compensation for weak quads. Don't use AFO during ambulation, only at night time. Don't correct equinus unless it's so bad that they can't balance anymore
810
True or False: bracing is important for the management of scoliosis in Duchenne's
False. Do not brace. Not effective and interferes w their already weak resp function
811
What are the indications for scoliosis correction in Duchenne's?
Curve >20 Life expectancy >2yrs PFTs >30%
812
True or False: scoliosis correction in Duchenne improves pulmonary function and mortality
False. Good for comfort. Higher parental satisfaction. But does not improve pulmonary function or mortality
813
RC: What slows the degradation of muscle strength in Duchenne? A. NSAIDs B. Baclofen C. Prednisone D. Gamma globulin
Answer: C (prednisone) Common corticosteroids used for Duchenne are prednisone and deflazacort
814
What is spinal muscular atrophy (SMA)?
Autosomal recessive mutation in survival motor neuron (SMN-1) gene. Causes loss of motor neurons in anterior horn. Present w proximal muscle weakness
815
What conditions are associated w SMA? (2)
1. Hip subluxation/dislocation 2. Scoliosis
816
Describe the clinical presentation of SMA
1. Symmetric progressive weakness that is worse in proximal muscles and worse in lower extremity 2. No reflexes 3. Fasciculations 4. Scoliosis 5. Hip subluxation/dislocation
817
What studies can be ordered for patients suspected of having SMA?
1. DNA testing 2. Muscle biopsy
818
What is the treatment for hip dislocation in SMA?
Observation Usually painless and high recurrence w open reduction
819
What is myelodysplasia? What are the different types?
Congenital abnormality related to failed neural tube closure Closed: spina bifida occulta Open: - Meningocele: protruding dural sac without cord - Myelomeningocele: protruding sac w cord - Rachischsis: exposed cord without covering
820
What are risk factors for myelodysplasia?
1. Folate deficiency 2. Maternal hyperthermia (fever) 3. Maternal diabetes 4. Valproic acid 5. Chromosomal abnormalities
821
What orthopaedic conditions are associated w myelodysplasia?
1. Pathologic # from disuse osteopenia 2. Spine: scoliosis (neuromuscular) and kyphosis 3. Hip dysplasia/dislocation 4. Congenital knee dislocation 5. External tibial torsion 6. Foot: clubfoot, vertical talus 7. Sprengel
822
What nonorthopaedic conditions are associated w myelodysplasia?
1. Type 2 Arnold-Chiari malformation: cerebellum and brain stem in foramen magnum 2. Hydrocephalus 3. Tethered cord 4. Neurologic bladder 5. Latex anaphylaxis
823
What is the ambulatory status in patients with myelodysplasia at each of the following levels: L2, L3, L4, L5 or S1?
L2: nonambulatory L3: Marginal household L4: Household ambulators L5: Community ambulators S1: normal ambulators
824
In patients w myelodysplasia, what sort of surveillance should be done at each followup?
1. Hip dysplasia/dislocation 2. Scoliosis
825
What is the association between level affected in myelodysplasia and the incidence of scoliosis?
Higher incidence of scoliosis w higher spinal level affected. 100% scoliosis w T spine defects
826
What are the treatment options for scoliosis in myelodysplasia?
- Observation. Bracing is not effective - ASF and PSF
827
True or False: anterior spinal fusion is necessary when surgically treating scoliosis in kids w myelodysplasia
True. Dysplastic posterior column. Anterior fusion decreases the failure rate
828
What are the indications for surgical management of scoliosis in kids w myelodysplasia? How distal should the fusion be?
Curves >50deg that interfere w sitting. Needs to include the pelvis
829
How does surgical management of scoliosis in kids w myelodysplasia affect functional outcomes?
Halts progression of scoliosis 75% lose ambulation postop No clear benefit for sitting or quality of life
830
In kids w myelodysplasia, which spinal level has the highest risk of hip dislocation?
L3. Unopposed hip flexion and adduction
831
What is the treatment for hip dislocation in kids w myelodysplasia?
Can do soft tissue releases for better sitting position. Otherwise, just observe
832
What is Friedreich's ataxia?
Autosomal recessive mutation of Frataxin gene. Causes spinocerebellar degeneration
833
What is the "classis triad" seen in Friedreich's ataxia?
1. Ataxia: wide based gait 2. Areflexia 3. Positive Babinski
834
What conditions are associated w Friedreich's ataxia?
1. Cavovarus foot 2. Scoliosis (neuromuscular) 3. Cardiomyopathy
835
What is Marfan syndrome?
Autosomal dominant connective tissue disorder due to mutated fibrillin-1 (FBN1) gene
836
What orthopaedic conditions are associated w Marfan's syndrome? (6)
1. Scoliosis 2. Protrusio acetabuli 3. Pes planovalgus 4. Dural ectasia 5. Myelodysplasia 6. Pectus excavatum or carinatum
837
What nonorthopaedic conditions are associated w Marfan's syndrome? (3)
1. Cardiac: aortic root dilation, aortic dissection, mitral valve prolapse 2. Superior lens dislocation 3. Spontaneous pneumothorax
838
What condition is the main cause of death in Marfan's syndrome? How is it managed to decrease mortality?
Aortic root dilatation Manage w beta blockers and BP management
839
Describe the clinical presentation of Marfan's syndrome
1. Dolichostenomelia: arm span greater than height 2. Arachnodactyly: long digits 3. Ligamentous laxity: recurrent dislocation, ankle sprains 4. Thumb sign: thumb extends beyond D5 when clasped in palm 5. Wrist sign: thumb and small fingers overlap when wrapped around opposite wrist 6. Scoliosis 7. Pes planus
840
In patients w Marfan's, what preoperative workup is needed?
1. Cardiology consult and echo 2. Before spine OR: MRI for dural ectasia
841
What are the indications for surgical management of scoliosis in Marfan's? What preoperative workup is needed?
Indication: curves >50deg Need MRI to assess for dural ectasia All Marfan's patients need cardio consult and echo
842
True or False: the rate of complications after scoliosis surgery is higher in Marfan's than in adolescent idiopathic scoliosis
True Higher rate of fixation failure (bone eroded from dural ectasia), infection, pseudarthrosis, dural tear and revision rate Same rate of blood loss, postop neuro deficit, length of hospital stay
843
What are the treatment options for protrusio acetabuli in patient's w Marfan's?
Skeletally immature: close triradiate cartilage. Controversial Skeletally mature: - Young without arthritis: valgus osteotomy - Old w arthritis: THA
844
What is arthrogryposis?
Group of diseases that cause multiple congenital joint contractures due to poor movement in utero. Symmetric and nonprogressive Decreased anterior horn cells. Muscle replaced by fibrosis
845
What are the causes of arthrogryposis? (3)
Contractures due to poor movement in utero 1. Neurogenic (90%): brain, spine 2. Myopathic: muscular dystrophy 3: Fetal akinesia
846
Describe the clinical presentation of arthrogryposis (10)
1. Shoulder IR and adducted 2. Wrist flexion and ulnar deviation 3. Thumb in palm 4. Teratologic hip 5. Congenital knee dislocation 6. Congenital patella dislocation 7. Clubfeet 8. Vertical talus 9. Cavovarus 10. Scoliosis: C-shaped
847
What are the perioperative risks associated w arthrogryposis? (4)
1. Difficult intubation from stiff jaw 2. Intraoperative hyperthermia 3. Drug distribution affected by low muscle mass 4. Aspiration
848
What are treatment options for elbow extension contracture in the management of arthrogryposis?
1. Stretching and splinting 2. Triceps V-Y lengthening 3. Posterior capsulectomy 4. Steindler flexorplasty 5. Tendon transfer to biceps. Donors: triceps, pec major, lat dorsi
849
In arthrogryposis, why should tendon transfer for elbow extension be done one arm at a time?
May result in flexion contracture. Can't reach perineum In this case, better for one arm to be in extension and the other in flexion.
850
RC: In a child w arthrogryposis and elbow extension contracture, all of the following are possible surgical options to restore elbow flexion, except A. Triceps transfer B. Steindler flexorplasty C. Pec major transfer D. Anterior deltoid transfer
Answer: D 2019
851
RC: 12yo presents w an olecranon # while playing. He has had a previous contralateral olecranon # last year. He has normal height but bad dentition. He has had previous fractures to both distal radius and left tibia. What should you do? A. Start bisphosphonates B. Skeletal survey for child abuse C. Refer for renal workup
Answer: A Child likely has OI. Bad teeth suggest dentinogenesis imperfecta. Has had fragility #s. Treatment w bracing and bisphosphonates can reduce fractures, pain, and deformity. It can improve ambulation. (In real life, it may not be wrong to do renal workup too for renal osteodystrophy, etc) 2017
852
What is osteogenesis imperfecta?
Abnormal collagen cross linking from mutation in collagen 1 Abnormal collagen 1 produced and decreased production Osteoblasts don't form sufficient osteoid and poor remodeling
853
What are the orthopaedic manifestations of OI? (8)
1. Fractures 2. Ligamentous laxity 3. Short status 4. Spine abnormalities 5. Olecranon apophyseal # 6. Congenital radial head dislocation 7. Acetab protrusio 6. Coxa vara 7. Long bone bowing and saber shin
854
True or False: fractures in OI heal at the same rate as normal bone
True. Heals normally, but doesn't remodel
855
What spine abnormalities are associated w OI? (3)
1. Scoliosis 2. Codfish vertebrae: from compression # 3. Basilar invagination
856
What are nonorthopaedic manifestations of OI? (8)
1. Blue sclera 2. Triangle facies 3. Hearing loss 4. Dentinogenesis imperfecta 5. Wormian skull bones 6. Hypermetabolism: malignant hyperthermia, heat intolerance, tachycardiac 7. Thin skin: subQ hemorrhage 8. Cardiac: mitral valve prolapse, aortic regurgitation
857
What is the Sillence classification?
Types of osteogenesis imperfecta 1. Autosomal dominant. Mildest form 2. Autosomal recessive. Lethal 3. Autosomal recessive. Most severe survivable form 4. Autosomal dominant. Moderate severity. 5. Autosomal dominant. Congenital radial head dislocation. Rad/ulna and tib/fib interosseous ossification
858
What are management options in preventing fractures in OI?
1. Early bracing 2. Bisphosphonates Ineffective treatment: calcitonin, steroids, vitC, vitD, fluoride, magnesium, flavonoids
859
What are the benefits of early bracing in OI?
1. Decrease deformity: bowing, saber shin 2. Decrease fractures
860
What are the benefits of bisphosphonates in OI? What is the maximum duration of treatment?
1. Reduces fracture rate 2. Improves bone pain 3. Improves ambulation 4. Increases cortical diameter 5. Increases cancellous bone volume Treatment for max of 2yrs
861
What is the effect of bisphosphonates on scoliosis in OI?
None
862
What should you do w bisphosphonates in the perioperative period for OI?
Should hold. Otherwise interferes w bone healing
863
What is the management of scoliosis in OI?
Bracing: not an option as ribs are too fragile Posterior fusion: controversial. Maybe for curves >50deg. Bisphosphonates may improve bone to allow instrumentation
864
What are the fracture management options in kids w OI?
Kids <2yo: observation Kids >2yo: fixation w telescoping rods
865
What are the benefits of telescoping rods in the fixation of fractures in OI?
1. Restore alignment 2. Prevent bowing 3. Prevent #s 4. Avoids revisions as bone grows
866
True or False: the fracture rate decreases after skeletal maturity in OI
TRUE
867
What are surgical considerations for hip #s in adults w OI?
1. Image entire femur 2. Assess bowing/deformity 3. Shorter limb 4. Coxa vara 5. Acetab protrusio 6. IM nail preferred: custom or pediatric implant. Flexinail isn't rigid enough
868
What is osteopetrosis?
Defective osteoclast resorption. Fail to acidify Howship's lacuna Results in: - Dense bone - Brittle bone: unable to remodel - Obliterated canals
869
What is the clinical presentation of osteopetrosis like? (6)
1. Cranial n palsy due to skull foramina overgrowth 2. Osteomyelitis due to poor marrow vascularity 3. Spondylosis 4. Coxa vara due to femoral neck stress #s and nonunion 5. Fractures: risk malunion and delayed healing 6. Pancytopenia: bone encroaches on marrow. Bleeding risk, frequent infections
870
What cranial nerves are commonly affected in osteopetrosis? (4)
1. Optic n: most common. Present w vision loss 2. Auditory 3. Trigeminal 4. Facial
871
What are XR findings in osteopetrosis? (7)
1. Thick cortical bone 2. Increased bone density 3. Lose medullary canal diameter: "bone on bone appearance" 4. Erlenmeyer flask: proximal humerus, distal femur 5. Rugger jersey spine 6. Block femoral metaphysis 7. Coxa vara
872
What is the indication for nonoperative fracture management in osteopetrosis? What are the outcomes?
Indication: most fractures, as long as in good alignment Requires prolonged casting Outcomes: - Delayed healing - Limited remodeling - Risk refracture and malunion
873
What is the indication for operative fracture management in osteopetrosis? Describe the technique and outcomes
Indication: proximal femur #s Technique: - Plates/screws - Avoid IM nails as no canal - Slow and steady drilling. Constant cooling and change drill bit Outcomes: - Risk HW failure - Less nonunion, malunion and coxa vara risk compared to nonop
874
Describe the technique for THA in patients w osteopetrosis
Femur: cannulated reamer, short stemmed implant, uncemented Acetab: sharp reamers and multiple screws
875
What is Larsen's syndrome?
Disorder characterized by - Ligamentous laxity - Flattened facies - Prominent forehead
876
What must be assessed preop in patients w Larsen's syndrome?
Cervical kyphosis. At risk of cord injury w intubation
877
What is the clinical presentation of Larsen's syndrome? (6)
1. Flattened facies 2. Radial head dislocations 3. Hip dislocations 4. Knee dislocations 5. Foot deformities: clubfeet, equinovarus, equinovalgus 6. Spine: scoliosis, kyphosis
878
What is the management of cervical kyphosis in patients w Larsen's syndrome? (2)
1. Posterior only fusion: kyphosis w no neuro deficits. Do before 18mo 2. Anterior + posterior fusion: kyphosis w neuro deficits
879
What is the management for hip dislocations in Larsen's syndrome?
Open reduction. Indicated when unilateral dislocation Closed reduction is rarely successful. Controversial
880
What is the Beighton score?
Score of 5+ out of 9 indicates hypermobility - Forward flexion w palms on floor and knees fully extended - Small finger extends >90deg - Thumb abducts to forearm - Elbow hyperextends - Knee hyperextends
881
What is Ehlers-Danlos syndrome? What are orthopaedic manifestations?
Connective tissue disorder from mutated COL5A1 or 2 1. Joint mobility or dislocation 2. Ligamentous laxity 3. Early OA 4. DDH 5. Foot deformities: clubfoot, pes planus 6. Scoliosis 7. Fractures
882
What are nonorthopaedic manifestations of Ehlers-Danlos syndrome?
1. Skin - elastic, easily bruised 2. Poor wound healing 3. Fragile soft tissues: arterial, intestinal, uterine 4. Cardiac: aortic root dilatation, mitral valve prolapse
883
How is Ehlers-Danlos syndrome diagnosed? What single study is mandatory in all patients?
Skin biopsy for diagnosis All require echo to R/O aortic root dilatation
884
Disproportionate dwarfism can be divided into short trunk and short limb. Which disorders fall under each category?
Short trunk: SED and Kniest Short limb: achondroplasia, pseudoachondroplasia, MED
885
Define rhizomelic, mesomelic, acromelic
Rhizomelic: proximal segment (humerus and femur) are hypoplastic Mesomelic: middle segment (rad/ulna, tib/fib) Acromelic: hands/feet
886
When first assessing dwarfism, what sort of workup should be performed for diagnosis?
1. Sitting and standing height 2. Skeletal survey 3. Genetics referral 4. Labs: calcium, phosphate, alkphosh, serum thyroxin, urine for storage products
887
What is achondroplasia? What is the clinical presentation?
Autosomal dominant, short limb disproportionate dwarfism FGFR3 mutation 1. Hypotonia in infancy and delayed milestones 2. Face: frontal bossing and midface hypoplasia 3. Foramen magnum stenosis 4. Lumbar stenosis 5. Thoracolumbar kyphosis 6. Rhizomelic dwarfism 7. Trident hands 8. Genu varum and fibular overgrowth 9. Radial head subluxation and humerus posterior bowing
888
What part of the bone is affected in achondroplasia?
Hypertrophic zone of physis
889
What are typical pelvic XR findings in achondroplasia?
Champagne glass pelvis: wider than deep Horizontal acetab roof Squared iliac
890
What are typical femur XR findings in achondroplasia?
Flared metaphysis Inverted V distal femur physis Normal distal epiphysis
891
What are typical spine XR findings in achondroplasia?
Short pedicles Decreased interpedicular distance Vertebral wedging in kyphosis Posterior vertebral scalloping
892
Why do achondroplastic infants have high mortality rates?
Foramen magnum stenosis
893
What is the clinical presentation of foramen magnum stenosis? What is the workup?
1. Central sleep apnea 2. Snoring 3. Difficulty swallowing 4. Hypotonia 5. Myelopathy: hyperreflexia, clonus, weakness Workup: sleep study and MRI
894
What is the treatment of foramen magnum stenosis in achondroplasia? At what age is it usually treated?
Usually requires tx by 2yo Foramen magnum decompression. Duraplasty if persistent compression after bone removed Intraop US to confirm decompression
895
What is the pathophysiology of thoracolumbar kyphosis in achondroplasia? What is the prognosis?
Infants w achondroplasia have trunk hypotonia and slump forward. Causes anterior vertebral wedging 90% improve by 18mo Persistent kyphosis causes lumbar hyperlordosis, worsened spinal stenosis, hip flexion contracture
896
What are management options for thoracolumbar kyphosis in achondroplasia?
1. Observation: most resolve due to trunk strength and walking 2. Bracing: TLSO until independent walking 3. Surgical correction: neuro compromise or >50deg
897
How does rhizomelia in achondroplasia negatively affect function?
Can't reach head or perineum Treatment: lengthening
898
RC: 3yo child presents w ataxic gait and rhizomelic dwarfism, frontal bossing and midface hypoplasia. He has a history of sleep apnea. He has a gibbus deformity and is hyperreflexic on exam. What is the next appropriate step? A. MRI of C1-2 B. MRI of foramen magnum C. EMG D. Sleep study
Answer: B Patient likely has achondroplasia. He presents w myelopathy and sleep apnea, likely from foramen magnum stenosis. All achondroplasia patients should have a sleep study and MRI of their foramen magnum. In this case, the patient already has symptoms suggestive of stenosis so they just jumped to MRI instead of formal sleep study. Reason is likely because stenosis causes high mortality in infants and delaying treatment for a sleep study may not be wise.
899
What is pseudoachondroplasia?
Short limbed disproportionate dwarfism due to mutated COMP gene Rhizomelia Delayed epiphysis ossification. Irregular and fragmented epiphysis
900
What is the clinical presentation of pseudoachondroplasia? (4)
1. Gait: waddling 2. Early OA 3. Genu valgum/varum 4. Odontoid hypoplasia causing atlantoaxial instability
901
What are typical hip XR findings in pseudoachondroplasia?
Delayed ossification. Head appears small and fragmented like Perthes or SED Sclerosis Incongruity Subluxation
902
What are management options for early hip OA in pseudoachondroplasia?
Early hip OA due to incongruity and subluxation 1. Varus femoral osteotomy to improve incongruity 2. Pelvic osteotomy. Salvage options only 3. THA
903
RC: All of the following are associated w atlantoaxial instability except A. Achondroplasia B. Pseudoachondroplasia C. Mucopolysaccharidoses D. Down's syndrome
Answer: A (achondroplasia) Achondroplasia is associated w foramen magnum stenosis, lumbar stenosis and kyphosis Pseudoachondroplasia has odontoid hypoplasia causing atlantoaxial instability Mucopolysaccharidoses (Morquio and Hurler Down associated w both atlantoaxial and atlantooccipital instability 2015
904
What is multiple epiphyseal dysplasia?
Short limb disproportionate dwarfism Autosomal dominant mutation in COMP and COL9 Irregular and delayed ossification at multiple epiphyses
905
What areas of the body are most commonly affected by MED? (2)
1. Proximal humerus 2. Proximal femur
906
Describe the clinical presentation of MED
1. Short limbs 2. Gait: waddling 3. Stubby fingers/toes 4. Hip: early OA 5. Valgus knees
907
Describe how the epiphyses appear on XR in MED
Multiple epiphyses affected (duh) Delayed ossification Fragmented Flattened
908
Describe how to differentiate MED from bilateral Perthes?
When bilateral Perthes suspected, order skeletal survey to R/O MED MED is different from Perthes in that: - Symmetric and bilateral presentation. Perthes is not synchronous - Early acetabular changes - No metaphyseal cysts
909
Describe typical knee XR findings in MED (3)
1. Valgus 2. Flat femoral condyles 3. Double layer patella
910
Describe typical hand and foot XR findings in MED
Short metacarpals and metatarsals. Result in stubby fingers and toes
911
What is the DDx of MED?
Spondyloepiphyseal dysplasia (SED): mutation in COL2 and involves spine. Shor trunk dwarfism MED has mutated COL9 and COMP. No spine involvement. Short limb dwarfism
912
What are treatment options for MED? (4)
1. NSAIDs and PT for early OA 2. Proximal femur osteotomy for subluxation. Varus osteotomy for containment. Valgus for hinge abduction 3. Pelvic osteotomy for containment. Usually salvage options 4. Knee: osteotomy vs hemiepiphysiodesis for genu valgum
913
What is spondyloepiphyseal dysplasia (SED)?
Short trunk disproportionate dwarfism due to mutated COL2A1. Causes defective epiphysis ossification. Affects spine and limbs
914
What is the clinical presentation of SED? (7)
1. Eyes: myopia (wear big glasses) and retinal detachment 2. Midface hypoplasia 3. Atlantoaxial instability (myelopathy) 4. Kyphoscoliosis 5. Thoracic dysplasia causing respiratory insufficiency 6. Gait: waddling 7. Coxa vara 8. Genu varum
915
What are typical C-spine XR findings in SED? What views should be ordered?
AP, lateral, open mouth views Flexion-extension 1. Atlantoaxial instability 2. Odontoid hypoplasia 3. Os odontoideum: dens separated from C2 body
916
What are typical hip XR findings in SED?
1. Horizontal acetab roof 2. Coxa vara 3. Delayed head ossification
917
Describe the general management of SED
1. Ophthalmology for myopia and retinal detachment 2. Pulmonology for resp insufficiency 3. Posterior atlantoaxial fusion for atlantoaxial instability 4. Posterior thoracolumbar fusion for scoliosis >50deg 5. Proximal femur valgus producing osteotomy
918
What are the indications for operative management of atlantoaxial instability in kids w SED?
1. ADI >8mm on flex-ex. Normal ADI in nonsyndromic kids is <5mm 2. Myelopathy
919
What are the indications for valgus producing proximal femur osteotomy in kids w SED?
1. Coxa vara <100deg 2. Symptomatic hip OA
920
RC: 7yo w SED. You are planning to do surgery for the patient's knee deformity. What do you order pre-op? A. C-spine flex-ex B. Scoliosis series C. MRI brain D. Polysomnography
Answer: A (flex-ex) SED at risk for atlantoaxial instability due to odontoid hypoplasia or os odontoideum 2015
921
What is Kniest dysplasia?
Short-trunk disproportionate dwarfism due to mutated COL2A1
922
What are orthopaedic manifestations of Kniest dysplasia?
1. Hypoplastic pelvis and spine 2. Dumbbell-shaped metaphyses 3. Coxa vara 4. Genu valgum 5. Kyphoscoliosis 6. Early OA
923
What are nonorthopaedic manifestations of Kniest dysplasia?
1. Flat facies 2. Cleft palate 3. Resp insufficiency 4. Eyes: myopia and retinal detachment 5. Ears: otitis media and hearing loss
924
What is diastrophic dysplasia?
Short-limb disproportionate dwarfism due to DTD gene Rhizomelia Causes defective epiphysis ossification
925
What is the clinical presentation of diastrophic dysplasia
1. Face: cleft palate and cauliflower ears 2. Arms: poorly developed. Hitchhiker thumb 3. Teratologic DDH 4. Genu valgum 5. Foot deformities: skew foot, clubfoot 6. Atlantoaxial instability 7. Cervical kyphosis 8. Scoliosis
926
What is the general treatment of diastrophic dysplasia?
1. Cauliflower ears: compressive bandages 2. DDH: femur and pelvic osteotomy 3. Genu valgum: corrective osteotomies 4. Foot deformity correction 5. Atlantoaxial instability: posterior fusion if high ADI or neuro sx 6. Cervical kyphosis: most resolve spontaneously. Fusion if doesn't resolve 7. Scoliosis: fusion if severe
927
What is mucopolysaccharidoses (MPS)?
Group of metabolic disorders due to deficiency lysosomal enzymes. Incomplete breakdown products (mucopolysaccharides) accumulate. Causes proportionate dwarfism
928
List common subtypes of mucopolysaccharidoses (MPS). What is the inheritance pattern?
Autosomal recessive 1. Sanfilippo: most common 2. Morquio 3. Hurler X-linked recessive 4. Hunter
929
What are diagnostic studies for MPS?
1. Toluidine blue spot test: detect excess mucopolysaccharide. If positive, specific blood test for type of MPS 2. Skin fibroblast culture: test enzyme activity 3. Chorionic villous sampling
930
True or False: bone marrow transplant in MPS improves life expectancy
True. But does not alter orthopaedic manifestations
931
What is San Filippo syndrome?
Mucopolysaccharidoses. Autosomal recessive. Most common MPS Accumulate heparan sulfate (found in urine) Multiple enzyme deficiencies
932
What is the clinical presentation of San Filippo?
1. Proportionate dwarfism 2. Mental retardation
933
What is the treatment of San Filippo?
Bone marrow transplant
934
What is Morquio syndrome?
MPS. Autosomal recessive Accumulate keratin sulfate in urine Deficient galactosamine-6-sulfate sulphatase or beta-galactosidase
935
What is the clinical presentation of Morquio syndrome?
1. Proportionate dwarfism 2. Normal intelligence 3. Atlantoaxial instability from odontoid hypoplasia 4. Thoracic kyphosis 5. Genu valgum 6. Corneal clouding
936
What is the treatment of Morquio?
1. OR for thoracic kyphosis and atlantoaxial instability if severe 2. Limb realignment osteotomy
937
What is Hurler syndrome?
Most severe MPS. Also known as gargoylism. Autosomal recessive Accumulate dermatan and heparan sulfate in urine Due to alpha-L iduronidase deficiency
938
What is the clinical presentation of Hurler syndrome?
1. Proportionate dwarfism 2. Mental retardation 3. Gargoyle facies 4. Corneal clouding 5. Atlantoaxial instability 6. Carpal tunnel
939
What is the treatment of Hurler syndrome?
Bone marrow transplant
940
Which mucopolysaccharidoses are associated w carpal tunnel?
Hunter and Hurler
941
What is Hunter syndrome?
MPS. X-linked recessive Accumulate dermatan and heparan sulfate in urine Present w proportionate dwarfism and mental retardation
942
RC: Which of the following is not true? A. Duchenne is associated w calf hypertrophy B. San Filippo is the most common mucopolysaccharidoses C. Mucopolysaccharidoses is proportionate dwarfism D. Marfan's is associated w arachnodactyly
Answer: A Duchenne is associated w calf pseudohypertrophy Some studies say Morquio is the most common MPS. It depends if the answer specifies incidence vs prevalence. San Filippo has the highest incidence but because the early death rate is so high, the prevalence is lower than Morquio 2019, 2015
943
RC: Which of the following is not true? A. San Filippo is the most prevalent type of mucopolysaccharidoses B. Marfan is associated w arachnodactyly C. Morquio is associated w atlantoaxial instability D. Duchenne's is associated w calf pseudohypertrophy
Answer: D Indeed Duchenne is associated w pseudohypertrophy. Previous questions mentioned just hypertrophy, which is wrong. San Filippo probably has the highest incidence but Morquio may be more prevalent. This is because San Filippo has a high early death rate. If it asks which MPS is the most common overall, it would be San Filippo (JAAOS 2013) 2013
944
What is Down syndrome?
Trisomy 21. Causes ligamentous laxity and muscle hypotonia. Chr 21 codes for COL6
945
What are orthopaedic manifestations of Down syndrome? (10)
1. Ligamentous laxity 2. Atlantooccipital instability 3. Atlantoaxial instability 4. Delayed motor milestones 5. Hip subluxation/dislocation 6. SCFE 7. Patella instability 8. Scoliosis 9. Spondylolisthesis 10. Pes planus
946
What are nonorthopaedic manifestations of Down syndrome?
1. Flattened facies, upward slanting eyes, epicanthal folds, single palmar crease 2. Cardiac: ASD/VSD 3. Hypothyroid 4. Premature aging 5. Duodenal atresia 6. Alzheimer
947
What is the management of atlantooccipital instability in Down syndrome?
Limit contact sports Posterior fusion if neuro deficits
948
What preoperative workup is required in Down syndrome?
C-spine flex ex for atlantoaxial instability
949
What are the indications for nonoperative vs operative management of atlantoaxial instability in Down syndrome?
Based on ADI and SAC - Normal ADI in nonsyndromic kids is <5mm. <10mm is acceptable in Down - SAC >14 is normal Nonop if ADI <10mm, SAC >14mm and neuro intact OR if ADI >10mm, SAC <14mm or neuro deficit
950
In Down syndrome kids w hip instability, where is the area of acetab deficiency?
Posterior acetab deficiency
951
What are the management options for Down syndrome kids w hip instability?
Based whether there are bony changes or dislocation. Options: - Abduction brace if neither - Femoral and pelvic osteotomy
952
What are specific management considerations in Down syndrome kids w SCFE?
R/O concomitant hypothyroidism Must pin contralateral side as well
953
RC: All of the following regarding Down syndrome and c-spine instability are true, except A. Only affects C1-2 B. >25% have instability C. C-spine XRs can't predict progression D. Screening XR for participation in sports it unnecessary if they are asymptomatic
Answer: A Down syndrome also causes occipitocervical instability C-spine XR can't predict progression. Screening XR also not effective or warranted prior to sports participation Down's patients should probably avoid impact sports though 2016
954
What is an accessory navicular? What attaches to it?
Autosomal dominant, normal variant. Tuberosity fails to fuse to rest of bone Post tib inserts onto tuberosity Presents as either: - Separate accessory bone - Accessory bone connected to navicular by synchondrosis - Bony medial extension of navicular
955
What foot conditions result from an accessory navicular? (2)
1. Pes planus 2. Posterior tib deficiency
956
What is the clinical presentation of accessory navicular?
Mostly asymptomatic Medial arch pain worse w overuse Repeated microfracture at synchondrosis Inflamed post tib tendon Pes planus Post tib insufficiency
957
What are the management options for an accessory navicular? (3)
1. Orthosis: medial wedge, longitudinal arch, UCBL cup 2. Cast immobilization 3. Excision
958
What is Kohler's disease?
Navicular AVN. Ages 4-7yo, 4x more common in boys Due to watershed in middle third Dorsum supplied by dorsalis pedis Plantar supplied by medial plantar artery
959
What is the prognosis for Kohler's disease?
Self limiting. Symptoms resolve after 1-3yrs. XR improves later
960
What is the clinical presentation of Kohler's disease?
Mostly asymptomatic Midfoot pain Antalgic gait
961
What are the XR findings in Kohler's disease?
Sclerosis, fragmentation, flattening of navicular
962
What is the treatment of Kohler's disease?
NSAIDs for symptom control Immobilize w cast if pain Surgery not indicated
963
What is Sever's disease?
Calcaneal apophysitis from jumping/running Ages 6-12yo
964
What is the treatment of Sever's disease?
Achilles tendon stretches NSAIDs Cast immobilization Surgery not indicated
965
What is Osgood Schlatter's disease?
Tibial tubercle apophysitis Ages 12-15yo Running and jumping sports
966
What is the clinical presentation of Osgood Schlatter? What provocative test can be performed?
Anterior knee pain, worse w kneeling Enlarged tib tubercle Provocative test: pain w resisted knee extension
967
What are typical XR findings of Osgood Schlatter?
Irregular, fragmented tibial tubercle
968
What is the DDx of suspected Osgood Schlatter disease?
Sinding-Larsen-Johansson syndrome: inferior patella apophysitis Proximal tibial osteochondroma Tib tubercle #
969
What is the treatment for Osgood Schlatter disease?
NSAIDs Quads stretching Cast immobilization
970
What is epiphyseal bracket?
AKA delta phalanx. Congenital disorder of epiphysis affecting phalanx. Distal and proximal phalanx connect w extension of epiphysis along the diaphysis. Forms C-shaped curve. Diaphysis appears D-shaped
971
What conditions are associated w epiphyseal bracket? (4)
1. Hallux varus 2. Clinodactyly 3. Polydactyly 4. Apert syndrome
972
What are the treatment options for epiphyseal bracket?
Pre-op splinting - loosen soft tissues 1. Physiolysis and fat interposition: skeletally immature. Resect bracket. Leave normal epiphysis at ends 2. Corrective osteotomy: after epiphyseal bracket closure
973
What is congenital hallux varus
AKA atavistic great toe Deformity at 1st MTP. Toe is short + thick Forms after walking age Possible causes: - Abductor hallucis overpowering adductor - 1st MT bracket epiphysis
974
What are XR findings in congenital hallux varus?
1. Short + thick 1st MT 2. Possible epiphyseal bracket
975
True or False: congenital hallux valgus can be managed w observation only
False. Should surgically correct in infancy. Worsens w time
976
What are treatment options for congenital hallux varus? (2)
1. Adductor hallucis release: mild deformity 2. Physiolysis and fat interposition of epiphyseal bracket 3. Farmer technique: create syndactyly between hallux + 2nd toe
977
What is toe syndactyly? What conditions is it associated with? Does it cause functional disability?
Fusion of bone or skin due to incomplete apoptosis in utero Autosomal dominant Assoc w Down syndrome + Klippel-Feil Cosmetic concern only
978
What is the treatment for toe syndactyly?
Observation: for simple syndactyly (soft tissue only) Digit release: for complex syndactyly (bone fusion)
979
What is toe polydactyly? Which toes are most commonly affected? Does it cause functional disability? What is the treatment?
Extra toes. Most commonly affects postaxial side Issues w shoes Treatment: - Observation for postaxial or central toes - Ablation for malaligned toes, esp if preaxial. Usually ablate border digit
980
What is toe oligodactyly? Which side is most commonly affected?
Absent toes Most common: lateral rays
981
What conditions are associated w toe oligodactyly?
1. Fibular hemimelia 2. Tarsal coalition 3. VACTERL 4. Fanconi 5. Hand and foot: polydactyly, syndactyly, brachydactyly, constriction ring
982
What is brachymetatarsia? Which toe is most commonly affected?
Hypoplasia of one or more toes Most common: 4th MT
983
What conditions are associated w brachymetatarsia?
1. Down syndrome 2. Turner's 3. Larsen 4. Diastrophic dwarfism
984
What are XR findings in brachymetatarsia?
Shortened MT Disrupted metatarsal parabola
985
What is the treatment for brachymetatarsia?
- Shoe modification: wider shoes - Metatarsal lengthening - Amputation
986
What is local gigantism (macrodactyly)? What are possible etiologies?
Macrodactyly of a finger or toe Possible etiology - Neurofibromatosis - AV malformation - Tumor - Acromegaly - Proteus
987
What are the treatment options of toe macrodactyly?
- Observation - Epiphysiodesis vs bony/soft tissue reduction - Amputation
988
What is the cause of overlapping toes? What toe is most commonly affected?
Toe overlaps w another due to EDL contracture Most common: 5th toe
989
What are the treatment options for overlapping toes?
1. Passive stretching and buddy taping 2. Surgical correction: - Butler procedure: release EDL - Create syndactyly w 4th toe
990
What is congenital curly toe? What is the cause? Which toes are most commonly affected?
Flexion + varus deformity of IP joint Due to FDL + FDB contracture Most common - lateral 3 toes
991
What is the treatment of congenital curly toes?
- Observation - Flexor tenotomy
992
What are the 3 signal centers of the limb bud during embryonic development?
1. Apical ectodermal ridge (AER) 2. Zone of polarizing activity 3. Wnt signalling center
993
What is the AER and how does it affect limb development? What abnormalities are associated w a defective AER?
Proximal-distal development. Secretes FGF Defective AER causes limb truncation such as: - Central deficiency (cleft hand) - Radial deficiency (radial clubhand)
994
What is the zone of polarizing activity and how does it affect limb development?
Secretes shh for anterior-posterior (radioulnar) axis High shh on ulnar side: 5th finger development Low shh on radial side: thumb development
995
What abnormalities are associated w a defective zone of polarizing activity?
Ulnar abnormalities: too much shh causes ulnar polydactyly, too little causes oligodactyly Radial abnormality: too much shh causes no thumb
996
What is radial deficiency?
AKA radial clubhand. Longitudinal deficiency of radius Often also deficient thumb
997
What syndromes are associated w radial deficiency?
1. TAR 2. Fanconi anemia 3. Holt-Oram syndrome 4. VACTERYL
998
What is TAR?
Thrombocytopenia, Absent Radius Autosomal recessive Assoc w Trisomy 18 Thumb is present
999
What is Fanconi anemia?
Autosomal recessive. Life threatening Causes pancytopenia. Need CBC to workup Need bone marrow transplant to survive
1000
What is Holt Oram syndrome?
Autosomal dominant Causes cardiac deficits: atrial/septal defects, arrythmias Workup w echo
1001
What tests should be ordered to rule out associated conditions in radial deficiency?
1. CBC 2. Renal US 3. Echo 4. Chromosomal challenge test: detects Fanconi between bone marrow failure
1002
What is VACTERYL?
Vertebral anomalies Anal atresia Cardiac abnormalities Tracheoesophageal fistula Renal agenesis Limb defects
1003
What is the Bayne and Klug classification?
Types of radial deficiency 1. Deficient distal epiphysis 2. Deficient distal + proximal epiphysis 3. Partial absence: proximal radius is present 4. Absent. Most common
1004
What is the clinical presentation of radial deficiency?
1. Hand and wrist are perpendicular towards radial side 2. Hypoplastic or absent thumb common 3. Elbow ROM may be limited
1005
What are the treatment options for radial deficiency?
1. Passive stretching 2. Observation 3. Wrist centralization 3. Wrist radialization
1006
What are the indications and goals of passive stretching for radial deficiency?
Indication: tight radial sided structures Goal: passive correction of radial wrist deviation. Needed preop
1007
What are the indications for observation of radial deficiency?
1. Minimal deformity 2. Good functional compensation 3. No elbow ROM (stuck in extension): rely on radial deviation for feeding 4. Biceps function deficient (^ same reasoning)
1008
What are goals of operative management for radial deficiency? Ideally, at what age should it be surgically treated?
Goals: - Limb length - Straighten forearm - Reconstruct thumb Forearm and thumb should be staged procedures May need preoperative stretching of radial tissues before wrist realignment Wrist alignment at 6-12mo, then thumb procedure 6mo later Complete all reconstruction by 18mo
1009
What sort of elbow functional is required if considering reconstructive surgery for radial deficiency?
Good elbow ROM, intact biceps function
1010
Radial structures need to be stretching preoperatively when considering wrist realignment for radial deviation. What are some ways of doing this?
Need to be able to passively correct radial wrist deviation Splinting Ex-fix distraction device
1011
What are contraindications to wrist realignment surgery for radial deficiency?
1. Older kids w good functional compensation 2. Elbow extension contracture or poor biceps function. They relay on radial deviation for feeding
1012
When performing wrist realignment surgery for radial deviation, what soft tissue structures may tether the wrist and prevent reduction? (4)
1. Radial wrist extensors 2. Brachioradialis 3. FCR 4. Radial anlage Resect anlage. Release distal insertions of these tendons ^
1013
For wrist realignment surgery for radial deficiency: if you are still unable to reduce the wrist after releasing soft tissue tethers, what are your next options?
1. Shortening: distal ulnar shaving or carpal resection 2. Ex-fix soft tissue distraction device
1014
Describe the general principles of wrist centralization for radial deficiency?
Align ulna w D3 Temporarily fix w pin through D3 to carpus to ulna Transfer ECU distally. Transfer FCU to dorsal wrist
1015
Describe the general principles of wrist radialization for radial deficiency
Overcorrect radial deviation Align ulna w D2 Transfer FCR, brachioradialis and radial wrist extensors ulnarly
1016
What conditions are associated w thumb deficiency?
1. Radial deficiency 2. TAR 3. Fanconi anemia 4. Holt Oram 5. VACTERYL
1017
What is the Blauth classification?
Grades of thumb deficiency 1. All structures present, just small 2. All bony structures present. Unstable MCP and thenar hypoplasia 3. Deficient metacarpal and abnormal FPL/EPL 3A. stable CMC joint 3B. unstable CMC 4. Floating thumb (pouce flottant). No metacarpal 5. Completely absent
1018
What is the clinical presentation of thumb hypoplasia? (6)
1. Smaller or absent thumb 2. Excessive MCP joint abduction 3. Hypoplastic thenar muscles 4. Pollex abductus: abnormal connection between FPL + EPL 5. Webspace tightness 6. Assess if MCP or CMC joint unstable
1019
What tests should be ordered to rule out associated conditions in thumb hypoplasia? (4)
1. CBC 2. Renal US 3. Echo 4. Chromosomal challenge test: detects Fanconi between bone marrow failure
1020
List the treatment options for thumb hypoplasia (4)
1. Observation 2. Opponensplasty 3. Opponensplasty and 1st MCP stabilization 4. Ablation and pollicization
1021
What are the indications for observation in the management of thumb hypoplasia?
Blauth type 1 (all structures present but hypoplastic), as long as there is sufficient thumb abduction
1022
What are the indications for opponensplasty in the management of thumb hypoplasia? What tendons are used?
Blauth type 1 (all structures present but hypoplastic) w insufficient thumb abduction 1. FDS of F4 2. Abductor digiti minimi Transferred to APB Note: if opponensplasty done for medial n palsy, EIP is also an option
1023
What are the indications for opponensplasty and 1st MCP stabilization? Describe the general principles of the procedure
Indications: Blauth type 2 (all bones present but unstable MCP) and 3A (hypoplastic MC, stable CMC jt) Must also do webspace deepening w z-plasty 3 options for MCP stabilization: 1. MCP fusion 2. UCL reconstruction w FDS tendon from opponensplasty 3. UCL reconstruction w free tendon graft
1024
What are the indications for ablation and pollicization in the management of thumb hypoplasia? Describe the general principles
Indications: type 3B - 5 Shorten D2 metacarpal Rotate and pronate: 120deg pronation, 4deg abduction, 15deg extension Reattach and balance muscles
1025
What is ulnar deficiency? Which joints are unstable as a result?
AKA ulnar clubhand Usually unstable elbow and stable wrist. But can be vice versa 10x less common than radial deficiency
1026
What conditions are associated w ulnar deficiency?
Not associated w systemic conditions like radial deficiency 1. PFFD 2. Fibular deficiency 3. Scoliosis 4. Phocomelia 5. Hand abnormalities: most have absent ulnar digits
1027
What is the clinical presentation of ulnar deficiency?
Shortened, bowed forearm Decreased elbow function Absent ulnar digits
1028
What is the Bayne classification?
Types of ulnar deficiency 0: hand deficiencies only 1: smaller ulna and both physis present 2: part of ulna missing (usually distal end) 3: absent ulna 4: radiohumeral synostosis
1029
What are treatment options for the management of ulnar deficiency?
1. Stretching and splinting 2. Radial head resection for one bone forearm. If syndactyly present then stage and do syndactyly release first. Improves stability but lose forearm ROM 3. Radiohumeral synostosis osteotomy
1030
What is madelung deformity? What is the inheritance pattern?
Disrupted distal radius physis (ulnar/volar aspect) due to tethering by Vicker's ligament Autosomal dominant Results in deficient growth, causing - Excessive radial inclination and volar tilt - Ulnar carpal impaction (positive ulnar variance)
1031
What is Vicker's ligament?
Pathologic short volar radiolunate ligament that causes tethering. Results in madelung deformity
1032
What is the clinical presentation of madelung deformity?
- Asymptomatic until adolescent - Ulnar impaction - Medial n irritation - Restricted prosupination - Carpus dislocates radial and volar
1033
What are typical XR findings in madelung deformity?
1. Dorsal/volar bowing of radius 2. Excessive radial inclination and volar tilt 3. Positive ulnar variance 4. DRUJ displaced dorsally 5. V-shaped deformity at DRUJ with lunate herniated at apex 6. Carpus subluxed radial and volar
1034
What are the treatment options for madelung deformity?
1. Activity modification: avoid repetitive impact. NWB until pain improves 2. Physiolysis w Vicker ligament release 3. Radial corrective osteotomy and ulnar shortening osteotomy 4. DRUJ arthroplasty: controversial. Indicated when DRUJ instability in additional to usual pain and limited ROM
1035
Describe the general principles of physiolysis in the management of madelung deformity
Volar approach Release Vicker's ligament Bar resection and fat grafting
1036
Describe the general principles of radial corrective osteotomy in the management of madelung deformity
Volar approach Dome osteotomy: allows coronal and sagittal correction Distal ulnar shortening osteotomy 2016, 2014, 2013
1037
RC: All of the following are true regarding Madelung's deformity, except A. Caused by an abnormality of the volar aspect of the distal radius growth plate B. There will be ulnar positive variance C. Caused by an abnormality of the dorsal ulnar aspect of the distal radius growth plate D. The carpus will dislocate volarly
Answer: C Madelung due to abnormal volar and ulnar aspect of distal radius physis
1038
Describe the clinical presentation of congenital radial head dislocation
1. Asymptomatic 2. Limited ROM, esp in extension and supination. AKA pronation contracture 3. Prominent radial head, posterior dislocation 4. Bilateral Differentiate from traumatic dislocation
1039
What are the treatment options for congenital radial head dislocation?
- Observation: 1st line - Radial head resection: in adulthood for pain, may improve ROM
1040
RC: a child presents w a congenital radial head dislocation. What is true about treatment w radial head excision? A. She will develop cubitus valgus B. Her radius will migrate proximally C. She is likely to develop a radioulnar synostosis D. The radial head will regrow
Answer: likely D Much discussion about this. Complications of excision include cubitus valgus, ulnar neuropathy, proximal migration, synostosis and reformation of the radial head. Reformation is the most common complication 2016
1041
What is congenital radioulnar synostosis? What is the inheritance pattern?
Proximal bony bridge between radius and ulna. Due to failure of separation in utero Forearm starts as single cartilaginous anlage that divides from distal to proximal Autosomal dominant
1042
What is the clinical presentation of congenital radioulnar synostosis?
1. Diagnosed at 6yo when noticed by parents 2. Limited prosupination, becomes fixed at 30deg pronation 3. Compensatory motions by shoulders and wrists
1043
What are typical XR findings in congenital radioulnar synostosis? (2)
1. Proximal synostosis 2. Radial head may be dislocated or malformed
1044
What is the Cleary classification?
Types of congenital radioulnar synostosis 1. No osseous synostosis 2. Osseous synostosis 3. Long osseous synostosis. Radial head hypoplastic, posteriorly dislocated 4. Short osseous synostosis w mushroom-shaped radial head, anteriorly dislocated
1045
What are the treatment options for congenital radioulnar synostosis?
1. Observation 2. Synostosis excision 3. Forearm derotational osteotomy
1046
What are the indications for observation in the management of congenital radioulnar synostosis?
Asymptomatic and unilateral
1047
What are the indications for surgical management in congenital radioulnar synostosis?
- Functionally limiting - Severe pronation >60deg - Bilateral
1048
Describe the general principles of synostosis excision in the management of congenital radioulnar synostosis. What are the outcomes?
Excise synostosis Interpose w vascularized fat Outcomes: slight gain in ROM but generally unsatisfactory No graft interposition causes 100% recurrence
1049
Describe the general principles of forearm derotational osteotomy for congenital radioulnar synostosis
Perform at 5yo 1. Osteotomy: 3 options - Radial and ulnar diaphysis at synostosis - Radial and ulnar diaphysis distal to synostosis and at different levels - Distal radius diaphysis alone 2. Correction: immediately at osteotomy, delayed 10d after osteotomy, or gradually w circular exfix 3. Stabilize correction: cast alone, circular exfix or per pins
1050
What are the risks of doing an osteotomy at the synostosis in the management of congenital radioulnar synostosis?
Narrow space. Risk of - Soft tissue tightness - Loss of correction - Neurovascular compromise
1051
What are the benefits of doing the radial and ulnar osteotomies at different levels in the management of congenital radioulnar synostosis?
Distributes rotational correction so - Less soft tissue tightness - Less neurovasc risk
1052
List specific complications associated w surgical management of radioulnar synostosis
1. Synostosis recurrence: less w vascularized fat graft 2. Malrotation/loss of correction due to casting after osteotomy 3. Compartment syndrome: assoc w large rotational corrections 4. Neuro deficit, esp PIN. Higher risk w synostosis osteotomy, immediate correction, large correction
1053
RC: What is true regarding congenital radioulnar synostosis? A. Supination contracture B. Does not improve w resection C. Is typically in the distal or medial forearm D. 2:1 male to female ratio
Answer: B (does not improve w resection) This is the most true answer. Causes pronation contracture. Resection w interposition is generally unsatisfactory w only slight gain in ROM. Typically occurs at proximal forearm 3:2 male to female ratio.
1054
What is cleft hand?
Lobster claw due to absent central digits Usually bilateral, absent metacarpals. May have syndactyly
1055
What is symphalangism?
Congenital digital stiffness. Due to failure of separation of IP joints More common in ulnar digits
1056
What is the treatment for symphalangism?
Observation. Usually adequate function. Capsulectomy and IP joint arthroplasty not effective Osteotomy and arthrodesis rarely needed
1057
What is camptodactyly?
Congenital digital flexion, usually at 5th PIP joint Possible causes - Abnormal lumbrical insertion/origin - Abnormal FDS insertion
1058
What is the Benson classification?
Types of camptodactyly 1. Isolated to 5th finger and presents in infancy. Most common 2. Isolated to 5th finger and presents in adolescence 3. Severe contractures of multiple digits. Presents at birth and assoc w a syndrome
1059
What is the treatment of camptodactyly?
Depending on Benson type: 1. Stretching and splinting 2. FDS transfer to lateral band if PIP extension achieved w active MCP extension (tenodesis) 3. Nonop. After maturity, can do corrective osteotomy and fusion if functional deficit
1060
What is clinodactyly?
Congenital curve of finger. Most common in 5th middle phalanx Assoc a Down's syndrome Significant angulation due to bracket epiphysis (delta phalanx)
1061
What is the treatment of clinodactyly?
Observation for most cases. Opening osteotomy if digit encroaches on adjacent digit
1062
What is finger syndactyly? Which fingers are most common?
Failure of apoptosis to separate fingers Most common: D3/4 > D4/5
1063
What conditions are associated w finger syndactyly?
1. Acrosyndactyly: digits fuse distally, fenestrations proximally 2. Poland syndrome 3. Apert syndrome 4. Carpenter syndrome: craniofacial malformation, obesity and syndactyly
1064
Describe the finger syndactyly classification
- Simple: only soft tissue - Complex: bony fusion - Complications: accessory phalanges or abnormal bones - Complete: skin extends to finger tips - Incomplete: skin fusion doesn't extend to tips
1065
At what age should finger syndactyly release be performed?
Acrosyndactyly: as neonate Syndactyly: at 1yo Bilateral syndactyly: do simultaneously if <18mo (less active), staged if >18mo (more active, difficult to immobilize both)
1066
What are the general principles of finger syndactyly release?
- Do all releases before school age - If multiple digits, stage 1 side of finger at a time to avoid compromising vasculature - Release digits w significant length differences first - Release border digits first as big differences in growth rates (D4/5, thumb/D2) - Can release D3/4 later - Zigzag flaps, dorsal flap reconstructs webspace - Only absorbable sutures (less inflammation)
1067
Regarding syndactyly release, what is the most common surgical complication?
Web creep. Treatment: reconstruct webspace
1068
RC: What complication is associated w surgical treatment of syndactyly? A. Web creep B. Nerve injury C. Vascular injury D. Growth arrest
Answer: A (web creep)
1069
RC: A young patient presents w syndactyly. What is true? A. Complex syndactyly means that nerves are commonly involved B. Synonychia cannot be surgically corrected C. Complete syndactyly extends to the PIP joint D. Reconstruction is indicated to improve function
Answer: A
1070
What is Poland syndrome?
Congenital disorder associated w chest wall hypoplasia and upper extremity disorders
1071
What is the clinical presentation of Poland syndrome?
1. Chest hypoplasia or absence of: pecs, deltoid, serratus, lat dorsi 2. Sprengel's 3. Scoliosis 4. Dextrocardia 5. Radioulnar synostosis 6. Hand abnormalities: syndactyly, symbrachydactyly, symphalangism, etc
1072
What is Apert syndrome? What is the clinical presentation?
Mutated FGFR2 gene 1. Bilateral complex syndactyly of hands and feet. (Rosebud hands) 2. Symphalangism 3. Radioulnar synostosis 4. Craniosynostosis: premature cranial fusion causing flattened skull 5. Hypertelorism: increased distance between paired body parts (wide set eyes)
1073
What is finger polydactyly?
Finger duplication Preaxial: thumb Postaxial: 5th finger Central
1074
What is the Wassel classification?
Types of preaxial (thumb) polydactyly 1. Bifid distal phalanx 2. Duplicated distal phalanx 3. Bifid proximal phalanx 4. Duplicated proximal phalanx. Most common 5. Bifid metacarpal 6. Duplicated metacarpal 7. Triphalangia
1075
What conditions are associated w thumb polydactyly?
- Pollex abductus: connected EPL and FPL tendons - Wassell type 6 (duplicated MC) associated w Holt Oram, Fanconi
1076
What are the operative options and indications for the management of thumb polydactyly?
Type 1 combination: type 1+2 (bifid or duplicated distal phalanx). Combine into tissues 1 digit Type 2 combination: type 3 + 4 (bifid or duplicated proximal phalanx). Ablate lesser digit (radial one). Preserve the other Type 3 combination: types 5-7 (bifid/duplicated MC or triphalangia). One digit has better proximal component. Other has better distal component. Segmental distal transfer (on-top plasty)
1077
What is postaxial polydactyly? What are the types? What is the treatment?
Duplication of D5. 10x more common in Blacks. Autosomal dominant Type A: well formed digit. Treatment: type 2 combination. Preserve radial digit. Ablate ulnar digit Type B: rudimentary skin tag. Treatment: amputate rudimentary digit
1078
What is central finger polydactyly and how may it affect function? What is the treatment?
Duplication of central fingers. Associated w syndactyly, which may cause angular deformity and impair motion Treatment: osteotomy to prevent angular deformity
1079
What is finger macrodactyly? Which fingers are most commonly affected?
Congenital digit enlargement D3 > thumb > D4 > D5
1080
What conditions are associated w macrodactyly?
Maffucci Ollier
1081
What are treatment options and indications for the management of macrodactyly?
1. Observation: mild cases 2. Epiphysiodesis: single digit after reaching adult length of same sex parent 3. Osteotomies and shortening: thumb or multiple digits involved, severe deformity 4. Amputation: not reconstructible
1082
What is constrictive ring syndrome? Which fingers are most commonly affected?
AKA Streeter's dysplasia In utero, loose bands of ruptured amnion entangle fetus. Causes hand deformities: syndactyly, acrosyndactyly, amputation Most common: central digits
1083
What conditions are associated w constrictive ring syndrome?
1. Craniofacial defects, cleft palate 2. Cardiac defects 3. Clubfoot
1084
What is the Patterson classification? What is the treatment for each type?
Type of constrictive ring syndrome 1. Simple constriction ring. Mild indent from ring. No other deformity. Observation or release band if compromises circulation 2. Deformity distal to ring: hypoplasia or lymphedema. Tx: circumferential z-plasty 3. Acrosyndactyly or syndactyly: fusion distal to ring. Tx: syndactyly release 4. Amputation. Tx: reconstruction
1085
True of False: in kids, trigger thumb is more common than trigger finger
True. Thumb is 10x more affected However, it is still relatively uncommon
1086
What is pediatric trigger thumb?
Acquired. Mismatch between thickened FPL and A1 pulley. Disrupts tendon gliding Tendon sheath is normal
1087
What is the clinical presentation of pediatric trigger thumb?
- Fixed flexion contracture: thumb doesn't often actually trigger (JAAOS 2012) - Nodule at MCP (Notta nodule)
1088
What are treatment options for pediatric trigger thumb?
1. Passive extension exercises 2. Extension splinting 3. A1 release
1089
What are the indications for nonoperative management of pediatric trigger thumb? What are the outcomes of nonop?
Indication: flexible deformity 50% resolve before 2yo. Few resolve if older than 2yo Intermittent extension splinting is more successful than passive stretching alone
1090
What are the indications for operative management of pediatric trigger thumb? Describe the general principles of the technique
Indication: fixed deformity. >2yo (spontaneous recovery unlikely) Transverse incision over MCP flexion crease Divide A1 Directly visualize passive IP extension for smooth gliding
1091
What are anatomic causes of pediatric trigger finger? What are associated conditions?
1. Thickened FDS/FDP 2. FDP caught at FDS decussation 3. A1, A2, A3 constriction Associated w mucopolysaccharidoses (Hunter and Hurler) The tendon sheath is normal
1092
Describe the general principles of pediatric trigger finger surgical management. How does it differ from adult management?
Bruner incision over A1 Release A1 and resect 1 slip of FDS (usually ulnar) Assess gliding. May also need to release - Remaining FDS slip - A2 - A3 In adults, A1 release alone is sufficient
1093
What complication is associated w release of pediatric trigger thumb?
Injury to radial digital n of thumb
1094
RC: All of the following are true regarding congenital trigger thumb, except A. The thumb rarely triggers B. Tendon sheath is relatively normal C. It is also known as congenital clasp thumb D. Rarely bilateral
Answer: C This question is a bit of a mess but C is definitely wrong The thumb doesn't trigger, usually presents as fixed flexion deformity The FPL tendon is thickened. The sheath is normal It does show up bilaterally 25% of cases (JAAOS 2012) 2016
1095
What is congenital clasped thumb? What is the pathophysiology?
Congenital flexion/adduction deformity that persists beyond 3mo Due to deficiency of EPB (most common) or EPL or both
1096
What are risk factors for congenital clasped thumb?
1. Consanguinity 2. Family hx
1097
Describe the clinical presentation of congenital clasped thumb (4)
1. Persistent flexion/adduction after 3mo 2. 1st webspace contracture 3. Adductor pollicis contracture 4. 1st MCP joint instability
1098
What conditions are associated w congenital clasped thumb? (3)
1. Congenital vertical talus 2. Congenital clubfeet 3. Arthrogryposis
1099
What is the Tsuyuguchi classification?
Types of congenital clasped thumb 1. Supple clasped thumb. Can passively correct 2. Clasped thumb w contracture. Cannot passively correct 3. Rigid clasped thumb assoc w arthrogryposis
1100
What are the treatment options for congenital clasped thumb? (3)
1. Splinting and stretching 2. EIP to EPL tendon transfer 3. Thumb reconstruction
1101
What are the indications for serial splinting and stretching for congenital clasped thumb?
1st line treatment for all types. Start at 6mo Good results for Tsuyuguchi type 1 (supple thumb) if EPL or EPB present
1102
What are the indications for EIP to EPL tendon transfer for congenital clasped thumb?
Type 1 and 2
1103
What are the indications for thumb reconstruction for congenital clasped thumb? What are the general principles of the procedure?
Indications: type 2 and 3 (neither are passively correctable) - 1st webspace widening - 1st webspace deepening: release thenars and MCP capsule - FPL z-lengthening in forearm - EIP to EPL tendon transfer
1104
RC: Which of the following is not associated w the development of future OA in the hip? A. Cam deformity B. SCFE C. Pincer
Answer: C Pincer doesn't cause OA. CHECK study, Agricola 2013
1105
RC: Which of the following is not associated w an eye condition? A. Neurofibromatosis B. Achondroplasia C. Marfan D. Homocysteineuria
Answer: B. Achondroplasia NF: optic glioma, iris hamartomas (Lisch nodules) Marfan: superior lens dislocation Homocysteineuria: inferior lens dislocation
1106
What is not true regarding bisphosphonate use in kids? A. Good oral bioavailability B. Creates sclerotic lines below the physis C. Does not cause growth arrest D. Medication stays hidden in the bone for a long time after administration
Answer: A Oral bisphosphonates have poor bioavailability (<5% absorbed). Most are given IV Shows characteristic sclerotic lines It binds to bones and is released slowly. Half life up to 10yrs Does not cause growth arrest
1107
After what age is toe-walking considered pathologic?
2yo Before 2yo, toe walking may be part of normal gait development.
1108
What is normal ankle ROM arc?
20deg dorsiflexion 40deg plantarflexion
1109
Describe the 3 rockers of ankle motion during gait
1. Heel strike and then ankle plantarflexes to lower foot. Eccentric tib ant contraction 2. Ankle at relative dorsiflexion as tibia moves forward over foot. Eccentric gastroc-soleus contraction 3. Push-off. Ankle plantarflexes. Concentric gastroc-soleus contraction
1110
How are the 3 rockers of ankle motion affected during toe walking?
Absent 1st rocker Increased plantarflexion during 3rd rocker
1111
What is the DDx of idiopathic toe walking? (9)
1. CP 2. Muscular dystrophy 3. Neuroaxis abnormalities: tethered cord, diastematomyelia, spina bifida 4. Autism 5. Schizophrenia 6. Global developmental delay 7. Charcot-Marie-Tooth 8. Ankylosing spondylitis 9. LLD
1112
True or False: many idiopathic toe walkers have normal dorsiflexion
TRUE
1113
What are options for nonoperative management of idiopathic toe walking? Which options is superior?
1. Observation: kids <2yo 2. Physiotherapy 3. Braces, night splints, walking cast 4. Botox and serial casting Variable outcomes in studies. We don't know yet which option is best
1114
What is the operative management of idiopathic toe walking? What are the indications?
Gastroc-soleus release: recession vs TAL. Level depends on Silfverskiold test Indications: fixed equinus contracture or failed nonop
1115
What is the risk of TAL for the management idiopathic toe walking that is less of a concern w gastroc recession?
TAL risks overcorrection. May weaken plantarflexors and cause calcaneal gait
1116
RC: All of the following are associated w idiopathic toe walking except: A. Decreased passive dorsiflexion B. Increased tone in upper extremities C. Autism D. Learning disability
Answer: B Dorsiflexion may be limited, but can also be normal in many Autism and developmental delay are associated Increased tone in the upper extremities may be due to a neuromuscular cause (like CP). CP may cause toe walking and so this would not be idiopathic
1117
RC: 4yo presents w toe walking, which is true? A. Thought to be autosomal dominant inheritance B. Most are due to neurologic etiology C. Usually resolve without treatment
Answer: A? Most are truly idiopathic. They do not resolve without treatment. Some studies suggest autosomal dominant inheritance 2017
1118
What is the most common location for a knee OCD lesion?
Posterolateral aspect of the medial femoral condyle
1119
RC: List 8 risk factors that would make a patient more prone to receive a blood transfusion in the context of pediatric spine surgery
1. Low preop hgb 2. Coagulopathy 3. Antiseizure medications (valproic acid) 4. Neuromuscular scoliosis: most important 5. Larger deformity 6. Long OR time 7. Multiple osteotomies 8. More levels involved
1120
RC: What is the inheritance pattern of duchenne muscular dystrophy? What protein is involved?
X-linked recessive. Dystrophin gene
1121
RC: What is the deformity of a typical clubfoot? A. Pronation, forefoot adduction, hindfoot varus, plantarflexed B. Pronation, forefoot adduction, hindfoot valgus, plantarflexed C. Pronation, forefoot abduction, hindfoot varus, dorsiflexed D. Pronation, forefoot adduction, hindfoot valgus, dorsiflexed
Answer: A. Pronation. Forefoot adduction. Hindfoot varus. Plantarflexed
1122
RC: What is not correct w respect to pediatric avulsion fractures? A. The majority of ASIS injuries can be managed nonoperative B. MRI is necessary to diagnose all of these injuries C. Tibial tubercle fractures usually need operative management D. The apophyseal region is the weakest
Answer: B. CT/MRI is only required in 2% of these injuries Most can be diagnosed via XR ASIS avulsion #s: nonop Tib tubercle #s do need OR depending on displacement and type Apophysis is indeed the weakest region

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